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Erschienen in: Journal of Inherited Metabolic Disease 5/2015

01.09.2015 | Original Article

Bone demineralisation in a large cohort of Wilson disease patients

verfasst von: Karl Heinz Weiss, Mart Van de Moortele, Daniel Nils Gotthardt, Jan Pfeiffenberger, Jessica Seeßle, Elena Ullrich, Evelien Gielen, Herman Borghs, Els Adriaens, Wolfgang Stremmel, Wouter Meersseman, Steven Boonen, David Cassiman

Erschienen in: Journal of Inherited Metabolic Disease | Ausgabe 5/2015

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Abstract

Aims and background

We compared the bone mineral density (BMD) of adult Wilson disease (WD) patients (n = 148), with an age- and gender-matched healthy control population (n = 148). Within the WD cohort, correlations of BMD with WD disease parameters, lab results, type of treatment and known osteoporosis risk factors were analysed.

Methods

Hip and lumbar spine absolute BMD and T-score were measured by dual-energy X-ray absorptiometry. Osteoporosis and osteopenia were defined as a T-score ≤ −2.5, and between −1 and −2.5, respectively.

Results

There were significantly more subjects with abnormal T-scores in the WD population (58.8 %) than in the control population (45.3 %) (χ2 = 6.65, df = 2, p = 0.036), as there were 50.0 % osteopenic and 8.8 % osteoporotic WD patients, vs. 41.2 % and 4.1 %, respectively, in the controls. Especially L2-L4 spine BMD measurements (BMD and T-scores) differed significantly between the WD population and matched controls. L2-L4 spine BMD for WD patients was on average 0.054 g/cm2 (5.1 %) lower than in matched normal controls (0.995 ± 0.156 vs 1.050 ± 0.135; p = 0.002). We found no significant correlation between BMD values and any of the WD disease parameters (e.g. the severity of liver disease), lab results, type of treatment or known osteoporosis risk factors. Duration of D-penicillamine treatment was negatively correlated with femoral BMD value, but in a clinically irrelevant manner, compared to age and gender. Importantly, BMD remained significantly lower in WD patients (n = 89) vs. controls after excluding WD patients with cirrhosis (p = 0.009).

Conclusions

Our study suggests that WD is intrinsically associated with bone demineralisation.
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Metadaten
Titel
Bone demineralisation in a large cohort of Wilson disease patients
verfasst von
Karl Heinz Weiss
Mart Van de Moortele
Daniel Nils Gotthardt
Jan Pfeiffenberger
Jessica Seeßle
Elena Ullrich
Evelien Gielen
Herman Borghs
Els Adriaens
Wolfgang Stremmel
Wouter Meersseman
Steven Boonen
David Cassiman
Publikationsdatum
01.09.2015
Verlag
Springer Netherlands
Erschienen in
Journal of Inherited Metabolic Disease / Ausgabe 5/2015
Print ISSN: 0141-8955
Elektronische ISSN: 1573-2665
DOI
https://doi.org/10.1007/s10545-015-9815-y

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