Bion E, Brenard R, Pariente E A, Lebrec D, Degott C, Maitre F, Benhamou J P
Service d'Hépatogastroentérologie, Hôpital de la Source, Orléans, France.
Gut. 1991 Feb;32(2):227-30. doi: 10.1136/gut.32.2.227.
Hepatic venous catheterisation and transvenous liver biopsy were performed in five patients with hepatic amyloidosis. In three patients, hepatic venous pressures were normal and histological examination of the liver biopsy specimen showed discrete and sparse perisinusoidal amyloid deposits. In the other two, however, the gradient between wedged and free hepatic venous pressures was increased (12 and 16 mmHg; normal 1-4 mmHg) and amyloid deposits were abundant and diffuse in the Disse's space. This study shows that portal hypertension in patients with hepatic amyloidosis is of the sinusoidal type and is related to the reduction of vascular space of hepatic sinusoids by massive perisinusoidal amyloid deposits. Furthermore, portal hypertension is associated with a poor prognosis in patients with hepatic amyloidosis.
对5例肝淀粉样变性患者进行了肝静脉插管和经静脉肝活检。3例患者肝静脉压力正常，肝活检标本的组织学检查显示肝窦周围有离散且稀疏的淀粉样沉积物。然而，另外2例患者，肝静脉楔压与自由肝静脉压之间的梯度增加（分别为12和16 mmHg；正常为1 - 4 mmHg），狄氏间隙有大量弥漫性淀粉样沉积物。本研究表明，肝淀粉样变性患者的门静脉高压属于窦性类型，与大量肝窦周围淀粉样沉积物导致肝窦血管间隙减少有关。此外，门静脉高压与肝淀粉样变性患者的预后不良相关。
