[Application of nasal intermittent positive pressure ventilation to a case of limb-girdle muscular dystrophy].

We applied nocturnal ventilation (NV) with nasal intermittent positive pressure ventilation with custom molded mask (NIPPV-C Mclermott, 1989), as well as NV with tracheostomy intermittent positive pressure ventilation (TIPPV) to a male patient with limb-girdle muscular dystrophy who had developed chronic respiratory failure at the age of 47. NV with both methods successfully corrected nocturnal hypoxemia, improved daytime arterial blood gas values, and achieved a stable clinical course without marked deterioration for four years. Daytime PaO2 higher than 60 Torr and PaCO2 lower than 70 Torr while breathing room air were maintained with both methods, whereas PaO2 was lower than 50 Torr and PaCO2 higher than 70 Torr before the implementation of NV. TIPPV was safely suspended repeatedly for as long as two weeks, maintaining daytime PaO2 higher than 50 Torr. NIPPV-C was also repeatedly suspended for two weeks. Occasionally PaO2 dropped as low as 40 Torr after periods without NV; however, it was restored to higher than 60 Torr after one or two nights' NIPPV-C. These facts suggest that NV had a restorative effect on respiratory muscle fatigue in the present case. While on NIPPV-C, nighttime SaO2 was higher than 90% for 94% of the total time, and between 80% and 90% for the remaining 6% of the time. Desaturation for short periods was thought to be due to oral air leakage, which made the method slightly less effective than TIPPV. However, the overall clinical effectiveness of NIPPV-C was comparable to that of TIPPV.(ABSTRACT TRUNCATED AT 250 WORDS)