Clinical phenotypes and the biological parameters of Congolese patients suffering from sickle cell anemia: A first report from Central Africa

Mikobi, Tite Minga; Lukusa-Tshilobo, Prosper; Aloni, Michel Ntetani; Akilimali, Pierre; Mvumbi-Lelo, Georges; Mbuyi-Muamba, J. M.

doi:10.1002/jcla.22140

Cited by 15 publications

(14 citation statements)

References 29 publications

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“…The diagnosis of alpha-thalassemia has been made by the Multiplex Ligation-dependent Probe Amplification (MLPA) technique. The procedures, as well as the reagents used for both techniques (RFLP and MLPA), have been carefully described by Mikobi et al 15,16…”

Section: Methodsmentioning

confidence: 99%

Homozygous Deletion Alfa-Thalassemia and Hereditary Persistence of Fetal Hemoglobin, Two Genetic Factors Predictive the Reduction of Morbidity and Mortality During Pregnancy in Sickle Cell Patients . A Report From Democratic Republic of Congo

Mikobi

Lukusa

Muamba

et al. 2019

Mediterr J Hematol Infect Dis

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FHb and alpha-thal are two genetic factors that modulate the clinical expression of sickle cell disease. Objective: to determine the beneficial role of FHb and alpha-thal on fetal and maternal morbidity during pregnancy in sickle cell patients. This is a documentary and analytical study that included 960 deliveries of homozygous sickle cell patients. The deliveries were divided into three genotype subgroups: Hb-SS / alpha-thal, HbSS / HPFH and HbSS. The diagnosis of SCD and the quantification of FHb were performed by the capillary electrophoresis technique. The diagnosis of SCD has been confirmed by the molecular test. The diagnosis of alpha-thal was made by the multiplex ligation dependent probe amplification (MLPA) technique. Sickle cell pregnancies were followed according to the protocol of care in force in our service. The variables of interest were: hematological variables, sickle cell crises during pregnancy, maternal and fetal complications. Statistical analyzes were performed with SPSS 20.0 software. Means and standard deviations were compared with the Student's t and Annova tests. The value of p <0.05 was considered the significance level. The Hb-SS / alpha-thal and HbSS / HPFH genotypes were observed in 101 and 121 women respectively. Otherwise 758 women had the HbSS genotype. The morbidity related to sickle cell complications in the mother and fetus, were less frequent in the Hb-SS / alpha-thal and HbSS / HPFH groups. The statistical differences were statistically significant. This study showed a significant protective effect of alpa-thal and HPFH during pregnancy in sickle-cell pregnant women. FHb and alpha-thal are two genetic factors that modulate the clinical expression of sickle cell disease. Objective: to determine the beneficial role of FHb and alpha-thal on fetal and maternal morbidity during pregnancy in sickle cell patients. This is a documentary and analytical study that included 960 deliveries of homozygous sickle cell patients. The deliveries were divided into three genotype subgroups: Hb-SS / alpha-thal, HbSS / HPFH and HbSS. The diagnosis of SCD and the quantification of FHb were performed by the capillary electrophoresis technique. The diagnosis of SCD has been confirmed by the molecular test. The diagnosis of alpha-thal was made by the multiplex ligation dependent probe amplification (MLPA) technique. Sickle cell pregnancies were followed according to the protocol of care in force in our service. The variables of interest were: hematological variables, sickle cell crises during pregnancy, maternal and fetal complications. Statistical analyzes were performed with SPSS 20.0 software. Means and standard deviations were compared with the Student's t and Annova tests. The value of p <0.05 was considered the significance level. The Hb-SS / alpha-thal and HbSS / HPFH genotypes were observed in 101 and 121 women respectively. Otherwise 758 women had the HbSS genotype. The morbidity related to sickle cell complications in the mother and fetus, were less frequent in the Hb-SS / alpha-thal and HbSS / HPFH groups. The statistical differences were statistically significant. This study showed a significant protective effect of alpa-thal and HPFH during pregnancy in sickle-cell pregnant women.

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Section: Methodsmentioning

confidence: 99%

Homozygous Deletion Alfa-Thalassemia and Hereditary Persistence of Fetal Hemoglobin, Two Genetic Factors Predictive the Reduction of Morbidity and Mortality During Pregnancy in Sickle Cell Patients . A Report From Democratic Republic of Congo

Mikobi

Lukusa

Muamba

et al. 2019

Mediterr J Hematol Infect Dis

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“…The majority (25/37; 68%) of studies were conducted in Nigeria. Other studies involved populations in Democratic Republic of Congo (DRC) [22][23][24], Ghana [25,26], Tanzania [27], Egypt [28], Cameroon [29], and Algeria [30]. In addition, two multi-country studies involved patients in Cameroon, Ivory Coast, Gabon, Mali, and Senegal [31,32].…”

Section: Resultsmentioning

confidence: 99%

Nutritional perspectives on sickle cell disease in Africa: A systematic review

Nartey

Spector

Adu‐Afarwuah

et al. 2021

Preprint

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Background: Sickle cell disease (SCD), an inherited blood disorder, predominantly affects individuals in sub-Saharan Africa. Research linking its pathophysiology and nutritional status in African patients has not been previously described. This systematic review aimed to assess the landscape of studies in sub-Saharan Africa focused on nutritional aspects of SCD and highlight gaps in knowledge that could inform priority-setting for future research.Methods: The study was conducted using the Preferred Reporting items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. All original studies involving individuals with any phenotypic variant of SCD and at least one nutritional status outcome; and were conducted between January 1995 and November 2020 in Africa were included. ‘Nutritional status’ was defined by studies on dietary intake, measurements of growth/anthropometry, or nutritional biomarkers. Studies were included if they involved Databases used were Ovid Embase, Medline, Biosis and Web of Science. Results: The search returned 526 articles with 76 studies included in the final analyses. Most investigations (67%) were conducted in Nigeria. Studies were categorized into one of three main categories: descriptive studies of anthropometric characteristics (49%), descriptive studies of macro- or micronutrient intakes (41%), and interventional studies (11%). Findings consistently found growth impairment, especially among children and adolescents from sub-Saharan Africa. Studies assessing macro- and micronutrients generally had small sample sizes and were exploratory in nature. Only four randomized trials were identified, which measured the impact of lime juice, long-chain fatty acids supplementation, ready-to-use supplementary food, and oral arginine on health outcomes. Conclusions: The findings reveal a moderate number of descriptive studies, most with small sample sizes, that focused on various aspects of nutrition and SCD in African patients. There was a stark lack of interventional studies that could be used to inform evidence-based changes in clinical practice. Findings of investigations were generally consistent with data from other regional settings, describing a significant risk of growth faltering and malnutrition among individuals with SCD. There is an unmet need for clinical research to better understand the potential benefits of nutritional-related interventions for patients with SCD in sub-Saharan Africa in order to promote optimal growth and improve health outcomes.

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“…The majority (23/31; 74%) of studies were conducted in Nigeria, including fourteen investigations from the major metropolitan area of Lagos. Other studies involved populations in Democratic Republic of Congo (DRC), [22,23] Ghana, [24] Tanzania, [25] Egypt, [26] and Algeria. [27] In addition, a multi-country study involved patients in Cameroon, Ivory Coast, Gabon, Mali, and Senegal.…”

Section: Resultsmentioning

confidence: 99%

Nutritional Perspectives on Sickle Cell Disease in Africa: A Systematic Review

Nartey

Spector

Adu‐Afarwuah

et al. 2020

Preprint

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Background: Sickle cell disease (SCD), an inherited blood disorder, predominantly affects individuals in sub-Saharan Africa. While there are established studies that links its pathophysiology and nutritional status, the extent of research in this area involving African patients has not been previously described. This systematic review aimed to assess the nutrition-focused landscape of SCD studies in Africa, summarize findings, and highlight gaps in knowledge that could inform priority-setting for future research.Methods: The study was conducted using the Preferred Reporting items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. We included all original studies, conducted between 1995 and 2018, on nutritional status of individuals with SCD in Africa. ‘Nutritional status’ was defined by studies on dietary intake, measurements of growth/anthropometry, and nutritional biomarkers. Studies of anemia in non-SCD individuals and those with sickle cell traits were excluded. Databases used were Ovid Embase, Medline, Biosis and Web of Science. Results: The search returned 366 articles from which 62 studies were included in the final analyses. Seventy percent of the investigations were conducted in Nigeria. Studies were categorized into one of three main categories: descriptive studies of anthropometric characteristics (50%), descriptive studies of macro- or micronutrient levels (35%), and interventional studies (15%). Findings consistently found growth impairment, especially among children and adolescents. Studies assessing macro- and micro-nutrients generally had small sample sizes and were exploratory in nature. Only two randomized placebo-controlled trials were identified, which measured the impact of fatty acid supplementation and lime juice on health outcomes. Conclusions: The findings reveal a moderate number of descriptive studies, with small sample size, that focused on various aspects of nutrition and sickle cell disease in African patients. There was a stark lack of interventional studies that could be used to inform evidence-based changes in clinical practice. Findings of investigations were generally consistent with data from other regional settings, describing a significant risk of growth faltering and malnutrition among individuals with SCD. There is an unmet need for clinical research to better understand the potential benefits of nutritional-related interventions for patients with SCD in sub-Saharan Africa in order to promote optimal growth and improve health outcomes.

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Clinical phenotypes and the biological parameters of Congolese patients suffering from sickle cell anemia: A first report from Central Africa

Abstract: In our study, fetal hemoglobin has an influence on the clinical severity and the biological parameters of SCA. The study provides data concerning the sickle cell anemia clinical and biological variability in our midst.

Cited by 15 publications

References 29 publications

Homozygous Deletion Alfa-Thalassemia and Hereditary Persistence of Fetal Hemoglobin, Two Genetic Factors Predictive the Reduction of Morbidity and Mortality During Pregnancy in Sickle Cell Patients . A Report From Democratic Republic of Congo

Homozygous Deletion Alfa-Thalassemia and Hereditary Persistence of Fetal Hemoglobin, Two Genetic Factors Predictive the Reduction of Morbidity and Mortality During Pregnancy in Sickle Cell Patients . A Report From Democratic Republic of Congo

Nutritional perspectives on sickle cell disease in Africa: A systematic review

Nutritional Perspectives on Sickle Cell Disease in Africa: A Systematic Review

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