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Wilson’s Disease

  • Stephanie I. ByerlyEmail author
Chapter

Abstract

Wilson’s disease (hepatolenticular degeneration) is a rare autosomal recessive inherited disorder with abnormal hepatic metabolism and biliary excretion of copper. Symptoms usually appear in the second and third decades of life. The liver can be affected to varying degrees, but the disease can also affect the renal, cardiac, and neurologic systems. Additionally, patients may experience pancreatitis, hypothyroidism, and infertility.

The progression of Wilson’s disease does not seem to be affected by pregnancy, and some patients may go into remission. Women must be maintained on treatment throughout their pregnancies; otherwise fulminant liver failure may occur. Treatments include d-penicillamine, trientine, zinc, antioxidants, and diet.

Anesthetic choice will be based on current status and organ involvement of the patient. Regional anesthesia is the preferred method for labor, vaginal delivery, and cesarean section in patients without coagulopathy.

Keywords

Wilson’s disease Ceruloplasmin Copper Pregnancy Obstetrical Anesthesia Regional General 

References

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Anesthesiology and Pain ManagementUniversity of Texas SouthwesternDallasUSA

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