title: Mortality in childhood progressive encephalopathy from 1985 to 2004 in Oslo, Norway: a population-based study.
abstract: OBJECTIVE
  The aims were to estimate case fatality and survival rates, standardized mortality ratio (SMR), and independent prognostic factors for survival, in a population-based cohort of progressive encephalopathy (PE) patients.
  
  
  METHODS
  We divided onset of disease into neonatal and postneonatal groups and aetiology into metabolic (n=55), neurodegenerative (n=27) and HIV encephalopathy (n=2) groups. Case fatality was the number of deaths divided by the number of patients. Cumulative survival probability at 10 years of follow-up and independent risk factors for mortality were analyzed using the Kaplan-Meier survival curve and the Cox model.
  
  
  RESULTS
  Case fatality was 36.9% and the mean and median follow-up times were 3109 and 2887 days. At 1 and 10 years, the cumulative probability of survival was 81% and 66%. Neonatal onset showed increased risk of death compared to postneonatal onset (RR 3.0; 95% CI 1.4-6.2). Metabolic aetiology showed increased risk of death compared to other aetiology (RR 1.25; 95% CI 1.10-1.46). The SMR of 37.7 for boys and 23.8 for girls was significantly increased (p<0.001) compared to the total Norwegian population stratified by gender and age.
  
  
  CONCLUSIONS
  Children with PE showed a vast excess in mortality compared to the general population stratified by gender and age. Neonatal presentation and metabolic aetiology were the most significant factors for increased risk of death.
authors: Department of Pediatrics, Ullevål University Hospital, Oslo, Norway. petter.stromme@medisin.uio.no
authors: Petter
authors: P
authors: Strømme
authors: Per
authors: P
authors: Magnus
authors: Øivind Juris
authors: ØJ
authors: Kanavin
authors: Terje
authors: T
authors: Rootwelt
authors: Berit
authors: B
authors: Woldseth
authors: Michael
authors: M
authors: Abdelnoor
mesh_terms: D015526:AIDS Dementia Complex / Q000401:mortality*; D001928:Brain Diseases, Metabolic / Q000401:mortality*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D019636:Neurodegenerative Diseases / Q000401:mortality*; D009664:Norway / Q000453:epidemiology; D012307:Risk Factors; D016019:Survival Analysis; D015996:Survival Rate