Endocrine Abstracts (2011) 26 P535

Prevalence of metabolic bone disease in Wilson's disease (WD) patients

Sofia Teixeira, Joana Domingos, Rui Carvalho, Helena Pessegueiro Miranda & Marina Magalhães


Centro Hospitalar do Porto, Porto, Portugal.


Background: Skeletal changes including demineralization have been reported in WD. The exact mechanism originating these changes isn’t completely understood but some authors have proposed a link to vitamin D (VitD) metabolism.

Aim: To investigate the prevalence of demineralization in a cohort of WD patients and correlate it with VitD status and severity of hepatic and neurological involvement.

Methods: Thirty-five patients (17 males and 18 females), mean age of 35.1±14.0 years, have done a complete liver profile, calcium, phosphorus, vitD and bone densitometry. Osteopenia and osteoporosis were defined by WHO criteria. VitD deficiency was defined as levels <50 nmol/l and insufficiency between 50 and 74 nmol/l. Hepatic and neurological disease was evaluated by MELD score, EuroWilson scale (EW) and functional independence measure (FIM). Statistical analysis was performed using SPSS. The significance level was established at 0.05.

Results: A total of 40% of the patients presented osteopenia and 13% osteoporosis. 63% had deficient and 27% insufficient VitD levels. Significant correlations were found between T-score at the femoral neck, EW and FIM (r=−0.55, r=0.47); T-score at lumbar spine, MELD, AST and ALP levels (r=−0.42, r=−0.39, r=−0.36). After multiple regression EW was significantly associated with decreased mineralization at femoral neck and MELD score and ALP levels were significantly correlated with demineralization at lumbar spine. It was found a significant correlation between VitD levels, AST and ALT levels (r=−0.36, r=−0.60), but there wasn’t significant association between VitD and severity of hepatic or neurological disease.

Conclusion: Decreased bone mineralization in our WD population doesn’t seem to be associated with low VitD levels, but to the degree of hepatic and neurologic impairment. We didn’t find any correlation between VitD deficiency and severity of neurologic or hepatic disease. We advocate that the high rates of demineralization and VitD deficiency found are significant and it is therefore advisable to evaluate and treat these two issues in this population.

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