{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T08:00:57Z","timestamp":1783065657864,"version":"3.54.6"},"reference-count":41,"publisher":"The Endocrine Society","issue":"7","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2009,7,1]]},"abstract":"<jats:p>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.<\/jats:p><jats:p>Objective: The aim was to study the relationship between CAI and sleep-related breathing disorders.<\/jats:p><jats:p>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.<\/jats:p><jats:p>Setting: The study was conducted in a pediatric intensive care unit and specialized sleep center.<\/jats:p><jats:p>Results: Median (interquartile range) age was 8.4 yr (6.5\u201310.2). After metyrapone administration, median (interquartile range) central apnea index (number\/hour) increased significantly from 2.2 (0.4\u20134.7) to 5.2 (1.5\u20137.9) (P = 0.007). The increase tended to be higher in children with CAI [2.8 (2.0\u20133.9) vs. 1.0 (\u22120.2 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).<\/jats:p><jats:p>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.<\/jats:p>","DOI":"10.1210\/jc.2008-2808","type":"journal-article","created":{"date-parts":[[2009,4,22]],"date-time":"2009-04-22T02:29:39Z","timestamp":1240367379000},"page":"2387-2393","source":"Crossref","is-referenced-by-count":34,"title":["The Relationship between Central Adrenal Insufficiency and Sleep-Related Breathing Disorders in Children with Prader-Willi Syndrome"],"prefix":"10.1210","volume":"94","author":[{"given":"Roderick F. A.","family":"de Lind van Wijngaarden","sequence":"first","affiliation":[{"name":"Dutch Growth Research Foundation (R.F.A.d.L.v.W., A.C.S.H.-K.), 3016 AH Rotterdam, The Netherlands"},{"name":"Erasmus University Medical Center Rotterdam\/Sophia Children\u2019s Hospital, Department of Pediatrics, Subdivisions of Endocrinology (R.F.A.d.L.v.W., A.C.S.H.-K.), 3015 GJ Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koen F. 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