Oral Corticosteroids and Onset of Cardiomyopathy in Duchenne Muscular Dystrophy

Objective To estimate the age when cardiomyopathy develops in boys with Duchenne muscular dystrophy (DMD) and to analyze the effect of corticosteroid treatment on the age of cardiomyopathy onset. Study design We identified a population-based sample of 462 boys with DMD, born between 1982 and 2005, i...

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Veröffentlicht in:The Journal of pediatrics 2013-10, Vol.163 (4), p.1080-1084.e1
Hauptverfasser: Barber, Brent J., MD, Andrews, Jennifer G., MBA, Lu, Zhenqiang, PhD, West, Nancy A., PhD, Meaney, F. John, PhD, Price, Elinora T., BA, Gray, Ashley, BS, Sheehan, Daniel W., MD, Pandya, Shree, PT, DPT, MS, Yang, Michele, MD, Cunniff, Christopher, MD
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container_end_page 1084.e1
container_issue 4
container_start_page 1080
container_title The Journal of pediatrics
container_volume 163
creator Barber, Brent J., MD
Andrews, Jennifer G., MBA
Lu, Zhenqiang, PhD
West, Nancy A., PhD
Meaney, F. John, PhD
Price, Elinora T., BA
Gray, Ashley, BS
Sheehan, Daniel W., MD
Pandya, Shree, PT, DPT, MS
Yang, Michele, MD
Cunniff, Christopher, MD
description Objective To estimate the age when cardiomyopathy develops in boys with Duchenne muscular dystrophy (DMD) and to analyze the effect of corticosteroid treatment on the age of cardiomyopathy onset. Study design We identified a population-based sample of 462 boys with DMD, born between 1982 and 2005, in 5 surveillance sites in the US. Echocardiographic and corticosteroid treatment data were collected. Cardiomyopathy was defined by a reduced fractional shortening (
doi_str_mv 10.1016/j.jpeds.2013.05.060
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John, PhD ; Price, Elinora T., BA ; Gray, Ashley, BS ; Sheehan, Daniel W., MD ; Pandya, Shree, PT, DPT, MS ; Yang, Michele, MD ; Cunniff, Christopher, MD</creator><creatorcontrib>Barber, Brent J., MD ; Andrews, Jennifer G., MBA ; Lu, Zhenqiang, PhD ; West, Nancy A., PhD ; Meaney, F. John, PhD ; Price, Elinora T., BA ; Gray, Ashley, BS ; Sheehan, Daniel W., MD ; Pandya, Shree, PT, DPT, MS ; Yang, Michele, MD ; Cunniff, Christopher, MD</creatorcontrib><description>Objective To estimate the age when cardiomyopathy develops in boys with Duchenne muscular dystrophy (DMD) and to analyze the effect of corticosteroid treatment on the age of cardiomyopathy onset. Study design We identified a population-based sample of 462 boys with DMD, born between 1982 and 2005, in 5 surveillance sites in the US. Echocardiographic and corticosteroid treatment data were collected. Cardiomyopathy was defined by a reduced fractional shortening (&lt;28%) or ejection fraction (&lt;55%). The age of cardiomyopathy onset was determined. Survival analysis was performed to determine the effects of corticosteroid treatment on cardiomyopathy onset. Results The mean (SD) age of cardiomyopathy onset was 14.3 (4.2) years for the entire population and 15.2 (3.4) years in corticosteroid-treated vs 13.1 (4.8) in non-treated boys. Survival analysis described a significant delay of cardiomyopathy onset for boys treated with corticosteroids ( P &lt; .02). By 14.3 years of age, 63% of non-treated boys had developed cardiomyopathy vs only 36% of those treated. Among boys treated with corticosteroids, there is a significant positive effect of duration of corticosteroid treatment on cardiomyopathy onset ( P &lt; .0001). For every year of corticosteroid treatment, the probability of developing cardiomyopathy decreased by 4%. Conclusions Oral corticosteroid treatment was associated with delayed cardiomyopathy onset. The duration of corticosteroid treatment also correlated positively with delayed cardiomyopathy onset. Our analysis suggests that a boy with DMD treated for 5 years with corticosteroids might experience a 20% decrease in the likelihood of developing cardiomyopathy compared with untreated boys.</description><identifier>ISSN: 0022-3476</identifier><identifier>EISSN: 1097-6833</identifier><identifier>DOI: 10.1016/j.jpeds.2013.05.060</identifier><identifier>PMID: 23866715</identifier><language>eng</language><publisher>United States: Mosby, Inc</publisher><subject>Administration, Oral ; Adolescent ; adrenal cortex hormones ; Adrenal Cortex Hormones - administration &amp; dosage ; Adrenal Cortex Hormones - therapeutic use ; Age of Onset ; boys ; Cardiomyopathies - complications ; Cardiomyopathies - drug therapy ; Cardiomyopathies - epidemiology ; cardiomyopathy ; Child ; Child, Preschool ; correlation ; Echocardiography ; Humans ; Male ; monitoring ; muscular dystrophy ; Muscular Dystrophy, Duchenne - complications ; Muscular Dystrophy, Duchenne - drug therapy ; Pediatrics ; probability ; Regression Analysis ; Time Factors ; Treatment Outcome</subject><ispartof>The Journal of pediatrics, 2013-10, Vol.163 (4), p.1080-1084.e1</ispartof><rights>Mosby, Inc.</rights><rights>2013 Mosby, Inc.</rights><rights>Copyright © 2013 Mosby, Inc. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c504t-36f803282c2fb25cb1ecdadc269e73c10a8b1a0731421214cad53c8677327c5c3</citedby><cites>FETCH-LOGICAL-c504t-36f803282c2fb25cb1ecdadc269e73c10a8b1a0731421214cad53c8677327c5c3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.jpeds.2013.05.060$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,776,780,3536,27903,27904,45974</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/23866715$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Barber, Brent J., MD</creatorcontrib><creatorcontrib>Andrews, Jennifer G., MBA</creatorcontrib><creatorcontrib>Lu, Zhenqiang, PhD</creatorcontrib><creatorcontrib>West, Nancy A., PhD</creatorcontrib><creatorcontrib>Meaney, F. John, PhD</creatorcontrib><creatorcontrib>Price, Elinora T., BA</creatorcontrib><creatorcontrib>Gray, Ashley, BS</creatorcontrib><creatorcontrib>Sheehan, Daniel W., MD</creatorcontrib><creatorcontrib>Pandya, Shree, PT, DPT, MS</creatorcontrib><creatorcontrib>Yang, Michele, MD</creatorcontrib><creatorcontrib>Cunniff, Christopher, MD</creatorcontrib><title>Oral Corticosteroids and Onset of Cardiomyopathy in Duchenne Muscular Dystrophy</title><title>The Journal of pediatrics</title><addtitle>J Pediatr</addtitle><description>Objective To estimate the age when cardiomyopathy develops in boys with Duchenne muscular dystrophy (DMD) and to analyze the effect of corticosteroid treatment on the age of cardiomyopathy onset. Study design We identified a population-based sample of 462 boys with DMD, born between 1982 and 2005, in 5 surveillance sites in the US. Echocardiographic and corticosteroid treatment data were collected. Cardiomyopathy was defined by a reduced fractional shortening (&lt;28%) or ejection fraction (&lt;55%). The age of cardiomyopathy onset was determined. Survival analysis was performed to determine the effects of corticosteroid treatment on cardiomyopathy onset. Results The mean (SD) age of cardiomyopathy onset was 14.3 (4.2) years for the entire population and 15.2 (3.4) years in corticosteroid-treated vs 13.1 (4.8) in non-treated boys. Survival analysis described a significant delay of cardiomyopathy onset for boys treated with corticosteroids ( P &lt; .02). By 14.3 years of age, 63% of non-treated boys had developed cardiomyopathy vs only 36% of those treated. Among boys treated with corticosteroids, there is a significant positive effect of duration of corticosteroid treatment on cardiomyopathy onset ( P &lt; .0001). For every year of corticosteroid treatment, the probability of developing cardiomyopathy decreased by 4%. Conclusions Oral corticosteroid treatment was associated with delayed cardiomyopathy onset. The duration of corticosteroid treatment also correlated positively with delayed cardiomyopathy onset. Our analysis suggests that a boy with DMD treated for 5 years with corticosteroids might experience a 20% decrease in the likelihood of developing cardiomyopathy compared with untreated boys.</description><subject>Administration, Oral</subject><subject>Adolescent</subject><subject>adrenal cortex hormones</subject><subject>Adrenal Cortex Hormones - administration &amp; dosage</subject><subject>Adrenal Cortex Hormones - therapeutic use</subject><subject>Age of Onset</subject><subject>boys</subject><subject>Cardiomyopathies - complications</subject><subject>Cardiomyopathies - drug therapy</subject><subject>Cardiomyopathies - epidemiology</subject><subject>cardiomyopathy</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>correlation</subject><subject>Echocardiography</subject><subject>Humans</subject><subject>Male</subject><subject>monitoring</subject><subject>muscular dystrophy</subject><subject>Muscular Dystrophy, Duchenne - complications</subject><subject>Muscular Dystrophy, Duchenne - drug therapy</subject><subject>Pediatrics</subject><subject>probability</subject><subject>Regression Analysis</subject><subject>Time Factors</subject><subject>Treatment Outcome</subject><issn>0022-3476</issn><issn>1097-6833</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2013</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkk1v1DAQhi0EotvCL0CCHLkkjO3Ezh5AQtvyIRXtofRseccT1iEbBztByr8nYQsHLpzm8rwzo2eGsRccCg5cvWmLdiCXCgFcFlAVoOAR23DY6lzVUj5mGwAhcllqdcEuU2oBYFsCPGUXQtZKaV5t2H4fbZftQhw9hjRSDN6lzPYu2_eJxiw02c5G58NpDoMdj3Pm--x6wiP1PWVfpoRTZ2N2PacxhuE4P2NPGtslev5Qr9j9h5uvu0_57f7j59372xwrKMdcqqYGKWqBojmICg-c0FmHQm1JS-Rg6wO3oCUvBRe8ROsqibXSWgqNFcor9vrcd4jhx0RpNCefkLrO9hSmZHgpdaW3QsgFlWcUY0gpUmOG6E82zoaDWU2a1vw2aVaTBiqzmFxSLx8GTIcTub-ZP-oW4NUZaGww9lv0ydzfLR2qRXNdw3Yd_PZM0CLip6doEnrqkZyPhKNxwf9nhXf_5LHzvUfbfaeZUhum2C-ODTdJGDB367nXa3MJoPTyA78Ax7mjQw</recordid><startdate>20131001</startdate><enddate>20131001</enddate><creator>Barber, Brent J., MD</creator><creator>Andrews, Jennifer G., MBA</creator><creator>Lu, Zhenqiang, PhD</creator><creator>West, Nancy A., PhD</creator><creator>Meaney, F. John, PhD</creator><creator>Price, Elinora T., BA</creator><creator>Gray, Ashley, BS</creator><creator>Sheehan, Daniel W., MD</creator><creator>Pandya, Shree, PT, DPT, MS</creator><creator>Yang, Michele, MD</creator><creator>Cunniff, Christopher, MD</creator><general>Mosby, Inc</general><scope>FBQ</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20131001</creationdate><title>Oral Corticosteroids and Onset of Cardiomyopathy in Duchenne Muscular Dystrophy</title><author>Barber, Brent J., MD ; Andrews, Jennifer G., MBA ; Lu, Zhenqiang, PhD ; West, Nancy A., PhD ; Meaney, F. John, PhD ; Price, Elinora T., BA ; Gray, Ashley, BS ; Sheehan, Daniel W., MD ; Pandya, Shree, PT, DPT, MS ; Yang, Michele, MD ; Cunniff, Christopher, MD</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c504t-36f803282c2fb25cb1ecdadc269e73c10a8b1a0731421214cad53c8677327c5c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2013</creationdate><topic>Administration, Oral</topic><topic>Adolescent</topic><topic>adrenal cortex hormones</topic><topic>Adrenal Cortex Hormones - administration &amp; dosage</topic><topic>Adrenal Cortex Hormones - therapeutic use</topic><topic>Age of Onset</topic><topic>boys</topic><topic>Cardiomyopathies - complications</topic><topic>Cardiomyopathies - drug therapy</topic><topic>Cardiomyopathies - epidemiology</topic><topic>cardiomyopathy</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>correlation</topic><topic>Echocardiography</topic><topic>Humans</topic><topic>Male</topic><topic>monitoring</topic><topic>muscular dystrophy</topic><topic>Muscular Dystrophy, Duchenne - complications</topic><topic>Muscular Dystrophy, Duchenne - drug therapy</topic><topic>Pediatrics</topic><topic>probability</topic><topic>Regression Analysis</topic><topic>Time Factors</topic><topic>Treatment Outcome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Barber, Brent J., MD</creatorcontrib><creatorcontrib>Andrews, Jennifer G., MBA</creatorcontrib><creatorcontrib>Lu, Zhenqiang, PhD</creatorcontrib><creatorcontrib>West, Nancy A., PhD</creatorcontrib><creatorcontrib>Meaney, F. John, PhD</creatorcontrib><creatorcontrib>Price, Elinora T., BA</creatorcontrib><creatorcontrib>Gray, Ashley, BS</creatorcontrib><creatorcontrib>Sheehan, Daniel W., MD</creatorcontrib><creatorcontrib>Pandya, Shree, PT, DPT, MS</creatorcontrib><creatorcontrib>Yang, Michele, MD</creatorcontrib><creatorcontrib>Cunniff, Christopher, MD</creatorcontrib><collection>AGRIS</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>The Journal of pediatrics</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Barber, Brent J., MD</au><au>Andrews, Jennifer G., MBA</au><au>Lu, Zhenqiang, PhD</au><au>West, Nancy A., PhD</au><au>Meaney, F. John, PhD</au><au>Price, Elinora T., BA</au><au>Gray, Ashley, BS</au><au>Sheehan, Daniel W., MD</au><au>Pandya, Shree, PT, DPT, MS</au><au>Yang, Michele, MD</au><au>Cunniff, Christopher, MD</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Oral Corticosteroids and Onset of Cardiomyopathy in Duchenne Muscular Dystrophy</atitle><jtitle>The Journal of pediatrics</jtitle><addtitle>J Pediatr</addtitle><date>2013-10-01</date><risdate>2013</risdate><volume>163</volume><issue>4</issue><spage>1080</spage><epage>1084.e1</epage><pages>1080-1084.e1</pages><issn>0022-3476</issn><eissn>1097-6833</eissn><abstract>Objective To estimate the age when cardiomyopathy develops in boys with Duchenne muscular dystrophy (DMD) and to analyze the effect of corticosteroid treatment on the age of cardiomyopathy onset. Study design We identified a population-based sample of 462 boys with DMD, born between 1982 and 2005, in 5 surveillance sites in the US. Echocardiographic and corticosteroid treatment data were collected. Cardiomyopathy was defined by a reduced fractional shortening (&lt;28%) or ejection fraction (&lt;55%). The age of cardiomyopathy onset was determined. Survival analysis was performed to determine the effects of corticosteroid treatment on cardiomyopathy onset. Results The mean (SD) age of cardiomyopathy onset was 14.3 (4.2) years for the entire population and 15.2 (3.4) years in corticosteroid-treated vs 13.1 (4.8) in non-treated boys. Survival analysis described a significant delay of cardiomyopathy onset for boys treated with corticosteroids ( P &lt; .02). By 14.3 years of age, 63% of non-treated boys had developed cardiomyopathy vs only 36% of those treated. Among boys treated with corticosteroids, there is a significant positive effect of duration of corticosteroid treatment on cardiomyopathy onset ( P &lt; .0001). For every year of corticosteroid treatment, the probability of developing cardiomyopathy decreased by 4%. Conclusions Oral corticosteroid treatment was associated with delayed cardiomyopathy onset. The duration of corticosteroid treatment also correlated positively with delayed cardiomyopathy onset. Our analysis suggests that a boy with DMD treated for 5 years with corticosteroids might experience a 20% decrease in the likelihood of developing cardiomyopathy compared with untreated boys.</abstract><cop>United States</cop><pub>Mosby, Inc</pub><pmid>23866715</pmid><doi>10.1016/j.jpeds.2013.05.060</doi><tpages>5</tpages></addata></record>
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subjects Administration, Oral
Adolescent
adrenal cortex hormones
Adrenal Cortex Hormones - administration & dosage
Adrenal Cortex Hormones - therapeutic use
Age of Onset
boys
Cardiomyopathies - complications
Cardiomyopathies - drug therapy
Cardiomyopathies - epidemiology
cardiomyopathy
Child
Child, Preschool
correlation
Echocardiography
Humans
Male
monitoring
muscular dystrophy
Muscular Dystrophy, Duchenne - complications
Muscular Dystrophy, Duchenne - drug therapy
Pediatrics
probability
Regression Analysis
Time Factors
Treatment Outcome
title Oral Corticosteroids and Onset of Cardiomyopathy in Duchenne Muscular Dystrophy
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