Central Adrenal Insufficiency Is Rare in Adults With Prader-Willi Syndrome.
J Clin Endocrinol Metab
; 105(7)2020 07 01.
Article
em En
| MEDLINE
| ID: mdl-32232324
ABSTRACT
CONTEXT 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. OBJECTIVE:
To prevent both over- and undertreatment with hydrocortisone, we 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).DESIGN:
Metyrapone test or ITT in adults with PWS (Nâ =â 82) and review of medical files for symptoms of hypocortisolism related to surgery (Nâ =â 645).SETTING:
Outpatient clinic. PATIENTS OR OTHERPARTICIPANTS:
Eighty-two adults with genetically confirmed PWS. MAIN OUTCOMEMEASURE:
For MTP, 11-deoxycortisolâ >â 230 nmol/L was considered sufficient. For ITT, cortisolâ >â 500 nmol/L (Dutch, French, and Swedish patients) orâ >â 450 nmol/L (British patients) was considered sufficient.RESULTS:
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.CONCLUSIONS:
Central adrenal insufficiency is rare (1.2%) in adults with PWS. Based on these results, we recommend against routinely prescribing hydrocortisone stress-doses in adults with PWS.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndrome de Prader-Willi
/
Insuficiência Adrenal
Tipo de estudo:
Diagnostic_studies
/
Prevalence_studies
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Risk_factors_studies
Limite:
Adolescent
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Adult
/
Female
/
Humans
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Male
/
Middle aged
Idioma:
En
Revista:
J Clin Endocrinol Metab
Ano de publicação:
2020
Tipo de documento:
Article
País de afiliação:
Holanda