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Table 2 Studies on Adrenal function in Prader-Willi syndrome

From: Endocrine manifestations and management of Prader-Willi syndrome

Study

N

Mean age, range (years)

Test

% Insufficient

Comment

De Lind van Wijingaarden 2008 [63]

25

9.7 (3.7-18.6), median

Overnight Single-dose Metyrapone

60

Baseline salivary cortisol levels not significantly different in sufficient versus insufficient

Grugni 2013 [64]

53

27.9 (18–45.2)

LDSST

15.1

 

Corrias 2012 [65]

84

7.7 (0.8-17.9)

LDTST

14.2

Lower cortisol peak with deletion subtype versus UPD

Connell 2010 [66]

25

7.2 (0.43-16.3)

ITT (15), HDSST (10), LDSST (4)

4

1 subject tested insufficiency by ITT

Nyunt 2010 [67]

41

7.7

LDSST

0

 

Farholt 2011 [68]

57

22 (0.58-48)

HDST

0

 

Farholt 2011 [68]

8

25 (16–33)

ITT

0

 
  1. LDSST = low-dose short Synacthen test (1 μg).
  2. LDTST = low-dose tetracosactrin stimulation test (1 μg).
  3. HDSST = high-dose short Synacthen test (250 μg).
  4. UPD = uniparental disomy.
  5. HDST = high-dose Synacthen test (250 μg).
  6. ITT – insulin tolerance test (0.15 units/kg).