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1.  Wildlife Warning Signs: Public Assessment of Components, Placement and Designs to Optimise Driver Response 
Simple Summary
Wildlife warning signs are aimed at reducing wildlife–vehicle collisions but there is little evidence that they are effective. Improving these sign designs to increase driver response may reduce wildlife–vehicle collisions. We examined drivers’ responses to different wildlife warning sign designs through a public survey. The presences of some sign components and sign position were assessed. Drivers’ responses to eight graphically displayed signs and animal- and vehicle-activated signs were ranked and participants indicated the sign to which they were most likely to respond. Three signs ranked highly. Animal- and vehicle-activated signs were also ranked highly by participants. More research into optimising wildlife warning sign designs is needed.
Wildlife warning signs are the most commonly used and widespread form of road impact mitigation, aimed at reducing the incidence of wildlife–vehicle collisions. Evidence of the effectiveness of currently used signs is rare and often indicates minimal change in driver behaviour. Improving the design of these signs to increase the likelihood of appropriate driver response has the potential to reduce the incidence of wildlife–vehicle collisions. This study aimed to examine and assess the opinions of drivers on wildlife warning sign designs through a public opinion survey. Three currently used sign designs and five alternative sign designs were compared in the survey. A total of 134 drivers were surveyed. The presence of temporal specifications and an updated count of road-killed animals on wildlife warning signs were assessed, as well as the position of the sign. Drivers’ responses to the eight signs were scaled separately at three speed limits and participants indicated the sign to which they were most likely to respond. Three signs consistently ranked high. The messages conveyed by these signs and their prominent features were explored. Animal-activated and vehicle speed-activated signs were ranked very highly by participants. Extensive field trials of various sign designs are needed to further this research into optimizing wildlife warning sign designs.
PMCID: PMC4494358  PMID: 26479756
wildlife–vehicle collisions; road signs; sign design; driver behaviour; mitigation; road ecology; urban wildlife
2.  A program for increasing manual signing by autistic and profoundly retarded youth within the daily environment 
A program was implemented to increase the manual signing of five profoundly retarded and four autistic youth within their daily environment. Each participant was nonvocal or minimally vocal. The program was based on modified incidental teaching strategies and was implemented by direct care personnel under supervision in an institutional setting. Specific components included rearranging the physical environment to prompt signing, altering routine staff-resident interactions to prompt, manually guide and/or reinforce signing; and conducting mini-training sessions. Additionally, staff modeled signs intermittently throughout the day. The program was sequentially implemented during two staff work shifts on each of two resident living modules. Observations conducted at four separate time periods during the day indicated that significant increases in signing occurred for all participating youth and that the increases generally maintained during follow-up checks at 5 and 17 weeks. Differential effects of the increased signing on frequency of vocalizations were noted across residents. A staff acceptability survey indicated favorable staff reports on the usefulness of signing to communicate with the youth. Results are discussed regarding the significance of manual signing for seriously developmentally disabled persons and the importance of ensuring that signing skills are used in the daily environment and not exclusively in formal training sessions. Also, areas for continued research are noted in terms of more refined analyses of client skills and subsequent progress in manual communication programs.
PMCID: PMC1308282  PMID: 7142061
sign language; incidental teaching; autistic children; retarded children; developmentally disabled
3.  Knowledge about Obstetric Danger Signs and Associated Factors among Mothers in Tsegedie District, Tigray Region, Ethiopia 2013: Community Based Cross-Sectional Study 
PLoS ONE  2014;9(2):e83459.
In many developing countries including Ethiopia, maternal morbidity and mortality still pose a substantial burden and thus progress towards the fifth Millennium Development Goal (MDG) remains slow. Raising awareness of women about the danger signs of pregnancy and childbirth is the first essential step in accepting appropriate and timely referral to obstetric care. However, in Ethiopia little is known about the knowledge level of mothers about obstetric danger signs. The objective of this study was to assess the status of knowledge of danger signs of pregnancy and childbirth among mothers who gave birth in the past two years prior to the survey in Tsegedie district, Tigray regional state, Ethiopia.
A Community based cross-sectional study was conducted from November 20, 2012 to June 30, 2013 on a randomly selected sample of 485 women who had at least one delivery in the past two years. Multistage sampling technique was employed to select the study participants. A pre-tested structured questionnaire was used to collect quantitative data. Focus group discussion and in-depth interviews were utilized to supplement the Quantitative data. Bivariate and multivariate data analysis was performed using SPSS version 17.0 software.
Four hundred eighty five mothers participated in the study making a response rate of 100%. Vaginal bleeding was the most commonly mentioned danger signs of pregnancy (49.1%) and childbirth (52.8%). Two hundred eighty five (58.8%) and 299 (61.6%) of respondents mentioned at least two danger signs of pregnancy and childbirth respectively. One hundred seventy (35.1%) and 154 (31.8%) of respondents didn't know any danger signs of pregnancy and childbirth respectively. Educational status of the mother, place of delivery and having functional radio were found to be independent predictors of knowledge of women about the danger signs of pregnancy and childbirth.
Educational status of the mother, place of delivery and having functional radio were independently associated with knowledge of women about obstetric danger signs. Thus, provision of information, education and communication targeting women, family and the general community on danger signs of pregnancy and childbirth and associated factors was recommended.
PMCID: PMC3916287  PMID: 24516516
4.  Men’s Knowledge of Obstetric Danger Signs, Birth Preparedness and Complication Readiness in Rural Tanzania 
PLoS ONE  2015;10(5):e0125978.
Men’s involvement in reproductive health is recommended. Their involvement in antenatal care service is identified as important in maternal health. Awareness of obstetric danger signs facilitates men in making a joint decision with their partners regarding accessing antenatal and delivery care. This study aims to assess the level of knowledge of obstetric complications among men in a rural community in Tanzania, and to determine their involvement in birth preparedness and complication readiness.
A cross-sectional survey was conducted where 756 recent fathers were invited through a two-stage cluster sampling procedure. A structured questionnaire was used to collect socio-demographic characteristics, knowledge of danger signs and steps taken on birth preparedness and complication readiness. Data were analyzed using bivariate and multivariable logistic regression to determine factors associated with being prepared, with statistically significant level at p<0.05.
Among the invited men, 95.9% agreed to participate in the community survey. Fifty-three percent could mention at least one danger sign during pregnancy, 43.9% during delivery and 34.6% during the postpartum period. Regarding birth preparedness and complication readiness, 54.3% had bought birth kit, 47.2% saved money, 10.2% identified transport, 0.8% identified skilled attendant. In general, only 12% of men were prepared. Birth preparedness was associated with knowledge of danger signs during pregnancy (AOR = 1.4, 95% CI: 1.8-2.6). It was less likely for men living in the rural area to be prepared (AOR=0.6, 95% CI; 0.5-0.8).
There was a low level of knowledge of obstetric danger signs among men in a rural district in Tanzania. A very small proportion of men had prepared for childbirth and complication readiness. There was no effect of knowledge of danger signs during childbirth and postpartum period on being prepared. Innovative strategies that increase awareness of danger signs as well as birth preparedness and complication readiness among men are required. Strengthening counseling during antenatal care services that involve men together with partners is recommended.
PMCID: PMC4423869  PMID: 25950814
5.  The quality–coverage gap in antenatal care: toward better measurement of effective coverage 
The proportion of pregnant women receiving 4 or more antenatal care (ANC) visits has no necessary relationship with the actual content of those visits. We propose a simple alternative to measure program performance that aggregates key services that are common across countries and measured in Demographic and Health Surveys, such as blood pressure measurement, tetanus toxoid vaccination, first ANC visit before 4 months gestation, urine testing, counseling about pregnancy danger signs, and iron–folate supplementation.
The proportion of pregnant women receiving 4 or more antenatal care (ANC) visits has no necessary relationship with the actual content of those visits. We propose a simple alternative to measure program performance that aggregates key services that are common across countries and measured in Demographic and Health Surveys, such as blood pressure measurement, tetanus toxoid vaccination, first ANC visit before 4 months gestation, urine testing, counseling about pregnancy danger signs, and iron–folate supplementation.
The proportion of pregnant women receiving 4 or more antenatal care visits (ANC 4+) is used prominently as a global benchmark indicator to track maternal health program performance. This has contributed to an inappropriate focus on the number of contacts rather than on the content and process of care. This paper presents analysis of specific elements of care received among women reporting 4 or more ANC visits.
We conducted secondary analysis using Demographic and Health Survey data from 41 countries to determine coverage for specific elements of antenatal care. The analysis was conducted for: (1) women who delivered during the 2 years preceding the survey and who reported receiving 4 or more ANC visits, and (2) all women who delivered during the preceding 2 years. The specific ANC services that we assessed were: blood pressure measurement, tetanus toxoid vaccination, first ANC visit at less than 4 months gestation, urine testing, counseling about danger signs, HIV counseling and testing, iron–folate supplementation (≥ 90 days), and at least 2 doses of sulfadoxine/pyramethamine for malaria prevention. The difference between expected (100%) and actual coverage (the quality–coverage gap) was calculated for each service across the 41 surveys.
Coverage for specific elements of care among women reporting 4 or more ANC visits was generally low for most of the specific elements assessed. Blood pressure and tetanus toxoid performed best, with median quality–coverage gaps of 5% and 18%, respectively. The greatest gaps were for iron–folate supplementation (72%) and malaria prevention (86%). Coverage for specific interventions was generally much lower among all pregnant women (reflecting population effective coverage) than among only those who had received ANC 4+ visits. Although ANC 4+ and average coverage across the elements of care correlated relatively well (Pearson r2  =  0.56), most countries had lower average coverage for the content of care than for ANC 4+ (among all pregnant women).
We argue for the adoption of a summary indicator that better reflects the content of antenatal care than does the current ANC 4+ indicator. We propose, as an alternative, the simple average of a set of ANC content indicators available through surveys and routine health information systems.
PMCID: PMC4168625  PMID: 25276575
6.  Using vital signs to assess children with acute infections: a survey of current practice 
GPs are advised to measure vital signs in children presenting with acute infections. Current evidence supports the value of GPs' overall assessment in determining how unwell a child is, but the additional benefit of measuring vital signs is not known.
To describe the vital signs and clinical features that GPs use to assess children (aged <5 years) with acute infections.
Design of study
Questionnaire survey.
All 210 GP principals working within a 10 mile radius of Oxford, UK.
Data were collected on reported frequency, methods, and utility of measuring vital signs. Description of clinical features was used to assess the overall severity of illness.
One hundred and sixty-two (77%) GPs responded. Half (54%, 95% confidence interval [CI] = 47 to 62) measured temperature at least weekly, compared to pulse (21%, 95% CI = 15 to 27), and respiratory rates (17%, 95% CI = 11 to 23). Almost half of GPs (77, 48%) never measured capillary refill time. Temperature was measured most frequently using electronic aural thermometers (131/152; 86%); auscultation or counting were used for pulse and respiratory rates. A minority used pulse oximeters to assess respiratory status (30/151, 20%). GPs' thresholds for tachypnoea were similar to published values, but there was no consensus on the threshold of tachycardia. Observations of behaviour and activity were considered more useful than vital signs in assessing severity of illness.
Vital signs are uncommonly measured in children in general practice and are considered less useful than observation in assessing the severity of illness. If measurement of vital signs is to become part of standard practice, the issues of inaccurate measurement and diagnostic value need to be addressed urgently.
PMCID: PMC2277108  PMID: 18494174
body temperature; child; heart rate; infection; observation; triage
7.  Use of an Appreciative Inquiry Approach to Improve Resident Sign-Out in an Era of Multiple Shift Changes 
Resident duty hour restrictions have resulted in more frequent patient care handoffs, increasing the need for improved quality of residents’ sign-out process.
To characterize resident sign-out process and identify effective strategies for quality improvement.
Mixed methods analysis of resident sign-out, including a survey of resident views, prospective observation and characterization of 64 consecutive sign-out sessions, and an appreciative-inquiry approach for quality improvement.
Internal medicine residents (n = 89).
An appreciative inquiry process identified five exemplar residents and their peers’ effective sign-out strategies.
Surveys were analyzed and observations of sign-out sessions were characterized for duration and content. Common effective strategies were identified from the five exemplar residents using an appreciative inquiry approach.
The survey identified wide variations in the methodology of sign-out. Few residents reported that laboratory tests (13%) or medications (16%) were frequently accurate. The duration of observed sign-outs averaged 134 ±73 s per patient for the day shift (6 p.m.) sign-out compared with 59 ± 41 s for the subsequent night shift (8 p.m.) sign-out for the same patients (p = 0.0002). Active problems (89% vs 98%, p = 0.013), treatment plans (52% vs 73%, p = 0.004), and laboratory test results (56% vs 80%, p = 0.002) were discussed less commonly during night compared with day sign-out. The five residents voted best at sign-out (mean vote 11 ± 1.6 vs 1.7 ± 2.3) identified strategies for sign-out: (1) discussing acutely ill patients first, (2) minimizing discussion on straightforward patients, (3) limiting plans to active issues, (4) using a systematic approach, and (5) limiting error-prone chart duplication.
Resident views toward sign-out are diverse, and accuracy of written records may be limited. Consecutive sign-outs are associated with degradation of information. An appreciative-inquiry approach capitalizing on exemplar residents was effective at creating standards for sign-out.
PMCID: PMC3286561  PMID: 21997480
sign-out; handoff of care; continuity of patient care; internship and residency; communication; patient transfer; medical errors
8.  Web Evaluation at the US National Institutes of Health: Use of the American Customer Satisfaction Index Online Customer Survey 
The National Institutes of Health (NIH), US Department of Health and Human Services (HHS), realized the need to better understand its Web users in order to help assure that websites are user friendly and well designed for effective information dissemination. A trans-NIH group proposed a trans-NIH project to implement an online customer survey, known as the American Customer Satisfaction Index (ACSI) survey, on a large number of NIH websites—the first “enterprise-wide” ACSI application, and probably the largest enterprise Web evaluation of any kind, in the US government. The proposal was funded by the NIH Evaluation Set-Aside Program for two years at a cost of US $1.5 million (US $1.275 million for survey licenses for 60 websites at US $18,000 per website; US $225,000 for a project evaluation contractor).
The overall project objectives were to assess the value added to the participating NIH websites of using the ACSI online survey, identify any NIH-wide benefits (and limitations) of the ACSI, ascertain any new understanding about the NIH Web presence based on ACSI survey results, and evaluate the effectiveness of a trans-NIH approach to Web evaluation. This was not an experimental study and was not intended to evaluate the ACSI survey methodology, per se, or the impacts of its use on customer satisfaction with NIH websites.
The evaluation methodology included baseline pre-project websites profiles; before and after email surveys of participating website teams; interviews with a representative cross-section of website staff; observations of debriefing meetings with website teams; observations at quarterly trans-NIH Web staff meetings and biweekly trans-NIH leadership team meetings; and review and analysis of secondary data.
Of the original 60 NIH websites signed up, 55 implemented the ACSI survey, 42 generated sufficient data for formal reporting of survey results for their sites, and 51 completed the final project survey. A broad cross-section of websites participated, and a majority reported significant benefits and new knowledge gained from the ACSI survey results. NIH websites as a group scored consistently higher on overall customer satisfaction relative to US government-wide and private sector benchmarks.
Overall, the enterprise-wide experiment was successful. On the level of individual websites, the project confirmed the value of online customer surveys as a Web evaluation method. The evaluation results indicated that successful use of the ACSI, whether site-by-site or enterprise-wide, depends in large part on strong staff and management support and adequate funding and time for the use of such evaluative methods. In the age of Web-based e-government, a broad commitment to Web evaluation may well be needed. This commitment would help assure that the potential of the Web and other information technologies to improve customer and citizen satisfaction is fully realized.
PMCID: PMC2483849  PMID: 18276580
Surveys; evaluation studies; satisfaction; Internet; World Wide Web; consumer health information
9.  Rural Community Knowledge of Stroke Warning Signs and Risk Factors 
Preventing Chronic Disease  2005;2(2):A14.
Rapid identification and treatment of ischemic stroke can lead to improved patient outcomes. Public education campaigns in selected communities have helped to increase knowledge about stroke, but most data represent large metropolitan centers working with academic institutions. Much less is known about knowledge of stroke among residents in rural communities.
In 2004, 800 adults aged 45 years and older from two Montana counties participated in a telephone survey using unaided questions to assess awareness of stroke warning signs and risk factors. The survey also asked respondents if they had a history of atrial fibrillation, diabetes, high blood pressure, high cholesterol, smoking, heart disease, or stroke.
More than 70% of survey participants were able to correctly report two or more warning signs for stroke: numbness on any side of the face/body (45%) and speech difficulties (38%) were reported most frequently. More than 45% were able to correctly report two or more stroke risk factors: smoking (50%) and high blood pressure (44%) were reported most frequently. Respondents aged 45 to 64 years (odds ratio [OR] 2.44; 95% confidence interval [CI], 1.78–3.46), women (OR 2.02; 95% CI, 1.46–2.80), those with 12 or more years of education (OR 1.96; 95% CI, 1.08–3.56), and those with high cholesterol (OR 1.68; 95% CI, 1.17–2.42) were more likely to correctly identify two or more warning signs compared with respondents without these characteristics. Women (OR 1.48; 95% CI, 1.07–2.05) and respondents aged 45 to 64 years (OR 1.35; 95% CI, 1.01–1.81) were also more likely to correctly identify two or more stroke risk factors compared with men and older respondents.
Residents of two rural counties were generally aware of stroke warning signs, but their knowledge of stroke risk factors was limited.
PMCID: PMC1327708  PMID: 15888225
10.  Clinical signs of early osteoarthritis: reproducibility and relation to x ray changes in 541 women in the general population. 
Annals of the Rheumatic Diseases  1991;50(7):467-470.
The definition and classification of early clinically apparent osteoarthritis both in clinical situations and in epidemiological surveys remains a problem. Few data exist on the between-observer reproducibility of simple clinical methods of detecting hand and knee osteoarthritis in the population and their sensitivity and specificity as compared with radiography. Two observers first studied the reproducibility of a number of clinical signs in 41 middle aged women. Good rates of agreement were found for most of the clinical signs tested (kappa = 0.54-1.0). The more reproducible signs were then tested on a population of 541 women, aged 45-65, drawn from general practice, screening centres, and patients previously attending hospital for non-rheumatic problems. The major clinical signs used had a high specificity (87-99%) and lower sensitivity (20-49%) when compared with radiographs graded on the Kellgren and Lawrence scale (2+ = positive). When analysis was restricted to symptomatic radiographic osteoarthritis, levels of sensitivity were increased and specificity was lowered. These data show that certain physical signs of osteoarthritis are reproducible and may be used to identify clinical disease. They are not a substitute for radiographs, however, if radiographic change is regarded as the 'gold standard' of diagnosis. As the clinical signs tested seemed specific for osteoarthritis they may be of value in screening populations for clinical disease.
PMCID: PMC1004459  PMID: 1877852
11.  Knowledge of Risk Factors and Warning Signs of Stroke 
In this review, we have summarized the findings of fifteen studies of knowledge of stroke warning signs and risk factors in both high- and low-risk populations. In general, there appears to be low levels of knowledge of both risk factors and stroke warning signs among the communities studied. Using free recall, between 20% and 30% of respondents could not name a single risk factor, and between 10% and 60% could not name a single warning sign of stroke. Providing survey respondents with a list of potential warning signs substantially improved the identification of warning signs. Respondents in older age groups and having lower levels of educational attainment tended to have less knowledge of risk factors and warning signs of stroke than those in younger age groups and those with more education. Public campaigns to improve stroke knowledge are needed, particularly in the older age groups where the risk of stroke is greater.
PMCID: PMC1993942  PMID: 17315400
stroke; awareness; risk factors; knowledge; survey
12.  Canine angiostrongylosis in Italy: occurrence of Angiostrongylus vasorum in dogs with compatible clinical pictures 
Parasitology Research  2013;112(7):2473-2480.
This study evaluated the perception of veterinarians in including the emerging metastrongyloid Angiostrongylus vasorum in the differential diagnosis for dogs referred with appropriate signs. Veterinarians at teaching hospitals, private practices or kennels collected faecal samples from 606 symptomatic dogs in six different areas of Italy. The samples were examined with the Baermann technique. Fourteen dogs scored positive for A. vasorum, all showing different clinical pictures, with the most common reason for inclusion in the study being a combination of respiratory signs along with general signs. The most reported sign was cough, followed by aspecific distresses. Other respiratory signs were dyspnoea, tachypnoea and breathing sounds, while bleeding diathesis occurred in one dog. Gastrointestinal disorders were diarrhoea and vomitus, while no neurological signs were detected. Four key cases are herein described, with a special focus on their clinical description, findings at the collateral diagnostic imaging and therapeutic options with moxidectin. This survey provides new data on distribution of A. vasorum in Europe and on the clinical impact of the disease. The results confirm that clinical angiostrongylosis overlaps a series of other conditions of dogs. Awareness among vet practitioners is discussed along with the importance of including A. vasorum on the list of differential diagnoses in canine clinical practice and of performing appropriate diagnostic methods.
PMCID: PMC3683398  PMID: 23595212
13.  A Comparative Analysis of 2 National Breast Reconstruction Surveys: Concerns Regarding Autologous and Microsurgical Breast Reconstruction 
Pertinent findings of two national breast reconstruction (BR) surveys from two different institutions at two separate times (2012 vs 2010) were analyzed for comparison.
An appraisal of sampling methodology, sample demographics, years of practice, practice affiliation, BR type, and annual volume between the two studies was conducted using statistical analysis.
Proper sampling technique and adequate sample size were used in both surveys to represent the typical population of general US plastic surgeons practicing BR. There were no differences in mean age or years of practice between the sample population in both studies. The percentage of plastic surgeons with academic affiliation was higher in the 2012 survey compared to the 2010 survey (28% vs 18%, P<0.05). Implant-based BR was the predominant method among surveyed plastic surgeons in both surveys. There was a substantial decrease in the proportion of plastic surgeons performing >20 cases per year in the 2012 survey compared to the 2010 survey (P<0.05). A high volume of BR, defined as >60 cases per year in the 2010 survey and >20 cases per year in the 2012 survey, correlated significantly with academic practice and multidisciplinary cancer centers, respectively (P<0.05). There was a significant decline in the percentage of plastic surgeons performing microsurgical BR from 2010 to 2012 (35% vs 25%, P<0.05).
Comparative data suggest that there are fewer high volume BR cases being performed by US plastic surgeons and that there has been a reduction among plastic surgeons in the use of microsurgical BR from 2010 to 2012. We believe that these findings may indicate early signs of the changes in BR trends in the US.
PMCID: PMC4174087  PMID: 25289351
14.  Integrated Mapping of Neglected Tropical Diseases: Epidemiological Findings and Control Implications for Northern Bahr-el-Ghazal State, Southern Sudan 
There are few detailed data on the geographic distribution of most neglected tropical diseases (NTDs) in post-conflict Southern Sudan. To guide intervention by the recently established national programme for integrated NTD control, we conducted an integrated prevalence survey for schistosomiasis, soil-transmitted helminth (STH) infection, lymphatic filariasis (LF), and loiasis in Northern Bahr-el-Ghazal State. Our aim was to establish which communities require mass drug administration (MDA) with preventive chemotherapy (PCT), rather than to provide precise estimates of infection prevalence.
Methods and Findings
The integrated survey design used anecdotal reports of LF and proximity to water bodies (for schistosomiasis) to guide selection of survey sites. In total, 86 communities were surveyed for schistosomiasis and STH; 43 of these were also surveyed for LF and loiasis. From these, 4834 urine samples were tested for blood in urine using Hemastix reagent strips, 4438 stool samples were analyzed using the Kato-Katz technique, and 5254 blood samples were tested for circulating Wuchereria bancrofti antigen using immunochromatographic card tests (ICT). 4461 individuals were interviewed regarding a history of ‘eye worm’ (a proxy measure for loiasis) and 31 village chiefs were interviewed regarding the presence of clinical manifestations of LF in their community. At the village level, prevalence of Schistosoma haematobium and S. mansoni ranged from 0 to 65.6% and from 0 to 9.3%, respectively. The main STH species was hookworm, ranging from 0 to 70% by village. Infection with LF and loiasis was extremely rare, with only four individuals testing positive or reporting symptoms, respectively. Questionnaire data on clinical signs of LF did not provide a reliable indication of endemicity. MDA intervention thresholds recommended by the World Health Organization were only exceeded for urinary schistosomiasis and hookworm in a few, yet distinct, communities.
This was the first attempt to use an integrated survey design for this group of infections and to generate detailed results to guide their control over a large area of Southern Sudan. The approach proved practical, but could be further simplified to reduce field work and costs. The results show that only a few areas need to be targeted with MDA of PCT, thus confirming the importance of detailed mapping for cost-effective control.
Author Summary
Integrated control of neglected tropical diseases (NTDs) is being scaled up in a number of developing countries, because it is thought to be more cost-effective than stand-alone control programmes. Under this approach, treatments for onchocerciasis, lymphatic filariasis (LF), schistosomiasis, soil-transmitted helminth (STH) infection, and trachoma are administered through the same delivery structure and at about the same time. A pre-requisite for implementation of integrated NTD control is information on where the targeted diseases are endemic and to what extent they overlap. This information is generated through surveys that can be labour-intensive and expensive. In Southern Sudan, all of the above diseases except onchocerciasis require further mapping before a comprehensive integrated NTD control programme can be implemented. To determine where treatment for which disease is required, integrated surveys were conducted for schistosomiasis, STH infection, LF, and loiasis, throughout one of ten states of the country. Our results show that treatment is only required for urinary schistosomiasis and STH in a few, yet separate, geographical area. This illustrates the importance of investing in disease mapping to minimize overall programme costs by being able to target interventions. Integration of survey methodologies for the above disease was practical and efficient, and minimized the effort required to collect these data.
PMCID: PMC2761732  PMID: 19859537
15.  Prevalence of Trachoma in Unity State, South Sudan: Results from a Large-Scale Population-Based Survey and Potential Implications for Further Surveys 
Large parts of South Sudan are thought to be trachoma-endemic but baseline data are limited. This study aimed to estimate prevalence for planning trachoma interventions in Unity State, to identify risk factors and to investigate the effect of different sampling approaches on study conclusions.
Methods and Findings
The survey area was defined as one domain of eight counties in Unity State. Across the area, 40 clusters (villages) were randomly selected proportional to the county population size in a population-based prevalence survey. The simplified grading scheme was used to classify clinical signs of trachoma. The unadjusted prevalence of trachoma inflammation-follicular (TF) in children aged 1–9 years was 70.5% (95% CI: 68.6–72.3). After adjusting for age, sex, county and clustering of cases at household and village level the prevalence was 71.0% (95% CI: 69.9–72.1). The prevalence of trachomatous trichiasis (TT) in adults was 15.1% (95% CI: 13.4–17.0) and 13.5% (95% CI: 12.0–15.1) before and after adjustment, respectively. We estimate that 700,000 people (the entire population of Unity State) require antibiotic treatment and approximately 54,178 people require TT surgery. Risk factor analyses confirmed child-level associations with TF and highlighted that older adults living in poverty are at higher risk of TT. Conditional simulations, testing the alternatives of sampling 20 or 60 villages over the same area, indicated that sampling of only 20 villages would have provided an acceptable level of precision for state-level prevalence estimation to inform intervention decisions in this hyperendemic setting.
Trachoma poses an enormous burden on the population of Unity State. Comprehensive control is urgently required to avoid preventable blindness and should be initiated across the state now. In other parts of South Sudan suspected to be highly trachoma endemic, counties should be combined into larger survey areas to generate the baseline data required to initiate interventions.
Author Summary
Large parts of South Sudan are thought to be trachoma endemic but baseline data, required to initiate interventions, are few. District-by-district surveys, currently recommended by the World Health Organization (WHO), are often not financially or logistically viable. We therefore adapted existing WHO guidelines and combined eight counties (equivalent to districts) of Unity State into one survey area, randomly sampling 40 villages using a population-based survey design. This decision was based on a trachoma risk map and a trachoma rapid assessment, both identifying the state as likely to be highly endemic. The survey confirmed trachoma as being hyperendemic throughout Unity State, meaning that large-scale intervention should be initiated now. Simulation studies were conducted to determine the likely outcome if fewer (n = 20) or more (n = 60) villages had been sampled, confirming that precision decreased or increased, respectively. Importantly, simulation results also showed that all three sample sizes would have led to the same conclusion, namely the need for large-scale intervention. This finding suggests that district-by-district surveys may not be required for areas where trachoma is suspected to be highly prevalent but that are lacking baseline data; instead districts may be combined into a larger survey area.
PMCID: PMC3323519  PMID: 22506082
16.  Defining Childhood Severe Falciparum Malaria for Intervention Studies 
PLoS Medicine  2007;4(8):e251.
Clinical trials of interventions designed to prevent severe falciparum malaria in children require a clear endpoint. The internationally accepted definition of severe malaria is sensitive, and appropriate for clinical purposes. However, this definition includes individuals with severe nonmalarial disease and coincident parasitaemia, so may lack specificity in vaccine trials. Although there is no “gold standard” individual test for severe malaria, malaria-attributable fractions (MAFs) can be estimated among groups of children using a logistic model, which we use to test the suitability of various case definitions as trial endpoints.
Methods and Findings
A total of 4,583 blood samples were taken from well children in cross-sectional surveys and from 1,361 children admitted to a Kenyan District hospital with severe disease. Among children under 2 y old with severe disease and over 2,500 parasites per microliter of blood, the MAFs were above 85% in moderate- and low-transmission areas, but only 61% in a high-transmission area. HIV and malnutrition were not associated with reduced MAFs, but gastroenteritis with severe dehydration (defined by reduced skin turgor), lower respiratory tract infection (clinician's final diagnosis), meningitis (on cerebrospinal fluid [CSF] examination), and bacteraemia were associated with reduced MAFs. The overall MAF was 85% (95% confidence interval [CI] 83.8%–86.1%) without excluding these conditions, 89% (95% CI 88.4%–90.2%) after exclusions, and 95% (95% CI 94.0%–95.5%) when a threshold of 2,500 parasites/μl was also applied. Applying a threshold and exclusion criteria reduced sensitivity to 80% (95% CI 77%–83%).
The specificity of a case definition for severe malaria is improved by applying a parasite density threshold and by excluding children with meningitis, lower respiratory tract infection (clinician's diagnosis), bacteraemia, and gastroenteritis with severe dehydration, but not by excluding children with HIV or malnutrition.
The accepted definition of severe malaria is appropriate for clinical purposes, but Philip Bejon and colleagues show its specificity in clinical trials may be improved by a parasite density threshold and by excluding children with certain conditions.
Editors' Summary
Malaria is responsible for over a million deaths every year, and most of those who die are children in Africa. Until a few years ago, not enough research was being done on malaria, but now many researchers are active in this field. Doctors describe some cases of malaria as being “severe.” Severe malaria in children is very hard to diagnose precisely. Current protocols for diagnosing severe malaria are very sensitive: that is, virtually all children who do have severe malaria will be correctly diagnosed as such. However, the protocols are not very specific: many children who do not have severe malaria, but whose symptoms are instead caused by other diseases, will be defined as suffering from severe malaria. This definition is acceptable for the clinical care of sick children, because it ensures that antimalarial drugs are given to all who might benefit from them, plus some additional children for whom those drugs are not required. However, this definition is not particularly useful for research purposes. When conducting a clinical trial aimed at preventing cases of malaria, it is important to evaluate whether the intervention being tested actually works. Therefore, a more specific method of calculating the number of malaria cases within a population is needed for this type of research.
Why Was This Study Done?
The current definition for diagnosing severe malaria includes a set of signs and symptoms that may be observed at the bedside or as a result of laboratory investigation, along with the detection of malaria parasites in the patient's blood. However, in many malarious areas, a large proportion of the population carries malaria parasites without signs of disease; at the same time, the signs and symptoms of malaria are shared with other diseases. The investigators here wanted to find out whether they could develop an accurate “case definition” of severe malaria that can be used in research.
What Did the Researchers Do and Find?
In this study, two groups of children were studied: first, 1,422 children admitted to the children's wards of the Kilifi District Hospital in Kenya, and second, 4,583 children from the surrounding community. Blood samples were taken in order to find out how common malaria parasites were in the children's blood, and standard clinical and laboratory data were also collected from the children admitted to the hospital. The researchers then compared these data using a computer and tried to find out whether, by excluding certain children who had particular signs, symptoms, or observations, from the diagnosis of severe malaria, they were able to improve the accuracy of their definition. Essentially, for each patient group, the authors calculated “malaria-attributable fractions,” i.e., the proportion of individuals studied whose disease was likely caused by malaria.
The researchers found that in areas with low and moderate transmission of malaria, the proportion of individuals whose disease could be attributed to malaria was high—nearly 85%. In areas with a high transmission rate of malaria, this fraction was much lower, but could be improved by including only children with a high proportion of parasites in their blood. Importantly, the researchers were also able to increase the recognition of children with disease likely caused by malaria by excluding individuals who had also been diagnosed with gastroenteritis, lower respiratory tract infections, meningitis, and bacterial infection in the blood. If all of these individuals were excluded—so only individuals with more than 2,500 parasites per microliter in their blood were regarded as having severe malaria—the “malaria-attributable fraction” rose to 95%.
What Do These Findings Mean?
These findings should not be directly used to change the clinical care of children with the signs and symptoms of severe malaria, but rather can be used within a clinical trial to create a “case definition” of malaria particular to that trial. This ability will help researchers more accurately find out whether the intervention being tested in their trial really does help to prevent cases of malaria or not.
Additional Information.
Please access these Web sites via the online version of this summary at
The World Health Organization Global Malaria Programme details the organization's activities in fighting malaria, and provides a number of helpful resources; a factsheet on children and malaria is also available
The US Centers for Disease Control and Prevention provides many malaria resources
General information about malaria, including illustrations, is available from the“Medline Plus” encyclopedia
PMCID: PMC1949845  PMID: 17713980
17.  Lungworm (Crenosoma vulpis) infection in dogs on Prince Edward Island. 
The Canadian Veterinary Journal  1999;40(8):555-559.
Crenosoma vulpis is a nematode lungworm that is highly prevalent in the red fox population of Atlantic Canada. Dogs are susceptible to infection with clinical signs consisting primarily of a chronic cough. A recent report of C. vulpis infection in 3 dogs on Prince Edward Island prompted an investigation into the importance of this parasite as a cause of chronic respiratory disease in Island dogs. A general prevalence was determined through the necropsy of dogs euthanized at the local humane society. Lungs were removed and examined for parasites using a lung flush technique. Rectal feces was collected and examined for first-stage larvae using the Baermann technique and zinc sulfate centrifugal flotation. Ten of 310 dogs (3.2%) were positive with 0-35 worms (mean = 11.0 +/- 13.4) recovered. First-stage larvae of C. vulpis were recovered in the rectal feces of the one animal in which no worms were recovered on lung flush. A second survey was conducted examining fecal samples with the Baermann technique from afebrile dogs with presenting signs of chronic cough that had no history of recent anthelmintic treatment and showed no signs of cardiac disease, based on physical examination. Fifteen of 55 dogs examined (27.3%) were definitively diagnosed as C. vulpis-positive. All of the infected dogs were treated with fenbendazole (50 mg/kg body weight, p.o. q24 h for 3-7 days). Clinical signs resolved in all of the dogs and fecal samples were negative 2-4 weeks posttreatment. It was concluded that C. vulpis infection was a significant cause of upper respiratory disease in dogs on Prince Edward Island and should be considered in all dogs with presenting signs of chronic cough.
PMCID: PMC1539780  PMID: 12001335
18.  Glaucoma-service provision in Scotland: introduction and need for Scottish Intercollegiate Guidelines Network guidelines 
To describe the pattern of glaucoma-service delivery in Scotland and identify areas for improvement, taking into account Scottish General Ophthalmic Services (GOS) arrangements and the Eye Care Integration project, and to design Scottish Intercollegiate Guidelines Network (SIGN) guidelines to refine the primary and secondary interface of glaucoma care.
Materials and methods
A glaucoma-survey questionnaire was sent to all consultant glaucomatologists in Scotland. The design of SIGN guidelines was based on the results of the questionnaire using SIGN methodology.
Over 90% of Scottish glaucoma care is triaged and delivered within hospital services. Despite GOS referral, information is variable. There are no consistent discharge practices to the community. These results led to defined research questions that were answered, thus formulating the content of the SIGN guidelines. The guideline covers the assessment of patients in primary care, referral criteria to hospital, discharge criteria from hospital to community, and monitoring of patients at risk of glaucoma.
With increasing age and limitations to hospital resources, refining glaucoma pathways between primary and secondary care has become a necessity. Scotland has unique eye care arrangements with both the GOS and Eye Care Integration project. It is hoped that implementation of SIGN guidelines will identify glaucoma at the earliest opportunity and reduce the rate of false-positive referrals to hospital.
PMCID: PMC4599172  PMID: 26491244
glaucoma; referral; community care; SIGN guidelines
19.  Self-reported awareness of the presence of product warning messages and signs by Hispanics in San Francisco. 
Public Health Reports  1994;109(2):275-283.
This study investigated the self-reported awareness of the presence of product warning messages and signs among random samples of Hispanics in San Francisco surveyed in 1990 and in 1991. The messages that were tested related to cigarettes, alcoholic beverages, and other consumer products. A random sample of 1,204 Hispanics (43.5 percent males) were interviewed by telephone in 1990. The corresponding figure for the second survey in 1991 was 1,569 Hispanics (41.1 percent males). In general, respondents reported low levels of awareness of the presence of product warning messages and signs. The exception was warning messages on cigarette packets which approximately 70 percent of the respondents reported having seen within the 12 months before the survey. There was an increase from 1990 to 1991 in the reported awareness of warning messages for wine, beer, and cigarettes. Smokers and drinkers of alcoholic beverages reported the highest levels of awareness of the relevant warning messages and signs. Length of exposure to warning messages and multiplicity of sources (for example, advertisements and products) seem to produce greater levels of awareness of the presence of product warning messages. Less acculturated, Spanish-speaking Hispanics are less likely to report being aware of the warnings, particularly those that appear only in English (for example, alcoholic beverages).
PMCID: PMC1403486  PMID: 8153279
20.  New monoclonal anti-mouse DC-SIGN antibodies reactive with acetone-fixed cells 
Journal of immunological methods  2010;360(1-2):66-75.
Mouse DC-SIGN CD209a is a type II transmembrane protein, one of a family of C-type lectin genes syntenic and homologous to human DC-SIGN. Current anti-mouse DC-SIGN monoclonal antibodies (MAbs) are unable to react with DC-SIGN in acetone fixed cells, limiting the chance to visualize DC-SIGN in tissue sections. We first produced rabbit polyclonal PAb-DSCYT14 against a 14-aa peptide in the cytosolic domain of mouse DC-SIGN, and it specifically detected DC-SIGN and not the related lectins, SIGN-R1 and SIGN-R3 expressed in transfected CHO cells. MAbs were generated by immunizing rats and DC-SIGN knockout mice with the extracellular region of mouse DC-SIGN.. Five rat IgG2a or IgM MAbs, named BMD10, 11, 24, 25, and 30, were selected and each MAb specifically detected DC-SIGN by FACS and Western blots, although BMD25 was cross-reactive to SIGN-R1. Two mouse IgG2c MAbs MMD2 and MMD3 interestingly bound mouse DC-SIGN but at 10 fold higher levels than the rat MAbs. When the binding epitopes of the new BMD and two other commercial rat anti-DC-SIGN MAbs, 5H10 and LWC06, were examined by competition assays, the epitopes of BMD11, 24, and LWC06 were identical or closely overlapping while BMD10, 30, and 5H10 were shown to bind different epitopes. MMD2 and MMD3 epitopes were on a 3rd noncompeting region of mouse DC-SIGN. DC-SIGN expressed on the cell surface was sensitive to collagenase treatment, as monitored by polyclonal and MAb. These new reagents should be helpful to probe the biology of DC-SIGN in vivo.
PMCID: PMC2924951  PMID: 20558171
Monoclonal Antibody; Polyclonal Antibody; DC-SIGN; CD209a; Dendritic Cells
21.  A Survey of Nursing Home Physicians to Determine Laboratory Monitoring Adverse Drug Event Alert Preferences 
Applied Clinical Informatics  2014;5(4):895-906.
We conducted a survey of nursing home physicians to learn about (1) the laboratory value thresholds that clinical event monitors should use to generate alerts about potential adverse drug events (ADEs); (2) the specific information to be included in the alerts; and (3) the communication modality that should be used for communicating them.
Nursing home physician attendees of the 2010 Conference of AMDA: The Society for Post-Acute and Long-Term Care Medicine.
A total of 800 surveys were distributed; 565 completed surveys were returned and seven surveys were excluded due to inability to verify that the respondents were physicians (a 70% net valid response rate). Alerting threshold preferences were identified for eight laboratory tests. For example, the majority of respondents selected thresholds of ≥ 5.5 mEq/L for hyperkalemia (63%) and ≤ 3.5 without symptoms for hypokalemia (54%). The majority of surveyed physicians thought alerts should include the complete active medication list, current vital signs, previous value of the triggering lab, medication change in the past 30 days, and medication allergies. Most surveyed physicians felt the best way to communicate an ADE alert was by direct phone/voice communication (64%), followed by email to a mobile device (59%).
This survey of nursing home physicians suggests that the majority prefer alerting thresholds that would generally lead to fewer alerts than if widely accepted standardized laboratory ranges were used. It also suggests a subset of information items to include in alerts, and the physicians’ preferred communication modalities. This information might improve the acceptance of clinical event monitoring systems to detect ADEs in the nursing home setting.
PMCID: PMC4287669  PMID: 25589905
Nursing homes; therapeutic drug monitoring; clinical decision support systems; adverse drug event
The aim of this study was to investigate the perception of King Saud University female students towards the implementation of premarital screening (PMS) in the Kingdom of Saudi Arabia (KSA).
Two consecutive surveys on knowledge, attitudes and practice (KAP) were conducted two and nine months after the compulsory implementation of PMS in KSA in 1/1/1425H. The female students of King Saud University were given health education lectures before the survey. The first survey was done with a designed close-ended questionnaire distributed at pre and post stages of the health education lecture. However, the second one explored the participants’ perception of the same items in open-ended questions summarized as a “consensus statement”. In fulfillment of their demands, the signed statement was mailed to the legislative authorities.
Results and discussion:
A total of 140 university female students attended the first lecture. The response rate for both pre and post lecture surveys were 132 (94.3%) and 128 (91.4%) respectively. A total of 112 out of 132 (84.8%) students in the pre test and 111 out of 128 (86.7%) in the post-test were single.
Of the married students 7/20 (35.0%) and 7/17 (41.2%) in pre and post tests had previously had PMS screening. The attitude of the students towards PMS was generally positive. One hundred and eight (81.8%) in the pre test and 110 (85.9%) in the post test saw the importance of PMS in controlling the commonest hereditary diseases. However, a smaller percentage of students (69.7% and 75.0%) in pre and post lecture respectively were in favor of the compulsory application of PMS in KSA. In spite of the positive attitude of all the students in the pre and post tests, fears were expressed towards the confidentiality of PMS test results and it was felt that social and psychological problems would ensue from abnormal results. This, however, does not represent the feeling of the entire population in KSA since the participants of the study formed a select group.
The second awareness lecture was attended by 319 students from the College of Education. They were subsequently requested to state their perceptions of PMS application with regard to its content, nature and method of application in KSA in their own words. The collected forms were summarized into a “consensus statement” and signed by all 319 students. They felt that the scope of PMS should be extended to investigate and screen for other diseases especially sexually transmitted diseases that would adversely affect the health of members of the family and the community as a whole. Their worry about the lack of screening for other diseases may be because a majority of the study group were single and would, therefore, refuse to get married on account of the risks to their future offspring. In addition, it may reflect their knowledge of the effects of globalization on the transmission of diseases.
Health education is an important means of improving the public's perception of newly-introduced health interventions. University students have a good perception of the compulsory implementation of PMS in KSA. Pre-marital screening could be extended to include a broader spectrum of health/genetic disorders and will be useful for early identification and possible intervention as well as the prevention of complications.
PMCID: PMC3410069  PMID: 23012110
Premarital Screening; Health Education; Kingdom of Saudi Arabia
23.  Broad Reach and Targeted Recruitment Using Facebook for an Online Survey of Young Adult Substance Use 
Studies of tobacco use and other health behaviors have reported great challenges in recruiting young adults. Social media is widely used by young adults in the United States and represents a potentially fast, affordable method of recruiting study participants for survey research.
The present study examined Facebook as a mechanism to reach and survey young adults about tobacco and other substance use.
Participants were cigarette users, age 18-25 years old, living throughout the United States and recruited through Facebook to complete a survey about tobacco and other substance use. Paid advertising using Facebook’s Ad program over 13 months from 2010 Feb 28 to 2011 Apr 4 targeted by age (18-25), location (United States or California), language (English), and tobacco- and/or marijuana-related keywords. Facebook approved all ads.
The campaign used 20 ads, which generated 28,683,151 impressions, yielding 14,808 clicks (0.7% of targeted Facebook members), at an overall cost of $6,628.24. The average cost per click on an ad was $0.45. The success of individual ads varied widely. There was a rise in both clicks and impressions as the campaign grew. However, the peak for clicks was 3 months before the peak for ad impressions. Of the 69,937,080 accounts for those age 18-25 in the United States, Facebook estimated that 2.8% (n = 1,980,240) were reached through tobacco and marijuana keywords. Our campaign yielded 5237 signed consents (35.4% of clicks), of which 3093 (59%) met criteria, and 1548 (50% of those who met criteria) completed the survey. The final cost per valid completed survey was $4.28. The majority of completed surveys came from whites (69%) and males (72%). The sample averaged 8.9 cigarettes per day (SD 7.5), 3.8 years of smoking (SD 2.9), with a median of 1 lifetime quit attempts; 48% did not intend to quit smoking in the next 6 months.
Despite wide variety in the success of individual ads and potential concerns about sample representativeness, Facebook was a useful, cost-effective recruitment source for young-adult smokers to complete a survey about the use of tobacco and other substances. The current findings support Facebook as a viable recruitment option for assessment of health behavior in young adults.
PMCID: PMC3374532  PMID: 22360969
social media, Facebook, participant recruitment, young adult, tobacco
24.  Expert opinion in the management of aqueous Deficient Dry Eye Disease (DED) 
BMC Ophthalmology  2015;15:133.
Dry eye disease (DED) affects millions of people worldwide. There are a variety of new treatments beyond traditional therapies such as preservative free artificial tears. Here, we conduct a survey to identify the most common treatments used among specialists and assess their interest in newer therapies.
An international survey was distributed to dry eye researchers and expert practitioners via an internet survey. The survey data collected were analyzed with descriptive statistics.
One hundred and fifteen respondents completed the survey; of these, 66 % were cornea specialists. The most commonly prescribed topical treatments included cyclosporine A (CSA) 0.05 % (71/104, 68 %), fluorometholone (FML) 0.1 % (59/99, 60 %), loteprednol etabonate 0.5 % (50/99, 51 %), and autologous serum eye drops (ASD; 48/97, 49 %). The most commonly prescribed non-topical medications included essential fatty acid supplements (72/104, 69 %), low-dose doxycycline (oral; 61/100, 61 %), and flaxseed supplements (32/96, 33 %) as well as punctal plugs (76/102, 75 %). Respondents reported treatment with topical corticosteroids for 2 to 8 weeks (46/86, 53 %), followed by less than 2 weeks (24/86, 28 %) and with topical CSA between 2 to 8 weeks (45/85, 53 %) followed by 2 to 6 months (24/85, 28 %). The top three signs and symptoms reported to indicate treatment response were, in order, fluorescein staining of the cornea, reduction in foreign body sensation, and reduction in burning sensation.
This survey offers insight into current expert opinion in the treatment of DED. The results of this survey are hypothesis generating and will aid in the design of future clinical studies.
PMCID: PMC4604775  PMID: 26462481
Dry eye disease; Keratoconjunctivitis sicca; Sjögrens
25.  Ocular Signs Correlate Well with Disease Severity and Genotype in Fabry Disease 
PLoS ONE  2015;10(3):e0120814.
Ocular signs in Fabry disease have generally been regarded to be primarily of diagnostic value. We explored whether ocular findings, alone or in particular in combination with the α-galactosidase A gene mutation, have predictive value for disease severity. Data from the Fabry Outcome Survey (FOS), a large, global database sponsored by Shire, were selected for adult patients who had undergone ophthalmological examination. Three ocular signs were assessed: cornea verticillata, tortuous conjunctival and/or retinal vessels, and cataract. Fabry disease severity was measured using FOS Mainz Severity Score Index and modifications thereof. Ophthalmological data were available for 1203 (699 female, 504 male) adult patients with eye findings characteristic of Fabry disease in 55.1%. Cornea verticillata had a similar distribution in women (51.1%) and men (50.8%), whereas tortuous vessels and Fabry cataract were somewhat more frequent in men than in women. Patients with cornea verticillata, selected as the principal ocular sign for this study, had more severe disease (median score, 20.0) versus those without ocular signs (11.0; P<0.001). This finding could be confirmed by applying age adjusted severity scores. Moreover, the prevalence of cornea verticillata was significantly higher in patients with null (male, 76.9%; female, 64.5%) and missense (male, 79.2%; female, 67.4%) mutations versus mild missense (male, 17.1%; female, 23.1%) and the p.N215S (male, 15.0%; female, 15.6%) mutations (P<0.01). Our analyses show a correlation between the prevalence of ocular changes in Fabry disease and disease severity. Consequently, information on ocular findings and α-galactosidase A gene mutation may help assess the risk for more severe Fabry disease. These observed findings are of notable clinical importance, as Fabry disease is characterized by high clinical course variability and only weak genotype-phenotype correlation at the individual patient level. Further confirmatory studies are needed.
PMCID: PMC4363518  PMID: 25781336

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