Abstract
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. Conclusion: 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.
Highlights
IntroductionDiffuse 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 [1, 2]
Diffuse parenchymal lung diseases in children or interstitial lung diseases in children represent a heterogeneous group of respiratory disorders that are mostly chronic and associated with high morbidity and mortality [1, 2]. They are characterized by impaired gas exchange and diffuse infiltrates on chest radiology. Children with these disorders may present with cough and dyspnea with evidence of a restrictive ventilatory defect [1]
Patients subjected to high-resolution CT because of either chest X-ray suggesting diffuse parenchymal lung diseases, or disagreement between subtle changes on chest X-ray and severe symptoms; 3) patients having had lung biopsies
Summary
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 [1, 2]. They are characterized by impaired gas exchange and diffuse infiltrates on chest radiology. Children with these disorders may present with cough and dyspnea with evidence of a restrictive ventilatory defect [1]. The clinico-radiologic-pathologic (CRP) diagnosis requires pathologic evidence, and pathologic standards in children are very difficult to obtain
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