Essential Site Maintenance: Authorea-powered sites will be updated circa 15:00-17:00 Eastern on Tuesday 5 November.
There should be no interruption to normal services, but please contact us at [email protected] in case you face any issues.

loading page

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

Corresponding Author:[email protected]

Author Profile

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