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  • Cooley's anemia
    "Ä𸮠ºóÇ÷(Þ¸úì), = thalassemia"
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Cooley's anaemia <haematology> A genetic disease that results when there is a defect in the synthesis of one (or more) of the subunits of haemoglobin. The subunits are referred to as alpha or beta globin chains. As a result of a genetic defect red blood cells are small and hypochromic (low haemoglobin in the cell). Anaemia is common. Incidence is approximately 4 out of 100,000 individuals. Genetic testing is recommended in pregnant females of Mediterranean descent for the purpose of identifying carriers of this potentially serious genetic disease.
Origin: Gr. Haima = blood
(27 Sep 1997)
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anaemia, Cooley's Better known today as thalassaemia (or as beta thalassaemia or thalassaemia major).The clinical picture of this important type of anaemia was first described in 1925 by the paediatrician Thomas Benton Cooley. Another name for the disease is Mediterranean anaemia. The name thalassaemia was coined by the Nobel Prise winning pathologist George Whipple and the professor of paediatrics Wm Bradford at Univ. Of Rochester because thalassa in Greek means the sea (like the Mediterrranean Sea) + -aemia means in the blood so thalassaemia means sea in the blood. Thalassaemia is not just one disease. It is a complex contingent of genetic (inherited) disorders all of which involve underproduction of haemoglobin, the indispensable molecule in red blood cells that carries oxygen. The globin part of normal adult haemoglobin is made up of 2 alpha and 2 beta polypeptide chains. In beta thalassaemia, there is a mutation (change) in both beta globin chains leading to underproduction (or absence) of beta chains, underproduction of haemoglobin, and profound anaemia. The gene for beta thalassaemia is relatively frequent in people of Mediterranean origin (for example, from Italy and Greece). Children with this disease inherit one gene for it from each parent. The parents are carriers (heterozygotes) with just one thalassaemia gene, are said to have thalassaemia minor, and are essentially normal. Their children affected with beta thalassaemia seem entirely normal at birth because at birth we still have predominantly foetal haemoglobin which does not contain beta chains. The anaemia surfaces in the first few months after birth and becomes progressively more severe leading to pallor and easy fatiguability, failure to thrive (grow), bouts of fever (due to infections) and diarrhoea. Treatment based on blood transfusions is helpful but not curative. Gene therapy will, it is hoped, be applicable to this disease.
(12 Dec 1998)
Cooley, Thomas <person> U.S. Paediatrician, 1871-1945.
See: Cooley's anaemia.
(05 Mar 2000)
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Cooley's anemia a fatal form of homozygous thalassemia (inherited from both parents) in which there is no hemoglobin; skeletal deformations; heart and spleen and liver enlarged
Ãâó: wordnet.princeton.edu/perl/webwn
Cooley's anemia (Coo
Ãâó: www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspz...
Cooley's a. thalassemia major.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
Cooley's anemia, disease see thalassemia.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
Cooley's d. thalassemia major.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
WordNet ÀÏ¹Ý ¿µ¿µ »çÀü °Ë»ö °á°ú : 2 ÆäÀÌÁö: 1
cooley\'s a fatal form of homozygous thalassemia (inherited from both parents) in which there is no hemoglobin
cooley\'s a fatal form of homozygous thalassemia (inherited from both parents) in which there is no hemoglobin
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