The effect of growth hormone on sleep‐related cardio‐respiratory control in Prader–Willi syndrome

Katz‐Salamon, Miriam; Lindgren, Ann Christin; Cohen, Gary

doi:10.1111/j.1651-2227.2012.02638.x

Cited by 29 publications

(18 citation statements)

References 28 publications

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“…In addition, children with PWS treated with hGH have demonstrated improvement in respiratory muscle strength response to carbon dioxide concentration, and nocturnal coupling of heart rate and blood pressure, all of which suggest improved cardiovascular and/or autonomic function [23■,24,25]. Thus, evidence indicates that cardiorespiratory complications from hGH do not develop in most PWS children receiving hGH, and that the observed cases of sudden death are more likely unrelated to hGH therapy [21].…”

Section: Introductionmentioning

confidence: 99%

Long-term effects of recombinant human growth hormone therapy in children with Prader–Willi syndrome

Wolfgram

Carrel

Allen

2013

Current Opinion in Pediatrics

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Purpose of review Recombinant human growth hormone (hGH) therapy in children with Prader–Willi syndrome (PWS) improves linear growth, body composition, physical strength and agility, and other metabolic parameters. These benefits must be weighed against potential adverse effects, including rare occurrences of sudden death. This review summarizes recent evidence important to a benefit–risk analysis of hGH use in children with PWS. Recent findings Studies consistently show that hGH improves stature, body composition, fat percentage and distribution, and other metabolic markers in children with PWS. Preliminary reports of improved cognitive development during hGH have also emerged. Scoliosis progression is influenced by growth rate, but frequency of occurrence and severity are not increased by hGH exposure. PWS genotype does not appear to affect response to hGH. Concerns about hGH-associated sudden death persist, but recent studies show either absence of change in sleep-disordered breathing or improved sleep cardiovascular function during hGH therapy. Summary Recent studies confirm and expand reported benefits of hGH therapy in children with PWS, including a possible salutary role in cognitive development. These findings support previous assertions that hGH can reduce morbidity and improve function in children with PWS, and suggest that potential risks of such treatment are favorably balanced by its benefits.

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Section: Introductionmentioning

confidence: 99%

Long-term effects of recombinant human growth hormone therapy in children with Prader–Willi syndrome

Wolfgram

Carrel

Allen

2013

Current Opinion in Pediatrics

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“…Animal studies have suggested that the posterior hypothalamus plays a role in the ventilatory response to CO 2 [16], so GH could also be exerting its effects at that level. During sleep, GH therapy in children with PWS leads to a modest improvement in SaO 2 during sleep but does not lead to significant changes in responsiveness to hyperoxia or hypercarbia, perhaps because the hypothalamus exerts less influence on respiratory drive during sleep [17]. …”

Section: Abnormalities Of Ventilatory Controlmentioning

confidence: 99%

Disorders of Sleep and Ventilatory Control in Prader-Willi Syndrome

Gillett

Perez

2016

Diseases

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Prader-Willi syndrome (PWS) is an imprinted genetic disorder conferred by loss of paternal gene expression from chromosome 15q11.2-q13. Individuals with PWS have impairments in ventilatory control and are predisposed toward sleep disordered breathing due to a combination of characteristic craniofacial features, obesity, hypotonia, and hypothalamic dysfunction. Children with PWS progress from failure to thrive during infancy to hyperphagia and morbid obesity during later childhood and onward. Similarly, the phenotype of sleep disordered breathing in PWS patients also evolves over time from predominantly central sleep apnea in infants to obstructive sleep apnea (OSA) in older children. Behavioral difficulties are common and may make establishing effective therapy with continuous positive airway pressure (CPAP) more challenging when OSA persists after adenotonsillectomy. Excessive daytime sleepiness (EDS) is also common in patients with PWS and may continue after OSA is effectively treated. We describe here the characteristic ventilatory control deficits, sleep disordered breathing, and excessive daytime sleepiness seen in individuals with PWS. We review respiratory issues that may contribute to sudden death events in PWS patients during sleep and wakefulness. We also discuss therapeutic options for treating sleep disordered breathing including adenotonsillectomy, weight loss, and CPAP. Lastly, we discuss the benefits and safety considerations related to growth hormone therapy.

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“…The authors concluded that rhGH improved arterial oxygenation and cardiovascular function during sleep. 91 In the February 2013 issue of The Journal of Pediatrics, Al-Saleh et al 92 presented the follow-up data of 15 children with PWS before and up to 2 years after starting rhGH. While two patients had to discontinue rhGH due to respiratory problems, the authors concluded that if there are no signs of SDB before and 6 weeks after the initiation of rhGH, the risk of developing SDB after that is small.…”

Section: Rhgh and Sleep Disordersmentioning

confidence: 99%

“…The effect of rhGH in young children with PWS was investigated in a prospective clinical case series study. 91 Sixteen patients with a median age of 16 months at enrollment were followed. The authors concluded that rhGH improved arterial oxygenation and cardiovascular function during sleep.…”

Section: Rhgh and 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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The effect of growth hormone on sleep‐related cardio‐respiratory control in Prader–Willi syndrome

Abstract: Growth hormone treatment improves arterial oxygenation and cardiovascular function during sleep; these changes are not owing to improved (stronger) chemoreflex-mediated autonomic drive.

Cited by 29 publications

References 28 publications

Long-term effects of recombinant human growth hormone therapy in children with Prader–Willi syndrome

Long-term effects of recombinant human growth hormone therapy in children with Prader–Willi syndrome

Disorders of Sleep and Ventilatory Control in Prader-Willi Syndrome

Endocrine management of children with Prader–Willi syndrome

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The effect of growth hormone on sleep‐related cardio‐respiratory control in Prader–Willi syndrome

Abstract: Growth hormone treatment improves arterial oxygenation and cardiovascular function during sleep; these changes are not owing to improved (stronger) chemoreflex-mediated autonomic drive.

Cited by 29 publications

References 28 publications

Long-term effects of recombinant human growth hormone therapy in children with Prader–Willi syndrome

Long-term effects of recombinant human growth hormone therapy in children with Prader–Willi syndrome

Disorders of Sleep and Ventilatory Control in Prader-Willi Syndrome

Endocrine management of children with Prader&ndash;Willi syndrome

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Product

Resources

About

Endocrine management of children with Prader–Willi syndrome