Clinical analysis of etiology of 25 cases of multiple cystic lung disease in children

Geng Gang; Li Guiju; Luo Jian; Luo Zhengxiu; Liu Enmei; Fu Zhou

doi:10.13406/j.cnki.cyxb.000897

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Clinical analysis of etiology of 25 cases of multiple cystic lung disease in children

更新时间：2024-01-17

- Clinical analysis of etiology of 25 cases of multiple cystic lung disease in children
- Journal of Chongqing Medical University Vol. 41, Issue 9, Pages: 945-949(2016)
- 作者机构：
  
  重庆医科大学附属儿童医院呼吸科
- 作者简介：
- 基金信息：
- DOI：10.13406/j.cnki.cyxb.000897
  CLC： R725.6
- Published：2016，
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[1]耿刚,黎桂菊,罗健,罗征秀,刘恩梅,符州.25例儿童多发囊性肺疾病的临床特点分析[J].重庆医科大学学报,2016,41(09):945-949.

Geng Gang, Li Guiju, Luo Jian, et al. Clinical analysis of etiology of 25 cases of multiple cystic lung disease in children. [J]. Journal of Chongqing Medical University 41(9):945-949(2016)
[1]耿刚,黎桂菊,罗健,罗征秀,刘恩梅,符州.25例儿童多发囊性肺疾病的临床特点分析[J].重庆医科大学学报,2016,41(09):945-949. DOI： 10.13406/j.cnki.cyxb.000897.

Geng Gang, Li Guiju, Luo Jian, et al. Clinical analysis of etiology of 25 cases of multiple cystic lung disease in children. [J]. Journal of Chongqing Medical University 41(9):945-949(2016) DOI： 10.13406/j.cnki.cyxb.000897.

摘要

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

012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。

Abstract

Objective:To explore the etiology and clinical features of multiple cystic lung disease(MCLD)in children. Methods:The clinical data of 25 cases of MCLD in Children’s Hospital Affiliated to Chongqing Medical University from March 2004 to December2012 were retrospectively analyzed. Results:(1)The causes of MCLD in children were expressed as follows: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 systemwere expressed as follows: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%)

anemia in 6 cases(24%)

lymph nodes in 5 cases(20%)

hepatosplenomegaly in 7 cases(28%)

skin rash in 3 cases(12%)

clubbing in 1 case(4%).(3)Computed tomographyexamination was performed on 25 cases

and allwere showed MCLD.(4)Three cases of idiopathic pulmonary fibrosis(IPF)had pulmonary function tests

and the results suggested restrictive ventilatory defect. Conclusion:The common causes for children with MCLD in this study are BPD

LCH

IPF. The extra-pulmonary manifestations are various and specific and are important. Thoracic CT is an important tool for diagnosing MCLD

关键词

囊性肺疾病临床特点儿童

Keywords

Cystic lung diseaseClinical manifestationschildren

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