Minute ventilation and respiratory resistance at baseline and during exercise for OVA and control rabbits.

<p>Minute ventilation and respiratory resistance at baseline and during exercise for OVA and control rabbits.</p

Wheal surface of the intradermal skin tests, total BAL cell count and BAL eosinophil count of OVA and control rabbits, expressed as mean (Standard Deviation).

<p>Wheal surface of the intradermal skin tests, total BAL cell count and BAL eosinophil count of OVA and control rabbits, expressed as mean (Standard Deviation).</p

Overall tracheal stimulations performed at rest and during exercise (electrically induced muscle contractions) for OVA and control rabbits (expressed as absolute value (percentage)).

<p>Overall tracheal stimulations performed at rest and during exercise (electrically induced muscle contractions) for OVA and control rabbits (expressed as absolute value (percentage)).</p

Rabbit exposed to an aerosol administrated in a closed Plexiglas box using an ultrasonic-nebulizer (SYST’AM, LS290).

<p>The box was conceived to allow the head of the rabbit to be maintained in front of the aerosol particles and drops cloud generated by the nebulizer. Aerosol sessions were performed 15–20 min daily from day 26 to day 28 either with ovalbumin (OVA rabbits) or with saline (control rabbits).</p

The 4-cluster solution.

<p>DMD patients were assigned to 4 clusters in the final hierarchical cluster analysis (linkage: Ward's method, metric: squared Euclidean distance) using CatPCA-derived XY coordinates in a plane defined by the cognition and motor function principal components (axes) (Cronbach's α: 0.859 and 0.721, respectively). Cognition was always altered to various extent in patients with early infantile DMD (A) and classical DMD (B), but was preserved in patients with moderate (C) and severe (D) pure motor DMD who differed markedly in motor function impairment. Overall, cluster A patients were most severely affected.</p

Data describing patients' history of disease, muscle and cognitive functions (quantitative variables are mean±SD).

<p>Data describing patients' history of disease, muscle and cognitive functions (quantitative variables are mean±SD).</p

Variables' contributions to the two principal components (Cronbach's α, a measure by default for the internal consistency of categorical principal components in the SPSS software, is 0.891 and 0.721), absolute values above 0.500 were retained to define the principal components.

<p>Variables' contributions to the two principal components (Cronbach's α, a measure by default for the internal consistency of categorical principal components in the SPSS software, is 0.891 and 0.721), absolute values above 0.500 were retained to define the principal components.</p

Simplified indicators of moderate pure motor DMD (cluster C) derived from series 1.

*<p>Yate's Chi square: all p = 0.00001, exact 95% confidence limits.</p>**<p>PPV and NPV, positive or negative predictive value.</p>***<p>III borderline normal, IV normal (see <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0004347#s2" target="_blank">methods</a>).</p

Genotype/phenotype correlations.

<p>Proportion of patients with a mutation upstream to exon 30 steadily increased from group A to D. This ascent correlated with spared cognition (mental status: p<0.0003) but not with motor function (age at ambulation loss: NS) (Fisher's exact test). Expectedly, the 3 patients with mutation after exon 63 affecting the brain specific DP71 transcript were classified in group A.</p

Simplified indicators applied to congenital and to moderate pure motor DMD in series 2.

*<p>Yate's Chi square: all p = 0.00001, exact 95% confidence limits.</p>**<p>PPV and NPV, positive or negative predictive value.</p>***<p>I severe mental retardation, II mild mental retardation, III borderline normal, IV normal (see <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0004347#s2" target="_blank">methods</a>).</p