Clinical course of 63 children with hereditary spherocytosis: a retrospective study

Detalhes bibliográficos
Autor(a) principal: Oliveira,Maria Christina Lopes Araujo
Data de Publicação: 2012
Outros Autores: Fernandes,Rachel Aparecida Ferreira, Rodrigues,Carolina Lins, Ribeiro,Daniela Aguiar, Giovanardi,Maria Fernanda, Viana,Marcos Borato
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Revista brasileira de hematologia e hemoterapia (Online)
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000100006
Resumo: 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 Clínicas, 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.
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spelling Clinical course of 63 children with hereditary spherocytosis: a retrospective studySpherocytosis, hereditaryCholelithiasisSplenectomyChildRetrospective studiesBACKGROUND: 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 Clínicas, 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.Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular2012-01-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000100006Revista Brasileira de Hematologia e Hemoterapia v.34 n.1 2012reponame:Revista brasileira de hematologia e hemoterapia (Online)instname:Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)instacron:ABHHTC10.5581/1516-8484.20120006info:eu-repo/semantics/openAccessOliveira,Maria Christina Lopes AraujoFernandes,Rachel Aparecida FerreiraRodrigues,Carolina LinsRibeiro,Daniela AguiarGiovanardi,Maria FernandaViana,Marcos Boratoeng2012-03-13T00:00:00Zoai:scielo:S1516-84842012000100006Revistahttp://www.rbhh.org/pt/archivo/https://old.scielo.br/oai/scielo-oai.phpsbhh@terra.com.br||secretaria@rbhh.org1806-08701516-8484opendoar:2012-03-13T00:00Revista brasileira de hematologia e hemoterapia (Online) - Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)false
dc.title.none.fl_str_mv Clinical course of 63 children with hereditary spherocytosis: a retrospective study
title Clinical course of 63 children with hereditary spherocytosis: a retrospective study
spellingShingle Clinical course of 63 children with hereditary spherocytosis: a retrospective study
Oliveira,Maria Christina Lopes Araujo
Spherocytosis, hereditary
Cholelithiasis
Splenectomy
Child
Retrospective studies
title_short Clinical course of 63 children with hereditary spherocytosis: a retrospective study
title_full Clinical course of 63 children with hereditary spherocytosis: a retrospective study
title_fullStr Clinical course of 63 children with hereditary spherocytosis: a retrospective study
title_full_unstemmed Clinical course of 63 children with hereditary spherocytosis: a retrospective study
title_sort Clinical course of 63 children with hereditary spherocytosis: a retrospective study
author Oliveira,Maria Christina Lopes Araujo
author_facet Oliveira,Maria Christina Lopes Araujo
Fernandes,Rachel Aparecida Ferreira
Rodrigues,Carolina Lins
Ribeiro,Daniela Aguiar
Giovanardi,Maria Fernanda
Viana,Marcos Borato
author_role author
author2 Fernandes,Rachel Aparecida Ferreira
Rodrigues,Carolina Lins
Ribeiro,Daniela Aguiar
Giovanardi,Maria Fernanda
Viana,Marcos Borato
author2_role author
author
author
author
author
dc.contributor.author.fl_str_mv Oliveira,Maria Christina Lopes Araujo
Fernandes,Rachel Aparecida Ferreira
Rodrigues,Carolina Lins
Ribeiro,Daniela Aguiar
Giovanardi,Maria Fernanda
Viana,Marcos Borato
dc.subject.por.fl_str_mv Spherocytosis, hereditary
Cholelithiasis
Splenectomy
Child
Retrospective studies
topic Spherocytosis, hereditary
Cholelithiasis
Splenectomy
Child
Retrospective studies
description 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 Clínicas, 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.
publishDate 2012
dc.date.none.fl_str_mv 2012-01-01
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dc.publisher.none.fl_str_mv Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular
publisher.none.fl_str_mv Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular
dc.source.none.fl_str_mv Revista Brasileira de Hematologia e Hemoterapia v.34 n.1 2012
reponame:Revista brasileira de hematologia e hemoterapia (Online)
instname:Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)
instacron:ABHHTC
instname_str Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)
instacron_str ABHHTC
institution ABHHTC
reponame_str Revista brasileira de hematologia e hemoterapia (Online)
collection Revista brasileira de hematologia e hemoterapia (Online)
repository.name.fl_str_mv Revista brasileira de hematologia e hemoterapia (Online) - Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)
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