Cumulative Prevalence of Valve Disease in MPS IVA Patients.

<p>Patients presenting with (dotted line) or without (solid line) valve thickening at first examination. Bars on the line indicate patients without valve thickening at first examination. Early valve thickening predisposes to the development of valve disease at later age. Created using SPSS software.</p

Cardiac Data for MPS IVA Patients with and without Valvular Thickening.

<p>Cardiac Data for MPS IVA Patients with and without Valvular Thickening.</p

Correlation Between Heart Rate and Cardiothoracic Ratio (CTR) in MPS IVA Patients.

<p>Patients with higher CTR had increased heart rates (r² = 0.52; CTR = 0.171*HR + 42.43; ANOVA F = 9.9, p = 0.04). Created using SPSS software.</p

Valve Involvement in MPS IVA Patients (n = 54).

<p>Valve Involvement in MPS IVA Patients (n = 54).</p

Inter-observer agreement of the Mercuri scores in the psoas and paraspinal muscles.

<p>Inter-observer agreement of the Mercuri scores in the psoas and paraspinal muscles.</p

Baseline Biometric Characteristics of MPS IVA Patients (n = 54).

<p>Baseline Biometric Characteristics of MPS IVA Patients (n = 54).</p

Fat fraction of the paraspinal muscles during follow-up.

<p>Images acquired at baseline (panel a), followed-up yearly (panel b-f) are shown. Fatty infiltrated muscle is colored red. The fat fraction increased from 0.38 (panel a) via 0.46 (b), 0.53 (c), 0.57 (d), 0.66 (e) to 0.74 (f).</p

MR-derived measurement of the fat fraction.

<p>A region of interest is selected from a T1-weighted TSE sequence in the left paraspinal musculature at the L3-level (panel a). Fat fraction is calculated using the equation FF = 1 –FCSA/CSA; a signal intensity threshold of 772 (derived from normal muscle–the medial multifidus muscle in this case) segregates muscle from fat tissue. Pixels beneath this threshold, colored red (panel b), constitute the FCSA (1096 mm²), while the entire area of the ROI constitutes the CSA (1466 mm²). These data result in an FF value of 0.25.</p

Inter-observer agreement concerning quantitative MR-derived data.

<p>Inter-observer agreement concerning quantitative MR-derived data.</p

Correlation Between Age and z Score of Aortic Root Dimensions in MPS IVA Patients.

<p>Patients with MPS IVA develop a dilatation of the aortic root in relation to BSA with ageing (r2 = 0.586; ANOVA F:95.717; p<0.005). Created using SPSS software.</p