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Abstract
Introduction. Les hémoglobinopathies sont très répandues à travers le monde et la drépanocytose constitue la forme la plus fréquente. Cette dernière représente au Niger un problème de santé publique avec une prévalence du portage de l’hémoglobine S (HbS) de 25%. Cette étude a pour but de faire le bilan des hémoglobinopathies diagnostiquées au Laboratoire de Biochimie de la Faculté des Sciences de la Santé (FSS) de l’Université Abdou Moumouni de Niamey.
Méthodologie. Il s’agit d’une étude rétrospective et prospective de 2000 à 2010. L’électrophorèse de l’hémoglobine était réalisée sur acétate de cellulose à PH alcalin.
Résultats. 6532 sujets ont été inclus dont 2921 hommes (46,57%) et 3351 femmes (53,42%). Les hémoglobines anormales représentaient 52,84% (n = 3120) et les hémoglobines normales 47,76% (n =3452). L’hémoglobine AS (HbAS) a été retrouvée dans 30,4% ; l’ Hb AC dans 3,2%, l’ HbSC dans 5,4% ; l’ HbSS dans 15,1% et l’ Hb CC dans 0,3%. La répartition des hémoglobines selon l’ethnie a montré que l’HbSC était présente chez les Gourmantché, peulhs, Touaregs, Haoussa et Djerma-Sonraï dans respectivement 46% ; 34,5% ; 33,6% ; 30,6% et 29%. Pour l’ HbSS, elle était présente dans ces ethnies dans respectivement 16,2% ; 10,6% ; 10,3%, 16,8% et 15,2%. Chez les Kanouri, ce taux est de 12, 4%.
Conclusion. Les hémoglobines S et C restent les plus fréquentes au Niger.
ABSTRACT
Aim. Abnormal hemoglobin is a public health problem in Niger with a prevalence of hemoglobin S (HbS) of 25%. The aim of the study was to review of haemoglobinopathies diagnosed in the biochemistry laboratory of the faculty of medicine in Niamey.
Methods. This was a prospective and retrospective study during 10 years from 2000 to 2010. Electrophoresis was performed on cellulose acetate at alkaline PH.
Results. We included 6532 subjects with 53.4% female and 46.6% male. Abnormal hemoglobin represented 52, 84% (n = 3120) and normal hemoglobin 47.76% (n=3452). Hemoglobin AS (HbAS) was encountered in 30.4%; Hb AC in 3, 2%, HbSC in 5.4%; HbSS in 15.1% and Hb CC in 0.3%. The distribution of hemoglobin by ethnicity showed that HbSC was present in Gourmantché, Fulani, Tuareg, Hausa and Zarma-Songhai in respectively 46%, 34.5%, 33.6%, 30.6% and 29%. For HbSS, the distribution in these ethnics groups was respectively 16.2%, 10.6%, 10.3%, 16.8% and 15.2%. In Kanuri, the rate was 12.4%.
Conclusion. The hemoglobin S and C are the two most common haemoglobinopathies in Niger.
Article Details
References
- - Weatherall D, Akinyanju O, Fucharoen S, Olivieri N, Musgrove P. Inherited Disorders of Hemoglobin. In: Jamison DT, Breman JG, Measham AR, Alleyne G, Claeson M, Evans DB, Jha P, Mills A, Musgrove P. Editors. Disease Control Priorities in Developing Countries. 2nd edition. Washington (DC): World Bank 2006; 34: 663-680.
- - Organisation mondiale de la Santé. Programme Maladies Non Transmissibles, y compris les affections buccodentaires et la Santé mentale. Rapport annuel OMS - Niger 2010: 20.
- - Livingstone F B. Abnormal Hemoglobins in Human Populations. Aldine pub. Co. Chicago, 1967: 482.
- - Serjeant GR, Higgs DR, Aldridge B, Hayes RJ, Weatherall DJ. Alpha thalassemia and homozygous sickle cell disease. Prog Clin Biol Res. 1981; 55:781-8.
- - Juneja N, S. Reed K. Path physiology and management of sickle cell pain crisis: therapy. Lancet 1995, 346:1408-1411.
- - Cabannes R, Fabritius H, Sangare A, Kple-Faget P - Hemoglobin variants: distribution in West Africa. CRC Press Inc ed, Florida, 1987; 2:11-25.
- - Segbena A.Y., Kueviakoe I., Messie A.K., Napo-Koura I.G, Vovor A, David M. Les anomalies de l’hémoglobine au centre hospitalier universitaire de Lomé, Togo. Med Trop 2002; 62: 51-54.
- - Mijiyawa M, Segbena A, Vovor A, Nubukpo P, David M, Amédégnato D. Hemoglobinopathies and rheumatology clinic patients in Lomé (Togo). Rev Rhum Engl 1994; 61: 165-169.
- - Segbena AY, Prehu C, Wajcman H, Bardakdjian-Michau J, Messie K, Feteke L, Vovor A, David M, Feingold J, Galacteros F. Hemoglobins in Togolese newborns: Hb S, Hb C, Hb Bart’s and a-Globin gene status. Am J Hematol 1998; 59: 208-213.
- - North ML, Piffaut MC, Duwig I, Locoh-Donou AM. Detection of haemoglobinop athies at birth in Togo. Nouv Rev Fr Hematol 1988 ; 30 : 237-241.
- - Sangare A, Sanogo I, Meite M, Ambofo Y, Abe S, Segbena A. Prévalence et profil génétique de l’a-thalassémie chez le nouveau-né en Côte d’Ivoire. Med Trop 1992; 52: 157-161.
- - Latoundji S, Anani L, Ablet E, Zohoun I. Morbidite et mortalite drépanocytaire au Benin. Médecine d'Afrique Noire: 1991, 38 (8/9).
- - Haj Khelil A, Denden S, Leban N, Daimi H, Lakhdhar R, Lefranc G, Ben Chibani J, Perrin P. Hemoglobinopathies in North Africa: a review. Hemoglobin 2010; 34:1–23.
- - Nafei A. Prevalence of hemogolobinopathies and study of their genetics. Tanta Med J 1992; 20:467–480.
- - Sunna EI, Gharaibeh NS, Knapp DD, Bashir NA. Prevalence of hemoglobin S and beta-thalassemia in northern Jordan. J Obstet Gynaecol Res 1996; 22:17–20.
- - Jain RC. Sickle cell and thalassaemic genes in Libya. Trans R Soc Trop Med Hyg 1985; 79:132–133
- - Fattoum S Hemoglobinopathies in Tunisia. An updated review of the epidemiologic and molecular data. Tunis Med 2006; 84:687–696.
- - Piel FB, Howes RE, Patil AP, Nyangiri OA, Gething PW, Bhatt S, Williams TN, Weatherall DJ, Hay SI. The distribution of haemoglobin C and its prevalence in newborns in Africa. Sci Rep. 2013; 3:1671.
References
- Weatherall D, Akinyanju O, Fucharoen S, Olivieri N, Musgrove P. Inherited Disorders of Hemoglobin. In: Jamison DT, Breman JG, Measham AR, Alleyne G, Claeson M, Evans DB, Jha P, Mills A, Musgrove P. Editors. Disease Control Priorities in Developing Countries. 2nd edition. Washington (DC): World Bank 2006; 34: 663-680.
- Organisation mondiale de la Santé. Programme Maladies Non Transmissibles, y compris les affections buccodentaires et la Santé mentale. Rapport annuel OMS - Niger 2010: 20.
- Livingstone F B. Abnormal Hemoglobins in Human Populations. Aldine pub. Co. Chicago, 1967: 482.
- Serjeant GR, Higgs DR, Aldridge B, Hayes RJ, Weatherall DJ. Alpha thalassemia and homozygous sickle cell disease. Prog Clin Biol Res. 1981; 55:781-8.
- Juneja N, S. Reed K. Path physiology and management of sickle cell pain crisis: therapy. Lancet 1995, 346:1408-1411.
- Cabannes R, Fabritius H, Sangare A, Kple-Faget P - Hemoglobin variants: distribution in West Africa. CRC Press Inc ed, Florida, 1987; 2:11-25.
- Segbena A.Y., Kueviakoe I., Messie A.K., Napo-Koura I.G, Vovor A, David M. Les anomalies de l’hémoglobine au centre hospitalier universitaire de Lomé, Togo. Med Trop 2002; 62: 51-54.
- Mijiyawa M, Segbena A, Vovor A, Nubukpo P, David M, Amédégnato D. Hemoglobinopathies and rheumatology clinic patients in Lomé (Togo). Rev Rhum Engl 1994; 61: 165-169.
- Segbena AY, Prehu C, Wajcman H, Bardakdjian-Michau J, Messie K, Feteke L, Vovor A, David M, Feingold J, Galacteros F. Hemoglobins in Togolese newborns: Hb S, Hb C, Hb Bart’s and a-Globin gene status. Am J Hematol 1998; 59: 208-213.
- North ML, Piffaut MC, Duwig I, Locoh-Donou AM. Detection of haemoglobinop athies at birth in Togo. Nouv Rev Fr Hematol 1988 ; 30 : 237-241.
- Sangare A, Sanogo I, Meite M, Ambofo Y, Abe S, Segbena A. Prévalence et profil génétique de l’a-thalassémie chez le nouveau-né en Côte d’Ivoire. Med Trop 1992; 52: 157-161.
- Latoundji S, Anani L, Ablet E, Zohoun I. Morbidite et mortalite drépanocytaire au Benin. Médecine d'Afrique Noire: 1991, 38 (8/9).
- Haj Khelil A, Denden S, Leban N, Daimi H, Lakhdhar R, Lefranc G, Ben Chibani J, Perrin P. Hemoglobinopathies in North Africa: a review. Hemoglobin 2010; 34:1–23.
- Nafei A. Prevalence of hemogolobinopathies and study of their genetics. Tanta Med J 1992; 20:467–480.
- Sunna EI, Gharaibeh NS, Knapp DD, Bashir NA. Prevalence of hemoglobin S and beta-thalassemia in northern Jordan. J Obstet Gynaecol Res 1996; 22:17–20.
- Jain RC. Sickle cell and thalassaemic genes in Libya. Trans R Soc Trop Med Hyg 1985; 79:132–133
- Fattoum S Hemoglobinopathies in Tunisia. An updated review of the epidemiologic and molecular data. Tunis Med 2006; 84:687–696.
- Piel FB, Howes RE, Patil AP, Nyangiri OA, Gething PW, Bhatt S, Williams TN, Weatherall DJ, Hay SI. The distribution of haemoglobin C and its prevalence in newborns in Africa. Sci Rep. 2013; 3:1671.