Skip to main content

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

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

 GSDIa Controls GSDIb Controls Significance (p)Reference range
MeanSEMeanSEMeanSEMeanSEIa vs CIb vs C 
ACTH (pmol/L)6.281.735.400.557.152.245.310.420.5450.2822.2–11.0
Cortisol (nmol/L)455.4441.74352.2719.30230.2259.37372.5635.470.0420.041<  15 years: 83–580
>  15 years:220–525
Androstenedione (nmol/L)1.160.331.440.232.240.282.280.350.4930.944Depending on Tanner stage
17OHP (nmol/L)1.370.251.090.132.070.531.380.110.2760.108Depending on Tanner stage
DHEAS (nmol/L)3392125534962724195175530682860.9380.549Depending on Tanner stage
Renina (pmol/L)0.140.040.180.010.200.050.160.010.6110.478<  5 years: 0.07–0.21
>  5 years: 0.06–0.08
Aldosteronea (pmol/L)25.426.5325.531.1117.645.4624.121.270.7500.432<  15 years: 1.80–28.80
>  15 years: 2.50–11.00
UFC (μg/24 h)b55.838.0265.305.7281.2947.9462.6710.260.3600.6101–10 years: 2–27
11–20 years:5–55
>  20 years: 20–90
  1. a 7 GSDIa and 6 GSDIb patients
  2. b 5 GSDIa and 3 GSDIb patients
  3. ACTH adrenocorticotropic hormone, 17OHP17-hydroxyprogesterone, DHEAS dehydroepiandrosterone sulphate, UFC 24-h urinary free cortisol