Rare inborn errors of metabolism with movement disorders: a case study to evaluate the impact upon quality of life and adaptive functioning

Table 2 HRQOL scores of children with IEM and movement disorders compared to scores in healthy children

	IEM and movement disorders n = 24	Healthy Dutch population *n =* 87	t-value	CI 95%	p -value*
	Mean value (SD)	Mean value (SD)	t-value	CI 95%	p -value*
Total score	49.63 (21.78)	87.60 (11.00)	-8.21	-28.78 to -47.16	<0.001
Physical functioning	39.71 (24.56)	92.20 (9.10)	-10.44	-43.31 to -63.67	<0.001
Emotional functioning	63.50 (23.18)	81.10 (17.40)	-3.42	-7.38 to -27.82	<0.001
Social functioning	49.71 (29.18)	90.30 (14.00)	-6.57	-25.32 to -52.86	<0.001
School functioning	51.29 (32.05)	82.50 (16.30)	-4.49	-17.41 to -45.01	<0.001

HRQOL scores were measured on the PedsQL 4.0 Generic Core Scales. Parent proxy-reports of children with IEM and movement disorders were compared to scores of healthy Dutch children [14].
CI Confidence interval, IEM Inborn error of metabolism, SD Standard deviation, *significance level after Bonferroni-Holm correction for multiple comparisons.
Bold text: statistically significant.

ISSN: 1750-1172