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Published in: BMC Endocrine Disorders 1/2020

Open Access 01-12-2020 | Obesity | Research article

Hair cortisol-a method to detect chronic cortisol levels in patients with Prader-Willi syndrome

Authors: Hasanain Hamid Shukur, Yolanda B. de Rijke, Elisabeth F. C. van Rossum, Laith Hussain-Alkhateeb, Charlotte Höybye

Published in: BMC Endocrine Disorders | Issue 1/2020

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Abstract

Background

Prader-Willi syndrome (PWS) is a multisymptomatic, rare, genetic, neurodevelopmental disorder in adults mainly characterized by hyperphagia, cognitive dysfunction, behavioral problems and risk of morbid obesity. Although endocrine insufficiencies are common, hypocortisolism is rare and knowledge on long-term cortisol concentrations is lacking. The aim of this study was to evaluate long-term cortisol levels in PWS by measurements of hair cortisol.

Methods

Twenty-nine adults with PWS, 15 men and 14 women, median age 29 years, median BMI 27 kg/m2, were included. Scalp hair samples were analyzed for cortisol content using liquid-chromatography tandem-mass spectrometry. In addition, a questionnaire on auxology, medication and stress were included. For comparison, 105 age- and sex-matched participants from the population-based Lifelines Cohort study were included as controls. The mean hair cortisol between the groups were compared and associations between BMI and stress were assessed by a generalized linear regression model.

Results

In the PWS group large variations in hair cortisol was seen. Mean hair cortisol was 12.8 ± 25.4 pg/mg compared to 3.8 ± 7.3 pg/mg in controls (p = 0.001). The linear regression model similarly showed higher cortisol levels in patients with PWS, which remained consistent after adjusting for BMI and stress (p = 0.023). Furthermore, hair cortisol increased with BMI (p = 0.012) and reported stress (p = 0.014).

Conclusion

Long-term cortisol concentrations were higher in patients with PWS compared to controls and increased with BMI and stress, suggesting an adequate cortisol response to chronic stress. Hair cortisol demonstrate promising applications in the context of PWS treatment and disease management.
Literature
1.
go back to reference Deal C, Tony M, Höybye C, Allen DB, Tauber M, Christiansen JS. Growth hormone research society workshop summary: consensus guidelines for recombinant human growth hormone therapy in Prader-Willi syndrome. J Clin Endocrinol Metab. 2013;98(6):E1072–87.CrossRef Deal C, Tony M, Höybye C, Allen DB, Tauber M, Christiansen JS. Growth hormone research society workshop summary: consensus guidelines for recombinant human growth hormone therapy in Prader-Willi syndrome. J Clin Endocrinol Metab. 2013;98(6):E1072–87.CrossRef
2.
go back to reference Cassidy SB, Schwartz S. Prader-Willi and Angelman syndromes. Disorders of genomic imprinting. Medicine (Baltimore). 1998;77:140–51.CrossRef Cassidy SB, Schwartz S. Prader-Willi and Angelman syndromes. Disorders of genomic imprinting. Medicine (Baltimore). 1998;77:140–51.CrossRef
3.
go back to reference Goldstone AP, Holland AJ, Hauffa BP, Hokken-Koelega AC, Tauber M. Recommendations for the diagnosis and management of Prader-Willi syndrome. J Clin Endocrinol Metab. 2008;93(11):4183–97.CrossRef Goldstone AP, Holland AJ, Hauffa BP, Hokken-Koelega AC, Tauber M. Recommendations for the diagnosis and management of Prader-Willi syndrome. J Clin Endocrinol Metab. 2008;93(11):4183–97.CrossRef
4.
go back to reference Ho AY, Dimitropoulos A. Clinical management of behavioral characteristics of Prader–Willi syndrome. Neuropsychiatr Dis Treat. 2010;6:107–18.PubMedPubMedCentral Ho AY, Dimitropoulos A. Clinical management of behavioral characteristics of Prader–Willi syndrome. Neuropsychiatr Dis Treat. 2010;6:107–18.PubMedPubMedCentral
5.
go back to reference Burman P, Ritzen EM, Lindgren AC. Endocrine dysfunction in Prader-Willi syndrome: a review with special reference to GH. Endocr Rev. 2001;22:787–99.CrossRef Burman P, Ritzen EM, Lindgren AC. Endocrine dysfunction in Prader-Willi syndrome: a review with special reference to GH. Endocr Rev. 2001;22:787–99.CrossRef
6.
go back to reference De Lind van Wijngaarden RF, Otten BJ, Festen DA, Joosten KF, de Jong FH, Sweep FC, Hokken-Koelega AC. High prevalence of central adrenal insufficiency in patients with Prader-Willi syndrome. J Clin Endocrinol Metab. 2008;93:1649–54.CrossRef De Lind van Wijngaarden RF, Otten BJ, Festen DA, Joosten KF, de Jong FH, Sweep FC, Hokken-Koelega AC. High prevalence of central adrenal insufficiency in patients with Prader-Willi syndrome. J Clin Endocrinol Metab. 2008;93:1649–54.CrossRef
9.
go back to reference Nyunt O, Cotterill AM, Archbold SMWJY, Leong GM, Verge CF, et al. Normal cortisol response of low-dose synacthen (1 microg) test in children with Prader-Willi syndrome. J Clin Endocrinol Metab. 2008;95:E464–7.CrossRef Nyunt O, Cotterill AM, Archbold SMWJY, Leong GM, Verge CF, et al. Normal cortisol response of low-dose synacthen (1 microg) test in children with Prader-Willi syndrome. J Clin Endocrinol Metab. 2008;95:E464–7.CrossRef
10.
go back to reference Farholt S, Sode-Carlsen R, Christiansen JS, Östergaard JR, Höybye C. Normal cortisol response to high-dose synacthen and insulin tolerance test in children and adults with Prader-Willi syndrome. J Clin Endocrinol Metab. 2011;96:E173–80.CrossRef Farholt S, Sode-Carlsen R, Christiansen JS, Östergaard JR, Höybye C. Normal cortisol response to high-dose synacthen and insulin tolerance test in children and adults with Prader-Willi syndrome. J Clin Endocrinol Metab. 2011;96:E173–80.CrossRef
11.
go back to reference Obrynba KS, Hoffman RP, Repaske DR, Anglin K, Kamboj MK. No central adrenal insufficiency found in patients with Prader-Willi syndrome with an overnight metyrapone test. J Pediatr Endocrinol Metab. 2018;31(7):809–14.CrossRef Obrynba KS, Hoffman RP, Repaske DR, Anglin K, Kamboj MK. No central adrenal insufficiency found in patients with Prader-Willi syndrome with an overnight metyrapone test. J Pediatr Endocrinol Metab. 2018;31(7):809–14.CrossRef
12.
go back to reference Manenschijn L, Koper JW, Lamberts SW, van Rossum EF. Evaluation of a method to measure long term cortisol levels. Steroids. 2011;76:1032–6.CrossRef Manenschijn L, Koper JW, Lamberts SW, van Rossum EF. Evaluation of a method to measure long term cortisol levels. Steroids. 2011;76:1032–6.CrossRef
13.
go back to reference Stolk RP, Rosmalen JG, Postma DS, de Boer RA, Navis G, Slaets JP, et al. Universal risk factors for multifactorial diseases: lifelines: a three-generation population-based study. Eur J Epidemiol. 2008;23:67–74.CrossRef Stolk RP, Rosmalen JG, Postma DS, de Boer RA, Navis G, Slaets JP, et al. Universal risk factors for multifactorial diseases: lifelines: a three-generation population-based study. Eur J Epidemiol. 2008;23:67–74.CrossRef
15.
go back to reference Wester VL, Noppe G, Savas M, van den Akker ELT, de Rijke YB, van Rossum EFC, et al. Hair analysis reveals subtle HPA axis suppression associated with use of local corticosteroids: the lifelines cohort study. J Psyconeuroendocrinology. 2017;80:1–6.CrossRef Wester VL, Noppe G, Savas M, van den Akker ELT, de Rijke YB, van Rossum EFC, et al. Hair analysis reveals subtle HPA axis suppression associated with use of local corticosteroids: the lifelines cohort study. J Psyconeuroendocrinology. 2017;80:1–6.CrossRef
16.
go back to reference Noppe G, de Rijke YB, Dorst K, Van den Akker EL, Rossum EF. LC-MS/MS-based method for long-term steroid profiling in human scalp hair. Clin Endocrinol. 2015;83:162–6.CrossRef Noppe G, de Rijke YB, Dorst K, Van den Akker EL, Rossum EF. LC-MS/MS-based method for long-term steroid profiling in human scalp hair. Clin Endocrinol. 2015;83:162–6.CrossRef
17.
go back to reference Kalra S, Einarson A, Karaskov T, Van Uum S, Koren G. The relationship between stress and hair cortisol in healthy pregnant women. Clin Invest Med. 2007;30:E103–7.CrossRef Kalra S, Einarson A, Karaskov T, Van Uum S, Koren G. The relationship between stress and hair cortisol in healthy pregnant women. Clin Invest Med. 2007;30:E103–7.CrossRef
18.
go back to reference Yamada J, Stevens B, de Silva N. Hair cortisol as a potential biologic marker of chronic stress in hospitalized neonates. Neonatology. 2007;92:42–9.CrossRef Yamada J, Stevens B, de Silva N. Hair cortisol as a potential biologic marker of chronic stress in hospitalized neonates. Neonatology. 2007;92:42–9.CrossRef
20.
go back to reference Van Uum SH, Sauvé B, Fraser LA, Morley-Forster P, Paul TL, Koren G. Elevated content of cortisol in hair of patients with severe chronic pain: a novel biomarker for stress. Stress. 2008;11:483–8.CrossRef Van Uum SH, Sauvé B, Fraser LA, Morley-Forster P, Paul TL, Koren G. Elevated content of cortisol in hair of patients with severe chronic pain: a novel biomarker for stress. Stress. 2008;11:483–8.CrossRef
21.
go back to reference Dettenborn L, Tietze A, Bruckner F, Kirschbaum C. Higher cortisol content in hair among long-term unemployed individuals compared to controls. Psychoneuroendocrinology. 2010;35:1404–9.CrossRef Dettenborn L, Tietze A, Bruckner F, Kirschbaum C. Higher cortisol content in hair among long-term unemployed individuals compared to controls. Psychoneuroendocrinology. 2010;35:1404–9.CrossRef
22.
go back to reference Manenschijn L, van Kruysbergen RG, de Jong FH, Koper JW, van Rossum EF. Shift work at young age is associated with elevated long-term cortisol levels and body mass index. J Clin Endocrinol Metab. 2011;96:E1862–5.CrossRef Manenschijn L, van Kruysbergen RG, de Jong FH, Koper JW, van Rossum EF. Shift work at young age is associated with elevated long-term cortisol levels and body mass index. J Clin Endocrinol Metab. 2011;96:E1862–5.CrossRef
23.
go back to reference Manenschijn L, Schaap L, van Schoor NM, van der Pas S, Peeters GM, Lips P, et al. High long-term cortisol levels, measured in scalp hair, are associated with a history of cardiovascular disease. J Clin Endocrinol Metab. 2013;98:2078–83.CrossRef Manenschijn L, Schaap L, van Schoor NM, van der Pas S, Peeters GM, Lips P, et al. High long-term cortisol levels, measured in scalp hair, are associated with a history of cardiovascular disease. J Clin Endocrinol Metab. 2013;98:2078–83.CrossRef
25.
go back to reference Russell E, Koren G, Rieder M, Van Uum S. Hair cortisol as a biological marker of chronic stress: current status, future directions and unanswered questions. Psychoneuroendocrinology. 2012;37(5):589–601.CrossRef Russell E, Koren G, Rieder M, Van Uum S. Hair cortisol as a biological marker of chronic stress: current status, future directions and unanswered questions. Psychoneuroendocrinology. 2012;37(5):589–601.CrossRef
26.
go back to reference Veldhorst MA, Noppe G, Jongejan MH, Jongejan MH, Kok CB, Mekic S, Koper JW, et al. Increased scalp hair cortisol concentrations in obese children. J Clin Endocrinol Metab. 2014 Jan;99(1):285–90.CrossRef Veldhorst MA, Noppe G, Jongejan MH, Jongejan MH, Kok CB, Mekic S, Koper JW, et al. Increased scalp hair cortisol concentrations in obese children. J Clin Endocrinol Metab. 2014 Jan;99(1):285–90.CrossRef
29.
go back to reference Stalder T, Steudte S, Alexander N, Miller R, Gao W, Dettenborn L, Kirschbaum C. Cortisol in hair, body mass index and stress-related measures. Biol Psychol. 2012 Jul;90(3):218–23.CrossRef Stalder T, Steudte S, Alexander N, Miller R, Gao W, Dettenborn L, Kirschbaum C. Cortisol in hair, body mass index and stress-related measures. Biol Psychol. 2012 Jul;90(3):218–23.CrossRef
30.
go back to reference Staufenbiel SM, Penninx BW, de Rijke YB, van den Akker EL, van Rossum EF. Determinants of hair cortisol and hair cortisone concentrations in adults. Psychoneuroendocrinology. 2015;60:182–94.CrossRef Staufenbiel SM, Penninx BW, de Rijke YB, van den Akker EL, van Rossum EF. Determinants of hair cortisol and hair cortisone concentrations in adults. Psychoneuroendocrinology. 2015;60:182–94.CrossRef
31.
go back to reference Hedgeman E, Ulrichsen SP, Carter S, Kreher NC, Malobisky KP, Braun MM, et al. Long-term health outcomes in patients with Prader–Willi Syndrome: a nationwide cohort study in Denmark. Int J Obes. 2017;41(10):1531–8. Hedgeman E, Ulrichsen SP, Carter S, Kreher NC, Malobisky KP, Braun MM, et al. Long-term health outcomes in patients with Prader–Willi Syndrome: a nationwide cohort study in Denmark. Int J Obes. 2017;41(10):1531–8.
32.
go back to reference Whittington JE, Holland AJ, Webb T, Bulter J, Clarke D, Boer H. Population prevalence and estimated birth incidence and mortality rate for people with Prader-Willi syndrome in one UK health region. J Med Genet. 2001;38:792–8.CrossRef Whittington JE, Holland AJ, Webb T, Bulter J, Clarke D, Boer H. Population prevalence and estimated birth incidence and mortality rate for people with Prader-Willi syndrome in one UK health region. J Med Genet. 2001;38:792–8.CrossRef
33.
go back to reference Wallace I, Cunningham S, Lindsay J. The diagnosis and investigation of adrenal insufficiency in adults. Ann Clin Biochem. 2009;46(Pt 5):351–67.CrossRef Wallace I, Cunningham S, Lindsay J. The diagnosis and investigation of adrenal insufficiency in adults. Ann Clin Biochem. 2009;46(Pt 5):351–67.CrossRef
Metadata
Title
Hair cortisol-a method to detect chronic cortisol levels in patients with Prader-Willi syndrome
Authors
Hasanain Hamid Shukur
Yolanda B. de Rijke
Elisabeth F. C. van Rossum
Laith Hussain-Alkhateeb
Charlotte Höybye
Publication date
01-12-2020
Publisher
BioMed Central
Keywords
Obesity
Obesity
Published in
BMC Endocrine Disorders / Issue 1/2020
Electronic ISSN: 1472-6823
DOI
https://doi.org/10.1186/s12902-020-00646-w

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