Le syndrome drépanocytaire de type hémoglobine SC : expérience du CHU Yalgado Ouédraogo de Ouagadougou (Burkina Faso)

Ayéroué, J; Kafando, Eléonore; Kam, Ludovic; Gue, Edmond; Vertongen, Françoise; Ferster, Alina; Cotton, Frédéric; Gulbis, Béatrice

doi:10.1016/j.arcped.2009.01.005

Cited by 10 publications

(10 citation statements)

References 13 publications

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“…The present study reported 718 SC genotypes versus 223 SS genotypes. These results are in line with the literature describing more SC carrier than SS individual including Simpore et al 12 with 660 SC and 196 SS recorded, Ayéroué et al 19 , 38 SC and 23 SS as well as Diarra et al 17 , 47 SC and 29 SS. It is noteworthy that the relative genotypic frequency of SC is higher than that of SS in west Africa (0.092 versus 0.029) (Table 2) probably because clinical and hematological parameters of heterozygous HbSC individuals are less critical compared to homozygous HbSS 14 .…”

Section: Discussionsupporting

confidence: 92%

Hemoglobin AE, AO-Arab and SO-Arab Genotypes in Burkina Faso: Hematological Parameters, Genotypic and Allelic Frequencies of Hemoglobinopathies

Traoré¹,

Zohoncon²,

Ouédraogo³

et al. 2020

J Human Clin Gen

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Introduction:The prevalence of hemoglobinopathies (HbC and HbS) is relatively high in West Africa, especially in Burkina Faso. The objectives of this study were to characterize the hematological parameters and to determine the genotypic and allelic frequencies of patients affected with hemoglobinopathies.Methods: Hemoglobin electrophoresis was carried out in a total of 7,789 patients attending Saint Camille Hospital of Ouagadougou during the period of study. Among them, hemogram was performed for 1014 patients. Results:The age of the study population ranged from 1 to 40 years, with a mean of 24.86 ± 12.69 years. The age group 16 to 35 were the most seen at medical center hospital and represented 3,035 out of 7,789 (38.96 %) having performed hemoglobin electrophoresis. The overall hemoglobin electrophoretic profiles revealed 223 SS, 718 SC, 2 SO-Arab, 2 AO-Arab, 3 AE, 152 CC, 799 AS, 1315 AC, and 4575 AA. The relative genotypic frequency was 58.73 % AA, 2.86 % SS and 9.22 % SC while the allelic frequencies were 0.7234 for HbA, 0.1500 for HbC, 0.1261 for HbS, 0.0002 for HbE and 0.0003 for HbO-Arab. The hemogram profiles of patients with major sickle cell syndrome (MSCS) revealed respectively for mean hemoglobin levels, number of red blood cells and mean corpuscular volume a values of 8.19 ± 1.39 g/dL ; 2.97 ± 0.73 10 12 /L ; 81.86 ± 12.82 fL in SS patients and 10.93 ± 1.68 g/dL ; 4.38 ± 0.77 10 12 /L ; 70.81 ± 7.11 fL in SC individuals. Conclusion:The results of the present study are in line with the previous one describing the high prevalence of HbC and HbS hemoglobinopathies in Burkina Faso. Indeed, the genotypic and allelic frequencies of patients with MSCS are increasing due to the accessibility of medical care for sickle cell patients. The study diagnoses for the first-time hemoglobin AE, AO-Arab and SO-Arab genotypes in Burkina Faso.

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Section: Discussionsupporting

confidence: 92%

Hemoglobin AE, AO-Arab and SO-Arab Genotypes in Burkina Faso: Hematological Parameters, Genotypic and Allelic Frequencies of Hemoglobinopathies

Traoré¹,

Zohoncon²,

Ouédraogo³

et al. 2020

J Human Clin Gen

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“…7,8,15,20 This low frequency is probably due to the deficit of medical devices in this health institution for detection of this sickle cell complications during follow-up of the patients.…”

Section: Discussionmentioning

confidence: 98%

“…7,8,15 However, this prevalence rate increases in Western countries with acute chest syndrome ranging to 15.2% to 24% of patients. 13,16,18 Our low prevalence is probably due to underestimation of this sickle cell complications associated with pneumonia episodes in our area.…”

Section: Discussionmentioning

confidence: 99%

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Acute Crises and Complications of Sickle Cell Anemia among Patients Attending a Pediatric Tertiary Unit in Kinshasa, Democratic Republic of Congo

Aloni¹,

Kadima²,

Ekulu³

et al. 2017

Hematology Reports

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In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated to affect 30,000 to 40,000 neonates per year. However, there is paucity of data on acute clinical manifestations in sickle cell children. In these circumstances, it is difficult to develop a health care policy for an adequate management of sickle cell patients. This was a seven years’ retrospective study of children admitted with acute sickle cell crisis in the Department of Pediatrics in University Hospital of Kinshasa, Kinshasa, the Democratic Republic of Congo. A total of 108 patients were identified as having SCA. There were 56 (51%) girls and 52 (49%) boys. Median age was 10.5 years (range 1-24 years). No child was diagnosed by neonatal screening. The median age of diagnosis of sickle cell anemia was 90 months (range: 8-250 months). The median age at the first transfusion was 36 months (range 4-168). In this series, 61 (56.5%) patients were eligible for hydroxyurea. However, this treatment was only performed in 4 (6.6%) of them. Pain episodes, acute anemic crisis and severe infection represent respectively 38.2%, 34.3% and 21.9% of events. Altered sensorium and focal deficit were encountered occasionally and represented 3.4% of acute events. Acute renal manifestations, cholelithiasis and priapism were rarely reported, in this cohort. In Kinshasa, the care of patients suffering from sickle cell anemia is characterized by the delayed diagnosis and low detection of organ complications compared to reports of Western countries. This situation is due to resources deficiencies.

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“…L'hémoglobinose SS était associée à un taux d'hémoglobine et d'hémoglobine fœtale plus bas (p10, 11]. Dans cette forme hétérozygote SC, l’âge de la manifestation clinique de la maladie était plus tardif, l'hémoglobine de base plus élevée, les signes cliniques plus atténués [12], les cavités cardiaques moins dilatées [13], l'HTAP est moins fréquente[10], la durée du recours à l'oxygénothérapie en cas de syndrome douloureux thoracique aigu plus courte [14]. Nous avons confirmé ces données: en effet, nous avons observé, en comparant ces deux formes d'hémoglobinopathie, une atteinte moins sévère dans la forme hétérozygote composite SC avec des taux d'hémoglobine de base moins bas et d'hémoglobine fœtale plus élevés, des taux de LDH moins élevés, des cavités cardiaques moins dilatées et l'HTAP moins fréquente que dans la forme homozygote SS.…”

Section: Résultatsunclassified

Aspects écho-cardiographiques au cours de la drépanocytose en Guadeloupe

Ondze-Kafata¹,

Sanouiller²,

Hédreville³

et al. 2014

Pan Afr Med J

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IntroductionL'atteinte cardiovasculaire représente le facteur pronostique majeur de la drépanocytose.MéthodesC’était une étude transversale et descriptive dans le service de cardiologie du centre hospitalier et universitaire de Pointe-à-Pitre.RésultatsNous avons inclus 82 patients, 23 hommes (28%) et 59 femmes (72%), âgés en moyenne de 40,0±12 ans. On avait 39 patients porteurs d'hémoglobinose SS (49,4%) et 40 porteurs d'hémoglobinose SC (50,6%). Le taux d'hémoglobine de base était en moyenne de 9,8±1,8 g/dl et celui de l'hémoglobine fœtale était en moyenne de 5,5±5,3 g/dl. La fraction de raccourcissement était de 36±4,9% en moyenne, la FeVG moyenne était de 65±5,9%. On avait 68 patients avec un profil mitral normal (82,9%) et 14 patients avec un profil pseudo normal (17,1%). On avait une dilatation ventriculaire gauche chez 26 patients (31,7%), ventriculaire droite chez 14 patients (17.1%), une dilatation auriculaire gauche chez 36 patients (43.9%), auriculaire droite chez 11 patients (13.4%). On avait une hypertrophie ventriculaire gauche chez 13 patients soit 15.9%. L'hypertension artérielle pulmonaire a été retrouvée chez 43 patients soit 52,4%. Après régression logistique, les facteurs suivants ont été associés à la présence d'une HTAP: âge (p=0,0024), le taux hémoglobine de base (p=0,032), le taux d'hémoglobine fœtale (p=0,011), le type SS (p=0,040), la dilatation de l'oreillette gauche (p=0,0012) et la dilatation de ventricule droit (p=0,047). Après comparaison, L'hémoglobinose SS s’était avérée plus sévère.ConclusionLa drépanocytose est une pathologie sévère dont le pronostic est aggravé par l'atteinte cardiaque.

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Le syndrome drépanocytaire de type hémoglobine SC : expérience du CHU Yalgado Ouédraogo de Ouagadougou (Burkina Faso)

Cited by 10 publications

References 13 publications

Hemoglobin AE, AO-Arab and SO-Arab Genotypes in Burkina Faso: Hematological Parameters, Genotypic and Allelic Frequencies of Hemoglobinopathies

Hemoglobin AE, AO-Arab and SO-Arab Genotypes in Burkina Faso: Hematological Parameters, Genotypic and Allelic Frequencies of Hemoglobinopathies

Acute Crises and Complications of Sickle Cell Anemia among Patients Attending a Pediatric Tertiary Unit in Kinshasa, Democratic Republic of Congo

Aspects écho-cardiographiques au cours de la drépanocytose en Guadeloupe

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