Differences in Adipose Tissue and Lean Mass Distribution in Patients with Collagen VI Related Myopathies Are Associated with Disease Severity and Physical Ability

Mutations in human collagen VI genes cause a spectrum of musculoskeletal conditions in children and adults collectively termed collagen VI-related myopathies (COL6-RM) characterized by a varying degree of muscle weakness and joint contractures and which include Ullrich Congenital Muscular Dystrophy (UCMD) and Bethlem Myopathy (BM). Given that collagen VI is one of the most abundant extracellular matrix proteins in adipose tissue and its emerging role in energy metabolism we hypothesized that collagen VI deficiency might be associated with alterations in adipose tissue distribution and adipokines serum profile. We analyzed body composition by means of dual-energy X-ray absorptiometry in 30 pediatric and adult COL6-RM myopathy patients representing a range of severities (UCMD, intermediate-COL6-RM, and BM). We found a distinctive pattern of regional adipose tissue accumulation which was more evident in children at the most severe end of the spectrum. In particular, the accumulation of fat in the android region was a distinguishing feature of UCMD patients. In parallel, there was a decrease in lean mass compatible with a state of sarcopenia, particularly in ambulant children with an intermediate phenotype. All children and adult patients that were sarcopenic were also obese. These changes were significantly more pronounced in children with collagen VI deficiency than in children with Duchenne Muscular Dystrophy of the same ambulatory status. High molecular weight adiponectin and leptin were significantly increased in sera from children in the intermediate and BM group. Correlation analysis showed that the parameters of fat mass were negatively associated with motor function according to several validated outcome measures. In contrast, lean mass parameters correlated positively with physical performance and quality of life. Leptin and adiponectin circulating levels correlated positively with fat mass parameters and negatively with lean mass and thus may be relevant to the disease pathogenesis and as circulating markers. Taken together our results indicate that COL6-RM are characterized by specific changes in total fat mass and distribution which associate with disease severity, motor function, and quality of life and which are clinically meaningful and thus should be taken into consideration in the management of these patients

Differences In Adipose Tissue And Lean Mass Distribution In Patients With Collagen Vi Related Myopathies Are Associated With Disease Severity And Physical Ability

Mutations in human collagen VI genes cause a spectrum of musculoskeletal conditions in children and adults collectively termed collagen VI-related myopathies (COL6-RM) characterized by a varying degree of muscle weakness and joint contractures and which include Ullrich Congenital Muscular Dystrophy (UCMD) and Bethlem Myopathy (BM). Given that collagen VI is one of the most abundant extracellular matrix proteins in adipose tissue and its emerging role in energy metabolism we hypothesized that collagen VI deficiency might be associated with alterations in adipose tissue distribution and adipokines serum profile. We analyzed body composition by means of dual-energy X-ray absorptiometry in 30 pediatric and adult COL6-RM myopathy patients representing a range of severities (UCMD, intermediate-COL6-RM, and BM). We found a distinctive pattern of regional adipose tissue accumulation which was more evident in children at the most severe end of the spectrum. In particular, the accumulation of fat in the android region was a distinguishing feature of UCMD patients. In parallel, there was a decrease in lean mass compatible with a state of sarcopenia, particularly in ambulant children with an intermediate phenotype. All children and adult patients that were sarcopenic were also obese. These changes were significantly more pronounced in children with collagen VI deficiency than in children with Duchenne Muscular Dystrophy of the same ambulatory status. High molecular weight adiponectin and leptin were significantly increased in sera from children in the intermediate and BM group. Correlation analysis showed that the parameters of fat mass were negatively associated with motor function according to several validated outcome measures. In contrast, lean mass parameters correlated positively with physical performance and quality of life. Leptin and adiponectin circulating levels correlated positively with fat mass parameters and negatively with lean mass and thus may be relevant to the disease pathogenesis and as circulating markers. Taken together our results indicate that COL6-RM are characterized by specific changes in total fat mass and distribution which associate with disease severity, motor function, and quality of life and which are clinically meaningful and thus should be taken into consideration in the management of these patients

The Oslo Health Study: Is bone mineral density higher in affluent areas?

<p>Abstract</p> <p>Background</p> <p>Based on previously reported differences in fracture incidence in the socioeconomic less affluent Oslo East compared to the more privileged West, our aim was to study bone mineral density (BMD) in the same socioeconomic areas in Oslo. We also wanted to study whether possible associations were explained by socio-demographic factors, level of education or lifestyle factors.</p> <p>Methods</p> <p>Distal forearm BMD was measured in random samples of the participants in The Oslo Health Study by single energy x-ray absorptiometry (SXA). 578 men and 702 women born in Norway in the age-groups 40/45, 60 and 75 years were included in the analyses. Socioeconomic regions, based on a social index dividing Oslo in two regions – East and West, were used.</p> <p>Results</p> <p>Age-adjusted mean BMD in women living in the less affluent Eastern region was 0.405 g/cm²and significantly lower than in West where BMD was 0.419 g/cm². Similarly, the odds ratio of low BMD (Z-score ≤ -1) was 1.87 (95% CI: 1.22–2.87) in women in Oslo East compared to West. The same tendency, although not statistically significant, was also present in men. Multivariate analysis adjusted for education, marital status, body mass index, physical inactivity, use of alcohol and smoking, and in women also use of post-menopausal hormone therapy and early onset of menopause, did hardly change the association. Additional adjustments for employment status, disability pension and physical activity at work for those below the age of retirement, gave similar results.</p> <p>Conclusion</p> <p>We found differences in BMD in women between different socioeconomic regions in Oslo that correspond to previously found differences in fracture rates. The association in men was not statistically significant. The differences were not explained by socio-demographic factors, level of education or lifestyle factors.</p

The relationship between educational level and bone mineral density in postmenopausal women

BACKGROUND: This study describes the influence of educational level on bone mineral density (BMD) and investigating the relationship between educational level and bone mineral density in postmenopausal women. METHODS: A total of 569 postmenopausal women, from 45 to 86 years of age (mean age of 60.43 ± 7.19 years) were included in this study. A standardized interview was used at the follow-up visit to obtain information on demographic, life-style, reproductive and menstrual histories such as age at menarche, age at menopause, number of pregnancies, number of abortions, duration of menopause, duration of fertility, and duration of lactation. Patients were separated into four groups according to the level of education, namely no education (Group 1 with 209 patients), elementary (Group 2 with 222 patients), high school (Group 3 with 79 patients), and university (Group 4 with 59 patients). RESULTS: The mean ages of groups were 59.75 ± 7.29, 61.42 ± 7.50, 60.23 ± 7.49, and 58.72 ± 7.46, respectively. Spine BMD was significant lower in Group 1 than that of other groups (p < 0.05). Trochanter and ward's triangle BMD were the highest in Group 4 and there was a significant difference between Group 1 and 4 (p < 0.05). The prevalence of osteoporosis showed an inverse relationship with level of education, ranging from 18.6% for the most educated to 34.4% for the no educated women (p < 0.05). Additionally, there was a significant correlation between educational level and spine BMD (r = 0.20, p < 0.01), trochanter BMD (r = 0.13, p < 0.01), and ward's BMD (r = 0.14, p < 0.01). CONCLUSIONS: The results of the study suggest that there is a significant correlation between educational level and BMD. Losses in BMD for women of lower educational level tend to be relatively high, and losses in spine and femur BMD showed a decrease with increasing educational level

CRONOEJECUCION y GESTIÓN ADAPTATIVA DE PAQUETES EN EL SISTEMA DE COMUNICACIONES MOVILES UMTS

RESUMEN. Fruto del imparable crecimiento que ha experimentado la transmisión de paquetes sobre los sistemas de comunicaciones móviles, ha surgido un creciente interés, tanto por parte de las grandes compañfas de telefonía móvil como de los fabricantes, por desarrollar algoritmos eficientes de gestión adaptativa de paquetes. El objetivo principal de la gestión adaptativa de paquetes en los sistemas de comunicaciones móviles es maximizar la capacidad del sistema proporcionando los niveles de cobertura y calidad de servicio deseados, para una cantidad de recursos radio e infraestructura desplegada dadas. En este articulo se presenta un estudio minucioso del proceso de cronoejecución o gestión adaptativa de paquetes en el sistema de comunicaciones móviles UMTS. De esta manera no sólo se describen los conceptos generales relacionados con la cronoejecución sino que también se proporciona una visión practica de los canales de datos estandarizados y de la implementación real de la cronoejecución en UMTS. Además, se ofrece una comparativa de distintos algoritmos típicos utilizados en la cronoejecución con dos nuevas técnicas, propuestas ambas con el objetivo de minimizar el retardo de los paquetes. Los resultados obtenidos demuestran que las mejores prestaciones del sistema se obtienen realizando conjuntamente la maximización de la tasa binaria transmitida yla minimización del retardo. PALABRAS CLAVE Conmutación de paquetes Cronoejecución UMTS Retardo de paquetes ABSTRACT Due to the high increase in the packet transmission over mobile communications systems, the big telecommunications operators together with the main vendors have focused their researching effort on the development of efficient packet scheduling algorithms. In the mobile communications systems the adaptive packet scheduling aims at maximizing the overall system capacity, guaranteeing the desired levels in coverage and quality of service, given a certain network infrastructure. This paper performs and in-depth analysis of the scheduling process within the UMTS mobile communications system. Not only the general concepts involved in scheduling are boned up but also a practical overview of the scheduling implementation is provided. Several algorithms are compared with two new proposals, both focused on minimizing the packet delay. Computer simulations have shown that the transmitted bit rate maximization carried out together with the delay minimization algorithm maximizes the resources and, moreover, distributes them efficiently minimizing the average service response time. Very important gains in terms of delay, resource allocation and number of active users have been obtained when comparing this new technique with respect to other scheduling algorithms proposed for UMTS. The higher efficiency in resource distribution is twice useful, first it causes the users to transmit faster, and second it reduces the load of the system allowing either more users to transmit or the current users to transmit even faster. KEYWORDS Packet Switching Scheduling UMTS Packet Dela

CRONOEJECUCION y GESTIÓN ADAPTATIVA DE PAQUETES EN EL SISTEMA DE COMUNICACIONES MOVILES UMTS

RESUMEN. Fruto del imparable crecimiento que ha experimentado la transmisión de paquetes sobre los sistemas de comunicaciones móviles, ha surgido un creciente interés, tanto por parte de las grandes compañfas de telefonía móvil como de los fabricantes, por desarrollar algoritmos eficientes de gestión adaptativa de paquetes. El objetivo principal de la gestión adaptativa de paquetes en los sistemas de comunicaciones móviles es maximizar la capacidad del sistema proporcionando los niveles de cobertura y calidad de servicio deseados, para una cantidad de recursos radio e infraestructura desplegada dadas. En este articulo se presenta un estudio minucioso del proceso de cronoejecución o gestión adaptativa de paquetes en el sistema de comunicaciones móviles UMTS. De esta manera no sólo se describen los conceptos generales relacionados con la cronoejecución sino que también se proporciona una visión practica de los canales de datos estandarizados y de la implementación real de la cronoejecución en UMTS. Además, se ofrece una comparativa de distintos algoritmos típicos utilizados en la cronoejecución con dos nuevas técnicas, propuestas ambas con el objetivo de minimizar el retardo de los paquetes. Los resultados obtenidos demuestran que las mejores prestaciones del sistema se obtienen realizando conjuntamente la maximización de la tasa binaria transmitida yla minimización del retardo. PALABRAS CLAVE Conmutación de paquetes Cronoejecución UMTS Retardo de paquetes ABSTRACT Due to the high increase in the packet transmission over mobile communications systems, the big telecommunications operators together with the main vendors have focused their researching effort on the development of efficient packet scheduling algorithms. In the mobile communications systems the adaptive packet scheduling aims at maximizing the overall system capacity, guaranteeing the desired levels in coverage and quality of service, given a certain network infrastructure. This paper performs and in-depth analysis of the scheduling process within the UMTS mobile communications system. Not only the general concepts involved in scheduling are boned up but also a practical overview of the scheduling implementation is provided. Several algorithms are compared with two new proposals, both focused on minimizing the packet delay. Computer simulations have shown that the transmitted bit rate maximization carried out together with the delay minimization algorithm maximizes the resources and, moreover, distributes them efficiently minimizing the average service response time. Very important gains in terms of delay, resource allocation and number of active users have been obtained when comparing this new technique with respect to other scheduling algorithms proposed for UMTS. The higher efficiency in resource distribution is twice useful, first it causes the users to transmit faster, and second it reduces the load of the system allowing either more users to transmit or the current users to transmit even faster. KEYWORDS Packet Switching Scheduling UMTS Packet Dela