Congenital hepatic fibrosis and portal hypertension in autosomal dominant polycystic kidney disease.

ABSTRACT:

Objectives

Autosomal dominant (ADPKD) and recessive (ARPKD) polycystic kidney diseases are the most common hepatorenal fibrocystic diseases (ciliopathies). Characteristics of liver disease of these disorders are quite different. All of the patients with ARPKD have congenital hepatic fibrosis (CHF) often complicated by portal hypertension. In contrast, typical liver involvement in ADPKD is polycystic liver disease, although rare atypical cases with CHF are reported. Our goal was to describe the characteristics of CHF in ADPKD.

Patients and methods

As a part of an intramural study of the National Institutes of Health on ciliopathies (www.clinicaltrials.gov, trial NCT00068224), we evaluated 8 patients from 3 ADPKD families with CHF. We present their clinical, biochemical, imagi

SUBMITTER: O'Brien K 

PROVIDER: S-EPMC8366680 | biostudies-literature | 2012 Jan

REPOSITORIES: biostudies-literature