Whitehead B, Rees P, Sorensen K, Bull C, Higenbottam T W, Wallwork J, Fabre J, Elliott M, de Leval M
Hospital for Sick Children, London, U.K.
J Heart Lung Transplant. 1993 Nov-Dec;12(6 Pt 1):903-8.
Between June 1988 and February 1993, combined heart-lung transplantation was performed in 30 children and adolescents aged 3.6 to 18.6 years (mean, 12.2 years) at The Hospital for Sick Children in London. Original diagnoses included cystic fibrosis (n = 25), Eisenmenger's syndrome (n = 4), and chronic graft-versus-host disease of the lung (n = 1). Posttransplantation maintenance immunosuppression comprised a triple regimen, with methylprednisolone and antithymocyte globulin given perioperatively and for episodes of allograft rejection. Actuarial survival was 63% (95% confidence interval: 42%-78%) at 1 year and 48% (95% confidence interval: 27%-66%) at 3 years. Obliterative bronchiolitis has been diagnosed in 13 patients (43%). Actuarial freedom from obliterative bronchiolitis in survivors was 76%, 59%, and 37% at 12, 24, and 36 months after transplantation, respectively. Recipients in whom obliterative bronchiolitis developed within the first year (n = 6) had more episodes of pulmonary rejection during the first 6 months after transplantation (mean, 5.7 episodes per patient) than those in whom "premature" obliterative bronchiolitis did not develop (mean, 3.2 episodes per patient). Infection of the pulmonary allograft was implicated to a lesser extent in predisposing to obliterative bronchiolitis. At 2, 3, and 6 months, tracheal stenosis developed in three patients, all of whom died with obliterative bronchiolitis within 10 months of transplantation. Noncompliance with therapy was considered a contributory factor in producing obliterative bronchiolitis in four adolescent recipients. The high incidence of obliterative bronchiolitis observed in this pediatric cohort may have a multifactorial cause.
1988年6月至1993年2月期间，伦敦大奥蒙德街儿童医院对30名年龄在3.6至18.6岁（平均12.2岁）的儿童和青少年进行了心肺联合移植。原发病诊断包括囊性纤维化（25例）、艾森曼格综合征（4例）和慢性肺移植物抗宿主病（1例）。移植后维持免疫抑制采用三联疗法，围手术期及同种异体移植排斥发作时给予甲泼尼龙和抗胸腺细胞球蛋白。1年时的精算生存率为63%（95%置信区间：42%-78%），3年时为48%（95%置信区间：27%-66%）。13例患者（43%）被诊断为闭塞性细支气管炎。存活者移植后12、24和36个月时无闭塞性细支气管炎的精算概率分别为76%、59%和37%。在第一年发生闭塞性细支气管炎的受者（6例）在移植后前6个月的肺部排斥发作次数（平均每位患者5.7次）多于未发生“过早”闭塞性细支气管炎的受者（平均每位患者3.2次）。肺同种异体移植感染在闭塞性细支气管炎的易患因素中所占比例较小。2、3和6个月时，3例患者发生气管狭窄，均在移植后10个月内死于闭塞性细支气管炎。在4名青少年受者中，治疗依从性差被认为是导致闭塞性细支气管炎的一个因素。在这个儿科队列中观察到的闭塞性细支气管炎的高发病率可能有多种原因。
