Jouvet P, Hubert P, Jan D, Niaudet P, Beringer A, Narcy C, Daudon M, Broyer M, Revillon Y
Service de Réanimation Médicochirurgicale Pédiatrique Polyvalente, Hôpital des Enfants-Malades, Paris.
Arch Fr Pediatr. 1991 Nov;48(9):637-9.
Hyperoxaluria type I (HPI) is a metabolic disorder secondary to liver alanine glyoxylate aminotransferase deficiency. Renal failure occurs due to the excessive production and precipitation of oxalate in the kidney. Combined liver-renal transplantation is the correct treatment for this condition when end-stage renal failure occurs as with renal transplantation alone the risk of recurrence of the same pathology in the transplanted kidney would be high. We report the case of a 4 year-old child with HPI suffering from terminal renal failure in whom a hepato-renal transplantation was performed: six months later, creatinine clearance was 62 ml/min/1.73 m2 and liver function tests were normal.
I型高草酸尿症(HPI)是一种继发于肝脏丙氨酸乙醛酸转氨酶缺乏的代谢紊乱疾病。肾衰竭是由于草酸盐在肾脏中过度产生和沉淀所致。当终末期肾衰竭发生时,肝肾联合移植是治疗这种疾病的正确方法,因为仅进行肾移植时,移植肾中相同病理情况复发的风险会很高。我们报告了一例4岁患有HPI且处于终末期肾衰竭的儿童接受肝肾移植的病例:六个月后,肌酐清除率为62 ml/min/1.73 m²,肝功能检查正常。