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Best Practice & Research: Clinical Gastroenterology, ISSN 1521-6918, 2010, Volume 24, Issue 5, pp. 573 - 584
Abstract Fibrocystic diseases affecting the liver and often also other organs like the kidneys are a clinically and genetically heterogeneous group of... 
Gastroenterology and Hepatology | PRKCSH | Joubert syndrome | Ciliopathies | Peribiliary cysts | Bardet-Biedl syndrome | PKD2 | PKD1 | PKHD1 | Early and severe disease manifestation | BBS | Autosomal dominant polycystic liver disease (PCLD) | JBTS | von Meyenburg complexes | Congenital hepatic fibrosis | SEC63 | Cilia | Biliary hamartoma | Fibrocystic diseases | Choledochal cysts | Jeune syndrome | Variable disease expression | MKS | Autosomal dominant polycystic kidney disease (ADPKD) | Caroli’s disease | Meckel-Gruber syndrome | Autosomal recessive polycystic kidney disease (ARPKD) | Ductal plate malformation | Caroli's disease | MOLECULAR CHARACTERIZATION | QUALITY-CONTROL | PCLD | SECRETORY PATHWAY | IMAGING EVALUATION | Autosomal dominant polycystic liver disease | INTRAHEPATIC PERIBILIARY GLANDS | BILE-DUCT CYSTS | PKHD1 MUTATIONS | GLUCOSIDASE-II | POLYCYSTIC KIDNEY-DISEASE | SURGICAL-MANAGEMENT | Caroli s disease | GASTROENTEROLOGY & HEPATOLOGY | Liver Diseases - genetics | Humans | Choledochal Cyst - diagnostic imaging | Liver Diseases - diagnostic imaging | Biliary Tract Diseases - diagnosis | Choledochal Cyst - classification | Polycystic Kidney, Autosomal Dominant - genetics | Cysts - diagnostic imaging | Liver Diseases - congenital | Polycystic Kidney, Autosomal Dominant - diagnosis | Liver - diagnostic imaging | Cysts - congenital | Ultrasonography | Polycystic Kidney, Autosomal Dominant - physiopathology | Liver Diseases - physiopathology | Bile Ducts, Intrahepatic - embryology | Hamartoma - diagnosis | Liver diseases | Genetic disorders
Journal Article
世界胃肠病学杂志:英文版, ISSN 1007-9327, 2017, Volume 23, Issue 30, pp. 5499 - 5507
AIM To develop a MRI-based method for accurate determination of liver volume(LV) and to explore the effect of long-term everolimus(EVR) treatment on LV in PCK... 
mTOR inhibitor | Somatostatin analogue | Magnetic resonance imaging | Fibrocystic liver disease | Liver volume measurement | KIDNEY-DISEASE | VOLUME | liver volume measurement | somatostatin analogue | INDIVIDUAL PATIENT DATA | MTOR | INHIBITION | SOMATOSTATIN | PCK RAT MODEL | POOLED ANALYSIS | GASTROENTEROLOGY & HEPATOLOGY | PROGRESSION | SIROLIMUS | Cysts - drug therapy | Liver - pathology | Phosphorylation | TOR Serine-Threonine Kinases - metabolism | Humans | Liver Diseases - pathology | Liver Diseases - diagnostic imaging | Gene Expression Profiling | Phosphatidylinositol 3-Kinases - metabolism | Hypertension, Portal - prevention & control | TOR Serine-Threonine Kinases - antagonists & inhibitors | Liver Cirrhosis - diagnostic imaging | Liver - drug effects | Liver - diagnostic imaging | Everolimus - therapeutic use | Female | Drug Evaluation, Preclinical | Proto-Oncogene Proteins c-akt - metabolism | Real-Time Polymerase Chain Reaction | Disease Models, Animal | Liver Cirrhosis - drug therapy | Somatostatin - analogs & derivatives | Rats | Random Allocation | Cysts - pathology | Magnetic Resonance Imaging | Animals | Signal Transduction - drug effects | Cysts - diagnostic imaging | Ribosomal Protein S6 - metabolism | Liver Cirrhosis - pathology | Peptides, Cyclic - therapeutic use | Liver Diseases - drug therapy | Somatostatin - therapeutic use | Basic Study
Journal Article
Gastroenterology, ISSN 0016-5085, 2013, Volume 144, Issue 1, pp. 112 - 121.e2
Background & Aims Autosomal recessive polycystic kidney disease (ARPKD), the most common ciliopathy of childhood, is characterized by congenital hepatic... 
Gastroenterology and Hepatology | Noncirrhotic Portal Hypertension | Genetics | Hepatorenal Fibrocystic Disease | Ductal Plate Malformation | ENCODES | LIVER VOLUME | LENGTH | INTRAHEPATIC BILE-DUCTS | CHILDREN | PKHD1 | SPLEEN | NORMAL VALUES | INFANTS | OUTCOMES | GASTROENTEROLOGY & HEPATOLOGY | Liver Transplantation | Hypertension, Portal - physiopathology | Ultrasonography, Doppler, Color | Hypertension, Portal - complications | gamma-Glutamyltransferase - blood | Humans | Middle Aged | Prothrombin Time | Child, Preschool | Infant | Male | Esophageal and Gastric Varices - etiology | Liver Cirrhosis - congenital | Splenomegaly - diagnostic imaging | Polycystic Kidney, Autosomal Recessive - genetics | Young Adult | Liver Cirrhosis - diagnostic imaging | Polycystic Kidney, Autosomal Recessive - complications | Adult | Female | Child | Liver Cirrhosis - genetics | Alkaline Phosphatase - blood | Severity of Illness Index | Endoscopy, Gastrointestinal | Organ Size | Kidney Transplantation | Serum Albumin | Platelet Count | Portal Pressure | Adolescent | Liver Cirrhosis - pathology | Cholangiopancreatography, Magnetic Resonance | Hypertension, Portal - blood | Mutation | Receptors, Cell Surface - genetics | Hypertension | Medical examination | Liver diseases | Genetic disorders | Aspartate | Thrombin | Diagnostic imaging | Fatty acids | Blood | Fibrosis | Medical genetics | Prothrombin | Polycystic kidney disease | Protein binding
Journal Article
American Journal of Physiology - Gastrointestinal and Liver Physiology, ISSN 0193-1857, 06/2018, Volume 314, Issue 6, pp. G677 - G689
Journal Article
Journal Article
Pediatric Nephrology, ISSN 0931-041X, 1/2016, Volume 31, Issue 1, pp. 113 - 119
Hepatorenal fibrocystic diseases (HRFCDs) are a group of monogenic disorders characterized by developmental abnormalities involving the liver and kidney. In... 
Pediatrics | Autosomal-recessive polycystic kidney disease | Ciliopathy | Nephrology | Nephronophthisis 13 | Medicine & Public Health | Congenital hepatic fibrosis | Caroli disease | Urology | Hepatorenal fibrocystic disease | GENOTYPE-PHENOTYPE CORRELATION | DIAGNOSIS | MANAGEMENT | CILIOPATHIES | POLYCYSTIC KIDNEY-DISEASE | DISORDERS | MECHANISMS | LIVER-DISEASE | CILIA | PATHOGENESIS | UROLOGY & NEPHROLOGY | PEDIATRICS | Polycystic Kidney Diseases - genetics | Predictive Value of Tests | Polycystic Kidney Diseases - diagnosis | Genetic Testing | Prognosis | Age Factors | Encephalocele - epidemiology | Humans | Genetic Diseases, Inborn - genetics | Child, Preschool | Caroli Disease - diagnosis | Infant | Male | Genetic Diseases, Inborn - epidemiology | Polycystic Kidney, Autosomal Recessive - diagnosis | Caroli Disease - genetics | Polycystic Kidney, Autosomal Recessive - genetics | Liver Cirrhosis - epidemiology | Renal Insufficiency, Chronic - epidemiology | Young Adult | Caroli Disease - epidemiology | Adult | Female | Republic of Korea - epidemiology | Child | Genetic Diseases, Inborn - diagnosis | Liver Cirrhosis - genetics | Encephalocele - genetics | Ciliary Motility Disorders - epidemiology | Encephalocele - diagnosis | Genetic Predisposition to Disease | Liver Cirrhosis - diagnosis | Risk Factors | Polycystic Kidney, Autosomal Recessive - epidemiology | Phenotype | Kidney Failure, Chronic - epidemiology | Adolescent | Ciliary Motility Disorders - diagnosis | Polycystic Kidney Diseases - epidemiology | Ciliary Motility Disorders - genetics
Journal Article
Intractable & Rare Diseases Research, ISSN 2186-3644, 05/2019, Volume 8, Issue 2, pp. 154 - 157
Goldston syndrome (GS) is a rare association of Dandy-Walker malformation (DWM) and cystic renal dysplasia with or without hepatic fibrosis. It is considered... 
Dandy walker malformation | Fibrocystic disease of liver | Cystic renal disease | Neonatal cholestasis | Syndromic association | syndromic association | neonatal cholestasis | cystic renal disease | fibrocystic disease of liver | Case Report
Journal Article
Hepatology Research, ISSN 1386-6346, 2006, Volume 35, Issue 2, pp. 147 - 150
Congenital hepatic fibrosis belongs to the fibrocystic diseases of the liver and represents ductal plate malformation of interlobular bile ducts, along with a... 
Fibrocystic diseases of the liver | Distal splenorenal shunting | Endoscopic variceal ligation | Portal hypertension | Congenital hepatic fibrosis | Ductal plate malformation
Journal Article
12/2011, ISBN 9780470654682, 42
Benign tumors of the liver are relatively common and may arise from hepatocytes or other cell types within the liver. Hepatocellular adenoma is a relatively... 
hemangioma | focal nodular hyperplasia | fibrocystic diseases | Adenoma | benign neoplasms | choledochal cysts | polycystic disease | Polycystic disease | Benign neoplasms | Choledochal cysts | Fibrocystic diseases | Hemangioma | Focal nodular hyperplasia
Book Chapter
Journal of Pediatric Gastroenterology and Nutrition, ISSN 0277-2116, 08/2013, Volume 57, Issue 2, pp. 161 - 166
BACKGROUND AND OBJECTIVE:Congenital hepatic fibrosis (CHF) and Caroli syndrome are frequently associated with renal cystic diseases. They have a variable... 
combined liver-kidney transplant | ARPKD | Caroli syndrome | portal hypertension | congenital hepatic fibrosis | hepatorenal fibrocystic disease | LIVER-TRANSPLANTATION | POLYCYSTIC KIDNEY-DISEASE | RISK | RENAL-TRANSPLANTATION | IMPACT | NUTRITION & DIETETICS | PEDIATRICS | MUTATIONS | SPECTRUM | GASTROENTEROLOGY & HEPATOLOGY | Hypertension, Portal - genetics | Liver - pathology | Liver Transplantation | Prevalence | Kidney - pathology | Renal Insufficiency, Chronic - etiology | Humans | Child, Preschool | Infant | Male | Esophageal and Gastric Varices - etiology | Genetic Diseases, Inborn - pathology | Genetic Diseases, Inborn - epidemiology | Esophageal and Gastric Varices - epidemiology | Liver Cirrhosis - epidemiology | Polycystic Kidney, Autosomal Recessive - pathology | Renal Insufficiency, Chronic - epidemiology | Caroli Disease - epidemiology | Hypertension, Portal - etiology | Polycystic Kidney, Autosomal Recessive - complications | Genetic Diseases, Inborn - complications | Caroli Disease - surgery | Female | Retrospective Studies | Child | Infant, Newborn | Caroli Disease - pathology | Cholangitis - epidemiology | Cholangitis - genetics | Liver Cirrhosis - complications | Esophageal and Gastric Varices - genetics | Kidney Transplantation | Polycystic Kidney, Autosomal Recessive - epidemiology | Cholangitis - etiology | Kidney Failure, Chronic - genetics | Hypertension, Portal - epidemiology | Phenotype | Kidney Failure, Chronic - epidemiology | Adolescent | Liver Cirrhosis - pathology | Polycystic Kidney, Autosomal Recessive - surgery | Liver Cirrhosis - surgery | Caroli Disease - complications | Genetic Diseases, Inborn - surgery | Kidney Failure, Chronic - etiology
Journal Article