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Case Ascertainment (case + ascertainment)
Selected AbstractsCase ascertainment and estimated incidence of drug-induced long-QT syndrome: study in Southwest FranceBRITISH JOURNAL OF CLINICAL PHARMACOLOGY, Issue 3 2008Mariam Molokhia WHAT IS ALREADY KNOWN ABOUT THIS SUBJECT , Drug-induced long-QT syndrome (LQTS) is a potentially fatal condition that has led to a number of postmarketing withdrawals in recent years. , However, many cases may not survive long enough to reach hospital, and only a small proportion are reported to pharmacovigilance agencies. , The extent to which genetic determinants of susceptibility to LQTS are specific to particular drugs, or common to several classes of drug, remains to be determined. WHAT THIS STUDY ADDS , We estimated population prevalence of drug-induced LQTS in the Midi-Pyrenees region, southwest France, using five different institutions and assessed feasibility of tracing potential cases (in addition to pharmacovigilance data), using hospital data and rigorous case definition. , These methods can be adapted to a wider region, used to augment pharmacovigilance reporting, and offer researchers the opportunity to study genetic susceptibility to drug-induced LQTS. AIMS The aim of this study was to investigate the incidence and reporting rate of drug-induced long-QT syndrome (LQTS) in France [defined by evidence of torsades de pointes (TdP), QT prolongation and exposure to a relevant drug] and to assess feasibility of case collection for drug-induced LQTS. METHODS A retrospective population-based study was carried out in Southwest France in five institutions: three main hospitals, one private clinic and one cardiac emergency unit, searched from 1 January 1999 to 1 January 2005 (population coverage of 614 000). The study population consisted of 861 cases with International Classification of Diseases-10 diagnostic codes for ventricular tachycardia (I147.2), ventricular fibrillation (I149.0) and sudden cardiac death (I146.1) from hospital discharge summaries, supplemented by cases reported to national or regional pharmacovigilance systems, and voluntary reporting by physicians, validated according to internationally defined criteria for drug-induced LQTS. RESULTS Of 861 patients coded with arrhythmias or sudden cardiac death, there were 40 confirmed surviving acquired cases of drug-induced LQTS. We estimated that the incidence of those who survive to reach hospital drug-induced LQTS is approximately 10.9 per million annually in France (95% confidence interval 7.8, 14.8). CONCLUSIONS Many cases of drug-induced LQTS may not survive before they reach hospital, as the reporting rate for drug-induced LQTS identified through the cardiology records and also reported to pharmacovigilance systems for the Midi-Pyrenees area is 3/40 (7.5%). Using the methods outlined it is possible to assemble cases to study genetic susceptibility to drug-induced LQTS and adapt these methods more widely. [source] Prevalence and epidemiologic characteristics of FASD from various research methods with an emphasis on recent in-school studiesDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 3 2009Philip A. May Abstract Researching the epidemiology and estimating the prevalence of fetal alcohol syndrome (FAS) and other fetal alcohol spectrum disorders (FASD) for mainstream populations anywhere in the world has presented a challenge to researchers. Three major approaches have been used in the past: surveillance and record review systems, clinic-based studies, and active case ascertainment methods. The literature on each of these methods is reviewed citing the strengths, weaknesses, prevalence results, and other practical considerations for each method. Previous conclusions about the prevalence of FAS and total FASD in the United States (US) population are summarized. Active approaches which provide clinical outreach, recruitment, and diagnostic services in specific populations have been demonstrated to produce the highest prevalence estimates. We then describe and review studies utilizing in-school screening and diagnosis, a special type of active case ascertainment. Selected results from a number of in-school studies in South Africa, Italy, and the US are highlighted. The particular focus of the review is on the nature of the data produced from in-school methods and the specific prevalence rates of FAS and total FASD which have emanated from them. We conclude that FAS and other FASD are more prevalent in school populations, and therefore the general population, than previously estimated. We believe that the prevalence of FAS in typical, mixed-racial, and mixed-socioeconomic populations of the US is at least 2 to 7 per 1,000. Regarding all levels of FASD, we estimate that the current prevalence of FASD in populations of younger school children may be as high as 2,5% in the US and some Western European countries. © 2009 Wiley-Liss, Inc. Dev Disabil Res Rev 2009; 15:176,192. [source] The changing prevalence of diagnosed diabetes and its associated vascular complications in a large region of the UK*DIABETIC MEDICINE, Issue 6 2010C. L. Morgan Diabet. Med. 27, 673,678 (2010) Abstract Aims, To characterize the prevalence of diabetes in a large health district in 2004 and compare it with a previous estimate made in 1996. Methods, The study population comprised the resident population of Cardiff and the Vale of Glamorgan. Routine record linkage was used to identify patients from various sources of hospital and mortality data. Patients with diabetes were identified according to biochemistry test results, coding on routine data or attendance at a diabetes-related clinic. Diabetes-related complications were ascribed according to coding on routine data. Results, It was possible to identify 17 088 people with diabetes alive on 1 January 2005. Of these patients, 9064 (53.0%) were male and 8024 (47.0%) were female. Mean age (± sd) was 59.6 ± 18.9 years for males and 61.2 ± 20.4 years for females. The crude prevalence of diabetes in 2005 was 3.9% (3.4% adjusted) compared with 2.5% in 1996 (2.3% adjusted). With the exception of females aged , 75 years, the prevalence of diabetes increased in all age- and sex-specific subgroups. Within the 2005 cohort, over two-thirds has no recorded complications compared with approximately one half of the 1996 cohort. The prevalence of individual complications decreased, with the exception of renal complications. Conclusions, The prevalence of identified diabetes appears to have increased substantially over a relatively short period of 9 years to 2004. The increase in prevalence was 46%, with an increase in numbers of patients with diabetes of 53%. A number of factors are likely to have contributed to this, including an increase in case ascertainment. [source] A national stroke quality register: 12 years experience from a participating hospitalEUROPEAN JOURNAL OF NEUROLOGY, Issue 8 2007P. Appelros Registration of all hospitalized stroke patients is practiced in Sweden in order to assess care quality. Data in this register, Riks-Stroke (RS), may be biased due to incomplete registration. The purpose of this paper was to report changes in stroke outcome in relation to fluctuations in registration. Patients registered in RS at a hospital during the period 1994,2005 were analyzed. Case fatality at 28 days, living conditions, and activities of daily living (ADL) performance at 3 months were correlated to the number of patients registered and follow-up frequency. A total of 4994 stroke cases were registered during the period. A high annual registration rate was significantly correlated to a high case fatality ratio. A low annual follow-up rate was associated with a low proportion of patients living in their own home without any need of help. Quality parameters are sensible for selection bias, which make them difficult to compare over time and between hospitals. We suggest that by weighing outcome data against stroke severity, safer conclusions may be drawn. Additionally, hospitals considering setting up quality registers should make every effort to attain complete case ascertainment at all times, including patients managed outside the hospital, in order to avoid selection bias. [source] A two-fold difference in the age-adjusted prevalences of Parkinson's disease between the island of Als and the Faroe IslandsEUROPEAN JOURNAL OF NEUROLOGY, Issue 6 2000L. Wermuth With the aim of comparing the previously found high prevalence of idiopathic Parkinson's disease (PD) in the Faroe Islands with the prevalence of PD in an area of Denmark, we used the same case-finding methods for case ascertainment and the same strict criteria to diagnose PD on the island of Als. During the last year before the prevalence date (1 January 1998), we found in various registries from pharmacies, hospital, private neurologist and general practioners 121 patients with suspected Parkinsonism out of 56 839 inhabitants on the island of Als. After exclusion of those who had other diseases, a total of 79 patients were left for further examinations. Among these we found 58 with PD. The overall prevalence of PD was estimated to be 102.0 and the age-adjusted prevalence to be 98.3 per 100 000 persons compared with 187.6 and 209.0 in the Faroe Islands. Compared with the previous results from the Faroe Islands (prevalence date 1 July 1995) we found an even lower mean age at onset of PD symptoms and at onset of treatment, a lower proportion of definite PD and a lower average dose of levodopa. We therefore conclude that the two-fold higher prevalence in the Faroe Islands than on the island of Als was not due to an early diagnosis and a higher ascertainment of cases with mild PD, which was suggested as being one possible explanation for our previous finding of a high prevalence of PD in the Faroe Islands. [source] Trends in childhood acute lymphoblastic leukemia in Western Australia, 1960,2006INTERNATIONAL JOURNAL OF CANCER, Issue 5 2008Elizabeth Milne Abstract Increases in the incidence of childhood acute lymphoblastic leukemia (ALL) have been reported in some countries, while other reports from similar geographical regions have indicated stable rates. The reasons for the discrepancies have been debated in the literature, with the focus on whether the observed increases are "real" or an artifact resulting from improvements in diagnosis, case ascertainment and population coverage over time. We used population-based data from Western Australia to investigate trends in the incidence of childhood ALL between 1960 and 2006. Age-standardized incidence rates (ASRs) and rate ratios (indicating annual percent change) were estimated using Poisson regression. Between 1960 and 2006, the ASR was 3.7 per 100,000 person-years, with an annual percent increase of 0.40% (95% CI: ,0.20, 1.00). Between 1982 and 2006, the ASR was 3.8, with an annual percent increase of 0.80% (95% CI = ,0.70 to 2.30). This increased to 1.42% (95% CI: ,0.30, 3.0) when a sensitivity analysis was undertaken to assess the effect of excluding the final 2 years of data. Annual increases of 3.7% (95% CI: ,0.50, 8.00) among children aged 5,14 years, and of 3.10% (95% CI: 0.50, 5.70) in girls, were observed for this latter period. These results were supported by national Australian incidence data available for 1982,2003. There may have been a small increase in the incidence of ALL since 1982 among girls and older children, but an overall increase appears unlikely. No impact of folate supplementation or fortification is apparent. © 2007 Wiley-Liss, Inc. [source] Association between pacifier use and breast-feeding, sudden infant death syndrome, infection and dental malocclusionINTERNATIONAL JOURNAL OF EVIDENCE BASED HEALTHCARE, Issue 6 2005Ann Callaghan RN RM BNurs(Hons) Executive summary Objective, To critically review all literature related to pacifier use for full-term healthy infants and young children. The specific review questions addressed are: What is the evidence of adverse and/or positive outcomes of pacifier use in infancy and childhood in relation to each of the following subtopics: ,breast-feeding; ,sudden infant death syndrome; ,infection; ,dental malocclusion. Inclusion criteria, Specific criteria were used to determine which studies would be included in the review: (i) the types of participants; (ii) the types of research design; and (iii) the types of outcome measures. To be included a study has to meet all criteria. Types of participants,The participants included in the review were healthy term infants and healthy children up to the age of 16 years. Studies that focused on preterm infants, and infants and young children with serious illness or congenital malformations were excluded. However, some total population studies did include these children. Types of research design, It became evident early in the review process that very few randomised controlled trials had been conducted. A decision was made to include observational epidemiological designs, specifically prospective cohort studies and, in the case of sudden infant death syndrome research, case,control studies. Purely descriptive and cross-sectional studies were excluded, as were qualitative studies and all other forms of evidence. A number of criteria have been proposed to establish causation in the scientific and medical literature. These key criteria were applied in the review process and are described as follows: (i) consistency and unbiasedness of findings; (ii) strength of association; (iii) temporal sequence; (iv) dose,response relationship; (v) specificity; (vi) coherence with biological background and previous knowledge; (vii) biological plausibility; and (viii) experimental evidence. Studies that did not meet the requirement of appropriate temporal sequencing of events and studies that did not present an estimate of the strength of association were not included in the final review. Types of outcome measures,Our specific interest was pacifier use related to: ,breast-feeding; ,sudden infant death syndrome; ,infection; ,dental malocclusion. Studies that examined pacifier use related to procedural pain relief were excluded. Studies that examined the relationship between pacifier use and gastro-oesophageal reflux were also excluded as this information has been recently presented as a systematic review. Search strategy, The review comprised published and unpublished research literature. The search was restricted to reports published in English, Spanish and German. The time period covered research published from January 1960 to October 2003. A protocol developed by New Zealand Health Technology Assessment was used to guide the search process. The search comprised bibliographic databases, citation searching, other evidence-based and guidelines sites, government documents, books and reports, professional websites, national associations, hand search, contacting national/international experts and general internet searching. Assessment of quality, All studies identified during the database search were assessed for relevance to the review based on the information provided in the title, abstract and descriptor/MeSH terms, and a full report was retrieved for all studies that met the inclusion criteria. Studies identified from reference list searches were assessed for relevance based on the study title. Keywords included: dummy, dummies, pacifier(s), soother(s), comforter(s), non-nutritive sucking, infant, child, infant care. Initially, studies were reviewed for inclusion by pairs of principal investigators. Authorship of articles was not concealed from the reviewers. Next, the methodological quality of included articles was assessed independently by groups of three or more principal investigators and clinicians using a checklist. All 20 studies that were accepted met minimum set criteria, but few passed without some methodological concern. Data extraction, To meet the requirements of the Joanna Briggs Institute, reasons for acceptance and non-acceptance at each phase were clearly documented. An assessment protocol and report form was developed for each of the three phases of review. The first form was created to record investigators' evaluations of studies included in the initial review. Those studies that failed to meet strict inclusion criteria were excluded at this point. A second form was designed to facilitate an in-depth critique of epidemiological study methodology. The checklist was pilot tested and adjustments were made before reviewers were trained in its use. When reviewers could not agree on an assessment, it was passed to additional reviewers and discussed until a consensus was reached. At this stage, studies other than cohort, case,control and randomised controlled trials were excluded. Issues of clarification were also addressed at this point. The final phase was that of integration. This phase, undertaken by the principal investigators, was assisted by the production of data extraction tables. Through a process of trial and error, a framework was formulated that adequately summarised the key elements of the studies. This information was tabulated under the following headings: authors/setting, design, exposure/outcome, confounders controlled, analysis and main findings. Results, With regard to the breast-feeding outcome, 10 studies met the inclusion criteria, comprising two randomised controlled trials and eight cohort studies. The research was conducted between 1995 and 2003 in a wide variety of settings involving research participants from diverse socioeconomic and cultural backgrounds. Information regarding exposure and outcome status, and potential confounding factors was obtained from: antenatal and postnatal records; interviews before discharge from obstetric/midwifery care; post-discharge interviews; and post-discharge postal and telephone surveys. Both the level of contact and the frequency of contact with the informant, the child's mother, differed widely. Pacifier use was defined and measured inconsistently, possibly because few studies were initiated expressly to investigate its relationship with breast-feeding. Completeness of follow-up was addressed, but missing data were not uniformly identified and explained. When comparisons were made between participants and non-participants there was some evidence of differential loss and a bias towards families in higher socioeconomic groups. Multivariate analysis was undertaken in the majority of studies, with some including a large number of sociodemographic, obstetric and infant covariates and others including just maternal age and education. As might be expected given the inconsistency of definition and measurement, the relationship between pacifier use and breast-feeding was expressed in many different ways and a meta-analysis was not appropriate. In summary, only one study did not report a negative association between pacifier use and breast-feeding duration or exclusivity. Results indicate an increase in risk for a reduced overall duration of breast-feeding from 20% to almost threefold. The data suggest that very infrequent use may not have any overall negative impact on breast-feeding outcomes. Six sudden infant death syndrome case,control studies met the criteria for inclusion. The research was conducted with information gathered between 1984 and 1999 in Norway, UK, New Zealand, the Netherlands and USA. Exposure information was obtained from a variety of sources including: hospital and antenatal records, death scene investigation, and interview and questionnaire. Information for cases was sought within 2 days after death, within 2,4 weeks after death and in one study between 3 and 11 years after death. Information for controls was sought from as early as 4 days of a nominated sudden infant death syndrome case, to between 1 and 7 weeks from the case date, and again in one study some 3,11 years later. In the majority of the studies case ascertainment was determined by post-mortem. Pacifier use was again defined and measured somewhat inconsistently. All studies controlled for confounding factors by matching and/or using multivariate analysis. Generally, antenatal and postnatal factors, as well as infant care practices, and maternal, family and socioeconomic issues were considered. All five studies reporting multivariate results found significantly fewer sudden infant death syndrome cases used a pacifier compared with controls. That is, pacifier use was associated with a reduced incidence of sudden infant death syndrome. These results indicate that the risk of sudden infant death syndrome for infants who did not use a pacifier in the last or reference sleep was at least twice, and possibly five times, that of infants who did use a pacifier. Three studies reported a moderately sized positive association between pacifier use and a variety of infections. Conversely, one study found no positive association between pacifier use at 15 months of age and a range of infections experienced between the ages of 6 and 18 months. Given the limited number of studies available and the variability of results, no meaningful conclusions could be drawn. Five cohort studies and one case,control study focused on the relationship between pacifier use and dental malocclusion. Not one of these studies reported a measure of association, such as an estimate of relative risk. It was therefore not possible to include these studies in the final review. Implications for practice, It is intended that this review be used as the basis of a ,best practice guideline', to make health professionals aware of the research evidence concerning these health and developmental consequences of pacifier use, because parents need clear information on which they can base child care decisions. With regard to the association between pacifier use and infection and dental malocclusion it was found that, due to the paucity of epidemiological studies, no meaningful conclusion can be drawn. There is clearly a need for more epidemiological research with regard to these two outcomes. The evidence for a relationship between pacifier use and sudden infant death syndrome is consistent, while the exact mechanism of the effect is not well understood. As to breast-feeding, research evidence shows that pacifier use in infancy is associated with a shorter duration and non-exclusivity. It is plausible that pacifier use causes babies to breast-feed less, but a causal relationship has not been irrefutably proven. Because breast-feeding confers an important advantage on all children and the incidence of sudden infant death syndrome is very low, it is recommended that health professionals generally advise parents against pacifier use, while taking into account individual circumstances. [source] The epidemiologic, health-related quality of life, and economic burden of gastrointestinal stromal tumoursJOURNAL OF CLINICAL PHARMACY & THERAPEUTICS, Issue 6 2007P. Reddy PharmD Summary Background and objectives:, Gastrointestinal stromal tumours (GIST) are uncommon tumours believed to arise from interstitial cells of Cajal or their precursors in the gastrointestinal (GI) tract, accounting for a small percentage of GI neoplasms and sarcomas. Given the recent recognition of GIST as a distinct cancer, as well as new treatment options available today, a review of the epidemiologic, health-related quality of life (HRQL), and economic burden of GIST is timely from a payer, provider and patient perspective and may provide guidance for treatment decision making and reimbursement. Methods:, A systematic literature review of PubMed and five scientific meeting databases, was conducted to identify published studies and abstracts describing the epidemiologic, HRQL and economic impact of GIST. Publications deemed worthy of further review, based on the information available in the abstract, were retrieved in full text. Results and discussion:, Thirty-four publications met the review criteria: 29 provided data on GIST epidemiology, one provided cost data, three reported HRQL outcomes, and one reported cost and HRQL outcomes. The annual incidence of GIST (cases per million) ranged from 6·8 in the USA to 14·5 in Sweden, with an estimated 5-year survival rate of 45,64%. On the Functional Illness of Chronic Therapy-fatigue instrument, GIST patients scored 40·0 compared with 37·6 in anaemic cancer patients (0 = worst; 52 = least fatigue). Total costs over 10 years for managing GIST patients with molecularly targeted treatment was estimated at £47 521,£56 146 per patient compared with £4047,£4230 per patient with best supportive care. Conclusions:, The incidence of GIST appears to be similar by country; the lower estimate in one country could be explained by differences in method of case ascertainment. Data suggest that the HRQL burden of GIST is similar to that with other cancers although this requires further exploration. The value of new therapies in GIST needs to consider not only cost but also anticipated benefits and the unmet medical need in this condition. [source] Notification of patients with acute flaccid paralysis since certification of Australia as polio-freeJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 8 2004K Whitfield Objective: Surveillance of patients presenting with acute flaccid paralysis (AFP) is the World Health Organization (WHO) recommended method for the detection of incident cases of poliovirus infection. Australia was certified free of circulating poliovirus in 2000 but is required to continue AFP surveillance until global certification. Although Australia reached the WHO nominated surveillance target in 2000 and 2001, it was not reached in 2002. Notification rates between states have been variable. We aim to investigate the difference in notification rates by state to determine whether different rates reflect different patterns of disease or different approaches to reporting. Methods: Notification rates were reviewed by state for the years 1997,2002. The completeness of case ascertainment was reviewed from published studies. Key informants described differences in AFP reporting in states with consistent differences in notification rates. Results: Australia achieved 75% of the WHO surveillance target for AFP cases between 1997 and 1999 and 98% between 2000 and 2002. After 2000, Queensland achieved 150% of its target while Victoria achieved less than 50%. New South Wales reached its target over the entire 6 years but other states and territories were not as consistent. Although the formal process for AFP reporting is uniform throughout Australia, many differences in approach were identified between Victoria and Queensland. Conclusion: Maintaining AFP surveillance at the required WHO standard will be more likely in Australia if the populous states are able to notify cases at the same rate as Queensland (since 2000) and New South Wales (in general). [source] Public health projects for preventing the recurrence of neural tube defects in the United States,,BIRTH DEFECTS RESEARCH, Issue 11 2009Julianne S. Collins Abstract BACKGROUND: The recurrence risk for neural tube defects (NTDs) in subsequent pregnancies is approximately 3%, or 40 times the background risk. Prevention projects target these high-risk women to increase their folic acid consumption during the periconceptional period, a behavior which decreases their recurrence risk by at least 85%. This study surveyed birth defect surveillance programs to assess their NTD recurrence prevention activities and to identify components of intervention projects that might be implemented in states with limited resources. METHODS: In 2005, the National Birth Defects Prevention Network developed and distributed an online survey to primary state birth defects surveillance contacts for the purpose of gathering information on NTD recurrence prevention activities in the United States. RESULTS: Responses came from 37 contacts in 34 states and Puerto Rico. There were 13 active NTD recurrence prevention projects, four past projects, and three planned projects. Fifteen past and present projects recommended that women with a prior NTD-affected birth take 4.0 mg of folic acid daily, and four projects provided folic acid to the women. Reasons given for not having an NTD recurrence prevention project included staffing limitations (53%), lack of funds (47%), lack of priority (18%), and confidentiality/privacy concerns (6%). CONCLUSIONS: Only 15 states and Puerto Rico had or were planning NTD recurrence prevention projects. An NTD recurrence prevention project using minimal resources should consist of timely case ascertainment, educational materials, and mechanisms for disseminating these materials. Birth Defects Research (Part A), 2009. © 2009 Wiley-Liss, Inc. [source] Prenatal diagnosis, pregnancy terminations and prevalence of Down syndrome in Atlanta,BIRTH DEFECTS RESEARCH, Issue 9 2004Csaba Siffel Abstract BACKGROUND The impact of prenatal diagnosis on the live birth prevalence of Down syndrome (trisomy 21) has been described. This study examines the prevalence of Down syndrome before (1990,1993) and after inclusion of prenatally diagnosed cases (1994,1999) in a population-based registry of birth defects in metropolitan Atlanta. METHODS We identified infants and spontaneous fetal deaths with Down syndrome (n = 387), and pregnancies electively terminated after a prenatal diagnosis of Down syndrome (n = 139) from 1990 to 1999 among residents of metropolitan Atlanta from a population-based registry of birth defects, the Metropolitan Atlanta Congenital Defects Program (MACDP). Only diagnoses of full trisomy 21 were included. Denominator information on live births was derived from State of Georgia birth certificate data. We compared the prevalence of Down syndrome by calendar period (1990,1993, 1994,1999), maternal age (<35 years, 35+ years), and race/ethnicity (White, Black, other), using chi-square and Fisher's exact tests. RESULTS During the period when case ascertainment was based only on hospitals (1990,1993), the prevalence of Down syndrome was 8.4 per 10,000 live births when pregnancy terminations were excluded and 8.8 per 10,000 when terminations were included. When case ascertainment also included perinatal offices (1994,1999), the prevalence of Down syndrome was 10.1 per 10,000 when terminations were excluded and 15.3 when terminations were included. During 1990,1993, the prevalence of Down syndrome was 24.7 per 10,000 among offspring to women 35+ years of age compared to 6.8 per 10,000 among offspring to women <35 years of age (rate ratio [RR] = 3.65, 95% confidence interval [CI] = 2.53,5.28). During 1994,1999, the prevalence of Down syndrome was 55.3 per 10,000 among offspring to women 35+ years compared to 8.5 per 10,000 among offspring to women <35 years (RR = 6.55, 95% CI = 5.36,7.99). There was no statistically significant variation in the prevalence of Down syndrome by race/ethnicity within maternal age and period of birth strata. During 1994,1999, the proportion of cases that were electively terminated was greater for women 35+ years compared to women <35 years (RR = 5.10, 95% CI = 3.14,8.28), and lower for Blacks compared to Whites among women 35+ years of age (RR = 0.33, 95% CI = 0.16,0.66). CONCLUSIONS In recent years, perinatal offices have become an important source of cases of Down syndrome for MACDP, contributing at least 34% of cases among pregnancies in women 35+ years of age. Variation in the prevalence of Down syndrome by race/ethnicity, before or after inclusion of cases ascertained from perinatal offices, was not statistically significant. Among Down syndrome pregnancies in mothers 35+ years we found a lower proportion of elective termination among Black women compared to White women. We suggest that future reports on the prevalence of Down syndrome by race/ethnicity take into account possible variations in the frequency of prenatal diagnosis or elective termination by race/ethnicity. Birth Defects Research (Part A) 70565,571, 2004. Published 2004 Wiley-Liss, Inc. [source] Pathologic Correlates of Dementia in Individuals with Lewy Body DiseaseBRAIN PATHOLOGY, Issue 3 2010Joshua A. Sonnen Abstract Cognitive impairment and dementia are more common in patients with Parkinson disease (PD) than age-matched controls and appear to become more frequent as PD progresses. However, estimates of dementia in patients with PD have varied widely, likely due in part to differences in case definition, case ascertainment and methodology. First, we review investigations of usual pathologic correlates of dementia in patients with brainstem (b) Lewy Body Disease (LBD) and report our findings from the initial 266 brain autopsies from a population-based study of brain aging and incident dementia. Our results showed that 2.6% of subjects were diagnosed with PD during life but that 20% had bLBD at autopsy. Seventy percent of individuals with bLBD had high level of one or more cerebral pathologic changes significantly associated with dementia: Alzheimer's disease (AD), cerebral (c) LBD or microvascular brain injury (µVBI); these were commonly co-morbid. Next we consider proposed contributors to cognitive impairment and dementia in the approximately 30% of patients with only bLBD, including regionally selective dendritic degeneration of neostriatal medium spiny neurons. Diseases contributing to cognitive impairment and dementia in patients with bLBD are heterogeneous, providing diagnostic challenges as well as multiple opportunities for successful intervention in patients with PD. [source] Prevalence of multiple sclerosis in Västerbotten County in northern SwedenACTA NEUROLOGICA SCANDINAVICA, Issue 4 2001P. Sundström Objective , To estimate the prevalence and clinical characteristics of multiple sclerosis (MS) in Västerbotten County in northern Sweden. Methods,Individuals with MS were identified from several sources. A follow-up interview and/or examination was performed in 94% of cases still living in the area during 1997,99. Onset adjusted prevalence and a definition of onset symptoms were applied. Results, A total of 313 cases were identified, resulting in an onset adjusted crude prevalence of MS for January 1990 of 125/105 (95% confidence interval (CI): 112,140). Female predominance was evident (163/105 (95% CI: 142,187) vs 86/105 (95% CI: 71,104)). Diagnostic coding registers were the most important source for identification of cases. Conclusions, The crude prevalence of MS in Västerbotten was higher than previous reports from other major areas in Scandinavia. The adjusted prevalence was significantly higher when compared with a previous study from Göteborg, south-western Sweden. The methodology used in this study gives a high degree of case ascertainment and increases the comparability of multiple sclerosis epidemiological studies. [source] Congenital diaphragmatic hernia: review of the literature in reflection of unresolved dilemmasACTA PAEDIATRICA, Issue 12 2009S Grisaru-Granovsky Abstract Background:, Congenital diaphragmatic hernia (CDH) is a rare but clinically and scientifically challenging condition. The introduction of ultrasound has enabled early prenatal detection and consequently, hope of early therapeutic intervention. Aim:, We undertook the task to review the recent developments in understanding the pathology of CDH as well as the history and current management strategies to aid perinatologists in consultations with parents of CDH-affected foetuses. Study design:, A Medline search was undertaken of all reports and reviews published between 1980 and 2008 using MeSH search terms ,diaphragmatic hernia', ,congenital' and ,newborn'. Results:, The true incidence of CDH is still difficult to estimate because of the high incidence of hidden mortality of CDH. Complete case ascertainment also poses difficulties in assessment of the impact of new therapeutic modalities on overall survival. Recent improvements in prenatal detection are a milestone in affording time for re-assessments and parental counselling. The true benefit of antenatal therapy is circumscribed and should be offered only in selected cases of isolated severe CDH as defined by existing guidelines. Postnatal intensive respiratory supportive therapy and innovative surgical techniques within specialized tertiary centres has had a major impact on survival of babies with CDH. Conclusion:, The high survival of ,selected cases' that are live births and benefit from optimal care will be difficult to improve by antenatal interventions. The multidisciplinary approach to basic research and randomized clinical trials will further define the best approach to the foetus and neonate with CDH. [source] | ||||||||||||||||||||||