Combined liver-kidney transplantation in children: Indications and outcome

Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA.
Pediatric Transplantation (Impact Factor: 1.44). 01/2009; 12(8):835-46. DOI: 10.1111/j.1399-3046.2008.01041.x
Source: PubMed


Although it remains a relatively infrequent procedure in children, CLKT has become a viable option for a select group of pediatric patients with severe liver and kidney disease. Most are performed for rare primary diseases such as PH1, but a selected few are performed in the setting of concomitant hepatic and renal failure of uncertain etiology and prognosis. This article reviews the indications for and outcomes following CLKT in children. While it focuses on the specific primary diseases which impact liver and kidney function simultaneously, it addresses the indications based on concomitant hepatic and renal failure, such as seen in the hepatorenal syndrome, as well.

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    ABSTRACT: PH1 is a metabolic disorder characterized by urolithiasis and the accumulation of oxalate crystals in the kidneys and other organs. Although patients often first present with renal failure, PH1 results from a deficiency of the hepatic peroxisomal enzyme AGT. Ultimately only liver transplantation will cure the underlying metabolic defect. Herein, we report the case of a three-month-old male infant diagnosed with PH and treated using a combined liver and en bloc-kidney transplant from a single donor. At the time of transplant, the patient was 11 months old and weighed 7.9 kg. He received a full size liver graft and en bloc kidneys from a two-yr-old donor. At 36 months post-transplant, the patient is steadily growing with normal renal and hepatic function. This is one of the first reports of successful liver and en bloc-kidney transplantation with abdominal compartment expansion by PTFE for the infantile form of PH1 in a high risk child before one yr of age. Prompt diagnosis and early referral to a specialized center for liver and kidney replacement offer the best chance for survival for infants with this otherwise fatal disease.
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    ABSTRACT: Since the implementation of the model for end-stage liver disease scoring system for organ allocation in orthotopic liver transplantation in 2002, the number of combined liver and kidney transplantations (CLKTs) that have been performed in the USA has increased significantly. To standardize the evaluation and selection of CLKT candidates, consensus conferences were held in March 2006 and September 2007. In this article, we review the studies on CLKT, especially concentrating on studies published in 2008 and 2009, to assess the impact of the model for end-stage liver disease system and two consensus conferences. The hepatorenal syndrome, usually a reversible cause of renal failure, has to be differentiated from other causes of chronic kidney disease that are potentially nonreversible and mandate CLKT. Despite published guidelines, it still remains difficult to clearly delineate appropriate candidates for CLKT, especially when the cause of renal disease remains controversial. Performing renal biopsies might help in decision-making. Chronic kidney disease patients with glomerular filtration rate less than 30 ml/min, hepatorenal syndrome patients with requirement of renal replacement therapy more than 8-12 weeks, and patients with renal biopsy findings of more than 30% fibrosis and glomerulosclerosis would get benefit receiving CLKT. In this era of organ shortage, with tens of thousands of patients listed for kidney transplantation, it is paramount that the organs should be scrupulously allocated to those in real need. However, patients with advanced renal disease should not receive orthotopic liver transplantation alone, which significantly decreases their survival.
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