Published in Semin Liver Dis on April 26, 2010
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Localization of genes for V+LDL plasma cholesterol levels on two diets in the opossum Monodelphis domestica. J Lipid Res (2010) 0.77
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Impaired fetal adrenal function in intrahepatic cholestasis of pregnancy. Med Sci Monit (2011) 0.76
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Clinical utility gene card for: progressive familial intrahepatic cholestasis type 3. Eur J Hum Genet (2013) 0.76
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Novel mutation in a Chinese patient with progressive familial intrahepatic cholestasis type 3. World J Gastroenterol (2015) 0.75
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Claudin-1 gene mutations in neonatal sclerosing cholangitis associated with ichthyosis: a tight junction disease. Gastroenterology (2004) 1.98
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Differences in presentation and progression between severe FIC1 and BSEP deficiencies. J Hepatol (2010) 1.60
MYO5B and bile salt export pump contribute to cholestatic liver disorder in microvillous inclusion disease. Hepatology (2014) 1.48
Anti-CD20 monoclonal antibody (Rituximab) treatment for Epstein-Barr virus-associated, B-cell lymphoproliferative disease in pediatric liver transplant recipients. J Pediatr Gastroenterol Nutr (2002) 1.47
ATP8B1 and ABCB11 analysis in 62 children with normal gamma-glutamyl transferase progressive familial intrahepatic cholestasis (PFIC): phenotypic differences between PFIC1 and PFIC2 and natural history. Hepatology (2010) 1.44
Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr (2010) 1.44
Widening spectrum of liver angiosarcoma in children. J Pediatr Gastroenterol Nutr (2011) 1.42
Experience with endoscopic management of high-risk gastroesophageal varices, with and without bleeding, in children with biliary atresia. Gastroenterology (2013) 1.42
Portopulmonary Hypertension in Liver Disease Presenting in Childhood. J Pediatr Gastroenterol Nutr (2015) 1.40
Progressive familial intrahepatic cholestasis. Orphanet J Rare Dis (2009) 1.38
Successful treatment of severe cardiomyopathy in glycogen storage disease type III With D,L-3-hydroxybutyrate, ketogenic and high-protein diet. Pediatr Res (2011) 1.28
Familial liver adenomatosis associated with hepatocyte nuclear factor 1alpha inactivation. Gastroenterology (2003) 1.22
Heterozygous bile salt export pump deficiency: a possible genetic predisposition to transient neonatal cholestasis. J Pediatr Gastroenterol Nutr (2006) 1.16
CFCI gene mutation and biliary atresia with polysplenia syndrome. J Pediatr Gastroenterol Nutr (2002) 1.14
Management of patients with biliary atresia in France: results of a decentralized policy 1986-2002. Hepatology (2006) 1.13
Improving outcomes of biliary atresia: French national series 1986-2009. J Hepatol (2013) 1.12
Oral cholic acid for hereditary defects of primary bile acid synthesis: a safe and effective long-term therapy. Gastroenterology (2009) 1.11
A missense mutation in ABCB4 gene involved in progressive familial intrahepatic cholestasis type 3 leads to a folding defect that can be rescued by low temperature. Hepatology (2009) 1.06
Altered hepatobiliary gene expressions in PFIC1: ATP8B1 gene defect is associated with CFTR downregulation. Hepatology (2006) 1.04
SRD5B1 (AKR1D1) gene analysis in delta(4)-3-oxosteroid 5beta-reductase deficiency: evidence for primary genetic defect. J Hepatol (2004) 0.99
Successful mutation-specific chaperone therapy with 4-phenylbutyrate in a child with progressive familial intrahepatic cholestasis type 2. J Hepatol (2012) 0.99
Liver diseases related to MDR3 (ABCB4) gene deficiency. Front Biosci (Landmark Ed) (2009) 0.92
Rat hepatocytes express functional P2X receptors. FEBS Lett (2007) 0.92
Long-term outcome of pediatric liver transplantation for biliary atresia: a 10-year follow-up in a single center. Liver Transpl (2005) 0.91
Progressive familial intrahepatic cholestasis type 3 revealed by oral contraceptive pills. J Hepatol (2003) 0.91
Mitochondrial Infantile Liver Disease due to TRMU Gene Mutations: Three New Cases. JIMD Rep (2013) 0.91
Claudin-1 involved in neonatal ichthyosis sclerosing cholangitis syndrome regulates hepatic paracellular permeability. Hepatology (2012) 0.90
Management of post-liver transplant-associated IgE-mediated food allergy in children. J Allergy Clin Immunol (2011) 0.90
CFC1 gene involvement in biliary atresia with polysplenia syndrome. J Pediatr Gastroenterol Nutr (2008) 0.90
Prognostic value of endoscopy in children with biliary atresia at risk for early development of varices and bleeding. Gastroenterology (2010) 0.89
Molecular genetics of 3beta-hydroxy-Delta5-C27-steroid oxidoreductase deficiency in 16 patients with loss of bile acid synthesis and liver disease. J Clin Endocrinol Metab (2003) 0.88
Mutation specific drug therapy for progressive familial or benign recurrent intrahepatic cholestasis: a new tool in a near future? J Hepatol (2010) 0.88
Relapsing features of bile salt export pump deficiency after liver transplantation in two patients with progressive familial intrahepatic cholestasis type 2. J Hepatol (2010) 0.87
Homozygosity mapping of a locus for a novel syndromic ichthyosis to chromosome 3q27-q28. J Invest Dermatol (2002) 0.87
Liver glycogen storage diseases due to phosphorylase system deficiencies: diagnosis thanks to non invasive blood enzymatic and molecular studies. Mol Genet Metab (2011) 0.87
CD25 appears non essential for human peripheral T(reg) maintenance in vivo. PLoS One (2010) 0.86
Hereditary fructose intolerance: frequency and spectrum mutations of the aldolase B gene in a large patients cohort from France--identification of eight new mutations. Mol Genet Metab (2008) 0.86
Identification of a human splenic marginal zone B cell precursor with NOTCH2-dependent differentiation properties. J Exp Med (2014) 0.86
Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy. Ann Surg (2014) 0.86
Giant cell hepatitis with autoimmune hemolytic anemia in early childhood: long-term outcome in 16 children. J Pediatr (2011) 0.85
Liver disease associated with ZZ alpha1-antitrypsin deficiency and ursodeoxycholic acid therapy in children. J Pediatr Gastroenterol Nutr (2008) 0.85
A new mutation in PRKAG2 gene causing hypertrophic cardiomyopathy with conduction system disease and muscular glycogenosis. Neuromuscul Disord (2006) 0.83
Oral Tocofersolan Corrects or Prevents Vitamin E Deficiency in Children With Chronic Cholestasis. J Pediatr Gastroenterol Nutr (2016) 0.83
Epstein-Barr virus infection and altered control of apoptotic pathways in posttransplant lymphoproliferative disorders. Pathobiology (2012) 0.82
Prenatal molecular diagnosis of inherited cholestatic diseases. J Pediatr Gastroenterol Nutr (2007) 0.81
Prophylactic endoscopic sclerotherapy of large esophagogastric varices in infants with biliary atresia. Gastrointest Endosc (2008) 0.81
AIRE gene analysis in children with autoimmune hepatitis type I or II. J Pediatr Gastroenterol Nutr (2009) 0.80
Secondary Mitochondrial Respiratory Chain Defect Can Delay Accurate PFIC2 Diagnosis. JIMD Rep (2013) 0.80
Detection of autoimmune regulator gene mutations in children with type 2 autoimmune hepatitis and extrahepatic immune-mediated diseases. J Pediatr (2005) 0.80
Innate immunity, purinergic system, and liver regeneration: a trip in complexity. Hepatology (2013) 0.79
Serum Autotaxin Activity Correlates With Pruritus in Pediatric Cholestatic Disorders. J Pediatr Gastroenterol Nutr (2016) 0.79
Role of purinergic P2X receptors in the control of liver homeostasis. Wiley Interdiscip Rev Membr Transp Signal (2012) 0.78
Alagille syndrome and nephroblastoma: Unusual coincidence of two rare disorders. Pediatr Blood Cancer (2008) 0.78
Molecular and biochemical characterization of a novel intronic single point mutation in a Tunisian family with glycogen storage disease type III. Mol Biol Rep (2013) 0.78
Initial liver transplantation for unresectable hepatoblastoma after chemotherapy. Pediatr Blood Cancer (2011) 0.78
Delayed postnatal presentation of biliary atresia in 2 premature neonates. J Pediatr Gastroenterol Nutr (2011) 0.77
Biliary atresia: does ethnicity matter? J Hepatol (2012) 0.77
NR1H4 analysis in patients with progressive familial intrahepatic cholestasis, drug-induced cholestasis or intrahepatic cholestasis of pregnancy unrelated to ATP8B1, ABCB11 and ABCB4 mutations. Clin Res Hepatol Gastroenterol (2012) 0.77
Albumin liver dialysis as pregnancy-saving procedure in cholestatic liver disease and intractable pruritus. World J Gastroenterol (2008) 0.76
Acute but transient neurological deterioration revealing adult polyglucosan body disease. J Neurol Sci (2012) 0.76
Sertraline as an Additional Treatment for Cholestatic Pruritus in Children. J Pediatr Gastroenterol Nutr (2017) 0.76
Progression to high-risk gastroesophageal varices in children with biliary atresia with low-risk signs at first endoscopy. J Pediatr Gastroenterol Nutr (2015) 0.76
Neonatal ichthyosis and sclerosing cholangitis syndrome: extremely variable liver disease severity from claudin-1 deficiency. J Pediatr Gastroenterol Nutr (2011) 0.76
Hepatorenal syndrome: diagnosis and effect of terlipressin therapy in 4 pediatric patients. J Pediatr Gastroenterol Nutr (2010) 0.76
Primary bile acid therapy during pregnancy in patients with 3β-hydroxy-Δ5 -C27 -steroid oxidoreductase deficiency. Pediatr Int (2011) 0.75
Polynesian ecology determines seasonality of biliary atresia. Hepatology (2011) 0.75
Acute leukemia presenting as acute hepatitis without liver failure. J Pediatr Gastroenterol Nutr (2014) 0.75
Neonatal ischemic liver failure: potential role of the ductus venosus. J Pediatr Gastroenterol Nutr (2004) 0.75
Budd-Chiari syndrome and essential thrombocythemia in a child: favorable outcome after transjugular intrahepatic portosystemic shunt. J Pediatr Gastroenterol Nutr (2008) 0.75
Aspergillus fumigatus endocarditis in a pediatric liver transplant recipient: favorable outcome without cardiac surgery. Pediatr Transplant (2008) 0.75
An Attempt to Determine the Prevalence of Two Inborn Errors of Primary Bile Acid Synthesis: Results of a European survey. J Pediatr Gastroenterol Nutr (2017) 0.75
Abdominal Arterial Anomalies in Children With Alagille Syndrome: Surgical Aspects and Outcomes of Liver Transplantation. J Pediatr Gastroenterol Nutr (2017) 0.75
Arthrogryposis, renal dysfunction, and cholestasis syndrome caused by VIPAR mutation. J Pediatr Gastroenterol Nutr (2014) 0.75