Oguchi Kenya, Takei Yo-Ichi, Ikeda Shu-Ichi
Third Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan.
Amyloid. 2006 Jun;13(2):99-107. doi: 10.1080/13506120600722662.
Liver transplantation for familial amyloid polyneuropathy (FAP) patients has been carried out worldwide and the outcomes seem to be promising. To clarify the severity of amyloid deposits on visceral organs, we evaluated the histopathological findings of biopsied renal and sural nerve specimens in 13 FAP patients with ATTR Val30Met by quantitative analysis, and compared them with the outcome of transplantation. Renal dysfunction with proteinuria seemed to correlate with the degree of amyloid deposits in glomeruli, not with that in medullary tissues. The severity of renal amyloid deposition did not consistently parallel that of myelinated nerve fiber loss in sural nerve. Three patients with proteinuria and severe amyloid deposits in glomeruli were considered to be unsuitable for transplantation. Ten patients underwent living donor liver transplantation and three resulted in unfavorable outcomes. These three had heavy amyloid deposits on renal tissues, especially in glomerular areas, but the severity of myelinated nerve fiber loss in their sural nerves was very similar to that in patients who made a good recovery. The prognosis after operation might be closely related to the severity of amyloid deposits in renal glomeruli. Renal biopsy is, therefore, recommended when determining the indications and contraindications for liver transplantation in FAP patients, although this biopsy is not routinely required.
家族性淀粉样多神经病（FAP）患者的肝移植已在全球范围内开展，且结果似乎很有前景。为了明确内脏器官中淀粉样沉积物的严重程度，我们通过定量分析评估了13例携带ATTR Val30Met突变的FAP患者肾活检和腓肠神经活检标本的组织病理学结果，并将其与移植结果进行比较。伴有蛋白尿的肾功能不全似乎与肾小球中淀粉样沉积物的程度相关，而非与髓质组织中的程度相关。肾淀粉样沉积的严重程度与腓肠神经中有髓神经纤维丢失的严重程度并不始终平行。3例有蛋白尿且肾小球中淀粉样沉积物严重的患者被认为不适合进行移植。10例患者接受了活体供肝移植，3例结果不佳。这3例患者肾组织尤其是肾小球区域有大量淀粉样沉积物，但其腓肠神经中有髓神经纤维丢失的严重程度与恢复良好的患者非常相似。术后预后可能与肾小球中淀粉样沉积物的严重程度密切相关。因此，在确定FAP患者肝移植的适应证和禁忌证时，建议进行肾活检，尽管并非常规需要进行此项活检。
