%0 Journal Article
%T Enfermedad por Hemoglobina H: primer caso de la variante de hemoglobina H tipo (-¦Á3.7/ --SEA) en Costa Rica.
%A Cart¨ªn-S¨¢nchez
%A Walter
%A Valverde-Mu£¿oz
%A Kathia
%A Su¨¢rez-Vargas
%A Carlos
%A L¨®pez-Villegas
%A Jorge
%J Acta M¨¦dica Costarricense
%D 2010
%I Scientific Electronic Library Online
%X hemoglobin h (hb h) disease is the most common form of thalassemia intermedia and has many features that require careful consideration in its management. in the majority of cases, the disease results from double heterozygosity for ¦Á0thalassemia due to deletions that remove both linked ¦Áglobin genes on one chromosome 16, and deletional ¦Á+ from single ¦Á-globin gene deletions on the other chromosome 16 resulting in a (--/-¦Á) condition. the excess ¦Â globin chain precipitates and forms a characteristic abnormal hemoglobin: hemoglobin h a ¦Â globin tetramer (¦Â4). in a steady state, patients with hb h disease have hemoglobin levels around 9 to 10 g/dl however, during a hemolytic crisis, which frequently occur in or after acute infections causing high fever, the hemoglobin may drop significantly and the patients can develop shock or renal shutdown. even though splenectomy leads to significant elevation of hemoglobin levels, it is not recommended because the majority of patients do well with said steady-state hemoglobin levels. we present here the first case of hemoglobin h (-¦Á3.7/ --sea) southeast asia variant described in costa rica.1
%K hemoglobin h
%K alpha-thalassemia
%K hemolytic anemia.
%U http://www.scielo.sa.cr/scielo.php?script=sci_abstract&pid=S0001-60022010000300009&lng=en&nrm=iso&tlng=en