Couetil J P, Soubrane O, Houssin D P, Dousset B E, Chevalier P G, Guinvarch A, Loulmet D, Achkar A, Carpentier A F
Department of Cardiovascular Surgery, Broussais Hospital, Paris, France.
Transpl Int. 1997;10(1):33-9. doi: 10.1007/BF02044339.
Between June 1990 and September 1995, 8 of 24 children with cystic fibrosis (CF) who were accepted either for combined transplantation or isolated liver transplantation died while waiting for a graft; 11 underwent transplantation and 5 are currently on the waiting list. Of the 11 children who had surgery, 7 (group 1) underwent one of the following procedures: heart-lung-liver (n = 4), sequential double lung-liver (n = 2), or bilateral lobar lung from a split left lung and reduced liver (n = 1). During the same period, the four other children (group 2) underwent isolated liver transplantation (three full-size livers, one partial liver). There was one perioperative death in each group. Pulmonary infection was the most common cause of morbidity in group 1. Other complications in group 1 included tracheobronchial stenosis (n = 2), biliary stricture (n = 2), and severe ascites (n = 2). All were successfully treated. Obliterative bronchiolitis developed in three patients. This was treated with FK 506. In group 2, pulmonary function tests improved or remained stable after liver transplantation. Surgical complications in group 2 included severe ascites (n = 1), biliary stricture (n = 1), and abscess of the liver (n = 1). Actuarial survival was 85.7% +/- 2% in group 1 at 1 year; it remained unchanged at 3 years and was 64.2% at 5 years.
1990年6月至1995年9月期间，24名接受联合移植或单纯肝移植的囊性纤维化（CF）患儿中，有8名在等待移植器官时死亡；11名接受了移植手术，5名目前仍在等待名单上。在接受手术的11名儿童中，7名（第1组）接受了以下手术之一：心肺肝联合移植（n = 4）、序贯双肺-肝移植（n = 2）或取自劈开左肺的双侧肺叶及缩小肝移植（n = 1）。同期，另外4名儿童（第2组）接受了单纯肝移植（3例全肝移植，1例部分肝移植）。每组各有1例围手术期死亡。肺部感染是第1组最常见的发病原因。第1组的其他并发症包括气管支气管狭窄（n = 2）、胆管狭窄（n = 2）和严重腹水（n = 2）。所有这些并发症均得到成功治疗。3例患者发生了闭塞性细支气管炎，采用FK 506进行治疗。在第2组中，肝移植后肺功能测试有所改善或保持稳定。第2组的手术并发症包括严重腹水（n = 1）、胆管狭窄（n = 1）和肝脓肿（n = 1）。第1组1年时的精算生存率为85.7%±2%；3年时保持不变，5年时为64.2%。
