GH therapy in pediatric patients with Prader-Willi syndrome (PWS) improves body composition, but discontinuation of GH after achieving adult height has been implicated in its deterioration. Although there is evidence for the deleterious effects of visceral adipose tissue (VAT) rather than subcutaneous adipose tissue (SAT) on the development of obesity-related complications, the effects of GH discontinuation on fat distribution in adults with PWS has not been fully investigated. Therefore, we utilized dual-energy X-ray absorptiometry (DEXA) and abdominal computed tomography (CT) to compare the fat distribution between before and 6 months or 12 months after the cessation of GH therapy in 7 adult PWS patients. GH therapy was initiated at a mean age of 4.1 ± 1.4 years and discontinued at a mean age of 18.9 ± 1.8 years. Serum IGF-1 levels were decreased by discontinuation of GH therapy. Fat mass was significantly increased 6 and 12 months after GH cessation, whereas muscle mass and bone mineral density were unchanged during both study periods. Abdominal CT analysis revealed that elevations in fat mass were due to increases in VAT rather than SAT. Circulating low-density lipoprotein (LDL) cholesterol levels were significantly elevated 6 months after GH cessation. In conclusion, discontinuation of GH therapy caused rapid increases in visceral adipose tissue and LDL cholesterol levels. These findings indicate that continuation of GH therapy may be a therapeutic option to maintain body composition; however, further studies regarding the long-term benefits and adverse effects of GH therapy in adults with PWS are required.
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26Bravata DM, Sanders L, Huang J, Krumholz HM, Olkin I, Gardner CD, Bravata DM Efficacy and safety of low-carbohydrate diets:a systematic review. JAMA 289:-. -.
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Rapid CommunicationDetermination of Total Energy Expenditure Using the Doubly labeled Water Method and Expired Gas Analysis in Short-stature Children Yukiko Nishimoto, , , Shinobu Ida, Yuri Etani, Naoyuki Ebine, Satoshi Nakae, and Shuichi Miyatani Received January , ; Accepted April , Summary: It is unclear which dietary intake standard should be used for children with abnormal body size. To assess energy expenditure in short-stature children who have no underlying disorders, we measured total energy expenditure (TEE), resting energy expenditure (REE) and respiratory quotient (RQ) using the doubly labeled water method and expired gas analysis in children with short stature ( males, females: age . . years), and calculated their physical activity levels (PAL). The measured values were then compared with estimated values based on subjects age-or height-adjusted age-matched references for basal metabolic rates. We also performed a food intake survey and calculated the percentage energy intake for the three major nutritional components. The measured TEE and REE in the short-stature children were significantly higher than those estimated from the age-matched references, but did not differ from the height-adjusted age-matched estimates. PAL obtained from the measured TEE and REE also did not differ from the estimated values based on either actual age-or height-adjusted age-matched references. Percentage energy intake from carbohydrate among total energy intake was lower than shown in the Dietary Reference Intakes for Japanese. These data suggest that metabolism is enhanced in short-stature children. In addition to possible delayed development and/or genetic mutation, as has been speculated, insufficient carbohydrate intake might also contribute to the energy imbalance in short-stature children.
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