Progression of cardiac amyloid deposition in hereditary transthyretin amyloidosis patients after liver transplantation
Progression of cardiac amyloid deposition in hereditary transthyretin amyloidosis patients after liver transplantation
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DOI：
10.1080/13506120701614032
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发表时间：
2007-12-01
影响因子：
5.5
通讯作者：
Benson, Merill D.
中科院分区：
文献类型：
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作者：
Liepnieks, Juris J.;Benson, Merill D.
据推测，甲状腺素运载蛋白（TTR）淀粉样变性可能是原位肝移植（奥尔特）后淀粉样纤维持续合成和沉积的结果。为了验证这一假设，从三名奥尔特后1年半至5年半死亡的患者的心脏组织中分离出淀粉样纤维：两名患者使用Val 30 Met，一名患者使用Thr 60 Ala TTR。在每种情况下，变异正常TTR的比例进行了测定，并与7例TTR淀粉样变性死亡，但没有肝移植患者的心脏组织中的变异正常的比例进行了比较。无奥尔特的TTR淀粉样变性患者的组织包括3例Val 30 Met，2例Thr 60 Ala，1例Delta Val 122和1例Val 122 Ile。来自非奥尔特患者的所有组织的变体TTR比正常TTR的量更大，除了Val 122 Ile之外，其比率为50：50。总体中位变异与正常比值为60：40，变异范围为50-70%。相比之下，奥尔特后患者的三种组织中变异TTR的平均百分比为25%（范围20-35）。这些数据与肝移植后心脏组织中正常TTR的持续沉积一致。
It has been hypothesized that transthyretin (TTR) amyloidosis may progress after orthotopic liver transplantation (OLT) as a result of continued amyloid fibril synthesis and deposition from normal TTR. To test this hypothesis amyloid fibrils were isolated from cardiac tissues of three patients who died 1 1/2 to 5 1/2 years after OLT: two with Val30Met and one with Thr60Ala TTR. The ratio of variant to normal TTR in each case was determined and compared with the ratio of variant to normal in cardiac tissues from seven patients who died with TTR amyloidosis but who had not had liver transplantation. Tissues from patients with TTR amyloidosis without OLT included three with Val30Met, two with Thr60Ala, one with Delta Val122, and one with Val122Ile. All tissues from patients without OLT had greater amounts of variant TTR than normal TTR except for the Val122Ile in which the ratio was 50:50. The overall median variant to normal ratio was 60:40 with a range of 50-70% variant. In contrast, the mean percentage of variant TTR in the three tissues from patients after OLT was 25% (range 20-35). These data are consistent with the continued deposition of normal TTR in cardiac tissue after liver transplantation.