Pediatric Pulmonary Medicine, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas 77030, USA.
Pediatr Pulmonol. 2013 May;48(5):490-6. doi: 10.1002/ppul.22634. Epub 2012 Sep 4.
Pediatric diffuse lung diseases comprise a heterogeneous group of rare lung disorders which may lead to end stage lung disease and referral for lung transplantation. Previous studies are limited by small numbers of patients with specific forms of diffuse lung disease. Children with all forms of diffuse lung disease who underwent lung transplantation at two pediatric centers were evaluated in terms of several pre- and post-transplant factors and compared to children with other end stage lung disorders.
A retrospective chart review was performed on all patients transplanted between October 1, 2002 and June 15, 2007 at Texas Children's Hospital and St. Louis Children's Hospital. Multiple pre-transplant characteristics and post-transplant morbidities and mortality were compared between diffuse lung disease, cystic fibrosis, and pulmonary vascular disease groups.
There were 31 diffuse lung disease (DLD), 57 cystic fibrosis (CF), and 16 pulmonary vascular disease (PVD) patients included in our analysis. Patients with DLD had significantly higher pre-transplant morbidity including lower percent predicted of forced expiratory volume in first second (P = 0.013) and more patients with pulmonary hypertension (P = 0.001) and hypercapnia (P = 0.031). Compared to CF patients, more DLD and PVD patients required invasive ventilation (P = 0.001) and care in the pediatric intensive care unit (P = 0.001). After transplant, there was a difference among the three groups with regards to number of acute allograft rejections but statistical limitations preclude knowing between which group the difference lies. A difference in time to bronchiolitis obliterans was found between the PVD and CF groups but not when compared to the DLD patients. The three groups had similar time to post-transplant lymphoproliferative disease, rate of infections, and survival.
Lung transplantation is as successful for patients with end stage diffuse lung disease as compared to other lung transplant candidates.
儿科弥漫性肺疾病包括一组异质性的罕见肺部疾病，可能导致终末期肺病和肺移植的转诊。以前的研究受到特定形式弥漫性肺病患者数量少的限制。在两个儿科中心接受肺移植的所有形式弥漫性肺疾病患儿，根据多个移植前和移植后因素进行了评估，并与其他终末期肺病患儿进行了比较。
对 2002 年 10 月 1 日至 2007 年 6 月 15 日在德克萨斯儿童医院和圣路易斯儿童医院接受移植的所有患者进行了回顾性图表审查。比较弥漫性肺病、囊性纤维化和肺血管疾病组之间的多种移植前特征和移植后发病率和死亡率。
我们的分析包括 31 例弥漫性肺病（DLD）、57 例囊性纤维化（CF）和 16 例肺血管疾病（PVD）患者。DLD 患者在移植前发病率较高，包括用力呼气第一秒的预计百分比较低（P = 0.013）和更多的肺动脉高压（P = 0.001）和高碳酸血症（P = 0.031）患者。与 CF 患者相比，更多的 DLD 和 PVD 患者需要有创通气（P = 0.001）和儿科重症监护病房（P = 0.001）的护理。移植后，三组之间在急性同种异体移植物排斥的次数上存在差异，但统计学限制排除了差异所在的组。在 PVD 和 CF 组之间发现了闭塞性细支气管炎的时间差异，但与 DLD 患者相比则没有。三组在移植后淋巴增殖性疾病、感染率和存活率方面相似。
与其他肺移植候选者相比，终末期弥漫性肺病患者的肺移植同样成功。
