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

Beauloye V, Dhondt K, Buysse W, Nyakasane A, Zech F, De Schepper J, Van Aken S, De Waele K, Craen M, Gies I, Francois I, Beckers D, Desloovere A, Francois G, Cools M - Orphanet J Rare Dis (2015)

Bottom Line: Similar correlations were found in controls.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.Our data do not support a link between CAI and central respiratory dysregulation.

View Article: PubMed Central - PubMed

Affiliation: Unité d'Endocrinologie pédiatrique, Cliniques Universitaires Saint-Luc, Université catholique de Louvain, avenue Hippocrate 10/1300, Brussels, B-1200, Belgium. veronique.beauloye@uclouvain.be.

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.

No MeSH data available.


Related in: MedlinePlus

a basal, b peak cortisol levels and c cortisol increase in PWS and control children after a glucagon (GT) or an insulin tolerance test (ITT). The black lines represent the medians
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License 1 - License 2
getmorefigures.php?uid=PMC4557896&req=5

Fig1: a basal, b peak cortisol levels and c cortisol increase in PWS and control children after a glucagon (GT) or an insulin tolerance test (ITT). The black lines represent the medians

Mentions: Basal cortisol levels did not differ between the PWS and the control children (p = 0.20) (Fig. 1a). Peak cortisol levels (Fig. 1b) and cortisol increase (Fig. 1c) were also not significantly different between the two groups (respectively p = 0.7 and p = 0.64). In both the PWS and control group, no difference was observed between the GT- or the ITT-induced peak cortisol response (respectively, p = 0.57 and p = 0.73) or cortisol increase (respectively, p = 0.24 and p = 0.63). Therefore results of both tests were combined for the correlation analysis. The variance of the cortisol levels was not statistically different between the two groups (PWS and controls) and between the two stimulation tests (ITT and GT) (PWS versus control group: basal cortisol levels, p = 0.55; peak cortisol levels, p = 0.505; cortisol increase, p = 0.57; ITT versus GT, even after adjustment for the group: basal cortisol levels, p = 0.44; peak cortisol levels, p = 0.49; cortisol increase, p = 0.16). No significant correlation between peak cortisol levels or cortisol increase was found with gender, BMI-z-score, GH peak or GH status neither in the PWS group nor in the control group (Table 2). However, the cortisol peak value showed a significant inverse correlation with age in both PWS and control children (Fig. 2) (respectively, p = 0.012 and p = 0.0067). The decrease with age of the peak cortisol levels after stimulation test was similar in the two groups (p = 0.13). A similar inverse correlation between cortisol increase during the stimulation test and age was present but only significant in the control group. A significant relationship between cortisol response and CA index (Fig. 3), or other PSG parameters in PWS children could not be demonstrated.Fig. 1


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

Beauloye V, Dhondt K, Buysse W, Nyakasane A, Zech F, De Schepper J, Van Aken S, De Waele K, Craen M, Gies I, Francois I, Beckers D, Desloovere A, Francois G, Cools M - Orphanet J Rare Dis (2015)

a basal, b peak cortisol levels and c cortisol increase in PWS and control children after a glucagon (GT) or an insulin tolerance test (ITT). The black lines represent the medians
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4557896&req=5

Fig1: a basal, b peak cortisol levels and c cortisol increase in PWS and control children after a glucagon (GT) or an insulin tolerance test (ITT). The black lines represent the medians
Mentions: Basal cortisol levels did not differ between the PWS and the control children (p = 0.20) (Fig. 1a). Peak cortisol levels (Fig. 1b) and cortisol increase (Fig. 1c) were also not significantly different between the two groups (respectively p = 0.7 and p = 0.64). In both the PWS and control group, no difference was observed between the GT- or the ITT-induced peak cortisol response (respectively, p = 0.57 and p = 0.73) or cortisol increase (respectively, p = 0.24 and p = 0.63). Therefore results of both tests were combined for the correlation analysis. The variance of the cortisol levels was not statistically different between the two groups (PWS and controls) and between the two stimulation tests (ITT and GT) (PWS versus control group: basal cortisol levels, p = 0.55; peak cortisol levels, p = 0.505; cortisol increase, p = 0.57; ITT versus GT, even after adjustment for the group: basal cortisol levels, p = 0.44; peak cortisol levels, p = 0.49; cortisol increase, p = 0.16). No significant correlation between peak cortisol levels or cortisol increase was found with gender, BMI-z-score, GH peak or GH status neither in the PWS group nor in the control group (Table 2). However, the cortisol peak value showed a significant inverse correlation with age in both PWS and control children (Fig. 2) (respectively, p = 0.012 and p = 0.0067). The decrease with age of the peak cortisol levels after stimulation test was similar in the two groups (p = 0.13). A similar inverse correlation between cortisol increase during the stimulation test and age was present but only significant in the control group. A significant relationship between cortisol response and CA index (Fig. 3), or other PSG parameters in PWS children could not be demonstrated.Fig. 1

Bottom Line: Similar correlations were found in controls.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.Our data do not support a link between CAI and central respiratory dysregulation.

View Article: PubMed Central - PubMed

Affiliation: Unité d'Endocrinologie pédiatrique, Cliniques Universitaires Saint-Luc, Université catholique de Louvain, avenue Hippocrate 10/1300, Brussels, B-1200, Belgium. veronique.beauloye@uclouvain.be.

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.

No MeSH data available.


Related in: MedlinePlus