Disorders Of The Renal System

Front 1

Minimal Change Disease
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and prognosis:

Back 1

Epi: Often young children, sometimes after URI.
Path: Normal on LM, fusion of foot processes on EM.
Clin:Nephrotic syndrome.
Treat:Response is excellent to prednisone.

Front 2

Membranous Glomerulonephritis
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and Prognosis:

Back 2

Epi: Can be primary or secondary to lupus, hepatitis, syphilis, drugs, or malignancy.
Path: Diffuse capillary and BM thickening on LM. Granular IgG or C3 deposits on IF. Subepithelial immune complex deposition on EM. Spike-and-dome appearance on silver methenamine stain.
Clin: Nephrotic syndrome accompanied by azotemia.
Treat:Cyclophosphamide and ACE inhibitors.

Front 3

Focal Segmental Glomerulonephritis
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and Prognosis:

Back 3

Epi: Associated with heroin use, morbid obesity, HIV infection. Older patients usually.
Path: Segmental sclerosis within capillary tufts of SOME glomeruli (focal). IgM and C3 on IF. Fusion of epithelial foot processes on EM.
Clin: Nephrotic syndrome. 80% have hematuria.
Treat: Prednisone. Most patients progress to ESRD.

Front 4

Diabetic nephropathy
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and Prognosis:

Back 4

Epi: Type I DM has 30-40% chance of DN after 20 years, type II has 15-20%. Most common cause of ESRD in US.
Path: Increase in mesangial matrix on LM, thickened GBM on EM.
Clin: Nephrotic syndrome, microalbuminuria and then proteinuria.
Treat:Glycemic control and treatment of HTN early, nothing once proteinuria sets in.

Front 5

Lupus nephropathy
Type I
Type II
Type III
Type IV
Type V

Back 5

Lupus nephropathy
Type I: Normal, no findings or treatment
Type II: Mesangial, focal and segmental glomerular involvement, slight proteinuria and hematuria.
Type III: Focal proliferative form, extensive damage to individual glomeruli. Treat III-V with immunosuppressives.
Type IV: Diffuse proliferative, with wire loop abnormalities on LM from immune complex deposition and gross thickening of GBM, endothelial cell proliferation on EM, and subendothelial deposition on IF. Nephrotic and nephritic syndromes.
Type V: Membranous form, looks like membranous glomerulonephritis.

Front 6

Poststreptococcal Glomerulonephritis
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and Prognosis:
Notes:

Back 6

Epi: Often seen in children following group A strep infection.
Path: Diffuse inflammatory reaction with surface hemorrhages grossly, swollen glomeruli with mesangial and endothelial proliferation, subepithelial humps on EM, granular IgG or C3 on IF. RBCs or casts seen in urine.
Clin:Nephritic syndrome and periorbital edema.
Treat: Spontaneously resolves.
Notes: Immune complex disease with strep antibody/antigen.

Front 7

Rapidly progressive (crescentic) glomerulonephritis
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and Prognosis:

Back 7

Epi: 50% are post-streptococcal. Immune type can be caused by lupus nephropathy, IgA nephropathy, Wegener granulomatosis or Goodpasture syndrome. Pauci-immune type doesn't have immune complex deposition or anti-GBM a.b.'s
Path: Crescent-moon shape between Bowman's capsule and glomerular tuft from fibrin deposition and parietal epithelial cell proliferation on LM. C-ANCA auto-immune a.b.'s in blood.
Clin: Nephritic syndrome with rapid progression to ESRD. Hemoptysis and anti-GBM a.b.'s in Goodpasture syndrome.
Treat: Dialysis and/or transplantation

Front 8

Alport Syndrome
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and Prognosis:

Back 8

Epi: Genetic disorder with mutation of alpha chain of type IV collagen.
Path: Pattern of thickening and attenuation of GBM, with longitudinal splitting of lamina densa.
Clin: Nephritis, nerve deafness, and eye disorders. Hematuria and erythrocyte casts in urine.
Treat: ACE-inhibitors and transplantation.

Front 9

Membranoproliferative glomerulonephritis
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and Prognosis:

Back 9

Epi: Type I is seen in SLE, HBV and HCV, and involves activation of classic and alternative complement pathways. Type II only involves alternative pathway.
Path: Reduplication of basement membrane and expansion of mesangium into capillaries (tram tracks!) on IM. Subendothelial deposits in type I, dense deposits within GBM in type II on EM.
Clin: Type I presents with nephrotic syndrome, type II presents with hematuria and CRF.
Treat: Corticosteroids and antiplatelet drugs.

Front 10

IgA Nephropathy (Berger Disease)
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and Prognosis:

Back 10

Epi: Most common acute glomerulonephritis in U.S. Primary renal disease of IgA deposition in glomerular mesangium. Can come after viral URI, GI infection, etc.
Path: Focal proliferative glomerulonephritis with diffuse mesangial widening on EM. Mesangial IgA deposits on EM.
Clin: Recurrent hematuria 1-2 days post-infection. Increased serum IgA in 50% of cases, normal complement levels.
Treat: Prednisone.

Front 11

Acute Pyelonephritis
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and Prognosis:
Notes:

Back 11

Epi: Caused by infection of renal parenchyma, more frequent among women.
Path: Affects renal cortex with sparing of glomeruli. Abscesses in renal interstitium may rupture-->WBCs in tubular lumen.
Clin: Fever, flank pain with CVA tenderness, polyuria, nausea.
Treat: IV antibiotics.
Notes: Renal papillary necrosis is a complication of APN in diabetics.

Front 12

Chronic Pyelonephritis
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and Prognosis:
Notes:

Back 12

Epi: Results from chronic urinary tract obstruction and recurrent UTI's.
Path:Asymmetric corticomedullary scarring, tubules contain proteinaceous casts reminiscent of thyroid follicles. Later, tubular atrophy and interstitial fibrosis.
Clin: Renal hypertension and ESRD. WBC casts in urine.
Treat: Renal transplantation if it progresses to ESRD.
Notes: Diffuse cortical necrosis (medulla spared) can occur, due to a combination of DIC and end-organ vasospasm.

Front 13

Acute Tubular Necrosis
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and prognosis:

Back 13

Epi: Precipitated by renal ischemia, crush injury, or nephrotoxic drugs like aminoglycosides.
Path: Focal tubular epithelial necrosis with ruptured GBM's and flattened, mitotic epithelial cells.
Clin: Signs of acute renal failure. Oliguria, tubular epithelial cell casts in urine, hyperkalemia.
Treat: Loop diuretics and fluid management.

Front 14

Adult Polycystic Kidney Disease
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and prognosis:

Back 14

Epi: Autosomal dominant, 90% due to mutation of APKD1 gene on chromosome 16.
Path: Replacement of renal parenchyma bilaterally with multiple large, variably sized cysts.
Clin: Hypertension, hematuria, palpable renal masses. Associated with mitral valve prolapse.
Treat: No therapy.

Front 15

Infant polycystic kidney disease:
Epidemiology:
Pathology:
Clinical manifestations:
Treatment and prognosis:

Back 15

Epi: Autosomal recessive.
Path: Closed, small, homogenous cysts.
Clin: Multiple cysts seen on CT at birth.
Treat: Death shortly after birth