Department of Surgery, Kumamoto Rosai Hospital, Kumamoto, Japan.
Department of Morphological and Physiological Sciences, Graduate School of Health Sciences, Kumamoto University, Chuo-ku, Kumamoto-shi, Kumamoto, Japan.
Transplant Proc. 2021 May;53(4):1313-1316. doi: 10.1016/j.transproceed.2021.03.001. Epub 2021 Apr 6.
Carpal tunnel syndrome is the most common compression syndrome of the peripheral nerve. Transthyretin amyloidosis and dialysis-related β2-microglobulin amyloidosis are known causes of carpal tunnel syndrome.
A Japanese woman showed carpal tunnel syndrome 16 years after a domino liver transplantation (DLT) from the donor with hereditary transthyretin amyloidosis. DLT indication was congenital extrahepatic portosystemic shunt, and the patient had been put on maintenance hemodialysis because of chronic kidney disease 6 years before DLT. Moreover, the amyloid precursor protein of the patient was histologically confirmed not to be β2-microglobulin, but transthyretin.
The existence of amyloid was speculated when the patient who underwent DLT from hereditary transthyretin amyloidosis showed carpal tunnel syndrome. Additionally, elucidating the amyloid precursor protein when the patient has another cause of amyloidosis is necessary.
腕管综合征是周围神经最常见的压迫综合征。转甲状腺素淀粉样变性和透析相关β2-微球蛋白淀粉样变性是腕管综合征的已知病因。
一名日本女性在接受遗传性转甲状腺素淀粉样变性供体的多米诺肝移植（DLT） 16 年后出现腕管综合征。DLT 指征为先天性肝外门体分流，患者在 DLT 前 6 年因慢性肾脏病接受维持性血液透析。此外，患者的淀粉样前体蛋白经组织学证实不是β2-微球蛋白，而是转甲状腺素。
当接受遗传性转甲状腺素淀粉样变性的 DLT 患者出现腕管综合征时，推测存在淀粉样物质。此外，当患者有其他淀粉样变性的病因时，阐明淀粉样前体蛋白是必要的。
