Expert Recommendations for ARPKD

A recent article (J Pediatr 2014; 165: 611-17) provides expert recommendations for the diagnosis and management of autosomal recessive polycystic kidney disease (ARPKD).

Some of the recommendations relevant to hepatologists:

  • Congenital hepatic fibrosis (CHF) “is presumed when portal HTN [hypertension] is present.”  Portal HTN is defined by splenomegaly (i.e.. spleen >2 cm below the left costal margin or >1 cm larger than ULN for age) and thrombocytopenia with a value of <150 mm3.  Other evidence of portal HTN includes varicose, ascites or hepatopulmonary syndrome.
  • “Liver biochemistries are not typically informative…one should monitor for associated neutropenia and thrombocytopenia.”
  • “Cholangitis may be difficult to diagnose definitively…should be considerd in any child with ARPKD with unexplained fever.” “Routine antibiotic prophylaxis is not indicated…antibiotic prophylaxis for 6-12 weeks after a cholangitis episode..may be considered.”
  • The “use of ursodeoxycholic acid as a choleric cannot be recommended.”
  • Hepatobiliary cancer is not a feature of ARPKD in children
  • “Limiting contact activities in individuals with a palpable spleen is highly controversial and not guided by evidence, but more by common sense.”
  • Recommends annual CBC, ultrasound at five years of age, and then follow-up ultrasound every 2-3 years.

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