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54 Cards in this Set
- Front
- Back
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#1 cause of nephrotic syndrome in kids?
in adults? in African Americans? Worldwide? |
Kids: Minimal change disease
Adults: Membranous GN Af Am: Collapsing FSGS Worldwide: IgA nephropathy |
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Associations with FSGS
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HIV
Heroin Obesity Unilateral agenesis Most are idiopathic |
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Associations with membranous GN?
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Solid tumors
Diabetes HBV, HCV, Syphilis, Malaria Lupus Drugs (Gold, Penicillamine, NSAIDs) |
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NSAIDs associated with which 4 renal diseases?
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Minimal change disease
Membranous glomerulopathy Papillary necrosis / Analgesic nephropathy Acute interstitial necrosis |
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Gene in ARPKD?
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PKHD! chromosome 6
Fibrocystin |
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Gene in ADPKD?
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PKD1 (16) polycystin 1
PKD2 (4) polycystin 2 |
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Clinical features of ADPKD
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Massive kidneys
Bilateral Liver MVP (25%) berry aneurysm (8%) |
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HCV associated with what 3 renal diseases?
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MPGN
MGN Cryo-GN |
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Gene in Alport's?
clinical? EM? |
Mutation COL4A5 xq22
Collagen IV alpha chain 5 usu XR Nephritis & sensorineural deafness Thickening and attenuation of GBM & TBM Moth-eaten |
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Other disease of collagen IV in kidney?
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Thin basement membrane disease (benign familial hematuria)
Dx on EM: THINNING OF GBM Mutation COL4A4 gene, alpha 3& 4 chains |
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What 3 diseases do we see isolated hematuria in?
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Thin basement membrane nephropathy
Alport's syndrome IgA nephropathy |
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Name 2 nephropathies that have subepithelial deposits on EM?
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Acute post-infectious GN (large humps)
Membranous GN (basement membrane fills in between the humps giving a SPIKED appearance) |
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Tram-track silver stain
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MPGN
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Associations with MPGN type I
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HBV
HCV Cryoglobulinemia alpha-1-antitrypsin def Schistosomiasis Chronic liver disease |
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Subendothelial deposits on EM
Large chunky IgG, IgM, C3 |
MPGN
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Low C3
Kid / young adult Disease? Unique autoantibody? |
MPGN Type II (DENSE DEPOSIT DISEASE)
see dense deposits in lamina densa IF stains C3 Circulating autoantibody to "C3 nephritic factor" |
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Name 2 nephropathies with MESANGIAL DEPOSITS
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IgA nephropathy
SLE Class II |
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Name 2 nephropathies that preferentially have more lambda light chains than kappa?
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Amyloid (AL type)
IgA nephropathy (Light chain deposition disease: 90% kappa!) |
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Hallmark of cyclosporine and tacrolimus toxicity?
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TUBULAR VACUOLIZATION
STRIPED FIBROSIS |
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"string of beads" or "sausage string" on imaging
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Fibromuscular dysplasia
#1 cause renovascular HTN young females |
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Multiple myeloma associations in kidney
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Amyloid (l>k)
Light chain deposition disease (k>l) Cast nephropathy (presents as ARF) |
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Most severe class of SLE in kidney?
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Class IV: Diffuse
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Worse px SLE classes?
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class III (Focal)
Class IV (Diffuse) |
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Better px SLE classes?
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Class II (mesangial prolif)
Class V (Membranous) |
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Class I SLE? Class VI?
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I: Minimal
VI: Advanced, sclerosing |
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Which SLE classes can be dx on light microscopy?
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III
IV V |
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"antibody-mediated rejection"
IF? |
Hyperacute: within minutes
Accelerated: days ANTI-DONOR antibodies C4d |
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"cell-mediated immune rejection"
time frame? histology? |
Acute rejection
months- years Banff criteria hallmark is TUBULITIS +/- vasculitis |
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"synpharyngitic gross hematuria"
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IgA nephropathy
Gross hematoria simultaneously with a URI More common in Asian patients MESANGIAL deposits lambda > kappa |
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Crescents on biopsy
NEGATIVE IF & EM |
Wegener's
"pauci-immune crescentic glomerulonephritis" cANCA to proteinase 3 50% RPGN |
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hemoptysis
Crescents on biopsy LINEAR IF |
Goodpasture's disease
anti-GBM 20% RPGN |
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Crescents on bx
GRANULAR IF |
SLE, post-infectious
"immune complex" 30% RPGN |
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Why don't we dx RPGN?
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Clinical dx
Must have rapid loss renal function & oliguria instead, "proliferative crescentic gn" |
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IF in SLE?
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FULL HOUSE
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"Bloodless glomeruli"
Fibrinoid necrosis afferent |
TTP/HUS
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histologic features of thrombotic microangiopathies
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Thrombi
Endothelial injury MUCOID INTIMAL EDEMA DOUBLE CONTOUR GBM |
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Types of TMAs?
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TTP/HUS
Scleroderma HTN Preeclampsia Antiphospholipid antibody syndrome Drug hereditary |
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Types of HUS?
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Diarrheal: E coli O157, Shigella
Non-D: Abn ADAMTS13, or sporadic |
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Kid
rash arthritis abdominal pain |
Henoch-Schonlein purpura
usually recover |
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Associations with fibrillary GN?
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CLL
other B cell lymphomas Recur > transplant Amyloid-like material but CONGO RED NEGATIVE EM: Random fibrils 20-30nm |
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Nephrotic syndrome
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Proteinuria (>3.5g/d)
Hypoalbuminemia (<3.5) Edema Hyperlipidemia |
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Nephritic syndrome
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Hematuria
Hypertension Decreased GFR +- proteinuria |
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association?
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Kimmelstein-Wilson nodule
DIABETES MELLITUS = nodular glomerulosclerosis |
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Histology of DM in kidney?
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KW nodules
THICKENED GBM Glomerular hypertrophy HYALINOSIS OF AFF & EFF A CAPSULAR DROP: hyalin in Bowmans capsule |
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Association?
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Cast nephropathy
Crystals are light chains See in Myeloma Can be accompanied by giant cells |
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Child
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Minimal change disease
Effacement of foot processes |
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Effacement of foot processes in adult with HIV?
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FSGS
IF: IgM & C3 (vs none in MCD) |
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Associations with adult MCD?
kids? |
Paraneoplastic (HL)
NSAIDS kids; allergies & post-immunization |
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seen in ?
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Tubuloreticular inclusions
HIV SLE Interferon treatment |
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IF for C3 is seen in?
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LOTS!
FSGS (IgM) MGN (subepithelial) (IgG) MPGN I (subendothelial) (IgM, IgG) MPGN II (DENSE lamina) Fibrillary GN (linear)(IgG4, k, l) Acute post-strep infxn (IgG)(Starry sky) |
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Starry sky IF for IgG & C3
Child |
post-infectious GN
High ASO titers, low C3 |
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Child
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post-infectious GN
Subepithelial humps without basement membrane filling in (vs membranous GN) |
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Membranous GN
SPIKES & HOLES in GBM |
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Membranous GN
SUBEPITHELIAL deposits, GBM fills in (vs post-infxs) |
