Thalassemia and other Hemoglobinopathies in Bangladeshi Children

: Background- Worldwide, Thalassemia and Hemoglobinopathies are the most common inherited disorders with a wide geographical variation in incidence. Bangladesh is situated in a thalassemia tending region but this hereditary disorder receives little attention yet and unfortunately there is no definitive national data regarding incidence and socio-demographic profile of Thalassemia and Hemoglobinopathies in the country. Objective- This study was undertaken to determine the current situation of the occurrence and socio-demographic profile of thalassemia and other hemoglobinopathies among Bangladeshi children. Methods- It was a prospective study conducted from January 2015 to December 2016.  All newly diagnosed children with Thalassemia major and other Hemoglobinopathies in Dhaka Shishu Hospital (children) Thalassemia center were included in the study. Consideration was given to total number of cases, age at presentation, sex distribution and types. Hb% was done in every patient after enrollment.   Result- Among total 432 affected children, male female ratio was 1.6:1 in which E-β Thalassemia, β Thalassemia major and Hb E disease were 68.50%, 31% and 0.5% respectively. Consanguineous marriage was found in 14% parents. Conclusions- E-β Thalassemia was found the commonest type of thalassemia among Bangladeshi children.

[1]  A. Mannan,et al.  A Demographic Approach for Understanding the Prevalence of β Thalassemia Patterns and Other Hemoglobinopathies: Selective Study in Chittagong City Perspective , 2013 .

[2]  T. Emran,et al.  A study of the prevalence of thalassemia and its correlation with liver function test in different age and sex group in the Chittagong district of Bangladesh , 2012, Journal of basic and clinical pharmacy.

[3]  Mz Hossain,et al.  Electrophoretic Pattern of Hereditary Haemoglobin Disorders in Bangladesh , 2010 .

[4]  D. Weatherall The inherited diseases of hemoglobin are an emerging global health burden. , 2010, Blood.

[5]  S. Islam,et al.  Haemoglobin E/Beta Thalassaemia- A Study in BSMMU , 2010 .

[6]  A. Cnaan,et al.  Survival and Complications in Thalassemia , 2005, Annals of the New York Academy of Sciences.

[7]  S. Sarnaik Thalassemia and related hemoglobinopathies , 2005, Indian journal of pediatrics.

[8]  Rahman Mj,et al.  Prevention & control strategy of thalassemia in Bangladesh , 2003 .

[9]  S. Fucharoen,et al.  Clinical Manifestation of &bgr;-Thalassemia/Hemoglobin E Disease , 2000, Journal of pediatric hematology/oncology.

[10]  R. S. Balgir Genetic epidemiology of the three predominant abnormal hemoglobins in India. , 1996, The Journal of the Association of Physicians of India.

[11]  G. Derchi,et al.  Management of cardiac complications in patients with thalassemia major. , 1995, Seminars in hematology.

[12]  H. Wong,et al.  Hb E beta +-thalassaemia in west Malaysia: clinical features in the most common beta-thalassaemia mutation of the Malays [IVS 1-5 (G-->C)]. , 1993, Singapore medical journal.

[13]  R. Grover,et al.  Current sickle cell screening program for newborns in New York City, 1979-1980. , 1983, American journal of public health.

[14]  M. Angastiniotis,et al.  PREVENTION OF THALASSAEMIA IN CYPRUS , 1981, The Lancet.