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目的:探讨儿童多发囊性肺疾病(multiple cystic lung disease,MCLD)的临床特点。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(临床表现、影像学资料、肺功能检查、病理活检等)。结果:125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%)。225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。43例特发性肺纤维化(idiopathic pulmonary fibrosis,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的常见病因为支气管肺发育不良(bonchopulmonary dysplasia,BPD)、朗格汉斯细胞组织细胞增生症(lngerhans cell histiocytosis,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。
Objective: To investigate the clinical features of multiple cystic lung disease (MCLD) in children. Methods: The clinical data (clinical manifestations, imaging data, pulmonary function tests, biopsy, etc.) of 25 children with MCLD admitted to our hospital from March 2004 to December 2012 were retrospectively analyzed. RESULTS: In 125 children with MCLD, 12 (48%) had bronchopulmonary dysplasia, 7 had Langerhans cell histiocytosis (28%), 3 had idiopathic pulmonary fibrosis (12%), and the etiology was unknown 3 cases (12%). Respiratory system performance in 225 children: 22 cases (88%) of shortness of breath, 16 cases (64%) of persistent or repeated coughs, and 15 cases (60%) of dyspnea. Extrapulmonary manifestations: 3 cases (12%) of ear empyema, 2 cases of head mass (8%), 6 cases of anemia (24%), 5 cases of lymph node enlargement (20%), 7 cases of hepatosplenomegaly 28%), rash in 3 cases (12%) and clubbing in 1 case (4%). 3 chest CT cystic lesions. Forty-three children with idiopathic pulmonary fibrosis (IPF) performed pulmonary function tests, all of which suggested limited ventilatory dysfunction. Conclusions: The common causes of MCLD in children are diagnostic value of bronchopulmonary dysplasia (BPD), Langerhans cell histiocytosis (LCH) and IPF. Chest CT is an important means of diagnosis and differential diagnosis of cystic lung disease.