title: Bone demineralisation in a large cohort of Wilson disease patients.
abstract: OBJECTIVE
  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%) (χ(2) = 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/cm(2) (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.
authors: Department of Internal Medicine IV, Heidelberg University Hospital, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany, karl-heinz.weiss@med.uni-heidelberg.de.
authors: Karl Heinz
authors: KH
authors: Weiss
authors: Mart
authors: M
authors: Van de Moortele
authors: Daniel Nils
authors: DN
authors: Gotthardt
authors: Jan
authors: J
authors: Pfeiffenberger
authors: Jessica
authors: J
authors: Seessle
authors: Elena
authors: E
authors: Ullrich
authors: Evelien
authors: E
authors: Gielen
authors: Herman
authors: H
authors: Borghs
authors: Els
authors: E
authors: Adriaens
authors: Wolfgang
authors: W
authors: Stremmel
authors: Wouter
authors: W
authors: Meersseman
authors: Steven
authors: S
authors: Boonen
authors: David
authors: D
authors: Cassiman
mesh_terms: D015502:Absorptiometry, Photon / Q000379:methods; D000293:Adolescent; D000328:Adult; D000368:Aged; D018488:Bone Demineralization, Pathologic / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000209:etiology*; D015519:Bone Density; D001851:Bone Diseases, Metabolic / Q000000981:diagnostic imaging / Q000453:epidemiology; D015331:Cohort Studies; D005260:Female; D005272:Femur Neck; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000000981:diagnostic imaging / Q000453:epidemiology; D006801:Humans; D008159:Lumbar Vertebrae; D008297:Male; D008875:Middle Aged; D010024:Osteoporosis / Q000000981:diagnostic imaging / Q000453:epidemiology; D012307:Risk Factors; D055815:Young Adult