Regional Variations in Sickle Cell Anemia in Saudi Arabia

Roberts, George T.; El-Badawi, Saad B.; Padmos, M. Andrew; Sackey, Kwesi

doi:10.5144/0256-4947.1988.320

Cited by 12 publications

(3 citation statements)

References 24 publications

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“…However, more recent and detailed studies have revealed that two forms of SCD exist in Saudi Arabs, one with a mild course and another with a severe course, similar to that encountered in other populations [14,15]. Significant clinical diversity has been demon strated in disease severity in the different regions of Saudi Arabia and other Middle-Eastern countries [3,[19][20][21], Sev eral genetic and/or environmental factors have been pro posed to account for the heterogeneity of disease presen tation. Interactions between Hb S and other abnormal genes have been considered as possible modulators of the clinical manifestations of SCD [7,9,11], In an attempt to investigate and compare the clinical and haematological presentation of SCD in the different regions of Saudi Arabia and the influence of coexisting aand (l°-thalassaemia, this study was conducted on individ uals identified as Hb S homozygotes during screening of three areas of Saudi Arabia.…”

Section: Introductionmentioning

confidence: 99%

Heterogeneity and Variation of Clinical and Haematological Expression of Haemoglobin S in Saudi Arabs

El‐Hazmi¹

1992

Acta Haematol

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Sickle cell haemoglobin (Hb S) occurs at a high frequency in the Eastern (EP), South-Western (SWP) and North-Western (NWP) Provinces of Saudi Arabia and the presentation of the Hb S is believed to exhibit clinical diversity in the different regions. Three areas of Saudi Arabia were screened to determine the frequency of Hb S and α- and β-thalassaemias and glucose-6-phosphate de-hydrogenase deficiency genes. Furthermore, in an attempt to investigate and compare the clinical and haematological presentation of sickle cell disease (SCD) in the different regions of Saudi Arabia, the individuals identified as Hb S homozygotes were investigated further. The patients were further classified on the basis of whether there was associated α- or β-thalassaemia. A severity index (SI) was calculated for each patient and the clinical presentations and laboratory findings were compared. The results showed significantly variable severity of SCD in patients from different regions. The patients from the EP generally had a mild clinical presentation, while in the SWP and NWP majority of the patients suffered from a severe disease as judged by the SI. No correlation could be established between Hb F level and SI, though the WBC level correlated positively with the SI. The lowest SI values were encountered in patients with associated α-thalassaemia who also had the lowest WBC count and MCV and the highest RBC count and packed cell volume.

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Section: Introductionmentioning

confidence: 99%

Heterogeneity and Variation of Clinical and Haematological Expression of Haemoglobin S in Saudi Arabs

El‐Hazmi¹

1992

Acta Haematol

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“…Previously it had been believed that the high level of foetal haemoglobin in patients from the Eastern region was responsible for the mild clinical symptoms [13,17,21]. Roberts et al reported that the high level of foetal haemoglobin is not only confined to patients from the Eastern region [21].…”

Section: Discussionmentioning

confidence: 99%

Orthopaedic complications in sickle cell disease

Sadat-Ali

Geeranavar

Assuhaimi

1992

International Orthopaedics

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The haematological and orthopaedic complications of patients with sickle cell disease from two different regions of Arabia are presented. Nineteen patients from the South-western region were matched for age and sex with an equal number from the Eastern region. The mean sickle cell haemoglobin was 75.95% in those from the South-west and 77.5% in those from the East. The haemoglobin concentration was marginally lower in the Eastern region patients at 9.19 g/% compared with 9.51 g/%. Bone and joint infections occurred in 17% of Eastern region patients and in 15% in those from the South-western region. There was no significant difference between the haematological parameters and the orthopaedic complications in the two regions. We conclude that these complications are severe in the Eastern region and the disease is not benign as previously thought. Aggressive treatment of orthopaedic complications is indicated in sickle cell disease in the Eastern region.

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“…Three studies, two from Riyadh 30,31 and one from the Eastern Province, 22 have compared the characteristics of patients from the Southwest with those from the Eastern Province. All conclude that patients originating in the Southwest had lower HbF and hemoglobin levels and a more symptomatic clinical course.…”

Section: The Clinical Picture In Southwestern Saudi Arabiamentioning

confidence: 99%

The Challenge of Sickle Cell Disease in Saudi Arabia

Serjeant

1990

Ann Saudi Med

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Regional Variations in Sickle Cell Anemia in Saudi Arabia

Cited by 12 publications

References 24 publications

Heterogeneity and Variation of Clinical and Haematological Expression of Haemoglobin S in Saudi Arabs

Heterogeneity and Variation of Clinical and Haematological Expression of Haemoglobin S in Saudi Arabs

Orthopaedic complications in sickle cell disease

The Challenge of Sickle Cell Disease in Saudi Arabia

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