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Wilson disease-the impact of hyperimmunity on disease activity:A case report

World Journal of Clinical Cases 2021年第6期9卷 1386-1393页

作者： Wolfgang Stremmel Thomas Longerich RenéLiere Vladimir Vacata josef van helden Ralf Weiskirchen Pracice for Gastroenterology Medical Center Baden-BadenBaden-Baden D-76530Germany Department of General Pathology University Heidelberg HospitalInstitute of PathologyUniversity of HeidelbergHeidelberg D-69115Germany MVZ Dr.Stein+Kollegen Labor MönchengladbachMönchengladbach D-41069Germany Institute of Molecular Pathobiochemistry Experimental Gene Therapy and Clinical ChemistryRheinisch-Westfaelische Technische Hochschule University Hospital AachenAachen D-52074Germany

BACKGROUND In Wilson disease lack of biliary copper excretion causes hepatocellular injury by accumulation of free toxic *** overspill to serum accounts for neuronal damage as second common *** with copper chelators or zinc targets the removal of this free ***,in some patients liver disease persists for unknown reason despite normalized free *** discovery of a hyperimmunity as a contributing pathogenetic factor was discovered in this case report with implication also for other liver *** SUMMARY A 9-year-old girl was diagnosed in August 2009 by family screening of having asymptomatic Wilson disease with elevated *** at time of diagnosis antinuclear antibodies(ANA)were elevated without hyperimmunoglobulinemia(immunoglobulin G,IgG).After one year of therapy with Dpenicillamine transaminases normalized together with free serum *** continuous therapy with copper chelators free copper remained normal until today,whereas transaminases raised to alanine aminotransferase values of 571 U/L in December *** hyperimmunity a tentative steroid course on top of Dpenicillamine improved ***,hyperimmunity may have impact on liver inflammation after control of the metabolic disturbance.A retrospective cohort study confirmed the common association of elevated transaminases with ANA,but no IgG *** This hyperimmune-triggered condition may represent a new entity which per se or on top of other liver diseases induces liver inflammation responsive to steroids.

关键词： Wilson disease Copper metabolism Antinuclear antibodies Diagnosis Steroid therapy Case report

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