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[Experiences with penicillamine in Wilson's disease]
Psychiatrie, Neurologie Und Medizinische Psychologie. Beihefte
|January 1, 1975
Summary
Early treatment of Wilson disease (morbus Wilson) with penicillamine is crucial, especially in asymptomatic stages. Continuous therapy can resolve symptoms, and monitoring copper balance aids dosage adjustments.
Area of Science:
- Neurology
- Hepatology
- Medical Biochemistry
Context:
- Wilson disease (morbus Wilson) is a genetic disorder of copper metabolism.
- Early diagnosis and intervention are critical for managing Wilson disease.
- Treatment strategies aim to reduce copper levels and prevent organ damage.
Purpose:
- To report on the principles of Wilson disease treatment using penicillamine.
- To evaluate treatment efficacy in patients with varying disease stages.
- To discuss methods for optimizing penicillamine dosage.
Summary:
- This study analyzed seventeen patients with Wilson disease and two in the pre-clinical stage.
- Treatment initiation in the asymptomatic stage is recommended for better outcomes.
- Penicillamine treatment showed favorable responses in pseudo-sclerosis symptoms compared to hypokinetic symptoms.
- Continuous penicillamine therapy over 3-4 years led to symptom resolution.
- Monitoring basal and penicillamine-induced copper secretion is proposed for dosage determination.
Impact:
- Highlights the importance of early Wilson disease treatment for improved prognosis.
- Demonstrates the long-term efficacy of penicillamine in managing Wilson disease symptoms.
- Suggests a refined approach to penicillamine dosage adjustment based on copper balance monitoring.