[Foveal choroidal thickness assessment with SD-OCT in high myopic glaucoma]. / Épaisseur choroïdienne fovéolaire en OCT-SD dans le glaucome du myope fort.

PURPOSE: To measure macular choroidal thickness (CT) using spectral domain optical coherence tomography (OCT) in high myopic eyes with primary angle-open glaucoma (POAG), and to investigate whether the choroid is thinner in these eyes compared to high myopic eyes without glaucoma. PATIENTS AND METHODS: We conducted a cross-sectional study of forty-eight eyes with high myopic glaucoma matched with 48 highly myopic eyes without glaucoma by age, central corneal thickness and axial length (AL). OCT scans were performed with the spectral domain OCT (Topcon 2000). The subfoveal CT was measured between the Bruch membrane and the internal aspect of the sclera. RESULTS: In the subgroup without glaucoma, matched with the subgroup with glaucoma (P=0.57), by age, central corneal thickness (P=0.33) and AL (P=0.10), the mean subfoveal CT was 96.32 µm ± 39.56 µm. In the subgroup with glaucoma, the mean subfoveal CT was 50.44 µm ± 16.36 µm. The comparison between the two subgroups found a statistically significant difference in subfoveal CT (P<10(-4)). CONCLUSIONS: Foveal choroidal thickness is reduced in highly myopic eyes with glaucoma. The choroidal thinning can be a useful parameter for the diagnosis and the follow-up of highly myopic patients with glaucoma.

High frequency of autoimmunization among transfusion-dependent Tunisian thalassaemia patients.

BACKGROUND: Limited data are available on the frequency of RBC alloimmunization and autoimmunization in transfusion-dependent Tunisian ß thalassaemia patients. MATERIALS AND METHODS: We analyzed the clinical and transfusion records of 130 patients (57 females and 73 males; mean age 119 months; range 12-11 months) with ß thalassaemia major and who had regular blood transfusions for periods ranging from 12 to 311 months. RESULTS: Of the 130 patients, ten (7.7%) developed RBC alloantibodies. The most common alloantibodies were directed against antigens in the Rh systems. Erythrocyte-autoantibodies as determined by a positive direct antiglobulin Coombs test, developed in 52(40%) patients with and without underlying RBC alloantibodies, thereby causing autoimmune haemolytic anaemia in eleven patients (21%). CONCLUSIONS: Autoimmunization to erythrocyte antigens is a frequent complication in patients with ß thalassaemia major. Several factors might have contributed to the high autoimmunization rate observed in this study, including non phenotypic blood exposure and alloantibody formation prior to positive Coombs test.

Diabetic acido-ketosis revealing thiamine-responsive megaloblastic anemia.

Thiamine-responsive megaloblastic anemia (TRMA) is a rare autosomal recessive disorder characterized by megaloblastic anemia, diabetes mellitus and progressive sensorineural deafness. We report the cases of two infants, aged 4 and 5 months, hospitalized for diabetic ketoacidosis requiring insulin therapy. Laboratory tests revealed megaloblasic anemia, thrombocytopenia and normal thiamine level. Neurosensorial investigations showed bilateral deafness and ophthalmic involvement. Treatment with oral thiamine normalized hematological disorders and controlled diabetes; however, thiamine therapy had no impact on neurosensorial disorders.