+ Site Statistics
+ Search Articles
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ PDF Full Text
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Translate
+ Recently Requested

Wilson's disease: long-term follow-up of a cohort of 24 patients treated with D-penicillamine

Wilson's disease: long-term follow-up of a cohort of 24 patients treated with D-penicillamine

European Journal of Gastroenterology and Hepatology 22(5): 564-571

Detailed data on long-term effectiveness of various drug therapies in Wilson's disease (WD) are lacking. Therefore, we retrospectively reviewed our patient cohort treated with D-penicillamine. This study reports on the clinical presentation, the diagnostic evaluation, and the disease course in 24 WD patients treated long-term (15+/-12 years, between 1969 and 2009) with D-penicillamine. The overall survival in our cohort was 91.6%. Twenty-two of 24 patients had liver disease at presentation, 17 of 24 patients (71%) had cirrhosis, 11 of whom had complications of cirrhosis. Six of 11 of these patients showed hepatological improvement (five of six) or stabilization (one of six), three of 11 were transplanted, one of 11 died, one of 11 discontinued follow-up. In the six of 17 cirrhotic patients without complications, improvement (four of six) or stabilization (two of six) occurred. Of all other patients (seven of 24), five of seven showed improvement (three of five) or stabilization (two of five), hepatological deterioration occurred only in one patient due to poor therapy compliance and one of seven discontinued follow-up. Neuropsychiatric symptoms were present in 13 of 24 at presentation and resolved in one of 13, decreased in seven of 13, stabilized in four of 13 and worsened in one of 13 patients (due to poor compliance). In general, we observed a favorable hepatological and neurological evolution with D-penicillamine. Despite the presence of liver disease or neuropsychiatric symptoms at baseline in all but one of the patients, we report beneficial results on liver and neurological disease after very long-term treatment with D-penicillamine, thereby adding to its reputation as 'first-line' therapy in WD.

(PDF emailed within 0-6 h: $19.90)

Accession: 056951973

Download citation: RISBibTeXText

PMID: 20042865

DOI: 10.1097/MEG.0b013e3283353df8

Related references

Unusual digestive lesions in a patient with Wilson's disease treated with long-term penicillamine. New England Journal of Medicine 313(1): 49, 1985

Changes in zinc metabolism during long-term D-penicillamine treatment of patients with Wilson-Konovalov disease. Zhurnal Nevropatologii i Psikhiatrii Imeni S.S. Korsakova 84(3): 356-359, 1984

The effect of long-term treatment with d-penicillamine on the humoral immune response in patients with Wilson's disease. Materia Medica Polona. Polish Journal of Medicine and Pharmacy 8(1): 48-50, 1976

The effect of long term treatment with penicillamine on the copper content in the liver in patients with Wilson's disease. Acta Hepato-Gastroenterologica 22(5): 292-296, 1975

Long-term penicillamine therapy for Wilson's disease. Southern Medical Journal 73(4): 530-531, 1980

The detection of antibodies against D-penicillamine. 3. Detection of antibodies against D-penicillamine in the serum of patients with Wilson's disease treated with D-penicillamine--preliminary results. Die Pharmazie 41(2): 126-127, 1986

Long-term follow-up of lung function parameters in patients with idiopathic pulmonary fibrosis treated with prednisone and azathioprin or d-penicillamine. Praxis und Klinik der Pneumologie 33 Suppl 1: 680-688, 1979

Long-term outcomes of patients with Wilson disease in a large Austrian cohort. Clinical Gastroenterology and Hepatology 12(4): 683-689, 2014

Ophthalmoscopic changes in a patient with Wilson's disease during long-term penicillamine therapy. Annals of Ophthalmology 10(9): 1227-1230, 1978

Long-term treatment of Wilson's disease with D-penicillamine. Report on 20 cases. Deutsche Medizinische Wochenschrift 92(37): 1657-1662, 1967

Laboratory changes and complications during long-term therapy of Wilson's disease using D-penicillamine. Vnitrni Lekarstvi 16(4): 344-353, 1970

Wilson's disease (experience of long-term treatment with penicillamine---con penicilamina. Revista Clinica Espanola 133(5): 451-454, 1974

Systemic sclerosis-like lesions during long-term penicillamine therapy for Wilson's disease. British Journal of Dermatology 116(1): 95-100, 1987

Liver copper determination in long-term treatment of Wilson's disease with D-penicillamine. Die Medizinische Welt 32(3): 109-110, 1981

Effects of long-term treatment in Wilson's disease with D-penicillamine and zinc sulphate. Journal of Neurology 243(3): 269-273, 1996