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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
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- 대한소아소화기영양학회 Pediatric Gastroenterology, Hepatology & Nutrition Pediatric Gastroenterology, Hepatology & Nutrition 제23권 제2호
- 발행연도
- 2020.1
- 수록면
- 174 - 179 (6page)
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We present an 8 years old girl who was diagnosed at 6 months of age of Progressive Familial Intrahepatic Cholestasis type 2. Although liver transplantation (LT) was classically considered curative for these patients, cholestasis recurrence with normal gamma-glutamyl transpeptidase (GGT), mediated by anti-bile salt export pump (BSEP) antibodies after LT (auto-antibody Induced BSEP Deficiency, AIBD) has been recently reported. Our patient underwent LT at 14 months. During her evolution, patient presented three episodes of acute rejection. Seven years after the LT, the patient presented pruritus with cholestasis and elevation of liver enzymes with persistent normal GGT. Liver biopsy showed intrahepatic cholestasis and giant-cell transformation with very low BSEP activity. Auto-antibodies against BSEP were detected therefore an AIBD was diagnosed. She was treated with Rituximab and immunoadsorption with resolution of the AIBD. As a complication of the treatment she developed a pneumocystis infection successfully treated with corticoids, cotrimoxazol and anidulafungin.
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참고문헌 (15)
참고문헌 신청
Strautnieks SS / 1998 / A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis / Nat Genet 20:233~238
Strautnieks SS / 2008 / Severe bile salt export pump deficiency : 82 different ABCB11 mutations in 109 families / Gastroenterology 134:1203~1214
Davit-Spraul A / 2009 / Progressive familial intrahepatic cholestasis / Orphanet J Rare Dis 4:1
Jansen PL / 1999 / Hepatocanalicular bile salt export pump deficiency in patients with progressive familial intrahepatic cholestasis / Gastroenterology 117:1370~1379
Alissa FT / 2008 / Update on progressive familial intrahepatic cholestasis / J Pediatr Gastroenterol Nutr 46:241~252
Strautnieks SS / 2008 / Severe bile salt export pump deficiency : 82 different ABCB11 mutations in 109 families / Gastroenterology 134:1203~1214
Davit-Spraul A / 2009 / Progressive familial intrahepatic cholestasis / Orphanet J Rare Dis 4:1
Jansen PL / 1999 / Hepatocanalicular bile salt export pump deficiency in patients with progressive familial intrahepatic cholestasis / Gastroenterology 117:1370~1379
Alissa FT / 2008 / Update on progressive familial intrahepatic cholestasis / J Pediatr Gastroenterol Nutr 46:241~252
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