title: Idiopathic hypereosinophilic syndrome in children: 3 cases with review of literature.
abstract: OBJECTIVE
  To highlight the varied clinical manifestations and management of idiopathic hypereosinophilic syndrome, a rare disorder in children.
  
  
  METHODS
  Retrospective review of case records of 3 patients who were diagnosed to have idiopathic hypereosinophilic syndrome (IHES) in a tertiary referral centre between 1997 and 2010 was performed. These 3 children presented with different symptoms and the first case had cardiac involvement. All had very high absolute eosinophil count (cells/mm(3)) 43,206, 9,082, 2,925, respectively. Diagnosis was confirmed by bone marrow biopsy in all three cases and supported by liver biopsy in the first two cases and inguinal lymphnode biopsy in the last case. All 3 children responded to treatment with steroids, with only second patient requiring hydroxyurea to control disease.
  
  
  RESULTS
  Case 1 and 2 are on follow up for 13 y and 10 y respectively and both are asymptomatic. Case 3 expired due to sepsis 1 mo after diagnosis.
  
  
  CONCLUSIONS
  Treatment with steroids with or without hydroxyurea gave good response in all 3 cases. Hence, they still remain the gold standard for the treatment of IHES in children.
authors: Department of Pediatrics, Kasturba Medical College, A-14, A Block, KMC Staff Quarters, Light House Hill Road, Mangalore, 575003, India. jayashreedoc@gmail.com
authors: Jayashree
authors: J
authors: Kanthila
authors: Nalini
authors: N
authors: Bhaskaranand
mesh_terms: D002675:Child, Preschool; D005260:Female; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D017681:Hypereosinophilic Syndrome / Q000188:drug therapy / Q000473:pathology; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use
chemical_list: D013256:Steroids; D006918:Hydroxyurea