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March 14, 1966

Wilson's Disease (Hepatolenticular Degeneration) and Pregnancy

Author Affiliations

From the Division of Neurology, University of Virginia School of Medicine, and the Neurological Service, University of Virginia Hospital, Charlottesville. Dr. McKinney is now at the Bowman Gray School of Medicine, Winston-Salem, NC.

JAMA. 1966;195(11):960-962. doi:10.1001/jama.1966.03100110128043

SUCCESSFUL PREGNANCY punctuating Wilson's disease (hepatolenticular degeneration) has been rarely reported.1-5 Most case reports on women in the child-bearing age mention scanty and irregular menses, and it is further likely that the early development of tremor or cirrhosis might constitute a bar to marriage and pregnancy.

Both Wilson's disease and pregnancy are associated with alteration in copper metabolism, in particular, that of ceruloplasmin. While this protein is markedly reduced in most cases of Wilson's disease, it is usually increased in pregnancy and by the administration of estrogens.6,7 In pregnant patients with Wilson's disease, ceruloplasmin was found by Sternlieb and Scheinberg5 to rise to almost normal concentrations, but other observers2 found no significant increase. Uniformly, however, there appears to have been significant clinical improvement in the severity of Wilson's disease during, and in the months following, pregnancy.

The following case report describes a patient with long-standing Wilson's

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