RESUMO
Background: Role of immunosuppression treatment in patients with inflammatory dilated cardiomyopathy is controversial. The aim of this review is to summarize current evidence for immunosuppressive therapy in inflammatory cardiomyopathy. Methods: A systematic literature search was performed using PubMed, Embase and MELDINE to identify trials comparing immunosuppressive therapy with either placebo or conventional medical therapy in adult patients with inflammatory cardiomyopathy. Combined primary outcome in our study was all cause mortality and heart transplantation. Secondary outcomes included improvement in left ventricular ejection fraction (LVEF) and left ventricular end diastolic dimension (LVEDD). Results: Five randomized controlled trials (RCTs) were identified and four trials with similar comparable groups, with a total of 359 adult patients were included for analysis. Pooled data demonstrated no reduction in all-cause mortality and heart transplantation amongst the immunosuppression or the placebo arm (OR 0.98, 95% CI 0.48-1.98). There was a significant improvement in LVEF (1.34%, 95% CI 0.37-2.30) in patients treated with immunosuppressive medications, however no difference was observed in LVEDD [-0.11mm (95% CI -1.92 – 1.71)] in the treatment arm. Conclusion: There was no survival benefit or reduction in heart transplantation events with a significant improvement in LVEF in inflammatory cardiomyopathy patients treated with immunosuppression therapy.
RESUMO
Tricho-dento-osseous dysplasia is a rare autosomal dominant disorder which involves increased bone density, enamel hypoplasia, enlarged pulp chambers, and molar taurodontism. We report a case of a 12 year old female presenting with tricho dento osseous syndrome.