Treatment with human growth hormone in patients with Prader-Labhart-Willi syndrome reduces body fat and increases muscle mass and physical performance

Twelve children with documented Prader-Labhart-Willi syndrome were treated with human growth hormone (24 U/m2/week) during 1 year. The children were divided into three groups: group 1: overweight and prepubertal (n = 6, age 3.8-7.0 years); group 2: underweight and prepubertal (n = 3, age 0.6-4.1 years); group 3: pubertal (n = 3, age 9.2-14.6 years). In group 1, height increased from -1.7 SD to -0.6 SD, while weight decreased from 1.1 SD to 0.4 SD, with a dramatic drop in weight for height from 3.8 SD to 1.2 SD. Hand length increased from -1.5 SD to -0.4 SD and foot length from -2.5 SD to -1.4 SD. Body fat, measured by dual X-ray energy absorptiometry, dropped by a third, whereas muscle mass increased by a fourth. Physical capability (Wingate test) improved considerably. The children were reported to be much more active and capable. In group 2, similar changes were seen, but weight for height increased, probably because muscle mass increase exceeded fat mass decrease. Changes in group 3 were similar as in group 1, even though far less distinct. Conclusion: Growth hormone treatment in Prader-Labhart-Willi syndrome led to dramatic changes: distinct increase in growth velocity, height and muscle mass, as well as an improvement in physical performance. Fat mass and weight for height decreased in the initially overweight children, and weight for height increased in underweight childre

A Critical Ear: Analysis of Value Judgments in Reviews of Beethoven's Piano Sonata Recordings

What sets a great music performance apart? In this study we addressed this question through an examination of value judgements in written criticism of recorded performance. One hundred reviews of recordings of Beethoven’s piano sonatas, published in the Gramophone between 1934 and 2010, were analyzed through a three-step qualitative analysis that identified the valence (positive/negative) expressed by critics’ statements and the evaluation criteria that underpinned their judgements. The outcome is a model of the main evaluation criteria used by professional critics: aesthetic properties, including intensity, coherence, and complexity, and achievement-related properties, including sureness, comprehension, and endeavor. The model also emphasizes how critics consider the suitability and balance of these properties across the musical and cultural context of the performance. The findings relate directly to current discourses on the role of evaluation in music criticism and the generalizability of aesthetic principles. In particular, the perceived achievement of the performer stands out as a factor that drives appreciation of a recording

Understanding music Problems in the philosophy of music

Available from British Library Document Supply Centre- DSC:D177022 / BLDSC - British Library Document Supply CentreSIGLEGBUnited Kingdo

Velocity Measurements and Turbulent Dispersion in Annular Liquid Film Flows in BWR Subchannels with Spacers

The thermal-hydraulic design characteristics of a boiling water reactor are strongly dependent on the physics of heat-removing mechanisms from nuclear rods. Performance is often limited by a lack of understanding of the heat and momentum transfer in thin liquid films close to dryout. This is particularly important with regard to power uprates of reactors. Flow control by functional spacers equipped with vanes adds complexity to the flow behavior. Instead of improving the heat removal, they can cause a local reduction of the film thickness under unfavorable conditions. This work presents a novel experimental technique to measure the velocity of the liquid phase and to study the turbulent mixing of a passive scalar in the film. The studies were performed in vertical annular flow in a double-subchannel geometry. The liquid film was labeled by either continuous or pulsed tracer injection. An electrically conductive tracer (salt solution) is injected into the film consisting of deionized water. The salt is traced using a liquid film sensor, a high-frequency nonintrusive conductivity-based technique. The experiments are conducted with and without a swirl-type spacer to quantify the effect of the spacers on the film flow for different gas and liquid flow rates. An ensemble averaging of the data obtained from individual injection pulses provides time-averaged liquid velocities in the film. The turbulent dispersion was characterized by analyzing the spread of the tracer in axial and lateral directions. Spacer vanes are found to induce a characteristic transverse movement of the liquid in the film. The effect of waves on mixing is analyzed and is found to enhance it

Building community through higher music education: a training program for facilitating musical engagement among older adults

Preparing music students to design and carry out community-based initiatives can be an effective way to enhance their employability and their wellbeing. With a large body of evidence now pointing to the benefits of musical engagement for older adults, both for individuals and for society as a whole, there is considerable opportunity and value in training aspiring professional musicians to work with and on behalf of those in their third and fourth age. This article describes a seminar designed by a Swiss conservatoire in collaboration with local nursing homes involving residents and music university students in a 10-week group music making program. On the basis of the positive results to emerge in terms of health, wellbeing and career preparation, we aim to provide information relevant for colleagues to replicate this seminar in other higher music education institutions. Moreover, this paper aims to shed light on the complexity of designing music students’ training so that they acquire the competences needed to deliver meaningful, community-based initiatives alongside their other professional training commitments, and to provide directions for future research. The development and implementation of these points could foster the increase and sustainability of innovative programs beneficial for older adults, musicians and local communities

Phenotypic heterogeneity of growth and psychometric intelligence in Prader-Willi syndrome: Variable expression of a contiguous gene syndrome or parent-child resemblance?

Phenotypic variability in children with Prader-Willi syndrome (PWS) was investigated with respect to variable expression of the contiguous gene syndrome and trait variance. In a prospective study, parent/child resemblance of anthropometric and psychometric measures was analyzed in 22 children with PWS (11 females and 11 males; 18 deletions, 4 uniparental disomy (UPD)) and in a control group (88 females and 88 males). The average child-midparent Z-score difference for height in females was –1.9 and in males –0.9, head circumference –1.7 and –1.0, and body mass index (BMI) 2.3 and 2.7, respectively. Intellectual performance of females and males was, on average, –2.7 and –2.6 below maternal performance. Range and standard deviation were moderately increased for height and head circumference, doubled for BMI, and unchanged for IQ. Parent/child correlations for anthropometric and psychometric measurements in the study group did not significantly differ from those of the control group. Exceptions were higher correlations between mothers and daughters for height (P < 0.05) and BMI (P < 0.01), and lower correlations for head circumference between midparent values and daughters (P < 0.05) than in the control group. In conclusion, parent–child resemblance in growth and intellectual development among children with PWS was found to be comparable to that noted in the normal population, indicating a strong determination by trait variance. Children with PWS differed significantly with respect to a lower trait level and—with the exception of IQ—a larger variability. The latter may indicate a variable expression of the contiguous gene syndrome

Familial Dysalbuminemic Hyperthyroxinemia in a Swiss Family Caused by a Mutant Albumin (R218P) Shows an Apparent Discrepancy between Serum Concentration and Affinity for Thyroxine1

Familial dysalbuminemic hyperthyroxinemia (FDH), is the most common cause of inherited increase in serum total T4 (TT4) in the Caucasian population. It is caused by a mutation (R218H) in the human serum albumin (HSA) gene, resulting in 10-fold higher affinity for T4 and, in heterozygous affected subjects, a TT4 level 2-fold higher than that in subjects expressing the wild-type HSA only. We now report FDH in a Swiss family, caused by HSA R218P, previously reported in subjects of Japanese origin. In this form of FDH, serum TT4 levels are 14- to 20-fold the normal mean, confirmed by measurements in serum extracts. TrT3 and TT3, concentrations are 7- and 2-fold above the mean, respectively. Thus, to maintain a normal free T4 level, the calculated affinity constant (Ka) of HSA R218P should be about 16-fold higher than that of HSA R218H. Surprisingly, the Ka values measured at saturation were similar: 5.4 × 106 and 6.4 × 106 mol/L−1 for HSA R218H, respectively. To determine how subjects with HSA R218P and R218P maintain a euthyroid state despite the markedly high serum TT4, the concentration of dialyzable T4 was measured at increasing amounts of TT4. At a TT4 level equivalent to that found in the subjects with HSA R218P, the absolute FT4 concentrations were 40, 432, and 1970 pmol/L for sera expressing HSAs R218P, R218H, and wild type, respectively. Thus, the affinity of HSA R218P for T4 must be higher than that of R218H to produce an 11-fold difference in FT4 at the same concentration of TT4. This difference was obliterated at saturating concentrations of TT4 used for the determination of Ka values by the method of Scatchard

Treatment with human growth hormone in patients with Prader-Labhart-Willi syndrome reduces body fat and increases muscle mass and physical performance

Twelve children with documented Prader-Labhart-Willi syndrome were treated with human growth hormone (24 U/m2/week) during 1 year. The children were divided into three groups: group 1: overweight and prepubertal (n=6, age 3.8-7.0 years); group 2: underweight and prepubertal (n=3, age 0.6-4.1 years); group 3: pubertal (n=3, age 9.2-14.6 years). In group 1, height increased from -1.7 SD to -0.6 SD, while weight decreased from 1.1 SD to 0.4 SD, with a dramatic drop in weight for height from 3.8 SD to 1.2 SD. Hand length increased from -1.5 SD to -0.4 SD and foot length from -2.5 SD to -1.4 SD. Body fat, measured by dual X-ray energy absorptiometry, dropped by a third, whereas muscle mass increased by a fourth. Physical capability (Wingate test) improved considerably. The children were reported to be much more active and capable. In group 2, similar changes were seen, but weight for height increased, probably because muscle mass increase exceeded fat mass decrease. Changes in group 3 were similar as in group 1, even though far less distinct. CONCLUSION: Growth hormone treatment in Prader-Labhart-Willi syndrome led to dramatic changes: distinct increase in growth velocity, height and muscle mass, as well as an improvement in physical performance. Fat mass and weight for height decreased in the initially overweight children, and weight for height increased in underweight children