Recomendaciones sobre el manejo integral para la transición óptima a la vida adulta del paciente con distrofia muscular de Duchenne

Introduction: Treatment for patients with Duchenne muscular dystrophy (DmD) has prolonged their walk ing ability and survival, resulting in an increasing number of DmD patients transitioning from adoles cence to adulthood. Methods: A panel of 58 expert specialists was convened, who had access to evidence resulting from a literature review before consensus. During the consensus meeting, recommendations on the transition to adulthood for DmD patients were developed based on available evidence and clinical expertise. A favorability threshold of 85 % was determined, and all recommendations were accepted with a favorability percentage greater than 90 %. Conclusion: This article provides management recom mendations for DmD patients during the childhood and adolescent period, aimed at prolonging walking ability, reducing the decline in respiratory function, and mitigating the consequences of dilated car diomyopathy, which are the main causes of preventable premature death that hinder the transition to adulthood. Emphasis is placed on systematic clinical and paraclinical evaluations, the use of deflazacort as the corticosteroid of choice, cardioprotectors, vitamin D, and bisphosphonates within pharmacologi cal management, as well as timely nutrition strategies, non-invasive respiratory care, spinal arthrodesis, and other orthopedic procedures, among the main indications.