Figure 944

Glomerulocystic kidney disease (GCKD). Contrast-enhanced CT, A, in a 23-year-old woman with the sporadic form of GCKD shows

multiple cysts, typically small cortical ones. This cystic pattern was verified in the nephrectomy specimen, B, obtained 8 months later at the time of kidney transplantation, and GCKD was confirmed by histopathologic examination with Masson's trichrome stain. C, Cysts consisted of a dilatation of Bowman's space surrounding a primitive-looking glomerulus.

GCKD may be sporadic or genetically dominant. Among the familial cases, some patients are infants who have early-onset autosomal-dominant polycystic disease. In others (children or adults) the disease is unrelated to PKD1 and PKD2 and may or not progress to end-stage renal failure [38]. (Courtesy of D. Droz.)

ARPKD: CLINICAL MANIFESTATIONS

Renal

Antenatal (ultrasonographic changes) Oligohydramnios with empty bladder Increased renal volume and echogenicity Neonatal period Dystocia and oligohydramnios Enlarged kidneys Renal failure

Respiratory distress with pulmonary hypoplasia (possibly fatal) Infancy of childhood Nephromegaly (may regress with time) Hypertension (often severe in the first year of life)

Chronic renal failure (slowly progressive, with a 60% probability of renal survival at 15 years of age and 30% at 25 years of age) Hepatic Portal fibrosis

Intrahepatic biliary tract ectasia

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