Abstract | BACKGROUND: AIM: 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%). CONCLUSION:
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Authors | Maria Christina Lopes Araujo Oliveira, Rachel Aparecida Ferreira Fernandes, Carolina Lins Rodrigues, Daniela Aguiar Ribeiro, Maria Fernanda Giovanardi, Marcos Borato Viana |
Journal | Revista brasileira de hematologia e hemoterapia
(Rev Bras Hematol Hemoter)
Vol. 34
Issue 1
Pg. 9-13
( 2012)
ISSN: 1806-0870 [Electronic] Brazil |
PMID | 23049376
(Publication Type: Journal Article)
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