[The Duchenne muscular dystrophy continuum: a modified staging classification].

OBJECTIVE

To clarify the classification of the stages of Duchenne muscular dystrophy (DMD) using the concept of a continuum, to provide a clinical and functional description of each stage and recommendations for therapeutic approaches at each stage.

MATERIAL AND

METHODS

A review of domestic and foreign studies devoted to the clinical course of DMD, with an emphasis on functional changes at various stages of the disease. Own data on the observation of more than 1.300 patients with DMD and BMD over the previous 11 years.

RESULTS

The proposed modified classification of DMD includes eight stages: 1 - stage of solitary clinical manifestations, 2 - early ambulatory, 3 - advanced ambulatory, 4 - late ambulatory, 5 - pre-non-ambulatory, 6 - early non-ambulatory, 7 - late non-ambulatory, 8 - terminal. The features of the clinical picture and functional changes at each stage are presented.

CONCLUSION

Understanding the concept of a continuous-stage (quasi-continuous) process in the progression of DMD is important for individualizing approaches to pathogenetic therapy, timely initiation of maintenance treatment (glucocorticosteroids, pathogenetic drugs, cardioprotectors, high doses of vitamin D3, bisphosphonates and other drugs) and the inclusion of standards or care, which will help improve the prognosis of the disease and improve the quality of life of both the patient and his family members.