Awad Omer

Non‐benign sickle cell anaemia in western Saudi Arabia

Seventy‐one Saudi and Yemeni Arabs with sickle cell anaemia from western Saudi Arabia aged between 11/2 and 42 years were studied. The mean steady state haemoglobin concentration of 8.1 g/dl was lower than that of 10.7 g/dl reported previously for sickle cell anaemia in eastern Saudi Arabia. The patients were divided into an SSLF group with fetal haemoglobin (HbF) of 10.0% or below (44 patients) and an SSHF group having HbF above 10.0% (27 patients). No significant differences were found in the haemoglobin concentrations, haematological indices and incidences of bone changes of the two groups. SSLF patients were significantly more prone to infections (P<0.01), however. Also, there was an overall high incidence of hepatomegaly (69.0%) and splenomegaly (54.9%) and hepatomegaly was significantly more common in the SSLF group (P<0.02).

Association of red cell glucose-6-phosphate dehydrogenase with haemoglobinopathies.

A total of 1,112 randomly selected Saudi Arabs, of both sexes, living in Jeddah and the surrounding areas were screened for the phenotypic distribution of red cell glucose-6-phosphate dehydrogenase (G6PD) and 6-phosphogluconate dehydrogenase (6PGD). They were also investigated for haemoglobin and for thalassaemia. Phenotyping of the haemoglobins and the red cell enzymes was carried out by starch gel electrophoresis and the dye-decolouration screening test, while the investigation for thalassaemia was carried out by globin-chain biosynthesis, followed by column chromatography. The red cell Gd- alleles were significantly associated with the sickle-cell gene in both the males (chi 2(1): AS-28.80; SS-4.89) and females (chi 2(1): AS-10.99; SS-13.16). A similar association was also observed between G6PD deficiency and thalassaemias in males (chi 2(1): alpha-thalassaemia - 3.13; beta-thalassaemia - 11.06) and females (chi 2(1): alpha-thalassaemia - 6.63). However, no such association was detected between red cell 6PGD types and haemoglobin genes. The results suggest that the red cell G6PD deficiency, sickle-cell and thalassaemia genes might have evolved as a result of the same ecological factor, probably malaria.

The Inter- and Intra-Tribal Distribution of Red Cell G6PD Phenotypes in Sudan

1,416 males and 564 female subjects from four Negroid and five Arab tribes and a group of mixed tribes of the Sudan were investigated for the phenotypic distribution of red cell glucose-6-phosphate dehydrogenase by starch gel electrophoresis. In general, the tribes of Negroid origin had higher frequency of GdA compared to the tribes of Arab ancestry. However, the Nilotes showed a lower frequency of GdA allele and the Mahass tribe claiming an Arab origin had a higher frequency of GdA. The immigrant groups from the neighbouring African countries also had a higher frequency of GdA. GdB (Khartoum) was present in low frequencies in both the Arab and Negroid tribes. A great deal of intratribal variation in the phenotypic distribution of G6PD was observed in the Nuba and Gáali tribes from different localities.

Red Cell Survival During Ramadan Fasting

DOI: http://dx.doi.org/10.5915/22-1-13520 Fasting the month of Ramadan is a unique metabolic exercise which has not been studied properly. We have carried out the first red cell survival study during Ramadan fasting. The subjects were six normal adults and three adult patients with sickle cell disease. All the subjects fasted the whole month. The ranges of 51CrT50 for normal subjects and patients were 25-40 and 10-12.5 days respectively. These are similar to the published values for non-fasting subjects. We did not note any harmful effects from Ramadan fasting on the red cell survival in our subjects to justify giving advice against fasting.