. Author manuscript; available in PMC: 2014 Oct 2.

Published in final edited form as: J Pediatr. 2014 Jun 16;165(3):528–533.e1. doi: 10.1016/j.jpeds.2014.05.013

Table I.

Baseline characteristics for enrolled patients

	Baseline
	40
Age (y)	11.5 ± 3.6
Children (from age 6 to ≤12)	25
Adolescents (from age 12 to ≤21)	15
Adults (>21)	0
Male, n (%)	23 (57.5%)
Height (cm)	139.4 ± 19.5
Height (Z-score)	−1.15 ± 0.93
Weight (kg)	37.1 ± 15.0
BMI (kg/m²)	18.2 ± 3.1
BSA (m²)	1.18 ± 0.31
eGFR (mL/min/1.73 m²)	63 ± 25 (44;58;85)
Daily immediate release cysteamine dose (mg/d)	1807 ± 520
Total daily DR-CYS dose (average % of previous immediate-release cysteamine dose)	83.7 ± 7.9
WBC (cystine) (nmol ½ cystine/mg protein)	0.79 ± 1.68
WBC (cystine) ≤1 nmol ½ cystine/mg protein; n (%)	34 (85%)

BSA, body surface area.

Values presented are the mean ± SD (range) and the (25th percentile; median; 75th percentile) unless otherwise noted.