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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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Chicago
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.
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.
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.
MLA
Beauloye, Veronique, KARLIEN DHONDT, W Buysse, 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.
@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 Fran\c{c}ois, I and Beckers, D and Desloovere, An and Fran\c{c}ois, G and Cools, Martine},
  issn         = {1750-1172},
  journal      = {ORPHANET JOURNAL OF RARE DISEASES},
  keyword      = {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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