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뒤시엔느형 근디스트로피에서 장기간의 주기적 스테로이드 치료 1예

A Case of Long Term Periodic Corticosteroid Treatment in Duchenne Muscular Dystrophy

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초록

Duchenne muscular dystrophy (DMD) is characterized by a predictable decline in skeletal muscle strength leading to loss of ambulation, respiratory failure, and death in the second and third decade of life. No cure is yet available, but nowadays the oral corticosteroid has become a standard care, although there is a lack of consensus on the optimal treatment regimen. However, oral corticosteroid therapy has not yet popularly offered to DMD patients due to its possible negative effects in our country. Therefore, we want to report our case of DMD with long term periodic deflazacort therapy, which led to improvement in muscle strength without any serious side effects to stop the medication. After the therapy, he showed improvement in functional status from wheelchair-bounded to independent ambulator with walking aid, and didn’t show aggravation in scoliosis and respiratory function. Osteoporosis was developed after one year of treatment, but it was effectively managed with medications.

Abstract

Duchenne muscular dystrophy (DMD) is characterized by a predictable decline in skeletal muscle strength leading to loss of ambulation, respiratory failure, and death in the second and third decade of life. No cure is yet available, but nowadays the oral corticosteroid has become a standard care, although there is a lack of consensus on the optimal treatment regimen. However, oral corticosteroid therapy has not yet popularly offered to DMD patients due to its possible negative effects in our country. Therefore, we want to report our case of DMD with long term periodic deflazacort therapy, which led to improvement in muscle strength without any serious side effects to stop the medication. After the therapy, he showed improvement in functional status from wheelchair-bounded to independent ambulator with walking aid, and didn’t show aggravation in scoliosis and respiratory function. Osteoporosis was developed after one year of treatment, but it was effectively managed with medications.

참고문헌 (10)

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  4. 문재호, "뒤시엔느형 근디스트로피 환자의 포괄적 임상고찰", 대한재활의학회지, 25(2), pp.241~248 (2001), 대한재활의학회
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  7. Angelini C, "Deflazacort in Duchenne dystrophy: study of long-term effect", Muscle Nerve, 17, pp.386~391 (1994)
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