Wilsonʼs Disease Treated with Trientine during Pregnancy

Devesa, Roque; Álvarez, Adoración Blanco; Heras, G. de las; Miguel, J.R. de

doi:10.1097/00005176-199501000-00018

Cited by 34 publications

(15 citation statements)

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“…There are also single case reports from Spain 46 and from France. 47 In the Spanish case, a 22-year-old woman with weeks failed, and a cesarean section had to be performed, with delivery of a healthy baby.…”

Section: Discussionmentioning

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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Section: Discussionmentioning

confidence: 99%

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

et al. 2000

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“…Thus, women who got pregnant have generally good copper control, and it is important to maintain copper balance during the whole pregnancy to prevent acute liver failure or emergence of new neurologic deficits. Successful pregnancies have been reported on trientine and d -penicillamine 75–77. Dose reduction of chelating medications is recommended in the range of 25%–50% with urine copper monitoring on a monthly basis until the delivery 8…”

Section: Treatment Of Wdmentioning

confidence: 99%

Update on the clinical management of Wilson's disease

Hedera

2017

TACG

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Wilson’s disease (WD), albeit relatively rare, is an important genetic metabolic disease because of highly effective therapies that can be lifesaving. It is a great imitator and requires a high index of suspicion for correct and timely diagnosis. Neurologic, psychiatric and hepatologic problems in WD are very nonspecific, and we discuss the most common clinical phenotypes. The diagnosis remains laboratory based, and here we review the most important challenges and pitfalls in laboratory evaluation of WD, including the emerging role of genetic testing in WD diagnosis. WD is a monogenic disorder but has very high allelic heterogeneity with >500 disease-causing mutations identified, and new insights into phenotype–genotype correlations are also reviewed. The gold standard of therapy is chelation of excessive copper, but many unmet needs exist because of possible clinical deterioration in treated patients and potential adverse effects associated with currently available chelating medications. We also review the most promising novel therapeutic approaches, including chelators targeting specific cell types, cell transplantation and gene therapy.

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“…2.1.16 Additional antiphlogistics and antirheumatics Trientine is also used for the treatment of Wilson's disease. No adverse pregnancy outcomes were observed in a total of 13 women who were treated throughout their whole pregnancy with trientine (Devesa 1995). As trientine may cause anemia due to iron deficiency, iron should be supplemented during treatment.…”

Section: Pharmacology and Toxicologymentioning

confidence: 96%