Nutritional intervention with hypocaloric diet for weight control in children and adolescents with Prader–Willi Syndrome

Prader–Willi syndrome (PWS) is a complex multisystem disorder due to the absent expression of the paternally active genes in the PWS critical region on chromosome 15 (15q11.2-q13). The syndrome is considered the most common genetic cause of obesity, occurring in 1:10,000–1:30,000 live births. Its main characteristics include neonatal hypotonia, poor feeding, and lack of appetite in infancy, followed by weight gain, lack of satiety, and uncontrolled appetite, frequently after the age of 2–3 years. The clinical picture includes short stature, multiple endocrine abnormalities (hypogonadism, growth hormone/insulin-like growth factor-I axis dysfunction, hypothyroidism, central adrenal insufficiency), dysmorphic features, scoliosis, osteoporosis, mental retardation, and behavioral and psychiatric problems. Subjects with PWS will become severely obese unless their food intake is strictly controlled. Constant and obsessive food seeking behavior can make life very difficult for both the family and caretakers. Prevention of obesity is mandatory in these patients from the first years of life, because once obesity develops it is difficult to maintain the control of food intake. In fact, PWS subjects die prematurely from complications conventionally related to obesity, including diabetes mellitus, metabolic syndrome, sleep apnea, respiratory insufficiency, and cardiovascular disease. The mechanisms underlying hyperphagia in PWS are not completely known, and to date no drugs have proven their efficacy in controlling appetite. Consequently, dietary restriction, physical activity, and behavior management are fundamental in the prevention and management of obesity in PWS. In spite of all available therapeutic tools, however, successful weight loss and maintenance are hardly accomplished. In this context, clinical trials with new drugs have been initiated in order to find new possibilities of a therapy for obesity in these patients. The preliminary results of these studies seem to be encouraging. On the other hand, until well-proven medical treatments are available, bariatric surgery can be taken into consideration, especially in PWS patients with life-threatening comorbidities.

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“…Many studies have been performed on diet restriction and physical activities in PWS, with different and sometimes conflicting results. 65 – 67 …”

Section: Current Standard Therapiesmentioning

confidence: 99%

Obesity management in Prader–Willi syndrome: current perspectives

Crinò¹,

Fintini²,

Bocchini³

et al. 2018

DMSO

106

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“…Relying on cognitive controls to resist food can be almost impossible for individuals with PWS ( 5 ), so physical restraints, such as locked food cabinets and refrigerator, are utilized in the homes of individuals with PWS. In past studies, individuals following low kcal diets had lower BMIs and lower body fat percentages ( 24 , 25 ). Restrictive diets, though, have been reported to result in shorter stature ( 26 ).…”

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confidence: 95%

“…In adults with PWS, hyperphagia may be due to an abnormality of the satiety response to food intake ( 21 , 22 ) or hormonal and neuroendocrine defects ( 23 ). Controlling food intake has contributed to the maintenance and prevention of weight gain and has been shown to improve anthropometric indices for individuals with PWS ( 24 ). Relying on cognitive controls to resist food can be almost impossible for individuals with PWS ( 5 ), so physical restraints, such as locked food cabinets and refrigerator, are utilized in the homes of individuals with PWS.…”

mentioning

confidence: 99%

The associations between diet and physical activity with body composition and walking a timed distance in adults with Prader–Willi syndrome

Woods

Knehans

Arnold

et al. 2018

Food & Nutrition Research

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BackgroundResearch on aging in Prader–Willi syndrome (PWS) is limited, although people with PWS are living longer. Individuals with PWS present with high fat mass, low lean mass, and low levels of physical activity (PA). Previous reports in children and young adults with PWS show inadequate nutrient intake and body fat percentage indicating obesity. Previous studies in PWS rarely included individuals beyond young adulthood, especially studies conducted in the United States. This study includes adults from 18 to 62 years of age, and includes 19 of the estimated 60 adult individuals with PWS in Oklahoma. Because individuals with PWS are living longer, information must be provided on aging with PWS. This study is a report of the initial data for a planned longitudinal study on aging with PWS.ObjectiveDetermine associations between body composition, diet, PA, and a timed walk for adults with PWS, and to assess adequacy of dietary intake for those individuals aging with PWS.DesignThis cross-sectional investigation determined dietary habits, PA, and body composition of adults with PWS, and tested associations between these variables.ResultsParticipants ranged in age from 18 to 62 years. They had healthier body composition, at 26.8% body fat, than previously reported. Mean body mass index (BMI) was in the overweight range at 26.7. Those who consumed higher amounts of fat (as a percent of total kilocalories) had statistically significant lower body fat percentage, but this may simply reflect that individuals with lower body fat percentages felt freer to consume fat. Mean steps taken per day was 7631.7 steps but only 16% of participants met healthy PA recommendations despite participating in daily structured exercise. All participants’ diets met Dietary Guidelines for macronutrient distribution, but 80% were deficient in calcium, 100% were deficient in dietary vitamin D, and 87% were deficient in fiber. Sample size was small, so it was difficult to reach statistical significance, despite seeing clinical significance.ConclusionsRecommend working toward healthy PA recommendations for all age groups by decreasing time in sedentary activity. Recommend increasing vitamin A and D fortified dairy products and high-fiber foods, and consider dietary supplementation, especially for calcium, vitamin D, and fiber.

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“…Current obesity treatments that focus on changes in feeding behavior/diet, exercise, or hormonal replacement therapy are ineffective for long-term weight loss maintenance in the majority of patients with PWS [ 58 ]. Although bariatric procedures, including RYGB, result in long-lasting weight reduction and remission of comorbidities in the majority of patients with obesity in the general population [ 59 ], it remains controversial whether or not to perform this invasive therapy in patients with PWS.…”

Section: Bariatric Surgery For the Treatment Of Syndromic Obesitymentioning

confidence: 99%

Bariatric Surgery for Monogenic Non-syndromic and Syndromic Obesity Disorders

et al. 2020

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Purpose of Review The global prevalence of obesity has increased rapidly over the last decades, posing a severe threat to human health. Currently, bariatric surgery is the most effective therapy for patients with morbid obesity. It is unknown whether this treatment is also suitable for patients with obesity due to a confirmed genetic defect (genetic obesity disorders). Therefore, this review aims to elucidate the role of bariatric surgery in the treatment of genetic obesity. Recent Findings In monogenic non-syndromic obesity, an underlying genetic defect seems to be the most important factor determining the efficacy of bariatric surgery. In syndromic obesity, bariatric surgery result data are scarce, and even though some promising follow-up results have been reported, caution is required as patients with more severe behavioral and developmental disorders might have poorer outcomes. Summary There is limited evidence in support of bariatric surgery as a treatment option for genetic obesity disorders; hence, no strong statements can be made regarding the efficacy and safety of these procedures for these patients. However, considering that patients with genetic obesity often present with life-threatening obesity-related comorbidities, we believe that bariatric surgery could be considered a last-resort treatment option in selected patients. Electronic supplementary material The online version of this article (10.1007/s11892-020-01327-7) contains supplementary material, which is available to authorized users.

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Nutritional intervention with hypocaloric diet for weight control in children and adolescents with Prader–Willi Syndrome

Cited by 17 publications

References 21 publications

Obesity management in Prader–Willi syndrome: current perspectives

Obesity management in Prader–Willi syndrome: current perspectives

The associations between diet and physical activity with body composition and walking a timed distance in adults with Prader–Willi syndrome

Bariatric Surgery for Monogenic Non-syndromic and Syndromic Obesity Disorders

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Nutritional intervention with hypocaloric diet for weight control in children and adolescents with Prader–Willi Syndrome

Cited by 17 publications

References 21 publications

Obesity management in Prader&ndash;Willi syndrome: current perspectives

Obesity management in Prader&ndash;Willi syndrome: current perspectives

The associations between diet and physical activity with body composition and walking a timed distance in adults with Prader–Willi syndrome

Bariatric Surgery for Monogenic Non-syndromic and Syndromic Obesity Disorders

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Product

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Obesity management in Prader–Willi syndrome: current perspectives

Obesity management in Prader–Willi syndrome: current perspectives