STEP 1 Week 1 Flashcards
(461 cards)
1
Q
Four impairments in glycogenolysis
A
2
Q
Characteristics of Pompe disease and cause
A
Cause: deficiency of acid alpha glucosidase
Normal glucose levels
Extreme cardiomegaly
Glycogen accumulation in lysosomes
3
Q
Muscle pierced for chest tube at 5th intercostal space, mid-axillary line
A
Serratus anterior
4
Q
Pierre Robin Sequence
A
5
Q
Progression of cells after MI
A
6
Q
Regulatory T-Cell function
A
7
Q
What is the IPEX disorder
A
Mutation in FOXP3 expression, leads to unrelgulated T and B cell activity
IPEX - Immune dysregulation, polyendocrinopathy, enteropathy, X-linked
See: autoimmune neteritis (villous atrophy,etc), excematous dermatitis, type 1 diabetes in infant
8
Q
Trigylceride metabolism to glucose
A
9
Q
Best long-term therapy for specific phobia
A
exposure-based CBT
10
Q
Ovarian venous drainage
A
Left ovarian vein into left renal vein
Right ovarian vein into IVC
11
Q
Distribution of data on a bell curve (percentiles)
A
12
Q
When is B-HCG detectable in serum and urine
A
β-hCG is produced by the syncytiotrophoblast after implantation, which generally occurs 6-7 days after fertilization at the earliest. β-hCG typically is detectable in the maternal serum approximately 8 days after fertilization, whereas it is detectable in the urine 14 days after fertilization. Therefore, a serum pregnancy test will be positive before a urine pregnancy test.
13
Q
What is hyperacute transplant rejection
A
Occurs minutes to hours
Preformed recipient antibodies against graft antigens
See: mottling and cyanosis, fibrinoid necrosis and capillary thrombotic occlusion
14
Q
What is acute transplant rejection
A
Usually <6 months
Donor antigens induces activation of naive immune cells - Cell mediated, sensitization of recipient T cells
See: lymphocytic infiltrate, cd4 deposition, neutrophils, necrotizing vasculitis
15
Q
What is chronic transplant rejection
A
Months to years later
Chronic low-grade immune response refractory to immune repression, mixed-cell and humoral
See: vascular wall thickening, interstitial fibrosis, parenchymal atrophy
16
Q
Process of tRNA amino acid binding
A
The 3’ CCA tail of tRNA serves as the amino acid binding site.Aminoacyl tRNA synthetase is the enzyme responsible for “loading” the appropriate amino acid to the 3’ terminal hydroxyl group of the CCA tail.
17
Q
How can buprenoprhine precipitate withdrawals
A
It is a partial opiod receptor agonist that binds with high affinity but low activity. It can displace other opioids leading to withdrawal
18
Q
Effects of unilateral renal artery stenosis
A
19
Q
Location and effect of adrenergic receptors
A
20
Q
What does staph secrete that allows Haemophillus to grow on blood agar
A
V Factor (NAD+)
Also helps release factor X (hemetin), both are necessary for haemophilus
21
Q
Pathophys of an acute hemolytic transfusion reaction
A
Type II hypersensitivty
IgG and IgM autoantibodies with complement activation (cell lysis)
22
Q
What are clavulinic acid, subactam, and tazobactam
A
beta-lactamase inhibitors
23
Q
Most common cause of nosocomial blood stream infections
A
Intravascular catheters (allows staph to enter bloodstream)
24
Q
What type of cells allow growth of HPV
A
Stratified squamous epithelium
Cervix, anus, true vocal cords
25
Manifestations of hypovolemia
26
Pathophys of rheumatoid arthritis
Activation of CD4+ T (Th1, Th17) cells and macrophasges secrete **IL-1 and TNF-alpha,** leading to articular destruction
IL-1 : induces matrix metalloproteinases and T cell response
TNF-alpha: inflammatory cells
Both activate osteoclasts
27
Causes of nonvalvular heart failure and changes in wall thickness and function
28
Effects on heart of volume overload vs pressure overload
Volume overload : eccentric hypertrophy (decompensated heart failure)
Pressure overload: Concentric hypertrophy (hypertensive heart disease)
29
What nerve is impacted in Bell's palsy
facial nerve (CN VII)
30
Functions of facial nerve (CN VII)
Motor output to the **muscles controlling movement of the face (including eye closure)**
Somatic sensation afferents from portions of the pinna and external auditory canal
Special sensation afferents for taste from the **anterior two-thirds of the tongue**
Parasympathetic innervation of the **submandibular and lacrimal glands**
Motor innervation of the stapedius muscle, which causes sound dampening
31
Changes to the lungs with age (lung capacity, FVC, RV)
Lung capacity is **unchanged** - chest compliance goes down (stiff ribs) and lung compliance goes up (loss of recoil)
FVC goes **down** (RV bigger portion of TLC)
RV goes **up**
32
Sources of nitrogens in purines
33
Sources of carbon in purines
34
Ribavirin MOA
Inhibits IMP dehydrogenase, blocks conevrsion of IMP to GMP
35
Mycophenolate MOA
Immunosuppresant
Blocks IMP dehydrogenase
36
6-mercaptopurine MOA
37
What is Lesch-Nyhan syndrome
**X-linked absence of HGPRT** (cant do purine salvage)
See excess uric acid production (juvenile gout), excess de novo purine synthesis (high PRPP, IMP)
SX: hypotonia, chorea, self-mutilation
38
Carbon and nitrogen sources for pyrimidines
39
What is orotic aciduria
**Autosomal recessive defect in UMP synthase**
## Footnote
SX: orotic acid in urine, megaloblastic anemia, hypersegmented neutrophils
TX: Uridine
40
Signs of orithine transcarbamylase deficiency
Causes a breakdown in urea cycle
See: increased carbamoyl phosphate and **orotic acid in urine, but also see increase in ammonia**
41
MOA of 5-FluroUracil
5-FU is a chemotherapy agent
Mimics uracil. **Blocks thymidilate synthase** and formation of dTMP
42
MOA of methotrexate
**Blocks dihydropholate reductase** so cant form THF, leads to blocked formation of dTMP
43
B12 vs Folate deficiency
44
Features of bulemia nervosa
45
What are the universal stop codons
UAA, UAG, UGA
Lead to releasing factor
46
Complete vs partial mole
47
Afferent vs efferent nerves for visual light reflex
Afferent: CN II
Efferent: CN III (edinger westphal for other eye, PNS)
48
Clinical features of selective IgA deficiency
49
50
MOA of carbon monoxide poisoning
CO **binds to heme** iron with a much higher affinity than oxygen, creates carboxyhemoglobin.
Impedes oxygen delivery and decreases cardiac output
51
What is type II error
Probability of concluding no difference exists when one actually does exist
52
What is Power
1 - Type II error
53
Clinical features of PCOS
54
Pathophys of PCOS
Increased activity of activity of **17α-hydroxylase; 17,20 lyase; and 3β-hydroxysteroid dehydrogenase.** The overexpression of these enzymes results in elevated androgen levels (eg, testosterone, androstenedione, DHEA) and the clinical consequences of hirsutism and acne.
Incresed risk for endometrial carcinoma
55
Pharyngeal and aortic arches derivatives (1-6)
56
Changes to glomerular filtration during pregnancy
Because blood volume and cardiac output goes up, **GFR increases**
Placental proteins also increae permeability of glomerular basement membrane, so see proteinuria
57
Source of single brain abscess vs multiple
Single - direct invasion from contiguous source
Multiple - hematogenous spread from distant infection
58
Exudative vs transudative pleural effusions
59
Regulation of glycogenolysis in liver vs muscle
In liver, regulated by glucagon and Epi through cAMP.
In muscle, regulated by muscle contraction (Ca2+) mostly and some cAMP
60
Characteristics of drugs that are mainly excreted by the liver
High lipophilicity
High volume of distribution
61
What is the drawback of using creatinine to measure GFR
Creatinine is secreted by the proximal tubules so the calculated GFR is 10-20% higher than actual GFR
62
Contents of superior occular vs inferior occular fissures vs optic canal
63
Features of TCA overdose
Most deaths come from cardiac arrhythmias and refractory hypotension
TX: Sodium Bicarbonate
64
Pathophys of rheumatic fever
Autoimmune reaction following GAS infection. **Antibodies against strep M protein and N-acteyl-beta-D-glucosamine** cross react
65
Infection in children marked by URI sx, stridor, brassy cough, barking cough, hoarsness, resp distress
Laryngotracheitis (**croup**)
caused by **parainfluenza virus - paramyxovirus**
66
Difference between GLUT 1, 2, and 4 transporters
67
Features of pyruvate kinase deficiency
Converts phosphoenylpyruvate to pyruvate
68
Sources of fuel over course of 24 hour fast
69
What is Von Gierke's Disease (Glycogen storage disease type 1)
**Glucose-6-phosphatase deficiency**
Sever hypoglycemia between meals, lethargy, seizures, lactic acidosis (Cori cycle)
Enlarged liver (cant get glycogen out)
70
What is Pompe's disease (glycogen storage disease II)
**Acid alpha-glucosidase deficiency**
Accumulation of ghlycogen inside lysosomes
Presents in childhood, severe
See enlarged muscles (cardiomegaly, macroglosia), hypotonia, no metabolic problems but have heart failure
71
What is Cori's disease (glycogen storage disease type III)
**Debranching enzyme deficiency**
Similar to Von Girkes but mild hypoglycemia and have muscle involvement
See enlarged liver and also hypotonia
72
What is McArdle's Disease (Glycogen storage disease V)
**Glycogen phosphorylase deficiency**
Adolescence or early adulthood
No liver problems, but have exercise problems - weakness and cramps
Myoglobinuria and urine turns dark after exercise
73
Uses of NADPH
74
What is respiratory burst
Killing of bacteria by phagocytes with oxygen
75
What is chronic granulomatous disease and features
76
How is fructose brought into glycolysis
77
What is essential fructosuria
**Deficiency of fructokinase**
Benign, fructose not taken up into liver, spills into urine
78
What is hereditary fructose intolerance
**Deficiency in aldolase B**
Get buildup up of fructose 1- phosphate and get depletion of ATP
See hypoglycemia, vomiting, hepatomegaly, FTT after weaning off breastmilk
79
How is galactose converted to glucose
80
What is classic galactosemia
**Deficiency in galactose 1-phosphate uridyltransferase (GALT)**
Galactose 1-phosphate builds up in liver and galactose is also converted to galactitol (accumulates in eye like sorbitol)
See hepatomegaly, jaundice, childhood cataracts
Have to avoid milk
81
Required cofactors for pyruvate dehydrogenase
NAD+
FAD+
CoEnzyme A
Thiamine
Lipoic Acid
82
What is the malate shuttle
Oxaloacete and NADH cannot cross membrane, but malate can. Malate is used to bring electrons in for NADH and then can be converted into oxaloacete inside mitochondria
83
How many ATP made per NADH/FADH2
3 per NADH
2 per FADH2
84
Inhibitors of electron transport chain
85
What tissues cannot use fatty acids for energy
RBCs (glycolysis only, no mitochondria)
Brain (glucose and ketones only)
86
What is the carnitine shuttle
Helps fatty acids undergo B-oxidation by moving fatty acyl coA into the mitochondria
87
Features of carnitine deficiency
Cant bring long chain fatty acids into mitochondria to break down fatty acids
88
What is a MCAD deficiency
Deficiency in medium chain Acyl-CoA dehydrogenase
AcetylCoA is low so gluconeogenesis is shut down
89
Pathophys of lactic acidosis in alcoholism
90
Genetic risk factors for Alzheimers
- Amyloid precursor protein on chromosome 21
- Presinilin 1 on chromosome. 14
- Presinilin 2 on chromosome 1
- ApoE4
91
Features of cystic fibrosis
92
Syphillis diagnostic serology
93
MOA of penicillins
Structurally simialr to **D-alanine-D-alanine**, bind to **transpeptidase** that inhibits formation of bacterial peptidoglycan cell way
94
Mechanisms of septic organ dysfunction (3)
**Poor oxygen use**
1. Bacterial products trigger free radicals that lead to mitochondrial damage = decreased oxydative phosphorylation and lactic acidosis
2. Vasodilation shunts blood quickly through organs
3. Increased capillary permeability causes edema that increases distance O2 must travel to mitochondria
95
Deposition seen in poststrep glomerulonephritis
Starry sky (granular) deposition of **IgG, IgM, and C3** immune complexes
96
Stages of alcohol withdrawal
97
What is integrin
Helps bind cells to one another by binding to fibronectin, collagen, and laminin
98
What is X-linked agammaglobulinemia
X-linked agammaglobulinemia is characterized by **low or absent circulating mature B cells** (ie, **CD19+, CD20+, CD21+** cells) and pan-hypogammaglobulinemia (**low IgG, IgM, IgA**). Affected patients have increased susceptibility to pyogenic bacteria, enteroviruses, and Giardia lamblia due to the absence of opsonizing and neutralizing antibodies.
99
Sources of ATP during exercise (3 phases)
1. Phosphocreatine shuttle
2. Glycolysis
3. Oxidative phosphorylation
100
Steps of insulin release (start with glucose receptor)
1. Glucose enters cell through GLUT-2, broken down by TCA cycle into ATP
2. ATP binds K+ channel and closes it, leading to depolarization
3. Calcium channel opens
4. Insulin release
101
Permeability of nephron regions to water
102
What is purulent pericarditis and most common causes
103
Structures derived from neural crest cells
**SOME SALT**
Schwann cells, odontoblasts, melanocytes, enterochromaffin cells, spinal membranes [pia and arachnoid], adrenal medulla/ganglia, laryngeal cartilage, tracheal cartilage
104
Visual pathway
105
Important HIV genes
**Pol gene** - encodes protease, reverse transcriptase, integrase
**Env gene** - surface glycoprotein, enables host immune escape
**Nef gene** - downregulates MHC 1 on infected cells
106
Major virulence factor of staph epidermis
Ability to produce **biofilm** - extracellular polysaccharide matrix
107
Hormonal control of pancreatic secretions
108
Prolonged exposure to loud noises causes hearing loss due to damage to the\_\_\_\_\_ of the \_\_\_\_\_\_.
Prolonged exposure to loud noises causes hearing loss due to damage to the **stereociliated hair cells** of the **organ of Corti.**
109
Higher frequency sounds are heard at the ____ of the cochlea
Earlier portion
Low frequency - farther in (cochlear cupula)
110
Risks and protective factors for epithelial ovarian cancer
The pathogenesis of epithelial ovarian cancer is linked to the frequency of trauma and repair at the ovarian surface. **Oral contraceptives, multiparity, and breastfeeding are protective by decreasing the frequency of ovulation. Risk factors include BRCA mutation, nulliparity, and infertility.**
111
What causes diastolic heart failure
Hypertension, obesity, infiltrative disorders (transythyretin-amyloid, sarcoidosis)
Caused by decreased LV compliance, see normal LVEF. and LV end-disatolic volume, but high LV filling pressures
112
Portions of hypothalamic nuclei that mediate food intake
**Venteromedial**. - mediate satiety, destruction = hyperohagia
**Lateral** - mediates hunger, destruction = anorexia
113
Portions of. hypothalamic nuclei that mediate heat
**Anterior**. - heat dissipation, destruction = hyperthermia
**Posterior** - heat conservation, destruction = hypothermia
114
Gram stain of common infectious urethritis organisms
N. Gonorrhea - Gram negative, see intracellulat diplococci
Chlamydia trachomatous and mycolplasma - do not graim stain because not enough peptidoglycan
115
Radial nerve function and sensory distribution
116
Function of Natural Killer cells
Recognize and kill cells with reduced MHC 1 expression (virus, tumor). Contain perforins and granzymes that lead to apoptosis
117
What is the alanine cycle
During starvation, alanine in muscles is transported to the liver. In the liver alanine is converted into pyruvate which can. be used to generate glucose
118
What is the Cori cycle
Muscle and RBC convert glucose to lactate which then goes to the liver. Lactate can then be converted to glucose to. be sent back to the muscle
119
Kwashiorkor vs Marasmus
Kwashiorkor is inadequate protein intake, see edema (low albumin)
Marasmus is insufficient calories, no edema
120
Hypoglycemia causes when after feeding vs fasting
After feeding: Galactosemia, hereditary fructose intolerance
Fasting: Glycogen storage disease
121
Sypmtoms of proprionic aciduria and methylmalonic aciduria
Poor feeding, hypotonia, vomiting, lethargy
Metabolic acidosis (anion gap)
Hypoglycemia and ketosis
**Hyperammonemia, elevated organic acids in serum/urine**
122
What is maple syrup urine disease
**Branched chain amino acid disorder - deficiency of alpha ketoacid dehydrogenase**
Get buildup of branched change AA and alpha ketoacids
123
Branched chain amino acids
Leucine, isoleucine, valine
124
Two causes of hypoketotic hypoglycemia and features
**Carnitine deficiency** - low carnitine levels, low acyl-carnitine levels
**MCAD deficiency** - High acylcarnitine. level and highicarboxylic acids
125
Features of ornithine transcarbamylase deficiency
High ammonia
High orotic acid
126
Hallmark of mitochodnrial disorder
**High alanine**
Pyruvate cannot be converted to. acetyl coA. (usually by. pyruvate dehydrogenase in mitochondria) so converted to alanine
127
Cell type and function
128
Cause and effect of midgut malrotation
Rotation of 180 instead of 270 degrees counterclockwise
Results in cecum in RUQ - intestinal obstruction due to bands connecting to mesentary over duodenum.
Can also get twisting around superior mesenteric artery
129
Lymph drainage of rectum
Above dentate line - internal illiac and inferior mesenteric, some superior mesenteric
Below dentate line - inguinal nodes
130
The live attenuated oral (sabin) polio vaccine creates a stronger ____ response than the inactivated (salk)
IgA - made in duodenal lumen
131
Contact dermititis, granulomatous inflamation, and reactive skin testing are all forms of \_\_\_\_
Type IV hypersensitivity - T cell mediated
132
Causes of eccentric vs concentric LV hypertrophy
Concentric - pressure overload
Eccentric - volume overload
133
How can cancer cells. mutate to resist anti-cancer drugs
The **human multidrug resistance (MDR1) gene codes for P-glycoprotein, a transmembrane ATP-dependent efflux pump** protein that has a broad specificity for hydrophobic compounds. This protein can both reduce the influx of drugs into the cytosol and can increase efflux from the cytosol, thereby preventing the action of chemotherapeutic agents.
134
EKG leads
135
Mechanical dysfunctions associated with MI timeframe and SX
136
Anion gap equation
Na+ - (Cl- + bicarb)
Normal: 10-14
137
Cause of an non-anion gap met acidosis
**Loss of bicarb**
Diarrhea, renal tubular acidosis, saline infusion
138
What muscles are targeted in pelvic floor strengthening
Levator ani
pubococcygeus, puborectablis, illeococcygeos
139
140
Difference between DNA replication in eukaryotes and prokaryotes
1. Prokaryotes have 5 DNA polymerases, eukaryotes have 3
2. Eukaryotic is much larger genome
3. Prokaryotic is circular and has one cite of replication. Eukaryotic is linear with multiple cites (faster)
141
Portosystemic anastamoses
142
Low dose aspirin mechanism and side effects
COX-1 inhibition, risk of gastric bleeding
143
Pathophys of myasthenia gravis
Autoimmune disease that leads to dicrease in number of acetylcholine receptors**, reduces the amplitude of the motor end plate potential**
144
Hemodynamic changes seen in aortic regurgitation
Will result in eccentric hypertrophy so larger LV
Causes an overall increase in systolic bp due to increase cardiac output and contractility
Aortic diastolic will be low and LV diastolic will be high
145
MOA of acetominophen toxicity
Formation of NAPQI that disrupts hepatocyte mitochondrial function and causes hepatic oxidative damage
146
Abortive and preventative therapy for migraines
147
Pathogenesis of primary aldosteronism
148
Techniques for motivational interviewing
**OARS**
Open ended questions, affirmations, reflect, summarize
149
Portion of hypothalamic nuclei involved in circadian rhythyms
suprachiasmatic
150
Symptoms of parvovirus B19
Symptomatic anemia, decreased erythropoesis, transient aplastic crisis. Worse in patients with hemoglobin disorders
See giant pronormoblasts and intranuclear inclusions
151
Features of the Phenylalanine hydroxylase reaction
Hydroxylation of phenylalanine is irreversible so phenylalanine cannot be derived from tyrosine.
**Phenylalanine hydroxylase uses the co-enzyme tetrahydrobiopterin (BH4)** to supply reducing equivalents for the hydroxylation reaction.
Over the course of the phenylalanine hydroxylase reaction tetrahydrobiopterin is oxidized to dihydrobiopterin.
Tetrahydrobiopterin is regenerated from dihydrobiopterin by dihydrobiopterin reductase in a reaction involving the oxidation of NADPH.
152
What is hartnup disease
Autosomal recessive **Absence of an AA transporter in the proximal tubule**
Leads to loss of tryptophan in the urine, can cause niacin deficiency that lead to sx (pellagra)
153
What is the alanine cycle
Use of alanine to transfer ammonia to the liver
154
Drugs that can cause a folate deficiency
Phenytoin
Methotrexate
Trimethoprim
155
What is the hallmark of a B12 deficiency
Hugh methymalonic acid
Neuropathy (legs\>arms)
156
What is pernicious anemai
Autoimmune destruction of gastric parietal cells (type 2 hsr) that leads to deficiency of intrinsic factor so **low B12**
Associated with. HLA-DR and gastric adenocarcinoma
157
What vitamin allows for conversion. of Fe3+ to Fe2+
Vitamin C
Improtant for Heme synthesis and collagen synthesis
158
Vitamin high in sarcoidosis
Vitamin D
Conversion to 1,25 hydroxyvitamin D independent of the kidney, so get high calcium
159
What is acrodermatitis enteropathica
Low Zinc
Dermatitis, decreased hair, diarrhea, poor growth
160
What is Lecithin-cholesterol acyl transferase (LCAT)
Catakyzes rdterification of cholesterol - packages it tightly in core of HDL
## Footnote
**Activated by A-1 on HDL**
161
What is the most common cause of community-acquired pneumonia and describe vaccine
Strep pneumoniae (Gram negative)
## Footnote
Two vaccines:
- Pneumococcal polysaccaride vaccine, 23 valent, moderate B cell response
- Pneumococcal conjugate vaccine, polysaccride conjugated to protein so get robust T cell response
162
Mechanism of cataracts
Nuclear sclerosis (layers), photooxidative damage to crystalline, osmotic damage
163
Gene and associations
164
Features of carcinoid syndrome
165
What types of cells are involved in superantigen. response
T- cells (IL-2)
Macrophages (IL-1)
166
MOA of opioid side effects
Direct activation. of mast cells - release of histamines
167
Conduct disorder vs Oppositional defiant disorder
Conduct disorder is more severe, includes aggressive behavior, stealing, destruction
Oppositional defiant disorder is less severe, more irritable mood and defiance
168
169
What type of collagen is found in scars?
Type I
170
Type I vs Type II muscle fibers
171
Symptoms of cauda equina syndrome
Sciatic nerve - back. pain, lower limb weakness
Pudendal nerve - saddle parasthesia
Pelvic splanchnic (PNS) - constipation and urinary retention
172
Risk factors and sx of anal squamous cell carcinoma
173
What are the retroperitoneal organs
SAD PUCKER
174
Impact of hyperthyroidism on SVR
Increased metabolic demand and direct effect of thyroid hormone on vascular smooth muscle leads to **vasodilation and decreased SVR**
175
What is a septic abortion and common organisms
Infected retained products of conception
**Staph. aureus**
E. coli and group B strep
176
Steps of insulin synthesis and secretion
177
Most common side effects if Amphotericin B
**Renal toxicity** - can get hypokalemia and hypomagensemia
## Footnote
**Normocytic anemia**
178
What is heparin induced thrombocytopenia (HIT)
IgG antibodies against heparin and platelet factor IV, induces platelet aggregation
TX: Direct thrombin inhibitor (hirudin, lepirudin, argatroban)
179
Fetal vs adult hemoglobin
Fetal hemoglobin is made up of 2 alpha and two delta subunits, and has a hgher affinity for oxygen (allowd for better delivery of oxgen to baby)
Adult hemoglobin is two alpha and two beta. Replaces fetal by 6 months
180
Clinical manifestations of hemochromatosis
181
What glucose transporter is responsive to insulin and where is it located
GLUT 4
Adipocytes and skeletal muscle
182
Common DNA damage cuased by UV radiation
Pyrimidine dimers
183
What is the cause of microsatellite instability
Deficiency of mismatch repair (DNA slippage that is not repaired)
184
Cause and SX of ataxia telangectasia
Deficiency in non-homologous end joining repair
185
How are herpes virus antivirals activated
Drugs for HSV and VZV are phorpylated twice, first by **viral thymidine kinase**
Drugs for EBV and CMV are only phosphorylated by human TK because those viruses wont phosphorylaye
186
What is adenosine deaminase
Enzyme in purine metabolism tha**t converts adenosine to inosine.** Without it, toxic metabolites are made that inhibit DNA and lead to apoptosis.
ADA deficiency is bad for highly dividing cells like lymphocytes (severe combined immunodeficiency)
187
Action potentials of pacemaker vs non-pacemaker cells
188
TCAs side effects
Coma
Cardio stuff
Confusion
Anticholinergics
189
Causes of cleft lip amnd cleft palate
Cleft lip- maxillary prominence fails to fuse with intermaxillary. segment
Cleft palata - Palantine shelves fail to fuse with each other or with the primary palate
**Caused by polygenetics and environment**
190
Causes of polyhydramnios
191
What causes hemosiderin laden macrophages in the lungs
Chronic passive lung congestion as a result of chronic **heart failure** - fluid backs up in the lungs and RBC are pushed into lung parenchyma
192
Pathogenesis of ascites in cirrhosis
193
What travel through the jugular foramen
CN IX, X, XI, jugular vein
194
What. is. porphyrea cutanea tarda
Deficiency in uroporphrynogen decarboxylase, distrupts heme synthesis
causes photosensitivity. - vesicles
195
How is drug delivery to the brain improved
Helping cross the BBB by **distrupting tight junctions**
Stopping cellular eflux transporters like i**nhibiting p-glycoprotein**
196
How to tell if anisacoria (pupil size difference) is due to PNS or SNS dysfunction
If worse in dim light - SNS disturbance, affected pupil cant dilate
If worse in bright light - PNS, affected pupil cant constrict
197
Recombination vs reassortment
Recombination is crossing-over and swapping of genetic material between nonsegmented viruses
Reassortment is with segmented viruses
198
Impact of aging on bone marrow
Increased bone marrow fat and decreased BM mass
199
Breath sounds, fremitus, resonance
200
Impact of kidney disease on vitamin d, phosphate, and PTH
201
How is the G1 to S phase regulated
Regulated by **cyclin dependent kinases** (CDK4/6). Cyclin D binds CDK and activates it to phosphylate Rb tumor suppresor gene. This. phophorylation inhibits Rb. and allows the cell cycle to go forward
202
Lithium side effects
LMNOP
Lithium
Movement problems
Nephrogenic diabetes insipidus
hypOthyroidism
Pregnancy (ebstein)
203
How to diagnose mucormycosis vs asperigilosus
Mucosal biopsy.
Mucor is non-septate with. right. angles along vessels
Apergillus is septate with acute angles
204
Where is norepinephrine produced
Mainly produced in the **locus ceruleus** - pigmented cells in posterior rostral pons near floor of fourth ventricle
205
What are snRNAs/U-RNAs
Form of RNA with lots of uracil that helps to splice out exons (forms splicesome)
206
What enzyme adds amino acids to tRNA
Aminoacyl-tRNA synthetase
207
What triggers maturation into Th1 cells
Macrophages with MHC II present to Th0 cells with **IL-12** that then differentiate into Th1 cells
208
Path of embolis that causes retinal artery occlusion
Internal carotid --\> opthalmic artery --\> retianl artery
209
What is a rectocele
Herniation of rectum into posterior vaginal wall due to weakened pelvic floor (levator ani)
210
Relative risk equation
Use for a cohort study
RR = (risk of disease in exposed)/(risk in unexposed)
211
Short and intermediate acting benzos
212
Long acting benzos
213
What happens to BP and SVR during exercise
There is only a mild elevation in BP becuase of large drop in SVR - local metabolites cause vasodilation
214
Manifestations of chikungunya
215
What is hemolytic uremic syndrome (HUS)
Shiga toxin samages epithelium. (glomerular damage) that causes microthrombi that consume platelets and damage RBC - shistocytes
216
How is calcium pumped out of cardiac cells after contraction
Pumped back into SR using SERCA (Ca2+ and ATP)
Pumped out in exhange for Na+ using NCX (1 ca = 3 na)
217
Where is potassium during DKA
Potassium is driven out of the cells into the plasma, so **low intracellular and high/normal extracellular**
Insulin will drive potassium back into cells so also have to monitor potassium and give as needed during therapy
218
TX for serotonin syndrome
Cyproheptadine
219
TX for benzo overdose
Flumazenil
220
Duration of pharmacological insulin
221
Histology as you moved down the airway
Bronchi have a ciliated pseudostratified columnar epithelium with mucin-secreting goblet cells and submucosal mucoserous glands.
The airway epithelium gradually changes to ciliated simple cuboidal by the level of the terminal bronchioles.
Bronchioles lack glands and cartilage, and the number of goblet cells decreases distally, ending before the terminal bronchioles.
Ciliated epithelium persists up to the respiratory bronchioles.
222
Progression of p53 to Rb
Double stranded break activates p53, which induces p21. p21 inhibits CDK-cyclin complex. This leads to hypophosphorylation of Rb and cell cycle arrest
## Footnote
**Occurs at G1-S**
223
What is Li-Fraumeni syndrome
Mutation in TP53 that encodes for p53 leads to inhibition of cell cycle arrest due to damage = accumulation of damage and malignancy
224
In the golgi:
\_\_\_\_\_ added to asparagine
\_\_\_\_\_\_\_ added to serine and threonine
\_\_\_\_\_\_\_ added to lysosomal proteins
N-oligosaccharides added to asparagine
O-oligosaccharides added to serine and threonine
Mannose-6-phosphate added to lysosomal proteins
225
What is I-cell disease
Defect in Golgi (trans) of the enzyme that adds phosphate to mannose-6-phosphate. This causes a defect in adding proteins to lysosomes and proteins are secreted extracellularly.
Coarse features, gingival hyperplasia, clouded cornea, restricted joints, high plasma levels of lysosomal enzymes
226
Coating of vesicles and where they go (clathrin, CopI/II)
Clathrin - bw plasma membrane and golgi
COPI - Golgi to RER
COPII - RER to golgi
227
Function of three types of filaments
Microfilaments - muscle contraction (actin), cytokinesis
Intermediate - maintain cell structure (messed up in many cancers)
Microtubules - movement, cell division
228
What is kartagener's syndrome
Triad of symptoms seen in Primary Ciliary Dyskinesia 0 dyniene messed up, cilia cant beat
1. Chronic sinusitus
2. Brinchiectasis
3. Situs inversus
Also see infertility
229
Mneumonic for collagen types
Be So Totally Cool, Read Books
1 - Bone, Skin, Tendons
2 - Cartilage
3 - Reticulin (blood vessels)
4 - Basement membrane
230
Main components of collagen
Glycine, proline, lysine
## Footnote
**Gly-X-Y**
231
Steps in collagen synthesis
1. hydroxylation of pro alpha chains (vitamin C)
2. Glycosylation and forming triple helix (OI)
3. exocytosis and cleavage of terminal ends
4. Cross links (Menkes)
232
Osteogenesis imperfecta mutations
COL1A1 and COL1A2
Type 1 collagen
233
Elastin components
Non-hydroxylated and non-glycosylated proline, glycine, lysine
234
What causes Marfans
**F**BN1 mutation on chromosone **F**ifteen causes defect in **F**ibrillin
See constellation of Sx:
Mitral valve prolapse, aortic dissection and aneurysm, lens subluxation (up and out)
235
What is McCune-Albright syndrome
Example of mosaicism (cells have to be mixed to survive)
Gs-protein activating mutation, see precocious puberty, fibrous growth in bones, cafe au laet spots
236
Steps pf meiosis
237
Where are oocytes arrested in oogenesis
Primary oocyte arrested in **Prophase of meiosis I**
At puberty, a few secondary oocytes arrest in **metaphase of meiosis II**
Completion of meiosis II at fertilization
238
What is a robertsonian translocation
Frequent cause of trisomy 21, often 14 and 21 (parent is a carrier)
239
Hardy-Weinberg equation
P2 + 2pq + q2 = 1
p2 and q2 are homozygotes
2pq is heterozygote
If x-linked, male risk = q, female = q2
240
What is used to screen for neural tube defects
AFP AND Acetylcholineesterase
241
What is Von-Hippel Lindau
Mutation in VHL gene - overexpression of angiogenic growth factor (like VEGF)
Hemangioblastoma, renal clear cell carcinoma, pheocromocytoma
242
What is the greatest risk factor for completed suicide
Previous attempted suicide
243
What are the significance of the colors in melanoma
Red - vessel ectasia (dilation) and inflammation
Black/brown - advancing melanocytes
White - T lymphocytes target and melanocytes regress
244
What is X-linked aggamaglobulinemia
Mutation in **BTK gene** that prevents maturation and exit of BM by pre-B cells. Have low B cell and immunoglobulin levels
Susceptible to enteroviruses and encapsulated bacteria
245
Features of chronic bronchitis
Thickened bronchial walls, lymphocytic infiltrate, mucous gland elargment, patchy squamous metaplasia
Tobacco is strongest predisposing factor
246
Postpartum mood disorders
247
Number needed to treat
1/ARR
248
What is deficient in vegan dieta
Calcium, vitamin d, B12
249
What is seen in injury to common peroneal (fibular) nerve
Often break of neck of fibula
Loss of dorsal foot sensation, inability to evert foot, inability to dorsiflex
250
What nerves are present in the cavernous sinus
III, IV, VI, V (opthalmic and maxillary)
251
Steps in base excision repair
252
How does Chagas disease lead to megaesophagus and megacolon
Destriction of myenteric and submucosal plexi
253
Three associated esophagitises associated with HIV
254
How is blood flow to kidney autoregulated in chronic hypertension
1. afferent arteriole reflexesively constrict at higher BP
2. High BP leads to hyperfiltration that leads to higher Na and Cl in urine, leading to release of adenosine which further constricts afferent a.
If BP is corrected too rapidly, blood flow to kidney drops and can cause acute tubular necrosis
255
Effect of BNP and ANP
BNP released by ventricle stretch, ANP by atrial stretch
Cause vasodilation and diuresis
256
Pathogenesis of anemia of chronic disease
Disease causes release of inflammatory citokines, including hepcidin
Hepcidin binds to iron transporters on enterocytes and macrophages leading to less iron recycling and utilization
**less overall circulating iron and total iron binding capacit**y
257
What is apnea of prematurity
Central stimulatory neurons of respiratory center are not fully developed, leads to periods of apnea and bradychardia
258
How does iodide block production of thyroid hormone
Competitively binds to iodine receptor for uptake into thyroid and prevents iodine organification which reduces thyroid hormone release (Wolf-Chaickoff)
259
Levodopa side effects
Peripheral : nausea, vomiting, tachyarrhythmias, postural hypotension, hot flashes
Central: anxiey, irritation
260
Difference in PO2 and PCO2 in difdusion-limited perfusion
Due to changes in diffusion like fibrosis, emphysema
High gradient between alveolar O2 and capillary O2 because catn diffuse
Less of a gradient in CO2 because CO2 diffuses much more readily than O2
261
Normal alveolar gas exchange numbers
262
TX for beta blocker overdose and MOA
Glucagon
stimulates adrenergic receptors to increae cAMP
263
Why is reflux common in pregnancy
Elevated estrogen and progesterone decrease LES tone. Later, fetus can compress stomach
264
What mutation is associated with sebborheic keratosis
Fibroblast growth factor receptor 3
265
Acromegaly impact on heart
Can lead to ventricular hypertrophy leading to systolic dysfunction and heart failure
266
What is Prader-Willi syndrome
Paternal allel is deleted or mutation on chormosome 15 - PWS, 25% due to maternal uniparental disomy
Hyperphagia, obesity, hypogonadism, intellectual disability
267
What is Angelman syndrome
Maternal allele is deleted or mutated on ch 15 - UBE3A
SAIL - seizures, ataxia, intellectual disability, laughter
5% from uniparental oarental disomy
268
Features of trisomy 21
5 As:
Advanced maternal age, Atresia (duodenal), ASD, Alzhiemers, AML/ALL
269
Pregnancy screens associated with down sydnrome
Poorly formed nasal bones, nuchal translucency
Increased B-hCG
Decreased PAPP-A and AFP
270
What is Edwards Syndrome
Trisomy 18
PRINCE : Prominent occiput, rocker bottom feet, intellectual disability, nondisjunction, ears are low set
Also see clenched fist, congenital heart defect
Death usually by 1 yr
271
Maternal screens seen with trisomy 18
Low PAPP-A and B-HCG
Basically low everything
272
What is Patau sundrome
Trisomy 13
Cleft palate, holoProsencephaly, polydactyly, cutis apalasia, polycystic kidney disease, heart defect
Also die by 1 yr, low everything
273
What is Ducehenne muscular dystrophy
X-linked recessive mutation in DMD gene that encodes for dystrophin (frameshift - truncated protein)
274
What is dystrophin
Binds actin to transmembrane proteins (dystroglycan)
275
Features od Duchenne Muscular Dystrophy
Myonecrosis - elevated CK and aldolase
Waddling gait, using hands to push. up from chair, weakness
Cardiomyopathy is common cause of death - fibrosis
276
What is Becker muscular dystrophy
Milder form of Duchenne's caused by non-frameshift mutation so get abnormal protein instead of truncated
277
What is Fragile X syndrome
X-linked dominant tinucleotide repeat (CGG) in FMR1 gene. Causes hypermethylation and less expression
Intellectual disabilities, long face, everted ears, mitral valve prolapse, hypermobile joints
278
What is Freidrich's Ataxia
GAA repeat in frataxin gene, Autosomal recessive
Ataxic gait, hypertrophic cardiomyopathy
279
What is myotonic dystrophy
CTG repeats in DMPK gene, autosomal dominant, progressive muscle. wasting with prolonged contractions
Cataracts, balding, hypogonadism, arrhythmias
280
What is cri-du-chat syndrome
deletion of part of hsort arm of chromosome 5
Intellectual disability, infant has high pitched cry (like cat), microcephaly, cardiac (VSD)
281
What is Williams syndrome
Deletion on part of chromosome 7, partially for gene for elastin
Elfin features, intellectual disability, extreme friendliness with strangers, supravalvular aortic stenosis, hypercalcemia
282
Kleinfelter's
Usually 47 XXY - nondisjunction during meiosis. of either parent
Hypogonadism (low testosterone but high FSH, LH)
Gynecomastia, delayed puberty, low body hair and sperm count
283
Turner Syndrome
Females 45 XO, can be mosaic 45X/46XX if mitotic nondysjunction in zygote
Short stature, broad chest, webbed neck, lymph obstruction, hypogonadism (streak ovaries), low inhibin b and estrogen (high LH and FSH)
Primary ammenorhea, bicuspid aortic valve, coarctation of aorta, high bp, horseshoe kidney, osteoporosis
284
What is supplied by the inferior mesenteric artery
Hindgut derivatives - distal 1/3 transverse, descending, sigmoid colon, and rectum
285
What dictates the severity of mitochondrial disorders
Heteroplasmy - some cells may recieve many damaged mitochondria and others very few
286
Dobutamine MOA
Beta adrenergic agonist, especially in the heart.
Increases contractility and heart rate, increasing Oxygen demand. Small decrease in BP (vasodilation)
287
Where are actin and myosin
288
What DNA viruses are enveloped
Hepadnavirus
Herpesvirus
Poxvirus
289
Describe the complement system
290
Pathophys of tetrology of fallot
Abnormal neural crest cell migration that leads to deviation of the infundibular septurm.
## Footnote
1. overriding aorta
2. VSD
3. Right pulmonary obstruction
4. Right Ventricle hypertrophy
291
V/Q ratio as you move down the lung
Ventilation/Perfusion(Q) is lowest at the base and highest at the apex
Ventilation is slightly higher in the base and slightly lower in the apex (gravity changes complaince). Perfusion is very high in the base and very low in the apex
292
Common sites of globe fractures
Medial into ethmoid air cells
Inferior into maxillary sinus
293
What does the mandibular nerve (CN V3) innervate
TMJ, floor of mouth, anterior tongue, lower part of face, tensor tympani (muffles sound), muscles of mastication
294
How does the lac operon in E. Coli work
295
What is the diptheria toxin
An AB toxin
Inhibits cell protein synthesis by catalyzing ATP-ribosylation on EF-2
296
Heparin MOA
Binds to antithrombin III
Then binds Xa and thrombin
297
What are RANK-L and OPG (osteoprotegrin)
RANK-L binds RANK on osteoclasts and increases formation and activity
OPG binds to RANK-L and stops it from inducing osteoclasts = less osteoclast function
298
Histo features of an aortic aneurysm
Cystic medial degeneration - smooth muscle replaced by basophillic extracellular matrix znd elastic fragments w cysts
299
What is achondroplasia
Limb shortening with a normal torso due to overexpression of FGFR3 gene that **inhibits chondrocyte formation**
300
What leads to formation of testosterone and inhibin
GnRH --\> LH. --\> Testosterone
GnRH ---\> FSH --\> inhibin
301
What is the odds ratio
The odds of disease of exposed individuals vs non-exposed individuals
302
How does digoxin slow HR
Increased parasympathetic (vagal) tone
303
What does CD14 bind
Macrophages
304
Markers for natural killer cells
CD16 and CD56
305
Function of glucagon-like peptide 1
Secreted by gastric L cells, stimulates release of insulin and decreases glucagon
306
MOA of DPP-4 ihibitors
Stop the breakdown of GLP-1 (done by DPP-4) so increase the glucose-dependent release of insulin
GLIPTINS
307
Process of male genital development
Presence of SRY gene on Y chromosome leads to expression of testes-determining factor
Leydig cells = testosterone --\> DHT = external and internal (Wollfian) male development
Sertoli cells = anti-mullerian hormone = involution of paramesonephric ducts
308
How to calculate specificity and sensitivity
309
Features of anti-glomerular basement mebrane antibodies
Target type IV collagen so see linear deposits of IgG and C3 with RPGN and crescent formation
310
Functions of the frontal lobe
Executive functioning (abstraction, impulse inhibition), personality, motor function, language
311
Features of cerebellar tremor
Action tremor (increases as hand gets closer)
ataxia, gait disorder
Seen most commonly in multiple sclerosis
312
Vitamin supplements for infants
Vitamin D (low in breast milk, usually low sun)
Vitamin K (given as shot at birth)
Iron (greater than 4 months, low weight)
313
MOA of fibrates
Activate PPAR alpha leading to **decreased VLDL production and increased LPL function**
314
Function of Th1 T cells vs Th2
Both are CD4 T cells
Th1 - "cell mediated" immunity, activate macrophages and CD 8 cells. Also B cells
Th2 - "humoral" immunity, activate B cells to produce IgE and IgE
315
Anti-inflammatory cytokine
IL-10
316
Infections that need TH1 response
Intracellular - TB, listeria
317
Relatiosnhip bw macrophages and Th1
Th1 produce IFN- gamma that activates macriphages
Macrophages produce IL-12 that activate Th1
Deficiency: mycobacterium infections
318
Function of granzymes
Activate caspases that lead to cell apoptosis
Present in CD8 T cells
319
Function of Th17 cells
Important for GI bacteria regulation
Induce neutrophullic inflammation
320
What is the AIRE gene and deficiency
Autoimmune regulator in thymus
Without AIRE get CHAR
chornic candidiasis, hypoparathyroidism, adrenal insufficiency, recurrent candida infections
321
B cell surface proteins
322
Self cell markers that block complement
Decay Accelerating Factor (DAF/CD55)
CD59 inhibits MAC
323
Risks associated with early complement defifiency
Low C1 - C4
Pyogenic sinus and respiratory tract infections
324
Risks associated with late complement defifiency
Low C5-C9
Susceptible to Neisseria
325
What is C1 esterase inhibitor deficiency
Causes activation of C1 leading to low C4. Causes high bradykinin that leads to angioedema
326
Formation of leukotrienes, prostoglandins, and thromboxanes
327
What is erythropoeitin
Released by renal cortex and medula during hypoxia that stimulates BM to produce more RBC, increasing. capacity for oxygen
328
Which non-glucose monosacchride is metabolised fastest
Fructose, bypasses PFK-1 in glycolysis
329
Most common cause of pathologic nipple discharge
Intraductal papilloma
See epithelial and myoepithelial cells lining a vascular core
330
Function of interferon alpha and beta
Produced by virally infected epithelial cells, bind to neighboring cells that are induced to make antiviral RNA enzymes - target double stranded RNA for destruction
331
Location of purely sensory strokes
ventral posterior thalamus
332
Neonatal tetanus prevention
Vaccinating women with inactivated tetanus toxin, will pass IgG on to baby
333
MOA of the four TB drugs
334
Treatment for PID
Have to treat for both gonorrhea and chlamydia
Third gen cephalosporin for gonoccocal
Azithromycin or doxy for chlamydia (not suscpetible to beta lactams)
335
Features of hypertrophic cardiomyopathy
Hypertrophic interventricular septum that can block outflow
See increased LV mass, decreased LV volume, preserved ejection fraction, impaired LV relaxation
336
Causes of premature cataracts
Glucorticoids (predisone)
DM II
trauma
radiation
337
Function of kinesin
Move vesicles along microtubules from - to + which means away from the nucleus (like towards nerve terminals)
338
Most common cause of fetal hydronephrosis
Narrowing of ureter at the uretropelvic junction
339
Stroke mechanisms in context of cocaine
1. Cerebral vasospasm
2. BP increased and sympathetic tone increased, can lead to dissection and rupture of small vessels
3. Increased activation of platelets
340
Changes seen in distributive shock
Seen in widespread vasodilation (anaphylaxis)
Decreased SVR, decreased PCWP (lower volume), decreased venous return (CVP)
341
MOA of shiga toxin
AB toxin
A portion stops protein synthesis leading to death of mucosal cells. Stops binding of tRNA to 60s subunit
342
Promoter regions on DNA
TATA is 25 nucleotides from transcription
CAAT is 75
343
How to calculate alveolar ventilation
(tidal volume - dead space) x RR
344
What is superior mesenteric artery syndrome
When the transverse protion of the dudoenum is traooed between SMA and aorta
Can be due to diminished fat, burns, lordosis
345
Effects of Vasoactive intestinal peptide
Stimulates pancreatic bicarb and chloride secretion, increased cAMP in intestinal cells that causes release of sodium, chloride, water secretion into bowel
346
What is seen in a VIPoma
WDHA syndrome - watery diarrhea, hypokalemia, achlorhydria
347
What causes a narrowed mediastinum on neonatal XRay
Transposition of great arteries
348
Examples of drugs that directly stimulate dopamine receptors
Ergot alkaloids: bromocriptine
Nonergots: pramipexole, ropinirole
349
MOA of senna and bisacodyl
Stimulant laxatives
Activate enteric nerves in myenteric plexus to induce peristalsis
350
What is calcineurin
Dephosphorylates nuclear factor of activated T cells (NFAT) that moves to the nucleus to induce production of IL-2 (activate more T cells)
351
Probablity of exact HLA match between siblings
25 %
2 genes - 1 each from each parent
352
What is graft vs host disease
Grafted T cells proliferate in host and see host cells as foreign - type IV hypersensitivity
Rash, jaundice, diarrhea, hepatosplenomegaly
353
What is DiGeorge Syndrome
Failure to develop 3rd and 4th pharyngeal puches due to **22q11 deletion**
**Lack of thymus - T cell deficiency**
Lack of parathyroid - hypocalcemia
354
What is Severed combines immunodeficiencies
Cytoline receptor defectors
**Adenosine deaminase deficiency** (nucleotide salvage pathways)
MHC II deficiency
RAG mutation - VDJ recombinant deficient
355
X-linked agammaglobinemia
Defect in BTK tyrosine kinase gene
No B cell maturation so no Ig
356
Pathophys of hyper IgM syndrome
There is a defect on the CD40 receptor so B cells cant activate helper T cells - only produce IgM
T cells dont produce IL-4 and IL-5 that induce class swithcing so low IgE, IgA, IgG
Reccurent pyogenic infections and mucosal infections
357
What is wiskott aldrich syndrome
WATER
Thrombocytopenia, eczema, recurrent infections
Mutatoin in WAS gene (defective cytoskleton and antigen presentation)
X-linked
358
What do the paramesonephric ducts. form
They fuse laterally to form the fallopian tubes, uterus, cervix, and upper vagina
359
Where is robosomal rna found
Nucleolus
360
What are P bodies
Structures found in cytoplasm that play a role in mRNA regulation and turnover - can cause translation termination and storage of mRNA
361
How does cortisol attenuate hypoglycemia
During a prolonged fast, cortisol is released and diffused across cell membranes. In the cytoplasm it binds to receptors bound to heat shock proteins. The receptor is release from the HSP and dimerize. They move into the nucleus and induce gluconeogenic. enzyme production
362
Treatment for turners syndrome and MOA
Growth hormone
Binds to JAK receptor in the liver and activates STAT which goes to the nucleus to induce production of IGF-1
363
Gonadotropin regulation in males
364
Features of aortic regurgitation
Diastolic decrescendo murmur
Bounding femoral and carotic pulses - widened pulse pressure
Bobbing head - stronger LV
365
What is trendelenburg sign
Hip drop with foot raise on contralateral side fue to weakness of gluteus medius/superior gluteal nerve
Due to giving shots superomedial - should give superolateral
366
How is bioavailability calculated
Area under the curve of the route / area under IV curve
367
TX for toxo encephalitis
pyramethamine and sulfadiazine (or clinda)
368
What is primary central nervous system lymphoma
Second most common cause of ring enhancing lesions in HIV - usually solitary
Caused by B cells - usually with EBV
369
What vitamins do gut bacteria produce
Vitamin K and folate
Can be overproduced in Rou-en-Y procedure that produces blind ended gastric pouch
370
Brain features od Alzheimers
Neurodegeneration, B-amyloid plaques, neurofibrillary tangles
371
Mneumonic for reflexes
S1 S2 buckle my shoe
L3 L4 kick the door
C5 C6 Pick up sticks
C7 C8 lay them straight
372
Process of platelet plug formation
VWF bind platelets on exposed epithelium
Platelet is activated and releases TXA2 to cause vasoconstriction, more substances released to form clot
Prostacyclin (PGI2) and NO oppose formation
373
How to measure for primary hypothyroidism
Use TSH levels because small changes in thyroxin cause large changes in TSH - can drop before thyroxin drop is noticeable
374
What two factors stop fungal infections
T cells stop sueprficial fungal infections (skin)
Neutrophils stop hematogenous fungal infections
375
Common pathogens seen in cystic fibrosis
Staph aureus, H. Flu, Pseudomonas aeurigonas, Burkholderia capacia
376
MOA of loop diuretics
Block NaK2Cl transporters
Can cause metabolic alkalosis because losing more Cl- --\> body becomes less negative so holds onto HCO3-
Can give carbonic anhydrase inhibitor (acetazolamid) to lose bicarb
377
MOA of verinicline
Partial agonist of nicotine receptor -- lowers cravings for tobacco and attenuates response to it
378
What is Fas ligand
Produced by T cells that keep reacting to self antigen. Binds to CD95 and causes apoptosis
379
Phenotype of regulatory T CELLS
CD4CD25FOXP3
380
What lupus tests are specific and which are sensitive
Specific : anti-dsDNA and Anti-Sm
Sensitive: Anti-ANA
381
Why can people with lupus have a false positive lupus test
People with LES can have anticardiolioin antibodies (type of antiphospholipid) that is used to test for syphillis (released by damaged cells)
382
Common causes of drug induced SLE
Hydralazine, isoniazid, procainamide
383
Pathophys of sjogrens
Lyphocytes - Type IV hypersensitivity
Destruction of salivary and lacrimal glands
384
Common comorbidity with sjogrens
Rheumatoid arthritis
Will have rheumatoid factor in blood
385
What causes neonatal lupus
The Anti-SSA ribonucleoprotein, crosses the placenta and can cause heart block
386
What is systemic sclerosis (scleroderma)
Autoimmune damage of vessels
Endothelial damage leads to inflammation and inflammation - increased endothelin, TGF -beta, PDGF
Leads to fibrosis
387
What amino acids cant be absporbed in cystinuria
cysteine, lysine, ornithine, arginine
COLA
Get cystine stones (hexagon)
388
What is Rett syndrome
X-linked condition marked by normal development until 6-18 months then regression of speech, repetivive hand movements, gait abnormaities
MECP2 gene causes arrested brain development
389
What can cause malaria despite prophylaxis
P. Falciparum is resistant to chloroquine, have to give mefloquine
The larvae live in schizonts inside the liver for up to 30 days, mefloquine only works in the blood. If stop before 30 days, can develop malaria
390
Course of the ureters
From kidneys, pass posetrior to gonadal vessels, then anterior to the internal ileac, then posterior to uterine artery
391
Describe the Ras pathway
Growth factors bind to a tyrosine kinase which converts GDP bound to inactive Ras to GTP, making active Ras. Ras then activates MAP kinase that goes to the nucleus
392
Impact of hypothyroidism on lipids
Hypothyroidism causes a decrease in the number of LDL receptors leading to higher LDL
Also have decreased activity of lipoprotein lipase so high triglycerides
393
Role of CTLA4
Blocks CD80 on APC which stops interaction with CD28 on T cells, causing anergy
394
What determines the maintenance dose
Steady state cocentration x clearance x interval
395
What is leukocyte adhesion deficiency
Deficiency in CD18, WBCs cant roll and migrate
see late separation of the umbilical cord, low pus, dysfunctional neutrophils
396
Glucorticoids MOA
Inactivation of NF-KB, a key inflammatpry transcription factor
397
Cancer associated with Sjogrens
B cell lymphoma - will see unilateral parotid gland enlargment
398
What is scleroderma and manifestations
Systemic sclerosis - vasculopathy, collagen deposition, fibrosis
Diffuse - skin, early visceral, kidney (use ACE)
Limited - **CREST** (calcinosis, anti-centromere antibody, raynauds, esophageal dysmotility, sclerodatyly, telangesctasia)
399
What is Potters sequence
Oligohydramnios causes Flat face, limb deformities, pulm hypoplasia
400
Odds ratio calculation
(a/b)/(c/d)
401
MOA of rituximab
MAB to CD20 on B cells, leads to reduction in B cells
402
What WBCs increase in steroid use
Neutrophils - steroids induce them to "demergination" off of vascular wells so get more circulating
403
What to moitor on testosterone therapy
PSA (can enlarge prostate)
Hematocrit (can make blood more coagulable)
404
Four components of informed consent
disclosure, understanding, voluntariness, authorization (documents)
405
What structures are injured with an anteromedial displacement of humerus
Brachial artery and medial nerve
406
Organ with the highest oxygen demand
Myocardial tissue
407
SX of pineal gland tumor
Hydrocephalus (blocks aqueductal obstruction) and Parnaud syndrome (cant gaze upward)
Usually a germinoma
408
Blood supply of the rectus abdominus
Inferior epigastric arteries
Enters at the arcuate line (above line, m has anterior and posterior sheath. Below, only anterior)
409
Side effects of succinylcholine
Malignant hyperthermia
Hyperkalemia - K leaks out of cell when polarized
Bradychardia or tachycardia
410
SX of cholangitis
Fever, RUQ pain, jaundice (charcot triad)
Can have hypotension and altered mental status
Often caused by obstruction of common bile duct
411
HBV life cycle
HBV has a partially double stranded DNA sequence
Goes into cell and dumps DNA material into nucleus where it is "fixed" by host DNA polymerase and then transcribed by host machinery into + RNA that goes into cytoplasm
RNA is reverse transcribed. by viral reverse transcriptase into the partially double stranded DNA. Host ribosomes make protein capsule
412
What is alternative splicing
Process by which one gene can code for various proteins - exclusion or inclusion of certain. introns can create a different protein
413
Pathophys of neonatal respiratory distress syndrome
With inadequate surfactant production by type II pneumocytes, the lungs have a higher surface tension and are less compliant. This cuases atelectasis - can see ground glass opacities
414
Changes seen in obesity induced restrictive lung disease
Low FEV1, Low FVC, low ERV, can be low TLC
Normal RV
415
What is beri-beri
Thiamine deficiency
Dry - peripheral neuropathy
Wet - neuropathy and cardiac involvment
416
Which diuretic to use with heart failure
Spironolactone
Aldosterone blocking can stop heart remodeling
417
SX of hyperaldosteronsim
Hypertension
Hypokalemia
Metabolic alkalosis
Sodium is retained in exchange for dumping of K and H
See muscle weakness and paresthesias
418
What organs cant undergo hyperplasia
Pemanent cells - myocytes, skeletal muscle, nerves
419
Hallmark of reversible cellular injury
Cellular swelliong - loss of microvilli, membrane blebbing, swelling of RER
NA/K pump is inhibited and Na builds up, pulling water in
420
Hallmark of irreversible cellular injury
Membrane damage - plasma membrane, mitochondrial (cyt c leaks out = apoptosis), lysosomal
421
Dosing of hydrophilic vs hydroophobic drugs in obese patients
Hydrophilic - lower dose than body weight becuase drug will stay extracellulary - wont go into larger adipose store
Hydrophobic - higher dose, drug has to saturate fat before it can take effect
422
Origin of melanocytes
Neural crest -- Melanoma
423
Function of TNF-alpha
macrophage activation, phagolysosomes, granulomas
424
Muscles that control defecation
Internal and external sphincter
Puborectalis muscle
425
Neuro finding on REM sleep behavior disorder
alpha-synuclein
426
What impacts creatinine levels
Muscle mass and meat intake
427
Order of veins off of the SVC
external jugular drains into subclavian then drains into brachiocephalic then into SVC
428
What CN innervate sensory of ear
V, VII, IX, X
429
Normal flow of pleural fluid
Into pleaural space from intercostal microvessels, out via parietal pleural lymphatics
430
Pathophys of TMJ
Dysfucntion of joint, hypersensitivity of mandibular nerve (V3) and muscle spasm (masseter, pterygoid)
431
SX of typhoid fever
432
Conditions that make TB more likely
Chronic kidney disease, DM, HIV, immunosuppression
433
Bone changes in osteoporosis in aging vs hyperparathyroidism
Aging - lamellar bone is reduced
PTH - Cortical thinning (subperiostial)
434
Pathogenesis of migraines
Cortical spreading depression is allowed by increased cerbreal excitability. The cortical spreading (aura) leads to release of CGRP (calcitonin gene related peptides) that transmit pain and cause vasodilation
Triptans block CGRP
435
explain transient warfarin hypercoagulability
Protein C and VII have short half lives and are depleted first by warfarin. Protein C is an anticoagulant so allows Vitamin K to go unchecked until depleted
436
TX for delirium
Treat underlying disease (abx)
Antipsychotics (usually firsy gen - haloperidol)
437
What is acute intermittent porphyria
Deficiency in PBG deaminase (in porphyrin synthesis)
5 Ps - painful abdomen, Port wine pee, polyneuropathy, psychological disturbances, precipitated by drugs (P-450 inducers)
438
What is lymphogranuloma venereum
Caused by chlamydia L1-L3
Starts as painless ulcer, then several large painful ulcers along inguinal nodes (boobos)
TX: Doxy
439
MOA of calcium channel blockers (dihydropyridines vs non)
440
Effects of ANP and BNP
Kidney: increases GFR, diuresis, renin inhibition
Adrenal: reduce aldrosterone
Vessels: vasodilate and increase permeability
441
Metabolic effects of glucagon
Primarily increases glycogenolysis
Then decreases glycolysis and increases gluconeogenesis
442
Process of loss of nucleus
Pyknosis - shrinking
Karyorrhexis - fragmentation
Karyolysis - dissolution
443
What is coagulative necrosis
Necrotic tissue that remains firm, retains its shape
Proteins coagulate, no nuclei
INFARACTION - wedge shaped
444
What is caseous necrosis
Friable, cottage cheease like necrosis
Liquefactive necrosis with TB or fungal infection
445
What is saponification
Calcium deposit on fat necrotic tissue due to binding of calcium to fatty acids
446
What is metastitc calcification
When there are high serum calcium levels that forces clacium into tissues
447
What is Bcl2
Stabilized the mitochondrial membrane. Without it Cyt-C leaks out and activates caspases = apoptosis
448
What are the three free radicals and what degrades each
Superoxide (O2-) -- superoxide dismutaes
Peroxide H202 -- catalse
OH free radical -- glutathione peroxidase
449
Process of carbon tetrachloride injury
Becomes ccl3 a free radical
Damages hepatic cells = swelling = ribosomes pop off RER so less protein formation
Less apolipoprotein formation = fatty liver
450
Primary vs secondary amyloidosis
primary - deposition of AL amyloid from Ig light chain
secondary - AA amyloid (from acute phase reactant SAA)
See nephrotic cyndrome, cardiomyopathy, tongue enlargment, hepatosplenomegaly
451
Features of gardnerella
Thin off-white discharge, positive whiff test
No inflammation
Anaerobic gram-variable, see clue cells
452
Effect of heart on a-1 agonism
Becuase of vasoconstriction, baroreceptors will cause increased vagal stimulation leading to an inhibition of pacemaker rate
453
Common features of turner syndrome
454
How to measure lung maturity of fetus
The phosphotidylcholine/sphingomyelin ratio
Phosphotidylcholine is a component of surfactant so should increase
Sphingeomyelin is a phospholipid and shouldnt increase as much
455
Location of pneumonia if aspiration lying down vs standing
Always in the right
456
MOA of sofosbuvir
RNA-dependent RNA polymerase inhibitor
Stops formation of proteins
HCV
457
Hormonal changes in pregnancy
458
Most common site of ectopic impulse in A fib
Pulmonary veins
459
Sounds heard with mitral regurgitation
Holosystolic murmur at apex of heart radiating to axila\
Can produce an S3 - sign of volume opverload in LV
460
Where are gastric parietal cells located
Upper glandular layer
461
Serotype associated with reactive arthritis and SX
HLA-B27
