지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2016.1
- 수록면
- 580 - 582 (3page)
이용수
초록· 키워드
오류제보하기
Bile Salt Export Pump (BSEP) Deficiency disease, including Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2), is a rare disease, usually leading within the first ten years to portal hypertension, liver failure, hepatocellular carcinoma. Often liver transplantation is needed. Sodium 4-phenylbutyrate (4-PB) seems to be a potential therapeutic compound for PFIC2. Psychiatric side effects in the adolescent population are little known and little studied since the drug used to treat children and infants. So we described a case of Caucasian boy, suffering from a late onset PFIC2, listed for a liver transplant when he was sixteen and treated with 4-FB (200 mg per kilogram of body weight per day). The drug was discontinued for the onset of bipolar and related disorders. This case illustrates possible psychiatric side effects of the drug.
목차
등록된 정보가 없습니다.
참고문헌 (24)
참고문헌 신청
Morotti RA / 2011 / Progressive familial intrahepatic cholestasis (PFIC) type 1, 2, and 3: a review of the liver pathology findings / Semin Liver Dis 31:3~10
Hori T / 2010 / Progressive familial intrahepatic cholestasis / Hepatobiliary Pancreat Dis Int 6:570~578
Soubrane O / 1990 / Orthotopic liver transplantation for Byler disease / Transplantation 50:804~806
Hayashi H / 2007 / 4-phenylbutyrate enhances the cell surface expression and the transport capacity of wild-type and mutated bile salt export pumps / Hepatology 45:1506~1516
Hasegawa Y / 2014 / Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1 / Orphanet J Rare Dis 9:89
Hori T / 2010 / Progressive familial intrahepatic cholestasis / Hepatobiliary Pancreat Dis Int 6:570~578
Soubrane O / 1990 / Orthotopic liver transplantation for Byler disease / Transplantation 50:804~806
Hayashi H / 2007 / 4-phenylbutyrate enhances the cell surface expression and the transport capacity of wild-type and mutated bile salt export pumps / Hepatology 45:1506~1516
Hasegawa Y / 2014 / Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1 / Orphanet J Rare Dis 9:89
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