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Liver Graft Failure and Bile Cast Nephropathy

Mrzljak A, Jurekovic , Novak R, Maksimovic B, Mikulic D, Galesic Ljubanovic D

The consequences of graft failure after liver transplantation (LT) range far beyond the liver. The kidneys are often affected, where persistent and progressive cholestasis can result in acute kidney injury...
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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

Quintero J, Juamperez , Gonzales E, Julio E, Mercadal-Hally M, Collado-Hilly M, Marín-Sánchez A, Charco R

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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Conceptual changes in small-for-size graft and small-for-size syndrome in living donor liver transplantation

Ikegami T, Kim JM, Jung DH, Soejima Y, Kim DS, Joh JW, Lee SG, Yoshizumi , Mori M

Early series in living donor liver transplantation (LDLT) in adults demonstrated a lower safe limit of graft volume standard liver volume ratio 25%–45%. A subsequent worldwide large LDLT series proposed...
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A Novel VPS33B Variant Identified by Exome Sequencing in a Patient with Arthrogryposis-Renal Dysfunction-Cholestasis Syndrome

Lee MJ, Suh CR, Shin JH, Lee JH, Lee Y, Eun BL, Yoo KH, Shim JO

Arthrogryposis-renal dysfunction-cholestasis (ARC) syndrome is a rare autosomal recessive multisystemic disease that is associated with the liver, kidney, skin, and central nervous and musculoskeletal systems. ARC occurs as a result...
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TNFα Induces Multidrug Resistance-Associated Protein 4 Expression through p38-E2F1-Nrf2 Signaling in Obstructive Cholestasis

Lian W, Liu X, Chen W

PURPOSE: To explore the molecular mechanism of the upregulation of multidrug resistance-associated protein 4 (MRP4) in cholestasis. MATERIALS AND METHODS: The mRNA and protein levels of MRP4 in liver samples from...
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A Case of Congenital Hepatoblastoma Presented with Hepatic Osteodystrophy Complicated by Multiple Bone Fractures

So YH, Kim DS, Yoon BA, Choi YD, Baek HJ, Kook H

Hepatic osteodystrophy is frequent complication in patients with chronic liver disease, particularly with chronic cholestasis. We report a male infant with congenital hepatoblastoma, who had osteodystrophy complicated by multiple bone...
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Novel ATP8B1 Gene Mutations in a Child with Progressive Familial Intrahepatic Cholestasis Type 1

Rhee ES, Kim YB, Lee S, Oh SH, Lee BH, Kim KM, Yoo HW

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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Progressive Familial Intrahepatic Cholestasis in Korea: A Clinicopathological Study of Five Patients

Kang HJ, Hong SA, Oh SH, Kim KM, Yoo HW, Kim GH, Yu E

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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A Case of Stevens-Johnson Syndrome with Vanishing Bile Duct Syndrome

An JH, Lee SG, Kim DH, Yoon MS, Lee HJ

  • KMID: 2451587
  • Korean J Dermatol.
  • 2019 Jun;57(5):258-261.
Stevens-Johnson syndrome (SJS) is a serious and potentially life-threatening disease. Vanishing bile duct syndrome (VBDS) is a rare cause of progressive cholestasis. Both syndromes are usually associated with the use...
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IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis

Tanaka A

Sclerosing cholangitis (SC) is defined as a condition with progressive stenosis and destruction of the bile ducts due to diffuse inflammation and fibrosis and currently includes three categories: primary sclerosing...
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Histopathological examination following side-by-side placement of metal stents for malignant hilar biliary obstruction

Nakagawara H, Yamao K, Gotoda T, Kikuta D, Takei A, Iwatsuka K, Takahashi , Ogawa M, Henmi A, Kobayashi , Moriyama M

BACKGROUND: Endoscopic-guided placement of metal stents for unresectable malignant hilar biliary obstruction (UMHBO) is performed using partial stent-in-stent or side-by-side (SBS) techniques. The latter involves placing sequential stents within the...
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Benign Recurrent Intrahepatic Cholestasis Type 2 in Siblings with Novel ABCB11 Mutations

Sohn MJ, Woo MH, Seong MW, Park SS, Kang GH, Moon JS, Ko JS

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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Vitamin D Improves Intestinal Barrier Function in Cirrhosis Rats by Upregulating Heme Oxygenase-1 Expression

Wang PF, Yao DH, Hu YY, Li Y

Intestinal barrier dysfunction always accompanies cirrhosis in patients with advanced liver disease and is an important contributor facilitating bacterial translocation (BT), which has been involved in the pathogenesis of cirrhosis...
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Effect of early and delay starting of enteral feeding in post-pancreaticoduodenectomy patients

Das BC, Haque M, Uddin MS, Nur-E-Elahi M, Khan ZR

BACKGROUNDS/AIMS: This study was undertaken to see the effect of early starting of enteral feeding after pancreatoduodenectomy (PD). The results were compared with existing nutritional practice in which enteral feeding...
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Huge Intramural Duodenal Hematoma Complicated with Obstructive Jaundice following Endoscopic Hemostasis

Kim HS, Kim HK, Kim WH, Hong SP, Cho JY

Intramural hematoma of the duodenum is a relatively unusual complication associated with the endoscopic treatment of bleeding peptic ulcers. Intramural hematomas are typically resolved spontaneously with conservative treatment alone. We...
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Imaging diagnosis of extrahepatic biliary tract obstruction with acquired portosystemic shunt in a cat

Hwang TS, Jang WS, Yoon YM, Jung DI, Lee HC

A 2-year-old, spayed female, Korean domestic short-hair cat was presented with depression and vomiting. The patient had history of weight loss lasting seven months. Physical examination revealed icterus in the...
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Mirror Syndrome Resulting from Metastatic Congenital Neuroblastoma to Placenta

Park SH, Namgoong J, Ko KN, Kim CJ, Lee PR, Jung E, Lee BS, Kim KS, Kim EA

Congenital neuroblastoma is a rare disease. Placental metastasis is extremely rare and poor prognosis has been reported in neonates. Mirror syndrome could occur in mother with placental metastasis with possibilities...
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Prospective Multicenter Study of the Challenges Inherent in Using Large Cell-Type Stents for Bilateral Stent-in-Stent Placement in Patients with Inoperable Malignant Hilar Biliary Obstruction

Yang MJ, Kim JH, Hwang JC, Yoo BM, Lee SH, Ryu JK, Kim YT, Woo SM, Lee WJ, Jeong S, Lee DH

BACKGROUND/AIMS: Although endoscopic bilateral stent-in-stent placement is challenging, many recent studies have reported promising outcomes regarding technical success and endoscopic re-intervention. This study aimed to evaluate the technical accessibility of...
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Clinical Usefulness of Direct/Total Bilirubin Ratio

Hur K, Park IK

BACKGROUND: The direct/total (d/t) bilirubin ratio can be used to distinguish the causes of jaundice in many patients who have increased levels of direct and indirect bilirubin. However, the reference...
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Evaluation of gallbladder and common bile duct size and appearance by computed tomography in dogs

Park HY, Cho YG, Lee YW, Choi HJ

The feasibility of using computed tomography (CT) to identify the common bile duct (CBD) and comparison with ultrasonography (US) results were evaluated in normal beagle dogs and dogs without hepatobiliary...
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