Body composition in Prader-Willi syndrome compared with nonsyndromal obesity: Relationship to physical activity and growth hormone function

Mil, G.A.H. van; Westerterp, Klaas R.; Gerver, Willem‐Jan M.; Lichtenbelt, Wouter D. van Marken; Kester, A.D.M.; Saris, W.H.M.

doi:10.1067/mpd.2001.118399

Cited by 54 publications

(37 citation statements)

References 45 publications

(41 reference statements)

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“…In contrast, a previous study in GH naïve children and adults with PWS demonstrated a higher fat mass and percentage compared to obese controls [10]. In addition, less lean mass in individuals with PWS has been demonstrated in early stages of development with this characteristic persisting into adulthood [6,10,11]. The present study shows lower total body and legs lean mass in PWS compared to obese children but similar to lean controls.…”

Section: Discussioncontrasting

confidence: 54%

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Update on Body Composition and Bone Density in Children with Prader-Willi Syndrome

et al. 2013

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Aim: To compare body composition in children with Prader-Willi syndrome (PWS) not naïve to growth hormone (GH) with obese and lean controls. Methods: Participants included 12 children with PWS, 12 children with obesity (body fat percentage >95th percentile for age and sex) and 12 lean children (body fat percentage <85th percentile for age and sex) matched by age and height. Fat mass, lean mass, bone mineral content (BMC), bone mineral density (BMD) and BMD z-score for total body, hips and lumbar spine were obtained through dual X-ray absorptiometry. Results: PWS had higher fat percentage in the legs (p = 0.04) but similar leg fat mass (p = 1.00) compared to obese. PWS exhibited lower lean mass in the body (p = 0.04) and legs (p = 0.02) than obese, but similar to lean (p = 1.00 and p = 0.89, respectively). PWS had lower hip BMC (p < 0.01), BMD (p < 0.01) and BMD z-score (p < 0.01) compared to obese but similar to lean. No other differences were found between PWS and obese (p > 0.05 for all). Conclusions: Children with PWS not naïve to GH present differences in fat and lean mass distribution compared to obese controls. BMC and BMD appear unaffected by PWS, except at the hips.

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Section: Discussioncontrasting

confidence: 54%

“…Factors such as lack of GH, hypogonadism and reduced spontaneous activity have been associated with this unique body composition [6,7,8,10]. A trend towards low BMD has also been suggested in children with PWS [11]. …”

Section: Introductionmentioning

confidence: 99%

Update on Body Composition and Bone Density in Children with Prader-Willi Syndrome

et al. 2013

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“…PWS patients have a significantly higher percentage of body fat than obese subjects in the arms and legs but not in the trunk (18,19). Therefore, the ratio of lean mass in the trunk to that in the limbs is significantly higher in PWS patients than in obese and normal-weight subjects (20).…”

Section: Discussionmentioning

confidence: 91%

Increased Density of Ghrelin-Expressing Cells in the Gastric Fundus and Body in Prader-Willi Syndrome

Choe¹,

Song²,

Paik³

et al. 2005

The Journal of Clinical Endocrinology & Metabolism

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Context:The levels of ghrelin, an orexigenic hormone secreted by oxyntic cells in the digestive tract, are elevated in Prader-Willi syndrome (PWS) and GH deficiency (GHD) patients. In this study, we hypothesized that the hyperghrelinemia observed in PWS is related to IGF-I or GH/IGF axis deficiency. Design:We investigated the densities of ghrelin-expressing cells (GECs), the amounts of ghrelin in gastric tissues, and ghrelin levels in plasma in 16 PWS patients and compared these results with those of 13 GHD patients and comparison groups (19 normal lean and 10 normal obese subjects). Results:In the gastric body and fundus, 2-to 3-fold increases in the numbers of GECs (P Ͻ 0.001) and in the amounts of ghrelin (P Ͻ 0.018) were noted in PWS patients vs. comparison groups, whereas GEC numbers in GHD patients were similar to those of the comparison group despite elevated fasting plasma ghrelin levels. In addition, IGF-I SD scores in PWS were not found to be correlated with GEC densities, the amounts of ghrelin expressed in gastric tissues, or plasma ghrelin levels. Conclusions:Our results suggest that IGF-I or GH/IGF axis deficiency appears to be unrelated to observed GEC increases in the stomach of patients with PWS. G HRELIN IS AN acylated peptide that is secreted byA-like endocrine cells of the oxyntic mucosa of the stomach and induces the secretion of GH (1, 2). Removing the acid-producing portion of the stomach was found previously to reduce circulating ghrelin by 80%, supporting the notion that the oxyntic mucosa is the major source of circulating ghrelin (3). Ghrelin can induce acute hunger, increase food intake, and reduce fat utilization in rodents and humans (1, 4). However, the direct orexigenic effect of ghrelin in its physiologic concentration range has not been elucidated in rodents or humans.Prader-Willi syndrome (PWS) is the most common form of human syndromic obesity. Ghrelin levels have been reported to be elevated in PWS (5-7). Moreover, unlike normal obese individuals, who have low fasting-plasma ghrelin concentrations, PWS patients can have 3-to 4-fold higher ghrelin fasting concentrations (5-7). Elevated plasma ghrelin levels in PWS may be related to low plasma insulin levels, because insulin plays an important role in the suppression of plasma ghrelin (8 -10).Recently, plasma ghrelin levels were reported to be elevated in patients with GH deficiency (GHD) (11,12). Because some PWS patients show attenuated plasma GH levels, and because total and free IGF-I have been reported to be low in PWS compared with obese individuals, partial GHD may also contribute to the hyperghrelinemia in PWS.In the present study, we hypothesized that the hyperghrelinemia observed in PWS is related to IGF-I or GH/IGF axis deficiency. We investigated plasma ghrelin levels, ghrelin-expressing cell (GEC) densities, and ghrelin quantities in the gastric tissues of PWS and GHD patients and in those of lean and obese normal subjects and correlated these results with total IGF-I levels. We found that GEC densities and ghreli...

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“…Parents and caregivers of people with PWS are routinely faced with two types of problems; the control of access to food to avoid and/or minimize other health problems (such as obesity, scoliosis, and cardiovascular and endocrine illnesses) 12 , and family burden, especially on the primary caregiver, due to the difficulties in the management of behavioral problems that start from early childhood 13,14,15 . Although scientific evidence has shown the neurobiological basis of the behavioral changes and patterns of hyperphagia, interventions which involve giving guidance to parents and caregivers in respect of parenting practices and the management of the children's behavioral problems are considered to be a priority; previous studies report that these interventions can not only improve a range of behavioral indicators in the children but also help support the caregiver in relation to the emotional difficulties they face [16][17][18][19][20][21] . Parents and/or caregivers in general are the main agents of change in any type of behavioral management of their children 22 .…”

Section: Introductionmentioning

confidence: 99%

Treino parental para manejo comportamental de crianças com Síndrome de Prader-Willi: impacto sobre a saúde mental e práticas educativas do cuidador

et al. 2016

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RESUMO Objetivo: verificamos os indicadores de impacto na saúde mental de cuidadores de um programa de treinamento parental para manejo de crianças e adolescentes com Síndrome de Prader-Willi. Métodos: a amostra foi composta por 5 mães de crianças/adolescentes com Síndrome de Prader-Willi entre 6 a 18 anos. Os instrumentos de coleta de dados foram: a) Questionário para verificação de conhecimentos das mães sobre a síndrome, b) Inventário de Auto-avaliação para Adultos de 18 a 59 anos, c) Inventário de Estilos Parentais/Práticas educativas maternas e paternas; d) Levantamento de dificuldades e preocupações sobre os filhos. O estudo foi realizado em quatro fases: pré-intervenção, intervenção, pós-intervenção e seguimento. Resultados: as mães passaram a adotar práticas parentais educativas baseadas na monitoria positiva, diminuíram as práticas de risco. Conclusão: houve melhora em indicadores de dificuldades emocionais das mães, apreenderam a identificar e manejar fatores que prejudicavam a saúde física, o comportamento e a aprendizagem escolar dos filhos e a necessidade do envolvimento de outros familiares no cuidado do filho.

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Body composition in Prader-Willi syndrome compared with nonsyndromal obesity: Relationship to physical activity and growth hormone function

Cited by 54 publications

References 45 publications

Update on Body Composition and Bone Density in Children with Prader-Willi Syndrome

Update on Body Composition and Bone Density in Children with Prader-Willi Syndrome

Increased Density of Ghrelin-Expressing Cells in the Gastric Fundus and Body in Prader-Willi Syndrome

Treino parental para manejo comportamental de crianças com Síndrome de Prader-Willi: impacto sobre a saúde mental e práticas educativas do cuidador

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