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dr taher classes HK > CVS 13 > Flashcards

CVS 13 Flashcards

1
Q

Chronic mucocutaneous candidiasis is linked to

A

AIRE gene

needed in T cell maturation (IL 2)

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2
Q

IL2 gamma chain defect

A 
SCID 
Cytosine receptor defect
RAG gene mutation
Adenosine deaminase def
MHC II def
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3
Q

causes of livedo reticularis

A

atheroembolic diseases

after coronary angiography/ angioplasty

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4
Q

small athero embolus can affect which organs

A

Brain —- AM PM
kidney —- PCT medulla and thick asc limb
colon — splenic flexure
liver —- zone III (pericentral)

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5
Q

what is the significance of zone III liver

A

metabolises
alcohol/ CCL4/ drugs/
highest conc of CYP450 here

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6
Q

specific drugs metabolized in zone iii of liver

A

sodium valproate
halothane
rifampin

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7
Q

smooth endo function

A

steroid synthesis
detox —– metabolism drugs (xenobiotics)
glucose 6 phosphatase —– glycogenolysis

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8
Q

which enzyme is def in von gierke and where is it present

A

glucose 6 phosphatase

presnt in smoooth ER

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9
Q

debranching enzyme vs branching enzyme

A

Anderson —- branching

Cori —— debranchin

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10
Q

which part of brain damaged due to ischemia

A
  1. hippocampus

2. purkinje axons ( leaving cerebellum)

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11
Q

any fibers LEAVING the cerebellum are ??

A

INHIBITORY

SCP —- superior cerebellar peduncle

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12
Q

Inferior cerebellar peduncle and Middle cerebellar peduncle

A

ICP —- medulla
MCP —- pons
afferent (excitatory)

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13
Q

mcc of acute messenteric ischemia

A

SMA

sudden onset of pain —– currant jelly stools

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14
Q

chronic mesenteric ischemia

A

post prandial pain

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15
Q

how are the pulses in blue toe sydnrome

A

occurs due to dislodging of cholestrol micro embolus

blocks small vessels so pulse = NORMAL

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16
Q

how are cholestrol crystals seen under microscope

A

needle shaped clefts

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17
Q

mechanism of cylophosphamide

A

alkylating agent bings the guanine N 7

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18
Q

KAWASAKI

A 
<4 yrs asian 
CRASH
Conjunctival injection
Rash involving palms n soles (desquamating)
Adenopathy --- cervical
Strawberry tongue
Hand foot changes + fever
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19
Q

which other condition can present with strawberry tongue

A

scarlet fever
GABS
erythrogenic EXOTOXIN A

sandpaper like rash/ blanching pharyngitis/ strawberry tongue

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20
Q

exotoxin A also produced by?

A

pseudomonas

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21
Q

most common and severe comp of kawasaki

A

coronary artery aneurysm

increase risk of rupture thrombosis

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22
Q

txt for kawasaki

A

aspirin

IV immunoglobulins

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23
Q

when to avoid givin aspirin

A

in viral infections — VZV / influenza

can cause REYES syndrome

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24
Q

how does aspirin cause reyes

A

aspirin metabolites inhibit B oxidation mitochondria

mitoch abnormality —–> fatty liver micro vesicular fatty change / hypoglycemia/ hepatomegaly/ vomiting / coma death

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25
Q

necrosis and inflammation in reyes

A

NO

26
Q

examples of uncoupling agents

A

Aspirin
thermogenin (brown fat in babies behind neck)
2-4 dimitrophenol

27
Q

uncoupling means

A

H+ ions migrate inside the mitoch without using F0—– F1 compartment

28
Q

what is the consequence of hyperammonemia related to alpha keto gluterate

A

consumes alpha KTG
rememeber aKTG can use ammonia to make glutamate and the use more ammonia to make glutamine

KTG is very imp for TCA cycle!!

29
Q

how does hyper ammonemia affect astrocytes

A

increases the level of glutamie in astrocytes
this increases the osmolality in the astrocytes
swollen astrocytes —- leasds to defective defective unloading of glutamine

30
Q

why is glutamine so important?

A

glutamate and aspartate are the excitatory neurotransmitters in the neurons

31
Q

conditions that can lead to increase in NH3

A

defective urea cycle
aspirin REYE syndrome
Liver disease
increase in nitrogen load due to GI Bleed

32
Q

how does hyperammonemia present

A 
CNS depression
astrexis flapping tremor
edema ( cerebral edema)
somnolence vomiting
lethargy coma death
33
Q

txt for hyper ammonemia

A

lactulose — acidification — NH4 made which can be excreted
antibiotics —- kill of the bacteria that makes excess nitrogen ( RIFAXIMIN NEOMYCIN)
phenyl acetate and butyrate —- interace with glycine and glutamine — increases their excretion — this helps restore balance between glutamine (exc) and glycine (inhib)

34
Q

buerger disease

A

thromboangitis obliterans — smoker – <40
segmental vasculitis with vein and nerve involvement

autoamputation 
digital gangrene
superficial phlebitis (nodular)
extremity claudication 
raynauds phenomena
VESSEL  WALL NOT INVOLVED
35
Q

what is BERGER disease

A

IgA nephropathy

36
Q

txt of buerger

A

smoking cessation
drugs-
veriniciline (nicotine partial agonist)
bupropion ( inhibit reupt of norepi and epi)

37
Q

MOA of
buspirone
bupenorphine
buterphenol

A

buspirone —- 5HTA1 partial agon

bupenorphine partial agonist at opiod mu rec`
buterphenol

38
Q

behcet sydrome

A

small vessel —- HLAb51 in trukish and med (TYPE 3)
ORO OCULO GENTIAL synd

oro— apthous ulcer
oculo — uveitis
genital —- ulcers

39
Q

what are the triggers for behcet

A

viral infections — herpes and parvo

immune complex within vessel wall — subside in a month

40
Q

Cutaneous small vessel vasculitis causes

A 
DRUGS
penicillins/cephalo
sulfonamides
phenytoin
allopurinol

VIRUS
hep B C and HIV

41
Q

cutaneous small vessel vascuilitis mannifestations

A

non blanching palpable purpura

LOWER EXTREMITY
small inflammed vessels/ fibrinoid necrosis /IC deposition

42
Q

cutaneous vasculitis time line

A

first 24 hours after taking drugs ——- perivascular inflammatory infiltrate — can see NEUTROPHILS ( fragmented nuclei) —– leukocytoclastic vasculitis

43
Q

churg strauss

A

think IgE and eosinophilic
granulomatous necrotizing vasculitis with eosniophilia
TYPE (I) hypersensitivity —- asthma and allergic sinusitis
IL 5 —– eosinophilia

MPO ANCA / PANCA

44
Q

churg straus mannifestations

A 
skin 
subcutaneous nodules
purpura
neuropathy (mononeuritis multiplex) foot wrist drop
heart ( restrictive cardiomyopathy)
kidney ( pauci immune GN)
45
Q

what is pauci immune GN and where can it be seen

A

negative immunofluorescence
no IgG / no complement C3

MPO (microscopic polyangitis)
Wegner
Churg strauss

46
Q

Microscopic polyangitis

A

MPO ANCA/ P ANCA
NO granuloma**

skin —- palpable purpura
kidneys RPGN
lungs
similar to wegners *

47
Q

wegner ( granulomatosis with polyangitis)

A

Granuloma +ve
Nsopharynx involvment
C ANCA/ PR 3 ANCA —- proteinase 3

otitis media mastoiditis
sinusitis 
nasal septal perforation
cough hemptysis**
hematuria --- RPGN

XRAY — granulomatous nodular densities

48
Q

IgA immune complex vasculitis

A

type III
Henoch schonlein purpura
NON THROMBOCYTOPENIC PURPURA — PLT NORMAL

mcc vasculitis in children 
skin--- extensor purpura (buttocks and legs)
arthralgias
GI bleed --- can lead to itusucception
renal --- igA berger nephropathy
49
Q

subepithelial deposits seen in

A

PSGN (nephritis)

membranous ( nephrotic)

50
Q

subendOthelial deposits

A

DPGN and MPGN

51
Q

common findings in nephritic synd

A 
hematuria 
proteinuria <3.5 g /day
inflammation of basement membrane
HTN 
oliguria 
azotemia
52
Q

types of nephritic syndrome

A 
RAPID
RPGN
Alport
PSGN
IgA
DGPN/MPGN
53
Q

nephrotic syndrome common findings

A

proteinuria >3.5 g
hypoalbuminemia
protein C S and antithrombin III also lost

overall edema and procoagulant state
DVT risk increased
renal vein thrombosis

also r/o infection increased because IMMUNOGLOBULINS are lost

Frothy urine — MALTESE sign

54
Q

where else is maltese sign seen

A

BABESIA

55
Q

acquired type II lipoprotein disorder causes

A

nephrotic syndrome

hypothyroidism

56
Q

how does nephrotic syndrome play a role in aquired type II lipoprotein disorder

A

loss of protein from blood —> low density—> compensation from liver increased VLDL and LDL production

57
Q

hypothyroidism and lipoprotein disorder link?

A

thyroid hormone used to make receptors!!

so LDL receptor in hypoT would be defective

58
Q

two MPO ANCA P ANCA vasculitis

A

MPO and churg strauss

MPO — no granuloma
CHurg —- necrotizing granuloma

59
Q

which vasculitis has wrist foot drop — neuropathy

A

churg strauss

60
Q

whuch vasculitis oftenfollows Upper resp infection in children
strept or parvo

A

HSP linkied to IgA nephropathy

61
Q

basket weave pattern on electron microscopy seen in?

A

alport XLD

cystic medial necrosis of aorta

dr taher classes HK (24 decks)

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