Combined liver‐kidney transplantation for children with autosomal recessive polycystic kidney disease (ARPKD): indication and outcome. (15th April 2013)

• Record Type: Journal Article
• Authors: Brinkert, Florian; Lehnhardt, Anja; Montoya, Carmen; Helmke, Knut; Schaefer, Hansjoerg; Fischer, Lutz; Nashan, Bjoern; Bergmann, Carsten; Ganschow, Rainer; Kemper, Markus J.
• Is Part Of: Transplant international. Volume 26:Number 6(2013:Jun.)
• Page Start: 640
• Page End: 650
• Publication Date: 2013-04-15
• Subjects: Transplantation of organs, tissues, etc -- Periodicals; 617.95405
• DOI: 10.1111/tri.12098
• Languages: English
• ISSNs: 0934-0874

Abstract

<abstract abstract-type="main" xml:lang="en" id="tri12098-abs-0001"> <title>Abstract</title> <p>In ARPKD, mutations in the <italic>PKHD1</italic> gene lead to remodeling of the kidneys and liver. These may result in progressive liver fibrosis with portal hypertension requiring combined liver and kidney transplantation (CLKT). There is currently no consensus on the indication for CLKT and data on long‐term outcomes are scarce. We analyzed in detail the pretransplant liver symptomatology, laboratory and ultrasound data, histological studies, and genotypes in eight patients undergoing CLKT. The median age was 10.1 years (range 1.7–16) and median follow‐up was 4.6 years (range 1.1–8.9). All patients had clinical signs of portal hypertension and abnormal ultrasound findings. Congenital hepatic fibrosis was present in all pretransplant biopsies (6 out of 8 patients) and in all explanted livers. All patients survived; liver and kidney graft survival was 72% and 88%, respectively. Liver and kidney function were stable in all patients with a median eGFR of 70 ml/min/1.73 m² (range 45–108 ml/min/1.73 m²). Height‐SDS improved significantly after 12, 24, and 36 months (<italic>P </italic>= 0.016, 0.022 and 0.018 respectively). The indication for CLKT remains challenging and controversial. A favorable outcome for patients with ARPKD can be achieved by using the degree of portal hypertension, longitudinal ultrasound examinations, and preoperative liver histology as parameters for CLKT.</p> </abstract>