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Nephrotic

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Disease

Most Frequent Clinical Presentation

Pathogenesis

Age Group Affected #1 cause of Nephrotic Syndrome in children, esp. boys younger than 6 yrs. old.

Treatment and Outcome -Responds well to corticosteroids. - No progression into chronic renal failure -Does not respond to corticosteroids. -Leads to renal failure.

Minimal Selective Change Disease proteinuria (Lipoid (Albumin) Nephrosis)

Loss of foot processes Loss of GBM polyanionic sites Appearance of villi on epithelial cells Idiopathic Lower renal mass (in obese) 2 causes: heroin use, HIV

Focal Segmental Glomerular Sclerosis

Non-selective proteinuria Hypertension Microscopic hematuria

Majority occur in older children. Also occurs in adults.

Disease

Most Frequent Clinical Presentation Persistent proteinuria

Pathogenesis

Age Group Affected #1 cause of Nephrotic syndrome in adults

Treatment and Outcome -Benefit of corticosteroids is unknown.

Membranous Nephropathy (Glomerulo nephritis)

Idiopathic 2 causes: carcinomas, SLE, hepatitis, Diabetes Mellitus, thyroiditis, drugs.

Diabetic Nephropathy (Diabetic Glomerulo sclerosis)

Proteinuria Diabetic No hematuria microangiopathy Thickened B.M. Massive mesangial growth "Kimmelstiel Wilson" nodular glomerulosclerosis Diffuse glomerulosclerosis

Diabetics

-Progresses to renal failure

Disease

Most Frequent Clinical Presentation Recurrent hematuria before age 20 Hypertension Deafness and ocular problems Recurrent hematuria Most frequent cause of asymptomatic hematuria.

Pathogenesis

Age Group Affected Symptoms appear before age 20

Treatment and Outcome -Progresses to renal failure

Alport Syndrome (Hereditary Nephritis)

Structural defect in Collagen IV leads to leaky basement membranes.

Benign Familial Hematuria (Thin B.M. Disease)

Reduced thickness of glomerular B.M.

Disease

Most Frequent Clinical Presentation Acute nephritis Abrupt oliguria, hematuria, facial edema, hypertension.

Pathogenesis

Age Group Affected Common renal disease in childhood

Treatment and Outcome -Return to normal in 8 weeks. Complete recovery without treatment (especially in kids) within 3 years.

Acute (PostStreptococcal) Glomerulo nephritis

Immune-complex mediated (Type-III hypersensitivity) Occurs after Streptococcal pharyngitis or Hepatitis-B High ASO-titer, low C3 Anti ds-DNA antibodies.

SLE Nephropathy

Degree of kidney involvement correlates with prognosis in SLE.

Disease

Most Frequent Clinical Presentation IgA Nephropathy (Berger's Disease): Most common primary Glomerulo nephritis

Pathogenesis

Age Group Affected Young men 15-30

Treatment and Outcome

Focal Segmental Glomerulo nephritis

Circulating IgA + fibronectin (due to chronic liver disease)

HenochSchonlein Purpura

Same as above, Mesangial cell Children plus proliferation, more systemic serious than above. disease: purpura of extremities, arthritis, colicky abdominal pain.

Disease

Most Frequent Pathogenesis Clinical Presentation S. Aureus Subepithelial immune deposits

Age Group Treatment Affected and Outcome Kidney disease resolves when infection is cured.

Endocarditis

Rapidly Progressive Crescentic Glomerulo nephritis

Wegener's: kidney + upper respiratory tract. Anuria Oliguria

Inflamed glomerular capillaries ANCA (+)

Must be treated or it will go to renal failure within weeks.

Disease

Pathogenesis

Age Group Affected

Treatment and Outcome

Goodpasture Syndrome Lung (hemoptysis) + (Anti-BM Antibody kidneys (hematuria) Disease)

Anti-B.M. Males 25-30 antibodies, against TypeIV collagen

Membranoproliferative Glomerulonephritis (Mesangiocapillary Glomerulonephritis)

B.M. thickening and cellular proliferation Mesangial expansion makes glomerular B.M. appear as though it were in two layers

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