scholarly journals CORRIGENDUM for “Central Adrenal Insufficiency Is Rare in Adults With Prader–Willi Syndrome”

2020 ◽  
Vol 105 (8) ◽  
pp. e3052-e3052
Keyword(s):  
Adrenal Insufficiency ◽  
Prader Willi Syndrome ◽  
Central Adrenal Insufficiency

No central adrenal insufficiency found in adults with prader-willi syndrome tested by multiple dose metyrapone test

2019 ◽  
Author(s):  
Anna Rosenberg ◽  
Karlijn Pellikaan ◽  
Kirsten Davidse ◽  
Stephany Donze ◽  
Anita Hokken-Koelega ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Multiple Dose ◽  
Prader Willi Syndrome ◽  
Central Adrenal Insufficiency

scholarly journals SAT-446 No Central Adrenal Insufficiency Found in Adults with Prader-Willi Syndrome Tested by Multiple-Dose Metyrapone Test

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Kirsten Davidse ◽  
Anna Rosenberg ◽  
Karlijn Pellikaan ◽  
Stephany Donze ◽  
Anita Hokken-Koelega ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Multiple Dose ◽  
Prader Willi Syndrome ◽  
Central Adrenal Insufficiency

P-102 The relationship between central adrenal insufficiency and sleep related breathing disorders in children with Prader-Willi syndrome

2008 ◽  
Vol 18 ◽  
pp. S57
Author(s):  
R. de Lind van Wijngaarden ◽  
S. van den Berg ◽  
K.F.M. Joosten ◽  
F.H. de Jong ◽  
C.G.J. Sweep ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Prader Willi Syndrome ◽  
Sleep Related Breathing Disorders ◽  
Breathing Disorders ◽  
Central Adrenal Insufficiency ◽  
The Relationship

Assessment of central adrenal insufficiency in children and adolescents with Prader-Willi syndrome

2012 ◽  
Vol 76 (6) ◽  
pp. 843-850 ◽  
Author(s):  
Andrea Corrias ◽  
Graziano Grugni ◽  
Antonino Crinò ◽  
Stefania Di Candia ◽  
Patrizia Chiabotto ◽  
...  
Keyword(s):  
Children And Adolescents ◽  
Adrenal Insufficiency ◽  
Prader Willi Syndrome ◽  
Central Adrenal Insufficiency

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

2020 ◽  
Vol 105 (7) ◽  
pp. e2563-e2571 ◽  
Author(s):  
Anna G W Rosenberg ◽  
Karlijn Pellikaan ◽  
Christine Poitou ◽  
Anthony P Goldstone ◽  
Charlotte Høybye ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Outcome Measure ◽  
Tolerance Test ◽  
Pituitary Hormone ◽  
Prader Willi Syndrome ◽  
Insulin Tolerance ◽  
Synacthen Test ◽  
Central Adrenal Insufficiency ◽  
Stress Dose ◽  
Hydrocortisone Treatment

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. 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.

Central adrenal insufficiency in young adults with Prader-Willi Syndrome

2013 ◽  
Vol 79 (3) ◽  
pp. 371-378 ◽  
Author(s):  
Graziano Grugni ◽  
Luciano Beccaria ◽  
Andrea Corrias ◽  
Antonino Crinò ◽  
Marco Cappa ◽  
...  
Keyword(s):  
Young Adults ◽  
Adrenal Insufficiency ◽  
Prader Willi Syndrome ◽  
Central Adrenal Insufficiency

scholarly journals High Prevalence of Central Adrenal Insufficiency in Patients with Prader-Willi Syndrome

2008 ◽  
Vol 93 (5) ◽  
pp. 1649-1654 ◽  
Author(s):  
Roderick F. A. de Lind van Wijngaarden ◽  
Barto J. Otten ◽  
Dederieke A. M. Festen ◽  
Koen F. M. Joosten ◽  
Frank H. de Jong ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Prader Willi Syndrome ◽  
High Prevalence ◽  
Central Adrenal Insufficiency

scholarly journals The Relationship between Central Adrenal Insufficiency and Sleep-Related Breathing Disorders in Children with Prader-Willi Syndrome

2009 ◽  
Vol 94 (7) ◽  
pp. 2387-2393 ◽  
Author(s):  
Roderick F. A. de Lind van Wijngaarden ◽  
Koen F. M. Joosten ◽  
Sandra van den Berg ◽  
Barto J. Otten ◽  
Frank H. de Jong ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Pediatric Intensive Care ◽  
Interquartile Range ◽  
Index Number ◽  
Prader Willi Syndrome ◽  
Central Apnea ◽  
Sleep Related Breathing Disorders ◽  
Breathing Disorders ◽  
Central Adrenal Insufficiency ◽  
The Relationship

Background: The annual death rate of patients with Prader-Willi syndrome (PWS) is high (3%). Many deaths of children are sudden and unexplained. Sleep apneas have been suggested to play a role in sudden deaths. Recently, we discovered that 60% of patients with PWS suffer from central adrenal insufficiency (CAI) during stress. Objective: The aim was to study the relationship between CAI and sleep-related breathing disorders. Design: In 20 children with PWS who underwent a metyrapone test (30 mg/kg at 2330 h), sleep-related breathing was evaluated by polysomnography before the metyrapone test. In addition, we recorded sleep-related breathing in 10 children with PWS during their metyrapone test. CAI was diagnosed when ACTH levels during the metyrapone test were below 33 pmol/liter at 0730 h. All tests were performed during healthy condition. Setting: The study was conducted in a pediatric intensive care unit and specialized sleep center. Results: Median (interquartile range) age was 8.4 yr (6.5–10.2). After metyrapone administration, median (interquartile range) central apnea index (number/hour) increased significantly from 2.2 (0.4–4.7) to 5.2 (1.5–7.9) (P = 0.007). The increase tended to be higher in children with CAI [2.8 (2.0–3.9) vs. 1.0 (−0.2 to 2.6); P = 0.09]. During polysomnography before the metyrapone test, sleep-related breathing was worse in children with CAI, who had a significantly higher central apnea index and tended to have a lower minimum oxygen saturation compared to those without CAI (P = 0.03 and P = 0.07). Conclusions: In children with PWS, the central apnea index increased significantly after metyrapone administration, particularly in those with CAI during stress. In addition, children with CAI had a higher central apnea index compared to those without several months before the metyrapone test.

scholarly journals SUN-308 Central Adrenal Insufficiency Is Rare in Adults with Prader-Willi Syndrome

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Anna G W Rosenberg ◽  
Karlijn Pellikaan ◽  
Christine Poitou ◽  
Anthony P Goldstone ◽  
Charlotte Hoybye ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Cortisol Level ◽  
Multiple Dose ◽  
Tolerance Test ◽  
Adrenal Crisis ◽  
Pituitary Hormone ◽  
Prader Willi Syndrome ◽  
Gh Treatment ◽  
Baseline Cortisol ◽  
Central Adrenal Insufficiency

Abstract Introduction: 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. This is partly due to the variable results of the synacthen test, compared with the more robust metyrapone test (MTP) and insulin tolerance test (ITT). In some countries, patients with PWS receive stress-dose corticosteroids during physical or psychological stress. Side effects of frequent corticosteroids 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 over- and undertreatment with corticosteroids, we assessed the prevalence of CAI in a large international cohort of adults with this rare disorder. Methods: The hypothalamic-pituitary-adrenal axis was tested in 81 adult subjects (55 Dutch, 10 British, 10 French, 6 Swedish) with genetically confirmed PWS. For multiple-dose MTP, 11-deoxycortisol >230 nmol/L (7.6 g/dL) was considered sufficient. For Dutch, French and Swedish patients who underwent ITT, cortisol >500 nmol/L (18.1 μg/dL) was considered sufficient. For British patients cortisol >450 nmol/L (16.3 μg/dL) was considered sufficient, as this center used a different assay. Additionally, we reviewed medical files of 645 adults with PWS from Italy (240), France (110), the Netherlands (110), Australia (60), Spain (45), Sweden (38) and the United Kingdom (42) for symptoms of hypocortisolism/adrenal crisis during surgery. Results: Data on 81 adult subjects (46 males and 35 females), median age (range) 25.2 yr (18.0 – 55.5), median BMI (range) 29.1 kg/m2 (20.0 – 62.0), with genetically confirmed PWS were collected. 33 subjects (41%) were using GH treatment since childhood. Multiple-dose MTP was performed in 45 subjects and ITT in 36 subjects. Both tests were well tolerated by all individuals. CAI was excluded in 80 of 81 patients. One patient with a peak cortisol level of 494 nmol/L (just below cut-off level of 500 nmol/L) was prescribed hydrocortisone for use during physical stress. There was no relation between baseline cortisol and ITT/multiple-dose MTP results. Even patients with a low baseline cortisol level (lowest: 102.0 nmol/L) had normal responses. Among the 645 patients whose medical files were reviewed, 200 had undergone surgery without perioperative corticosteroids treatment. None of them displayed any features of hypocortisolism/adrenal crisis. Conclusions: CAI is rare (1.2%) in adults with PWS. Based on these results, we recommend against routinely prescribing corticosteroids stress-doses in adults with PWS. Funding: CZ foundation.

No central adrenal insufficiency found in patients with Prader-Willi syndrome with an overnight metyrapone test

2018 ◽  
Vol 31 (7) ◽  
pp. 809-814 ◽  
Author(s):  
Kathryn S. Obrynba ◽  
Robert P. Hoffman ◽  
David R. Repaske ◽  
Kathryn Anglin ◽  
Manmohan K. Kamboj
Keyword(s):  
Adrenal Insufficiency ◽  
Low Dose ◽  
False Positive Rate ◽  
Standard Test ◽  
Prader Willi Syndrome ◽  
Acth Stimulation Test ◽  
Acth Stimulation ◽  
Positive Rate ◽  
Central Adrenal Insufficiency ◽  
Method Of Assessment

Abstract Background Individuals with Prader-Willi syndrome (PWS) have hypothalamic dysfunction and may have central adrenal insufficiency (CAI). The prevalence of CAI in PWS remains unknown. Methods Twenty-one subjects with PWS aged 4–53 years underwent a low dose adrenocorticotropic hormone (ACTH) stimulation test (LDAST) (1 μg/m2, maximum 1 μg) followed by an overnight metyrapone test (OMT). Metyrapone (30 mg/kg, maximum 3 g) was administered at 2400 h. Cortisol, 11-deoxycortisol (11-DOC) and ACTH levels were collected the following morning at 0800 h. OMT was the standard test for comparison. Peak cortisol ≥15.5 μg/dL (427.6 nmol/L) on LDAST and 0800 h 11-DOC ≥7 μg/dL (200 nmol/L) on OMT were classified as adrenal sufficiency. Results Twenty subjects had 0800 h 11-DOC values ≥7 μg/dL on OMT indicating adrenal sufficiency. One subject had an inconclusive OMT result. Six of the 21 (29%) subjects had peak cortisol <15.5 μg/dL on LDAST. Conclusions We found no evidence of CAI based on OMT, yet 29% of our PWS population failed the LDAST. This suggests that the LDAST may have a high false positive rate in diagnosing CAI in individuals with PWS. OMT may be the preferred method of assessment for CAI in patients with PWS.

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