Rapamycin-based rescue therapy after chronic rejection in a pediatric liver transplant patient
Rapamycin-based rescue therapy after chronic rejection in a pediatric liver transplant patient
复制标题
小儿肝移植患者慢性排斥反应后基于雷帕霉素的挽救治疗
作者：
J. Iglesias;J. Ortega;J. A. López;Luz Sánchez;E. Allende;M. Asensio;C. Margarit
Received: 1 July 2002 Revised: 13 March 2003 Accepted: 20 March 2003 Published online: 24 June 2003 Springer-Verlag 2003 Dear Editors: Liver transplantation is a therapeutic procedure accepted by the scientific community for the treatment of a range of end-stage liver diseases in all age groups. However, acute and chronic graft rejections remain major problems, which lead to re-transplantation in a large number of cases [1]. Much has been published on the management of chronic failure of the transplanted liver, and, thanks to the new immunosuppressive drugs now available, there are many therapeutic approaches [2, 3]. Rapamycin, a macrocyclic lactone produced by Streptomyces hygroscopicus, resembles tacrolimus structurally and binds the same immunophilin FK506 12-kDa binding protein (FKBP-12) [4], with potent immunosuppressive properties. Its mechanism of action lies in blocking the signal pathway between the IL-2 receptor and nucleus, thereby suppressing cellular proliferation of T lymphocytes and B lymphocytes without affecting the calcineurin pathway [5]. The use of this new immunosuppressive agent has been reported in several articles on adult liver, kidney, or pancreas transplantation [6, 7, 8, 9, 10, 11]; however, only two reports have been published on children with liver and kidney transplants [12, 13]. The case of a pediatric patient saved from chronic graft rejection by rapamycin-based therapy is described herein. The patient was a 12-year-old girl with a history of extrahepatic biliary atresia and Kasai porto-enterostomy before the age of 45 days. At 4 years of age she underwent orthotopic liver transplantation and suffered an episode of acute graft rejection 15 days after transplantation, which was managed with steroid pulses, and, because of steroid resistance, OKT 3 after 13 days, with good response. She was discharged and receiving immunosuppression therapy consisting of tacrolimus (FK506) and steroids, which were slowly tapered. She remained complication-free during 7 years of therapy with FK506 and with good blood trough concentrations until the age of 11 years, at which time she developed tonsillitis and cervical lymph node enlargement; malignancy was ruled out by biopsy. One month later, jaundice was observed. Laboratory values were: total bilirubin 40 mg/dl, direct bilirubin 29 mg/dl, AST 819 U/l, ALT 756 U/l, GGT 662 U/l, urea 36 mg/dl, and creatinine 0.3 mg/dl. Prothrombin time was always above 70%. Viral serology tests and CMV antigenemia were all negative. Anti-nuclear antibodies, anti-mitochondrial antibodies, liver-kidney microsomal antibodies, smooth muscle antibodies, peri-nuclear antineutrophil cytoplasmic antibodies, Transpl Int (2003) 16: 765–767 DOI 10.1007/s00147-003-0608-0 LETTER TO THE EDITORS
