Table 1.

Diagnostic criteria for ARPKD. Modified from Zerres et al37

Imaging criteria Characteristic findings on US, as defined by Garel et al38

Clinical criteria Absence of renal cysts in both parents by US Signs of hepatic fibrosis Pathoanatomical proof of ARPKD in an affected sibling Parenteral consanguinity

Notes:

^*To meet diagnostic criteria, patients must meet imaging criteria and at least one of the clinical criteria