Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet): Case Definition in Surveillance for Childhood-Onset Duchenne/Becker Muscular Dystrophy

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Date: 
Tuesday, September 28, 2010
Author(s): 
Katherine D. Mathews
Chris Cunniff
Jiji R. Kantamneni
Emma Ciafaloni
Timothy Miller
Dennis Matthews
Valerie Cwik
Charlotte Druschel
Lisa Miller
F. John Meaney
John Sladky
Paul A. Romitti
Journal Title: 
Journal of Child Neurology
Abstract: 

The Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet) is a multisite collaboration to determine the prevalence of childhood-onset Duchenne/Becker muscular dystrophy and to characterize health care and health outcomes in this population. MD STARnet uses medical record abstraction to identify patients with Duchenne/Becker muscular dystrophy born January 1, 1982 or later who resided in 1 of the participating sites. Critical diagnostic elements of each abstracted record are reviewed independently by >4 clinicians and assigned to 1 of 6 case definition categories (definite, probable, possible, asymptomatic, female, not Duchenne/Becker muscular dystrophy) by consensus. As of November 2009, 815 potential cases were reviewed. Of the cases included in analysis, 674 (82%) were either ‘‘definite’’ or ‘‘probable’’ Duchenne/Becker muscular dystrophy. These data reflect a change in diagnostic testing, as case assignment based on genetic testing increased from 67% in the oldest cohort (born 1982-1987) to 94% in the cohort born 2004 to 2009.

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Citation: 

Mathews, Katherine D., Chris Cunniff, Jiji R. Kantamneni, Emma Ciafaloni, Timothy Miller, Dennis Matthews, Valerie Cwik et al. "Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet): case definition in surveillance for childhood-onset Duchenne/Becker muscular dystrophy." Journal of child neurology 25, no. 9 (2010): 1098-1102.