Haemoglobinopathies and  β -Thalassaemia  among the Tribals Working in the Tea  Gardens of Assam, India

Teli, Anju Barhai; Deori, Rumi; Saikia, Sidhartha Protim

doi:10.7860/jcdr/2016/22010.9002

Cited by 9 publications

(6 citation statements)

References 12 publications

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“…4,15 The prevalence and carrier rates of β-thalassemia are relatively high in Southeast Asia (Table 1). [16][17][18][19][20][21][22][23][24][25][26] For example, the reported prevalence ranges from 1.25% to 1.66% in India 21,22 but is approximately 2.21% in China. 18,19 Carrier rates range from 0.5% in Myanmar 16 to 12.8% in Malaysia.…”

Section: Pre Valen Ce Of β -Thal a Ss Emiamentioning

confidence: 99%

Changing patterns in the epidemiology of β‐thalassemia

Kattamis

Forni

Aydınok

et al. 2020

European J of Haematology

196

114

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β-thalassemias are a heterogeneous group of hereditary hemoglobinopathies characterized by defects in the β-globin chain of hemoglobin and autosomal recessive inheritance. Homozygous or compound heterozygous forms have an imbalance in the production of α-and non-α-globin chains, resulting in ineffective erythropoiesis and decreased production of normal hemoglobin A. 1 Patients with β-thalassemia major have severe chronic hemolytic anemia and require regular blood transfusions from early childhood. 1-3 Chronic blood transfusion therapy is typically combined with iron chelation therapy (ICT) to prevent complications due to iron overload, such as cardiac morbidity, liver disease, and endocrine dysfunction. 1-3 Patients with β-thalassemia intermedia have symptoms in between carriers and those with β-thalassemia major: Anemia is often moderate, but patients may still have morbidity due to ineffective erythropoiesis and hemolysis, including ulcers, pulmonary hypertension, and pain. Some patients require occasional blood transfusions, although less frequently than patients with β-thalassemia major. 1,2 Historically, the prevalence of β-thalassemia has been highest in the Mediterranean region, the Middle East, and Southeast Asia and lowest in Northern Europe and North America. 4 Due to migration patterns, β-thalassemia is increasingly more common in non-endemic regions,

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Section: Pre Valen Ce Of β -Thal a Ss Emiamentioning

confidence: 99%

Changing patterns in the epidemiology of β‐thalassemia

Kattamis

Forni

Aydınok

et al. 2020

European J of Haematology

196

114

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“…Due to presence of tribes in Datia region it was expected that sickle cell disease could be prevalent, but results were contradictory 11,12,13 . The general prevalence of sickle cell anaemia and β-thalassemia trait lies between 1-44% and 3-17% respectively 13 . In this study a prevalence of β-thalassemia minor was found to be and sickle cell trait 0.66% respectively in Datia District.…”

Section: Discussionmentioning

confidence: 99%

Screening for Various Haemoglobinopathies to Observe Prevalence and Patterns in Population of District Datia (M.P.), India

Mishra

Rajasubramaniam

Balakrishna

et al. 2022

Preprint

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Purpose: Haemoglobinopathies are the most common inherited disorder of Red Blood Cells across globe and one of the major public health problems in many regions of India. Variation in ethnic and regional prevalence is observed. The prevalence and pattern of haemoglobinopathies in Datia region has not been reported limiting the knowledge of diseases profile in this region. Present work showed the broad view of various haemoglobinopathies in the local area and population at risk. Efforts were made to identify prevalence of Sickle cell anaemia, β-thalassemia major and G-6-P-D deficiency among patients attending various OPDs of District Hospital, Datia, Madhya Pradesh, India. Methods: All anaemia patients referred by Govt. hospitals/facilities (Datia District hospital) exhibiting anaemia were screened for various haemoglobinopathies. A total of 605 cases exhibiting anemia received from September 2017 to March 2020 at the Model Rural Health Research Unit (MRHRU), Datia, M.P. were tested for different haemoglobinopathies. Samples were received from District Hospital Datia. The samples were tested by solubility and cellulose acetate electrophoresis and HPLC and G6PD deficiency.Results: A total 605 patients were screened for haemoglobinopathies from District Hospital, Datia. It was found that 13 cases (2.14%) were of β thalassemia trait and other rare variants followed by 4 cases (0.66%)of sickle cell trait. A total of 3 cases (0.5%) of β thalassemia major was observed in total samples of 605.Conclusion: Very low prevalence rate of Sickle cell anaemia and thalassemia was observed. Large scale screening in the region is required for identifying the true burden.

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“…Tribals of Chhattisgarh, West Bengal, Odisha, and Jharkhand are Austro-Asiatic speakers of Proto-Australoid race, whereas the tribals of Northeast India are Tibeto-Burman speaking group of Mongoloid origin. Reports on existence of Hb S and Hb E separately among the tea tribe of Assam are well known [ 8 ]. However, compound heterozygosity for Hb S and Hb E was not reported earlier from any community from the multiethnic and multiracial population of Northeast India.…”

Section: Discussionmentioning

confidence: 99%

Compound heterozygosity for hemoglobin S and hemoglobin E in a family of Proto-Australoid origin: a case report

Basumatary

Baruah²,

Sarma³

et al. 2021

J Med Case Reports

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Background Hemoglobin S and E are commonly occurring hemoglobin variants among distinctly separate tribal populations of Central and Northeast India, respectively. Combined heterozygosity for hemoglobin S and E or hemoglobin SE disease is a benign clinical condition with rare incidence. Reports of approximately 46 hemoglobin SE cases are available worldwide. We conducted a screening program to study the prevalence of hemoglobin variants among the tribal population working in the tea estates of Northeast India. A total of 551 subjects were screened, and complete blood count was performed. Based on their hematological profiles, hemoglobin typing was done for 218 subjects. Case presentation We describe a case of an adolescent male of Munda tribe diagnosed as double heterozygous for hemoglobin S and E. On screening of the nuclear family of the subject, the mother was found to have hemoglobin E disease and father as hemoglobin S trait. Both siblings of the subject were diagnosed as hemoglobin E trait. Conclusion This is the first case of compound heterozygous for hemoglobin S and E to be reported from the tea tribes of Assam, India.

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Haemoglobinopathies and β -Thalassaemia among the Tribals Working in the Tea Gardens of Assam, India

Cited by 9 publications

References 12 publications

Changing patterns in the epidemiology of β‐thalassemia

Changing patterns in the epidemiology of β‐thalassemia

Screening for Various Haemoglobinopathies to Observe Prevalence and Patterns in Population of District Datia (M.P.), India

Compound heterozygosity for hemoglobin S and hemoglobin E in a family of Proto-Australoid origin: a case report

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