Cooley s anemia

In the treatment of poisoning by lead or other metal ions, higher concentrations of chelant can be safely obtained in humans by administering Na2CaEDTA rather than Na EDTA. The metal ion is bound by displacing small amounts of Ca " that the body can tolerate. Use of Na EDTA would result in calcium chelation and thus serious depletion of calcium in the body fluids (44). Removal of iron in Cooley s anemia is accompHshed by using chelants that are relatively specific for iron (45). 

An Attempt to Unify Some Observations In Thalassemia and In Related Conditions". Proc. Third Cooley s Anemia Conf. April, 1973. Ann. N. Y. Acad. Scl., (1974), 232 107. Hulsman, T. H. J. "Normal and Abnormal Hemoglobins". 

Anticancer, antiviral, treatment of arthritis, antibacterial, antifungal, antifouling, treatment of Cooley s anemia, algicides, molluscicides, contrast agents, radiology agents... 

The use of chelating or complexing agents to treat metabolic dysfunction. The classical example is the use of D-penicillamine to treat Wilson s disease, which is caused by an inability of the body to metabolize copper in the normal way. Another example is the use of desferrioxamine for iron overload in Cooley s anemia, which is caused by a fault in hemoglobin synthesis. 

Iron 3-5 g 10 mg (males) 18 mg (females) Electron transport (cytochromes), oxygen carrier (haemoglobin), storage/transport (ferritin), enzymes, immune system Widespread geographically fatigue, anemia Danger in hemochromatosis, Cooley s anemia, acute poisoning, Bantu siderosis... 

P -Thaiassemia (p-Thaiassemia Major). Sometimes called Cooley s anemia after the physician who in 1925 first described the condition in the children of Italian and Greek immigrants in New York by noting that these children failed to grow, had frequent infections, appeared pale and malnourished, had splenomegaly, and had facial bone changes. 

Design of Polymeric Iron Chelators for Treating Iron Overload in Cooley s Anemia... 

Cooley s Anemia is a genetic disorder relatively rare in the United States, but found more often among peoples of the Mediterranean area and within a band extending through India and southeast Asia ( 1 ). In addition to severe anemia, the disease causes bone malformation and general deterioration and distruction of the vital organs leading eventually to death. 

Several powerful iron chelators, some recently synthesized specifically for the Cooley s Anemia program, are being tested to ascertain their potential as drugs for use in iron chelation therapy for treating iron overload in Cooley s Anemia. Some of the more promising ones include ... 

Acknowledgment. The direct support of this work by the Cooley s Anemia Foundation is greatly appreciated. The biological testing was performed by the Mason Research Institute, Worcester, Mass, and R. Grady, Rockefeller University, New York, under contracts with the National Institutes of Health, NIAMDD. 

Characteristics of the more common /3-thalassemia syndromes are summarized in Table 28-4. Not included are two different abnormal heterozygotes. Anemia results from precipitation of excess a-globin chains, premature red cell destruction in bone marrow and the circulation, and deficiency of functional hemoglobin tetramer. /S-Thalassemia major, or Cooley s anemia, occurs when /3-globin synthesis is markedly depressed or absent. The ineffective erythropoiesis causes massive erythroid proliferation, skeletal deformities, and extramedullary erythropoiesis. The usual treatment is frequent blood transfusion. 

Thalassemia Cooley s anemia (thalassemia major) + -1- P -mRN A markedly reduced -genes present... 

HB Lepore Cooley s anemia 0 0 (and25%Hb Lepore) 75% Absence of normal J3- and 5-mRNA Normalj3-and 5-genes absent, 5j3-fusiongene present... 

Defective erythropoiesis (dyserythropoiesis) impaired hemoglobin synthesis leading to lack of utilization and consequent accumulation of iron in mitochondria, e.g., from inhibition of ALA synthase activity by dietary vitamin 85 deficiency inhibition of heme synthesis by lead impairment of pyridoxine metabolism in alcoholic patients familial sideroblastic anemias and Cooley s anemia. 

Repeated blood transfusions, e.g., in Cooley s anemia or sickle cell disease. 

In hemochromatosis secondary to refractory anemias (e.g., Cooley s anemia, sickle cell anemia), patients require repeated blood transfusions to survive childhood and adulthood. Therapy consists of administration of ironchelating agents. Deferoxamine, an iron chelator isolated from Streptomyces pilosis, has the structure ... 

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