Hyperhidrosis: a new and often early symptom in Fabry disease. International experience and data from the Fabry Outcome Survey.

Hypohidrosis is a classic feature of Fabry disease; in contrast, hyperhidrosis has only been rarely described. The aim of the study is to characterise the baseline descriptive data on hyperhidrosis (frequency, age at onset, sex ratio and outcome with and without enzyme replacement therapy) in hemizygous male and heterozygous female patients with Fabry disease. We describe case histories of five patients with Fabry disease and hyperhidrosis seen at three different centres. We have also analysed a cohort of 21 paediatric patients in the UK and a large European cohort of patients enrolled in the Fabry Outcome Survey (FOS). Five patients (three female, two male) with hyperhidrosis were originally identified, although each had additional symptoms related to Fabry disease. The age at onset of hyperhidrosis was less than 18 years in four cases. In the cohort of 21 paediatric patients (12 female, nine male), one female had hyperhidrosis; the age at onset of this symptom was 11 years. In the FOS cohort, 66 of 714 patients with Fabry disease had hyperhidrosis (44 of 369 females, 11.9%; 22 of 345 males, 6.4%). The female predominance was observed in seven of nine countries from which data were analysed. Hyperhidrosis is an increasingly recognised feature of the Fabry disease phenotype. It is more prevalent in females than in males and often appears in childhood or adolescence. The efficacy of enzyme replacement therapy on this recently recognised symptom should be assessed.

Economic survey of Latin America and the Caribbean 2012: policies for an adverse international economy

Includes bibliography

Findings of the survey on international classifications in Latin America

Spanish document available at the Library

Learning for well being : Studies using the International Adult Literacy Survey

This thesis is a collection of five independent but closely related studies. The overall purpose is to approach the analysis of learning outcomes from a perspective that combines three major elements, namely lifelonglifewide learning, human capital, and the benefits of learning. The approach is based on an interdisciplinary perspective of the human capital paradigm. It considers the multiple learning contexts that are responsible for the development of embodied potential – including formal, nonformal and informal learning – and the multiple outcomes – including knowledge, skills, economic, social and others– that result from learning. The studies also seek to examine the extent and relative influence of learning in different contexts on the formation of embodied potential and how in turn that affects economic and social well being. The first study combines the three major elements, lifelonglifewide learning, human capital, and the benefits of learning into one common conceptual framework. This study forms a common basis for the four empirical studies that follow. All four empirical studies use data from the International Adult Literacy Survey (IALS) to investigate the relationships among the major elements of the conceptual framework presented in the first study. Study I. A conceptual framework for the analysis of learning outcomes This study brings together some key concepts and theories that are relevant for the analysis of learning outcomes. Many of the concepts and theories have emerged from varied disciplines including economics, educational psychology, cognitive science and sociology, to name only a few. Accordingly, some of the research questions inherent in the framework relate to different disciplinary perspectives. The primary purpose is to create a common basis for formulating and testing hypotheses as well as to interpret the findings in the empirical studies that follow. In particular, the framework facilitates the process of theorizing and hypothesizing on the relationships and processes concerning lifelong learning as well as their antecedents and consequences. Study II. Determinants of literacy proficiency: A lifelong-lifewide learning perspective This study investigates lifelong and lifewide processes of skill formation. In particular, it seeks to estimate the substitutability and complementarity effects of learning in multiple settings over the lifespan on literacy skill formation. This is done by investigating the predictive capacity of major determinants of literacy proficiency that are associated with a variety of learning contexts including school, home, work, community and leisure. An identical structural model based on previous research is fitted to the IALS data for 18 countries. The results show that even after accounting for all factors, education remains the most important predictor of literacy proficiency. In all countries, however, the total effect of education is significantly mediated through further learning occurring at work, at home and in the community. Therefore, the job and other literacy related factors complement education in predicting literacy proficiency. This result points to a virtual cycle of lifelong learning, particularly to how educational attainment influences other learning behaviours throughout life. In addition, results show that home background as measured by parents’ education is also a strong predictor of literacy proficiency, but in many countries this occurs only if a favourable home background is complemented with some post-secondary education. Study III. The effect of literacy proficiency on earnings: An aggregated occupational approach using the Canadian IALS data This study uses data from the Canadian Adult Literacy Survey to estimate the earnings return to literacy skills. The approach adapts a labour segmented view of the labour market by aggregating occupations into seven types, enabling the estimation of the variable impact of literacy proficiency on earnings, both within and between different types of occupations. This is done using Hierarchical Linear Modeling (HLM). The method used to construct the aggregated occupational classification is based on analysis that considers the role of cognitive and other skills in relation to the nature of occupational tasks. Substantial premiums are found to be associated with some occupational types even after adjusting for within occupational differences in individual characteristics such as schooling, literacy proficiency, labour force experience and gender. Average years of schooling and average levels of literacy proficiency at the between level account for over two-thirds of the premiums. Within occupations, there are significant returns to schooling but they vary depending on the type of occupations. In contrast, the within occupational return of literacy proficiency is not necessarily significant. The latter depends on the type of occupation. Study IV: Determinants of economic and social outcomes from a lifewide learning perspective in Canada In this study the relationship between learning in different contexts, which span the lifewide learning dimension, and individual earnings on the one hand and community participation on the other are examined in separate but comparable models. Data from the Canadian Adult Literacy Survey are used to estimate structural models, which correspond closely to the common conceptual framework outlined in Study I. The findings suggest that the relationship between formal education and economic and social outcomes is complex with confounding effects. The results indicate that learning occurring in different contexts and for different reasons leads to different kinds of benefits. The latter finding suggests a potential trade-off between realizing economic and social benefits through learning that are taken for either job-related or personal-interest related reasons. Study V: The effects of learning on economic and social well being: A comparative analysis Using the same structural model as in Study IV, hypotheses are comparatively examined using the International Adult Literacy Survey data for Canada, Denmark, the Netherlands, Norway, the United Kingdom, and the United States. The main finding from Study IV is confirmed for an additional five countries, namely that the effect of initial schooling on well being is more complex than a direct one and it is significantly mediated by subsequent learning. Additionally, findings suggest that people who devote more time to learning for job-related reasons than learning for personal-interest related reasons experience higher levels of economic well being. Moreover, devoting too much time to learning for personal-interest related reasons has a negative effect on earnings except in Denmark. But the more time people devote to learning for personal-interest related reasons tends to contribute to higher levels of social well being. These results again suggest a trade-off in learning for different reasons and in different contexts.

A survey of paediatric HIV programmatic and clinical management practices in Asia and sub-Saharan Africa--the International epidemiologic Databases to Evaluate AIDS (IeDEA)

INTRODUCTION There are limited data on paediatric HIV care and treatment programmes in low-resource settings. METHODS A standardized survey was completed by International epidemiologic Databases to Evaluate AIDS paediatric cohort sites in the regions of Asia-Pacific (AP), Central Africa (CA), East Africa (EA), Southern Africa (SA) and West Africa (WA) to understand operational resource availability and paediatric management practices. Data were collected through January 2010 using a secure, web-based software program (REDCap). RESULTS A total of 64,552 children were under care at 63 clinics (AP, N=10; CA, N=4; EA, N=29; SA, N=10; WA, N=10). Most were in urban settings (N=41, 65%) and received funding from governments (N=51, 81%), PEPFAR (N=34, 54%), and/or the Global Fund (N=15, 24%). The majority were combined adult-paediatric clinics (N=36, 57%). Prevention of mother-to-child transmission was integrated at 35 (56%) sites; 89% (N=56) had access to DNA PCR for infant diagnosis. African (N=40/53) but not Asian sites recommended exclusive breastfeeding up until 4-6 months. Regular laboratory monitoring included CD4 (N=60, 95%), and viral load (N=24, 38%). Although 42 (67%) sites had the ability to conduct acid-fast bacilli (AFB) smears, 23 (37%) sites could conduct AFB cultures and 18 (29%) sites could conduct tuberculosis drug susceptibility testing. Loss to follow-up was defined as >3 months of lost contact for 25 (40%) sites, >6 months for 27 sites (43%) and >12 months for 6 sites (10%). Telephone calls (N=52, 83%) and outreach worker home visits to trace children lost to follow-up (N=45, 71%) were common. CONCLUSIONS In general, there was a high level of patient and laboratory monitoring within this multiregional paediatric cohort consortium that will facilitate detailed observational research studies. Practices will continue to be monitored as the WHO/UNAIDS Treatment 2.0 framework is implemented.

The significant effect of endometriosis on physical, mental and social wellbeing: results from an international cross-sectional survey.

STUDY QUESTION To what extent do the management of endometriosis and the symptoms that remain after treatment affect the quality of life in women with the disease? SUMMARY ANSWER Many women with endometriosis had impaired quality of life and continued to suffer from endometriosis-associated symptoms even though their endometriosis has been managed in tertiary care centres. WHAT IS KNOWN ALREADY The existing literature indicates that quality of life and work productivity is reduced in women with endometriosis. However, most studies have small sample sizes, are treatment related or examine newly diagnosed patients only. STUDY DESIGN, SIZE, DURATION A cross-sectional questionnaire-based survey among 931 women with endometriosis treated in 12 tertiary care centres in 10 countries. PARTICIPANTS/MATERIALS, SETTING, METHODS Women diagnosed with endometriosis who had at least one contact related to endometriosis-associated symptoms during 2008 with a participating centre were enrolled into the study. The study investigated the effect of endometriosis on education, work and social wellbeing, endometriosis-associated symptoms and health-related quality of life, by using questions obtained from the World Endometriosis Research Foundation (WERF) GSWH instrument (designed and validated for the WERF Global Study on Women's Health) and the Short Form 36 version 2 (SF-36v2). MAIN RESULTS AND THE ROLE OF CHANCE Of 3216 women invited to participate in the study, 1450 (45%) provided informed consent and out of these, 931 (931/3216 = 29%) returned the questionnaires. Endometriosis had affected work in 51% of the women and affected relationships in 50% of the women at some time during their life. Dysmenorrhoea was reported by 59%, dyspareunia by 56% and chronic pelvic pain by 60% of women. Quality of life was decreased in all eight dimensions of the SF-36v2 compared with norm-based scores from a general US population (all P < 0.01). Multivariate regression analysis showed that number of co-morbidities, chronic pain and dyspareunia had an independent negative effect on both the physical and mental component of the SF-36v2. LIMITATIONS, REASONS FOR CAUTION The fact that women were enrolled in tertiary care centres could lead to a possible over-representation of women with moderate-to-severe endometriosis, because the participating centres typically treat more complex and referred cases of endometriosis. The response rate was relatively low. Since there was no Institute Review Board approval to do a non-responder investigation on basic characteristics, some uncertainty remains regarding the representativeness of the investigated population. WIDER IMPLICATIONS OF THE FINDINGS This international multicentre survey represents a large group of women with endometriosis, in all phases of the disease, which increases the generalizability of the data. Women still suffer from frequent symptoms, despite tertiary care management, in particular chronic pain and dyspareunia. As a result their quality of life is significantly decreased. A patient-centred approach with extensive collaboration across disciplines, such as pain specialists, psychologists, sexologists and social workers, may be a valuable strategy to improve the long-term care of women with endometriosis. STUDY FUNDING/COMPETING INTEREST(S) The WERF EndoCost study is funded by the World Endometriosis Research Foundation (WERF) through grants received from Bayer Schering Pharma AG, Takeda Italia Farmaceutici SpA, Pfizer Ltd and the European Society of Human Reproduction and Embryology. The sponsors did not have a role in the design and conduct of the study; collection, management, analysis and interpretation of the data; and preparation, review or approval of the manuscript. L.H. is the chief executive and T.D. was a board member of WERF at the time of funding. T.D. holds the Merck-Serono Chair in Reproductive Medicine and Surgery, and the Ferring Chair in Reproductive Medicine at the Katholieke Universiteit Leuven in Belgium and has served as consultant/research collaborator for Merck-Serono, Schering-Plough, Astellas and Arresto.

International variation in GP treatment strategies for subclinical hypothyroidism in older adults: a case-based survey

Background: There is limited evidence about the impact of treatment for subclinical hypothyroidism, especially among older people. Aim: To investigate the variation in GP treatment strategies for older patients with subclinical hypothyroidism depending on country and patient characteristics. Design and setting: Case-based survey of GPs in the Netherlands, Germany, England, Ireland, Switzerland, and New Zealand. Method: The treatment strategy of GPs (treatment yes/no, starting-dose thyroxine) was assessed for eight cases presenting a woman with subclinical hypothyroidism. The cases differed in the patient characteristics of age (70 versus 85 years), vitality status (vital versus vulnerable), and thyroid-stimulating hormone (TSH) concentration (6 versus 15 mU/L). Results: A total of 526 GPs participated (the Netherlands n = 129, Germany n = 61, England n = 22, Ireland n = 21, Switzerland n = 262, New Zealand n = 31; overall response 19%). Across countries, differences in treatment strategy were observed. GPs from the Netherlands (mean treatment percentage 34%), England (40%), and New Zealand (39%) were less inclined to start treatment than GPs in Germany (73%), Ireland (62%), and Switzerland (52%) (P = 0.05). Overall, GPs were less inclined to start treatment in 85-year-old than in 70-year-old females (pooled odds ratio [OR] 0.74 [95% confidence interval [CI] = 0.63 to 0.87]). Females with a TSH of 15 mU/L were more likely to get treated than those with a TSH of 6 mU/L (pooled OR 9.49 [95% CI = 5.81 to 15.5]). Conclusion: GP treatment strategies of older people with subclinical hypothyroidism vary largely by country and patient characteristics. This variation underlines the need for a new generation of international guidelines based on the outcomes of randomised clinical trials set within primary care