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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
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 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
Scientific Reports, ISSN 2045-2322, 12/2017, Volume 7, Issue 1, pp. 1601 - 15
Journal Article
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