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KMID : 0882419930450030400
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Korean Journal of Medicine
1993 Volume.45 No. 3 p.400 ~ p.406
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A Case of Pulmonary Veno-occlusive Disease in Primary Pulmonary Hemoglobinuria
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서홍석
강경호/김혜경/임도선/박창규/김영훈/심완주/오동주/박정의 노영무
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Abstract
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rimary pulmonary hypertension has been subdivided into several subsets, including plexogenic, theromoembolic, pulmonary veno-occlusive disease, and others. Pulmonary veno-occlusive disease (PVOD) is one of the least cause of vlinical pulmonary
hypertension,
with approximately 100 patients reported in would medical literature, and no case reported in
Korea.
Here we report on a 35-year-old woman had primary pulmonary hypertension presenting
with massive hemoptysis and secondary atelectasis.Her simple chest x-ray, C-T scan,
pulmonary angiogram and cardiac catheterization findings were compatible with typical
primary pulmonary hypertension except elevated pulmonary capillary wedge pressure up to 26
mmHg. That was pathognomonic feature of pulmonary venoocclusive disease subset of
primary pulmonary hypertension. The laboratory findings were compatible with primary
pulmonary hypertension and could rule out the econdary pulmonary hypertension and other
cardiac diseases. Left lingular segment atelectasis due to massive hemoptysis clots was
resolved by removal of large blood clots by bronchoscopy. Her pulmonary artery pressure
was not response to the vasodilator trials including nifedipine and captopril.
We report a case of PVOD with a review of the literature.
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KEYWORD
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