nephritics and nephrotics Flashcards
acute post streptococcal golmerulonephitis
nephritic
LM - glomeruli diffuse, enlarge, and hypercellular
if - “starry sky” granular appearence (lumpy bumpy) due to igg igm and c3 deposition along the gmb and mesangium. proliferation of the mesangium.
em - subepithelial humps due to immne complexes
most frequently seen in children. occurs 2-4 weeks after group a nephritognic infection of skin or pharynx.
resolves by itsef
type 3 hyper
presents with peripheral and periorbial edema, cola colored urine, hpertension and increased creatinine.
positive strep titiers/serolgies (increased ASO and increased ab dnaase). decreased compliment (c3)
PMNs, adults have worse prognosis. they can develop renal failure later, or they can progress to RPGN
diffuse proliferative glomerlonephritis
nephritic/nephrotic
often due to sle or membranoproliferative glomeruloneprhitis
lm - wire looping of cappilaries
em - sunednothelial and sometimes intramembranous igg based ICs often with c3. anti-dsdna complexes. full house igg iga igm c3 c1q
if - granular
common cause of death in sle
both DPGN and MPGN can often co present as both neprhi\rtic and nephrotic syndrome
most common lupus disease
- worst form, often leads to DPGN and renal failure
decreased compliment
iga nephropathy (berger disease)
nephritic
lm - mesangial proliferation
em - mesangial IC complexes
if - iga-based IC deposits in mesangium.
renal pathology of henoch-schonlein purpura
episodic gross hematuria (casts) that occurs conccurently with respiratory or gi tract infections (iga is secreted by mucosal linings)
BB said days after
dont confuse with buerger disease
most common. can slowly progress to renal failure. u world say RPGN possible.
alport syndrome
nephritic
mutation in type IV collagen -> thinning and splitting of the basement membrane. most commonly x-linked dominant.
eye problems (retinopathy, lens dislocation), glomerulonephritis, sensironeual deafness, cant see, cant pee, cant hear a bee.
basket weave appeareance on EM
immune complexes in mesagangium on IF
progressive renal failure
membranproliferative glomerulonephritis
nephirtic/nephrotic
type 1 - igg c3 sunendthelial immune complex deposits with granular if. tram track on he and thick glomerlar basement membrane. more common and seen on pas and HE. caused by mseangial ingrowth
type 2 also called dense depoit disease in GBM. no if for igg.
type 1 can be 2nd to hep b/c or idiopathic.
type 2 is associated with c3 nephritic factor (igg ab that stabilizes c3 convertase -> persistent compliment activation -> decreased c3 levels)
varying bun/cr and renal fxn. roteinuria could be in neprhotic range.
poor response to steroids, progresses to renal failure
radidly progressive (crescentic) glomeruloneprhitis
nephritc
LM and IF - cresecent moon shape in bowman shape. crscents consist of fibrin and plasma proteins (eg c3b) with glomerular parietal cells, monocytes, and macs
type one - linear - good pastures
a3 collagen chain
treat with emergent plasmapharesis
poor prognosis rapidly progresses to renal failure (days to weeks)
PSGN
sle (DPGN)
iga nephropathy
heno scholein purpura
clinical pictre and IF help resolve picture
minimal change disease (lipoid nephrosis)
nephrotic
LM - normal glomerulus, lipid may be seen in PCT
IF - negative
em - effacement of foot processes and fusion, gmb losses negative charge.
most common cause of nephrotic syndrome in children. often primary (idiopathic) and often caused by recent infection, immunization, URI, immune stimulus (bee sting). rarely may be 2nd to hodgkin lymphoma (ctokine mediated damage by reid sternerg cells)). - i think cytokines damage in primary and secondary.
excellent response to corticosteroids
selective charge loss and only albumin
focal segmental glomerulonephrits
nephrotic syndrome
LM - segmental slcerosis and hyalinosis (pinkening)
if - often negative but may b positive for non specific focal deposits f igm c3 and c1
em - effacemet of foot process similar to minimal change disease
uptodate primary - unkown secondary - reduced mass - nephron loss - single kidney - surgical removal - other causes
most common cause of neprhotic sndrome in african americans and hispanics.
HIV, infection, sickle cell disease, heorin abuse, massive obseitsy, intereron treatment, chronic kidney disease due to congenital malformations.
inconsistent response to steroids -> renal failure often
membranous neprhopathy (membranou gomerulonephritis)
diffuse
no inflmmation compared to MPGN. no hypercellulatiry.
lm - difuse fapillary and gbm basement thickening.
IF - granular as a result of immune complex deposition. nephrotic presentation of SLE. Igg and c3
EM - spike and dome appearace with subepithelial deposits.
most common cause of primary nephrotic syndrome in caucasion adults. can be primary (autoABs agaisnt phospholiase on a2) or secondary to drugs (catopril, nssaids, penicilamine, gold), infections (hbv, hcv, syphillis), sle, or solid tumrs (coon, lung, melanoma, malaria, diabets
poor response to steroids -> progress to renal failure in adults, less likely in kids
amyloidosis (renal)
lm - congo red stain shows apple green birefringence under polarized light due to amyloid deosition in mesangium
kidney is the most commonlyinvolved organ in systemic amyloidosis. associated with chornic conditions that predispose to amyloid deposition (al amyloid or aa amyloid)
diabetic glomerulonephopathy
LM - mesangial expansion, gbm thickening, eosinophillic nodulat glomerulosclerris (kw bodies)
wind up with renal failure, htn, and nephortic syndrom
glycation o GBM -> increased permeability and thickening. glycation of fferect (hyaline arteriolosclerosis) -> increased gfr -> mesagnial expansion
microlabunuria protect with ace
most common cause of end stage renal failure in the states.
