Table 1.
Patient characteristics.
| HCTD (N = 56) | MFS (N = 37) | LDS (N = 6) | EDS (N = 13) | |
|---|---|---|---|---|
| Sex, female N (%) | 23 (41) | 16 (43) | 3 (50) | 4 (31) |
| Age (years), median (IQR) | 11.6 (8.8–15.8) | 10.5 (8.0–15.0) | 13.6 (10.1–16.4) | 14.4 (9.2–16.5) |
| BMI, median (IQR) | 15.9 (14.2–20.7) | 15.6 (14.0–16.6) | 17.0 (13.9–19.1) | 18.1 (17.0–21.0) |
| Beighton score/9, median (IQR) | 3 (1–6) | 2 (0–4) | 3 (0–5) | 6 (5–7) |
| GJH (Beighton ≥6), N (%) | 16 (29) | 5 (14) | 1 (17) | 10 (77) |
| Pain intensity - VAS | ||||
| past 7 days, mean (SD) | 1.3 (1.9) | 1.2 (1.7) | 1.3 (2.1) | 1.6 (2.3) |
| Z-scores, mean (SD) | .66 (.94) | .61 (.86) | .63 (1.0) | .81 (1.2) |
| Fatigue - Promis V2.0 shortform reported by the child | ||||
| T score, mean (SD) | 41.7 (9.8) | 40.7 (10.1) | 41.2 (9.5) | 43.9 (9.9) |
| Z-scores, mean (SD) | −.83 (.98) | −.88 (.95) | −.83 (.95) | −.62 (1.0) |
| Fatigue - Promis V2.0 shortform reported by the parent | ||||
| T score, mean (SD) | 46.5 (11.2) | 44.8 (11.4) | 49.3 (11.0) | 49.9 (11.1) |
| Z-scores, mean (SD) | −.35 (1.1) | −.52 (1.1) | −.07 (.96) | −.01 (1.1) |
| Use of CVM, N (%)* | 20 (36) | 16 (43) | 4 (67) | 0 (0) |
| ARBs | 9 (45) | 7 (44) | 2 (50) | |
| BBs | 10 (50) | 8 (50) | 1 (25) | |
| Both ARBs & BBs | 1 (5) | 0 (0) | 1 (25) |
HCTD, heritable connective tissue disorders; MFS, marfan syndrome; LDS, loeys-dietz syndrome; EDS, ehlers-danlos syndromes; VAS, visual analogue scale; GJH, generalized joint hypermobility; CVM, cardiovascular medication; ARBs, angiotensin receptor blockers; BBs, beta-blockers.