Medici Valentina, Di Leo Vincenza, Lamboglia Francesca, Bowlus Christopher L, Tseng Szu-Ching, D'Incà Renata, Irato Paola, Burra Patrizia, Martines Diego, Sturniolo Giacomo C
Department of Surgical and Gastroenterological Sciences, Section of Gastroenterology, University of Padua, Padua, Italy.
Scand J Gastroenterol. 2007 Dec;42(12):1495-500. doi: 10.1080/00365520701514495.
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.
Twenty-three Wilson's disease patients undergoing long-term treatment were enrolled in the study.
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.
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.
肝豆状核变性中铁代谢的生理学机制在很大程度上尚不清楚，关于铁蓄积的实际存在情况和进展的数据也很匮乏。本研究的目的是评估随访肝活检、血清和尿前铁调素中的铁代谢参数，包括肝脏铁浓度。
本研究纳入了23例接受长期治疗的肝豆状核变性患者。
与接受锌治疗的患者相比，接受青霉胺治疗的患者肝脏铁含量显著增加。尽管铁代谢的生化模式正常，但肝豆状核变性患者的血清和尿前铁调素浓度显著高于健康志愿者。
长期青霉胺治疗似乎导致肝脏中铁蓄积更为明显。这一观察结果可能为修订肝豆状核变性长期使用青霉胺的治疗方式提供依据。与健康志愿者相比，肝豆状核变性患者血清和尿前铁调素显著升高这一发现表明铁调素在肝豆状核变性铁代谢中起作用，但这需要通过对这些患者肝脏铁调素表达的研究来证实。
