Variant characterisation and clinical profile in a large cohort of patients with Ellis-van Creveld syndrome and a family with Weyers acrofacial dysostosis.

Background: Ellis-van Creveld syndrome (EvC) is a recessive disorder characterised by acromesomelic limb shortening, postaxial polydactyly, nail-teeth dysplasia and congenital cardiac defects, primarily caused by pathogenic variants in or . Weyers acrofacial dysostosis (WAD) is an ultra-rare dominant condition allelic to EvC. The present work aimed to enhance current knowledge on the clinical manifestations of EvC and WAD and broaden their mutational spectrum.

Long-term prognostic impact of anticoagulation on patients with atrial fibrillation undergoing hemodialysis.

Introduction And Objectives: Evidence for the efficacy and safety of oral anticoagulation with dicumarines in patients with atrial fibrillation (AF) on hemodialysis is controversial. The aim of our study is to evaluate the long-term prognostic implications of anticoagulation with dicumarines in a cohort of patients with non-valvular AF on a hemodialysis program due to end-stage renal disease.

Methods: Retrospective, observational study with consecutive inclusion of 74 patients with AF on hemodialysis.

[Comparison of the efficacy and safety of dronedarone and flecainide as maintenance antiarrhythmic therapy for sinus rhythm in atrial fibrillation].

Introduction And Objectives: Dronedarone and flecainide are the first pharmacological choice to reduce recurrence of atrial fibrillation (AF); however, there are no studies comparing them. A study was performed to compare the efficacy in terms of recurrence of AF and safety of both drugs.

Methods: A retrospective cohort study was conducted that included 123 consecutive patients treated with flecainide or dronedarone due to paroxysmal AF (76.

[Analysis of the safety culture in a Cardiology Unit managed by processes].

Unlabelled: Safety culture is one of the requirements for preventing the occurrence of adverse effects. However, this has not been studied in the field of cardiology. The aim of this study is to evaluate the safety culture in a cardiology unit that has implemented and certified an integrated quality and risk management system for patient safety.