Renal Week 1 - Nephrotic/nephritic Flashcards by Cameron Niswander

2 protein components of podocyte slit diaphragm

Nephrin

Podocin

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Classic and common mechanisms of pitting edema in nephrotic syndrome

Classic (low V, low P): low serum albumin -> low oncotic pressure -> fluid leaks out -> increased ADH/Na retention -> more fluid out
Common (high V, high P): primary defect in Na excretion -> retention

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Complications of nephrotic syndrome

Hypercoagulable state: protein deficiency, increased ATIII production and renal vein thrombosis
Infection (loss of Ig and complement)
Decreased vitamin D
Hypercholesterolemia
HypoNa/K

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Management of nephrotic syndrome

Low salt diet
Diuretics
BP control
Decrease GFR (ACEI)
Replace Vit D binding proteins

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Hereditary Nephrotic Syndrome

Mostly kids
Mutation in nephrin or podocin
Foot process fusion on EM

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Minimal change dz: age, UA, cell markers/pathogenesis

2-8 years old
UA: 4+ protein, hyaline casts
CD-80 expression on podocytes (usually on APCs)
Foot process fusion on EM
Circulating factor damaging podocyte

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Associations of minimal change dz

Allergy
Hodgkin’s Lymphoma
NSAIDs

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Treatment of minimal change dz

Steroids

Course of oral cytoxan for frequent relapsers

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FSGS: age, UA, pathogenesis

20-40 years
UA: 4+ protein, hyaline casts, micro hem
Viral factor: HIV infecting podocyte
Genetic factor: APOL1 polymorphism (AA, sleeping sickness protection)

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FSGS associations

Idiopathic
HIV associated
Sickle cell
Obesity
Heroin nephropathy

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FSGS treatment

Steroids
Calcineurin inhibitors or other immunosuppresion
Anti-viral for HIV

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HIV Nephropathy

FSGS
Tubular dilation
Reticulo-endothelial inclusions

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Membranous nephropathy associations

Idiopathic
CA (Lung esp)
Lupus
Hep B (tx interferon)
Heavy metals

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Membranous nephropathy: age, pathogenesis

Adults
Immune complex disease
Thickened BM
Subepithelial deposits on podocyte side of BM (granular deposits on IF)
Ab binding to PL A2 podocyte receptor

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Membranoproliferative GN type I associations

Hep C (autoimmune, palpable purpura)
Low grade systemic infection
Neoplasm
CT disease (lupus)

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MPGN type I presentation, pathogenesis

Endothelial deposits (WBC)
Complement is low due to activation (C3 and C4)
GBM thickening and hypercellularity

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MPGN type II

Same presentation at type I

Low C3, nml C4 -> alternative pathway activation

Nephrotic syndromes: primary renal dz

Hereditary
MCD
FSGS
MN
MPGN (I and II)

Nephrotic syndromes: systemic

Diabetes
Amyloid and Light chain dz
SLE (membranous)

Nephritic syndromes: primary renal

Post strep GN
IgA nephropathy
RPGN (anti-GBM, idiopathic)

Nephritic syndromes: systemic

Vasculitis (ANCA associated)
Immune complex (SLE, HSP)

Nephrotic syndrome: overall theme and major criteria

Increased glomerular permeability

Albuminuria
Hypoalbuminemia
Edema
Hyperlipidemia

Nephrotic mechanisms of injury

Non-inflammatory
Podocyte injury and retraction: loss of size selectivity and loss of polyanion
Injury to clathrin pit, absorbed and secreted as deposit
Progress into CRF

Renal amyloidosis: types, path, stain

Kidney involved in 85%
Different LC types: AA/AL
Fluffy pink in glomeruli and vessels
Red congo stain/polarized light/granny apple green
Spilled uncooked spaghetti

Causes of chronic renal failure

Most common: diabetes | Next: vascular/HTN dz

DM in CRF

Hyaline arteriolar disease (increased thickness) | Diabetic GS: hypercellularity, diffuse/nodular mesangial expansion, BM thickening

Uremia definition

Group of manifestations with severe loss of all renal functions

Nephritic syndrome: major criteria

Hematuria (RBC and casts)

Signs of aggressive disease and usual association

RPGN (2x Scr or 50%

Treatment of nephritic syndrome

Treat symptoms Immunosuppression Plasma exchange

4 examples of glomerulonephritis

IgA nephropathy (HSP = systemic,

IgA nephropathy: deposition, prognosis, type

Mesangial immune complex deposition Focal 30% regress, 30% to ESRD, 40% chronic renal dz Nephritic or nephrotic

Lupus nephritis: deposition, presentation, type, pathoneumonic finding

Mesangial (hematuria) Subendothelial (hematuria, nephritic syndrome) Subepithelial (proteinuria) >90% scarring -> nephritic and progressive failure Nephritic or nephrotic or both Pathoneumonic = tubuloreticular body

Anti-GBM disease: deposition, presentation

Antibody to glomerular antigen (linear) Usually bad: pulmonary-renal, crescents, RPGN Big treatment

ANCA associated vasculitis: deposition, presentation, unique treatment

ANCA but few immune complexes seen Usually bad: pulmonary-renal, RPGN Normal treatment + B cell targeted therapy

ANCA vasculitis examples

Granulomatosis with polyangiitis (C-ANCA, PR3) Microscopic polyangiitis (P-ANCA, MPO) Eosinophilc granulomatosis (Churg-Strauss)(more P-ANCA) Renal limited vasculitis (usually P-ANCA)

Two diseases that mimic nephritic dz

Benign Familial Hemauria (thin BM dz) Alport's dz (nephritis, deaf, ocular lesions) Collagen IV mutation, basket-weave pattern on microscopy

Proliferative glomerulonephritis: types and consequences

Endocapillary: occlusion of capillary loops Mesangial: >3 nuclei in space Epithelial: crescents, fill Bowman's space

Post-infectious GN: etiology, morphology, stain, tx

Post strep, acute nephritic Diffuse proliferative Starry sky on IF Supportive therapy

IF Staining and diseases

Linear: Goodpasture's, Anti-GBM Granular: IgA, SLE, endocarditis, idiopathic None: vasculitis, idiopathic

Renal lupus WHO classes

``` I = no change II = mesagnial glomerulitis III = Focal, segmental IV = Diffuse (>55%) V = membranous ```