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Sickle cell disease in Orissa State, India.
Lancet (London, England)
|November 22, 1986
Summary
Indian patients with sickle cell disease (SCD) show distinct characteristics compared to other populations. This study highlights unique hematological and clinical features in Indian SCD patients, differing significantly from West African origins.
Area of Science:
- Hematology
- Genetics
- Clinical Medicine
Background:
- Sickle cell disease (SCD) is a genetic blood disorder with varying clinical manifestations globally.
- Understanding regional differences in SCD is crucial for targeted healthcare and research.
Purpose of the Study:
- To characterize the clinical and hematological profile of homozygous sickle cell (SS) disease patients in Orissa, India.
- To compare these features with those of patients from Jamaica and populations of West African origin.
Main Methods:
- Retrospective analysis of 131 patients with homozygous sickle cell disease in Orissa, India.
- Comparative analysis of hematological parameters, clinical outcomes, and disease incidence.
Main Results:
- Indian patients exhibit higher alpha thalassaemia frequency, elevated fetal hemoglobin, total hemoglobin, and red cell counts.
- Lower mean cell volume, mean cell hemoglobin concentration, and reticulocyte counts were observed in Indian patients.
- Increased frequency and later peak incidence of splenomegaly, common hypersplenism, frequent painful crises and dactylitis, but rare leg ulceration were noted.
Conclusions:
- Homozygous sickle cell disease in Orissa presents a unique phenotype, distinct from West African-origin populations.
- The disease profile in Indian patients closely resembles that observed in Saudi Arabia's Eastern Province.