Health services use among children diagnosed with medium-chain acyl-CoA dehydrogenase deficiency through newborn screening: A cohort study in Ontario, Canada

Authors

Maria D. Karaceper, University of Ottawa
Sara D. Khangura, University of Ottawa
Kumanan Wilson, University of Ottawa
Doug Coyle, University of Ottawa
Marni Brownell, Max Rady College of Medicine, University of Manitoba
Christine Davies, Children's Hospital of Eastern Ontario, Ottawa
Linda Dodds, Dalhousie University, Faculty of Medicine
Annette Feigenbaum, Hospital for Sick Children University of Toronto
Deshayne B. Fell, University of Ottawa
Scott D. Grosse, Coordinating Center for Health Promotion
Astrid Guttmann, Hospital for Sick Children University of Toronto
Steven Hawken, University of Ottawa
Robin Z. Hayeems, Hospital for Sick Children University of Toronto
Jonathan B. Kronick, Hospital for Sick Children University of Toronto
Anne Marie Laberge, CHU Sainte-Justine - Le Centre Hospitalier Universitaire Mère-Enfant
Julian Little, University of Ottawa
Aizeddin Mhanni, University of Manitoba
John J. Mitchell, Centre Universitaire de Santé McGill, Hôpital de Montreal Pour Enfants
Meranda Nakhla, Centre Universitaire de Santé McGill, Hôpital de Montreal Pour Enfants
Murray Potter, McMaster University
Chitra Prasad, Western UniversityFollow
Cheryl Rockman-Greenberg, University of Manitoba
Rebecca Sparkes, Alberta Children's Hospital
Sylvia Stockler, Children's and Women's Health Centre of British Columbia
Keiko Ueda, Children's and Women's Health Centre of British Columbia
Hilary Vallance, Children's and Women's Health Centre of British Columbia

Document Type

Article

Publication Date

3-22-2019

Journal

Orphanet Journal of Rare Diseases

Volume

14

Issue

1

URL with Digital Object Identifier

10.1186/s13023-019-1001-0

Abstract

Background: We describe early health services utilization for children diagnosed with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency through newborn screening in Ontario, Canada, relative to a screen negative comparison cohort. Methods: Eligible children were identified via newborn screening between April 1, 2006 and March 31, 2010. Age-stratified rates of physician encounters, emergency department (ED) visits and inpatient hospitalizations to March 31, 2012 were compared using incidence rate ratios (IRR) and incidence rate differences (IRD). We used negative binomial regression to adjust IRRs for sex, gestational age, birth weight, socioeconomic status and rural/urban residence. Results: Throughout the first few years of life, children with MCAD deficiency (n = 40) experienced statistically significantly higher rates of physician encounters, ED visits, and hospital stays compared with the screen negative cohort. The highest rates of ED visits and hospitalizations in the MCAD deficiency cohort occurred from 6 months to 2 years of age (ED use: 2.1-2.5 visits per child per year; hospitalization: 0.5-0.6 visits per child per year), after which rates gradually declined. Conclusions: This study confirms that young children with MCAD deficiency use health services more frequently than the general population throughout the first few years of life. Rates of service use in this population gradually diminish after 24 months of age.