a Record control number WOS:000307744000012

a ISSN 1590-8658

a Original cataloging agency NEU GrandLibrary

a Language code of text/sound track or separate title eng

a Classification number WI 140

b Item number .L58 2012

a Personal name Unal, Fatih.

h Medium Electronic Document

a Title Liver involvement in children with Familial Mediterranean fever

c Statement of responsibility, etc Fatih Unal, Murat Cakir, Masallah Baran, Cigdem Arikan, Hasan Ali Yuksekkaya, Sema Aydogdu.

a Place of publication, distribution, etc Elsevier Science Inc.,

c Date of publication, distribution, etc 2012.

a Summary, etc Aim: Familial Mediterranean fever is characterised by recurrent, febrile, inflammatory attacks of the serosal membranes. Prolonged inflammatory response is triggered secondary to cytokine stimulation due to reduced activity of pyrin. Inflammatory cytokines play major role in the pathogenesis of acute liver injury; and chronic, recurrent cytokine production may cause chronic hepatitis/cirrhosis. We aimed to analyse liver involvement in children with Familial Mediterranean fever. Patients: The study included 58 patients with Familial Mediterranean fever. Patients with liver involvement were examined in detail.Results: Liver involvement was seen in 11 of 58 patients (18.9%). Two patients (3.4%) had abnormal liver enzymes during the diagnostic evaluation, whilst 9 patients (15.5%) were admitted with the features of liver diseases, and had final diagnosis of Familial Mediterranean fever (2 had Budd-Chiari syndrome, Shad chronic hepatitis/cirrhosis, 2 had acute hepatitis). None of the demographic factors or laboratory findings was different between the patients with or without liver involvement M694V allele was more common in patients with liver involvement but did not reach significant difference (50% vs. 33.6%, p = 0.21). All the patients showed clinical and laboratory improvement after colchicine.Conclusion: Paediatric hepatologists must keep Familial Mediterranean fever in mind in the patients with cryptogenic hepatitis/cirrhosis especially in regions where hereditary inflammatory diseases are common. Crown Copyright (C) 2012 Published by Elsevier Ltd on behalf of Editrice Gastroenterologica Italiana S.r.l. All rights reserved.

a Topical term or geographic name as entry element Gastroenterology & Hepatology

a Topical term or geographic name as entry element Near East University Article

a Topical term or geographic name as entry element Yakın Doğu Üniversitesi Makale

a Topical term or geographic name as entry element Children

a Topical term or geographic name as entry element Familial Mediterranean Fever

a Topical term or geographic name as entry element Liver involvement

g Relationship information AUG 2012, Vol 44 Issue 8, p689-693

x International Standard Serial Number 15908658

t Title Digestive And Liver Disease

u Uniform Resource Identifier http://library.neu.edu.tr:2048/login?url=http://dx.doi.org/10.1016/j.dld.2012.01.003