title: Successful management of severe intrahepatic cholestasis of pregnancy: report of a first Japanese case.
abstract: BACKGROUND
  Intrahepatic cholestasis of pregnancy (ICP) is a cholestasis condition caused by elevated levels of serum bile acids that mainly occurs in the third trimester of pregnancy. Maternal symptoms include pruritus; elevation of transaminases, biliary enzymes, and bilirubin levels; and abnormal liver function tests. Fetal symptoms include spontaneous preterm labor, fetal distress, and intrauterine death. It is more prevalent in the Caucasians and is rarely found in Asian countries, including Japan. The etiology of ICP has been reported as involving various factors such as, environmental factors, hormone balance, and genetic components. The genetic factors include single-nucleotide polymorphisms (SNPs) in the genes of canalicular transporters, including ABCB4 and ABCB11. It has also been reported that the combination of these SNPs induces severe cholestasis and liver dysfunction.
  
  
  METHODS
  Here, we report for the first time a 24-year Japanese case of severe ICP diagnosed by typical symptoms, serum biochemical analysis, and treated with the administration of ursodeoxycholic acid which improved cholestasis and liver injury and prevented fetal death. The sequence analysis showed SNPs reported their association with ICP in the ABCB11 (rs2287622, V444A) and ABCB4 (rs1202283, N168N) loci.
  
  
  CONCLUSIONS
  The risk of ICP has been reported to be population-specific, and it is rare in the Japanese population. Our case was successfully treated with ursodeoxycholic acid and the genetic sequence analysis has supported the diagnosis. Because genetic variation in ABCB4 and ABCB11 has also been reported in the Japanese population, we need to be aware of potential ICP cases in pregnant Japanese women although further studies are necessary.
authors.affiliation: Division of Gastroenterology and Hepatology, Graduate School of Medical and Dental Sciences, Niigata University, 1-757 Asahimachido-ri, Chuo-ku, Niigata 951-8510, Japan. kenya-k@med.niigata-u.ac.jp.
authors.forename: Kenya
authors.initials: K
authors.lastname: Kamimura
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Abe
authors.forename: Naomi
authors.initials: N
authors.lastname: Kamimura
authors.forename: Masayuki
authors.initials: M
authors.lastname: Yamaguchi
authors.forename: Maiko
authors.initials: M
authors.lastname: Mamizu
authors.forename: Kanna
authors.initials: K
authors.lastname: Ogi
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Takahashi
authors.forename: Ken-Ichi
authors.initials: K
authors.lastname: Mizuno
authors.forename: Hiroteru
authors.initials: H
authors.lastname: Kamimura
authors.forename: Yuji
authors.initials: Y
authors.lastname: Kobayashi
authors.forename: Manabu
authors.initials: M
authors.lastname: Takeuchi
authors.forename: Kunihiko
authors.initials: K
authors.lastname: Yoshida
authors.forename: Kyoko
authors.initials: K
authors.lastname: Yamada
authors.forename: Takayuki
authors.initials: T
authors.lastname: Enomoto
authors.forename: Koichi
authors.initials: K
authors.lastname: Takakuwa
authors.forename: Minoru
authors.initials: M
authors.lastname: Nomoto
authors.forename: Miki
authors.initials: M
authors.lastname: Obata
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Katsuragi
authors.forename: Yukio
authors.initials: Y
authors.lastname: Mishima
authors.forename: Ryo
authors.initials: R
authors.lastname: Kominami
authors.forename: Tomoteru
authors.initials: T
authors.lastname: Kamimura
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Aoyagi
mesh_terms: D044466:Asian People; D002756:Cholagogues and Choleretics / Q000627:therapeutic use*; D002780:Cholestasis, Intrahepatic / Q000188:drug therapy*; D005260:Female; D006801:Humans; D011247:Pregnancy; D011248:Pregnancy Complications / Q000188:drug therapy*; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid