F R Livingston1, R Arens, S L Bailey, T G Keens, S L Ward. 1. Division of Neonatology and Pediatric Pulmonology, Childrens Hospital of Los Angeles, University of Southern California School of Medicine 90027, USA.
Abstract
STUDY OBJECTIVE: Prader-Willi syndrome (PWS) is characterized by a number of abnormalities of hypothalamic function, such as hyperphagia, short stature, temperature instability, hypogonadotropic hypogonadism, and neurosecretory growth hormone deficiency. Patients with PWS are reported to have sleep-disordered breathing and have blunted hypercapnic ventilatory responses secondary to abnormal peripheral chemoreceptor function. Thus, we hypothesized that hypercapnic arousal responses would be abnormal in PWS. DESIGN: Hypercapnic arousal responses were tested in ten nonobese children and adults with PWS, aged 17.7 +/- 2.5 (SEM) years, 70% female, and nine control subjects, aged 14.2 +/- 2.6 years, 67% female. Hypercapnic challenges were performed during stage 3/4 non-rapid eye movement sleep. RESULTS: The PWS subjects had a significantly higher arousal threshold to hypercapnia compared with the controls (53 +/- 1.0 vs 46 +/- 1.7 mm Hg; p < 0.01). The PWS subjects had significantly higher baseline end-tidal CO2 levels (42 +/- 0.8 vs 38 +/- 1.1 mm Hg; p < 0.01) and more central apneas greater than 15 s/h of sleep (1.5 +/- 0.3 vs 0.1 +/- 0.1; p < 0.01). CONCLUSIONS: Elevated hypercapnic arousal thresholds during sleep are found in PWS subjects; these may be a manifestation of abnormal peripheral chemoreceptor function and may further contribute to sleep-disordered breathing in PWS patients.
STUDY OBJECTIVE:Prader-Willi syndrome (PWS) is characterized by a number of abnormalities of hypothalamic function, such as hyperphagia, short stature, temperature instability, hypogonadotropic hypogonadism, and neurosecretory growth hormone deficiency. Patients with PWS are reported to have sleep-disordered breathing and have blunted hypercapnic ventilatory responses secondary to abnormal peripheral chemoreceptor function. Thus, we hypothesized that hypercapnic arousal responses would be abnormal in PWS. DESIGN:Hypercapnic arousal responses were tested in ten nonobese children and adults with PWS, aged 17.7 +/- 2.5 (SEM) years, 70% female, and nine control subjects, aged 14.2 +/- 2.6 years, 67% female. Hypercapnic challenges were performed during stage 3/4 non-rapid eye movement sleep. RESULTS: The PWS subjects had a significantly higher arousal threshold to hypercapnia compared with the controls (53 +/- 1.0 vs 46 +/- 1.7 mm Hg; p < 0.01). The PWS subjects had significantly higher baseline end-tidal CO2 levels (42 +/- 0.8 vs 38 +/- 1.1 mm Hg; p < 0.01) and more central apneas greater than 15 s/h of sleep (1.5 +/- 0.3 vs 0.1 +/- 0.1; p < 0.01). CONCLUSIONS: Elevated hypercapnic arousal thresholds during sleep are found in PWS subjects; these may be a manifestation of abnormal peripheral chemoreceptor function and may further contribute to sleep-disordered breathing in PWSpatients.
Authors: Brian E Cade; Han Chen; Adrienne M Stilp; Kevin J Gleason; Tamar Sofer; Sonia Ancoli-Israel; Raanan Arens; Graeme I Bell; Jennifer E Below; Andrew C Bjonnes; Sung Chun; Matthew P Conomos; Daniel S Evans; W Craig Johnson; Alexis C Frazier-Wood; Jacqueline M Lane; Emma K Larkin; Jose S Loredo; Wendy S Post; Alberto R Ramos; Ken Rice; Jerome I Rotter; Neomi A Shah; Katie L Stone; Kent D Taylor; Timothy A Thornton; Gregory J Tranah; Chaolong Wang; Phyllis C Zee; Craig L Hanis; Shamil R Sunyaev; Sanjay R Patel; Cathy C Laurie; Xiaofeng Zhu; Richa Saxena; Xihong Lin; Susan Redline Journal: Am J Respir Crit Care Med Date: 2016-10-01 Impact factor: 21.405
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