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PubMed2004

The burden of alcohol misuse on emergency in-patient hospital admissions among residents from a health board region in Ireland.

AIMS: To identify in-patient emergency admissions to acute hospitals of residents from a health board region in the Republic of Ireland with an acute alcohol intoxication diagnosis; to profile the admissions and to assess whether the increase in alcohol consumption in Ireland has been mirrored by an increase in alcohol related emergency admissions over the same time period. DESIGN: A retrospective review of hospital admissions using Hospital In-Patient Enquiry (HIPE) data and alcohol consumption trends using data from the Central Statistics Office (CSO). SETTING: Acute hospitals in the Republic of Ireland. PARTICIPANTS: All residents from a health board region admitted with a recorded alcohol intoxication emergency admission to non-private acute hospitals in Ireland. MEASUREMENTS: All in-patient emergency admissions for which an acute alcohol intoxication diagnosis (ICD Codes 303.0 and 305.0) was recorded of residents from one health board region were extracted from the HIPE system for years 1997-2001 inclusive. Pearson's chi2 test was used to compare proportions in groups of categorical data and chi2 test for trend was used to identify linear trends. Age standardized rates were calculated for each year and trend analysis carried out. Demographic data on the patients were also extracted from the database. FINDINGS: There were 3289 acute alcohol intoxication admissions to acute hospitals of residents from the study region recorded for years 1997-2001 inclusive. There were 777 acute alcohol intoxication admissions in 2001 compared to 432 admissions in 1997, an increase of 80%. Age standardized rates showed a significant increasing linear trend (P < 0.001). Over half the admissions occurred on weekends. The average length of stay was 2.7 days (95% CI 2.5-2.8) with just under a quarter (24.3%) of these admissions being discharged on the same day. The majority of these patients were male (71.5%), 40.9% were under 30 years old and over half (59.4%) were single. CONCLUSIONS: This study shows that alcohol intoxication accounted for a substantial number of emergency in-patient admissions to acute hospitals in one health board region in Ireland and that the age standardized recorded acute alcohol related emergency admission rate increased significantly over the 5-year period, 1997-2001. This increase mirrored the national increase in alcohol consumption over the same time period.

Adolescent↗
PubMed1983

Edentulousness in the United Kingdom and Ireland.

Surveys of adult (16 yr and over) dental health were conducted in England/Wales and Scotland (n = 5967) and in Ireland (n = 1764) and Northern Ireland (n = 1176) in 1979. From the results of these surveys a comparison is made between levels of edentulousness, attendance patterns, attitude to loss of teeth and the wearing of full dentures. Scotland had the highest level of edentulousness (39%) and Ireland the lowest (26%). England/Wales had a level of 29% and Northern Ireland 33%. The percentage edentulous increased considerably ith age and females had a higher level of edentulousness than males. Regular attendance at the dentist was lowest in Ireland and in all countries there was a greater preference for filling rather than extraction of teeth. Levels of edentulousness do not appear to be directly related to any of the parameters investigated.

Adolescent↗
PubMed2006

Trends in blind registration in the adult population of the Republic of Ireland 1996-2003.

BACKGROUND/AIM: The Republic of Ireland has a centralised database of all registered, blind people in the country. The last study of the national blind register was undertaken in 1996. The current study sought, firstly, to investigate and identify any recent changes in the register composition. Secondly, there is concern that many eligible people are not appropriately registered. To examine this further, registration levels among patients attending an Irish outpatient ophthalmology clinic were determined. METHODS: Criteria for blind registration in Ireland are (1) a best corrected visual acuity of 6/60 or less in the better eye, or (2) a visual field subtending an angle of 20 degrees or less. The National Council for the Blind in Ireland (NCBI) is the sole custodian of a national registration database recording all eligible, registered people. This computerised database was analysed to provide information on the demographics and blind registration condition of those on the register in 2003. This information was compared with the results of the 1996 study. To assess the accuracy of the current register, the registration status of eligible patients attending the outpatient clinic of a busy, tertiary referral ophthalmology department, over a 9 week period, was studied. RESULTS: 6862 adults were registered as blind on the NCBI register in Ireland in 2003, representing an increase of 37% since 1996. The leading causes of registration were age related macular degeneration (ARMD) (25%), glaucoma (12%), and retinitis pigmentosa (7%). Comparing the 1996 and 2003 data, dramatic increases in the numbers registered caused by ARMD (from 812 to 1729 people, a 113% increase) and diabetic retinopathy (DR) (from 147 people to 323 people, a 120% increase) were found. The numbers registered as a result of glaucoma were relatively stable (795 in 1996 and 811 in 2003). A substantial drop, of 53%, was noted in the number of people registered as a result of cataracts, from 561 people to 261. Of the 672 new cases registered in 2003, ARMD accounted for 44%, glaucoma 13%, and DR 7%. Over the 9 week study period 75 patients, out of a total 2320 patients who attended the outpatient department, fulfilled the blind registration criteria. It was found that 21% (16 of 75) of the eligible clinic outpatients had not been appropriately registered. CONCLUSION: An overall increase in adult blind registration of 37% in the Republic of Ireland was found between 1996 and 2003. There were large increases in registered blindness as a result of ARMD (113%) and DR (120%). A notable decrease in registration as a result of cataracts was discovered. Vigilance by clinicians is necessary to ensure that eligible patients are registered.

Adolescent↗
PubMed1994

Deaths from primary brain cancers, lymphatic and haematopoietic cancers in agricultural workers in the Republic of Ireland.

STUDY OBJECTIVE: To ascertain if agricultural workers in the Republic of Ireland had a higher than expected mortality from brain and haematopoietic cancers than occurred in the general population. DESIGN: The Central Statistics Office of Ireland provided computer analysis of all deaths coded as cancer of the brain, ICD 191, and of lymphatic and haematopoietic cancers, ICD codes 200-208, by socioeconomic, sex, and age groups, from 1971 to 1987. The deaths were then analysed by socioeconomic group and compared with the expected number of deaths in the general population. SETTING: A cluster of four deaths from primary brain cancer, three from leukaemia, and one from Hodgkin's disease, occurred in the research and technical staff of the former Agricultural Institute of the Republic of Ireland in men under the age of 65. This raised the question, were farmers more likely to get these forms of cancer due to exposure to herbicides or fertilisers? SUBJECTS: All deaths in the Republic of Ireland from 1971 to 1987 by socioeconomic group. MAIN RESULTS: Although deaths reported as due to primary brain cancers had increased in all socioeconomic groups in the two time periods studied, there was no greater increase in farmers and a smaller increase in other agricultural workers and fishermen. Deaths from Hodgkin's disease and multiple myeloma, and to a smaller extent from leukaemia, had also increased. The increase in reported mortality of these haematopoietic cancers in farmers was no greater and in other agricultural workers it was less than in the general population. CONCLUSION: There was no evidence that farmers had any greater increase in mortality from these cancers than the general population. The cluster of brain and haematopoietic cancers in research and technical staff at the Agricultural Institute of the Republic of Ireland does not reflect a high risk of these cancers among the general farming population, but strongly supports the need for a compilation of a register of causes of death of laboratory workers in a number of countries.

Adolescent↗
PubMed2004

Latitudinal variation in the prevalence of multiple sclerosis in Ireland, an effect of genetic diversity.

BACKGROUND: Northern Ireland has a high and rising prevalence rate of multiple sclerosis (MS). The most recent survey in 1996 found a rate of 168.7/100 000. Recorded prevalence rates for the south of Ireland, including County Wexford, have been markedly lower and seemed to suggest the existence of a prevalence gradient within the island. OBJECTIVES: To compare the prevalence of multiple sclerosis in Co. Wexford in the south east of Ireland and Co. Donegal in the north west, and to establish whether a variation in prevalence of MS exists within Ireland. METHODS: Patients were referred from multiple sources. Review of clinical case records and/or patient examination confirmed the diagnosis. RESULTS: In Co. Wexford, 126 patients were found to have clinically definite or probable multiple sclerosis with a prevalence rate of 120.7/100 000 (95% confidence intervals (CI) 100.6 to 143.8), which is similar to other areas of similar latitude within the British Isles. In Co. Donegal, 240 people had clinically definite or probable MS with a prevalence rate of 184.6/100 000 (95% CI 162.0 to 209.5). The difference in prevalence rates is statistically significant (Z = 3.94, p = <0.001). CONCLUSION: There is a latitudinal variation in the prevalence rate of MS between the north and the south of Ireland. The increased prevalence of MS seen in Co. Wexford is likely to represent better case ascertainment and improved diagnostic accuracy rather than an actual increase in prevalence. The north/south variation in prevalence may represent a variation in the genetic predisposition to MS between the background populations of the two counties.

Adolescent↗
PubMed1997

Epidemiology of bovine spongiform encephalopathy in Northern Ireland 1988 to 1995.

The epidemiology of bovine spongiform encephalopathy (BSE) in Northern Ireland from 1988, when it was first confirmed, to the end of 1995 is described. All cases of BSE were subjected to a detailed epidemiological investigation, complemented by data from the national animal health records on every bovine animal. Data are presented on 1680 cases. Many of the epidemiological features of the disease were similar to those reported in Great Britain, but the incidence in Northern Ireland was approximately one-tenth that in Great Britain. The epidemic increased to a peak of 56 cases per month in January 1994, and decreased to nine cases in December 1995. Statutory intervention banning the use of meat and bone meal in ruminant feed in January 1989 has produced a marked and continuing reduction in the incidence. The majority of the cases were in Northern Ireland cattle, but 83 cases were imported from Great Britain and five from the Republic of Ireland. Many of the key epidemiological features have remained constant throughout the epidemic: the greater incidence of BSE in dairy herds than in beef suckler herds, the low within-herd incidence, the variation in incidence with herd size, the breed distribution, the distribution of the reported clinical signs and the proportion of purchased cases. Although the source of the BSE epidemic in Northern Ireland has not been established conclusively, the evidence suggests that the importation of meat and bone meal and protein concentrates from Great Britain may have been responsible.

Animals↗
PubMed1994

A case-control investigation of perinatal risk factors for childhood IDDM in Northern Ireland and Scotland.

OBJECTIVE: To identify perinatal risk factors for childhood insulin-dependent diabetes mellitus (IDDM) and determine if they differ between early-onset and late-onset disease. RESEARCH DESIGN AND METHODS: We selected 258 diabetic children in Northern Ireland and 271 diabetic children in Scotland from population-based registers. For each diabetic child, five matched control subjects were drawn from the same population. All perinatal data were recorded routinely at birth. Odds ratios (ORs) were estimated for parental age, social class, breast-feeding, deprivation measures, and other perinatal variables. RESULTS: Scottish data indicated an increased risk among children born to older mothers (OR = 2.43, 95% confidence interval [CI] 1.49-3.97 for mothers > or = 35 years of age relative to those < 25 years of age). Northern Ireland data showed no such effect. Only Northern Ireland data showed an excess risk in children of professional or managerial families (OR = 1.51, 95% CI 1.11-2.04). A small but nonsignificant reduction in risk among breast-fed children was observed only after adjustment for social class (OR = 0.76, 95% CI 0.54-1.07). Deprivation measures were associated with reductions in risk. Children delivered by cesarean section were at increased risk in both Northern Ireland (OR = 1.66, 95% CI 1.10-2.50) and Scottish (OR = 1.70, 95% CI 1.12-2.59) data. In Northern Ireland data only, children of first pregnancies were at increased risk (OR = 1.41, 95% CI 1.03-1.93). Both data sets indicated that a first pregnancy was a more important risk factor for early-onset disease than for late-onset disease. CONCLUSIONS: Many reported risk factors are weak and show inconsistencies between studies. They may be secondary to more direct, as-yet-undiscovered risk factors. Although irrelevant in the majority of cases, the increased risk associated with delivery by cesarean section deserves further study.

Adult↗
PubMed2006

Prevalence of Arcobacter spp. in raw milk and retail raw meats in Northern Ireland.

A 1-year study was undertaken to determine the prevalence of Arcobacter spp. in raw milk and retail raw meats on sale in Northern Ireland. Retail raw poultry samples (n = 94), pork samples (n = 101), and beef samples (n = 108) were obtained from supermarkets in Northern Ireland, and raw milk samples (n = 101) were kindly provided by the Milk Research Laboratory, Department of Agriculture and Rural Development, Belfast, Northern Ireland. Presumptive arcobacters were identified by previously described genus-specific and species-specific PCR assays. Arcobacter spp. were found to be common contaminants of retail raw meats and raw milk in Northern Ireland. Poultry meat (62%) had the highest prevalence, but frequent isolations were made from pork (35%), beef (34%), and raw milk (46%). Arcobacter butzleri was the predominant species isolated from retail raw meats and was the only species isolated from raw milk samples. Arcobacter cryaerophilus was detected less frequently, and Arcobacter skirrowii was detected only as a cocontaminant. To our knowledge, this is the first report of Arcobacter spp. prevalence in a diverse range of products of animal origin in Northern Ireland.

Animals↗
PubMed1993

Oral cancer in North Western Ireland: a sixteen year retrospective study.

OBJECTIVE: To provide baseline data on oral cancer epidemiology in North Western Ireland. BACKGROUND: Information on oral cancer in the Republic of Ireland is limited. Few studies have been conducted and, frequently, studies from other countries have been used to estimate the extent of the problem. STUDY DESIGN: A retrospective study of records identified oral cancer patients who presented between 1973 and 1988. Only squamous cells carcinomas of covering epithelium were included (ICD-9, 140-146 and 149). RESULTS: The commonest site was lip (81%). Lip lesions tended to be smaller than intraoral lesions and less likely to show lymph node involvement. Among lip cancer patients, there was a marked predominance of men and of outdoor workers. CONCLUSIONS: Intraoral cancer incidences in North Western Ireland approximate those in other parts of Ireland and many other Western countries. Lip cancer incidences are higher than those in other parts of Ireland and many Western countries. Survival rates are similar to those found in other studies.

Adult↗
PubMed1997

Genetic drift and gene flow in post-famine Ireland.

This study examines the genetic impact of the Great Famine (1846-1851) on the regional genetic structure of Ireland. The Great Famine resulted in a rapid decrease in population size throughout Ireland in a short period of time, increasing the possibility of genetic drift. Our study is based on migration and anthropometric data collected originally in the 1930s from 7211 adult Irish males. These data were subdivided into three time periods defined by year of birth: 1861-1880, 1881-1900, and 1901-1920. Within each time period the data were further subdivided into six geographic regions of Ireland. Estimates of Wright's FST were calculated from parent-offspring migration data and from 17 anthropometric variables (10 head measures, 7 body measures). Over time, the average population size decreased, but average rates of migration increased. The estimates of FST at equilibrium from migration matrix analysis suggest that the net effect of these opposite effects is a reduction in among-group variation. Closer examination shows that within each time period the rate of convergence to equilibrium is slow, meaning that the expected levels of genetic homogeneity revealed from migration matrix analysis are not likely to be seen over short intervals of time. Estimates of FST from anthropometric data show either relatively little change in microdifferentiation or some increase, depending on which variables are analyzed. Investigation of a simple model of demographic and genetic change shows that, given the demographic changes in post-Famine Ireland, FST could in theory increase, decrease, or remain the same over short intervals of time. Overall, the Great Famine appears to have had minimal impact on the genetic structure of Ireland on a regional level. Comparison with studies focusing on local genetic structure shows the opposite. It appears that the level of genetic impact depends strongly on the level of analysis; local populations are affected to a greater extent by demographic shifts than regional populations. We also provide formulas for the standard errors of FST from metric traits and related statistics.

Adolescent↗
PubMed1998

Seasonal variations in mortality caused by cardiovascular diseases in Norway and Ireland.

BACKGROUND: Seasonal variations in mortality resulting from cardiovascular diseases (CVD) have been demonstrated in many countries, with the highest levels observed during the coldest months of the year. We studied the seasonal changes in CVD mortality in Norway and the Republic of Ireland, two countries which are demographically quite similar, but climatically different; we also examined the relation between CVD mortality and air temperature. METHODS: Registered monthly data for mortality from CVD for the period 1985-1995 were obtained from the Norwegian Central Bureau of Statistics and the Irish Central Statistics Office. Meteorological data were provided by the Norwegian Institute of Meteorology and Met Eireann, in Ireland. Monthly mortality ratio for both men and women aged 60 and older was calculated from the mortality date. Mean monthly air temperatures for the two countries were calculated from the meteorological data. RESULTS: For the 10-year period investigated, the lowest and highest monthly mortality ratios were on average found in August and January, respectively, and mean excess winter mortality, expressed as the difference between the August and January values for the entire 10-year period, was 22% (Norway) and 35% (Ireland). However, when the percentage difference in the months with the respective highest and lowest mortality ratios were calculated for each year, the average of these differences for each of the 10 individual years was 29% and 45%. Mortality ratio was found to increase much more steeply with decreasing air temperature in Ireland than in Norway. CONCLUSION: Although the seasonal variation between CVD mortality in both countries is similar, the different relation with climatic conditions may result from differences in housing standards, allowing outdoor temperatures to have a greater influence on indoor temperature in Ireland than in Norway.

Adolescent↗
PubMed1999

Prehistoric birds from New Ireland, Papua New Guinea: extinctions on a large Melanesian island.

At least 50 species of birds are represented in 241 bird bones from five late Pleistocene and Holocene archaeological sites on New Ireland (Bismarck Archipelago, Papua New Guinea). The bones include only two of seabirds and none of migrant shorebirds or introduced species. Of the 50 species, at least 12 (petrel, hawk, megapode, quail, four rails, cockatoo, two owls, and crow) are not part of the current avifauna and have not been recorded previously from New Ireland. Larger samples of bones undoubtedly would indicate more extirpated species and refine the chronology of extinction. Humans have lived on New Ireland for ca. 35,000 years, whereas most of the identified bones are 15,000 to 6,000 years old. It is suspected that most or all of New Ireland's avian extinction was anthropogenic, but this suspicion remains undetermined. Our data show that significant prehistoric losses of birds, which are well documented on Pacific islands more remote than New Ireland, occurred also on large, high, mostly forested islands close to New Guinea.

Animals↗
PubMed2003

Epidemiology of MRSA: the North/South study of MRSA in Ireland 1999.

The North/South Study of methicillin-resistant Staphylococcus aureus (MRSA) in Ireland, 1999, includes a joint review of the epidemiology of MRSA across both jurisdictions on the island of Ireland. Data were gathered on all MRSA cases identified in laboratories in Northern Ireland (the North) and in the Republic of Ireland (the South) over a two-week period. The prevalence rate per 100000 population was 11.4 in the North and 14.0 in the South, with a marked variation across geographical regions. MRSA cases were located throughout hospitals and the community, were slightly more common in males than females, and occurred in all age groups, especially in the elderly. The majority of cases were inpatients in acute hospitals and were distributed across all types of wards. Most cases were colonized with MRSA but 5% of cases in the North and 10% in the South had invasive infection. Invasive infection was associated with intravascular lines and invasive procedures/surgery. Continuous surveillance is recommended to monitor the epidemiology of MRSA and the effectiveness of control measures.

Adolescent↗
PubMed2002

MRSA bacteraemia: North/South Study of MRSA in Ireland 1999.

Retrospective aggregate data on all Staphylococcus aureus isolates recovered from blood cultures during 1998 were collected in both jurisdictions on the island of Ireland, Northern Ireland (North) and the Republic of Ireland (South), as part of the North/South Study of MRSA in Ireland 1999. A postal questionnaire was used to gather the data, and all diagnostic microbiology laboratories in the North and 98% of laboratories in the South participated. S. aureus bacteraemia occurred at rates of 20.4 per 100,000 population in the North and 24.5 per 100,000 in the South (missing data from one laboratory). In the North, 22% of patients who had blood cultures positive for S. aureus had methicillin-resistant S. aureus (MRSA) and 25% of S. aureus isolates were MRSA (some patients had more than one isolate). In the South, 31% of patients who had blood cultures positive for S. aureus had MRSA and 36% of S. aureus isolates were MRSA. There was a marked variation in rates between different regions. The percentage of patients with blood cultures positive for S. aureus that had MRSA was considerably lower in the North (22%) than in the South (31%), and in both jurisdictions was lower than that found in England and Wales in 1999 (37%). It is recommended that data on S. aureus bacteraemia and methicillin-resistance rates (already available in many laboratories) are gathered at regional and national level for the surveillance of antimicrobial resistance.

Bacteremia↗
PubMed2001

The North/South Ireland Food Consumption Survey: vitamin intakes in 18-64-year-old adults.

OBJECTIVE: To estimate vitamin intakes and assess the contribution of different food groups to vitamin intakes in adults aged 18-64 years in Ireland as estimated in the North/South Ireland Food Consumption Survey. Intakes are reported for retinol, carotene, total vitamin A, vitamin D, vitamin E, thiamin, riboflavin, pre-formed niacin, total niacin equivalents, vitamin B6, vitamin B12, folate, biotin, pantothenate and vitamin C. The adequacy of vitamin intakes in the population and the risk of occurrence of excessive vitamin intakes are also assessed. DESIGN: Food consumption was estimated using a 7-day food diary for a representative sample (n=1379; 662 men and 717 women) of 18-64-year-old adults in the Republic of Ireland and Northern Ireland selected randomly from the electoral register. Vitamin intakes were estimated using tables of food composition. RESULTS: In general, the percentage of the population with vitamin intakes below the average requirement (AR) was low. Mean daily intake of total vitamin A was below the AR in 20.2% and 16.6% of men and women, respectively, and mean daily intake of riboflavin was below the AR in 12.5% and 20.6% of men and women, respectively. Mean daily folate intakes were below the AR for folate in 11.2% and 6.6% of women aged 18-35 years and 36-50 years, respectively. Only 2.2% of women aged 18-35 years and 5.2% of women aged 36-50 years achieved the recommended folate intake of 600 microg day(-1) for women of reproductive age for the prevention of neural tube defects. A high proportion of the population has a low dietary vitamin D intake and is largely dependent on sunlight exposure to maintain adequate vitamin D status. Except for pre-formed niacin, the 95th percentile intake of vitamins did not exceed the tolerable upper intake level (UL) for any group and was much less than the UL for most vitamins. Although 20.8% of men and 6.3% of women exceeded the UL for pre-formed niacin (which is 35 mg, based on nicotinic-acid-induced flushing), the large contribution of meat and fish to the intake of niacin (as nicotinamide) suggests that the risk of overexposure to nicotinic acid is much lower than this and is probably solely related to supplement use. A small proportion of men (4.0%) and women (1.2%) aged 51-64 years had retinol intakes that exceeded the UL (3000 microg) and while the 95th percentile intake of women in the 18-50 year age group was well below the UL, 1.5% of 18-35-year-old and 2.4% of 36-50-year-old women had mean daily retinol intakes above the UL. About 2.0% of women had intakes of vitamin B6 that exceeded the UL (25 mg). There were significant differences by age and sex in nutrient densities of vitamin intakes between men and women and between age groups, which may be explained by differences in consumption of particular food groups as well as different patterns of supplement use. CONCLUSION: Nutritional adequacy of the population for most vitamins was good. Folate intake in women of childbearing age is not meeting current recommendations for the prevention of neural tube defects. The public health significance of the relatively high proportion of men and women with inadequate intakes of vitamin A and riboflavin and with low dietary intakes of vitamin D is unclear and should be investigated further. With the possible exception of niacin (flushing) and vitamin B6 (neuropathy), there appears to be little risk of the occurrence of adverse effects due to excessive consumption of vitamins in this population, based on current dietary practices.

Adolescent↗
PubMed2002

The prevalence of "pure" autosomal dominant hereditary spastic paraparesis in the island of Ireland.

OBJECTIVES: Hereditary spastic paraparesis (HSP) is clinically and genetically heterogeneous. "Pure" autosomal dominant (AD) HSP is most common and eight genetic loci are identified to date. Previous studies have included autosomal recessive and sporadic cases in prevalence calculations. This study aimed to determine the prevalence and features of pure ADHSP in the island of Ireland. METHODS: Index cases were identified from a 5 year survey of all adult and paediatric neurologists, clinical geneticists, hospital records, and hospital inpatient enquiry system data in Ireland, north and south. Families were examined by two neurologists and classified as affected or unaffected according to specific criteria. The prevalence date was set at 1 June 2000 and the midyear population estimate for the previous year was 5.436 million. RESULTS: Eighty two patients with pure ADHSP and a further 12 obligate carriers from 19 families were identified. In total 69 patients with pure ADHSP were alive and resident in Ireland at the prevalence date. Twenty nine per cent of these were asymptomatic but with signs of paraparesis. CONCLUSIONS: The prevalence of ADHSP in Ireland to estimated to be 1.27/100 000 population. The high proportion of asymptomatic cases and obligate carriers means that this condition is likely to be underdiagnosed.

Adolescent↗
PubMed2000

Investigation of a cluster of children with Down's syndrome born to mothers who had attended a school in Dundalk, Ireland.

OBJECTIVES: To investigate a reported cluster of Down's syndrome in offspring of former pupils of a girls' school in Ireland, to establish the prevalence of Down's syndrome among live births in the area around the school, and to review the literature on the possible causes of reported clusters of Down's syndrome. METHODS: Questionnaire survey of obstetric and personal histories of women who had attended the girls' school at Dundalk, County Louth, Republic of Ireland, at some time during 1956-7, and also of women who had attended another, nearby, girls' school during the same period. Comparison of observed numbers of cases of Down's syndrome identified by these surveys with maternal age adjusted expected numbers for the reported live births. Laboratory tests were conducted to verify and characterise the cases of Down's syndrome constituting the cluster. Retrospective collection and collation of data on Down's syndrome occurring among live births, and the compilation of maternal age specific incidences, in County Louth and in Newry and Mourne District in neighbouring Northern Ireland, during 1961-80. These rates were compared with reference rates and rates for other areas of Ireland. RESULTS: Six children with Down's syndrome were confirmed among 387 reported live births to women who had been pupils at the girls' school in Dundalk during 1956-7, compared with 0.69 expected (nominal p<10(-4)). Five of the affected births were to mothers under 30 years of age, against 0.15 expected (nominal p<10(-6)), although only four of these mothers were attending the school at any one time. The origin of the non-disjunction was found to be maternal first meiotic in four children, mitotic after fertilisation in another (with the youngest mother), and in the remaining one could not be determined. The marked excess of Down's syndrome in births to young mothers did not extend to offspring of former pupils of the other Dundalk girls' school surveyed, or to live births in County Louth generally or in adjacent Newry and Mourne District. CONCLUSION: A striking, highly localised, excess of Down's syndrome in births to young mothers who had attended a girls' school in Dundalk during 1956-57 has been confirmed. However, not all of the mothers of the affected children attended the school concurrently and the origin of non-disjunction in one child was an error occurring after conception. Some exposure essentially confined to girls attending the school at this time is a possible, although unlikely, explanation, but a review of potential risk factors does not suggest what this could be. Previous suggestions that an influenza epidemic or contamination from the Windscale nuclear reactor fire might be implicated, both of which occurred in October 1957, can be effectively dismissed because three of the women with affected offspring had left the school by then and had moved away from Dundalk, and Down's syndrome in the child of another mother originated in an error after fertilisation. Owing to the retrospective nature of the investigation and the characteristics of the cases, chance is the most likely explanation for the cluster.

Adult↗
PubMed1993

Extended major histocompatibility complex haplotypes in celiac patients in the west of Ireland.

The highest reported prevalence of celiac disease (gluten-sensitive enteropathy) is found in the West of Ireland. Recent genetic data have suggested that major histocompatibility complex-linked loci may have a dominant genetic effect for disease susceptibility in this population compared with a recessive effect in other groups. To further understand the role of the MHC in celiac disease in the West of Ireland, we analyzed markers for 22 MHC haplotypes from celiac patients and compared them with 18 nontransmitted haplotypes found in the parents of celiac children, and with reported haplotypes from other populations. An extended MHC haplotype including [HLA-B8, DR3, DQw2, Bf*S, C4A*Q0, and C4B*1] accounted for 50% of celiac haplotypes but only 27% of nontransmitted parental haplotypes. Compared with other reported haplotypes in celiacs, patients from the West of Ireland show a higher prevalence of HLA-A1 as a component of this extended haplotype, suggesting that although the core haplotype is similar between Irish patients and others, the celiac population in the West of Ireland differs at other HLA loci. We did not observe any other common haplotypes among our patients unlike the situation in other populations. These differences may underlie the possible dominant effect of HLA-linked loci and the unusually high prevalence of celiac disease in the Irish population. We also found that the serum levels of complement components C3c, C4, and factor B were significantly lower among celiac patients than nonceliacs. The lower serum level of C4 appears to be related to the presence of deletions and null alleles at the C4A and C4B loci in celiacs.

Adult↗