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Medicina (Guayaquil) ; 14(2): 156-161, mar. 2009.
Article in Spanish | LILACS | ID: lil-617742

ABSTRACT

La esferocitosis hereditaria conocida también como enfermedad de Minkowski-Chauffard, es una patología genética que forma parte de las anemias hemolíticas, caracterizada por la producción de hematíes de forma esferoidal, por un defecto en la membrana del mismo, lo cual hace que se destruyan en el bazo. A menudo se diagnostica durante la niñez, pero es posible que los casos más leves se descubran de manera incidental en la vida adulta. Realizamos una revisión de la esferocitosis hereditaria, a propósito de la presentación de un paciente del hospital “Luis Vernaza” a quien se diagnosticó esta enfermedad a los 48 años de edad y se aplicó tratamiento médico y quirúrgico con resultados favorables.


Hereditary spherocytosis also known as Minkowski-Chauffard syndrome, is a genetic disease that belong in the group of the hemolytic anemias. It is (characterized) by the production of spheroid blood cells because of a membrane defect, which makes them destroy themselves inside the spleen. It is frequently diagnosed during childhood, but it is possible that minor cases are discovered in an incidental way during adulthood. We carried out a revision of hereditary spherocytosis, because of patient in Hospital Luis Vernaza who was diagnosed this disease at the age of 48 and had medical and surgical treatment with favorable outcome.


Subject(s)
Male , Adult , Anemia, Hemolytic, Congenital , Congenital Abnormalities , Spherocytosis, Hereditary , Gallstones , Hemolysis , Jaundice , Splenomegaly
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