Beta thalassaemiatriat in Western Nigeria

Background: Genes for thalassaemia; haemoglobin S; Glucose-6-phosphate dehydrogenase which confer resistance to malaria are found in high frequencies in Nigeria; 25of the population being carriers of the sickle cell trait while another 25are hemizygous for the G6PD gene. The frequency of alpha thalassaemia is equally high among Nigerians but there is little information on beta thalassaemia in this population. A recent study however suggest a high prevalence of beta thalassaemia in the same population; hence the need for this study. Methods: Haemoglobin A2 and HbF were determined in healthy adults who have haemoglobin A genotype by elution after electrophoresis and alkaline denaturation methods respectively. Results: The mean HbA2 among the subjects was 3.3(range 2.0-5.6) while the mean HbF was 2.6(range 0.4-8.8). Twenty-six percent of the subjects had HbA2 values higher than 3.9while 86had HbF values greater than 1; twenty-four percent had elevated HbA2 and HbF. The mean HbA2 value was 2.7among those with HbF 1; 3.6among those with HbF 1-3and 3.1among those with HbF 3. Conclusion: These findings confirm that the frequency of beta thalassaemia in western Nigeria is higher than previously thought and that many of the individuals studied may be silent carriers of the beta thalassaemia trait. Its presence may also have been masked by the high prevalence of alpha thalassaemia in the same environment. It is therefore important to consider beta thalassaemia trait as a differential diagnosis in patients who present with haemolytic anaemia in this environment

Control of sickle cell disorder in Africa

Sickle cell disorder (SCD); understandably; concerns millions of Africans. There is no doubt that the prevalence of the condition is increasing; especially among the urban educated elite and in othercommunities who have access to effective basic health care. There is; however; a palpable lack of information and education about the disorder; which; with the increasing prevalence; has encouraged the growth of myths; misinformation; inappropriate treatment; frustration and stigmatization. The frustration has kindled the desire in many Africans to do something about sickle cell disorder. What needs to be done often appears to be deceptively easy; but it is usually not critically considered and therefore continues to be confusing and controversial even among health careprofessionals. In this regard; one frequently hears talk of eradication of the disorder by enactment of legislation while the wider context of control is invariably overlooked. In this paper; I shall rely heavily on my experience in Nigeria and discuss some issues pertaining to the management and control of SCD in Africa in the hope that it will promote a better understanding of its complexities and help readers identify credible and effective strategies and solutions in their own communities

Splenectomie au cours des maladies drépanocytaires et thalassemiques chez l'enfant au CHU de Yopougon: indications et évolutions post opératoire

La splenectomie est indiquee dans le traitement de certaines complications evolutives des hemoglobinopathies majeures. Chez l'enfant la rate intervient dans l'immunisation et son ablation expose a des infections severes. PATIENTS ET METHODE : 14 dossiers medicaux d'enfants operes de juillet 1991 a septembre 2004 pour splenomegalie sur rate hematologique ont ete analyses retrospectivement. Ont ete inclus; les enfants de 0 15 ans des deux sexes pour lesquels le dossier comportait un hemogramme pre operatoire; post operatoire immediat et un mois post operatoire. Tous les enfants operes avaient une anti biotherapie post operatoire et une couverture vaccinale. Il dossiers avaient ete exploitables pour l'etude. AU PLAN EPIDEMIOLOGIQUE : L'age moyen etait de 9;2 ans avec des extremes de 6 et 13 ans. Le sex ratio etait de 1;75 (4 filles pour 7 garcons); la taille n'a pu etre etudie pour manque de donnees. AU PLAN DIAGNOSTIC : La splenomegalie etait de type III dans 73pour cent des cas; l'anemie etait presente chez tous nos patients; 72;3pour cent des patients avaient une drepanocytose majeure. L'hypersplenisme etait la principale indication. AU PLAN THERAPEUTIQUE : La frequence annuelle de transfusion avant la splenectomie etait de 6. La splenectomie totale avait ete realisee chez enfants porteurs de drepanocytose majeure; la splenectomie partielle etait realisee chez les enfants porteurs de thalassemie. Toutes les splenectomies ont ete realisees par la voie transversale sus ombilicale gauche. Le poids moyen des rates operees etait de 783g. AU PLAN EVOLUTIF : L'augmentation du nombre de globules rouges et du taux d'hemoglobine etait significative. La frequence annuelle de transfusion etait de 3 dans le post operatoire. L'incidence des sepsis etait de 27;3pour cent apres une splenectomie totale. Aucune infection n'a ete observee apres splenectomie partielle. Deux recidives d'anemie chronique etait observees apres splenectomie partielle. La mortalite etait nulle