Presse Med
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Apeced syndrome is a rare disease, with autosomal recessive transmission and associated with mutations of the AIRE gene, which is involved in central and peripheral immune tolerance mechanisms. Its diagnosis is classically based on the combination of any two of the following three major criteria: chronic mucocutaneous candidiasis, hypoparathyroidism and autoimmune chronic adrenocortical insufficiency (Addison disease). One single criterion is sufficient to diagnosis a sibling of a patient already diagnosed. ⋯ Potentially fatal disease (hepatitis or severe malabsorption) requires immunosuppressant therapy. Before beginning this aggressive treatment, underlying infectious foci, especially of candidiasis, must be sought and treated to prevent the development of extremely serious systemic infections in this context. A workup for splenic atrophy is also recommended.
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After publication of the 2005 international recommendations on resuscitation, a French panel of experts on cardiac arrest published guidelines adapted to French practice. Despite the absence of placebo-controlled trials, adrenaline remains the standard vasopressor for cardiac arrest, at a dose of 1 mg about every 4 minutes. Amiodarone has replaced lidocaine in treatment of refractory ventricular fibrillation. ⋯ Fibrinolysis should be considered only for cardiac arrest due to pulmonary embolism. Isotonic saline solution is recommended for infusions, and routine administration of sodium bicarbonate is not recommended. Intravenous access is necessary, but when unavailable rapidly,intraosseous is preferred to endotracheal delivery in adults and children
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An imbalance between prostacyclin (PGI2) and thromboxane A2 is observed in patients with scleroderma. Iloprost is a stable analogue of PGI2 with a plasma half-life of 20-30 min. Intravenous iloprost is effective in the treatment of Raynaud's phenomenon related to scleroderma, decreasing the frequency and severity of attacks. ⋯ Inhaled iloprost is an effective treatment for NYHA class III pulmonary arterial hypertension, either idiopathic primary or associated with a particular condition, such as scleroderma. Intravenous iloprost improves kidney vasospasm in patients with scleroderma. The possible benefits of sequential intravenous iloprost on the natural course of scleroderma require further investigation.
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The incidence of atrial fibrillation increases rapidly with population age. Atrial fibrillation essentially presents a problem of long-term management. ⋯ Anticoagulation treatment is indicated in most cases. Radiofrequency ablation by pulmonary vein isolation is an unquestionably innovative treatment but is reserved for selected patients.