Imbalanced cortisol concentrations in glycogen storage disease type I: evidence for a possible link between endocrine regulation and metabolic derangement

Table 2 Baseline hormone serum levels in GSDI patients and control subjects

	GSDIa		Controls		GSDIb		Controls		Significance (p)		Reference range
	Mean	SE	Mean	SE	Mean	SE	Mean	SE	Ia vs C	Ib vs C
ACTH (pmol/L)	6.28	1.73	5.40	0.55	7.15	2.24	5.31	0.42	0.545	0.282	2.2–11.0
Cortisol (nmol/L)	455.44	41.74	352.27	19.30	230.22	59.37	372.56	35.47	0.042	0.041	< 15 years: 83–580 > 15 years:220–525
Androstenedione (nmol/L)	1.16	0.33	1.44	0.23	2.24	0.28	2.28	0.35	0.493	0.944	Depending on Tanner stage
17OHP (nmol/L)	1.37	0.25	1.09	0.13	2.07	0.53	1.38	0.11	0.276	0.108	Depending on Tanner stage
DHEAS (nmol/L)	3392	1255	3496	272	4195	1755	3068	286	0.938	0.549	Depending on Tanner stage
Renin^a (pmol/L)	0.14	0.04	0.18	0.01	0.20	0.05	0.16	0.01	0.611	0.478	< 5 years: 0.07–0.21 > 5 years: 0.06–0.08
Aldosterone^a (pmol/L)	25.42	6.53	25.53	1.11	17.64	5.46	24.12	1.27	0.750	0.432	< 15 years: 1.80–28.80 > 15 years: 2.50–11.00
UFC (μg/24 h)^b	55.83	8.02	65.30	5.72	81.29	47.94	62.67	10.26	0.360	0.610	1–10 years: 2–27 11–20 years:5–55 > 20 years: 20–90

^a 7 GSDIa and 6 GSDIb patients
^b 5 GSDIa and 3 GSDIb patients
ACTH adrenocorticotropic hormone, 17OHP17-hydroxyprogesterone, DHEAS dehydroepiandrosterone sulphate, UFC 24-h urinary free cortisol

ISSN: 1750-1172