Abstract
The sickle cell gene in India represents a separate occurrence of the HbS mutation (the Asian haplotype), which has occurred against a genetic background characterised by high levels of fetal haemoglobin and widely varying frequencies of alpha thalassaemia. These features, which tend to inhibit sickling, change the expression of the disease, which, in India, may be further modified by poor nutrition, malaria and other infections, and limited public health resources. Sickle cell disease in Jamaica is predominantly of African origin (the Benin haplotype) and faces some similar challenges. This review assesses similarities and differences between disease expression in the two countries and seeks to explore lessons from Jamaica, which may be relevant to Indian health care. In particular, it addresses common causes of hospital admission as detailed from Indian clinical experience: anemia, bone pain crisis, and infections.
Highlights
The sickle cell gene in India was first reported among tribal people in the Nilgiri Hills of Tamil Nadu in the south of the country [1]
In peoples of African origin, there are three major beta globin haplotypes (Benin, Bantu, and Senegal), and the clinical features of the Benin haplotype, which dominates in North America and the Caribbean, have been well documented, but only limited information is available on the Asian haplotype, which accounts for the disease in India and the Eastern Province of Saudi Arabia
This review has focused on the most common causes of hospital admission in children with Asian HbSS in Nagpur, India, and allowed some comparisons with Jamaican data
Summary
The sickle cell gene in India was first reported among tribal people in the Nilgiri Hills of Tamil Nadu in the south of the country [1]. An early study of 131 patients with Asian HbSS from Burla Medical College in Western Odisha [3] found a disease relatively mild compared to Jamaica with little chronic leg ulceration and priapism, hypersplenism appeared common.
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