Difference in iron metabolism may partly explain sex-related variability in the manifestation of Wilson's disease.
Copper
Iron
Phenotype
Sex
Wilson’s disease
Journal
Journal of trace elements in medicine and biology : organ of the Society for Minerals and Trace Elements (GMS)
ISSN: 1878-3252
Titre abrégé: J Trace Elem Med Biol
Pays: Germany
ID NLM: 9508274
Informations de publication
Date de publication:
Dec 2020
Dec 2020
Historique:
received:
07
05
2020
revised:
05
08
2020
accepted:
26
08
2020
pubmed:
17
9
2020
medline:
28
9
2021
entrez:
16
9
2020
Statut:
ppublish
Résumé
Wilson's disease (WD) is a hereditary disorder characterized by abnormal metabolism of copper. For unknown reasons, the clinical picture of this disease appears to be sex-dependent. Because the metabolism of copper and iron is interrelated, we aimed to evaluate whether the variability in the clinical picture of WD could be explained by the sex difference in iron metabolism. A total of 138 WD patients were examined in this study: 39 newly diagnosed, treatment naive patients and 99 individuals already treated with decoppering drugs. The serum concentration of ceruloplasmin (Cp) and copper were measured using an enzymatic colorimetric assay and by atomic absorption spectroscopy, respectively. The parameters of iron metabolism were determined by using standard laboratory methods and enzyme immunoassays. In the treatment naive group men had a higher median serum concentration of ferritin (290.5 vs. 81.0 ng/mL, p < 10 Iron metabolism differs between men and women with WD, which may partly explain the sex difference noted in the disease manifestation.
Sections du résumé
BACKGROUND/AIM
OBJECTIVE
Wilson's disease (WD) is a hereditary disorder characterized by abnormal metabolism of copper. For unknown reasons, the clinical picture of this disease appears to be sex-dependent. Because the metabolism of copper and iron is interrelated, we aimed to evaluate whether the variability in the clinical picture of WD could be explained by the sex difference in iron metabolism.
METHODS
METHODS
A total of 138 WD patients were examined in this study: 39 newly diagnosed, treatment naive patients and 99 individuals already treated with decoppering drugs. The serum concentration of ceruloplasmin (Cp) and copper were measured using an enzymatic colorimetric assay and by atomic absorption spectroscopy, respectively. The parameters of iron metabolism were determined by using standard laboratory methods and enzyme immunoassays.
RESULTS
RESULTS
In the treatment naive group men had a higher median serum concentration of ferritin (290.5 vs. 81.0 ng/mL, p < 10
CONCLUSION
CONCLUSIONS
Iron metabolism differs between men and women with WD, which may partly explain the sex difference noted in the disease manifestation.
Identifiants
pubmed: 32937238
pii: S0946-672X(20)30202-9
doi: 10.1016/j.jtemb.2020.126637
pii:
doi:
Substances chimiques
Copper
789U1901C5
Iron
E1UOL152H7
Ceruloplasmin
EC 1.16.3.1
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
126637Informations de copyright
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