SESSION TYPE: Pediatric Chest Disease
PRESENTED ON: Sunday, October 21, 2012 at 10:30 AM - 11:45 AM
PURPOSE: Childhood Interstitial Lung Diseases (ILD) are difficult to diagnose and are associated with high but variable morbidity and mortality in children. Recent multicenter and multidisciplinary efforts have focused on improved recognition and classification of interstitial and diffuse lung diseases in children. Purpose: To identify childhood ILD cases at our institution and describe the clinical features, classification, and outcomes.
METHODS: Pediatric ILD cases seen at Vanderbilt Children’s Hospital from 1994-2011 were identified through lung biopsy and clinical billing queries. Clinical data, imaging, and lung biopsies were retrospectively reviewed.
RESULTS: We identified 98 cases; 63 (67.8%) had undergone lung biopsy, of which >75% were performed since 2003. The mean age at biopsy was 7.9±6.4 years, with 28.6% presenting by age 2 years. Entities encountered in the biopsy group included: disorders of the immunocompromised host (n=21), disorders of the normal host (n=15), Neuroendocrine cell Hyperplasia of Infancy (NEHI; n=7), disorders related to systemic disease processes (n=7), ILD of unknown etiology (n=4), lung growth abnormalities (n=2), disorders masquerading as ILD (n=2), pulmonary interstitial glycogenosis (n=2), surfactant dysfunction disorders (n=2; 1 SFTPC, 1 ABCA3), and diffuse developmental disorders (n=1). Cases identified on clinical and radiographic criteria were: connective tissue associated ILD (n=18), ILD of unknown etiology (n=6), bronchiolitis obliterans (n=4), surfactant dysfunction disorders (2 SFTPC, 1 ABCA3), NEHI (n=2), and pulmonary hemorrhage and hypersensitivity pneumonitis (n=1 each). Overall mortality was 17.3% (mean age 8.8±7.0 years) and not significantly different in the biopsy versus non-biopsy groups. Mortality was 27% for immunocompromised patients, accounting for 43.8% of the total mortality. Among survivors (mean age 11.8±7.8 years), 21.9% required supplemental oxygen at last follow up. Retrospective review has already led to the identification of 5 previously unrecognized NEHI cases.
CONCLUSIONS: The spectrum of childhood ILD at our institution is similar to prior multicenter reports.
CLINICAL IMPLICATIONS: In light of improved diagnostic strategies and recently recognized entities, this study demonstrates the value of comprehensive case review in achieving definitive diagnosis for patients with ILD.
DISCLOSURE: The following authors have nothing to disclose: Jennifer Soares, Mohammad Fazili, Paul Moore, Melissa Hilmes, Gail Deutsch, Joyce Johnson, Lisa Young
No Product/Research Disclosure InformationMonroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN