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

on behalf of the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet)

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalBirth Defects Research
DOIs
StateAccepted/In press - Jan 1 2018
Externally publishedYes

Fingerprint

Population Surveillance
Muscular Dystrophies
International Classification of Diseases
Health
Research
Application programs
Myotonic Dystrophy
Duchenne Muscular Dystrophy
Statistics
Network protocols
Medical Records
Meta-Analysis
Software
Extremities
Population

Keywords

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

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Embryology
  • Toxicology
  • Developmental Biology
  • Health, Toxicology and Mutagenesis

Cite this

Muscular Dystrophy Surveillance, Tracking, and Research Network pilot : Population-based surveillance of major muscular dystrophies at four U.S. sites, 2007–2011. / on behalf of the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet).

In: Birth Defects Research, 01.01.2018.

Research output: Contribution to journalArticle

@article{1461e0901106480db5dc4e94e1f5d85a,
title = "Muscular Dystrophy Surveillance, Tracking, and Research Network pilot: Population-based surveillance of major muscular dystrophies at four U.S. sites, 2007–2011",
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 = "active surveillance, Clinical Modification (ICD-9-CM) codes, International Classification of Diseases, MD STARnet, medical record abstraction, muscular dystrophies, Ninth Revision, population-based",
author = "{on behalf of the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet)} and Do, {Thuy Quynh N.} and Natalie Street and Jennifer Donnelly and Adams, {Melissa M.} and Cunniff, {Christopher M} and Fox, {Deborah J.} and Weinert, {Richard O.} and Joyce Oleszek and Romitti, {Paul A.} and Westfield, {Christina P.} and Julie Bolen",
year = "2018",
month = "1",
day = "1",
doi = "10.1002/bdr2.1371",
language = "English (US)",
journal = "Birth Defects Research",
issn = "2472-1727",
publisher = "John Wiley and Sons Ltd",

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TY - JOUR

T1 - Muscular Dystrophy Surveillance, Tracking, and Research Network pilot

T2 - Population-based surveillance of major muscular dystrophies at four U.S. sites, 2007–2011

AU - on behalf of the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet)

AU - Do, Thuy Quynh N.

AU - Street, Natalie

AU - Donnelly, Jennifer

AU - Adams, Melissa M.

AU - Cunniff, Christopher M

AU - Fox, Deborah J.

AU - Weinert, Richard O.

AU - Oleszek, Joyce

AU - Romitti, Paul A.

AU - Westfield, Christina P.

AU - Bolen, Julie

PY - 2018/1/1

Y1 - 2018/1/1

N2 - 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.

AB - 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.

KW - active surveillance

KW - Clinical Modification (ICD-9-CM) codes

KW - International Classification of Diseases

KW - MD STARnet

KW - medical record abstraction

KW - muscular dystrophies

KW - Ninth Revision

KW - population-based

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U2 - 10.1002/bdr2.1371

DO - 10.1002/bdr2.1371

M3 - Article

C2 - 30070776

AN - SCOPUS:85052625958

JO - Birth Defects Research

JF - Birth Defects Research

SN - 2472-1727

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