Sickle cell disease: reappraisal of the role of foetal haemoglobin levels in the frequency of vaso-occlusive crisis

Antwi‐Boasiako, Charles; Frimpong, Emmanuel; Ababio, G. K.; Dzudzor, Bartholomew; Ekem, Ivy; Gyan, Ben; Sodzi-Tettey, NA; Antwi, DA

doi:10.4314/gmj.v49i2.7

Cited by 15 publications

(18 citation statements)

References 34 publications

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“…However, this finding agrees with a previously published 11.3% SCT reported among blood donors in Ghana [ 37 ]. This study thus supports the estimated annual prevalence of SCD in Ghana with its associated high rates of morbidity and mortality [ 38 ].…”

Section: Discussionsupporting

confidence: 87%

Significantly elevated foetal haemoglobin levels in individuals with glucose 6-phosphate dehydrogenase disease and/or sickle cell trait: a cross-sectional study in Cape Coast, Ghana

et al. 2017

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BackgroundPreviously published data have demonstrated that sickle red blood cells produce twice as much reactive oxygen species (ROS) suggesting that co-inheritance of sickle cell disease (SCD) and glucose 6-phosphate dehydrogenase (G6PD) enzymopathy could lead to more severe anaemia during sickling crises. Elevated foetal haemoglobin (Hb F) levels have been shown to have positive modulatory effects on sickling crises and disease outcomes. This study sought to assess how inheritance of G6PD enzymopathy affects the level of Hb F and haemoglobin concentration in adults in steady state.MethodsThis cross-sectional study selected 100 out-patients (41 males and 59 females) visiting the University of Cape Coast hospital, between January, 2016 and May, 2016. Cellulose acetate electrophoresis (pH 8.2–8.6), methaemoglobin reductase test, modified Betke alkaline denaturation methods were used to investigate haemoglobin variants, qualitative G6PD status, and %Hb F levels in venous blood samples drawn from these participants. Data was analysed with GraphPad Prism 6 and SPSS and significance set at p < 0.05.ResultsForty one percent of the participants demonstrated qualitative G6PD enzymopathy whereas only 10% demonstrated Hb AS type (Sickle cell trait, SCT). 5% of the participants co-inherited SCT and G6PD enzymopathy. %Hb F levels in G6PD deficient males was significantly higher than in G6PD deficient females [(p = 0.0003, 2.696% (males) vs 1.975% (females)], although the %Hb F levels was comparable in non-G6PD deficient individuals. %Hb F levels were significantly elevated in males with SCT only (p < 0.05), or G6PD enzymopathy only (p < 0.0001), or SCT + G6PD enzymopathy (p < 0.0001) compared to males with none of these pathologies even though their respective haemoglobin levels were comparable. Male participants with G6PD enzymopathy + SCT co-inheritance had significantly elevated %Hb F when compared to their counterparts with only G6PD enzymopathy (p < 0.001). Male gender [(p = 0.001, OR: 6.912 (2.277–20.984)] partial defective G6PD enzyme [(p = 0.00, OR: 7.567E8 (8.443E7–6.782E9)] SCT [(p = 0.026, OR: 4.625 (1.196–17.881)] were factors associated with raised %Hb F levels ≥2.5.ConclusionThe inheritance of G6PD defect and/or SCT significantly elevate %Hb F levels in the steady state even though haemoglobin levels are not affected.

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Section: Discussionsupporting

confidence: 87%

Significantly elevated foetal haemoglobin levels in individuals with glucose 6-phosphate dehydrogenase disease and/or sickle cell trait: a cross-sectional study in Cape Coast, Ghana

et al. 2017

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“…The population of Ghana is about 25 million [ 14 ], and the prevalence of Sickle Cell Disease is 1.9% of all births per year [ 15 ]. The out-patient morbidity reports compiled by the Ghana Health Service in 2002 and 2003 ranked SCD at the 37th and 36th positions, respectively [ 16 ]. KBTH is the largest hospital in Ghana with a bed capacity of about 2000 and 17 clinical and diagnostic departments including a Sickle Cell Clinic [ 17 ].…”

Section: Methodsmentioning

confidence: 99%

Levels of Soluble Endothelium Adhesion Molecules and Complications among Sickle Cell Disease Patients in Ghana

et al. 2018

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Background: Soluble adhesion molecules are involved in the gathering and joining of inflammatory cells to vascular endothelium. Therefore, they serve as potential markers of endothelial dysfunction in vascular diseases including sickle cell disease (SCD). In Ghana, there are scarcely any report on the levels of adhesion molecules among SCD patients. The current study aimed to determine plasma levels of ICAM-1, VCAM-1 and E-Selectin as markers of endothelial dysfunction in SCD patients in steady state, complications and controls. Methodology: This was a cross-sectional study involving 60 HbAA controls, 46 HbSS steady state, 57 HbSS VOC, 18 HbSC VOC, 21 HbSS with leg ulcer and 11 HbSS with priapism. Blood samples were collected from all the study subjects (n = 213) and processed into plasma. The plasma levels of VCAM-1, ICAM-1 and E-Selectin concentrations of SCD patients and controls were measured using a double sandwich ELISA technique. Demographic information was also collected from the study subjects. Results: Levels of all soluble proteins (ICAM-1, VCAM-1 and E-Selectin) were significantly higher in HbSS steady-state patients compared to non-SCD controls (p < 0.001). Generally, SCD patients with complications had relatively higher levels of the soluble proteins compared to those in the steady-state. Of the SCD patients with complications, those with vaso-occlusion crisis (HbSS VOC) had relatively higher levels of ICAM-1, VCAM-1 and E-Selectin at (62.42 ng/mL ± 26.09), (634.99 ng/mL ± 324.31) and (236.77 ng/mL ± 114.40) respectively; Conclusion: Although levels of adhesion molecules were high in all the SCD patients with complications, those with vaso-occlusive crisis had higher levels. This might reflect an ongoing endothelial dysfunction in these patients. SCD patients with vaso-occlusive crisis presents with a more severe pathophysiology condition.

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“…The population of Ghana is about 25 million [14], and the prevalence of sickle cell disease is 1.9% of all births per year [15]. The out-patient morbidity reports compiled by the Ghana Health Service in 2002 and 2003 ranked SCD at positions 37th and 36th, respectively [16]. There are over 100 hospitals in Ghana and a national health insurance scheme has been in operation since 2004.…”

Section: Methodsmentioning

confidence: 99%

Risk of Asymptomatic Bacteriuria among People with Sickle Cell Disease in Accra, Ghana

et al. 2017

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Asymptomatic bacteriuria (ASB) is benign except in certain medical conditions such as pregnancy and immunosuppression. In Ghana, there are hardly any studies on urinary infections among sickle cell disease (SCD) patients, and the few studies carried out in Africa focused on pediatric SCD populations. The current study aimed to investigate the risk of ASB among SCD patients at a tertiary hospital in Ghana. This was a cross-sectional study involving 110 SCD patients and 110 age and sex matched healthy controls. Urine specimens were collected from all the study subjects and analyzed by standard microbiological methods. Demographic information were also collected from the study subjects. The overall ASB prevalence was significantly higher among SCD patients (17.2%) than among the control group (8.2%), and the relative risk was 2.11 (p = 0.0431; CI = 1.00-4.45). Being female was as a predictor of ASB among the SCD patients (OR = 14.76; CI = 11.23-18.29; p = 0.0103). The most common organism isolated from the study participants was coagulase negative Staphylococcus species (4.1%), followed by Escherichia coli (2.7%); etiology of ASB in the SCD patients was more diverse compared to healthy people. All the E. coli isolates were susceptible to amikacin, sparfloxacin and norfloxacin but resistant to ampicillin.

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Sickle cell disease: reappraisal of the role of foetal haemoglobin levels in the frequency of vaso-occlusive crisis

Cited by 15 publications

References 34 publications

Significantly elevated foetal haemoglobin levels in individuals with glucose 6-phosphate dehydrogenase disease and/or sickle cell trait: a cross-sectional study in Cape Coast, Ghana

Significantly elevated foetal haemoglobin levels in individuals with glucose 6-phosphate dehydrogenase disease and/or sickle cell trait: a cross-sectional study in Cape Coast, Ghana

Levels of Soluble Endothelium Adhesion Molecules and Complications among Sickle Cell Disease Patients in Ghana

Risk of Asymptomatic Bacteriuria among People with Sickle Cell Disease in Accra, Ghana

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