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


Type of adrenal insufficiency: central (CAI)


Testing: low dose tetracosactrin (Synacthen) stimulation test (LDSST), using 1 µg


CAI should be suspected if the patient shows certain clinical and laboratory findings.


Clinical signs and symptoms of CAI may include:

(1) abnormal weight gain or weight loss

(2) vomiting

(3) recurrent infections

(4) excessive fatigue

(5) muscle and joint pain

(6) headache

(7) dizziness


Laboratory findings may include:

(1) hypoglycemia

(2) hyponatremia

(3) hyperkalemia (with chronic kidney disease)

(4) anemia

(5) lymphocytosis

(6) eosinophilia


Basal morning cortisol should be assessed every 3-6 months in all patients with PWS.


Mandatory CAI exclusion: basal cortisol < 138 nmol/L.

Some perform LDSST when the basal cortisol < 250 nmol/L.


Comorbid conditions may include:

(1) hypogonadism

(2) inadequate growth hormone

(3) hyperphagia with obesity

(4) impaired body temperature regulation

(5) hypothyroidism

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