Oral Zinc Sulphate as Long-Term Treatment in Wilson’s Disease (Hepatolenticular Degeneration)

Hoogenraad, T. U.; Koevoet, R.; Korver, E.G.W.M. De Ruyter

doi:10.1159/000115077

Cited by 133 publications

(49 citation statements)

References 4 publications

(5 reference statements)

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“…70,81 Zinc Zinc was first used in the 1960s to treat Wilson's disease. 82,83 Its mode of action is through inhibition of copper uptake by intestinal mucosa. Zinc induces enterocyte metallothionein which has a greater affinity for copper (than for zinc) inhibiting its portal absorption.…”

Section: Trientinementioning

confidence: 99%

Insights into the management of Wilson’s disease

Kathawala

Hirschfield

2017

Therap Adv Gastroenterol

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Abstract:Wilson's disease is a rare, inherited autosomal recessive disease of copper metabolism, in which the causative gene, ATP7B, results in absent or reduced function of the ATP7B transporter important for biliary excretion of copper and incorporation of copper into caeruloplasmin. Affected patients accumulate excessive copper within the liver, brain and other tissues. A disease mainly of children, adolescents and young adults; clinical features vary from the asymptomatic state to chronic liver disease, acute liver failure, and neuropsychiatric manifestations. Diagnosis requires a high index of suspicion and is based on a combination of clinical signs, biochemical tests, hepatic copper content assay and mutation analysis of the ATP7B gene; to date, there are more than 500 mutations of ATP7B in patients with Wilson's disease. Early recognition and treatment can result in an excellent prognosis whereas untreated disease is almost always fatal. Drug therapies include chelating agents, such as penicillamine or trientine, and zinc salts. Liver transplantation is curative correcting the underlying pathophysiology and is traditionally indicated in acute liver failure or end-stage liver disease refractory to medical therapy. This review provides an overview of various aspects of Wilson's disease including molecular basis of the disease, clinical features, diagnostic and management strategies with their current limitations.

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Section: Trientinementioning

confidence: 99%

Insights into the management of Wilson’s disease

Kathawala

Hirschfield

2017

Therap Adv Gastroenterol

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“…11 In January, 1997 the Food and Drug Administration approved zinc for the maintenance therapy of Wilson' s disease, based on our work in developing this therapy. 1,[12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28] Schouwink 29 and Hoogenraad et al [30][31][32] have also used zinc to treat Wilson' s disease. Because zinc is essentially nontoxic, and the other two agents have toxic side-effects, in our opinion zinc is now the preferred maintenance therapy for Wilson' s disease.…”

mentioning

confidence: 99%

Treatment of Wilson's disease with zinc. XVII: Treatment during pregnancy

et al. 2000

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Therapy of Wilson's disease continues to evolve. In 1997, zinc acetate was added to the list of drugs approved by the Food and Drug Administration, which includes penicillamine and trientine. The mechanism of zinc's anticopper action is unique. It induces intestinal cell metallothionein, which binds copper and prevents its transfer into blood. As intestinal cells die and slough, the contained copper is eliminated in the stool. Thus, zinc prevents the intestinal absorption of copper. It is universally agreed that pregnant Wilson's disease patients should remain on anticopper therapy during pregnancy. There are numerous reports of such patients stopping penicillamine therapy to protect their fetus from teratogenicity, only to undergo serious deterioration and even death from renewed copper toxicity. Penicillamine and trientine have teratogenic effects in animals, and penicillamine has known teratogenic effects in humans. In this report we discuss the results of 26 pregnancies in 19 women who were on zinc therapy throughout their pregnancy. The evidence is good that zinc protects the health of the mother during pregnancy. Fetal outcomes were generally quite good, although one baby had a surgically correctable heart defect and one had microcephaly. (HEPATOLOGY 2000;31:364-370.)

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“…Schouwink [50] gave zinc to two Wilson's disease patients as part of his thesis work, although this work was never published in the general literature. Hoogenraad's group [51][52][53] later followed up one of Schouwink's patients and also gave zinc, as the sulfate salt, to additional patients.…”

Section: Zinc Therapymentioning

confidence: 99%