Glycosylated haemoglobin level in saudi children with sickle cell anaemia

الملخص EN

Objective: To evaluate the glucose-6-phosphate dehydrogenase (G6PD) status in ameliorating the disease in sickle cell paediatric patient.

Design: Prospective study of patient with sickle cell anaemia and without G6PD deficiency.

Setting: Paediatric Department, Qatif Central Hospital, Saudi Arabia.

Subject: Sickle cell paediatric patient.

Results: The mean HbA,C in patient with SS disease was 6.5 0.9 %, while that in ٌ normal paediatric patient was 6.3 1.5 % (P value > 0.6).

On the other hand the ٌ HbA,C in patient with SS disease and G6PD deficiency was 6.9 1.08 % which is ٌ statistically different from patient with SS disease and normal G6PD status (5.6 1.08 %) (P value < 0.04).

ٌ Conclusion: G6PD deficiency may be beneficial in reducing the haemolytic crises in Saudi paediatric SS patient.

Bahrain Med Bull 1996;18(1): Sickle cell disease is a chronic haemolytic disorder, manifested by a wide variety of clinical haematological features.

The prevalence of sickle cell anaemia in Saudi Arabia is well recognised1,2.

The incidence of the disease in the Eastern part of Saudi Arabia is particularly high3 .

The natural history of the disease has been reported to show mild clinical manifestation with moderate haemolytic anaemia4,5.

The glycosylated haemoglobin (HbA,C) level has been used as an index for the survival time of the red cells in sickle cell anaemia and other related Hb disorders6,7.

We have done this study to evaluate the influence of G6PD deficiency on reducing the haemolytic crises in patient with sickle cell anaemia using the HbA,C as an indicator for the rate of haemolysis.