Homozygous sickle cell disease in Central India & Jamaica: A comparison of newborn cohorts.

Homozygous sickle cell disease in Central India & Jamaica: A comparison of newborn cohorts. Indian J Med Res. 2020 Apr;151(4):326-332 Authors: Jain D, Tokalwar R, Upadhye D, Colah R, Serjeant GR Abstract Background & objectives: Homozygous sickle cell (SS) disease in Central India runs a more severe clinical course than reports from other areas of India. The current study was undertaken to compare the disease in Central India (Nagpur) with that in Jamaica, both populations defined by newborn screening. Methods: The Nagpur cohort included infants born to sickling-positive mothers from May 2008 to 2012, examined by high-pressure liquid chromatography and DNA analysis. The Jamaican cohort screened 100,000 consecutive non-operative deliveries between June 1973 and December 1981, analyzed by haemoglobin (Hb) electrophoresis and confirmed by family studies and compatible HbA2levels. Results: In Nagpur, 103 SS patients were detected, but only 78 (76%) were followed up. In Jamaica, 311 cases were followed from birth and compliance with follow up remained 100 per cent up to 45 years. In the Nagpur cohort all had the Asian haplotype, and 82 per cent of Jamaicans had at least one Benin chromosome; none had the Asian haplotype. Compared to Jamaica, Nagpur patients had higher foetal Hb, less alpha-thalassaemia, later development of splenomegaly and less dactylitis. There were also high admission rates for febrile illness a...
Source: The Indian Journal of Medical Research - Category: Biomedical Science Authors: Tags: Indian J Med Res Source Type: research