Faust Dominik, Akoglu Bora, Ristic Gordana, Milovic Vladan
Asklepios Hospital, Department of Medicine, Langen, Germany.
Vojnosanit Pregl. 2009 Jun;66(6):482-6. doi: 10.2298/vsp0906482f.
Amyloidosis represents a group of different diseases characterized by extracellular accumulation of pathologic fibrillar proteins in various tissues and organs. Severe amyloid deposition in the liver parenchyma has extrahepatic involvement predominantly in the kidney or heart. We evaluated the effect of ursodeoxycholic acid, in four patients with severe hepatic amyloidosis of different etiologies, who presented with increased alkaline phosphatase and gamma-glutamyl transferase.
The study included four patients who presented with amyloidosis-associated intrahepatic cholestasis. Three of them had renal amyloidosis which developed 1-3 years before cholestasis occurred, the remaining one having intrahepatic cholestasis as the primary sign of the disease. Amyloidosis was identified from liver biopsies in all patients by its specific binding to Congo red and green birefringence in polarized light. The biochemical nature and the class of amyloid deposits were identified immunohistochemically. In addition to their regular treatment, the patients received 750 mg ursodeoxycholic acid per day. After 2-4 weeks all patients had a significant decrease of serum alkaline phosphatase and gamma-glutamyl transferase, and their general status significantly improved.
Treatment with ursodeoxycholic acid may be beneficial in patients with hepatic amyloidosis, and do extend indications for the use of ursodeoxycholic acid in amyloidotic cholestatic liver disease.
淀粉样变性是一组不同的疾病，其特征是病理纤维蛋白在各种组织和器官中细胞外积聚。肝实质中严重的淀粉样沉积主要累及肾脏或心脏等肝外器官。我们评估了熊去氧胆酸对4例不同病因的严重肝淀粉样变性患者的疗效，这些患者的碱性磷酸酶和γ-谷氨酰转移酶升高。
该研究纳入了4例表现为淀粉样变性相关肝内胆汁淤积的患者。其中3例在胆汁淤积发生前1 - 3年出现肾淀粉样变性，其余1例以肝内胆汁淤积为疾病的主要表现。所有患者均通过肝活检组织与刚果红的特异性结合及偏振光下的绿色双折射鉴定出淀粉样变性。通过免疫组织化学鉴定淀粉样沉积物的生化性质和类别。除常规治疗外，患者每天接受750 mg熊去氧胆酸治疗。2 - 4周后，所有患者的血清碱性磷酸酶和γ-谷氨酰转移酶均显著下降，全身状况明显改善。
熊去氧胆酸治疗可能对肝淀粉样变性患者有益，并确实扩展了熊去氧胆酸在淀粉样变性胆汁淤积性肝病中的应用指征。
