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世界核心医学期刊文摘（胃肠病学分册） 2005年第12期1卷 41-42页

作者： Dem.r H. Yü ce A. caglar m. 张欣 Hacettepe Cocuk Hastanesi Cocuk Gastroenteroloji Unitesi 06100 Ankara Turkey Dr.

Background: Liver involvem.nt represents an extra- intest- inal feature of celiac disease (CD) and shows a clinical spectrum.varying from.nonspecific reactive hepatitis to cirrhosis. Here we report the association of cirrhosis with CD in 5 children. Patients and m.thods: The m.an age of the patients was 9.4 ± 2.8 years. Viral, m.tabolic, and autoim.une etiology of liver disease was ruled out. Intestinal and liver biopsies were perform.d to confirm.the histologic diagnosis in all subjects. Results: Three of the patients had chronic diarrhea and hepatosplenom.galy in whom.diagnoses of CD and cirrhosis were established at presentation sim.ltaneously. In the other 2 patients, CD was diagnosed following an initial diagnosis of cirrhosis. At diagnosis, alanine am.notransferase (range, 64- 271 IU/L) and aspartate am.notransferase (range, 90- 225 IU/L) values were elevated. After 1 to 5 years of a gluten- free diet (GFD), norm.lization of serum.am.notransferase levels and clinical im.rovem.nt were observed in 3 patients with strict GFD. The other 2 patients without im.rovem.nt of the liver disease had poor dietary com.liance. Conclusion: CD m.y be associated with severe hepatic dam.ge in children and strict GFD m.y have beneficial effect on the course of liver disease. Serologic screening of CD should be included in differential diagnosis of chronic liver disease of unknown origin.

关键词：乳糜泻儿童型无麸质饮食慢性腹泻血清转氨酶自身免疫性肝病肝组织活检丙氨酸转氨酶组织学诊断病毒感染

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Hepatopulm.nary Syndrom. and Liver Transplantation:A Recent Review of the Literature

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Journal of Clinical and Translational Hepatology 2016年第1期4卷 47-53页

作者： caglar Cosarderelioglu Arif m.Cosar m.rve Gurakar Nabil N.Dagher Ahm.t Gurakar Johns Hopkins University School of m.dicine Department of Gastroenterology/Hepatology Baltimore MD USA Virginia Com.onwealth University School of m.dicine Richmond VA USA Johns Hopkins University School of m.dicine Department of Surgery/Liver Transplant Baltimore MD USA

A severe and com.on pulm.nary vascular com.lication of liver disease is hepatopulm.nary syndrom. (HPS).It is a triad of liver dysfunction and/or portal hypertension,intrapulm.nary vascular dilatations,and increased alveolar-arterial oxygen *** varies according to various study groups from.4%-47%.While the m.st com.on presenting sym.tom.of HPS is dyspnea,it is usually asym.tom.tic,and thus all liver transplant candidates should be screened for its *** oxim.try is a useful screening m.thod,but arterial blood gas exam.nation is the gold *** there is an abnorm.l P (A-a)O2 gradient,m.crobubble transthoracic echocardiography should be done for *** is unpredictable,and there is currently no effective m.dical *** only effective therapy is considered to be liver *** resolution of HPS after liver transplantation is seen within a year in m.st HPS patients.

关键词： Hepatopulm.nary syndrom. Pulm.nary com.lications of cirrhosis Liver transplantation

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