Maldevelopment of intrapulmonary bronchial cartilage in congenital diaphragmatic hernia
Maldevelopment of intrapulmonary bronchial cartilage in congenital diaphragmatic hernia
复制标题
先天性膈疝肺内支气管软骨发育不良
DOI：
10.1002/ppul.24799
复制
发表时间：
2020
影响因子：
3.1
通讯作者：
Itoh Kyoko
中科院分区：
文献类型：
--
作者：
Tando So;Sakai Kohei;Takayama Shohei;Fukunaga Kenji;Higashi Mayumi;Fumino Shigehisa;Aoi Shigeyoshi;Furukawa Taizo;Tajiri Tatsuro;Ogi Hiroshi;Itoh Kyoko
研究背景肺发育不良是先天性横隔疝(CDH)患儿发病和死亡的重要原因。本研究旨在验证我们的假设，即肺软骨发育异常和肺泡发育不成熟可能在CDH肺发育不良中起核心作用。方法对10例CDH患者尸检肺组织进行回顾性分析，并与9例年龄匹配的对照组进行比较，以评价CDH患者肺软骨和肺泡成熟度的变化。CDH患者同侧(左侧)支气管软骨至胸膜的最短距离明显缩短，与放射状肺泡计数(RAC)呈正相关。对于小于20 000 μm2的小支气管软骨，CDH组左肺平均软骨面积明显减少，右肺有减小趋势。另外，CDH患者左肺内直径小于2 0 0 μm的支气管周围软骨有变小的趋势。相反，对于直径为200~400 μm的支气管周围软骨，CDH患者左肺软骨总面积与支气管径的比值虽有较大差异，但有较大的差异。结论远端和近端支气管周围相反方向的软骨异常支持了支气管软骨发育异常在CDH肺发育不良中的重要作用。
BackgroundPulmonary hypoplasia is an important cause of morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). This study aimed to verify our hypothesis that the abnormal development of bronchial cartilage as well as alveolar immaturity, might play a central role in hypoplasia of the lung in human CDH.MethodWe retrospectively analyzed autopsied lungs from 10 CDH cases and compared with nine age‐matched controls to assess the bronchial cartilage and alveolar maturity using morphological techniques.ResultKi‐67 and thyroid transcription factor‐1 (TTF‐1) expression in the alveoli significantly increased in bilateral lungs with CDH. The shortest distance from the bronchial cartilage to the pleura was significantly shorter in ipsilateral (left) lungs with CDH, showing a positive correlation with the radial alveolar count (RAC). Regarding the small bronchial cartilages less than 20 000 μm2, the average cartilage area significantly decreased in left lungs with CDH, and tended to decrease in right lungs with CDH. In addition, cartilage around the bronchi less than 200 μm in diameter tended to be smaller in left lungs with CDH. In contrast, regarding the cartilage around the bronchi 200 to 400 μm in diameter, the ratio of the total cartilage area relative to the bronchial diameter tended to be higher in left lungs with CDH, although there was a large variation.ConclusionsThese opposite directional cartilage abnormalities around the distal and more proximal bronchi support our hypothesis that abnormal development of bronchial cartilage might play an important role in the hypoplastic lung in CDH.