Open AccessMeasurement of urinary copper excretion after 48-h d-penicillamine cessation as a compliance assessment in Wilson�s disease
Author(s) -
Karolina Dzieżyc
Publication year - 2015
Publication title -
functional neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.505
H-Index - 42
eISSN - 1971-3274
pISSN - 0393-5264
DOI - 10.11138/fneur/2015.30.4.264
Subject(s) - penicillamine , excretion , urinary system , medicine , copper , wilson's disease , urology , chelation therapy , gastroenterology , disease , chemistry , organic chemistry , thalassemia
Treatment of Wilson's disease (WD) with anti-copper agents is effective in most compliant patients. During long-term treatment with chelating agents, a two-day interruption of the treatment should result in normal urinary copper concentrations (<50 μg/dl). The aim of this study was to establish the usefulness of this method as a compliance assessment in these patients. We examined consecutive patients treated with d-penicillamine (DPA) undergoing routine follow-up studies at our center. We performed 24-h urinary copper excretion analysis 48 h after interruption of chelating therapy. Thirty-two patients were enrolled. After DPA cessation, normalization of copper excretion was observed in 91% of reportedly compliant patients. The specificity and sensitivity values of this test were 87% and 77%, respectively. Measurement of 24-h urinary copper excretion after a 48-h interruption of DPA therapy in patients with WD is a reliable method for confirming patients' compliance.