Combined liver-kidney transplantation in a child with primary hyperoxaluria |
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| Authors: | Martin S. Polinsky Steven Dunn Bruce A. Kaiser Seth L. Schulman Barbara J. Wolfson I. Bruce Elfenbein H. J. Baluarte |
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| Affiliation: | (1) Division of Pediatric Nephrology, St. Christopher's Hospital for Children, Temple University School of Medicine, Erie Avenue at Front Street, 19134 Philadelphia, PA, USA;(2) Department of Surgery, St. Christopher's Hospital for Children, Temple University School of Medicine, Erie Avenue at Front Street, 19134 Philadelphia, PA, USA;(3) Department of Radiology, St. Christopher's Hospital for Children, Temple University School of Medicine, Erie Avenue at Front Street, 19134 Philadelphia, PA, USA;(4) Department of Pathology, St. Christopher's Hospital for Children, Temple University School of Medicine, Erie Avenue at Front Street, 19134 Philadelphia, PA, USA |
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| Abstract: | A 3.5-year-old boy presented with end-stage renal disease and bilateral nephrocalcinosis. Renal biopsy demonstrated marked parenchymal calcium oxalate deposition and a diagnosis of primary hyperoxaluria (PH) was made. Following 2 years of hemodialysis he received two renal allografts which were lost at 7 and 11 months, respectively, due to biopsy-proven recurrent oxalosis. Combined liver-kidney transplantation was then performed, after which renal and hepatic function initially stabilized. The patient died on the 28th postoperative day, of in fectious complications and progressive respiratory insufficiency. However, comparisons between the patterns of urinary oxalate excretion noted after the isolated renal and liver-kidney transplants indicated that, following the latter, successful biochemical correction of the enzyme defect responsible for type 1 PH had occurred. |
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| Keywords: | Liver-kidney transplantation Primary hyperoxaluria |
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