[The effect of nasal IPPV on patients with respiratory failure during sleep due to Duchenne muscular dystrophy].

In order to investigate respiratory failure during sleep in patients with Duchenne muscular dystrophy (DMD), overnight arterial oxygen saturation (SaO2) and end-tidal CO2 (EtCO2) monitoring by capnographoximeter was performed. We supposed that average of EtCO2 over 60 mmHg documented by continuous overnight capnograph study indicated the need for introducing nocturnal respiratory assistance. Accordingly, four patients who showed EtCO2 over 60 mmHg were initiated to treat with nocturnal nasal intermittent positive pressure ventilation (NIPPV). The first ventilator settings were adjusted for patient comfort and to attain near normal arterial blood gas values while the patients were awake on NIPPV. After the patients were able to tolerate NIPPV for the whole night, overnight recording of SaO2 and EtCO2 on nocturnal NIPPV were made to assure the adequacy of ventilation and to provide basis for adjusting ventilator settings. Subsequently, appropriate nocturnal NIPPV could normalize overnight SaO2 and EtCO2, improve daytime arterial PO2 and PCO2, and reverse symptoms of chronic alveolar hypoventilation in these patients. According to further decline in pulmonary function, efficacy of NIPPV must be checked periodically by overnight monitoring, and ventilator resettings should be done if necessary. We believe that early awareness and appropriate management of respiratory failure during sleep by NIPPV are important to postpone tracheostomy for patients with DMD.