Fetal hemoglobin during infancy and in sickle cell adults

婴儿期和镰状细胞成人的胎儿血红蛋白

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Abstract

BACKGROUND: Fetal hemoglobin has been implicated in the modulation of sickle cell crisis though it is functional during infancy. OBJECTIVE: The purpose of this study was to determine the waning time of fetal hemoglobin (HbF) and its persistence in later life. METHOD: Ninety infants aged 0-12 months, admitted at hospital, were tested for their HbF levels. Adult patients numbering 690 were also examined for their sickle cell status and a sickle positive patient of SS type with HbF had her family members recruited and their sickle cell types determined. RESULTS: The results revealed that HbF was highest (98%) at birth, decreasing at 5% per week till 6 months when it wane off. Ten infants aged 6-12 months had HbF persisting at a level of 10% or more. Adult patients examined showed proportions of their sickle cell types as AS forming 51%, AC 20%, SS 19%, and SC 10%. An SS adult patient with mild sickle cell crisis had an ASF father who had no crisis and a mother and brother with AS each who had severe crisis. CONCLUSION: These findings suggest that HbF wanes off during infancy but persist in some adults and may modulate crisis in these adults. This has implications in sickle cell management.

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