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.