SwePub
Sök i LIBRIS databas

  Utökad sökning

WFRF:(Poitou C)
 

Sökning: WFRF:(Poitou C) > Central Adrenal Ins...

  • Rosenberg, AGW (författare)

Central Adrenal Insufficiency Is Rare in Adults With Prader-Willi Syndrome

  • Artikel/kapitelEngelska2020

Förlag, utgivningsår, omfång ...

  • 2020-03-31
  • The Endocrine Society,2020

Nummerbeteckningar

  • LIBRIS-ID:oai:prod.swepub.kib.ki.se:144544520
  • http://kipublications.ki.se/Default.aspx?queryparsed=id:144544520URI
  • https://doi.org/10.1210/clinem/dgaa168DOI

Kompletterande språkuppgifter

  • Språk:engelska
  • Sammanfattning på:engelska

Ingår i deldatabas

Klassifikation

  • Ämneskategori:ref swepub-contenttype
  • Ämneskategori:art swepub-publicationtype

Anmärkningar

  • ContextPrader–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.ObjectiveTo 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).DesignMetyrapone test or ITT in adults with PWS (N = 82) and review of medical files for symptoms of hypocortisolism related to surgery (N = 645).SettingOutpatient clinic.Patients or Other ParticipantsEighty-two adults with genetically confirmed PWS.Main Outcome MeasureFor MTP, 11-deoxycortisol > 230 nmol/L was considered sufficient. For ITT, cortisol > 500 nmol/L (Dutch, French, and Swedish patients) or > 450 nmol/L (British patients) was considered sufficient.ResultsCentral 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.ConclusionsCentral 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.

Biuppslag (personer, institutioner, konferenser, titlar ...)

  • Pellikaan, K (författare)
  • Poitou, C (författare)
  • Goldstone, AP (författare)
  • Hoybye, CKarolinska Institutet (författare)
  • Markovic, T (författare)
  • Grugni, G (författare)
  • Crino, A (författare)
  • Caixas, A (författare)
  • Coupaye, M (författare)
  • Van den Berg, SAA (författare)
  • Van der Lely, AJ (författare)
  • De Graaff, LCG (författare)
  • Karolinska Institutet (creator_code:org_t)

Sammanhörande titlar

  • Ingår i:The Journal of clinical endocrinology and metabolism: The Endocrine Society105:7, s. E2563-E25711945-71970021-972X

Internetlänk

Hitta via bibliotek

Till lärosätets databas

Kungliga biblioteket hanterar dina personuppgifter i enlighet med EU:s dataskyddsförordning (2018), GDPR. Läs mer om hur det funkar här.
Så här hanterar KB dina uppgifter vid användning av denna tjänst.

 
pil uppåt Stäng

Kopiera och spara länken för att återkomma till aktuell vy