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Randolph P. Matthews, MD, PhD

Attending Physician

Assistant Professor of Pediatrics, Perelman School of Medicine at the University of Pennsylvania

Abstracts

2013

S.F. EauClaire, S. Cui, Z. Cofer, J. Tobias, C. Kim, H. Hakonarson, R.P. Matthews: Analysis of differential gene methylation in livers from biliary atresia patients uncovers a potential role platelet-derived growth factor (PDGF). 26th annual meeting of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition October 2013.

V. Tang, E.A. Tsai, N.B. Spinner, and R.P. Matthews: Evidence from human and zebrafish that ADD3 is a biliary atresia susceptibility gene. 26th annual meeting of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition October 2013 Notes: Oral presentation.

V. Sapp, L. Gaffney, S.F. EauClaire, R.P. Matthews: Characterizing Fructose-Induced Steatohepatitis in Zebrafish Larvae. The Liver Meeting, annual meeting of the American Association for the Study of Liver Diseases November 2013 Notes: Oral presentation.

V. Tang and R.P. Matthews: Effects of histone deacetylation inhibitors on biliary formation in zebrafish The Liver Meeting, annual meeting of the American Association for the Study of Liver Diseases November 2013.

2012

Randolph P. Matthews, Shuang Cui, Melissa Leyva-Vega, Ellen A. Tsai, Steven F. EauClaire, Barbara A. Haber, Nancy B. Spinner: Human and zebrafish evidence for GPC1 as a biliary atresia risk gene. FASEB Liver Biology Conference July 2012.

H. Lin, R. Rutstein, J. Cantor, S. Anupindi, R.P. Matthews: Cholangiocarcinoma in a Young Woman with Perinatally Acquired HIV. 25th annual meeting of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition October 2012.

R.P. Matthews, L. Gaffney, A.J. Edens, S.F. EauClaire: Multiple models of nonalcoholic steatohepatitis in zebrafish underscore the importance of metabolic and inflammatory pathways. 25th annual meeting of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition October 2012.

S.F. EauClaire, S. Cui, A. Alloy, J. Tobias, C. Kim, H. Hakonarson, R.P. Matthews: Methylation microarray studies of biliary atresia livers uncover potential role for PDGF. The Liver Meeting, annual meeting for the American Association for the Study of Liver Disease November 2012.

2011

Shuang Cui, Megan M. Ross, Steven F. EauClaire, and Randolph P. Matthews: Injection of interferon-gamma protein into developing zebrafish larvae leads to biliary defects. 24th annual meeting of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition October 2011.

Steven F. EauClaire and Randolph P. Matthews: A mutation in the ints2 gene leads to severe biliary defects in zebrafish. 24th annual meeting of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition October 2011.

Shuang Cui, Melissa Leyva-Vega, Ellen Tsai, Steven F. EauClaire, Nancy B. Spinner, and Randolph P. Matthews: Glypican 1 regulates zebrafish biliary development and is a potential susceptibility gene for biliary atresia. The Liver Meeting November 2011.

2010

Cui S, Capecci L, Matthews R. Disruption of planar cell polarity leads to developmental biliary defects [poster presentation]. 9th International Conference on Zebrafish Development and Genetics; 2010 June; Madison, WI. 

Matthews RP, EauClaire SF, Mugnier M, Lorent K, Cui S, Ross M, et al. DNA hypomethylation causes bile duct defects in zebrafish and is a distinguishing feature of biliary atresia [oral presentation]. 23rd annual meeting of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition; 2010 Oct; New Orleans, LA.

2009

Matthews RP, Ma L, EauClaire SA. Zebrafish pekin mutants demonstrate the importance of intracellular trafficking in hepatobiliary development [poster presentation]. Digestive Disease Week; 2009 May; Chicago, IL.

Matthews RP, Cui S, Capecci L. Defects in planar cell polarity pathway activity are associated with developmental biliary anomalies [oral presentation]. Digestive Disease Week; 2009 May; Chicago, IL.

EauClaire SA, Ma L, Matthews RP. Biliary and pigmentation defects in zebrafish pekin mutants [poster presentation]. Digestive Disease Week; 2009 May; Chicago, IL.

Matthews RP, Cui S, Capecci L. Defects in planar cell polarity pathway activity are associated with developmental biliary anomalies and are associated with specific infantile biliary disorders [oral presentation]. 22nd annual meeting, North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition; 2009 Nov; National Harbor, MD.

Matthews RP, EauClaire SF, Edens A. Zebrafish models of hepatic steatosis [poster presentation]. 22nd annual meeting, North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition; 2009 Nov; National Harbor, MD. 

Awards

2013, CHOP Resident Faculty Honor Roll

2012, Society for Pediatric Research

2007-2008, Foerderer-Murray New Investigator Award, The Children's Hospital of Philadelphia

2005, Young Faculty Investigator Award, North American Society for Gastroenterology, Hepatology, and Nutrition

1996-1998, Achievement Reward for College Scientists (ARCS) Fellowship

1989, Phi Beta Kappa

1989, Sigma Xi

Books

Chapters

2011

Shuang Cui, Jessi Erlichman, Pierre Russo, Barbara A. Haber, and Randolph P. Matthews*: Intrahepatic biliary anomalies in a patient with Mowat-Wilson syndrome uncover a role for the zinc finger homeobox gene zfhx1b in vertebrate biliary development. J. Pediatr. Gastroenterol. Nutr. 52(3): 339-344, March 2011 Notes: *corresponding author.

2009

Matthews RP. Zebrafish as a model system for liver development and disease. In: Arias IM, et al, editors. The Liver: Biology and Pathobiology. 5th edition; 2009.

2008

Matthews, RP: Metabolic liver disease: tyrosinemia, galactosemia, and hereditary fructose intolerance. In: Liacouras C and Piccoli D, editors. Pediatric Gastroenterology: The Requisites in Pediatrics. Mosby; 2008. Page: 267-275.

Friedlander JE, Matthews RP. Chronic abdominal pain. In: Shah S, editor. Pediatric Practices: Infectious Disease. McGraw-Hill; 2008.

2007

Matthews RP. Metabolic liver disease: glactosemia, tyrosinemia and hereditary fructose intolerance. In: Liacouras CA, Piccoli DA, editors. Essentials of Pediatric Gastroenterology. Mosby Elsevier; 2007.

2004

Matthews RP, Haber BA, Mamula P, Piccoli DA.: Cases in pediatric gastroenterology from The Children's Hospital of Philadelphia. A 5-year-old girl with massive hepatosplenomegaly, fever, and rash. MedGenMed 6: 14, 2004.

2003

Matthews RP. Chronic abdominal pain. In: Schwartz W, editor. Clinical Handbook of Pediatrics 3rd edition. Philadelphia: Lippincott, Williams and Wilkins; 2003.

Editorial and Academic Positions

Editorial positions

2013-present, BMC Systems Biology (ad hoc reviewer)
2013-present, Epigenetics (ad hoc reviewer)
2012-present, BBA - Molecular Basis of Disease (ad hoc reviewer)
2012-present, Frontiers in Pediatric Gastroenterology, Hepatology and Nutrition (Review Editor)
2012-present, Zebrafish (ad hoc reviewer)
2010-present, Hepatology (ad hoc reviewer)
2010-present, Disease Models and Mechanisms (ad hoc reviewer)
2009-present, Development (ad hoc reviewer)
2009-present, Developmental Biology (ad hoc reviewer)
2009-present, Genesis (ad hoc reviewer)
2008-present, Gastroenterology (ad hoc reviewer)
2008-present, Developmental Dynamics (ad hoc reviewer)
2007-present, Journal of Pediatric Gastroenterology and Nutrition (ad hoc reviewer)
2001-present, Pediatric Emergency Medicine (ad hoc reviewer)

Academic positions

2012-present, Therapeutic Standards Committee (CHOP)

2011-present, Disciplinary Hearing Panel for the University of Pennsylvania

2008-present, Fellowship selection committee, The Children's Hospital of Philadelphia Division of Gastroenterology, Hepatology, and Nutrition, ad hoc interviewer

2010, Internal review committee for Pediatric Endocrinology Fellowship, The Children's Hospital of Philadelphia, member

2009-2013, Undergraduate Student Scholars Program (UPenn Center for Molecular Studies in Digestive and Liver Disease), Selection Committee member

2004-present, Residency Selection Committee, The Children's Hospital of Philadelphia, ad hoc interviewer

Leadership

Memberships in professional organizations

2012-present, Society for Pediatric Research
2002-present, American Association for the Study of Liver Disease

  • 2011-present, Member, Pediatric Special Interest Group
  • 2013-present, 
Member, Pediatric Special Interest Group Single Topic Conference subcommittee

2001-present, North American Society for Gastroenterology, Hepatology, and Nutrition

  • 2011-present, Chair, Study Section Grant Review 
  • 2009-2011, member, Research Committee

 

Original Papers

2013

Shuang Cui, Melissa Leyva-Vega, Ellen A. Tsai, Steven F. EauClaire, Joseph T. Glessner, Hakon Hakonarson, Marcella Devoto, Barbara A. Haber, Nancy B. Spinner, and Randolph P. Matthews: Evidence from human and zebrafish that GPC1 is a biliary atresia susceptibility gene. Gastroenterology 144: 1107-1115, May 2013.

Shuang Cui, Steven F. EauClaire, Randolph P. Matthews: Interferon-gamma directly mediates developmental biliary defects. Zebrafish 10: 177-183, May 2013.

Henry Lin, Richard Rutstein, Joshua Cantor, Sudha Anupindi, and Randolph P. Matthews: Cholangiocarcinoma in a Young Woman with Perinatally Acquired HIV. J. Pediatr. Gastroenterol. Nutr. Page: e43-46, June 2013.

Shah, S., Conlin, L.K., Gomez, L., Aagenaes, Ø., Eiklid, K., Knisely, A.S., Mennuti, M., Matthews, R.P., Spinner, N.B., and Bull, L.N.: CCBE1 mutation identified in two siblings, one of whom manifested Lymphedema-Cholestasis syndrome, and the other, Fetal Hydrops. PLoS ONE 8: e75770, September 2013.

Wilkins, B.J., Lorent, K., Matthews, R.P., and Pack, M: p53-mediated biliary defects caused by knockdown of cirh1a, the zebrafish homolog of the gene responsible for North American Indian Childhood Cirrhosis. PLoS ONE in press.

2012

Steven F. Eauclaire, Shuang Cui, Liyuan Ma, James Matous, Florence L. Marlow, Tripti Gupta, Harold A. Burgess, Eliott W. Abrams, Lee D. Kapp, Michael Granato, Mary C. Mullins, and Randolph P. Matthews.: Mutations in vacuolar H(+)-ATPase subunits lead to biliary developmental defects in zebrafish. Dev Biol 365(2): 434-444, May 2012.

2011

Leyva-Vega M, Weiss P, Ganesh J, Conlin L, Spinner N, Matthews R. Significant liver disease in a patient with Y116H mutation in the MVK gene. Am. J. Med. Genet. 2011 Jun;155A(6):1461-4. doi: 10.1002/ajmg.a.33915. Epub 2011 May 5. No abstract available.

Matthews R, EauClaire S, Mugnier M, Lorent K, Cui S, Ross M, et al. DNA hypomethylation causes bile duct defects in zebrafish and is a distinguishing feature of infantile biliary atresia. Hepatology. 2011 Mar;53(3):905-14. doi: 10.1002/hep.24106. Epub 2011 Feb 11. Read the abstract

Cui S, Erlichman J, Russo P, Haber B, Matthews R: Intrahepatic biliary anomalies in a patient with Mowat-Wilson syndrome uncover a role for the zinc finger homeobox gene zfhx1b in vertebrate biliary development. J. Pediatr. Gastroenterol. Nutr. 2011 Mar;52(3):339-44. Read the abstract

2010

Cullinane AR, Straatman-Iwanowska A, Zaucker A, Wakabayashi Y, Bruce CK, Luo G, Rahman F, et al. Mutations in VIPAR cause an arthrogryposis, renal dysfunction and cholestasis syndrome phenotype with defects in epithelial polarization. Nat Genet. 2010 Apr;42(4):303-12. Epub 2010 Feb 28. Read the abstract

2009

Matthews RP, Lorent K, Mañoral-Mobias R, Huang Y, Gong W, Murray IV, et al. TNFalpha-dependent hepatic steatosis and liver degeneration caused by mutation of zebrafish S-adenosylhomocysteine hydrolase. Development. 2009 Mar;136(5):865-75. Read the abstract

Hand NJ, Master ZR, Eauclaire SF, Weinblatt DE, Matthews RP, Friedman JR. The microRNA-30 family is required for vertebrate hepatobiliary development. Gastroenterology. 2009 Mar;136(3):1081-90. Epub 2008 Dec 9. Read the abstract

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