Sleep-Disordered Breathing in Prader-Willi Syndrome and its Association with Neurobehavioral Abnormalities

O’Donoghue, Fergal J; Camfferman, Danny; Kennedy, J. D.; Martin, A. Lynn; Couper, T; Lack, Leon; Lushington, Kurt; McEvoy, R. Doug

doi:10.1016/j.jpeds.2005.07.021

Cited by 67 publications

(53 citation statements)

References 30 publications

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“…1,4 High BMIs are also associated with higher serum triglyceride and fasting glucose levels, 5 unsatisfactory oral health status, 6 and more sleep disturbance. 7 Although the abdominal obesity of adults with intellectual disabilities has been studied less often, abdominal obesity assessed via waist circumference (WC) is associated with higher fasting plasma glucose and triglyceride levels, 5 and hypertension. 8 Elevated morbidity among adults with intellectual disabilities is also evident for those who are underweight.…”

Section: Artículo Originalmentioning

confidence: 99%

“…[5][6][7][8] However, the degree to which adults with intellectual disabilities in Latin America experience overweight/obesity has rarely been examined and rates of abdominal obesity have not been examined. With the exception of one previous study which described the BMI status of Latin American Special Olympics participants for the period 2003 -2009 as part of a larger international sample, 10 studies of BMI among adults with intellectual disability in Latin America have been generally small in sample size and derived from single country samples, specifically: Argentina, 11 Brazil, 6,12 and Mexico.…”

Section: Artículo Originalmentioning

confidence: 99%

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Body mass index and waist circumference of Latin American adult athletes with intellectual disability

et al. 2017

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Objectives. To examine both body mass index (BMI) status and waist circunference (WC) in a large international sample of adult Special Olympics participants from Latin America. It also explored the association of age and sex with obesity in this population. Materials and methods. BMI and WC records from a total of 4 174 (2 683 male and 1 491 female) participant records from the Special Olympics International Health Promotion database were examined. Results. The prevalence of overweight and obesity was quite high (i.e. > 40%), but generally lower than studies involving adults with intellectual disabilities from Europe and the USA. Chi-square analyses revealed that both increasing age and being female significantly predicted levels of overweight, obesity, and WC. Conclusions. These results suggest that efforts need to be made to prevent and reduce rates of overweight and obesity among Latin American Special Olympics participants, particularly women.

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Section: Artículo Originalmentioning

confidence: 99%

Section: Artículo Originalmentioning

confidence: 99%

Body mass index and waist circumference of Latin American adult athletes with intellectual disability

et al. 2017

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“…However, SRBD may disrupt sleep and induce ESD in PWS. In patients with PWS, OSA is associated with behavioral disturbances, such as autistic-related behavior and impulsiveness 13) . A recent study in young PWS infants found that OSAS was associated with lower mental development 14) .…”

Section: Sleep Related Breathing Disorders In Patients With Pwsmentioning

confidence: 99%

How to Understand Sleep and Sleep Problems in Patients with Prader-Willi Syndrome?

Joo

2015

Journal of mucopolysaccharidosis and rare disease

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Sleep problems occur frequently among patients with Prader-Willi syndrome (PWS). The most common problem is excessive daytime sleepiness (EDS) that are closely related to of sleep-related breathing disorder (SRBD) such as obstructive sleep apnea (OSA) and congenital hypoventilation syndrome. Obesity, craniofacial dysmorphism and muscular hypotonia of patients with PWS may increase the risk of SRBD. Sleep apneas can interrupt the continuity of sleep, and these disruptions result in a decrease in both the quality and quantity of sleep. In addition to SRBD, other sleep disorders have been reported, such as hypersomnia, a primary abnormality of the rapid eye movement (REM) sleep and narcolepsy traits at sleep onset REM sleep. Patients with PWS have intrinsic abnormalities of sleep-wake cycles due to hypothalamic dysfunction. The treatment of EDS and other sleep disorders in PWS are similar to standard treatments. Correction of sleep hygiene such as sufficient amount of sleep, maintenance of regular sleep-wake rhythm, and planned naps are important. After comprehensive evaluation of sleep disturbances, CPAP or surgery should be recommended for treatment of SRBD. Remaining EDS or narcolepsy-like syndrome are controlled by stimulant medication. Bright light therapy might be beneficial for disturbed circadian sleep-wake rhythm caused by hypothalamic dysfunction.

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“…82 Obstructive sleep apnea (OSA) is a primary disorder, and is considered a minor criterion for the diagnosis of PWS. 83 OSA may lead to cardiovascular complications, such as systemic arterial hypertension and cor pulmonale, with increased severity and a high impact on morbimortality.…”

Section: Sleep Disordersmentioning

confidence: 99%

“…12 In PWS patients, OSA may also be related to autistic behavior and impulsivity. 82 Excessive daytime sleepiness and rapid eye movement sleep abnormalities are common issues in PWS and can be considered primary disorders, although OSA may aggravate them. 12 DeMarcantonio et al 84 retrospectively evaluated the need for tonsillectomy in PWS patients with OSA.…”

Section: Sleep Disordersmentioning

confidence: 99%

Endocrine management of children with Prader–Willi syndrome

Medeiros¹,

Bordallo²,

Souza³

et al. 2013

PHMT

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Prader-Willi syndrome is a rare genetic condition afflicting nearly 1/15,000 live births. Clinical features include neonatal hypotonia, poor weight gain in early infancy followed by binge eating from childhood to adulthood, severe obesity, developmental delay, short stature, and hypogonadism of both central and peripheral etiology. Central hypothyroidism and adrenal insufficiency may occur. Sleep disordered breathing, by obstruction of upper airways associated with central hypoventilation, is a common feature. Most of these characteristics are assumed to be the result of a hypothalamic dysfunction. The most important complication and the most difficult to manage is the obesity. This review aims at discussing the most recent strategies to manage the endocrine complications of Prader-Willi syndrome patients, with a special approach on the treatment of obesity, hypogonadism, and short stature. We summarize the indication and effects of recombinant human growth hormone therapy on growth, cognitive development, and body composition, and discuss the effects of recombinant human growth hormone therapy on the resulting sleep disorders.

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Sleep-Disordered Breathing in Prader-Willi Syndrome and its Association with Neurobehavioral Abnormalities

Cited by 67 publications

References 30 publications

Body mass index and waist circumference of Latin American adult athletes with intellectual disability

Body mass index and waist circumference of Latin American adult athletes with intellectual disability

How to Understand Sleep and Sleep Problems in Patients with Prader-Willi Syndrome?

Endocrine management of children with Prader–Willi syndrome

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Sleep-Disordered Breathing in Prader-Willi Syndrome and its Association with Neurobehavioral Abnormalities

Cited by 67 publications

References 30 publications

Body mass index and waist circumference of Latin American adult athletes with intellectual disability

Body mass index and waist circumference of Latin American adult athletes with intellectual disability

How to Understand Sleep and Sleep Problems in Patients with Prader-Willi Syndrome?

Endocrine management of children with Prader&ndash;Willi syndrome

Contact Info

Product

Resources

About

Endocrine management of children with Prader–Willi syndrome