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Showing 3 results for Beta-Thalassemia
Zahra Razavi (md), Hasan Bazmamoun (md), Mohammad Sadegh Saba (md), Volume 10, Issue 4 (12-2008)
Abstract
Background and Objective: The thalassemic syndromes are geneticaly structural disturbance of Alfa and Beta globin chains. In major Beta-thalassemia the life expectancy depended on frequent blood transfusions that lead to over storage and deposition of Iron in different body-organs (Hemochromatosis) including parathyroid glands, which may cause hupoparathyroidism. This study was done to evaluate the frequency of hypoparathyroidism in thalassemic patients referred to teaching hospitals in Hamedan – Iran. Materials and Methods: This descriptive, cross-sectional study was done on 56 thalassemic patients, which received blood transfusion. Serum Ca , P , PTH , total protein , albumin , Ferritin and BUN cratinine were checked two weeks after last transfusion. The hypoparathyroidism was defined when calcium was less than 8mg/dl and phosphorus more than 5.5mg/dl and PTH less than 10 ng/dl. Results: In this study, 14.2% of patients had hypoparathyroidism. 37.5% of hypoparathyroid ones had clinical manifestation of hypocalcemia. There were statistically meaningful association (P<0.05) between The hypoparathyroidism with desferal administration, splenectomy and diabetes. Conclusion: This study showed that the frequency of hypoparathyroidism in Beta-thalassemic patients receiving blood transfusion was relatively high, so clinical and labratory evaluation for endocrine glands specially parathyroid is recommended since early second decay of life.
Valizadeh F (md), Mousavi A (bsc), Hashemi-Soteh Mb (phd), Volume 14, Issue 1 (3-2012)
Abstract
Background and Objective: According to world health organization statistics, at least 5.2% of world population is carrier for a main hemoglobin disorder. Previous reports showed that more than 10% of people are carrier for beta-thalassemia Northern Iran. This study was done to determine the prevalance of hemoglobinopathies in premarriage individuals referred to Babolsar, Iran.
Materials and Methods: This descriptive study was carried out on 8500 individuals (4200 women and 4300 men) whome were attended the thalassemia counseling program in Babolsar, North of Iran during 2006-09. After performing the CBC test, for those MCV and MCH were less than 80 and 27 respectively, Hemoglobin A2 was evaluated. Subjects whome were volunteers for more comprehensive tests, basic and acidic electrophoresis and genetic tests were applied, subsequently.
Results: 1200 (14.11%) subjects had low hematological indexes. 474 (5.57%) subjects had high HbA2 and were classified as beta-thalassemia carriers and 726 (8.54%) had normal HbA2 level and were classified as alpha-thalassemia carriers. 6 (1.2%) subjects were identified with HbF level more than 10 and were identified as carriers for beta-gene cluster deletion carrier. Also, 16 (3.2%) individuals had HbE, 16 (3.2%) had HbS, 4 had HbD and 4 had HbH (0.33% in 1200 and 0.047% in 8500 subjects). Genetic study of 317 individuals for beta carriers and 145 subjects for alpha-carriers showed IVSII-1G>A (74.5%) in beta-globin and single gene deletion of 3.7 (47.5%) in alpha-globin genes were the most frequent mutations.
Conclusion: This study showed that carriers for alpha - thalassemia (8.5%) are more frequent compared with beta- thalassemia (5.57%). Also other hemoglobin variants included HbS, HbE, HbD or different beta-gene cluster deletions in the region are considerable and should be screened.
Salehi M, Mirbehbahani Nb, Jahazi A, Aghebati Amlashi Y, Volume 16, Issue 1 (3-2014)
Abstract
Background and Objective: Thalassemias is the most common inherited disease in the world. This study was designed to assess general health of patients with beta-thalassemia major in Gorgan, northern Iran. Materials and Methods: This descriptive study was performed on 163 patients with beta-thalassemia major whom referred to Thalghani hospital in Gorgan, northern Iran. Socio-Psychosocial status of patients was assessed by using general health questionnaire-28 (GHQ-28). Results: Socio-Psychosocial status of 44.2%, 46% and 9.8% of patients was normal, suspected and suffered from psychosocial disorders, respectively. Social dysfunction (12.9%) and depression (10.4%) were the most prevalent and anxiety was the less prevalent (4.9%) in the patients. There were significant correlations between psychosocial status and mother’s ocupation (P<0.05), patients level of education (P<0.05) and history of associated diseases (P<0.05). Conclusion: Social dysfunction and depression were the two major general health complications in beta-thalassemia patients in this region.
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