Post natal decline of fetal hemo globin in homo zygous sickle cell disease relationship to parental hemo globin f levels
Mason, K.P.; Grandison, Y.; Hayes, R.J.; Serjeant, B.E.; Serjeant, G.R.; Vaidya, S.; Wood, W.G.
British Journal of Haematology 52(3): 455-464
ISSN/ISBN: 0007-1048 Accession: 006152190
The decline of fetal Hb (Hb F) from birth to 6 yr was compared in a cohort of 266 Jamaican children with homozygous sickle cell (SS) disease and in 243 matched controls with a normal Hb (AA) genotype. Hb F levels were significantly higher in the SS cases from 1 mo. onward but, unlike the normal controls, no sex difference was apparent. The Hb F levels in SS disease were significantly correlated with parental Hb F levels, suggesting that genetic factors regulating adult Hb F levels are active at earlier stages in development. Some of these genetic determinants of Hb F production may be linked to the .beta.-like globin gene complex and may be in linkage disequilibrium with the .beta.s allele.