Examination of Factors Affecting the Development of Osteoporosis in Children with Duchenne Muscular Dystrophy Duchenne Musküler Distrofisi Olan Çocuklarda Osteoporoz Gelişimini Etkileyen Faktörlerin İncelenmesi

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Güzin Y., Dağdaş S. M., Ateş Ö., ALATAŞ Ö., Özbay Yıldız A., Tunçay B., ...Daha Fazla

Journal of Behcet Uz Children's Hospital, cilt.15, sa.2, ss.95-104, 2025 (ESCI, Scopus, TRDizin)

Özet

Objective: Duchenne muscular dystrophy (DMD), which is primarily treated with glucocorticoids, is the most common genetic progressive neuromuscular disease in children, which can lead to osteoporosis and fractures. This study analyzed factors affecting osteoporosis before and after loss of ambulation and its relationship with fractures in DMD patients. Method: This retrospective study included 40 DMD patients. Clinical and laboratory findings and bone mineral densitometry (BMD) values were analyzed. Results: The median age at diagnosis was 3 years (Q1-Q3: 1-3.5). Osteoporosis was detected in 80% by femoral neck Z-score and 40% by vertebral Z-score, with all vertebral osteoporosis cases also meeting femoral neck osteoporosis criteria. Femoral neck Z-score worsened after loss of ambulation (p<0.05), while the lumbar Z-score remained stable. Fractures occurred in 35% of patients, with vertebral fractures in 17.5%. All vertebral fractures were associated with vertebral osteoporosis. No correlation was found between fractures and Dual-energy X-ray absorptiometry scores before loss of ambulation (p>0.05), and Z-scores were not significant predictors of fractures. The median age for glucocorticoid initiation was 48 months, with no significant difference between prednisolone and deflazacort regarding osteoporosis duration, scoliosis, or loss of ambulation (p>0.05). Scoliosis was present in 60% of patients before loss of ambulation, but no significant relationship was found between BMD and scoliosis. Conclusion: The results of this study did not show a direct correlation between BMD before the loss of ambulation and the future risk of fractures. Therefore, BMD alone may not be a sufficient predictor of scoliosis progression in DMD patients.