Marina Guitton Rodrigues , Paula Marcela Vilela Castro , Tiago Careli de Almeida , Bruno Carrijo Cunha , Fernanda Ribeiro Danziere , Francisco Antonio Sergi Filho , Beimar Edmundo Zeballos Sempertegui , Juan Rafel Branez , Leonardo Toledo Mota , Marcelo Perosa de Miranda , Regina Gomes dos Santos , e Tércio Genzini
{"title":"Case report: Liver Transplantation in a highly dependent Down Syndrome patient","authors":"Marina Guitton Rodrigues , Paula Marcela Vilela Castro , Tiago Careli de Almeida , Bruno Carrijo Cunha , Fernanda Ribeiro Danziere , Francisco Antonio Sergi Filho , Beimar Edmundo Zeballos Sempertegui , Juan Rafel Branez , Leonardo Toledo Mota , Marcelo Perosa de Miranda , Regina Gomes dos Santos , e Tércio Genzini","doi":"10.1016/j.tpr.2020.100054","DOIUrl":null,"url":null,"abstract":"<div><p>Despite the malformations commonly associated with Down Syndrome, the average life expectancy of individuals nowadays may reach 60-70 years of age. With these increased survival rates, the spectrum of diseases reported in these patients has been growing, particularly autoimmune diseases. This report describes a 44-year-old patient with Down Syndrome with a high degree of family dependence and end-stage liver disease (MELD-Na 38) caused by overlapping Primary Sclerosing Cholangitis and Autoimmune Hepatitis, who needed Liver Transplantation. Here, we point out two relevant aspects: the overlap of rare autoimmune diseases in patients with Trisomy 21 and the performance of Liver Transplantation in these patients. We then highlight the ethical dilemma between organ scarcity and the use of the graft in patients with neurocognitive disorders.</p></div>","PeriodicalId":37786,"journal":{"name":"Transplantation Reports","volume":"5 3","pages":"Article 100054"},"PeriodicalIF":0.0000,"publicationDate":"2020-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.tpr.2020.100054","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Transplantation Reports","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2451959620300160","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
Abstract
Despite the malformations commonly associated with Down Syndrome, the average life expectancy of individuals nowadays may reach 60-70 years of age. With these increased survival rates, the spectrum of diseases reported in these patients has been growing, particularly autoimmune diseases. This report describes a 44-year-old patient with Down Syndrome with a high degree of family dependence and end-stage liver disease (MELD-Na 38) caused by overlapping Primary Sclerosing Cholangitis and Autoimmune Hepatitis, who needed Liver Transplantation. Here, we point out two relevant aspects: the overlap of rare autoimmune diseases in patients with Trisomy 21 and the performance of Liver Transplantation in these patients. We then highlight the ethical dilemma between organ scarcity and the use of the graft in patients with neurocognitive disorders.
期刊介绍:
To provide to national and regional audiences experiences unique to them or confirming of broader concepts originating in large controlled trials. All aspects of organ, tissue and cell transplantation clinically and experimentally. Transplantation Reports will provide in-depth representation of emerging preclinical, impactful and clinical experiences. -Original basic or clinical science articles that represent initial limited experiences as preliminary reports. -Clinical trials of therapies previously well documented in large trials but now tested in limited, special, ethnic or clinically unique patient populations. -Case studies that confirm prior reports but have occurred in patients displaying unique clinical characteristics such as ethnicities or rarely associated co-morbidities. Transplantation Reports offers these benefits: -Fast and fair peer review -Rapid, article-based publication -Unrivalled visibility and exposure for your research -Immediate, free and permanent access to your paper on Science Direct -Immediately citable using the article DOI