title: Severe chronic neutropenia in Chinese children in Hong Kong.
abstract: OBJECTIVE
  Severe chronic neutropenia (SCN) is a rare and heterogeneous disorder in children. The epidemiology, clinical features and outcomes of SCN in Chinese children were reviewed.
  
  
  METHODS
  A retrospective analysis of case records was undertaken for 18 children with SCN managed during a 12-year period in a university teaching hospital in Hong Kong.
  
  
  RESULTS
  The median (range) age of the patients at initial presentation was 6.5 months (4 days-19 months). The initial and lowest median absolute neutrophil counts (ANC) were 0.29 x 109 /L and 0.06 x 109 /L, respectively. Patients with congenital SCN had significantly fewer neutrophils in peripheral blood at diagnosis. Only five subjects received granulocyte colony-stimulating factor (G-CSF) treatment. All children were free from serious infection on follow up for 51 months. Only one child suffered from long-term infection-related morbidity. One patient with chronic neutropenia was subsequently shown to have common variable immunodeficiency.
  
  
  CONCLUSIONS
  Most children with SCN in our series had favourable clinical outcomes. Our results support the recommendation that G-CSF should be used only in those with recurrent or severe infections.
authors.affiliation: Department of Paediatrics, Prince of Wales Hospital, Hong Kong SAR, China. leung2142@cuhk.edu.hk
authors.forename: T F
authors.initials: TF
authors.lastname: Leung
authors.forename: C K
authors.initials: CK
authors.lastname: Li
authors.forename: K L
authors.initials: KL
authors.lastname: Kwok
authors.forename: K W
authors.initials: KW
authors.lastname: Chik
authors.forename: M M
authors.initials: MM
authors.lastname: Shing
authors.forename: P M
authors.initials: PM
authors.lastname: Yuen
mesh_terms: D002681:China / Q000208:ethnology; D002908:Chronic Disease; D013293:Enterococcus faecalis / Q000302:isolation & purification; D005260:Female; D000069585:Filgrastim; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006723:Hong Kong / Q000453:epidemiology; D006801:Humans; D007223:Infant; D008297:Male; D016920:Meningitis, Bacterial / Q000150:complications / Q000382:microbiology; D009503:Neutropenia / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology*; D011552:Pseudomonas Infections / Q000150:complications; D011994:Recombinant Proteins; D012189:Retrospective Studies
chemical_list: D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor; D000069585:Filgrastim