title: Coagulation Parameters in Wilson Disease.
abstract: OBJECTIVE
  Wilson disease (WD) is an autosomal recessive disorder of copper metabolism. Alterations of copper metabolism have been associated with changes in coagulation factors. The aim of the present study was the analysis of coagulation factors in WD patients.
  
  
  METHODS
  100 patients attending a tertiary WD outpatient clinic were analyzed in a prospective cross sectional cohort study. Out of peripheral venous blood samples coagulation factors were assessed including: full blood count, INR, partial thromboplastin time (PTT), clotting factors II, V, VII, VIII, IX, X, XI, XII, XIII, von Willebrand factor/-antigen, fibrinogen, antithrombin III, protein S, protein C, activated protein C (APC) resistance. Subgroup analyses of the blood tests were performed for sex, initial clinical presentation, WD treatment and liver function.
  
  
  RESULTS
  Subgroup analysis by liver function showed decreased levels of factors II, V, VII and X. Subgroup analysis by gender or clinical course of the disease did not reveal significant coagulation changes. In patients treated with trientine significantly decreased levels of factors II, VII and antithrombin III and increased von Willebrand factor/-antigen levels were detected. Factor VIII levels were significantly reduced in patients receiving zinc.
  
  
  CONCLUSIONS
  Although significant differences of some coagulation parameters in subgroup analysis were found, no clinically relevant alterations of the coagulation system in WD patients could be detected.
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Heidelberg, Heidelberg, Germany.
authors.forename: Mark
authors.initials: M
authors.lastname: Schaefer
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Heidelberg, Heidelberg, Germany.
authors.forename: Laura
authors.initials: L
authors.lastname: Weber
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Heidelberg, Heidelberg, Germany.
authors.forename: Daniel
authors.initials: D
authors.lastname: Gotthardt
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Heidelberg, Heidelberg, Germany.
authors.forename: Jessica
authors.initials: J
authors.lastname: Seessle
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Heidelberg, Heidelberg, Germany.
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Stremmel
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Heidelberg, Heidelberg, Germany.
authors.forename: Jan
authors.initials: J
authors.lastname: Pfeiffenberger
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Heidelberg, Heidelberg, Germany. med.uni-heidelberg.de.
authors.forename: Karl Heinz
authors.initials: KH
authors.lastname: Weiss
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D001777:Blood Coagulation / Q000187:drug effects; D001779:Blood Coagulation Factors / Q000032:analysis*; D002614:Chelating Agents / Q000627:therapeutic use; D003430:Cross-Sectional Studies; D005260:Female; D006527:Hepatolenticular Degeneration / Q000097:blood* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D019934:International Normalized Ratio; D008111:Liver Function Tests; D008297:Male; D010314:Partial Thromboplastin Time; D010396:Penicillamine / Q000627:therapeutic use; D011237:Predictive Value of Tests; D011446:Prospective Studies; D062606:Tertiary Care Centers; D014266:Trientine / Q000627:therapeutic use; D017967:Zinc Compounds / Q000627:therapeutic use
chemical_list: D015415:Biomarkers; D001779:Blood Coagulation Factors; D002614:Chelating Agents; D017967:Zinc Compounds; D010396:Penicillamine; D014266:Trientine