Social and demographic characteristics of a Polish cohort with Wilson disease and the impact of treatment persistence
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(2019) 14:167
RESEARCH
Open Access
Social and demographic characteristics of a Polish cohort with Wilson disease and the impact of treatment persistence Wojciech Maselbas1, Tomasz Litwin2 and Anna Czlonkowska1,2*
Abstract Background: Wilson disease (WD) is a genetic disorder involving impaired copper metabolism, which presents with hepatic, neurological, and/or psychiatric manifestations. WD requires lifelong pharmacotherapy and treatment persistence may be problematic. We studied social characteristics, education, and work-related activities and how they are affected by WD symptoms and treatment persistence. Methods: In a cross-sectional study, data on demographic characteristics, achieved education level, household and marital status, plus a primary source of income were collected from 202 Polish subjects (mean ± standard deviation age of 36.4 ± 9.9 years at assessment) with WD. Results: Overall, WD appeared to have a negative impact on achieved level of education and influenced the ability to work as compared with the general Polish population. Patients with neurological manifestations less often achieved upper-secondary/post-secondary or higher education compared with those with hepatic manifestations (65.5% vs. 83.6%; p = 0.003). They also significantly less frequently stated salary (19.6% vs. 56.2%; p < 0.0001) as the primary income and more often were on disability pension (53.3% vs. 26.0%; p = 0.0003). The percentage of married patients with WD appeared lower than in the general population (47.0% vs. 54.6%), although the difference was not significant (p = 0.2). The 27.6% of patients who were non-persistent with WD treatment less frequently achieved upper/post-secondary or higher education compared with persistent patients (66.0% vs. 76.3%; NS) and their primary source of outcome was significantly less often a salary (18.9% vs. 40.3%; p = 0.001). Conclusions: Neurological manifestations had an adverse effect on education level and work ability. Treatment nonpersistence had a further negative impact regardless of the disease form. Patients with WD should receive appropriate treatment, with the need for persistence emphasized and monitored to avoid a detrimental effect on their lives. Keywords: Wilson disease, Neurological manifestations, Treatment persistence
Introduction Wilson disease (WD) is an autosomal recessive disorder of impaired copper metabolism caused by mutations in the ATP7B gene, which encodes a copper-transporting ATPase involved in copper transportation across cell membranes [1]. Decreased ATP7B function leads to reduced incorporation of copper into ceruloplasmin and impaired biliary excretion. The general prevalence of WD is thought to be
* Correspondence: [email protected] 1 Department of Experimental and Clinical Pharmacology, Medical University of Warsaw, Warsaw, Poland 2 2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland
around 1 in 30,000 people but prevalence varies among populations and WD may be underrecognized and underdiagnosed [2, 3]. The
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