Polinsky M S, Dunn S, Kaiser B A, Schulman S L, Wolfson B J, Elfenbein I B, Baluarte H J
Division of Pediatric Nephrology, St. Christopher's Hospital for Children, Temple University School of Medicine, Philadelphia, PA 19134.
Pediatr Nephrol. 1991 May;5(3):332-4. doi: 10.1007/BF00867495.
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 infectious 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.
一名3.5岁男孩出现终末期肾病和双侧肾钙质沉着症。肾活检显示实质内有明显的草酸钙沉积，诊断为原发性高草酸尿症（PH）。经过2年的血液透析后，他接受了两次肾移植，但分别在7个月和11个月时因活检证实的复发性草酸沉着症而移植肾失功。随后进行了肝肾联合移植，术后肾和肝功能最初保持稳定。患者在术后第28天死于感染性并发症和进行性呼吸功能不全。然而，对单纯肾移植和肝肾联合移植后尿草酸排泄模式的比较表明，在后者之后，负责1型PH的酶缺陷已得到成功的生化纠正。
