두시엔느 근디스트로피 환자에서 비침습적 환기보조 중 발생한 기흉−증례 보고−

Abstract

Duchenne muscular dystrophy (DMD) is a severe X-linked recessive disorder characterized by rapid progression of muscle wasting and weakness. Long term noninvasive intermittent positive pressure ventilation (NIPPV) is an important treatment for neuromuscular patients with chronic respiratory failure. NIPPV is easy to administer and life-threatening complications are rare. We report two cases of pneumothorax associated with long term NIPPV in DMD. Given the increasing utilization of chronic NIPPV, we suggest that caregivers and patients must be aware of this potentially life-threatening complication.