Büşra YILMAZ EKİCİ, Sabiha Zeynep Aydenk KÖSEOĞLU

Duchenne Musküler Distrofi’si olan Çocuklarda Güncel Tıbbi Beslenme Tedavisi ve Önemi

İlerleyici bir kas erimesi hastalığı olan Duchenne musküler distrofisi (DMD), distrofin proteini eksikliğine bağlı olarak ortaya çıkan önemli nöromusküler bir hastalıktır. Bu hastalarda ilerleyen yaş dönemlerinde solunum, kalp, beslenme gibi fonksiyonlarında sorunların arttığı dikkati çekmektedir. Yaşla birlikte artan bu sorunlar, obezite ve yetersiz beslenme gelişme riskini artırmakta ve hastalığın prognozunun daha kötüye gitmesine neden olmaktadır. DMD hastalığının kesin tedavisi olmamakla birlikte tedaviye ek olarak, hastaların obezite ve malnütrisyondan korunması, beslenme ve gastrointestinal sorunların varlığının doğrultusunda diyetsel değişikliklerinin yapılması önem taşımaktadır. DMD’li hastalarda kas bütünlüğünü korumaya yardımcı olacak bir beslenme düzeninin oluşturulması, erken yaşta başlayan kemik sağlığı problemlerini ve kilo üzerindeki olumsuz etkilerini azaltmada önemli rol oynamaktadır. Aynı zamanda uygulanan beslenme düzenine, sadece DMD’li hastanın değil tüm ailenin uyması, çocuğun bu düzene daha kolaylıkla alışmasını ve sürdürmesini sağlayacaktır. Bu nedenle DMD’li çocuklarda beslenme sorunları erken dönemde belirlenerek uygun tedavi yaklaşımları planlanmalı ve bu konuda multidisipliner bir ekiple tedavinin sürdürülmesi daha uygun olacaktır.

Anahtar Kelimeler:

duchenne musküler distrofi, çocuk, obezite, malnütrisyon, beslenme sorunu, diyet tedavisi

Current Medical Nutrition Therapy and Its Importance in Children with Duchenne Muscular Dystrophy Abstract

Duchenne muscular dystrophy (DMD), a progressive muscle wasting disease, is an important neuromuscular disease that occurs due to dystrophin protein deficiency. It is noteworthy that in these patients, problems in functions such as breathing, heart and nutrition increase in advancing age. These problems, which increase with age, increase the risk of obesity and malnutrition and cause the prognosis of the disease to worsen. Although there is no definitive treatment for DMD, in addition to treatment, it is important to protect patients from obesity and malnutrition, and to make dietary changes in line with the presence of nutrition and gastrointestinal problems. Establishing a diet that will help protect muscle integrity in patients with DMD plays an important role in reducing early-onset bone health problems and their negative effects on weight. At the same time, compliance with the applied diet, not only for the patient with DMD, but also for the whole family, will enable the child to get used to and maintain this order more easily. For this reason, nutritional problems in children with DMD should be determined at an early stage and appropriate treatment approaches should be planned, and it would be more appropriate to continue the treatment with a multidisciplinary team.

Keywords:

duchenne muscular dystrophy, child, obesity, malnutrition, nutritional problem, diet therapy,

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