Skalski Michal, Kornasiewicz Oskar, Raszeja-Wyszomirska Joanna, Konieczka Agata, Mlynarczyk Monika, Grat Michal
Department of General, Transplant and Liver Surgery, Public Central Teaching Hospital, Medical University of Warsaw, 1A Banacha St., 02-097 Warsaw, Poland.
J Clin Med. 2024 Sep 25;13(19):5702. doi: 10.3390/jcm13195702.
Data regarding the outcomes of liver transplantation in disabled, highly dependent, and legally incapacitated adults are scarce, likely due to the infrequency of these procedures in such populations. Multicenter studies in adult transplant centers have shown that patients with coexisting intellectual and developmental disabilities (IDDs) may be denied transplantation because of their expected low longevity and the complexities associated with managing post-transplant care. We examined the long-term patient and graft outcomes in highly dependent, incapacitated patients with IDDs who underwent elective transplantation for chronic liver disease. Six adult patients who underwent liver transplantation for primary biliary cholangitis ( = 2), hepatitis C cirrhosis ( = 2), Wilson's disease ( = 1), and autoimmune hepatitis ( = 1) were included. The main causes of their disability were infantile cerebral palsy, myotonia, and Niemann-Pick disease. Four of the six patients were women, with a median age of 26 (range: 23-36) years. Only one patient died during follow-up. Their 1- and 5-year survival rates were 100 and 75%, respectively, which were not statistically different from those of the general cohort of electively transplanted patients (95.8 and 90.1%, respectively) ( = 0.35). Adult patients who are highly dependent, disabled, or legally incapable should not be denied liver transplantation because of poor long-term survival rates. Physiological disorders and psychiatric comorbidities should not prevent patients from receiving life-saving surgeries due to poor postoperative compliance or low quality of life.
关于残疾、高度依赖他人且无法律行为能力的成年人肝移植结果的数据很少，这可能是由于这类人群中进行此类手术的频率较低。成人移植中心的多中心研究表明，患有智力和发育障碍（IDD）的患者可能会因为预期寿命短以及移植后护理管理的复杂性而被拒绝移植。我们研究了因慢性肝病接受择期移植的高度依赖他人、无行为能力的IDD患者的长期患者和移植物结果。纳入了6例因原发性胆汁性胆管炎（n = 2）、丙型肝炎肝硬化（n = 2）、威尔逊病（n = 1）和自身免疫性肝炎（n = 1）接受肝移植的成年患者。他们残疾的主要原因是婴儿期脑瘫、肌强直和尼曼-匹克病。6例患者中有4例为女性，中位年龄为26岁（范围：23 - 36岁）。随访期间仅1例患者死亡。他们的1年和5年生存率分别为100%和75%，与择期移植患者的总体队列（分别为95.8%和90.1%）相比，差异无统计学意义（P = 0.35）。高度依赖他人、残疾或无法律行为能力的成年患者不应因长期生存率低而被拒绝肝移植。生理障碍和精神合并症不应因术后依从性差或生活质量低而阻止患者接受挽救生命的手术。
