Declaración de posición. Recomendaciones sobre el uso de escalas de seguimiento y evaluación y manejo de la salud ósea en la distrofia muscular de Duchenne

Duchenne muscular dystrophy (dmd) is a disease characterized by progressive muscle weakness. Glucocorticoids have been shown to prolong the time of ambulation, decrease the rate of progression of dmd complications, and also extend survival. However, their prolonged use is associated to side effects such as alterations in bone microarchitecture and weight gain. The initiation of treatment and the monitoring of disease progression depends on the patient’s functional status, which is assessed using motor function scales that predict functional outcomes and evaluate the impact of interventions. This article provides recommendations on the use of monitoring scales and the evaluation and management of bone health in patients with dmd. Materials and methods: An expert panel of 26 physicians from different specialties with knowledge and experience in managing patients with Duchenne muscular dystrophy was formed. Two specialists drafted recommendations on the use of monitoring scales and two others on the evaluation and management of bone health in dmd, based on literature review and their knowledge and experience on the topic. Conclusions: It is recommended to monitor patients with Duchenne muscular dystrophy every six months, using functional scales that may vary according to the patient’s clinical status. Additionally, assessment of bone health, measurement of 25-oh vitamin D levels, and nutritional recommendations for calcium intake are essential. In patients progressing to osteoporosis, intravenous bisphosphonate management should be performed.