Proceedings of the 16th Annual Meeting of the Society for Pediatric Dermatology

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/75156/1/j.1525-1470.1992.tb00329.x.pd

Tricuspid valve disease with significant tricuspid insufficiency in the fetus: Diagnosis and outcome

AbstractThe echocardiographic studies and clinical course of 27 fetuses (mean gestationl age 26.9 weeks) diagnosed in utero with tricuspid valve disease and significant tricuspid regurgitation were reviewed. The diagnosis of Ebstein's anomaly was made in 17 of the fetuses, 7 had tricuspid valve dysplasia with poorly developed but normally attached leaflets and 2 had an unguarded tricuspid valve orifice with little or no identifiable tricuspid tissue. One fetus was excluded from data analysis because a more complex heart lesion was documented at autopsy. All fetuses had massive right atrial dilation and most who were serially studied had progressive right-sided cardiomegaly. Hydrops fetalis was found in six cases and atrial flutter in five.Associated cardiac lesions included pulmonary stenosis in five cases and pulmonary alresia in six. Four fetuses with normal forward pulmonary artery flow at the initial examination were found at subsequent study to have retrograde pulmonary artery and ductal flow in association with the development of pulmonary stenosis (n = 1) and pulmonary atresia (n = 3). On review of the clinical course of the 23 fetuses (excluding 3 with elective abortion), 48% of the fetuses died in utero and 35% who were liveborn died despite vigorous medical and, when necessary, surgical management, many of whom had severe congestive heart failure. Of the four infants who survived the neonatal period, three had a benign neonatal course, all of whom were diagnosed with mild to moderate Ebstein's anomaly; only one had pulmonary outflow obstruction. An additional finding at autopsy was significant lung hypoplasia documented in 10 of 19 autopsy reports.Tricuspid valve anomalies with tricuspid insufficiency can be identified echocardiographically in the fetus and should be searched for in the presence of right atrial enlargement. The prognosis for the fetus diagnosed in utero with significant tricuspid valve disease is extremely poor, with a prenatal course that includes progressive right heart dilation, with cardiac failure and lung hypoplasia in many and development of pulmonary stenose or pulmonary atresia later in gestation in some

Diagnosing tuberculous pleural effusion using clinical data and pleural fluid analysis A study of patients less than 40 years-old in an area with a high incidence of tuberculosis

SummaryBackgroundTuberculous pleural effusions (TPE) are common. The diagnosis is often problematic. As the determination of ADA is often unavailable in some countries, the aim of this study was to evaluate the diagnostic usefulness of other data from pleural fluid analysis, in young patients from populations with high prevalence of tuberculosis (TB).MethodsWe analysed 218 patients with pleural effusion (165 tuberculous, 21 infectious, 11 neoplastic, 16 miscellaneous, 3 idiopathic). We performed two regression models; one included pleural fluid ADA values (model 1), and the other without ADA (model 2).ResultsModel 1 selected two variables (ADA >35U/L) and lymphocytes (>31.5%) and correctly classified 216/218 effusions (1 false negative, 1 false positive). Model 2 (without ADA) selected three variables: lymphocytes (>31.5%), fever and cough, and correctly classified 207/218 effusions (8 false negatives, 3 false positives). The sensitivity of models 1 and 2 was 99.4% and 95.2%, specificity 98.1% and 94.3% and accuracy 99% and 95%.ConclusionsIn geographic areas with high prevalence of TB and a low prevalence of HIV, in young patients (≤40 years), it is possible to confidently diagnose TPE with either of the two regression tree models, with the utility of ADA providing superior sensitivity, specificity, and accuracy

Validation of test performance characteristics and minimal clinically important difference of the 6-minute walk test in patients with idiopathic pulmonary fibrosis

SummaryBackgroundThe 6-minute walk test distance (6MWD) has been shown to be a valid and responsive outcome measure in patients with idiopathic pulmonary fibrosis (IPF). The analyses were based, however, on a single phase 3 trial and require validation in an independent cohort.ObjectiveTo confirm the performance characteristics and estimates of minimal clinically important difference (MCID) of 6MWD in an independent cohort of patients with IPF.MethodsPatients randomized to placebo in the phase 3 CAPACITY trials who had a baseline 6MWD measurement were included in these analyses. The 6MWD and other functional parameters (lung function, dyspnea, and health-related quality of life) were measured at baseline and 24-week intervals. Validity and responsiveness were examined using Spearman correlation coefficients. The MCID was estimated using distribution- and anchor-based methods.ResultsThe analysis comprised 338 patients. Baseline 6MWD was significantly correlated with lung function measures, patient-reported outcomes, and quality-of-life measures (validity). Compared with baseline 6MWD, change in 6MWD (responsiveness) showed stronger correlations with change in lung function parameters and quality-of-life measures. Dyspnea measured by the University of California San Diego Shortness of Breath Questionnaire showed the strongest correlations with 6MWD (baseline: coefficient −0.35; 48-week change: coefficient −0.37; both p < 0.001). The distribution-based analyses of MCID using standard error of measurement yielded an MCID of 37 m, and distribution-based analyses by effect size resulted in 29.2 m. The MCID by anchor-based analysis using criterion referencing (health events of hospitalization or death) was 21.7 m.ConclusionsThe 6MWD is a valid and responsive clinical endpoint, which provides objective and clinically meaningful information regarding functional status and near-term prognosis. These results confirm previous findings in an independent cohort of patients with IPF