PPT-Non-Transfusion-Dependent Thalassemia

Ashutosh Lal MD Northern California Thalassemia Center UCSF Benioff Childrens Hospital Oakland Thalassemia Syndromes Many diagnoses Oakland Data n203 What is the proportion of nontransfusiondependent thalassemia. Ellis J. Neufeld MD, PhD. Egan Family Foundation Chair in Transitional Medicine, Harvard Medical School. Associate Chief, Dana-Farber Boston Children’s Center for . Cancer and Blood Disorders . Chair, Medical Advisory Board, CAF. Thalassemia. Prof. Firoza Begum. High Risk Pregnancy & Infertility Specialist. Chairman, Fetomaternal Medicine . Dept. , BSMMU. Introduction. Hemoglobinopathies. are among the most common inherited diseases.. C TAGALOG Ano ang Thalassemia?ng Thalassemia ay isang uri ng makakuha ng sapat na oxygen ang dugo, atTaon-taon ay dumarami ang bilang ngmga sanggol na ipinapanganak na mayAno ang mga palatandaan ngMay C TAGALOG Ano ang Thalassemia?ng Thalassemia ay isang uri ng makakuha ng sapat na oxygen ang dugo, atTaon-taon ay dumarami ang bilang ngmga sanggol na ipinapanganak na mayAno ang mga palatandaan ngMay Thalassemia. Beta-thalassaemia is a global disease - most prevalent in South Asia, the Far East, the Middle East, and Mediterranean countries. . Distribution is attributed largely to natural selection of heterozygote carriers because of protection against falciparum malaria. . MD; FRCP (London); . FRCPEdin. ; . FRCSEdin. T. halassemia. Hemoglobin molecule. : is the iron-containing oxygen-transport . metalloprotein. in the red blood cells of all vertebrates.. The normal . haemoglobin. Thalassemia . is an inherited blood disorder in which the body makes an abnormal form of hemoglobin. Hemoglobin is the protein molecule in red blood cells that carries oxygen. Hemoglobin is formed by the combination of . : are genetic(AR) disorders in . globin. chain production. . In individuals with β. -thalassemia. , there is either: . complete. absence of β-globin gene production . (β. 0. -thalassemia). Kakavoulis. . Nikolaos. Patras Ioannis. What is . Thalassaemia. ?. . Thalassaemia. is a group of inherited disorders of hemoglobin synthesis characterized by . reduced . or . absen. ce. . of one . Silent carrier . α. -/. αα. . Normal complete blood count . α. -Thalassemia trait . αα. /- - (. α. -thalassemia 1) . or . α . -/. α . - (. α. -thalassemia 2) . Mild microcytic anemia . Hemoglobin H . THALASSEMIA. It result from reduced or absent production of beta chain in globin.. Common Hereditary Hemoglobin disorder(HHD). A. ) . Beta- . Thalassemia:Major,Intermedia,Minor. (trait . or carrier). .” . . –Robert Beal, past director of International Federation of Red Cross. Transfusions: History. 1660s: First experiments in blood transfusion, transfused dog blood to humans. Patient died and experiments were banned.. DEFINITION: . Thalassemia is blood disorder passed down through families (inherited) in which the body makes an abnormal form or inadequate amount of . haemoglobin. .. CAUSE:. . Haemoglobin. made of 2 proteins: . F.I.B.M.S (Int. Medicine) , F.I.B.M.S (Hematology). DEFINITION. Thalassemias . are inherited blood disorders characterized by defect . in the rate of synthesis of one . of the . globin chains.. Resultant imbalance of globin chain production may cause ineffective .