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患有乳糜泻的儿童的肝移植

Liver transplantation in a child with celiac disease.

作者信息

Pavone Piero, Gruttadauria Salvatore, Leonardi Salvatore, Sorge Giovanni, Minervini Marta Ida, Greco Filippo, La Rosa Mario, Marino Ignazio

机构信息

Department of Pediatrics, University of Catania, Via Santa Sofia 78, 95123 Catania, Italy.

出版信息

J Gastroenterol Hepatol. 2005 Jun;20(6):956-60. doi: 10.1111/j.1440-1746.2005.03223.x.

Abstract

Recently, an atypical form of celiac disease (CD) has been identified, in which gastrointestinal symptoms are less pronounced. Other organs can be more or less severely affected, and the disease might be unrecognized and undiagnosed. In some cases, hypertransaminasemia has been indicated as the first symptom of CD in infancy. A direct relationship between liver damage and glutensensitivity has been proven by the disappearance of biochemical signs and histological lesions of the liver after the initiation of the gluten-free diet. The present case report is of a 14-year-old girl affected by CD and severe hepatic failure who underwent a liver transplant. To our knowledge, this case is the first report of liver involvement related to CD which is so severe as to require a liver transplant in a child. Because most patients with CD remain undiagnosed, and, as observed in the present report, untreated CD with subclinical hepatic involvement can lead to more serious liver disease, a more aggressive diagnostic work-up for CD in the general population is warranted.

摘要

最近,一种非典型的乳糜泻(CD)被发现,其胃肠道症状不太明显。其他器官可能或多或少受到严重影响,并且该疾病可能未被识别和诊断。在某些情况下,高转氨酶血症被认为是婴儿期CD的首发症状。无麸质饮食开始后,肝脏生化指标和组织学病变消失,证明了肝损伤与麸质敏感性之间存在直接关系。本病例报告的是一名14岁患有CD和严重肝衰竭的女孩,她接受了肝移植。据我们所知,该病例是与CD相关的肝脏受累严重到需要儿童进行肝移植的首例报告。由于大多数CD患者仍未被诊断出来,并且如本报告中所观察到的,未经治疗的伴有亚临床肝脏受累的CD可导致更严重的肝脏疾病,因此有必要对普通人群进行更积极的CD诊断检查。

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