Wilson’s Disease: An Update, with Emphasis on New Approaches to Treatment

Brewer, George J.; Yuzbasiyan‐Gurkan, Vilma

doi:10.1159/000171218

Cited by 28 publications

(11 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…A potential problem with penicillamine is that, in addition to its hypersensitivity-type reaction, about 10% of patients eventually demonstrate some form of immunologically induced intolerance to it. In 1969, Walshe [4] reported the introduction of triethylene tetramine for the management of a 17-year-old boy who developed nephrotic synWilson's disease is an autosomal recessive disorder characterized by an accumulation of a toxic amount of copper in the liver, kidney, brain, cornea and other tissues [1,2]. Since its introduction in 1956 by Walshe [3], D-penicillamine has been the drug of first choice for both initial and maintenance therapy.…”

Section: Introductionmentioning

confidence: 99%

Wilson's Disease Treatment by Triethylene Tetramine Dihydrochloride (Trientine, 2HCI): Long-Term Observations

Morita

Yoshino²,

Watari³

et al. 1992

Dev Pharmacol Ther

View full text Add to dashboard Cite

Wilson's disease is an autosomal recessive disorder characterized by an accumulation of a toxic amount of copper in the body. Triethylene tetramine dihydrochloride (trientine, 2HC1) is a new chelating agent that may be effective in the removal of excess copper but long-term efficacy has not yet been investigated. Here we report the use of trientine over more than 8 years in 2 patients with Wilson's disease who could not tolerate D-penicillaminc. We found no significant side effect, except a decreased serum iron concentration without clinical symptoms of anemia. In annual examinations at a steady state, the serum copper levels remained below 20 μg/100 ml. The 24-hour urinary copper excretion was less than that found using D-penicillamine, while the basal copper excretion, after 5 days abstinence from trientine, was maintained below 100 μg/day. Both hepatic and neurological manifestations except bulbar symptoms were recovered without any initial deterioration.

show abstract

Section: Introductionmentioning

confidence: 99%

Wilson's Disease Treatment by Triethylene Tetramine Dihydrochloride (Trientine, 2HCI): Long-Term Observations

Morita

Yoshino²,

Watari³

et al. 1992

Dev Pharmacol Ther

View full text Add to dashboard Cite

show abstract

“…There has been a recent wave of concern about penicillamine treatment [33,43] which has renewed interest in its mode of action and a search for alternate treatments. Treatment with zinc is gaining credibility [7,22].…”

Section: Practical Considerationsmentioning

confidence: 99%

“…Zinc (as sulphate or acetate) has been promoted as a treatment for Wilson disease by groups in Holland [22] and in Michigan [7], first to maintain control of copper balance after initial treatment with penicillamine and latterly as a treatment used from the time of diagnosis. Zinc induces MT synthesis in gut mucosal cells.…”

Section: Practical Considerationsmentioning

confidence: 99%

“…Copper entering these cells displaces zinc from this MT and is retained in the cells long enough to be lost back into the lumen as the mucosal cells are shed. To be maximally effective, the zinc needs to be given as a substantial dose (50 mg of zinc per dose) and to be taken I h before each meal [7]. It would be expected that the effect of zinc would be very gradual since it is acting by reducing the absorption of zinc and depends upon the natural turnover of copper in the affected organs to eventually reduce the levels.…”

Section: Practical Considerationsmentioning

confidence: 99%

See 1 more Smart Citation

Copper and liver disease

Danks

1991

Eur J Pediatr

View full text Add to dashboard Cite

There has been some limited progress in the understanding of the basic defect in Wilson disease and the gene concerned has been located to the chromosome region 13q14. Treatment with zinc has emerged as a definite alternative to penicillamine administration and some shortcomings and/or hazards of both forms of therapy have emerged as their modes of action have been studied more carefully. Tetrathimolybdate may have a place in treatment, especially when rapid complexing of copper is important. Hepatic copper accumulation occurs in a number of cholestatic diseases and they play an important part in pathogenesis and can occasionally lead to neurologic toxic effects. Copper overload in the newborn period when biliary excretion of copper is inefficient may establish a vicious cycle of copper accumulation and liver damage in Indian childhood cirrhosis and less frequently in babies in other countries.

show abstract

“…Chronic liver dis ease is not a prominent feature of these syndromes. Con versely, familial chronic liver diseases such as hemochro matosis, Wilson's disease, ai-antitrypsin deficiency, ga lactosemia, and glycogen storage diseases are not associat ed with clinical marrow failure [3][4][5],…”

Section: Introductionmentioning

confidence: 99%

A New Syndrome of Familial Aplastic Anemia and Chronic Liver Disease

et al. 1997

View full text Add to dashboard Cite

This report describes a new familial syndrome characterized by a combination of bone marrow failure and chronic liver disease. This disorder appears to be genetic in origin with an autosomal dominant inheritance and was characterized by hyperactivity of the immune system with increased activated cytotoxic T lymphocytes in peripheral blood and bone marrow and the presence of γ-interferon messenger RNA in bone marrow of several cases.

show abstract

Wilson’s Disease: An Update, with Emphasis on New Approaches to Treatment

Cited by 28 publications

References 10 publications

Wilson's Disease Treatment by Triethylene Tetramine Dihydrochloride (Trientine, 2HCI): Long-Term Observations

Wilson's Disease Treatment by Triethylene Tetramine Dihydrochloride (Trientine, 2HCI): Long-Term Observations

Copper and liver disease

A New Syndrome of Familial Aplastic Anemia and Chronic Liver Disease

Contact Info

Product

Resources

About