Clinical course of 63 children with hereditary spherocytosis: a retrospective study
AUTOR(ES)
Oliveira, Maria Christina Lopes Araujo, Fernandes, Rachel Aparecida Ferreira, Rodrigues, Carolina Lins, Ribeiro, Daniela Aguiar, Giovanardi, Maria Fernanda, Viana, Marcos Borato
FONTE
Revista Brasileira de Hematologia e Hemoterapia
DATA DE PUBLICAÇÃO
2012
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.
Documentos Relacionados
- Comments on 'Clinical course of 63 children with hereditary spherocytosis: a retrospective study': with the particular question: 'Should HS be treated the same way throughout the world?'
- Cardiopulmonary bypass in hereditary spherocytosis: a case report
- Analysis of the SLC4A1 gene in three Mexican patients with hereditary spherocytosis: report of a novel mutation
- Retrospective case-control study of diffusely adhering Escherichia coli and clinical features in children with diarrhea.
- ERYTHROCYTE CARBOHYDRATE METABOLISM IN HEREDITARY SPHEROCYTOSIS*