转甲状腺素
心脏淀粉样变性
淀粉样变性
淀粉样多发性神经病
淀粉样蛋白(真菌学)
肝移植
医学
病理
多发性神经病
移植
病态的
内科学
疾病
发病年龄
作者
Masahide Yazaki,Takahiko Tokuda,Akihiro Nakamura,Takeo Higashikata,Jun Koyama,Kenichi Higuchi,Yasu Harihara,Satoshi Baba,Fuyuki Kametani,Shu‐ichi Ikeda
标识
DOI:10.1006/bbrc.2000.3203
摘要
Patients with familial amyloid polyneuropathy (FAP) are now cured by liver transplantation, but cardiac amyloidosis would further progress even after liver transplantation in some patients. To clarify the pathological mechanism of the progress of cardiac amyloidosis in FAP, we investigated cardiac tissues obtained from 6 FAP patients with 3 different types of TTR mutations. One of them had undergone liver transplantation and one year later died of cardiac amyloidosis. We determined clinical severity of cardiac involvement of those patients and characterized amyloid fibril proteins depositing in their cardiac muscles by immunohistochemistry, mass spectrometry and isoelectric focusing. All the patients had cardiac dysfunction and increased cardiac weight. Diffuse deposition of TTR-related amyloid was seen in their myocardium on microscopic examination. Amyloid fibrils of the heart were composed of wild-type TTR as well as variant TTR at a ratio of about 1:1 in 5 patients without liver transplantation. In the patient with a transplanted liver, about 80% of the cardiac amyloid consisted of wild-type TTR. Wild-type TTR contributes greatly to the development of amyloid deposition in the heart of FAP patients regardless of the types of TTR mutations.
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