CVS 13 Flashcards
Chronic mucocutaneous candidiasis is linked to
AIRE gene
needed in T cell maturation (IL 2)
IL2 gamma chain defect
SCID Cytosine receptor defect RAG gene mutation Adenosine deaminase def MHC II def
causes of livedo reticularis
atheroembolic diseases
after coronary angiography/ angioplasty
small athero embolus can affect which organs
Brain —- AM PM
kidney —- PCT medulla and thick asc limb
colon — splenic flexure
liver —- zone III (pericentral)
what is the significance of zone III liver
metabolises
alcohol/ CCL4/ drugs/
highest conc of CYP450 here
specific drugs metabolized in zone iii of liver
sodium valproate
halothane
rifampin
smooth endo function
steroid synthesis
detox —– metabolism drugs (xenobiotics)
glucose 6 phosphatase —– glycogenolysis
which enzyme is def in von gierke and where is it present
glucose 6 phosphatase
presnt in smoooth ER
debranching enzyme vs branching enzyme
Anderson —- branching
Cori —— debranchin
which part of brain damaged due to ischemia
- hippocampus
2. purkinje axons ( leaving cerebellum)
any fibers LEAVING the cerebellum are ??
INHIBITORY
SCP —- superior cerebellar peduncle
Inferior cerebellar peduncle and Middle cerebellar peduncle
ICP —- medulla
MCP —- pons
afferent (excitatory)
mcc of acute messenteric ischemia
SMA
sudden onset of pain —– currant jelly stools
chronic mesenteric ischemia
post prandial pain
how are the pulses in blue toe sydnrome
occurs due to dislodging of cholestrol micro embolus
blocks small vessels so pulse = NORMAL
how are cholestrol crystals seen under microscope
needle shaped clefts
mechanism of cylophosphamide
alkylating agent bings the guanine N 7
KAWASAKI
<4 yrs asian CRASH Conjunctival injection Rash involving palms n soles (desquamating) Adenopathy --- cervical Strawberry tongue Hand foot changes + fever
which other condition can present with strawberry tongue
scarlet fever
GABS
erythrogenic EXOTOXIN A
sandpaper like rash/ blanching pharyngitis/ strawberry tongue
exotoxin A also produced by?
pseudomonas
most common and severe comp of kawasaki
coronary artery aneurysm
increase risk of rupture thrombosis
txt for kawasaki
aspirin
IV immunoglobulins
when to avoid givin aspirin
in viral infections — VZV / influenza
can cause REYES syndrome
how does aspirin cause reyes
aspirin metabolites inhibit B oxidation mitochondria
mitoch abnormality —–> fatty liver micro vesicular fatty change / hypoglycemia/ hepatomegaly/ vomiting / coma death
necrosis and inflammation in reyes
NO
examples of uncoupling agents
Aspirin
thermogenin (brown fat in babies behind neck)
2-4 dimitrophenol
uncoupling means
H+ ions migrate inside the mitoch without using F0—– F1 compartment
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!!
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
why is glutamine so important?
glutamate and aspartate are the excitatory neurotransmitters in the neurons
conditions that can lead to increase in NH3
defective urea cycle
aspirin REYE syndrome
Liver disease
increase in nitrogen load due to GI Bleed
how does hyperammonemia present
CNS depression astrexis flapping tremor edema ( cerebral edema) somnolence vomiting lethargy coma death
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)
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
what is BERGER disease
IgA nephropathy
txt of buerger
smoking cessation
drugs-
veriniciline (nicotine partial agonist)
bupropion ( inhibit reupt of norepi and epi)
MOA of
buspirone
bupenorphine
buterphenol
buspirone —- 5HTA1 partial agon
bupenorphine partial agonist at opiod mu rec`
buterphenol
behcet sydrome
small vessel —- HLAb51 in trukish and med (TYPE 3)
ORO OCULO GENTIAL synd
oro— apthous ulcer
oculo — uveitis
genital —- ulcers
what are the triggers for behcet
viral infections — herpes and parvo
immune complex within vessel wall — subside in a month
Cutaneous small vessel vasculitis causes
DRUGS penicillins/cephalo sulfonamides phenytoin allopurinol
VIRUS
hep B C and HIV
cutaneous small vessel vascuilitis mannifestations
non blanching palpable purpura
LOWER EXTREMITY
small inflammed vessels/ fibrinoid necrosis /IC deposition
cutaneous vasculitis time line
first 24 hours after taking drugs ——- perivascular inflammatory infiltrate — can see NEUTROPHILS ( fragmented nuclei) —– leukocytoclastic vasculitis
churg strauss
think IgE and eosinophilic
granulomatous necrotizing vasculitis with eosniophilia
TYPE (I) hypersensitivity —- asthma and allergic sinusitis
IL 5 —– eosinophilia
MPO ANCA / PANCA
churg straus mannifestations
skin subcutaneous nodules purpura neuropathy (mononeuritis multiplex) foot wrist drop heart ( restrictive cardiomyopathy) kidney ( pauci immune GN)
what is pauci immune GN and where can it be seen
negative immunofluorescence
no IgG / no complement C3
MPO (microscopic polyangitis)
Wegner
Churg strauss
Microscopic polyangitis
MPO ANCA/ P ANCA
NO granuloma**
skin —- palpable purpura
kidneys RPGN
lungs
similar to wegners *
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
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
subepithelial deposits seen in
PSGN (nephritis)
membranous ( nephrotic)
subendOthelial deposits
DPGN and MPGN
common findings in nephritic synd
hematuria proteinuria <3.5 g /day inflammation of basement membrane HTN oliguria azotemia
types of nephritic syndrome
RAPID RPGN Alport PSGN IgA DGPN/MPGN
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
where else is maltese sign seen
BABESIA
acquired type II lipoprotein disorder causes
nephrotic syndrome
hypothyroidism
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
hypothyroidism and lipoprotein disorder link?
thyroid hormone used to make receptors!!
so LDL receptor in hypoT would be defective
two MPO ANCA P ANCA vasculitis
MPO and churg strauss
MPO — no granuloma
CHurg —- necrotizing granuloma
which vasculitis has wrist foot drop — neuropathy
churg strauss
whuch vasculitis oftenfollows Upper resp infection in children
strept or parvo
HSP linkied to IgA nephropathy
basket weave pattern on electron microscopy seen in?
alport XLD
cystic medial necrosis of aorta
