| BackgroundRespiratory muscle weakness is a major cause of morbidity and mortality in children with Duchenne muscular dystrophy (DMD).Foreign study of DMD respiratory function found that sniff nasal inspiratory pressure (SNIP) is a sensitive indicator than lung function. In healthy children of abroad, a range of age-stratified normative SNIP values has been obtained.However, there is no related research in our country. SNIP whether can be used as a sensitive indicator to evaluate the change of the Chinese DMD early respiratory function is unknown. Therefore, quantify SNIP in DMD patients and the normal children in our country are very important.ObjectiveTo quantify SNIP in DMD children and the extent of impaired to compare with normal children. To determine whether the SNIP is a sensitive indicator than lung function.If there is a correlation among SNIP, lung function and overnight polysomnography.Methods148 patients (mean 8.3 years, range 5.0-14.8 years) in our DMD multidisciplinary joint outpatient with diagnosed Duchenne muscular dystrophy underwent SNIP assessment. 35 patients (mean 8.0 years, range 5.0-12.9 years) with DMD underwent pulmonary function tests.24 patients (mean 9.1 years, range 5.3-14.5 years) with DMD underwent overnight polysomnography. The other 311 healthy male children (mean 9.4 years, range 5.0-14.0 years) as normal control group with SNIP test.Results1、SNIP average of 148 DMD children was 61.3 (±17.3) cm H2O. SNIP average of 311 normal children was 77.8( ± 22.9) cm H20.5.0-6.9 years the SNIP average of DMD group and normal control group was 56.1 cm H2O cmH2O and 59.1cmH2O, respectively. There is no significant difference between the two groups (t=0.99, P=0.32). 7.0-8.9 years the SNIP average of DMD group and normal control group was 60.4 cmH20 and 76.9 cm H2O, respectively. There is very highly significant difference between the two groups (t=5.94, P<0.001).9.0-10.9 years the SNIP average of DMD group and normal control group was 68.5cm H2O and 85.3 cm H2O, respectively. There is highly significant difference between the two groups (t=3.41, P=0.001).11.0-14.9 years the SNIP average of DMD group and normal control group was 61.1 cm H2O and 83.6 cm H2O, respectively. There is very highly significant difference between the two groups (t=3.97, P<0.001).The SNIP of healthy controls and DMD group increased until 12.2 and 9.8 years of age, respectively, and DMD group declined thereafter, while healthy controls did not change with age. The SNIP was significantly impaired in the group with DMD when compared to the healthy controls, especially after the age of 9.8.2、35 cases of DMD patients with pulmonary function test. DMD patients under the age of 10 tests of VC%, FVC%, FEV1% values were greater than 80%, that is normal.2 patients with 11-12 of VC%, FVC% values are less than 80%, that is lower than normal. FEV1%(85.2%) is still in the normal range.SNIP with the important indicators of lung function of VC%(P=0.14) FVC%(P= 0.49), and FEV1%(P= 0.79) correlation analysis showed no correlation.3、24 cases of DMD patients with overnight polysomnography test. AHI is 8.1±4.4 times per hour, AI is 2.1±3.0 times per hour, HI is 5.7±4.4 times per hour, SPO2 was 89.5±3.4%.we found that 70.8%(17)patients AHI> 5 times per hour,45.8%(11) patients SPO2< 90%. We also found that 70.8%(17) patients with apnea, all for the OS A. SNIP with the important indicators of overnight polysomnography of AHI、AI、HI、 SPO2 correlation analysis showed no correlation.Conclusion1、DMD patients SNIP obvious decline when compared with normal children, and when the lung function in the normal range it has fallen. SNIP can early found DMD patients with respiratory dysfunction.2、The study found no significant correlations between SNIP and indexes of overnight polysomnography, which SNIP does not predict DMD patients with nocturnal breathing. |
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