Nephrotic Syndromes Flashcards
A conformational change in what chain of the type IV collagen of GBM results in Anti-GBM Glomerulonephritis?
The α3 chain
Sometimes Anti-GBM antibodies cross-react with basement membranes of the lung alveoli. This can result in a systemic Anti-GBM syndrome called. . . ?
Goodpasture Syndrome
Anti-GBM is associated with what type of inflammation?
Activation of complement via the CLASSICAL pathway primarily. However, alternative and mannose-binding lectin pathways can also be activated.
What are five additional (i.e. excluding complement activation) mediators of glomerular damage in Anti-GBM Nephritis?
- Monocytes/macrophages
- Sensitized T cells
- Platelets
- Resident Glomerular Cells
- Thrombin
Name two proteins associated with the integrity of slit diaphragms.
Nephrin and Podocin (mutations in these structural proteins can result in rare hereditary forms of nephrotic syndrome)
Name 4 distinguishing characteristics of nephrotic syndrome.
- Massive proteinuria
- Hypoalbuminemia
- Generalized Edema
- Hyperlipidemia and lipiduria
Anasarca is commonly associated with what class of kidney disorders?
Nephrotic syndrome
BUZZ WORD: “Foot Process Effacement” is associated with. . .
Minimal Change Disease
BUZZ WORD: “Spike and Dome” is associated with. . .
Membranous Nephropathy
BUZZ WORD: “Subepithelial Humps” is associated with. . .
Post-Infectious Glomerulonephritis (PIGN)
BUZZ WORD: “Tram Tracks” is associated with. . .
Membranoproliferative Glomerulonephritis (MPGN)
BUZZ WORD: “Basket Weave” is associated with. . .
Alport Syndrome
BUZZ WORD: “Wire Loops” is associated with. . .
Lupus Nephritis
BUZZ WORD: “Onion-skin” is associated with. . .
Hypertensive Nephropathy or Scleroderma
Minimal-Change Disease is a disorder most common in what population?
Children
What is diagnostic for a Minimal-Change Disease in electron microscopy?
Podocyte Effacement
What is the prognosis for children with Minimal-Change Disease?
Good. More than 90% respond to a short course of corticosteriod therapy.
Focal Segmental Glomerulosclerosis (FSGS) can be primary or secondary. What are the etiologies?
- Primary - idiopathic
- Secondary to:
- HIV
- Heroin abuse
- Other nephropathies (e.g. IgA nephropathy)
- Inherited
Inherited or Congenital FSGS is associated with a mutation in what gene?
APOL1 - primarily affects African-Americans
FSGS is associated with what circulating factor?
suPAR - activates B3 integrin (anchor for podocytes in GBM)
What do the affected glomeruli of FSGS exhibit?
- Increased mesangial matrix
- Obliterated capillary lumina
- Deposition of hyaline masses and lipid droplets
What is a morphologic variant of FSGS?
Collapsing Glomerulopathy - Characterized by collapse of the glomerular tuft and podocyte hyperplasia. Carries a particularly poor prognosis.
What characterizes membranous nephropathy morphologically?
Subepithelial immunoglobulin-containing deposits (thick GBM), as well as thickening of the capillaries and mesangial matrix. “Spike and Dome”
Membranous nephropathy occurs secondarily to what disorders:
- Infections (Hep B, Syphilis, Schistosomiasis, Malaria)
- Malignancies (esp. carcinoma of the lung)
- SLE
- Exposure to gold/mercury
- Drugs (penicillamine, captopril, NSAIDs)
What antigen is most often recognized in cases of membranous nephropathy?
Phospholipase A2 Receptor - an endogenous podocyte antigen.
What is unique/peculiar about membranous nephropathy as a renal disorder?
It is a disorder in which complement is recruited and there is MAC formation, however it is not considered a nephritis because there are no inflammatory cells involved in the process.
What are the morphologic characteristics of Membranoproliferative Glomerulonephritis (MPGN type I)?
- Hypercellularity in the glomerular tuft.
- Expanded mesangium
- Capillary wall changes - double contour (“Tram Tracks”)
Dense Deposit Disease (formerly MPGN type II) is associated with what protein?
C3 Nephritic Factor (also less commonly mutations in factor H)
