Experts and Doctors on biliary atresia in Philadelphia, Pennsylvania, United States


Locale: Philadelphia, Pennsylvania, United States
Topic: biliary atresia

Top Publications

  1. Hand N, Horner A, Master Z, Boateng L, Leguen C, Uvaydova M, et al. MicroRNA profiling identifies miR-29 as a regulator of disease-associated pathways in experimental biliary atresia. J Pediatr Gastroenterol Nutr. 2012;54:186-92 pubmed publisher
    ..We have used reporter assays to confirm that Igf1 and Il1RAP are direct targets of miR-29...
  2. Zahm A, Hand N, Boateng L, Friedman J. Circulating microRNA is a biomarker of biliary atresia. J Pediatr Gastroenterol Nutr. 2012;55:366-9 pubmed
    ..Circulating microRNAs (miRNAs) are a new class of noninvasive biomarkers with encouraging diagnostic utility...
  3. Cui S, Leyva Vega M, Tsai E, Eauclaire S, Glessner J, Hakonarson H, et al. Evidence from human and zebrafish that GPC1 is a biliary atresia susceptibility gene. Gastroenterology. 2013;144:1107-1115.e3 pubmed publisher
    ..Based on genetic analysis of patients with BA and zebrafish, GPC1 appears to be a BA susceptibility gene. These findings also support a role for Hedgehog signaling in the pathogenesis of BA. ..
  4. Cui S, Eauclaire S, Matthews R. Interferon-gamma directly mediates developmental biliary defects. Zebrafish. 2013;10:177-83 pubmed publisher
    ..These results support an importance of IFN? in mediating biliary defects, and also demonstrate the feasibility of direct injection of intact protein into developing zebrafish larvae. ..
  5. Matthews R, Russo P, Berry G, Piccoli D, Rand E. Biliary atresia associated with a fatty acid oxidation defect. J Pediatr Gastroenterol Nutr. 2002;35:624-8 pubmed
  6. Erlichman J, Hohlweg K, Haber B. Biliary atresia: how medical complications and therapies impact outcome. Expert Rev Gastroenterol Hepatol. 2009;3:425-34 pubmed publisher
    ..As more children with BA survive into adulthood with their native liver, it is important to understand which factors predict good and poor outcomes. ..
  7. 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;53:905-14 pubmed publisher
  8. 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;52:339-44 pubmed publisher
    ..Our studies underscore the importance of genetic contributions in the etiology of infantile hepatobiliary disorders, including biliary atresia. ..
  9. Tsai E, Grochowski C, Loomes K, Bessho K, Hakonarson H, Bezerra J, et al. Replication of a GWAS signal in a Caucasian population implicates ADD3 in susceptibility to biliary atresia. Hum Genet. 2014;133:235-43 pubmed publisher
    ..The role of ADD3 in biliary development is unclear, but our findings suggest that this gene may be functionally relevant for the development of BA. ..