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

AGW Rosenberg; Karlijn Pellikaan; Christine Poitou; Anthony P. Goldstone; Charlotte Höybye; Tania P. Markovic; Graziano Grugni; Antonino Crinò; Assumpta Caixàs; Muriel Coupaye; Sjoerd van den Berg; AJ (Aart-Jan) van der Lely; Laura de Graaff

doi:10.1210/clinem/dgaa168

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

AGW Rosenberg
, Karlijn Pellikaan
, Christine Poitou
, Anthony P. Goldstone
, Charlotte Höybye
, Tania P. Markovic
, Graziano Grugni
, Antonino Crinò
, Assumpta Caixàs
, Muriel Coupaye
, Sjoerd van den Berg
, AJ (Aart-Jan) van der Lely
, Laura de Graaff^*

^*Corresponding author for this work

Sorbonne Université
European Reference Network on Rare Endocrine Conditions (ENDO-ERN)
International Network for Research, Management and Education on Adults with PWS
Academic Centre for Growth Disorders
Imperial College London
Karolinska University Hospital
Royal Prince Alfred Hospital
The University of Sydney
IRCCS Istituto Auxologico Italiano - Milano
IRCCS Ospedale pediatrico Bambino Gesù - Roma
Autonomous University of Barcelona

Research output: Contribution to journal › Article › Academic › peer-review

34 Citations (Scopus)

93 Downloads (Pure)

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 (N = 82) and review of medical files for
symptoms of hypocortisolism related to surgery (N = 645).
Setting: Outpatient clinic

Original language	English
Pages (from-to)	e2563-e2571
Number of pages	9
Journal	The Journal of clinical endocrinology and metabolism
Volume	105
Issue number	7
Early online date	31 Mar 2020
DOIs	https://doi.org/10.1210/clinem/dgaa168
Publication status	Published - Jul 2020

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SDG 3 Good Health and Well-being

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Central Adrenal Insufficiency Is Rare in Adults With Prader–Willi SyndromeFinal published version, 238 KBLicence: CC BY

Cite this

Rosenberg, AGW., Pellikaan, K., Poitou, C., Goldstone, A. P., Höybye, C., Markovic, T. P., Grugni, G., Crinò, A., Caixàs, A., Coupaye, M., van den Berg, S., van der Lely, AJ., & de Graaff, L. (2020). Central Adrenal Insufficiency Is Rare in Adults With Prader–Willi Syndrome. The Journal of clinical endocrinology and metabolism, 105(7), e2563-e2571. https://doi.org/10.1210/clinem/dgaa168

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title = "Central Adrenal Insufficiency Is Rare in Adults With Prader–Willi Syndrome",

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 (N = 82) and review of medical files for symptoms of hypocortisolism related to surgery (N = 645). Setting: Outpatient clinic",

author = "AGW Rosenberg and Karlijn Pellikaan and Christine Poitou and Goldstone, \{Anthony P.\} and Charlotte H{\"o}ybye and Markovic, \{Tania P.\} and Graziano Grugni and Antonino Crin{\`o} and Assumpta Caix{\`a}s and Muriel Coupaye and \{van den Berg\}, Sjoerd and \{van der Lely\}, \{AJ (Aart-Jan)\} and \{de Graaff\}, Laura",

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doi = "10.1210/clinem/dgaa168",

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Rosenberg, AGW, Pellikaan, K, Poitou, C, Goldstone, AP, Höybye, C, Markovic, TP, Grugni, G, Crinò, A, Caixàs, A, Coupaye, M, van den Berg, S , van der Lely, AJ & de Graaff, L 2020, 'Central Adrenal Insufficiency Is Rare in Adults With Prader–Willi Syndrome', The Journal of clinical endocrinology and metabolism, vol. 105, no. 7, pp. e2563-e2571. https://doi.org/10.1210/clinem/dgaa168

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AU - Rosenberg, AGW

AU - Pellikaan, Karlijn

AU - Poitou, Christine

AU - Goldstone, Anthony P.

AU - Höybye, Charlotte

AU - Markovic, Tania P.

AU - Grugni, Graziano

AU - Crinò, Antonino

AU - Caixàs, Assumpta

AU - Coupaye, Muriel

AU - van den Berg, Sjoerd

AU - van der Lely, AJ (Aart-Jan)

AU - de Graaff, Laura

PY - 2020/7

Y1 - 2020/7

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JO - The Journal of clinical endocrinology and metabolism

JF - The Journal of clinical endocrinology and metabolism

IS - 7

ER -

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

Abstract

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