Affiliations:
1Pediatric Pulmonary Medicine, Department of Child
Health, University of Missouri Children’s Hospital
2Department of Laboratory Medicine and Pathology,
Seattle Children’s Hospital, University of Washington Medical Center
3Pediatric Pulmonology, Rainbow Babies & Children’s
Hospital, Case School of Medicine
4Pediatric Pulmonary Medicine, Children’s Hospital of
Colorado
5Division of Pulmonary Medicine, Boston Children’s
Hospital, Harvard Medical School
6Neonatal-Perinatal Medicine, Department of
Pediatrics, Washington University School of Medicine, St. Louis
Children’s Hospital
7Division of Pediatric Pulmonology, Department of
Pediatrics, Vanderbilt University Medical Center
8Division of Pulmonology, Department of Pediatrics,
Children’s Hospital of Pittsburgh, University of Pittsburgh School of
Medicine
9 Division of Pediatric Pulmonology, Department of
Pediatrics, University of North Carolina School of Medicine
10Pulmonary Medicine, Pediatrics, Stanford University
School of Medicine
11Pulmonary and Sleep Medicine, Children’s Mercy
Hospital – Kansas City
12Department of Pediatrics, Donald and Barbara Zucker
School of Medicine at Hofstra / Northwell, Steven and Alexandra Cohen
Children’s Medical Center
13Division of Pulmonary and Sleep Medicine, Department
of Pediatrics, University of Minnesota System, School of Medicine
14Division of Pediatric Respiratory Sciences, Johns
Hopkins University
15Pediatric Pulmonology, Department of Pediatrics,
Indiana University School of Medicine, Riley Hospital for Children at
Indiana University Health.
16Pediatrics, Ann and Robert H Lurie Children’s
Hospital of Chicago Northwestern University Feinberg School of Medicine
17 Division of Pediatric Pulmonology / Sleep Medicine,
Children’s Hospital Los Angeles, Keck School of Medicine, University of
Southern California
18Pediatrics, University of Michigan C. S. Mott
Children’s Hospital
19 Division of Pediatric Pulmonology, Columbia
University Irving Medical Center, Vagelos College of Physicians and
Surgeons
20Pediatric Pulmonology, San Francisco Benioff
Children’s Hospital, University of California
21Pediatrics, Doernbecher Children’s Hospital, Oregon
Health & Science University
22Division of Pulmonary and Sleep Medicine, Department
of Pediatrics, Children’s Hospital of Philadelphia, University of
Pennsylvania Perelman School of Medicine
Funding Source : Supported in part by the NIH/NHLBI K24 HL143281
(LRY)
Running Title : U.S. ChILD Registry
Key Words : interstitial lung disease, rare lung disease, NEHI
Corresponding Author :
Lisa R. Young, M.D.
Children’s Hospital of Philadelphia
3615 Civic Center Boulevard, 4th Floor, Suite 416
Philadelphia, PA 19104
E-mail: youngL5@chop.edu
Phone: 215-590-3749
ABSTRACT :
Childhood interstitial and diffuse lung disease (chILD) encompasses a
broad spectrum of rare disorders. The Children’s Interstitial and
Diffuse Lung Disease Research Network (chILDRN) established a
prospective registry to advance knowledge regarding etiology, phenotype,
natural history, and management of these disorders. This longitudinal,
observational, multicenter registry utilizes single-IRB reliance
agreements, with participation from 25 chILDRN centers across the U.S.
Clinical data are collected and managed using the Research Electronic
Data Capture (REDCap) electronic data platform. We report the study
design and some elements of the initial Registry enrollment cohort,
which includes 683 subjects with a broad range of chILD diagnoses. The
most common diagnosis reported was neuroendocrine cell hyperplasia of
infancy (NEHI), with 155 (23%) subjects. Components of underlying
disease biology were identified by enrolling sites, with cohorts of
interstitial fibrosis, immune dysregulation, and airway disease being
most commonly reported. Prominent morbidities affecting enrolled
children included home supplemental oxygen use (63%) and failure to
thrive (46%). This Registry is the largest longitudinal chILD cohort in
the U.S. to date, providing a powerful framework for collaborating
centers committed to improving the understanding and treatment of these
rare disorders.