Hereditary persistence of foetal haemoglobin in a tribal family of Orissa, India.
Article
in English
| IMSEAR
| ID: sea-118630
ABSTRACT
BACKGROUND:
The hereditary persistence of foetal haemoglobin (HPFH) is an autosomal co-dominant, rare, inherited condition. It occurs due to failure of switching off of the production of gamma-chains during the neonatal period leading to a high level of foetal haemoglobin in adult life but without any anaemia. During screening a randomly selected Paraja Bhuyan tribal population for haemoglobinopathies in the Sundargarh district of western Orissa, HPFH was detected in a family.METHODS:
Horizontal haemoglobin electrophoresis was carried out to identify abnormal haemoglobins and quantitation of the haemoglobin A2 fraction was done by the elution method at pH 8.9. Haemoglobin F was estimated. Haematological parameters were studied using an automated blood cell counter. The acid elution-staining test was used to demonstrate the intracellular distribution of haemoglobin F-containing erythrocytes.RESULTS:
Four members of the tribal family had a high level (6.5%-13.7%) of foetal haemoglobin--the mother and 3 children. None of them had any apparent clinical or haematological abnormality except for mild pallor in the two younger children. The add elution-staining test revealed pancellular distribution of foetal haemoglobin in the erythrocytes of all the affected family members.CONCLUSION:
Genetic traits such as hereditary persistence of foetal haemoglobin, although rare, are prevalent in India.
Full text:
Available
Index:
IMSEAR (South-East Asia)
Main subject:
Thalassemia
/
Female
/
Humans
/
Male
/
Fetal Hemoglobin
/
Child
/
Chronic Disease
/
Risk Factors
/
Population Groups
/
Erythrocytes
Type of study:
Etiology study
/
Prognostic study
/
Risk factors
Country/Region as subject:
Asia
Language:
English
Year:
2004
Type:
Article
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