Metabolic responses to walking in children with Prader‐Willi syndrome on growth hormone replacement therapy

Hyde, Adam M.; Chavoya, Frank A.; Silveira, Fabiano Vanroo; Beam, William; Rubin, Daniela A.

doi:10.1002/ajmg.a.40509

Cited by 3 publications

(5 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A total of 483 studies were identified, of which 447 did not meet the inclusion criteria. Twenty‐two studies, whose details are summarized in Tables and , were finally included and evaluated (Figure ). The retrieved articles included a total of 766 subjects (356 patients with PWS [242 children and/or adolescents and 114 adults]).…”

Section: Resultsmentioning

confidence: 99%

“…A total of 483 studies were identified, of which 447 did not meet the inclusion criteria. Twenty-two studies, 10,15,16,[21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39] whose details are summarized in Tables 2 and 3 Mullins (1987)…”

Section: Participants' Characteristicsmentioning

confidence: 99%

“…Ten studies 10,15,16,[22][23][24][25] reported the acute effects (either physiological response or physical performance) of exercise in patients with PWS using the following types of exercise tests: cycle ergometer, 16,22,23 lower-body strength, 10,24,33 treadmill along with upper-body strength and flexibility, 15 only treadmill, 25,34 or the Bruininks-Oseretsky test to analyse motor proficiency. 35 Eight out of ten studies were carried out in children and/or adolescents, 16,[22][23][24][25] with the remainder of studies conducted in adults.…”

Section: Acute Response To Exercisementioning

confidence: 99%

See 2 more Smart Citations

Physical exercise and Prader‐Willi syndrome: A systematic review

Morales

Valenzuela²,

Pareja‐Galeano

et al. 2019

Clinical Endocrinology

Self Cite

View full text Add to dashboard Cite

Summary Objective The aim of this systematic review was to summarize evidence on the acute responses of individuals with Prader‐Willi syndrome (PWS) to physical exercise, and on the effectiveness of long‐term exercise interventions to improve the clinical manifestations of this syndrome. Design/methods Relevant articles were identified in the electronic databases PubMed, Medline, CINAHL and SPORTDiscus (from inception to December 2018). Twenty‐two studies including a total of 356 patients with PWS met all inclusion criteria and were included in the review. Results Patients with PWS present with a decreased physical performance and impaired cardiorespiratory (maximal oxygen consumption, heart rate recovery after exercise) and hormonal (growth hormone release) responses to exercise. Most long‐term exercise interventions have proven to decrease body mass while improving physical performance. Some benefits have also been reported in biochemical (glucose homeostasis, lipid profile) and biomechanical (gait pattern) variables, although there is controversy regarding the effects on body composition. No exercise‐related adverse events have been reported in patients with PWS. Conclusion Physical exercise seems to be safe and effective for improving several phenotypes in PWS, notably physical fitness. However, further research is needed to confirm these results and especially to corroborate whether exercise per se or combined with dietary intervention is an effective coadjuvant treatment for reducing body mass in these patients.

show abstract

Section: Resultsmentioning

confidence: 99%

Section: Participants' Characteristicsmentioning

confidence: 99%

Section: Acute Response To Exercisementioning

confidence: 99%

See 1 more Smart Citation

Physical exercise and Prader‐Willi syndrome: A systematic review

Morales

Valenzuela²,

Pareja‐Galeano

et al. 2019

Clinical Endocrinology

Self Cite

View full text Add to dashboard Cite

show abstract

“…As for the results of presented clinical trials, most of them show positive results of GH-replacement therapy in PWS patients, not only in case of better motor skills or body composition [21,24,25,26], but also concerning their social and adaptive functioning [22,23]. As for adaptive functioning development, it was stated that only the long-term therapy has its significant effects (>2 years).…”

Section: Discussionmentioning

confidence: 99%

Effects of growth hormone-replacement therapy in patients with Prader-Willi syndrome - review of recent clinical trials

Piwoński

Żak

Kozłowska

et al. 2020

J Educ Health Sport

View full text Add to dashboard Cite

Introduction and objective: Prader-Willi syndrome (PWS) is a severe genetic disorder being manifested by several symptoms such as infantile hypotonia and poor thriving outcomes, low height, hyperphagia, endocrine, reproductive and internal malfunctions and malformations. In the disease treatment, growth hormone (GH)-replacement therapy is commonly carried on. In this article, we sum up the latest clinical trials' results concerning the effectiveness and safety of GH-replacement therapy. Abbreviated description of the state of knowledge: Taking into consideration six clinical trials performed in the years 2019-2020, most of them showed positive results of GHreplacement therapy in PWS patients, not only in case of better motor skills or body composition, but also concerning their social and adaptive functioning. Moreover, as for the cessation of GH treatment, some authors seem to highlight no deterioration in cognitive functioning in patients who attained adult height after GH-treatment. Summary: GH-replacement therapy seems to be a good therapeutic option for PWS-affected individuals. Nonetheless, in our research we did not come across on any clinical trials with more than one-year-long observations. Thus, on the long term, these results can not exclude a gradual deterioration of cognitive functioning.

show abstract

“…As with any consistent exercise intervention, these individuals experience biochemical (glucose homeostasis, lipid profile) and biomechanical (gait pattern) improvement. 16,17 Including resistance training as part of the regular exercise routine increases muscle strength, thereby, hopefully increasing their energy expenditure over time. 18 All of this information shows that the first tenant of weight control in adolescents with PWS has to be increasing physical exercise and movement.…”

mentioning

confidence: 99%

<p>Dietary Management for Adolescents with Prader–Willi Syndrome</p>

Miller

Tan

2020

AHMT

View full text Add to dashboard Cite

Prader-Willi syndrome (PWS) is a complex, multisystem neurodevelopmental disorder affecting approximately 1 in 25,000 live births. PWS is caused by absence of expression of paternally inherited imprinted genes on chromosome 15q11-q13. The syndrome typically occurs due to one of three genetic mechanisms: paternal deletion of involved genes, maternal uniparental disomy, or imprinting center defects. These genetic anomalies lead to well-described clinical phenotype that includes hypotonia, hypothalamic dysfunction, social and behavioral issues, life-threatening hyperphagia, and elevated probability of obesity. Adolescents with PWS are at the highest risk for development of life-threatening obesity due to increased access to food, decreased physical activity, and hyperphagia. Currently, the only treatment for the hyperphagia is environmental control, including locked kitchens and continuous supervision of the affected individual. Caloric intake must be restricted to prevent obesity, which subsequently increases the hunger drive even more. Research and clinical practice have demonstrated that increasing physical activity along with insuring a wellbalanced, nutritionally dense diet can improve overall weight control in adolescents with PWS.

show abstract

Metabolic responses to walking in children with Prader‐Willi syndrome on growth hormone replacement therapy

Abstract: This is the author manuscript accepted for publication and has undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as

Cited by 3 publications

References 18 publications

Physical exercise and Prader‐Willi syndrome: A systematic review

Physical exercise and Prader‐Willi syndrome: A systematic review

Effects of growth hormone-replacement therapy in patients with Prader-Willi syndrome - review of recent clinical trials

<p>Dietary Management for Adolescents with Prader–Willi Syndrome</p>

Contact Info

Product

Resources

About