Vitamin D in Patients With Duchenne Muscular Dystrophy

Yıl 2019, , 307 - 315, 28.06.2019

Filiz Meryem Sertpoyraz , Bedile İrem Tiftikcioglu Figen Baydan Bakiye Tunçay Oya Halıcıoğlu Baltalı

https://doi.org/10.17098/amj.582015

Öz

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.

Anahtar Kelimeler

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

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