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STEP1 Flashcards > Heme/onc and micro + review > Flashcards

Heme/onc and micro + review Flashcards

1
Q

Hematuria, flank pain, low-grade fever after upper respiratory infection

A

IgA nephropathy

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2
Q

Tay-Sachs vs. Niemann-Pick

A

Niemann-Pick: Hepatosplenomegaly
Lipid laden foamy macrophages

Tay-Sachs: NO hepatosplenomegaly

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3
Q

Nonsense vs. Frameshift

A

Nonsense: adding a stop codon leading to truncated proteins. Only affects protein translation (NOT RNA transcription)

Frameshift: adding or removing a number of bases not divisible by 3
alter the reading frame and thus the mRNA produced

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4
Q

PRPP synthetase mutation

A

Leads to increased production and breakdown of purines thus more uric acid–> GOUT

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5
Q

Pyruvate dehydrogenase deficiency

A

build up of lactate and alanine (via ALT)

tx: lysine and leucine (ketogenic nutrients)

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6
Q

Possible products from pyruvate

A

Alanine
lactate (using NADH)
Oxaloacetate
Acetyl-CoA

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7
Q

Transamination leads to and requires?

A

rxn between aa and alpha kept acid
Leads to transfer of N from alanine to Glutamate
moving NH3 from muscle to liver for urea cycle

Requires Pyridoxine (B6)

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8
Q

Biotin rxns

A

pyruvate carboxylase (pyruvate to oxaloacetate)
Acetyl-CoA carboxylase (acetyl-CoA to malonyl-CoA)
Propionyl-CoA carboxylase (propinyl-CoA to methylmalonyl CoA)

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9
Q

Consequence of high-oxygen affinity Hb

A

trouble releasing O2 in the peripheral tissues–> renal hypoxia–> increased epo synthesis–> erythrocytosis

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10
Q

DNA polymerase I

A

5 to 3 exonuclease activity
removes RNA primer
repair damage

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11
Q

DNA polymerase III exonuclease

A

3 to 5 activity

5 to 3 polymerase

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12
Q

Where does the herpes virus hang out?

A

In the nucleus of cells

thus envelope is made up of nuclear membrane

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13
Q

Desmopressin MOA

A

For hemophilia A and vWbrand dz: increases circulating factor VIII and endothelial secretion of vWF to stop bleeding

For CDI and Nocturnal enuresis: binds V2 receptors–> increase aquaporin channels

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14
Q

Von Recklinghausen

A

NF1

neurofibromas, optic nerve gliomas, lisch nodules (eye), cafe au lait spots

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15
Q

Osler-Weber-Rendu

A

hereditary hemorrhagic telangiectasis

epistaxis, GI bleed or hematuria

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16
Q

Sturge-Weber

A 
STURGE
Sporadic
port wine Stain
Tram track calcifications
Unilateral
Retardation 
Glaucoma
GNAQ
Epilepsy
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17
Q

Cause of extramedullary hematopoiesis

A

Chronic hemolytic anemia (Beta thalassemia)

see erythroid precursors in liver and spleen

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18
Q

skin and subcutaneous tissue necrosis after warfarin

A

protein C deficiency

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19
Q

carcinoid histology

A

neatly arranged cells in trabecular pattern surrounded by a fibrovascular storm

dense core membrane bound neurosecretory granules

+ chromogranin, synaptophysin, CD57

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20
Q

tx for methanol poisoning

A

folic acid

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21
Q

Treatment for thyroid storm

A

Propranolol
PTU
Prednisolone

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22
Q

Relative risk

A

Cohort study

RR= [a/(a+b)]/[c/(c+d)]

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23
Q

Attributable risk

A

difference in risk between exposed and not exposed

AR= [a/a+b] - [c/c+d]

ARP=100*(RR-1/RR)

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24
Q

Relative risk reduction

A

RRR=1-RR

proportion of risk reduction attributable to intervention

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25
Q

Absolute risk reduction (ARR)

A

ARR=[c/c+d] - [a/a+b]

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26
Q

Number needed to treat

A

NNT=1/ARR

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27
Q

Hormone levels in Klinefelter syndrome

A

primary issue: Dysfunctional leydig cells

Decreased T
increased LH and FSH
increased estrogen

small testes and dysgenesis of seminiferous tubules

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28
Q

Nausea and vomiting, headaches, vertical gaze palsy. paplledemia

A

Pineal mass

increased ICP

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29
Q

Signaling pathway of steroid hormones

A
  1. enters cell (lipophilic)
  2. binds to intracellular receptor
  3. hormone-receptor complex then undergoes a conformational change (reveal DNA-binding site)
  4. binds to DNA enhancer elements
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30
Q

Normal sized head and trunk but short limbs

A

Achondroplasia

defect in FGFR3 (AD)

associated with advanced paternal age

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31
Q

woman with bitemporal hemianopsia and amenorrhea

A

Prolactinoma

from lactotroph cells

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32
Q

Glucagon target

A

The liver!!

increases glucose production via glycogenolysis and gluconeogenesis

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33
Q

Glucagonoma

A

Dermatitis (necrolytic migratory erythema), Diabetes, Diarrhea, anemia

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34
Q

excess Vit. D

A

hypercalcemia
muscle weakness, constipation, mental status changes
impaired concentration of urine

bone pain

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35
Q

Where is Urea reabsorbed

A

Mostly in proximal tubule

with ADH also reabsorbed in medullary collecting duct

36
Q

Desmopressin effect on renal clearance of urea

A

decreases clearance

increases reabsorption in medullary collecting duct increasing medullary osmotic gradient and leading to more concentrated urine

37
Q

Pathophys of PAH

A

BMPR2

dysfunctional endothelial and smooth muscle cell proliferation

THEN vascular remodeling, elevated pulmonary vascular resistance–> progressive pulmonary HTN

38
Q

Cardiac drug that lengthens QRS duration in a rate-dependent manner

A

Use-dependent Flecanide (class 1C anti arrhythmic)

39
Q

Why are reticulocytes blue on wright-giemsa stain?

A

residual ribosomal RNA

40
Q

Histology of follicular lymphoma

A

B cells predominantly centrocytes

packed follicles that obscure normal LN architecture

41
Q

Causes of folate deficiency

A

Poor diet (alcoholics and elderly)

Increased demand (hemolytic anemia, pregnancy, cancer)

folate antagonists (methotrexate)

42
Q

Mutation in polycythemia vera

A

JAK2

cytoplasmic tyrosine kinase

43
Q

Li-Fraumeni

A 
mut in p53
Sarcomas
breast
brain
adrenal
44
Q

MOA Enoxaparin

A

Binds ATIII

a LMWH

45
Q

Consequences of Multiple Myeloma

A 
CRAAB
hyperCalcemia
Renal failure
Anemia (normocytic)
AL amyloid 
Bone lytic lesions 
Back pain
46
Q

Trastuzumab

A

inhibits tyrosine kinase
Antibody dependent cytotoxicity

treat HER2+ breast cancer

47
Q

JAK2 mutation

A

Polycythemia vera

makes hematopoietic cells more sensitive to growth factors

48
Q

Microsomal monooxygenase role

A

cytochrome P450 monooxygenase

found in hepatic microsomes
metabolizes steroids, alcohol and toxins making them easier to excrete

can make pro-carcinogens into carcinogens

49
Q

HBV life cycle

A

DNA VIRUS!!

Incorporates into host DNA

can continue to produce HBx protein–> inactivates p53 tumor suppressor gene

50
Q

effect of carbon tetrachloride

A

free radical injury

lipid peroxidation

51
Q

tumor stage

A

extent of expansion

most important for PROGNOSIS

52
Q

Tumor grade

A

degree of tumor differentiation

mitotic figures, aneuploidy and cell differentiation

53
Q

anaplastic tumors

A

loss of structural differentiation and function of cells–primative cells of the same tissue

GIANT CELLS

54
Q

Function of BRCA1 and 2

A

Tumor supressor gene

DNA repair of dsDNA breaks

55
Q

Qualitative plt disorders

A 
Uremic plt dysfunction 
Bernard-soulier syndrome (GpIb)
Glanzmann thrombasthenia (GpIIb/IIIa)
56
Q

localized loss of sensation and positive rhomberg

A

Medial leminscus (goes to VPL from contralateral dorsal column)

57
Q

Treatment for supra ventricular tachyarrhythmia

A

Adenosine
carotid massage
Valsalva maneuver

58
Q

Type IV Renal tubular acidosis

A

Caused by hypoaldo
HYPERkalemia (other types of RTA are hypokalemic)

low urine pH because hyperkalemia inhibits ammonia excretion

59
Q

Painful vesicle on external genitalia with bilateral inguinal adenopathy

A

Chancroid

Haemophilus ducreyi

tx: Ceftriaxone

60
Q

Differential for genital ulcers

A

Primary syphilis (painless)
Herpes (painful)
Chancroid (painful)

61
Q

Baby with abnormal skull and facial features, growth deficiency, underdeveloped nails, and mild developmental delay

A

Fetal hydantoin syndrome

due to phenytoin

62
Q

SE of Phenytoin

A 
Nystagmus 
diplopia
ataxia
gingival hyperplasia
megaloblastic anemia 
Fetal hydantoin syndrome 
steven-johnson
osteopenia
63
Q

Bacitracin MOA

A

Inhibits peptidoglycan synthesis
prevents transfer of mucopeptides into the growing bacterial cell wall

SE:Nephrotoxicity

64
Q

Organs effected by ADPKD

A

LIVER–but usually asymptomatic

Also: cerebral vessels (berry aneurysms)
mitral valve prolapse
cysts in seminal vesicles

65
Q

what artery runs with the recurrent laryngeal nerve?

A

R subclavian a.

66
Q

Matinance dose

A

MD=Cp x CL/F

67
Q

Loading dose

A

LD= Cp x Vd/F

68
Q

Half life

A

t=0.7 x Vd/ CL

69
Q

Tx for metastatic renal cell carcinoma

A

Recombinant IL-2

aldesleukin

70
Q

SE of protease inhibitors

A

Metabolic (lipodystrophy, dyslipidemia, insulin resistance)

71
Q

Painful abdomen, Port-wine colored urine, polyneuropathy, psychological disturbances

A

Acute intermittent porphyria

Enzyme: porphobilinogen deaminase

build up of prophobilinogen, delta-ALA, coporphobilinogen

tx: glucose and heme (inhibits ALA synthesis)

72
Q

TNF alpha

A

mediates septic shock
activates endothelium
causes WBC recruitment
vascular leak

73
Q

what innervates the uvula

A

Vagus nerve! if damage the uvula deviates away from the side of the lesion

also soft palate elevation, swallowing, coughing, talking, aortic arch chemo and baroreceptors

74
Q

Hereditary sphereocytosis treatment

A

splenectomy

75
Q

Isotretinoin

A

Acne treatment

pregnancy category X

other preg X: Warfarin, Methotrexate, danazol (tx for endometriosis), synthetic progestins

76
Q

Risks associated with H. pylori infection

A

Increased risk of duodenal ulcers, gastric carcinoma, B-cell non-Hodgkin gastric lymphoma

MALT

77
Q

Hyperventilation, tinnitus, abdominal pain, n/v, sweating, dizziness after OD

A

Aspirin

tx: Bicarb

78
Q

Function of protein C

A

cleavage of factor Va and VIIIa

requires vitamin K

79
Q

Aschoff bodies

A

Noncaseating granuloma with multinucleate gian cells

80
Q

Amatoxin target

A

RNA polymerase II

stops mRNA synthesis

81
Q

GVH in the liver

A

T cells infiltrate and destroy small intrahepatic bile ducts

increased Alk phos

Looks like Primary biliary cholangitis

82
Q

Myocardial thinning and fibrosis

A

Infiltrative cardiomypoathy (sacroid, amyloid), chagas, myocarditis, prior MI

83
Q

Differential for ring enhancing lesions with mass effect in HIV pts

A

Toxo–most common

Primary central nervous system lymphoma (B-cell origin associated with EBV)

84
Q

Replication of Hep B

A

partially dsDNA–>dsDNA–>+ssRNA–>makes viral proteins and ssDNA–>dsDNA

85
Q

Acid fast staining takes advantage of what?

A

mycolic acid in the cell wall

STEP1 Flashcards (9 decks)

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