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      Carbohydrate Metabolism Is Not Impaired after 3 Years of Growth Hormone Therapy in Children with Prader-Willi Syndrome

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          Background/Aim: In children with Prader-Labhart-Willi syndrome (PWS), the insulin secretion is reduced, despite obesity, being ascribed to the growth hormone (GH) deficiency of hypothalamic origin. Besides, an increased prevalence of diabetes mellitus was described in this syndrome. Hence, we addressed the questions of how body composition and insulin secretion are interrelated and what impact GH therapy has on the carbohydrate metabolism in PWS. Methods: We measured weight, lean and fat mass (by dual-energy X-ray absorptiometry), triglycerides, HbA<sub>1c</sub>, and fasting insulin and glucose levels in 17 children (age range 1.5–14.6 years) with PWS to examine whether the carbohydrate metabolism is altered during 36 months of therapy with 8 mg GH/m<sup>2</sup> body surface/week. In a subgroup of 8 children, the insulin secretion was longitudinally assayed during oral glucose tolerance at 0 and 12 months of therapy. Results: Before therapy, the insulin secretion was lower and markedly delayed as compared with reference data and did not rise during therapy. The glucose tolerance was impaired in 2 of 12 children examined by oral glucose tolerance test before therapy and normalized during therapy. Fasting insulin and insulin resistance being normal at the beginning, significantly increased at 12 months and returned to initial levels at 36 months of GH therapy. Fasting glucose as well as HbA<sub>1c</sub> and triglyceride levels were always normal. The fat mass before GH therapy was increased (39.5%) and dropped into the upper normal range (28.3%) during 3 years of therapy, being correlated with fasting insulin concentration and indices of insulin sensitivity before and after 1 year of therapy. Conclusions: Children with PWS are characterized by an intact insulin sensitivity with a decrease and a delay of insulin secretion, regardless of moderate obesity or GH treatment. In the present setting, the carbohydrate metabolism is not impaired by GH therapy, but by the excessively increased fat mass.

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          Eating behavior in Prader-Willi syndrome, normal weight, and obese control groups.

          Hyperphagia in Prader-Willi syndrome (PWS) is hypothesized to be due to hypothalamic dysfunction; thus the study of individuals with PWS might illustrate how hypothalamic dysfunction affects eating behavior. The aim of this study was to document the microstructure of the eating behavior in patients with PWS and to compare it with that of members of obese and normal weight control groups of the same age. Nine subjects with PWS (age, 10 +/- 4 years), 20 normal weight subjects (age, 12 +/- 3 years), and 20 obese subjects (age, 12 +/- 4 years) were served an excess lunch meal (hash) on a hidden scale built into a table and connected to a computer. The plate of food is placed on top of the scale, and when the food is eaten, the change in food weight is registered continuously. An eating curve is displayed online. After the meal, the eating data are fitted to a polynomial, and the computer calculates the amount of food eaten, time of consumption, eating rate (initial and total), and rate of deceleration. Subjects with PWS were found to have a longer duration of eating (P =.04) and a slower initial eating rate (P =. 01) compared with members of both obese and normal weight groups. In subjects with PWS, 56% of the eating curves were non-decelerating (linear or accelerating) compared with 10% of the normal weight group and 30% of the obese group (P =.02). The microstructure of the eating behavior in subjects with PWS differs from that of members of obese and normal weight control groups. Thus the eating behavior found in subjects with PWS might be due to decreased satiation rather than increased hunger.
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            Sustained benefit after 2 years of growth hormone on body composition, fat utilization, physical strength and agility, and growth in Prader-Willi syndrome.

            Obesity and hypotonia in children with Prader-Willi syndrome (PWS) are accompanied by abnormal body composition resembling a growth hormone (GH)-deficient state. Hypothalamic dysfunction in PWS includes decreased GH secretion, suggesting a possible therapeutic role for GH treatment. Although recent studies have demonstrated short-term benefits of treatment with GH, a critical question is whether beneficial changes persist or wane with prolonged therapy. Effects of 24 months of GH treatment (1 mg/m(2)/d) on growth, body composition, strength and agility, pulmonary function, resting energy expenditure, and fat utilization were assessed in 35 children with PWS. Percent body fat, lean muscle mass, and bone mineral density were measured by dual-energy x-ray absorptiometry. Indirect calorimetry was used to determine resting energy expenditure and to calculate the respiratory quotient. Compared with baseline evaluations, increased height velocity (SD score -1.1 +/- 2.5 to 2.2 +/- 2.3; P <. 001), reduced percent body fat (46.4% +/- 8.4% to 40.3% +/- 10.0%, P <.001), and improved respiratory muscle function and physical strength and agility (sit-ups, weight-lifts, running speed, and broad jump; P <.01) were observed after 24 months of GH treatment. A decline in respiratory quotient (0.81 +/- 0.07 to 0.75 +/- 0.06; P <. 01) and a trend toward increased resting energy expenditure were also observed. Changes in response to GH occurred predominantly during the initial 12 months of GH therapy. Children with PWS had sustained increases in lean body mass, decreases in percent body fat, improvements in physical strength and agility, and increased fat oxidation after 24 months of GH therapy. However, between 12 and 24 months, the growth rate slowed. Consequently, encouraging initial results require even more prolonged study to draw conclusions regarding the long-term value of GH therapy in changing body composition in children with PWS.
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              Detection of Insulin Resistance by Simple Quantitative Insulin Sensitivity Check Index QUICKI for Epidemiological Assessment and Prevention

               J Hrebícek (2002)

                Author and article information

                Horm Res Paediatr
                Hormone Research in Paediatrics
                S. Karger AG
                08 May 2003
                : 59
                : 5
                : 239-248
                aFoundation Growth Puberty Adolescence, Zürich, bDepartment of Paediatrics, University of Zürich, and cInstitute of Paediatric Endocrinology, Basel, Switzerland
                70224 Horm Res 2003;59:239–248
                © 2003 S. Karger AG, Basel

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                Page count
                Figures: 4, Tables: 3, References: 48, Pages: 10
                Original Paper


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