Case report demonstrates the fatal course of pulmonary veno-occlusive disease in a pediatric patient, suggesting lung transplantation offers the best survival outcome.
We describe the observation of a 12 yr old girl who died of pulmonary veno-occlusive disease (PVOD). Diagnosis was based on histological examination of an open lung biopsy. The differential diagnosis, pathogenesis and possible therapies are discussed. Although medical therapy can sometimes give some temporary relief, lung transplantation might offer these patients a better chance of survival and a better quality of life.
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Vries et al. (1991) studied this question.
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