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
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.Pediatric Transplantation 06/2009; 13(7):940-2. DOI:10.1111/j.1399-3046.2009.01189.x · 1.44 Impact Factor
Chapter: Other Cystic Kidney DiseasesComprehensive Clinical Nephrology, 01/2010: pages 543-559; , ISBN: 9780323058766
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ABSTRACT: Solid organ transplantation has become the first line of treatment for a growing number of life-threatening pediatric illnesses. With improved survival, research into the long-term outcome of transplant recipients has become important to clinicians. Adherence to medical instructions remains a challenge, particularly in the adolescent population. New immunosuppressant approaches promise to expand organ transplantation in additional directions. Extension of transplantation into replacement of organs such as faces and hands raises complex ethical issues.Child and adolescent psychiatric clinics of North America 04/2010; 19(2):285-300, viii-ix. DOI:10.1016/j.chc.2010.02.002 · 2.88 Impact Factor
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