A patient with Prader-Willi Syndrome (PWS) may develop adrenal insufficiency due to dysfunction of the hypothalamic-pituitary axis. The authors are from Medical University of Silesia in Poland.
Patient selection: Prader-Willi Syndrome
Steroid used: hydrocortisone
Situations:
(1) basic replacement
(2) stress situation or infection
(3) more severe stress situation or surgery
Situation
Daily Dose
In Divided Doses
basic replacement
7.5 to 15 mg per sq meter
2 to 4
stress or infection option 1
30 to 50 mg per sq meter
3
stress or infection option 2
2 to 3 times basic daily dose
2 to 4
Parameters for more severe situation:
(1) body weight in kilograms
(2) age in years
Option 1: based on body weight, with initial bolus given before surgery
Body Weight
Initial Bolus
Followed by (in Divided Doses)
<= 10 kg
2 mg/kg
25 mg per 24 hours
10.1 to 20 kg
2 mg/kg
50 mg per 24 hours
> 20 kg, prepubertal
2 mg/kg
100 mg per 24 hours
> 20 kg, postpubertal
2 mg/kg
150 mg per 24 hours
Option 2: based on age
Age in Years
Initial Bolus
Followed by (in 3 Divided Doses)
0 to 3 years
25 mg
25 mg per 24 hours
3 to 12 years
50 mg
50 mg per 24 hours
> 12 years
100 mg
100 mg per 24 hours
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