The Clinical Characteristics of Autosomal Recessive Polycystic Kidney Disease (ARPKD): An Update of the North American Experience

Abstract 1961 Poster Session II, Sunday, 5/2 (poster 188)

Background: ARPKD is one of the most common pediatric renal cystic diseases, yet limited clinical information is available to guide decision-making by clinicians and parents. More optimal patient management will require improved tools for prenatal diagnosis, detailed information about the clinical course, and identification of specific prognostic factors. While recent genetic studies have provided the basis for reliable, haplotype-based diagnosis in at-risk pregnancies, the natural history of ARPKD has not been well-characterized. The objective of the current investigation is to describe the baseline clinical features and survival characteristics of ARPKD patients drawn from tertiary care centers in the United States and Canada.

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