Prominent Intrapulmonary Bronchopulmonary Anastomoses and Abnormal Lung Development in Infants and Children with Down Syndrome
Prominent Intrapulmonary Bronchopulmonary Anastomoses and Abnormal Lung Development in Infants and Children with Down Syndrome
复制标题
DOI：
10.1016/j.jpeds.2016.08.063
复制
发表时间：
2017-01-01
影响因子：
5.1
通讯作者：
Galambos, Csaba
中科院分区：
文献类型：
--
作者：
Bush, Douglas;Abman, Steven H.;Galambos, Csaba
目的确定受损的肺血管和肺泡发育的组织学特征的频率，并确定存在肺内支气管肺结核病（IBA）的婴儿和儿童谁死于Down syndrome.Study Design的尸检报告和肺组织学的回顾性审查13名儿童唐氏综合征（年龄：0-8岁）进行。对异常肺发育的组织学特征进行了鉴定和半定量，包括IBA的存在。还进行了IBA的三维重建。与4例年龄匹配的先天性心脏病患者进行尸检，在此时间periods.Results唐氏综合征，69%死于心脏事件，77%有先天性心脏病，46%有肺动脉高压的临床诊断。所有唐氏综合征患者的肺组织学表现为肺泡简化，92%的患者在远端肺中存在双毛细血管网络。唐氏综合征受试者的肺经常具有肺动脉高压重构的特征（85%），并且在所有唐氏综合征受试者中观察到突出的支气管血管和IBA。这些功能更频繁的唐氏综合征与对照subjects.Conclusions儿童唐氏综合征谁死于心肺疾病往往有受损的肺泡和血管发育的组织学证据，包括存在突出的IBA和肺动脉高压。我们推测唐氏综合征患儿存在肺表面积减少和IBA募集的风险，这可能会使唐氏综合征患者的气体交换恶化。
Objectives To determine the frequency of histologic features of impaired lung vascular and alveolar development and to identify the presence of intrapulmonary bronchopulmonary anastomoses (IBA) in infants and children who died with Down syndrome.Study design A retrospective review of autopsy reports and lung histology from 13 children with Down syndrome (ages: 0-8 years) was performed. Histologic features of abnormal lung development were identified and semiquantified, including the presence of IBA. Three-dimensional reconstructions of IBA were also performed. Comparisons were made with 4 age-matched patients without Down syndrome with congenital heart defects who underwent autopsies during this time period.Results Of the 13 subjects with Down syndrome, 69% died from cardiac events, 77% had a congenital heart defect, and 46% had a clinical diagnosis of pulmonary hypertension. Lung histology from all subjects with Down syndrome demonstrated alveolar simplification, and 92% had signs of persistence of a double capillary network in the distal lung. The lungs from the subjects with Down syndrome frequently had features of pulmonary arterial hypertensive remodeling (85%), and prominent bronchial vessels and IBA were observed in all subjects with Down syndrome. These features were more frequent in subjects with Down syndrome compared with control subjects.Conclusions Children with Down syndrome who died of cardiopulmonary diseases often have histologic evidence of impaired lung alveolar and vascular development, including the presence of prominent IBA and pulmonary hypertension. We speculate that children with Down syndrome are at risk for reduced lung surface area and recruitment of IBA, which may worsen gas exchange in subjects with Down syndrome.