• Ann Acad Med Singap · Mar 2001

    Review

    A case series of six children with primary pulmonary hypertension.

    • S C Quek, C L Yip, M L Wong, K Y Chan, and C L Wong.
    • Department of Paediatrics, National University of Singapore, 5 Lower Kent Ridge Road, Singapore 119074.
    • Ann Acad Med Singap. 2001 Mar 1;30(2):118-21.

    IntroductionPrimary pulmonary hypertension is an uncommon but serious disease in children. Management is difficult despite recent advances in pharmacotherapy.Clinical PictureWe reviewed patients with this condition with respect to their presenting symptoms, investigations, treatment and outcome.TreatmentThese children were treated with individualised combinations of oxygen, diuretics and calcium antagonists.OutcomeIn our follow-up of 5 children, 4 had died.ConclusionThis 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.

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