Department of Gastroenterology and Hepatology, University Medical Center Groningen, University of Groningen, The Netherlands.
Amyloid. 2009;16(3):133-41. doi: 10.1080/13506120903090726.
Liver transplantation aims to halt the progression of the disease in patients with familial amyloidotic polyneuropathy (FAP) caused by hereditary transthyretin-related (ATTR) amyloidosis. Insight in health-related quality of life of these transplanted FAP-patients can be of help to optimize health care delivery. The aim of this cross-sectional study was to assess the health-related quality of life of patients with FAP long-term after transplantation. Nine patients with a post-transplant follow-up of 4 years or more were included in the study. During the annual checks, health-related quality of life was measured with the Short Form-36 (SF-36). Data were compared with non-FAP transplanted patients with the same duration of follow-up and with the normal Dutch population. Pre-transplant, all patients had signs of mild to moderate peripheral polyneuropathy. The results showed that in patients with FAP health-related quality of life was stable in the first 4 years after transplantation. The domain of physical well-being at 4 years after transplantation was significantly lower compared to non-FAP transplanted patients and control Dutch population. The domain of emotional well-being was comparable with non-FAP controls. However, on most health areas patients with FAP scored lower than the non-FAP transplanted patients and the Dutch controls. After four years, the three patients with FAP with longest follow-up (9-12 years) deteriorated in all health domains, except in self-perceived mental health. This study, including only a small number of patients with FAP, shows a relatively low health-related quality of life after liver transplantation, which may deteriorate further with longer follow-up.
肝移植旨在阻止遗传性转甲状腺素蛋白相关（ATTR）淀粉样变性引起的家族性淀粉样多神经病（FAP）患者疾病的进展。了解这些接受肝移植的 FAP 患者的健康相关生活质量有助于优化医疗保健服务。本横断面研究的目的是评估接受肝移植后 FAP 患者的健康相关生活质量。该研究纳入了 9 名接受肝移植后随访 4 年或以上的 FAP 患者。在每年的检查中，使用 36 项简短健康调查问卷（SF-36）测量健康相关生活质量。将数据与具有相同随访时间的非 FAP 移植患者和正常荷兰人群进行比较。移植前，所有患者均有轻度至中度周围神经病的迹象。结果表明，在接受肝移植后的前 4 年内，FAP 患者的健康相关生活质量保持稳定。移植后 4 年时，身体状况领域的得分明显低于非 FAP 移植患者和荷兰对照组。情感健康领域的得分与非 FAP 对照组相当。然而，在大多数健康领域，FAP 患者的得分均低于非 FAP 移植患者和荷兰对照组。4 年后，3 名随访时间最长（9-12 年）的 FAP 患者在所有健康领域的状况都恶化了，除了自我感知的心理健康。本研究纳入的 FAP 患者数量较少，表明肝移植后健康相关生活质量相对较低，随着随访时间的延长，可能会进一步恶化。
