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.

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.

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

scholarly journals Adrenal insufficiency in a woman secondary to standard-dose inhaled fluticasone propionate therapy

2014 ◽  
Vol 2014 ◽  
Author(s):  
Casey M Hay ◽  
Daniel I Spratt
Keyword(s):  
Fluticasone Propionate ◽  
Adrenal Insufficiency ◽  
Cortisol Level ◽  
Standard Dose ◽  
Clinical Signs ◽  
Unknown Origin ◽  
Adrenal Crisis ◽  
List Type ◽  
Morning Cortisol ◽  
Low Serum

Summary A 55-year-old woman with asthma presented with adrenal insufficiency of unknown origin. She was referred to our Division of Reproductive Endocrinology to further evaluate an undetectable morning cortisol level discovered during the evaluation of a low serum DHEA-S level. She was asymptomatic other than having mild fatigue and weight gain. Her medication list included 220 μg of inhaled fluticasone propionate twice daily for asthma, which she was taking as prescribed. On presentation, the undetectable morning cortisol level was confirmed. A urinary measurement of fluticasone propionate 17β-carboxylic acid was markedly elevated. Fluticasone therapy was discontinued and salmeterol therapy initiated with supplemental hydrocortisone. Hydrocortisone therapy was discontinued after 2 months. A repeat urinary fluticasone measurement 4 months after the discontinuation of fluticasone therapy was undetectably low and morning cortisol level was normal at 18.0 μg/dl. Inhaled fluticasone is generally considered to be minimally systemically absorbed. This patient's only clinical evidence suggesting adrenal insufficiency was fatigue accompanying a low serum DHEA-S level. This case demonstrates that adrenal insufficiency can be caused by a routine dose of inhaled fluticasone. Missing this diagnosis could potentially result in adrenal crisis upon discontinuation of fluticasone therapy. Learning points Standard-dose inhaled fluticasone can cause adrenal insufficiency. Adrenal insufficiency should be considered in patients taking, or who have recently discontinued, inhaled fluticasone therapy and present with new onset of nonspecific symptoms such as fatigue, weakness, depression, myalgia, arthralgia, unexplained weight loss, and nausea that are suggestive of adrenal insufficiency. Adrenal insufficiency should be considered in postoperative patients who exhibit signs of hypoadrenalism after fluticasone therapy has been withheld in the perioperative setting. Routine screening for hypoadrenalism in patients without clinical signs or symptoms of adrenal insufficiency after the discontinuation of inhaled fluticasone therapy is not indicated due to the apparently low incidence of adrenal insufficiency caused by fluticasone.

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 The incidence of adrenal crisis in the postoperative period of HPA axis insufficiency after surgical treatment for Cushing’s syndrome

2019 ◽  
Vol 181 (2) ◽  
pp. 201-210 ◽  
Author(s):  
Leonie H A Broersen ◽  
Femke M van Haalen ◽  
Tina Kienitz ◽  
Olaf M Dekkers ◽  
Christian J Strasburger ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Hpa Axis ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Adrenal Crisis ◽  
Pituitary Hormone ◽  
Increased Risk ◽  
Potential Risk Factors ◽  
Life Threatening ◽  
Glucocorticoid Deficiency

Background Adrenal crisis, the most feared complication of adrenal insufficiency, is a potentially life-threatening state of acute glucocorticoid deficiency. After successful surgery for Cushing’s syndrome, many patients develop (transient) adrenal insufficiency. The incidence of adrenal crisis in patients treated for hypercortisolism is unknown. Methods Cohort study included consecutive patients with Cushing’s syndrome with adrenal insufficiency after surgery from Leiden and Berlin from 2000 to 2015. We summarized the incidence of adrenal crisis, compared patients with and without adrenal crisis regarding potential risk factors for its occurrence and assessed the effect of better education in time on incidence of adrenal crisis. Results We included 106 patients, of whom 19 patients had a total of 41 adrenal crises. There were 9.0 crises per 100 patient-years at risk (95% confidence interval (CI): 6.7–12.0). All crises occurred while on hydrocortisone replacement. The risk ratio for a recurrent crisis was 2.3 (95% CI: 1.2–4.6). No clear change in incidence of adrenal crisis due to better education in time was observed. There was no difference in recurrence rate between patients with, and without any crisis, but patients with adrenal crisis had more often pituitary deficiencies. Conclusions The incidence of adrenal crises after treatment for Cushing’s syndrome is substantial, and patients who suffered from an adrenal crisis have higher risk for recurrent crisis. Adrenal crisis tends to present early after remission of Cushing’s syndrome, which is probably the period of severest HPA axis suppression, despite in general higher hydrocortisone replacement doses for withdrawal complaints in this period. Additional pituitary hormone deficiencies may be a risk marker for increased risk of adrenal crisis. However, further risk factor analysis is needed to identify risks for a first crisis. Effective education methods to prevent adrenal crises should be identified and implemented, including stress instructions by trained nursing staff before hospital discharge.

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 Case Series: Four Cases of Anorexia Nervosa Concomitant with Central Adrenal Insufficiency

2021 ◽  
Author(s):  
Sunao Matsubayashi ◽  
Madoka Tanaka ◽  
Takeshi Hara
Keyword(s):  
Anorexia Nervosa ◽  
Adrenal Insufficiency ◽  
Case Series ◽  
Addison’S Disease ◽  
Tolerance Test ◽  
Addison's Disease ◽  
Level Of Evidence ◽  
Insulin Tolerance ◽  
History Of ◽  
Central Adrenal Insufficiency

Abstract Objective: Widespread attention has been paid to the misdiagnosis of life-threatening Addison's disease as anorexia nervosa. However, there are no reports on the possible comorbidity of Addison's disease and other adrenal insufficiencies with anorexia nervosa.Methods: A case-series presentation of anorexia nervosa concomitant with central adrenal insufficiency.Results: Four anorexia nervosa patients (21-35 years old, all females) complained of severe fatigue during their treatment. After a thorough examination of the hypothalamus-pituitary-adrenal axis using stimulation with a rapid adenocorticotropic hormone test of 250-µg Cortrosyn®, a corticotropin-releasing hormone test, and an insulin tolerance test, central adrenal insufficiency was diagnosed. Two of the four patients had a history of exogenous steroids for their history of comorbidity. One of the residual two patient had Rathke’s cleft cyst. After the initiation of hydrocortisone replacement the patient's fatigue symptoms improved and they　were able to return to school and their workplace. In some cases, their weight obsession was reduced after the initiation of hydrocortisone replacement.Conclusion: Anorexia nervosa may be concomitant with central adrenal insufficiency partly in relation to exogenous steroids used for their history of comorbidity, which needs to be kept in mind when treating such patients.Level of EvidenceLevel V, descriptive study.

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