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


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.
Medici
Valentina
V
Department of Surgical and Gastroenterological Sciences, Section of Gastroenterology, University of Padua, Padua, Italy. valentina.medici@ucdmc.ucdavis.edu
Di Leo
Vincenza
V
Lamboglia
Francesca
F
Bowlus
Christopher L
CL
Tseng
Szu-Ching
SC
D'Incà
Renata
R
Irato
Paola
P
Burra
Patrizia
P
Martines
Diego
D
Sturniolo
Giacomo C
GC
D000293:Adolescent; D000328:Adult; D001706:Biopsy; D002614:Chelating Agents / Q000627:therapeutic use*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000188:drug therapy* / Q000378:metabolism; D006801:Humans; D007501:Iron / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D010396:Penicillamine / Q000627:therapeutic use*; D018709:Statistics, Nonparametric; D016896:Treatment Outcome; D019287:Zinc Sulfate / Q000627:therapeutic use*
D002614:Chelating Agents; D019287:Zinc Sulfate; D007501:Iron; D010396:Penicillamine