Table 1.
Demographic characteristics and aortic root diameter measurements among patients with different types of mucopolysaccharidoses (MPSs) (n = 125).
| MPS I | MPS II | MPS III | MPS IV | MPS VI | All | |
|---|---|---|---|---|---|---|
| n | 11 | 49 | 25 | 29 | 11 | 125 |
| Gender (M/F) | 5/6 | 49/0 | 13/12 | 13/16 | 5/6 | 85/40 |
| Age (years) | 7.0 (8.1) | 7.2 (5.0) | 8.1 (4.4) | 9.8 (5.1) | 8.9 (5.0) | 8.1 (5.3) |
| Age range (years) | 0.1–18.9 | 0.1–19.1 | 1.8–18.5 | 1.1–18.4 | 2.2–17.1 | 0.1–19.1 |
| Height z score | −2.59 (3.96) | −1.81 (2.77) | −0.73 (1.75) | −6.16 (3.43) | −5.13 (3.15) | −2.96 (3.56) |
| Weight z score | −0.92 (1.14) | 0.04 (1.94) | −0.13 (1.51) | −2.09 (1.13) | −1.78 (1.10) | −0.73 (1.80) |
| BSA (m2) | 0.65 (0.41) | 0.78 (0.25) | 0.92 (0.24) | 0.69 (0.20) | 0.69 (0.10) | 0.77 (0.26) |
| AoD (cm) | 1.67 (0.39) | 2.10 (0.47) | 2.19 (0.44) | 2.11 (0.31) | 1.99 (0.16) | 2.07 (0.42) |
| AoD z score | 1.03 (1.13) | 2.12 (1.43) | 1.68 (1.57) | 3.03 (1.48) | 2.06 (0.80) | 2.14 (1.50) |
| AoD z score > 2, n (%) | 3 (27%) | 25 (51%) | 7 (28%) | 19 (66%) | 5 (45%) | 59 (47%) |
| r value (AoD z score versus age) | −0.566 | 0.394 | 0.624 | 0.457 | 0.161 | 0.353 |
| p-value | p < 0.05 | p < 0.01 | p < 0.01 | p < 0.01 | p > 0.05 | p < 0.01 |
Data are mean (standard deviation). MPS, mucopolysaccharidosis; M/F: male/female; BSA: body surface area; AoD, aortic root diameter.