help button home button
AJRCCM
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH
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
This Article
Right arrow Full Text (PDF)
Right arrow All Versions of this Article:
200703-393OCv1
200703-393OCv2
176/11/1120    most recent
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Deutsch, G. H
Right arrow Articles by Travis, W. D
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Deutsch, G. H
Right arrow Articles by Travis, W. D

Submitted on March 9, 2007
Accepted on September 20, 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, Claire Langston10*, Eric A Albright11, Frederic B Askin12, Pauline M Chou13, Carlyne M Cool14, Susan C Coventry15, Ernest Cutz16, Mary M Davis17, Csaba Galampos18, Kathleen Patterson19, and William D Travis20

1 Division of Pathology, Cincinnati Childrens Hospital Medical Center, Cincinnati, OH, USA, 2 Division of Pulmonary Medicine, Cincinnati Childrens 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 Childrens Hospital Medical Center, Cincinnati, OH, USA, 7 Department of Radiology, Cincinnati Childrens 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 Childrens Hospital Medical Center, Cincinnati, OH, USA, 10 Department of Pathology, Texas Children's Hospital, Houston, TX, USA, 11 Department of Anatomic Pathology, Children's Hospital, Columbus, OH, USA, 12 Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, USA, 13 Department of Pathology, Children's Memorial Hospital, Chicago, IL, USA, 14 Department of Pathology, University of Colorado Health Science Center, Denver, CO, USA, 15 epartment of Pathology, Kosair Children's Hospital, Louisville, KY, USA, 16 Department of Pathology, The Hospital For Sick Children, Ontario, Canada, 17 Division of Pediatric Pathology, James Whitcomb Riley Hospital for Children, Indianopolis, Indiana, USA, 18 Department of Pathology, Children's Hopsital of Pittsburgh, Pittsburgh, PA, USA, 19 Department of Laboratories, Children's Hopital and Regional Medical Center, Seattle, WA, USA, 20 Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY, 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




This article has been cited by other articles:


Home page
 Eur Respir JHome page
W. Lenney, A. L. Boner, L. Bont, A. Bush, K-H. Carlsen, E. Eber, B. Fauroux, M. Gotz, A. Greenough, J. Grigg, et al.
Medicines used in respiratory diseases only seen in children
Eur. Respir. J., September 1, 2009; 34(3): 531 - 551.
[Abstract] [Full Text] [PDF]

Home page
 J. Med. Genet.Home page
L Guillot, R Epaud, G Thouvenin, L Jonard, A Mohsni, R Couderc, F Counil, J de Blic, R A Taam, M Le Bourgeois, et al.
New surfactant protein C gene mutations associated with diffuse lung disease
J. Med. Genet., July 1, 2009; 46(7): 490 - 494.
[Abstract] [Full Text] [PDF]

Home page
 ThoraxHome page
A Bush
Interstitial lung disease guideline
Thorax, June 1, 2009; 64(6): 548 - 548.
[Full Text] [PDF]

Home page
 Am. J. Respir. Crit. Care Med.Home page
A. Bush
Update in Pediatric Lung Disease 2008
Am. J. Respir. Crit. Care Med., April 15, 2009; 179(8): 637 - 649.
[Full Text] [PDF]

Home page
 Proc Am Thorac SocHome page
S. C. Sweet
Pediatric Lung Transplantation
Proceedings of the ATS, January 15, 2009; 6(1): 122 - 127.
[Abstract] [Full Text] [PDF]

Home page
 J. Clin. Pathol.Home page
D M Hwang and K S Cunningham
Color atlas and text of pulmonary pathology, 2nd edition
J. Clin. Pathol., November 1, 2008; 61(11): 1231 - 1232.
[Full Text] [PDF]

Home page
 NeoReviewsHome page
W. A. Gower, S. E. Wert, and L. M. Nogee
Inherited Surfactant Disorders
NeoReviews, October 1, 2008; 9(10): e458 - e467.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Roentgenol.Home page
S. Vrielynck, T. Mamou-Mani, S. Emond, P. Scheinmann, F. Brunelle, and J. de Blic
Diagnostic Value of High-Resolution CT in the Evaluation of Chronic Infiltrative Lung Disease in Children
Am. J. Roentgenol., September 1, 2008; 191(3): 914 - 920.
[Abstract] [Full Text] [PDF]

Home page
 ChestHome page
L. R. Young, L. M. Nogee, B. Barnett, R. J. Panos, T. V. Colby, and G. H. Deutsch
Usual Interstitial Pneumonia in an Adolescent With ABCA3 Mutations
Chest, July 1, 2008; 134(1): 192 - 195.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Respir. Crit. Care Med.Home page
A. Bush
Update in Pediatric Lung Disease 2007
Am. J. Respir. Crit. Care Med., April 1, 2008; 177(7): 686 - 695.
[Full Text] [PDF]

Home page
 Am. J. Respir. Crit. Care Med.Home page
A. G. Nicholson and A. Bush
Classification of Diffuse Lung Disease in Infants: The Reality of Groups
Am. J. Respir. Crit. Care Med., December 1, 2007; 176(11): 1060 - 1061.
[Full Text] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH
Proc. Am. Thorac. Soc. Am. J. Respir. Cell Mol. Biol.
Copyright © 2007 American Thoracic Society 		  CCM abstracts