Prader-Willi Syndrome A Review for Pediatric Nutrition Professionals

Pereira, Rosanna; Schalk, A. F.; Geraghty, Maureen E.

doi:10.1177/1941406409348555

Cited by 8 publications

(12 citation statements)

References 24 publications

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“…The nutritional goal for infants with PWS is to promote proper growth without leading to overweight or obesity. To do this, the infants with PWS should be kept between the 50th and 75th percentile of his/her weight for height [98]. Of interest, the basal metabolic rate, the minimum energy required to maintain vital body functions [93], in infants with PWS is slower than an infant without PWS, suggesting that infants with PWS require fewer calories than the healthy counterparts.…”

Section: Nutritional Management Of Obesity In Pwsmentioning

confidence: 99%

“…Of interest, the basal metabolic rate, the minimum energy required to maintain vital body functions [93], in infants with PWS is slower than an infant without PWS, suggesting that infants with PWS require fewer calories than the healthy counterparts. In preschool and school-age children with PWS the guidelines recommend from 10 to 11 cal per centimeter of length to keep the weight and 8 to 9 cal per centimeter to reduce body weight [98]. For young children, the guidelines recommend from 600 to 800 cal per day while for older children and adults from 800 to 1100 cal per day [19].…”

Section: Nutritional Management Of Obesity In Pwsmentioning

confidence: 99%

“…For young children, the guidelines recommend from 600 to 800 cal per day while for older children and adults from 800 to 1100 cal per day [19]. In addition, it is important in subjects with PWS to take a multivitamin, and above all take a vitamin D supplement if necessary, to compensate for the reduced intake of vitamins and minerals that may be suboptimal due to a restrictive diet [10,98]. When subjects with PWS develop obesity in adulthood, body weight should be assessed frequently and the total energy intake adjusted accordingly.…”

Section: Nutritional Management Of Obesity In Pwsmentioning

confidence: 99%

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Obesity in Prader–Willi syndrome: physiopathological mechanisms, nutritional and pharmacological approaches

Muscogiuri

Barrea

Faggiano

et al. 2021

J Endocrinol Invest

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Prader–Willi syndrome (PWS) is a genetic disorder caused by the lack of expression of genes on the paternally inherited chromosome 15q11.2-q13 region. The three main genetic subtypes are represented by paternal 15q11-q13 deletion, maternal uniparental disomy 15, and imprinting defect. Clinical picture of PWS changes across life stages. The main clinical characteristics are represented by short stature, developmental delay, cognitive disability and behavioral diseases. Hypotonia and poor suck resulting in failure to thrive are typical of infancy. As the subjects with PWS age, clinical manifestations such as hyperphagia, temperature instability, high pain threshold, hypersomnia and multiple endocrine abnormalities including growth hormone and thyroid-stimulating hormone deficiencies, hypogonadism and central adrenal insufficiency due to hypothalamic dysfunction occur. Obesity and its complications are the most common causes of morbidity and mortality in PWS. Several mechanisms for the aetiology of obesity in PWS have been hypothesized, which include aberration in hypothalamic pathways of satiety control resulting in hyperphagia, disruption in hormones regulating appetite and satiety and reduced energy expenditure. However, despite the advancement in the research field of the genetic basis of obesity in PWS, there are contradictory data on the management. Although it is mandatory to adopt obesity strategy prevention from infancy, there is promising evidence regarding the management of obesity in adulthood with current obesity drugs along with lifestyle interventions, although the data are limited. Therefore, the current manuscript provides a review of the current evidence on obesity and PWS, covering physiopathological aspects, obesity-related complications and conservative management.

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Section: Nutritional Management Of Obesity In Pwsmentioning

confidence: 99%

Section: Nutritional Management Of Obesity In Pwsmentioning

confidence: 99%

Section: Nutritional Management Of Obesity In Pwsmentioning

confidence: 99%

See 1 more Smart Citation

Obesity in Prader–Willi syndrome: physiopathological mechanisms, nutritional and pharmacological approaches

Muscogiuri

Barrea

Faggiano

et al. 2021

J Endocrinol Invest

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“…Typical daily caloric intakes range from 800 to 1,500 kcal, with the higher amount of daily calories offered to those who maintain a BMI below 23 kg/m 2 . Individual caloric requirements are calculated according to height as recommended by current guidelines, namely 7–8 kcal/cm for weight loss and 10–14 kcal/cm for weight maintenance (Hoffman et al, 1992; Miller & Tan, 2020; Pereira et al, 2009). Typical diet recommendations include 40%–45% carbohydrates, 25%–30% protein, and 30% fats.…”

Section: Study Participants and Methodsmentioning

confidence: 99%

Long‐term weight control in adults with Prader‐Willi syndrome living in residential hostels

Hirsch

Benarroch

Genstil

et al. 2021

American J of Med Genetics Pt A

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Hyperphagia leading to severe obesity with increased morbidity and mortality is the major manifestation of Prader‐Willi syndrome. Caring for these individuals in a home environment is challenging and stressful for caregivers and families. Residential hostels specifically for PWS adults offer programs of diet, exercise, and vocational opportunities, but long‐term effects of PWS hostel living have not been reported. We studied long‐term changes in body mass index (BMI) for PWS adults living in residential hostels compared with age‐matched controls living with families at home. The study included all 34 individuals (18 men) aged >17 years with genetically confirmed PWS living in residential hostels. BMI was recorded at the time of yearly clinic visits and compared to 23 PWS adults (10 men) living at home. BMI on entering the hostel was 36.3 ± 11.0 kg/m2 and decreased to 27.0 ± 5.6 kg/m2 (p < 0.001) after 6.9 ± 3.9 years. For 21 residents, a slight rise of BMI to 28.8 kg/m2 was observed 5.1 ± 2.5 years after the lowest value was achieved. BMI of 23 PWS adults at home was 36.8 ± 12.7 kg/m2 versus 27.9 ± 7.1 kg/m2 for hostel residents in the same age range (p = 0.008). From 2008 to 2019, there were five deaths among PWS individuals aged 18–40 years living at home, compared with one death (a 43‐year‐old man) among hostel residents. Adults with PWS living in hostels lose weight, maintain BMI values in a normal to mildly overweight range, and have lower mortality in contrast to individuals in a family home environment.

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“…Commonly used medications may also be associated with negative side effects that translate to an increased risk for lifestyle disease (e.g., weight gain secondary to the use of anti-depressants) in people with ID [21]. Specific conditions such as Prader Willi syndrome alter appetite and increase the risk for weight gain and obesity [22][23][24]. Conversely, a reduced capacity to chew and swallow may make eating more difficult and require the texture of foods to be modified.…”

Section: Introductionmentioning

confidence: 99%

Observed Dietary Intake in Adults with Intellectual Disability Living in Group Homes

2019

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Background: There is limited information on the dietary intakes of people with intellectual disability (ID) living in group homes. Objective: To describe and evaluate dietary intake in people with ID. Method: Dietary intake was assessed in a convenience sample of people with ID living in group homes. Dietary assessment used three-day weighed food records and digital food photography. Intakes were compared to the Nutrient Reference Values (NRVs) and dietary recommendations. Results: A sample of 33 adults, (men (M): n = 14; women (W): n = 19), mean age 51 ± 14 years, was recruited from seven group homes. Mean daily energy intake was low (M: 7.4 MJ; W: 7.0 MJ; p = 0.46), similar to levels recommended for bed rest. Many participants had intakes below the estimated average requirements (EARs) for the nutrients, magnesium (M: 86%; W: 63%), calcium (M: 43%; W: 78%), iodine (M: 43%; W: 47%) and zinc (M: 43%). Less than half of the recommended daily servings were consumed for vegetables (men and women) and dairy foods (women). Conclusion: Nutrient intake and diet quality of the participants in the group homes studied was poor. Education and policy to support healthier diets is required to improve dietary intake of people with intellectual disability, living in group homes.

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Prader-Willi Syndrome A Review for Pediatric Nutrition Professionals

Cited by 8 publications

References 24 publications

Obesity in Prader–Willi syndrome: physiopathological mechanisms, nutritional and pharmacological approaches

Obesity in Prader–Willi syndrome: physiopathological mechanisms, nutritional and pharmacological approaches

Long‐term weight control in adults with Prader‐Willi syndrome living in residential hostels

Observed Dietary Intake in Adults with Intellectual Disability Living in Group Homes

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