Armitage J M, Fricker F J, Kurland G, Hardesty R L, Michaels M, Morita S, Starzl T E, Yousem S A, Jaffe R, Griffith B P
Department of Surgery, University of Pittsburgh School of Medicine, PA 15213.
J Thorac Cardiovasc Surg. 1993 Feb;105(2):337-45; discussion 346.
The application of lung transplantation to the pediatric population was a natural extension of the success realized in our adult transplantation program, which began in 1982. Twenty pediatric patients (age range 3 to 18 years) have had heart-lung (n = 11), double lung (n = 8), and single lung (n = 1) transplantation procedures. The causes of end-stage lung disease were primary pulmonary hypertension (n = 7), congenital heart disease (n = 5), cystic fibrosis (n = 4), pulmonary arteriovenous malformation (n = 2), graft-versus-host disease (n = 1), and desquamative interstitial pneumonitis (n = 1). Four (20%) patients had thoracic surgical procedures before the transplantation operation. The survival was 80% at a mean follow-up of 2 years. Immunosuppressive drugs included cyclosporine (n = 9) or FK 506 (n = 11) based therapy with azathioprine and steroids. Children were followed up by means of spirometry, transbronchial biopsy, and primed lymphocyte testing of bronchoalveolar lavage fluid. The mean number of treated episodes of rejection was 1.4 at 30 days, 0.5 at 30 to 90 days, and 1.4 at more than 90 days, and the first treated rejection episode occurred on average 28 days after the operation. Obliterative bronchiolitis developed in four (25%) of 16 patients surviving more than 100 days. Results of pulmonary function tests have remained good in almost all recipients. The greatest infectious risk was that of cytomegalovirus: one death and one case of pneumonia. Posttransplantation lymphoproliferative disease was diagnosed in two (12.5%) patients; both recovered. The most common complications were hypertension (25%) and postoperative bleeding (15%). Early results indicate that lung transplantation is a most promising therapy for children with severe vascular and parenchymal lung disease.
肺移植应用于儿科患者是我们始于1982年的成人移植项目所取得成功的自然延伸。20名儿科患者（年龄范围3至18岁）接受了心肺移植（n = 11）、双肺移植（n = 8）和单肺移植（n = 1）手术。终末期肺病的病因包括原发性肺动脉高压（n = 7）、先天性心脏病（n = 5）、囊性纤维化（n = 4）、肺动静脉畸形（n = 2）、移植物抗宿主病（n = 1）和脱屑性间质性肺炎（n = 1）。4名（20%）患者在移植手术前接受过胸外科手术。平均随访2年时生存率为80%。免疫抑制药物包括以环孢素（n = 9）或FK 506（n = 11）为基础的治疗方案，联合硫唑嘌呤和类固醇。通过肺量计、经支气管活检以及支气管肺泡灌洗液体外预激淋巴细胞检测对儿童进行随访。移植后30天平均治疗排斥发作次数为1.4次，30至90天为0.5次，90天以上为1.4次，首次治疗排斥发作平均发生在术后28天。16名存活超过100天的患者中有4名（25%）发生了闭塞性细支气管炎。几乎所有接受者的肺功能测试结果都保持良好。最大的感染风险是巨细胞病毒感染：1例死亡和1例肺炎。2名（12.5%）患者被诊断为移植后淋巴细胞增生性疾病；两人均康复。最常见的并发症是高血压（25%）和术后出血（15%）。早期结果表明，肺移植对于患有严重血管性和实质性肺病的儿童是一种非常有前景的治疗方法。
