Hearing in adults with Pompe disease

Hearing loss has been recognized as an important cause of morbidity in infants with Pompe disease, a metabolic disorder caused by deficiency of acid α-glucosidase. It is unknown whether hearing is also affected in adult Pompe patients. We have studied the prevalence, severity, and type of hearing loss in 58 adult patients using tympanometry and pure-tone audiometry. Compared to normative data (International Organisation for Standardisation standard 7029), 72% of patients had impaired hearing thresholds at one or more frequencies in at least one ear. All measured frequencies were equally affected. All patients had a sensorineural type of hearing loss, pointing to cochlear or retrocochlear pathology. Categorised according to the standards of the World Health Organisation 21% of patients had a clinically relevant hearing loss (16% slight, 3% moderate, 2% profound). Though this suggests that hearing loss occurs in a considerable number of patients with Pompe disease, this prevalence is similar to that in the general population. Therefore, we conclude that hearing loss is not a specific feature of Pompe disease in adults

Informal caregivers of persons with dementia, their use of and needs for specific professional support: a survey of the national dementia programme

<p>Abstract</p> <p>Background</p> <p>This paper describes both the use of and needs for informal caregivers of people with dementia, based on a questionnaire survey organized within the National Dementia Programme in the Netherlands. The National Dementia Programme is a quality collaborative of the Dutch Alzheimer's Association, the Institute of Quality of Healthcare (CBO) and the Knowledge Centre on Ageing (Vilans), instigated by the Ministry of Health, Welfare and Sport, to improve integrated care for people with dementia and their informal caregivers. The support needs of informal caregivers are important to improve caregiver well-being and delaying institutionalization of the person with dementia.</p> <p>Methods</p> <p>In the period April 2006 - January 2007, the National Dementia Programme questionnaire was completed by 984 informal caregivers. Descriptive statistics were used to analyze the use of and needs for additional professional support by informal caregivers. Chi-square tests were used to assess the relationships between characteristics of the caregivers (spouses, sons/daughters, sons/daughters in-law) and support needs on one hand and to assess the relationship between the living situation of the person with dementia (living at home or living in a nursing home or home for the elderly) and support needs on the other hand.</p> <p>Results</p> <p>Almost all informal caregivers (92.6%) received some professional support. However, two thirds (67.4%) indicated they had one or more needs for additional professional support. Informal caregivers often need additional professional advice about what to do when their relative is frightened, angry of confused. Spouses reported different needs than sons or daughters (in-law): spouses relatively often need emotional support and sons or daughters (in-law) more often need information and coordination of dementia care.</p> <p>Conclusions</p> <p>Most of the informal caregivers report that they need additional information and advice, e.g. about how to cope with behavioral problems of their relative, about the progression of the illness trajectory, emotional support and coordination of dementia care. Future support programmes, e.g. in the field of case management, should address the specific needs of informal caregivers.</p

Effectiveness of an intervention at construction worksites on work engagement, social support, physical workload, and need for recovery: results from a cluster randomized controlled trial

<p>Abstract</p> <p>Background</p> <p>To prolong sustainable healthy working lives of construction workers, a worksite prevention program was developed which aimed to improve the health and work ability of construction workers. The aim of the current study was to investigate the effectiveness of this program on social support at work, work engagement, physical workload and need for recovery.</p> <p>Methods</p> <p>Fifteen departments from six construction companies participated in this cluster randomized controlled trial; 8 departments (n=171 workers) were randomized to an intervention group and 7 departments (n=122 workers) to a control group. The intervention consisted of two individual training sessions of a physical therapist to lower the physical workload, a Rest-Break tool to improve the balance between work and recovery, and two empowerment training sessions to increase the influence of the construction workers at the worksite. Data on work engagement, social support at work, physical workload, and need for recovery were collected at baseline, and at three, six and 12 months after the start of the intervention using questionnaires.</p> <p>Results</p> <p>No differences between the intervention and control group were found for work engagement, social support at work, and need for recovery. At 6 months follow-up, the control group reported a small but statistically significant reduction of physical workload.</p> <p>Conclusion</p> <p>The intervention neither improved social support nor work engagement, nor was it effective in reducing the physical workload and need for recovery among construction workers.</p> <p>Trial registration</p> <p>NTR1278</p

Hypertrophy of mature xenopus muscle fibres in culture induced by synergy of albumin and insulin

The aim of this study was to investigate effects of albumin and insulin separately as well as in combination on mature muscle fibres during long-term culture. Single muscle fibres were dissected from m. iliofibularis of Xenopus laevis and attached to a force transducer in a culture chamber. Fibres were cultured in a serum-free medium at slack length (mean sarcomere length 2.3 μm) for 8 to 22 days. The medium was supplemented with (final concentrations): (1) bovine insulin (6 nmol/L or 200-600 nmol/L), (2) 0.2% bovine albumin or (3) 0.2% bovine albumin in combination with insulin (120 nmol/L). In culture medium with insulin, 50% of the muscle fibres became in-excitable within 7-12 days, whereas the other 50% were stable. Caffeine contractures of in-excitable muscle fibres produced 80.4±2.4% of initial peak tetanic force, indicating impaired excitation-contraction (E-C) coupling in in-excitable fibres. In the presence of albumin, all cultured muscle fibres were stable for at least 10 days. Muscle fibres cultured in medium with insulin or albumin exclusively did not hypertrophy or change the number of sarcomeres in series. In contrast, muscle fibres cultured with both albumin and insulin showed an increase in tetanic force and fibre cross-sectional area of 19.6±2.8% and 32.5±4.9%, respectively, (means±SEM.; P=0.007) after 16.3±1.7 days, whereas the number of sarcomeres in series remained unchanged. We conclude that albumin prevents muscle fibre damage and preserves E-C coupling in culture. Furthermore, albumin is important in regulating muscle fibre adaptation by a synergistic action with growth factors like insulin. © 2008 The Author(s)

Communication, advice exchange and job satisfaction of nursing staff: a social network analyses of 35 long-term care units

Background: The behaviour of individuals is affected by the social networks in which they are embedded. Networks are also important for the diffusion of information and the influence of employees in organisations. Yet, at the moment little is known about the social networks of nursing staff in healthcare settings. This is the first study that investigates informal communication and advice networks of nursing staff in long-term care. We examine the structure of the networks, how they are related to the size of units and characteristics of nursing staff, and their relationship with job satisfaction. Methods: We collected social network data of 380 nursing staff of 35 units in group projects and psychogeriatric units in nursing homes and residential homes in the Netherlands. Communication and advice networks were analyzed in a social network application (UCINET), focusing on the number of contacts (density) between nursing staff on the units. We then studied the correlation between the density of networks, size of the units and characteristics of nursing staff. We used multilevel analyses to investigate the relationship between social networks and job satisfaction of nursing staff, taking characteristics of units and nursing staff into account. Results: Both communication and advice networks were negatively related to the number of residents and the number of nursing staff of the units. Communication and advice networks were more dense when more staff worked part-time. Furthermore, density of communication networks was positively related to the age of nursing staff of the units. Multilevel analyses showed that job satisfaction differed significantly between individual staff members and units and was influenced by the number of nursing staff of the units. However, this relationship disappeared when density of communication networks was added to the model. Conclusions: Overall, communication and advice networks of nursing staff in long-term care are relatively dense. This fits with the high level of cooperation that is needed to provide good care to residents. Social networks are more dense in small units and are also shaped by characteristics of staff members. The results furthermore show that communication networks are important for staff's job satisfaction. (aut. ref.

Prospective research on musculoskeletal disorders in office workers (PROMO): study protocol

BACKGROUND: This article describes the background and study design of the PROMO study (Prospective Research on Musculoskeletal disorders in Office workers). Few longitudinal studies have been performed to investigate the risk factors responsible for the incidence of hand, arm, shoulder and neck symptoms among office workers, given the observation that a large group of office workers might be at risk worldwide. Therefore, the PROMO study was designed. The main aim is to quantify the contribution of exposure to occupational computer use to the incidence of hand, arm, shoulder and neck symptoms. The results of this study might lead to more effective and/or cost-efficient preventive interventions among office workers. METHODS/DESIGN: A prospective cohort study is conducted, with a follow-up of 24 months. In total, 1821 participants filled out the first questionnaire (response rate of 74%). Data on exposure and outcome is collected using web-based self-reports. Outcome assessment takes place every three months during the follow-up period. Data on computer use are collected at baseline and continuously during follow-up using a software program. DISCUSSION: The advantages of the PROMO study include the long follow-up period, the repeated measurement of both exposure and outcome, and the objective measurement of the duration of computer use. In the PROMO study, hypotheses stemming from lab-based and field-based research will be investigated

Anti-tumour activity of bisphosphonates in preclinical models of breast cancer

There is increasing evidence of anti-tumour effects of bisphosphonates from pre-clinical studies, supporting a role for these drugs beyond their traditional use in treatment of cancer-induced bone disease. A range of model systems have been used to investigate the effects of different bisphosphonates on tumour growth, both in bone and at peripheral sites. Most of these studies conclude that bisphosphonates cause a reduction in tumour burden, but that early intervention and the use of high and/or repeated dosing is required. Successful eradication of cancer may only be achievable by targeting the tumour cells directly whilst also modifying the tumour microenvironment. In line with this, bisphosphonates are demonstrated to be particularly effective at reducing breast tumour growth when used in combination with agents that directly target cancer cells. Recent studies have shown that the effects of bisphosphonates on breast tumours are not limited to bone, and that prolonged anti-tumour effects may be achieved following their inclusion in combination therapy. This has opened the field to a new strand of bisphosphonate research, focussed on elucidating their effects on cells and components of the local, regional and distal tumour microenvironment. This review highlights the recent developments in relation to proposed anti-tumour effects of bisphosphonates reported from in vitro and in vivo models, and summarises the data from key breast cancer studies. Evidence for effects on different processes and cell types involved in cancer development and progression is discussed, and the main outstanding issues identified

Reconstitution of the Costunolide Biosynthetic Pathway in Yeast and Nicotiana benthamiana

The sesquiterpene costunolide has a broad range of biological activities and is the parent compound for many other biologically active sesquiterpenes such as parthenolide. Two enzymes of the pathway leading to costunolide have been previously characterized: germacrene A synthase (GAS) and germacrene A oxidase (GAO), which together catalyse the biosynthesis of germacra-1(10),4,11(13)-trien-12-oic acid. However, the gene responsible for the last step toward costunolide has not been characterized until now. Here we show that chicory costunolide synthase (CiCOS), CYP71BL3, can catalyse the oxidation of germacra-1(10),4,11(13)-trien-12-oic acid to yield costunolide. Co-expression of feverfew GAS (TpGAS), chicory GAO (CiGAO), and chicory COS (CiCOS) in yeast resulted in the biosynthesis of costunolide. The catalytic activity of TpGAS, CiGAO and CiCOS was also verified in planta by transient expression in Nicotiana benthamiana. Mitochondrial targeting of TpGAS resulted in a significant increase in the production of germacrene A compared with the native cytosolic targeting. When the N. benthamiana leaves were co-infiltrated with TpGAS and CiGAO, germacrene A almost completely disappeared as a result of the presence of CiGAO. Transient expression of TpGAS, CiGAO and CiCOS in N. benthamiana leaves resulted in costunolide production of up to 60 ng.g−1 FW. In addition, two new compounds were formed that were identified as costunolide-glutathione and costunolide-cysteine conjugates