Analysis of clinical features and complications in patients with β-Thalassemia in the region of Strumica

Sinokapovska, Silvana and Dimova, Cena (2015) Analysis of clinical features and complications in patients with β-Thalassemia in the region of Strumica. In: International Symposium at Faculty of Medical Sciences “Current achievements and future perspectives in medical and biomedical research”, 24 Nov 2015, Stip, Macedonia.

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Abstract

Thalassemias are hemoglobinopathies and are hereditary deseases which are characterized by disturbance in synthesis of one or more polypeptide chains of globin. Thalassemias are type of anemia of quantitative nature, where synthesized chain of hemoglobin are with normal structure, but not in adequate quantities. β-thalassemia is a desease characterized by production of large quantities of α-chains which exceedes the production of β-chains, whereas β-chains accumulate and precipitate into precursors of red blood cells and change their permeability, which leads to lysis of red blood cells. Within Research Center for Genetic Engineering and Biotechnology (RCGEB) is operating national reference laboratory for hemoglobinopathies, founded in 1970, where over the past 40 years examined more than 30,000 individuals from our country. The analysis revealed that the average frequency of beta-thalassemia in Macedonia is 2.6%, of alpha thalassemia is 1.5%, delta-beta-thalassemia is 0.2%, while the share of Swiss type of hereditary persistence of fetal hemoglobin (HPFH) is 0.3%.The objective of this study was to show the prevalence, or the presence, of a certain type of thalassemia in the region of Strumica, R. of Macedonia, the complications that arise in specific cases, and the diagnosis and treatment of this inherited disease, not only for successfully identifying the extent of the presence of thalassemias in this region, but also to display the patient status in this region.
The data analysis indicates the presence of many characteristic sympthoms and clinical manifestation of many features of thalassemias, which confirms the theoretical framework for this hemoglobinopathy.
In many patients it is observed decline in values of hemoglobin, reduces number of red blood cells, and disruption of liver function, followed by decreased function of liver enzymes.
Therapy that is administered to the patients is of basic character, a continuous blood transfusion in patients with milder form of thalassemia, and excess iron chelators, whilst in the patients with severe forms of thalassemia spelenctomy was performed.
The results shows that the experimental group of patients present many comorbidities and complications thay complays with the medical theory about thalassemias.

Item Type:	Conference or Workshop Item (Poster)
Subjects:	Medical and Health Sciences > Basic medicine Medical and Health Sciences > Clinical medicine Medical and Health Sciences > Health sciences Medical and Health Sciences > Other medical sciences
Divisions:	Faculty of Medical Science
Depositing User:	Cena Dimova
Date Deposited:	22 Jan 2016 13:10
Last Modified:	22 Jan 2016 13:10
URI:	https://eprints.ugd.edu.mk/id/eprint/14924

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