title: Hereditary spherocytosis in children: profile and post-splenectomy outcome.
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 post-splenectomy sepsis.
  
  
  CONCLUSIONS
  Anemia, hepato-splenomegaly and jaundice are commonest clinical features of hereditary spherocytosis. Post-splenectomy sepsis is uncommon.
authors.affiliation: Pediatric Hematology-oncology unit, Advanced Pediatric Center and *Department of Hematology, PGIMER, Chandigarh, India. Correspondence to: Dr Deepak Bansal, Hematology-oncology unit, Department of Pediatrics, Advanced Pediatric Center, PGIMER, Chandigarh, India. deepakbansaldr@gmail.com.
authors.forename: Anirban
authors.initials: A
authors.lastname: Das
authors.forename: Deepak
authors.initials: D
authors.lastname: Bansal
authors.forename: Reena
authors.initials: R
authors.lastname: Das
authors.forename: Amita
authors.initials: A
authors.lastname: Trehan
authors.forename: R K
authors.initials: RK
authors.lastname: Marwaha
mesh_terms: D000293:Adolescent; D017487:Ankyrins / Q000172:deficiency*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007194:India; D007223:Infant; D008297:Male; D011183:Postoperative Complications; D012189:Retrospective Studies; D013103:Spherocytosis, Hereditary / Q000175:diagnosis / Q000601:surgery*; D013156:Splenectomy; D016896:Treatment Outcome
chemical_list: D017487:Ankyrins