DIT- Things to know COPY Flashcards

1
Q
Loss of pain and temp on C/L body
Loss of pain and temp on ipsl face
Hoarseness
Difficulty swallowing
Loss of gag reflex
Vertigo
Nystagmus
N/V
A

Lateral Medullary syndrome (Wallenberg)

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2
Q

Lateral Medullary syndrome (Wallenberg) (6)

A

Spinothalamic tract damage
- Loss of pain and temp sensation over c/l body

Spinal Trigeminal nucleus damage
- Loss of pain and temp sensation over ipsl face

CN IX and X damage
- Hoarseness, difficulty swallow, loss of gag reflex

Descending sympathetic tract
- Ipsilateral Horner syndrome

Vestibular nuclei damage
- Vertigo, nystagmus, N/V

Inferior cerebellar peduncle damage
- Ipsilateral cerebellar deficits
(ataxia, past pointing)

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3
Q

Vessel occluded in stroke of lateral medulla

A

PICA

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4
Q

Internuclear opthalmoplegia

A

Medial longitudinal fasciculus

Eyes dont track together

Medial rectus palsy (right)
Left eye: horizontal nystagmus

Abducting eye: nystagmus
Adducting eye: unable to adduct

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5
Q

Locked in syndrome

A

Can only move eyes

basilar A. stroke affects both sides of superior pons

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6
Q
Dysphagia
Dysphonia
Dysarthria
C/L spastic hemiparesis
Ipsl ptosis
Pupillary dilation
Lateral strabismus (down and out)
A

Weber syndrome

Anterior midbrain infarction resulting from occlusion of paramedian branches of the posterior cerebral artery

Oculomotor nerve
Cerebral peduncle lesion

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7
Q

Lumbar puncture layers (9)

A
  1. Skin/ superficial fascia
  2. Supraspinous lig
  3. Interspinous lig
  4. Ligamentum Flavum
  5. Epidural space
  6. Dura mater
  7. Subdural space
  8. Arachnoid membrane
  9. Subarachnoid space
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8
Q

Last place blood from brain travels before exiting cranium through jugular foramen

-becomes

A

Sigmoid sinus

Internal jugular vein

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9
Q

Carotid sheath contains (3)

A

Internal jugular v.
Common carotid A.
Vagus n.

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10
Q

Clinical features of normal pressure hydrocephalus

A

Wet wacky wobbly

Urinary incontinence
Dementia
Gait disturbances

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11
Q

Magnetic gait

A

Normal pressure hydrocephalus

Wet wacky wobbly

Urinary incontinence
Dementia
Gait disturbances

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12
Q

Fetal component that secretes hCG

A

Synctiotrophoblast

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13
Q

Maternal component of the placenta

A

Decidua basalis

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14
Q

Migraines are made worse by foods that contain

A

Tyramine

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15
Q

Jaw muscle pain when chewing

A

Giant cell arteritis

Temporal arteritis

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16
Q

Pt using topical retinoic acid for acne

A

Vit A toxicity –>

Idiopathic intracranial HTN

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17
Q

Elevated ESR headache

A

Giant cell arteritis

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18
Q

Headache + Extraocular muscular palsies

A

Cavernous sinus thrombosis

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19
Q

Scintillating scotomata prior to HA

A

Migraine

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20
Q

Stages that an embryo goes through between conception and development of inner cell mass

A

Zygote –> Morula –> blastocyst

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21
Q

Adult brain tumor pneumonic

A

MGM studios

Metastasis
Glioblastoma
Meningioma
Schwannoma

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22
Q

Pediatric brain tumor pneumonic

A

Animal kingdom
Magic kingdom
EpCot

Prepubescent minds eaten with cancer

Pilocytic astrocytoma
Medulloblastoma
Ependymoma
Craniopharyngioma

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23
Q
Rapidly progressive primary brain tumor
Irregular necrotic center
Surrounded by edema
GFAP stain
Pseudopalasating pattern "snake like"
A

Glioblastoma

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24
Q

Slow growing primary brain tumor
“Whorled”
Surface of brain
Psammoma bodies

  • originates
  • risk
A

Meningioma

Arachnoid cells

Risk

  • Radiation
  • Neurofibromatous type 2
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25
Benign brain tumor S-100 Tennitus, hearing loss Vertigo, unsteady gait -assoc with
Schwannoma Bilateral assoc w/ NF2
26
Fried egg brain tumor
Oligodendroglioma
27
Slow growing brain tumor Frontal lobe Perinuclear cytoplasmic clearing
Oligodendroglioma
28
Hypogonadism or acromegaly | Bitemporal hemianopia
Pituitary adenoma
29
Things that have fried egg appearance
Oligodendroglioma HPV Seminoma
30
Two brain tumors associated with NF-2
Meningioma | Schwannoma
31
Posterior fossa brain tumor GFAP Rosenthal fibers
Pilocytic astrocytoma Rosenthal fibers: eosinophilic corkscrew fibers
32
Rathke's pouch Compresses optic chiasm Supratentorial tumor of childhood
Craniopharyngioma
33
Malignant brain tumor Cerebellar Compress 4th ventricle Homer-wright rosettes
Medulloblastoma Cells circle around tangle of fibers
34
Compress 4th ventricle | Peri-vascular pseudo-rosettes
Ependymona Circular arrangement of cell around vessel
35
Eosinophilic corkscrew fibers
Rosenthal fibers Pilocytic astrocytoma
36
Which cranial nerve relays the following information 1) Hypoxia measure by carotid body 2) Motor information on swallowing 3) BP from aortic arch 4) Salivation from the sublingual glands 5) Salivation from the parotid gland 6) BP from carotid body
1) CN IX 2) IX, X 3) X 4) VII 5) IX 6) IX
37
Which type of collagen abnormal in alport syndrome Alport syndrome
Type IV collagen BM Nephritis and kidney failure Hearing loss Eye problems (cataracts) Can see, cant pee, cant hear high C
38
Which type of collagen abnormal in Classic Ehler Danlos syn Which type in Vascular EDS
Type V and I (Type 1: bone) Type III Type (III: stretchy, blood vessles, uterus)
39
Collagen synthesis 3*
Preprocollagen alpha chains synthesized in rough ER of fibroblast ( glycine-proline- X) or (glycine-x-hydroproline) Hydroxylation of lysine and proline (requires Vit C) Glycosylation of the hydroxylated lysin --> procollagen Exocytosis Cleave terminal regions of procollagen --> Tropocollagen (proteolysis) Crosslink tropocollagen to make collagen fibrils (covalent bond)
40
What is seen in early onset Alz dis vs late onset
Early onset - Presenilin 1 - presenilin 2 - amyloid precursor protein (APP) chr 21 Late onset - Apo E4
41
Dementia w/ Lewy body inclusion
Pinkish eosinophilic cytoplasmic inclusions, made up of protein called alpha-synuclein
42
Inclusions of Tau proteins Atrophy of frontal and temporal lobe Dementia and behavioral changes Dementia and progressive aphagia (inability to understand)
Pick's dis Frontotemporal dementia
43
``` Rapidly progressive dementia Personality changes Muscle spasm Myoclonus Weeks to months ```
Creutzfeldt-Jakob disease Prions aka spongiform encephalopathy
44
Dementia plus 1) Visual hallucinations 2) Progressive aphasia 3) Ataxia & loss pupillary light reflex 4) Megaloblastic anemia and peripheral neuropathy 5) Resting tremor and bradykinesia 6) Uninhibited social behavior 7) Urinary incontinence and magnetic gait 8) Syncopal episodes 9) Dysarthria and liver dis 10) Myoclonus
1) Lewy body dis 2) FTD (Pick) 3) Tertiary syphilis 4) B12 5) LBD or Parkinsons dis 6) FTD (Pick) 7) Normal pressure hydrocephalus 8) Lewy body dis 9) Wilson dis (copper build up) 10) Creutzfeltd-Jakob dis
45
Identify pigment inclusion 1) Herpes simplex 2) Rabies 3) Parkinson dis 4) Assoc w/ aging 5) Dark pigment in substantia nigra and locus coeruleus, not seen in parkinson dis 6) Eosinophilic, rod like seen in hippocampus of Alz pts 7) Diagnostic of Alz dis 8) Filamentous inclusion that stain w/ silver, do not survival neuronal death 9) Filamentous inclusion that stain w/ PAS and ubiquitin
1. Cowdry Type A 2. Negri bodies 3. Lewy body 4. Lipofuscin granules 5. Melanin 6. Hirano bodies 7. Neurofibrillary plaques and tangles 8. Pick bodies 9. Lewy body
46
Dementia work up (5)
``` RPR- syphilis HIV B12 TSH MRI ```
47
Dominant parietal lobe lesion (4) Non-dominant parietal lobe lesion
Gerstman syndrome - agraphia - acalculial - Finger agnosia (inability to distinguish fingers) - L to R disorientation Hemispatial neglet (Ignore c/l side of body)
48
Cholinergic excess pneumonic
DUMBBELSS ``` Diarrhea Urination Miosis Bronchospasm Bradycardia Excitation of skeletal m./ CNS Lacrimination Salivation Sweating ```
49
Ptosis Diplopia Worsens as day goes on
Myasthenia gravis Ab to acetycholine receptor Thymus pathology
50
Nicotinic receptor and Muscarinic receptor are what type of receptor
Nicotonic: Ligand gated Na/K channels Muscarinic: G protein coupled
51
Anxiety is assoc with altered levels of neurotransmitters
Decrease GABA Decrease Serotonin Increase NE
52
Uvela deviates to right, damage to what 2 things
Left vagus n. | Left nucleus ambiguus
53
What beside alpha feto protein is abnormal in fetus with neural tube defect
Increase acetylcholinesterase (amniotic)
54
Patient can not adduct left eye on lateral gaze, convergence is normal What is damaged
Intranuclear opthalmoplegia Medial longitudinal fasciculus
55
Pharyngeal pouches from
Endoderm
56
Sympathetic receptor on vasculature of skeletal m. on heart on lungs
vasculature of skeletal m.: Alpha 1 Beta2 (more) Heart: Beta 1 Lungs: Beta 2
57
alpha 1 alpha 2 Beta 1 Beta 2
Alpha 1: vascular smooth m. contraction - increase resistance and BP - bladder sphincter contraction Alpha 2: Inhibit NE release Beta 1: Heart - Tachycardia - increase contractility - release renin Beta 2: Lungs - Vasodilation - bronchodilation - insulin release - decrease uterine tone
58
Pharyngeal pouches structures
1st pouch: mastoid air cells, middle ear cavity, eustacian tubes 2nd pouch: lining of palatine tonsils 3rd pouch: thymus, inferior parathyroid glands 4th pouch: superior parathyroid glands
59
Structures in cavernous sinus
``` Oculomotor Trochlear n. Abducens Opthalmic div V1 Maxillary div V2 ``` Internal carotid A.
60
Catecholamine synthesis
Phenylalanine (BH4 -> BH2) --> Tyrosine [Phenylalanine hydroxylase] Tyrosine (BH4--> BH2) --> DOPA [Tyrosine hydroxylase) DOPA (Vit B6) --> Dopamine [DOPA decarboxylase] Dopamine (Vit C) --> NE [Dopamine beta-hydroxylase] NE --> EPI [PNMT] (+) Cortisol
61
Cofactors in metabolism of NE
Catechol-o-methyltransferase (COMT) - methylates NE ``` Monoamine oxidase (MAO) - oxidized NE ```
62
Damage to which artery would produce each 1) Bilateral loss of lateral visual fields 2) Broca or wernicke aphasia 3) Unilateral lower extremity sensory and/or motor loss 4) Unilateral facial and upper extremity sensory and motor loss
1) Anterior communicating A. 2) Middle cerebral A. 3) Anterior cerebral A. 4) Middle cerebral A.
63
Diuretic used for idiopathic intracranial HTN
Acetazolamide
64
When does implantation of blastocyst take place
6 days after fertilization
65
Therapeutic index (TI)
LD50/ED50 Lethal dose/ effictive dose Higher the TI safer the drug
66
Low therapeutic index drugs
Some Drugs With Low Therapeutic index ``` Seizure drugs Digoxin Warfarin Lithium Theophylline ```
67
Which regions of the brain compose the limbic system
``` Amygdala Septal nucleus Mamillary bodies Fornix Hippocampus ```
68
What structure grows to close the opening/ canal between the atrial chamber and ventricular chamber into two smaller openings
Superior endocardial cushion Inferior endocardial cushion
69
Aortic arches
Most likely to be test on 3rd and 4th 1st - Part of maxillary A. 2nd - stapedial A. - hyoid A. 3rd - common carotid a. - proximal part of internal carotid A. 4th - Left: arch of adult aorta - Right: Proxiaml part of right subclavian a. 6th - proximal part of pulmonary A. - Ductus arteriosus
70
R to L shunts
1. Persistant Truncus arteriosus 2. Transposition of the great vessels (2 vessels) 3. Tricuspid atresia 4. Tetralogy of Fallot 5. Total anamalous pulmonary venous return
71
Ebstein anomaly
Tricupsid leaflets are displaced into right ventricle Hypoplastic right ventricle Lithium use Widely split S2
72
Tetralogy of Fallot
IHOP Interventriculat septal defect (USD) Hypertrophy of RV Overriding aorta Pulmonic stenosis
73
Boot shaped heart in infant
Tetralogy of fallot
74
Maternal alcohol use can lead to heart defects
Tetralogy of fallot VSD ASD
75
Pregestational diabetes possible heart defect
Transposition of great vessels
76
Congenital Rubella possible heart defect
PDA | Pulmonary artery stenosis
77
DiGeorge syndrome possibe heart defect
Tetralogy of fallot | Truncus arteriosus
78
Turner syndrome possible heart defect
Coarctation of the aorta | Bicuspid aortic valve
79
Trisomy 21 possible heart defect
ASD or VSD
80
Most common congenital cardiac anomaly
VSD
81
Most common congenital cause of early cyanosis
Tetralogy of Fallot
82
Rib notching heart defect
Coarctation of aorta
83
Enzymes involved in catabolism of NE
``` Catechol-o-methyltransferase (COMT) Monoamine oxidase (MAO) ```
84
Cardiac output is equal to
Stroke vol (SV) x HR
85
Stroke vol is equal to
End-diastolic vol minus end-systolic vol
86
Fick Principle
CO= Rate of O2 consumption/ arterial O2 content- venous O2 content
87
Mean arterial pressure (P) =
MAP = Cardiac output (Q) x Peripheral resistance (R) P= QxR MAP= 2/3 diastolic pressure + 1/3 systolic pressure
88
Pulse pressure =
systolic pressure - diastolic pressure The higher the stroke volume the higher the pulse pressure
89
Digoxin does what
Increase contractility
90
Ejection fraction (EF) =
SV/ EDV
91
CHF does what to capillary hydrostatic pressure (Pc)
Increases it
92
Liver disease does what to starling forces
Decreases plasma colloid osmotic pressure
93
What increased interstitial colloid osmotic pressure
Lymphatic obstruction
94
Affect on starling forces 1. HF 2. Liver failure 3. Oliguric renal failure 4. Infections 5. Nephrotic syndrome 6. Lymphatic blockage 7. Burns 8. Diuretic administration 9. IV infusion of albumin 10. Venous insufficiency
1. HF= Increase Pc 2. Liver failure= Decrease TTc 3. Oliguric renal failure= Increase Pc 4. Infections= Increase Kf 5. Nephrotic syndrome= Decrease TTc 6. Lymphatic blockage= Increase TTi 7. Burns= Increase Kf 8. Diuretic administration= Decrease Pc 9. IV infusion of albumin= Increase TTc 10. Venous insufficiency= Increase Pc
95
Drug causes eye dilation
Anticholinergic | Atropine
96
Wide splitting of S2
Wider on inspiration Pulmonic stenosis RBBB
97
Fixed splitting of S2
Split on both inspiration and expiration equally atrial septal defect
98
Paradoxical splitting of S2
Split on expiration Aortic stenosis LBBB
99
Which heart sound is associated with dilated congestive heart failure
S3 Mitral regurg L To R shunt
100
Which heart sound is associated with chronic HTN
S4 Hypertrophic cardiomyopathy Aortic stenosis LV hypertrophy Post MI
101
Inspiration makes what murmur louder
Tricuspid
102
What murmur is louder with expiration
Mitral
103
Hang Grip makes what murmur louder
Most quieter except Hypertrophic cardiomyopathy
104
Bounding pulses, head bobbing, diastolic murmur
Aortic regurg
105
Underlying mechanism of organophosphate poisoning
Inhibit acetylcholinesterase
106
MOA of N-acetylcysteine for acetaminophen overdose
Regenerates glutathione
107
Equation for 1/2 life
T1/2= (0.7 x VD)/ CL
108
What two antibodies most useful in diagnosing rheumatoid arthritis
Rheumatoid factor | Anti-citrullinated protein Ab
109
How do NSAIDS cause renal disease
Block prostaglandin Syn | Constrict renal vessels
110
Epidermis pneumonic
Come Lets Get Sun Burnt ``` Corneum Lucideum Granulosum Spinosum (only palms and soles) Basale ```
111
Components of Zona Occludens
TIght junction Claudins Occludin
112
Components of Zona adherens
Bridge to skeleton | E-cadherin actin
113
Components of Macula adherens
Desmosomes, resist shearing forces Simple/ stratified squamous epithelium Desmoglein
114
Components of Gap junctions
Cardiac cells Connexon
115
Components of Hemidesmosomes
Connect to BM Integrins
116
Auer rods
AML 15;17
117
15;17
AML
118
8;14
Burkitt lymphoma Starry sky Basophilic cytopasms containing prominenet lipid vacuoles
119
9;22
CML Blood smear shows leukocytosis with many immature myeloid cells (horseshoe nucleus)
120
12;21
B cell- ALL Anemia Thrombocytopenia Lymphoblasts
121
14;18
Follicular lymphoma
122
Firm flesh color papules on face with umbilicated center is
Molluscum contagiosum from Pox virus See Molluscum bodies: eosinophilic cytoplasmic inclusions
123
Ballooning multinucleated giant cells
Herpes simplex Enlarged cells w/ intranuclear inclusions, cell fusion produc giant cells
124
Basaloid cells with peripheral palisading nuclei
Basal cell carcinoma Slow growing papules, ulceration or rolled border
125
Cells with enlarged nuclei and perinuclear halos
Veruca vulgaris due to HPV
126
Encapsulated round yeast forms
Cryptococcus neoformans cerebrospinal fluid shows budding encapsulated yeast
127
What is at risk with an anterior/ posterior knee displaccement
Popliteal artery
128
What is commonly damaged by blunt trauma of lateral knee, vessel
Common peroneal nerve
129
Concentrations of fluids within - Ileum - Pancreas - Salivary gland - Sweat gland
Ileum - Alkaline - bicarb > Cl - increase K Pancreas - bicarb and chloride change with flow rate - high flow: high bicarb low Cl - low flow: low bicarb high Cl - fixed Na and K concentration Salivary gland - isotonic solution - Lower [Na] [Cl] - Higher [K] Eccrine glands - hypotonic - composed of sodium chloride, some potassium - minimal bicarb
130
Acute stress disorder become PTSD
> 1 month
131
Cerebellar degeneration vs cobalamin deficiency
Cerebellar degeneration - tremors - gait impairment Cobalamin deficiency - Decreased vibration sensation - Gait impairment - Positive Babinski - Hyperactive/ hypo reflexes
132
Difficulty chewing Double vision Ptosis Associated with
Myasthenia gravis Associated with thymic hyperplasia
133
Loss of heterozygosity
Inherits one mutation and must develop the second one for tumor to form
134
Defective DNA repair
Xeroderma pigmentosum
135
Parental imprinting
Prader-Willi | Angelmann syndromes
136
Blue green image
potassium hydroxide (KOH) mount Fungi
137
Low CD3 | Normal B cells
Di George
138
Allelic heterogeneity
Multiple genes same phenotype
139
Hereditary hemochromatosis
AR Excessive absorption of iron ``` Hyperpigmentation (bronze diabetes) Arthalgia, arthropathy Hepatomegaly DM Progressive weakening Dry mouth Hypogonadism (small testes) ```
140
COL1A1 mutation
Osteogenesis imperfecta Type I collagen Short stature Blue sclerae
141
Infant suffering from recurrent infections, abnormal gene for cytoplasmic tyrosine kinase
Bruton aggammaglobulinemia Bruton's tyrosine kinase (BTK) X linked
142
Genetic mutations chromosome 7
Cystic fibrosis Ehlers Danlos Osteogenesis imperfecta
143
Genetic mutations chromosome 16
Polycystic kidney disease | Tuberous sclerosis
144
Increase in branched chain alpha-keto acid What can also be deficent
Maple syrup urine disease Alpha ketoacid dehydrogenase require thaimine pyrophosphate (Vit B1) as coenzyme
145
Pyridoxine order to go with
Isoniazid
146
Previous MI Crampy abdominal pain Blood in stool Elderly
Ischemic colitis Mucosal hemorrhage and patchy areas of necrosis
147
Not eating for days Low ketones Vomiting and lethargy
Medium-chain acyl-CoA dehydrogenase deficiency Hypoketotic hypoglycemia
148
Glucose 6 phosphatase deficiency
Von Gierke disease
149
Glycogen phosphorylase deficiency
McArdle
150
Warfarin with CYP 450 inducer leads to what Vmax and KM
Warfarin metabolism increased | Increased V max
151
Chromogranin
Carcinoid tumor Secretes serotonin
152
Diabetes medications 1. Acarbose 2. Canagliflozin 3. Glyburide 4. Pioglitazone 5. Repaglinide
Acarbose -intestinal brush border alpha glucosidase inhibitor decreases postprandial hyperglycemia by reducing GI absorption of glucose Canagliflozin - sodium glucose cotransporter 2 inhibitors, act on kidney to decrease reabsorption of filtered glucose Glyburide (sulfonylureas) and Meglitinides (repaglinide) - bind to and close ATP sensitive K channels in pancreatic beta membrane - depolarize and open L type Ca channel - Increase Ca influx, insulin release Pioglitazone (Thiazolidinediones) peroxisome proliferator-activated receptor gamma (PPAR-y) - decrease insulin resistance - expression glut 4 - adiponectin
153
Celiac disease location
Distal duodenum | Proximal jejunum
154
Chronic diarrhea Drainage of abscess Erythema near perineum
Crohns terminal ileum
155
Abnormal formation of lamellar bone is seen in
Paget disease of bone
156
Autosomal dominant cancer mutations disease
``` Lynch syndrome Familial adenomatous polyposis VHL Li-Fraumeni MEN1/2 ```
157
Laryngeal nerves | -Internal laryngeal supplies
Internal laryngeal: Supraglottic External laryngeal: cricothyroid L. recurrent laryngeal: posterior cricoarytenoid, lateral cricoarythenoid, oblique arytenoid, transverse arytenoid, thyroarytenoid
158
DNA virus
Papillomaviruses | Poxviruses (replicates in cytosol)
159
Acute onset anemia Evidence of hemolysis (icterus, elevated reticulocyte count) Tx w/ TMP-SMX
G6pD deficiency Erythrocyte enzyme deficiency
160
Unmyelinated nerve fibers
Post ganglionic autonomic nervous system axons
161
Positive sense ssRNA virus 1) Meningitis 2) common cold
Picornavirus Coronoavirus- cold
162
negative sense ssRNA virus
Arenavirus- hemorrhagic fever
163
Prader Willi syndrome
Loss of paternally inherited allele ``` Tantrums Excessive food seeking Obesity Small hands Short stature Hypogonadism Intellectual disability ```
164
Tantrums Excessive food seeking behavior Obesity Small hands
Prader Willi syndrome Loss of paternally inherited allele
165
Angelmann syndrome
Loss of maternal allele Epilepsy/ seizures Ataxic gait Innappropraite laughter/ smiling
166
Cobblestone mucosa
Crohns disease
167
Aggressive biting fingers, tongue and lips
Hypoxanthine-guanine phosphoribosyltransferase deficiency (HGPRT) Lesch-Nyhan syndrome X linked recessive
168
Stroke blindness
Homonymous hemianopsia
169
acanthosis nigrcans associated iwth
velvety hyper pigmented plaques on the skin in axillae and neck insulin resistance GI malignancy
170
Cystic fibrosis type of mutation
Frame shift
171
Hyperflexible joints | Stretchy skin
Ehlers danlos Collagen defect
172
Drug A. Absolute risk reduction for drug A versus standard therapy 4%. The incidence of recurrent PE in standard therapy group was 6%. There were 24 pts who developed recurrent PE in drug A group. How many total subjects in drug A group?
ARR= ER control- ER treatment (Event rate) 0.4= 0.6- x ER treatment= 0.2 ER treatment= Number of events/ number of subjects 0.2= 24/ number of subjects =1200
173
IFN-y fxn
activates macrophages | promotes TH1 differentiation
174
IL-4
produced by TH2 | activates B cells
175
Stimulates neutrophil migration to sites of inflammation
Leukotriene B4
176
Acute pancreatitis is due to activation of waht
Trypsin
177
Elastin composition
Nonpolar aminoacids Not hydroxylated Lysyl oxidase copper-dep enzyme oxidatively deaminates lysine residues of tropoelastin, facilitating desmosine cross links [Collagen synthesis - Disulfide bridges - post translational hydroxylation - triple helix assembly]
178
Reverse T3 (rT3)
inactive form of T3 generated by entirely from the peripheral conversion of T4
179
Diphtheria toxin and Exotoxin A (pseudomonas)
Inactivates EF-2 vis ribosylation, thus inhibiting host cell rpotein synthesis
180
Tc-pertechnetate scan
Meckel diverticulum Failed obliterationo fthe vitelline duct
181
Promotor regions -disrupted
CAAT TATA - Prevent initiation of transcription
182
Palpitations Tachycardia Irregularly irregular rhythm
Atrial fibrillation No P waves
183
Neutralizes reactive oxygen species
Superoxide dismutase
184
Kidney biopsy | Green straight lines
Anti-glomerular basement membrane disease (Goodpasture dis) Collagen IV Seen glomerular crescents
185
Origin of melanoma
Neural crest
186
Receptor of T cells to identify tumor cells and induce apoptosis
Programmed death ligand 1 (PD-1)
187
Yellow nodules on palmar creases. Father has MI at 56. Accumulation of lipid laden macrophages Lack of ApoE3 and ApoE4. What is impaired?
Familial dysbetalipoproteinemia AR -defect in ApoE Impaired chylomicron remnant uptake by liver cells Accumulation cholestesrol and triglyceride levels
188
Hyperlipoproteinemias types
Type I (AR) - Familial chylomicroenemia - Defect: Lipoprotein lipase - Defect: ApoC-2 production - Elevated chylomicrons - Acute pancreatitits*, Lipemia retinalis, eruptive xanthomas ``` Type IIA - Familial hypercholesterolemia - Defect: LDL receptor - Defect: ApoB-100 - Elevated LDL* (Impaired LDL particle uptake by hepatocytes) -Premature atherosclerosis* - Tendon xanthomas, xanthelasmas (yellow deposit around eyes) ``` Type III - Familial dysbetalipoproteinemia - Defect ApoE - Elevated: chylomicron & VLDL remnants - Premature atherosclerosis* - Tuboeruptive and palmar xanthomas Type IV - Familial hypertriglyceridemia - Defect Polygenic - Elevated VLDL - Associated with coronary disease, pancreatitis and diabets
189
Defect in Fibrillin-1
Marfan ``` Microfibrils Blood vessels (aorta) Long thin extremities long fingers Aortic root dilation ```
190
Vit D overdose similar to what condition
Sarcoidosis, Tb Have PTH independent conversion due to expression of 1-alpha-hydroxylse in activated macrophages
191
Oculomotor (CN III) palsy secondary to a compressive aneurysm located where
Posterior communicating artery
192
Bronchiolitis
Rhinorrhea and congestion Followed by cough, low grade fever and increased work of breathing
193
Repetitive vomiting leads to what acid-base disturbance
Metabolic alkalosis
194
Conjunctival injection
Red eyes
195
Glucagon mediator responsible for decrease glycogen stores adn glucose
Gs Adenylate cyclase Protein kinase A
196
Insulin resistance leads to increase in waht
Free fatty acid level
197
How to measure mean arterial pressure
placing an arterial catheter directly in central arterial circulation (common femoral or radial artery) and reflects the average aortic pressure
198
Measuring pressure in pulmonary artery is also measuring pressure where?
Left atrial pressure
199
Ammonium and bicarbonate generated from AA Urea and ornithine produced from pyruvate produced from AA oxaloacetate produced from AA
glutamine arginine Alanine aspartate
200
Lymphatic drainage Testis glans penis Upper 2/3 vagina, uterus, cervix, prostate Lower 1/2 vagina, vulva, scrotum
Testis: para-aortic Glans penis: deep inguinal nodes Upper 2/3 vagina, uterus, cervix, prostate: External iliac nodes, internal iliac nodes Lower 1/2 vagina, vulva, Scrotom: superficial inguinal nodes
201
Broad, nonseptate hyphae that branch at wide 90 angles
Mucor Rhizopus Absidia
202
Immediate hypersensitivity txn
Type 1 Cell surface bound antibody bridging by antigen
203
Three primary ketones
Acetoacetate Beta-hydroxybutyrate (BHB) Beta-hydroxybutyric acid
204
Septic aborption organisms
Staph aureus Ecoli Group B strep
205
Intellectual disability Hx of seizures Abnormal pallor of substantia nigra, locus ceruleus and vagal nucleus dorsalia
Phenylalanine hydroxylase deficiency PKU Cant convert phenylalaine to tyrosine
206
Tennis racquet granules
Langerhan cells
207
9 month old 2 minute general seizures Fever 3 days later afebrile Maculopapular rash on trunk
Human herpesvirus 6
208
Exertional dyspnea Easy fatigue Gait unstable when eyes closed Impaired vibratory sensation of lower extremiteis Marked pallor of conjunctivae, nail beds and palms
Vit B 12 deficency Co factor for methylmalonyl-CoA Elevated Serum methylmalonic acid and homocysteine
209
Increased intracranial pressure in infant
Intracranial hemorrhage due to vit K deficiency Impaired clotting factor carboxylation
210
Acute compartment syndrome (ACS) affects what in the leg
Foot extensors Anterior tibial A. Deep peroneal (Fibular) nerve
211
Pleiotrophy
One mutation multiple things wrong w/ person
212
Power of the study?
1- beta beta= type II error, probability to conducting there is no difference
213
MHC to CD8 composed of
``` MHC class I beta microglobulin ```
214
MCH to CD4 composed of
MHC class II alpha and beta chains
215
Hepatitis B C D vs A E
BCD transmitter parenterally (IV, blood) AE: fecal oral
216
Hepatitis pregnant women
Hep E unenveloped ssRNA
217
Takes place in cytosol vs mitochondria
Cytosol - Glycolysis - FA synthesis - Pentose phosphate pathway Mitochondria - beta oxidation of fatty acids - citric acid cycle carboxylation of pyruvate (gluconeogenesis)
218
Interferon alpha and beta do what
halt protein synthesis Only works on dsDNA
219
Sorbitol conversion
Sorbitol -- sorbitol dehydrogenease --> fructose
220
Start codon
AUG
221
Stop codon
UAA UAG UGA
222
OCD tx
SSRI
223
``` Fever myalgia Profound fatigue Swollen LN in neck and behind ears Lymphocytosis Splenomegaly ```
EBV transferred vis kissing
224
Splenomegaly South asia Anemia thrombocytopenia
Malaria
225
Splenomegaly South asia Progressive splenic enlargement WL over months
Leishmaniasis
226
scabes tx
topical permethrin
227
Tx for staph aureus cellulitis
Topical mupirocin
228
Anemia Hypercoagulability Pancytopenia
Paroxysmal nocturnal hemoglobinuria (PNH) Mutation PIGA Deficiency: glycosylphosphatidylinositol (GPI) anchor Deficiency: CD55 CD59
229
Positive VDRL w/ vulvar lesion
Tertiarty syphilis Gumma
230
Tx Manic depression
Valproic acid Lithium Carbamazepine
231
Tx w/ antibiotic Proteinuria Hematuria
Acute poststreptococcal glomerulonephritis
232
HTN Hematuria Proteinuria
Nephritic syndrome
233
Breast dimpling signals involvement of
Suspensory ligaments
234
HTN HA Adrenal mass
Pheochromocytomas From Neural crest
235
Narcolepsy caused by
hypocretin-1 | hypocretin-2
236
Elevated 14-3-3 protein CSF
Creutz-jakob Prions
237
Temporal lobe encephalitis
Herpes simplex virus Tx acyclovir - effects viral DNA polymerase
238
Meningitis w/ increased protein and normal glucose
Viral
239
Meningitis w/ low glucose and elevated protein
Bacterial
240
CHF leads to increase in what force
Capillary hydrostatic pressure (Pc)
241
Replicative process 1) DS dna --> DS dna template --> ds DNA progeny 2) DS dna -> +RNA template -> partially ds-DNA 3) ssDNA --> ds DNA template --> ss DNA progeny 4) ss +RNA --> ds DNA template --> ss +RNA progeny 5) ss +RNA --> - RNA template --> ss + RNA progeny 6) ss -RNA --> +RNA template --> ss -RNA progeny
1) Adenovius, herpesvirus, poxvirus 2) Hepatitis virus 3) Parvovirus B19 4) Retroviruses (HIV) 5) poliovirus 6) Influenza virua, measles virus, rabies virus
242
Metastatic bone pneumonic
Permanently Relocated Tumors That Like Bones ``` Prostate Renal cell cancer Testes Thyroid Lung Breast ```
243
Osteopetrosis due to deficient
Carbonic anhydrase II
244
Polyostotic Fibrous dysplasia associated with
Bone replaced by collagen and fibroblast Assoc with McCune Albright syn - Precocious puberty - Cafe au lait - Fibrous dysplasia bone
245
Precocious puberty Cafe au lait spots Fibrous dysplasia bone
McCune albright syndrome
246
unhappy Triad
Anterior cruciate lig Medial cruciate lig Lateral/ medial meniscus
247
Ankle sprain (3)
Anterior talofibular lig Calcaneofibular lig Posterior talofibular lig
248
What structures damaged in anterior shoulder dislocation
Axillary n. Posterior circumflex artery Supraspinatus tendon Anterior glenohumoral ligaments
249
What is damaged with posterior dislocation of the hip
Medial and lateral circumflex femoral a. Femoral v Sciatic nerve Head of femur
250
Polymyositis
Inflammatory muscle disease Proximal muscle weakness CD8 t cells induced injury to muscle fibers Increase Ck and aldolase
251
Fibromyalgia
Excess tenderness at 11 of 18 sites Fatigue Tx. Pregabalin, milnacipran
252
Duchenne muscular dystrophy
X linked frame shift mutation Absent Dystrophin gene INcreased creatine kinase
253
Becker muscular dystrophy
Inframe shift mutation Mutated dystrophin gene
254
Polymyalgia rheumatica
Joint pain Stiffness in proximal joints Malaise, fever, WL No weakness Tx prednisone
255
S-100 tumor marker
Melanoma
256
Melanoma histologically
Nests of melanocytes that fill dermis and obsecure epithelial dermal junction
257
Albinism due to
Lack of melanin pigment due to lack of tyrosinase enzyme
258
Vitillago due to
Decreased number of melanocytes
259
Melasma
Dark discoloration common in pregnant women those take OCPs or hormone replacement Patchy cheeks or lip Due to stimulation of melanocytes by estrogen and progesterone
260
Cellulitis organisms looks like
Staph. aureus Strep pyogenes Painful and tender to touch rapidly spreads
261
cellulitis tx - MRSA - non-MRSA
Non-MRSA( no abscess) - oral dicloxacillin, cephalexin MRSA: - oral trimethoprim/sulfamethoxazole - clindamycin
262
Necrotizing fasciitis organism Tx
Strep. pyogenes Anaerobic bacteria Tx: Debridement IV carbapenem clindamycin
263
Painful flaccid blister biopsy shows Ab against test unique feature
Pemphigus vulgaris acantholysis Ab desmosomes Positive Nikolsky sign oral lesions
264
Tight raised blister
Bullous pemphigoid Ab to hemidesmosomes Eosinophils within blister Negative nikolsky no oral lesion
265
Blister IgA in tips of dermal papillae
Dermatitis herpetiformis assoc w/ celiac
266
Erythema multiforme
Deposition of immune complexes Following infection or drug exposure Target lesions
267
Pruritic, purple, polygonal papules and plaques - microscopically see - assoc with
Lichen planus Sawtooth pattern at dermal-epidermal junction Hepatitis C
268
Acanthosis nigricans - hyperplasia of - assoc w/
velvety hyperpigmentation of skin Stratum spinosum Hyperinsulinemia and visceral malignancies
269
Erythema nodosum assoc with
Sarcoidosis, histoplasmosis, Tb, leprsy
270
Pruritic vesicles associated with celiac disease
Dermatitis herpetiformis
271
Ligaments of ovaries/ uterus w/ vessels
Cardinal ligament - uterine vessels Cervix to pelvic sidewall Infundibulopelvic ligament (suspensory lig of ovary) - ovarian vessels - ovary to pelvic side wal Broad ligament - uterus, fallopian tubes, and ovaries to pelvic wall - contain ovarian lig, round lig, and suspensory lig of ovary
272
Ligaments of ovaries/ uterus with no vessels
Round ligament of uterus - gubernaculum - uterus thorugh inguinal canal to labia majorum Ovarian ligament - ovary to uterus - gubernaculum
273
Nerves damaged w/ prostatectomy --> erectile dysfunction Release Leads to
Cavernous n. release NO (vasodilation) Increase cGMP Decrease Ca
274
Nervous system components of male sexual response
Erection (point) - parasym Emission & ejaculation (shoot) - sympathetic
275
R ovarian/ testis vein drain to
R gonadal v to IVC
276
L ovarian/ testis vein drin to
L gonadal v. to L. renal vein --> IVC
277
Indirect inguinal hernia Direct inguinal hernia
Indirect inguinal hernia - Most common - Congenital due to failure of closure of processus vaginalis - Protrude through deep inguinal ring, lateral to inferior epigastric vessels and pass through inguinal canal Direct inguinal hernia - Weakening of abdominal wall in Hesselbach's triangle - Increase pressure heavy lifting - Directly through abdominal wall medial to inferior epigastric vessel, exit out superficial inguinal ring
278
Hesselbach's triangle
Inguinal lig Inferior epigastric vessel Lateral border of rectus abdominis
279
Mitral regurg vs Aortic regurg
Mitral regurg - Left lateral decubitis best heard - Enhanced w/ squatting - Expiration - Holosystolic Aortic regurg - Widening of pulse pressure - Diastolic - Decrescendo
280
Epispadias associated with
opening of penis on dorsal surface (top) Exstrophy of bladder - exposure of bladder interior to the outside world
281
Male reproductive development process
Mesonephric duct SRY gene on Y chromosome Produce testis-determining factor -Induces gonad differentaition - Develop of two cells types Sertoli - Produce Mullerian inhibitor factor - Paramesonephric duct degenerates Leydig cells - Produce testosterone - Mesonephric duct --> internal male (but not prostate)
282
What induces urogenital sinus and genital tubercle to become male external genitalia
DHT
283
Paramesonephric duct forms
Fallopian tube, uterus and upper portion of vagina
284
What forms the lower portion of the vagina
Urogenital sinus --> sinovaginal bulbs --> lower vagina
285
Genital homologs 1) Glans penis 2) Corpus spongiosum and corpus cavernosum 3) Bulbourethral (Cowper) glas 4) Prostate gland 5) Ventral shaft of penis 6) Scrotum
1) Glans penis= glans clitoris 2) Corpus spongiosum and corpus cavernosum= vestibular bulbs 3) Bulbourethral (Cowper) glands= Greater vestibular (Bartholin) glands 4) Prostate gland= Urethral and paraurethral (skene) glands 5) Ventral shaft of penis= Labia minora 6) Scrotum= Labia majora
286
Genital homologs 1) Glans clitoris 2) Vestibular bulbs 3) Greater vestibular (Bartholin) glands 4) Urethral and paraurethral (skene) glands 5) Labia minora 6) Labia majora
1) Glans penis= glans clitoris 2) Corpus spongiosum and corpus cavernosum= vestibular bulbs 3) Bulbourethral (Cowper) glands= Greater vestibular (Bartholin) glands 4) Prostate gland= Urethral and paraurethral (skene) glands 5) Ventral shaft of penis= Labia minora 6) Scrotum= Labia majora
287
Gubernaculum and Processus vaginalis in males and females
Males - Gubernaculum: anchors testes to scrotum - Processus vaginalis: forms tunica vaginalis Females - Gubernaculum: becomes ovarian ligament and round ligament of uterus (anchors uterus to labia majora) - Processus vaginalia: obliterated
288
Sertoli cells stimulated by | secrete (3)
Anti-mullerian hormone Inhibin (FSH feedback) Androgen binding protein - maintain levels testosterone - helps mature spermatocytes
289
Leydig cells - located - stimulated by - secretes
Interstitium LH Secretes testosterone
290
Amenorrhea | Loss of smell
Kallman syndrome Defect of X linked KAL chain Decrease GnRH --> Low LH and FSH
291
Redness swelling and tenderness of scrotom relieved by support of the testes - Organisms - Tx
Epididymitis GC/Chlamydia Anal: E coli GC/Chlamydia: Ceftrixone (IM) then doxycycline Ecoli: Fluoroquinolone
292
Lesions on penis Gray, solitary, crusty plaque on the penile shaft or scrotum Red, velvety plaque involving the glans of the penis Multiple papular lesions, not invasive
Bowen disease - progress to invasive SCC Erythroplasia of Queyrat - the tip of the penis Bowenoid papulosis -affects younger individuals
293
95% of penile cancers
SCC assoc w/ HPV
294
Peyronie disease
Angulation of penis Leading to painful erections Inflammation of tunica albuginea
295
Priapism Lead to assoc w/
Persistent penile erection Ischemia and clotting of blood retained in penis Assoc w/ sickle cell disease and spinal cord injuries
296
Balanitis - organism - more common
Inflammation of glans penis Candida Uncircumcised Diabetes
297
Prostatitis - Organism - Tx
<35: Gonorrhea, Chlamydia >35: E coli, klebsiella, serratia, enterobacter, proteus Tx: Fluoroquinolone (levofloxacin), TMP-SMX
298
Estradiol Estrone Estriol
Estradiol - Most potent estrogen - Produced ovaries - need both granulosa and theca cells to produce Estrone - aromatase conversion - adipose tissue Estriol - produced by placenta - weakest
299
hCG - fxn - alpha and beta subunit
Prevents degradation of corpus luteum Shares alpha subunit with LH, FSH, and TSH Beta subunit specific for hCG - detectable in blood 1 wk - detectable in urine 2 wk
300
Prolactin vs oxytocin
Prolactin - milk production Oxytocin - milk ejection (letdown)
301
Menopause - definition - labs
12 months of amenorrhea Decrease estradiol Decrease Inhibin Increase GnRH, LH, FSH Estrone is predominant, aromatase still working
302
Byproducts of MAO and COMT enzymatic activity of dopamine, NE, Epinephrine
Dopamine --> homovanillic acid (HVA) NE --> Vanillylmandelic acid (VMA) Epi --> Metanephrine
303
Smooth white plaques on vulva Parchment paper Fusion of labia majora and minora - due to - tx - risk of
Lichen sclerosus Thinning of epidermis Corticosteroids SCC of vulva
304
Koilocytosis
Enlarged nuclei and perinuclear halo Fried egg Seminoma Dysgerminioma HPV
305
Vaginal tumors (3)
SCC ``` Clear cell adenocarcinoma - DES exposure - T shaped uterus - Vaginal adenosis (patches of columnar epithelium on ectocervix and vagina) ``` Sarcoma botryoides (Embryonal rhabdomyosarcoma) - infants, <5 y.o - bunch of grapes out of vagina
306
HPV 16 and 18 MOA
E6 binds p53 --> degradation | E7 binds Rb and inactivates
307
Post coidal bleeding | Pelvic or low back pain
Cervical cancer Lesion on surface of cervix
308
Bunch of grapes growing out of vagina
Sarcoma botryoides Embryonal rhabdomyosarcoma
309
T shaped uterus | Patches of columnar epithelium on ectocervix and vagina
(Vaginal adenosis) Clear cell adenocarcinoma due to DES exposure
310
Cervical cancer presentation
abnormal vaginal bleeding - post coidal Vaginal discharge Pelvic or low back pain Bowel or bladder symptoms Ureteral obstruction - Pyelonephritis - uremia
311
Endometrium layers | Which are shed
Stratum compactum Stratum spongiosum Stratum basalis Basalis only one not shed Stratum functionalis - Stratum compactum - Stratum basalis
312
Endometritis - what cells do you see in endometirum - tx
Plasma cells Broad spectrum Ab Post partum - Gentamicin + clindamycin
313
Adenomyosis
Endometrial tissue extends into myometrium enlarged globular uterus tender to palpation
314
Whorled pattern
Leiomyomas
315
Nontender enlarged uterus | Infertility
Leiomyomas
316
Rapidly enlarging uterus -from
Leiomyosarcoma Rare malignant Denovo from myometrium
317
Dermoid cyst aka
Benign cystic teratoma
318
String of pearls
PCOS
319
PCOS symptoms - also see (3)
Need 2 of 3 Anovulation/ Oligoovulation - menstrual irregular - infertility Hyperandrogenism - acne - hirsuitism (hair on face) Polycystic ovarian on US ALso see - Obesity - insulin resistance - increased risk endometrial hyperplasia
320
Midcycle pelvic pain associated with ovulation
Mittelschmerz
321
Ascites in otherwise healthy female think
ovarian carcinoma
322
Ovarian tumor | Psamomma bodies
Serous tumor
323
Abundant mucinous ascites
appendical cancer
324
Brenner tumor
Benign ovarian tumor | Urinary tract like epithelium
325
Struma ovarii
Teratoma contains functional thyroid tissue --> hyperthyroidism
326
Rosettes of tumor cells surrounding eosinophilic spaces
Call- Exner body Granulosa cell tumors yellow produce estrogen
327
Meigs syndrome
Ovarian tumor Ascites Pleural effusion
328
SIgnet ring cells
FIlled w/ mucin Pushes nucleus to periphery Metastatic gastric adenocarcinoma to bilateral ovaries Krukenberg tumor
329
Swollen hydropic villi | Snow strom appearance
Hydatidiform mole
330
Hydatidiform mole hallmarks
Swollen hydropic villi Look like grape clusters US: Snowstorm appearance
331
Bloody brown vaginal discharge - arises from - metastasizes to
Choriocarcinoma Complete mole, miscarriage, normal pregnancy, ectopic pregnancy Lung
332
Triple or quadruple screen in pregnancy
AFP Estriol hCG Inhibin
333
Trisomy 18 lab values
Decrease AFP Decrease estriol Decrease hCG
334
Trisomy 21 lab values
Decrease AFP Decrease estriol Increase hCG Increase inhibin
335
Partial mole vs complete mole
Partial mole - 69 XXX, XXY, XYY - Fetal parts - Normal size uterus - increase hCG - No risk choriocarcinoma Complete mole - 46, XX or YY - No fetal parts - Large uterus - BIG increase hCG - Risk of choriocarcinoma
336
Type of ovarian cyst associated with molar pregnancies
Increase hCG Theca-lutein cyst
337
Cause of placental abruption
``` Hx prior placental abruption HTN Trauma Smoking Cocaine use** ```
338
HTN Proteinuria or end organ dysfunction
Preeclampsia ``` Thrombocytopenia Renal insufficiency Increased LFT Pulmonary edema Cerebral or visual symptoms - HA, visual disturbances, seizure ```
339
Hemolysis Elevated liver enzymes Low platelets
HELLP syndrome
340
Patients who have migraines with aura are at an increased risk for what other neurologic condition?
Stroke
341
Trisomy 21 what happens w/ chromosomes | - gene effect seen
Maternal nondisjunction of homologous chromosomes during anaphase of meiosis I Mosaicism
342
Rocker bottom feet Micrognathia Intellectual disability
Trisomy 18
343
``` Rocker bottom feet Intellectual disability Microcephaly Cleft lip and palate Holoprosencephaly Polydactyly ``` labs? US?
Trisomy 13 Patau Decrease hCG, PAPP-A US: increase nuchal translucency
344
Kleinfelter labs
Testicular atrophy Decrease testosterone Decrease inhibin Increase FSH
345
Commonly seen w/ Double Y males
Tall Severe achne Antisocial behavior
346
High-pitched crying and newing also seen (4) Mutation
Cri-du-chat syndrome Microcephaly Severe intellectual disability Epicanthal folds Cardiac abnormalities 5p deletion
347
William syndrome - characteristics (5) - mutation
``` Elfin facies Intellectual disability Hypercalcemia (hypersensitivity to Vit D) Good verbal skills Friendly w/ strangers ``` Microdeletion of long arm of chr 7
348
What should be suspected as a cause of HA in a patient using topical retinoic acid for tx of acne
Idiopathic intracranial hypertension (IIH)
349
What is associated iwth berry aneurysms mutation?
Autosomal dominant polycystic kidney dis APKD1 mutation on chr 16
350
AD disease6
1) Achondroplasia 2) Autosomal dominant polycystic kidney dis 3) Familial adenomatous polypopsis 4) Gardner syndrome 5) Familial hypercholesterolemia 6) Hereditary hemorrhagic telangiectasis 7) Hereditary spheocytosis 8) Huntington dis 9) Li-Fraumeni syndrome 10) Marfan syndrome 11) MEN 12) NF1 13) NF2 14) Tuberous sclerosis 15) VHL
351
Trinucleotide repeat disorders
Huntington Myotonic dystrophy Fragile X syndrome Friedreich ataxia
352
Bilateral acoustic neuroma Hearing loss Tinnitus mutation
NF2 Chr 22 AD
353
Cafe au lait spots Neural tumor Lisch nodules (pigment hamartoma in eye) Scoliosis mutation
NF I AD mutation chr 17
354
Huntington mutation
Chr 4 | CAG repeats
355
Hereditary spherocytosis defect in
Spectrin or ankyrin
356
MEN syndrome
MEN1: MEN 1 gene - PPP - Parathyroid adenomas - Pituitary adenomas - Pancreatic tumor MEN2A: RET gene - PPM - Parathyroid adenomas - Pheochromocytoma - Medually thyroid cancer MEN2B: RET gene - PMM - Pheochromocytoma - Medullary thyroid cancer - Mucosal neuromas
357
``` Facial lesions Hypopigmented ash leaf spots Cortical and retinal hamartomas seizures intellectual diability ```
Tuberous sclerosis Mutation of hamartin or tuberin gene
358
Hemangioblastomas of retina, cerebellum, medulla | Phenochromocytomas
von Hippel lindau disease Deletion of VHL Expression of HIF
359
Fragile X syndrome
X linked trinucleotide repeat Mutation for FMR1 gene which codes for FMRP cytoplasmic protein Involved in mRNA translation of axons and dendrites CGG repeat ``` Macro-orchidism Long face Large jaw Everted ears Mitral valve prolapse Intellectual disability ```
360
``` Macro-orchidism Long face Large jaw Everted ears Mitral valve prolapse Intellectual disability ```
Fragile X syndrome
361
``` AR trinucleotide repeat disorder Staggering gait Falling Nystagmus Pes cavus Kyphoscoliosis ```
Friedreicha ataxia GAA Mutation for gene that codes for frataxin (mitochondrial protein that is involved in iron detoxifying and storage)
362
AD disease fits 1) Associated with mitral valve prolapse, liver disease, berry aneurysms 2) Neural tumors and pigmented iris hamartomas 3) Very strong association with colon cancer 4) MI before age 20 5) Hemangioblastomas of retina/ cerebellum/ medulla 6) Increased MCHC, hemolytic anemia 7) Bilateral acoustic neuromas 8) Facial lesions, seizure disorder, cancer risk 9) Caudate atrophy, dementia 10) Cystic medial necrosis of aorta 11) Defect of FGF receptor 3 12) Assoc w/ floppy mitral valve, dissecting aortic aneurysm
1) ADPKD 2) NF1 3) Familal adenomatous polyposis 4) Familial hypercholestrolemia 5) VHL 6) Hereditary spherocytosis 7) NF2 8) Tuberous sclerosis 9) Huntington's dis 10) Marfan syn 11) Achondroplasia 12) Marfan syndrome
363
X linked recessive pneumonic
Oblivious Females will Give Her Boys Her (x)Linked Disorders ``` Ocualr albinism Fabry dis Wiskott aldrich G6PD defiency Hunter syndrome Bruton agammaglobulinemia Hemophilia A/B Lesch-Nyhan syn Duchenne muscular dystrophy ```
364
Lysosomal storage disease
``` Fabry dis (X linked recessive) Gaucher dis Krabbe Metachromatic leukodystrophy Niemann Pick Tay-sach ``` ``` Hurler syndrome Hunter syndrome (X linked recessive) ```
365
Painless papules on lower abdomen peripheral neuropathy glomerulopathy (renal failure)
Fabry disease Deficient: alpha- galactosidase Accumulation: ceramide trihexoside; globotriaosylceramide
366
``` Hepatosplenomegaly Cherry red spot Painful bony lesions Anemia Fatigue thrombocytopenia ``` -Microscopically
Gaucher disease Deficient: glucocerebrosidase Accumulation: Glucocerebroside Prominent blue cytoplasmic fibrils (crumpled tissue paper)
367
``` Hepatosplenomegaly Cherry red spot Thrombocytopenia Ataxia Dysarthria Dysphagia Gradual worsening of intellectual fxn ``` - Mircoscopically
Niemann-Pick disease Deficient: Sphingomyelinase Accumulation: sphingomyein Foam cells
368
Irritability Regression of motor skills Macrocepahly Cherry red spot
Tay-Sachs Deficient: hexosaminidase A Accumulation: GM2 ganglioside
369
``` Peripheral neuropathy Seizures Optic atrophy Developmental delay Weakness ```
Krabbe disease Deficient: galactocerebrosidase Accumulation: galactocerebroside
370
Muscle wasting Weakness Progressive vision loss Dementia
Metachromatic leukodystrophy Deficient: arylsulfatase Accumulation: cerebroside sufate
371
``` Progressive deterioration Coarse facial features Hepatosplenomegaly Intellectual disability Poor growth (dwarfism) Corneal clouding ```
Hurler syndrome Deficient: alpha-L-iduronidase Accumulation: heparan sulfate and dermatan sulfate
372
``` 2 y.o Progressive deterioration Coarse facial features Hepatosplenomegaly Intellectual disability Poor growth (dwarfism) Aggressive behavior ```
Hunter syndrome Deficient: iduronate sulfatase Accumulation: heparan sulfate and dermatan sulfate
373
Hepatomegaly Hypoglycemia Seizure Lactic acidosis
Von Gierke Glycogen storage disease
374
Cardiomegaly | Severe hypotonia
Pompe disease Lysosomal alpha-1,4, glucosidase deficiency
375
What type lysosomal storage diseases have myelin sheath pathology leading to peripheral neuropathy
Krabbe disease Metachromatic leukocystrophy
376
Signet ring cells | ER and PR positive
LCIS
377
Large bulky breast tumor | Leaf like projections on histology
Phyllodes tumor
378
Bloody nipple discharge
intraductal papilloma
379
What is a feature of gram positive bacteria cell wall
Lipoteichoic acids Induce cytokine production IL-1, TNF-alpha
380
What is the unique featuers of gram negative bacteria cellw all
Outer membrane - endotoxin (LPS) - induce IL-1, TNF alpha ``` Periplasmic space (between membranes) - beta lactamase ```
381
Quellung reaction
Anti-capsular serum added to bacteria Capsule appears swollen under microscope
382
Bacteria w/ capsule pneumonic
Even Some Pretty Nasty Killers have Shiny Bodies ``` E coli Strep pneumonia Pseudomonas Neisseria meningitidis Klebsiella Haemophilius influenza type B Salmonella Group B strep ``` Cryptococcus neoformans - encapsulated yeast
383
Mycoplasma vs mycobacteria
Mycoplasma - sterols for plasmsa membrane - No cell wall Mycobacteria - Mycolic acid cell wall - Rich in lipids, resistant to staining
384
Bacteria that dont stain well
Mycoplasma Mycobacteria Treponema - due to corkscrew and small - dark field microscopy Legionella pneumophilia - stain poorly due to unusual branch chain fatty acid in wall
385
Giemsa stain
Giemsa Chinese Painted wHore Bows To Rich ``` Chylamydia Plasmodium Histoplasma Borrelia Trypanosomes Rickettsiae ```
386
PAS stain for
Tropheryma whipplei Whipple disease
387
Ziehl neelsen stain
Acid fast organism
388
India ink
Fungus Crytococcus neoformans
389
Silver stain
Fungi -Pneumocystis jirovecii Legionella
390
Neurotoxins
Interfere with neural transmission Botulinum toxin Tetanus toxin
391
Enterotoxins
Affect intestines Shigella Vibero cholera
392
Superantigens
Exotoxins that simulatenously bind MHC II receptor and T cell receptor Polyclonal expansion of T cells Large amounts of cytokines Staph Aureus Strep Pyogenes
393
Diphtheria toxin
Inactivates EF-2 Inhibits protein synthesis Corynebacterium diphtheriae
394
Inactivates EF-2
Exotoxin A - pseudomonas Diphtheria toxin
395
Shiga toxin
Cleaves host rRNA at adenine base in 60S ribosomal subunit Inhibition of protein synthesis Shigella
396
Ecoli toxin
Verocytotoxin (Shiga toxin) - Bloody diarrhea - HUS ``` Heat labile toxin - stimulates adenylyl cyclase - Increase cAMP Heat stabile toxin -stimulate guanylyl cyclase - increase cGMP ETEC ```
397
Heat stable toxin
ETEC | Yersinia enterocolitica
398
Pseudoappendicitis
Yersinia enterocolitica Heat stable toxin Increase cGMP
399
Anthrax toxin
Edema factor (adenylyl yclase) Lethal factor Protective antigen Bacillus anthracis
400
Vibrio cholerae
Cholera toxin stimulates adenylyl cyclase increase cAMP chloride and water cross lumen rice water stools
401
Bordetella pertussis
Pertussis toxin binds and inactivates inhibitory G proteins --> increase cAMP
402
Clostridium tetani
Tetanospasmin blocks release of inhibitory NT gaba and glycine
403
Botulinum toxin
Exotoxins A->G inhibit release of ACh at NMJ CLostridium botulinum Spoiled canned goods Honey
404
Clostridium perfringens toxins
Alpha toxin (phospholipase) - Gas gangrene - Myonecrosis Enterotoxin - Food poisoning - Food left out too long once cooked
405
Endotoxin triggers (3)
1. Macrophages - IL-1 IL-6= fever - TNF= cell death - NO= hypotension, development septic shock 2. Complement - C3a= release histamine, hypotension, edema - C5a= neutrophil chemotaxis 3. Tissue factor - coagulation cascade --> DIC
406
Toxic associated with Staph aureus - hemolysis - tissue destruction w/ mRSA affects both neutrophils and macrophages - food poisoning - release of cytokines, high fever, hypotension, diffuse rash - Scalded skin syndrome in newborns
Hemolysis - alpha-toxin (alpha hemolysin) - sphingomyelinase C (beta) - leukocidin (gamma) - delta-toxin Panton-Valentine leukocidin Enterotoxin (superantigen) Toxic shock syndrome toxin 1 (superantigen) Exfoliative toxin
407
Toxins secreted by Strep pyogenes
Streptolysin O - hemolysis (oxygen labile) Streptolysin S - hemolysis (oxygen stable) Streptococcal pyrogenic exotoxins Type A, B, C/ erythrogenic toxins - red rash (erythro-) and fever (pyro-) of scarlet fever (superantigen)
408
Degrades H202 into H20 and O2
Catalase
409
Increases bidning to host tissues, induce coagulation or blood clotting
Coagulase
410
S. epidermidis vs s. saprophyticus
S. epidermidis - Novobiocin sensitive - heart valves S. saprophyticus - Novobiocin resistant - UTI
411
Gram positive diplococci
Strep. pneumoniae
412
Viridans streptococci
Colonizer of the oral cavity Cause infective endocarditis No capsule Partial hemolysis
413
Chronic granulomatous disease
NADPH oxidase deficiency H2O2 easily broken down by catalase (+) organisms Susceptible in infections by staph
414
Contaminated deli meat
Listeria
415
meningitis in very young and old
Listeria
416
Potato salad
Staphyloccus aureus Rapid vomiting
417
Fried rice Reheated rice
Bacillus cereus
418
Pigment producing bacteria (4)
Staph aureus - Large golden colonies Actinomyces israelii - yellow (sulfur) granules - yellow sand Pseudomonas aeruginosa - blue green pigment ``` Serratia marcescens (MARsecens) - red pigment ```
419
What ovarian tumor matches each of hte following 1) Estrogen secreting leading to precocious puberty 2) Produces AFP 3) Psammoma bodies 4) Testosterone secreting leading to virilization
1) Granulosa theca cell tumor 2) Yolk sac tumor 3) serous cystadenocarcinoma 4) Sertoli-leydig cell tumor
420
Gram (+) cocci Catalase negative Dental plaque
S. mutans (most) S. sanguinis - preexisting heart flow problems
421
Common disease caused by Group A strep (pyogenes)
Acute pharyngitis Cellulitis Invasive infection Acute post-streptococcal glomerulonephritis Rheumatic fever Toxic shock syndrome
422
Impetigo caused by
Staph aureus | Strep pyogenes
423
Otitis media and sinusitis Sudden onset chills Lobular consolidation Rust colored sputum
Streptococcus pneumoniae
424
Group A Strep immunologic complications
Body immune response to bacteria ``` Jones Joints Pancarditis (endo/myo/pericarditis) Nodules (SQ) Erthema marginatum Sydenham chorea ```
425
Neonatal sepsis
Group B strep Listeria Ecoli
426
Gram positive bacteria associated iwth colon cancer
Streptococcus bovis Group D strep Endocarditis Found in gut
427
Bacteria cause subscute endocarditis
``` Coagulase neg Staph Viridans group streptococci Strep bovis Enterococci HACEK organisms ``` ``` HACEK - not gram (+) Haemophilus aphrophilus Aggregatibacter spp Cardiobacterium hominis Eilcenella corrodens Kingella kingae ```
428
Spore forming
Clostridium spp Bacillus anthracis Coxiella burnetti
429
Clostiridum difficile - toxins - diagnosis - tx
Toxin A (enterotoxin) - binds brush border - secretion and inflammation ``` Toxin B (cytotoxin) - disruption actin cytoskeleton ``` Pseudomembrnaous colitis Stool toxin assay (PCR or ELISA) Tx - Metronidazole - Oral vancomycin - Fidxomicin (recurrent)
430
Mediastinal widening on CXR
``` Bacillus anthracis Aortic dissection Ruptured esophagus Lymphoma Aortic aneurysm due to syphillus ```
431
Large painless ulcer w/ central necrosis (eschar)
Cutaneous anthrax | Wound
432
Obligate anaerobes (3) Tx
Clostridium species Actinomyces (Gram +) Bacteroides (Gram -) Tx metronidazole Clindamycin
433
Facial infection of jaw/mandible | Sinus tracts draining yellow pus
Actinomyces israelii
434
Decaying vegetation | Infection in immunocompromised
Nocardia
435
Whitish patch on posterior oropharynx, makes greyish membrane, will bleed if scrap off Tx
Corynebacterium diphtheriae Erythromycin or penicillin Antitoxin Vaccinate
436
Chocolate agar | - requires
Haemophilus influenza Factor V and X
437
Epiglottitis
Haemophilus influenza
438
Buffered charcoal yeast extract agar - requires - transmission - tx
Legionella pneumophila Iron Cysteine Aerosols from water sources macrolides Fluoroquinole
439
Spiral shaped rod urease positive causes tx
Helicobacter pylori Gastric adenocarcioma ``` Tx: Triple therapy - PPI - Clarithromycin - Amoxicillin or metronidazole ``` Quadruple therapy - PPI - Bismuth - Metronidazole - Tetracycline
440
Lactose non-fermenters Urease producing -Creates what
Proteus spp ``` Alkaline urine Struvite stones (Staghorn calculi) ```
441
Associated w/ reactive arthrits
``` Shigella Salmonella Campylobacter jejuni Yersinia Chlamydia ```
442
Question marked shaped Rodent urine Phase 1 Phase 2
Leptospira interrogans Phase 1 Fever chills Body ache HA Phase 2 Hyponatremia Renal failure Liver damage w/ jaundice
443
``` Bulls eye Fever HA Body ache Fatigue ``` Tx
Borrelia burgdorferi Ixodes tick Tx Doxycycline (early stage) Amoxicilin (children <8) Doxycycline or Ceftriaxone (late)
444
Bilateral facial nerve palsy Tx
Borrelia burgdorferi Ixodes tick Tx Doxycycline (early stage) Amoxicilin (children <8) Doxycycline or Ceftriaxone (late)
445
Argyll robertson pupil
Pupil constricts with accomodation but doesnt react w/ direct light Neurosyphilis
446
Rash involving palms and soles | Maculopapular
Kawasaki disease Coxsackie A virus Rocky mountain spotted fever Syphillis Meningococcemia You drive Kawaski CARS with your hands and feet
447
Sensory ataxia Lancinating pain: sudden stabs of pain in body Loss of proprioception and vibratory sense
Tabes dorsalis Posterior dorsal columns of SC and dorsal root Neurosyphilis
448
Zoonotic bacteria 1) Cat scratch 2) Rodents to Tick 3) Cattle fluids 4) Birds and pigs 5) Birds 6) Infected placenta or contaminated fluid of newborn farm animals 7) Lone star tick 8) Rabbit or squirrel 9) Infected animal urine 10) Nine-banded armadillo 11) dog bit 12) Lice 13) Fleas 14) Tick, rash on palms 15) Fleas, prairie dogs
1) Bartonella henselae 2) Borrelia burgdorferi 3) Brucellosis 4) Campylobacter 5) Chlamydophila psittaci 6) Coxiella burneii 7) Ehrlichia chaffeensis 8) Francisella tularensis 9) Leptospira 10) Mycobacterium leprae 11) Pasteurella multocida 12) Rickettsia prowazekii 13) Rickettsia typhi 14) Rickettsia rickettsii (rocky mountain spotted fever) 15) Yersinia pestis
449
What is the classic presenting symptoms in a patient with Lyme disease
Erythema migrans (bulls eye)
450
Leprosy Hansen disease two types
Lepromatous disease - weak immune system - disfuguring Tuberculoid disease - strong immuen response - fewer skin lesions
451
Clue cell - organism - characteristic (2) - change - tx (2)
Vaginale pithelial cell covered in coccobacilli Gardnerella vaginalia - gray vaginal discharge - fishy smell - elevated pH >4.5 Oral metronidazole Metronidazole cream
452
Headache Fever Rash on wrist and ankles moves to palms soles and trunk
Ricketta ricksttsii Rocky mountain spotted fever
453
HA | Fever, Rash centrally spreads to extremities
Rickettsia prowazekii -Lice Rickettsia typhi - Fleas
454
Febrile Non specific Morulae within monocytes or granulocytes Vector Tx
Ehrlichia chaffeensis -Lone star tick Anaplasma phagocytophilum - Ixodes scapularis Tx: Doxycycline
455
Acute flu like illness w/ pneumoniae Works on farm - chronic assoc w/
Coxiella burnetii - Q fever - Spore forming - chronic assoc w/ endocarditis
456
Chlamydia Types 1) A-C 2) D-K 3) L1-L3 Tx
A-C - Acute eye infection - Can lead to scarring and blindness D-K - Urethritis, cervicitis, PID - Neonatal pneumonia and conjunctivitis L1-L3 - Lymphogranuloma venereum - Genital ulcer - Unilateral lymphadenopathy - Groove sign - Proctitis Tx: Azithromycin
457
Atypical pneumonia
Legionella pneumophila Chlamydophila pneumoniae Mycoplasma pneumoniae
458
``` Malaise, HA Low grade fever Nonproductive cough Diffuse interstitial infiltrates Coughing for weeks ``` CXR: patchy reticulonodular infiltrate Tx Diagnosis
Mycoplasma pneumonia College kids- dorms Military recruits Macrolide (azithromycin) Doxycycline (empiric) Fluroquinoles (empiric) Cold agglutinins - Nonspecific IgM Ab to erythrocytes
459
Lymphatic drainage 1) Arm lateral breast 2) Posterior calf, dorsolateral foot 3) Thigh 4) Stomach, upper duodenum 5) Lower duodenum, jejunum, ileum, proximal 2/3 of colon 6) Distal 1/3 of colon, upper rectum 7) Lower rectum (above pectinate line) 8) Anal canal (below pectinate line) 9) Test, ovaries, uterus kidneys 10) Scrotum, vulva 11) Right arm and right side of face 12) Rest of body
1) Arm lateral breast = Axillary LN 2) Posterior calf, dorsolateral foot = Popliteal LN 3) Thigh = Superficial inguinal LN 4) Stomach, upper duodenum = Celiac LN 5) Lower duodenum, jejunum, ileum, proximal 2/3 of colon = Superior mesenteric 6) Distal 1/3 of colon, upper rectum = Inferior mesenteric 7) Lower rectum (above pectinate line) = Internal iliac 8) Anal canal (below pectinate line) = superficial inguinal 9) Testes, ovaries, uterus kidneys = para-aortic 10) Scrotum, vulva = superficial inguinal 11) Right lymphatic duct 12) Thoracic duct --> L subclavian
460
Peyers patches are located
Lamina propria and submucosa of ileum
461
Which muscles and nerves are derived from the first brachial arch?
M & T Muscles of mastication - Masseter - Medial pterygoid - Lateral pterygoid - Temporalis Mylohyoid Tensor tympani Tensor veli palatini Anterior 2/3 of tongue Mandibular and maxillary division of Trigeminal n.
462
What is the cell type in peyer's patches
M (microfold) cells Take up Ag from lumen of gut and transcytose to other side of cell
463
Dendritic cell surface markers
MHC I MHC II B7 protein (CD80 or 86) CD40
464
HLA-B27 HLA-DR3, HLA-DR4
Seronegative spondoloarthropathies "PAIR" Psoriatic arthritis, ankylosing spondylitis, inflammatory bowel disease, reactive arthritis Type 1 diabetes mellitus
465
S-100 CD1a Tennis rackets
Langerhans cell histiocytosis Excessive proliferation of langerhan cells but are poor at presenting Ag
466
Positive selection | - occurs where
Selecting for T cells that can bind to MHC Loss CD4 or CD8 Cortex of thymus
467
Negative selection | - occurs where
Selectingout T cells w/ receptor for self antigen Corticomedullary junction
468
Induces Th1 differentiation Th1 produces Functions of those produced
IL-12 --> Th1 Th1 --> IL-2, INF-gamma IL-2: proliferation of T cells induce more Th1 IFN-gamma: activate macrophages, suppress Th2
469
Induces TH2 differentiation Th2 produces Functions of those produced
IL-4 --> Th2 Th2 --> IL-4, IL-5, IL-10 IL-4, IL-5= proliferation of B cells, induce production of Th2 cells Il-10= inhibit production Th1, inhibits macrophages
470
T cell activation
2 signals TCR and CD4/CD8 binds to MHC II/I B7 protein (CD80/86) on APC binds to CD28 of T cell
471
Transcription factor the helps control the development of Treg cells
FOXP3 Forkhead Box Protein P3 Found on X chromosome Mutation: suppress immune system - condition called IPEX ``` Immune dysregulation Polyendocrinopathy - Type I diabetes - Thyroiditis Enteropathy - Severe diarrhea - Failure to thrive X linked ```
472
Male infant Severe chronic diarrhea Neonatal diabetes or thyroiditis Eczema
IPEX Mutation of FOXP3
473
B cell activation
1) Th cell activated 2) B cell endocytoses Ag and present to Th cell 3) B cell has CD40 and binds to CD40L on T cell 4) Th2 produce IL-4, IL-5 induce B cell proliferation and class switching
474
NK cells activity induced by
IL-12 IL-2 IFN-alpha IFN-beta
475
IFN-alpha and IFN-beta fxn
Induce NK cells Induce near by cells to inhibit viral protein synthesis
476
CD16 - does what - found on - function
Binds to Ab (on constant region Ab) ``` Found on: NK cells Macrophages Monocytes Neutrophils Eosinopohils ``` Antibody dependent cell mediated cytotoxicity (ADCC) - Enemy cell get coated with IgG antibody, NK cells and other cells w/ CD16 surface marker are able to recognize Ab coated cells and kill them
477
VDJ recombination begins with Initated by
Breaks in dsDNA at Recombination Signal sequences RSSs Initiated by RAG-1 and RAG-2
478
Surface markers B cells T cells T cytotoxic T helper NK cells Macrophages Dendritic
1) B cells - CD19, 20, 21 - IgG, IgM - B7 - CD40 - MHC II 2) T cells - CD3 - TCR - CD28 3) T cytotoxic - CD3 - TCR - CD28 - CD8 4) T helper - CD3 - TCR - CD28 - CD4 - CD40 ligand 5) NK cells - CD16 - CD56 6) Macrophages - CD14 - MHC II - B7 - CD40 7) Dendritic - MHC II - B7 - CD 40
479
IgG (5) IgA (5) IgE (4) IgM (4) IgD (1)
1) IgG - Delayed immune response - Fixes complement - Crosses placenta ** - Opsonizes bacteria ** - Neutralize viruses and bacterial toxins 2) IgA - Secreted by MALT - Mucous membranes and GI - Monomor in circulation - Dimer in secretion (Mucus, tears, saliva, breast milk) 3) IgE - Immunity to parasites - Activates eosinophils - Mast cells and basophils - Type I hypersensitivity 4) IgM - Surface of B cells (monomer) - Circulation (pentamer) - Primary immune response - Does NOT cross placenta 5) IgD - surface of B cells
480
Live Virus vaccinations pneumonic
Attention! Please Vaccinate Young Infants with MMR Regularly Attenuated virus vaccines ``` Polio (sabin, oral) Varicella Yellow fever Intranasal influenza MMR Rotavirus ```
481
Egg based vaccines
Influenza Yellow fever (MMR) tiny amount
482
Bacteria vaccine - toxin - capsular polysaccharides - killed bacteria - live attenuated bacteria
Toxin - Tetanus toxoid Capsular polysaccharides - S. pneumoniae - H. influenzae Killed bacteria - Vibrio cholerae Live attenuated bacteria - Typhoid vaccine - BCG vaccine
483
Amyloidosis types (6)
1) Immunologic (primary) - Ig light chain - AL fibril 2) Secondary - SAA (serum amyloid associated protein) - AA fibril 3) Type 2 diabetes - Amylin (secreted by pancreas) - AIAPP 4) Medullary thyroid CA - Calcitonin - A-CAL 5) Alzheimer - beta-amyloid - APP (amyloid precursor protein) 6) Dialysis associated - beta2- microglobulin - Abeta2M
484
Opsonization
C3b | IgG
485
Stimulates mast cells and basophils (complement)
C3a
486
C5a does what
Neutrophil chemotaxis
487
Deficiency of C1 esterase inhibitor Deficiency of C3 Deficiency of MAC Deficiency of DAF
1) Def C1 esterase inhibitor - Hereditary angioedema - Elevated bradykinin - Never take ACE inhibitor, also increase bradykinin levels 2) Def C3 - Recurrent pyogenic sinus infections and resp tract infections - encapsulated organisms - Strep pneu, Haemophilus influ - Increased sensitivity to type II infections 3) Neisseria bactermia susceptible 4) Def in DAF (CD55) - Protect self form spontaneous complement activation through alternative pathway - Paroxysmal noctural hemogobinuria (PNH)
488
Paroxysmal nocturnal hemoglobinuria (PNH) deficient in what clinical (3) Tx (3)
TBC deficient in two surface molecules that protect against MAC 1) Glycosylphosphotidylinositol (GPI) which anchors Decay accelerating factor (CD55) to plasma membrane 2) MAC inhibitory protein (CD59) Clinical - break down RBC intravascular - hemosiderinura (red urine) - thrombosis (platelets destroyed) Transfusion Warfarin Eculizumab
489
Which organisms cannot be stained w/ gram stain
``` Treponema Rickettsia Chlamydia Legionella Mycoplasma Mycobacteria ```
490
Macrophages secrete
IL-1, IL-6, TNF-alpha (acute phase) | IL-12
491
Neutrophils chemotactic agents
IL-8 C5a Leukotriene B 4
492
Cytokines functions pneumonic
Hot T bone stEAk ``` IL-1: fever IL-2: stimulates T cells IL-3 stimualtes bone marrow IL-4: IgE adn IgG IL-5: IgA and eosinophils ```
493
Differential diagnosis Eosinophilia pneuonic
CANADA P Collagen vascular disease (PAN, dermatomyositis) Atopic disease (allergies, asthma) Neoplasm Adrenal insufficiency (addison dis) Drugs (NSAIDS, penicillins, cephalosporins) Acute interstitial nephritis Parasites (stronglyloides, ascaris) HIV Hyper igE coccidioidomycosis
494
Chvostek sign | Trousseau sign
Chovstek: tap on cheek, causes facial spasm Troussseau: blood pressure cuff, leads to spasm of arm Hypocalemia
495
6 month old boy Recurrent bacterial infections Low immunoglobulins - defect - inheritence
Bruton agammaglobulinemia Defective tyrosine kinase X linked
496
Recurrent candida infections and respiratory infections Diarrhea Failure to thrive No thymus seen on imaging - deficiency - decrease in
Severe combined immunodeficiency (SCID) Decrease B and T cells Adenosine deaminase deficiency
497
Cant follow object Talangiectasias of face Recurrent infections clinical (2) sensitive (2) risk (2) elevated
Ataxia-telangiectasia IgA deficiency T cell deficiency ``` Cerebellar ataxia Telangiectasias after 5 y.o Radiation sensitivity (avoid x ray) Increased risk: lymphoma leukemia -Elevated AFP (8 months) ```
498
Ataxia-telangiectasia mneumonic "ATAXIA"
``` "ATAXIA" Ataxia Telangiectasia, tracking eye difficulties Acute leukemia and lymphoma Xray sensitivity IgA deficiency AFP ```
499
Wiskott-Aldrich syndrome mneumonic
``` WAITER Wiskott Aldrich Immunodeficiency Thrombocytopenia and purpura Eczema Recurrent pyogenic infections ``` X linked Low IgM, high IgA
500
1) Defect tyrosine kinase 2) Elevated AFP 3) Adenosine deaminase deficiency 4) Low IgM, high IgA 5) Lack NADPH oxidase 6) Defect LYST gene 7) STAT3 mutation 8) Abnormal integrins 9) Two rows of teeth 10) Delayed separation umbilical cord
1) Bruton agammaglobulinemai 2) Ataxia-telangiectasia 3) SCID 4) Wiskott-Aldrich sydrnome 5) Chronic granulomatous disease (CGD) 6) Chediak-Higashi sydnrome 7) Hyper IgE syndrome (Job syn) 8) Leukocyte adhesion deficiency 9) Hyper IgE syndrome (Job syn) 10) Leukocyte adhesion deficiency
501
Chronic granulomatous disease (CGD)
X linked Lack of NADPH oxidase Cant destroy catalase + Staph aureus, aspergillus TMP-SMX Itraconazole
502
Giant cytoplasmic granules in PMN Partial albinism Recurrent respiratory tract and skin infections Neurologic disorders
Chediak- Higashi syndrome LYST gene defect (lysosomal transport) Defective phagocyte lysosomes
503
``` High level IgE and eosinophils Eczema Recurrent cold S. aureus abscesses Coarse facial features Two rows teeth ```
Hyper IgE syndrome STAT3 signaling protein mutation Impaired differentiation of TH12 Impaired recruitment of neutrophils
504
X linked immunodeficiencies
Bruton agammaglobulinemia Chronic granulomatous disease Wiskott aldrich syndrome SCID
505
Eczema Recurrent cold abscesses high serum IgE
Hyper IgE syndrome
506
Large phagosomal vesicles with neutrophils
Chediak=Higashi disease
507
Tuberous sclerosis mutations | - what is the gene for
TSC1 or TSC2 ``` TSC1= hamartin protein TSC2= tuberin protein ```
508
Seizures Intellectual disability Angiofibromas (tiny bumps/ tumors on nose and cheeks) - also seen
Tuberous sclerosis ``` Hypomelanotic macules (white spots) Retinal hamartomas (white circumscribed tumor of eye) Cortical tuber (white mass in brain benign) ```
509
``` Flaccid paralysis Hyporeflexia weakness Faciculations Fever, HA, malaise Lymphocytic pleocystois (increase lymphocytes in CSF) Slightly elevated protein Normal glucose ```
Polio
510
Infantile flaccid paralysis
Most infants die before 1 year AR Werdnig-hoffman Death of neuron in the anterior horn of Spinal cord
511
``` Rapidly progessive weakness Muscle atrophy Fascicultations Spasticity Difficulty speaking and swallowing ``` - Defect - Tx
ALS Superoxide dismutase 1 Riluzole - lengthens survival - decrease glutamate release
512
Tabes dorsalis - assoc w/ - affects - test
Tertiary syphilis Obliterates dorsal columns Light tough and proproceptive cant get to CNS Romberg sign
513
Bilateral loss of pain and temperature sensation in upper extremities (cape like) Hand muscle weakness and atrophy
Syringomyelia
514
``` Ipsl UMN sign Ipsl loss tactile vibrtaion and proprioception C/L pain and temp below lesion LMN at level of lesion At lesion ipsl pain /temp ```
Brown sequard syndrome complete hemisection spinal cord
515
Brown sequard syndrome
``` Ipsl UMN sign Ipsl loss tactile vibrtaion and proprioception C/L pain and temp below lesion LMN at level of lesion At lesion ipsl pain /temp ```
516
Deep nuclei of cerebellum from medial to lateral
Fast Gerbals Exercise Daily Fastigial nucleus Globose nucleus Emboliform nucleus Dentate nucleus
517
Involuntary flailing of one arm
Hemiballismus Subthalamic nucleus
518
which portions of the hypothalamus are inhibited by leptin ? which are stimulated
Inhibits the lateral nucleus Stimulates teh ventromedial nucleus
519
Dopamineric signals from the ______ to _______ induce movement
Substantia nigra to neostriatum
520
Parkinson - signs - seen in brain - loss of
``` Hypokinesia Festinating gait Pill rolling tremor Cogwheel rigidity Mask like facies ``` Depigmentation of substantia nigra Loss of dopamine
521
Also has parkinsonian features
Synthesizing meperidine Creates MPTP -- MAO--> MPP
522
Athetosis
Slow writhing snke like movements in hands and fingers
523
Akathisia
Compulsion to move
524
Huntingtons Disease
Huntington chorea C's ``` CAG trinucleotide repeat Chromosome cuatro (4) Cuarenta (age 40) Chorea Cognitive decline Caudate atrophy ``` Excessive excitation by glutamate --> death of neurons Increase dopamine Decrease GABA Decrease ACh
525
Fracture to shaft of humerus Fracture of surgical neck of humerus
Radial n. - Wrist drop Axillary n. Posterior circumflex A.
526
Poststreptococcal glomerulonephritis deposits
IgG IgM C3 Immune complex deposition
527
Enteropeptidase activity
Trypsin
528
Gram neg rod Green metallic sheen on eosin methylene blue agar Hemolysis on blood agar
Ecoli
529
Green metallic sheen on eosin methylene blue agar
Ferments lactose
530
Pyruvate kinase deficiency
Pyruvate kinase needed to convert phosphoenolpyruvate to pyruvate, resulting in generation of ATP Deficiency means less ATP, less gradient Disruption of RBC membrane Splenic red pulp hyperplasia to remove RBCs Splenomegaly
531
RAS is usually active when bound to
GTP MAP kinase pathway
532
``` Fever chills, fatigue, dyspnea Temp Ring shaped and Maltese cross Splenectomy Intraerythrocytic inclusions ```
Babesiosis Tick borne infection Babesia microti
533
Heteroplasmy
condition of having different mitochondrial genomes
534
Frothy foamy urine caused by Starling forces?
Proteinuria or bile salts in urine Low plasma oncotic pressure
535
Absence of CD18
Leukocyte adhesion deficiency CD18 needed for formation of integrins Persistent leukocytosis
536
Average total body water is extracellular fluid Plasma volume
41 liters 14 L 3 L (within extracellular)
537
Posterior duodenal wall ulcer erodes into
Gastroduodenal artery
538
Mass in right temporal love vision problems
Affects meyer's loop Left homonymous superior quadrantanopia Left top quadrant of both eyes
539
Hepatoduodenal ligament contains
Common bile duct Hepatic artery Portal vein
540
Amnio acids modified in golgi
Tyrosine Serine Threonine Aspargine
541
Failure of what process results in I cell disease
Addition of mannose-6- receptor marker to lysosome enzymes
542
What develops week 4 Week 8 Week 10
Week 4: 4 chamber heart, 4 limb buds Week 8: fetal movement 8 and "Gait" Week 10: sex specific genitalia ("ten"italia")
543
Neural crest derivitives
Magic Cops ``` Melanocytes Aorticopulmonary septum Ganglia (autonomic, dorsal root, enteric) Iris stroma Chromaffin cells Cranial nervs Odontoblasts/ ossicles Parafollicular C cells Sclerae ```
544
Emybryonic origin of each 1) Anterior pituitary 2) Posterior pituitary 3) Parotid glands 4) Sublingual glands 5) Sclera 6) Lens 7) Retina 8) Mammary glands 9) Sweat glands
1) Anterior pituitary= surface ectoderm 2) Posterior pituitary= neural tube 3) Parotid glands= surface ectoderm 4) Sublingual glands= endoderm 5) Sclera= neural crest 6) Lens= surface ectoderm 7) Retina= Neural tube 8) Mammary glands= surface ectoderm 9) Sweat glands= surface ectoderm
545
G1 --> S
cyclin D binds/ activates CDK4 --> phosphorylation of Rb protein Rb protein released from E2F E2F left free to transcribe/ synthesize components needed for progression through S (cyclin E, DNA polymerase, thymidine kinase) Cyclin E binds/ activates CDK2 --> the cell is allowed into S pahse
546
G2 --> M
Cyclin A - CDK2 complex --> mitotic prophase Cyclin B- CDK1 complex is activated by cdc25 --> breakdown of nuclear lamins and initiation of mitosis
547
Trafficking proteins (3)
1) COP-11 - antrograde trafficking - protein from ER to golgi 2) COP-1 - retrograde from golgi to ER 3) Clathrin - farside of golgi - helps golgi to form vesicles to transport enzymes called hydrolases to lysosomes
548
Post translational modifications of proteins and proteoglycans in golgi apparatus
1) GLycosylation of core proteins to form proteoglycans 2) Sulfation of proteoglycans and selected tyrosine residues 3) Add O-oligosaccharides to serine and threonine residues 4) Adds mannose-6-phosphate to asparagine residues located on lysosomal enzymes
549
I cell disease
Deficiency of mannose phosphorylation No mannose-6-phosphate to target lysosomal proteins Secreted out of cell instead of into lysosomes Corneal clouding, coarse facies, hepatosplenomegaly, skeletal abnormalities, restricted joint movement
550
Corneal clouding, coarse facies, hepatosplenomegaly, skeletal abnormalities, restricted joint movement
I cell disease
551
Beta oxidation of very long chain fatty acids and branched chain fatty acids - synthesis of
Peroxisomes Synthesis of plasmalogens (phospholipids in myelin)
552
Retrograde movement on axon
Dynein
553
Antrograd movement on axon
Kinesin
554
Situs inversus Chronic sinusitis Bronchiectasis Infertility
Kartagner syndrome Dynein doesnt work Immotile cilia
555
what intermediate filaments are found in each of the following? ``` Connective tissue Muscle tissue Epithelial tissue Axons Nuclear envelope and DNA Schwann cells ```
CT= Vimentin Muscle tissue= desmin Epithelial tissue= cytokeratin Axons= neurofilaments Nuclear envelop and DNA= Nuclear lamins Schwann cells= Glial fibrillary acid proteins
556
Kartagener syndrome
Situs inversus Bronchiectasis Chronic sinsitis
557
Which arachidonic acid product causes each of the following effects 1) Increased bronchial tone 2) Increased platelet aggregation 3) Decreased platelet aggregation 4) Increased uterine tone 5) Decreased uterine tone 6) Increased vascular tone 7) Decreased vascular tone
1) LTD4, LTE4, LTC4 2) TXA A2 3) PGL2 4) PGE2, PGF2alpha 5) PGI2 6) TXA A2 7) PGI2, PGE1
558
Needed for Granuloma formation
IFN-gamma: forms granuloma TNF-alpha: maintains granuloma
559
Blue sclerae Hearing loss Multiple fractures -inheritence
``` Osteogenesis imperfecta (OI) - Autosomal dominant ```
560
Cant see Cant pee Cant hear high C
Alport syndrome Type IV collagen Nephritis and kidney failure Hearing loss Eye problems (Cataracts)
561
Role of Vit C in collagen production
Hydroxylation of lysine and proline
562
Damaged in Guillain-Barre syndrome CSF changes Classic manifestations
Schwann cell Normal cell count Increase protein Symmetric ascending muscle weakness Bilateral facial paralysis (palsy) Preceded by infection No sensory loss
563
Holoproencephaly - conditions - mutation
Trisomy 13 patau Fetal alcohol SHH mutation
564
Chiari Malformation
Herniation of cerebellar tonsils
565
Enlarged posterior fossa Cerebellar vermis fails to develop Dilation of 4th ventricle
Dandy Walker
566
Brocas
Language production Understand Cant talk
567
Wernicke's area
Comprehension of language Can talk in sentences Doesnt make sense
568
Lesion to which area of brain is responsible for each of the following 1) Hemispatial neglet syndrome 2) Poor repetition 3) Poor comprehension 4) Poor verbral expression 5) Personality changes and disinhibition 6) Agraphia and acalculia
1) Nondominant angular gyrus, parietal 2) Arcuate fasciculus 3) Wernicke 4) Broca 5) Frontal lobe 6) Dominant angular gyrus, parietal
569
What regulates the progression of the cell cycle from the G1 phase to S phase
p53 | Rb
570
Which arachidonic acid product has actions that oppose that of prostacyclin
Thromboxane A2
571
Disease which has abnormal breakdown of elastin
Alpha 1 anti-trypsin deficiency
572
Associated with berry aneurysms
ADPKD | Ehler Danlos
573
Fearless Curious Puts things in mouth Hypersexual
Bilateral lesion in amygdala Kluver-Bucy syndrome
574
Nystagmus Ataxia Encephalopathy Amnesia
Wernicke-Korsakoff syndrome ``` Thiamine deficiency (Vit B) - alcoholics ``` Mamillary body lesion
575
Located in anterior hypothalamus - damage to this neuron causes dilute urine - secretes antidiuretic hormone
Supraoptic nucleus
576
Stimulation increases appetite Damage causes anorexia adn WL Leptin inhibits this nucleus
Lateral nucleus
577
Regulates teh secretion of TSH Regulates teh secretion of ACTH Secretes oxytocin
Paraventricular nucleus
578
Receives input from retina Controls circadian rhythms Master clock
Suprachiasmatic nucleus
579
Damage leads to savage behavior Damage leads to obesity Stimulated by leptin
Ventromedial nucleus
580
Thermoregulation (cooling) | Damage causes hyperthermia
Anterior nucleus
581
Involved in memory | Damage causes wernicke korsakoff
Mamillary nucleus
582
Regulates appetite Pulsatile secretion of FnRH Secretes dopamine Secretes GHRH
Arcuate nucleus
583
Regulates hunger | Damage leads to obesity and a savage behavior
Dorsomedial nucleus
584
``` Does thermoregulation (warming) Damage causes hypothermia ```
Posterior nucleus
585
Secretes GnRH | nucleus?
Preoptic area nucleus
586
Which amino acids are found in nuclear localization signals
PAL Proline Arginine Lysine
587
Cavernous sinus becomes infected. What neurological deficits
Ophthalmoplegia - CN III: down and out - CN VI: unable to abduct Diplopia Pain/ numbness in upper face ``` Oculomotor n. Trochlear n. Abducens n. Opthalmic div of CN V1 Maxillary div of CN V2 ```
588
Nerve for 1) dorsal foot 2) lower leg 3) Sole of foot 4) lateral lower leg
1) Common fibular nerve 2) Sciatic n. 3) Tibial n. 4) common fibular n.
589
Merkel corpuscle - adaption - sensation - location
Slow Static pressure Superficial
590
Meissner corpuscle - adaption - sensation - location
Rapid Light tough Superficial
591
Ruffini - adaption - sensation - location - shape
Slow Pressure, position sense Deep Spindle shaped
592
Pacinian - adaption - sensation - location - shape
Rapid Vibration Deep Onion-shaped
593
Previous infection In the last week -Muscle weakness in legs -Muscles weakness spread to hands and trunk - can also see
Guillain-Barre syndrome Bilateral facial paralysis no sensory loss
594
Slowly adapting vs fast adapting
Slowly adapting - sends a continuous electric signal thoughout stimulus - Merkel, Ruffini Fast adapting - sends a electrical signal only at the beginning and end of continuous stimulus - Meissner, Pacinian
595
Bilateral facial muscle paralysis or weakness
Guillain-Barre | Lyme disease with Bells palsy
596
``` Abrupt onset of pain HA at the eyebrow Colored halos Rainbows of light Red teary eye, hazy cornea, fixed Mid dilated pupil (not reactive to light) Firm to palpation ```
Acute angle-closure glaucoma Lens against iris
597
Patients complains of glare with vision | Eye sight has improved, was near sighted
Cataract
598
White or bluish ring on periphery of cornea Elderly -due to
Arcus senilis Accumulation of cholesterol
599
Clouding of cornea
Mucopolysaccharidosis (hurler syndrome) Deficient: alpha-L-iduronidase Accumulation: heparan sulfate and dermatan sulfate
600
Enlarged optic disc > 50% of optic cup
Glaucoma
601
Auditory pathway (10)
1) Tympanic membrane 2) Middle ear ossicles (malleus --> incus --> stapes 3) Hair cells 4) Spiral (cochlear ganglion) 5) Cochlear nuclei 6) C/L superior olivary nucleus 7) Lateral lemniscus 8) Inferior colliculus 9) Medial geniculate nucleus 10) Primary auditory cortex
602
Intermittent vertigo Tinnitus Hearing loss
Meniere disease Imbalance of fluid and electrolyte composition of the endolymph
603
Acute otitis externa organisms
S. aureus | Pseudomonas aeruginosa
604
Acute otitis media organisms
S. pneumonia Nontypable Haemophilius influenzae M. Catrrhalis
605
Facial angiofibromas Ash-leaf spots of depigmentation Hx of seizures Intellectual disability What neoplasms is this patient at an increased risk of developing
Tuberous sclerosis Cardiac rhabdomyoma Astrocytomas Angiomyolipoma
606
Awake (open) Awake (closed) Non-REM stages REM stages
Awake (open): beta waves Awake (closed): alpha Stage N1: Theta waves Stage N2: Sleep spindles and K complexes - Bruxism (teeth grinding) Stage N3: Delta waves - Sleepwalking, night terror, bedwetting (enuresis) REM: Beta waves
607
Upper motor neuron lesion classic signs LMN classic signs
UMN - Spastic paralysis - Hyperreflexia LMN - Flaccid paralysis - Hyporeflexia - Atrophy - Fasciculations
608
What can be damaged with parotid gland surgery
Facial n. | Runs through parotid gland
609
Common infectious organism of tonsils and adenoids Common infectious organism of salivary gland
S. pyogenes S. aureus, viridans group streptococci
610
Tumor of parotid gland
Pleomorphic adenomas | benign
611
Child Recurrent staphylococcus abscesses Neutrophils fail to respond because chemotactic stimuli are deficient
Leukocyte adhesion deficiency Hyperimmunoglobulin E syndrome
612
What upper GI problem associated with findings 1) Biopsy of pt with esophagitis reveals large pink intranuclear inclusions and host cells chromatin that is pushed to the nucleus
HSV esophagitis
613
What upper GI problem associated with findings Biopsy of a patient with esophagitis reveals enlarged cells, intranuclear and cytoplasmic inclusions, and a clear perinuclear halo
CMV esophagitis
614
An esophageal biopsy reveals a lack of ganglion cells between the inner and outer muscular layers
Achalasia
615
Protrusion of the mucosa in the upper esophagus
Plummer-vinson syndrome
616
Outpouching of the esophagus found just above the LES
Epiphrenic diverticulum
617
Goblet cells seen in the distal esophagus
Barrett esophagus
618
A PAS stain on a biopsy obtained from a patient with esophagitis reveals hyphate orgnaisms
Candida esophagitis
619
Esophageal pouch found in the upper esophagus
Zenker diverticulum
620
Dark urine Clay stools Jaundice - due to
Extrahepatic biliary atresia Incomplete recanalization of the bile duct during development of the bile duct
621
Non-bilious vomiting Shortly after birth Constricting ring around duodenum -due to
Annual pancreas Failure of ventral pancreatic bud to rotate properly
622
Mega-esophagus | Cardiomegaly
Chagas disease | Trypanosoma cruzi infection
623
Dysphagia Glossitis And ____
Plummer-vinson Iron deficiency anemia
624
Directly inhibits parietal cells from generating acid
Somatostatin
625
Gastrin secreted by Secretion stimulated by
G cells of antrum Phenylalanine Tryptophan Calcium
626
Stomach biopsy reveals neutrophils above the basement membrane, loss of surface epithelium and fibrin-containing purulent exudate
Acute gastritis
627
What is seen in chronic gastritis
Stomach biopsy reveals lymphoid aggregates in the lamina propria, columnar absorptive cells and atrophy of the glandular structures
628
Spiral shaped bacteria
H. pylori
629
A 54 y.o male presents with abdominal pain, diarrhea, bilateral lower extremity edema and 5 lb weight loss over last 3 months. An esophagogastroduodenoscopy is performed and cerebriform rugae are observed throughout the body of the stomach. A biopsy of the region shows an abundance of mucous cells forming glands in the shape of corkscrews, but very few parietal and chief cells. most likely diagnosis is - increased risk of
Menetrier disease Gastric adenocarcinoma
630
Menetrier disease 5 things
1. Hypertrophy of mucous producing cells, rugae of stomach hypertrophy look like gyri 2. Atrophy of parietal cells 3. Decrease gastric acid production 4. Enteric protein loss --> hypoalbuminemia --> edema 5. Increase risk of gastric adenocarcinoma
631
Signet ring cell in ovary
Krukenberg tumor gastric cancer metasisi to ovary
632
Non bilious projectile vomiting | Palpable oliver mass in epigastric
Pyloric stenosis - pylorus is hypertrophied - narrowing of gastric outlet
633
What enzymes do obligate anaerobes lack
Catalse | Superoxide dismutase
634
WHich mycobacterium species fits each 1) Causes leprosy 2) Causes pulmonary TB-like symptoms in COPD patients 3) Causes cervical lymphadenitis in children 4) Cuases a disseminated disease in AIDS patients 5) Associated iwth hand infection in aquarium handlers
1) Myco. leprae 2) Myco. kansasii 3) Myco. scrofulaceum 4) Myco. avium intracellulure (MAI) Myco. avium complex (MAC) 5) Myco. Marinum
635
Stimulates gallbladder contraction Faciliates pancreatic HCO3 secretion Increase insulin release Inhibits secretion of all GI hormones Relaxes smooth muscle and sphincter through out GI Produces peristaltic waves
Cholecystokinin (CKK) - I cells - Gall bladder contraction, decrease gastric emptying, increase pancreatic secretion Secretin - S cells - Neutralize gastric acid - Decrease gastric acid production Gastric inhibitory peptide (GIP) - K cells - Decrease gastric acid production - Increase insulin release Somatostatin - D cells in GI tract - delta cells in pancreas Vasoactive intestinal peptide (VIP) - Relaxes smooth m and sphincter - Increase secretion of electrolytes and water - Copious watery diarrhea Motillin
636
Hepatoduodenal ligament contains
Portal triad - Hepatic artery - portal vein - common bile duct
637
Retroperitoneal
``` SAD PUCKER Suprarenal (adrenal) glands Aorta Duodenum (2nd-->4th part) Pancreas (not tail) Ureters Colon (ascending and descending) Kidney Esophagus (lower 2/3) Rectum ```
638
Four obligate aerobic bacteria
Nagging Pest Must Breathe Nocardia Pseudomonas aeruginosa Mycobacterium tuberculosis Bacillus
639
Carbohydrate breakdown and transport
Carbs - immediately: salivary amylase in mouth Carbs --> disaccharides (pancreatic amylase) Disaccharides --> monosaccharides (intestinal brush border) Sodium dependent transport - GLucose - Galactose ``` Faciliate diffusion (GLUT5) - Fructose ```
640
Protein breakdown and transport
Protein --> Amino acids & dipeptides, tripeptides (pancreatic proteases) Sodium dependent transport - Amino acids Hydrogen gradient - dipeptides, tripeptides
641
Lipid breakdown and transport
Lipids --> triacylglycerol ( salivary lipase, stomach) Triacylglycerol --> Fatty acids, 2-monoacylglycerol ( Pancreatic lipase) Bile acids emulsify hydrolyzed product Forms micelles --> Triglycerides, cholesterol Gets balled up into chylomicrons, transported to liver
642
Location of absorption Iron Folate Vit B12
Duodenum Duodenum and jejunum Terminal ileum
643
Bite cell
G6PD deficiency Due to removal of Heinz bodies by macrophages in the spleen
644
``` Deficiency ApoB-48, ApoB-100 Malabsorptive Steatorrhea Failure to thrive Ataxia acanthocyes "spur cell" on microscope Lipid-laden enterocytes ``` - inheritence - mutation - missing - later manifestations - tx
Abetalipoproteinemia AR Mutation in gene that encodes for microsomal transfer protein (MTP) Chylomicrons, VLDL and LDL absent Retinitis pigmentosa progressive ataxia acanthocytosis Restriction of long chain fatty acids Large doses oral Vit E
645
``` Defective ApoB-100 Increased LDL, cholesterol, VLDL Premature atherosclerosis Tendon xanthomas Xanthelasmas ```
Familial hypercholesterolemia - AD - Absent LDL receptors Defective LDL particle uptake by hepatocytes
646
``` Deficiency ApoC-2 Increased chylomicrons Acute pancreatitis Lipemia retinalis Eruptive xanthomas ```
Familial chylomicronemia AR Creamy white retinal blood vessels Localized lipid deposits in skin, red bumps
647
Deficiency ApoE Premature atherosclerosis Lipid droplets in palms of hand Lipid laden macrophages What is impaired
Familial dysbetalipoprotenemia Chylomicron remnant uptake by liver cells Increase chylomicron and VLDL remnants
648
Celiac disease Ab to
Gliadin | Tissue transglutaminase
649
``` Weight loss Lymphadenopathy Hyperpigmentation Cardiac symptoms Arthralgias Neurlogic symptoms PAS+ Foamy macrophages in lamina propria ```
``` Whippe disease Tropheryma whipplei (Gram +) ``` Penicillin Ampicillin Tetracycline
650
Small intestinal mucosa laden with distended macrophages in the lamina propria
Whipple disease
651
Horrible abdominal pain Mild tenderness to palpation Dilated loops of bowel Bowel wall thickening
Intestinal ischemia
652
Blood supply proximal to pectinate line Blood supply distal to pectinate line
Superior rectal artery from inferior mesenteric artery Inferior rectal artery (br. of pudendal a.)
653
Peutz-Jeghers syndrome
AD Multiple benign hamartomas in GI tract Hyperpigmented areas on lips, mouth, hands genitalia Increase risk of malignancy
654
Colon cancer APC pathway
``` Loss of APC gene K-RAS mutation Loss of tumor suppressor genes -p53 - DCC (chr 18q) ```
655
colon cancer microsatellite pathway
Loss common HNPCC (lynch) Dysfunction of DNA mismatch repair enzymes
656
LOTs of polyps
Familial adenomatous polyposis | AD mutation of the APC gene
657
Gardner syndrome (4)
Familial adenomatous polyposis Bone and soft tissue tumors Lipomas Retinal hyperplasia
658
Lynch syndrome
HNPCC Nonpolyposis colorectal cancer AD Proximal colon cancer
659
Patient with urinary tract infection caused by Proteus vulgaris is at risk for what type of renal stone
Ammoniummagnesium phosphate stones (Staghorn calculi)
660
Chlamydophila psittaci Chlamydophila pneumoniae Chlamydia trachomatis - A, B, C - D-K - L1 L2 L3
Chlamydophila psittaci - bird exposure - pneumonia Chlamydophila pneumoniae - interstitial pneumoniae - walking pneumonia Chlamydia trachomatis - A, B, C (trachoma eye infxn) - D-K (PID, urethritis, conjunctivitis) - L1 L2 L3 (lymphogranuloma venerum, primary ulcer and LAD)
661
Ventral pancreatic bud --> Dorsal pancreatic bud -->
Ventral --> uncinate process, part of head of pancreas, proximal portion of main pancreatic duct Dorsal --> Body and tail, most of head (superior aspect) , small accessory pancreatic duct
662
What tells the pancreas to start secreting
CCK Vagus n. Secretin
663
Pancreatitis causes
``` "Pancreatitis" hyperParathyroidism** Alcohol** Neoplasm Choleithiasis** Rx (drugs)** ERCP** Abdominal surgery hyperTriglyceridemia ** Infection Trauma Idiopathic Scorpion sting ```
664
Sit wont sit down Severe pain radiates to back What will be elevated
Acute pancreatitis Increased serum lipase
665
Painless jaundice -markers
Pancreatic adenocarcinoma CD19-9 CEA
666
Trousseua syndrome
Hypercoagulability Venous thrombosis Migratory thrombophlebitis Associated with pancreatic cancer
667
A 59-year-old man with no prior medical history presents to the physician with marked hyperglycemia, diarrhea, and weight loss. A CT scan of the abdomen reveals a pancreatic mass. A trial period on an oral hypoglycemic agent has not helped reduce his glucose levels. His physical examination is signifi cant for the rash shown in the image. Which of the following is the most likely diagnosis?
Glucagonoma Tx Octreotide
668
Pancreatic mass Increased insulin Hypoglycemia Elevated C- peptide
Insulinoma
669
``` Pancreatic mass Hyperglycemia Steatorrhea Gallstones Decrease hydrocholic acid (achlorhydria) ```
Somatostatinoma
670
Young child has recurrent lung infections and granulomatous lesions. This is most likely due to what underlyign defect in neutrophils
Chronic granulomatous disease Deficiency of NADPH oxidase
671
Spore forming bacteria
Bacillus anthracis Bacillus cereus Clostridum spp (perfringens, tetani, botulinum, difficle) Coxiella burnetti
672
6 month old gets flaccid paralysis after eating honey. Organism? MOA?
Clostridum botulinum Inhibits release of acetylcholine
673
``` Jaundice male neonate Lethargic Icterus of skin and sclerae Muscle tone decreased Liver no enlarged Total bilirubin high Normal direct bilirubin ``` Phenobarbital does not lower bilirubin levels
Unconjugated hyperbilirubinemia Crigler-najjar Syn (type I)
674
hCG stimulates corpus luteum to produce progesterone until placenta takes over at
8 weeks
675
Adult vs fetal hemoglobin
Adult - Two alpha - two beta Fetal - two alpha - two gamma
676
What allows bilirubin to be taken out of blood
UDP-glucuronyl transferase
677
A healthy 25 y.o man comes in to routine examination. His lab tests show a serum bilirubin level of 4 mg/dL and a direct bilirubin of 0.3 mg/dL. Liver fxn tests are nromal. Which of hte following explains the serum and indirect bilirubin levels
B. Glucuronosyltransferase deficiency Gilbert
678
Phenobarbital in Crigler Najjar
Phenobarbital Type II have decrease in bilirubin Type I no change
679
New born presents with kernicterus, jaundice adn elevation of serum unconjugated bilirubin. Patient dies after 1st birthday. Most likely had a deficiency of?
Uridine diphosphate-glucuronsltransferase Crigler-Najjar tyep I
680
Autopsy of patient has black liver
Dubin Johnson Increase direct bilirubin
681
Increased direct bilirubin | no black liver
Rotor syndrome
682
Encapsulated bacteria? Test for encapsulated bacteria
Even Some Pretty Nasty Killers Have Shiny Bodies ``` E.coli Strep pneu. Pseudomonas Neisseria meningitis Klebsiella H. influenza Salmonella B Group B strep ``` Quellung rxn
683
A 39-year-old white woman who suffers from polycythemia vera presents to the clinic complaining of severe and constant right upper quadrant pain over the past 2 days. Physical examination reveals an enlarged liver. What other finding would most likely be seen at presentation? (A) Ascites (B) Asterixis (C) Esophageal varices (D) Hyperpigmented skin (E) Spider angiomata
A. Ascites Budd-chiari
684
A 34-year-old woman who is at 26 weeks of gestation and who has a history of multiple spontaneous abortions presents with severe abdominal pain, jaundice, ascites, and mental status change. Ultrasonography reveals an obscure hepatic venous connection to the inferior vena cava and absence of any waveform in the hepatic veins. She has a positive serum antiphospholipid antibody titer. Which of the following is the most likely diagnosis? (A) Budd-Chiari syndrome (B) Congestive heart failure (C) Polymyalgia rheumatica (D) Portal vein thrombosis (E) Veno-occlusive disease
A. Budd-chiari syndrome Complete obstruction to blood flow by acute clot in hepatic veins or inferior vena cava Hepatomegaly Pain Ascites Jaundice
685
A 57-year-old white man presents to his primary care physician with dyspnea. He says that he likes to maintain his yard and garden, but that he has recently had trouble doing the work, and becomes short of breath even walking up the one fl ight of stairs in his house. On further questioning, he says that sometimes he wakes up short of breath in the middle of the night. Physical examination demonstrates pitting ankle edema. Which of the following fi ndings would also be expected in this patient? (A) Decreased sympathetic outfl ow (B) Decreased venous pressure (C) Increased aldosterone secretion (D) Increased effective arterial blood volume (E) Increased glomerular fi ltration rate
C. increased aldosterone secretion CHF
686
A 26-year-old man presents to the clinic with bradykinesia, rigidity, and resting tremor. Serum aminotransferase levels are mildly elevated. A liver biopsy is shown in the image. What is the chance that this patient’s sister will have the same condition?
B. 25% Wilsons disease
687
A 19-year-old woman is bothered by a tremor at rest, which becomes progressively worse over the next 6 months. She exhibits paranoid ideation with auditory hallucinations and is diagnosed with an acute psychosis. On physical examination, she has scleral icterus. A slit lamp examination shows corneal Kayser-Fleischer rings. Laboratory findings include total serum protein, 5.9 g/dL; albumin, 3.1 g/dL; total bilirubin, 4.9 mg/dL; direct bilirubin, 3.1 mg/dL; AST, 128 U/L; ALT, 157 U/L; and alkaline phosphatase, 56 U/L. Which of the following additional serologic test findings is most likely to be reported in this patient? A Decreased α1-antitrypsin level B Decreased ceruloplasmin level C Increased α-fetoprotein level D Increased ferritin level E Positive antimitochondrial antibody F Positive HbsAg
B. Decreased ceruloplasmin level Wilson disease
688
``` Cirrhosis Diabetes Hyperpigmentation CHF Testicular atrophy ```
"Bronze diabetes" Hemochromatosis INcreased risk of HCC AR disease - HFE gene --> makes hepcidin Increased ferritin
689
Lung and liver disease
Alpha 1 antitrypsin disease AD Impaired ability to protect organs for elastase
690
What three bacteria are obligate intracellular bacteria
Rickettsia Coxiella Chlamydia
691
Hepatitis A - type - genus - family - transmission - infection type - signs
ssRNA virus non-enveloped Hepatovirus Picronavirus Fecal/oral, poor sanitation Acute Fever, vomiting, jaundice, RUQ pain
692
Hepatitis E - type - genus - family - transmission - infection type - More likely to cause fulminant hepatic failure in
ssRNA Hepevirdae Orthohepevirus Fecal/oral route, contaminated water Acute Pregnant women
693
Hepatitis B - type - genus - family - transmission - infection type - Tx
ds DNA Orthohepadnavirus Hepadnaviridae Perinatally, sexual contact, blood Chronic Tenofovir
694
Hepatitis D - type - genus - family - transmission - infection type - feature - Tx
ssRNA deltavirdae Blood, sexual contact Infects w/ hepatitis B Chronic Pegylated IFN-alpha
695
Hepatitis C - type - genus - family - transmission - infection type - Tx
ssRNA hepacivirus Flavivirdae Blood, sexual contact Chronic Ledipasvir-sofosbuvir Ombitasvir-paritaprevir-ritonavir + dasabuvir
696
ANA | anti-smooth muscle Ab
Autoimmune hepatitis | Type 1
697
Anti-liver-kidney microsomal Ab | Anti-liver cytosol antibody
Autoimmune hepatitis | Type 2
698
Coarse facial features Abscesses Eczema
Hyper IgE syndrome T cell
699
Thrombocytopenia Purpura Infections Eczema
Wiskott aldrich B cell disorder
700
Delayed separation of umbilicus
Leukocyte adhesion deficiency
701
Neurologic defects, partial albinism | recurrent infections
Chediak-Higashi syndrome
702
P450 enzymes in the liver are found in what zone of hepatic lobule
Zone 3
703
Beads on a string + pANCA - due to - tx
Primary sclerosing cholangitis (PSC) COncentric fibrosis of bile ducts liver transplant
704
A 43-year-old man with a 20-year history of ulcerative colitis presents to the physician with complaints of worsening bloody diarrhea, progressive fatigue, pruritus, visual disturbances, and arthralgias. On physical examination, he is found to have icteric sclera, fi nger clubbing, and several small ulcerations with necrotic edges on both legs. Endoscopic retrograde cholangiopancreatography (ERCP) shows alternating strictures and dilations of the bile ducts. Which of the following conditions is consistent with these ERCP fi ndings? (A) Cholelithiasis (B) Pancreatic carcinoma (C) Primary biliary cirrhosis (D) Primary hemochromatosis (E) Primary sclerosing cholangitis
(E) Primary sclerosing cholangitis
705
25. A 46-year-old white woman with rheumatoid arthritis presents with severe pruritus. She denies any history of alcohol or drug use. On physical examination, she is found to have icteric sclera, palpebral xanthomas, and hepatomegaly. She tests positive for antimitochondrial antibody and increased alkaline phosphatase activity. Which of the following is most likely responsible for this patient’s presentation? (A) Destruction of intrahepatic bile ducts (B) Hepatic parenchymal destruction (C) Obstruction of extrahepatic bile ducts (D) Portal vein thrombosis (E) Stenosis of extrahepatic and intrahepatic bile ducts
(A) Destruction of intrahepatic bile ducts The triad of jaundice (icteric sclera), hypercholesterolemia (palpebral xanthomas), and pruritus with positive antimitochondrial antibody titers and elevated alkaline phosphatase activity is classic for primary biliary cirrhosis. Primary biliary cirrhosis is a cholestatic disease with chronic, progressive, and often fatal liver injury characterized by the destruction of medium-sized intrahepatic bile ducts with eventual liver failure. Liver transplantation is the defi nitive treatment.
706
25. A 46-year-old white woman with rheumatoid arthritis presents with severe pruritus. She denies any history of alcohol or drug use. On physical examination, she is found to have icteric sclera, palpebral xanthomas, and hepatomegaly. She tests positive for antimitochondrial antibody and increased alkaline phosphatase activity. Which of the following is most likely responsible for this patient’s presentation? (A) Destruction of intrahepatic bile ducts (B) Hepatic parenchymal destruction (C) Obstruction of extrahepatic bile ducts (D) Portal vein thrombosis (E) Stenosis of extrahepatic and intrahepatic bile ducts
(A) Destruction of intrahepatic bile ducts The triad of jaundice (icteric sclera), hypercholesterolemia (palpebral xanthomas), and pruritus with positive antimitochondrial antibody titers and elevated alkaline phosphatase activity is classic for primary biliary cirrhosis. Primary biliary cirrhosis is a cholestatic disease with chronic, progressive, and often fatal liver injury characterized by the destruction of medium-sized intrahepatic bile ducts with eventual liver failure. Liver transplantation is the defi nitive treatment.
707
A 42-year-old woman comes to the physician with severe itching for the past 4 days. Her physical examination is signifi cant for hepatomegaly and three xanthomas on her right lower extremity. Laboratory studies reveal a normal total bilirubin level and elevated serum cholesterol and alkaline phosphatase levels. A liver biopsy shows granulomatous destruction of medium- sized intrahepatic bile ducts. Which of the following autoantibodies is most likely to be signifi cantly elevated in this patient? (A) Anticentromere antibodies (B) Antihistone antibodies (C) Antimitochondrial antibodies (D) Antinuclear antibodies (E) Rheumatoid factor
C. anti mitochondrial ab primary biliary cholangitis (cirrhosis)
708
Granulomas Cirrhosis Middle age female Anti-mitochondrial antibody - due to - tx
Piarmy biliary cholangitis (PBC) Autoimmune T cells attack bile ductules within teh liver parenchyma Ursodiol (ursodeoxycholic acid) - bile acid that decreases the synthesis of cholesterol in the liver Liver transplant
709
Lysosomal storage disease - accumulation of CM2 ganglioside - associated with renal failure
Tay-Sach Fabry disease
710
Inhaled coin goes where
Right mainstem bronchus
711
Physiologic dead space equation
Vd= Vt x (PaCO2- PeCO2)/ PaCO2 ``` Vd= dead space Vt= tidal volume PaCO2= Pco2 arterial PeCO2= Pco2 expired air ```
712
Pulmonary vascular resistance equations P= R=
P= Q x R Q= perfusion ``` R= 8 nl/ #R^4 n= viscosity l= length of vessel r= radius #= pie ```
713
Beta 2 stimulation
INcrease radius | Decrease resistance
714
Moves oxygen hemoglobin dissociation curve right (5)
``` Co2 Acidosis Increase 2,3 DPG Increase exercise Increase temp ```
715
Form of hemoglobin that has low affinity for oxygen - what favors this form
Taut (T form) Favors tissues, give up oxygen readily Favor the T form - Co2 - H - Increased temp - 2,3 DPG
716
Hemoglobin with high affinity for oxygen
R form (relaxed)
717
Methemoglobin - form - affinity - causes - caused by - tx
Ferric form (3+) of iron Decreased affinity for oxygen Increased affinity for cyanide --> Tissue hypoxia ``` Nitrates and nitrites Antimalarial drugs (Chloroquine, primaquine) Dapsone Sulfonamide Local anesthetics (lidocaine) Metoclopramide ``` Tx - Methylene blue - Vit C - Cimetidine (gradually lowers)
718
Which immunodeficiency matches 1) Neutrophils fail to respond to chemotactic stimuli 2) Adenosine deaminase deficiency 3) Failure of endodermal development 4) Defective tyrosine kinase gene 5) Associated wth high levels of IgE and eosinophils
1) Leukocyte adhesion deficiency 2) Hyper IGE syndrome 3) SCID 4) Bruton agammaglobulienmia 5) Hyper IgE syndrome
719
What occurs in COPD with oxygen levels
Decrease arterial PO2 Physiologic shunt Decrease O2 extraction ration Decreases blood O2 content
720
Alveolar gas equation
PAo2= PIo2- PaCo2/ R PAo2= 150 - PaCO2/0.8
721
CAG - cause - inheritence - signs CGG - cause - inheritence - signs CTG - cause - inheritence - signs GAA - cause - ineritence - signs
Huntington disease - AD - - Fragile X syndrome - gene hypermethylation - X linked dominant - Protruding chin, giant gonads Myotonic dystrophy - AD - Cataracts, Toupee (early balding) , gonadal atrophy Friedreich ataxia - AR - Ataxic gait
722
Defects in DNA mismatch repair
Lynch syndrome increased risk of hereditary non-polyposis colorectal cancer (HNPCC)
723
``` Headache fever inattentiveness Chemotherapy Increased opening pressure Increased protein, low glucose Image: Cds ``` Tx.
Cryptococal meningoencephalitis Initial: Amphotericin B Flucytosine Long term maintenance therapy: Fluconazole
724
Left ventricular myocardial blood supply occurs when
During diastole Ventricle is relaxed
725
Intrapleural pressure when airway pressure is zero
-5 --> -7.5 with inspiration
726
Manic vs hypomanic
Manic - severe - 1 week - May have psychotic features Hypomanic - >= 4 consecutive days
727
Bipolar I Bipolar II Cyclothymic disorder
Bipolar 1 - Manic episode - Depressive episode common Bipolar 2 - Hypomanic - >1 major depressive episode Cyclothymic - >2 years fluctuating, mild hypomanic, depressive symptoms
728
1) Parasite eggs in stool 2) Perianal egg deposition 3) Proglottids in the stool 4) Rhabdoitiform larvae in the stool 5) Trophozoites and cysts in the stool
1) Schistosoma mansoni or S. japonicum 2) Enterobius vermicularis (pinworms) 3) Intestinal tape worms (Taenia solium, T saginata, Diphyllobothrium latum) 4) Strongyloides stercoralis 5) Protozoal infections (Giardia lamblia, entamoeba histolyticsa)
729
``` Conjunctiva pallor SOB Constipation Decreased concentration Microcytic anemia - Decreased MCV ``` What do you see on peripheral smear?
Lead poisoning Basophilic stippling
730
1) Heinz bodies 2) Howell-Jolly bodies 3) RIng sideroblasts 4) Schistocytes 5) Basophilic stippling
1) Inclusions of denatured hemoglobin after Oxidant stress in pts w/ G6PD deficiency 2) DNA remnant incusion in peripheral RBC taht are normal removed by spleen, indicates decreased or absent splenic function 3) Formed from precipitation of iron granules in developing erythrocytes due to defect in heme synthesis pathway (See in bone marrow no peripheral smear) 4) Are fragments of RBC formed from mechanical hemolysis, hemolytic uremic syndrome, thrombocytopenic purpura, and disseminated intravascular coagulation
731
Lactose deficiency see what in intestine
Normal
732
Tennis elbow | - muscle
Lateral epicondylitis Wrist extension Extensor carpi radialis brevis
733
Prophylactic medication Jaundice Dark urine RBC w/ dark inclusions -inheritence pattern?
G6PD deficiency Heinz bodies X recessive
734
Relative erythrocytosis
aka Relative Polycythemia Normal RBC mass Decrease in plasma volume
735
``` Fever Headache Nuchal rigidity Normal glucose elevated protein ```
Aseptic meningitis Enteroviruses - coxsackievirus - echovirus - poliovirus
736
Ovarian cancer risk factors
Repeat ovulation | - nulliparity
737
Failure to thrive Developmental delay Cant lift head Orotic acid crystals Supplementation of what is needed?
Hereditary orotic aciduria - AR - disorder of pyrimidine synthesis - defect in uridine 5- monophosphate (UMP) synthase Supplement Uridine
738
Ascorbic acid
Vit C
739
Posterior left 12th rib fracture injury Anterior right rib 8-11 fracture Anterior left ribs 8-11 fracture Fracture ribs 1-6
L kidney R liver Spleen visceral pleural of lungs
740
Changes what gene expression 1) 14;18 2) 9;22 3) 8;14 4) Breast cancer
1) Follicular lymphoma - Bcl-2 overexpression 2) CML - Bcr-abl 3) Burkitt lymphoma - C-myc overexpression 4) Erb-B2 overexpression, HER2
741
Increased hemoglobin=
Erythrocytosis Excess erythropoietin porduction
742
1) Linear IgG and C3 deposits on IF microscopy 2) Glomerular basement membrane splitting 3) Glomerular basement fibrin deposition 4) Subepithelial humps on electron microscopy 5) Diffuse capillary wall thickening on light microscopy 6) Glomerular crescents on LM 7) C3 granular straining along GBM
1) Anti-GBM disease 2) Membranoproliferative glomerulonephritis (MPGN) and Alport syndrome 3) Rapidly progressive glomerulonephritis (anti-gbm) 4) Poststreptococcal glomerulonephritis 5) Membranous glomerulopathy 6) Rapidly progressive glomerulonephritis, Anti- GBM disease 7) Poststreptococcal glomerulonephritis
743
Metabolic vs respiratory compensation
Metabolic acidosis - Decrease pH - Decrease PCo2 - Decrease HCO2 Respiratory acidosis - Decrease pH - Increase PCO2 - Increase HCo2 PCO2= 1.5 [HCO3] + 8 (+-2)
744
Rapid onset sepsis Altered consciousness Organ failure Petechial/ ecchymotic rash virulence factor
N. meningitidis Lipo-oligosaccharide (LOS)
745
Reaction rate in skeletal muscles is dependent on ____ which is activated by
Phosphorylase kinase (PK) Ca
746
Skeletal pain Deformity (bowing of legs) Focal warmth
Paget disease
747
How HIV attaches
Attaches to host cells using the viral surface glycoprotein gp120 This glycoprotein bins to teh CD4 as the primary receptor and teh chemokine receptor CC45 as the coreceptor Conformation change in gp120 exposes teh underlying transmembrane glycoprotien gp41 which mediates viral fusion
748
Nef | tat
HIV regulatory genes Nef: downregulates teh expression of class I major histocompatibility complex proteins on teh surface of infected cells, limits immune recognition by cytotoxic T cells tat: is a transcriptional activator that promotes viral gene expression
749
(+) for synaptophysin Chromogranin neuron-specific enolase
Small cell carcinoma of the lung | Carcinoid tumor
750
Temporal hemorrhage affects what nerve
Herniation CN III
751
Missouri caves
Histoplasma capsulatum Macropahges iwth intracellular small yeasts
752
1) Budding yeast with thick capsule 2) Macropahges with intracellular small yeast 3) Round spherules iwth multiple endospores 4) Septate hyphae with dichotomous branching 5) Yeasts with pseudohyphae and blastoconidia
1) Cryptococcus neoformans - bird droppings - immunocompromised - meningoencephalitis 2) Histoplasma capsulatum - Missouri caves - fever nonproductive cough 3) Coccidioides immitis - pulmonary disease 4) Aspergilus fumigatus - V shaped branching - AIDS 5) Candida species - immunocompromised
753
Pig farmer
Loeffler syndrome Ascaris infection Eosinophilic invasion due to parasitic infection
754
Most common cause of viral conjunctivitis
Adenovirus Ds non enveloped linear DNA virus
755
Listeria is acquired how
Contaminated food Unpasteurized milk Uncooked meats
756
Decrease in RPF causes what in GFR and FF
Decrease in GFR | Increase FF
757
Anti cholinergic effects
``` Hot as a hare Dry as a bone Red as a beet Blind as a bat Mad as a hatter Full as a flask Fast as a fiddle ```
758
Alpha 1 agonist effects
increase smooth muscle tone of iris dilator (mydriasis) Blood vessel constriction Vasoconstriction reduce nasal congestion
759
DNA binding protein
c-Jun c-Fos
760
Chalky white lesions in mesentery, fat cell destruction and calcium depositions Abdominal pain
Acute pancreatitis
761
Infection from puppy
Campylobacteri jejuni
762
Exertional shortness of breath Progressive dyspnea Lightheadness during exertion Tricuspid regurg
Pulmonary hypertension
763
Glucagon MOA
Increases hepatic glucose production via glycogenlysis and gluconeogenesis
764
What can you suffer from if to quick nitroprusside infusion tx
cyanide toxicity tx sulfur
765
Nissl substance
Normal axonal injury reaction
766
VSD sign
holosystolic murmur over left sternal border
767
Fundal varices
splenic vein thrombosis
768
COPD exacerbation due to
virus: rhinovirus, influenza virus, parainfluenza (most common) bacterial: h. influenza, moraxella catarrhalis, strep. pneumoniae
769
Hyperventilation results in
Decrease in arterial partial pressure of CO2
770
Wrinkles due to
Decrased collagen fibril production
771
Increase RBC | Intense itching after showering
Polycythemia vera Mutation Jak2 Hematopoietic cells more sensitive to growth factors Decrease EPO
772
Projection
Think wife is cheating cause they are
773
Displacement
Mad at boss yells at wife
774
Transference
See psychiatrist as parent
775
5 y.o difficulty breathing dysphagia drooling fever Swollen and cherry red epiglottis sign
H. influenzae type B thumb sign
776
Child sticking nose in air
Esophagitis | H. infuenzae type B
777
Cyst in brain Not IC Seizures
Taenia solium Pork tapeworm Feces contaminated food
778
Mutations in pol gene in HIV patient
Inconsistent use of antiretroviral--> antiretroviral resistance
779
Patient in shocks gets IV fluids causes
increase in preload Stretches myocardium Increases the end-diastolic sarcomere length
780
Levothyroxine supplement TSH <0.01 Risk
Too high supplement Atrial fibrillation
781
Copper is eliminated
hepatic excretion into bile
782
Corynebacterium diphtheriae is virulent how
phage conversion permitting exotoxin production
783
Permissive effect
One hormone allows another to exert it s maximal effect
784
``` Lethary confusion Persistent atrial fibrillation CHF Nauseas and decreased appetitie, vomiting Vision difficulties ``` Hyperkalemic
Digoxin toxicity
785
Nitrates MOA
Venodilators (large veins) Increase peripehral venous capacitance --> reducing cardiac preload and left ventricular end-diastolic pressure adn volumne
786
Release of NE from peripheral autonomic nerve terminals will effect
sympathetic output to the detrusor muscle Most organs Smooth muscle glands
787
``` Fever Lethargy urinalysis: positive for proteinuria and hematuria Decreased hemoglobin Elevated reticulocyte count Decreased platelet GI illness scattered petechial rash ``` renal biopsy
Thrombocytopenic thrombotic purpura-hemolytic uremic syndrome Platelet rich thrombi in glomeruli and arterioles
788
Crescent- shaped mass of cellular proliferation and leukocytes
RPGN macroscopic hematuria HTN Progressive renal failure Good pasture
789
Diffuse proliferation and subepithelial immunoglobulin deposits
Post streptococal glomerulonephritis
790
Mesangial IgA deposition and proliferation
Henoch-Schonlein purpura Palpable purpura arthritis
791
Patchy necrosis of tubular epithelium and loss of basement membrane
Acute tubular necrosis Rising creatinine Muddy brown granular casts on urinarlysis
792
RB mutation BRAF HER2 KRAS RET
RB: Retinoblastoma, osteosarcoma BRAF: Hairy cell luekemia, melanoma HER2: breast cancer KRAS: colorectal cancer, non-small cell lung cancer RET: MEN2
793
Calculate False positives Study enrolled 300 patients, 100 have a UTI by urinalysis. New dipstick test 70% sensitive and 90% specific
``` 0.9= D/ 200 TN= 180 ``` False positive= 200-180= 20
794
Diarrhea HIV patient Hemorrhagic nodules or polypoid masses Spindle cells
Kapsoi sarcoma | -HHV8
795
What can not be metabolized to pyruvate and cause more lactic acid if pyruvate dehydrogenase deficiency
Lysine Leucine Ketogenic AA
796
Myeloid cells
``` Myeloblast Metamyelocyte Band Neutrophils Basophils Eosinophils ```
797
UV damage
formation of abnromal covalent bonds between adjacent thymine or cytosine rediues (pyrimidien dimers)
798
Hemosiderin laden macrographes
Chronic passive lung congestion in setting of heart failure (left ventricular systolic dysfunction
799
Collagen synthesis occurs when
RER proline and lysine residues are hydroxyalted Vit C cofactor here
800
Internal hemorrhoid above pectinate line drain into External hemorrhoids drain
Superior rectal vein from inferior mesenteric vein External drain via inferior rectal vein into internal pudendal vein which communicates with internal iliac veins
801
Branches of deep external pudendal artery supply the External iilac vein receives drainage from Left colic artery branches off, supplies The superior vesical artery supplies
Supply the scrotum and labia majora External iliac vein receives drainage from the inferior epigastric and deep circumflex iliac veins, serve the anterior abdominal wall and iliac crest respectively Branches of inferior mesenteric a. to supply the transverse and descending colon portions of the urinary bladder and ductus deferens
802
Complications of herpes zoster
long term residual pain postherpetic neuralgia
803
Beta 1 receptor found
Cardiac tissue Renal JC cells Not vascular smooth muscle Nonselective beta blocker who affect all three
804
Vit B12 deficiency Type 1 DM Hashimoto thyroiditis
Pernicious anemia CD4 mediated immune response against parietal cells Loss of parietal cells mass - decrease in intrinsic factor - decreased secretion of hydrochloric acid Elevated pH that stimulates gastrin secretion
805
Tx for alcohol wanting to quit with help
Naltrexone
806
Apolipoprotein E4 allele
At risk Alz disease
807
Oval budding yeast forms germ tubes at 37 C
``` Candida albicans (true hyphae at 37) ``` Normal commensal of skin and GI
808
Small ovoid yeast in lungs
Histoplasma capsulatum - dimorphic - inhaled as microconidia - pneumonia
809
Noncardiogenic pulmonary edema can be distinguished from cardiogenic pulmonary edema by a
normal pulmonary capillary wedge pressure
810
Pancreatitis SOB adn hypoxemia New bilateral opacities
Acute respiratory distress syndrome Indirect pulmonary insults cause excessive cytokine release leading to endothelial activation, neutrophilic migration to the lungs, degranulation with release of toxic mediators Increase capilalry permeability Formation of hyalien membrane
811
Exercise effect on PaO2, PaCO2, CO and HR
Increase HR and CO No change PaO2, PaCO2
812
Crescent formation 3 types
Rapidly progressive glomerulonephritis (RPGN) 1) Antiglomerular basement membrane 2) Immune complex RPGN - post-streptococcal glomerulonephritis, SLE, IGA, henoch schonlein purupra - decrease C3 C4 3) Pauci-immune RPGN - no immunoglobulin or complement deposits - elevated antineurtrophil cytoplasmic antibodies (ANCA)
813
``` SOB Cough Exacerbated when lay down MI Bilateral crackles at lung base S3 ```
Left sided heart failure --> elevation of end-diastolic pressure --> decrease lung compliance
814
zollinger-Ellison syndrome associated with also at risk for
MEN 1 ``` primary hyperparathyroidism ( hypercalcemia, constipation, kidney stones) Recurrent nephrolithiasis ``` Pituitary adenomas
815
Calcifications in epigastric area abdominal pain Chronic diarrhea Consumes alcohol
Chronic pancreatitis Digestive enzyme deficiency --> diarrhea
816
Vomiting/ nausea due to
``` GI irritation (infections chemotherapy, distention) - increase serotonin release and activation of 5HT ``` ``` Central nausea (migraines) - use dopamine receptor antagonist to stop ``` Vestibular nausea - dopamine receptor stimulation and H1 stimulation - promethazine tx both
817
Seen in hashimoto on biopsy
Intense lymphoplasmacytic infiltrate with active germinal centers Hurthle cells
818
Thyroid biopsy 1) Branching papillae with cells containing empty -appearing nuclei 2) Dense fibrous tissue extending beyond the thyroid capsule 3) Follicular hyperplasia with tall cells projecting into the follicular lumen 4) Intense lymphoplasmacytic infiltrate with active germinal centers 5) widespread inflammatory infiltrates, giant cellls, and disrupted follicles
1) Papillary thyroid cancer - orphan annie eye 2) Reidel thyroiditis 3) Graves disease 4) Hashimoto 5) Subacute granulomatous (de Quervain) thyroiditis
819
Ureters anterior to
Internal iliac artery
820
Drugs with pt fever, joint pains, rash of face and trunk, Positve anti-nuclear ab
Drug induced lupus ``` SHIPP Sulfonamides Hydralazine Isoniazid Phenytoin Procainamide ```
821
Pencillin binding proteins
Transpeptidases
822
Smokers have 5x the risk of esopahgeal carcinoma compared to non smokers (relative risk = 5.0, 95% CI 2.9-7.1) What percentage of SCC of the esophagus in smokers can be attributed to smoking?
Attributable risk percentage in exposed (ARP) ARP exposed= 100 x (risk in exposed - risk in unexposed)/ risk in exposed ARP= 100 x [(RR-1)/ RR] ARP= 100 x [(5-1)/5]= 80%
823
Ionizing radiaton tx for cancer does what
DNA ds breaks free radical formation
824
What is released as O2 saturation curve moves up
protons CO2
825
Anal fissures occur where
Posterior midline distal to dentate line
826
Multiple ulcers Mucosal erosions Large cells iwth basophili cintranuclear and intracytoplasmic inclusions
CMV | herpesvirus
827
Patent foramen ovale
wide and fixed splitting | no change with respiration
828
``` Headache Fever Malaise Cough Nodular infiltrates Clumping once room temp ```
Mycoplasma pneumoniae Walking pneumoniae Cross reactive IgM Activate complement --> erythrocyte lysis Cold agglutinins
829
``` Action potential conduction velocity Point 1- 0.05 Point 2 - 0.3 Point 3- 1.1 Point 4- 2.2 ``` Points correspond to what location
AV node Ventricular muscle Atrial muscle Purkinje system
830
Insulin effect
decrease glucagon secretion increase glycogen synthesis Increase glucose uptake
831
Cirrhosis Agitated and confused. Jerky irregular flexion-extension tremor in hands What is elevated in astrocytes
Hepatic encephalopathy Increased nitrogen, ammonia Ammonia + glutamate --> glutamine (non-neuroactive compond)
832
Demyeliantion of pons
overly rapid correction of electrolyte abnromalities
833
Neurodegenerative disease presents at birth as "floppy baby"
Werdnig Hoffman disease
834
Perfusion is greatest where in the lung -V/Q ratio Exercise effect of V/Q
Bases - V/Q <1 Exercise - V/Q moves down toward 1 - Vasodilation in apex - perfusion increases
835
V/Q --> zero V/Q --> infinity
V/Q --> zero - low ventilation, high perfusion - air way obstruction - pneumonia, pulmonary edema, lung cancer - supplemental O2 ineffective V/Q --> infinity - high ventilation, low perfusion - blood flow obstruction - supplemental O2 effective
836
Responses to exercise - O2 - Co2 - ventilation rate - V/Q in apex vs base - Pulmonary blood flow - pH - arterial PO2 - arterial pCO2 - Venous CO2 content
Increase O2 consumption Increase CO2 production Increase ventilation rate V/Q more uniform Increase pulmonary blood flow due to increase CO Decrease pH No change in arterial PO2 and PCO2 Increase venous CO2 content
837
Normal A-a gradient Causes of elevated A-a gradient
10-15 mmHg ``` High FiO2 Shunting o f blood Diffusion limitation (pulmonary fibrosis) V/Q mismatch Advanced age ``` Pressure alveolar oxygen to pressure arterial oxygen
838
Name the exotoxin 1) Causes scarlet fever 2) Inactivates EF-2 3) Blocks release of the inhibitory neurotransmitter glycine
``` 1) Erythogenic toxin Pyogenic toxins (s. pyogenes) ``` ``` 2) Diphtheria toxin Exotonin A (pseudomonas) ``` 3) Tetanospasmin (c. tetani)
839
Underlying cause of achalasia? How is it diagnosed?
Failure of LES to relax due to loss of myenteric (Auerbach) plexus due to loss of postganglionic inhibitory neurons ( which contain NO and VIP) Dysphagia solids and liquids ``` Diagnosis - Manometry (Uncoordinated or absent peristalsis) - Barium swallow (dilated esophagus with an area of distal stenosis ) (birds beak) ```
840
Clinical derangements that come about in a patient with acute mountain sickness
Headache Fatigue Extreme cases - Acute cerebral edema due to hypoxia induced vasodilation - acute pulmonary edema
841
Partial pressure of oxygen difference at sea level vs 20,000
Sea level= 760 mmHg 20,000= 349 mm Hg Partial pressure of oxygen is 21% of barometric pressure Partial pressure is higher at sea level
842
Response to high altitude (6)
Increase ventilation - have to breathe more - blow off more CO2 Increased erythropoietin - HCT 40% --> 64% - Hgb 15 --> 20 g/dL Increase 2,3 BPG - unloads oxygen to tissue better Increase mitochondria Increase efficiency of O2 utilization Increase renal excretion of bicarb
843
pH = Breathing more causes what pH change
HCO3/ pCO2 Respiratory alkalosis Decreases pCO2 --> raises pH
844
Physiologic changes that occur in chronic mountain sickness (6)
Increase RBC mass and hematocrit Increased blood viscosity and decrease tissue blood flow Elevated pulmonary artery pressure (pulmonary artery constricts in response to hypoxia) Right sided heart enlargement ( due to increase pulmonary artery pressure) Peripheral artery pressure falls Congestive heart failure
845
Positive G-force in which visual black out occurs Due to G force in space lift off
4-6 G Insufficient blood return to heart Insufficient pumping of blood to brain 8-9 G
846
Nitrogen narcosis
Nitrogen dissolves into neuronal membrane, which causes reduced neuronal excitability Diver becomes jovial/ careless Loss strength and coordination
847
Symptoms of decompression sickness
aka "Bends" Nitrogen bubbles undissolving from blood, make bubbles in blood vessels can occlude Joint/ muscle pains in arms and legs Neurologic problems (dizziness, paralysis, syncope) "The chokes (Bubbles occlude the lung capillaries --> SOB, pulmonary edema, death) ``` "BENDS" Breathign problems Extremity pain Neurologic changes Death ```
848
VIrchow triad for
DVT "SHE" Stasis - severely ill, paralyzed, long trips, cast Hypercoagulability - sickle cell, polycythemia, estrogen excess, cancer, pregnancy, OCPs, smoking Endothelial damage - vessel trauma, fracture, froeign body, bacterial infection
849
Homan's sign
Pain with ankle dorsiflexion DVT
850
``` Tall thin young male Smoker Uneven chest expansion Unilateral Chest pain Hyperresonance Dimished breath sounds ``` Due to
Primary spontaneous Pneumothorax Due to rupture of apical subpleural blebs or cysts
851
Location for needle for tension pneumothorax
Above 2nd rib
852
``` Pleuritic chest pain SOB Cough Tachypnea Tachycardia Low grade fever ``` Elevated D dimer Normal CXR Large A-a gradient ECG?
Pulmonary embolism (PE) ECG S1Q3T3 - deep S in lead I - large Q and inverted T in lead III
853
Elevated D-dimer
DVT | PE
854
The C's of huntington disease
``` Chorea Cuarenta (40) CAG repeats Chr Cuatro 4 Cognitive decline Caudate atrophy ```
855
FEV1/ FVC normal Obstructive restrictive
80% <80% obstructive > 80% restrictive
856
Hexagonal, double pointed, needle like crystals in bronchial secretions
Charcot-Leygen crystals - eosinophilic granules Asthma
857
Spiral shaped mucus plugs
Curschmann spirals Desquamated epithelium Asthma Obstructive lung disease
858
Pulsus paradoxus
Abnormal drop of 10 mm/Hg in systolic pressure during inspiration Decreased intrathoracic pressure --> increased right ventricle blood return Right ventricle push into left ventricle --> poor cardiac output and drop in systolic pressure Decreased capacity of LV ``` Cardiac tamponade Asthmatics Severe pulmonary embolism COPD Croup ```
859
Hyperplasia of both goblet cells and submucosa glands
Chronic bronchitis
860
Emphysema increased what
Compliance (loss of elastic fibers)
861
Right main stem bronchi same size Productive cough Increased sputum production FEV1/FVC <80% Causes (4)
Bronchiectasis Allergic bronchopulmonary aspergillosis Kartagener syndrome - sinus inversus Cystic fibrosis (most common) Primary ciliary dyskinesia
862
Hallmark of obstructive lung disease
FEV1/FVC= < 80% Difficulty getting air out Increased lung volumes
863
Hallmark of restrictive lung disease
FEV1/FVC= >80% Decreased lung volume Can not get air in
864
Reid index
Thickness/ glands/ thickness of wall Chronic bronchitis 50% composed of mucus glands elevated reid index= normal
865
What disease is associated with the Ab 1) Anti- mitochondrial 2) Anti- neutrophil cytoplasmic 3) Antiplatelet Ab
1) anti-mitochondrial Primary biliary cholangitis 2) Anti-neutrophil cytoplasmic cANCA - granulomatosis w/ polyangiitis pANCA - Eosinophilic granulomatosis with pollyangiitis (churg-strauss) - Microscopic polyangiitis - Pauci-immune crescentric glomerulonephritis - primary sclerosing cholangitis - ulcerative collitis 3) Immune thrombocytopenic purpura (ITP)
866
Female homolog? 1) Corpus spongiosum 2) Bulbourethral glands 3) Ventral shaft of penis
1) Corpus spongiosum - Vestibular bulb 2) Bulbourethral glands - Vestibular (Bartholin) glands 3) Labia minora
867
Most common salivary gland tumor
Pleomorphic adenoma
868
ARDS pathway
Shock, infection, toxic gas inhalation, aspiration, pancreatitis, heroin OD, sepsis, trauma, uremia, amniotic fluid embolism --> Inflammatory cells/ mediators and oxygen free radicals --> Damage to endothelial or alveolar epithelial (type I) cells --> Diffuse alveolar damage (DAD)
869
Sarcoidosis mneumonic tx
A GRUELING Disease ``` ACE enzyme increase Gammaglobulinemia Rheumatoid factor Uveitis Erythema nodosum Lmphadnopathy (bilateral, hilar) Idiopathic Noncaseating Granulomas Vit D increase (hypercalcemia or hypercalcinuria) ``` Glucocorticoids
870
Honeycomb lung on CT Patchy interstitial fibrosis - mutations - risk factors - survival - also seen
Idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis) Mutations: telomerase, mucin MUC5B 3 year survival Usual interstitial pneumonia
871
A 68-year-old man has had worsening dyspnea with a nonproductive cough for the past 9 months. On physical examination, he is afebrile and normotensive. On auscultation of the chest, diffuse dry crackles are heard in all lung fields. A chest radiograph shows irregular opacifications throughout both lungs. A transbronchial biopsy is obtained and the microscopic findings with trichrome stain are shown in the figure. Laboratory studies include negative serologic tests for ANA, anti–DNA topoisomerase I, ANCA, and anticentromere antibody. Despite glucocorticoid therapy, his condition does not improve, and he dies 2 years later. What is the most likely diagnosis? A Goodpasture syndrome B Hypersensitivity pneumonitis C Idiopathic pulmonary fibrosis D Sarcoidosis E Systemic sclerosis
C. idiopathic pulmonary fibrosis
872
A 50-year-old man has increasing dyspnea with idiopathic pulmonary fibrosis, which was diagnosed 18 months ago. Physical examination shows elevated jugular venous pressure and pedal edema. Laboratory studies show serum AST of 221 U/L, ALT of 234 U/L, alkaline phosphatase of 48 U/L, lactate dehydrogenase of 710 U/L, total bilirubin of 1.2 mg/dL, albumin of 3.5 g/dL, and total protein of 5.4 g/dL. The figure shows the microscopic appearance of a liver biopsy specimen. Which of the following terms best describes these findings? A Apoptosis B Centrilobular congestion C Cholestasis D Hemosiderin deposition E Macrovesicular steatosis F Mallory-Denk bodies G Portal fibrosis
B. Centrilobular congestion
873
What is low in restrictive lung disease
TLC FVC Both FEV1 and FVC decreased FEV1/FVC = > 80%
874
Innumerable calcified nodules Eggshell calcification in hilar nodes - increased susceptibility to - increased risk of
Silicosis Susceptibility to TB Increased risk of cancer
875
Sandblasting Mining Demolition Cement/concrete
Silicosis
876
Shipbuilding Roofing Pipe fitting plumbing
Asbestosis
877
``` Fibers in lung bases Dumbbells on stain (prusian blue) Pleural plaques Pleural effusions interstitial fibrosis ``` - seen w/ cells - cancer
Asbestosis Macrophages release inflammatory mediators and free radicals Cancer - Bronchogenic carcinoma - mesothelioma
878
Tennis racket shaped cytoplasmic organelles | Young adult smoker
Pulmonary Langerhans Cell histiocytosis Focal collections of langerhans often accompanied by eosinophils Burbbeck granules Quit smoking
879
Lung disease + glomerulonephritis think
Goodpasture syndrome Granulomatosis with polyangiitis (Wegener's granulomatosis)
880
Necrotizing granulomas vasculitis most prominent in lungs and upper airways Focal necrotizing glomerulonephritis
Granulomatosis with polyangiitis
881
Bilateral hilar adenopathy | uveitis
Sarcoidosis
882
CD surface marker fits each of the following 1) Found on all NK cells and binds the constant region of IgG 2) Inhibits complement C9 binding 3) Endotoxin receptor found on macrophages
1) CD16 2) CD55/59 3) CD14
883
Lung cancer 1) in peripheral lung 2) in central lung 3) most common in non smokers 4) hypercalcemia 5) giant cells 6) ACTH, ADH 7) Ab to presynaptic Ca channels leading to? 8) Mutations in Adenocarcinoma 9) Mutations in TP53, Rb
1) adenocarcinoma, large cell carcinoma 2) SCC, small cell carcinoma 3) Adenocarcinoma 4) SCC 5) Large cell carcinoma 6) Small cell carcinoma 7) Small cell carcinoma Lambert Eaton syndrome 8) "MARKER" MET, ALK, RET, KRAS, EGFR, ROS 9) Small cell carcinoma
884
Lambert Eaton syndrome
Muscle weakness that improves iwth use Ab against Ca channels Small cell carcinoma
885
Tumor in apex of lung - symptoms
Pancoast tumor ``` Hoarseness (compress recurrent laryngeal n.) Horner syndrome - ptosis - miosis -anhidrosis (affect cervical sympathetic plexus) ```
886
Lung cancer metastasize to Receive metastases from
``` To: Brain Bone Liver Adrenal glands ``` ``` Receive from: Breast Colon Prostate Bladder ```
887
Which lysosomal storage disease 1) Accumulation of dermatan sulfate 2) Deficiency in hexosaminidase
1) Hurler dis, hunter syn, scheic syn | 2) Tay-sach disease
888
Which infectious agent fits 1) Common cause of pneumonia in immunocompromised patients 2) Most common cause atypical pneumonia 3) Agent for penumonia in alcoholics 4) Can cause an interstitial pneumonia in bird handlers 5) Pneumonia in pts w/ hx of exposure to bats and bat droppings 6) Pneumonia in pt who visited south california, new mexico, or west texas 7) Pneumonia associated iwth currant jelly sputum 8) Q fever 9) Assocaited with pneumonia acquired from air conditioners 10) Most common cause of pneumonia in children 1 year old or younger 11) Pneumonia in neonate 12) Pneumonia in children and young adults (college, militrary, prison inmates) 13) Viral pneumonia 14) Causes wool-sorter's disease (life-threatening pneumonia) 15) Common pneumonia in ventilator patients and those with CF 16) Pontiac fever
1) Pneumocystis jirovecii 2) Mycoplasma pneumoniae 3) Klebsiella pneumoniae 4) Chlamydophila psittaci 5) Histoplasma 6) Coccidioides immitis 7) Klebsiella pneumoniae 8) Coxiella burnetii 9) Legionella pneumophilia 10) RSV 11) Group B strep, E coli 12) Mycoplasma pneumoniae 13) RSV 14) Bacillus anthracis 15) Pseudomonas, MRSA 16) Legionella pneumophila
889
Lung abscess bacteria Tx Complication of
Anaerobes ``` Peptostreptococcus Prevotella Bacteroids Fusobacterium S. aureus Klebsiella pneumoniae Gram negative bacteria ``` First line for anaerobe - Clindamycin Aspiration pneumonia - alcoholics - drug abusers - general anesthesia
890
Test for sleep apnea
Polysomnography
891
GI problem associated 1) 50 y.o woman, pruritus w.o jaundice, positive AMA 2) GI bleeding, buccal pigmentation 3) 60 y.o female, RA, no alcohol hx, fatigue, and right abdominal pain, elevated ANA adn ASMA, elevated serum IgG lvel, no viral serologic markers 4) 23 y.o woman no alcohol hx; elevated levels of LKM-1 ab, no viral serologic markers, liver biopsy with infiltration of the portal and periportal area with lymphocytes 5) Fatal disease of unconjugated bilirubin resulting from a complete lack of UDPGT activity
1) Primary biliary cirrhosis 2) Peutz-jegher syndrome 3) Autoimmune hepatitis 4) Autoimmune hepatitis 5) Crigler- Najjar type I
892
What substances act on smooth muscle myosin light chain kinase? How does this affect blood pressure
Dihydropyridine Ca channel blockers Epinephrine (acting at beta2 receptor) Prostaglandin E2 Relaxation of vascular smooth m -> vasodilation --> decrease BP
893
Watery diarrhea Dyspnea and wheezing Flushed and red Mass in right liver lobe and ileum What is it? Treatment?
Carcinoid syndrome - secrete serotonin Hepatic metastasis Octreotide for symptomatic patients
894
``` Developmental delay Difficulty seeing board Tall, thin habitus Elongated limbs Lens subluxation Thrombus and infarct ``` Supplement?
Homocystinuria Error of methionine metabolism AR deficiency cystathionine beta-synthase that requires pyridoxine (Vit B6) as cofactor
895
A 6-year-old girl is found to be nearsighted during a vision screening at school, and the school nurse tells the parents the child should be fi tted for corrective lenses. Her mother is upset because her daughter is already much taller than her classmates, has an awkward gait, and was recently diagnosed with scoliosis. She is afraid that the glasses will only add to her daughter’s problems at school, where her classmates frequently tease her. When the ophthalmologist observes that the patient’s right lens is dislocated, he suspects that her symptoms are in fact related to an enzyme defi ciency. As a result of this defi ciency, which of the following amino acids is essential in this patient’s diet? (A) Cysteine (B) Lysine (C) Methionine (D) Tryptophan (E) Tyrosine
The correct answer is A. Homocystinuria is an inborn error of metabolism caused by a defect in cystathionine synthase, the enzyme that converts homocysteine to cystathionine. Cystathionine is later converted to cysteine, so patients with this enzyme defi ciency are required to supplement their diets with exogenous cysteine. In addition to marfanlike features and subluxation of the lens, these patients are at increased risk of a variety of cardiovascular derangements, includin
896
Arteries off splenic artery
Short gastric Left gastroepiploic
897
CXR reveals a round density with an air fluid level in right lung Copious sputum - What is it - Key player in formation
Lung abscess Neutrophils that release cytotoxic granules (lysosomes) containing myeloperoxidase Copious sputum (foul smelling)
898
intermittent claudication (pain comes and goes) due to Chest pain relieved by rest Cause?
Atherosclerosis Lipid filled arterial intimal plaques
899
Onion-like concentric thickening of arteriolar walls Homogenous acellular thickening of the arteriolar wall
Hyperplastic arteriosclerosis Hyaline arteriolosclerosis - homogenous deposition of hyaline material in the intima and medial of small arteries and arterioles
900
``` Man to ER Found unconscious Temp White patches on mucosa Bilateral crackles in lungs Bilateral interstitial infiltrates ``` Silver stain
Pneumocystisi pneumonia atypical fungal infection Opportunistic infection
901
Silver stain
Fungi - coccidoides - pneumocystis jirovecci Legionella Helicobacter pylori
902
Hernia that originates medial to the inferior epigastric vessels Defect in
Direct hernia MDs dont LIe - medial to inferior epigastric = direct - lateral = indirect Weakness of transversalis fascia
903
Cause of indirect hernia Location
Lateral to inferior epigastric vessels Due to patent processus vaginalis
904
Cause of femoral hernia Location
Emerges inferiro to inguinal ligament Widening of the femoral ring is defect
905
Epistasis
Phenomenon in which the allele of one gene affects the expression of alleles in another gene
906
Imprinting
Phenomenon in which an offspring's genes are expressed in a parent specific manner. Caused by DNA methylation
907
Person is exercising what value will increase What will decrease
Venous blood mean CO2 content will increase pH decreases - Lactic acid produced Decrease physiologic dead space - due to decrease in pulmonary vascular resistance Mean O2 content of venous blood remains the same or decreases
908
S. bovis risk of
Streptococcus gallolyticus Causes subacute bacterial endocarditis Part of normal flora of colon Associated with colon cancer
909
Still get Haemophilus influenzae even though had vaccine how?
The strain responsible for the patients disease does not produce a capsule H. influenzae nontypeable Vaccine only against more invasive type b strain
910
Diuretic that causes hypercalcemia
Thiazide diuretic
911
Nerve associated with cremasteric reflex
Genitofemoral n.
912
Nerve that passes through spermatic cord through superficial inguinal ring
Ilioinguinal nerve.
913
Nerve damaged in appendectomy causes decreased sensation to suprapubic region
Iliohypogastric n.
914
``` Fever Malaise sore throat VERY tired Palatal petechiae cervical LAD Splenomegaly ``` -Assoc with
Mono | - EBV
915
EBV associated with
Burkitt lymphoma Nasopharyngeal carcinomas Mononucleosis
916
Severe HTN emergency >180/120 Manifests as (2)
Fibrinoid necrosis Hyperplastic arteriolosclerosis - onion skin
917
Glutamate --> alpha ketoglutarate for TCA cycle requires Oxaloacetate --> aspartate
Transamination Vit B 6 Pyridoxine
918
Biotin is cofactor for
Vit B7 ``` carboxylase enzymes - pyruvate carboxylase Acetyl-COA carboxylase Propionyl--CoA carboxylase 3-methylcrotonyl-CoA- carboxylase ```
919
Vessel with lowest oxygen content
Coronary sinus
920
Emysema causes what to lung compliance Asthma on lung compliance What shifts the graph right and down? decrease compliance
Increase Shift left and up No change Pulmonary fibrosis
921
What hormone increases bicarb secretion? Cell type?
Secretin | - Duodenal S cells
922
Skin lesion on eyelid ma Lipid laden macrophages Assoc with
Xanthelasma Associated iwth primary or secondary hyperlipidemias - Primary biliary cirrhosis
923
Most common cause of overdose death
Opioids
924
Aortric regurgitation Mediastinal widening + fluorescent treponemal ab Suggest? - Due to - Begins with
Aortic aneurysm Syphilis Vasa vasorum endarteritis adn obliteration
925
H. influenzae type b virulence factor
Polysaccaride capsule composed of polyribosylribitol phosphate (PRP)
926
Goes with? Lecithinase M protein Protein A Trehalose dimycolate Polyribosylribitol phosphate (PRP)
Lecithinase: toxin A, clostridium perfringens - results in cell lysis and gas gangrene M protein: strep. pyogenes -Binds factor H to prevent opsonization and destruction by alternative complement pathway Protein A: staph aureus - prevent opsonization by binding Fc region of immunoglobulins Trehalose dimycolate: cellw all component , mycobacterium tuberculosis - protects from being killed by macrophages and stimulates granuloma formation Polyribosylribitol phosphate: Haemophilus influenzae type b - capsule protects against phagocytosis and complement mediated lysis by binding factor H (prevents complement C3b deposition on host cells
927
Rebound anxiety, tremor, insomnia, palpitations, diaphoresis | Seizure
Benzodiazepine withdrawal Lorazepam
928
Itching after hot showers Fatigue Rash worse with rubbing Flushing Clusters of mast cells
Systemic mastocytosis Excessive histamine
929
Excessive histamine causes
Mastocytosis Gastric hypersecretion
930
Hyperestrogenic states can cause
Gynecomastia Spider angiomata - estrogen effect on arteriolar dilation
931
Continous IV infusion with first order kinetics takes __ half lives to reach 95%
4-5
932
High urinary excretion of ketones suggests
absolute insulin deficiency | Diabetes type I
933
Huntingtons neuroimaging
Caudate atrophy with enlargement of the lateral ventricles Enlargement of frontal horns of the lateral ventricles
934
Seen with? 1) Diffuse ventriculomegaly without sulcal widening 2) Enlargement of frontal horns of the lateral ventricles 3) Hypoplastic vermis with a dilated fourth ventricle 4) Pronouced atrophy of bilateral hippocampi 5) Regional atrophy of frontal and temporal lobes
1) Normal pressure hydrocephalus 2) Huntington's dis 3) Dandy-walker malformation 4) Early onset alzheimer dis 5) Frontotemoral dementia
935
Winging scapula can occur due to what nerve and what event
Long thoracic nerve to serratus anterior Axillary LN dissection
936
Histopathologic evaluation of syphilis lesion
Intense plasma cell rich infiltrate with proliferative endarteritis
937
What is this? 1) Acantholysis with superficial dermal lymphocytic infiltrate 2) Dysplastic spndle cells iwht viral genome that form vascular channels 3) Intense plasma cell-rich infiltrate with proliferative endarteritis 4) Panniculitis and sepal inflammation with multinucleated giant cells 5) Papillomatous epidermal hyperplasia with cytoplasmic vacuolization
1) Pemphigus vulgaris 2) Kaposi sarcoma 3) Syphilis 4) Erythema nodosum 5) Anogenital warts, HPV-6
938
``` Headaches Dizziness Facial plethora (reddish) Splenomegaly Peptic ulcer disease Increased erythrocytes ``` - Mutation
Polycythemia vera - uncontrolled erythrocyte production Abnormal transduction of erythropoietin growth signals Mutation in JAK2 Erythropoietin receptor has no intrinsic kinase activity and must interact with JAK2 a cytoplasmic tyrosine kinase
939
Mutation of 1) Growth stimulating transcription factor 2) Tumor suppressor protein
1) c-myc - burkitt lymphoma 2) p53 - Li-fraumeni syndrome
940
Protein 4.5 g antinuclear ab glomerular capillary wall thickening Irregular spikes protruding from glomerular basement membrane
Membranous glomerulopathy
941
HIV process involving glycosylation
Only glycosylated HIV polyprotein is gp160 - product of the env gene gp160 is extensively glycosylated in the rough ER and golgi cleaved into envelope proteins gp120 and gp41 gp120: mediates viral attachment gp41: mediates viral fusion
942
HIV pol gene encodes for
reverse transcriptase integrase protease RT: ssRNA --> ds DNA Integrase: integrate into host genome Protease: cleave HIV polyproteins into individual proteins after trasncription in rough ER
943
Acondroplasia inheritence
AD
944
``` Anorexia Nausea Dark-colored urine Trip to mexico Fever Right upper quadrant pain ``` liver biopsy?
Acute viral hepatitis HAV Hepatic swelling
945
Seen with? 1) Fibrinoid necrosis of liver 2) Liquefactive necrosis 3) Hepatocyte swelling 4) Regenerative nodules
1) Fibrinoid necrosis= immune vasculitis or malignant hypertension 2) Liquefactive necrosis= severe viral hepatitisor entamoeba histolytica infection 3) Hep A acute infection 4) Regenerative nodules= cirrhosis form chronic hepatitis
946
For viral particle to be coupled to MHC class I it first has to be involved with what
Ubiquitin ligase
947
Increase in fructose 2,6 bisphosphate causes a decrease in
Inhibits gluconeogenesis Decrease Alanine --> glucose
948
Thiamine deficiency Get thiamine infusion What neurologic finding will persist despite treatment?
Memory loss Korsakoff syndrome
949
Airway resistance from trachea --> medium sized bronchi --> bronchioles --> terminal bronchioles
Airway resistance increases from trachea to medium sized bronchi Decreases after that due to lower respiratory tract are arranged in parallel
950
N gonorrhoeae infection and repeat infections
High variability of gonococcal surface antigens (porins, opa proteins and lipooligosaccharide) limits effectiveness of antibody response Previous infections results in almost no protective immunity against future infections
951
Decrease in PAH level would be due to
Carrier transport saturation
952
Vaginal discharge Grayish white Fishy smelling What is seen on microscopy?
Epithelial cells covered with gram-variable rods
953
Identify 1) Epithelial cells covered with gram-variable rods 2) Epithelial cells with rare leukocytes 3) Gram-negative intracellular diplococci 4) leukocytes and pear-shaped organisms 5) Pseudohyphae with leukocytes
1) Gardnerella 2) normal vaginal discharge 3) Gonorrhea 4) trichomonas vaginitis 5) Candida vaginitis
954
Back pain that is better when leaning on stroller Thickening of what?
Ligamentum flavum Spinal stenosis
955
Paroxysmal atrial fibrillation can be caused by aberrant electrical foci in the
Aberrrant electrical foci in the pulmonary veins
956
Alpha 1 effects Examples
Increase blood pressure Contraction of internal urethral sphincter Mydriasis (eye dilation) - contraction of pupillary dilator muscle Epinephrine NE Phenylephrine Methoxamine
957
Beta 1 effects examples
Heart - Increase HR, contractility and conductance Epinephrine Dopamine Dobutamine Isoproterenol
958
Beta 2 effects examples
Vasodilation Decrease diastolic BP Bronchodilation Relaxation (uterus) Isoproterenol Terbutaline Ritodrine
959
What releases growth factors in formation of atheroma
platelets
960
``` Rapid onset Headache Fever Altered mental status Nuchal rigidity Pruritic rash ``` Type of vaccine?
Meningococcal meningitis Capsular polysaccharide Ag
961
Vaccine with inactivated (killed organisms)
RIP Always Rabies Influenza (injection) Polio (oral) Hep A
962
Vaccine with live attenuated organisms
"Attention Teachers! Please vaccinate SMALL, Beautiful Young INFants with MMR Regularly!" ``` Adenovirus Typhoid Polio Varicella Small pox BCG Yellow fever Influenza (intranasal) MMR Rotavirus ```
963
Vaccine with virus-like particles Subunit type
Induces only the Ag that stimulate the immune system Hep B HPV
964
Hypercalcemia | Lung mass
Hypercalcemia of malignancy Parathyroid hormone-related peptide (PTHrP)
965
Multiple polyps found in colon due to mutation of
Familial adenomatous polyposis APC mutation
966
What mutation 1) Lung adenocarcinoma 2) CML, ALL 3) Follicular lymphoma 4) non-hodgkin lymphoma 5) Neuroblastoma 6) Melanoma 7) MEN 2A and 2B 8) Colorectal cancer assoc w/ FAP 9) Hairy cell leukemia 10) Osteosarcoma 11) Li-Fraumeni 12) Tuberous sclerosis 13) Burkitt lymphoma 14) Colon cancer 15) Diffuse large B cell lymphoma
1) ALK 2) BCR-ABL 3) BCL-2 4) BRAF 5) n-myc 6) BRAF, CDKN2A 7) RET 8) APC 9) BRAF 10) Rb 11) TP53 12) TSC1/2 13) c-MYC 14) KRAS 15) BCL-2
967
``` Exertional dyspnea Dry cough Bilateral hilar adenopathy Reticular pulmonary infiltrates ACE and Ca elevated ``` Predominant cell type?
Sarcoidosis CD4 lymphocytes
968
Painless lymph node enlargement Translocation?
Follicular lymphoma t(14;18)
969
Most common cause of bilateral fetal hydronephrosis in boys
Posterior urethral valves
970
Heart murmur Best heard when patient sits up and leans forward Peak intensity?
Aortic regurgitation Just after aortic valve closure
971
What causes the green color of sputum
Neutrophil myeloperoxidase
972
Pathologic finding of varicella zoster
Multinucleated cells with intranuclear inclusions
973
Identify 1) Acantholysis and intrercellular IgG deposits 2) Eosinophilic cytoplasmic inclusions 3) Microabscesses at the tips of dermal papillae 4) Multinucleated cells with intranuclear inclusions 5) Koilocytosis of the superfical epidermal layers
1) Pemphigus vulgaris 2) Molluscum contagiosum (poxvirus) 3) Dermatitis herpetiformis 4) Varicella zoster 5) HPV of skin w/ warts
974
Serotonin syndrome
``` Mental status changes Hyperthermia Sympathetic hyperactivity Clonus Hyperreflexia ```
975
Neuroleptic malignant syndrome Tx
``` Mental status changes Hyperthermia Sympathetic hyperactivity Diffuse rigidity (lead pipe) Hyporeflexia ``` Dantrolene Dopaminergic drugs - Bromocriptine (DA agonist) - Amantadine (Increase DA release)
976
Meningitis --> bleeding from venous puncture sites Results in what cells
Disseminated intravascular coagulopathy (DIC) Schistocytes
977
Bite cells
Oxidant induced damage G6PD deficiency
978
Surgery --> Fever, lethary, N/V Scleral icterus RUQ tenderness Elevated AST, ALT and bilirubin What drug used during surgery
Acute hepatitis Inhaled halogenated --> hepatocellular damage -flurane
979
High fever Nasal discharge Bilateral conjunctival injection Several small white spots with an erythematous base on buccal mucosa No vaccines What else is seen
Measles Maculopapular rash
980
Measles vaccine is
Live attenuated
981
Vesicular rash
Chicken pox
982
``` Myalgia Fatigue HA Chills Fever ``` Sick and recovered Mild jaundice Hepatosplenomegaly Elevated indirect bilirubin Ringed inclusions
Malaria Trophozoites (ringed inclusions) on Giemsa Dormant hepatic phase
983
Virus that infects by CD21 Virus that infects by glycoprotein gp120 Virus that infects via the blood group P antigen (globoside)
1) EBV 2) HIV 3) Parvovrus B19
984
Damage seen due to megaloblastic anemia
Vit B12 deficiency Damage to dorsal column of spinal cord Axonal degeneration of the peripheral nerves Loss of poition and vibration sense Gait problems
985
Pear shaped organism Watery diarrhea Small bowel shows? Immune mechanism?
Giardia lamblia (parasite) Villus atrophy and crypt hyperplasia CD4 T cells and secretory IgA production
986
Lesion to cerebellar vermis leads to
truncal ataxia | Gait ataxia
987
Pain in both knees Pain in multiple joints Blue-black spots on sclerae Diffuse darkening of the auricular helices Deficiency?
Alkaptouria AR Deficiency of homogentisic acid dioxygenase Accumulated homogentisic acid
988
Decreased ceruloplasmin
Wilson disease (copper buildup)
989
Proteinuria Hematuria lactate dehdyrogenase elevated
Renal infarction Renal vein thrombosis due to antithrombin III loss
990
HTN is defined as
>= 140/90 3 different occasions
991
Tearing chest pain that radiates to the back See on CXR? TX?
Aortic dissection Widening of the mediastinum Large aorta, double circle Lower BP - Beta blocker
992
Substances that act on smooth muscle mysoin light chains kinase How does this affect blood pressure
Dihydropyridine Ca channel blockers Epinephrine (acting at beta 2 receptor) Prostaglandin E2 Relaxation of vascular smooth m. Vasodilation Decrease BP
993
Loss of forearm pronation nerve
Median
994
Guillain- Barre syndrome preceded by what infection
Symmetric ascending muscle weakness Campylobacter jejuni (CMV, HIV, EBV, mycoplasma pneuonae)
995
Where do neurons of the lateral corticospinal tract synapse prior to exiting the spinal cord
At the cell body of the anterior horn
996
Endogenous hormones that stimulate gastric acid secretion Endogenous hormone that inhibit gastric acid secretion Drugs that regulate secretion
Stimulate - Histamine - Acetyl choline - Gastrin Inhibit - Prostaglandins - Somatostatin - Secretin - GIP Drugs that regulate - PPIs - H2 blockers - Antimuscarinic drugs (atropine)
997
What type of lipoproteins accumulates first in atherosclerosis What cell adheres next Steps
LDL Macrophages and foam cells 1) Endothelial dysfunction 2) Accumulation of lipoprotein 3) Monocytes adhesion 4) Migration into intima transformation --> macrophages and foam cells 5) Factor release (platelets, macrophages) 6) Smooth muscle proliferation, deposition extracellular matrix elastin and collagen 7) Lipid accumulation
998
Onion skin appearance of vessels
Arteriolosclerosis (Not atherosclerosis) Hyperplasia of vascular smooth muscle cells Hyaline thickening of arterioles
999
Most common MI
Anterior wall MI | LAD
1000
Inferior wall MI artery Lateral wall MI artery
Inferior: Posterior descending A (RCA most common) Lateral: circumflex
1001
Chest pain in younger adult Occurs at rest - Due to - ECG changes - Tx
``` Prinzmetal angina (variatn angina) ``` Coronary artery spasm ST segment elevation Dihydropyridine Ca channel blockers (Nifedipine)
1002
Classic presentation of congenital pyloric stenosis (5)
2-6 weeks age Nonbillous projectile vomiting Palpable "olive" RUQ Hypochloremic metabolic alkalosis Hypokalemia
1003
Relays what type of information from thalamus 1) Ventral posterior lateral 2) Lateral geniculate 3) Ventral posterior medial 4) Ventral anterior
1) Somatosensory info from body 2) Visual retinal to occipital lobe 3) Somatosensory from face 4) Motor basal ganglia to cortex
1004
Changes seen on EKG after MI Hours 1-2 days Weeks
Hours - ST elevation - R wave decreases - Q wave appears 1-2 days - T wave inverts - Q wave deepens Weeks - ST normal - T wave normal - Q wave persists
1005
Dressler syndrome
Post MI pericarditis Chest pain Pericardial freiction rub Persistent fever occuring several weeks after MI
1006
Type of hyperbilirubinemia that responds to phenobarbital
Crigler Najjar type II | Gilbert
1007
ECG leads the correspond 1) Left anterior descending 2) Left circumflex 3) Right coronary 4) Right coronary (posterior descending )
1) Anterior wall V1-V4, V5 2) Lateral wall aVL, V5, V6, lead I 3) Inferior wall II, III, aVF 4) Posterior wall - R precordial ECG, V4
1008
MI Sudden onset mitral regurgitation New holosystolic murmur Cardiac tamponade
Papillary muscle rupture Intraventricular septal rupture (VSD) Ventricular wall rupture - bleeding into pericardium
1009
Cardiac tamponade
Compression of heart by fluid Decrase Cardiac output Equilibration of diastolic pressures in all 4 heart chambers ``` Hypotension Distended neck veins Distant heart sounds Increased HR Pulsus paradoxus ``` Electrical alternans
1010
Kartagener syndrome
Situs inversus Bronchiectasis Recurrent sinusitis Dynein arm defect
1011
A 2-year-old boy presents to the pediatrician with fever, facial tenderness, and a green, foulsmelling nasal discharge. The patient is diagnosed with sinusitis, and the physician notes that he has a history of recurrent episodes of sinusitis. X-ray of the chest is ordered because of the fever; it reveals some dilated bronchi and shows the heart situated on the right side of his body. A congenital disorder is diagnosed. Which other finding would this patient be most likely to have? (A) Defective chloride transport (B) Elevated blood sugar (C) Infertility (D) Reactive airway disease (E) Tetralogy of Fallot
C. infertility Kartagener's syndrome
1012
Bronchiectasis
Abnromal bronchial dilation caused by chronic infection Purulent sputum
1013
A 19-year-old man has a history of recurrent mucoid rhinorrhea with chronic sinusitis and otitis media since childhood. He has experienced multiple bouts of pneumonia. His temperature is 37.7° C. On examination of his chest, there is tactile fremitus, rhonchi, and rales in lower lung fields. Nasal polyps are noted. A chest radiograph shows bronchial dilation with bronchial wall thickening, focal atelectasis, and areas of hyperinflation; his heart shadow appears mainly on the right. Which of the following abnormalities is he most likely to have? A α1-Antitrypsin deficiency B Atopy C Chloride ion channel dysfunction D Ciliary dyskinesia E HIV infection
D. Ciliary dyskinesia
1014
Carcinoid syndrome
B FDR Bronchospasm and wheezing Flushing Diarrhea Right sided heart lesions
1015
Painful red nodules on finger and toe pads Erythematous maculoes on palms and soles Splinter hemorrhages in fingernails Fever Organisms? Acute vs subacute
Endocarditis - inflammation of valves S. Aureus Viridans streptococci Enterococcus Coagulase neg staph Acute - s. aureus - days - normal valves Subacute - Viridans streptococci - wks --> months - previously damaged valves
1016
Systolic murmer louder with valsalva - due to Softer with squatting - due to Tx
Hypertrophic cardiomyopathy - Hypertrophy of interventricular septum - outflow tract obstruction Reduces preload Increases afterload Beta blocker Verapamil
1017
Endocarditis with streptococcus bovis also check
Colon cancer
1018
Endocarditis with negative blood cultures
HACEK - Haemophilus - Actinobacillus - Cardiobacterium - Eikenella - Kingella Coxiella burnetii (Q fever) Bartonella Brucella
1019
Endocarditis mneumonic Treatment
``` Bacteria FROM JANE Fever Roth spots (eye) Osler nodes (tender fingertip) Murmur Janeway lesions (painless) Anemia Nail bed hemorrhage Emboli ``` Prophylactic Penicillin Empiric IV vancomycin (covers staph and strep)
1020
Retinal hemorrhages with pale centers
Roth spots Infective endoarditis
1021
Valve affected in infective endocarditis Valve affected in IV drug user infective endocarditis
Mitral valve Tricuspid valve
1022
Kussmaul's sign
JVD during inspiration Right sided heart problem Decreased capacity of RV Constrictive pericarditis (>>tamponade)
1023
Aschoff bodies | Anitschkow cells
Aschoff bodies - granuloma with giant cells - area of fibrinoid necrosis infiltrated by lymphocytes and giant cells Anitschkow cells - activated histocytes with owl eye appearance
1024
EKG change with pericarditis
Diffuse ST elevation
1025
Most common primary cardiac tumor in adults
Myxomas Left atrial myxoma Ball valve obstructs mitral valve --> syncopal episodes Plop sound
1026
Tuberous sclerosis is associated iwth what three tumors
Rhabdomyoma Astrocytoma Angiomylipoma
1027
What heart pathology 1) Focal myocardial inflammation with multinucleate giant cells 2) Chest pain and coarse rubbing heart sounds in patient with Cr of 5.0 3) Tree barking of the aorta 4) ST elevation in all ECG leads 5) ECG shows electrical alternans 6) Child with fever, joint pain, cutaneous nodules, four weeks after throat infection
1) Aschoff bodies - Rheumatic heart disease 2) Uremic pericarditis 3) Tertiary syphilis 4) Pericarditis 5) Cardiac tamponade 6) Acute rheumatic fever
1028
Primary biliary cholangitis vs primary sclerosing cholangitis
PBC - Autoimmune - (+) AMA - Middle aged female ``` PSC - (+) p-ANCA - Males > 40 - Assoc: UC and cholangiocarcinoma ERCP: alternating beading stricturing ```
1029
``` Fever Jaundice RUQ pain Hypotension Altered mental status ```
Reynold's pentad for cholangitis
1030
Pulseless disease Poor pulses in extremities Young asain female - Histology - Elevated
Takayasu arteritis Granulomatous inflammation of arteries of teh arotic arch Elevated ESR
1031
Unilateral headache Jaw claudication Vision loss - Histology - Elevated - Assoc with - Tx
Giant cell arteritis aka temporal arteritis Inflammation of branches of external carotid artery - temporal arteries Vision loss (occlusion of opthalamic artery) Elevated ESR Polymyalgia rheumatic - pain and joint stiffness High dose steroids for several months
1032
``` Child Fever 5 days Conjunctivitis Rash on trunk Cervical lymph nodes swollen Red tongue Erythema on hands and feet ``` Potential for? Tx?
Kawasaki disease (Mucocutaneous lymph node syndrome) Potential for coronary aneurysms - rupture - thormbosis --> MI Tx - VI immunoglobulin - Hig dose aspirin
1033
Only child you give asprin to?
Kawaski disease
1034
Gangrene of fingers Smoker Male in 40s
THromboangititis obliterans Buerger disease Small medium peripheral arteries adn veins Cold sensitivity Stop smoking
1035
Hep B Vasculitis (-) anca involves spares assoc with Tx
Polyarteritis nodosa (PAN) Involves kidneys, skin, GI, heart spares lungs Associated iwth hep B adn C Corticosteroids
1036
Recent upper respiratory infection Palpable purpura on butt and legs Abdominal pain Protein and blood in urine - also have what disease - Tx
Henoch-Schonlein purpura (IgA vasculitis) Renal dease - IgA nephropathy (berger's disease) Nephritis andhematuria Self limiting
1037
``` Patient with asthma Sinusitis Multinucleated giant cells and eosinophils + p-ANCA Increased IgE ```
Eosinophilic granulomatosis with polyangiitis | Churg-Strauss syndrome
1038
A 53-year-old man with a long-standing history of allergic rhinitis and asthma presents with uveitis, mild hearing loss, numbness and tingling in his right hand, and diffuse joint pain for the past 10 days. Physical examination shows weak to absent left knee patellar reflexes (right knee reflex strong and intact). Laboratory studies show a markedly elevated eosinophil count. A diagnosis is made, and the patient is treated with cyclophosphamide. Further laboratory studies show elevated serum levels of the most common autoantibody associated with this condition. What structure is primarily targeted by the autoantibodies that are most likely elevated in this patient’s serum? (A) Acetylcholine receptors (B) Neutrophils (C) Oligodendrocytes (D) RBCs (E) TSH receptors
B
1039
p-ANCA - Ag - Ab c-ANCA - Ag - Ab
p-ANCA - Myeloperoxidase (MPO) - MPO-ANCA c-ANCA - Proteinase 3 (PR3) - PR3-ANCA
1040
Affects both kidneys and lungs
Granulomatosis with polyangiitis (GPA) | Goodpasture
1041
``` Dyspnea Hemoptysis Heamturia Saddle nose +c-ANCA Necrotizing granulomas ``` Tx
Granulomatosis with polyangiitis (GPA) Wegners Focal necrotizing vasculitis
1042
A 46-year-old man comes to the clinic with a cough that is occasionally productive of blood, diffuse muscle and joint pain in the upper extremities, and blood in his urine for the past several days. On further questioning, the patient reveals that he has had chronic sinusitis for the past several years. The patient has a low grade fever and nasal ulcerations. Laboratory studies show a markedly elevated erythrocyte sedimentation rate, and staining for antibodies to cytoplasmic antigens of neutrophils is positive.Biopsy of a nasal ulceration reveals vasculitis and necrotizing granulomas. Which of the following is the most likely diagnosis? (A) Alport’s syndrome (B) Giant cell (temporal) arteritis (C) Goodpasture’s syndrome (D) Takayasu’s arteritis (E) Wegener’s granulomatosis
E. wegeners
1043
(+) p-ANCA
Eosinophilic granulomatosis with polyangiitis Microscopic polyangiitis
1044
(+) c-ANCA
Granulomatosis with polyangiitis (GPA)
1045
Necrotizing vasculitis of lungs kidneys skin Crescentic GN Palpable purpura + p-ANCA Doesnt have
Microscopic polyangiitis No naropharyngeal involvement
1046
``` Recurrent nose bleeds Gi bleeding Hematuria Telangiectasias Skin discolorations Microcytic anemia ``` - inheritence
Hereditary Hemorrhagic Telangiectasia aka osler-weber rondu syndrome AD Due to vascular malformations in mucosa --> prone to bleeding
1047
Microcytic anemia
Iron deficiency Thalassemias Lead poisoning
1048
Normocytic Hemolytic anemia
``` Sickle cell HbC disease Pyruvate kinase deficiency G6PD deficiency Paroxysmal nocturnal hemoglobunuria Hereditary spherocytosis ```
1049
Megaloblastic macrocytic anemia
Folate def Vit B12 def orotic aciduria Fanconi anemia
1050
Can cause perforation of nasal septum | Vasculitis
Granulomatosis with polyangiitis (wegener's granulomatosis)
1051
Anti smooth muscle Ab Antimitochondrial Ab
Autoimmune hepatitis Primary biliary cholangitis
1052
Chronic granulomatous disease suspectable to
Lack NADPH oxidase activity Catalase positive s. aureus aspergillus
1053
``` Port wine stain Intellectual disability Hemiplegia Calcification of outer boarder of brain Also see ```
Sturge-Weber disease - affects capillaries Seizures Early onset glaucoma Hemiplegia (paralysis one side body) Leptomeningeal angioma
1054
Red polyp on skin Can ulcerate and bleed Assoc w/ trauma and pregnancy
Pyogenic granuloma
1055
Red prominent bumps smooth dots
Baronella henselae Bacillary angiomatosis
1056
Large dark brown spots on legs
Kaposi sarcoma (KS) Herpes virus 8
1057
Raynaud phenomenon associ with
Blue fingers in cold SLE CREST scleroderma Thromboangiitis obliterans (Buerger dis) Mixed CT disease
1058
What cell type secretes histamine that stimulates the histamine receptor on parietal cells
Enterochromaffin -like cells (ECL cells)
1059
Cohort study gives
Relative risk | A/A+B) / (C/ C+D
1060
Cross sectional gives
Prevalence
1061
Plummer vinson
Dysphagia (esophageal webs) Glossitis Iron deficiency
1062
what changes depending on disease prevalence?
PPV increases with increasing prevalence NPV decreases with increase prevalence
1063
Sensitivity equation (2)
A/ A+C 1- false negative rate
1064
What is seen in distal duodenum of patients with celiac sprue
Blunting of villi Hyperplastic crypts Lymphocytes in lamina propria
1065
Attributable risk (AR) equation
AR= incidence disease (exposed) - incidence of disease (unexposed) AR= (A/ A+B) - (C/C+D)
1066
Odds ratio
(A/B)/ (C/D)
1067
Relative risk
Probability to getting disease in exposed group compared to unexposed group (A/A+B) / (C/C+D)
1068
Absolute risk reduction (ARR)
(C/C+D) - (A/A+B)
1069
Number needed to harm
1/ AR AR= (A/ A+B) - (C/C+D)
1070
Number needed to treat (NNT)
NNT= 1/ ARR ARR= (C/C+D) - (A/A+B)
1071
``` NNT? 100 acute stroke patients given tPA 40 have complete recovery 400 acute not give tPA 80 have complete recovery ```
60 40 320 80 (320/ 400) - (60/100)= 0.2 NNT= 1 / 0.2= 5
1072
Most common benign salivary gland neoplasm Most common malignant slaivary gland neoplasma Most common location for salivary gland neoplasm
Pleomorphic adenoma Mucoepidermoid carcinoma Parotid gland
1073
Positive skew
Tail toward positive direction (to right) Mean > Median > Mode
1074
Negative Skew
Tail on left | Mean < Median < Mode
1075
Small p value
indicates that the study values didnt occurred on chance alone Reject null Statistically significant
1076
Type I error
alpha Stating there is an effect when there isnt one Accusing an innocent man False positive
1077
Type II error
beta Stating there is no effect when there is one You blindly let the guilty man go free False negative
1078
Power=
Probability that a test will correctly reject the null hypothesis Increases with increased number of study participants 1-beta
1079
Confidence interval =
CI= mean +- Z (SEM) 90% CI; Z= 1.5 95% CI; Z= 2 99% CI; Z= 2.5 SEM= standard dev/ squr root ( sample size)
1080
One standard deviation away from zero percentage 2 standard deviation away 3 standard deviation away
68% 95% 99.7%
1081
Male vs female Diseased vs not diseased Comparing percentages of members Test?
Chi-squared
1082
Correlation coefficient (r)
Measure of relationship of two variables ``` r= - 0.8 straight negative line r= - 0.4 spread out up negative line r=0 no relationship r= 0.4 spread out positive line r= 0.8 straight positive line ```
1083
Blood pressure between men and woemn test
T test
1084
What hormones stimulate pancreatic secretion
Acetylcholine CCK Secretin
1085
IPV vaccine
Poliomyelitis
1086
Meningococcal vaccine recommendation
11 or 12 yo | Boooster at 16
1087
What diarrheal illnesses should be reported
Salmonella | Shigella
1088
E/M ICD-10 CPT
E/M= evaluation and management codes ICD-10= international classification of diseases (diagnosis) CPT= current procedure terminology (produre done)
1089
Capitation
Fixed payment for a period of time or number of patients (ER shift, concierge practice)
1090
Which department in a hospital oversees the maximization of the quality of care while minimizing the cost of care?
Utilization management department
1091
In patient with elevated alkaline phosphatase, elevation in which other serum lab markers would point toward a diagnosis of liver disease?
AST ALT GGT
1092
Ethical duty to act in the patients best interest
Beneficience
1093
Physicians must strive to treat people fairly
Justice
1094
Dont need informed consent from parents or guardians for (7)
``` Emancipated minors STDS Pregnancy Drug addiction Child of a minor Minor serving a sentence of confinement Emergency situation ```
1095
Right sided heart failure signs
Peripheral signs Lower extremity edema Jugular venous distention Hepatosplenomegaly
1096
What areas of the hypothalamus regulate the autonomic nervous system
Anterior hypothalamus: parasympathetic Posterior: sympathetic
1097
Pneumonia in neonates (Birth to 28 days Pneumonia in children Pneumonia in adults
Neonates - Group B streptococcus - E coli - Chlamydia trachomatis - HSV Children - Viruses (RSV) - Streptococcus pneumoniae - Mycoplasma pneumoniae - Chlamydophila pneumoniae Adults - Streptococcus pneumoniae - H. influenza - Mycoplasma pneumoniae - Chlamydophila pneumoniae - Influenza virus Elderly - Streptococcus pneumoniae - H. influenza - Influenza virus - S. aureus - Gram negative rods (klebsiella )
1098
Organism taht causes pneumonia in each of these patients 1) Alcoholic patient 2) Patient with decrased level of consciousness 3) Pt w/ cystic fibrosis 4) Pt w/ HIV and CD4<200 5) Post-influenza 6) Acquired from patio-cooling water mister 7) Travel to southwestern U.S. 8) Exposure to bird/ bat droppings in ohio missiissippi river 9) Patient with pet parrot
1) Klebsiella 2) Anaerobes 3) Pseudomonas 4) Pneumocystis jirovecii 5) Staphylococcus aureus 6) Legionella pneumophilia 7) Coccidioides immitis 8) Histoplasma capsulatum 9) chlamydophila psittaci
1099
Meningitis by age Neonates Infants/children Adults Elderly adults
Neonates - Group B strep - E.coli - Listeria monocytogenes Infants/ children - Streptococcus pneumoniae - Neisseria meningitides - H. influenza type B - viruses (enteroviruses) Adults - strep pneumoniae - Neisseria meningitidis - enteroviruses Elderly - Streptococcus pneumoniae - Listeria - Gram negative rods (ecoli)
1100
Meningitis gram positive diplococci
Streptococcus pneumoniae
1101
Meningitis gram negative coccobacilli
H. influenzae
1102
Meningitis gram positive rod
LIsteria monocytogenes
1103
Variola virus
Pox virus Small pox Respiratory droplets Contaminated objects Rash around mouth --> spread to face --> trunk extremities
1104
What type of meningitis? 1) Unvaccinated child 2) Outbreak in dorm 3) Meningitis with petechial rash 4) Patient with HIV 5) RBCs in CSF + temporal lobe involvement on MRI
1) H. influenza type B 2) N. meningitis 3) N. meningitides 4) Cryptococcus neoformans 5) HSV encephalitis
1105
CSF findings bacterial meningitis ``` Opening pressure WBC count Cell type Protein Glucose Gram stain ```
Increased pressure Increased WBC Neutrophils HIGH protein Decreased glucose Gram stain positive
1106
CSF findins TB/ fungal meningitis ``` Opening pressure WBC count Cell type Protein Glucose Gram stain ```
Increased pressure INcreased WBC Lymphocytes Increased protein Decreased glucose No stain
1107
CSF findings in viral meningitis ``` Opening pressure WBC count Cell type Protein Glucose Gram stain ```
Normal or increased opening pressure WBC increased Lymphocytes Normal or increased protein Normal glucose No stain
1108
Meningitis Slightly increased protein Normal glucose Incrased opening pressure
Viral meningitis
1109
1) Newborn with chronic diarrhea, Failure to thrive Chronic candidiasis 2) Child with eczema, coarse facial features and cold abscesses 3) Child with partial albinism, pepripheral neuropathy, and recurrent infections
1) Severe combined immunodeficiency (SCID) 2) Hyper IgE syndrome 3) Chediak- Higashi disease
1110
Frothy yellow green Fishy odor pH > 4.5 Pear shaped Tx
Trichomonas vaginalis Metronidazole
1111
Thick white cottage cheese like 4-4.5 pH Tx
Candida spp Azoles Nystatin
1112
Thin gray-white fishy odor >4.5 pH Clue cells Tx
Gardnerella vaginalis Part of normal flora Metronidazole Clindamycin
1113
Budding yeast and peudohyphae
Candidiasis
1114
Strawberry cervix
Trichomoniasis
1115
Perinuclear cytoplasmic clearing
Koilocytes HPV
1116
What can have painful inguinal lymphadenopathy
Bubo Lymphogranuloma venereum Chlamydia trachomatis
1117
Identify 1) Multiple painful ulcers 2) Painful single ulcer 3) Painless ulcer, raised indurated edges 4) School of fish 5) Granulomatous lesions on skin ( large areas of dying flesh)
1) Herpes simplex 2) Haemophilus ducreyi 3) Syphilis 4) Haemophilus ducreyi 5) Tertiary syphilis - Gummatous syphilis
1118
Adhesion to liver | Violin strings
FItz-Hugh Curtis Extension of PID
1119
WBC cast
Formed in tubules of kidney Pyelonephritis
1120
Gram negative rod Swarming motility Urease positive Forms what
Proteus mirabilis Hydrolyzes urea to ammonia Urine more alkaline Struvite stones Magnesium ammonium phosphate stones Staghorn calculus
1121
Staghorn calcus
Urease producing bacteria Proteus mirabilis Kelbsiella
1122
Rash followed by desquamation of hands and feet (sloughing of skin) Snuffles (blood tinged nasal secretions) Skeletal abnromalities Hepatomegaly
Early manifestations Congenital syphilis (during first 2 years)
1123
``` Frontal bossing Interstitial keratitis Hutchinson teeth Saddle nose Perforation of hard palate Sabershins ```
Late mnaifestation of congenital syphilis
1124
Infects transfered through placenta or during birth
TORCHeS ``` Toxopolasmosis Other (parvovirus B19) Rubella CMV HIV/HSV Syphilis ```
1125
Pregnant lady and cat feces or undercooked meat - What happens
Toxoplasmosis Chorioretinitis Hydrocephalus Intarcranial calcifications
1126
Child | Red rash on cheek spread to chest and shoulder
Parvovirus B19
1127
Severe fetal anemia Ascites Pleural effusions
Hydrops fetalis Parvovirus B19
1128
``` Newborn Cataracts Harsh machine like murmur Deafness Blueberry muffin rash ```
Rubella
1129
Newborn Jaundice Hepatosplenomegaly Sensorineural hearing loss
CMV
1130
Nosocomial infection 1) Catheter assoc UTI 2) Ventilator assoc pneumonia 3) Central lines infxn 4) Surgical wounds/ decubitus ulcers 5) Parenteral nutrition
1) E. coli Gram neg rods Pseudomonas Candida 2) Pseudomonas Staph aureus 3) Staph epidermdiis 4) Staph aureus 5) Candida
1131
Osteomelitis | Gangrene organism
Clostridium perfringens Alpha toxin
1132
Wolff parkinson White syndrome
D wave (curving of slope between P and R wave Ventricular pre-excitation syndrome Abnormal fast accessory conduction pathway from atria to ventricle (bypass AV) Ventricles begin to partially depolarize earlier --> SVT
1133
Infantile cataracts
Galactokinase deficiency Classic galactosemia Rubella
1134
Vit B12 used for
Methionine synthase
1135
Herpes virus
dsDNA
1136
Calcium stone due to diuretic
Thiazide diuretic
1137
Cataracts and deafness
Rubella
1138
Migraine NT
Excessive glutamate
1139
Liver abscess
E histolytic Protozoan Amebic dysentery
1140
Bloody dirarrhea | Eosinophils
E. histolytica
1141
Saccular aneurysm
aka berry aneurysm ACA
1142
Empysema
Increase compliance | Increases alveolar dead space
1143
Hesselbach triangle?
Inguinal ligament Inf. epigastric Lateral border rectus abdominis
1144
Carbamoyl phosphate synthetase (1 and 2)
CPS-1 - urea cycle - mitochondria - source of nitrogen: ammonia CPS-2 - pyrimidine synthesis - cytosol - source of nitrogen: glutamine
1145
Rate limiting step in pyrimidine synthesis
Carboamoyl phosphate synthetase 2
1146
Rate limiting step in purine synthesis Inhibited by
Glutamine PRPP amidotransferase 6-mercaptopurine
1147
Sources of carbon in sythesis of purines? In pyrimidine synthesis?
Pyrimidine: aspartate, CO2 Purine= CO2, glycine, tetrahydrofolate
1148
What medication inhibits each 1) Ribonucleotide reductase 2) Dihydrofolate reductase 3) Thymidylate synthase 4) Inosine monophosphate dehydrogenase 5) PRPP amidotransferase`1)
1) Hydroxyurea 2) Trimethoprim Methotrexate 3) 5-fluorouracil 4) Mycophenolate 5) 6-mercaptopurine
1149
How many adenine residues are found in a molecule of DNA if one strand contains A= 2000 G= 500, C= 1500 T=1000
2000 + 1000= 3000
1150
Self-mutilating behavior intellectual disability Gout - Deficency - inheritence - tx
Lesch-Nyhan syndrome Deficency HGPRT X linked Allopurinol Febuxostate
1151
Orotic acid in urine + elevated serum ammonia - inheritence - causes
Ornithine transcarbamylace deficiency X linked recessive Excess carbamoyl phosphate --> orotic acid Hyperammonemia Decreased BUN No megaloblastic anemia
1152
Orotic acid in urine + normal serum ammonia - deficency - inheritence - feature seen - tx
Orotic aciduria Deficiency of UMP synthase AR Megaloblastic anemia Tx: Uridine supplementation
1153
Megaloblastic anemia that does not improve with B12 and folate
Orotic aciduria
1154
Histones consist of what amino acids
Lysine and arginine
1155
Pyrimidine synthesis requires what four things
Aspartate Co2 Glutamine ATP
1156
Purine synthesis requires what 5 things
Cats PURRR until they GAG and Cough on Fur balls Purine synthesis GAG= glycine, aspartate, glutamine Cough= CO2 Fur balls= folic acid or tetrahydrofolate
1157
SCID due to
Adenosine deaminase deficiency
1158
DNA Polymerase I: DNA polymerase III:
I: Breaks down RNA primer fills in gap w/ DNA III: leading strand 5 --> direction Lagging stand, short discontinous segments
1159
DNA enzyme that can go in 3--> 5 direction and 5--> 3 direction
DNA polymerase I 3--> 5 remove 1 nucleotide at a time 5--> 3 remove 1-10 nucleotides
1160
DNA polymerase alpha DNA polymerase beta DNA polymerase delta DNA polymerase episilon DNA polymerase gamma
Alpha - makes own primer - replicates first 20 nucleotides Beta - DNA repair Delta - Build okazaki fragments on lagging strand Episilon: Elongates teh leading strand Gamma: Replicates mitochodnrial DNA
1161
Base excision repair (5)
Glycosylase - recognized damaged bases and removes them Lyase - cuts 3' end of back bone Endonuclease - cuts the 5- end and removes backbone DNA polymerase beta - Fills gaps Ligase -seals
1162
Mismatch repair
Nick daughter strand upstream and remove a whole bunch of bases Redo replication and fill gap
1163
DNA repair that repairs both strands
Nonhomologous end joining
1164
Hereditary nonpolyposis colorectal cancer DNA defect | -risk of
Defect mismatch repair Increased risk of colon cancer
1165
Xeroderma pigmentosum DNA repair defect - inheritence - cant do - increased risk
AR Defective nucleotide excision repair Sensitive to sunlight Cant remove pyrimidine dimers from UV radiation Increased risk of skin cancer, melanoma, SCC, basal cell
1166
Bloom syndrome - DNA defect - Sensitive to - increased risk
Mutation of helicase - DNA replication - DNA repair Hypersensitive to sunlight Increased risk of leukemia and lymphoma
1167
Ataxia-telangiectasia defect - syndrome - elevated - sensitive to
Defect repair of dsDNA breaks IgA deficiency Cerebellar ataxia Smooth persuit with eye Elevated alpha fetoprotein after 8 months Sensitive to ionizing radiation
1168
BRCA1 and BRCA 2 DNA repair defect
dsDNA breaks
1169
What strand of DNA nucelotide opposes this DNA strand? 5' - ATTGCGTA-3'
5'- TACGCAAT- 3'
1170
Which eukaryotic DNA polymerase performs this function 1) Replicates lagging strand 2) Synthesizes RNA primer 3) Repairs DNA 4) Replicates mitochondrial DNA 5) Replicates leading strand DNA
1) DNA polymerase delta (first 20 alpha) 2) Alpha 3) Beta 4) Gamma 5) Epsilon (alpha first 20)
1171
What gets compressed in pregnant female to decrease blood pressure when supine
Inferior vena cava
1172
DNA promoter regions
- 25 Hogness/ TATA box - 75 CCAAT box - 10 Pribnow/ TATAAT box
1173
Operator region of transcription
Binds repressor or inducer Located between promoter region and start site
1174
Response elements of trascription
Enhancer region and repressor region Increase or decrease the rate of transcription Location: close to, far from or within the promotor region
1175
mRNA tRNA rRNA
mRNA - messenger RNA - coupled to DNA template - transcribed into proteins - largest type - made in nucleoplasm tRNA - transfer RNA - matches to mRNA and brings AA, transfers mRNA to polypeptide chain - Smallest type - made in nucleoplasm rRNA - Part of ribosome - binds AA together - Most abundant - made in nucleus
1176
Start codon
AUG Methionine
1177
Stop Codons
UGA UAA UAG
1178
What breaks down lactose into two particles
beta-galactosidase Into glucose and galactose
1179
What do you need for transcription of beta-galactosidase. When is it present
CAP (catabolite activating protein) -Present when glucose absent Allolactose - lactose metabolite binds to lac repressor protein and changes shape - Present with abundant lactose
1180
5- cap - what is it - provided by
1- methyl-guanosine cap Provided by s-adenosyl-methionine (SAM)
1181
specific signal to start polyadenylate tail
AAUAAA
1182
Substances that inhibit reuptake of NE
``` Cocaine TCAs SNRIs - venlafaxine - duloxetine Methylphenidate Bupropion ```
1183
When enzyme matches amino acids to tRNA
Aminoacyl tRNA synthetase
1184
Tendon xanthomas Cafe au alit spot Boot shaped heart
Familial hypercholesterolemia NF1, McCune albright syndrome RVH --> tetralogy of fallot
1185
Triad of tuberous sclerossi
Seizures Intellectual disability Angiofibromas (reddish borwn bumps on nose/ cheeks)
1186
Autosomal dominant inheritence Disease?
Both male and female Every generation affected 50% in any generation ``` Von Hippel Lindau Von Willebrand Dis ALS Retinoblastoma MEN Tuberous sclerosis Hereditary spherocytosis Huntington's disease Marfan Ehler's danlos syndrome Neurofibromatosis Familial adenomatous polyposis Osler-weder-Rondu Achondroplasia Family hypercholesterolmia ADPKD ```
1187
X linked dominant disease
Fragile X
1188
Autosomal recessive | Type
25% offspring ONly 1 generation ``` Alpha 1 AT Beta-thalassemia PKU Hemochromatosis Wilson's dis Bernard-Soulier Hartnup syndrome Glanzman thrombasthenia Fanconi Cystic fibrosis Pompe dis Sphingoolipidoses Mucopolysaccharidoses Sickle cell disease Cori disease McArdle Albinism Von Gierke ```
1189
X linked recessive
50% sons affected ``` Hunter Lesch-Nyhan Fabry disease Meinke's disease Wiskott Aldrich syndrome G6PDH Bruton's agammaglobulinemia Duchenne's muscular dystrophy Hemophilia A Hemophilia B Diabetes insipidus ```
1190
Single gene has more than one effect Mutations oat different loci have same phenotype
Pleiotrophy Locus heterogeneity
1191
Prader-Willi
POP Prader willi Overeating Obesity Paternal gene
1192
Angelmann
``` MAMA Maternal gene Angelmann Mood- happy laughter Ataxia ```
1193
Southern blot Northern Blot Western blot Southwestern blot
Southern - DNA sample, DNA probe Northern - RNA sample, DNA probe Western - Protein sample, Ab probe Southwestern - Transcription factors, DNA binding proteins - DNA binding proteins sample, Oligonucltide probe
1194
Direct ELISA test for
Antigens
1195
Indirect ELISA test for
Antibodies
1196
Hallmark of COPD on pulmonary function testing
Decreased FEV1/ FVC
1197
Schizophrenic brain
Smaller Enlarged ventricles Thin cortex Increase dopamine in mesolimbic Decrease dopamine in mesocortical
1198
RNA made from different polymerase RNA polymerase I RNA polymerase II RNA polymerase III
I= rRNA II= mRNA III= tRNA
1199
Hallmark of restrictive lung disease
Decrease in total lung capacity | Normal FEV1/ FVC ratio
1200
Dystonia
sustained muscle contraction
1201
Adjustment disorder
Identifiable psychosocial stressor <6 months after stressor gone
1202
Virchow node
Left supraclavicular LN Gastric cancer metastasis
1203
Pattern of behaviors that violate social norms and rights of others
Conduct disorder Theft Violence Fire Cruelty to animals < 18 conduct > 18 antisocial personality disorder
1204
Trichotillomania Tx
Hair pulling disorder Education Cognitive behavioral Fluoxetine (SSRI) Clomipramine (TCA)
1205
Redirection of emotions or impulses to a neutral person or object
Displacement
1206
Partially remaining at a more childish level of development Surgeon throws tantrum in operating room because last case ran late
Fixation
1207
Separating feelings from ideas and events Describing murder with not emotional response
Isolation
1208
Replacing a warded-off idea or feeling with an (unconsciously derived) emphasis on its opposite Lustful thoughts so go to church
Reaction formation
1209
Alleviatin negative feelings via unsolicited generosity, which provides gratification mafia boss makes large donation of charity
Altruism
1210
Replacing an unacceptable with with a course of action that is similar to the wish but socially acceptable Teenagers aggression towards parents because of high expectations is channeled into excelling in sports
Sublimations
1211
30 year old says you are the best doctor shes ever had, nurses are the worst. Threatens to change doctors when you wont do a particular lab test Personality disorder?
Borderline personality disorder Splitting Fear of abandonment Self mutilation
1212
Women comes into office wearing all black, and excessive lipstick. Talks to everyone in waiting room. Wearing feather boa Also?
Histrionic personality disorder Sexually provocative
1213
A patient with IL-12 receptor deficiency is at particualr risk for what type of infection?
Mycobacterial
1214
Alcohol has affects on what receptors
GABA
1215
``` Alcohol in hospital for 2-3 days Sudden confusion Nightmares Agitation Disorientation Hallucinations Fever HTN Seizures ``` Tx
Delirium Tremens Benzo Alcohol
1216
Wernicke-Korsakoff damages what
Medial thalamus and mamillary bodies of posterior hypothalamus
1217
What embryologic structure of the heart gives rise to each of the following adult structures? 1) Smooth parts of the left and right ventricles 2) Smooth part of the right atrium 3) Trabeculated left and right atria 4) Trabeculated parts of the left and right ventricles
1) Smooth parts of the left and right ventricles = Bulbus cordis 2) Smooth part of the right atrium = Right horn of sinus venosus 3) Trabeculated left and right atria = primitive atria 4) Trabeculated parts of the left and right ventricles = primitive ventricle
1218
Mesonephros - does what - becomes
Fetal kidney (1st trimester) Becomes male genital system
1219
Potter sequence due to
Failure of ureteric bud to develop No collecting system Nothing to induce metanephros to develop into nephron Bilateral renal agenesis
1220
Horseshoes kidney gets stuck on Associated iwth
Inferior mesenteric artery Turners syndrome
1221
What remains in capillaries after filtration
Blood cells Platelets Large proteins Exits through efferent arterioles
1222
Juxtaglomerular apparatus composed of
Juxtaglomerular cells - secrete renin Macula densa - specialized sensory cells - senses if Na in lumen of distal nephron is too low
1223
Three things that cause JG cells to secrete renin
Beta adrenergic stimulation Low Na in DCT Low pressure in afferent arteriole
1224
Effect of GFR, RPF, FF 1) Increase serum protein 2) Ureter stone obstruction 3) ACE inhibitors 4) NSAIDS
1) Osmotic load keeps fluid in GFR: Decrease RPF: No change FF: Decrease 2) High hydrostatic pressure in Bowman capsule, favors blood staying in capillaries GFR: decrease RPF: No change FF: decrease 3) Dilate efferent GFR: Decrease RPF: Increase FF: Decrease 4) Afferent constriction GFR: decrease RPF: decrease FF: no change
1225
How much of body is water? Extracellular fluid Interstitial fluid Plasma volume Intracellular fluid
60% total 40% Intracellular 20% Extracellular - 75% Interstitial fluid - 25% Plasma
1226
Renal clearance=
UV/P Urine concentration of substance x urine flow rate / plasma concentration substance
1227
Substance that gives GFR Normal GFR
Inulin Creatinine 90-135 (think 100)
1228
Substance that gives Renal plasma flow
PAH
1229
Filtration fraction=
GFR/ RPF Normally 20%
1230
Prostaglandin effect of GFR, RPF, FF
Vasodilators | Dilate afferent arterioles
1231
Angiotensin II on GFR, RPF, FF
Constrict efferent arterioles
1232
Filtered load= Reabsorbed amount= Secretion=
FL= GFR x P (plasma) Reabsorbed= filtered - excreted Secretion= excreted - filtered
1233
A 55-year-old woman has had worsening problems with memory and the ability to carry out tasks of daily living over the past year. She has had watery diarrhea for the past 3 months. Physical examination shows red, scaling skin in sun-exposed areas. Deep tendon reflexes are normal, and sensation is intact. Which of the following diseases is she most likely to have? A Beriberi B Cheilosis C Hypothyroidism D Marasmus E Pellagra
Pellagra 3D's Hartnup disease Deficient transporter for neutral amino acids (tryptophan) Inability to make niacin (Vit B3)
1234
What is HUS
Hemolytic uremic syndrome Associated w/ E.coli 0157:H7 Hemolytic anemia (red blood cells being destroyed) Thrombocytopenia Acute kidney injury
1235
What is absorbed in PCT first half
All glucose All amino acids 2/3 fluid 2/3 electrolytes Reabsorbs primarily - Bicarb - Na
1236
Na transporter - PCT (early) - PCT (late) - Thick ascending - Early distal tubule
PCT (early) - Na/ X (glucose, AA, lactate) into cell PCT (late) - Na reabsorption is with chloride Thick ascending - Na/K/ 2CL transporter Early distal tubule - Na/ Cl-
1237
Relative concentrations of ultrafiltrate along proximal tubule (graph)
"Plasma Concentrations Increase Unless Cleared by Kidneys" ``` PAH Creatinine Inulin Urea Chloride Potassium ``` Sodium Water HCO3 Aminoacids Glucose
1238
What do you need to get an anion from interstitium to lumen What do you need to get an cation from interstitium to lumen
alpha-ketoglutarate ``` Cation - Na/K ATPase (2 Na/ 3 K) - electrochemical gradient - Cation move down gradient in to cell Cation/H exchange into lumen ```
1239
Responsible for water reabsorption | Impermeable to Na
Thin descending limb Water follows osmotic gradient into hypertonic medulla Concentrates lumen
1240
Thick ascending limb - fxn - what also gets in - what doesnt - Result
Actively absorbing Na, Cl, and K (passive in PCT) Ca and Mg sneak inbetween cells Impermeable to water Dilutes urines
1241
PTH acts on
Ca in early distal convoluted tubule
1242
ADH works on
V2 vasopressin receptors on principal cells in collecting tubule Tells cells to insert aquaporins into lumen surface of cells Allows water to be resorbed Lithium - Enters through Na channel - Blocks aquaporin
1243
Potassium-sparing diuretics (2 types)
"SEAT" Aldosterone antagonists - Spironolactone - Eplerenone Inhibit epithelial Na channel - Amiloride - Triamterene
1244
What class of drug inhibits Na/2Cl/K symporter in thick ascending limb
Loop diuretics Cause isotonic water excretion
1245
Two cells in collecting duct and function
Principal cells - reabsorb water and Na - secrete K Intercalated cells - Secrete H or HCO3 - reabsorb K
1246
Two types of intercalated cells
``` Alpha cells (A cells) - Secrete H ``` ``` Beta cells (B cells) Secrete HCO3 ```
1247
What diuretic directly affects principal cells
Potassium sparing diuretics
1248
What effect does aldosterone have on the principal cells and intercalated cells of the collecting duct?
Principal= reabsorptionof Na and secretion of K Intercalated cells: stimulates secretion of H
1249
What bacteria secrete enterotoxins?
``` Vibrio cholerae Enterotoxigenic E.coli Staph aureua Shigella Yersinia Clostridium spp. ```
1250
Charcot's neurological triad
Associated iwth MS Scanning speech Intention tremor nystagmus
1251
Damage to which part of substantia nigra causes hypokinesia
Substantia nigra pars compacta
1252
What shifts potassium out of cells --> hyperkalemia
``` Low insulin Beta blockers Acidosis Digoxin Cell lysis ```
1253
What shifts potassium into cells --> hypokalemia
Insulin beta agonist Alkalosis Cell creation
1254
You have a patient with an EKG with tall peaked T waves Tx?
Hyperkalemia 1st: IV calcium - to prevent arrhythmias - wont correct hyperkalemia To correct hyperkalemia: Beta agonist (albuterol) IV bicarb IV insulin + dextrose
1255
Atrial natriuretic peptide - Released from - Due to - MOA - Effect
Release by atria of heart due to increased atrial volume and atrial pressure ANP on kidneys --> constrict efferent arterioles and dilates afferent arterioles Increase GFR, diuresis
1256
SIADH caused by
Too much ADH ``` Small cell lung cancer COPD, pneumonia Head trauma Stroke CNS infection Drugs: cyclophosphamide ```
1257
Locked in syndrome
Central pontine myelinolysis Correct hyponatremia too quickly Causes lysis of myelin and pons
1258
Diabetes insipidus
Too little ADH Cant concentrate urine Central - Abnormal ADH production by hypothalamus Nephrogenic - Kidneys unresponsive to ADH - Lithium, hypercalcemia, mutation
1259
Patient with excessive thirst and polyuria What to do?
1) First think DM - check urine and serum glucose 2) Check urine and serum osmolality - Low urine osmolality - High serum osmolality 3) Water deprivation test - Healthy: dehydrate --> secrete ADH --> concentrate urine - DI: urine osm stays low 4) Desmopressin challenge - ADH analog - Central: increase osm - Nephrogenic: no change
1260
Polymyositis vs polymyalgia rheumatica
Polymyositis - muscle weakness - increased CK and aldolase - Increased ANA - Positive Anti-Jo Polymyalgia rheumatica - Joint pain - No muscle pain - No weakness - Normal Ck and aldolase
1261
MPTP exposure causes depletion of waht
Dopamine
1262
pH pCO2 HCO3
``` pH= 7.35-7.45 pCO2= 35-45 HCO3= 22-28 ```
1263
Anion gap
[Na]- [Cl] - [HCO3] 10-12 normal
1264
Adding acids to blood does what to anion gap
Add acid, buffered by bicarb Lowers bicarb amount Increases anion gap --> High anion gap
1265
High anion gap acidosis
"MUDPILES" ``` Methanol Uremia (renal failure) Diabetic ketoacidosis Propylene glycol Iron tablets/ Isoniazid Lactic acid (shock or coding) Ethylene glycol Salicylastes (aspirin) ```
1266
Normal anion gap acidosis
DIarrhea Renal tubular acidosis Spironolactone Acetazolamide
1267
Normal anion gap metabolic acidosis | Urine pH > 5.5
Renal tubular acidosis Type I Alpha intercalated cells in collecting tubule unable to secrete H Hypokalemia
1268
Normal anion gap metabolic acidosis Urine pH < 5.5 Hyperkalemia
Renal tubular acidosis Type 4 Often due to hypoaldosteronism If low not putting potassium into urine Hyperkalemia Prevents PCT from generating NH4
1269
Normal anion gap metabolic acidosis Urine pH < 5.5 Hypokalemia
Renal tubular acidosis Type 2 Proximal tubule defect of HCO3 reabsorption Hypokalemia hypophosphatemia
1270
Renal tubular acidosis three types
Type 1 - Impaired hydrogen (H) excretion 1 letter Type 4 - impaired ammonium (NH4) excretion - Type 4= NH4 = aldo Type 2 - impaired BIcarb reabsorption
1271
pH equation
HCO3/ pCO2
1272
Nephritis vs Nephrosis
NephrOsis - Proteinuria > 3.5 g day - Hyperlipidemia - Hypoalbuminemia Nephritis - Proteinuria < 3.5g - HTN - Hematuria - Hardly any urine (oliguria) I= eye= symptom you can see
1273
Poststreptococcal glomerulonephritis
PSG= 3 letters Lump3- Bump3 C3 3 wks poststrep Type 3 rxn Subepithelial humps Coca-cola urine Decrease C3 Increase anti-streptolysin O Decrease anti-DNase B
1274
Rapidly progressive glomerulonephritis
"Crescentic" Rapidly crescentic glomerulonephritis Crescentic shaped Made up of fibrin and complement and cellular debris
1275
Diffuse proliferative glomerulonephritis
Dilupus Wire loops= wire lupus SLE
1276
Thin distinct membrane
Wire loop SLE Anti ds DNA Lupus Diffuse proliferative glomerulonephritis
1277
Vision problems Deafness Protein in urine Coughing up blood
Cant see Cant pee Cant hear high C Alport syndrome Splitting of basement membrane Defect in Type IV collagen Al-4 Type IV 4 things Lung, kidney, ear and eyes
1278
Goodpasture syndrome
Goodpasture= 2-pasture Type II hypersensitivity 2 organs (lung + kidneys) Ab vs Type IV collagen
1279
Wegeners Gramulomatosis
Granulomatosis w/ polyangiitis ``` We-C-ners C over stick figure - nasopharynx - lungs - kidney ``` C-anca
1280
``` Palpable purpura on butt and legs Abdominal pain Increased IgA RBC casts Hematuria ```
IgA Nephropathy Henoch-Schonlein purpura
1281
What nerve most likely damaged in fibula neck fracture
Common peroneal n.
1282
Defining features of nephrotic syndrome
Proteinuria > 3.5 g/day Hypoalbuminemia Peripheral edemia Hyperlipidemia
1283
Flattening or effacement of podocyte foot process Child Post URI Tx
Minimal change disease Corticosteriods Minimal - kids= minimal age - foot= minimal body part
1284
HIV patient | Proteinuria
Focal segmentla glomerulosclerosis (FSGS)
1285
Spike and Dome
Membranous Glomerulonephropathy Thickening of basement membrane Hep B and Hep C Subepithelial immune deposits
1286
Membranous glomerulonephropathy
" suck some dick to become a MEMBER of the club" Blow job= Dome Who sucks dick= Hepatitis
1287
Membranoproliferative glomerulonephritis
Prolife "Train Track" Hep B and C Subendothelial humps
1288
Focal segmental Glomerulosclerosis (FSG)
FSG HIV Half glomerulus affected
1289
Round acellular nodules
Kimmelstiel wilson nodule Diabetic nephropathy
1290
Congo red stain | Apple green birefringence
Amyloidosis
1291
Glomerular histology reveals multiple mesangial nodules. THis lesion is indicative of what disease
Diabetic nephropathy
1292
Prerenal azotemia vs intrinsic renal disease
Prerenal - Urine Na < 20 mEq/L - FE(Na) < 1% - BUN:CR >20:1 Intrinsic - urine Na > 40 FE (Na) > 2% BUN:Cr 10-15:1
1293
Urinary Casts made up of Secreted by
Tamm-Horsfall mucoprotein Secreted by tubule epithelial cells
1294
Muddy Brown Casts Drugs that cause
Acute tubular necrosis Most common cause of acute kidney injury Acute necrosis or death of epithelial cells Aminoglycosides Cisplatin
1295
Azotemia
Nitrogen in blood High urea High creatinine
1296
Fever Eosinophilia Azotemia Maculopapular rash
Acute interstitial nephritis (AIN)
1297
Gross hematuria Flank pain Azotemia HTN
Renal papillary necrosis
1298
1) Hyaline casts 2) RBC casts 3) WBC casts 4) Epithelial cell casts 5) Granular casts 6) Fatty casts 7) Waxy casts
1) Hyaline casts= Normal patient with concentrated urine 2) RBC casts = Glomerular bleeding (glomerulonephritis, vasculitis) Yellow brown 3) WBC casts= Tubular interstitial disease, acute pyelonephritis, proliferative glomerulonephritis 4) Epithelial cell casts= Acute tubular necrosis, acute interstitial nephritis, glomerulonephritis 5) Granular casts= Acute tubular necrosis, muddy brown 6) Fatty casts= nephrotic syndrome, contain cholesterol (maltese cross) 7) Waxy casts= end stage renal disease
1299
Basic metabolic panel for patient with renal failure
``` INcreased BUN adn Cr Increased K Decrease Calcium Decrased bicard (metabolic acidosis) ```
1300
WBC casts
Pyelonephritis
1301
What is associated iwth AD polycystic kidney disease
Hepatic cysts Intracrnail aneurysms Mitral valve prolapse
1302
ADPKD vs ARPKD
ADPKD - mutation: PKD1 (chr 16) , PKD2 (chr 4) ARPKD - mutation PKHD1 (chr 6) - infancy oliguria --> potter
1303
Small shrunken kidneys and fiborsis
Autosomal dominant tubulointerstitial kidney disease aka medullary cystic kidney disease Progressive renal failure
1304
Stone 1) Envelope (square) 2) rectangle (coffin lid) 3) hexagon 4) rhombus or rosette
1) Calcium oxalate 2) struvite 3) cystine 4) uric acid
1305
Renal cell carcinoma - arises from - deletion --> - triad - secretion - pathology
PCT in the cortex Deletion on Chr 3 (Von Hippel Lindau dis) Flank pain, hematuria palpable abdominal mass Polycythemia - erythrocytosis due to excess erythropoeitin production Solid tumor wit "clear cells" full of lipids and carbohydrates
1306
Intellectual disability Absence of iris renal tumor Child Also see
Wlims tumor (nephroblastoma) | Also see genitourinary malformations Cryptorcidism, bicornuate uterus
1307
Painless hematuria Major risk
Transitional cell carcinoma (urinary tract) Smoking Aniline dyes Naphthylamine dyes Cyclophosphamide
1308
WAGR complex
Wilms tumor Aniridia (no iris) Genitourinary malformation Mental retardation
1309
Organism that causes bladder cancer
Squamous cell bladder cancer schistosoma haematobium Middle east Urinary blood fluke Trematode
1310
Developmental milestones 3 m 6 m 9 m 12 mo
3 mo 3= before everything else - roll - hands together - laugh and squeal - smile 6 mo "Six= S's" - Sit up - Switch objects between hands - Shmooze - Self feed - Stranger danger 9 mo "9= P's (9 in penis) - Pull itself (crawl) - Pincer grasp - Papa - play - permance (object) 12mo "TWELVE= T's" - Two legs (stand, walk) - Track ( object tracking), block in a cup - 2 words - 2 of use (separation anxiety) - drink from cup
1311
Apgar score
1 and 5 minutes after delivery 0-10 Appearance 0= cyanotic 1= blue extremities 2= pink Pulse 0= absent 1= < 100 2= > 100 Grimace 0= no response 2= cries and pulls away from stimulus Activity 0= limp and floppy baby 2= flexed arms and legs, resist extension Respiration 0= absent effort 1= slow irregular or labored 2= crying and vigorous breathing
1312
What zoonotic bacterium causes each 1) Cat scratch fever 2) Lyme disease 3) Recurrent fever from variable surface antigens 4) Bloody diarrhea 5) Q fever 6) Tularemia 7) Leptospirosis 8) Cellulitis and osteomyelitis from cat or dog bites
1) Cat scratch fever - Bartonella 2) Lyme disease - Borrelia burgdorferi 3) Recurrent fever from variable surface antigens - Borrelia recurrentis 4) Bloody diarrhea - Campylobacter (puppies, livestock, fecal oral, sexual) 5) Q fever - Coxiella burnetti (spores from tic feces, cattle placenta) 6) Tularemia - Francisella tularesis 7) Leptospirosis - Leptospira spp (animal urine) 8) Cellulitis and osteomyelitis from cat or dog bites - Pasteurella multocida
1313
First step in glucose utilization
Phosphorylate it to trap in muscle Hexokinase Glucokinase - liver and beta cells
1314
Main enzymes in glycolysis pathway
1. Hexokinase/ glucokinase 2. Phosphofructokinase-1 3. Pyruvate kinase
1315
Muscle biopsy on patient reveals elevated glycogen levels, elevated fructose-6- phosphate, and decreased pyruvate. What enzyme is deficient?
Phosphofructokinase-1 deficiency
1316
Insulin on glycolysis pathway Glucagon on pathway
Doesn't directly stimulate glycolysis Stimulates Phosphofructokinase-2 (fed) --> Forms fructose-2,6 BP Glycogen formation stimulated by insulin GLucagon - stimulates FBPase-2 (fasting) - -> Fructose-6-P
1317
Increase in fructose-2,6 BP leads to
Decrease conversion of alanine --> glucose
1318
To get ATP need Which requires That is from What is needed for that That comes from Get that from This occurs where TCA and oxidative phosphorylation occur where
ATP from Oxidative phosphorylation NADH needed for oxidative phosphorylation is from TCA cycle Need acetyl-CoA for TCA cycle Acetyl-CoA from decarboxylation of pyruvate Pyruvate is from glycolysis of glucose Glycolysis in cytosol TCA and oxidative phosphorylation are in mitochondria
1319
Aerobic metabolism two different types of shuttle
Malate-aspartate shuttle - Heart liver and kidneys - 32 ATP Glycerol-3-phosphate shuttle - brain adn skeletal muscles - 30 ATP
1320
GLUT Transports GLUT 1 GLUT 2 GLUT 3 GLUT 4 GLUT5
GLUT1 - RBC - Endothelium of BBB - mediates basal glucose uptake (low level) - Takes up glucose regardless of insulin GLUT2 - Found on cells that regulate glucose - Hepatocytes, pancreatic beta cells GUT3 - Neurons - Placenta GLUT4 - Skeletal muscle - Adipose tissue - INSULIN dependent GLUT5 - Fructose uptake in GI tract
1321
Gluconeogenesis steps
Pyruvate --> Oxaloacetate (pyruvate carboxylase w/ biotin) (+) acetyl-CoA Oxaloacetated -->Phosphoenol pyruvate (PEP carboxykinase ) ... Fructose-1,6-BP --> Fructose6P (Fructose-1,6-bisphosphatase) RL (+) ATP (-) AMP, Fructose 2,6 BP Glucose 6 phosphate --> Glucose (Glucose-6-phosphatase)
1322
Rate limiting enzymes 1) De novo pyrimidine synthesis 2) De novo purine synthesis 3) Glycolysis 4) Gluconeogenesis 5) Glycogen synthesis 6) Glycogenolysis 7) TCA cycle 8) Hexose monophosphate shunt
1) carbamoyl phosphate synthetase-2 2) glutamine PRPP amidotransferase 3) Phosphofructokinase -1 4) Fructose-1,6- bisphosphatase 5) Glycogen synthase 6) Glycogen phosphorylase 7) isocitrate dehydrogenase 8) glucose-6-phosphate dehydrogenase
1323
What irreversible enzymes are involved in gluconeogenesis
Pyruvate carboxylase w/ biotin PEP carboxykinase Fructose-1,6- bisphosphatase Glucose-6- phosphatase
1324
RL step of gluconeogenesis
Fructose-1,6- bisphosphatase
1325
Order molecules based on energy the contain ``` Pyruvate Adenosine monphosphate Glucose Adenosine Adenosine triphsphate ```
Glucose > pyruvate > ATP > AMP > adenosine
1326
Flow of aqueous humor
Formed in capillary bed of ciliary body - -> posterior chamber - -> flows between angle formed by lens and iris diaphragm - -> anterior chamber --> reabsorbed in canal of schlemm
1327
Glucose -6- phosphate --> glucose
Glucose-6- phosphate Last step in glycogenolysis and gluconeogenesis Deficency= Von Gierke
1328
Glycogenolysis To break alpha-1,4 glycosidic linkage need To break alpha- 1,6 glycosidic linkage need
alpha-1,4= glycogen phosphorylase (RL) alpha-1,6= alpha 1,6 glucosidase (aka debraching enzyme) - two functions - take 3 of last 4 glucose and move to end of other branch (transferase) - remove last glucose (alpha 1,6 glucosidase)
1329
Epinephrine effect of glycogen regulation
Beta adrenergic receptor stimulate adenylyl cyclase --> Glycogen phosphorylase --> Glycogenolysis
1330
Glycogen synthase - pathway - regulated by
Glucose 1 phospahte --> glycogen Glycogen synthesis (+) insulin
1331
Glycogen phosphorylase - pathway - regulated by
Glycogenolysis Glycogen --> Glucose -1- phosphate (+) glucagon (+) Epinephrine (+) cAMP
1332
Von Gierke
Lack of glucose-6-phosphatase Accumulation of Glucose-6-phosphate in liver Hepatomegaly, Hypoglycemia Hyperlipidemia, Hyperuricemia (fat/protein catabolism) - cant get free glucose have to get energy from fats and proteins Von GEEK disease - Fat - No Muscle (hyperuricemia) - Fatty liver
1333
Cori Disease
Defect in Debranching enzyme (alpha-1,6- glycosidic linkage) Same symptoms as Von Gierke Only different is abnormal glycogen structure Milder hypoglycemia Hepatomegaly No elevation in lactate levels No elevation in uric acid Cori disease= coral reef
1334
McArdle disease
Deficiency in glycogen phosphorylase Deficiency in Muscle McArdle= Muscle Muscle cramps on exertion Myoglobinuria Hypoglycemia on exertion Osmotic pressure from glycogen draws water into cells Cell lyses --> myoglobin into serum Rhabdomyolysis - -> Myoglobin in blood to kidney - -> Myoglobinuria - -> Renal failure
1335
Pompe disease
Deficiency of alpha-1,4, glucosidase in LYSOSOMES Infantile form (severe) - severe muscle weakness - CARDIOMEGALY and heart failure - Only live 1 year Adult form - No cardiac involvement - Gradual onset of skeletal muscle weakness - Diaphragm weakness - Respiratory failure POMPE volcano= build up in volcano, cardiomegaly, explodes
1336
A 2-year-old child with failure to thrive since infancy now exhibits a seizure. Physical examination shows hepatomegaly and ecchymoses of the skin. Laboratory studies show a blood glucose level of 31 mg/dL. A liver biopsy specimen shows cells filled with clear vacuoles that stain positive for glycogen. Which of the following conditions is most likely to produce these findings? A Hurler syndrome B McArdle disease C Pompe disease D Tay-Sachs disease E Von Gierke disease
E. Von Gierke Def glucose-6-phosphatase
1337
A 25-year-old woman stops going to her aerobic exercise class because of severe muscle cramps that have occurred during every session for the past 2 months. Four hours after each session, she notices that her urine is a brown color. On physical examination, she has normal muscle development and strength. An inherited defect in which of the following substances is most likely to explain her findings? A Dystrophin B Fibrillin C Glucose-6-phosphatase D Lysosomal glucosidase E Muscle phosphorylase F Spectrin
E. Muscle phosphorylase McArdle
1338
A 6-month-old male infant has failure to thrive and abdominal enlargement. His parents are concerned that he has shown minimal movement since birth. On physical examination, the infant has marked muscle weakness and hepatosplenomegaly. A chest radiograph shows marked cardiomegaly. He dies of congestive heart failure at age 19 months. The microscopic appearance of myocardial fibers at autopsy is shown in the figure. A deficiency of which of the following enzymes is most likely to be present in this infant? A Glucocerebrosidase B Glucose-6-phosphatase C Hexosaminidase A D Homogentisic acid oxidase E Lysosomal glucosidase F Sphingomyelinase
E. Lysosomal glucosidase Pompe
1339
An 8-month-old boy is brought to the pediatrician by his parents because he has recently lost the ability to crawl or hold his toys. On examination the patient is tachypneic and breathing with considerable effort; the liver is palpable fi ve fi ngerwidths below the right costal margin. X-ray of the chest reveals cardiomegaly. He has a diffi cult time sitting upright and cannot squeeze the physician’s fi ngers or the ring of his pacifi er with any noticeable force. Despite a number of interventions, the child’s symptoms continue to worsen until his death 2 weeks later. On autopsy, it is likely that this patient’s cells will contain an accumulation of which of the following substances? (A) Glucose (B) Glycogen (C) Oxaloacetate (D) Pyruvate (E) Urea
B. Glycogen Pompe disease
1340
A 14-year-old high school freshman presents to her family doctor for a sports physical. She has not played organized sports in the past but is in good physical shape. She mentions that she experienced severe leg cramps after trying out for the soccer team last week. The night after the tryouts, she noticed that her urine had a reddish tinge. She has no other medical complaints. Her physician orders an ischemic forearm exercise test, which reveals no increase in venous lactate. Which of the following enzymes is most likely defi cient in this patient? (A) Cystathionine synthase (B) Glucose-6-phosphatase (C) α-1,6-Glucosidase (D) Glycogen phosphorylase (E) Lysosomal α−1,4-glucosidase
D. Glycogen phosphorylase McArdle's disease
1341
What glycogen storage disease matches 1) Glycogen phosphorylase deficiency 2) Glucose-6-phosphatase deficiency 3) Lactic acidosis, hyperlipidemia, hyperuricemia (gout) 4) Alpha -1,6- glucosidase deficiency 5) alpha-1,4- glucosidase deficiency 6) Cardiomegaly 7) Diaphragm weakness leading to respiratory failure 8) Increased glycogen in liver, severe fasting hypoglycemia 9) Hepatomegaly, hypoglycemia, hyperlipidemia (normal kidneys, lactate, and uric acid) 10) Painful muscle cramps, myoglobinuria with strenuous exercise 11) Severe hepatosplenomegaly, enlarged kidneys
1) McArdle 2) Von Gierke 3) Von Gierke 4) Cori disease 5) Pompe 6) Pompe 7) Pompe 8) Von Gierke 9) Cori 10) McArdle 11) Von Gierke
1342
What is the landmark for a pudendal nerve lbock
Palpate for ischial spine
1343
Which enzyme catalyzes the RL step in carbohydrate digestion
Oligosaccharide hydrolases (at intestinal brush border)
1344
What vitamin deficiency results from Hartnup disease?
Deficiency tryptophan --> Niacin deficiency (vit B3) Pellagra
1345
Electron transport chain steps Energy required steps inhibited by
1) NADH reductase (NADH --> NAD) (Needs 2.5 ATP) (-) Amytal (-) Rotenone (-) MPP 2) Succinate dehydrogenase w/ Coenzyme Q (Succinate --> fumarate) (Needs FADH2, 1.5 ATP) 3) Cytochrome b + c1 (-) antimycin A 4) Cytochrome a +a3 (1/2 O2 +2H --> H2O) (-) CN (-) N3 - (-) CO (-) H2S 5) ATPase (ADP + Pi --> ATP) (-) Oligomycin A
1346
Cofactors for Pyruvate Dehydrogenase
Pyruvate --> acetyl-CoA ``` "TLC For Nobody" Thiamine pyrophosphate (vit B1) Lipoid acid Coenzyme A (vit B5) FAD- Vit B2 Nad- Vit B3 ```
1347
Garlic breath Vomiting Rice water stool
Arsenic exposure Inhibits lipoic acid No acetyl-CoA for TCA cycle
1348
Pyruvate dehydrogenase deficiency
Cant make acetyl CoA Back up of pyruvate - -> Lactate (Lactic acidosis) - -> Alanine (neurologic defects) X linked Arsenic exposure B vitamin deficiency
1349
TCA cycle main points
Acetyl-CoA --> citrate (Citrate synthase) Isocitrate --> alpha ketoglutarate (isocitrate dehydrogenase) (NADH + CO2) RL a-ketoglutarate --> succinyl-CoA (a-ketoglutarate dehydrogenase) (NADH + Co2) Highly regulated, needs all cofactors as pyruvate dehydrognase ``` "TLC For Nobody" Thiamine pyrophosphate (vit B1) Lipoid acid Coenzyme A (vit B5) FAD- Vit B2 Nad- Vit B3 ```
1350
Uncoupling agents on Electron transport chain
Uncouples the electron transport chain from ATPase, so it increases the permeability of inner mitochondria membrane to hydrogen ions, hydrogen ions can leak back in (wasted as heat) Thermogenin (brown fat, hybernation) High dose aspirin 2,4-dinitrophenol (2,4-DNP)
1351
What four end products can be made from pyruvate
Oxaloacetate (Pyruvate carboxylase) Acetyl-CoA (pyruvate dehydrogenase) Lactate ( Lactae dehydrogenase) Alanine (Alanine Transaminase)
1352
What four B vitamins are needed to make cofactors for pyruvate dehydrogenase
B1 (thiamine) B2 (riboflavin) B3 (niacin) B5 (pantothenic acid) plus lipoic acid
1353
Which TCA cycle enzyme requires the same cofactors as pyruvate dehydrogenase
alpha ketoglutarate dehydrogenase
1354
What cofactor is required by all transaminases
Pyridoxal phospahte (derivative of vitamin B6)
1355
Two main nitrogen transporters in blood
Alanine | Glutamine
1356
Oxidative burst pathway
O2 --> O2 (-) superoxide ion (NADPH oxidase) O2 (-) --> H2O2 hydrogen peroxide (Superoxide dismutase) H2O2 --> (+ Cl) --> HOCl hypochlorous acid (Myeloperoxidase)
1357
Steps to neutralize H2O2
1) React w/ reduced glutathione (antioxidant) --> glutathione disulfide + H2O [Glutathione peroxidase] 2) Glutathione disulfide --> GSH (Need NADPH) --> GSH + NADP+ [Glutathione peroxidase] 3) NADP+ --> NADPH (Need G6P) --> NADPH + 6PG [ Glucose-6-phosphate dehydrogenase]
1358
Deficiency in G6PD --> What substances can cause
Decrease in NADPH Decrease in GSH Cant neutralize H2O2 ``` "Spleen Purges Nasty Inclusions From Damaged Cells" Sulfonamides Primaquine Nitrofurantoin Isoniazid Fava beans Dapsone Chloroquine ```
1359
Hemolytic anemia | Bit cells
G6PD deficiency X linked
1360
Build up of fructose in urine | Asymptomatic
Essential fructosuria Deficency of fructokinase
1361
``` Hypoglycemic Vomiting Hepatomegaly Jaundice Elevated Fructose-1-P ```
Fructose intolerance Deficiency of aldolase B
1362
Infantile cataracts galactosuria galactosemia
Galactokinse deficiency Deficiency of galactokinase Accumulation of galactitol
1363
A 3-month-old infant presents with failure to thrive, poor feeding, and lethargy. A physical examination reveals an enlarged liver and jaundice. Laboratory analysis reveals an elevated blood galactitol level and increased urinary reducing substance. Which of the following could correctly describe the levels of intermediates of galactose metabolism in this patient? (A) Decreased galactose (B) Decreased uridine diphosphoglucose (C) Elevated glucose-1-phosphate (D) Increased galactose-1-phosphate (E) Increased glycogen
D. Increased galactose-1-phosphate
1364
``` Infantile cataracts Failure ot thrive Jaundice Hepatomegaly Intellectual disability ```
Classic galactosemia Deficiency of galactose-1-phosphate uridyltransferase Excess Galactose-1- phosphate Excess Galactose Excess Galactitol
1365
RL step of pentose phosphate pathway
Glucose 60 phosphate dehydrgenase G6PD
1366
Pentose phosphate pathway what is it used for pathway what tissues use it
aka HMP shunt Provides a source of NADPH from G6P G6P -->2 NADPH, Ribulose 5P [ G6PD] RBCs Liver Adrenal cortex mammary glands (during lactation)
1367
What disorder is caused by a deficiency of each enzyme 1) Galactokinase 2) Aldolase B 3) Lactase 4) Galactose-1- phosphate uridyltransferase 5) Fructokinase
1) Galactokinse deficiency 2) Fructose intolerance 3) Lactose intolerance 4) Galactosemia 5) Essential fructosuria
1368
Ketogenesis RL enzyme
HMG-CoA synthase
1369
Child super skinny arms and legs, proturbarant belly
Kwashiorkor Protein malnutrition - Fatty liver disease (cant make ApoB 100) Edema Anemia Skin lesions Depigmentation ``` "Flames" Fatty Liver Anemia Malnutrition Edema Skin lesions ```
1370
Child stick and bones
Marasmus Total energy malnutritoon (protein, fat, carbohydrates) Muscle wasting Subcutaneous fat loss
1371
Lipid transport pathway
1) Dietary fat broken down [ Pancreatic lipase] 2) Fat particles absorbed by enterocytes an dpacked into chylomicrons 3) Chylomicrons released with help of Apo-B-48 - W/O = abetalipoprotinemia 4) Chylomicrons pass cells that draw free fatty acids off [ Lipoprotein Lipase] 5) End up w/ chylomicron remnant --> Liver 6) Liver synthesizes VLDL from remnant 7) VLDL secreted into circulation with help of Apo-B- 100 8) Free fatty acids pulled off VLDL, VLDL becomes IDL 9a) IDL reabsorbed by liver 9b) IDL hydrolyzed further to become LDL [ Hepatic lipase] 10) LDL reabsorbed by liver or other cells, absorbed by endocytosis by LDL receptor
1372
Apo Proteins 1) Apo-B-48 2) Apo-B-100 3) ApoE 4) ApoA-I 5) ApoC-II
1) Helps release of chylomicrons from enterocytes - Without you get abetalipoprotinemia 2) Helps VLDL secretion into circulation from liver 3) Mediates extra remnant uptake 4) Activates LCAT, found on HDL 5) Cofactor for lipoprotein lipase
1373
HDL pathway
1) HDL produced by liver contains Apo-A-I 2) Has increased protein low TG = Nacent HDL 3) Takes up cholesterol [Lecithin-cholesterol acyltransferase (LCT)] 4) Now HDL-2 Exchanges cholesterol esters for TG with other lipoproteins [Cholesterol ester transfer protein (CETP)] 5) HDL full a. Hydrolyzed by Hepatic lipase b. Travels back to liver, Scavenger receptor B1 (SRB1) pulls of cholesterol esters
1374
Fatty acid metabolism - precursor molecule - location - RL enzymes
Precursor: acetyl-CoA Laction: cytoplasm of hepatocytes RL: Acetyl-CoA carboxylase
1375
Fatty acid degradation - Location - RL - Two outcomes
Mitochondria Carnitine acyltransferase 1 (carnitine palmitoyltransferase 1) Build up of ketones or Move into TCA cycle
1376
Weakness Hypotonia Hypoketotic hypogycemia
Deficiency of carnitine acyltransferase 1 Accumulation of LCFA into cytopasm - cant transfer to mitochondria
1377
Abetalipoproteinemia - ineritence - deficency - mutation - lipoproteins - signs - intestinal biopsy - peripheral smear
AR Decrease ApoB-48 and ApoB-100 Mutation MTP gene Decrease chylomicron adn VLDL synthesis and secretion Steatorrhea Ataxia Night blindness Enterocytes swollen with TGs Acanthocytosis of RBC - Misshaped and spiky
1378
Type I hyperchylomicroenemia - inheritence - deficiency - lipoproteins - signs - extra
AR Lipoprotein lipase deficiency or defective ApoC-11 Increase chylomicrons, TG and cholesterol Pancreatitis Hepatosplenomegaly Pruritic xanthomas No risk of atherosclerosis
1379
Type IIa familial hypercholesterolemia - inheritence - defect - lipoproteins - signs
AD Absent of LDL receptor Elevated LDL in blood Tendinous xanthomas (achilles tendon) Corneal arcus Accelerated atherosclerosis MI in 20s
1380
Type IV hypertriglyceridemia - inheritence - defect - lipoproteins - signs
AD Overproduction of VLDL High level of TG Pancreatitis
1381
A 30-year-old man is diagnosed with type I familial dyslipidemia. He has had recent laboratory studies showing elevated triglycerides and normal cholesterol levels. Which of the following explains the pathophysiology of this disease? (A) Apolipoprotein E defi ciency (B) LDL cholesterol receptor defi ciency (C) Lipoprotein lipase defi ciency (D) VLDL cholesterol clearance defi ciency (E) VLDL cholesterol overproduction
C. Lipoprotein lipase deficiency Type I dyslipidemia
1382
Which apolipoprotein 1) Activates LCAT 2) Mediates chylomicron secretion 3) Mediates VLDL secretion 4) Cofactor for lipoprotein lipase 5) Mediates uptaek of remnant particles
1) ApoA-1 2) ApoB-48 3) ApoB-100 4) ApoC-11 5) ApoE
1383
What is the rate limiting enzyme for each pathway 1) Fatty acid synthesis B) Beta oxidation of FA 3) ketone body synthesis 4) Cholesterol synthesis
1) Acetyl-CoA carboxylase 2) Carnithine acyltransferase I 3) HMG CoA synthase 3) HMG CoA reductase
1384
Essential amino acids
``` "Private (PVT) TIM HaLL" Phenylalanine Valine Threonine Tryptophan Isoleucine Methionine Histidine Leucine Lysine ```
1385
Basic charged amino acids
Lysine Argine Histidine (no charge)
1386
Acidic amino acids
Aspartate and glutamate | negatively charged
1387
Phenylalanine breakdown
1) Phenylalanine --> Tyrosine (BH4) [Phenylalanine hydroxylase ] (-) def --> PKU 2) Tyrosine --> DOPA (BH4) [Tyrosine hydroxylase] (-) def --> parkinson, albinism 3)DOPA --> Dopamine (Vit B6) [Dopa decarboxylase (-) inhibited by carbadopa to tx parkinsons 4) Dopamine --> NE (Vit C) [DOPA beta-hydroxylase] 5) NE --> Epi (SAM) (+) cortisol
1388
PKU
Deficiency of phenylalanine hydroxylase Deficiency BH4 Musty odor Intellectual disability Light skin Seizure
1389
An 18-month-old, light-skinned African American child has a developmental delay characterized by mental retardation and inability to walk. The child’s urine has a distinctly “mousy” odor. On physical examination, there is no lymphadenopathy or hepatosplenomegaly. Laboratory studies show hemoglobin, 14 g/dL; platelet count, 302,700/mm3; WBC count, 7550/mm3; glucose, 80 mg/dL; total protein, 7.1 g/dL; albumin, 5 g/dL; and creatinine, 0.5 mg/dL. A mutation involving a gene that encodes which of the following enzymes is most likely to be present in this child? A Adenosine deaminase B α1-Antitrypsin C Galactose-1-phosphate uridyltransferase D Glucose-6-phosphatase E Lysosomal acid maltase F Phenylalanine hydroxylase G Sphingomyelinase
F. PKU
1390
``` A woman gives birth to a full term baby. Upon delivery, the baby is small and has a musty odor. Upon questioning the woman says that she did not smoke or drink alcohol during the pregnancy, and she only drinks diet soda and water. Which of the following amino acids is most likely to be deficient in the newborn? (A) Alanine (B) Cysteine (C) Glutamine (D) Phenylalanine (E) Proline (F) Serine (G) Tyrosine ```
G. Tyrosine PKU
1391
Deficency of DOPA beta hydroxylase
``` Excess dopamine Deficiency NE and Epi Nasal congestion Ptosis Orthostatic hypotension ```
1392
Precursor for 1) Arginine 2) Tryptophan 3) Histidine 4) Glutamate 5) Glycine
1) Arginine --> Creatine Urea Nitric oxide 2) Tryptophan Niacin --> NAD/NADP (req B6) Serotonin --> Melatonin 3) Histidine --> Histamine (req B6) 4) Glutamate --> GABA (Req B6) Glutathione 5) Glycine --> Porphyrin (Req B6) --> Heme
1393
Deficency of Vit B6 -->
Lack of inhibition | --> seizures
1394
RL enzyme of urea cycle
Carbamoyl phosphate 1 (CPS-1) Mitochondria
1395
Urea cycle
CPS-1 in mitochondria RL step Ornithine transcarbamylase - carbamoyl phosphate --> citrulline
1396
Ornithine Transcarbamylase deficiency
Most common urea cycle disorder X linked Cant undergo urea cycle - build up nitrogen and ammonia Excess carbamoyl phosphate --> orotic acid Orotic acid in blood and urine Decreased VUN Hyperammonemia ``` Child Slurring speech Vomiting Cerebral edema Blurring vision Hepatoencephalopathy ```
1397
A 9-month-old boy is brought to the emergency department after his mother is unable to rouse him. His past medical history is signifi cant for the onset of seizures at the age of 4 months and for a delay in reaching developmental milestones. On examination, the patient is found to have poor muscle tone and an enlarged liver. Laboratory studies show a blood urea nitrogen level of 3.2 mg/dL, a creatinine level of 0.4 mg/ dL, and a serum ammonia level of 300 mg/dL. A plasma amino acid analysis fails to detect citrulline, while his urinary orotic acid level is increased. This patient suffers from a defi ciency of which of the following enzymes? (A) Argininosuccinate lyase (B) Carbamoyl phosphate synthetase II (C) Glutamate dehydrogenase (D) Ornithine transcarbamoylase
D. Ornithine trasncarbamoylase
1398
What does each cell secrete 1) G cells 2) I cells 3) S cells 4) D cells 5) Parietal cells
1) G cells - Gastrin 2) I cells - CCK 3) S cells - secretin 4) D cells - Somatostatin 5) Parietal cells - Gastric acid & intrinsic factor
1399
A 35-year-old man presents to the physician with arthritic pain in both knees along with back pain. He states that the pain has been present for months. In an effort to obtain relief, he has taken only aspirin, but this has been of little benefit. The patient is afebrile, and his slightly swollen knee joints are neither hot nor tender to palpation; however, the pain does restrict his motion. The cartilage of his ears appears slightly darker than normal. No tophi are present. A urine specimen is taken for analysis of uric acid content and turns black in the laboratory while standing. A defect in which of the following is the most likely underlying cause of the patient’s condition? (A) Galactokinase (B) Homogentisic acid oxidase (C) α-Ketoacid dehydrogenase (D) Orotate phosphoribosyltransferase (E) Phenylalanine hydroxylase
B. Homogentisate Oxidase Alkaptonuria - Deficiency of homogentisate oxidase AR Build up homoentic acid in urine Darkens CT and urine Arthralgias
1400
A 6-year-old girl is found to be nearsighted during a vision screening at school, and the school nurse tells the parents the child should be fitted for corrective lenses. Her mother is upset because her daughter is already much taller than her classmates, has an awkward gait, and was recently diagnosed with scoliosis. She is afraid that the glasses will only add to her daughter’s problems at school, where her classmates frequently tease her. When the ophthalmologist observes that the patient’s right lens is dislocated, he suspects that her symptoms are in fact related to an enzyme deficiency. As a result of this deficiency, which of the following amino acids is essential in this patient’s diet? (A) Cysteine (B) Lysine (C) Methionine (D) Tryptophan
A. Cysteine Homocystinuria Homocysteine --> cystathionine - Deficency of cystathionine synthase - decreased affinity of cystathionine synthase for Bit B6 Homocysteine --> Methionine - Deficiency of homocysteine methyltransferase - Deficiency of Vit B12
1401
``` Intellectual disability Tall stature Osteoporosis Kyphosis Atherosclerosis Subluxation of lens (down ward) ```
Homocystinuria
1402
The mother of a 16-month-old girl is concerned because she noticed that her daughter became lethargic and irritable 2 days after an ear infection. The mother recalls periodically noticing sweet-smelling urine in her child’s diaper but that otherwise her development has been normal. An inability to metabolize which of the following would explain these symptoms? (A) Glucosylceramide (B) Histidine (C) Phenylalanine (D) Sphingomyelin (E) Tyrosine (F) Valine
F. Valine Maple syrup urine disease Urine smells like maple syrup (sweet) Deficiency of branched chain alpha-ketoacid dehydrogenase complex - build up of branched chain amino acids "I LoVe Maple Syrup" - Isoleucine - Leucine - Vaine Intellectual deficiencies Severe CNS defects High mortality
1403
39-year-old woman presents to the physician with complaints of itchy skin and diarrhea. On examination, the patient appears slightly confused and is having trouble remembering what she did earlier in the day. A physical examination shows that the patient has a large, deeply pigmented tongue with thickened papillae. On further questioning, it is discovered that the patient has been taking isoniazid for suspected tuberculosis infection. Which of the following supplements should this patient take? (A) Ascorbic acid (B) Biotin (C) Folic acid (D) Niacin (E) Ribofl avin (F) Thiamine (G) Vitamin D
D. Niacin Patient has Pellagra
1404
Patient presents to office with itchy skin and diarrhea. She is confused to the day. Examination shows an large deeply pigmented tongue. Genetic cause
Hartnup disease AR Defective neutral aminoacid transporter Tryptophan not absorbed No Niacin Pellagra
1405
What goes with there 1) homogentisic acid oxidase deficency 2) Alpha ketoacid dehydrogenase def 3) Tyrosine def 4) Decreased tryptophan 5) Phenylalanine hydroxylase def 6) Cystathionine synthase def 7) B6 def 8) COLA def 9) Tetrahydrobiopterin def 10) Decreased Niacin 11) Sweet smelling urine 12) Musty smell 13) Dark urine 14) Increased skin cancer 15) Lens dislocation 16) Cystine stones
1) Alkaptonuria 2) Maple syrup urine 3) Albinism 4) Hartnup 5) PKU 6) Homocystinuria 7) Homocystinuria 8) Cystinuria 9) PKU 10) Hartnup 11) Maple syrup urine 12) pKU 13) Alkaptonuria 14) Albinism 15) Homocystinuria 16) Cystinuria
1406
Ferritin
Storage form of iron Acute phase reactant - Alot of inflammation release ferritin to bind up iron so bacteria cant use it
1407
Transferrin
Transport ferric molecules Increased in iron deficiency
1408
Zinc - use - deficency
Healing Imune system Carbonic anhydrase - CO2 --> bicarb ``` Delayed wound healing Decrase body and facial hair Hypogonadism Decreased ability to smell Decreased taste Rash around eyes, mouth, nose, anus Impaired night vision Infertility ```
1409
Lead poisoning
``` Decreased IQ Hearing problems Growth impairment Wrist/ foot drop Lead lines Anemia ``` Basophilic stippling Tx: Chelating agent - succimer or EDTA Pediatric - dimercaprol + succimer
1410
Basophilic stippling | Ringed sideroblasts
Lead poisoning
1411
Mecury poisoning affects Key feature
Kidneys and brain Acrodynia: peeling of fingertips
1412
Acrodynia
Peeling of fingertips Kawaski disease Mercury posoning
1413
Fat soluble vitamins Absrobed in
Vit AdEK ileum
1414
Sarcoidosis and vit D
Increases coversion of 25-OH Vit D --> 1,25 (OH2) Vit D Hypercalcemia
1415
Vit K does post-translational modification of what clotting factors
"diSCo started in 1972" Protein S Protein C Clotting factor 10, 9, 7 and 2
1416
Vit E - fxn - deficency
Antioxidant Prevents free radials on RBCs ``` Deficiency - hemolytic anemia - Spinocerebellar degeneration (ataxia) - peripheral neuropathy - proximal muscle weakness ```
1417
Vit C - fxn - deficiency
Hydroxyaltion of prolyl and lysyl residues of collagen Required for dopamine --> NE [Dopamine beta hydroxylase] Antioxidant Iron absorption in gut Deficiency - cant make collagen - scurvy
1418
Differential diagnosis for eosinophilia
CANADA-P ``` Collagen vascular disease Atopic disease Neoplasma Adison disease Drugs Acute interstitial nephritis Parasites ```
1419
Vit B1
Thiamine Deficiency - Wernicke-Korsakoff - Beriberi
1420
A 9-month-old infant has failure to thrive following a premature birth with low birth weight. The infant has chronic cholestatic hepatobiliary disease. The infant is now at the 40th percentile for height and the 25th percentile for weight creased vibration and pain sensation, muscle weakness, and abnormalities of eye movement. Laboratory studies show hemoglobin, 9.2 g/dL; hematocrit, 27.6%; MCV, 86 μm3; platelet count, 208,000/mm3; WBC count, 6080/mm3; total protein, 6.4 g/dL; albumin, 3.4 g/dL; glucose, 70 mg/dL; and creatinine, 0.3 mg/dL. A deficiency of which of the following vitamins is most likely to contribute to these findings? A Vitamin A B Vitamin B1 C Vitamin B3 D Vitamin B12 E Vitamin C F Vitamin E G Vitamin K
F. Vitamin E
1421
The symptoms of thiamine defi ciency were fi rst described over 4,000 years ago. In adults, these symptoms commonly include polyneuropathy of the distal extremities, resulting in paresthesias and motor dysfunction. In addition to neuropathy, how else can isolated thiamine defi ciency present in adults? (A) Chronic infection (B) Convulsions, hyperirritability, and jaundice (C) Excessive diarrhea, dermatitis, and dementia (D) Kidney failure (E) Peripheral edema and congestive heart failure
(E) Peripheral edema and congestive heart failure BeriBeri
1422
Beriberi Dry vs wet
Thiamine deficiency ``` Dry Beriberi - peripheral neuropathy (toe drop, wrist drop, foot drop) - Muscle weakness - Hyporeflexia or areflexia ``` Wet Beriberi - Peripheral vasodilation - High output heart failure - peripheral edema - cardiomegaly
1423
Vit B2
Riboflavin FMN and FAD Cofactors for redox reactions Deficiency - Dermatitis - Cheilosis - Glossitis
1424
Vit B3
Niacin NAD and NADP Redox reactions Derived from Tryptophan Deficiency - pellagra
1425
Vit B5
Pantothenate Component of coenzyma A Fxn in transfer of acyl groups Deficiency -dermatitis Enteritis -alopecia-adrenal insufficiency
1426
Vit B6
pyridoxine coenzyme for transaminations and deaminations Conversion of AA precursors ``` Deficiency - cheilosis -glossitis - convulsions ** (B6 essential for GABA) -Peripheral neuropathy ```
1427
Vit B7
Biotin Apoenzyme for caboxylation rxns
1428
Vit B9
Folic acid Synthesis and repair of DNA Rapid cell division and growth Tetrahydrofolate Deficiency - Meds (phenytonin, sulfonamides, TMP, MTX) Neural tube defect Megaloblastic anemia Glossitis Diarrhea Depression Confusion
1429
Megaloblastic anemia
RBC large Macrocytic anemia Hypersegmented neutrophils Folate and B12
1430
Vit B12
Cobalamin Need Intrinsic factor to be absorbed in duodenum Cofactor w/ homocysteine methyltransferase --> THF ``` Deficiency - Anemia Ataxia Paresthesias Memory loss Dementia ``` Vegetarians Pernicious anemia
1431
A patient presents with convulsions and irritability, what vitamin deficiency
Vitamin B6
1432
Which vitamin deficiency 1) Peripheral neuropathy, glossitis 2) Neural tube defects 3) Dermatitis, diarrhea, dementia 4) Megaloblastic anemia 5) Pernicious anemia
1) B12, B6 2) Folic acid (B9) 3) B3 4) B12 or folic acid (B9) 5) B12
1433
Tumor markers for pancreatic cancer
CA 19-9 | CEA (also elevated in gastric and colon cancer)
1434
Uses cAMP and protein kinase A as secondary messengers
``` FSH LH ACTH TSH hCG MSH GHRH, CRH PTH, Calcitonin Glucagon V2 Vasopressin receptor ```
1435
Uses IP3 as second messenger
GnRH TRH Oxytocin V1 vasopressin receptor
1436
Uses tyrosine kinase receptor as second messenger
``` Growth hormone Prolactin Inulin insulin-like growth factor Platelet derived growth factor Fibroblast growth factor Cytokines ```
1437
Uses cGMP as secondary messenger
Nitric oxide | Atrial natriuetic peptide
1438
Uses steroid receptors as second messenger
``` Estrogen, progesterone, testosterone Glucocorticoids Aldosterone Thyroid hormone Vitamin D ```
1439
What are the two nervous tissue bundles that run through the GI tract and where re they located
Meissner plexus: submucosa Auerbach's plexus: between layers of the muscularis propria
1440
What disesases specifically damage tehn aterior horn?
Polio West nile virus ALS Werdnig-Hoffman dis (spinal muscular atrophy)
1441
ACTH is synthesized as part of
Large precursor called POMC
1442
POMC contains
Lipotropin MSH beta-endorphin
1443
Somatostatin fxn
Reduces splanchnic blood flow Reduces GI motility adn GB contraction Inhibits secretion of most GI hormones
1444
Sheehan syndrome
Postpartum hemorrhage leading to underperfusion of the pituitary Pituitary necrosis and hypopituitarism Agalactorrhea Amonorrhea after delivery Hypothyroidism
1445
Pituitary Anterior lobe forms from - type of cells Posterior lobe - forms from - type of cells
Anterior lobe - forms from rathke's pouch - ectodermal diverticulum Posterior lobe - forms from invagination of the hypothalamus - neuroectoderm
1446
What increases ADH What decreases ADH
Increase ADH - Nicotine - Opiates Decrease ADH - Ethanol Atrial natriuretic factor - decreased serum osmolarity
1447
What hormones share a common alpha subunit
FSH LH TSH hCG
1448
what do type II pneumocytes need to mature
Cortisol
1449
Adrenal cortex from Adrenal medull afrom
Cortex= mesoderm Medulla= ectoderm
1450
Tumor in adrenal medulla of kids Causes?
Adrenal neuroblastoma Sustained HTN
1451
Deficiency in 21 alpha hydroxylase
Masculization Hypotension Cant make aldosterone or cortisol Hypotension Hyponatremia HYPERkalemia High 1-OH progesterone Cortisol deficiency --> increase ACTH, hyperplasia of adrenal glands Most common
1452
Deficiency in 11b- hydroxylase
Masculization Hypertension Build up of 11-deoxycorticosterione Still have mineralocorticoid properties Cortisol deficiency --> increase ACTH, hyperplasia of adrenal glands
1453
Deficiency of 17a- hydroxylase
HTN Ambiguous genitalia Can only make mineralocorticoids Salt and H20 retention Hypokalemia Cortisol deficiency --> increase ACTH, hyperplasia of adrenal glands
1454
3beta-hydroxysteroid dehydrogenase deficiency
``` Inability to produce - Glucocorticoids Mineralocorticoids Androgens Estrogens ``` Excessive sodium Early death Ambigous genitalia
1455
Adrenal steroids are made from
Cholesterol
1456
Dexamethasone suppression test, high dose works on
ACTH-producing pituitary tumor
1457
Cortisol effect on bones and immune system
Reduces bone formation --> osteoporosis Suppress immune system
1458
Waterhouse-Friderichsen syndrome
Acute primary adrenal insufficiency due to adrenal hemorrhage - Severe meningococcal sepsis - DIC Petechial rash Coagulopathy
1459
Primary adrenal insufficiency deficient in Tx
Cortisol and aldosterone Tx: glucocorticoid
1460
Conn syndrome
Aldosterone-secreting adrenal tumor Hyperaldosteronism HTN Hypokalemia Metabolic alkalosis Decrease in Renin levels
1461
``` Hypotension Hyponatremia Hyperkalemia Fatigue Anorexia Weight loss Hyperpigmentation ```
Primary adrenal insufficiency (addison disease)
1462
A 42-year-old woman has had increasing weakness, nausea, vomiting, watery diarrhea, and a 5-kg weight loss over the past 7 months. She has generalized muscle weakness, muscle wasting, and increased skin pigmentation on physical examination. After an upper respiratory tract infection lasting 1 week, she develops abdominal pain and faintness and lapses into a coma. Her temperature is 36.9° C, pulse is 83/min, respirations are 17/min and shallow, and blood pressure is 80/40 mm Hg. Laboratory findings show Na+, 129 mmol/L; K+, 3.5 mmol/L; Cl−, 95 mmol/L; CO2, 23 mmol/L; glucose, 48 mg/ dL; and creatinine, 0.6 mg/dL. Atrophy of which of the following tissues is most likely to be present? A Adrenal cortex B Hypothalamus C Islets of Langerhans D Parafollicular C cells in thyroid E Pineal gland F Thyroid epithelium
A. Adrenal cortex
1463
A 55-year-old man has experienced increasing lethargy for the past 7 months. Physical examination shows hyperpigmentation of the skin. Vital signs include temperature of 36.9° C, pulse of 70/min, respirations of 14/min, and blood pressure of 95/65 mm Hg. Laboratory studies include a serum cortisol level of 3 μg/mL at 8:00 AM with a serum corticotropin level of 65 pg/mL. Which of the following diseases most often occurs in patients with this disorder? A Type 2 diabetes mellitus B Classic polyarteritis nodosa C Hashimoto thyroiditis D Systemic lupus erythematosus E Ulcerative colitis
C. Hashimoto thyroiditis Addison disease
1464
A 44-year-old woman has become increasingly listless and weak and has had chronic diarrhea and a 5-kg weight loss over the past 7 months. She also notices that her skin seems darker, although she rarely goes outside because she is too tired for outdoor activities. On physical examination, she is afebrile, and her blood pressure is 85/50 mm Hg. A chest radiograph shows no abnormal findings. Laboratory findings include serum Na+, 120 mmol/L; K+, 5.1 mmol/L; glucose, 58 mg/dL; urea nitrogen, 18 mg/dL; and creatinine, 0.8 mg/ dL. The serum corticotropin level is 82 pg/mL. Which of the following is most likely to account for these findings? A Adenohypophyseal adenoma B Autoimmune destruction of the adrenals C Pancreatic neuroendocrine tumor D Metastatic carcinoma with lung primary E Neisseria meningitidis infection of the adrenals F Sarcoidosis of the lung and adrenals
B Autoimmune destruction of the adrenals
1465
30 y.o man undergoing an evaluation for hypertension. Has a history of multiple episodes of hypokalemia. Lab shows a potassium of 3.0 despite replacement and metabolic alkalosis. What is the cause of HTN?
Primary hyperaldosteronism
1466
Which causes of vaginal discharge/ vaginitis are associated iwth a high vaginal pH? Wjocj are associated iwth a low vaginal pH?
Low vaginal pH - physiologic dischrage Candida High vaginal pH - gardnerella Trichomonas
1467
Erythropoietin=secreting tumors
Pheochromocytoma Renal cell carcinoma Hemangioblastoma (vascular tumor of CNS) Hepatocellular carcinoma
1468
Neuroblastoma - tumor of - type of cells - see in - causes - associated with - histology - tumor marker
Home Wright rosettesTumor of the adrenals Tumor of sympathetic ganglion cells Most common tumor of adrenal medulla in children Mild HTN N-myc oncogene Homer Wright rosettes Bombesin tumor marker
1469
Homer Wright rosettes
Adrenal neuroblastoma
1470
N-myc oncogene
Adrenal neuroblastoma