title: Improved survival in homozygous sickle cell disease: lessons from a cohort study.
abstract: OBJECTIVE
  To examine whether simple interventions in a sickle cell clinic improve survival in sickle cell disease.
  
  
  METHODS
  Survival curve analysis and hazard ratios in a cohort study followed from birth.
  
  
  METHODS
  MRC Laboratories (Jamaica) at the University of the West Indies, and Victoria Jubilee Hospital, Kingston, Jamaica.
  
  
  METHODS
  315 patients with homozygous sickle cell disease detected during the screening of 100,000 consecutive non-operative deliveries between June 1973 and December 1981 at the main government maternity hospital, Kingston, Jamaica.
  
  
  METHODS
  Prophylactic penicillin to prevent pneumococcal septicaemia, parental education in early diagnosis of acute splenic sequestration, close monitoring in sickle cell clinic.
  
  
  METHODS
  Survival.
  
  
  RESULTS
  Survival appeared to improve, the log rank test for trend comparing the first, second, and last third of the study reaching borderline significance (P = 0.05). Combined deaths from acute splenic sequestration and pneumococcal septicaemia-meningitis declined significantly (test for trend, P = 0.02).
  
  
  CONCLUSIONS
  Early diagnosis and simple prophylactic measures significantly reduce deaths associated with homozygous sickle cell disease.
authors.affiliation: MRC Laboratories, (Jamaica), University of the West Indies, Kingston, Jamaica.
authors.forename: A
authors.initials: A
authors.lastname: Lee
authors.forename: P
authors.initials: P
authors.lastname: Thomas
authors.forename: L
authors.initials: L
authors.lastname: Cupidore
authors.forename: B
authors.initials: B
authors.lastname: Serjeant
authors.forename: G
authors.initials: G
authors.lastname: Serjeant
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000235:genetics / Q000401:mortality* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D006720:Homozygote; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007563:Jamaica / Q000453:epidemiology; D008581:Meningitis / Q000401:mortality / Q000517:prevention & control; D010290:Parents / Q000193:education; D010406:Penicillins / Q000627:therapeutic use; D011008:Pneumococcal Infections / Q000401:mortality / Q000517:prevention & control; D016016:Proportional Hazards Models; D018805:Sepsis / Q000401:mortality / Q000517:prevention & control; D013158:Splenic Diseases / Q000175:diagnosis / Q000401:mortality; D015996:Survival Rate
chemical_list: D010406:Penicillins