The role of ceramide trihexoside (globotriaosylceramide) in the diagnosis and follow-up of the efficacy of treatment of Fabry disease: a review of the literature.

Fabry disease is caused by a deficiency of a-galactosidase A which leads to the progressive intra-lysosomal accumulation of ceramide trihexoside (CTH), also known as globotriaosylceramide (Gb3), in different cell types and body fluids. The clinical manifestations are multisystemic and predominantly affect the heart, kidney and central nervous system. The role of CTH in the pathophysiological process of Fabry disease is not established, and the link between the degree of accumulation and disease manifestations is not systematic. The use of CTH as a diagnostic tool has been proposed for several decades. The recent introduction of a specific treatment for Fabry disease in the form of enzyme replacement therapy (ERT) has led to the need for a biological marker, in place of a clinical sign, for evaluating the efficacy of treatment and also as a tool for following the long term effects of treatment. The ideal biomarker must adhere to strict criteria, and there should be a correlation between the degree of clinical efficacy of treatment and a change in its concentration. This review of the literature assesses the utility of CTH as a diagnostic tool and as a marker of the efficacy of ERT in patients with Fabry disease. Several techniques have been developed for measuring CTH; the principles and the sensitivity thresholds of these methods and the units used to express the results should be taken into consideration when interpreting data. The use of CTH measurement in Fabry disease should be re-evaluated in light of recent published data.

Posterior cortical atrophy: review of the recent literature.

Posterior cortical atrophy (PCA) is a group of neurodegenerative dementing disorders characterized by initial predominant visual complaints followed by progressive decline in cognitive functions. The visuospatial and visuoperceptual defects arise from the dysfunction of, respectively, the dorsal (occipito-parietal) and the ventral (occipito-temporal) streams. Clinical symptoms, results of neuropsychological examination, and findings of posterior cerebral atrophy and/or posterior hypoperfusion/hypometabolism contribute to the diagnosis. However, owing to the insidious onset of PCA and the non-specificity of initial symptoms, the diagnosis is often delayed. Specific etiologies include Alzheimer's disease, dementia with Lewy bodies, subcortical gliosis, corticobasal degeneration, and prion-associated diseases. Alzheimer's disease accounts for at least 80 % of PCA cases. Recent research has concentrated on better defining the clinical presentation of PCA, improving neuroimaging analysis, testing new neuroimaging techniques, and developing biological measurements. Selected recent papers on PCA are reviewed in this article.

Resolution of foveal detachment in dome-shaped macula after treatment by spironolactone: report of two cases and mini-review of the literature.

Dome-shaped macula (DSM) was recently described in myopic patients as a convex protrusion of the macula within a posterior pole staphyloma. The pathogenesis of DSM and the development of associated serous foveal detachment (SFD) remain unclear. The obstruction of choroidal outflow and compressive changes of choroidal capillaries have been proposed as causative factors. In this paper, we report two cases of patients with chronic SFD associated with DSM treated with oral spironolactone. After treatment, there was a complete resolution of SFD in both patients. To the best of our knowledge, this is the first report of successful treatment of SFD in DSM by a mineralocorticoid receptor antagonist.

The Irish Infancy Narratives and their Relationship with Latin Sources

(Résumé de l'ouvrage) In 1927 M. R. James published Latin Infancy Gospels, identified by him in two related but not identical manuscripts (one the British Library Arundel 404; the other from Hereford), together with a parallel text from the Irish manuscript known as the Leabhar Breac. Later researches brought to light more manuscripts of this Latin work, and also of the Irish text. James recognized that his apocryphal Latin Infancy text was compiled from a combination of the Protevangelium of James and a hitherto unknown text which he named "The Source". Recent research has identified a full Latin translation of the Protevangelium of James. A hitherto unrecognized Irish Infancy Narrative has also been identified in the Dublin manuscript known as the Liber Flavus Fergusiorum. A deep study of this related tradition was called for. This has been carried out over the past ten years by an Irish team in conjunction with Professor Daniel Kaestli and AELAC. The fruits of this labour are published in these two volumes. Volume 13 has a general introduction with a historical sketch of New Testament apocrypha in Ireland and a history of research on the subject. This is followed by a comparison of the Infancy Narratives in the Leabhar Breac and the Liber Flavus Fergusiorum. There are special introductions to these Infancy texts, followed by critical editions of the Irish texts, accompanied by English translations and rich annotation. Next there is similar treatment of the Irish versified Narrative (from ca. 700) of the Childhood Deeds of Jesus (commonly known as the Infancy Narrative (or Gospel) of Thomas. There is then (in volume 14, but with continuous pagination) the edition and translation of an Irish thirteenth-century poem with elements from Infancy Narratives, and both Latin and Irish texts on the wonders at Christ's birth, accompanied by translations and notes. The edition of the Irish material is followed by a critical edition of the full Arundel and Hereford forms of the Infancy Narrative (here referred to as the "J Compilation"), together with a detailed study of all the questions relating to this work. The volume concludes with a critical edition (by Rita Beyers) of the Latin text of the Protevangelium of James, accompanied by a detailed study of the work.. The work contains a detailed study of the Latin translations of the Protevangelium of James and the transmission of this work in the West. The "J Compilation" (a combination of the Protevangelium and texts of Pseudo-Matthew) can be traced back in manuscript transmission to ca. 800,and must have originated some time earlier. Behind it stands an earlier "I ("I" for Irish) Compilation" without influence from Pseudo-Matthew, the form found in the Irish witnesses. It is argued that M. R. James's "Source" may be of Judaeo-Christian origin and may really be the Gospel of the Nazoreans. Among the indexes there is a list of all the Irish words found in the texts. This edition of the Irish and related Latin texts is a major contribution to the study of the apocryphal Infancy Narratives. It should also be of particular interest to Celtic scholars, to students of Irish ecclesiastical learning, and in general to all medievalists.

A soil emergence trap for collections of phlebotomine sand flies

The identification of breeding sites of sand flies is of great epidemiological interest. A soil emergence trap for investigating potential sand fly breeding sites is described. The trap was tested in two rural areas in the Mogi Guaçu River Valley where the American cutaneous leishmaniasis is an endemic disease. Seventy-three sand fly individuals of three species, Lutzomyia intermedia s. l., L. whitmani and L. pessoai, were collected on the forest floor and peridomicile.

Literature Review: Equality of Opportunity in Relation to the DHSSPS Draft Equality Scheme

This review of the literature on equality of opportunity issues was commissioned by the Department of Public Health, Social Sevices and Public Safety.

Audit of the Irish health system for value for money

In April 2000, Deloitte & Touche in conjunction with the York Health Economics Consortium were chosen by The Department of Health & Children to carry out an examination of the health services over the past ten years Download the Report here

Dietary habits of the Irish population: results from SLÃÅN Annual Report 2003

Dietary habits of the Irish population: results from SLÃÂÅN Annual Report 2003 The National Nutritional Surveillance Centre was established in 1992, in the Department of Health Promotion, National University of Ireland,Galway. In 2003 the Centre moved to the Department of Public Health Medicine and Epidemiology, University College Dublin. Its main functions are to provide nutrition-related information to relevant organizations in an accessible form and to monitor trends in health status in relation to food supply, availability and consumption. Click here to download PDF 1.8mb

Dietary habits of the Irish population: results from SLÃÅN Annual Report 2003

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Mental Disorders in Older Irish People: Incidence, Prevalence and Treatment

The National Council for the Elderly is an advisory body to the Minister for Health on all aspects of ageing and the welfare of the elderly. One of its terms of reference is to advise the Minister on measures to promote the health of the elderly. As one of its contributions towards the realisation of this objective the Council published a report in October 1996 entitled, Mental Disorders in Older Irish People: Incidence Prevalence and Treatment. The report provides a profile of mental disorders in the older Irish population by bringing together in one publication the information which exists on the prevalence, incidence and treatment of mental disorders in older people. It will be a valuable source of information for planning and developing mental health services for older people. Download the Report here  

Speech by Mr. Micheál Martin TD – Meeting The Challenges Of Cultural Diversity In The Irish Healthcare Sector

It gives me great pleasure to accept the invitation to address this conference on “Meeting the Challenges of Cultural Diversity in the Irish Healthcare Sector” which is being organised by the Irish Health Services Management Institute in partnership with the National Consultative Committee on Racism and Interculturalism. The conference provides an important opportunity to develop our knowledge and understanding of the issues surrounding cultural diversity in the health sector from the twin perspectives of patients and staff. Cultural diversity has over recent years become an increasingly visible aspect of Irish society bringing with it both opportunities and challenges. It holds out great possibilities for the enrichment of all who live in Ireland but it also challenges us to adapt creatively to the changes required to realise this potential and to ensure that the experience is a positive one for all concerned but particularly for those in the minority ethnic groups. In the last number of years in particular, the focus has tended to be on people coming to this country either as refugees, asylum seekers or economic migrants. Government figures estimate that as many as 340,000 immigrants are expected in the next six years. However ethnic and cultural diversity are not new phenomena in Ireland. Travellers have a long history as an indigenous minority group in Ireland with a strong culture and identity of their own. The changing experience and dynamics of their relationship with the wider society and its institutions over time can, I think, provide some valuable lessons for us as we seek to address the more numerous and complex issues of cultural diversity which have arisen for us in the last decade. Turning more specifically to the health sector which is the focus of this conference, culture and identity have particular relevance to health service policy and provision in that The first requirement is that we in the health service acknowledge cultural diversity and the differences in behaviours and in the less obvious areas of values and beliefs that this often implies. Only by acknowledging these differences in a respectful way and informing ourselves of them can we address them. Our equality legislation – The Employment Equality Act, 1998 and the Equal Status Act, 2000 – prohibits discrimination on nine grounds including race and membership of the Traveller community. The Equal Status Act prohibits discrimination on an individual basis in relation to the nine grounds while for groups it provides for the promotion of equality of opportunity. The Act applies to the provision of services including health services. I will speak first about cultural diversity in relation to the patient. In this respect it is worth mentioning that the recognition of cultural diversity and appropriate responses to it were issues which were strongly emphasised in the public consultation process which we held earlier this year in the context of developing National Anti-Poverty targets for the health sector and also our new national health strategy. Awareness and sensitivity training for staff is a key requirement for adapting to a culturally diverse patient population. The focus of this training should be the development of the knowledge and skills to provide services sensitive to cultural diversity. Such training can often be most effectively delivered in partnership with members of the minority groups themselves. I am aware that the Traveller community, for example, is involved in in-service training for health care workers. I am also aware that the National Consultative Committee on Racism and Interculturalism has been involved in training with the Eastern Regional Health Authority. We need to have more such initiatives. A step beyond the sensitivity training for existing staff is the training of members of the minority communities themselves as workers in our health services. Again the Traveller community has set an example in this area with its Primary Health Care Project for Travellers. The Primary Health Care for Travellers Project was established in 1994 as a joint partnership initiative with the Eastern Health Board and Pavee Point, with ongoing technical assistance being provided from the Department of Community Health and General Practice, Trinity College, Dublin. This project was the first of its kind in the country and has facilitated The project included a training course which concentrated on skills development, capacity building and the empowerment of Travellers. This confidence and skill allowed the Community Health Workers to go out and conduct a baseline survey to identify and articulate Travellers’ health needs. This was the first time that Travellers were involved in this process; in the past their needs were assumed. The results of the survey were fed back to the community and they prioritised their needs and suggested changes to the health services which would facilitate their access and utilisation. Ongoing monitoring and data collection demonstrates a big improvement in levels of satisfaction and uptake and ulitisation of health services by Travellers in the pilot area. This Primary Health Care for Travellers initiative is being replicated in three other areas around the country and funding has been approved for a further 9 new projects. This pilot project was the recipient of a WHO 50th anniversary commemorative award in 1998. The project is developing as a model of good practice which could inspire further initiatives of this type for other minority groups. Access to information has been identified in numerous consultative processes as a key factor in enabling people to take a proactive approach to managing their own health and that of their families and in facilitating their access to health services. Honouring our commitment to equity in these areas requires that information is provided in culturally appropriate formats. The National Health Promotion Strategy 2000-2005, for example, recognises that there exists within our society many groups with different requirements which need to be identified and accommodated when planning and implementing health promotion interventions. These groups include Travellers, refugees and asylum seekers, people with intellectual, physical or sensory disability and the gay and lesbian community. The Strategy acknowledges the challenge involved in being sensitive to the potential differences in patterns of poor health among these different groups. The Strategic aim is to promote the physical, mental and social well-being of individuals from these groups. The objective of the Strategy on these issues are: While our long term aim may be to mainstream responses so that our health services is truly multicultural, we must recognise the need at this point in time for very specific focused responses particularly for groups with poor health status such as Travellers and also for refugees and asylum seekers. In the case of refugees and asylum seekers examples of targeted services are screening for communicable diseases – offered on a voluntary basis – and psychological support services for those who have suffered trauma before coming here. The two approaches of targeting and mainstreaming are not mutually exclusive. A combination of both is required at this point in time but the balance between them must be kept under constant review in the light of changing needs. A major requirement if we are to meet the challenge of cultural diversity is an appropriate data and research base. I think it is important that we build up our information and research data base in partnership with the minority groups themselves. We must establish what the health needs of diverse groups are; we must monitor uptake of services and how well we are responding to needs and we must monitor outcomes and health status. We must also examine the impact of the policies in other sectors on the health of minority groups. The National Health Information Strategy, currently being developed, and the recently published National Strategy for Health Research – Making Knowledge Work for Health provide important frameworks within which we can improve our data and research base. A culturally diverse health sector workforce – challenges and opportunities The Irish health service can benefit greatly from successful international recruitment. There has been a strong non-national representation amongst the medical profession for more than 30 years. More recently there have been significant increases in other categories of health service workers from overseas. The Department recognises the enormous value that overseas recruitment brings over a wide range of services and supports the development of effective and appropriate recruitment strategies in partnership with health service employers. These changes have made cultural diversity an important issue for all health service organisations. Diversity in the workplace is primarily about creating a culture that seeks, respects, values and harnesses difference. This includes all the differences that when added together make each person unique. So instead of the focus being on particular groups, diversity is about all of us. Change is not about helping “them” to join “us” but about critically looking at “us” and rooting out all aspects of our culture that inappropriately exclude people and prevent us from being inclusive in the way we relate to employees, potential employees and clients of the health service. International recruitment benefits consumers, Irish employees and the overseas personnel alike. Regardless of whether they are employed by the health service, members of minority groups will be clients of our service and consequently we need to be flexible in order to accommodate different cultural needs. For staff, we recognise that coming from other cultures can be a difficult transition. Consequently health service employers have made strong efforts to assist them during this period. Many organisations provide induction courses, religious facilities (such as prayer rooms) and help in finding suitable accommodation. The Health Service Employers Agency (HSEA) is developing an equal opportunities/diversity strategy and action plans as well as training programmes to support their implementation, to ensure that all health service employment policies and practices promote the equality/diversity agenda to continue the development of a culturally diverse health service. The management of this new environment is extremely important for the health service as it offers an opportunity to go beyond set legal requirements and to strive for an acceptance and nurturing of cultural differences. Workforce cultural diversity affords us the opportunity to learn from the working practices and perspectives of others by allowing personnel to present their ideas and experience through teamwork, partnership structures and other appropriate fora, leading to further improvement in the services we provide. It is important to ensure that both personnel units and line managers communicate directly with their staff and demonstrate by their actions that they intend to create an inclusive work place which doesn´t demand that minority staff fit. Contented, valued employees who feel that there is a place for them in the organisation will deliver a high quality health service. Your conference here today has two laudable aims – to heighten awareness and assist health care staff to work effectively with their colleagues from different cultural backgrounds and to gain a greater understanding of the diverse needs of patients from minority ethnic backgrounds. There is a synergy in these aims and in the tasks to which they give rise in the management of our health service. The creative adaptations required for one have the potential to feed into the other. I would like to commend both organisations which are hosting this conference for their initiative in making this event happen, particularly at this time – Racism in the Workplace Week. I look forward very much to hearing the outcome of your deliberations. Thank you.

Evaluation of the Irish Pilot Programme for the Education of Health Care Assistants

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An evaluation of the first phase of the Irish Cervical Screening Programme from the woman's perspective

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Integrating the Gender Perspective in Irish Health Policy: A Case Study

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