Table 1.
Characteristics of patients with cystinosis at time of grip strength measurement
| Characteristics | Median (IQR) or n (%) |
|---|---|
| Age at diagnosis, mo | 17 (12–24) |
| Age at cysteamine initiation, mo | 18 (13–30) |
| Male sex | 38 (50) |
| History of dialysis | 30 (39) |
| History of kidney transplantation | 50 (66) |
| Age at inclusion, yr | 26.0 (15.0–32.0) |
| Pediatric patients (vs. adults) | 28 (37) |
| Type of treatment at inclusion | |
| Immediate release cysteaminea | 25 (33) |
| Delayed release cysteamineb | 50 (66) |
| Self-reported adherence, % | |
| <50 | 2 |
| 50–75 | 7 |
| >75 | 64 |
| eGFR at inclusion, ml/min per 1.73 m2 | 55 (37–74) |
| CKD stage | |
| 1 | 4 (7) |
| 2 | 21 (36) |
| 3A | 12 (21) |
| 3B | 13 (22) |
| 4 | 8 (14) |
CKD, chronic kidney disease; eGFR, estimated glomerular filtration rate; IQR, interquartile range.
Missing: age at diagnosis (n = 1), age at cysteamine initiation (n = 3), age at inclusion (n = 1), self-reported adherence (n = 3), and eGFR (n = 18).
a
Immediate release cysteamine (Cystagon; Mylan Pharmaceuticals, Canonsburg, PA).
b
Delayed release cysteamine (Procysbi; Horizon Therapeutics, Dublin, Ireland).