Department of Neurosciences, Centro Hospitalar Lisboa Norte, Hospital de Santa Maria, Lisbon, Portugal.
Muscle Nerve. 2010 Nov;42(5):836-9. doi: 10.1002/mus.21806.
Familial amyloid polyneuropathy (FAP) is a progressive neuropathy with autonomic dysfunction. Domino liver transplantation (DLT), in which the liver of an FAP patient is transplanted into another patient, is routinely applied to compensate for the shortage of available organs. We report a patient who developed a clinical picture of FAP 9 years after a DLT from an FAP donor. Electrophysiological, neuropathological, and autonomic tests were administered. The patient presented with typical clinical features of FAP. Electrophysiological investigation confirmed a moderate sensorimotor axonal and autonomic neuropathy. Sural nerve biopsy confirmed the presence of amyloid deposits in the endoneurium. Skin biopsy at the ankle showed reduced intraepidermal nerve fiber density. Our report shows that FAP can develop in a recipient of an FAP liver. This suggests that careful longitudinal study is required to evaluate the risk of FAP polyneuropathy in patients who undergo domino liver transplantation.
家族性淀粉样多神经病（FAP）是一种进行性神经病，伴有自主功能障碍。多米诺肝移植（DLT），即将 FAP 患者的肝脏移植到另一名患者体内，是常规用于弥补可用器官短缺的方法。我们报告了一例患者，该患者在接受 FAP 供体的 DLT 后 9 年出现了 FAP 的临床表现。进行了电生理学、神经病理学和自主功能测试。患者表现出典型的 FAP 临床特征。电生理学研究证实存在中度感觉运动轴索性和自主神经病。腓肠神经活检证实神经内膜存在淀粉样沉积物。踝关节皮肤活检显示表皮内神经纤维密度降低。我们的报告表明，FAP 可在 FAP 肝脏受者中发生。这表明需要进行仔细的纵向研究，以评估接受多米诺肝移植的患者发生 FAP 多发性神经病的风险。
