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Familial pulmonary veno-occlusive disease: a case report.
  1. C G Voordes,
  2. J R Kuipers,
  3. J D Elema


    A case of pulmonary veno-occlusive disease in a male infant who died at the age of 3 months is presented. Both intra- and extrapulmonary veins were involved. Two years earlier a brother had died of the same disease at the age of 8 weeks, but, in that case, the disease was restricted to the intrapulmonary veins. It is suggested that the disease may have been caused by a viral infection, the mother acting as a carrier. The simultaneous occurrence of intra- and extraparenchymal pulmonary vein occlusion indicates that some instances of isolated extraparenchymal pulmonary vein atresia or obstruction may also have been examples of pulmonary veno-occlusive disease.

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