title: 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 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.
authors: Department of Pediatrics, University of Bologna, S. Orsola-Malpighi Hospital Bologna, Italy. Electronic address: salvatore_cazzato@aosp.bo.it.
authors: Salvatore
authors: S
authors: Cazzato
authors: Emanuela
authors: E
authors: di Palmo
authors: Vincenzo
authors: V
authors: Ragazzo
authors: Silvia
authors: S
authors: Ghione
mesh_terms: D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D017563:Lung Diseases, Interstitial / Q000151:congenital / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy
keywords: Bronchiolitis obliterans; Computed tomography; Diffuse lung disease; Interstitial lung disease; Surfactant