[Teratogenic risk during treatment of Wilson disease].
Untreated Wilson's disease usually causes infertility or abortion, as a result of increased intrauterine copper level. Therefore, a chelation treatment is necessary during the whole pregnancy. The most used is D-Penicillamine whose teratogenic risks such as cutis laxa, dermatopathy or complex mesenchyme abnormalities are paradoxically rare in the new borns of treated Wilson's disease mothers, perhaps owing to hypercupremia that protects the foetus from excessive copper deficiency. Yet, it's wise to reduce chelation treatment about a quarter fold and to add 50 mg vitamin B6 weekly as we did in our case whose child was born normal.
D000328:Adult; D000013:Congenital Abnormalities / Q000517:prevention & control; D003300:Copper / Q000032:analysis; D005260:Female; D005333:Fetus / Q000187:drug effects*; D006527:Hepatolenticular Degeneration / Q000628:therapy*; D006801:Humans; D007231:Infant, Newborn; D010396:Penicillamine / Q000008:administration & dosage / Q000009:adverse effects*; D011247:Pregnancy; D011736:Pyridoxine / Q000627:therapeutic use; D012306:Risk
D003300:Copper; D010396:Penicillamine; D011736:Pyridoxine
Risque tératogène au cours de la maladie de Wilson traitée.