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先天性膈疝在囊性纤维化患者中的迟发表现。

Late presentation of congenital diaphragmatic hernia in patients with cystic fibrosis.

机构信息

Division of Pulmonary Medicine, Department of Pediatrics, The Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania 15201, USA.

出版信息

Pediatr Pulmonol. 2010 Apr;45(4):403-5. doi: 10.1002/ppul.21173.

Abstract

Late presentation of congenital diaphragmatic hernia (CDH) is unusual, especially in patients with cystic fibrosis (CF). To our knowledge, cases of CDH in CF patients and the combined effects on lung function have not been previously described. Here we report two cases of late presenting CDH in CF patients and describe effects on lung function. Late presentation of CDH in CF patients can cause gastrointestinal or respiratory symptoms and requires a high index of suspicion as well as proper interpretation of imaging. In patients with CF and CDH, lung function abnormalities could include obstructive, restrictive defects, or combined defects.

摘要

先天性膈疝(CDH)的迟发表现并不常见,尤其是在囊性纤维化(CF)患者中。据我们所知,CF 患者的 CDH 病例及其对肺功能的综合影响以前尚未描述过。在这里,我们报告了两例 CF 患者的迟发性 CDH 病例,并描述了对肺功能的影响。CF 患者的 CDH 迟发表现可引起胃肠道或呼吸道症状,需要高度怀疑,并对影像学进行正确解读。在 CF 合并 CDH 的患者中,肺功能异常可能包括阻塞性、限制性缺陷或混合性缺陷。

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