Evaluarea managementului respirator la copiii cu distrofie musculară Duchenne

Duchenne muscular dystrophy is an X-linked genetic dis­order that involves the gene responsible for the synthesis of dys­trophin. Decreased dystrophin levels in the body cause a progressive loss in muscle strength that affects ske­le­tal muscle structures and evolves towards disability. Death occurs after a variable period, depending on the case manage­ment. Impaired lung function is gradual and progresses to chronic respiratory failure. The severity of respiratory impairment is assessed by careful and periodic monitoring. Along with the information pro­vided by spirometry and muscle inspiratory and ex­pi­ra­tory pressures, the ap­pli­ca­tion of the pediatric sleep questionnaire, the study of poly­somno­graphic sleep and the monitoring of CO2 levels during sleep are practiced. All these provide information about the impact of this disease on the respiratory system, about the time of sleep respiratory disorders onset and select patients who need support through assisted cough and noninvasive ventilation. The authors present the results of respiratory monitoring in children diagnosed with Duchenne mus­cu­lar dystrophy and sleep respiratory disorders and ob­serve the impact over time of the non­inva­sive nocturnal ven­ti­la­tion treatment.