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Steroid Therapy and Cardiac Function in Duchenne Muscular Dystrophy

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Abstract

Duchenne muscular dystrophy leads to progressive deterioration in skeletal and cardiac muscle function. Steroids prolong ambulation and improve respiratory muscle strength. The authors hypothesized that steroid treatment would stabilize cardiac muscle function. Echocardiograms performed from 1997 to 2004 for 111 subjects 21 years of age or younger with Duchenne muscular dystrophy were restrospectively reviewed. The medical record was reviewed for steroid treatment. Untreated and steroids-treated subjects did not differ in age, height, weight, body mass index, systolic and diastolic blood pressure, or left ventricular mass. The shortening fraction was lower in the untreated group. Of those treated, 29 received prednisone and 19 received deflazacort. There was no difference in the shortening fraction between the two treated subgroups. Treated subjects not receiving steroids still had a normal shortening fraction, which was no different from the shortening fraction of those still receiving treatment. As compared with the treated subjects, the untreated subjects 10 years of age or younger were 4.4 times more likely to have a shortening fraction less than< 28% (p = 0.03), and the untreated subjects older than 10 years were 15.2 times more likely to have a shortening fraction less than< 28% (p < 0.01). This retrospective study suggests that the progressive decline in cardiac function of patients with Duchenne muscular dystrophy can be altered by steroid treatment. The effect appears to be sustained beyond the duration of treatment and independent of steroid type.

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References

  1. WD Biggar M Gingras DL Fehlings VA Harris CA Steele (2001) ArticleTitleDeflazacort treatment of Duchenne muscular dystrophy J Piediatr 138 45–50 Occurrence Handle1:CAS:528:DC%2BD3MXhtF2msr0%3D

    CAS  Google Scholar 

  2. BJ Boland PL Silbert RV Groover PC Wollan MD Silverstein (1996) ArticleTitleSkeletal, cardiac, and smooth muscle failure in Duchenne muscular dystrophy Pediatr Neurol 14 7–12 Occurrence Handle10.1016/0887-8994(95)00251-0 Occurrence Handle1:STN:280:BymB3szhs1c%3D Occurrence Handle8652023

    Article  CAS  PubMed  Google Scholar 

  3. MH Brooke GM Fenichel RC Griggs JR Mendell RT Moxley SuffixIII et al. (1987) ArticleTitleClinical investigation of Duchenne muscular dystrophy: Interesting results in a trial of prednisone Arch Neurol 44 812–817 Occurrence Handle1:STN:280:BiiA38rnsFQ%3D Occurrence Handle3632393

    CAS  PubMed  Google Scholar 

  4. K Bushby F Muntoni A Urtizberea R Hughes R Griggs (2004) ArticleTitleTreatment of Duchenne muscular dystrophy. Defining the gold standards of management in the use of corticosteroids. Report of the 124th ENMC International Workshop Neuromuscul Disord 14 526–534 Occurrence Handle1:STN:280:DC%2BD2cvjt1GrtA%3D%3D Occurrence Handle15336694

    CAS  PubMed  Google Scholar 

  5. AM Connolly J Schierbecker R Renna J Florence (2002) ArticleTitleHigh-dose weekly oral prednisone improves strength in boys with Duchenne muscular dystrophy Neuromuscul Disord 12 917–925 Occurrence Handle12467746

    PubMed  Google Scholar 

  6. S DeSilva DB Drachman D Mellits RW Kuncl (1987) ArticleTitlePrednisone treatment in Duchenne muscular dystrophy: Long-term benefit Arch Neurol 44 818–822 Occurrence Handle1:STN:280:BiiA38rnsFU%3D Occurrence Handle3632394

    CAS  PubMed  Google Scholar 

  7. RB Devereux DR Alonso EM Lutas et al. (1986) ArticleTitleEchocardiographic assessment of left ventricular hypertrophy: Comparison to necropsy findings Am J Cardiol 57 450–458 Occurrence Handle1:STN:280:BimC38jgslw%3D Occurrence Handle2936235

    CAS  PubMed  Google Scholar 

  8. L D’Orsogna JP O’Shea G Mllier (1988) ArticleTitle Cardiomyopathy of Duchenne muscular dystrophy Pediatr Cardiol 96 205–213

    Google Scholar 

  9. HP Gutgesell et al. (1977) ArticleTitleEvaluation of left ventricular size and function by echocardiography: Results in normal children Circulation 56 457–462 Occurrence Handle1:STN:280:CSiB38vgsVU%3D Occurrence Handle884801

    CAS  PubMed  Google Scholar 

  10. SB Heymsfield T McNish JV Perkins JM Felner (1978) ArticleTitleSequence of cardiac changes in Duchenne muscular dystrophy Am Heart J 95 283–294 Occurrence Handle1:STN:280:CSeC3c%2FjsVc%3D Occurrence Handle622971

    CAS  PubMed  Google Scholar 

  11. GW Manning CJ Cropp (1958) ArticleTitleElectrocardiogram in progressive muscular dystrophy Br Heart J 20 416–420 Occurrence Handle1:STN:280:CyeC3czotVw%3D Occurrence Handle13560702

    CAS  PubMed  Google Scholar 

  12. AY Manzur T Kuntzer M Pike A Swan (2004) ArticleTitleGlucocorticoid corticosteroids for Duchenne muscular dystrophy Cochrane Database Syst Rev 2 CD003725 Occurrence Handle15106215

    PubMed  Google Scholar 

  13. LE Mesa AL Dubrovsky J Corderi P Marco D Flores (1991) ArticleTitleSteroids in Duchenne muscular dystrophy: Deflazacort trial Neuromuscul Disord 1 261–266 Occurrence Handle1:STN:280:By2A2MzntFU%3D Occurrence Handle1822804

    CAS  PubMed  Google Scholar 

  14. JK Perloff AC Leon Particlede SuffixJr D O’Doherty (1996) ArticleTitleThe cardiomyopathy of progressive muscular dystrophy Circulation 33 625–648

    Google Scholar 

  15. JK Perloff WC Roberts AC deLeon D O’Doherty (1967) ArticleTitleDistinctive electrocardiography of Duchenne’s progressive muscular dystrophy: An electrocardiographic–pathologic correlative study Am J Med 42 179–188 Occurrence Handle10.1016/0002-9343(67)90017-4 Occurrence Handle1:STN:280:CCiD1M3ps1A%3D Occurrence Handle6018530

    Article  CAS  PubMed  Google Scholar 

  16. K Sasaki K Sakata E Kachi S Hirata T Ishihara K Ishikawa (1998) ArticleTitleSequential changes in cardiac structure and function in patients with Duchenne type muscular dystrophy: A two-dimensional echocardiographic study Am Heart J 135 IssueID6 pt 1 937–944 Occurrence Handle1:STN:280:DyaK1c3pt1Snug%3D%3D Occurrence Handle9630096

    CAS  PubMed  Google Scholar 

  17. J Schott M Jacobi MA Wald (1955) ArticleTitleElectrocardiographic patterns in the differential diagnosis of progressive muscular dystrophy Am J Med Sci 229 517–524 Occurrence Handle1:STN:280:CyqD28zgsF0%3D Occurrence Handle14361418

    CAS  PubMed  Google Scholar 

  18. CK Silversides GD Webb VA Harris WD Biggar (2003) ArticleTitleEffects of deflazacort on left ventricular functions in patients with Duchenne muscular dystrophy Am J Cardiol 91 769–772 Occurrence Handle1:CAS:528:DC%2BD3sXhvFeqtbs%3D Occurrence Handle12633823

    CAS  PubMed  Google Scholar 

  19. PL Wahi KC Bhargava S Mohindra (1971) ArticleTitleCardiorespiratory changes in progressive muscular dystrophy Br Heart J 33 533–537 Occurrence Handle1:STN:280:CS6B2M%2FhvF0%3D Occurrence Handle5557466

    CAS  PubMed  Google Scholar 

  20. BL Wong C Christopher (2002) ArticleTitleCorticosteroids in Duchenne muscular dystrophy: A reappraisal J Child Neurol 17 183–190 Occurrence Handle12026233

    PubMed  Google Scholar 

  21. O Yilmaz A Karaduman H Topaloglu (2004) ArticleTitlePrednisolone therapy in Duchenne muscular dystrophy prolongs ambulation and prevents scoliosis Eur J Neurol 11 541–544 Occurrence Handle10.1111/j.1468-1331.2004.00866.x Occurrence Handle1:STN:280:DC%2BD2czntVOjtg%3D%3D Occurrence Handle15272899

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatrics, Division of Pediatric Cardiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, 45229, USA

    L.W. Markham, R.L. Spicer, P.R. Khoury & L.H. Cripe

  2. Department of Neurology, Cincinnati Children’ Hospital Medical Center, Cincinnati, OH, 45229, USA

    B.L. Wong

  3. Department of Pediatrics and Neurology, University of Iowa, Iowa city, Iowa , 52242, USA

    K.D. Mathews

Authors
  1. L.W. Markham
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  2. R.L. Spicer
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  3. P.R. Khoury
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  4. B.L. Wong
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  5. K.D. Mathews
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  6. L.H. Cripe
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Correspondence to L.W. Markham.

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This work is supported by a grant from the Parent Project Muscular Dystrophy.

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Markham, L., Spicer, R., Khoury, P. et al. Steroid Therapy and Cardiac Function in Duchenne Muscular Dystrophy. Pediatr Cardiol 26, 768–771 (2005). https://doi.org/10.1007/s00246-005-0909-4

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