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45 Cards in this Set
- Front
- Back
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What is the most frequent cause of nephrotic syndrome in children?
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Minimal Change Disease
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What are the clinical features of MCD?
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Nephrotic syndrome w/selective proteinuria
Normal BP, normal renal fxn, normal serologies |
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What is the prognosis for MCD?
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60-80% recover after steroids
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What are the only patients with MCD who progress?
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Those who actually have FSGS
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What is the pathogenesis of MCD?
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Serum from patient-->increased glomerular permeability & loss of GBM anionic charge
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What are the 2 types of FSGS?
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Primary: idiopathic
Secondary: known underlying cause of disease (morbid obesity, "healed" focal GN, reduced renal mass, HIV infection) |
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What is the most frequent cause of nephrotic syndrome in young & middle-aged adults?
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Focal Segmental Glomerular Sclerosis
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What is a predisposing factor for FSGS?
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African-American race
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What are the clinical features of FSGS?
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Severe (nephrotic-range) proteinuria that is nonselective
May have hypertension or microscopic hematuria |
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What is the outcome of FSGS?
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Most cases are steroid-resistant-->progress to ESRD
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Significance of MYH9?
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On chromosome 22
contributes to FSGS progression in African-Americans |
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What contributes to FSGS progression in African-Americans?
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MYH9 on gene 22
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Does FSGS recur in renal allografts?
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1/3 do
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What is the pathogenesis of FSGS?
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Podocyte injury
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What is NPHS1 related to?
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Nephrin gene associated w/FSGS
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What is NPHS2 related to?
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Podocin gene associated w/FSGS
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What are the clinical features of glomerulopathy secondary to obesity or metabolic syndrome?
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Proteinuria (usually not nephrotic range but can be)
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What is the histology seen in glomerulopathy secondary to obesity or metabolic syndrome?
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Large glomeruli w/FSGS
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What is the pathogenesis of glomerulopathy secondary to obesity or metabolic syndrome?
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glomerular hyperfiltration-hyperperfusion injury
Insulin resistance Inappropriate RAAS activation Hyperaldosteronemia Oxidative stress Adipokiins: leptin & adiponectin |
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What is leptin associated with?
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Pathogenesis of glomerulopathy secondary to obesity or metabolic syndrome
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What is adiponectin associated with?
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Pathogenesis of glomerulopathy secondary to obesity or metabolic syndrome
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What is the treatment for glomerulopathy secondary to obesity or metabolic syndrome?
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Weight loss
Metabolic control RAS blockade-->anti-aldosterone agents |
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What is the most frequent cause of nephrotic syndrome in the non-diabetic elderly?
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Membranous glomerulonephritis/membranous nephropathy
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What are the clinical syndromes of membranous GN?
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Nephrotic syndrome
No gross hematuria Normal renal function |
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What can membranous GN be secondary to?
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SLE
Hep B & C drugs (penicillamine, NSAIDs), neoplasms |
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What is the outcome of membranous GN?
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30-40% develop spontaneous remission
Slow if any progression 20% develop ESRD |
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What is the pathogenesis of membranous GN?
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In-situ immune complex formation
Usually phospholipase A2 receptor |
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What commonly involves phospholipase A2?
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Primary membranous GN
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What is the most common cause of ESRD in the US?
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Diabetic nephropathy
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What are the clinical features of diabetic nephropathy?
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Microalbuminuria followed by proteinuria that reaches nephrotic-range
Decreasing GFR Increasing serum creatinine HTN is common |
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What is the outcome of diabetic nephropathy?
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Progressive to ESRD
Transplant-->good prognosis |
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What is the pathogenesis of diabetic nephropathy?
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Increased glycosylation of glomerular ECM
Increased GF production |
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What is the morphology of diabetic nephropathy?
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Thickened GBM
K-W nodules |
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What is the epidemiology of lupus nephritis?
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Young black women mostly
60% of SLE px have renal involvement |
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What is the clinical presentation of lupus nephritis?
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Variety of symptoms
Microscopic hematuria Nephritic syndrome Nephrotic syndrome Asymptomatic proteinuria Any combination of the above |
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What is the morphology of lupus nephritis?
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Variety of LM patterns
IF: C3, C1q, IgG, IgM, IgA |
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What is class I lupus nephritis?
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NL kidney w/mesangial deposits
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What is class II lupus nephritis?
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Mesangial hypercellularity & IC deposition
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What is class III lupus nephritis?
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Intra and/or extracapillary GN in <50% of glomeruli, mesangial & capillary loop deposits
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What is class IV lupus nephritis?
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diffuse prolfierative GN (>50%), mesangial & capillary loop deposits
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What is class V lupus nephritis?
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membranous GN
Subepithelial glomerular capillary deposits |
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What is class VI lupus nephritis?
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chronic sclerosing GN
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What do you treat class III and IV lupus nephritis with?
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cellcept
prednisone |
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What kind of outcome do classes II & V of lupus nephritis have?
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good
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Does lupus nephritis recur in renal allografts?
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no
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