Classic Presentations

Question 1

Lesch Nyhan syndrome

X linked recessive, HGPRT deficiency

Answer 1

Gout
Intellectual disability
Self mutilating behavior in a boy

Question 2

Kartangener syndrome (dynein arm defect affecting cilia)

Answer 2

Situs inversus
Chronic sinusitis
Bronchiectasis
Infertility

Question 3

Osteogenesis imperfecta (type I collagen defect)

Answer 3

Blue sclera

Fx can be mistaken for child abuse

Question 4

Ehler’s Danlos type V classic type

Answer 4

Joint hyper mobility
Hyperextensible skin
Easy bruising
MVP

Question 5

Ehlers Danlos type III vascular subtype

Answer 5

Thin skin
Lobeless ears
BERRY ANEURYSM
Rupture kid hollow organs

Question 6

Marfan (fibrillin defect)

Answer 6

Arachnodactyly
Lens dislocation (upward)
AORTIC DISSECTION
Hyperflexible joints

Question 7

McCune Albright syndrome (Gs protein activating mutation)

Answer 7

Unilateral cafe au lait spot
Polyostotic fibrous dysplasia
PRECOCIOUS PUBERTY
multiple endocrine abnormalities

Question 8

Muscular dystrophy (MC Duchenne, X linked recessive frameshift mutation of dystrophin gene)

Answer 8

Calf pseudohypertrophy

Gowers sign

Question 9

Becker muscular dystrophy (X linked NON FRAMESHIFT deletions in dystrophin, less severe than DMD)

Answer 9

Slow progressive weakness in boys

Question 10

Patau syndrome (trisomy 13)

Answer 10

Infant with cleft lip/palate
Microcephaly or holoprosencephaly
Polydactyly
CUTIS APLASIA - lack of skin on top of head

Question 11

Edwards syndrome (trisomy 18)

Answer 11

Infant with microcephaly
ROCKER BOTTLM FEET
CLENCHED HANDS
Structural heart defects

Question 12

Down syndrome (trisomy 21)

Answer 12

Single palmar crease

Question 13

Wet beri beri (thiamine B1 deficiency)

Answer 13

Dilated cardiomyopathy
EDEMA
Alcoholism or malnutrition

Question 14

Pellagra (niacin B3 deficiency)

Answer 14

Dermatitis- sun exposed areas
Dementia
Diarrhea

Question 15

Scurvy (vit C deficiency, can’t hydroxylate proline/lysine for collagen synthesis)

Answer 15

Swollen gums
Mucosal bleeding
Poor wound healing
Petechiae

Question 16

McArdle disease (myophosphorylase deficiency, glycogen storage disease)

Answer 16

Chronic exercise intolerance with myalgia, fatigue, painful cramps, myoglobinuria

Question 17

Cori disease (debranching enzyme deficiency) OR von Gierke’s disease (glucose 6 phosphatase deficiency, MORE SEVERE)

Answer 17

Infant with hypoglycemia, hepatomegaly

Question 18

Pompe disease (lysosomal and glycogen storage disease, lysosomal alpha 1,4 glucosidase deficiency)

Answer 18

Myopathy (infantile hypertrophic cardiomyopathy)

Exercise intolerance

Question 19

Tay Sachs (ganglioside accumulation) or Niemann Pick disease (sphingomyelin accumulation), central retinal artery occlusion

Answer 19

Cherry red spot on macula

Question 20

Gaucher disease (glucocerebrosidase deficiency)

Answer 20

Gaucher cells
Hepatosplenomegaly
Pancytopenia
Osteoporosis
BONE CRISES
AVN FEMORAL HEAD

Question 21

Familial hypercholesyerolemia (decreased LDL signaling)

Answer 21

Achilles’ tendon xanthoma

Question 22

IgA deficiency

Answer 22

Anaphylaxis following blood transfusion

Question 23

Bruton disease (X linked agammaglobulinemia)

Answer 23

Male child
Recurrent infections
NO MATURE B CELLS

Question 24

Hyper IgE syndrome (Job syndrome: neutrophil chemotaxis abnormality)

Answer 24

Recurrent cold (non inflamed) abscesses
Eczema
High serum IgE
High EOSINOPHILS

Question 25

Scarlet fever OR Kawasaki disease

Answer 25

Strawberry tongue

Question 26

C. difficile infection

Answer 26

Abdominal pain Diarrhea Leukocytosis Recent antibiotic use

Question 27

Pott disease (vertebral TB)

Answer 27

Back pain Fever Night sweats

Question 28

Waterhouse Friderichsen syndrome (meningococcemia)

Answer 28

ADRENAL HEMORRHAGE Hypotension DIC

Question 29

Klebsiella pneumoniae pneumonia

Answer 29

Red CURRANT JELLY SPUTUM in alcoholic or diabetic patients

Question 30

Erythema migrans from Ixodes tick (Lyme: Borrelia)

Answer 30

Large rash with bulls eye

Question 31

NONPAINFUL, indurated: chancre (primary syphilis, Treponema pallidum) OR PAINFUL with exudate: chanchroid (Haemophilus ducreyi)

Answer 31

Ulcerated genital lesion

Question 32

Neurosyphilis (Argyll Robertson pupil)

Answer 32

Pupil accommodates but doesn’t react

Question 33

Condylomata lata (secondary syphilis)

Answer 33

Smooth, moist, painless, wart like lesions on genitals

Question 34

Jarisch Herxheimer reaction (rapid lysis of spirochetes results in endo toxin like release)

Answer 34

Fever, chills, HA, myalgia following antibiotic treatment for syphilis

Question 35

Pasturella multocida (cellulitis at inoculation site)

Answer 35

Dog or cat bite resulting in infection

Question 36

Coxsackie A Secondary syphilis Rocky Mountain Spotted Fever

Answer 36

Rash on palms and soles

Question 37

Mucor or Rhizopus fungal infection

Answer 37

Black eschar on face of patient with diabetic keto acidosis

Question 38

Congenital toxoplasmosis

Answer 38

Chorioretinitis Hydrocephalus Intracranial calcifications

Question 39

Erythema infectiosum/fifth disease (slapped cheek appearance, caused by Parvo B19)

Answer 39

Child with fever later develops red rash on face that spreads to body

Question 40

Measles

Answer 40

Cough, conjunctivitis, fever, coryza, diffuse rash

Question 41

Koplik spots (measles [rubeola] virus)

Answer 41

Small irregular red spots on buccal/lingual mucosa with BLUE WHITE CENTERS

Question 42

Aortic regurgitation

Answer 42

Bounding pulses Wide pulse pressure Diastolic heart murmur HEAD BOBBING

Question 43

Aortic stenosis

Answer 43

Systolic ejection murmur (crescendo descrescendo)

Question 44

Patent Ductus Arteriosus | Close w Indomethacin, OPEN with PGE analogs

Answer 44

Continuous machine like murmur

Question 45

Angina Stable: with moderate exertion Unstable: minimal exertion or at rest

Answer 45

CP on exertion

Question 46

Angina (- troponins) OR NSTEMI (+ troponins)

Answer 46

CP with ST depressions on EKG

Question 47

Dressler syndrome (autoimmune mediated post MI fibrinous pericarditis, 2 weeks to several months after acute episode)

Answer 47

CP Pericardial effusion/FRICTION RUB Persistent fever following MI

Question 48

Osler nodes (infective endocarditis, immune complex deposition)

Answer 48

Painful, red raised lesions on pads of fingers/toes

Question 49

Janeway lesions (infective endocarditis, septic emboli/microabscesses)

Answer 49

PAINLESS erythematous lesions on palms and soles

Question 50

Bacterial endocarditis

Answer 50

Splinter hemorrhages in finger nails

Question 51

Roth spots (bacterial endocarditis)

Answer 51

Retinal hemorrhages with PALE centers

Question 52

Becks triad of cardiac tamponade

Answer 52

Distant heart sounds Distended neck veins Hypotension

Question 53

Kawasaki disease (mucocutaneous lymph node syndrome, treat with IVIG and aspirin)

Answer 53

Cervical LAD, desquamating rash, coronary aneurysms, red conjunctiva, strawberry tongue, hand/foot changes

Question 54

Henoch Schonlein purpura (IgA vasculitis affecting skin and kidneys)

Answer 54

Palpable purpura on buttocks/legs Joint pain Abdominal pain (peds) Hematuria

Question 55

Hereditary hemorrhagic telangiectasia (Osler Weber Rendu syndrome)

Answer 55

``` Telangiectasias Recurrent epistaxis Skin discoloration AVMs GI bleed Hematuria ```

Question 56

Primary adrenocortical insufficiency - HIGH ACTH, HIGH MSH, (ie Addison disease)

Answer 56

Skin hyperpigmentation Hypotension Fatigue

Question 57

Carcinoid syndrome (right sided cardiac valvular lesions, HIGH 5 HIAA)

Answer 57

Cutaneous flushing Diarrhea Bronchospasm

Question 58

Hypothyroidism

Answer 58

Cold intolerance Brittle hair Weight gain

Question 59

Myxedema (caused by hypothyroidism, Graves’ disease [pretibial])

Answer 59

Cutaneous/dermal edema d/t deposition of mucopolysaccharides in connective tissue

Question 60

Chvostek sign (hypocalcemia)

Answer 60

Facial muscle spasm upon tapping

Question 61

Sheehan syndrome (postpartum hemorrhage leading to pituitary infarction)

Answer 61

No lactation postpartum Absent menstruation Cold intolerance

Question 62

Diabetic ketoacidosis (Kussmaul respirations)

Answer 62

Deep labored breathing/hyperventilation

Question 63

MEN 1 (autosomal dominant)

Answer 63

Pancreatic Pituitary Parathyroid tumors

Question 64

MEN 2B (autosomal dominant RET mutation)

Answer 64

Thyroid tumors Pheochromocytoma Ganglioneuromatoses Marfanoid habitus

Question 65

MEN 2A (autosomal dominant RET mutation)

Answer 65

Thyroid and parathyroid tumors | Pheochromocytoma

Question 66

Courvoisier sign (distal malignant obstruction of biliary tree)

Answer 66

Jaundice | Palpable, non tender gallbladder

Question 67

Mallory Weiss syndrome (alcoholic and bulimic patients)

Answer 67

Vomiting blood following longitudinal lacerations at GE junction

Question 68

Plummer Vinson syndrome (May progress to esophageal squamous cell carcinoma)

Answer 68

Dysphagia (esophageal webs) Glossitis Iron deficiency anemia

Question 69

Virchow node (abdominal metastasis)

Answer 69

Enlarged, hard left supraclavicular node

Question 70

Whipple disease (Tropheryma whipplei, PAS + foamy macrophages)

Answer 70

``` PAS the FOAMY WHIPPed cream in a CAN: Cardiac symptoms (endocarditis) Arthralgias Neurological symptoms Diarrhea ``` Abnl D xylose test

Question 71

Rovsings sign (acute appendicitis)

Answer 71

Severe RLQ pain with LLQ palpation

Question 72

McBurney sign (acute appendicitis)

Answer 72

Severe RLQ pain with deep tenderness

Question 73

Peutz Jeghers syndrome (inherited benign polyposis can cause obstruction, high cancer risk, mainly GI)

Answer 73

Hamartomatous GI polyps | Hyperpigmentation of mouth/feet/hands/genitals

Question 74

Gardner syndrome (subtype of FAP, chromosomal instability)

Answer 74

Multiple colon polyps Osteomas/soft tissue tumors Impacted/supernumerary teeth

Question 75

Budd Chiari syndrome (posthepatic venous thrombosis)

Answer 75

``` Abdominal pain Ascites Hepatomegaly Congestive liver disease- nutmeg liver Assoc with hypercoagulable state, polycythemia vera, postpartum, HCC ```

Question 76

Crigler Najjar syndrome (congenital unconjugated hyperbilirubinemia)

Answer 76

Severe jaundice in neonate

Question 77

Wilson disease (Kaiser Fleischer rings d/t copper accumulation)

Answer 77

Golden brown rings around peripheral cornea

Question 78

Cholelothiasis (gallstones)

Answer 78

``` Fat Female Forty Fertile Familial ```

Question 79

Cancer of pancreatic head obstructing bile duct

Answer 79

Painless jaundice

Question 80

Burton line (lead poisoning)

Answer 80

Bluish line on gingiva

Question 81

Fanconi anemia (genetic loss of DNA crosslink repair, often progresses to AML)

Answer 81

Short stature Cafe au lait spots Thumb/radial defects HIGH INCIDENCE OF TUMORS/LEUKEMIA, aplastic anemia

Question 82

Paroxysmal nocturnal hemoglobinuria (CD55/59 - on floe cytometry)

Answer 82

Red/pink urine | Fragile RBCs

Question 83

Cold agglutinin disease (autoimmune hemolytic anemia caused by Mycoplasma pneumoniae, infectious mononucleosis, CLL)

Answer 83

Painful blue fingers/toes | Hemolytic anemia

Question 84

Platelet disorders (Glanzmann thrombasthenia, Bernard Soulier, HUS, ITP, TTP)

Answer 84

Petechiae Mucosal bleeding Prolonged bleeding time

Question 85

B symptoms of lymphoma

Answer 85

Fever Night sweats Weight loss

Question 86

Mucosal fungoides (cutaneous T cell lymphoma) or Sezary syndrome (mycosis fungoides + malignant T cells in blood)

Answer 86

Skin patches/plaques Pastries microabscesses Atypical T cells

Question 87

CLL

Answer 87

WBCs that look smudged | “Crushed Little Lymphocytes”

Question 88

Erb Duchenne palsy (superior trunk [C5-C6] brachial plexus injury)

Answer 88

Neonate with arm paralysis following difficult birth, arm in “waiters tip” position

Question 89

Anterior cruciate ligament injury

Answer 89

+ anterior drawer sign

Question 90

Paget disease of bone (HIGH osteoblastic followed by HIGH osteoclastic activity)

Answer 90

Bone pain Bone enlargement Arthritis

Question 91

Osteoarthritis (osteophytes on PIP [Bouchard nodes], DIP [Heberden nodes])

Answer 91

Swollen, hard, painful joints in elderly patient, pain worse with activity

Question 92

Gout/podagra (hyperuricemia)

Answer 92

Sudden swollen/big toe joint | Tophi

Question 93

Sjogren syndrome (autoimmune destruction of exocrine glands)

Answer 93

Dry eyes Dry mouth Arthritis

Question 94

Reactive arthritis associated with HLA B27

Answer 94

Urethritis Conjunctivitis Arthritis in MALE

Question 95

Systemic lupus erythematosus

Answer 95

Butterfly rash | Raynaud phenomenon in YOUNG FEMALE

Question 96

Raynaud phenomenon (vasospasm in extremities)

Answer 96

Painful fingers/toes changing color from white to blue to red with cold or stress

Question 97

Scleroderma (CREST)

Answer 97

Anticentromere antibodies

Question 98

Kaposi sarcoma, associated with HHV 8

Answer 98

Dark purple skin/mouth nodules in patient with AIDS

Question 99

Pemphigus vulgaris (blistering)

Answer 99

Anti desmoglein (anti desmosome) antibodies

Question 100

Lichen planus (6 Ps)

Answer 100

``` 6 Ps: Pruritic Purple Polygonal planar papules Plaques ```

Question 101

Dating error, anencephaly, spina bifida (open NTDs)

Answer 101

HIGH AFP in amniotic fluid/maternal serum

Question 102

Cerebellar lesion

Answer 102

Ataxia Nystagmus Vertigo Dysarthria

Question 103

Babinski sign (UMN lesion)

Answer 103

Toe extensions/fanning upon player scrape

Question 104

Kluver Bucy syndrome (bl amygdala lesion)

Answer 104

Hyperphagia Hypersexuality Hyoeroralality

Question 105

Basal ganglia lesion

Answer 105

Resting tremor Athetosis Chorea

Question 106

Epidural hematoma (middle meningeal artery rupture)

Answer 106

Lucid interval after traumatic brain injury

Question 107

Subarachnoid hemorrhage

Answer 107

“Worst headache of my life”

Question 108

Parkinson’s disease (loss of dopaminergic neurons in substantial nigra pars compacta)

Answer 108

``` Resting tremor Rigidity Akinesia Postural instability Shuffling gait ```

Question 109

Huntington disease (autosomal dominant CAG repeat expansion)

Answer 109

Chorea Dementia Caudate degeneration

Question 110

Multiple sclerosis

Answer 110

Nystagmus Intention tremor Scanning speech Bl internuclear ophthalmoplegia

Question 111

Guillain Barre syndrome (acute inflammatory demyelinating polyradiculopathy subtype, C jenuni can precede it)

Answer 111

Rapidly progressive limb weakness that ascends following GI/upper respiratory tract infection (C jejuni)

Question 112

Neurofibromatosis type 1

Answer 112

``` AD CHROM 17 100% penetrance Cafe au lait spots Lisch nodules (iris hamartoma) Cutaneous neurofibromas Pheochromocytomas Optic gliomas ```

Question 113

Nevus flammeus (benign, associates with Sturge Weber syndrome)

Answer 113

Vascular birthmark (port wine stain) on face

Question 114

von Hippel Landau disease (autosomal dominant tumor suppressor mutation)

Answer 114

Bl renal cell carcinoma Hemangioblastomas Angiomatosis Pheochromocytomas

Question 115

Neurofibromatosis type 2

Answer 115

Bl vestibular schwannomas

Question 116

UMN damage

Answer 116

Hyperreflexia Hypertonia Babinski sign present

Question 117

LMN damage

Answer 117

Hyporeflexia Hypotonia Atrophy FASCICULATIONS

Question 118

Spinal cord lesion

Answer 118

Spastic weakness Sensory loss Bowel/bladder dysfunction

Question 119

LMN facial nerve (CN VII) palsy (UMN damage spares forehead)

Answer 119

Unilateral facial drooping involving forehead

Question 120

Meniere disease

Answer 120

Episodic vertigo Tinnitus Hearing loss

Question 121

Horner syndrome (sympathetic chain lesion)

Answer 121

Ptosis Miosis Anhydrosis

Question 122

Internuclear ophthalmoplegia (damage your MLF, may be unilateral or bilateral)

Answer 122

Conjugate horizontal gaze palsy | Horizontal diplopia

Question 123

Fanconi syndrome (multiple combined dysfunction of PCT)

Answer 123

``` Polyuria RTA type II Growth failure Electrolyte imbalances HYPOPHOSPHATEMIC RICKETS ```

Question 124

Secondary to EPO injection

Answer 124

Athlete with polycythemia

Question 125

Nephrotic syndrome

Answer 125

Periorbital or peripheral edema Proteinuria (> 3.5g/day) Hypoalbuminemia HYPERCHOLESTEROLEMIA

Question 126

Alport syndrome (type IV collagen mutation)

Answer 126

Hereditary nephritis Sensorineural hearing loss Retinopathy Lens dislocation

Question 127

Turner syndrome (45XO)

Answer 127

``` STREAK OVARIES Congenital heart disease (bicuspid aortic valve) Horseshoe kidney CYSTIC HYGROMA short stature Webbed neck Lymphedema Swollen hands/feet at birth ```

Question 128

Paget disease of breast (sign of underlying neoplasm)

Answer 128

Red itchy swollen rash of nipple/areola

Question 129

Peyronie disease (connective tissue disorder)

Answer 129

Fibrous plaques in soft tissue of penis with abnormal curvature

Question 130

Chronic bronchitis (hyperplasia of mucus cells, “blue blister”)

Answer 130

Hypoxemia Polycythemia Hypercapnia

Question 131

Emphysema (“pink puffer,” centriacinar [smoking] or panacinar [alpha 1 antitrypsin deficiency])

Answer 131

Pink complexion Dyspnea Hyperventilation

Question 132

Sarcoidosis (noncaseating granulomas, bl hilar lymphadenopathy, hypercalcemia)

Answer 132

Bilateral hilar lymphadenopathy, uveitis

Question 133

Granulomatous infections (7)

Answer 133

``` Tb and leprosy Fungal PNA (Histo, Blasto, Coccidio) Bartonella (cat scratch disease) Brucella Listeria in infants (granulomatosis infantiseptica) Schistosomiasis (worm) Syphilis (gummas) ```

Question 134

Granulomatous infections (7)

Answer 134

``` Tb and leprosy Fungal PNA (Histo, Blasto, Coccidio) Bartonella (cat scratch disease) Brucella Listeria in infants (granulomatosis infantiseptica) Schistosomiasis (worm) Syphilis (gummas) ```

Question 135

Granulomatous infections (7)

Answer 135

``` Tb and leprosy Fungal PNA (Histo, Blasto, Coccidio) Bartonella (cat scratch disease) Brucella Listeria in infants (granulomatosis infantiseptica) Schistosomiasis (worm) Syphilis (gummas) ```

Question 136

Obligate aerobes (4)

Answer 136

Nocardia (opportunistic inf) Pseudomonas Mycobacteria tb Bordatella pertussis Use oxygen to form ATP contain superoxide dismutase and catalase

Question 137

Obligate anaerobes (4)

Answer 137

Actinomyces (gums, dental abscesses) Bacteroides (abd abscesses) Fusobacterium Clostridium (botulinum, perfringens, tetani) Common among normal flora and gut Lack catalase and superoxide dismutase Don’t cause communicable dx, often live near mucosal surfaces OFTEN IN ABSCESSES Aminoglycosides ineffective (req oxygen to get into cells) Use fermentation Produce gases and short chain FAs- foul smell

Question 138

Abdominal abscess/perforation tx and why

Answer 138

Contain GN and Bacteroides fragilis (anaerobe) B fragilis R to many abx Tx: METRONIDAZOLE and GN agent

Question 139

Aspiration PNA tx and why

Answer 139

Mouth anaerobes enter lungs Peptostreptoccus, Fusobacterium, Prevotella Tx: CLINDAMYCIN

Question 140

Facultative anaerobes

Answer 140

Can live without oxygen but can use it if it’s available Staph, Strep, E. coli

Question 141

Obligate intracellular bacteria (2)

Answer 141

Chlamydia (cannot synthesize ATP) Rickettsia (depends on host ATP) Will not Gram stain well- inside other cells Difficult to culture Rickettsia dx clinically or serology (ab tests) Chlamydia dx Nucleic Acid Amplification Test (DNA test)

Question 142

Pyknosis

Answer 142

Nucleus condensing

Question 143

Karyorrhexis

Answer 143

Nucleus fragments

Question 144

5 positive acute phase reactants

Answer 144

``` CRP SERUM AMYLOID A FERRITIN HEPCIDIN FIBRINOGEN ```

Question 145

Negative acute phase reactants

Answer 145

Levels FALL in inflammation ALBUMIN TRANSFERRIN TRANSTHYRETIN

Question 146

3 major cytokines of acute inflammation

Answer 146

IL1 TNF alpha IL6 All endogenous pyrogens - need endogenous pyrogen to circulate systemically leading to increased PGE2 which works locally on hypothalamus

Question 147

Inflammatory phase of wound healing

Answer 147

First 24h to 3 days after wound NEUTROPHILS, platelets Inflammation and hemostasis CLOT FORMATION NEUTROPHIL INVASION via inc vasc perm

Question 148

3 to 7 days after wound

Answer 148

MACROPHAGES replace neutrophils ANGIOGENESIS Fibroblast infiltration Granulation tissue formation Type III collagen laid down Wound contraction by MYOFIBROBLASTS

Question 149

7+ days after wound

Answer 149

Remodeling Type III collagen replaced by TYPE I LYSYL OXIDASE LINKS COLLAGEN - need ZINC

Question 150

Sarcomas spread

Answer 150

Hematogenously

Question 151

Carcinomas spread

Answer 151

Via lymph

Question 152

4 carcinomas that spread hematogenously

Answer 152

Follicular thyroid carcinoma Choriocarcinoma Renal cell carcinoma HCC

Question 153

Cross sectional study

Answer 153

Snap shot in time, absence of time period DISEASE PREVALENCE Can show risk factor ASSOCIATION with dx but NOT establish causality

Question 154

Cohort study

Answer 154

Patients identified by EXPOSURE Compares group WITH exposure to group WITHOUT exposure Did exposure change likelihood of dx? RELATIVE RISK Can be prospective or retrospective NOT USED FOR RARE DISEASES

Question 155

Case control study

Answer 155

Identifies patient by DISEASE or NO DISEASE ODDS RATIO Need matching to decrease confounding variables USED FOR RARE DISEASES

Question 156

Parvo 19 infection causes what in SCD pts?

Answer 156

Aplastic crisis Virus infects RBC line in BM

Question 157

Xeroderma pigmentosum

Answer 157

AR Caused by defects in NUCLEOTIDE EXCISION REPAIR Severe sunburn with minimal UV exposure, signs of skin damage (freckles, actinic keratoses, hyperpigmentation, eye lesions)

Question 158

Histoplasma

Answer 158

Small intracellular yeasts in MACROPHAGES and grow in culture as small yeasts with NO TRUE CAPSULE

Question 159

Blastomyces

Answer 159

Broad based budding yeast

Question 160

Cryptococcus

Answer 160

Fungal meningitis | Prominent capsule

Question 161

Paracoccidioides

Answer 161

Multiple budding yeast surrounding mother cell. | Appears like “pilots wheel”

Question 162

Coccidioides

Answer 162

Non budding speherules filled with endospores

Question 163

Aspergillus

Answer 163

Monomorphic fungi with dichotomous branching at acute angles

Question 164

Sublimation

Answer 164

Person takes thought, feeling, or emotion that is socially or personally unacceptable and expresses it in a more acceptable way. Example- girl has friend die, girl writes to her friend for hours in her diary. Girl is taking overwhelming sadness and using writing to express her sadness in a more acceptable, less impairing way

Question 165

Hib vaccine components

Answer 165

Capsular polysaccharide coat (polyribitol phosphate) coupled to protein carried (diphtheria toxoid) making a T cell dependent vaccine which stimulates B cells to undergo isotope switching More robust immune response with protein toxoid present

Question 166

Negative regulator of PFK 1

Answer 166

Citrate When TCA slows down, citrate accumulates and moves into cytosol from mitochondria. Citrate negatively regulates PFK1 to decrease glycolysis activity Citrate is positive regulator of acetyl coA carboxylase- rate limiting enzyme of FA synthesis

Question 167

Plasmodium falciparum

Answer 167

High cyclic fevers- dt rupture of RBCs and release of merozoites Intracellular rings in RBCs Anopheles mosquito inoculate humans with sporozoites via bite. Sporozoites travel to liver and infect hepatocytes- latent phase

Question 168

Congenital rubella

Answer 168

Cataracts, deafness, PDA, hepatosplenomegaly, pulmonary stenosis Admin of live attenuated vaccine of MMR a month before pregnancy can prevent congenital rubella In adults- mild febrile illness, upper respiratory illness, LAD, MACULOPAPULAR RASH, joint pain

Question 169

Ataxia telangiectasia

Answer 169

Mutation in ATM, cannot repair dsDNA breaks by NHEJ Cerebellar atrophy, spider angiomas, IgA deficiency, lymphopenia, HIGH AFP HIGH INCIDENCE OF LYMPHOMAS AND LEUKEMIAS Primary immunodeficiency of B and T cells

Question 170

Huntington dx

Answer 170

AD trinueotide repeat expansion disorder CAG repeat expansion

Question 171

Marfan syndrome

Answer 171

AD defect in FBN1 Death caused by aneurysmal dilation and aortic dissection

Question 172

SANDman neurotransmitters for sleep

Answer 172

Serotonin- initiates sleep ACh- higher in REM, assoc with erections in men Norepi- lower in REM, ACh:NE ratio triggers REM Dopamine- arousal and wakefulness, levels rise with waking

Question 173

Fabry disease

Answer 173

Lysosomal storage dx X linked recessive Deficiency of alpha galactosidase A- intracellular accumulation of globotriaosylceramide ``` Neuropathy - pain in hands and feet Angiokeratomas DECREASED SWEAT RENAL DISEASE Cardiac disease CVA/TIA early age ```

Question 174

Krabbe dx

Answer 174

Galactocerebrosidase deficiency ``` Peripheral neuropathy Absence of reflexes Developmental delay Optic atrophy Seizures Globoid cells ``` Brain shows loss of myelin AR

Question 175

Tay Sachs dx

Answer 175

Deficiency of of Hexosaminidase GM2 ganglioside accumulation AR Progressive neurodegeneration ``` Devo delay Exaggerated startle response Cherry red spot on macula Lysosomes with onion skinning NO HEPATOSPLENOMEGALY ```

Question 176

Niemann Pick dx

Answer 176

AR deficiency in sphingomyelonase Cherry red spot on macula HEPATOSPLENOMEGALY Foam cells

Question 177

Gaucher dx

Answer 177

Glucocerebrosidase deficiency AR ``` peripheral neuropathy Hepatosplenomegaly Pancytopenia Diffuse bone pain AVN Interstitial Lung dx Gaucher cells ```

Question 178

Parvo virus in adults

Answer 178

Arthralgia and arthritis Commonly involves small joints of hands, wrists, knees, feet

Question 179

Clostridium perfringens cell lysis toxin

Answer 179

Alpha toxin- lecithinase (phospholipase) that causes myonecrosis and hemolysis Jaundice and renal failure can occu secondary to hemoglobinuria and myoglobinuria

Question 180

G6PD

Answer 180

X linked

Question 181

Pyruvate kinase deficiency

Answer 181

AR

Question 182

Yersinia enterolitica

Answer 182

Transmitted from pet feces, pork, contaminated milk Causes MESENTERIC ADENITIS and can mimic appendicitis, Crohn dx May cause reactive arthritis in adults

Question 183

Primary Tb

Answer 183

Located in lung parenchyma and hilar LN and generate granulomatous rxn called Ghon complex Reactivation dx shows organisms moving to upper lobe causing cavitation lesion

Question 184

Bullous pemphigoid

Answer 184

Autoimmune skin condition Abs target hemidesmosome- dystonin Hemidesmosomes attach epithelial cells to basal lamina of basement membrane - fluid collects under epithelial cell and basal lamina Negative Nikolsky sign Large, tense subepidermal blisters that don’t easily rupture

Question 185

Pemphigus vulgaris

Answer 185

Autoimmune skin condition Bullous skin condition with abs against desmosome components- desmoglein 1 and desmoglein 3 Involves oral mucosa Desmosomes connect lateral surfaces of two adjacent cells together- space between cells develops- flaccid intraepidermal blisters

Question 186

Subacute sclerosing panencephalitis

Answer 186

Rare late complication of MEASLES (rubeola) Defective virus persists in brain Change in behavior and personality, memory, myoclonic jerks, blindness, spasticity 7 to 10 years after infection

Question 187

Paramyxoviruses

Answer 187

Negative sense, ssRNA, helical, enveloped Mumps, measles, RSV, parainfluenza Mumps can cause: orchitis, pancreatitis, bl parotitis

Question 188

Lymphogranuloma venereum

Answer 188

Caused by CHLAMYDIA TRACHOMATIS serotypes L1-L3 Primary lesion is shallow ulcer/ self healing pupule PAINFUL draining enlarged abscessed LN termed “buboes” contain stellate abscesses

Question 189

Dengue virus

Answer 189

RNA flavivirus transmitted by Aedes mosquitos ssRNA, positive sense, enveloped, arbovirus N/V, RETRO ORBITAL EYE PAIN, leukopenia, joint pain, positive tourniquet test Vascular leak syndrome leads to fluid accumulation, shock, organ damage

Question 190

OCD treatment

Answer 190

TCAs- clomipramine SSRIs Clomipramine inhibit BOTH serotonin and NE reuptake

Question 191

Progressive multi focal leukoencephalopathy (PML) viral encephalitis caused by JC polygoma virus

Answer 191

Viral encephalitis caused by JC polyomavirus Infects oligodendrocytes, causing demyelination Extensive multi focal lesions in hemispheric or cerebellar white matter Eosinophilic intranuclear inclusions Affects immunocomp patients

Question 192

Lambert Eaton syndrome

Answer 192

Autoimmune neuromuscular condition marked by proximal muscle weakness and decrease DTRs, which improve after prolonged muscle contraction Abs in LES against PRE synaptic Ca channels LEMS assoc with malignancies (SCLC)

Question 193

EBV linked malignancies

Answer 193

EBV infects B cells, becomes latent and can reactivate and cause cancer Burkitt lymphoma Hodgkin lymphoma Nasopharyngeal carcinoma B and T cell lymphomas

Question 194

Entamoeba histolytica

Answer 194

Protozoa “Cart wheel” distribution of chromatic around nucleus and presence of ingested RBCs Bloody diarrhea, stool cysts, liver abscesses Amebiasis- bloody d, liver abscess w anchovy paste exudate, RUQ pain. Bx shows FLASK SHAPED ULCERS Eats RBCs

Question 195

Classic galactosemia

Answer 195

AR GALT deficiency - can’t metabolize lactose from diet Growth failure, CATARACTS, liver disease, MR Restrict lactose

Question 196

Wiskott Aldrich syndrome

Answer 196

X linked recessive Thrombocytopenia Recurrent sinus infections Eczema Dt WAS gene mutation- T cell cytoskeleton abnormalities

Question 197

CMV vs rubella congenital deafness

Answer 197

CMV- blueberry muffin baby, seizures, hepatosplenomegaly Rubella- cataracts, blueberry muffin baby, congenital heart dx (PDA)

Question 198

Tricuspid regurgitation

Answer 198

Holosystolic murmur that increases in intensity in inspiration Best heard at left sternal border at second and third intercostal space

Question 199

Aortic stenosis

Answer 199

Mid systolic ejection murmur Starts after S1 and ends before S2 Crescendo- decrescendo (diamond shaped) Best heard at RIGHT second interspace

Question 200

Mitral regurgitation

Answer 200

High pitched holosystolic murmur prominent over cardiac apex with patient in L lateral decubitus position Radiates to AXILLA

Question 201

Pulmonic regurgitation

Answer 201

Early diastolic murmur starts with S2 and ends before S1 Descrescendo May increase with inspiration High pitched blowing over L second and third intercostal spaces

Question 202

ANP and BNP

Answer 202

Released form atria and ventricle in response to myocardial wall stretch dt intravascular volume expansion Increase GFR, natriuresis, and diuresis

Question 203

Cardiac response to exercise

Answer 203

Initiated by muscle hypoxia and mediated by SNS 1. Low TPR- activates SNS- inc contractility and HR- inc CO, inc preload Vasodilation occurs dt ADENOSINE, K, LACTATE in plasma 2. INC SBP 3. DBP lowers/no change 4. Inc PP

Question 204

SBP determined by

Answer 204

SC (CO)

Question 205

DBP influenced by

Answer 205

TPR If peripheral vessels dilated- drop in DBP If peripheral vessels constricted- higher DBP

Question 206

HCM

Answer 206

Interventricular septal hypertrophy that obstructs LV outflow Systolic murmur decreases with with maneuvers that INCREASE LV VOLUME (inc preload by passive leg raise) or increase after load (handgrip)- alleviates outflow obstruction Inc LV muscle mass Small LV cavity Preserved EF IMPAIRED RELAXATION OF HYPERTROPHIED LV- diastolic dysfunction

Question 207

Midsystolic click

Answer 207

MVP

Question 208

S3

Answer 208

Occurs in diastole during RAPID FILLING PHASE best heard at apex Caused by sudden limitation of ventricular movement during passive ventricular filling Normal in young, pregnant Abnormal in pts >40- suggests abnormal VL cavity enlargement (eccentric hypertrophy) from chronic mitral regurgitation, aortic regurgitation, DCM

Question 209

S4

Answer 209

Low frequency, late diastolic sound coinciding with atrial systole High atrial pressure- LA must push against stiff LV wall Caused by sudden rise in EDP as blood strikes stiff ventricle, suggests concentric hypertrophy from prolonged HTN, aortic stenosis Always abnormal

Question 210

S3

Answer 210

Occurs in diastole, best heard at apex Caused by sudden limitation of ventricular movement during passive ventricular filling Normal in young, pregnant Abnormal in pts >40- suggests abnormal VL cavity enlargement (eccentric hypertrophy) from chronic mitral regurgitation, aortic regurgitation, DCM

Question 211

S4

Answer 211

Low frequency, late diastolic sound coinciding with atrial systole Caused by sudden rise in EDP as blood strikes stiff ventricle, suggests concentric hypertrophy from prolonged HTN, aortic stenosis

Question 212

Large a wave (increase atrial contraction pressure) on venous tracing

Answer 212

Tricuspid stenosis | RHF/pulm HTN

Question 213

Cannon a wave (atria against closed tricuspid valve)

Answer 213

Complete heart block PAC/PVC Ventricular tachycardia

Question 214

Absent a wave (no organized atrial contraction)

Answer 214

Atrial fribrillation

Question 215

Giant V wave

Answer 215

Tricuspid regurgitation

Question 216

Carotid massage

Answer 216

Tricks carotid sinus into thinking high BP Result- lowered HR, vasodilation, lowered BP - veins/arteries dilate, decreased HR Can have syncope/passing out while shaving or buttoning shirt Carotid sinus senses both high and low BP. Signals to brain via CN9

Question 217

Coronary blood flow during tachycardia

Answer 217

Less time in diastole, less flow to coronary arteries

Question 218

Hypertonic solution infusion

Answer 218

Expands ECF Reduce ICF Raise osmolality of both fluid compartments

Question 219

Parinaud syndrome

Answer 219

``` Pinealoma Upgaze paralysis Inability to converge No rxn to light High ICP ```

Question 220

What changes ICF volume

Answer 220

ECF osmolality

Question 221

Low ECF OSMOLALITY

Answer 221

High ICF volume

Question 222

High ECF osmolality

Answer 222

Low ICF volume

Question 223

Aortic dissection

Answer 223

Tearing, excruciating CP Intimal layer tear- blood extravasates in media and creates false lumen HTN MC predisposing factor and defects in CT Aortic regurgitation develops dt dilation of ascending aorta WIDENED MEDIASTINUM

Question 224

BRCA1

Answer 224

Tumor suppressor | Chrom 17q

Question 225

BRCA2

Answer 225

Chrom 13q

Question 226

Chromosome 17

Answer 226

BRCA1 NF1 p53

Question 227

Chromosome 3

Answer 227

VHL- capillary hemangioblastomas , RCC, pheochromocytoma

Question 228

Chromosome 5

Answer 228

APC - mutated in FAP

Question 229

Chromosome 11

Answer 229

WT1 and 2- Wilms tumor

Question 230

Chrom 13

Answer 230

Retinoblastoma, osteosarcoma

Question 231

Chrom 18

Answer 231

DCC- FAP

Question 232

Chrom 22

Answer 232

NF2- acoustic neuromas, meningiomas

Question 233

MVP cause

Answer 233

Defects in CT tissue that predispose myxomatous degeneration of mitral leaflets and chordae tendinae Midsystolic click followed by MR murmur Murmur disappears with squatting/ maneuvers that increase LVEDV

Question 234

Granulomatosis with polyangiitis

Answer 234

c ANCA positive (PR3-ANCA) Systemic vasculitis with LUNG manifestations (purulent nasal discharge, cavitary lung lesions) SADDLE NOSE DEFORMITY RENAL INSUFFICIENCY (High BUN/Cr) and hematuria Bx shows necrotizing arteritis with granulomatous inflammation and NO COMPLEMENT DEPOSITION

Question 235

Wilson dx presentation

Answer 235

Movement abnormalities (Parkinson’s like) Psychiatric symptoms (personality changes, psychosis) Liver failure, chronic hepatitis, cirrhosis KAYSER FLEISCHER RINGS

Question 236

Syringomyelia

Answer 236

Loss of UE pain and temperature sensation, UE weakness, hyporeflexia, LE weakness, hyperreflexia Central cystic dilation of cervical spinal cord causing damage to central white commissure and anterior horns

Question 237

Subarachnoid hemorrhage complication

Answer 237

Communicating hydrocephalus Presents with deteriorating mental status Results from blood induced impairment of ABSORPTION OF CSF BY ARACHNOID GRNAULATIONS

Question 238

Polyarteritis nodosa

Answer 238

Segmental transmural fibrinoid necrotizing inflammation of medium arteries Manifestation arise dt ischemia from arterial lumen narrowing/thrombosis or bleeding from microaneurysms Affects kidneys, skin, peripheral nerves, GIT Assoc with hepB/C (immune complexes)

Question 239

Vitamin E deficiency

Answer 239

Occurs in pts with fat malabsorption Symptoms mimic Friedrich ataxia- ATAXIA (generation of spinocerebellar tracts), LOSS OF POSITION AND VIBRATION (degeneration of dorsal columns) and loss of DTRs (peripheral nerve degeneration) Neuro dysfunction and hemolysis bc vitamin E is antioxidant

Question 240

Friedrich ataxia

Answer 240

AR Trinucleotide repeat disorder- GAA in frataxin Gene ``` HCM Kyphoscoliosis High arches Ataxia Loss of position and vibration Spastic weakness ```

Question 241

Milrinone

Answer 241

PDE3 inhibitor that inhibits cAMP degradation 1. In cardiomyocytes- Increased intracellular Ca - positive inotrope to improve SV and CO 2. In VSMCs- increase Ca uptake by SR which reduces myosin light chain kinase interaction and stimulates vasodilation- vasodilation- reduces preload and afterload

Question 242

Nitroprusside

Answer 242

Used in HTN emergency, shirt acting Increases cAMP and NO release Venous AND arteriolar vasodilation - low preload (low venous return) and low afterload CN toxicity with prolonged use - CN inhibits ETC, toxic with prolonged infusions

Question 243

Anthracyclines (doxorubicin)

Answer 243

Binds topoisomerase II to cleanse DNA Binds iron to generate free radicals Tox- dilated cardiomyopathy Prevented by dexrazoxane

Question 244

Bleomycin

Answer 244

Indices free radical formation Tox- pulmonary fibrosis

Question 245

Cisplatin

Answer 245

Crosslinks DNA to inhibit DNA synthesis Nephroxtoxic, ototoxic, peripheral neuropathy

Question 246

Cyclophosphamide

Answer 246

Cross links dna to inhibit dna synthesis Hemorrhagic cystitis Bladder cancer

Question 247

Paclitaxel

Answer 247

Inhibits microtubule disassembly Neuropathy

Question 248

Vinceistine/vinblastine

Answer 248

Blocks beta tubulin to inhibit microtuble formation Neuropathy

Question 249

Dihydropyridines

Answer 249

Amlodipine, felodipine, nifedipine CCBs Vascular smooth muscle- peripheral vasodilation, no effect on contractility or conduction

Question 250

Nondihydropyridines

Answer 250

Verapamil, diltiazem Verapamil- cardiac muscle, reduces HR and contractility Diltiazem- VSM and cardiac muscle- vasodilation and reduces HR and contractility

Question 251

Clopidogrel

Answer 251

Irreversibly blocks P2Y12 on ADP receptors on platelet surfaces Prevents platelet aggregation

Question 252

Rifampin

Answer 252

Inhibits bacterial DNA dependent RNA pol GI side effects, rash, red orange body fluids, cytopenias

Question 253

Isoniazid

Answer 253

Inhibition of mycolic acid synthesis Neurotoxic (give B6/pyridoxine) Liver toxic

Question 254

Pyrazinamide

Answer 254

Unk mech Liver toxic, hyperuricemia

Question 255

Ethambutol

Answer 255

Inhibition of arabinosyl transferase Optic neuropathy

Question 256

What cells form fibrous cap in atherosclerosis

Answer 256

VSMCs

Question 257

Why is pO2 in left heart lower than pulmonary capillaries?

Answer 257

pO2 in LA and LV lower than pulmonary capillaries dt mixing of oxygenated blood from pulmonary weins with deoxygenated blood from bronchial circulation and thebesian veins (small cardiac veins)

Question 258

Directive counseling

Answer 258

Ethically appropriate when only one medical treatment is medically reasonable ie a woman with placenta previa counseled for C section bc medically necessary

Question 259

Major stimulator of respiratory drive in healthy patients

Answer 259

Arterial partial pressure of CO2 Even a minor increase PaCO2 stimulates central chemoreceptors and triggers increased ventilation

Question 260

Respiratory drive in COPD patients

Answer 260

COPD pts have decreased sensitivity to arterial partial pressure of CO2 (dt chronic CO2 retention) and May also have profound hypoxemia, therefore arterial partial pressure of O2 become significant contributor to respiratory drive

Question 261

Peripheral chemoreceptors

Answer 261

Carotid and aortic bodies Primary sites for sensing arterial O2 and are stimulated by hypoxemia and can be suppressed by supplemental oxygen

Question 262

Central chemoreceptors

Answer 262

Located in medulla Involved in respiratory response to hypercapnia - CO2 readily diffuses thru BBB and forms H ions in CSF- decreased pH detected by medullary neurons, triggering increase in respiration - bbb impermeable you H ions therefore blood pH has little effect on central chemoreceptors

Question 263

Beta 1 receptors

Answer 263

Heart, kidney Increase HR, contractility Stimulate renin release BLOCKADE- decrease CO, ECV, and BP

Question 264

Beta 2 receptors

Answer 264

Peripherally- liver and muscles, lungs Dilated blood vessels Bronchodilate Blockade does not lead to lowered BP

Question 265

Beta 1 selective antagonists

Answer 265

Atenolol, metoprolol, esmolol Used for HTN- lower CO, ECV- lowered BP Metoprolol given in systolic HF- blocks SNS stimulation of heart, reduces mortality

Question 266

Beta1,2 nonselective blockers

Answer 266

Propranolol, timolol, nadolol Nadolol and propranolol used in PORTAL HTN Beta1 block- low CO, ECV- low BP Beta2 block- decreases portal flow Timolol- glaucoma Beta1 and 2- aqueous humor production

Question 267

Beta1,2, alpha1 blockers

Answer 267

Carvedilol, labetalol Labetalol- HTN emergency, rapid reduction in BP Carvedilol- blocks SNS stimulation of heart, reduces mortality Alpha 1 block- fall in TPR and BP

Question 268

BB partial agonists

Answer 268

Pindolol (b1b2) acetbutolol (b1) Intrinsic sympathomimetic activity - B agonist when sympathetic activity LOW - B blocker when sympathetic activity HIGH Can cause ANGINA via b1

Question 269

BB side effects

Answer 269

Fatigue ED Depression Hyperlipidemia

Question 270

BB and DM

Answer 270

Can precipitate hypoglycemia Blockade of epinephrine effects (epi raises glucose levels), blockade-> hypoglycemia Tremors/tachycardia associated with hypoglycemia masked by BB SWEATING WILL NOT BE MASKED- muscarinic effect

Question 271

BB In COPD/Asthma

Answer 271

Caution B2 receptors- brinchodilators B1 CARDIOSELECTIVE USED

Question 272

Decompensated HF and BB

Answer 272

B1 blockers lower CO-> worsen symptoms Can be used in compensated HF

Question 273

BB overdose

Answer 273

Depression of myocardial contractility-> shock Bradycardia/AV block TREAT WITH GLUCAGON - activates adenylyl Cyclades at different site from beta receptors - increase cAMP—> increases intracellular Ca—> increased contraction and HR

Question 274

Alpha blockers (-osin)

Answer 274

Tamsulosin, doxazosin, terazosin, alfuzosin Alpha1 receptors in periphery- vasoconstriction BLOCK alpha1-> vasodilation -> decreases TPR —> decreased BP USED IN BPH Side effect- postural hypotension Tamsulosin- uroselective, less hypotension effect

Question 275

Alpha2 receptors

Answer 275

Presynaptic receptors on neurons that release NE onto VSMCs to cause vasoconstriction NE feeds back on alpha2 receptor and once activated, neurons releases less epinephrine Alpha2 AGONISTS effective for HTN

Question 276

Alpha2 agonists

Answer 276

Clonidine - rebound HTN side effect from abrupt cessation of drug- severe HTN- symptoms of HTN AND SNS OVERACTIVITY (sweating, nervousness, HA, CP) - causes SEDATION Methyldopa - DRUG OF CHOICE IN PREGNANCY - causes sedation - May cause HEMOLYTIC ANEMIA

Question 277

CCP classes

Answer 277

Dihydropyridines (nifedipine, amlodipine)- vasodilators Nondihydropyridines (verapamil, diltiazem)- negative chronotropes/inotropes, SIMILAR TO B1 BLOCKERS

Question 278

Dihydropyridines SE

Answer 278

Amlodipine, nifedipine EDEMA- increases capillary hydrostatic pressure dt PRECAPILLARY ARTERIOLAR VASODILATION

Question 279

Dihydropyridine CCB sure of action

Answer 279

Preferentially dilate PRE CAPILLARY ARTERIOLE Mechanism of edema side effect

Question 280

Nondihydropyridines (verapamil, diltiazem)

Answer 280

Used in HTN, heart disease - arrhythmias- a five - stable angina- lower o2 demand Side effect- negative inotropes, can precipitate HF Constipation Hyperprolactinemia- seen in verapamil, blocks Ca in CBS-> low dopamine release. Low prolactin can cause hypogonadism, low libido, irregular menses, galactorrhea

Question 281

Side effect of CCB, phenytoin, cyclosporine

Answer 281

Gingival hyperplasia

Question 282

Angiotensin II Effects (5)

Answer 282

``` SNS activation Renal Na/Cl reabsorption Arteriolar vasoconstriction Adrenal aldosterone secretion Pituitary ADH secretion ``` Net result- increased salt and water retention, INCREASE PRELOAD, INCREASE AFTERLOAD, INCREASE BP

Question 283

ACEi and ARB side effects

Answer 283

``` Hyperkalemia(low aldosterone) Renal failure (low GFR) ``` ACEi specifically- angioedema and cough dt high bradykinin

Question 284

Aliskiren

Answer 284

Direct renin inhibitor REDUCES AMOUNT OF ANGIOTENSIN I

Question 285

Hydralazine

Answer 285

``` Direct arteriolar vasodilator (reduces afterload) Combined with nitrates for HF Rarely used for HTN Safe in pregnancy Causes drug induced lupus ```

Question 286

Drug induced lupus drugs

Answer 286

Hydralazine Procainamide INH Anti histone abs

Question 287

Fenoldapam

Answer 287

Dopamine1 receptor - arteriolar vasodilation and increased renal perfusion - increased salt and water excretion - maintains renal perfusion while vasodilation HTN emergency

Question 288

Rheumatic fever

Answer 288

Weeks after GAS pharyngitis Common in Peds T2HSR Antibodies to bacterial M proteins cross react

Question 289

JONES criteria for RF

Answer 289

``` Joint pain (polyarthritis) Heart- endocarditis, valvulitis, myocarditis, pericarditis Nodules- subQ Erythema marginatum (rash on trunk) Sydenham chorea ```

Question 290

Rheumatic HD

Answer 290

``` Damage to heart valves by RF MITRAL VALVE MC involved Often presents years after ARF, pts do not remember acute symptoms COMMON IN DEVELOPING COUNTRIES - immigrants to US ```

Question 291

MI complications- arrhythmia

Answer 291

COD 24h after MI, occurs within few days of MI

Question 292

Post infarction pericarditis

Answer 292

1-3 days post MI | friction rub

Question 293

Papillary muscle rupture

Answer 293

2-7 days after MI POSTERIOMEDIAL PAPULLARY RUPTURE Can cause severe mitral regurgitation

Question 294

Interventricular septal rupture

Answer 294

3-5 days Macrophage mediated VSD HYPOTENSION, RHF, edema

Question 295

Pseudoaneurysm

Answer 295

3 to 14 days MC inferior wall Rupture in wall, blood leaks out into pericardium/scar tissue covers it, can rupture

Question 296

Free wall rupture

Answer 296

5 to 14 days Free wall rupture, cardiac tamponade LV wall rupture

Question 297

True aneurysm

Answer 297

MC anterior infarct Weeks to months Myocardial wall replaced with scar tissue, bulges out, doesn’t contract, blood stasis, thrombus

Question 298

Aortic stenosis clinically

Answer 298

Systolic crescendo descrescendo murmur Syncope- failure to increase CO dt high afterload Angina- increased LVEDP decreases coronary blood flow LHF- high LVEDP

Question 299

AS causes

Answer 299

1. Senile aortic stenosis- wear and tear, collagen breakdown, Ca deposition 2. Bicuspid aortic valves 3. Rarely- RHD

Question 300

Mitral stenosis

Answer 300

Decreases preload Causes by RF MC symptom- dyspnea bc LA pressure very high that translates back to lungs-> pulmonary congestion Diastolic rumble w opening snap

Question 301

Tricuspid stenosis

Answer 301

Diastolic murmur at LLSB Caused by RF and Carcinoid syndrome

Question 302

Pulmonic stenosis

Answer 302

Congenital defect in peds - fused commusures with thick leaflets Carcinoid syndrome

Question 303

aortic regurgitation

Answer 303

Blood leaks across aortic valve Diastolic problem Increased preload, increase SV, INCREASED AFTERLOAD (more SV-> aorta-> less compliance Blowing diastolic murmur Caused by dilated aortic root, bicuspid aortic valve, endocarditis, RHD

Question 304

Aortic regurgitation clinically

Answer 304

Wide PP (very low diastolic pressure w high CO) Head bobbing, water hammer pulses

Question 305

Mitral regurgitation

Answer 305

Increased preload, increased SV BLOOD LEAKS ACROSS MITRAL VALVE, increased LA PRESSURE-> starting mechanism-> increase LV filling from LA-> increased preload and SV REDUCED AFTERLOAD

Question 306

Fick equation for CO

Answer 306

CO= o2 consumption/(arterial o2 - venous o2) Swanz Ganz catheter gives CO O2 consumption proportional to body size Arterial content o2- o2 sat on finger, ABG Venous o2 content - o2 from swanz ganz

Question 307

Flow equation

Answer 307

Used to determine SVR SVR= MAP-RAP/CO Swanz ganz gives SVR

Question 308

Cardiogenic shock

Answer 308

Hallmark: LOW CO HIGH CARDIAC PRESSURES High SVR (SNS response) Classic cause- MI, also seen in advanced HF (depressed LVEF) Tx- inotropes- milrinone, dobutamine

Question 309

Hypovolemic shock

Answer 309

Poor fluid intake, GI loss High fever, insensible losses in lungs Hemorrhage LOW CO LOW CARDIAC PRESSURES HIGH SVR(SNS response) Blood transfusion, IVF

Question 310

Distributive shock

Answer 310

``` LOW SVR Sepsis (MC) Anaphylaxis Neurogenic (massive intracerebral bleed) Cardiac output classically high Cardiac pressures variable ``` Abnormal distribution of body fluids- diffuse vasodilation and/endothelial dysfunction Fluid leaks out of vascular space and into tissue beds Tx w vasopressors- phenylephrine, epi, NEVER

Question 311

Cold skin on exam

Answer 311

High SVR, low CO CARDIOGENIC HYPOVOLEMIC

Question 312

Warm skin on exam

Answer 312

Low SVR, high CO distributive

Question 313

High jugular venous pressure

Answer 313

High RA pressure Cardiogenic shock

Question 314

Pulmonary rales

Answer 314

High LA pressure Cardiogenic shock

Question 315

Obstructive shock

Answer 315

Obstruction to flow from heart Low CO, high SVR Low CO despite NORMAL contractility Tamponade Tension pneumothorax- high pressure in thorax, impairs hearts ability to full even tho heart working normally Massive PE Tx w resolve obstruction

Question 316

Pericarditis

Answer 316

MC pericardial disorder Inflammation of pericardium Immune mediated Can recur after tx

Question 317

Pericarditis clinically

Answer 317

CP - sharp - worse w deep breath (pleuritic) - worse lying flat - improves sitting up/leaning fwd Pericardial friction rub Fever, elevated ESR, fever, white count Diffuse ST elevations in ALL LEADS PR segment depression

Question 318

Pericarditis etiology

Answer 318

Usually idiopathic Viral - classically- coxsackie virus, often after viral URI Bacterial - spread of PNA - complication of surgery - tb Fungal Uremia from renal failure Post MI- fibrinous (days after MI), Dressler’s syndrome (weeks after MI) Autoimmune pericarditis (SLE, RA)

Question 319

Pericarditis treatment

Answer 319

NSAIDS Steroids Colchicine- inhibits WBCs, useful in gout and familial Mediterranean fever, added to NSAIDs to lower risk of recurrence

Question 320

Tamponade

Answer 320

Accumulation of pericardial fluid High pericardial pressure Filling restrictions of cardiac chambers Acute accumulation - bleeding, small amnt of fluid Chronic inflammation - cancer, larger vol of fluid On CXR- water bottle heart

Question 321

Tamponade causes

Answer 321

Cancer Mets Uremia Pericarditis Trauma Tx- drain fluid

Question 322

Tamponade clinically

Answer 322

Becks triad- distant heart sounds, elevated JVP, hypotension Seen in rapidly developing traumatic effusions Severe impairment LV function- LOW CO Slower effusions- pericardium stretches/dilates

Question 323

Pulses paradoxus

Answer 323

Classic in tamponade Normal- systolic BP always falls slightly on inspiration In tamponade- exaggerated fall in SBP Inspiration-> inc VR-> inc RV -> septum bulges into LV -> decreases LV size-> drops CO

Question 324

Pulsus paradoxus in ASTHMA and COPD

Answer 324

Inspiration- Low left side flow caused by pulmonary pressure fluctuation Exaggerated in lung dx- lung disease patients can have high pressure in lungs LARGE drop in left sided flow—> pulsus paradoxus

Question 325

Electrical alternans

Answer 325

In tamponade | Some QRS complexes bigger and smaller because heart is swinging in effusion fluid

Question 326

Constrictive pericarditis

Answer 326

Fibrous, CALCIFICATION scar in pericardium Loss of elasticity: stiff, thickened, sticky Can result from: - pericarditis - chest radiation - heart surgery

Question 327

Constrictive pericarditis clinically

Answer 327

``` Dyspnea Prominent RHF - elevated JVP - LE edema - liver congestion (nutmeg liver- rare cause of cirrhosis) ```

Question 328

Kussmaul sign

Answer 328

Classic finding in pericardial constriction - normal- inspiration causes increase in VR - slight fall in JVP Kussmaul sign- HIGH JVP WITH INSPIRATION - ventricle cannot accept increases VR Bc RV surrounded by calcifies sack and cannot expand to accept blood - constrictive pericarditis - restrictive cardiomyopathy - RV MI NOT IN TAMPONADE

Question 329

Pericardium innervation

Answer 329

Phrenic nerve Pericarditis- referred pain to shoulder (shared nerves)

Question 330

0-4h post MI

Answer 330

No changes

Question 331

4-12h post MI

Answer 331

``` Necrosis Edema Hemorrhage Wavy fibers Neutrophils Dark mottling gross ```

Question 332

12-24h post MI

Answer 332

Tissue inflammation

Question 333

1-3 d post MI

Answer 333

Hyperemia Necrosis Acute inflammation Neutrophils

Question 334

3 to 14d post MI

Answer 334

Macrophages and granulation tissue Central yellow brown softening

Question 335

2 weeks to mos after MI

Answer 335

Contracted scar complete Grey white scar

Question 336

Restrictive heart disease

Answer 336

Something infiltrates the myocardium - granulomas (sarcoidosis) - amyloid (amyloidosis) Heart cannot Relax and full SEVERE DIASTOLIC DYSFUNCTION

Question 337

Restrictive HD

Answer 337

Normal LVEF Normal left ventricular volume Restricted filling- high atrial pressure-> dilates L and R atria Classic imaging finding- BL ATRIAL ENLARGEMENT Normal LV function/size

Question 338

Restrictive heart disease clinically

Answer 338

Dyspnea Prominent RHF- RV very sensitive to problems with impaired filling - elevated JVP - LE edema - liver congestion (may lead to cirrhosis, nutmeg liver)

Question 339

Restrictive HD causes (Puppy LEASH)

Answer 339

``` Post radiation fibrosis Loeffler endocarditis Endocardial fibroelastosis Amyloidosis Sarcoidosis Hemochromatosis ```

Question 340

Loeffler syndrome

Answer 340

Hyper eosinophilic syndrome Eosinophilic infiltration of organs Eczema Lung fibrosis

Question 341

Eosinophils infiltrate which heart layer

Answer 341

ENDOMYOCARDIAL- interface between myocardium and blood | - thrombus formation can occur

Question 342

Endocardial fibroelastosis

Answer 342

Endocardial thickening (innermost myocardium) Infants 1st year of life PROLIFERATION OF FIBROUS (COLLAGEN) AND ELASTIC FIBERS

Question 343

Pulmonary trunk position in relation to aorta

Answer 343

Pulmonary trunk is ANTERIOR | Aorta POSTERIOR

Question 344

Aortic dissection

Answer 344

Tear in intima (directly next to blood) Blood “dissects” intima and media

Question 345

Type A dissection

Answer 345

Involves ascending aorta/arch TREATED SURGICALLY

Question 346

Type B dissection

Answer 346

Descending aorta Treated medically by controlling HTN/ symptoms

Question 347

Recurrent laryngeal nerve compression

Answer 347

Aortic dissection | LA enlargement

Question 348

AD s/s

Answer 348

Tearing CP Blood pressure differential between arms Widened mediastinum on CXR

Question 349

Medial layer of aorta

Answer 349

Tensile strength and elasticity Key proteins- COLLAGEN and ELASTIN Weakness of medial layer- dissection/aneurysms Common aneurysm feature- medial damage/destruction

Question 350

Vasa vasorum

Answer 350

Network of small vessels in adventitial layer Supplies blood to medial layer in thick vessels (aorta) Thickening (HTN)-> weakening of medial layer

Question 351

Cystic medial necrosis

Answer 351

Devo of cysts and necrosis in medial layer Occurs with age, BICUSPID AORTIC VALVE, MARFAN SYNDROME Common in ASCENDING THORACIC ANEURYSMS

Question 352

Aortic dissection RF

Answer 352

1. Aortic damage - HTN 2. Abnormal collagen - marfan, ehlers danlos 3. Others- bicuspid aortic valve (Turner syndrome), tertiary syphilis (aortitis)

Question 353

Aortic aneurysm

Answer 353

Dilation/bulge of aorta - risk of rupture True aneurysms - all layers involved AAA more common

Question 354

TAA

Answer 354

RF for dissection Assoc with CYSTIC MEDIAL DEGENERATION Usually in proximal/ascending aorta Usually in association with another dx: Marfan, Turner, bicuspid aortic valve, SYPHILIS Aortic root dilation May lead to AORTIC REGURGITATION

Question 355

AAA

Answer 355

More common ASSOC W ATHERSCLEROSIS INFRARENAL AORTA MOST AFFECTED AND MC SITE SMOKING STRONGEST RF, HTN, hyperlipidemia 10X MC IN MEN, 65 yo+ Pulsatile mass

Question 356

Aortic rupture

Answer 356

Dt trauma OR DECELERATION INJURY Occurs in ISTHMUS- transition from proximal arch to rigid descending aorta

Question 357

Hypertrophic cardiomyopathy

Answer 357

Aka HOCM, IHSS AD- variable expression Can be sporadic Mutations in CARDIAC SARCOMERE PROTEINS - BETA MYOSIN HEAVY CHAIN - MYOSIN BINDING PROTEIN SINGLE POINT MISSENSE MUTATION Myofibrillar disarray and fibrosis

Question 358

HCM

Answer 358

1. Arrhythmias- thick myocardium vulnerable to arrhythmias, V TACH- SUDDEN CARDIAC DEATH Exercise(catecholamines) increases SCD risk, avoid exercise 2. Outflow obstruction - outflow tract obstructed by thickened myocardium - HF, CP, exercise induced syncope Tx w beta blockers (decrease contractility) CCBs (verapamil) 3. Mitral valve problem- systolic anterior motion (SAM) of mitral valve, leads to mitral regurgitation

Question 359

HCM murmur

Answer 359

Systolic ejection murmurs (occurs between S1 and S2, crescendo descrescendo) Caused by outflow tract obstruction Sounds like AS unless do maneuvers S4, holosystolic murmur of MR, paradoxical split of S2

Question 360

HCM and Valsalva and standing

Answer 360

Increase thoracic pressure-> compress veins-> lower venous return Less VR, less preload, smaller LV cavity, obstructing septum moves further into outflow tract Murmur INCREASES With Valsalva and standing

Question 361

HCM and squatting and raising the legs

Answer 361

Forces blood volume speed in legs to return to heart Preload roses, LV size increases, less obstruction Murmur DECREASES with squatting and leg raising

Question 362

Aortic stenosis vs HCM

Answer 362

Both cause systolic ejection murmur AS- fixed obstruction, less preload-> less flow->quieter AS murmur

Question 363

HCM associations

Answer 363

Maternal diabetes- infants will have transient HCM Friedrich Ataxia- AR CNS dx, spinocerebellar syndrome, concentric LVH, septal hypertrophy

Question 364

Athletes heart

Answer 364

Combo of Endurance and strength training Increase in LV size (eccentric hypertrophy) Increase in wall thickness (concentric hypertrophy) LVEF IS NORMAL

Question 365

Fabry disease

Answer 365

Lysosomal storage dx Deficiency of alpha galactosidase a Neuropathy, skin lesions, lack of sweat LVH

Question 366

Endocarditis FROM JANE

Answer 366

Fever Roth spots-retinal lesions, red w pale center Osler nodes- PAINFUL bumps on finger/toe pads Murmur Janeway lesions- painless red macular on palms and soles Anemia Nail bed hemorrhages (splinter hemorrhages) Emboli

Question 367

Staph aureus endocarditis

Answer 367

Tricuspid valve in IVDA Acute form- patients get sick very quickly, rapid infection Can infect patients with NORMAL HEART VALVES

Question 368

Viridans streptococcus endocarditis

Answer 368

Mouth flora Endocarditis AFTER DENTAL PROCEDURE Infect DAMAGED heart valves Viridans synthesize DEXTRAN, dextran adheres to fibrin (fibrin found with endothelial damage) Classic predisposing condition- MVP SUBACUTE ENDOCARDITIS- slow onset of symptoms

Question 369

Enterococcus endocarditis

Answer 369

Normal gut flora Subacute endocarditis ASSOC WITH GI/GU MANIPULATION - abd surgery - urinary catheter - TURP for BPH tx Common in older men

Question 370

Prosthetic valve endocarditis

Answer 370

Occurs with mechanical or biologic valve STAPH EPIDERMIDIS Rarely cured w abx, requires repeat surgery b S epidermidis causes infection of prosthetic material

Question 371

Culture negative endocarditis

Answer 371

Endocarditis with negative blood cultures Caused by rare, hard to culture orgs 1. Coxiella burnetii 2. Bartonella

Question 372

Coxiella burnetii

Answer 372

Zoonotic infection (transferred from animals) Obligate intracellular FARM ANIMALS- cattle, sheep, goats Infects animal placenta Humans inhale aerosolized bacteria from animals Causes Q fever, culture negative endocarditis

Question 373

Q fever

Answer 373

Coxiella burnetii Chronic Q fever- endocarditis Acute Q fever-flu like illness, asymptomatic

Question 374

Bartonella Quintana and henslae

Answer 374

GN Quintana- Transmitted by LICE Patients with poor hygiene Henslae- found in cats, causes cat scratch fever

Question 375

Non bacterial thrombotic endocarditis aka liebman sacks endocarditis aka marantic endocarditis

Answer 375

Lesions found on BOTH SIDES OF VALVE Mitral valve MC Formed by thrombus, immune complexes Seen in hypercoaguable states- malignancy, SLE

Question 376

S aureus and mecA gene

Answer 376

In MRSA, mecA gene allows resistance to beta lactam abx mecA produces PBP2a- has low affinity for beta lactams and continues to cross link peptidoglycan Treat MRSA WITH NON BETA LACTAM (TMP-SMX, clindamycin, dox, vancomycin)

Question 377

Molluscum cantagiosum

Answer 377

Poxvirus Firm, flesh colored, shiny, dome shaped with central indentation papules Autoinnoculation, sexual transmission Can be itchy Intracytoplasmic eosinophilic inclusion bodies

Question 378

Medical therapy for aortic dissection

Answer 378

Aimed at reducing aortic wall shear stress to limit extension of dissection Give anti impulse therapy to decrease rate of change in aortic blood pressure GIVE ESMOLOL

Question 379

Esmolol

Answer 379

Beta 1 block, five for medical management of aortic dissection Short half life Reduces shear stress by: 1. Neg inotropy decreases LV contraction velocity 2. Negative chronotropy decreases HR

Question 380

Digoxin MOA

Answer 380

1. Increases parasympathetic vagal tone | 2. Positive inotrope via inhibition of NaK ATPase pump

Question 381

Fibrate MOA (gemfibrozil, fenofibrate)

Answer 381

Activate peroxisome proliferator activates receptor alpha (PPAR) which leads to decreases liver VLDL production and increased LPL activity

Question 382

Lipoprotein lipase (LPL)

Answer 382

Hydrolyzes triglycerides in chylomicrons and VLDL to release FFAs- used for E or converted back to TGs for storage in adipose tissue

Question 383

Cystic medial degeneration

Answer 383

Myxomatous changes with pooling mucopolysaccharides in media layer Predisposes patients to development of aortic aneurysms Medial degeneration in younger pts dt Marfans

Question 384

Etanercept

Answer 384

TNF a inhibitor Decoy receptor for TNF a - binds TNFa to keep it away from functional TNFa receptors

Question 385

Nitrates anti angina therapy

Answer 385

Venodilation with decrease in LVEDV and wall stress, resulting in decreased myocardial oxygen demand ``` VENODILATION REDUCED PRELOAD (decreased LVEDV and pressure) ```

Question 386

Dobutamine

Answer 386

b1 agonist Increased CO

Question 387

Dopamine

Answer 387

Low dose D1- increased renal perfusion and increased CO High dose- a1 agonist, increased SVR

Question 388

Epinephrine

Answer 388

Low dose- b1 agonist- increased CO HIGH DOSE- a1 agonist- increased SVR

Question 389

Isoproteronol

Answer 389

B1= b2 agonist Lower SVR and increased CO

Question 390

NE

Answer 390

A1=b1 Increased SVR

Question 391

Phenylephrine

Answer 391

A1 Increased SVR

Question 392

Buerger disease

Answer 392

Aka thromboangitis obliterans SEGMENTAL SMALL AND MEDIUM VESSEL VASCULITIS SMOKING ASSOC DISTAL ISCHEMIC ULCERATIONs (fingertips) Limb claudication Inflammatory intraluminal thrombi with vessel wall sparing EXTENDS INTO CONTIGUOUS VEINS AND NERVES

Question 393

Phentolomine

Answer 393

Alpha receptor blocker Antidote to NE extravasation - NE leaks out of IV into tissue causes intense alpha1 mediated vasoconstriction which can cause local tissue necrosis

Question 394

Blood supply to SA node

Answer 394

RCA

Question 395

Rental artery stenosis

Answer 395

Atherosclerotic narrowing of renal artery Fibromuscular dysplasia in F Refractory HTN, abd bruits, AKI after ACEi Shrunken kidney secretes high levels of renin- activates RAAS which results in systemic HTN

Question 396

Diastolic HF

Answer 396

Decreased ventricular compliance Normal LVEF, normal LVEDV, ELEVATED FILLING PRESSURES HTN, obesity, infiltration dx (transthyretin related amyloidosis, sarcoidosis) cause DHF

Question 397

Sotalol

Answer 397

Beta blocker and class III antiarrythmic (K blocking) Side effects- bradycardia, Pro arrhythmia, tdp do prolong QT Used for paroxysmal a fib

Question 398

QT interval

Answer 398

Ventricular depolarization and repolarization

Question 399

AAA risk factors

Answer 399

``` Greater than 60 Smoking HTN MALE Family hx ``` These risk factors lead to chronic transmittal inflammation and ECM degradation within walls of the aorta - weakening and progressive expansion or aortic wall, typically BELOW RENAL ARTERIES

Question 400

Cause of Janeway lesions

Answer 400

Septic embolization from valvular vegetations

Question 401

Cause of Osler nodes

Answer 401

Immune complex deposition in skin

Question 402

B1 necessary for (4)

Answer 402

Dehydrogenase enzymes Alpha ketoacid dehydrogenase (branched chain keto acid dehydrogenase) Alpha ketogluterate dehydrogenase (TCA cycle) Pyruvate dehydrigenase (links glycolysis to TCA) Transkatolase (HMP shunt)

Question 403

B2 req for

Answer 403

Riboflavin FAD, FMN Deficiency- cheilosis and corneal vascularization

Question 404

Niacin (b3) req for

Answer 404

NAD, NADPH DERIVED FROM TRYPTOPHAN SYNTH req B6 and B2 Not found in CORN

Question 405

3 niacin deficiencies

Answer 405

1. Hartnup dx 2. Carcinoid syndrome 3. INH tx

Question 406

B5 req for

Answer 406

Pantothenic acid Necessary for coenzyme A and fatty acid synthase Deficiency- adrenal insufficiency, burning feet

Question 407

B6 req for

Answer 407

Pyridoxine REQ PHOSPHORYLATION TO NECOME ACTIVE ``` Transamination rxns Glycogen phosphorylase Cystathione synthesis Heme synthesis Niacin synthesis Histamine synthesis NEUROTRANSMITTER SYNTHESIS ```

Question 408

B6 deficiency

Answer 408

Sideroblastic anemia, convulsions, peripheral neuropathy

Question 409

B7 biotin req for

Answer 409

Adds 1 C Pyruvate carboxylase (pyruvate to OAA)- gluconeogenesis Acetyl coA carboxylase (acetyl coA to malon coA)- FA synthesis Proprionyl coA carboxylase (propionylcA to methylmalonyl coA)- FA breakdown

Question 410

Thyroid peroxidase

Answer 410

Multifunctional enzyme Occurs in FOLLICULAR CELLS OF THYROID Catalysts 1. oxidation of iodide (I2) 2. organification of iodine into MIT/DIT 3. couples MIT/DIT into T3/T4 TPO abs common in autoimmune thyroid

Question 411

Thyroid hormones

Answer 411

T3 (converted from T4 by 5’iodinase to T3 in periphery) T4 (major product) Thyroglobulin produced by thyroid follicular cells, contains many Tyrosine molecs Proteolysis of T3/T4 from thyroglobulin releases T3/T4 into plasma

Question 412

Wolff Chaikoff effect

Answer 412

Excess iodine temporarily inhibits TPO- less T3/T4 (protective autoregulatory effect) Amiodarone can cause hypothyroidism via Wolff Chaikoff effect Amiodarone also mimics 5’ deiodinase- lower T3, increased TSH in response

Question 413

Thyroxine binding globulin (TBG)

Answer 413

Binds T4 Produced by liver Less TBG, less available T4/T3 to tissues

Question 414

Thyroid hormone effects 6bs

Answer 414

Bonds nuclear receptor, modified gene transcription Brain maturation Bone growth Beta adrenergic effects- INCREASE B1 receptors in heart- high CO, HR, SV (beta blocker alleviate adrenergic symptoms in thyrotoxicosis) Basal metabolic rate via INCREASE NA/K ATPase Blood sugar (increase glycogenolysis and gluconeogenesis) Break down lipids (increase lipolysis) Hypothyroid pt- hyperlipidemia Hyperthyroid pt- hyperglycemia

Question 415

Cretinism

Answer 415

Congenital hypothyroidism Babies may appear normal bc exposed to maternal T3/T4 (crosses placenta) ``` MR Coarse facial features Short stature Umbilical hernia Enlarged tongue ``` Treatable MR

Question 416

TSH

Answer 416

Released by anterior pituitary Binds follicular cells ACTIVATES CAMP/PKA system Increase T3/T4 release Stimulates thyroid cell growth and TG synthesis TSH controlled by TRH (hypothalamus) -high serum T3/T4 exerts NEGATIVE EFFECT on TSH and TRH

Question 417

Calcitonin

Answer 417

Synthesized by parafollicular cells, C CELLS Lowers serum calcium - inhibits osteoclasts- suppresses resorption of bone - inhibits renal resorption of Ca, phosphate - increases Ca in urine

Question 418

Mechanism of hyperlipidemia in hypothyroidism

Answer 418

Downregulation of LDL receptors Normally T3 upregulates LDL receptor gene activation

Question 419

Hypothyroid myopathy

Answer 419

Weakness, cramps, myalgia INCREASED CREATINE KINASE

Question 420

Goiter

Answer 420

Enlarged thyroid High TSH (stimulates thyroid growth) but unable to produce T3/T4 Seen in iodine def (3rd world countries) Seen in Graves dx- thyroid stimulating abs

Question 421

Best initial test for suspected thyroid problem

Answer 421

TSH - tests what the thyroid tissues sees - TSH abnormal before T3/T4

Question 422

Primary thyroid disease

Answer 422

Disorder of thyroid gland TSH opposite thyroid hormone Hypothyroidism - high TSH w low T3/T4 Hyperthyroidism - low TSH w high T3/T4

Question 423

Euthyroid sick syndrome and rT3

Answer 423

Reverse T3 usually parallels T4 Critically ill pts have low TSH and low T3/T4 Can look like central hypothyroidism CHECK rT3- rT3 RISES in critical illness If rT3 low- central hypothyroidism If rT3 high- sick euthyroid syndrome

Question 424

Graves dx

Answer 424

Autoimmune dx Exopthalmos and pretibial myxedema (shins) T lymphocyte activation of fibroblasts - fibroblasts contain TSH receptor -> stimulates -> secret glycosaminoglycans which draw in water- swelling

Question 425

Methimazole and Propylthiouracil

Answer 425

Thionamides Inhibit TPO PTU also inhibits 5’ deiodinase (blunts peripheral T4 to T3 conversion)

Question 426

Thionamides AE

Answer 426

Agranulocytosis - rare drop in WBC - may present as fever, infection after starting drug - WBC improves after stopping drug - aplastic anemia reported

Question 427

Methimazole and pregnancy

Answer 427

CONTRAINDICATED Teratogen PTU used in early pregnancy than switch to methimazole bc PTU hepatotoxic

Question 428

Thyroid storm tx

Answer 428

Propranolol- blocks T4 to T3 conversion Thionamides- methimazole, PTU SSKI(saturated solution of KI) - iodide loaf shuts down T4 production via Wolff Chaikoff effect STEROIDS- reduce T4 to T3 conversion, suppress autoimmune damage, treat potential concomitant adrenal insufficiency

Question 429

Hot thyroid nodule

Answer 429

Takes up I131 Not cancerous lesion

Question 430

Cold nodule

Answer 430

Chance of cancer Often biopsies via FNA

Question 431

Jod basedow phenomenon

Answer 431

Iodine induced hyperthyroidism Occurs in stress of IODINE DEFICIENCY - introduction of iodine, hyperthyroidism Often occurs in pts w toxic adenomas

Question 432

Amiodarone hyperthyroidism

Answer 432

1. In pts with pre existing thyroid dx- graves or multinodular- Amiodarone provides iodine- excess hormone production 2. Destructive thyroiditis- excess release of preformed T3/4, occurs in pts WITHOUT pre existing dx

Question 433

Goitrogen

Answer 433

Substances that inhibit thyroid hormone production MC iodine LITHIUM INHIBITS RELEASE OF THYROID

Question 434

Amiodarone and hypothyroidism

Answer 434

Wolff Chaikoff effect Also mimics T4- inhibits 5’ deiodinase

Question 435

Hashimoto’s thyroiditis

Answer 435

Chronic autoimmune thyroiditis LYMPHOCYTES INFILTRATE thyroid gland - autoimmune dx (T call attacks thyroid, B cell activation) - HLA DR3/5 - massive lymphocytic infiltrate - Hurthle cells- enlarged eosinophilic follicular cells) - abs produced but not involved in pathophys INCREASED RISK OF NON HODGKIN B CELL LYMPHOMA

Question 436

Subacute thyroiditis (de Quervain’s)

Answer 436

GRANULOMATOUS inflammation of thyroid Young F Tender, enlarged thyroid Hyperthyroidism to hypothyroidism Tx w anti inflammatories

Question 437

Reidel’s thyroiditis

Answer 437

Fibroblast activation/proliferation ``` FIBROUS TISSUE (collagen) deposits in thyroid ROCK HARD THYROID, extends beyond thyroid ``` ASSOC WITH IgG4 plasma cells

Question 438

Follicular adenoma

Answer 438

BENIGN proliferation of normal folliclular tissue Completely surrounded by fibrous capsule Followed over time FBA cannot distinguish between adenoma/cancer bc cannot see entire capsule

Question 439

Papillary thyroid cancer

Answer 439

MC type Increased risk with PRIOR RADIATION EXPOSURE Presents as thyroid nodule Tx with surgery and radioactive iodine ablation

Question 440

Papillary carcinoma bx

Answer 440

Psammoma bodies- calcifications with layered pattern Nuclear grooves - dark lines in nucleus Orphan Annie eye nuclei Dx made by NUCLEAR FINDINGS

Question 441

Follicular carcinoma

Answer 441

Breaks thru, INVADES fibrous capsule FNA CANNOT DISTINGUISH BETWEEN ADENOMAS/CANCER Followed over time

Question 442

Medullary carcinoma

Answer 442

Cancer of parafollicular cells, C cells Produces CALCITONIN- used for monitoring AMYLOID DEPOSITS IN THYROID MEN 2A and 2B associated with medullary thyroid ca

Question 443

Anaplastic carcinoma

Answer 443

ELDERLY PTS HIGHLY MALIGNANT- invades local tissues - dysphagia (esophagus) - hoarseness (recurrent laryngeal nerve) - dyspnea (trachea) POOR PROGNOSIS UNDIFFERNETIATED CELLS

Question 444

Cortisol and immune system

Answer 444

Suppresses IS Sequesters B and T cells in spleen/nodes BLOCKS NEUTROPHIL MIGRATION- high peripheral neutrophil count Block histamine release from mast cells, lowers eosinophil counts - basis for steroids as immunosuppressants

Question 445

Cortisol and NFKB

Answer 445

INACTIVATES NFKB - key inflammatory transcription factor - mediates response to TNF a - controls synthesis of inflammatory mediators - COX2, PLA2, lipoxygenase

Question 446

rT3

Answer 446

Inactive form generated from peripheral conversion of T4 PARALLELS T4 ACTIVITY If T4 high- rT3 high If T4 low- rT3 low

Question 447

COPD

Answer 447

Air trapping and hyperinflation Higher baseline lung volumes Increased residual volume Increased fraction of air in lungs that is not involved in respiration

Question 448

Mesothelioma

Answer 448

Malignant mesothelial cells Assoc w asbestos PLEURAL THICKENING ON IMAGING - Lung encased in rind like calcified mass

Question 449

Mesothelioma IHC

Answer 449

Spindle cells CYTOKERATINS CALRETININ

Question 450

Primary adrenal insufficiency

Answer 450

Aka addisons dx Abd pain, weight loss, salt craving, orthostasis, electrolyte disturbance Autoimmune destruction of adrenal cortex- all 3 layers destroyed

Question 451

PAI pathophys

Answer 451

Reduced aldosterone- renal and salt wasting with hypovolemia, hypotension, hyperkalemia Hypovolemia- ADH release- water rentention and hyponatremia Reduced cortisol- decreases circulating epi with compensatory rise in NE

Question 452

Second hand smoke and infants

Answer 452

Increased risk for PNA, asthma, recurrent otitis media Cigarette smoke affects macrophage function and impairs mucociliary clearance

Question 453

Chromogranin and synaptophysin

Answer 453

Stain neuroendocrine cells SCLC, carcinoid tumor

Question 454

Cytokeratin

Answer 454

Epithelial cells Epithelial tumors- squamous cell carcinoma

Question 455

DesMin

Answer 455

Muscle Muscle tumors - rhabdomyosarcoma

Question 456

GFAP

Answer 456

NeuroGlia (astrocytes, Schwann cells, oligodendrocytes) Astrocytoma, glioblastoma

Question 457

Neurofilament

Answer 457

Neurons Neuronal tumors

Question 458

PSA

Answer 458

Prostate epithelium Prostate ca

Question 459

S100

Answer 459

Neural crest cells Melanoma, schwannoma, langerhans histiocytosis

Question 460

TRAP

Answer 460

Tartrate resistant acid phosphatase Hairy cell leukemia

Question 461

ViMENtin

Answer 461

Mesenchymal tissue - fibroblast, endothelial cells, macrophage) Mesenchymal tumors - sarcoma - also endometrial carcinoma, RCC, meningioma

Question 462

Cortisol and glucose

Answer 462

More glucose produced in liver, more gluconeogenesis (more synthesis of glucose 6 phosphatatse, PEPCK) Less glucose taken up peripherally Net result- high serum glucose More glycogen storage in liver- inc glycogen synthase

Question 463

Cortisol and lipolysis

Answer 463

Activates lipolysis in adipocytes - high FFAs, total cholesterol, TGs Stimulate adipocyte growth KEY EFFECT- FAT DEPOSITION

Question 464

Cortisol and insulin resistance

Answer 464

Cortisol enhances effects of glucagon and epi Leads to insulin resistance Long term steroid use- DM

Question 465

Cortisol and muscle, skin, bones

Answer 465

Muscle atrophy Blunted epidermal cell division, less collagen and fibroblast inhibition - thin skin, easy bruising, striae Bones: inhibition of osteoblasts - steroids-> osteopenia and osteoporosis

Question 466

Ketoconazole

Answer 466

Blocks ergosterol synth Inhibits 17,20 lyase- less androstenedione/testosterone KEY SIDE EFFECT- gynecomastia Also blocks 17 alpha hydroxylase, desmolase - BLOCK CORTISOL SYNTH- used to tx Cushing’s syndrome

Question 467

Branchial cysts

Answer 467

Result from incomplete obliteration if 2/3/4 pharyngeal CLEFT Literally placed along the SCM muscle

Question 468

Congenital adrenal hyperplasia

Answer 468

Cortisol making enzyme deficiency syndrome ALL AUTOSOMAL RECESSIVE

Question 469

CAH

Answer 469

NO cortisol cholesterol diverted into either ALDOSTERONE or ANDROGENS HIGH ACTH (since adrenals NOT able to make cortisol) low cortisol-> High ACTH -> adrenal hyperplasia -> increased NON cortisol hormone synthesis

Question 470

Low cortisol s/s

Answer 470

BABY Hypoglycemia- cortisol raises BG level N/V in adults

Question 471

Androgens excess/def

Answer 471

Depends on sex of child (XX/XY) Excess 1 XX- ambiguous genitalia 2 XY - precocious puberty Deficiency 1 XX- normal genitalia 2 XY- female or ambiguous genitalia

Question 472

ACTH effects in CAH

Answer 472

High ACTH can cause SKIN HYPERPIGMENTATION Melanocyte stimulating hormone (MSH) is a common precursor protein in pitutary with ACTH Inc melanin synthesis with increased ACTH synthesis in CAH

Question 473

21 alpha hydroxylase deficiency

Answer 473

LOW CORTISOL LOW ALDOSTERONE HIGH ANDROGENS MC CAUSE Girls- AMBIGUOUS GENITALIA Boys- precocious puberty N/v, volume depletion, HYPERKALEMIA

Question 474

11 beta hydroxylase deficiency

Answer 474

HIGH 11 DEOXYCORTICOSTERONE- HIGH MINERALCORTICOID ACTIVITY- hypertension and hypokalemia OVER PRODUCTION ANDROGENS

Question 475

17 alpha hydroxylase def

Answer 475

High aldosterone- HTN, hypokalemia Low cortisol Low androgens XY- female or ambiguous genitalia, NO uterus/Fallopian tubes (sertoli cells-MIH), undescneded testes XX Normal at birth, primary amenorrhea are puberty, theca cells lack androgens- low estradiol Often dx at puberty

Question 476

3 beta hydroxylase steroid dehydrogenase deficiency

Answer 476

NO ALDOSTERONE, cortisol, androgens

Question 477

Elevated 17 hydroxyprogesterone seen in

Answer 477

21 alpha hydroxylase deficiency MC CAH

Question 478

Cushing syndrome

Answer 478

Clinical features dt excess cortisol MC cause- corticosteroid medication- prescribed for anti inflamm

Question 479

Cushing disease

Answer 479

Pituitary ACTH Secreting tumor One cause of Cushing syndrome

Question 480

Cortisol and GnRH

Answer 480

Low FSH and LH Menstrual irregularities in F Hirsutism ED in men

Question 481

ACTH independent Cushings (low ACTH)

Answer 481

Glucocorticoid therapy Adrenal adenoma

Question 482

ACTH dependent ACTH

Answer 482

``` Cushings disease (pituitary ACTH secreting tumor) Ectopic ACTH (SCLC) ``` High ACTH-> adrenal hyperplasia-> high cortisol

Question 483

Ketoconazole

Answer 483

Blocks desmolase- first step in cortisol synthesis Treats Cushings Potent inhibitor of androgen synthesis - side effect- gynecomastia

Question 484

Adrenal insufficiency

Answer 484

Insufficient cortisol production Primary AI (addison’s) - failure of adrenal gland - cortisol and aldosterone low - ACTH HIGH Secondary AI - failure of pituitary ACTH release - ONLY cortisol low

Question 485

Adrenal insufficiency symptoms

Answer 485

Loss of cortisol - POSTURAL HYPOTENSION - N/ V/D - hypoglycemia Loss of aldosterone - hyperkalemia - acidosis - sodium loss in Irwin -> hypovolemia

Question 486

Primary adrenal insufficiency and ACTH

Answer 486

ACTH will be high Leads to skin pigmentation - MSH shares common precursor in pituitary as ACTH - high melanin synthesis

Question 487

Addison’s dx common causes

Answer 487

1. Autoimmune adrenalitis - ab and cell mediated dx - abs to 21 hydroxylase - adrenal gland atrophy - loss of Cortez, medulla soared 2. Infections - Tb - fungal (histoplasmosis, cryptococcus) - CMV

Question 488

Secondary adrenal insufficiency impt pts

Answer 488

1 no skin findings - ACTH not elevated | 2 no hyperkalemia - aldosterone not effected

Question 489

Secondary adrenal insufficiency

Answer 489

Problem in pituitary- not release enough ACTH to maintain cortisol tx MC cause- glucocorticoid therapy - steroids chronically suppress ACTH release from pituitary - leads to adrenal atrophy over time - sudden dc- adrenals can’t compensate quickly enough and produce enough cortisol to maintain cortisol levels

Question 490

Primary aldosteronism

Answer 490

MINERALCORTICOID excess HTN, hypokalemia (weakness, cramps), metabolic alkalosis Dt bl idiopathic hyperaldosteronism or aldosterone producing adenoma High aldosterone, low renin Tx resistant HTN

Question 491

Secondary hyperaldosteronism

Answer 491

seen in pts with renovascular HTN, JG cell tumors (renin producing), edema (HF, cirrhosis, nephrotic syndrome)

Question 492

Spironolactone

Answer 492

Drug of choice in primary aldosteronism K sparing diuretic Blocks aldosterone effects

Question 493

Pheochromocytoma

Answer 493

Catecholamine secreting tumor- secretes dopamine, epi, NE Chromaffin cells of adrenal medulla - derived from NCC Episodic presentation Dx by normetanephrine and metanephrines in urine Tx w phenoxybenzaprine (irrev alpha antag) followed by beta blockers and surgery

Question 494

Neuroblastoma

Answer 494

Tumor of sympathetic ganglion Derived from NCC Adrenal gland MC location Firm, irregular mass that CROSSES midline (vs Wilms tumor) Occurs in CHILDREN- younger onset, better prognosis - abd pain - dx w HVA/VMA urine levels Can synthesize catecholamines, but rarely causes HTN or pheo effects

Question 495

Opsoclonus myoclonis ataxia

Answer 495

Rare paraneoplastoc syndrome Rapid eye movements, rhythmic jerking, ataxia Half of OMA pts have neuroblastoma

Question 496

Neuroblastoma stain and oncogene

Answer 496

Bombesin and NSE+ N myc amplification Assoc with poor prognosis

Question 497

MIBG scan

Answer 497

Metaiodobenzylguanidine - analog of NE - used to dx pheo and neuroblastomas - labeled with radioactive iodine THYROID GLAND MUST BE PROTECTED - admin with KI (non radioactive)- will be taken up by thyroid instead

Question 498

ATP attachment to sarcomere causes

Answer 498

Myosin head to detach from actin filament This resets myosin head to “contract” next time it binds actin

Question 499

Myositis ossificans

Answer 499

Formation of lamellar bone in extra skeletal tissue often triggered by trauma Painful, firm, Mobile mass within muscle - intramuscular calcification - MC in quads and brachialis Benign meta plastic bone and proliferating fibroblasts with no motorized atypia

Question 500

Liposarcoma

Answer 500

Develop in LE muscles | Painless, compress surrounding structures

Question 501

Osteosarcoma

Answer 501

Pleomorphic neoplastic spindle cells and new woven bone Tumor arises in metaphysis and mass not mobile

Question 502

Lupus labs

Answer 502

ANA anti dsDNA Anti smith

Question 503

ACTH dependent Cushing syndrome

Answer 503

Cushing disease or ectopic ACTH syndrome Bl hyperplasia involving sons fasciculata and reticularis Zona glomerulosa primarily regulates by ANGII

Question 504

SGLT2 inhibitors (empagliflozin)

Answer 504

Decrease transcription of glucose and Na in PCT-> urine glucose wasting and lowered bs Also increases Na delivery to macula dens-> decreases renin secretion, lower glomerular pressures, reduced hyperfiltration, and delayed nephropathy

Question 505

First order kinetics

Answer 505

Fixed PROPORTION of drug metabolized

Question 506

Zero order kinetics

Answer 506

Constant amount of drug is metabolized regardless of concentration or dose

Question 507

17 alpha hydroxylase def

Answer 507

Impaired synthesis of androgens, estrogens, and cortisol Normal mineralcorticoid production Boys appear phenotypically female at birth - hypogonadism - HTN - hypokalemia

Question 508

SCD

Answer 508

Abrupt cessation of organized cardiac activity leading to hemodynamics collapse and inadequate tissue perfusion ) MC cause by MI dt malignant ventricular arrhythmia (v fib, v tach) that’s triggered by electrical instability of ischemic myocardium

Question 509

Hypertensive heart disease

Answer 509

Concentric LV hypertrophy, uniformly thickened LV wall Transverse thickening of cardiomyocytes with prominent hyperchromatic nuclei, interstitial fibrosis

Question 510

Dilated cardiomyopathy

Answer 510

Systolic dysfunction Eccentric hypertrophy- sarcomeres added in SERIES

Question 511

Hypertrophic cardiomyopathy

Answer 511

Diastolic dysfunction Concentric hypertrophy- sarcomeres added in parallel Most cases autosomal dominant- mutation increases myosin binding protein C, beta myosin heavy chain Can be caused by Friedrich ataxia (AR)

Question 512

Fetal effects of maternal DM

Answer 512

Macrosomia, hypoglycemia after birth, fetal beta cell hyperplasia and hyperinsulinemia

Question 513

What determines drug half live?

Answer 513

Clearance Volume of distribution

Question 514

MI with new systolic murmur resolving after revascularization

Answer 514

Mitral regurgitation Dt papillary muscle dysfunction MI causes ischemia of papillary muscle and adjacent LV wall it’s mounted on-> hypokinesis and out displacement of papillary muscle

Question 515

Psuedomonas

Answer 515

MOTILE

Question 516

Nitrates

Answer 516

Metabolized to NO and S nitrothiols in VSMCs-> increased cGMP-> vasodilation LARGE VEINS AFFECTED - increased venous capacity - reduced preload-> decreases LV wall stress and myocardial o2 demand to relieve anginal symptoms

Question 517

Most important RF for intimal tears leading to dissection

Answer 517

HTN

Question 518

Myocardial hibernation

Answer 518

State of chronic myocardial ischemia where myocardial metabolism and function are reduced to match reduction in coronary blood flow Prevents myocardial necrosis Decreased contractility and LV systolic dysfunction

Question 519

Alpha 1 second messenger

Answer 519

Increased IP3

Question 520

Alpha 2

Answer 520

Decreases cAMP

Question 521

Beta1

Answer 521

Increased cAMP

Question 522

Beta 2

Answer 522

Increased cAMP

Question 523

Muscarinic 2

Answer 523

Decreased cAMP

Question 524

Muscarinic 3

Answer 524

Increased IP3

Question 525

Trastuzumab

Answer 525

Mab for HER2 positive breast ca Blocks downstream signaling that promotes cell proliferation and encourages malignant cell apoptosis CARDIOTOXICITY - HER2 has role in minimizing oxidative stress on cardiomyocytes - decrease in myocardial contractility with no myocardial destruction or fibrosis

Question 526

Insulin release

Answer 526

Glucose enters beta cells via GLUT2 Glucose-> G6P via GLUCOKINASE ATP produced-> closure of K channels Depolarization-> VGCC open Ca influx->insulin release from vesicles

Question 527

Insulin receptor

Answer 527

Tyrosine kinase receptor

Question 528

Two insulin downstream paths

Answer 528

1. PIK3 | 2. RAS/MAP kinase

Question 529

PIK3 pathway

Answer 529

Glycogen formation FA synthesis Increase GLUT4 glucose transporter on cell surface

Question 530

Biguanides

Answer 530

Metformin, first line in T2DM Oral Primary effect- lowers hepatic glucose production (inhibits gluconeogenesis) Lowers FFAs Reduced glucose absorption from GIT Reduces glucagon levels Increase insulin sensitivity, NO RISK OF HYPOGLYCEMIA

Question 531

Biguanides AE

Answer 531

GI upset Lactic acidosis - can increase conversion of glucose to lactate - too much-> lactic acidosis - ALMOST ALWAYS OCCURS WITH RENAL DISEASE, liver dx/alcohol abuse, hypoxia, serious acute illness Must hold metformin FOR IV CONTRAST - held in pts w low GFR - held when pts acutely ill

Question 532

Sulfonylureas

Answer 532

Bond sulfonylurea receptor in pancreas CLOSE K CHANNELS IN BETA CELLS - changed RMP, depolarization (Ca influx) HIGH INSULIN RELEASE

Question 533

Sulfonylurea drugs

Answer 533

Chlorpropamide Glyburide, glipizide Gimepiride Last 2 more commonly used

Question 534

Sulfonylurea AE

Answer 534

Hypoglycemia - glucagon levels fall - may occur with exercise or meal skip WEIGHT GAIN

Question 535

Chlorpropamide AE

Answer 535

Flushing with alcohol Inhibits acetaldehyde dehydrogenase (disulfiram) Hyponatremia (increase ADH activity)

Question 536

Meglitinides (repaglinide, nateglinide)

Answer 536

Similar mechanism to sulfonylureas Close K channels in beta cells- increase insulin secretion Give prior to meals, short acting Hypoglycemia side effect NO SULFUR- can give to pts w sulfa allergy

Question 537

TZDs- pioglotazone, rosiglotazone

Answer 537

Decreases insulin resistance Oral Act on PPAR gamma receptors - nuclear receptor - highest level in adipose - modulates gene expression TZD binds PPAR gamma- this complex binds RETINOIDS X RECEPTORS - modifies gene transcription

Question 538

TZD mechanism

Answer 538

GLUT4 transcription upregulated Adiponectin- increase insulin sensitivity Antagonism of TNF alpha insulin resistance

Question 539

TZD AE

Answer 539

Weight gain - may cause proliferation of adipocytes - fluid retention - increased Na retention in nephron Hepatotoxic risk Do not use in advanced heart failure

Question 540

Glucosidase inhibitors Acarbose Miglitol Voglibose

Answer 540

Competitive inhibitors of intestinal alpha glucosidases Slows absorption of glucose Less spike in glucose after meals AE- GI upset

Question 541

Pramlintide

Answer 541

Amylin analogs Amylin protein stored in beta cells- secreted with insulin Suppresses glucagon release Delays gastric emptying Reduced appetite Allows insulin to work more effectively

Question 542

Pramlintide use

Answer 542

Given SQ with meals Given with insulin (type 1 and type 2) HYPOGLYCEMIA can result

Question 543

Incretins

Answer 543

Hormones that increase insulin secretion GIP- produces by K cells of SI, increases insulin secretion GLP1- produces by L cells of SI, stimulates insulin release, blunts glucagon release, slows gastric emptying Incretins explain why oral glucose metabolized faster than IV

Question 544

GLP 1 analogs | Exenatide, liraglutide

Answer 544

Both SQ GI upset

Question 545

DPP4 inhibitors - sitagliptin, linagliptin

Answer 545

DPP4- enzyme expressed on many cells, inhibits release of GIP and GLP1 Inhibit DPP4-> increased GLP1 Oral AE- infections

Question 546

Renal failure and metformin

Answer 546

AVOID Dt lactic acidosis

Question 547

Advanced HF DM drugs to avoid

Answer 547

TZDs(fluid retention) | Metformin(lactic acidosis)

Question 548

Initial test for DM diagnosis

Answer 548

Fasting plasma glucose >126 HbA1c >6.5% Non fasting glucose of >200 in a symptomatic patient

Question 549

MEN1

Answer 549

Primary hyperparathyroidism (adenoma, hyperplasia) Pituitary tumors (prolactin, visual effects) Pancreatic tumors (gastrinomas)

Question 550

Beta1 receptors located

Answer 550

Cardiac tissue Renal JG cells Both Gs-> cAMP

Question 551

Alpha1

Answer 551

Gq Gq alpha subunit activates phospholipase C-> degrades DAG bad IP3 PKC activates by DAG Ca released from SR by IP3

Question 552

Alpha2

Answer 552

Gi

Question 553

Beta1 beta2

Answer 553

Gs Alpha subunit activates adenylyl cyclase Converts ATP to cAMP. Elevated cAMP leads to activation of protein kinase A

Question 554

Vitamin A deficiency clinically

Answer 554

Night blindness Severe eye dryness and corneal ulceration Hyperkeratosis Growth retardation

Question 555

Vitamin A deficiency causes

Answer 555

Insufficient dietary intake Pancreatic insufficiency Cholestatic liver disease/biliary obstruction Intestinal malabsorption (IBD, bariatric surgery)

Question 556

Neurophysins

Answer 556

Involved in posttranslational processing and stabilization of oxytocin and vasopressin within neurosecretory vesicles during transport to posterior pituitary

Question 557

Fidaxomicin

Answer 557

Macrolide related Inhibits sigma unit of RNA polymerase - bactericidal against CDIFF

Question 558

Porcelain gallbladder

Answer 558

Increased risk of adenocarcinoma of gallbladder

Question 559

Posterior pituitary

Answer 559

Derived from neural ectoderm Contains axons and nerve terminals Releases ADH and oxytocin Paraventricular- oxytocin Supraoptic- ADH

Question 560

Anterior pituitary

Answer 560

Outgrowth of oral cavity- RATHKES POUCH ``` Makes 5 hormones: FSH LH ACTH PROLACTIN GROWTH HORMONE ```

Question 561

Prolactin

Answer 561

Regulates milk production in mothers Under inhibitory control from DOPAMINE from hypothalamus Dopamine inhibit lactotrophs via binding D2 receptors Prolactin feedback on hypothalamus-> increased dopamine release-> low prolactin

Question 562

TRH and prolactin

Answer 562

TRH INCREASES PROLACTIN SECRETION TRH elevated in hypothyroidism - hypothyroidism predisposes to hyperprolactinemia

Question 563

Prolactin and pregnancy

Answer 563

Estrogen stimulates prolactin release - stimulates transcription, release from lactotrophs Marked increase in lactotrophs during pregnancy PITUITARY GROWS IN SIZE DURING PREGNANCY Prolactin inhibits GnRH RELEASE - ceases ovulation/menstruation

Question 564

Cabergoline, bromocriptine

Answer 564

Dopamine agonists Used to tx Parkinson’s and prolactinomas Dopamine agonists will inhibit prolactin release via D2 receptors

Question 565

Pituitary adenoma symptoms

Answer 565

HA | Bitemporal hemianopsia- optic chiasm compression

Question 566

Prolactinoma

Answer 566

Mc hormone secreting tumor HA, vision loss F- amenorrhea, fractures (LOW BONE DENSITY from low estrogen) M- loss of libido, impotence Dx- serum prolactin, CNS imaging Tx w bromocriptine, capergoline

Question 567

Hyperprolactinemia in F

Answer 567

Amenorrhea (prolactin inhibits GnRH secretion-> lack of LH, FSH Galactorrhea- high prolactin

Question 568

Prolactinoma in M

Answer 568

Hypogonadotropic hypogonadism - low FSH/LH Decreased libido, impotence, infertility, gynecomastia

Question 569

Dopamine Antagonists

Answer 569

Antipsychotics- haldol, risperidone Antiemetics- metoclopromide BLOCK D2- increased prolactin Side effects- amenorrhea, breast engorgement, galactorrhea, sexual dysfunction Can cause Parkinson’s symptoms

Question 570

Craniopharyngoma

Answer 570

Benign tumor Peds 10 to 14 Symptoms from compression- Hypopituitarism HA, vision problems Behavioral changes - frontal lobe dysfunction DERIVED FROM REMNANTS OF RATHKES POUCH

Question 571

Empty sella syndrome

Answer 571

Enlarged sella turcica partially filled w CSF Rarely compress pituitary - hypopititarism MC IN OBESE HTN WOMAN

Question 572

Pituitary apoplexy

Answer 572

Sudden hemorrhage into pituitary Occurs w pre existing adenoma Pt on blood thinners Severe onset HA Diplolpia (compresses occulomotor nerves) Hypopit- shock from loss of cortisol

Question 573

Growth hormone

Answer 573

Protein hormone Impt for linear height growth Released in pulsatile manner Between pulses- levels can be undetectable

Question 574

Growth hormone stimulates by

Answer 574

GHRH Exercise Sleep

Question 575

Growth hormone inhibited by

Answer 575

Glucose Somatostatin IGF1 (direct and indirect)

Question 576

GH receptor

Answer 576

Membrane bound JAK STAT - activation of cytoplasmic tyrosine kinase - alters gene expression

Question 577

GH and liver

Answer 577

Liver has many GH receptors GH-> liver-> IGF1 secreted IGF1 measured in serum as indicator of GH function

Question 578

Anti insulin hormones

Answer 578

Glucagon Cortisol Epi GH

Question 579

IGF1 Effects

Answer 579

Indirect GH effects via IGF1 Chondrocytes- increased linear growth Muscle- lean muscle mass Increase organ size

Question 580

GH inhibited by

Answer 580

Somatostain Glucose IGF1

Question 581

Acromegaly

Answer 581

GH excess in adults Coarse facial features, hands and feet enlarge DIABETES- GH anti insulin, insulin resistance enlargement of joints and organs- CV disease from HTN, LVH

Question 582

Oral glucose test for GH excess

Answer 582

Glucose should suppress growth hormones Normal- GH falls within 2 hours Post glucose levels high

Question 583

Oxytocin

Answer 583

Produces by praventricular nuclei of hypothalamus Milk release in response to sucking Causes contraction of uterus - pitocin- synthetic oxytocin, induces labor, used to help control post partum bleeding

Question 584

Somatostatin

Answer 584

Inhibits release of many hormones Released by D cells in GIT INHIBITS GH RELEASE Used therapeutically in Acromegaly, carcinoid syndrome, glucagonoma/insulinoma, UGIB

Question 585

Parathyroid hormone

Answer 585

Protein hormone Binds cell surface receptors in bone and kidney Synthesized by CHEIF CELLS in parathyroid gland

Question 586

PTH effects

Answer 586

INCREASE SERUM CA LOWER PLASMA PHOS INCREASE URINE PHOSPHATE

Question 587

PTH regulation

Answer 587

Stimulated by low plasma Ca High plasma phos Low vitamin D Calcium activates calcium sensing receptors (CaSRs)-> lowers PTH

Question 588

Magnesium and PTH

Answer 588

High mag- lower PTH by activating CaSRs Low mag- increase PTH release and increase GI and renal Mag absorption along with Ca

Question 589

Very low Mg and PTH

Answer 589

Very low Mg inhibits PTH release Some Mg needed for normal CaSR function Abnormal function-> suppression of PTH release HYPOCALCEMIA OFTEN SEEN IN SEVERE HYPOMAGNESEMIA

Question 590

Continuous PTH

Answer 590

BONE RESORPTION | - increase serum Ca

Question 591

Low dose once daily PTH admin

Answer 591

Bone formation - increased bone mass TERIPARATIDE- tx of osteoporosis

Question 592

Primary hyperparathyroidism

Answer 592

Overactive gland Inappropriate secretion of PTH NOT dt low calcium Commonly caused by parathyroid adenoma HYPERCALCEMIA (cortical bone breakdown) and phosphaturia HIGH CA AND HIGH PTH

Question 593

1 hyperparathyroidism labs

Answer 593

Urinary calcium HIGH OR NORMAL High PTH-> high Ca urinary reabsorption-> high Ca High serum Ca-> high urinary Ca bc high plasma Ca overwhelms uptake

Question 594

1 hyperparathyroidism s/s

Answer 594

Recurrent kidney stones malignancy Stones, bones, groans, psychiatric overtones

Question 595

Osteitis fibrosa cystica

Answer 595

Classic bone dx of long standing untreated hyperparathyroidism Bone pain and fractures Subperiosteal bone resorption - bones of fingers - irregular/indented edges to bone Brown tumors (osteoclastoma) - collections of giant osteoclasts in bone - appears as black spaces on XR

Question 596

2 hyperparathyroidism | -HIGH PTH, LOW CA

Answer 596

Renal failure patients Chronic low Ca-> increased PTH Results IN RENAL OSTEODYSTROPHY - bone pain(predominant symptom) - fractures (weak bones 2 to chronic high PTH levels) - if severe and untreated, can lead to osteitis fibrosa cystica

Question 597

3 hyperparathyroidism | - VERY HIGH PTH

Answer 597

Consequence of chronic renal failure Chronically low Ca-> chronically high PTH Parathyroid becomes autonomous VERY HIGH PTH LEVELS Req parathyroidectomy

Question 598

Ca and ADH

Answer 598

Dehydration Ca blunts effects of ADH (nephrogenic DI) Polyuria, polydipsia Can lead to renal failure

Question 599

Familial hypocalciuric hypercalcemia

Answer 599

AUTOSOMAL DOMINANT Abnormal Ca sensing receptors (CaSRs) - GPCRs - in KIDNEY and PARATHYROID Pts have higher than normal set point for calcium - NORMAL PTH-> HIGH CALCIUM More renal absorption of calcium, LOW URINARY CALCIUM (key finding)

Question 600

Autoimmune polyendocrine syndrome type 1

Answer 600

AUTOSOMAL RECESSIVE Mutation in autoimmune regulator (AIRE) gene Triad: 1 mucocutaneous candidiasis 2 autoimmune hypoparathyroidism 3 Addison’s disease

Question 601

DiGeorge syndrome (thymic aplasia)

Answer 601

Immunodef syndrome Failure of 3/4 pharyngeal pouch to form

Question 602

DiGeorge syndrome triad

Answer 602

Loss of thymus (loss of T cells, recurrent infections) Loss of parathyroid glands (hypocalcemia, tetany) Congenital heart defects

Question 603

DiGeorge syndrome presentation

Answer 603

Infancy/ childhood Hypocalcemia (hypoparathyroidism) Recurrent infections Congenital heart defect

Question 604

Medullary thyroid carcinoma

Answer 604

Cancer of parafollicular cells (C cells) Produces calcitonin Lowers serum Ca

Question 605

Prophylactic surgery in MEN2 syndromes

Answer 605

Thyroidectomy Risk of thyroid cancer 100% for pts with MEN2

Question 606

Receptor tyrosine kinase ligands

Answer 606

Insulin Growth factors

Question 607

JAK/STAT Path

Answer 607

Janus kinases (JAK) are kinase enzymes JAKS phosphorylate STAT STAT goes to nucleus and transcribes genes

Question 608

JAK/STAT ligands

Answer 608

Cytokines Bone marrow stimulating factors Prolactin and growth hormone

Question 609

JAK2 mutation

Answer 609

Assoc w myeloproliferative disorders Gene for cytoplasmic tyrosine kinase Mutation-> increased tyrosine phosphorylation Progenitor cells- hypersensitivity to cytokines More growth, longer survival

Question 610

cGMP 2nd messenger system

Answer 610

BNP/ANP NO

Question 611

Lipid hormone

Answer 611

Intercellular receptor

Question 612

BNP/ANP/NO receptor

Answer 612

cGMP

Question 613

Insulin, growth factor receptor

Answer 613

Tyrosine kinase

Question 614

Cytokine/bone marrow receptor

Answer 614

JAK/STAT

Question 615

Prolactin, growth hormone

Answer 615

JAK/STAT

Question 616

IP3

Answer 616

ADH (V1 receptor) Histamine (H1) Gastrin Angiotensin II

Question 617

cAMP

Answer 617

Histamine H2 | ADH V2