Showing papers by "Raffaella Origa published in 2008"

Onset of cardiac iron loading in pediatric patients with thalassemia major

Abstract: We reviewed cardiac T2* assessments from 77 thalassemia major patients between the ages of 2.5 and 18 years to study optimal timing of cardiac iron screening by magnetic resonance imaging. No patient under 9.5 years of age showed detectable cardiac iron in contrast to 36% of patients between the ages of 15–18 years old, corresponding to an odds-ratio of 1.28 (28%) per year. All patients with cardiac iron had received at least 35 grams of transfusional iron. Liver iron and ferritin failed to predict cardiac iron loading. Initiation of cardiac magnetic resonance imaging assessment should be determined according to age and transfusional burden rather than indices of iron overload. When appropriate chelation therapy has been administered since birth, cardiac magnetic resonance imaging can be postponed until 8 years of age when anesthesia is not required. Patients with suboptimal chelation, increased transfusional requirements, or who have initiated transfusions later in life should be tested sooner.

No evidence of cardiac iron in 20 never- or minimally-transfused patients with thalassemia intermedia.

Abstract: Iron loading in non-transfused thalassemia intermedia (TI) patients is mainly due to an increased intestinal absorption secondary to chronic anemia and varies from 2 to 5 grams per year.[1][1] In thalassemia major (TM) iron derived from red blood cell breakdown accumulates first in the

Effect of food, type of food, and time of food intake on deferasirox bioavailability: recommendations for an optimal deferasirox administration regimen

Abstract: Deferasirox (ICL670) is representative of a new class of tridentate iron chelators, formulated as tablets for dispersion. Deferasirox has exhibited high potency and a clinically manageable safety profile in preclinical models and in an extensive clinical program. The effect of food and time of food intake on the pharmacokinetics of deferasirox was investigated in healthy volunteers and patients with transfusional hemosiderosis. The bioequivalence of a single oral dose of deferasirox (20 mg/kg) was assessed following administration either before a high-fat or standard breakfast or concurrent with a standard breakfast in comparison with fasted conditions in healthy volunteers. The bioavailability of deferasirox was determined following a single oral dose (20 mg/kg) under fed and fasted conditions in patients. These data show that the type of food, caloric content, and fat content of the meal influence the bioavailability of deferasirox when consumed concomitantly. In contrast, this is not the case when deferasirox is administered at least 30 minutes before a meal. In conclusion, it is recommended that deferasirox be administered at least 30 minutes prior to meals. When this is not feasible, deferasirox should be administered consistently at the same time before meals to limit the sources of variability that affect absorption.

Glutathione S-transferase gene polymorphism and cardiac iron overload in thalassaemia major

Abstract: Chorlton, I., Karnei, Jr, R.F., King, F.M. & Norris, H.J. (1974) Primary malignant reticuloendothelial disease involving the vagina, cervix and corpus uteri. Obstetrics and Gynecology, 44, 735–738. Dursun, P., Gultekin, M., Bozdag, G., Usubutun, A., Uner, A., Celik, N.Y., Yuce, K. & Ayhan, A. (2005) Primary cervical lymphoma, report of two cases and review of the literature. Gynecologic Oncology, 98, 484–489. Engin, H., Türker, A., Abali, H., Uner, A. & Günalp, S. (2004) Successful treatment of primary non-Hodgkin’s lymphoma of the vagina with chemotherapy. Archives of Gynecology and Obstetrics, 269, 208–210. Freeman, C., Berg, J. & Cutler, S. (1972) Occurrence and prognosis of extranodal lymphomas. Cancer, 29, 252–260. Hariprasad, R., Kumar, L., Bhatla, D.M., Kukreia, M. & Papaiah, S. (2006) Primary uterine lymphoma, report of 2 cases and review of literature. American Journal of Obstetrics and Gynecology, 195, 308– 313. Lagoo, A. & Robboy, S. (2005) Lymphoma of the female genital tract, current status. International Journal of Gynecological Pathology, 25, 1–21. Sandvei, R., Lote, K., Svendsen, E. & Thunold, S. (1990) Successful pregnancy following treatment of primary malignant lymphoma of uterine cervix. Gynecologic Oncology, 38, 128–131.

Once-daily oral deferasirox for the treatment of transfusional iron overload.

Abstract: The increasing use of blood transfusions, combined with extended patient survival, has led to an increase in the number of patients at risk of developing transfusional iron overload. Clinical data have shown that the once-daily oral iron chelator deferasirox is effective in adults and children with various transfusion-dependent anemias, including β-thalassemia and the myelodysplastic syndromes. Deferasirox has a defined, clinically manageable safety profile. The most common treatment-related adverse events are mild gastrointestinal disorders, skin rash and mild, nonprogressive serum creatinine increases. The deferasirox clinical trial program is continuing in Phase II/III extension phases and Phase IV trials. Long-term data continue to support the efficacy and safety of deferasirox. Convenient, effective and tolerable chelation therapy with deferasirox is a significant development in the treatment of transfusional iron overload.