Maggiore Giuseppe, Caprai Silvia
Department of Reproductive Medicine and Child Development University of Pisa, Gastroenterology and Liver Unit and IsMeTT, University of Pittsburgh Medical Center, Palermo, Italy.
Indian J Pediatr. 2006 Sep;73(9):809-11. doi: 10.1007/BF02790391.
Celiac disease may present as a cryptogenic liver disorder being found in 5-10 % of patients with a persistent and cryptogenetic elevation of serum aminotransferase activity. In fact, a wide spectrum of liver injuries in children and adults may be related to CD and in particular: (1) a mild parenchymal damage characterised by absence of any clinical sign or symptom suggesting a chronic liver disease and by non-specific histological changes reversible on a gluten-free diet; (2) a chronic inflammatory liver injury of autoimmune mechanism, including autoimmune hepatitis, primary sclerosing cholangitis and primary biliary cirrhosis, that may lead to fibrosis and cirrhosis, generally unaffected by gluten withdrawal and necessitating an immunosuppressive treatment; (3) a severe liver failure potentially treatable by a gluten-free diet. Such different types of liver injuries may represent a spectrum of a same disorder where individual factors, such as genetic predisposition, precocity and duration of exposure to gluten may influence the reversibility of liver damage. A rigorous cross-checking for a asymptomatic liver damage in CD individuals and conversely, for CD in any cryptogenic liver disorder including end-stage liver failure is recommended.
乳糜泻可能表现为一种隐源性肝脏疾病,在血清转氨酶活性持续且原因不明升高的患者中,有5% - 10%可发现该病。事实上,儿童和成人中广泛的肝脏损伤可能与乳糜泻相关,尤其是:(1)轻度实质损害,其特征是没有任何提示慢性肝病的临床症状或体征,且组织学改变不具特异性,在无麸质饮食后可逆转;(2)自身免疫机制引起的慢性炎症性肝损伤,包括自身免疫性肝炎、原发性硬化性胆管炎和原发性胆汁性肝硬化,可能导致纤维化和肝硬化,通常不受撤麸质饮食影响,需要进行免疫抑制治疗;(3)严重肝衰竭,可能通过无麸质饮食治疗。这些不同类型的肝损伤可能代表同一疾病的不同表现,个体因素,如遗传易感性、麸质暴露的早熟程度和持续时间,可能影响肝损伤的可逆性。建议对乳糜泻患者进行无症状肝损伤的严格筛查,反之,对任何隐源性肝脏疾病(包括终末期肝衰竭)患者进行乳糜泻筛查。