Metabolic syndrome in adult patients with Prader–Willi syndrome

Grugni, Graziano; Crinò, Antonino; Bedogni, Giorgio; Cappa, Marco; Sartório, Alessandro; Corrias, Andrea; Candia, Stefania Di; Gargantini, L; Pagano, Claudio; Ragusa, Letizia; Salvatoni, Alessandro; Spera, S.; Vettor, Roberto; Chiumello, G; Brambilla, Paola

doi:10.1016/j.numecd.2012.11.006

Cited by 47 publications

(54 citation statements)

References 33 publications

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“…Unlike in obesity, visceral fat deposits are reduced, and insulin sensitivity is higher. 6,7 In spite of these metabolic benefits, PWS patients often die at an early age because of complications usually linked to obesity, which include respiratory problems, heart disease, and type 2 diabetes mellitus (T2DM). 8,9 Choking (mainly during bouts of heavy eating) and stomach rupture or necrosis of tissue have also been described.…”

Section: Clinical Problems Managementmentioning

confidence: 99%

Prader–Willi syndrome: clinical problems in transition from pediatric to adult care

Crinó

Fintini²,

Bocchini³

et al. 2016

RRED

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Prader-Willi syndrome (PWS) represents the most common form of genetic obesity. Thanks to the advances in medical care and technology, many persons with PWS live longer and survive to adulthood. Currently, because of the many physical and behavioral manifestations, transitional health care is not easy for these patients and is considered a very important issue. Moreover, very few studies have examined these transitional problems in young adults with PWS. In recent years, there has been great interest in improving transition planning and support for young people with PWS reaching adulthood. In this article, we underline the main clinical problems in transition and give some advice to make this period less difficult and easier for adolescents with PWS. Special attention should be paid to obesity, diabetes mellitus, hypertension, osteoporosis, and sleep apnea during the period of transition. In PWS, for an effective transition from childhood to adulthood, a multidisciplinary team is needed, and should maintain the same approach to food, environment, and psychiatric issues. For comprehensive care, it is necessary to involve adult endocrinologists and other medical specialists in conjunction with the pediatric team. Parental involvement is, however, a great help for supervising adolescents with PWS during this particular period.

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Section: Clinical Problems Managementmentioning

confidence: 99%

Prader–Willi syndrome: clinical problems in transition from pediatric to adult care

Crinó

Fintini²,

Bocchini³

et al. 2016

RRED

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“…However, when the presence of obesity was excluded from the analysis and the frequency of ≥2 altered parameters was considered, a significantly lower frequency in the clustering of metabolic risk factors was still present in the non-obese PWS group as compared to both obese groups, thus confirming the main role played by obesity status in this field. Grugni et al 8) reported Mets frequency evaluated in a large cohort of PWS adults (87 obese PWS and 85 matched obese control and 21 non-obese PWS). Mets was found in 1/21 (4.8%) non-obese PWS, 36/87 (41.4%) obese PWS, and 39/85 (45.9%) obese controls.…”

Section: Metabolic Syndrome In Prader-willi Syndromementioning

confidence: 99%

“…However, obesity remains a critical problem in PWS teenagers and adults because it leads to severe complications, such as sleep disorder, cardiac or respiratory failure, and physical disability 6) . Obesity status also plays on a crucial role in development of metabolic syndrome (Mets) in PWS children and adults [7][8][9][10] . Several studies focused on the metabolic outcomes of PWS adults.…”

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confidence: 99%

“…PWS patients had a better metabolic profile, such as lower insulin resistance and healthier lipid profile compared with matched primary obese subjects 11) . However, Grugni et al 8) reported that when matched for body mass index (BMI), PWS adults had the same prevalence of metabolic syndrome (41.4%) and insulin resistance index as obese controls. In this context, we review for the obesity and metabolic syndrome of PWS adults.…”

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confidence: 99%

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Obesity and Metabolic Syndrome in Adults with Prader-Willi Syndrome

Kim¹

2015

Journal of mucopolysaccharidosis and rare disease

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“…16,17 However, type 2 diabetes (25%), hypertension and cardiovascular disease (38%) are described in adults with PWS. 18 Grugni et al 19 compared the frequency of metabolic syndrome components in 108 adult patients with PWS (87 obese patients and 21 nonobese patients) with 85 obese nonsyndromic patients. The study revealed that obese patients with PWS have a higher frequency of hyperglycemia and elevated systolic blood pressure when compared with obese controls.…”

Section: Obesitymentioning

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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Metabolic syndrome in adult patients with Prader–Willi syndrome

Cited by 47 publications

References 33 publications

Prader–Willi syndrome: clinical problems in transition from pediatric to adult care

Prader–Willi syndrome: clinical problems in transition from pediatric to adult care

Obesity and Metabolic Syndrome in Adults with Prader-Willi Syndrome

Endocrine management of children with Prader–Willi syndrome

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Metabolic syndrome in adult patients with Prader–Willi syndrome

Cited by 47 publications

References 33 publications

Prader&ndash;Willi syndrome: clinical problems in transition from pediatric to adult care

Prader&ndash;Willi syndrome: clinical problems in transition from pediatric to adult care

Obesity and Metabolic Syndrome in Adults with Prader-Willi Syndrome

Endocrine management of children with Prader&ndash;Willi syndrome

Contact Info

Product

Resources

About

Prader–Willi syndrome: clinical problems in transition from pediatric to adult care

Prader–Willi syndrome: clinical problems in transition from pediatric to adult care

Endocrine management of children with Prader–Willi syndrome