Annals of the Academy of Medicine, Singapore · 2001
A case series of six children with primary pulmonary hypertension.
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Quek S C, Yip C L, Wong M L, Chan K Y, Wong C L
Аннотация
INTRODUCTION: Primary pulmonary hypertension is an uncommon but serious disease in children. Management is difficult despite recent advances in pharmacotherapy.
CLINICAL PICTURE: We reviewed patients with this condition with respect to their presenting symptoms, investigations, treatment and outcome.
TREATMENT: These children were treated with individualised combinations of oxygen, diuretics and calcium antagonists.
OUTCOME: In our follow-up of 5 children, 4 had died.
CONCLUSION: 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.
CLINICAL PICTURE: We reviewed patients with this condition with respect to their presenting symptoms, investigations, treatment and outcome.
TREATMENT: These children were treated with individualised combinations of oxygen, diuretics and calcium antagonists.
OUTCOME: In our follow-up of 5 children, 4 had died.
CONCLUSION: 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.