OBJECTIVES : Wilson’s disease (WD) is an autosomal recessive inherited condition in which copper excretion via bile is diminished due to gene mutation. Consequence is copper accumulation in the body leading to a variety of symptoms. German registry data showed that 49% WD patients suffer from liver damage, 26% from neurological deficits, 13% from both. Liver failiure is in rare cases short term fatal and may be rescued by liver transplantation. Incidence is estimated around 1: 30,000. Very little is known about the costs of WD, despite it demands life time treatment and is in many cases disabling. Here we examine the hospitalization cost of WD in Germany as first part of cost of disease analysis.

METHODS : Inpatient care data from Federal Statistical Office 2005-2018 and quality reports for 2016 of all German hospitals were evaluated for WD encodings. Calculation based on DRG charges from 2016. Analysis with IBM SPSS-25.

RESULTS : 186 patients hospitalized with WD in2017. In 153 cases the resulting DRG could be identified. Additionally in 46 cases with side diagnosis WD the DRG was reported. 7.5% were liver transplant DRGs, in 5.0% neurologic, 4.0% hepatologic, and 83.5% metabolism DRGs. Average length of stay was 19.3 days (d) with liver transplants, 6.3d with neurologic, 4.9d hepatologic and 3.8d metabolism DRGs. Average cost of stay for liver transplant was 24,205 Euro, partly only preparatory stays, resulting in 44.5% of the annual total cost of 1,336,901 Euro. All other DRG-groups had average costs between 2,432 and 2863 Euro, summing up to 55.5% of total costs.

CONCLUSIONS : Total hospital cost for WD in Germany was 1.3 million Euro in 2017, with 186 cases with primary diagnosis WD. Cost driver are the liver transplantations and their preparation which make close to half of the costs. The hospitalization costs for primarily neurologic or hepatologic reasons were rather similar.