Can a woman who has Dubin-Johnson syndrome (DJS) use hormonal contraception?
My patient is 38 years old and her liver function test results are normal. She wants to use the progestogen-only contraceptive. This is a difficult but fascinating question. DJS is a rare autosomal recessive disorder manifested by chronic conjugated hyperbilirubinemia. DJS has recently been found to be associated with mutations in the multidrug resistance protein 2 (MRP2) gene. MRP2 is a glycoprotein in the apical membrane of hepatocytes, and it transports anionic conjugates from hepatocytes to the bile. As well as conjugated bilirubin, this protein transports oestrogens and progesterone metabolites. This explains why the jaundice of DJS increases during pregnancy and when taking the combined oral contraceptive Pill (COCP). However, there are many other bile acid transporters, so the presence of exogenous oestrogens or progesterone metabolites should not cause cholestasis or liver damage, even though it may lead to an increase in jaundice. Also, the oestrogens or progesterone metabolit
