Abstract:
BACKGROUND:Muscular dystrophies are inherited myogenic disorders characterized by progressive muscle wasting and weakness of variable distribution and severity. They are a heterogeneous group characterized by variable degree of skeletal and cardiac muscle involvement. The most common and the most severe form of muscular dystrophy is DMD. Currently, there is no curative treatment for muscular dystrophies. Several drugs have been studied to retard the progression of the muscle weakness. There is much controversy about steroid usage in muscular dystrophy with respect to regimen, adverse effects, and whether long term benefits outweigh side effects. This study is to assess steroid efficacy in children with muscular dystrophy. MATERIALS AND METHODS:All children with diagnosed muscular dystrophy by muscle biopsy, immunohistochemistry and/or genetic test were enrolled in the study. They were started on either prednisolone (0.75 mg/kg/day) or deflazacort 0.9 mg/kg/day based on affordability. All were followed up every 6 months with clinical assessment, quality of life questionnaire and clinical and laboratory assessment of side effects. Outcome measures of children on deflazacort and prednisolone at 1 year followup were summarized as numbers and percentages and were compared using Fisher's exact test. RESULTS:Twenty two children with muscular dystrophy were included (prednisolone group: 10 and deflazacort group: 12). The mean age was 7.7 years at an average followup of 26.4 months. Twenty children were diagnosed to have Duchenne's; one had Becker's muscular dystrophy while one had sarcoglycanopathy by Type 2C. All children from prednisolone group maintained their ambulatory status at 2 and 4 years followups while three on deflazacort lost their ability to walk at an average age of 11.3 years. All activities of daily living were found to be better in prednisolone group. Muscle function and time taken to walk improved in prednisolone group. Weight gain in children on prednisolone was three times more. CONCLUSIONS:Prednisolone is more beneficial than deflazacort at doses of 0.75 mg/kg/day and 0.9 mg/kg/day, respectively, however it is associated with adverse effects.
journal_name
Indian J Orthopjournal_title
Indian journal of orthopaedicsauthors
Petnikota H,Madhuri V,Gangadharan S,Agarwal I,Antonisamy Bdoi
10.4103/0019-5413.189609subject
Has Abstractpub_date
2016-09-01 00:00:00pages
551-557issue
5eissn
0019-5413issn
1998-3727pii
IJOrtho-50-551journal_volume
50pub_type
杂志文章abstract::Sports medicine physicians have a keen clinical and research interest in the anterior cruciate ligament (ACL). The biomechanical, biologic, and clinical data researchers generate, help drive injury management and prevention practices globally. The current concepts in ACL injury and surgery are being shaped by technolo...
journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
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journal_title:Indian journal of orthopaedics
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journal_title:Indian journal of orthopaedics
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journal_title:Indian journal of orthopaedics
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更新日期:2008-07-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/0019-5413.80039
更新日期:2011-05-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
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journal_title:Indian journal of orthopaedics
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章,评审
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/0019-5413.136240
更新日期:2014-07-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/0019-5413.111493
更新日期:2013-05-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/0019-5413.114918
更新日期:2013-07-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/0019-5413.80049
更新日期:2011-05-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/ortho.IJOrtho_464_17
更新日期:2018-11-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/0019-5413.136311
更新日期:2014-07-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/0019-5413.132525
更新日期:2014-05-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/0019-5413.108912
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/0019-5413.96369
更新日期:2012-05-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.1007/s43465-020-00252-x
更新日期:2020-09-10 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/0019-5413.168768
更新日期:2015-11-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
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更新日期:2019-03-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/0019-5413.159678
更新日期:2015-07-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/ortho.IJOrtho_590_17
更新日期:2019-03-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/0019-5413.201709
更新日期:2017-03-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
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更新日期:2018-11-01 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 社论
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更新日期:2020-04-08 00:00:00
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journal_title:Indian journal of orthopaedics
pub_type: 杂志文章
doi:10.4103/ortho.IJOrtho_547_17
更新日期:2019-03-01 00:00:00