Cooleys anemia

This patient is of Mediterranean descent, making thalassemia more likely. Furthermore, the microcytic (small red blood cell size) anemia in the face of elevated hemoglobin A2 and F is consistent with P-thalassemia minor. Patients with P-thalassemia major (Cooley anemia) typically have severe anemia requiring frequent transfusions and shortened life expectancy. Infants will appear healthy after birth, but as the hemoglobin F levels fall, the infant becomes severely anemic. Females with P-thalassemia major who survive beyond childhood are usually sterile. 

E. The correct diagnosis is thalassemia minor because the patient had been asymptomatic until age 7 years. If he had thalassemia major or Cooley anemia, he would have exhibited symptoms as early as his first birthday. Pernicious anemia leads to a macrocytic or megaloblastic anemia, whereas aplastic anemia is characterized by normal sized erythrocytes. 

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. 

Whipple GH, Bradford WL. Mediterranean disease thalassemia (erythroblastic anemia of Cooley) associated pigment abnormalities simulating hemochromatosis. J Pediatr 1936 9 279-311. 

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 ... 

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