儿童气管支气管肿大的鉴别诊断难点

ya A. Il’ina, Lidiya E. Vorob’eva, Mayya D. Bakradze, A. S. Polyakova
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引用次数: 0

摘要

气管支气管肿大是一种罕见的儿童发育缺陷,以气管扩张、支气管粗大为特征,在成人实践中多作诊断和治疗,其临床诊断表现具有明显的Mounier-Kuhn综合征特征。在这方面,需要在儿童中早期发现这种病理,并将其与其他疾病和气管支气管树畸形区分开来,这些疾病和畸形掩盖了肺部和上呼吸道的复发性炎症过程,主要是囊性纤维化和原发性纤毛运动障碍等。本文报告一例继发性气管支气管肿大的儿童,临床表现明显为穆尼埃-库恩综合征,伴有支气管扩张、肺纤维化和全身性结缔组织病的征象。它讨论了胸部计算机断层扫描在诊断儿童这种缺陷的可能性,以及它的原因。将本例患者的气管和主支气管的大小与同年龄、性别的囊性纤维化或弥漫性肺纤维化患儿进行比较,以确定遗传性结缔组织疾病在继发性气管支气管肥大的发展中更显著的作用。作者建议将儿童气管支气管扩张和穆尼埃-库恩综合征的概念分开,并在这些+3SD或以上的患者中识别气管扩张的阈值。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Difficulties in the Differential Diagnosis of Tracheobronchomegaly in Children
Tracheobronchomegaly is a rare developmental defect in children, which is characterized by the expansion of the trachea and large bronchi, the diagnosis and treatment of which are made more often in adult practice, when the clinical diagnostic picture acquires the obvious features of Mounier-Kuhn syndrome. In this connection, the need arises for the early detection of this pathology in children and its differentiation from other diseases and malformations of the tracheobronchial tree, which mask a recurrent inflammatory process in the lung and upper respiratory tract, and primarily with those, such as cystic fibrosis and primary ciliary dyskinesia.The paper describes a case of secondary tracheobronchomegaly in a child with obvious clinical manifestations of Mounier-Kuhn syndrome in the presence of bronchiectasis, pulmonary fibrosis, and signs of systemic connective tissue disease. It discusses the possibilities of chest computed tomography in the diagnosis of this defect in children, as well as its causes. The sizes of the trachea and main bronchi in this patient are compared with those in the groups of children of the same age and sex who have cystic fibrosis or diffuse pulmonary fibrosis to identify a more significant role of hereditary connective tissue diseases in the development of secondary tracheobronchomegaly. The authors propose to separate the concepts of tracheobronchomegaly and Mounier-Kuhn syndrome in children and to recognize the threshold for tracheal expansion in these patients having +3SD or more.
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