- Alagille Syndrome with Multiple Xanthomas
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Byun SY, Kwon SH, Kim BR, Park JT, Park KC, Youn SW, Na JI, Huh CH
- KMID: 2246256
- Korean J Dermatol.
- 2015 Jan;53(1):71-72.
No abstract available. -
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- Alagille Syndrome Mimicking Biliary Atresia Confirmed by Jagged1 (JAG1) Gene Analysis in a Newborn: A Case Report
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Yun HJ, Cho CJ, Kim YW, Kim EY, Cho HM, Kim Y, Jang HI, Kim KS
- KMID: 2388979
- Neonatal Med.
- 2017 Aug;24(3):140-144.
- doi: 10.5385/nm.2017.24.3.140
Alagille syndrome is a complex autosomal dominant disorder secondary to defects in the Notch signaling pathway, primarily caused by mutations in the Jagged1 (JAG1) gene. The liver, heart, skeleton, face... -
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- Jagged1 mutation analysis in Alagille syndrome patients
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Ko JS, Yang HR, Kim KM, Seo JK
- KMID: 2278564
- Korean J Pediatr.
- 2006 May;49(5):519-522.
- doi: 10.3345/kjp.2006.49.5.519
PURPOSE: Alagille syndrome is an autosomal dominant disorder with developmental abnormalities affecting the liver, heart, eyes, vertebrae, and craniofacial region. The Jagged1(JAG1) gene, which encodes a ligand of Notch, has... -
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- Clinical manifestation and outcome of alagille syndrome in Korea: a study of 30 patients
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Shin JY, Kim YJ, Seo JK
- KMID: 2279783
- Korean J Pediatr.
- 2006 Oct;49(10):1067-1072.
- doi: 10.3345/kjp.2006.49.10.1067
PURPOSE: The purpose of this study was to examine the clinical courses and long-term outcomes of children with Allagille syndrome in Korea, and to evaluate the prognostic potentials of identified... -
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- A Case of Alagille Syndrome with Atresia of the Hepatic Duct
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Kim HS, Koh H, Chung KS, Oh JT, Park YN, Kim MJ
- KMID: 2275317
- Korean J Pediatr Gastroenterol Nutr.
- 2008 Mar;11(1):65-69.
A two-month-old baby had acholic stool, neonatal hyperbilirubinemia and congenital heart disease. Atresia of the hepatic duct was confirmed by open cholangiography, which showed a non-opacified intrahepatic bile duct. Liver... -
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- Alagille Syndrome: A Case Report
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Shim H, Park C, Kim SI, Park YN
- KMID: 2275528
- Korean J Pathol.
- 2004 Feb;38(1):56-59.
Alagille syndrome is a rare autosomal dominant disorder showing complicated systemic manifestations, although the hepatic manifestations are predominant in many patients. We report a case of Alagille syndrome in a... -
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- A Case of Alagille Syndrome
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Kim ES, Lee DW, Chung KS, Kim SI, Park YN
- KMID: 2275176
- Korean J Pediatr Gastroenterol Nutr.
- 2002 Sep;5(2):192-198.
Alagille syndrome is characterized by paucity of interlobular bile ducts, chronic cholestasis, characteristic facial abnormalities, cardiovascular abnormalities, posterior embryotoxon, vertebral arch defects, skeletal abnormalities, and glomerular renal involvement. We experienced... -
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- A Case of Alagille Syndrome
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Hwang EK, Lee GH, Ryoo E, Cho KH, Kim GH, Lee HS, Kim JH, Park SH, Kim HS
- KMID: 2207959
- J Korean Pediatr Soc.
- 1998 Mar;41(3):410-414.
Alagille syndrome is characterized by chronic cholestasis, posterior embryotoxon, skeletal abnormalities, cardiovascular abnormalities, and a typical face with prominent forehead and pointed chin. Its histological feature includes paucity of interlobular... -
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- Clinical Evaluation of Syndromic and Nonsyndromic Intrahepatic Bile Duct Paucity
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Han SJ, Choi BH, Kang KH, Kim KM
- KMID: 1557090
- Korean J Pediatr Gastroenterol Nutr.
- 1999 Sep;2(2):178-184.
PURPOSE: The aims of this study were to evaluate the clinical manifestations and prognosis of the syndromic and nonsyndromic intrahepatic bile duct paucity (IHBDP). METHODS: We studied histology of 42 infants... -
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- A Case of Alagille's Syndrome
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Kim S, Choe WS, Chun YS, Yoon HK
- KMID: 2078706
- Korean J Nucl Med.
- 2000 Apr;34(2):154-158.
This is a case report of a 5-month-old male who was brought in to hospital for evaluation of jaundice from birth. The baby had a history of ileal atresia operated 2... -
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- A case of Alagille syndrome presenting with chronic cholestasis in an adult
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Kim J, Yang B, Paik N, Choe YH, Paik YH
- KMID: 2400673
- Clin Mol Hepatol.
- 2017 Sep;23(3):260-264.
- doi: 10.3350/cmh.2016.0057
Alagille syndrome (AGS) is a complex multisystem disorder that involves mainly the liver, heart, eyes, face, and skeleton. The main associated clinical features are chronic cholestasis due to a paucity... -
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- Outcome of Alagille Syndrome Patients Who Had Previously Received Kasai Operation during Infancy: A Single Center Study
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Lee HP, Kang B, Choi SY, Lee S, Lee SK, Choe YH
- KMID: 2068794
- Pediatr Gastroenterol Hepatol Nutr.
- 2015 Sep;18(3):175-179.
- doi: 10.5223/pghn.2015.18.3.175
PURPOSE: Infants with Alagille syndrome (AGS) are occasionally misdiagnosed as biliary atresia and subsequently undergo Kasai operation. The purpose of this study was to investigate the outcome of patients with... -
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- Alagille syndrome and a JAG1 mutation: 41 cases of experience at a single center
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Ahn KJ, Yoon JK, Kim GB, Kwon BS, Go JM, Moon JS, Bae EJ, Noh CI
- KMID: 2085448
- Korean J Pediatr.
- 2015 Oct;58(10):392-397.
- doi: 10.3345/kjp.2015.58.10.392
PURPOSE: Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and... -
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- Cholestasis beyond the Neonatal and Infancy Periods
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Khalaf R, Phen C, Karjoo S, Wilsey M
- KMID: 2160470
- Pediatr Gastroenterol Hepatol Nutr.
- 2016 Mar;19(1):1-11.
- doi: 10.5223/pghn.2016.19.1.1
Cholestasis results from impairment in the excretion of bile, which may be due to mechanical obstruction of bile flow or impairment of excretion of bile components into the bile canaliculus.... -
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- A Case of Nonsyndromic Paucity of Interlobular Bile Ducts in Down Syndrome
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Chang CH, Kim JH, Lee SJ, Lee DS, Kim DK, Choi SM, Kim WT, Jang TJ
- KMID: 2041990
- J Korean Pediatr Soc.
- 1999 Jun;42(6):858-862.
The nonsyndromic paucity of interlobular bile ducts, which belongs to intrahepatic biliary atresia, is characterized by conjugated hyperbilirubinemia, suggesting cholestasis in newborn infants it has little relationship with extrahepatic congenital... -
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