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结节病中的危及生命情况。

Life-threatening situations in sarcoidosis.

作者信息

James D G

机构信息

Royal Free Hospital, London, U.K.

出版信息

Sarcoidosis Vasc Diffuse Lung Dis. 1998 Sep;15(2):134-9.

PMID:9789890
Abstract

Life-threatening situations in sarcoidosis are extremely rare. They may be due to failure of vital organs--lungs, heart, kidney, liver and brain--and usually due to irreversible fibrosis. Respiratory failure follows irreversible pulmonary fibrosis and the development of cor pulmonale. Cardiac sarcoidosis is more sinister for it may be silent, ill-recognised with sudden death or high morbidity. It needs sophisticated techniques to uncover this latent iceberg. Renal failure may be due to granulomatous interstitial nephritis and/or nephrocalcinosis. Hepatic failure is due to intrahepatic cholestasis, portal hypertension and bleeding oesophageal varices. Neurosarcoidosis carries a mortality of 10 per cent, over twice that of sarcoidosis overall. The treatment of each situation is discussed including organ transplantation.

摘要

结节病中危及生命的情况极为罕见。它们可能是由于重要器官——肺、心脏、肾脏、肝脏和大脑——功能衰竭引起的,通常是由于不可逆的纤维化。呼吸衰竭继发于不可逆的肺纤维化和肺心病的发展。心脏结节病更为凶险,因为它可能没有症状,难以识别,可导致猝死或高发病率。需要复杂的技术来发现这座潜在的冰山。肾衰竭可能是由于肉芽肿性间质性肾炎和/或肾钙质沉着症。肝功能衰竭是由于肝内胆汁淤积、门静脉高压和食管静脉曲张出血。神经结节病的死亡率为10%,是结节病总体死亡率的两倍多。文中讨论了每种情况的治疗方法,包括器官移植。

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