The assessment of sniff nasal inspiratory pressure in patients with Duchenne muscular dystrophy in China.

OBJECTIVE

Progressive weakness of respiratory muscles remains one of the leading causes of death among patients with Duchenne muscular dystrophy (DMD). Currently, there are few pulmonary function data among Chinese DMD patients. This study was carried out to evaluate the sniff nasal inspiratory pressure (SNIP) change among a group of Chinese DMD patients, and compare it with the SNIP value of patients with neuromuscular disorders in other countries.

METHODS

SNIP data were collected in three research groups that consists of 581 subjects: 125 DMD boys who have taken steroid (Age 5.0-13.3, DMD-steroid group), 145 DMD steroid-naive boys (Age 5.0-13.9, DMD-nonsteroid group), and 311 healthy controls (Age 5.0-14.0, Control group).

RESULTS

The SNIP for DMD-nonsteroid group, DMD-steroid group and Control group were: 56.5 (±14.3) cm HO,66.4 (±15.5) cm HO and 78.9 (±21.5) respectively. The SNIP in the DMD-nonsteroid group became significantly different from that of the healthy controls since age 7.0-8.9. The significant difference of SNIP between DMD-steroid group and DMD-nonsteroid group at age 7.0-10.9. The peak value of SNIP in the DMD-nonsteroid group appeared at age 8.7, and decreased dramatically thereafter, while in DMD-steroid group and the Control group peaked at 10.2 years and 12.2 years respectively. There was a bit difference between SNIP in this group and that in previous researches which may be due to geographical distribution and ethnic backgrounds.

CONCLUSION

This study strengthens the previous findings that SNIP can be used to evaluate respiratory dysfunction during the early stage of young patients with neuromuscular disorders, and demonstrates that steroid is effective in slowing the decrease of SNIP in this group of Chinese DMD boys.