25.6.2013(pathology) 48 Flashcards
(155 cards)
Extra renal manifestations of ADPKD
Berry aneurysm Cysts in other organs Liver(most common) Pancreas Spleen Ovary Colonic diverticula MVP and AR
Mutation in ARPKD
PKHD 1 which codes for fibrocystin
Chr of ADPKD
Chr 16
Heyman antigen
Megalin
Site of deposition based on charge of molecules
Mesangium (Neutral)
Subepithelial(cation)
Subendothelial(anion)
Antigens responsible for PSGN
Endostreptosin
Nephritis strain associated protein(NSAP)
Starry sky appearance of BM and mesangium is seen in
PSGN
Level of complement proteins in PSGN
Transient reduction for 6-8wks
Type II RPGN
HSP
SLE
post infectious
Deposits in MPGN
Subendothelial deposits of IgG,C1q,C4(Type 1)
Intramembranous deposits of C3 alone(Type 2)
Complement activation in MPGN
Both classical and alternative pathways are activated in MPGN1
Only alternative pathway activated in MPGN2
EM appearance of MPGN
Tram track or double contour appearance(MPGN1)
Dense deposit disease(MPGN2)
NPHS1
Nephrin
Hereditary form of congenital nephrotic syndrome
Finnish type
NPHS2
Podocin
Steroid resistant nephrotic syndrome
autosomal recessive FSGS
Autosomal dominant FSGS
A actinin-4
Mutation causing adult onset FSGS
TRPC6
Deposits in membranous glomerulopathy
Subepithelial
HPE of collapsing glomerulopathy
Collapse and sclerosis of entire glomerular tufts
Microcysts in tubules
Cause of collapsing glomerulopathy
Podocin expression of vpr and nef
Most common cause of collapsing glomerulopathy in children
Cystinosis
EM appearance of collapsing glomerulopathy
Tubuloreticular inclusions induced by interferon alpha
Secondary causes of Berger disease
Liver failure
Intestinal disease
Alternative complement pathway alone is activated in
MPGN2
Berger disease
Deposits in FSGS
C3
IgM