Central Adrenal Insufficiency Is Rare in Adults With Prader–
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Prader–Willi syndrome (PWS) is associated with several hypothalamic-pituitary hormone deficiencies. There is no agreement on the prevalence of central adrenal insufficiency (CAI) in adults with PWS. In some countries, it is general practice to prescribe stress-dose hydrocortisone during physical or psychological stress in patients with PWS. Side effects of frequent hydrocortisone use are weight gain, osteoporosis, diabetes mellitus, and hypertension—already major problems in adults with PWS. However, undertreatment of CAI can cause significant morbidity—or even mortality.

To prevent both over- and undertreatment with hydrocortisone, This study assessed the prevalence of CAI in a large international cohort of adults with PWS. As the synacthen test shows variable results in PWS, we only use the metyrapone test (MTP) and insulin tolerance test (ITT).

Metyrapone test or ITT in adults with PWS (N = 82) and review of medical files for symptoms of hypocortisolism related to surgery was undertaken in an Outpatient clinic. Patients were Eighty-two adults with genetically confirmed PWS.

For MTP, 11-deoxycortisol; more than 230 nmol/L was considered sufficient. For ITT, cortisol ; more than 500 nmol/L (Dutch, French, and Swedish patients) or more than; 450 nmol/L (British patients) was considered sufficient. Central adrenal insufficiency was excluded in 81 of 82 patients. Among the 645 patients whose medical files were reviewed, 200 had undergone surgery without perioperative hydrocortisone treatment. None of them had displayed any features of hypocortisolism.

Conclusively, Central adrenal insufficiency is rare (1.2%) in adults with PWS. Based on these results, researchers recommend against routinely prescribing hydrocortisone stress-doses in adults with PWS.

Source: https://academic.oup.com/jcem/article/105/7/dgaa168/5813913

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