Recurrent Fetal Loss and Antiphospholipid Antibodies: Clinical and Therapeutic Aspects

Recurrent fetal losses indicate screening for antiphospholipid antibodies, especially after the third consecutive fetal loss, or when they occur after 12 weeks gestation or when the mother presents with thrombosis or other ailments of antiphospholipid syndrome. Fetal loss may be caused by thromboses of placental vasculature. There is no agreement concerning the mechanism of thromboses: protein C pathway and/or annexin V are the best candidates. When fetal loss occurs early during gestation, murine models suggest that antiphospholipid antibodies can also act on trophoblasts by inhibiting syncytia formation. Among the high risk patients with more than two fetal losses, an association of aspirin and heparin given early during gestation is successful in 70–80% of cases

La maladie de Fabry chez l'adulte: aspects cliniques et progrès thérapeutiques [Fabry disease in adulthood: clinical aspects and therapeutic progress].

INTRODUCTION: Fabry disease is an X-linked recessive abnormality of glycosphingolipid metabolism that is due to deficiency of the lysosomal enzyme alpha-galactosidase A. CURRENT KNOWLEDGE AND KEY POINTS: A majority of hemizygous men develop severe multisystemic disease (classic form), dominated by renal failure, progressive neurological and cardiac involvement. Nevertheless, some affected men retain sufficient enzyme activity and long remain asymptomatic (atypical form); their main manifestation is hypertrophic cardiomyopathy. Female heterozygous carriers are usually asymptomatic; 15% of them, however, have severe involvement of one or several organs. Laboratory, histologic and molecular diagnosis identifies 100% of hemizygous and over 80% of heterozygous subjects. FUTURE PROSPECTS AND PROJECTS: With developments in molecular genetics, it is now possible to produce the human recombinant enzyme alpha-galactosidase A. Two recent studies had proven that this therapeutic approach was able to be clinically and histologically effective in men. In addition, the results of a trial of gene therapy in a Fabry gene knocked-out mouse appear promising

[Visceral lesions in hypereosinophilia].

International audienceThe hypereosinophilic syndrome is an ill-defined nosological entity with predominant risks of cardiac and/or neurological lesions. In the light of new data on the effector cytotoxic effects of eosinophils, we have tried to establish new criteria of severity by purifying the circulating eosinophils of 14 patients with hypereosinophilic syndrome and testing their toxicity. This study has revealed the existence of low density ("hypodense") eosinophils with potential cytotoxicity in vitro. The worst clinical forms of the syndrome were observed in the group of patients with positive eosinophil toxicity tests. The significance of these cellular changes (hypodensity, eosinotoxicity) and their relationship with the clinical manifestations are discussed