Turkiye Klinikleri Journal of Pediatrics

.: ORIGINAL RESEARCH
Investigation of the Oxidative Process by Measuring Total Antioxidant Capacity and Total Oxidant Capacity in Patients with Mucopolysaccharidosis: Cross-Sectional Prospective Study
Mukopolisakkaridoz Hastalarında Total Antioksidan Kapasite ve Total Oksidan Kapasite Ölçümleri Yapılarak Oksidatif Sürecin Araştırılması: Kesitsel Prospektif Çalışma
Eylem Şerife KALKANa , Aynur KÜÇÜKÇONGAR YAVAŞb , Özlem ÜNAL UZUNc , Mehmet GÜNDÜZb
a Ankara City Hospital, Clinic of Pediatrics, Ankara, Türkiye
bAnkara City Hospital, Clinic of Pediatric Metabolism, Ankara, Türkiye
cKocaeli University Faculty of Medicine, Department of Pediatric Metabolism, Kocaeli, Türkiye
Turkiye Klinikleri J Pediatr. 2024;33(1):1-7
doi: 10.5336/pediatr.2023-98909
Article Language: EN
Full Text
ABSTRACT
 Objective: To compare the oxidative stress status between the patients with mucopolysaccharidosis who received and did not receive enzyme replacement therapy and healthy control group. Material and Methods: A retrospective study of mucopolysaccharidosis patients with analysis of the total oxidant and antioxidant capacity levels. Results: We included 29 mucopolysaccharidosis patients aged between 1-18 years and 50 healthy children. Total antioxidant capacity and total oxidant capacity parameters between this patients and healthy control group were compared. The median age of the patients who were included in the study was 9.0 years and the median age of the cases included in the control group was 8.0 years. Total antioxidant capacity, total oxidant capacity and Oxidative Stress Index (OSI) values did not differ significantly in patients receiving enzyme replacement therapy compared to patients who did not receive enzyme replacement therapy. A comparison of the oxidant and antioxidant system parameters between the patient and control groups revealed a distinct difference, with the control group exhibiting a considerably higher total antioxidant capacity than mucopolysaccharidosis patients, and the patient group showing a significantly higher total oxidant capacity and OSI. Conclusion: In this study, it was observed that the antioxidant defence system decreased in patients with mucopolysaccharidoses. It is thought that, in addition to standard treatments, the administration of antioxidant treatments and supporting nutrition in mucopolysaccharidosis patients will increase the quality of life of the patients. Regular sleep, eating healthy foods and doing regular exercise would also increase the effectiveness of these supplements.

Keywords: Mucopolysaccharidoses; oxidative stress; oxidants; antioxidants
ÖZET
 Amaç: Bu çalışmanın amacı, enzim replasman tedavisi alan ve almayan mukopolisakkaridozlu hastalar ile sağlıklı kontrol grubu arasındaki oksidatif stres durumunu karşılaştırmaktır. Gereç ve Yöntemler: Total oksidan kapasite ve total antioksidan kapasite düzeylerinin analizi ile mukopolisakkaridoz hastalarının oksidatif stres seviyelerinin retrospektif incelenmesi. Bulgular: Çalışmaya 1-18 yaş arası 29 mukopolisakkaridoz hastası ve aynı yaş grubunda 50 sağlıklı çocuk dâhil edildi. Bu hastalar ile sağlıklı kontrol grubu arasındaki toplam antioksidan kapasite ve toplam oksidan kapasite parametreleri karşılaştırıldı. Çalışmaya alınan hastaların yaş ortalaması 9,0 iken, kontrol grubuna alınan olguların yaş ortalaması 8,0 yaş idi. Enzim replasman tedavisi alan hastalarda total antioksidan kapasite, total oksidan kapasite ve Oksidatif Stres İndeksi (OSİ) değerleri, enzim replasman tedavisi almayan hastalara göre anlamlı farklılık göstermedi. Hasta grubu ve kontrol grubu değerleri oksidan ve antioksidan sistem parametreleri açısından karşılaştırıldığında, kontrol grubunun total antioksidan kapasite değeri mukopolisakkaridoz hastalarına göre anlamlı olarak yüksek olmasına rağmen total oksidan kapasite ve OSİ değerleri hasta grubunda anlamlı olarak yüksek bulundu. Sonuç: Bu çalışmada, mukopolisakkaridozlu hastalarda antioksidan savunma sisteminin azaldığı gözlendi. Mukopolisakkaridoz hastalarında standart tedavilere ek olarak antioksidan tedaviler ve beslenmeyi destekleyici uygulamaları hastaların yaşam kalitelerini artırabilir. Düzenli uyku, sağlıklı besinler yemek ve düzenli egzersiz yapmak da bu takviyelerin etkinliğini artırabilir.

Anahtar Kelimeler: Mukopolisakkaridozlar; oksidatif stres; oksidanlar; antioksidanlar
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