Clinical Scope and Healthcare Utilization in Childhood Interstitial Lung
Disease at a Tertiary Center
Abstract
Childhood interstitial lung disease (chILD) is a heterogeneous group of
diffuse lung diseases (DLD) that can be challenging to diagnose. With
relative rarity of individual entities, data are limited on disease
prevalence, care patterns, and healthcare utilization. The objective of
this study was to evaluate chILD prevalence and review diagnostic and
clinical care patterns at our center. A single-center, retrospective
cohort study was conducted of patients receiving care at the Children’s
Hospital of Philadelphia (CHOP) between January 1, 2019, and December
31, 2021. Through query of selected ICD-10 billing codes relevant for
chILD/DLD, a total of 306 patients were identified receiving care during
this period. Respiratory symptom onset was documented to have developed
before two years of age for 40% of cases. The most common diagnostic
categories included those with oncologic disease (21.2%), bronchiolitis
obliterans (10.1%), and connective tissue disease (9.5%). Genetic
testing was performed in 49% of cases, while 36% underwent lung
biopsy. Hospitalization at CHOP had occurred for 80.4% of patients,
with 45.1% ever hospitalized in an intensive care unit. One-third of
children had required chronic supplemental oxygen. Seven (2.3%)
patients died during this three-year period. Collectively, these data
demonstrate the scope of chILD and extent of health care utilization at
a large volume tertiary care center. This approach to cohort
identification and EMR-driven data collection in chILD provides new
opportunities for cohort analysis and will inform the feasibility of
future studies.