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Diffuse Lung Disease in Biopsied Children 2 to 18 Years of Age. Application of the chILD Classification Scheme
Leland L Fan1, Megan K Dishop2, Csaba Galambos2
11 Department of Pediatrics, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, Colorado.
Insights
This study evaluated a classification for pediatric diffuse lung disease in children aged 2-18 years. Immunocompromised children with interstitial lung disease had poor survival rates.
Area of Science:
- Pediatric Pulmonology
- Thoracic Pathology
- Critical Care Medicine
Background:
- Children's Interstitial and Diffuse Lung Disease (chILD) is a complex group of lung disorders in children.
- A prior classification scheme for chILD was effective in infants (0-2 years) but unproven in older children.
- This study addresses the need to evaluate the classification scheme in a broader pediatric age group (2-18 years).
Purpose of the Study:
- To describe the spectrum of biopsy-proven chILD in North American children aged 2-18 years.
- To apply and evaluate a previously established chILD classification scheme in this age group.
- To identify mortality risk factors in children with diffuse lung disease.
Main Methods:
- A multicenter study included 191 patients aged 2-18 years who underwent lung biopsies for diffuse lung disease.
- Pediatric lung pathologists reviewed biopsies and applied the chILD classification scheme.
- Logistic regression analysis identified risk factors associated with mortality.
Main Results:
- The most frequent category was Disorders of the Immunocompromised Host (40.8%); Disorders of Infancy were least common (4.7%).
- Immunocompromised patients had the highest mortality rate (52.8%).
- Mechanical ventilation, clinical status at biopsy, tachypnea, hemoptysis, crackles, and pulmonary hypertension (in immunocompetent patients) were associated with mortality.
Conclusions:
- The chILD classification scheme showed overlap with adult diagnoses in older children, with fewer infant-specific diagnoses.
- Survival was less than 50% for immunocompromised patients with diffuse lung disease requiring lung biopsy.
Rationale:
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.
Objectives:
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.
Measurements And Main 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.
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