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glomerular disease
glomerular disease
97
Medical
Graduate
03/01/2012

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Term
focal glomeruloneprhtitis
Definition
only few gloemruli abnormal
Term
diffuse gloemrliar nerphtitis
Definition
all glomeruli abnormal
Term
proliferative glomerunerphtitis
Definition
more than 100 nuceil i naffected cgloemruli
Term
membarnous glomerulonepropathy
Definition
thick gbm no proliferatie change
Term
mebranorpoflierative gloemrulonerphtiits
Definition
thick gbm, hypercellualr gloemruli
Term
focal segmental glomeruloscelrosis
Definition
fibrosis involving only asegmetn of involved glomerulus
Term
crescentic glomeruloneprhtitis
Definition
prolfieration of parietal epithealialcelsaorudn gloemrulus
Term
lienar patterns found in
Definition
anti-gbm disease- good pastures -
Term
lumpy bumpy pattern of IF
Definition
indiecates imunocomplxdeposistion in gloemrulus
Term
fusino of podocytes
Definition
presents in all nephrotic syndromes- page 413D
Term
Immunecomplexes pathogenesis
Definition
activate c5a-> attracts neutrophils.
Term
Nephritic sydnroem primary mechanism of damage
Definition
neutrophils.
Term
lwo comeplemetn levels associated with
Definition
diffuse nephritic idsroders
Term
enphrtoic sydnrome classic syptoms
Definition
hypoalbumininemia, 3.5g/24 hours proteinuria, lipiduria, hyperlipidemia, edema
Term
edema of neprhotic sydnrome rpomotes,
Definition
ADH, and aldosterone secretion ,elading to MORE edema
Term
nephrotic sydnroem m.o. injury and effect
Definition
cyotkiens injury to podcytes- leadign to loss of negative charge of GBm and podocyte fusion.
Term
effects of losso fo protein in nephrotic sydnrome
Definition
icnreased caoguability (loss of ATIII),
Hypogamaglobulinemia- due to loss of gamma-globulisn in urine
Term
msot common nephrotic disease inc hildren
Definition
minimal change disease
Term
association of minimal change disease with
Definition
hodkins lymphoma, preceeding URI.
Term
minimal change light microscopy and IF
Definition
look normal , nothing there
Term
minimal change idsease
Definition
fusion of podocytes, b ut NO depostis seen
Term
minimal change disease prognosis
Definition
good,rearely pr ogresses to esrd
Term
FSGs epi
Definition
most common priamry gloemrular disroder leadign ot ESRD. Common among HIV patients, iV heroine use.
Term
IF in fsgs
Definition
granular deposits of IGM and c3
Term
fsgs clincial featuers
Definition
proteinuria, microscopic hematuria, hypertension
Term
fsgs prognosis
Definition
poor, commonly rpogresses to CRF.
Term
membranosu glomeruneprotpathy
Definition
most common cause of nephrotic sydnrome in adults. usually idioapthic.
Term
women with membarnous glomeruneproptahty hsa
Definition
SLE, unti lrpoven othewise
Term
light miscrocopy of membranosu gleomeruo
Definition
thickened membrane , with no incresei cellularity
Term
IF of membarnous
Definition
granular deposits. of igg and c3
Term
EM of membarnosu gloemruloneproapthy
Definition
Em subepithelial deposites engulfed by BM, creating spikes onsilver stain.
Term
m typephospholipae a2 recepytor marker
Definition
marker of idiopathic membarnosu glgeomerunepropathy ONLY the idiopathic form
Term
elements requierd for MN to occur
Definition
antibody dep in subeptihelial location, compelment activaiton, c5b-9 (MAC) formation, and disabled regulatory rpoteins for cell defense.
Term
diabetes mellitus glomerunepropathy
Definition
usually diffuse glomeruloscelrosis sometimes nodular glomerulosclerosis.
Term
nodular gloemrulosclerosis rpevalcnee amogn diabetics
Definition
type 1> type II
Term
diabetic gloemrulosclerosis is usually preceeded by
Definition
diabetic retinopathy- without retinopathy, unlikely that it is of diabetic origin
Term
diabetic gloemrulosclerosis correlates wel wltih
Definition
presence of htn and poor glycemic contorl
Term
pathogenesis of diabetic glomerulosclerosis , proteinuria
Definition
nonenzymatic glycsolation fo gbm increases its permeability ,

NEG also produced the hyalien arteriolsoclerosis- specifically to the efferent arteriole.
Term
damage to capillary endotheliall cells in diabetic glomerular cause
Definition
conversion of glucose to sorbitol inside celsl which is ostmotically active and absorbs water itno cels.
Term
prgoression of diabetic gloemrulosclerosis
Definition
4 stages: clincally silent disease with hyeprifltration and supranormal GFR (due to hyaline arteriloscleroriss). then siletn diseaese with microalbuminuiar, then heavy proteinuira and decline i nGFR.
Term
diabetic mciroangiopathy is due to
Definition
icnreased deposition of type IV collagen.
Term
first labrotory amngiestation fo labroterory diabeteric gloemrulo
Definition
mciroalbuminaruia.
Term
prescription whe microabluminuria is present in diabetic gloemrulo
Definition
ACei nhibitor to coutner ATIII effects on constriciton of efferent arterile
Term
miscroscopic findings of diabetic glomerulo
Definition
efferent haylien arteriolosclerosis, nodular masss in mesangial matrix due ot incresaed type IV colaglen dep
Term
amyloidoses pathogenesis
Definition
soluble proteins aggregate and deposite as insolbule fibrils. initailly starting with a misfoldign event.
Term
primary amyloid caused by
Definition
(AL)light chain rpoduced yb clonal palsma cels.
Term
secondary ayloid cause
Definition
AA . serum amyloid A, porduced yb lvier as aacute phase reactant in resposne to longstanding iflammation.
Term
amyloidosis on light microscopy
Definition
amorohpous hyalien deposits , nodular apeparance- similiar to DM. Green birefringence with congro red .
Term
amyloidosis EM
Definition
see fibrils.
Term
prognosis of amyloidosis
Definition
poor, most dyign of cardiac, renal failure or infeciton.
Term
defnitive diagnosis of renalamylodisosis
Definition
renal biopsy (may be clued in via protein electrophoresis)
Term
pathogenesis of preeclampsia
Definition
(nephrotic) decreased diamter of spira larteries suipplying blood to placenta= impaired trophoblast invasion= placental sichemia and imapired prostaglandin metabolism- intravascular coagulation adn vasoconstriction.
Term
histological changes of preeclampsia
Definition
glomeular endothelial ce lswelling. adn subendothelial deposition of hyalin and fibrin like material.
Term
nephritic syndroem clinical featuers
Definition
hypertension due to salt retension, periorbital puffiness-- salt retention, hematuria, rbc casts.
Term
nephrotic syndrome clinical features
Definition
protienuria more htan 3.5g/24 hours. pitting edema and ascited due to hyopoalubminemia. fatty casts
Term
iga enphrophtary empi
Definition
nephritic,most ocmmon cause of priamry gloemruloneprhitis
Term
iga neprophaty paghtoegenesis
Definition
ifnecitous atnigen bidns to igA- the complex then binds to fibronectum and gets trapepdi nmesangium
Term
LIght micrsocopy igA gloemrulo
Definition
focal prolifertiave areas of glomernproptahy- masangial matrix icnreasen adn ceullarity.
Term
EM igA glomerulo
Definition
dense deposits in mesagnium
Term
IF igA-
Definition
frequent activation of alternative comeplement pathway with c3.
Term
igA gloemrulo clinical
Definition
episocidc bouts of hemautria 9both microscopic or gorsss) usually follwing URI. very slow progression to CRF in half of cases.
Term
henoch schonlein prupra
Definition
IGa gleomrulo association wtih arhtirits, vasculitis, and nonthormcytopenci prupura.
Term
Alports sydnroem e pi
Definition
,nephritic x- linekd ocnodition, mostly in males
Term
alports sydnroem characteristic defect
Definition
mutation of type Iv collagen (alpha 5 chain). with antibodies to type IV colalgen in GBM.
Term
Em of alprot sydnrome***
Definition
irregular thiceknign adn splitting of lamina densai nBM
Term
alport syndroem clnical pciture
Definition
microscopichematuria, proetineuaria and seosniirneural hearing lsos.
Term
post streptococcal glomeruneprhittis time period
Definition
l week to 3 weeks after inefction.
Term
psgn disese type
Definition
nephritic.
Term
PSGN light microscopy
Definition
diffuse prolfierative- icnreases mesnagial and neutorphillic hyeprcelullarity
Term
PSGN
Definition
hemturia following 1-3 weeks streptococal ifnection. more common in chidlren. have some hypoclomplementemia, but usually resovles.
Term
bacterail endocarditis
Definition
most commonly caused by strep or staph. presents with fevere , mailase ,hepatosplenomgaly, may remeslbe PsGN (this sliden ot htat important)
Term
Crygobluinemia def
Definition
(nephritic) imunoglobulinwhich precipiatate in cold and sidssovle with warming of serum. Usually anti IgG antibodies forming immuncomplexes withIgG- leading to MPGN.

Diansois amde via systemic complaints ( there are plenty of extrarenal manifestations that precipitate , years before renal manfiestations), and cryoglobuin levels. (not that important slide)
Term
MPGN epi
Definition
(nephritic) usually young adults, mostly idiopathic. can be seen with hep C
Term
type I mpgn
Definition
subendothelial deposits pronounced cellualr prolif
Term
type II mpgn
Definition
deposits throughout bm and less celluarity.

associated with nephrtic factor- auto Ab anti-C3convertase- sustained activaiton fo C3 resulting in very low levels.
Term
mpgn light light micrsocopy/EM
Definition
mesangial depotis and proliferation. see cytoplasmic extnesions of mesangial cells in between capillary BM. split basement mebrane - tram track effect.
Term
MPGN clincial picture
Definition
typical nephriticpicture that progresses to enprhotic sydnrome and ESRD.
Term
RPGN disease course
Definition
( nephritic) rapi losso f renal function oprogression to ARF quickly and poor prognosis
Term
RPGn hischotlica l characteritic
Definition
proliferative GN with , fibrocelualr changes in Bowmans space.
Term
three tyeps of RPGN distinguished by IF
Definition
granular Immune compl deposition, ribbon like deposition of Anti-GBm antibody, and the last have no immuen ocmpelx dep
Term
RPGN method of diagnosis
Definition
seroanalysis to detemrien whether ANCA, ANti BM autoantibodies, or Immune ocmplexes.

If ANCA psoitive and you see systemic necrotizign arteritis is is polyarterits nodosa.

if you ANCA positive nad you see poulmoanry necrotizing granuloams it isi Wegenrs granuloamtosis.

if Anti GBm autoantiboides postiive and postiive for lung hemmorhage it is good pastures .

If is immune complex postiive and its anti dina ab- it is SLe, if anti strep ab- it is psgn, if cryblobulin positive it is cryoglobulinaema.
Term
RPGN method of diagnosis
Definition
seroanalysis to detemrien whether ANCA, ANti BM autoantibodies, or Immune ocmplexes.

If ANCA psoitive and you see systemic necrotizign arteritis is is polyarterits nodosa.

if you ANCA positive nad you see poulmoanry necrotizing granuloams it isi Wegenrs granuloamtosis.

if Anti GBm autoantiboides postiive and postiive for lung hemmorhage it is good pastures .

If is immune complex postiive and its anti dina ab- it is SLe, if anti strep ab- it is psgn, if cryblobulin positive it is cryoglobulinaema.
Term
ANCA MOA
Definition
pmns are stimualted to release anca antigen by viral ifenciton. ANCA attaches and degrades PMN-resuling in ROS release from PMN- leadingto vascular injury.

DAMGE WITHOTU IMMUEN COMPLX DEP.
Term
calssification of lupus neprhittis
Definition
( nephritic)class I - noram llight microscopy mesangial immuen deposits. class II- classI plus mesagnial expansion and hyeprcellularity.
Class III- focal proliferative, class IV- difuse prolfierative
classs V- irregular bm thickening in combo with III/IV.

class VI- advancd dlomerular sclerosis.
Term
LSe miscroscopailly/Em
Definition
most commolnly difufsep rolfieraitive.
granular immunofloruesece.
Eubendothelial immuen complx deposition.
Term
tyep fo antibodies in SLE
Definition
antidna antibody complxies.
Term
THrombotic microangiopathy mechanism
Definition
damge of vascualr endothelium leadsto palteletaggregation and hemolytic anemia secodnary to shearing of rbcs squeeing through these narrowed vessels.
Term
causes of TMA
Definition
malignat hypertension, autoimune disorders,emdications
Term
tma diese causingi t
Definition
HSU, an d TTP
Term
TTP pathogenesis
Definition
defect in Metalloproteinase ADAMtS-13, leadginto fialure to degrade ultra-large VOn WIlelbrand factor- leads to platelt aggregation, thrombus formation in microcirculation.
Term
HUS variants
Definition
dplus (typical), with diarrhea, and d-, without diarrhea.
Term
typical HUS clincial
Definition
preceded by rpodrome of blodoy diarhea and abd pain due to infeciton wtih salmonella, shigella, and verotoxin producing strains of e. coli.
Term
hUS/ttp miscroscopci apeparnace
Definition
endothelial cel swelling of gloemrulus. wdiend subendothelial cel lspace- with fibirn and lipids- capiallary lumin occlusded with patletl rich thrombi.
Term
hUS/TTP clinical features
Definition
thrombocytopenia, non-immune hemolytic anemia
Term
THIn bM disease clincial features/micsroscopy
Definition
"benign familiarh ematuria".
thign GBMS but normal renal funciton, with persistent microscopic hematuria
Term
two most ocmmo ncauses of chornic gloemrulonephritis
Definition
first RPGN, then FSG
Term
last table , slide is extremley helpful in this lecture
Definition
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