Published ahead of print on September 20, 2007, doi:10.1164/rccm.200703-393OC
Am. J. Respir. Crit. Care Med., Volume 176, Number 11, December 2007, 1120-1128
A more recent version of this article appeared on December 1, 2007
Submitted on March 9, 2007
Accepted on October 11, 2007
Diffuse Lung Disease in Young Children: Application of a Novel Classification Scheme
Gail H Deutsch1, Lisa R Young2, Robin R Deterding3, Leland L Fan4, Sharon D Dell5, Judy A Bean6, Alan S Brody7, Lawrence M Nogee8, Bruce C Trapnell9, and Claire Langston and the Pathology Cooperative Group on behalf of the chILD Research Cooperative10*
1 Division of Pathology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA,
2 Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA,
3 Department of Pediatrics, The Children's Hospital, Denver, CO, USA,
4 Department of Pediatrics, Texas Children's Hospital, Houston, TX, USA,
5 Division of Respiratory Medicine, The Hospital For Sick Children, Ontario, Canada,
6 Division of Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA,
7 Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA,
8 Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA,
9 Division of Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA,
10 Department of Pathology, Texas Children's Hospital, Houston, TX, USA
* To whom correspondence should be addressed. E-mail: czlangst{at}texaschildrenshospital.org.
Rationale: Considerable confusion exists regarding nomenclature, classification and management of pediatric diffuse lung diseases due to the relative rarity and
differences in the spectrum of disease between adults and young children. Objectives: A multi-disciplinary working group was formed to: 1) Apply consensus terminology and diagnostic criteria for disorders presenting with
diffuse lung disease in infancy; and 2) Describe the distribution of disease entities, clinical features, and outcome in young children who currently undergo
lung biopsy in North America. Methods: Eleven centers provided pathologic material, clinical data and imaging
from all children less than 2 years of age who underwent lung biopsy for diffuse lung disease from 1999 to 2004.
Results: Multidisciplinary review categorized 88% of 187 cases. Disorders more prevalent in infancy, including primary developmental and lung growth abnormalities, neuroendocrine cell hyperplasia of infancy, and surfactantdysfunction disorders, comprised the majority of cases (53%). Lung growth disorders were often unsuspected clinically and under-recognized histologically.
Cases with known surfactant mutations had characteristic pathologic features. Age at biopsy and clinical presentation varied among categories. Pulmonary
hypertension, presence of a primary developmental abnormality or ABCA3 mutation was associated with high mortality, while no deaths occurred in cases of
pulmonary interstitial glycogenosis or neuroendocrine cell hyperplasia of infancy. Conclusions: This cross-sectional study identifies a diverse spectrum of lung disorders, largely unique to young children. Application of a classification scheme grouped clinically distinct patients with variable age of biopsy and mortality. Standardized terminology and classification will enhance accurate
description and diagnosis of these disorders.
Key words: infant, pulmonary, interstitial lung disease, lung biopsy
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