Hyperplasia of pulmonary neuroendocrine cells in a case of childhood pulmonary emphysema
Hyperplasia of pulmonary neuroendocrine cells in a case of childhood pulmonary emphysema
复制标题
DOI：
10.1378/chest.112.2.553
复制
发表时间：
1997-08-01
期刊：
影响因子：
9.6
通讯作者：
Canny, G
中科院分区：
文献类型：
--
作者：
Alshehri, M;Cutz, E;Canny, G
肺气肿是非常罕见的儿童在第一个十年的生活。文献中记录的少数病例均为α（1）-抗胰蛋白酶缺乏所致。我们提出的情况下，一个6岁的白色男孩与慢性咳嗽和呼吸困难的努力。肺活检显示全腺泡型肺气肿与开放的气道和弥漫性增生的肺神经内分泌细胞后，发现蛙皮素，这些细胞产生的肽免疫染色。我们推测，特发性弥漫性增生的蛙皮素产生的肺神经内分泌细胞可能有助于不寻常的慢性阻塞性肺疾病的发病机制在儿童。
Pulmonary emphysema is very uncommon in children in the first decade of life. The few cases documented in the literature were all due to alpha(1)-antitrypsin deficiency. We present the case of a 6-year-old white boy with chronic cough and dyspnea on exertion. Lung biopsy showed panacinar type emphysema with patent airways and diffuse hyperplasia of pulmonary neuroendocrine cells revealed after immunostaining for bombesin, a peptide produced by these cells. We speculate that idiopathic diffuse hyperplasia of bombesin-producing pulmonary neuroendocrine cells may contribute to the pathogenesis of unusual COPD in childhood.