Sickle Cell Disease in the Sudan: Clinical and Biochemical Aspects

Mohamed, Abdelrahim Osman

doi:10.3109/03009739209179297

Cited by 2 publications

(2 citation statements)

References 113 publications

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“…In some areas sickle cell trait was present in 24 per cent of the newborn and 29 per cent of those aged over five years. The SCA presentation was severe and it was frequently fatal in early childhood and was accompanied with major complications81–84. Analysis of the haplotypes associated with the S gene indicated that the most abundant haplotypes are the Cameroon, Benin, Bantu and Senegal haplotypes85.…”

Section: Frequency and Distribution Of Sickle Cell Gene Among Arabsmentioning

confidence: 99%

Sickle cell disease in Middle East Arab countries

El‐Hazmi¹,

Al-Hazmi²,

Warsy³

2011

Indian J Med Res

120

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The sickle cell (HbS) gene occurs at a variable frequency in the Middle Eastern Arab countries, with characteristic distribution patterns and representing an overall picture of blood genetic disorders in the region. The origin of the gene has been debated, but studies using β-globin gene haplotypes have ascertained that there were multiple origins for HbS. In some regions the HbS gene is common and exhibits polymorphism, while the reverse is true in others. A common causative factor for the high prevalence and maintenance of HbS and thalassaemia genes is malaria endemicity. The HbS gene also co-exists with other haemoglobin variants and thalassaemia genes and the resulting clinical state is referred to as sickle cell disease (SCD). In the Middle Eastern Arab countries, the clinical picture of SCD expresses two distinct forms, the benign and the severe forms, which are related to two distinct β-globin gene haplotypes. These are referred to as the Saudi-Indian and the Benin haplotypes, respectively. In a majority of the Middle Eastern Arab countries the HbS is linked to the Saudi-Indian haplotype, while in others it is linked to the Benin haplotype. This review outlines the frequency, distribution, clinical feature, management and prevention as well as gene interactions of the HbS genes with other haemoglobin disorders in the Middle Eastern Arab countries.

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Section: Frequency and Distribution Of Sickle Cell Gene Among Arabsmentioning

confidence: 99%

Sickle cell disease in Middle East Arab countries

El‐Hazmi¹,

Al-Hazmi²,

Warsy³

2011

Indian J Med Res

120

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“…ickle cell disease (SCD) is an inherited autosomal recessive disorder of the βglobin gene caused by mutation in position 6 with replacement of glutamate by valine. The disease is characterized by haemolytic anaemia, intermittent episodes of vascular occlusions that can cause both acute and chronic pain, increased susceptibility to infections and end organ damage 1 . There are several factors affecting the clinical severity of the disease which is extremely variable [2][3][4] .…”

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Plasma level of von Willebrand factor: An indicator of severity in sickle cell disease

Omer¹,

Satti²,

Ao³

2009

Sud Jnl Med Sci.

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Background: Sickle cell anaemia is a congenital hemolytic disorder caused by mutation in the βglobin gene at position 6 with replacement of glutamic acid by valine. Patients who are homozygous for this mutation suffer from hemolytic anaemia and other serious complications. The underlying pathology of much of these complications is the occurrence of recurrent vasoocclusion due to microthrombi formation resulting in organs ischaemia. Methods: In this study we investigated the role of vWF as a determinant of sickle cell disease severity through its contribution to the formation of such microthrombi. The clinical disease severity was determined using two different scoring methods, and vWF antigen level in the plasma was estimated by using ELISA technique. Results and discussion: Seventy Sudanese patients were investigated in this study. They were 35 females and 35 males, the mean of their ages± standard deviation was 6.8 ± 4.7 years. Seventeen controls with normal haemoglobin were also included with mean age of 6.5 years. Thirty four patients presented during vasoocclusive crisis and 36 presented in steady state. vWF was high (179.83%) among patients compared to controls (82.4%), p< 0.001. there was positive correlation between severity score and vWF level in the plasma in steady state in the two scoring methods used (r = 0.79, p = 0.008 for method I and r = 0.78, p = 0.009 for method II). So, Severity of sickle cell disease increases with elevation of vWF level in the plasma in the steady state.

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Sickle Cell Disease in the Sudan: Clinical and Biochemical Aspects

Cited by 2 publications

References 113 publications

Sickle cell disease in Middle East Arab countries

Sickle cell disease in Middle East Arab countries

Plasma level of von Willebrand factor: An indicator of severity in sickle cell disease

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