Neonatal cholestasis: emerging molecular diagnostics and potential novel therapeutics

scientific article published on 01 June 2019

Neonatal cholestasis: emerging molecular diagnostics and potential novel therapeutics is …
instance of (P31):
scholarly articleQ13442814

External links are
P356DOI10.1038/S41575-019-0132-Z
P698PubMed publication ID30903105

P2093author name stringRonald J Sokol
Amy G Feldman
P2860cites workNeonatal cholestasisQ80945524
Intrahepatic cholestasis: summary of an American Association for the Study of Liver Diseases single-topic conferenceQ81753891
Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screeningQ83747342
Selective use of endoscopic retrograde cholangiopancreatography in the diagnosis of biliary atresia in infants younger than 100 daysQ84401207
Comparison of different diagnostic methods for differentiating biliary atresia from idiopathic neonatal hepatitisQ84784853
Shear wave elastography helps differentiate biliary atresia from other neonatal/infantile liver diseasesQ85343346
Newborn Bilirubin Screening for Biliary AtresiaQ88095507
Nuclear receptors and liver disease: Summary of the 2017 basic research symposiumQ91059985
Identification of Polycystic Kidney Disease 1 Like 1 Gene Variants in Children With Biliary Atresia Splenic Malformation SyndromeQ91140075
Colesevelam hydrochloride: evidence for its use in the treatment of hypercholesterolemia and type 2 diabetes mellitus with insights into mechanism of actionQ24613405
High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitisQ24655731
Recent advances in understanding and managing cholestasisQ26748537
The SPLIT research agenda 2013Q26825184
New paradigms in the treatment of hepatic cholestasis: from UDCA to FXR, PXR and beyondQ27013066
Sequencing technologies - the next generationQ27860568
Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant careQ28075831
Bile acid nuclear receptor FXR and digestive system diseasesQ28085493
A multicenter, controlled trial of ursodiol for the treatment of primary biliary cirrhosis. UDCA-PBC Study GroupQ28243457
Side chain structure determines unique physiologic and therapeutic properties of norursodeoxycholic acid in Mdr2-/- miceQ30535328
Portal hypertension in children and young adults with biliary atresiaQ33402861
Phytosterols promote liver injury and Kupffer cell activation in parenteral nutrition-associated liver diseaseQ33805839
Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1Q33929416
Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in TaiwanQ34153781
Cholangiocyte biology and cystic fibrosis liver diseaseQ34464126
The toxin biliatresone causes mouse extrahepatic cholangiocyte damage and fibrosis through decreased glutathione and SOX17Q34522256
Experience of nitisinone for the pharmacological treatment of hereditary tyrosinaemia type 1.Q34590786
Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trialQ34999853
Parenteral nutrition supplementation in biliary atresia patients listed for liver transplantationQ35631885
Intestinal Microbiota, Lipids, and the Pathogenesis of Intestinal Failure-Associated Liver DiseaseQ35679248
PoopMD, a Mobile Health Application, Accurately Identifies Infant Acholic Stools.Q35722159
Molecular Genetic Dissection and Neonatal/Infantile Intrahepatic Cholestasis Using Targeted Next-Generation SequencingQ35917843
Patients with biliary atresia have elevated direct/conjugated bilirubin levels shortly after birthQ36071207
Design and validation of the biliary atresia research consortium histologic assessment system for cholestasis in infancyQ36104831
Immunization of pediatric solid-organ transplantation candidates: immunizations in transplant candidatesQ36263626
Whatever happened to "neonatal hepatitis"?Q36352863
Pharmacological inhibition of apical sodium-dependent bile acid transporter changes bile composition and blocks progression of sclerosing cholangitis in multidrug resistance 2 knockout mice.Q36464051
Studies of Pediatric Liver Transplantation 2002: patient and graft survival and rejection in pediatric recipients of a first liver transplant in the United States and CanadaQ44920745
Percutaneously inserted central catheter for total parenteral nutrition in neonates: complications rates related to upper versus lower extremity insertionQ46090187
Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosisQ46408092
Biliary atresia: clinical profiles, risk factors, and outcomes of 755 patients listed for liver transplantationQ46673007
24-norUrsodeoxycholic acid is superior to ursodeoxycholic acid in the treatment of sclerosing cholangitis in Mdr2 (Abcb4) knockout miceQ46939923
Diagnosis and treatment of tyrosinemia type I: a US and Canadian consensus group review and recommendationsQ47137137
Large-scale proteomics identifies MMP-7 as a sentinel of epithelial injury and of biliary atresia.Q48250842
Percutaneous cholecysto-cholangiography in the diagnosis of obstructive jaundice in infantsQ48925836
Reducing the Underimmunization of Transplant RecipientsQ50077047
Targeted pharmacotherapy in progressive familial intrahepatic cholestasis type 2: Evidence for improvement of cholestasis with 4-phenylbutyrateQ50438744
Newborn Direct or Conjugated Bilirubin Measurements As a Potential Screen for Biliary Atresia.Q50896607
Cost-effective analysis of screening for biliary atresia with the stool color card.Q51053787
Universal screening for biliary atresia using an infant stool color card in Taiwan.Q51114193
Neonatal Cholestasis.Q51340539
Role of abdominal sonography in the preoperative diagnosis of extrahepatic biliary atresia in infants younger than 90 days.Q51376366
Transient cholestasis in newborn infants with perinatal asphyxia.Q51678912
Province-Wide Biliary Atresia Home Screening Program in British Columbia: Evaluation of First Two Years.Q52640526
Inborn Errors of Metabolism with Hepatopathy: Metabolism Defects of Galactose, Fructose, and Tyrosine.Q52675188
Neonatal Cholestasis.Q54236162
Association of variants of ABCB11 with transient neonatal cholestasis.Q54277571
Placebo-Controlled Randomized Trial of an Intestinal Bile Salt Transport Inhibitor for Pruritus in Alagille SyndromeQ57070329
Inhibition of intestinal bile acid absorption improves cholestatic liver and bile duct injury in a mouse model of sclerosing cholangitisQ57105327
Oral Cholic Acid Is Efficacious and Well Tolerated in Patients With Bile Acid Synthesis and Zellweger Spectrum DisordersQ57122674
EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitisQ59384401
Contribution of acoustic radiation force impulse (ARFI) elastography to the ultrasound diagnosis of biliary atresiaQ60691307
Ursodeoxycholic acid in the treatment of primary biliary cirrhosisQ72401649
Long-term effects of ursodeoxycholic acid in primary biliary cirrhosis: results of a double-blind controlled multicentric trial. UDCA-Cooperative Group from the Spanish Association for the Study of the LiverQ73715566
Biliary atresia: the Canadian experienceQ79949877
4-phenylbutyrate enhances the cell surface expression and the transport capacity of wild-type and mutated bile salt export pumpsQ80407604
Biliatresone, a Reactive Natural Toxin from Dysphania glomulifera and D. littoralis: Discovery of the Toxic Moiety 1,2-Diaryl-2-PropenoneQ36585337
Increased frequency of double and triple heterozygous gene variants in children with intrahepatic cholestasisQ36753645
Nuclear receptors as drug targets in cholestatic liver diseasesQ36786270
Screening and outcomes in biliary atresia: summary of a National Institutes of Health workshop.Q36895500
Jaundice in babies: implications for community screening for biliary atresiaQ36903399
Newborn Screening for Biliary AtresiaQ37036993
Medical treatment of cholestatic liver diseaseQ37074707
Mechanisms of disease: Inborn errors of bile acid synthesisQ37199631
Growth failure and outcomes in infants with biliary atresia: a report from the Biliary Atresia Research ConsortiumQ37435062
Recent insights into the function and regulation of the bile salt export pump (ABCB11).Q37580701
Parenteral nutrition-associated cholestasis in premature babies: risk factors and predictorsQ37621499
Liver fibrosis in biliary atresiaQ37763083
HIDA, percutaneous transhepatic cholecysto-cholangiography and liver biopsy in infants with persistent jaundice: can a combination of PTCC and liver biopsy reduce unnecessary laparotomy?Q37904859
Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: systematic review and meta-analysis of the literatureQ38092007
Long term outcomes after pediatric liver transplantationQ38186718
The therapeutic potential of chemical chaperones in protein folding diseases.Q38211124
Alterations in lipid metabolism mediate inflammation, fibrosis, and proliferation in a mouse model of chronic cholestatic liver injury.Q38331124
Prevention of cholesterol gallstone disease by FXR agonists in a mouse model.Q38333973
Incidence of Biliary Atresia and Timing of Hepatoportoenterostomy in the United StatesQ38660435
Biliary atresia and other cholestatic childhood diseases: Advances and future challenges.Q38831107
Cholestasis in Preterm InfantsQ38846691
Guideline for the Evaluation of Cholestatic Jaundice in Infants: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and NQ38901059
Key Histopathologic Features of Liver Biopsies That Distinguish Biliary Atresia From Other Causes of Infantile Cholestasis and Their Correlation With Outcome: A Multicenter Study.Q38989429
Hospitalizations for Respiratory Syncytial Virus and Vaccine-Preventable Infections in the First 2 Years After Pediatric Liver TransplantQ39020496
New therapeutic concepts in bile acid transport and signaling for management of cholestasis.Q39044702
Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasisQ39062043
A biliary HCO3- umbrella constitutes a protective mechanism against bile acid-induced injury in human cholangiocytes.Q39471132
Stool color card screening for biliary atresiaQ39693838
A Randomized, Placebo-Controlled Trial of Cenicriviroc for Treatment of Nonalcoholic Steatohepatitis with Fibrosis.Q40082820
Expression of bile acid synthesis and detoxification enzymes and the alternative bile acid efflux pump MRP4 in patients with primary biliary cirrhosisQ40183754
Hepatic uptake of conjugated bile acids is mediated by both sodium taurocholate cotransporting polypeptide and organic anion transporting polypeptides and modulated by intestinal sensing of plasma bile acid levels in miceQ40215280
Farnesoid X Receptor Activates Transcription of the Phospholipid Pump MDR3Q40628895
Regulation of multidrug resistance-associated protein 2 (ABCC2) by the nuclear receptors pregnane X receptor, farnesoid X-activated receptor, and constitutive androstane receptorQ40768989
Taking the next step forward - Diagnosing inherited infantile cholestatic disorders with next generation sequencing.Q41221551
Late referral for biliary atresia--missed opportunities for effective surgeryQ41823817
Prolonged follow-up of patients in the U.S. multicenter trial of ursodeoxycholic acid for primary biliary cirrhosisQ41871213
Mitochondrial hepatopathies: advances in genetics, therapeutic approaches, and outcomesQ41977878
Jaundice at 14 days of age: exclude biliary atresiaQ42129961
Analysis of gene mutations in children with cholestasis of undefined etiologyQ42728589
Folding defects in P-type ATP 8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate.Q43240642
Improving outcomes of biliary atresia: French national series 1986-2009.Q43638929
Niemann-pick disease type C in neonatal cholestasis at a North American CenterQ44079921
P433issue6
P304page(s)346-360
P577publication date2019-06-01
P1433published inNature Reviews Gastroenterology & HepatologyQ2108255
P1476titleNeonatal cholestasis: emerging molecular diagnostics and potential novel therapeutics
P478volume16

Reverse relations

cites work (P2860)
Q91642461Inherited Cholestatic Diseases in the Era of Personalized Medicine
Q104283798Modeling Outcomes in Children With Biliary Atresia With Native Liver After 2 Years of Age
Q101133373Molecular and cellular mechanisms of liver fibrosis and its regression
Q97646037Neonatal Cholestasis Over Time: Changes in Epidemiology and Outcome in a Cohort of 154 Patients From a Portuguese Tertiary Center
Q97593197Pediatric Cholestasis: Epidemiology, Genetics, Diagnosis, and Current Management
Q98185020The Functional Role of the Secretin/Secretin Receptor Signaling During Cholestatic Liver Injury

Search more.