家族性淀粉样变性多发性神经病(FAP)是一种常染色体显性遗传性疾病。在葡萄牙,它主要与转甲状腺素蛋白(TTR)突变有关,和存在长度依赖性感觉运动多发性神经病的患者,常伴有自主神经功能障碍。FAP的治疗选择包括肝移植,由于缺乏器官,在称为多米诺肝移植(DLT)的过程中,FAP肝脏开始植入患有严重肝病的患者体内。我们报道了一个68岁的葡萄牙男子的病例,丙型肝炎后肝硬化肝移植,他向家庭医生介绍了双脚和体重减轻的敏感性下降,最初归因于糖尿病神经病变和糖尿病药物的调整,分别。症状演变为双脚的热和疼痛敏感性的变化,双手灵敏度降低,腹泻,和逐步减肥。此时,患者接受DLT的披露提示医源性淀粉样多发性神经病的正确诊断.该病例强调了DLT后诊断和治疗医源性淀粉样多发性神经病的挑战,强调及时识别DLT接收者的重要性,通过结构化监测对这些患者的积极警惕,并提高医疗保健提供者对这种做法的认识,以便可以识别疾病的早期迹象。
Familial amyloidotic polyneuropathy (FAP) is an autosomal dominant hereditary disorder. In Portugal, it is mainly linked to transthyretin (TTR) mutation, and patients present with length-dependent sensory-motor polyneuropathy, often accompanied by autonomic dysfunction. Treatment options for FAP include liver transplant, and due to the lack of organs, FAP livers began being implanted in patients with severe liver disease in a process known as domino liver transplantation (DLT). We report a
case of a 68-year-old Portuguese man, with post-hepatitis C-related cirrhosis liver transplantation, who presented to his family doctor with decreased sensitivity in both feet and weight loss, which were initially attributed to diabetic neuropathy and an adjustment in diabetic medication, respectively. Symptoms evolved to changes in both feet\'s thermal and painful sensitivity, reduced sensitivity in both hands, diarrhea, and progressive weight loss. At this time, the patient\'s disclosure of receiving a DLT prompted the correct diagnosis of iatrogenic amyloid polyneuropathy. This
case underscores the challenges in diagnosing and managing iatrogenic amyloid polyneuropathy following DLT, highlighting the importance of prompt identification of DLT recipients, active vigilance of these patients via structured monitoring, and increased healthcare providers\' awareness of this practice so that early signs of the disease may be recognized.