title: A case series of six children with primary pulmonary hypertension.
abstract: BACKGROUND
  Primary pulmonary hypertension is an uncommon but serious disease in children. Management is difficult despite recent advances in pharmacotherapy.
  
  
  METHODS
  We reviewed patients with this condition with respect to their presenting symptoms, investigations, treatment and outcome.
  
  
  METHODS
  These children were treated with individualised combinations of oxygen, diuretics and calcium antagonists.
  
  
  RESULTS
  In our follow-up of 5 children, 4 had died.
  
  
  CONCLUSIONS
  This condition is irreversible and progressive with a high mortality rate. A better understanding of, and research into, the pathogenesis would hopefully lead to the formulation of improved therapeutic strategies for this condition.
authors.affiliation: Department of Paediatrics, National University of Singapore, 5 Lower Kent Ridge Road, Singapore 119074.
authors.forename: S C
authors.initials: SC
authors.lastname: Quek
authors.forename: C L
authors.initials: CL
authors.lastname: Yip
authors.forename: M L
authors.initials: ML
authors.lastname: Wong
authors.forename: K Y
authors.initials: KY
authors.lastname: Chan
authors.forename: C L
authors.initials: CL
authors.lastname: Wong
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000503:physiopathology* / Q000628:therapy*; D008297:Male; D011379:Prognosis