OBJECTIVES

: Wilson disease (WD) is an inherited condition in which copper excretion via bile is diminished due to gene mutations. Consequence is toxic accumulation of copper predominantly in liver and brain, leading to a variety of symptoms. WD prevalence in Germany is often estimated around 1:30,000. Little is known about the medical treatment and hospitalization rates. Here we used health insurance billing data to estimate current number and care of WD patients.

METHODS : Inpatient care data from Federal Statistical Office 2005-2017 and ambulatory billing data from the federal physicians’ organization 2009-2017 were evaluated for WD encodings and treatment schemes. Analysis with IBM SPSS-25.

RESULTS

: 2017 in German statutory sick funds 1,368 patients were diagnosed WD and received specific therapy. Thereof 828 (60.5%) D-penicillamine, 217 (15.9%) trientine, 174 (12.7%) zinc-acetate and 149 (10.9%) other zinc-salts. 46.4% treated patients are women, they make 45.7% of all chelator and 48.1% of all zinc therapies. WD-prevalence is estimated 1:60.000. Shares of therapy were over time. Patients younger than 24 received more frequently chelators. Diagnosed patients under 15 were 159 (2009) and 161 (2017). Regional distribution of WD was inconspicuous.

186 patients were hospitalized with WD as main diagnosis in 2017. 45.1% female, who stay shorter in hospital (6.2 days) than men (8.5 days). 45.2% of cases neurologic departments, 29.0% gastroenterological, 19.9 pediatric, 3.2% transplant units, 2.7% other. Regional distribution of hospitalization differs, from 4.9 per M in Rhineland to 11.8 per M in Pomerania. Mean age at hospitalization is 30.1 years (m 29.9y, f 30.3). Around 48% treatments in university hospitals.

CONCLUSIONS : German billing data allow for precise estimation of treated WD patients. From 2009 to 2017 treatment patterns were stable change. Younger patients are treated more frequently with chelators than adults. Hospitalizations are partly driven by regional habits. A national registry would support further insights.