Evaluating RANKL and OPG levels in patients with Duchenne muscular dystrophy

Ali, S Akhtar; Kang, Harsimar; Olney, Robert C.; Ramos‐Platt, Leigh; Ryabets-Lienhard, Anna; Cheung, Clement C.; Georgia, Senta; Pitukcheewanont, Pisit

doi:10.1007/s00198-019-05077-5

Cited by 5 publications

(1 citation statement)

References 17 publications

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“…In adult thalassemic patients, RANK/RANKL-OPG system disorder increases in favour of osteoclasts. Many studies have noted that the RANKL/OPG ratio increases in patients with thalassemia major, and this situation shows the role of this system in the pathogenesis of osteoporosis [ 13 – 15 ]. Toumba and Skordis [ 6 ] reported that in thalassemia patients, progressive “ageing” of the bone starts even in childhood by the gradual development of an imbalance between augmented osteoclastic resorption and insufficient osteoblastic bone formation.…”

Section: Discussionmentioning

confidence: 99%

Assessment of biochemical bone markers of osteoporosis in children with thalassemia major

Çelik

Sangün²,

Ünal

et al. 2022

Ital J Pediatr

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Background Beta thalassemia major (β-TM) is a common cause of skeletal morbidity and is associated with increased bone fracture risk, particularly in inadequately transfused children. The aim of this study was to investigate some potential biochemical markers as possible early predictors of BMD variations in children with β-TM. Methods The study included 38 children with β-TM and 40 sex-age matched controls. All patients were subjected to BMD assessment by dual-energy X-ray absorptiometry (DEXA). Serum beta-crosslaps (beta-CTx), osteoprotegerin (OPG), receptor activator of nuclear factor-kappa B ligand (RANKL), urinary deoxypyridinoline (DPD) and ferritin levels were compared between the groups. Results Serum OPG levels were significantly lower in thalassemic children than in controls. The mean ratio of RANKL/OPG was significantly higher in the thalassemic patients than in the control group. Osteoporosis was detected in 10 (3 female and 7 male) of 38 patients (26.3%) according to the femur Z score and in 6 of them (4 male and 2 female) (15.8%) according to the spine Z score. Conclusions Serum OPG concentrations can be used as a biochemical marker in screening patients with beta-thalassemia major for the development of osteoporosis.

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Section: Discussionmentioning

confidence: 99%