%0 Journal Article
%T Clinical course of 63 children with hereditary spherocytosis: a retrospective study
%A Oliveira
%A Maria Christina Lopes Araujo
%A Fernandes
%A Rachel Aparecida Ferreira
%A Rodrigues
%A Carolina Lins
%A Ribeiro
%A Daniela Aguiar
%A Giovanardi
%A Maria Fernanda
%A Viana
%A Marcos Borato
%J Revista Brasileira de Hematologia e Hemoterapia
%D 2012
%I Sociedade Brasileira de Hematologia e Hemoterapia
%R 10.5581/1516-8484.20120006
%X background: hereditary spherocytosis (hs) is an inherited hemolytic anemia that is caused by deficiency or dysfunction of erythrocyte cytoskeletal proteins. aim: the aim of this study was to describe the clinical course of hereditary spherocytosis in patients treated in the pediatric hematology unit, hospital das cl¨ªnicas, universidade federal de minas gerais. methods: sixty-three under 16-year-old patients with hereditary spherocytosis were retrospectively evaluated between january 1988 and december 2007. hereditary spherocytosis was diagnosed based on clinical history, physical examination and on a positive osmotic fragility curve. patients underwent screening for cholelithiasis by ultrasonography. they were classified into three groups: mild, moderate and severe. the events of interest were need for blood transfusion, cholelithiasis, splenic sequestration, aplastic crisis, and splenectomy. differences between subgroups were evaluated by the two-sided log-rank test. results: the mean age at diagnosis was 5.2 years and most patients were classified as moderate (54%). patients with the severe form of the disease were younger (p-value = 0.001) and needed more blood transfusions (p-value = 0.004). seventeen patients (27%) developed cholelithiasis, 14 (22.2%) splenic sequestration and three (4.8%) aplastic crises. twenty-two patients (34.9%) were splenectomized with the main indication being splenic sequestration in nine patients (41%). conclusions: the clinical course of patients with hereditary spherocytosis in this study was relatively benign however cholelithiasis was a common complication.
%K spherocytosis
%K hereditary
%K cholelithiasis
%K splenectomy
%K child
%K retrospective studies.
%U http://www.scielo.br/scielo.php?script=sci_abstract&pid=S1516-84842012000100006&lng=en&nrm=iso&tlng=en