Neonatal Screening for genetic blood diseases

Previous neonatal screening in 1986 showed that the incidence of sickle cell disease [SCD] is 2.1% and [SCT] is l1%. Since 1984 the Ministry of Health [MOH] instituted a prevention campaign. The incidence has been falling gradually since then. To update the national data on the incidence of SCD among the newborns and to compare it with the previously available data. All Bahraini newborns delivered at the [MOH] maternity hospitals for a period of three months from February to April 2002 were targeted. Cord blood samples were analyzed by HPLC. Two thousand newborns constituted the study population five were excluded. Eighteen were found to be affected with SCD with an incidence of 0.9%. SCT was found in 325 [16.3%]. G6PD deficiency was found in 18% of males, and 10% of females. Parental age distribution and consanguinity were documented. Bahrain has for the first time recorded less than 1% babies with SCD

Glucose-6-Phosphate dehydrogenase deficiency in Bahraini blood donors

Glucose -6- phosphate dehydrogenase deficiency, a genetic defect, underlying the biochemical red cell abnormality is the most common red cell enzyme disorder and occurs world wide. Blood from donors with G6PD-deficiency survives normally unless the recipient is given certain oxidant drugs which may lead to a rapid destruction of the G6PD-deficient cells. 2680 male Bahraini blood donors attending the Central Blood Bank, Salmaniya Medical Centre were screened for G6PD-deficiency. The result of the study showed that 26.4% blood donors were G6PD- deficient. It is proposed to estimate quantitative enzyme levels to identify G6PD variants in the population to assess the extent of its severity, and a clinical trial to assess the effect of blood transfusion on patients receiving G6PD-deficient donor cells