title: Success of kidney transplantation in oxalosis is unrelated to residual hepatic enzyme activity.
abstract: We evaluated hepatic alanine glyoxylate aminotransferase (AGT) activity in percutaneous hepatic biopsy material obtained from four children with long-term renal allograft function following transplantation for primary hyperoxaluria type 1 (PH 1). The study was performed to determine whether these successes had occurred because relatively high residual levels of AGT activity had introduced a selection bias. The children ranged from seven months to eight years at transplant and are currently well 7 to 11 years later, with no oxalate deposition on repeated allograft biopsies and creatinine clearances of 80 to 128 ml/min/1.73 m2. AGT activity ranged from 0 to 13.8%, and in two of three patients with detectable levels the AGT was in mitochondria rather than peroxisomes. These results indicate that long-term renal allograft success can occur in spite of severe AGT deficiency. Thus, the therapeutic choice of kidney alone versus combined kidney-liver transplant cannot currently be made by measuring residual hepatic AGT in PH 1. Kidney transplant alone remains a reasonable initial therapeutic alternative for patients with recent onset of renal insufficiency due to PH 1.
authors: Department of Pediatrics, Shaare Zedek Medical Center, Jerusalem, Israel.
authors: A
authors: A
authors: Katz
authors: D
authors: D
authors: Freese
authors: C J
authors: CJ
authors: Danpure
authors: J I
authors: JI
authors: Scheinman
authors: S M
authors: SM
authors: Mauer
mesh_terms: D000293:Adolescent; D000410:Alanine Transaminase / Q000378:metabolism*; D002648:Child; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000150:complications / Q000201:enzymology / Q000601:surgery*; D007668:Kidney / Q000201:enzymology / Q000503:physiopathology; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000503:physiopathology / Q000601:surgery; D016030:Kidney Transplantation / Q000502:physiology
chemical_list: D000410:Alanine Transaminase