Asymptomatic Congenital Hyperinsulinism due to a Glucokinase-Activating Mutation, Treated as Adrenal Insufficiency for Twelve Years
Table 3
Results of examinations on the patient after 12 years.
(a) Glucose diurnal rhythm.
Clock time
7:30
11:30
17:30
20:00
23:00
Plasma glucose
10 mg hydrocortisone (8:00)
(mg/dL)
63
80
59
83
81
(mmol/L)
3.5
4.4
3.2
4.6
4.5
5 mg hydrocortisone (8:00)
(mg/dL)
58
68
67
84
80
(mmol/L)
3.2
3.7
3.7
4.6
4.4
no hydrocortisone
(mg/dL)
48
64
57
77
67
(mmol/L)
2.6
3.5
3.1
4.2
3.7
(b) Circadian variation in cortisol.
Clock Time
8:00
17:00
23:00
ACTH (pg/mL)
21.3
10.2
8.3
Cortisol (μg/dL)
8.8
2.8
1.1
(c) Cortisol response to the ACTH rapid stimulation test (250 μg, intravenous bolus).
Time (min)
0
30
60
Cortisol (μg/dL)
6.8
22.2
24.5
Interpretation of results. The normal peak cortisol value poststimulation should be an increment no less than 18 µg/dL.
(d) Cortisol and ACTH responses to the CRH stimulation test (100 μg, intravenous bolus).
Time (min)
0
15
30
60
90
120
ACTH (pg/mL)
15
62.8
75.3
53.6
38
33.8
Cortisol (μg/dL)
4.5
9
15.8
15.5
13
12.6
Interpretation of results. The normal peak ACTH value poststimulation should be an increment no less than 20 pg/mL. Cortisol should be an increment no less than 5 µg/dL.
