Zeijen R N, Sels J P, Flendrig J A, Arends J W
Department of Internal Medicine, University Hospital Maastricht, The Netherlands.
Neth J Med. 1991 Jun;38(5-6):257-61.
Despite the fact that hepatic involvement is frequently seen in systemic amyloidosis, major clinical symptoms or impaired hepatic functional capacity are rare. We describe a patient with primary hepatic amyloidosis, severe intrahepatic cholestasis and portal hypertension, a combination previously reported only three times in the literature. In case of an unexplained intrahepatic cholestasis or portal hypertension the possibility of amyloidosis should be considered and a Congo red staining should be performed.
尽管肝受累在系统性淀粉样变性中很常见，但主要临床症状或肝功能受损却很少见。我们描述了一名原发性肝淀粉样变性患者，伴有严重的肝内胆汁淤积和门静脉高压，这种组合在文献中此前仅报道过三次。对于不明原因的肝内胆汁淤积或门静脉高压，应考虑淀粉样变性的可能性，并进行刚果红染色。
