Clinical and demographic characteristics associated with Total Motor Composite Scores and Percentiles.
Age, a lifetime history of hospitalizations and the presence of any comorbidities were each associated with TMC and TMC% scores. Age was inversely associated with TMC. For every one-year increase in age, there was an estimated 1.2 point (95% CI: 0.3 to 2.1) decrease (p=0.0089) in TMC, and an estimated 3.5% (95% CI: 0.9% to 6.2%) decrease (p=0.0099) in TMC%. A history of hospitalization was also associated with significantly lower TMC scores (linear trend test, p= 0.0014) and TMC% (linear trend test, p=0.0005). Those diagnosed with any comorbidity had a significantly lower TMC score (8.9 points lower, CI 5.3- 12.5; p<0.0001) and lower TMC % (27.8% lower, CI 17.3% to 38.4%; p<0.0001). Figure 2 illustrates TMC scores by age, hospitalizations, and presence of any comorbidities.
In univariable regression models, ppFEV1 and ppFVC were positively correlated with both TMC and TMC% (Figure 3 A and B ). For every 1 percent increase in ppFEV1 there was an estimated 0.61 point (95%CI: 0.15 to 1.06) increase in TMC (p=0.0101). For every 1 percent increase in ppFVC there was an estimated 0.61 point (95%CI: 0.20 to 1.01) increase in TMC (p=0.0043). For every 1 percent increase in ppFEV1 there was an estimated 19.0% (95%CI: 4.4% to 33.6%) increase in TMC% (p=0.0118). For every 1 percent increase in ppFVC there was an estimated 19.2% (95%CI: 6.2% to 32.2%) increase in TMC% (p=0.0046).
Children with the F508del/Other genotype had the highest rate of total motor skill delays (27% with a TMC% below 18%). Comparatively, the F508del homozygous genotype group and the Other/Other group had a 6% and 10% rate of TMC% below 18%. Although this difference was not statistically significant (p=0.0706), it may be clinically meaningful. There were no observed associations between TMC and TMC% and body mass index (BMI). The only observed difference in the six BOT-2 subset components and scores between sexes was in running speed and agility with girls scoring higher than boys in this category (Supplemental table 1 ).