Autosomal recessive polycystic kidney disease
 Discussion of Autosomal recessive polycystic kidney disease
- Autosomal recessive polycystic kidney disease is a hereditary condition characterized by renal cystic disease and hepatic fibrosis in varying degrees of severity.
- Neonatal patients have prominent renal enlargement with renal failure and minimal liver dysfunction.
- Approximately 30%–50% of affected infants die during the perinatal period owing to pulmonary hypoplasia and pulmonary insufficiency.
- Those that survive have progression of the renal and liver disease, with increasing amounts of fibrosis.
- The hepatic fibrosis might be progressive but is not of clinical importance in this group of patients.
 Imaging Findings for Autosomal recessive polycystic kidney disease
- At US, the kidneys are massively enlarged and diffusely echogenic bilaterally.
- Corticomedullary differentiation is absent.
- High-resolution US (linear-array transducer, 7.5 mHz or greater) allows visualization of numerous cylindrical cysts in the medulla and cortex, which represent ectatic collecting ducts.
Patient #1: MRI demonstrates typical imaging features of autosomal recessive polycystic kidney disease
 See Also
- Acquired cystic kidney disease
- Autosomal dominant polycystic kidney disease
- Cystic disease of the kidneys
 External Links
 References for Autosomal recessive polycystic kidney disease
- Maria-Gisela Mercado-Deane, James E. Beeson, and Susan D. John. US of Renal Insufficiency in Neonates. RadioGraphics 2002 22: 1429-1438.