Diffuse Lung Disease in Biopsied Children 2 to 18 Years of Age. Application of the chILD Classification Scheme.
BACKGROUND
Children's Interstitial and Diffuse Lung Disease (chILD) is a heterogeneous group of disorders that is challenging to categorize. In previous study, a classification scheme was successfully applied to children 0 to 2 years of age who underwent lung biopsies for chILD. This classification scheme has not been evaluated in children 2 to 18 years of age.


OBJECTIVE
This multicenter interdisciplinary study sought to describe the spectrum of biopsy-proven chILD in North America and to apply a previously reported classification scheme in children 2 to 18 years of age. Mortality and risk factors for mortality were also assessed.


METHODS
Patients 2 to 18 years of age who underwent lung biopsies for diffuse lung disease from 12 North American institutions were included. Demographic and clinical data were collected and described. The lung biopsies were reviewed by pediatric lung pathologists with expertise in diffuse lung disease and were classified by the chILD classification scheme. Logistic regression was used to determine risk factors for mortality.


RESULTS
A total of 191 cases were included in the final analysis. Number of biopsies varied by center (5-49 biopsies; mean, 15.8) and by age (2-18 yr; mean, 10.6 yr). The most common classification category in this cohort was Disorders of the Immunocompromised Host (40.8%), and the least common was Disorders of Infancy (4.7%). Immunocompromised patients suffered the highest mortality (52.8%). Additional associations with mortality included mechanical ventilation, worse clinical status at time of biopsy, tachypnea, hemoptysis, and crackles. Pulmonary hypertension was found to be a risk factor for mortality but only in the immunocompetent patients.


CONCLUSIONS
In patients 2 to 18 years of age who underwent lung biopsies for diffuse lung disease, there were far fewer diagnoses prevalent in infancy and more overlap with adult diagnoses. Immunocompromised patients with diffuse lung disease who underwent lung biopsies had less than 50% survival at time of last follow-up.
1 Department of Pediatrics, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, Colorado.
Leland L
LL
Fan
2 Department of Pathology and Laboratory Medicine, University of Colorado School of Medicine, Aurora, Colorado.
Megan K
MK
Dishop
2 Department of Pathology and Laboratory Medicine, University of Colorado School of Medicine, Aurora, Colorado.
Csaba
C
Galambos
3 Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Frederic B
FB
Askin
4 Department of Pathology, Washington University School of Medicine, St. Louis, Missouri.
Frances V
FV
White
5 Department of Pathology, Baylor College of Medicine, Houston, Texas.
Claire
C
Langston
1 Department of Pediatrics, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, Colorado.
Deborah R
DR
Liptzin
6 Department of Biostatistics and Informatics, Colorado School of Public Health, University of Colorado, Aurora, Colorado.
Miranda E
ME
Kroehl
7 Department of Pathology, University of Washington School of Medicine, Seattle, Washington.
Gail H
GH
Deutsch
8 Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, Tennessee.
Lisa R
LR
Young
9 Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
Geoffrey
G
Kurland
10 Department of Pediatrics, University of California San Diego and Rady Children's Hospital, San Diego, California.
James
J
Hagood
11 Department of Pediatrics, The Hospital of Sick Children, Toronto, Ontario, Canada; and.
Sharon
S
Dell
12 Department of Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Bruce C
BC
Trapnell
1 Department of Pediatrics, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, Colorado.
Robin R
RR
Deterding
D000293:Adolescent; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000473:pathology*; D016867:Immunocompromised Host; D016015:Logistic Models; D008168:Lung / Q000473:pathology*; D017563:Lung Diseases, Interstitial / Q000145:classification* / Q000401:mortality* / Q000473:pathology*; D008297:Male; D009656:North America; D035583:Rare Diseases; D012307:Risk Factors
interstitial lung disease; pathology; rare pediatric lung disease