Mendeloff E N
Department of Surgery, Washington University School of Medicine, St. Louis, MO, USA.
Semin Thorac Cardiovasc Surg. 1998 Jul;10(3):202-12. doi: 10.1016/s1043-0679(98)70038-3.
Cystic fibrosis (CF) is an inherited disease in which the fundamental physiological defect is failure of cAMP regulation of chloride transport. More than 90% of patients with CF will die of chronic, suppurative, obstructive lung disease, with the median survival in the United States currently being 29 years of age. Currently, although other therapies are being aggressively investigated, bilateral lung transplantation offers the only hope for short-term and mid-term survival in patients with CF and end-stage pulmonary disease. Since 1989, 103 bilateral sequential lung transplants (BLT) for CF have been performed at our institution (46 pediatric, 48 adult, 9 redo) at a mean age of 21+/-10 years. Cardiopulmonary bypass was used in all but one pediatric (age <18) transplantation, and in 15% of adults. The hospital mortality rate was 4.9%, with 80% of early deaths related to infection. Bronchial anastomotic complications occurred with equal frequency in the pediatric and the adult populations (7.3%). One- and 3-year actuarial survival rates are 84% and 61%, respectively (no significant difference between pediatric and adult age groups; average follow-up 2.1+/-1.6 years). Mean forced expiratory volume in 1 second increased from 25%+/-9% pretransplantation to 79%+/-35% 1 year posttransplantation. Acute rejection occurred 1.7 times per patient-year, with the majority of these episodes taking place the first 6 months posttransplantation. Need for treatment of lower respiratory infections occurred 1.2 times per patient in the first year after transplantation. Actuarial freedom from bronchiolitis obliterans was 63% at 2 years and 43% at 3 years. Redo transplantation was performed only in the pediatric population, and was associated with an early mortality of 33%. Eight living donor transplants (4 primary transplants, 4 redo transplants) were performed with an early survival of 87.5%. Patients with end-stage CF can undergo BLT with morbidity and mortality comparable with that observed in pulmonary transplantation for other disease entities.
囊性纤维化（CF）是一种遗传性疾病，其基本生理缺陷是环磷酸腺苷（cAMP）对氯离子转运的调节功能失效。超过90%的CF患者将死于慢性化脓性阻塞性肺病，在美国，目前患者的中位生存期为29岁。目前，尽管其他治疗方法正在积极研究中，但双侧肺移植是CF和终末期肺病患者短期和中期生存的唯一希望。自1989年以来，我们机构已为CF患者进行了103例双侧序贯肺移植（BLT）（46例儿科患者、48例成人患者、9例再次移植），平均年龄为21±10岁。除1例儿科（年龄<18岁）移植外，所有移植均使用了体外循环，15%的成人移植使用了体外循环。医院死亡率为4.9%，80%的早期死亡与感染有关。儿科和成人患者支气管吻合口并发症的发生率相同（7.3%）。1年和3年的精算生存率分别为84%和61%（儿科和成人年龄组之间无显著差异；平均随访2.1±1.6年）。移植前1秒用力呼气量平均为25%±9%，移植后1年增加到79%±35%。急性排斥反应的发生率为每位患者每年1.7次，其中大多数发作发生在移植后的前6个月。移植后第一年，每位患者下呼吸道感染的治疗需求为1.2次。闭塞性细支气管炎的精算无病生存率在2年时为63%，在3年时为43%。再次移植仅在儿科患者中进行，早期死亡率为33%。进行了8例活体供体移植（4例初次移植，4例再次移植），早期生存率为87.5%。终末期CF患者可以接受BLT，其发病率和死亡率与其他疾病实体的肺移植相当。
