Interstitial lung disease in children

Abstract Children's interstitial lung disease (ILD) includes a wide range of rare respiratory disorders associated with high morbidity and mortality. Genetic factors, systemic disease processes, nonspecific inflammatory or fibrotic patterns of repair seen in a number of clinical settings are in...

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Bibliographic Details
Published in:Early human development 2013-10, Vol.89, p.S39-S43
Main Authors: Cazzato, Salvatore, di Palmo, Emanuela, Ragazzo, Vincenzo, Ghione, Silvia
Format: Article
Language:English
Subjects:
Advanced Basic Science
Bronchiolitis obliterans
Child
Child, Preschool
Computed tomography
Diffuse lung disease
Humans
Infant
Interstitial lung disease
Lung Diseases, Interstitial - congenital
Lung Diseases, Interstitial - diagnosis
Lung Diseases, Interstitial - etiology
Lung Diseases, Interstitial - therapy
Neonatal and Perinatal Medicine
Surfactant
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Summary:Abstract Children's interstitial lung disease (ILD) includes a wide range of rare respiratory disorders associated with high morbidity and mortality. Genetic factors, systemic disease processes, nonspecific inflammatory or fibrotic patterns of repair seen in a number of clinical settings are involved in the ILD pathogenesis. Specific disorders more prevalent in young children include diffuse developmental disorders, alveolar growth abnormalities, genetic surfactant disorders, pulmonary interstitial glycogenosis and neuroendocrine cell hyperplasia of infancy. It may be difficult to recognize these entities and this can lead to delayed treatment. The diagnostic approach is based on a combination of history/physical examinations, imaging studies, pulmonary function testing, genetic testing, bronchoalveolar lavage (BAL) and in most cases an open lung biopsy. Although some disease types overlap with those seen in adults, in this review emphasis is placed on entities unique to the pediatric population focusing on clinical characteristics, histologic definitions, radiologic–pathologic correlation and therapeutic strategies.
ISSN:0378-3782
1872-6232
DOI:10.1016/j.earlhumdev.2013.07.021