Abstract
Objective
To describe profile of 82 children with hereditary spherocytosis diagnosed over a period of 27 years (1985–2011) from a single center.
Methods
Retrospective analyses of case records.
Results
The mean (SD) age at diagnosis was 6.7 (2.8) years; 7 (8.5%) were diagnosed in infancy. Pallor (100%), icterus (67%), undocumented fever (28%), splenomegaly (96%) and hepatomegaly (73%) were the most frequent findings. Cholelithiasis was observed in 26%. Twenty-six (32%) underwent splenectomy and were followed for a median duration of 4.5 years. Two (7.7%) children developed postsplenectomy sepsis.
Conclusion
Anemia, hepato-splenomegaly and jaundice are commonest clinical features of hereditary spherocytosis. Post-splenectomy sepsis is uncommon.
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Das, A., Bansal, D., Das, R. et al. Hereditary spherocytosis in children: Profile and post-splenectomy outcome. Indian Pediatr 51, 139–141 (2014). https://doi.org/10.1007/s13312-014-0348-5
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DOI: https://doi.org/10.1007/s13312-014-0348-5