[A 22-year-old mother with severe pruritus and increasing jaundice two weeks after starting hormonal contraception].
Intermittent cholestatic liver disease may indicate an inherited deficiency of bile salt transport proteins. Episodes of cholestasis may start during pregnancy or during use of oral contraceptives or other medication. We describe the case of a 22-year-old mother with increasing jaundice and severe pruritus two weeks after starting hormonal contraception. A few months before she was suffering from intrahepatic cholestasis of pregnancy (ICP). Liver biopsy showed bland cholestasis with canalicular bile plugs. Treatment with ursodeoxycholic acid was not effective. Finally, rifampicin induced a complete remission of the cholestasis. Genetic testing showed a heterozygous mutation in the ABCB11 gene encoding the bile salt export pump (BSEP). Rifampicin activates nuclear receptors and may induce alternative pathways for the excretion of bile salts in patients with ABCB11 deficiency.
Medizinische Klinik II, Klinikum Worms gGmbH, Akademisches Lehrkrankenhaus der Universität Mainz, Germany.
C
C
Guth
Department of Gastroenterology and Hepatology, Tytgat Institute for Liver and Intestinal Research, Academic Medical Centre University of Amsterdam, Amsterdam, The Netherlands.
U
U
Beuers
Medizinische Klinik II, Klinikum Worms gGmbH, Akademisches Lehrkrankenhaus der Universität Mainz, Germany.
K
K
Beckh
D000328:Adult; D002779:Cholestasis / Q000150:complications* / Q000175:diagnosis*; D003278:Contraceptives, Oral, Hormonal / Q000008:administration & dosage / Q000009:adverse effects*; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D006801:Humans; D007565:Jaundice / Q000139:chemically induced* / Q000175:diagnosis; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis*; D011537:Pruritus / Q000139:chemically induced* / Q000175:diagnosis
D003278:Contraceptives, Oral, Hormonal