Liver involvement in children with Familial Mediterranean fever

Digestive and Liver Disease : Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver
Fatih UnalSema Aydoğdu

Abstract

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. The study included 58 patients with Familial Mediterranean fever. Patients with liver involvement were examined in detail. 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, 5 had 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 ...Continue Reading

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Citations

Mar 13, 2015·Clinical Rheumatology·Donato RiganteLuca Cantarini
May 15, 2015·Thrombosis Journal·Micaela La ReginaRaffaele Manna
Jun 26, 2013·Digestive and Liver Disease : Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver·Thomas AparicioPauline Afchain
Jan 30, 2015·Prague Medical Report·Flora TzifiAchilleas Attilakos
Feb 9, 2013·Expert Review of Clinical Immunology·Susie W Ng, Uma Mahadevan
Nov 5, 2016·Annals of the Rheumatic Diseases·Nienke M Ter HaarJoost Frenkel
Mar 21, 2020·Liver International : Official Journal of the International Association for the Study of the Liver·Thibault FraisseStéphanie Ducharme-Bénard
Sep 20, 2019·Italian Journal of Pediatrics·Maria Cristina MaggioGiovanni Corsello
Feb 23, 2020·Mediators of Inflammation·Carla GaggianoLuca Cantarini
Jun 15, 2017·The American Journal of Surgical Pathology·Thomas W CzeczokTaofic Mounajjed
Aug 8, 2021·Journal of Tropical Pediatrics·Fatma İlknur VarolElif Yeşilada

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