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Tunis Med ; 95(12): 229-233, 2017 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-29878289

RESUMEN

BACKGROUND: - Hemoglobin C is a hemoglobin variant encountered worldwide. The regionswith high prevalence are West Africa and South-East Asia.The objective of this study is to report cases of hemoglobin C disease brought together during these last twelve years in the Laboratory of Biochemistry and Toxicology of RabatMilitary Hospital Mohammed V (MHIMV). METHODS: - This was a retrospective study including111 cases of hemoglobin C disease collected in the Laboratory of Biochemistry of the MHIMVover the past 12 years. A questionnairewasfulfilledwith the epidemiological data,clinical data and the results of the biological explorations. The screening of the hemoglobin variant in this study included several biochemical (hemoglobin electrophoresis at acid and alkalinepH) and hematological tests. RESULTS: - Sex-ratio was equal to 1,22. The age at the time of diagnosis ranges between 4 and 80years old, with the mean of 38. North-West regions of Morocco seem most affected. The most frequent reasons for prescription of the hemoglobin's studywere: biological abnormalities, splenomegaly and anemic syndrome. Blood smear reveals frequently anisopoikilocytosis and red blood target. The biochemical tests contribute to the diagnosis and reveal various and varied etiological groups: heterozygous A/C (75%),homozygous C/C (8%), double heterozygous S/C (9%),C/ß+-thal (6%) andC/O-Arab (2%). Conclusion - The results of the present descriptive study are in line with the literature data. The importance of genetic counseling and the installation of a national card of systematic neonatal tracking seemto be unavoidable.


Asunto(s)
Enfermedad de la Hemoglobina C/diagnóstico , Enfermedad de la Hemoglobina C/epidemiología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Electroforesis de las Proteínas Sanguíneas , Niño , Preescolar , Estudios de Cohortes , Femenino , Pruebas Genéticas , Pruebas Hematológicas , Hemoglobina C/análisis , Hemoglobina C/genética , Hemoglobina C/metabolismo , Enfermedad de la Hemoglobina C/sangre , Enfermedad de la Hemoglobina C/genética , Humanos , Masculino , Persona de Mediana Edad , Marruecos/epidemiología , Estudios Retrospectivos , Adulto Joven
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