Pregnancy

Liver Diseases in Pregnancy
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Wilson Disease:
Wilson disease is an inherited recessive disorder of
copper transport in the golgi apparatus of the hepatocyte.
It leads to the accumulation of copper in the liver,
brain and other organs. It is a disease of the young thus
affecting women of childbearing age. In the absence
of treatment, cirrhosis of the liver, fulminant hepatic
failure and advanced psychiatric and neurologic damage
may occur. While fertility is decreased in patients
with Wilson disease, women have become pregnant
when appropriately diagnosed and treated. Treatment
should be initiated as soon as diagnosis is made during
pregnancy and regardless of the stage of pregnancy.
Treatment should be maintained throughout pregnancy
because its interruption could result in fulminant
liver failure. Dose reduction in the order of 25-50%
of pre-pregnancy maintenance therapy for both chelators
D- penicillamine and trientene is required so that
copper is not depleted from the body and wound healing
is not impaired if C-section is to be performed. 45
In addition, Copper depletion may be associated with
congenital malformations.
While fertility is decreased
in patients with Wilson
disease, women have become
pregnant when appropriately
diagnosed and treated.
Trientene is FDA category C and its dose should be
decreased to less than 500mg /day in pregnancy.
Zinc sulfate (FDA Category C) is probably safe during
pregnancy and breastfeeding. It is usually given at
a dose of 50 mg orally three times daily and does not
require dose adjustment. 49
Primary Biliary Cirrhosis and Primary Sclerosing
Cholangitis:
A few cases of pregnancy have been described in
women with primary biliary cirrhosis (PBC). This is
partly due to the later age at presentation of the disease.
Although reports suggest an increased risk for premature
delivery, still births and liver failure, there are
no good data on the outcome of pregnancy in women
with PBC. When pregnancy occurs, PBC may induce
a new onset pruritus. Diagnosis and management are
similar to the non pregnant state. Upper endoscopy is
recommended 50 in order to screen for esophageal varices.
Nonselective beta blockers and banding ligation
should be given if necessary. Ursodeoxycholic acid or
UDCA (FDA category B) is the treatment of choice
due to its safety as discussed earlier.
Primary sclerosing cholangitis is rarely described in
pregnancy. Abdominal pain and pruritus seem to be
the major symptoms in pregnancy. Diagnosis and
therapy are similar to the non pregnant state.
Budd-Chiari Syndrome:
D-penicillamine is FDA Category D. It has been associated
with teratogenic abnormalities in 5% of infants
(cutis laxa, micrognathia, low-set ears, inguinal
hernias, and joint mobility). 46 The suggested dose in
pregnancy is