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Evaluation of the hypothalamic-pituitary-adrenal axis and its relationship with central respiratory dysfunction in children with Prader-Willi syndrome

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Abstract
Background: Children with Prader-Willi Syndrome (PWS) have been considered at risk for central adrenal insufficiency (CAI). Hypothalamic dysregulation has been proposed as a common mechanism underlying both stress-induced CAI and central respiratory dysfunction during sleep. Objective: To evaluate CAI and sleep-related breathing disorders in PWS children. Patients and methods: Retrospective study of cortisol response following either insulin tolerance test (ITT) or glucagon test (GT) in 20 PWS children, and comparison with 33 non-Growth Hormone deficient (GHD) controls. Correlation between sleep related breathing disorders and cortisol response in 11 PWS children who received both investigations. Results: In PWS children, the cortisol peak value showed a significant, inverse correlation with age (Kendall's t = -0.411; p = 0.012). A similar though non-significant correlation was present between cortisol increase and age (t = -0.232; p = 0.16). Similar correlations were found in controls. In only 1 of 20 PWS children (5 %), ITT was suggestive of CAI. Four patients had an elevated central apnea index but they all exhibited a normal cortisol response. No relationship was found between peak cortisol or cortisol increase and central apnea index (respectively p = 0.94 and p = 0.14) or the other studied polysomnography (PSG) parameters. Conclusions: CAI assessed by ITT/GT is rare in PWS children. Our data do not support a link between CAI and central respiratory dysregulation.
Keywords
NORMAL CORTISOL RESPONSE, Prader-Willi, GROWTH-HORMONE THERAPY, Sleep disorders, Adrenal insufficiency, INSULIN TOLERANCE-TEST, STIMULATION TEST, DOSE SYNACTHEN, ACTH TEST, INSUFFICIENCY, SLEEP, DIAGNOSIS, ADULTS

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MLA
Beauloye, Veronique et al. “Evaluation of the Hypothalamic-pituitary-adrenal Axis and Its Relationship with Central Respiratory Dysfunction in Children with Prader-Willi Syndrome.” ORPHANET JOURNAL OF RARE DISEASES 10 (2015): n. pag. Print.
APA
Beauloye, V., Dhondt, K., Buysse, W., Nyakasane, A., Zech, F., De Schepper, J., Van Aken, S., et al. (2015). Evaluation of the hypothalamic-pituitary-adrenal axis and its relationship with central respiratory dysfunction in children with Prader-Willi syndrome. ORPHANET JOURNAL OF RARE DISEASES, 10.
Chicago author-date
Beauloye, Veronique, Karlien Dhondt, W Buysse, A Nyakasane, F Zech, Jean De Schepper, Sara Van Aken, et al. 2015. “Evaluation of the Hypothalamic-pituitary-adrenal Axis and Its Relationship with Central Respiratory Dysfunction in Children with Prader-Willi Syndrome.” Orphanet Journal of Rare Diseases 10.
Chicago author-date (all authors)
Beauloye, Veronique, Karlien Dhondt, W Buysse, A Nyakasane, F Zech, Jean De Schepper, Sara Van Aken, Kathleen De Waele, Margarita Craen, I Gies, I François, D Beckers, An Desloovere, G François, and Martine Cools. 2015. “Evaluation of the Hypothalamic-pituitary-adrenal Axis and Its Relationship with Central Respiratory Dysfunction in Children with Prader-Willi Syndrome.” Orphanet Journal of Rare Diseases 10.
Vancouver
1.
Beauloye V, Dhondt K, Buysse W, Nyakasane A, Zech F, De Schepper J, et al. Evaluation of the hypothalamic-pituitary-adrenal axis and its relationship with central respiratory dysfunction in children with Prader-Willi syndrome. ORPHANET JOURNAL OF RARE DISEASES. 2015;10.
IEEE
[1]
V. Beauloye et al., “Evaluation of the hypothalamic-pituitary-adrenal axis and its relationship with central respiratory dysfunction in children with Prader-Willi syndrome,” ORPHANET JOURNAL OF RARE DISEASES, vol. 10, 2015.
@article{7221663,
  abstract     = {Background: Children with Prader-Willi Syndrome (PWS) have been considered at risk for central adrenal insufficiency (CAI). Hypothalamic dysregulation has been proposed as a common mechanism underlying both stress-induced CAI and central respiratory dysfunction during sleep. 
Objective: To evaluate CAI and sleep-related breathing disorders in PWS children. 
Patients and methods: Retrospective study of cortisol response following either insulin tolerance test (ITT) or glucagon test (GT) in 20 PWS children, and comparison with 33 non-Growth Hormone deficient (GHD) controls. Correlation between sleep related breathing disorders and cortisol response in 11 PWS children who received both investigations. 
Results: In PWS children, the cortisol peak value showed a significant, inverse correlation with age (Kendall's t = -0.411; p = 0.012). A similar though non-significant correlation was present between cortisol increase and age (t = -0.232; p = 0.16). Similar correlations were found in controls. In only 1 of 20 PWS children (5 %), ITT was suggestive of CAI. Four patients had an elevated central apnea index but they all exhibited a normal cortisol response. No relationship was found between peak cortisol or cortisol increase and central apnea index (respectively p = 0.94 and p = 0.14) or the other studied polysomnography (PSG) parameters. 
Conclusions: CAI assessed by ITT/GT is rare in PWS children. Our data do not support a link between CAI and central respiratory dysregulation.},
  articleno    = {106},
  author       = {Beauloye, Veronique and Dhondt, Karlien and Buysse, W and Nyakasane, A and Zech, F and De Schepper, Jean and Van Aken, Sara and De Waele, Kathleen and Craen, Margarita and Gies, I and François, I and Beckers, D and Desloovere, An and François, G and Cools, Martine},
  issn         = {1750-1172},
  journal      = {ORPHANET JOURNAL OF RARE DISEASES},
  keywords     = {NORMAL CORTISOL RESPONSE,Prader-Willi,GROWTH-HORMONE THERAPY,Sleep disorders,Adrenal insufficiency,INSULIN TOLERANCE-TEST,STIMULATION TEST,DOSE SYNACTHEN,ACTH TEST,INSUFFICIENCY,SLEEP,DIAGNOSIS,ADULTS},
  language     = {eng},
  pages        = {8},
  title        = {Evaluation of the hypothalamic-pituitary-adrenal axis and its relationship with central respiratory dysfunction in children with Prader-Willi syndrome},
  url          = {http://dx.doi.org/10.1186/s13023-015-0312-z},
  volume       = {10},
  year         = {2015},
}

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