Renal function recovery in children undergoing combined liver kidney transplants.
ABSTRACT Combined liver kidney transplant (CLKT) is a recognized treatment option for end-stage renal disease due to primary hyperoxaluria (PH-I) and cystic disorders, yet there is only limited data on posttransplant renal function recovery. The objective of this study was to assess postoperative renal function of children with PH-I (group A) undergoing CLKT and to compare this with a cohort of children (group B) who received CLKT for other indications.
Twenty-three patients underwent CLKT between 1994 and 2008 (group A: 9 patients; median age 8.6 [1.6-16.7] years; group B: 14 patients; median age 8.5 [1.9-14.6] years). The median follow-up was 88 (14-112) and 22 (4-109) months. Both groups were transplanted with comparable organs. Eight (8/9) and six (6/14) patients received preoperative renal support in each group, respectively, whereas an equal proportion of them required early postoperative renal support (4/8; 50% and 3/6; 50%, respectively). Glomerular function was significantly different between groups until first year posttransplant (median estimated glomerular filtration rate: groups A vs. B; at pretransplant, 3 mo, 6 mo, and 12 mo posttransplant, respectively; 11.06 vs. 12.61 [P=0.4], 40.78 vs. 75.83 [P=0.03], 42.59 vs. 80.56 [P=0.04] and 53.57 vs. 76.75 [P=0.005]). Overall 1-year survival is 89% versus 90% and 5-year survival is 89% versus 62%, respectively.
Children with PH-I receiving CLKT seem to have delayed recovery of renal function compared with polycystic disease, possibly due to mobilization of systemic oxalate. Consideration should be given to earlier or preemptive transplantation for children with PH-I.
SourceAvailable from: Mikko P Pakarinen[Show abstract] [Hide abstract]
ABSTRACT: Simultaneous combined liver-kidney transplantation (CLKT) is a rare operation in pediatric patients so that annually only 10-30 operations are performed worldwide. The main indications for CLKT are primary hyperoxaluria type 1 and autosomal recessive polycystic kidney disease. In addition, CLKT is indicated in individual patients with metabolic or cirrhotic liver diseases and end-stage kidney disease. The surgery and immediate post-operative management of CLKT remain challenging in infants and small children. The patients should be operated on before they become severely ill or develop major systemic manifestations of their metabolic disorder. The liver allograft is immunologically protective of the kidney graft in simultaneous CLKT, often resulting in well-preserved kidney function. The long-term outcome after CLKT is nowadays comparable to that of isolated liver and kidney transplantations.Pediatric Nephrology 05/2013; 29(5). DOI:10.1007/s00467-013-2487-7 · 2.88 Impact Factor
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ABSTRACT: In ARPKD, mutations in the PKHD1 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 (P = 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.Transplant International 04/2013; DOI:10.1111/tri.12098 · 3.16 Impact Factor
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ABSTRACT: Primary hyperoxaluria type-1 (PH1) is a rare inherited autosomal recessive disorder in which a deficiency of the hepatic enzyme alanine-glyoxylate aminotransferase leads to endogenous oxalate overproduction, renal failure, systemic oxalate deposition and death. As hemodialysis provides insufficient oxalate clearance, patients ultimately require both liver and kidney transplantation for correction of the metabolic abnormality and oxalate excretion. Herein, we describe a young adult male with end-stage renal disease and systemic oxalosis causing progressive disabling multi-organ dysfunction while awaiting transplantation. We review the literature regarding liver-kidney transplantation and suggest that for patients with PH1, a standardized assessment of organ dysfunction and functional impairment may improve identification of patients requiring urgent transplantation thereby reducing the morbidity and mortality that can occur with delayed transplantation.08/2011; 2(3):126-32.