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.
"However, mobilization of tissue oxalate and continued hyperoxaluria for months or years post-transplant can lead to renal allograft damage [2,10]. One series of pediatrics simultaneous liver-kidney transplants demonstrated decreased renal function at 3, 6 and 12 months post-transplantation in patients with PH1 compared to those with other diagnoses . "
[Show abstract][Hide abstract] 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.
International Journal of Organ Transplantation Medicine 08/2011; 2(3):126-32.
[Show abstract][Hide abstract] ABSTRACT: jGRASP is an integrated development environment that provides automatic generation of visualizations to improve the comprehensibility of software. These visualizations, which are particularly well suited for CS1 and CS2 using Java, include Control Structure Diagrams, UML Class Diagrams, and dynamic Object Views for data structures such as stacks, queues, linked lists, and binary trees. The workshop will provide an in-depth introduction to jGRASP and show how to use the visualizations to improve the learning experience. Participants are encouraged to bring programs from their own courses and experiment with the visualizations for these during the workshop. jGRASP is freely available (http://www.jgrasp.org/).
[Show abstract][Hide abstract] ABSTRACT: Liver transplantation is curative, life saving or both for a range of inherited diseases affecting the liver. Indications, timing and outcome of transplantation for these diseases are the focus of this review.
Liver transplant represents a mode of gene replacement therapy for several disorders, including Wilson disease, hemochromatosis, tyrosinemia, urea cycle defects and hypercholesterolemia in which the primary defect residing in the liver results in hepatic complications or severe extrahepatic disease. Liver transplant is also an important therapeutic modality in multisystemic genetic disorders with major hepatic disease such as glycogen storage disease types I, III and IV and porphyria. For familial amyloidosis and primary hyperoxaluria, liver replacement eliminates the source of the injurious products that results in extrahepatic disease. Innovations in medical and surgical management of these patients have led to improved outcomes providing an important benchmark for future gene therapy of these disorders.
Recent developments have refined the indications for liver transplant in the treatment of inherited metabolic diseases. The full potential of liver transplant in these disorders can be harnessed by careful patient selection, optimizing timing and perioperative metabolic management of these patients.
Current opinion in organ transplantation 06/2010; 15(3):269-76. DOI:10.1097/MOT.0b013e3283399dbd · 2.88 Impact Factor
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