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.