Echocardiographic Assessment of Patients with Fabry Disease.

Fabry disease is an X-linked lysosomal storage disorder that results from a deficiency of α-galactosidase A. Increased left ventricular wall thickness has been the most commonly described cardiovascular manifestation of the disease. However, a variety of other structural and functional abnormalities have also been reported. Echocardiography is an effective noninvasive method of assessing the cardiac involvement of Fabry disease. A more precise and comprehensive characterization of Fabry cardiomyopathy using conventional and novel echocardiographic techniques may lead to earlier diagnosis, more accurate prognostication, and timely treatment. The aim of this review is to provide a comprehensive overview of the structural and functional abnormalities on echocardiography that have thus far been described in patients with Fabry disease and to highlight potential areas that would benefit from further research.

Extended Screening Costs Associated With Selecting Donors for Fecal Microbiota Transplantation for Treatment of Metabolic Syndrome-Associated Diseases.

BACKGROUND: Knowledge of the impact of the gut microbiome on conditions other than Clostridium difficile infection has been rapidly increasing, and the potential usefulness of fecal microbiota transplantation (FMT) in these indications is being explored. The need to exclude donors with an increased risk of these diseases has left uncertainties regarding the cost and feasibility of donor screening. The aim of this study was to compare our experience to other donor-screening programs and report the costs associated with establishing a donor-screening program, for the treatment of metabolic syndrome-related conditions. METHODS: Forty-six potential donors (PDs) had their medical histories and physical examinations undertaken by a physician. Blood, stool, and urine were screened for 31 viral, bacterial, fungal, and protozoan agents in addition to biochemical characteristics. The price of advertising, doctor's visits and diagnostic tests were calculated to determine the cost of finding a donor. RESULTS: Of the PDs screened, 5 of 46 passed the history, examination, blood, stool, and urine tests. The most common reasons for exclusion included a body mass index >25 or the detection of Blastocystis hominis, Dientamoeba fragilis, or Helicobacter pylori. Four of five eligible donors had subsequent travel or illness that contraindicated donation, so only 1 of 46 PDs was suitable. The total cost for finding a single suitable donor was $15190 US dollars. This screening was performed in Canada, and costs in the United States would be substantially higher. CONCLUSIONS: New potential therapeutic uses for FMT have created a demand for stricter exclusion criteria for donors. This study illustrates that screening many individuals to find a donor and the subsequent associated costs may make central processing and shipment a more reasonable alternative.

Best method for right atrial volume assessment by two-dimensional echocardiography: validation with magnetic resonance imaging.

AIM: Echocardiographic methods for estimating right atrial (RA) volume have not been standardized. Our aim was to evaluate two-dimensional (2D) echocardiographic methods of RA volume assessment, using RA volume by magnetic resonance imaging (MRI) as the reference. METHODS AND RESULTS: Right atrial volume was assessed in 51 patients (mean age 63 ± 14 years, 33 female) who underwent comprehensive 2D echocardiography and cardiac MRI for clinically indicated reasons. Echocardiographic RA volume methods included (1) biplane area length, using four-chamber view twice (biplane 4C-4C); (2) biplane area length, using four-chamber and subcostal views (biplane 4C-subcostal); and (3) single plane Simpson's method of disks (Simpson's). Echocardiographic RA volumes as well as linear RA major and minor dimensions were compared to RA volume by MRI using correlation and Bland-Altman methods, and evaluated for inter-observer reproducibility and accuracy in discriminating RA enlargement. All echocardiography volumetric methods performed well compared to MRI, with Pearson's correlation of 0.98 and concordance correlation ≥0.91 for each. For bias and limits of agreement, biplane 4C-4C (bias -4.81 mL/m(2) , limits of agreement ±9.8 mL/m(2) ) and Simpson's (bias -5.15 mL/m(2) , limits of agreement ±10.1 mL/m(2) ) outperformed biplane 4C-subcostal (bias -8.36 mL/m(2) , limits of agreement ±12.5 mL/m(2) ). Accuracy for discriminating RA enlargement was higher for all volumetric methods than for linear measurements. Inter-observer variability was satisfactory across all methods. CONCLUSIONS: Compared to MRI, biplane 4C-4C and single plane Simpson's are highly accurate and reproducible 2D echocardiography methods for estimating RA volume. Linear dimensions are inaccurate and should be abandoned.

Are two heads better than one? Comparing dyad and self-regulated learning in simulation training.

CONTEXT: The optimal learner to simulator ratio for procedural skills training is not known. Research in motor learning suggests observational training in pairs, termed 'dyad training', may be as effective as directed self-regulated learning (DSRL). OBJECTIVES: This study was conducted to compare the relative effectiveness and efficiency of dyad versus DSRL training of simulation-based lumbar puncture (LP). METHODS: We conducted a two-group randomised equivalence trial. First-year internal medicine residents (n = 50) were randomly assigned to learn LP either in dyads or as individual learners on a simulator, using a directed self-regulated approach (i.e. the learning sequence was defined for them, but they defined the pace of learning). Participants were videotaped performing a simulated LP on a pre-test, an immediate post-test, and a 6-week delayed retention test. In duplicate, blinded raters independently evaluated all trainee performances using a previously validated 5-point global rating scale (GRS) and 35-item checklist. RESULTS: Our analyses showed no significant differences (p = 0.69) on pre-test, post-test or retention test GRS scores between the dyad (mean ± standard deviation [SD] scores by test: 2.39 ± 0.57, 3.48 ± 0.62, 3.12 ± 0.85, respectively) and DSRL (mean ± SD scores by test: 2.67 ± 0.50, 3.34 ± 0.77, 3.21 ± 0.79, respectively) groups. Both groups improved significantly from pre-test to post-test (p < 0.001) and retained that performance following the 6-week delay. Dyad participants experienced significantly greater pre-test to post-test gains than DSRL participants (p = 0.02). There was no significant difference in total practice time between the groups (20.94 minutes for individuals and 24.20 minutes for dyads; p = 0.175). CONCLUSIONS: Our results indicate that learning in pairs is as effective as independent DSRL. Dyad training permits the more efficient use of simulators as two learners use the same resources as an individual.

Vision health disparities in the United States by race/ethnicity, education, and economic status: findings from two nationally representative surveys.

PURPOSE: To assess vision health disparities in the United States by race/ethnicity, education, and economic status. DESIGN: Cross-sectional, nationally representative samples. METHODS: We used national survey data from the National Health and Nutrition Examination Survey (NHANES) and the National Health Interview Survey (NHIS). Main outcome measures included, from NHANES, age-related eye diseases (ie, age-related macular degeneration [AMD], cataract, diabetic retinopathy [DR], glaucoma) and from NHIS, eye care use (ie, eye doctor visits and cannot afford eyeglasses when needed) among those with self-reported visual impairment. The estimates were age- and sex-standardized to the 2000 US Census population. Linear trends in the estimates were assessed by weighted least squares regression. RESULTS: Non-Hispanic whites had a higher prevalence of AMD and cataract surgery than non-Hispanic blacks, but a lower prevalence of DR and glaucoma (all P < .001 in NHANES 2005-2008). From 1999 to 2008, individuals with less education (ie, high school) and lower income (poverty income ratio [PIR] <1.00 vs ≥ 4.00) were consistently less likely to have had an eye care visit in the past 12 months compared with their counterparts (all P < .05). During this period, inability to afford needed eyeglasses increased among non-Hispanic whites and Hispanics (trend P = .004 and P = .007; respectively), those with high school education (trend P = .036), and those with PIR 1.00-1.99 (trend P < .001). CONCLUSIONS: Observed vision health disparities suggest a need for educational and innovative interventions among socioeconomically disadvantaged groups.

Immunization in urbanized villages of Delhi.

OBJECTIVE: To assess the immunization coverage of BCG, DPT, OPV, Measles, MMR and Hepatitis B vaccines in two urbanized villages of East Delhi and study the factors affecting the coverage. METHODS: Children of age 24-47 months were selected using systematic random sampling. Information on socio-demographic factors and immunization status was obtained by house-to- house visit. Immunization coverage of all vaccines was computed and analysis of association between immunization coverage and socio-demographic factors was done. RESULTS: The coverage levels were 82.7% for BCG, 81.5% for DPT/OPV 1, 76.8% for DPT/OPV 2, 70.7% for DPT/OPV 3 and 65.3% for measles vaccine. It was 41.4% and 41.6% for DPT booster and MMR vaccine. Higher education of mother (OR=1.96) and father (OR=1.80), father's occupation (OR=1.86), residential status (OR=1.76), place of birth (OR=2.64) and presence of immunization card (OR=5.8) were significant determinants for complete immunization on univariate analysis. On regression analysis mother's education (OR=1.43), presence of immunization card OR=2.05 and place of birth (OR=3.80) remained significant. CONCLUSION: Immunization evaluation surveys have shown a wide variation across regions, states and different strata of the society.

The assessment of technical and interpretative proficiency in echocardiography.

OBJECTIVES: We sought to assess the relationship between traditional measures of proficiency in echocardiography and an objective assessment of technical and interpretative skills. BACKGROUND: Determination of competency in echocardiography is currently based on the number of months of training, echocardiograms scanned, and echocardiograms interpreted. It has not been established whether completion of these requirements is a surrogate for competency. METHODS: In all, 22 cardiology fellows underwent an echocardiography objective structured clinical examination (OSCE). RESULTS: There was a correlation between the number of echocardiograms scanned and the interpretation (r = 0.45, P = .038) and scanning (r = 0.42, P = .048) scores. There was a weak correlation between the number of echocardiograms interpreted and interpretation scores (r = 0.33); and number of months of training and the scanning (r = 0.39) and interpretation (r = 0.42) scores. CONCLUSIONS: Technical and interpretative proficiency in echocardiography is not related to traditional measures. An objective assessment of acquisition and interpretation of echocardiographic data should be incorporated into the assessment of proficiency in echocardiography.