title: Liver disease in autosomal recessive polycystic kidney disease: clinical characteristics and management in relation to renal failure.
abstract: OBJECTIVE
  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.
authors.affiliation: *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.
authors.forename: Topi T
authors.initials: TT
authors.lastname: Luoto
authors.forename: Mikko P
authors.initials: MP
authors.lastname: Pakarinen
authors.forename: Timo
authors.initials: T
authors.lastname: Jahnukainen
authors.forename: Hannu
authors.initials: H
authors.lastname: Jalanko
mesh_terms: D000293:Adolescent; D000328:Adult; D001659:Biliary Tract / Q000473:pathology; D001660:Biliary Tract Diseases / Q000453:epidemiology / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D002761:Cholangitis / Q000453:epidemiology / Q000209:etiology; D004932:Esophageal and Gastric Varices / Q000453:epidemiology / Q000209:etiology; D005260:Female; D018197:Hepatoblastoma / Q000401:mortality / Q000601:surgery; D006801:Humans; D006971:Hypersplenism / Q000453:epidemiology / Q000209:etiology; D006975:Hypertension, Portal / Q000453:epidemiology / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000473:pathology / Q000601:surgery; D016030:Kidney Transplantation / Q000009:adverse effects; D008099:Liver / Q000473:pathology* / Q000601:surgery; D008107:Liver Diseases / Q000453:epidemiology / Q000209:etiology* / Q000601:surgery; D016031:Liver Transplantation / Q000009:adverse effects; D008297:Male; D017044:Polycystic Kidney, Autosomal Recessive / Q000150:complications* / Q000601:surgery; D051437:Renal Insufficiency / Q000209:etiology* / Q000601:surgery; D013163:Splenomegaly / Q000453:epidemiology / Q000209:etiology; D015996:Survival Rate; D055815:Young Adult