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Items: 1 to 50 of 134

1.

Neurodevelopmental Outcomes in Pre-School and School Aged Children with Biliary Atresia and their Native Liver.

Squires JE, Ng VL, Hawthorne K, Henn L, Sorensen LG, Fredericks EM, Alonso EM, Murray KF, Loomes KM, Karpen SJ, Cavallo LA, Molleston JP, Bezerra JA, Rosenthal P, Squires RH, Wang KS, Schwarz KB, Arnon R, Magee JC, Sokol RJ; Childhood Liver Disease Research Network (ChiLDReN).

J Pediatr Gastroenterol Nutr. 2019 Sep 6. doi: 10.1097/MPG.0000000000002489. [Epub ahead of print]

PMID:
31503218
2.

Correlation of Immune Markers With Outcomes in Biliary Atresia Following Intravenous Immunoglobulin Therapy.

Kim S, Moore J, Alonso E, Bednarek J, Bezerra JA, Goodhue C, Karpen SJ, Loomes KM, Magee JC, Ng VL, Sherker AH, Smith C, Spino C, Venkat V, Wang K, Sokol RJ, Mack CL; Childhood Liver Disease Research Network.

Hepatol Commun. 2019 Mar 25;3(5):685-696. doi: 10.1002/hep4.1332. eCollection 2019 May.

3.

A Phase I/IIa Trial of Intravenous Immunoglobulin Following Portoenterostomy in Biliary Atresia.

Mack CL, Spino C, Alonso EM, Bezerra JA, Moore J, Goodhue C, Ng VL, Karpen SJ, Venkat V, Loomes KM, Wang K, Sherker AH, Magee JC, Sokol RJ; and The ChiLDReN Network.

J Pediatr Gastroenterol Nutr. 2019 Apr;68(4):495-501. doi: 10.1097/MPG.0000000000002256.

PMID:
30664564
4.

Identification of Polycystic Kidney Disease 1 Like 1 Gene Variants in Children With Biliary Atresia Splenic Malformation Syndrome.

Berauer JP, Mezina AI, Okou DT, Sabo A, Muzny DM, Gibbs RA, Hegde MR, Chopra P, Cutler DJ, Perlmutter DH, Bull LN, Thompson RJ, Loomes KM, Spinner NB, Rajagopalan R, Guthery SL, Moore B, Yandell M, Harpavat S, Magee JC, Kamath BM, Molleston JP, Bezerra JA, Murray KF, Alonso EM, Rosenthal P, Squires RH, Wang KS, Finegold MJ, Russo P, Sherker AH, Sokol RJ, Karpen SJ; Childhood Liver Disease Research Network (ChiLDReN).

Hepatology. 2019 Sep;70(3):899-910. doi: 10.1002/hep.30515. Epub 2019 Mar 21.

PMID:
30664273
5.

Placebo-Controlled Randomized Trial of an Intestinal Bile Salt Transport Inhibitor for Pruritus in Alagille Syndrome.

Shneider BL, Spino C, Kamath BM, Magee JC, Bass LM, Setchell KD, Miethke A, Molleston JP, Mack CL, Squires RH, Murray KF, Loomes KM, Rosenthal P, Karpen SJ, Leung DH, Guthery SL, Thomas D, Sherker AH, Sokol RJ; Childhood Liver Disease Research Network.

Hepatol Commun. 2018 Sep 24;2(10):1184-1198. doi: 10.1002/hep4.1244. eCollection 2018 Oct.

6.

Impact of Steroid Therapy on Early Growth in Infants with Biliary Atresia: The Multicenter Steroids in Biliary Atresia Randomized Trial.

Alonso EM, Ye W, Hawthorne K, Venkat V, Loomes KM, Mack CL, Hertel PM, Karpen SJ, Kerkar N, Molleston JP, Murray KF, Romero R, Rosenthal P, Schwarz KB, Shneider BL, Suchy FJ, Turmelle YP, Wang KS, Sherker AH, Sokol RJ, Bezerra JA, Magee JC; ChiLDReN Network.

J Pediatr. 2018 Nov;202:179-185.e4. doi: 10.1016/j.jpeds.2018.07.002. Epub 2018 Sep 21.

PMID:
30244988
7.

Clinical Consequences of Cardiomyopathy in Children With Biliary Atresia Requiring Liver Transplantation.

Gorgis NM, Kennedy C, Lam F, Thompson K, Coss-Bu J, Akcan Arikan A, Nguyen T, Hosek K, Miloh T, Karpen SJ, Penny DJ, Goss J, Desai MS.

Hepatology. 2019 Mar;69(3):1206-1218. doi: 10.1002/hep.30204. Epub 2019 Feb 17.

PMID:
30076624
8.

TGR5 activation induces cytoprotective changes in the heart and improves myocardial adaptability to physiologic, inotropic, and pressure-induced stress in mice.

Eblimit Z, Thevananther S, Karpen SJ, Taegtmeyer H, Moore DD, Adorini L, Penny DJ, Desai MS.

Cardiovasc Ther. 2018 Oct;36(5):e12462. doi: 10.1111/1755-5922.12462. Epub 2018 Aug 22.

PMID:
30070769
9.

Bone Density in Children With Chronic Liver Disease Correlates With Growth and Cholestasis.

Loomes KM, Spino C, Goodrich NP, Hangartner TN, Marker AE, Heubi JE, Kamath BM, Shneider BL, Rosenthal P, Hertel PM, Karpen SJ, Molleston JP, Murray KF, Schwarz KB, Squires RH, Teckman J, Turmelle YP, Alonso EM, Sherker AH, Magee JC, Sokol RJ; Childhood Liver Disease Research Network.

Hepatology. 2019 Jan;69(1):245-257. doi: 10.1002/hep.30196. Epub 2018 Dec 27.

PMID:
30063078
10.

Novel Bile Acid Therapies for Liver Disease.

Karpen SJ.

Gastroenterol Hepatol (N Y). 2018 Feb;14(2):117-119. No abstract available.

11.

BILIARY ATRESIA: Clinical and Research Challenges for the 21st Century.

Bezerra JA, Wells RG, Mack CL, Karpen SJ, Hoofnagle JH, Doo E, Sokol RJ.

Hepatology. 2018 Mar 31. doi: 10.1002/hep.29905. [Epub ahead of print]

PMID:
29604222
12.

Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver: Results from the ChiLDReN Study.

Ng VL, Sorensen LG, Alonso EM, Fredericks EM, Ye W, Moore J, Karpen SJ, Shneider BL, Molleston JP, Bezerra JA, Murray KF, Loomes KM, Rosenthal P, Squires RH, Wang K, Arnon R, Schwarz KB, Turmelle YP, Haber BH, Sherker AH, Magee JC, Sokol RJ; Childhood Liver Disease Research Network (ChiLDReN).

J Pediatr. 2018 May;196:139-147.e3. doi: 10.1016/j.jpeds.2017.12.048. Epub 2018 Mar 5.

13.

Organic solute transporter-β (SLC51B) deficiency in two brothers with congenital diarrhea and features of cholestasis.

Sultan M, Rao A, Elpeleg O, Vaz FM, Abu-Libdeh B, Karpen SJ, Dawson PA.

Hepatology. 2018 Aug;68(2):590-598. doi: 10.1002/hep.29516. Epub 2018 May 11.

14.

Initial assessment of the infant with neonatal cholestasis-Is this biliary atresia?

Shneider BL, Moore J, Kerkar N, Magee JC, Ye W, Karpen SJ, Kamath BM, Molleston JP, Bezerra JA, Murray KF, Loomes KM, Whitington PF, Rosenthal P, Squires RH, Guthery SL, Arnon R, Schwarz KB, Turmelle YP, Sherker AH, Sokol RJ; Childhood Liver Disease Research Network.

PLoS One. 2017 May 11;12(5):e0176275. doi: 10.1371/journal.pone.0176275. eCollection 2017.

15.

Level of γ-glutamyltransferase in 2-Year-old Children With Biliary Atresia Associates With Progression of Portal Hypertension.

Freeman AJ, Ng VL, Harpavat S, Hrycko A, Apted Z, Bulut P, Leong T, Karpen SJ.

Clin Gastroenterol Hepatol. 2017 Jul;15(7):1133-1135. doi: 10.1016/j.cgh.2017.02.025. Epub 2017 Mar 1. No abstract available.

PMID:
28259742
16.

In Children With Nonalcoholic Fatty Liver Disease, Cysteamine Bitartrate Delayed Release Improves Liver Enzymes but Does Not Reduce Disease Activity Scores.

Schwimmer JB, Lavine JE, Wilson LA, Neuschwander-Tetri BA, Xanthakos SA, Kohli R, Barlow SE, Vos MB, Karpen SJ, Molleston JP, Whitington PF, Rosenthal P, Jain AK, Murray KF, Brunt EM, Kleiner DE, Van Natta ML, Clark JM, Tonascia J, Doo E; NASH CRN.

Gastroenterology. 2016 Dec;151(6):1141-1154.e9. doi: 10.1053/j.gastro.2016.08.027. Epub 2016 Aug 26.

17.

Bile acid excess induces cardiomyopathy and metabolic dysfunctions in the heart.

Desai MS, Mathur B, Eblimit Z, Vasquez H, Taegtmeyer H, Karpen SJ, Penny DJ, Moore DD, Anakk S.

Hepatology. 2017 Jan;65(1):189-201. doi: 10.1002/hep.28890. Epub 2016 Nov 29.

18.

Inhibition of ileal bile acid uptake protects against nonalcoholic fatty liver disease in high-fat diet-fed mice.

Rao A, Kosters A, Mells JE, Zhang W, Setchell KD, Amanso AM, Wynn GM, Xu T, Keller BT, Yin H, Banton S, Jones DP, Wu H, Dawson PA, Karpen SJ.

Sci Transl Med. 2016 Sep 21;8(357):357ra122. doi: 10.1126/scitranslmed.aaf4823.

19.

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 Nutrition.

Fawaz R, Baumann U, Ekong U, Fischler B, Hadzic N, Mack CL, McLin VA, Molleston JP, Neimark E, Ng VL, Karpen SJ.

J Pediatr Gastroenterol Nutr. 2017 Jan;64(1):154-168. doi: 10.1097/MPG.0000000000001334.

PMID:
27429428
20.

Bile acids and nonalcoholic fatty liver disease: Molecular insights and therapeutic perspectives.

Arab JP, Karpen SJ, Dawson PA, Arrese M, Trauner M.

Hepatology. 2017 Jan;65(1):350-362. doi: 10.1002/hep.28709. Epub 2016 Aug 4. Review.

21.

New-Onset Scleral Icterus in a School-Aged Female.

Thompson AB, Bilhartz JL, Abramowsky CR, Rapkin L, Karpen SJ, Berkowitz FE.

Clin Pediatr (Phila). 2017 Jan;56(1):93-96. doi: 10.1177/0009922816650599. Epub 2016 Jul 19. No abstract available.

PMID:
27193342
22.

Total Serum Bilirubin within 3 Months of Hepatoportoenterostomy Predicts Short-Term Outcomes in Biliary Atresia.

Shneider BL, Magee JC, Karpen SJ, Rand EB, Narkewicz MR, Bass LM, Schwarz K, Whitington PF, Bezerra JA, Kerkar N, Haber B, Rosenthal P, Turmelle YP, Molleston JP, Murray KF, Ng VL, Wang KS, Romero R, Squires RH, Arnon R, Sherker AH, Moore J, Ye W, Sokol RJ; Childhood Liver Disease Research Network (ChiLDReN).

J Pediatr. 2016 Mar;170:211-7.e1-2. doi: 10.1016/j.jpeds.2015.11.058. Epub 2015 Dec 24.

23.

Inflammation-associated upregulation of the sulfated steroid transporter Slc10a6 in mouse liver and macrophage cell lines.

Kosters A, Abebe DF, Felix JC, Dawson PA, Karpen SJ.

Hepatol Res. 2016 Jul;46(8):794-803. doi: 10.1111/hepr.12609. Epub 2015 Nov 19.

24.

Nuancing insulin's actions in liver through rapid NCOR1 phosphorylation.

Karpen SJ.

Hepatology. 2015 Nov;62(5):1344-5. doi: 10.1002/hep.28014. Epub 2015 Aug 29. No abstract available.

PMID:
26223211
25.

Quality of Life and Its Determinants in a Multicenter Cohort of Children with Alagille Syndrome.

Kamath BM, Chen Z, Romero R, Fredericks EM, Alonso EM, Arnon R, Heubi J, Hertel PM, Karpen SJ, Loomes KM, Murray KF, Rosenthal P, Schwarz KB, Subbarao G, Teckman JH, Turmelle YP, Wang KS, Sherker AH, Sokol RJ, Magee JC; Childhood Liver Disease Research Network (ChiLDReN).

J Pediatr. 2015 Aug;167(2):390-6.e3. doi: 10.1016/j.jpeds.2015.04.077. Epub 2015 Jun 6.

26.

Genetic contributors and modifiers of biliary atresia.

Mezina A, Karpen SJ.

Dig Dis. 2015;33(3):408-14. doi: 10.1159/000371694. Epub 2015 May 27. Review.

27.

Saturated fat and cholesterol are critical to inducing murine metabolic syndrome with robust nonalcoholic steatohepatitis.

Mells JE, Fu PP, Kumar P, Smith T, Karpen SJ, Anania FA.

J Nutr Biochem. 2015 Mar;26(3):285-92. doi: 10.1016/j.jnutbio.2014.11.002. Epub 2014 Dec 6.

28.

Total serum bilirubin predicts fat-soluble vitamin deficiency better than serum bile acids in infants with biliary atresia.

Venkat VL, Shneider BL, Magee JC, Turmelle Y, Arnon R, Bezerra JA, Hertel PM, Karpen SJ, Kerkar N, Loomes KM, Molleston J, Murray KF, Ng VL, Raghunathan T, Rosenthal P, Schwartz K, Sherker AH, Sokol RJ, Teckman J, Wang K, Whitington PF, Heubi JE; Childhood Liver Disease Research and Education Network.

J Pediatr Gastroenterol Nutr. 2014 Dec;59(6):702-7. doi: 10.1097/MPG.0000000000000547.

29.

P2Y2 purinergic receptor activation is essential for efficient hepatocyte proliferation in response to partial hepatectomy.

Tackett BC, Sun H, Mei Y, Maynard JP, Cheruvu S, Mani A, Hernandez-Garcia A, Vigneswaran N, Karpen SJ, Thevananther S.

Am J Physiol Gastrointest Liver Physiol. 2014 Dec 1;307(11):G1073-87. doi: 10.1152/ajpgi.00092.2014. Epub 2014 Oct 9.

30.

Intestinal transport and metabolism of bile acids.

Dawson PA, Karpen SJ.

J Lipid Res. 2015 Jun;56(6):1085-99. doi: 10.1194/jlr.R054114. Epub 2014 Sep 10. Review.

31.

Medical status of 219 children with biliary atresia surviving long-term with their native livers: results from a North American multicenter consortium.

Ng VL, Haber BH, Magee JC, Miethke A, Murray KF, Michail S, Karpen SJ, Kerkar N, Molleston JP, Romero R, Rosenthal P, Schwarz KB, Shneider BL, Turmelle YP, Alonso EM, Sherker AH, Sokol RJ; Childhood Liver Disease Research and Education Network (CHiLDREN).

J Pediatr. 2014 Sep;165(3):539-546.e2. doi: 10.1016/j.jpeds.2014.05.038. Epub 2014 Jul 9. Erratum in: J Pediatr. 2015 Jan;166(1):211.

32.

Not all (bile acids) who wander are lost: the first report of a patient with an isolated NTCP defect.

Karpen SJ, Dawson PA.

Hepatology. 2015 Jan;61(1):24-7. doi: 10.1002/hep.27294. Epub 2014 Nov 20. No abstract available.

33.

Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial.

Bezerra JA, Spino C, Magee JC, Shneider BL, Rosenthal P, Wang KS, Erlichman J, Haber B, Hertel PM, Karpen SJ, Kerkar N, Loomes KM, Molleston JP, Murray KF, Romero R, Schwarz KB, Shepherd R, Suchy FJ, Turmelle YP, Whitington PF, Moore J, Sherker AH, Robuck PR, Sokol RJ; Childhood Liver Disease Research and Education Network (ChiLDREN).

JAMA. 2014 May 7;311(17):1750-9. doi: 10.1001/jama.2014.2623.

34.

Bile acids reach out to the spinal cord: new insights to the pathogenesis of itch and analgesia in cholestatic liver disease.

Dawson PA, Karpen SJ.

Hepatology. 2014 Apr;59(4):1638-41. doi: 10.1002/hep.26786. Epub 2014 Feb 14. No abstract available.

35.

Cardiomyopathy reverses with recovery of liver injury, cholestasis and cholanemia in mouse model of biliary fibrosis.

Desai MS, Eblimit Z, Thevananther S, Kosters A, Moore DD, Penny DJ, Karpen SJ.

Liver Int. 2015 Apr;35(4):1464-77. doi: 10.1111/liv.12438. Epub 2014 Jan 12.

36.

Impaired bile acid handling and aggravated liver injury in mice expressing a hepatocyte-specific RXRα variant lacking the DNA-binding domain.

Kosters A, Felix JC, Desai MS, Karpen SJ.

J Hepatol. 2014 Feb;60(2):362-9. doi: 10.1016/j.jhep.2013.09.026. Epub 2013 Oct 10.

37.

Phytosterols promote liver injury and Kupffer cell activation in parenteral nutrition-associated liver disease.

El Kasmi KC, Anderson AL, Devereaux MW, Vue PM, Zhang W, Setchell KD, Karpen SJ, Sokol RJ.

Sci Transl Med. 2013 Oct 9;5(206):206ra137. doi: 10.1126/scitranslmed.3006898.

38.

Sexually dimorphic genome-wide binding of retinoid X receptor alpha (RXRα) determines male-female differences in the expression of hepatic lipid processing genes in mice.

Kosters A, Sun D, Wu H, Tian F, Felix JC, Li W, Karpen SJ.

PLoS One. 2013 Aug 19;8(8):e71538. doi: 10.1371/journal.pone.0071538. eCollection 2013.

39.

Do therapeutic bile acids hit the sweet spot of glucose metabolism in NAFLD?

Karpen SJ.

Gastroenterology. 2013 Sep;145(3):508-10. doi: 10.1053/j.gastro.2013.07.017. Epub 2013 Jul 25. No abstract available.

PMID:
23891611
40.

Long-term medical management of the pediatric patient after liver transplantation: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation.

Kelly DA, Bucuvalas JC, Alonso EM, Karpen SJ, Allen U, Green M, Farmer D, Shemesh E, McDonald RA; American Association for the Study of Liver Diseases; American Society of Transplantation.

Liver Transpl. 2013 Aug;19(8):798-825. doi: 10.1002/lt.23697. No abstract available.

41.

Extrahepatic anomalies in infants with biliary atresia: results of a large prospective North American multicenter study.

Schwarz KB, Haber BH, Rosenthal P, Mack CL, Moore J, Bove K, Bezerra JA, Karpen SJ, Kerkar N, Shneider BL, Turmelle YP, Whitington PF, Molleston JP, Murray KF, Ng VL, Romero R, Wang KS, Sokol RJ, Magee JC; Childhood Liver Disease Research and Education Network.

Hepatology. 2013 Nov;58(5):1724-31. doi: 10.1002/hep.26512. Epub 2013 Sep 19.

42.

Inflammatory mediators increase SUMOylation of retinoid X receptor α in a c-Jun N-terminal kinase-dependent manner in human hepatocellular carcinoma cells.

Schneider Aguirre R, Karpen SJ.

Mol Pharmacol. 2013 Aug;84(2):218-26. doi: 10.1124/mol.113.085555. Epub 2013 May 20.

43.

Liver transplantation in cystic fibrosis: a report from Baylor College of Medicine and the Texas Children's Hospital.

Harring TR, Nguyen NT, Liu H, Karpen SJ, Goss JA, O'Mahony CA.

Pediatr Transplant. 2013 May;17(3):271-7. doi: 10.1111/petr.12057. Epub 2013 Mar 15.

PMID:
23489441
44.

Portal hypertension in children and young adults with biliary atresia.

Shneider BL, Abel B, Haber B, Karpen SJ, Magee JC, Romero R, Schwarz K, Bass LM, Kerkar N, Miethke AG, Rosenthal P, Turmelle Y, Robuck PR, Sokol RJ; Childhood Liver Disease Research and Education Network.

J Pediatr Gastroenterol Nutr. 2012 Nov;55(5):567-73. doi: 10.1097/MPG.0b013e31826eb0cf.

45.

Efficacy of fat-soluble vitamin supplementation in infants with biliary atresia.

Shneider BL, Magee JC, Bezerra JA, Haber B, Karpen SJ, Raghunathan T, Rosenthal P, Schwarz K, Suchy FJ, Kerkar N, Turmelle Y, Whitington PF, Robuck PR, Sokol RJ; Childhood Liver Disease Research Education Network (ChiLDREN).

Pediatrics. 2012 Sep;130(3):e607-14. Epub 2012 Aug 13.

46.

Discordant presentation of biliary atresia in premature monozygotic twins.

Fallon SC, Chang S, Finegold MJ, Karpen SJ, Brandt ML.

J Pediatr Gastroenterol Nutr. 2013 Oct;57(4):e22-3. doi: 10.1097/MPG.0b013e31826a1044. No abstract available.

PMID:
22847464
47.

Progressive familial intrahepatic cholestasis type 3: overlapping presentation with Wilson disease.

Ramraj R, Finegold MJ, Karpen SJ.

Clin Pediatr (Phila). 2012 Jul;51(7):689-91. doi: 10.1177/0009922812451076. Epub 2012 Jun 4. No abstract available.

48.

Curiouser and curiouser!

Karpen SJ.

J Hepatol. 2012 Aug;57(2):237-8. doi: 10.1016/j.jhep.2012.05.006. Epub 2012 May 23. No abstract available.

49.

Presentation of atopic disease in a large cohort of pediatric liver transplant recipients.

Shroff P, Mehta RS, Chinen J, Karpen SJ, Davis CM.

Pediatr Transplant. 2012 Jun;16(4):379-84. doi: 10.1111/j.1399-3046.2012.01684.x. Epub 2012 Apr 11.

PMID:
22489822
50.

Patients with biliary atresia have elevated direct/conjugated bilirubin levels shortly after birth.

Harpavat S, Finegold MJ, Karpen SJ.

Pediatrics. 2011 Dec;128(6):e1428-33. doi: 10.1542/peds.2011-1869. Epub 2011 Nov 21.

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