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People with chronic anemia such as thalassemia or sickle cell anemia often require regular red blood cell transfusions. Repeated infusions produce toxic and ultimately fatal iron accumulation in multiple tissues of the body as insoluble ferritin. This chronic iron overload occurs because the body cannot eliminate iron efficiently. Chronic iron overload is a serious symptom, and organ failure can occur due to the iron deposits produced. When the heart or liver is affected, the condition can be life-threatening. Iron overload is treated by administering iron chelators, which drive iron deposits into soluble complexes that can be excreted from the body. Many patients, such as those with thalassemia, sideroblastic anemia, myelodysplastic syndrome and aplastic anemia, require long-term dependence on blood transfusion treatment, which leads to increased iron deposition in the body and secondary transfusion-related iron overload, which causes tissue damage and functional damage to multiple organs. Cardiac complications including myocardial infarction, congestive heart failure, and arrhythmia are the most common causes of death. Iron chelators are a new type of iron removal treatment based on directional binding of iron ions in the body, effectively increasing iron excretion, reducing iron content in the body and its pathological deposition in various organs. It is also the only effective drug currently used to treat iron overload. The application of iron chelators has greatly reduced the mortality and improved the quality of life of patients with long-term blood transfusions, and its clinical application has been receiving widespread attention and attention. The iron chelators currently used clinically mainly include deferoxamine (DFO), deferiprone (DFP), and deferiprone (DFS).

Is Enrig listed on the market?

Enrig was approved by the FDA in November 2005 for use in patients 2 years and older with chronic iron overload caused by blood transfusions. In December 2012, Enrig was approved by the European Commission for the treatment of chronic iron overload in patients aged 10 years and above with non-transfusion-dependent thalassemia (NTDT) syndrome who require chelation therapy due to contraindications or insufficiency of deferoxamine mesylate therapy. On January 23, 2013, the FDA approved the new indication of Enriga for the treatment of chronic iron overload in patients with non-transfusion-dependent thalassemia (NTDT) aged 10 years and above.

The recommended starting daily dose is 20 mg/kg. For patients who receive monthly transfusions of more than 14 mL/kg of packed red blood cells (i.e., more than 4 units/month in adults) and who need to reduce excess iron exposure, a starting dose of 30 mg/kg/day may be considered. For patients who receive monthly transfusions of less than 7 mL/kg of packed red blood cells (i.e., less than 2 units/month in adults) and who need to maintain body iron balance, a starting dose of 10 mg/kg/day may be considered.