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. 2018 Nov 15;110(19):1404-1411.
doi: 10.1002/bdr2.1371. Epub 2018 Aug 2.

Muscular Dystrophy Surveillance, Tracking, and Research Network pilot: Population-based surveillance of major muscular dystrophies at four U.S. sites, 2007-2011

ThuyQuynh N Do  1 , Natalie Street  1 , Jennifer Donnelly  2 , Melissa M Adams  3 , Christopher Cunniff  4 , Deborah J Fox  5 , Richard O Weinert  2 , Joyce Oleszek  6 , Paul A Romitti  7 , Christina P Westfield  5 , Julie Bolen  1 ; Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet)
Affiliations

Muscular Dystrophy Surveillance, Tracking, and Research Network pilot: Population-based surveillance of major muscular dystrophies at four U.S. sites, 2007-2011

ThuyQuynh N Do et al. Birth Defects Res. .

Abstract

Background: For 10 years, the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet) conducted surveillance for Duchenne and Becker muscular dystrophy (DBMD). We piloted expanding surveillance to other MDs that vary in severity, onset, and sources of care.

Methods: Our retrospective surveillance included individuals diagnosed with one of nine eligible MDs before or during the study period (January 2007-December 2011), one or more health encounters, and residence in one of four U.S. sites (Arizona, Colorado, Iowa, or western New York) at any time within the study period. We developed case definitions, surveillance protocols, and software applications for medical record abstraction, clinical review, and data pooling. Potential cases were identified by International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes 359.0, 359.1, and 359.21 and International Classification of Diseases, Tenth Revision (ICD-10) codes G71.0 and G71.1. Descriptive statistics were compared by MD type. Percentage of MD cases identified by each ICD-9-CM code was calculated.

Results: Of 2,862 cases, 32.9% were myotonic, dystrophy 25.8% DBMD, 9.7% facioscapulohumeral MD, and 9.1% limb-girdle MD. Most cases were male (63.6%), non-Hispanic (59.8%), and White (80.2%). About, half of cases were genetically diagnosed in self (39.1%) or family (6.2%). About, half had a family history of MD (48.9%). The hereditary progressive MD code (359.1) was the most common code for identifying eligible cases. The myotonic code (359.21) identified 83.4% of eligible myotonic dystrophy cases (786/943).

Conclusions: MD STARnet is the only multisite, population-based active surveillance system available for MD in the United States. Continuing our expanded surveillance will contribute important epidemiologic and health outcome information about several MDs.

Keywords: Clinical Modification (ICD-9-CM) codes; International Classification of Diseases; MD STARnet; Ninth Revision; active surveillance; medical record abstraction; muscular dystrophies; population-based.

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Conflict of interest statement

CONFLICT OF INTEREST

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

FIGURE 1
FIGURE 1
Comparison of ICD-9-CM codes in the identification of major types of MDs from the 2007 to 2011 MD STARnet pilot. Codes listed are ICD-9-CM codes: 359.0: congenital hereditary MD (includes CMD), 359.1: hereditary progressive MD (includes DBMD, Distal, EDMD, FSHD, LGMD, OPMD, and MD-NOS), 359.21: myotonic muscular dystrophy (myotonic dystrophy). *Other indicates that cases were identified by ICD-10 code or other method. MD STARnet = muscular dystrophy surveillance, tracking and research network; MD = muscular dystrophy; CMD = congenital muscular dystrophy; DBMD = Duchenne and Becker muscular dystrophy; EDMD = Emery–Dreifuss muscular dystrophy; FSHD = facioscapulohumeral muscular dystrophy; LGMD = limb-girdle muscular dystrophy; DM = myotonic dystrophy; OPMD = oculopharyngeal muscular dystrophy; MD-NOS = muscular dystrophy-not otherwise specified
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