PPT-Mediterranean Anemia-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 . Ashutosh Lal, MD. Northern California Thalassemia Center. UCSF Benioff Children’s Hospital Oakland. Thalassemia Syndromes: . Many diagnoses. Oakland Data (n=203). What is the proportion of . non-transfusion-dependent thalassemia. Anemia:. Deficiency of hemoglobin in the blood.. Causes: . Too few RBCs.. Too little hemoglobin in cells. Decreased packed cell volume.. This may be due to. :. decreased production of RBCs.. increased destruction.. Be able to classify the different types of anemia. Understand the signs and symptoms associated with anemia.. Being able to interpret lab abnormalities to diagnose different causes of anemia. . Introduction to Anemia. Be able to classify the different types of anemia. Understand the signs and symptoms associated with anemia.. Being able to interpret lab abnormalities to diagnose different causes of anemia. . Introduction to Anemia. .. . Anemia…. What is Anemia?. A condition characterized by a decrease in RBCs mass, hemoglobin or RBCs count.. Adult male: . Hb. .  13 g/. dL. , or . Hct. .: 41%.. Adult Females: . Hb.  12 g/. 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 red blood cell mass . or . blood hemoglobin concentration. . In practice, anemia most commonly is defined by reductions in one or both of the following:. Hematocrit (HCT) – The HCT is the fractional volume of a whole blood sample occupied by RBCs, expressed as a percentage. As an example, the normal HCT in a child age 6 to 12 years is approximately 40 percent.. a sign . of an underlying . disorder. There are several kinds of anemia. . A. . physiologic . approach classifies anemia according to whether. the deficiency in erythrocytes is caused by a defect in . referance. range for the age and sex. The normal ranges vary with age . -neonate < 14g/dl. -1-12mths < 10mg/dl. -1-12y< 11 g/dl. Causes of anemia can be from. Reduced cell production. Increased destruction. a pathophysiological condition in which the body cannot meet its demands for oxygen. . M: . Hb. <13.5 . Hct. <41 . F. : . Hb. <12 . Hct. <36 . Erythrocyte Indices . Hgb. M: 14.0-18.0 . 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 . 1- what is the mode of inheritance in the vast majority f spherocytosis cases?. Autosomal dominant. Autosomal recessive. X-linked dominant. X linked recessive. 2- The amino acid present at the sixth position of the normal alpha-globin chain is replaced by which one of the following amino acids in sickle cell disease?. 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 . Dr.Methal A. Alrubaie. Assistant professor. Objectives. Classify the types of anemia & explain the etiology behind each type.. Describe how an anemic women presented clinically.. Study the effect of anemia on both mother & fetus..