Imperial College London
Alternate

Dr Tony Goldstone

Faculty of Medicine, Department of Brain Sciences

Reader in PsychoNeuroEndocrinology
 
 
 
//

Contact

 

+44 (0)20 7594 5989tony.goldstone Website

 
 
//

Location

 

S25Commonwealth BuildingHammersmith Campus

//

Summary

 

Publications

Citation

BibTex format

@article{Rosenberg:2020:clinem/dgaa168,
author = {Rosenberg, AGW and Pellikaan, K and Poitou, C and Goldstone, AP and Høybye, C and Markovic, T and Grugni, G and Crinò, A and Caixàs, A and Coupaye, M and Van, Den Berg SAA and Van, Der Lely AJ and De, Graaff LCG},
doi = {clinem/dgaa168},
journal = {Journal of Clinical Endocrinology and Metabolism},
pages = {e2563--e2571},
title = {Central adrenal insufficiency is rare in adults with Prader-Willi syndrome},
url = {http://dx.doi.org/10.1210/clinem/dgaa168},
volume = {105},
year = {2020}
}
Download

RIS format (EndNote, RefMan)

TY  - JOUR
AB  - 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. PATIENTS OR OTHER PARTICIPANTS: Eighty-two adults with genetically confirmed PWS. MAIN OUTCOME MEASURE: For 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. 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.
AU  - Rosenberg,AGW
AU  - Pellikaan,K
AU  - Poitou,C
AU  - Goldstone,AP
AU  - Høybye,C
AU  - Markovic,T
AU  - Grugni,G
AU  - Crinò,A
AU  - Caixàs,A
AU  - Coupaye,M
AU  - Van,Den Berg SAA
AU  - Van,Der Lely AJ
AU  - De,Graaff LCG
DO  - clinem/dgaa168
EP  - 2571
PY  - 2020///
SN  - 0021-972X
SP  - 2563
TI  - Central adrenal insufficiency is rare in adults with Prader-Willi syndrome
T2  - Journal of Clinical Endocrinology and Metabolism
UR  - http://dx.doi.org/10.1210/clinem/dgaa168
UR  - https://www.ncbi.nlm.nih.gov/pubmed/32232324
UR  - http://hdl.handle.net/10044/1/81324
VL  - 105
ER  -
Download