[Dermatological features of auto-inflammatory recurrent fevers]. / Peau et fièvres récurrentes auto-inflammatoires.

Auto-inflammatory diseases are characterized by unexplained and recurrent attacks of systemic inflammation often involving the skin, joints, or serosal membranes. They are due to a dysfunction or dysregulation of the innate immunity, which is the first line of defense against pathogens. Early recognition of these diseases by the clinician, especially by pediatricians encountering such pathologies in pediatric patients, is primordial to avoid complications. Skin manifestations, common in most auto-inflammatory diseases, are helpful for prompt diagnosis. After a brief physiopathological review, we will describe auto-inflammatory recurrent fevers by their main dermatological presentations: urticarial lesions, neutrophilic dermatoses, panniculitis, other maculopapular eruptions, dyskeratosis, skin vasculitis, and oral aphthous. We finally suggest a decision tree to help clinicians better target genetic exams in patients with recurrent fevers and dermatological manifestations.

[LEFT ATRIAL APPENDAGE CLOSURE IN A PATIENT WITH SYSTEMIC SCLEROSIS]. / LE CAS CLINIQUE DU MOIS. Fermeture percutanée de L'auricule gauche chez une patiente souffrant de sclérodermie.

We report the clinical history of a 69 year-old female who suffered from systemic sclerosis and in whom we performed a percutaneous left atrial appendage closure due to recurrent gastrointestinal bleedings under anticoagulant therapy for chronic atrial fibrillation. We review the impact of scleroderma on the cardiac and digestive systems and discuss the issue of anticoagulation and its alternatives in uncommon clinical situations. We also describe the indications, technical aspects and potential complications of percutaneous left atrial appendage closure.

[A left intraventricular mass]. / Le cas clinique du mois. A propos d'une masse intra-ventriculaire gauche.

We report the case of a 29-years-old male presenting with a large mass inserted at the hypokinetic apex of the left ventricle. Without any early regression under anticoagulant therapy and taking into account recent neurological manifestations, surgical extraction was decided. The mass corresponded to a chronic thrombus lying on a non-transmural myocardial necrosis. This case gives us the opportunity to review all causes of intracardiac masses.

Incomplete myocardial rupture following inferior myocardial infarction: a case report.

In an era of early and invasive therapeutic approaches, myocardial rupture has become an uncommon complication of myocardial infarction. While septal wall rupture most often leads to devastating haemodynamic consequences, free wall rupture is usually fatal. We report a case of a 48-year-old man in whom an incomplete myocardial rupture located in the inferior part of the interventricular septum was promptly detected during the acute phase of an inferior myocardial infarction treated by early percutaneous coronary angioplasty. A conservative rather than a surgical approach was decided with a favourable short-term outcome.

[Clinical value of cardiac resynchronization therapy in patients with congestive heart failure]. / Resynchronisation ventriculaire dans la décompensation cardiaque réfractaire.

Besides major progress in the pharmacologic treatment of severe chronic heart failure, cardiac resynchronization therapy (CRT) has developped over the last ten years. We report the follow-up of the 36 first patients with a CRT device implanted from July 2000 to November 2002 at the CHR de la Citadelle Hospital in Liege. After a mean follow up of 6 months, no death was observed. The functional benefit of resynchronization is validated by a significant improvement in the NYHA class, an increase in the walking distance measured by the six minute walk test from 268 +/- 103 to 342 +/- 106 meters (p < 0.004) and by a not significant rise in the VO2 max (from 11.1 +/- 2.8 to 14 +/- 10 ml/kg/min; P=0.1). The quality of life, assessed by the Minnesota-Living-In-CHF score, improves from 49 +/- 20 to 35 +/- 22 after the six month follow-up (P=0.02) The echocardiogram also shows a better left ventricular ejection fraction at six months, from 24 +/- 7% to 31 +/- 7% (P<0.05). Based on a better NYHA functional class, responders (n=24; 71%) and non responders (n=10; 29%) were compared; a correlation between the functional class change and the improvement of the ejection fraction was documented, but not with the reduction in QRS width. Our registry, with the potential pitfalls of a monocentric prospective study, confirms the feasability, safety and efficacy of CRT in severe chrbnic heart failure uncompletely corrected pharmocalogically. It remains however approximately 30% of non responding patients, in whom the current clinical studies should help identify the right criteria to predict and discriminate responders.