Skip Navigation LinksHome > August 2014 - Volume 59 - Issue 2 > Liver Disease in Autosomal Recessive Polycystic Kidney Disea...
Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0000000000000422
Original Articles: Hepatology and Nutrition

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

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Abstract

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.

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

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