Introduction: The prevalence and clinical significance of central adrenal insufficiency (CAI) in adult patients with Prader Willi Syndrome (PWS) remains unclear. Aim: To assess the prevalence of CAI in adults with PWS and to analyse the effects of replacement therapy with hydrocortisone (HCT) in patients with suspected CAI. Material and Methods: Twenty one adult patients with PWS were evaluated. Based on peak cortisol at the 30 minute of the high dose short Synacthen test (HDSST), patients were divided into three groups: CAI (central adrenal insufficiency) - peak cortisol \<500nmol/L, intermediate (partial AI) - peak cortisol ≥500 nmol/L and \<600 nmol/L and AS (adrenal sufficiency) - peak cortisol ≥ 600 nmol/L. In patients with diagnosed CAI HCT replacement treatment was initiated. Body weight, body fat percentage, signs, and symptoms of CAI were evaluated after 6 and 12 months of treatment.
Study Type
OBSERVATIONAL
Enrollment
21
Central Clinical Hospital Medical University of Warsaw
Warsaw, Poland
The prevalence of CAI in adult PWS patients based on HDSST.
The diagnosis of CAI was based on the high-dose short Synacthen test (HDSST). The following cut-off points were used: 1. CAI group (suspected CAI): • cortisol \< 500 nmol/L at the 30th minute 2. AS group (adrenal sufficiency - CAI excluded): • cortisol ≥ 600 nmol/L at the 30th minute 3. Intermediate group • cortisol ≥500 nmol/L and \<600 nmol/L at the 30th minute
Time frame: At first visit (before HCT treatment).
Symptoms of CAI before hydrocortisone substitution: fatigue
Incidence of the fatigue in the CAI, intermediate and the AS groups.
Time frame: Before HCT treatment
Symptoms of CAI after 6 month hydrocorisone treatment: fatigue
Incidence of the fatigue in the CAI, intermediate and the AS groups.
Time frame: After 6 month HCT treatment
Symptoms of CAI after 12 month hydrocorisone treatment: fatigue
Incidence of the fatigue in the CAI, intermediate and the AS groups.
Time frame: After 12 month HCT treatment
Symptoms of CAI before hydrocortisone substitution: loss of appetite
Incidence of loss of appetite in the CAI, intermediate and the AS groups.
Time frame: Before HCT treatment
Symptoms of CAI after 6 month hydrocorisone treatment: loss of appetite
Incidence of loss of appetite in the CAI, intermediate and the AS groups.
Time frame: After 6 month HCT treatment
Symptoms of CAI after 12 month hydrocorisone treatment: loss of appetite
Incidence of loss of appetite in the CAI, intermediate and the AS groups.
Time frame: After 12 month HCT treatment
Symptoms of CAI before hydrocortisone substitution: muscle weakness
Incidence of the muscle weakness in the CAI, intermediate and the AS groups.
Time frame: Before HCT treatment
Symptoms of CAI after 6 month hydrocortisone treatment: muscle weakness
Incidence of the muscle weakness in the CAI, intermediate and the AS groups.
Time frame: After 6 month HCT treatment
Symptoms of CAI after 12 month hydrocortisone treatment: muscle weakness
Incidence of the muscle weakness in the CAI, intermediate and the AS groups.
Time frame: After 12 month HCT treatment
Symptoms of CAI before hydrocortisone substitution: myalgia
Incidence of the myalgia in the CAI, intermediate and the AS groups.
Time frame: Before HCT treatment
Symptoms of CAI after 6 month hydrocorisone treatment: myalgia
Incidence of the myalgia in the CAI, intermediate and the AS groups.
Time frame: After 6 month HCT treatment
Symptoms of CAI after 12 month hydrocorisone treatment: myalgia
Incidence of the myalgia in the CAI, intermediate and the AS groups.
Time frame: After 12 month HCT treatment
Symptoms of CAI before hydrocortisone substitution: arthralgia
Incidence of the arthralgia in the CAI, intermediate and the AS groups.
Time frame: Before HCT treatment
Symptoms of CAI after 6 month hydrocorisone treatment: arthralgia
Incidence of the arthralgia in the CAI, intermediate and the AS groups.
Time frame: After 6 month HCT treatment
Symptoms of CAI after 12 month hydrocorisone treatment: arthralgia
Incidence of the arthralgia in the CAI, intermediate and the AS groups.
Time frame: After 12 month HCT treatment
Symptoms of CAI before hydrocortisone substitution: weight loss
Incidence of the weight loss (\>3 kg) in the CAI, intermediate and the AS groups.
Time frame: Before HCT treatment
Symptoms of CAI after 6 month hydrocorisone treatment: weight loss
Incidence of the weight loss (\>3 kg) in the CAI, intermediate and the AS groups.
Time frame: After 6 month HCT treatment
Symptoms of CAI after 12 month hydrocorisone treatment: weight loss
Incidence of the weight loss (\>3 kg) in the CAI, intermediate and the AS groups.
Time frame: After 12 month HCT treatment
Symptoms of CAI before hydrocortisone substitution: nausea
Incidence of the nausea in the CAI, intermediate and the AS groups.
Time frame: Before HCT treatment
Symptoms of CAI after 6 month hydrocorisone treatment: nausea
Incidence of the nausea in the CAI, intermediate and the AS groups.
Time frame: After 6 month HCT treatment
Symptoms of CAI after 12 month hydrocorisone treatment: nausea
Incidence of the nausea in the CAI, intermediate and the AS groups.
Time frame: After 12 month HCT treatment
Symptoms of CAI before hydrocortisone substitution: vomiting
Incidence of the vomiting in the CAI, intermediate and the AS groups.
Time frame: Before HCT treatment
Symptoms of CAI after 6 month hydrocorisone treatment: vomiting
Incidence of the vomiting in the CAI, intermediate and the AS groups.
Time frame: After 6 month HCT treatment
Symptoms of CAI after 12 month hydrocorisone treatment: vomiting
Incidence of the vomiting in the CAI, intermediate and the AS groups.
Time frame: After 12 month HCT treatment
Symptoms of CAI before hydrocortisone substitution: abdominal pain
Incidence of the abdominal pain in the CAI, intermediate and the AS groups.
Time frame: Before HCT treatment
Symptoms of CAI after 6 month hydrocorisone treatment: abdominal pain
Incidence of the abdominal pain in the CAI, intermediate and the AS groups.
Time frame: After 6 month HCT treatment
Symptoms of CAI after 12 month hydrocorisone treatment: abdominal pain
Incidence of the abdominal pain in the CAI, intermediate and the AS groups.
Time frame: After 12 month HCT treatment
BMI before hydrocortisone substitution in PWS patients with CAI
Measurement of weight and height will be combined to report BMI in kg/m\^2.
Time frame: Before HCT treatment
BMI after 6 month hydrocortisone substitution in PWS patients with CAI
Measurement of weight and height will be combined to report BMI in kg/m\^2.
Time frame: After 6 month HCT treatment
BMI after 12 month hydrocortisone substitution in PWS patients with CAI
Measurement of weight and height will be combined to report BMI in kg/m\^2.
Time frame: After 12 month HCT treatment
Body fat percentage before hydrocortisone substitution in PWS patients with CAI
Measurement of the body fat percentage using bioelectrical impedance method.
Time frame: Before HCT treatment
Body fat percentage after 6 month hydrocortisone substitution in PWS patients with CAI
Measurement of the body fat percentage using bioelectrical impedance method.
Time frame: After 6 month HCT treatment
Body fat percentage after 12 month hydrocortisone substitution in PWS patients with CAI
Measurement of the body fat percentage using bioelectrical impedance method.
Time frame: After 12 month HCT treatment
Concentration of morning cortisol
Results of morning cortisol in the entire study group.
Time frame: At first visit (before HCT treatment).
Concentration of ACTH
Results of ACTH in the entire study group.
Time frame: At first visit (before HCT treatment).
Concentration of dihydroepiandrosterone sulfate
Results of dihydroepiandrosterone sulfate measurements in the entire study group.
Time frame: At first visit (before HCT treatment).
Laboratory findings - short Synacthen test
Results of the short Synacthen test in the entire study group.
Time frame: At first visit (before HCT treatment).
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