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Liver Disease in Autosomal Recessive Polycystic Kidney Disease: Clinical Characteristics and Management in Relation to Renal Failure

Luoto, Topi T.*; Pakarinen, Mikko P.*; Jahnukainen, Timo; Jalanko, Hannu

Journal of Pediatric Gastroenterology & Nutrition: August 2014 - Volume 59 - Issue 2 - p 190–196
doi: 10.1097/MPG.0000000000000422
Original Articles: Hepatology and Nutrition

Objectives: We correlated liver and kidney manifestations in a national cohort of patients with autosomal recessive polycystic kidney disease (ARPKD).

Methods: A total of 27 consecutive patients with ARPKD were included. Hepatobiliary disorders were comparatively evaluated in 2 groups: children in group 1 (n = 10) displayed renal failure as infants and those in group 2 (n = 17) had normal kidney function through the first year of life.

Results: Median follow-up time was 10.6 (range, 0.4–40) years. Portal hypertension was diagnosed in 13 patients (48%) at the median age 5.0 (1.5–27.9) years. Esophageal varices developed in 8 patients (30%) at age 8.0 (2.1–11.9) years; 4 patients (15%) had variceal bleeding, and hypersplenism/splenomegaly occurred in 52%, similarly in both groups. Biliary tract dilatation was detected at 2.8 years in group 1 and at 7.9 years in group 2, significantly more frequently in group 1 (60% vs 18%, P = 0.039), causing cholangitis in 2 (20%) versus none in group 2 (P = 0.055). A total of 10 patients (37%) underwent cadaveric liver transplantation (LT) at a median age of 6.6 (1.0–20.0) years. In 1 patient LT was performed because of hepatoblastoma. Nine of these were combined liver–kidney transplantations (CLKT). Patients in group 1 required LT earlier (4.1 years vs 18.2 years, P = 0.017) and more frequently (70% vs 18%, P = 0.01). Overall survival beyond neonatal period was 85%. Two patients died because of infectious complications after CLKT, and 1 patient because of recurrent hepatoblastoma.

Conclusions: Although correlation of renal and liver manifestations was variable, biliary dilatation was associated with early renal failure. CLKT may be a treatment for patients with ARPKD with marked hepatobiliary complications.

*Section of Pediatric Surgery, Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki and Helsinki University Central Hospital

Department of Pediatric Nephrology and Transplantation, Children's Hospital, University of Helsinki and Helsinki University Central Hospital, Finland.

Address correspondence and reprint requests to Topi T. Luoto, Section of Pediatric Surgery, Children's Hospital, Stenbäckinkatu 11, PL 281, 00029-HUS, Helsinki, Finland (e-mail:

Received 5 November, 2013

Accepted 29 April, 2014

The study was supported by the Foundation for Pediatric Research, the Helsinki University Central Hospital Fund, and the Sigrid Juselius Foundation.

The authors report no conflicts of interest.

© 2014 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,