DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20170021

An epidemiological study on the clinico-hematological profile of pediatric patients with congenital hemolytic anemia

Santosh Kumar, Deepa Singh, Abhay Garg

Abstract


Background: Among the inherited disorders of blood, haemoglobinopathy and thalassaemia constitute a major bulk of congenital hemolytic anemia in India.

Methods: The present cross sectional study was conducted on pediatric patients aged 0-15 years admitted in pediatric ward of Department of Pediatrics, MGM Medical College and LSK Hospital, Kishanganj, Bihar, India between December 2015 to November, 2016. The data on socio-demographic profile, relevant clinical history and examination and hematological parameters were assessed.

Results: Out of 211 patients evaluated, most common cause of congenital hemolytic anemia was Hb E Beta thalassemia (39.8%), followed by beta thalassemia (27.9%), beta thalassemia trait (14.2%), Hb E disease (11.3 %) and Hb E trait (6.6%). There was male preponderance (male 63%, female 37 %). The mean hemoglobin was found to be lowest in patients of β thalassemia (5.1 gm/gl) and HbE β thalassemia (5.8 gm/dl). The mean total serum bilirubin was found to be highest among β Thalassemia patients (3.0 mg/dl). Hepatomegaly was the most common clinical finding among the study population (57.8%), followed by splenomegaly (54.9%) and hemolytic facies and jaundice (both 53%).

Conclusions: The incidence of HbE beta thalassemia is relatively high in comparison to other varieties of thalassemias and is a major public health problem in this area of the country.


Keywords


Beta thalassemia, Congenital hemolytic anemia, Hb E beta thalassemia

Full Text:

PDF

References


Balgir RS. The burden of haemoglobinopathies in India and the challenges ahead. Current Science. 2000;79(11):1536-47.

Chattopadhyay K, Biswas R, Bhattacherjee S, Bandyopadhyay R. An epidemiological study on the clinico-hematological profile of patients with congenital hemolytic anemia in a tertiary care hospital of Kolkata. Indian J Prev Soc Med. 2012;43(4):1-6.

Angastiniotis M, Modell B, Englezos P, Boulyjenkov V. Prevention and control of hemoglobinopathies. Bull World Health Organ. 1995;73(3):375-86.

Balgir RS. The genetic burden of hemoglobinopathies with special reference to community health in India and the challenges ahead. Indian J Hematology Blood Transfusion. 2002;20(1):2-7.

Varawalla NY, Old JM, Sarkar R, Venkatesan R, Weatherall DJ. The spectrum of beta thalassemia mutations on the Indian subcontinent: the basis for prenatal diagnosis. Brit J Hematol. 1991;78(2):242-7.

Ghai OP. Gupta P, Paul VK. Essential pediatrics. 6th edition. New Delhi: Interprint; 2004. Hematological disorders.100-101.

Nasa LG, Caocci G, Argiolu F. Unrelated donor stem cell transplantation in adult patients with thalassemia. Bone Marrow Transplant. 2005;36(11):971-5.

Chatterjea JB. Some aspects of hemoglobin E and its genetic interference with Thalassemia. Ind J Med Res. 1965;53:377.

Sujatha R, Sreekantha, Niveditha SR, Avinash SS, Remya, Vinodchandran, Rangaswamy R. The study of recent biochemical and pathological aspects of thalassemia. Int J Research Health Sci. 2013;1(3):140-52.

Shivashankara R, Jailkhani R, Kini A. Hemoglobinopathies in Dharwad, North Karnataka: A Hospital-Based Study. Journal Clinical Diagnostic Research. 2008;2:593-9.

Archana AD, Kavita D, Pragna R. Biochemical patterns of hemoglobinopathies and thalassemia syndrome in a tertiary care hospital of Telangana. International J Healthcare Sci. 2014;2(2):385-8.

Weatherall DJ, Clegg JB. Thalassemia - a global health problem. Nat Med. 1996;2:847-9.

Deyde VM, Lo BB, Aw T. Hb hope/HbS and HbS/ß- thal double compound heterozygosity in a Mauritanian family: clinical and biochemical studies. Ann Hematol. 2003;82:423.

Cunningham MJ. Update on thalassemia: Clinical care and complications. Pediatr Clin North Am. 2008;55:447-60.

Bernard SS. Genetic counseling for thalassemia in the islamic republic of Iran. Johns Hopkins University Press. 2009;52(3):364-76.

Erlandson ME, Brilliant R , Smith CH. Comparison of sixty-six patients with thalassemia major and thirteen patients with thalassemia intermedia including evaluations of growth, development and prognosis. Ann Ny Acad Sci. 1964;7:727-35.

Hazell JW, Modell CB. ENT complications in thalassaemia major. J Laryngol Otol. 1976;90(9):877-81.