25例儿童多发囊性肺疾病病因的临床分析

The clinical analysis of etiology of 25 cases of multiple cystic lung disease in children

摘要:

【摘要】目的：探讨儿童多发囊性肺疾病（Multiple cystic lung disease，MCLD）的病因及临床特点。方法：回顾性分析我院2004年3月~2012年12月收治的25例MCLD患儿的临床资料。结果：1、25例儿童MCLD中，支气管肺发育不良12例（48%），朗格汉斯细胞组织细胞增生症7例（ 28%），特发性肺纤维化3例（12%），病因不明3例（12%）。2、25例患儿呼吸系统表现：气促22例（88%），持续或反复咳嗽16例（64%），呼吸困难15例（60%）。肺外表现：耳溢脓3例（12%），头部包块2例（8%），贫血6例（24%），淋巴结肿大5例（20%），肝脾肿大7例（28%），皮疹3例（12%），杵状指1例（4%）。3、胸部CT均有囊腔病变。4、3例IPF的患儿行肺功能检查，结果均提示有限制性通气功能障碍。结论：儿童MCLD的常见病因为BPD、LCH、IPF，其伴随的肺外表现有诊断价值。胸部CT是诊断及鉴别囊性肺疾病的重要手段。

Abstract:

【Abstract】 Objective：To explore the etiology and clinical features of Multiple cystic lung disease (MCLD) in children. Methods：Retrospective analysis of the clinical data of 25 cases of MCLD in Children's Hospital Affiliated to Chongqing University of Medical Sciences from March 2004 to December 2012. Results：(1) The causes of MCLD in children were: 12 cases (48%) with Bronchopulmonary dysplasia (BPD), 7 cases (28%) with Langerhans cell histiocytosis (LCH), 3 cases (12%) with idiopathic pulmonary fibrosis, and 3 case of unknown reason. (2) Clinical manifestations of respiratory system: 22 cases (88%) had tachypnea, 16 cases (64%) had cough , 15 cases (60%) had dyspnea, Extrapulmonary manifestations were various and specific: ear pus overflow in 3 cases (12%), head mass in 2 cases (8%), 6 patients with anemia (24%),lymph nodes in 5 cases (20%), 7 (28%) cases with hepatosplenomegaly, 3 cases of skin rash (12%), clubbing in 1 cases(4%). (3) 25 cases were had Computed tomography(CT) examination, and were all showed MCLD . (4) 3 cases of IPF had pulmonary function tests, the results suggestive restrictive ventilatory defect. Conclusion：(1) The most causes for children with MCLD in this study were BPD, LCH, IPF. (2) The common clinical manifestations of MCLD in children was respiratory system, and were unspecific, but extra-pulmonary manifestations were various and specific, they were important value in diagnosis of MCLD. (3) The diagnosis of MCLD should combine with the history, extrapulmonary manifestations, pulmonary function tests and chest CT. (4) Histopathological examination is a diagnosis tool of some disease, if the disease could not diagnosis through the clinical manifestations and image features, the pathological biopsy should be performed.