Autosomal recessive polycystic kidney disease

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[edit] 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.

[edit] Imaging Findings for Autosomal recessive polycystic kidney disease

[edit] US

  • 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.

[edit] Images

Patient #1: MRI demonstrates typical imaging features of autosomal recessive polycystic kidney disease

[edit] See Also

[edit] External Links

[edit] References for Autosomal recessive polycystic kidney disease

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