Pulmonary veno-occlusive disease in siblings: Case reports and morphometric study

摘要: Two cases of fatal veno-occlusive disease are described: the first in a boy 14 years old and second his sister, who died, at age 13, only few months later. In both, symptom was effort-induced dyspnea course rapid. The chest radiograph showed diffuse opacities, whereas that girl Kerley lines bilaterally. autopsy, diagnosis severe confirmed, with partial or complete occlusion much pulmonary venous bed by organized thrombi. vascular system processed for light microscopic morphometry injection radiopaque medium into veins one lung arteries other. Although there little filling system, arterial filled down to capillary level. Pre-acinar intra-acinar medial hypertrophy extension smooth muscle precapillary arteries. concentration patent relative alveoli not significantly less than normal, strikingly reduced. Possible pathogenetic mechanisms discussed.