Disorders Of The Embden-Meyerhof Cycle

Disorders of the Embden-Meyerhof cycle are autosomal recessive diseases of erythrocyte metabolism that lead to hemolytic anemia.

Pyruvate kinase deficiency is one such enzyme defect. In all these Stoffwechselwegdefekten hemolytic anemia occurs only in homozygotes. The exact mechanism of hemolysis is unknown. It found no spherocytes, but a small number of irregular spherically shaped red blood cells may be present. In general, the determination of ATP and diphosphoglycerate leads to the identification of each metabolic defect and allows the assignment in the metabolic chain, so that further investigations can be carried out. There is no specific treatment for these types of hemolytic anemia, and most patients will need other than the administration of folic acid of 1 mg po once a day no special treatment during acute hemolysis. Hemolysis and anemia persist even after a splenectomy, although in some cases, particularly in patients with pyruvate kinase deficiency, is actually improving.

Disorders of the Embden-Meyerhof cycle are autosomal recessive diseases of erythrocyte metabolism that lead to hemolytic anemia. Pyruvate kinase deficiency is one such enzyme defect. In all these Stoffwechselwegdefekten hemolytic anemia occurs only in homozygotes. The exact mechanism of hemolysis is unknown. It found no spherocytes, but a small number of irregular spherically shaped red blood cells may be present. In general, the determination of ATP and diphosphoglycerate leads to the identification of each metabolic defect and allows the assignment in the metabolic chain, so that further investigations can be carried out. There is no specific treatment for these types of hemolytic anemia, and most patients will need other than the administration of folic acid of 1 mg po once a day no special treatment during acute hemolysis. Hemolysis and anemia persist even after a splenectomy, although in some cases, particularly in patients with pyruvate kinase deficiency, is actually improving.

Health Life Media Team

Leave a Reply