The relationship between central adrenal insufficiency and sleep-related breathing disorders in children with Prader-Willi syndrome
| Autor: | C. G. J. (Fred) Sweep, Roderick F. A. de Lind van Wijngaarden, Barto J. Otten, Al W. de Weerd, Koen F. M. Joosten, Anita C. S. Hokken-Koelega, Frank H. de Jong, Sandra van den Berg |
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| Přispěvatelé: | Pediatrics, Internal Medicine |
| Rok vydání: | 2009 |
| Předmět: |
Male
medicine.medical_specialty Polysomnography Endocrinology Diabetes and Metabolism Clinical Biochemistry Aetiology screening and detection [ONCOL 5] Neurological disorder Central adrenal insufficiency Biochemistry Diagnostic Techniques Endocrine Sleep Apnea Syndromes Endocrinology Translational research [ONCOL 3] Interquartile range Internal medicine medicine Adrenal insufficiency Humans Child Pediatric intensive care unit Sleep disorder Metyrapone medicine.diagnostic_test business.industry Hormonal regulation [IGMD 6] Biochemistry (medical) medicine.disease Child Preschool Female Sleep business Prader-Willi Syndrome Adrenal Insufficiency medicine.drug |
| Zdroj: | Journal of Clinical Endocrinology and Metabolism, 94, 2387-93 Journal of Clinical Endocrinology and Metabolism, 94, 7, pp. 2387-93 Journal of Clinical Endocrinology and Metabolism, 94(7), 2387-2393. Endocrine Society |
| ISSN: | 0021-972X |
| Popis: | Contains fulltext : 81198.pdf (Publisher’s version ) (Closed access) BACKGROUND: The annual death rate of patients with Prader-Willi syndrome (PWS) is high (3%). Many deaths of children are sudden and unexplained. Sleep apneas have been suggested to play a role in sudden deaths. Recently, we discovered that 60% of patients with PWS suffer from central adrenal insufficiency (CAI) during stress. OBJECTIVE: The aim was to study the relationship between CAI and sleep-related breathing disorders. DESIGN: In 20 children with PWS who underwent a metyrapone test (30 mg/kg at 2330 h), sleep-related breathing was evaluated by polysomnography before the metyrapone test. In addition, we recorded sleep-related breathing in 10 children with PWS during their metyrapone test. CAI was diagnosed when ACTH levels during the metyrapone test were below 33 pmol/liter at 0730 h. All tests were performed during healthy condition. Setting: The study was conducted in a pediatric intensive care unit and specialized sleep center. RESULTS: Median (interquartile range) age was 8.4 yr (6.5-10.2). After metyrapone administration, median (interquartile range) central apnea index (number/hour) increased significantly from 2.2 (0.4-4.7) to 5.2 (1.5-7.9) (P = 0.007). The increase tended to be higher in children with CAI [2.8 (2.0-3.9) vs. 1.0 (-0.2 to 2.6); P = 0.09]. During polysomnography before the metyrapone test, sleep-related breathing was worse in children with CAI, who had a significantly higher central apnea index and tended to have a lower minimum oxygen saturation compared to those without CAI (P = 0.03 and P = 0.07). CONCLUSIONS: In children with PWS, the central apnea index increased significantly after metyrapone administration, particularly in those with CAI during stress. In addition, children with CAI had a higher central apnea index compared to those without several months before the metyrapone test. |
| Databáze: | OpenAIRE |
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