Table 1 The distribution of Cosenza mutations in some provinces of Iran We also studied the association of Mediterranean and Cosenza mutation with (1311C→T) haplotype. The haplotype analysis revealed that unlike Mediterranean G6PD, mutation was associated with (1311C) haplotype. Fifty eight samples, which have not Mediterranean and mutations, are kept for identification of other G6PD mutations. Conclusion The findings of the present study
indicate that G6PD Cosenza is a common mutation in Khuzestanian G6PD deficient individuals. Acknowledgment This study was financially supported by a grant (no. 3587) from the Shahid Cabozantinib Chamran University of Ahvaz, Iran. Conflict of Interest: None Inhibitors,research,lifescience,medical declared
Duchenne muscular dystrophy (DMD) is a congenital, chronic degenerative muscle disorder that results in loss of ambulation, respiratory compromise and cardiac dysfunction (1). Corticosteroids are the standard of care for the treatment of DMD (1-4). Prednisone, prednisolone and deflazacort are the corticosteroids used to treat DMD. Inhibitors,research,lifescience,medical Corticosteroids have been shown to prolong independent ambulation, improve pulmonary
Inhibitors,research,lifescience,medical function, delay the onset of cardiomyopathy and reduce the incidence of scoliosis (1, 2, 5). Here we examine the Canadian clinical experience with deflazacort. Deflazacort is an oxazoline derivative of prednisolone with anti-inflammatory and immunosuppressive activity (6, 7). The effect of deflazacort on the progression of symptoms in DMD as well as the side effect profile have been characterized (8-18). Compared to prednisone, deflazacort has been shown in other diseases to cause fewer side effects including better preservation of bone mass (19-22), less weight gain (19, 20, 22, 23), better lipid profile Inhibitors,research,lifescience,medical (20, 22, 24) and less glucose intolerance (24, 25). A direct comparison of deflazacort and prednisone in DMD has Inhibitors,research,lifescience,medical been studied in a multicenter, double-blind, randomized trial of 18 patients over one year of treatment (14). There was no significant difference in motor outcomes however, there
was less weight gain in the group treated with deflazacort compared to prednisone (2.17 kg vs. 5.08 kg) (14). Two patients developed small cataracts in the deflazacort group and none were observed in the prednisone group. Other side effects were equally distributed including Phosphatidylinositol diacylglycerol-lyase behaviour changes, increased appetite, cushingoid appearance, hirsutism and gastric symptoms (14). There is an international study planned to compare two prednisone dosing schedules to daily deflazacort (26). Biggar et al. (9) compared two different deflazacort protocols; one from Toronto (0.9 mg/kg daily) and one from Naples (0.6 mg/kg/d for the first 20 days of the month). Benefits were seen with both protocols, however, the higher daily dose, Toronto protocol, resulted in prolonged ambulation (77% at 15 years compared to 25% at 15 years) and patients were less likely to develop scoliosis (16% compared to 30%).