RESUMO
Background: Ongoing transfusional iron load (TIL) is an important determinant while deciding starting and subsequent dose adjustment of deferasirox during course of chelation therapy. So present study aims to find out effect of different dosing of deferasirox over the serum ferritin level in children with thalassemia major with impact of rate of transfusional iron load.Methods: This one year observational study was carried out in 35 transfusion dependent ?-thalassemic patients aged 2-18 years. Patients with baseline serum ferritin 1000-1500ng/ml and/or receiving TIL 0.2-0.3mg/kg/day were started 20mg/kg/day deferasirox and patients with ferritin>1500ng/ml and/or having TIL > 0.3mg/kg/day were started 30mg/kg/day deferasirox. Serum ferritin was repeated in every three months. Dose adjustments were performed on serum ferritin trends in steps of 5-10mg/kg /day to maximum 40mg/kg/day. Evaluation of relationship between dose adjustment, percentage of reduction in serum ferritin and TIL was done.Results: Group-1 patients(42.8%) had TIL 0.2 to 0.3mg/kg/day whereas Group-2(37.1%) and Group-3(20%) children had TIL >0.3-0.4mg/kg/day and >0.4 mg/kg/day respectively. Starting dose of deferasirox in 25.7% patients was 20mg/kg/day and in rest were 30mg/kg/day. Average dose of deferasirox in group-1 was significantly lower as compared to group-2 and group-3 patients ( p< 0.05). Significant decline in mean serum ferritin was observed in all three groups (p < 0.05). There was a significant positive correlation between TIL and average drug dose prescribed (r=0.5411and p=0.0007) but negative insignificant correlation was observed with percentage of reduction in serum ferritin(r=0.0027and p=0.98).Conclusions: Deferasirox 30mg/kg/day significantly reduces serum ferritin and is well tolerated in majority of patients having TIL 0.3-0.4mg/kg/day where as 20mg/kg/day is required in patients having low transfusional iron intake.
RESUMO
Globins are heme proteins that are capable of reversible oxygen binding. All globins can be classified into three families: the M (myoglobin-like), S (sensor) and T (truncated) globins. M and S globins exhibit the canonical 3/3 α-helical fold, and T globins are characterized by a 2/2 α-helical fold. Globins in the genomes of myxobacteria have not been characterized till date. Myxobacteria have very large genomes relative to other bacteria and have a unique life cycle that involves the aggregation of cells into fruiting bodies under starvation conditions. The diversity of globin like sequences in 14 sequenced genomes of myxobacteria is presented in this review. In myxobacterial globins some unusual domain architectures are identified that have not been characterized in bacteria so far; these are: i) a unique chimeric group I 2/2 HbN in the genome of Corallococcus coralloides DSM 2259; ii) M globin chimera harboring a central and a C-terminal globin domain in Sorangium cellulosum ‘so ce 56’ and Plesiocystis pacifica SIR-1 respectively; iii) two tandem globin domains on the same M globin polypeptide in the genomes of Sorangium cellulosum.