Abstract
Background
Wilson's disease (WD) is an inherited disorder of copper metabolism. Although well
documented in many disorders, gender hasn't been directly addressed in WD; therefore,
our aim was to evaluate gender differences in WD.
Methods
We analyzed data on 627 consecutive WD patients entered into our registry (1958–2010).
Results
We observed a male predominance in our population of WD patients (327 males vs. 290
females; p<0.05). At disease diagnosis, 510/627 patients were symptomatic, most patients had
the neuropsychiatric WD form (345/510; p<0.01). The neuropsychiatric form occurred predominantly in men versus women (209/278
vs. 136/232; p<0.01), especially the rigidity-tremor (71/111 vs. 40/111; p<0.05), rigidity (23/33 vs. 10/33; p=0.07) and psychiatric forms (46/71 vs. 25/71; p=0.06). The hepatic form occurred more frequently in women (96/165 vs. 69/165; p<0.01) and women developed the neuropsychiatric form 2 years later than men (29.4 vs. 27.1; p<0.05).
Conclusions
According to our findings, the neuropsychiatric form of WD is predominant at diagnosis
in both genders. The hepatic form of WD occurs more frequently in women, and women
develop the neuropsychiatric form of disease almost 2 years later than men. We speculate these differences may be due to the protective
effect of estrogens and iron metabolism differences.
Keywords
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Article info
Publication history
Published online: September 14, 2011
Accepted:
August 22,
2011
Received in revised form:
August 18,
2011
Received:
April 17,
2011
Identification
Copyright
© 2011 Elsevier B.V. Published by Elsevier Inc. All rights reserved.