Abstract
Few short-term studies of weight loss have been performed in adult patients with Prader–Willi syndrome (PWS) undergoing metabolic rehabilitation. We performed a retrospective cohort study of 45 adult obese PWS patients undergoing a long-term multidisciplinary metabolic rehabilitation program based on diet and physical activity. Body composition was evaluated by dual-energy X-ray absorptiometry in 36 (80%) patients. The mean (95% CI) weight change was −3.6 (−7.6 to 0.4, p = 0.08) kg at 3 years and −4.6 (−8.5 to −0.8, p = 0.02) kg at 6 years, and that of BMI was −1.7 (−3.4 to 0.1, p = 0.06) kg/m2 at 3 years and −2.1 (−3.8 to −0.4, p = 0.02) kg/m2 at 6 years. A decrease of about 2% in fat mass per unit of body mass was observed, which is in line with the expectations for moderate weight loss. A possibly clinically relevant decrease in total and low-density lipoprotein cholesterol was also observed. These long-term results are important for patients with PWS, which is characterized by severe hyperphagia, behavioral disturbances, and cognitive impairment and is generally considered “resistant” to classical weight loss interventions.
Highlights
Prader–Willi syndrome (PWS) is a multisystemic disorder caused by lack of expression of genes on the paternally inherited chromosome 15q11.2-q13 region [1]
The resting energy expenditure (REE) of subjects with PWS is lower, even if it is similar to that of subjects with simple obesity when it is standardized on fat-free mass (FFM) [12,13]
At 6 years, we found that the average weight loss was slightly less than 5% and that the average body mass index (BMI) loss (−2.1 kg/m2) was similar to that reported by Grolla et al as an average of repeated measures for a median follow-up time of 0.5 years [17]
Summary
Prader–Willi syndrome (PWS) is a multisystemic disorder caused by lack of expression of genes on the paternally inherited chromosome 15q11.2-q13 region [1]. PWS is characterized by hyperphagia and childhood-onset morbid obesity [5], which is likely to contribute substantially to the high mortality rate associated with the disorder [6]. Obesity associated with PWS is the most common syndromic obesity and is characterized by a peculiar body composition [8,9,10,11]. Subjects with PWS have, an expanded fat mass (FM) and a lower fat-free mass (FFM) when compared to age- and sex-matched obese subjects without PWS. The resting energy expenditure (REE) of subjects with PWS is lower, even if it is similar to that of subjects with simple obesity when it is standardized on FFM [12,13]. For rehabilitation programs based on lifestyle changes, the total energy expenditure (TEE) of subjects with PWS is usually decreased because of reduced activity energy expenditure [14]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
