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

Heme/onc and micro + review Flashcards

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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
Absolute risk reduction (ARR)
ARR=[c/c+d] - [a/a+b]
26
Number needed to treat
NNT=1/ARR
27
Hormone levels in Klinefelter syndrome
primary issue: Dysfunctional leydig cells Decreased T increased LH and FSH increased estrogen small testes and dysgenesis of seminiferous tubules
28
Nausea and vomiting, headaches, vertical gaze palsy. paplledemia
Pineal mass | increased ICP
29
Signaling pathway of steroid hormones
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
30
Normal sized head and trunk but short limbs
Achondroplasia defect in FGFR3 (AD) associated with advanced paternal age
31
woman with bitemporal hemianopsia and amenorrhea
Prolactinoma from lactotroph cells
32
Glucagon target
The liver!! | increases glucose production via glycogenolysis and gluconeogenesis
33
Glucagonoma
Dermatitis (necrolytic migratory erythema), Diabetes, Diarrhea, anemia
34
excess Vit. D
hypercalcemia muscle weakness, constipation, mental status changes impaired concentration of urine bone pain
35
Where is Urea reabsorbed
Mostly in proximal tubule with ADH also reabsorbed in medullary collecting duct
36
Desmopressin effect on renal clearance of urea
decreases clearance increases reabsorption in medullary collecting duct increasing medullary osmotic gradient and leading to more concentrated urine
37
Pathophys of PAH
BMPR2 dysfunctional endothelial and smooth muscle cell proliferation THEN vascular remodeling, elevated pulmonary vascular resistance--> progressive pulmonary HTN
38
Cardiac drug that lengthens QRS duration in a rate-dependent manner
Use-dependent Flecanide (class 1C anti arrhythmic)
39
Why are reticulocytes blue on wright-giemsa stain?
residual ribosomal RNA
40
Histology of follicular lymphoma
B cells predominantly centrocytes packed follicles that obscure normal LN architecture
41
Causes of folate deficiency
Poor diet (alcoholics and elderly) Increased demand (hemolytic anemia, pregnancy, cancer) folate antagonists (methotrexate)
42
Mutation in polycythemia vera
JAK2 | cytoplasmic tyrosine kinase
43
Li-Fraumeni
``` mut in p53 Sarcomas breast brain adrenal ```
44
MOA Enoxaparin
Binds ATIII a LMWH
45
Consequences of Multiple Myeloma
``` CRAAB hyperCalcemia Renal failure Anemia (normocytic) AL amyloid Bone lytic lesions Back pain ```
46
Trastuzumab
inhibits tyrosine kinase Antibody dependent cytotoxicity treat HER2+ breast cancer
47
JAK2 mutation
Polycythemia vera | makes hematopoietic cells more sensitive to growth factors
48
Microsomal monooxygenase role
cytochrome P450 monooxygenase found in hepatic microsomes metabolizes steroids, alcohol and toxins making them easier to excrete can make pro-carcinogens into carcinogens
49
HBV life cycle
DNA VIRUS!! Incorporates into host DNA can continue to produce HBx protein--> inactivates p53 tumor suppressor gene
50
effect of carbon tetrachloride
free radical injury lipid peroxidation
51
tumor stage
extent of expansion most important for PROGNOSIS
52
Tumor grade
degree of tumor differentiation | mitotic figures, aneuploidy and cell differentiation
53
anaplastic tumors
loss of structural differentiation and function of cells--primative cells of the same tissue GIANT CELLS
54
Function of BRCA1 and 2
Tumor supressor gene DNA repair of dsDNA breaks
55
Qualitative plt disorders
``` Uremic plt dysfunction Bernard-soulier syndrome (GpIb) Glanzmann thrombasthenia (GpIIb/IIIa) ```
56
localized loss of sensation and positive rhomberg
Medial leminscus (goes to VPL from contralateral dorsal column)
57
Treatment for supra ventricular tachyarrhythmia
Adenosine carotid massage Valsalva maneuver
58
Type IV Renal tubular acidosis
Caused by hypoaldo HYPERkalemia (other types of RTA are hypokalemic) low urine pH because hyperkalemia inhibits ammonia excretion
59
Painful vesicle on external genitalia with bilateral inguinal adenopathy
Chancroid Haemophilus ducreyi tx: Ceftriaxone
60
Differential for genital ulcers
Primary syphilis (painless) Herpes (painful) Chancroid (painful)
61
Baby with abnormal skull and facial features, growth deficiency, underdeveloped nails, and mild developmental delay
Fetal hydantoin syndrome due to phenytoin
62
SE of Phenytoin
``` Nystagmus diplopia ataxia gingival hyperplasia megaloblastic anemia Fetal hydantoin syndrome steven-johnson osteopenia ```
63
Bacitracin MOA
Inhibits peptidoglycan synthesis prevents transfer of mucopeptides into the growing bacterial cell wall SE:Nephrotoxicity
64
Organs effected by ADPKD
LIVER--but usually asymptomatic Also: cerebral vessels (berry aneurysms) mitral valve prolapse cysts in seminal vesicles
65
what artery runs with the recurrent laryngeal nerve?
R subclavian a.
66
Matinance dose
MD=Cp x CL/F
67
Loading dose
LD= Cp x Vd/F
68
Half life
t=0.7 x Vd/ CL
69
Tx for metastatic renal cell carcinoma
Recombinant IL-2 | aldesleukin
70
SE of protease inhibitors
Metabolic (lipodystrophy, dyslipidemia, insulin resistance)
71
Painful abdomen, Port-wine colored urine, polyneuropathy, psychological disturbances
Acute intermittent porphyria Enzyme: porphobilinogen deaminase build up of prophobilinogen, delta-ALA, coporphobilinogen tx: glucose and heme (inhibits ALA synthesis)
72
TNF alpha
mediates septic shock activates endothelium causes WBC recruitment vascular leak
73
what innervates the uvula
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
Hereditary sphereocytosis treatment
splenectomy
75
Isotretinoin
Acne treatment pregnancy category X other preg X: Warfarin, Methotrexate, danazol (tx for endometriosis), synthetic progestins
76
Risks associated with H. pylori infection
Increased risk of duodenal ulcers, gastric carcinoma, B-cell non-Hodgkin gastric lymphoma MALT
77
Hyperventilation, tinnitus, abdominal pain, n/v, sweating, dizziness after OD
Aspirin tx: Bicarb
78
Function of protein C
cleavage of factor Va and VIIIa requires vitamin K
79
Aschoff bodies
Noncaseating granuloma with multinucleate gian cells
80
Amatoxin target
RNA polymerase II stops mRNA synthesis
81
GVH in the liver
T cells infiltrate and destroy small intrahepatic bile ducts increased Alk phos Looks like Primary biliary cholangitis
82
Myocardial thinning and fibrosis
Infiltrative cardiomypoathy (sacroid, amyloid), chagas, myocarditis, prior MI
83
Differential for ring enhancing lesions with mass effect in HIV pts
Toxo--most common Primary central nervous system lymphoma (B-cell origin associated with EBV)
84
Replication of Hep B
partially dsDNA-->dsDNA-->+ssRNA-->makes viral proteins and ssDNA-->dsDNA
85
Acid fast staining takes advantage of what?
mycolic acid in the cell wall

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