Measurement of urinary copper excretion after 48-h d-penicillamine cessation as a compliance assessment in Wilson's disease.

Abstract

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.

• Authors: Karolina Dzieżyc, Tomasz Litwin, Grzegorz Chabik, Anna Członkowska
• Journal: Functional neurology (Funct Neurol) 2015 Oct-Dec Vol. 30 Issue 4 Pg. 264-8 ISSN: 1971-3274 [Electronic] Italy
• PMID: 26727705 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Chelating Agents
• Copper
• Penicillamine

Topics

• Adult
• Aged
• Chelating Agents (administration & dosage, therapeutic use)
• Copper (urine)
• Female
• Follow-Up Studies
• Hepatolenticular Degeneration (diagnosis, drug therapy)
• Humans
• Male
• Middle Aged
• Penicillamine (administration & dosage, therapeutic use)
• Time Factors
• Treatment Outcome
• Young Adult