Document Detail


Hematological profile of twenty-nine tribal compound cases of hemoglobinopathies and G-6-PD deficiency in rural Orissa.
MedLine Citation:
PMID:  18923215     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Hematogenetic disorders are commonly encountered in Orissa state in Central-Eastern India. Hemoglobinopathies and G-6-PD deficiency are the most frequently occurring hereditary hemolytic disorders causing high morbidity and mortality in vulnerable people. AIMS: There is no study available reporting combined condition of hemoglobinopathies and G-6-PD deficiency in a single individual from India. This study aims to assess the coincidence of G-6-PD enzyme deficiency with different hemoglobinopathies and beta-thalassemia and to evaluate the influence of combined conditions on the hematological expression. SETTINGS AND DESIGN: The study was carried out in rural Orissa with a random sampling procedure. MATERIALS AND METHODS: Following the standard methodology and techniques, this study highlights 29 tribal cases of compound occurrence of hemoglobinopathy with G-6-PD deficiency in a randomly conducted study in Sundargarh district of Orissa. STATISTICAL ANALYSIS: Results were subjected to statistical analysis. RESULTS: Both female heterozygotes and homozygotes of G-6-PD deficiency in association with different hemoglobinopathies showed reduced values of hematological indices: hemoglobin level, MCV, MCH, MCHC and RBC in comparison to normals. Red cell indices were found further reduced in male G-6-PD deficiency concurrence with hemoglobinopathies in homozygous condition, i.e. sickle cell disease (HbSS) or hemoglobin E disease (HbEE). Hematological indices were significantly lower except WBC counts and fetal hemoglobin in male G-6-PD deficiency with co-existing homozygous sickle cell disease in comparison with counterpart sickle cell trait and normal controls. CONCLUSIONS: Hemoglobin polymorphism with G-6-PD deficiency is advantageous to the community against lethal effects of malaria especially against Plasmodium falciparum at population level, but their combination is harmful at the individual level because of low levels of red cell indices to cope with the routine human physiology.
Authors:
R S Balgir
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Indian journal of medical sciences     Volume:  62     ISSN:  0019-5359     ISO Abbreviation:  Indian J Med Sci     Publication Date:  2008 Sep 
Date Detail:
Created Date:  2008-10-16     Completed Date:  2009-06-04     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0373023     Medline TA:  Indian J Med Sci     Country:  India    
Other Details:
Languages:  eng     Pagination:  362-71     Citation Subset:  IM    
Affiliation:
Division of Human Genetics, Regional Medical Research Centre ((ICMR), Chandrasekharpur, Bhubaneswar, Orissa, India. balgirrs@yahoo.co.in
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Child
Child, Preschool
Erythrocyte Indices
Female
Glycogen Storage Disease Type I / blood,  complications*,  ethnology
Hemoglobinopathies / blood,  complications*,  ethnology
Hemoglobins / analysis
Humans
India / epidemiology
Male
Middle Aged
Young Adult
Chemical
Reg. No./Substance:
0/Hemoglobins

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