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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
necrosis and inflammation in reyes
NO
26
examples of uncoupling agents
Aspirin thermogenin (brown fat in babies behind neck) 2-4 dimitrophenol
27
uncoupling means
H+ ions migrate inside the mitoch without using F0----- F1 compartment
28
what is the consequence of hyperammonemia related to alpha keto gluterate
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
how does hyper ammonemia affect astrocytes
increases the level of glutamie in astrocytes this increases the osmolality in the astrocytes swollen astrocytes ---- leasds to defective defective unloading of glutamine
30
why is glutamine so important?
glutamate and aspartate are the excitatory neurotransmitters in the neurons
31
conditions that can lead to increase in NH3
defective urea cycle aspirin REYE syndrome Liver disease increase in nitrogen load due to GI Bleed
32
how does hyperammonemia present
``` CNS depression astrexis flapping tremor edema ( cerebral edema) somnolence vomiting lethargy coma death ```
33
txt for hyper ammonemia
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
buerger disease
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
what is BERGER disease
IgA nephropathy
36
txt of buerger
smoking cessation drugs- veriniciline (nicotine partial agonist) bupropion ( inhibit reupt of norepi and epi)
37
MOA of buspirone bupenorphine buterphenol
buspirone ---- 5HTA1 partial agon bupenorphine partial agonist at opiod mu rec` buterphenol
38
behcet sydrome
small vessel ---- HLAb51 in trukish and med (TYPE 3) ORO OCULO GENTIAL synd oro--- apthous ulcer oculo --- uveitis genital ---- ulcers
39
what are the triggers for behcet
viral infections --- herpes and parvo immune complex within vessel wall --- subside in a month
40
Cutaneous small vessel vasculitis causes
``` DRUGS penicillins/cephalo sulfonamides phenytoin allopurinol ``` VIRUS hep B C and HIV
41
cutaneous small vessel vascuilitis mannifestations
non blanching palpable purpura LOWER EXTREMITY small inflammed vessels/ fibrinoid necrosis /IC deposition
42
cutaneous vasculitis time line
first 24 hours after taking drugs ------- perivascular inflammatory infiltrate --- can see NEUTROPHILS ( fragmented nuclei) ----- leukocytoclastic vasculitis
43
churg strauss
think IgE and eosinophilic granulomatous necrotizing vasculitis with eosniophilia TYPE (I) hypersensitivity ---- asthma and allergic sinusitis IL 5 ----- eosinophilia MPO ANCA / PANCA
44
churg straus mannifestations
``` skin subcutaneous nodules purpura neuropathy (mononeuritis multiplex) foot wrist drop heart ( restrictive cardiomyopathy) kidney ( pauci immune GN) ```
45
what is pauci immune GN and where can it be seen
negative immunofluorescence no IgG / no complement C3 MPO (microscopic polyangitis) Wegner Churg strauss
46
Microscopic polyangitis
MPO ANCA/ P ANCA NO granuloma**** skin ---- palpable purpura kidneys RPGN lungs similar to wegners *
47
wegner ( granulomatosis with polyangitis)
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
IgA immune complex vasculitis
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
subepithelial deposits seen in
PSGN (nephritis) | membranous ( nephrotic)
50
subendOthelial deposits
DPGN and MPGN
51
common findings in nephritic synd
``` hematuria proteinuria <3.5 g /day inflammation of basement membrane HTN oliguria azotemia ```
52
types of nephritic syndrome
``` RAPID RPGN Alport PSGN IgA DGPN/MPGN ```
53
nephrotic syndrome common findings
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
where else is maltese sign seen
BABESIA
55
acquired type II lipoprotein disorder causes
nephrotic syndrome | hypothyroidism
56
how does nephrotic syndrome play a role in aquired type II lipoprotein disorder
loss of protein from blood ---> low density---> compensation from liver increased VLDL and LDL production
57
hypothyroidism and lipoprotein disorder link?
thyroid hormone used to make receptors!! | so LDL receptor in hypoT would be defective
58
two MPO ANCA P ANCA vasculitis
MPO and churg strauss MPO --- no granuloma CHurg ---- necrotizing granuloma
59
which vasculitis has wrist foot drop --- neuropathy
churg strauss
60
whuch vasculitis oftenfollows Upper resp infection in children strept or parvo
HSP linkied to IgA nephropathy
61
basket weave pattern on electron microscopy seen in?
alport XLD | cystic medial necrosis of aorta

dr taher classes HK (24 decks)

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