Clinical course of 63 children with hereditary spherocytosis: a retrospective study
Autor(a) principal: | |
---|---|
Data de Publicação: | 2012 |
Outros Autores: | , , , , |
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. |
id |
ABHHTC-1_a15f9333a83689254ef8dfdb79c5e9a7 |
---|---|
oai_identifier_str |
oai:scielo:S1516-84842012000100006 |
network_acronym_str |
ABHHTC-1 |
network_name_str |
Revista brasileira de hematologia e hemoterapia (Online) |
repository_id_str |
|
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 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000100006 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000100006 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.5581/1516-8484.20120006 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
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) |
repository.mail.fl_str_mv |
sbhh@terra.com.br||secretaria@rbhh.org |
_version_ |
1754213111540219904 |