Research & Literature

ACG Clinical Guideline: Primary Sclerosing Cholangitis.

Lindor KD, Kowdley KV, Harrison ME,

The American journal of gastroenterology 2015; (110(5)):646-59; quiz 660 doi:10.1038/ajg.2015.112.

Liver Transplantation for Cholestatic Liver Diseases in Adults.

Khungar V, Goldberg DS

Clinics in liver disease 2016; (20(1)):191-203.

Pathogenesis and Management of Pruritus in PBC and PSC.

Kremer AE, Namer B, Bolier R, et al.

Digestive diseases (Basel, Switzerland) 2015; (33 Suppl 2()):164-75 doi:10.1159/000440829.

Performance of magnetic resonance elastography in primary sclerosing cholangitis.

Eaton JE, Dzyubak B, Venkatesh SK, et al.

Journal of gastroenterology and hepatology 2016; (31(6)):1184-90 doi:10.1111/jgh.13263.

Biliary atresia: Clinical advances and perspectives.

Nizery L, Chardot C, Sissaoui S, et al.

Clinics and research in hepatology and gastroenterology 2016; (40(3)):281-287 doi:10.1016/j.clinre.2015.11.010.

Erythrokeratodermia variabilis et progressiva.

Ishida-Yamamoto A

The Journal of dermatology 2016; (43(3)):280-5 doi:10.1111/1346-8138.13220.

Mutations in DCDC2 (doublecortin domain containing protein 2) in neonatal sclerosing cholangitis.

Grammatikopoulos T, Sambrotta M, Strautnieks S, et al.

Journal of hepatology 2016; (65(6)):1179-1187 doi:10.1016/j.jhep.2016.07.017.

Development of a Novel Tool to Assess the Impact of Itching in Pediatric Cholestasis.

Kamath BM, Abetz-Webb L, Kennedy C, et al.

The patient 2018; (11(1)):69-82 doi:10.1007/s40271-017-0266-4.

Acute-on-chronic hepatitis. A case report of autoimmune hepatitis/primary sclerosing cholangitis/ulcerative colitis overlap syndrome in a 15-year-old patient.

Nalepa A, Woźniak M, Cielecka-Kuszyk J, et al.

Clinical and experimental hepatology 2017; (3(1)):28-32 doi:10.5114/ceh.2017.65501.

[NISCH syndrome, a rare cause of neonatal cholestasis: A case report].

Szepetowski S, Lacoste C, Mallet S, et al.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie 2017; (24(12)):1228-1234 doi:10.1016/j.arcped.2017.09.016.

Primary sclerosing cholangitis.

Dyson JK, Beuers U, Jones DEJ, et al.

Lancet (London, England) 2018; (391(10139)):2547-2559 doi:10.1016/S0140-6736(18)30300-3.

Anesthetic Care of 2 Siblings With Neonatal Ichthyosis and Sclerosing Cholangitis Syndrome: Case Reports.

Khalifa C, Boliaki Botombe G, Stephenne X, Veyckemans F

A&A practice 2018; (11(8)):216-218 doi:10.1213/XAA.0000000000000785.

Update in the Care and Management of Patients with Primary Sclerosing Cholangitis.

Sedki M, Levy C

Current gastroenterology reports 2018; (20(7)):29 doi:10.1007/s11894-018-0635-8.

[Neonatal sclerosing cholangitis caused by DCDC2 variations in two siblings and literature review].

Li JQ, Lu Y, Qiu YL, Wang JS

Zhonghua er ke za zhi = Chinese journal of pediatrics 2018; (56(8)):623-627 doi:10.3760/cma.j.issn.0578-1310.2018.08.013.

Non-Invasive Prediction of High-Risk Varices in Patients with Primary Biliary Cholangitis and Primary Sclerosing Cholangitis.

Moctezuma-Velazquez C, Saffioti F, Tasayco-Huaman S, et al.

The American journal of gastroenterology 2019; (114(3)):446-452 doi:10.1038/s41395-018-0265-7.

Infant skin care: updates and recommendations.

Johnson E, Hunt R

Current opinion in pediatrics 2019; (31(4)):476-481 doi:10.1097/MOP.0000000000000791.

Fat soluble vitamins deficiency in pediatric chronic liver disease: The impact of liver transplantation.

Veraldi S, Pietrobattista A, Liccardo D, et al.

Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver 2020; (52(3)):308-313 doi:10.1016/j.dld.2019.10.005.

Dermatology Part 2: Ichthyoses and Psoriasis.

Sticherling M

Handbook of experimental pharmacology 2020; (261()):153-175 doi:10.1007/164_2019_345.

Effects of partial internal biliary diversion on long-term outcomes in patients with progressive familial intrahepatic cholestasis: experience in 44 patients.

Foroutan HR, Bahador A, Ghanim SM, et al.

Pediatric surgery international 2020; (36(5)):603-610 doi:10.1007/s00383-020-04641-z.

Role of Endoscopy in Primary Sclerosing Cholangitis.

Bhat P, Aabakken L

Clinical endoscopy 2021; (54(2)):193-201 doi:10.5946/ce.2020.019-IDEN.

[Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis].

Ahn DW

The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi 2020; (75(5)):246-256 doi:10.4166/kjg.2020.75.5.246.

The Sclerosing Cholangitis Outcomes in Pediatrics (SCOPE) Index: A Prognostic Tool for Children.

Deneau MR, Mack C, Perito ER, et al.

Hepatology (Baltimore, Md.) 2021; (73(3)):1074-1087 doi:10.1002/hep.31393.

Gut Microbiome: A Potential Modifiable Risk Factor in Biliary Atresia.

Jain V, Alexander EC, Burford C, et al.

Journal of pediatric gastroenterology and nutrition 2021; (72(2)):184-193 doi:10.1097/MPG.0000000000002973.

Long-term outcome following cholecystocolostomy in 41 patients with progressive familial intrahepatic cholestasis.

Li Q, Chong C, Sun R, et al.

Pediatric surgery international 2021; (37(6)):723-730 doi:10.1007/s00383-021-04871-9.

Malnutrition in Pediatric Chronic Cholestatic Disease: An Up-to-Date Overview.

Tessitore M, Sorrentino E, Schiano Di Cola G, et al.

Nutrients 2021; (13(8)) doi:10.3390/nu13082785.

The need for early Kasai portoenterostomy: a Western Pediatric Surgery Research Consortium study.

Kelley-Quon LI, Shue E, Burke RV, et al.

Pediatric surgery international 2022; (38(2)):193-199 doi:10.1007/s00383-021-05047-1.

Autosomal recessive congenital ichthyosis caused by a pathogenic missense variant in CLDN1.

Mohamad J, Samuelov L, Assaf S, et al.

American journal of medical genetics. Part A 2022; (188(10)):2879-2887 doi:10.1002/ajmg.a.62924.

Longitudinal changes in quantitative magnetic resonance imaging metrics in children and young adults with autoimmune liver disease.

Mahalingam N, Trout AT, Zhang B, et al.

Abdominal radiology (New York) 2023; (48(6)):1933-1944 doi:10.1007/s00261-022-03733-9.

Age at surgery and native liver survival in biliary atresia: a systematic review and meta-analysis.

Hoshino E, Muto Y, Sakai K, et al.

European journal of pediatrics 2023; (182(6)):2693-2704 doi:10.1007/s00431-023-04925-1.

Primary Sclerosing Cholangitis-Autoimmune Hepatitis Overlap Syndrome: Significant Barriers in Liver Disease Diagnosis and Treatment Experienced by the Latino Community.

Guifarro DA, De Oliveira-Gomes D, Beas R, et al.

Cureus 2023; (15(3)):e36126 doi:10.7759/cureus.36126.

Fat-Soluble Vitamins Deficiency in Pediatric Cholestasis: A Scoping Review.

Degrassi I, Leonardi I, Di Profio E, et al.

Nutrients 2023; (15(11)) doi:10.3390/nu15112491.

Current Therapeutics in Primary Sclerosing Cholangitis.

Tan N, Lubel J, Kemp W, et al.

Journal of clinical and translational hepatology 2023; (11(5)):1267-1281 doi:10.14218/JCTH.2022.00068S.

Medium-chain triglycerides and the impact on fat absorption, growth, nutritional status and clinical outcomes in children with cholestatic liver disease: A scoping review.

Mancell S, Manwani K, Dhawan A, Whelan K

Clinical nutrition (Edinburgh, Scotland) 2023; (42(11)):2159-2172 doi:10.1016/j.clnu.2023.09.010.

CLDN1 Arg81His founder variant causes ichthyosis, leukocyte vacuoles, alopecia, and sclerosing cholangitis (ILVASC) syndrome in Moroccan Jews.

Eskin-Schwartz M, Dolgin V, Didkovsky E, et al.

Clinical genetics 2024; (105(1)):44-51 doi:10.1111/cge.14432.

CLDN1 knock out keratinocytes as a model to investigate multiple skin disorders.

Arnold KA, Moran MC, Shi H, et al.

Experimental dermatology 2024; (33(5)):e15084 doi:10.1111/exd.15084.

Assessment of disease severity with magnetic resonance cholangiography in pediatric-onset primary sclerosing cholangitis.

Vanhanen E, Ovissi A, Kolho KL, Tenca A

Journal of pediatric gastroenterology and nutrition 2024; (79(3)):644-651 doi:10.1002/jpn3.12319.

Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group.

van Rheenen PF, Kolho KL, Russell RK, et al.

Journal of pediatric gastroenterology and nutrition 2025; (80(2)):374-393 doi:10.1002/jpn3.12378.

Real-world Multi-institutional Data From the Midwest Pediatric Surgery Consortium (MWPSC) to Assess the Effect of Delayed Kasai Procedure on Biliary Drainage in Patients With Biliary Atresia.

Farooqui ZA, Hartman SJ, Stetson AE, et al.

Journal of pediatric surgery 2025; (60(5)):162250 doi:10.1016/j.jpedsurg.2025.162250.

Claudin-1 is a mediator and therapeutic target in primary sclerosing cholangitis.

Del Zompo F, Crouchet E, Ostyn T, et al.

Journal of hepatology 2025; (83(6)):1305-1319 doi:10.1016/j.jhep.2025.08.005.

Neonatal ichthyosis-sclerosing cholangitis syndrome caused by a novel CLDN1 mutation: a case report and literature review.

Ghosh U, Agrawal A, Srinivasan VM, et al.

Clinical and experimental pediatrics 2025; (68(11)):858-867 doi:10.3345/cep.2025.00906.

Spanish clinical practice guidelines for the diagnosis and management of cholestatic liver diseases in adult and pediatric population: Joint report from AEEH and SEGHNP.

Díaz-González Á, Molera C, Gómez-Domínguez E, et al.

Gastroenterologia y hepatologia 2026; (49(4)):502629 doi:10.1016/j.gastrohep.2025.502629.

Primary sclerosing cholangitis in children: a single-center experience and evaluation of prognostic markers.

Vlckova E, Hradsky O, Semjonov V, et al.

Translational gastroenterology and hepatology 2026; (11()):18 doi:10.21037/tgh-25-57.

Sarcopenia and nutritional impact in pediatric patients with chronic liver disease: Clinical and management strategies.

Kehar M, Ayala-German AG, Pandey A, et al.

Journal of pediatric gastroenterology and nutrition 2026; (82(6)):1329-1340 doi:10.1002/jpn3.70367.

Nutrition support of children with chronic liver disease: A rapid review and consensus statements from Australasian society of parenteral and enteral nutrition (AuSPEN) Paediatric liver group.

Burgess D, Collins T, Landorf E, et al.

Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver 2026; (58(7)):862-870 doi:10.1016/j.dld.2026.05.006.

Do hepatic and biliary functional data from gadoxetate-enhanced MRI add value in predicting outcomes in primary sclerosing cholangitis (PSC)?

Sandrasegaran K, Shah AS, Thompson CP, et al.

European radiology 2026; doi:10.1007/s00330-026-12710-5.