METHODS:
The National Registry for Childhood Interstitial and Diffuse Lung Disease is a longitudinal, observational, multicenter study which was initially approved by the Vanderbilt University Institutional Review Board (IRB #160427) in 2016. In 2019, the Children’s Hospital of Philadelphia (CHOP) became the data coordinating center and IRB of record (IRB #19-016138), now utilizing IRB Reliance Exchange. An open call for site participation was issued within the chILDRN in 2016 and offered through 2020, with sites volunteering to participate. Protocol and database training for participating site personnel are facilitated by the coordinating center via an online format. Data use agreements are coordinated from CHOP and aligned across participating sites.
Enrollment criteria are: age 0 to 21 years; diagnosis or suspected diagnosis of chILD; subject/parental/guardian permission (informed consent); and where appropriate, child assent. Both prevalent and incident patients seen clinically at chILDRN participating institutions or referred to a participating site for remote enrollment with informed consent may be enrolled. Data from clinical care are collected and organized using Research Electronic Data Capture (REDCap) electronic data capture tools hosted at CHOP 16,17Cross-sectional baseline data consist of demographics, clinical status, disease process / disease mechanism, and diagnosis or classification. The severity of illness score as developed by Fan, et al18, is used to classify clinical status at the time of Registry enrollment.
Descriptive statistics were used to characterize the study cohort. The utilization of genetic testing and lung biopsy were compared among different diagnoses or diagnostic categories using Chi-square tests. Plots were performed using GraphPad Prism version 9. Subjects included in the current analysis were enrolled prior to December 1, 2022, and with a minimum dataset of demographic information and disease classification. Missing data elements were handled as missing completely at random given ongoing data enrollment format of the prospective registry, with subject N reported for each data analysis.