Effect of penicillamine and zinc on iron metabolism in Wilson's disease

Valentina Medici, Vincenza Di Leo, Francesca Lamboglia, Christopher Bowlus, Szu Ching Tseng, Renata D'Incà, Paola Irato, Patrizia Burra, Diego Martines, Giacomo C. Sturniolo

Research output: Contribution to journalArticlepeer-review

22 Scopus citations


Objective. The physiology of iron metabolism in Wilson's disease is largely unknown, and there is a paucity of data on the real presence and progression of iron accumulation. The purpose of this study was to assess the iron metabolism parameters, including hepatic iron concentration, in follow-up liver biopsies and serum, and urinary pro-hepcidin. Material and methods. Twenty-three Wilson's disease patients undergoing long-term treatment were enrolled in the study. Results. Hepatic iron content was significantly increased in penicillamine-treated patients compared with zinc-treated patients. Serum and urinary pro-hepcidin concentrations were significantly higher in Wilson's disease patients than in healthy volunteers, despite a normal biochemical pattern of iron metabolism. Conclusions. Long-term penicillamine treatment seems to be responsible for a more marked iron accumulation in the liver. This observation may justify a revision of long-term Wilson's disease treatment modalities with penicillamine. The finding that serum and urinary pro-hepcidin is significantly increased in Wilson's disease patients compared with healthy volunteers suggests a role for hepcidin in iron metabolism in Wilson's disease, but this needs to be confirmed by a study of hepatic hepcidin expression in these patients.

Original languageEnglish (US)
Pages (from-to)1495-1500
Number of pages6
JournalScandinavian Journal of Gastroenterology
Issue number12
StatePublished - 2007


  • Copper
  • Hepcidin
  • Iron
  • Wilson's disease

ASJC Scopus subject areas

  • Gastroenterology


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