Beta Thalassemia Major in a Developing Country: Epidemiological, Clinical and Evolutionary Aspect

Abstract

Beta-thalassemia major (TM) remains to be one of the major health problems particularly in developing countries. Tunisia is a part of the Mediterranean countries mostly affected by this disease which is highly concentrated in small towns in families with low-income earners. The main objectives of this study are to provide a description of the demographic, clinical features and transfusion-related complications in patients with TM living in Tunisia. A standardized questionnaire was sent to clinicians throughout 33 different medical institutions caring for thalassemic patients. 391 transfusion dependant thalassemic patients with a median age of 10.7 years (range 3 months- 31 years) were included in the study. The majority originated from the north west of the country. A moderate iron overload between 1501 and 2500 ng/ml was found in 61patients, while 81 patients (26.9%) had a ferritin level more than 2500 ng/ml and greater than 5000ng/ml in 21 patients (6.9%). 51 patients died from complications related to their disease. Heart failure was the main cause of death. The incidence of cardiac, endocrine, and infectious complications will be reviewed. Preventive measures such as health education, carrier screening and premarital screening remain the best ways for lowering the incidence of these diseases, which might be reflected in financial saving, social s and health benefits.

Hereditary Hematologic Disorders in Najaf Province-Iraq

“…About one-third (33%) of the cases were in the age group 7-10 years, followed by the age group 3-6 years. This result is in agreement with Bejaoui and Guirat reporting a median age of 10.7 years among thalassemic patients in Tunisia (35,38). In contrast to these results, in 2016, lower mean age of 3.7 years was reported in a similar study carried out in India.…”

“…Most importantly, our results show a highly increased frequency of close family members' marriages, most often cousins, in patients with HHD (78.24%) as compared with the expected consanguinity rate of 45.8%. One study, conducted in Tunisia, reported a consanguinity rate of 75.3% among the patients' parents (38). Another study reported that 40.6% of β-thal patients occurred in the offspring of first-cousin marriages (31).…”

Ischemia-modified albumin as a marker of vascular dysfunction and subclinical atherosclerosis in β-thalassemia major

“…Mokhtar and associates [53] reported the presence of cardiovascular morbidities in 41.3% of β-thalassemia patients with cardiomyopathy being the most frequent (6.9%), followed by arrhythmia (1.6%) and PH (0.4%). Another study done by Bejaoui and Guirat [54] showed that cardiac disorders occurred in 19.8% of the studied β-TM patients with higher incidence of PH (12.5%) and heart failure (39%) being the major leading cause of death.…”

Β Thalassemia; A Case Report of Oral and Maxillofacial Manifestation From Population of Karachi Pakistan

“…Thalassemia minor. 12 In thalassemia due to lack of total or partial production of α or β or globin results serious effects on their bodies, 2,3,9,11,13-16 Beta thalassemia is also responsible of causing various manifestations and complications, resulting multisystem complications due to chronic anemia, iron overload, adverse effects of chelation, and infection due to transfusion. Thus, just a simple hemolytic disease 'anemia' change to a chronic disease with involving various organs with various degree of deformities.…”