Childhood Interstitial Lung Diseases: An 18-year Retrospective Analysis
- 1 October 2013
- journal article
- case report
- Published by American Academy of Pediatrics (AAP) in Pediatrics
- Vol. 132 (4), 684-691
- https://doi.org/10.1542/peds.2013-1780
Abstract
Childhood interstitial lung diseases (ILD) occur in a variety of clinical contexts. Advances in the understanding of disease pathogenesis and use of standardized terminology have facilitated increased case ascertainment. However, as all studies have been performed at specialized referral centers, the applicability of these findings to general pulmonary practice has been uncertain. The objective of this study was to determine the historical occurrence of childhood ILD to provide information reflecting general pediatric pulmonary practice patterns. Childhood ILD cases seen at Vanderbilt Children's Hospital from 1994 to 2011 were retrospectively reviewed and classified according to the current pediatric diffuse lung disease histopathologic classification system. A total of 93 cases were identified, of which 91.4% were classifiable. A total of 68.8% (64/93) of subjects underwent lung biopsy in their evaluations. The largest classification categories were disorders related to systemic disease processes (24.7%), disorders of the immunocompromised host (24.7%), and disorders more prevalent in infancy (22.6%). Eight cases of neuroendocrine cell hyperplasia of infancy (NEHI) were identified, including 5 that were previously unrecognized before this review. Our findings demonstrate the general scope of childhood ILD and that these cases present within a variety of pediatric subspecialties. Retrospective review was valuable in recognizing more recently described forms of childhood ILD. As a significant portion of cases were classifiable based on clinical, genetic, and/or radiographic criteria, we urge greater consideration to noninvasive diagnostic approaches and suggest modification to the current childhood ILD classification scheme to accommodate the increasing number of cases diagnosed without lung biopsy.Keywords
This publication has 26 references indexed in Scilit:
- Abnormal infant pulmonary function in young children with neuroendocrine cell hyperplasia of infancyPediatric Pulmonology, 2012
- A national internet-linked based database for pediatric interstitial lung diseases: the French networkOrphanet Journal of Rare Diseases, 2012
- Imaging of Childhood Interstitial Lung DiseasePediatric Allergy, Immunology, and Pulmonology, 2010
- Direct Diagnosis in Radiology Series.American Journal of Roentgenology, 2010
- Incidence and classification of pediatric diffuse parenchymal lung diseases in GermanyOrphanet Journal of Rare Diseases, 2009
- Genetic Disorders of Surfactant DysfunctionPediatric and Developmental Pathology, 2009
- Diffuse Lung Disease in Young ChildrenAmerican Journal of Respiratory and Critical Care Medicine, 2007
- Evaluating Infants and Children with Interstitial Lung DiseaseSeminars in Respiratory and Critical Care Medicine, 2007
- Deficiency of Pulmonary Surfactant Protein B in Congenital Alveolar ProteinosisNew England Journal of Medicine, 1993
- Clinical spectrum of chronic interstitial lung disease in childrenThe Journal of Pediatrics, 1992