Vitamin D in Patients With Duchenne Muscular Dystrophy

Abstract

Objectives: Duchenne Muscular Dystrophy (DMD) is one of the neuromuscular junction dystrophies, characterized by deficiency of dystrophin and inherited as an X-linked recessive trait. It has a progressive nature, and results in loss of ambulation and death. Poor bone-marrow health is an important issue in terms of morbidity in DMD. Vitamin D is an important pre-prohormone related to bone health. Although steroids used in treatment of DMD are effective on progression of the disease they have adverse effects on bone heatlh and 25(OH)-vitamin D (vitamin D) levels. We aimed in the present study to investigate levels of vitamin D and its relationship with steroid use and ambulation in the patients with DMD.

Material and Metods: The present study is a case-control study including 96 male patients aged between 2 to 18 years with diagnosis of DMD and 48 healthy volunteers with matching gender and age distribution.

Results: Deficiency or insufficiency of vitamin D level was found in 95.83% of the patients with DMD. The patients with DMD were compared to the healthy volunteers and significantly lower level of vitamin D was found in the group of patients (p = 0.003). No significant difference was found in levels of vitamin D between the patients with DMD who were using steroids and those not using steroids and between the patient who were ambulated or not ambulated (p = 0.741 and p=0.785).

Conclusion: Majority of the patients with DMD had deficient or insufficient level of vitamin D. Thus, in the terms of protecting bone health it is important to assess level of vitamin D regularly from the early stages of the disease and during the routine controls, to implement additional treatments in the case of deficiency or insufficiency.

Keywords

• Duchenne Muscular Dystrophy,Vitamin D,Steroid

Duchenne Muskuler Distrofili Hastalarda Vitamin D Düzeyi

Öz

Amaç: Duchenne Musküler Distrofi (DMD), kavşak tipi distrofilerden olup X’e bağlı resesif geçen, distrofin eksikliği karakterize kas hastalığıdır. İlerleyicidir, ambulasyon kaybı ve erken dönemde ölümlere yol açar. Zayıf kemik sağlığı DMD’de morbidite açısından önemli bir sorundur. Vitamin D, kemik sağlığı açısından önemli bir preprohormondur. DMD tedavisinde kullanılan steroidlerin hastalığın progresyonu üzerinde etkin tedavisi olmasına rağmen kemik sağlığı ve vitamin D üzerinde olumsuz etkileri bulunmaktadır. DMD’li hastalarda serum 25(OH)vitamin D (vitamin D) düzeylerini, vitamin D’nin steroid kullanımı ve ambulasyonla ilişkisini değerlendirmeyi amaçlandık.

Materyal-Metot: Vaka kontrol olan çalışmaya doksan altı kesin DMD tanısı almış, 2-18 yaş, erkek hasta ve kırk sekiz benzer yaş ortalaması ve cinsiyette sağlıklı gönüllüler alındı.

Bulgular: DMD’li hastalarda %95,83 oranında vitamin D düzeylerinde eksiklik – yetersizlik saptandı. DMD ‘li hastalar ile sağlıklı gönüllüler karşılaştırıldı, vitamin D düzeylerinde hasta grubunda anlamlı düşüklük saptandı (p=0.003). DMD’li steroid alan ve almayanlar ile ambulasyonu olan ve olmayan hastalar arasında vitamin D düzeyleri açısından fark saptanmadı (p=0.741 ve p=0.785).

Sonuç: DMD’li hastalarda, yüksek oranda vitamin D düzeylerinde eksiklik-yetersizlik mevcuttu. Bu nedenle D vitamin düzeylerinin erken dönemden itibaren ve rutin kontrollerde düzenli değerlendirilmesi ve eksiklik-yetersizliğinde ilave tedavilerinin yapılması kemik sağlığını korumak açısından oldukça önemlidir.

Anahtar Kelimeler

• Duchenne Muskuler Distrofi,vitamin D,Steroid

Kaynakça

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