Table 1.
Characteristics, anthropometrics, and clinical findings of study subjects at study visit
| Characteristic | MUL patients (n = 23) |
Control subjects (n = 23) |
P | ||
|---|---|---|---|---|---|
| Mean or median | SD or range | Mean or median | SD or range | ||
| Male sex, n | 11 | 11 | |||
| Age, y | 10.3 | 1.9–15.2 | 10.0 | 1.2–15.8 | NS |
| Weight, kg | 14.8 | 6.6–47.0 | 33.0 | 13.0–68.7 | < 0.001 |
| Weight-for-age, Z | –5.17 | 2.78 | 0.20 | 0.96 | < 0.001 |
| Weight-for-height, Z | –2.11 | 1.70 | 0.08 | 0.94 | < 0.001 |
| Weight-for-height, % | –13.3 | 8.83 | 0.92 | 12.7 | < 0.001 |
| Height, cm | 110.5 | 69.5–157 | 146.4 | 93–175.5 | < 0.001 |
| Height-for-age, Z | –4.47 | –10.4–1.10 | 0.20 | –3.34–1.85 | < 0.001 |
| Lean body mass∗ | 14.7 | 6.8–36.1 | 29.5 | 11.3 – 46.2 | < 0.001 |
| Waist-to-hip ratio | 0.93 | 0.73–1.23 | 0.85 | 0.71–1.22 | 0.007 |
| Body surface area, m2 | 0.66 | 0.36–1.43 | 1.15 | 0.58–1.78 | < 0.001 |
| Thorax circumference, mm | 508 | 395–790 | 655 | 507–800 | < 0.001 |
| Laboratory data | |||||
| Alanine aminotransferase, U/l | 35 | 19 | NA | ||
| Pro-BNP, ng/l | 385 | 682 | |||
| Clinical findings | |||||
| Estimated jugular venous pressure, mm Hg | 7 | 3 | Veins not observable | ||
| Liver (cm from right costal margin) | 2 | 0–5 | Liver not palpable | ||
| Modified Ross score | 1 | 0–13 | 0 | ||
| Modified Ross class | 1 | 1–3 | 1 | ||
| Systolic BP, mm Hg | 95 | 13 | 104 | 10 | 0.009 |
| Systolic BP, Z | –0.14 | 1.32 | 0.27 | 0.67 | 0.210 |
| Diastolic BP, mm Hg | 58 | 7 | 60 | 7 | 0.405 |
| Diastolic BP, Z | –0.16 | 1.22 | 0.13 | 0.52 | 0.310 |
| Heart rate, bpm | 91 | 20 | 76 | 14 | 0.005 |
Data are presented as mean and standard deviation (SD), median and range, or count and percentage, unless otherwise specified.
BP, blood pressure. bpm, beats per minute; NA, not assessed; NS nonsignificant. pro-BNP, pro-hormone brain natriuretic peptide.
∗
N = 18 for lean body mass for mulibrey nanism (MUL) patients and individually matched control subjects.