先天性胆道閉鎖症の肺病変

概要

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The children with congenital biliary atresia (CBA) tend to develop pulmonary complications postoperatively, e. g. pneumonitis and reccurrent atelectasis. Their management was often very difficult. Even when diseases free, their chest X-ray films show diffuse parenchyma! changes of the lungs. Alteration of the pulmonary structure and on pulmonary surfactant were studied in 18 CBA autopsy cases. The most characterisitc morphologic changes of the lungs included thickening of the interalveolar septa, increase in the cellularity of alveolar walls and formation of the multiple A-V fistula. With electron microscopy, the most remarked findings were swelling and increased vacuolization of the endothelial celles, increase in the microthrombi in the pulmonary capillaries, prolifiration of the type II alveolar pneumocytes and decrease in the number of the type I pneumocytes. As for the surfactant, the amount of disaturated phosphatidyl choline increased in the lung in CBA in comparison with controls. These changes were confirmed in the experimental study. The Wister rats with ligated common bile duct developed progressive hepatic and pulmonary changes. The hepatic change consisted of generalized fibrosis and bile duct proliferation. The pulmonary changes were exactly same as seen in human CBA.
日本小児外科学会の論文
1982-08-20