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Evaluation of the hypothalamic-pituitaryadrenal axis and its relationship with central respiratory dysfunction in children with Prader-Willi syndrome.
- Source :
- Orphanet Journal of Rare Diseases; 9/2/2015, Vol. 10 Issue 1, p1-8, 8p
- Publication Year :
- 2015
-
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 τ = -0.411; p = 0.012). A similar though non-significant correlation was present between cortisol increase and age (τ = -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. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 17501172
- Volume :
- 10
- Issue :
- 1
- Database :
- Complementary Index
- Journal :
- Orphanet Journal of Rare Diseases
- Publication Type :
- Academic Journal
- Accession number :
- 109505506
- Full Text :
- https://doi.org/10.1186/s13023-015-0312-z