Xu Dan, Chen Zhimin, Chen Huizhong, Huang Rongyan, Zhao Shunying, Liu Xiuyun, Zhou Chunju, Peng Yun, Yuan Xinyu, Zou Jizhen, Zhang Hailing, Zhao Deyu, Liu Enmei, Zheng Yuejie, Zhong Lili, Lu Min, Lu Jirong, Nong Guangmin
Department of Pulmonology, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Department of Respiratory Diseases, Children's Hospital, Affiliated to the Capital Institute of Pediatrics, Beijing, China.
PLoS One. 2015 Jan 8;10(1):e0116930. doi: 10.1371/journal.pone.0116930. eCollection 2015.
Diffuse parenchymal lung diseases in children (chDPLD) or interstitial lung diseases in children (chILD) represent a heterogeneous group of respiratory disorders that are mostly chronic and associated with high morbidity and mortality. However, the incidence of chDPLD is so low that most pediatricians lack sufficient knowledge of chDPLD, especially in China. Based on the clinico-radiologic-pathologic (CRP) diagnosis, we tried to describe (1) the characteristics of chDPLD and (2) the ratio of each constituent of chDPLD in China. Data were evaluated, including clinical, radiographic, and pathologic results from lung biopsies. We collected 25 cases of chDPLD, 18 boys and 7 girls with a median age of 6.0 years, from 16 hospitals in China. The most common manifestations included cough (n = 24), dyspnea (n = 21), and fever (n = 4). There were three cases of exposure-related interstitial lung disease (ILD), three cases of systemic disease-associated ILD, nineteen cases of alveolar structure disorder-associated ILD, and no cases of ILD specific to infancy. Non-specific interstitial pneumonia (n = 9) was the two largest groups.
Non-specific interstitial pneumonia is the main categories of chDPLD in China. Lung biopsy is always a crucial step in the final diagnosis. However, clinical and imaging studies should be carefully evaluated for their value in indicating a specific chDPLD.
儿童弥漫性实质性肺疾病（chDPLD）或儿童间质性肺疾病（chILD）是一组异质性的呼吸系统疾病，大多为慢性，且发病率和死亡率较高。然而，chDPLD的发病率很低，以至于大多数儿科医生对其了解不足，在中国尤其如此。基于临床-放射-病理（CRP）诊断，我们试图描述（1）chDPLD的特征以及（2）中国chDPLD各组成部分的比例。对数据进行了评估，包括肺活检的临床、影像学和病理结果。我们从中国16家医院收集了25例chDPLD病例，其中18例为男孩，7例为女孩，中位年龄为6.0岁。最常见的表现包括咳嗽（n = 24）、呼吸困难（n = 21）和发热（n = 4）。有3例与暴露相关的间质性肺疾病（ILD）、3例与全身性疾病相关的ILD、19例与肺泡结构紊乱相关的ILD，无婴儿期特有的ILD病例。非特异性间质性肺炎（n = 9）是最大的两个类别。
非特异性间质性肺炎是中国chDPLD的主要类型。肺活检始终是最终诊断的关键步骤。然而，应仔细评估临床和影像学研究在提示特定chDPLD方面的价值。
