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The U.S. National Registry for Childhood Interstitial and Diffuse Lung Disease: Report of Study Design and Initial Enrollment Cohort
  • +32
  • Rebekah Nevel,
  • Gail Deutsch,
  • Daniel Craven,
  • Robin Deterding,
  • Martha Fishman,
  • Jennifer Wambach (Guest Editor),
  • Alicia Casey,
  • Katie Krone,
  • Deborah R. Liptzin,
  • Michael O'Connor,
  • Geoffrey Kurland,
  • Jane Taylor,
  • William Gower,
  • James Hagood,
  • Carol Conrad,
  • Jade Tam-Williams,
  • Elizabeth Fiorino,
  • Samuel Goldfarb,
  • Sara Sadreameli,
  • Lawrence Nogee,
  • Gregory Montgomery,
  • Aaron Hamvas,
  • Terri Laguna,
  • Manvi Bansal,
  • Cheryl Lew,
  • Maria Santiago,
  • Antonia Popova,
  • Aliva De,
  • Marilynn Chan,
  • Michael Powers,
  • Maureen B Josephson ,
  • Devaney Camburn,
  • Laura Voss,
  • Yun Li,
  • Lisa Young
Rebekah Nevel
University of Missouri School of Medicine
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Gail Deutsch
Seattle Children's Hospital
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Daniel Craven
Rainbow Babies and Children's Hospital
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Robin Deterding
Children's Hospital Colorado
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Martha Fishman
Boston Children's Hospital
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Jennifer Wambach (Guest Editor)
Washington University School of Medicine in Saint Louis
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Alicia Casey
Boston Children's Hospital
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Katie Krone
Boston Children s Hospital
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Deborah R. Liptzin
Children's Hospital Colorado
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Michael O'Connor
Vanderbilt University Medical Center
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Geoffrey Kurland
Children's Hospital of Pittsburgh
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Jane Taylor
UPMC Children's Hospital of Pittsburgh Child Development Unit
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William Gower
University of North Carolina at Chapel Hill School of Medicine
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James Hagood
University of North Carolina System
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Carol Conrad
Stanford University
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Jade Tam-Williams
Children's Mercy Hospital
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Elizabeth Fiorino
Weill Cornell Medicine
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Samuel Goldfarb
University of Minnesota System
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Sara Sadreameli
Johns Hopkins University
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Lawrence Nogee
JHMI
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Gregory Montgomery
Indiana University
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Aaron Hamvas
Ann and Robert H Lurie Children's Hospital of Chicago
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Terri Laguna
Ann and Robert H Lurie Children's Hospital of Chicago
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Manvi Bansal
Children\'s Hospital of Los Angeles
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Cheryl Lew
CHLA
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Maria Santiago
Steven and Alexandra Cohen Children's Medical Center
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Antonia Popova
University of Michigan C. S. Mott Children's Hospital
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Aliva De
Columbia University Medical Center
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Marilynn Chan
University of California, San Francisco (UCSF) Benioff Children's Hospital
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Michael Powers
Oregon Health Sci. Univ.
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Maureen B Josephson
CHOP
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Devaney Camburn
The Children's Hospital of Philadelphia
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Laura Voss
Children's Hospital of Philadelphia
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Yun Li
University of Pennsylvania Perelman School of Medicine
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Lisa Young
Children's Hospital of Philadelphia

Corresponding Author:youngl5@email.chop.edu

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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.
22 Mar 2023Submitted to Pediatric Pulmonology
26 Mar 2023Submission Checks Completed
26 Mar 2023Assigned to Editor
26 Mar 2023Review(s) Completed, Editorial Evaluation Pending
07 Apr 2023Reviewer(s) Assigned
16 May 2023Editorial Decision: Revise Minor
27 May 20231st Revision Received
28 May 2023Submission Checks Completed
28 May 2023Assigned to Editor
28 May 2023Reviewer(s) Assigned
28 May 2023Review(s) Completed, Editorial Evaluation Pending
14 Jun 2023Editorial Decision: Accept