- A case of benign recurrent intrahepatic cholestasis
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Park JH, Sim SS, Kim SY, Jeon HJ, Kim CY
- KMID: 1691579
- J Korean Pediatr Soc.
- 1991 Dec;34(12):1745-1752.
No abstract available. -
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- Familial Benign Recurrent Intrahepatic Cholestasis
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Lee JK, Rhee PL, Lee JH, Lee KT, choi SH, Noh JH, Kim JJ, Koh KC, Paik SW, Rhee JC
- KMID: 2240694
- Korean J Gastroenterol.
- 1997 Apr;29(4):549-553.
Benign recurrent intrahepatic cholestasis(BRIC) is a very rare cholestatic condition of unknown etiology and its main clinical feature is multiple recurrent episodes of cholestasis with no extrahepatic bile duct obstruction.... -
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- A Case of Fetal Cholelithiasis Related to Maternal Intrahepatic Cholestasis of Pregnancy
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Yon DK, An JW, Kim JH, Jeon JH, Heo JS
- KMID: 2379661
- Neonatal Med.
- 2017 May;24(2):92-96.
- doi: 10.5385/nm.2017.24.2.92
Despite the improved accuracy and increasing use of prenatal ultrasonography, fetal cholelithiasis is a rarely detected disease, and its natural history and clinical significance are not yet well defined. Many... -
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- A Case of Nonfamilial Benign Recurrent Intrahepatic Cholestasis
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Kim OY, Sung BY, Kowg GD, Yoon HS, Shin YM, Oh HT, Kim MK, Kim HK, Kim JH, Ri KU, Shon HI
- KMID: 2143307
- Korean J Hepatol.
- 1998 Jun;4(2):188-193.
Benign recurrent intrahepatic cholestasis (BRIC) is a rare desease, which usually manifests between the age of 10 and 20. Its main clinical feature is multiple recurrent episodes of cholestasis without extrahepatic bile... -
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- Novel ATP8B1 Gene Mutations in a Child with Progressive Familial Intrahepatic Cholestasis Type 1
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Rhee ES, Kim YB, Lee S, Oh SH, Lee BH, Kim KM, Yoo HW
- KMID: 2457750
- Pediatr Gastroenterol Hepatol Nutr.
- 2019 Sep;22(5):479-486.
- doi: 10.5223/pghn.2019.22.5.479
Progressive familial intrahepatic cholestasis (PFIC) is a group of severe genetic disorders, inherited in an autosomal recessive manner, causing cholestasis of hepatocellular origin, later progressing to biliary cirrhosis and liver... -
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- The etiologies of neonatal cholestasis
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Ko JS, Seo JK
- KMID: 2140811
- Korean J Pediatr.
- 2007 Sep;50(9):835-840.
- doi: 10.3345/kjp.2007.50.9.835
Any infant noted to be jaundiced at 2 weeks of age should be evaluated for cholestasis with measurement of total and direct serum bilirubin. With the insight into the clinical... -
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- Presentation of Progressive Familial Intrahepatic Cholestasis Type 3 Mimicking Wilson Disease: Molecular Genetic Diagnosis and Response to Treatment
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Boga S, Jain D, Schilsky M
- KMID: 2068799
- Pediatr Gastroenterol Hepatol Nutr.
- 2015 Sep;18(3):202-208.
- doi: 10.5223/pghn.2015.18.3.202
Progressive familial intrahepatic cholestasis type 3 (PFIC3) is an autosomal recessive disorder of cholestasis of hepatocellular origin, typically seen in infancy or childhood caused by a defect in the ABCB4... -
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- Increased Microfilaments in Hepatocytes and Biliary Ductular Cells in Cholestatic Liver Diseases
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Chung KW, Han NI, Choi SW, Ahn BM, Yoon SK, Nam SW, Lee YS, Han JY, Sun HS
- KMID: 648790
- J Korean Med Sci.
- 2002 Dec;17(6):795-800.
- doi: 10.3346/jkms.2002.17.6.795
To assess the extent of microfilaments in cholestatic liver diseases we examined the cytoplasmic microfilaments in intrahepatic and extrahepatic cholestasis in man by electron microscopy. Study subjects were two... -
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- Dermatoses of Pregnancy: Clues to Diagnosis, Fetal Risk and Therapy
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Ambros-Rudolph CM
- KMID: 2171915
- Ann Dermatol.
- 2011 Aug;23(3):265-275.
- doi: 10.5021/ad.2011.23.3.265
The specific dermatoses of pregnancy represent a heterogeneous group of pruritic skin diseases that have been recently reclassified and include pemphigoid (herpes) gestationis, polymorphic eruption of pregnancy (syn. pruritic urticarial... -
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- Benign Recurrent Intrahepatic Cholestasis with a Single Heterozygote Mutation in the ATP8B1 Gene
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Lee YS, Kim MJ, Ki CS, Lee YM, Lee Y, Choe YH
- KMID: 2315331
- Pediatr Gastroenterol Hepatol Nutr.
- 2012 Jun;15(2):122-126.
Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive inherited disorder characterized by multiple recurrent episodes of severe cholestatic jaundice without obstruction of extrahepatic bile duct. We present the... -
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- Early Diagnosis of ABCB11 Spectrum Liver Disorders by Next Generation Sequencing
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Lee SJ, Kim JE, Choe BH, Seo AN, Bae HI, Hwang SK
- KMID: 2384825
- Pediatr Gastroenterol Hepatol Nutr.
- 2017 Jun;20(2):114-123.
- doi: 10.5223/pghn.2017.20.2.114
PURPOSE: The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. METHODS: Fifty patients... -
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- Benign Recurrent Intrahepatic Cholestasis Type 2 in Siblings with Novel ABCB11 Mutations
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Sohn MJ, Woo MH, Seong MW, Park SS, Kang GH, Moon JS, Ko JS
- KMID: 2440595
- Pediatr Gastroenterol Hepatol Nutr.
- 2019 Mar;22(2):201-206.
- doi: 10.5223/pghn.2019.22.2.201
Benign recurrent intrahepatic cholestasis (BRIC), a rare cause of cholestasis, is characterized by recurrent episodes of cholestasis without permanent liver damage. BRIC type 2 (BRIC2) is an autosomal recessive disorder... -
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- Predictive Factors for Prognosis of Neonatal Intrahepatic Cholestasis: Non-Familial, Non-Metabolic, Non-Syndromic Cholestasis
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Kim HS, Lee CH, Kim IJ, Park JH
- KMID: 2275203
- Korean J Pediatr Gastroenterol Nutr.
- 2004 Sep;7(2):208-214.
PURPOSE: The prognosis of neonates with cholestasis is not clear. Some factors, such as high peak bilirubin levels and liver histologic findings have been claimed to affect the prognosis adversely.... -
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- Neonatal Intrahepatic Cholestasis Caused by Citrin Deficiency in Korean Infants
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Ko JS, Song JH, Park SS, Seo JK
- KMID: 1785752
- J Korean Med Sci.
- 2007 Dec;22(6):952-956.
- doi: 10.3346/jkms.2007.22.6.952
Citrin is a liver-type mitochondrial aspartate-glutamate carrier encoded by the SLC25A13 gene, and its deficiency causes adult-onset type II citrullinemia and neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). Here,... -
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- Comparison of Tc-99m DISIDA Hepatobiliary Scintigraphy and Percutaneous Needle Biopsy in the Diagnosis of Biliary Atresia from Intrahepatic Cholestasis
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Kim WS, Park WH, Choi SO, Kim SP
- KMID: 2422262
- J Korean Assoc Pediatr Surg.
- 1997 Jun;3(1):6-14.
- doi: 10.13029/jkaps.1997.3.1.6
To differentiate biliary atresia from intraheaptic cholestasis, Tc-99m DISIDA hepatobiliary scintigraphies and percutaneous needle biopsies of 60 consecutive infants were evaluated retrospectively. Twenty three patients had biliary atresia and 37... -
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- A Case of Idiopathic Adulthood Ductopenia
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Jang HJ, Kim JH, Hong SJ, Kae SH, Chae SW, Sohn JH, Lee J
- KMID: 1864978
- Korean J Gastroenterol.
- 2002 Mar;39(3):233-237.
Idiopathic adulthood ductopenia (IAD), named about 10 years ago, is a chronic cholestatic entity of unknown origin that affects predominantly young and middle-aged adult. IAD should be considered in patients... -
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- Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient
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Quintero J, Juamperez , Gonzales E, Julio E, Mercadal-Hally M, Collado-Hilly M, MarÃn-Sánchez A, Charco R
- KMID: 2471627
- Pediatr Gastroenterol Hepatol Nutr.
- 2020 Mar;23(2):174-179.
- doi: 10.5223/pghn.2020.23.2.174
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... -
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- Fibrosing cholestatic hepatitis: a report of three cases
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Lee HK, Yoon GS, Min KS, Jung YW, Lee YS, Suh DJ, Yu E
- KMID: 1731246
- J Korean Med Sci.
- 2000 Feb;15(1):111-114.
- doi: 10.3346/jkms.2000.15.1.111
Fibrosing cholestatic hepatitis is an aggressive and usually fatal form of viral hepatitis in immunosuppressed patients. We report three cases of fibrosing cholestatic hepatitis in various clinical situations. Case 1... -
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- Progressive Familial Intrahepatic Cholestasis in Korea: A Clinicopathological Study of Five Patients
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Kang HJ, Hong SA, Oh SH, Kim KM, Yoo HW, Kim GH, Yu E
- KMID: 2454605
- J Pathol Transl Med.
- 2019 Jul;53(4):253-260.
- doi: 10.4132/jptm.2019.05.03
BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of autosomal recessive liver diseases that present as neonatal cholestasis. Little is known of this disease in Korea. METHODS: The records... -
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- Bipolar and Related Disorders Induced by Sodium 4-Phenylbutyrate in a Male Adolescent with Bile Salt Export Pump Deficiency Disease
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Vitale G, Simonetti , Pirillo M, Taruschio , Andreone P
- KMID: 2354482
- Psychiatry Investig.
- 2016 Sep;13(5):580-582.
- doi: 10.4306/pi.2016.13.5.580
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... -
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