Published Research

Total Records: 86; Page 1 of 9
TitleMediaAuthorsPublicationPublication Year Descending order
Identification of PKD1L1 Gene Variants in Children with the 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 N, 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, ChiLDReN
Hepatology (2019)
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 N, 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, ChiLDReN Hepatology2019
Clinically Evident Portal Hypertension: An Operational Research Definition for Future Investigations in the Pediatric Population.
Bass LM, Shneider BL, Henn LL, Goodrich NP, Magee JC, Childhood Liver Disease Research and Education Net
Journal of Pediatric Gastroenterology and Nutrition (2019)
Bass LM, Shneider BL, Henn LL, Goodrich NP, Magee JC, Childhood Liver Disease Research and Education Net Journal of Pediatric Gastroenterology and Nutrition2019
A Phase I/IIa Trial of Intravenous Immunoglobulin (IVIg) Therapy Following Portoenterostomy in Infants with Biliary Atresia.
Mack CL, Spino C, Alonso EM, Bezerra JA, Moore J, Goodhue C, Ng VL, Karpen SJ, Venkat VL, Loomes KM, Wang KS, Sherker AH, Magee JC, Sokol RJ, ChiLDReN
Journal of Pediatric Gastroenterology and Nutrition (2019)
Mack CL, Spino C, Alonso EM, Bezerra JA, Moore J, Goodhue C, Ng VL, Karpen SJ, Venkat VL, Loomes KM, Wang KS, Sherker AH, Magee JC, Sokol RJ, ChiLDReN Journal of Pediatric Gastroenterology and Nutrition2019
Correlation of Immune Markers With Outcomes in Biliary Atresia Following Intravenous Immunoglobulin Therapy
Kim SJ, Moore J, Alonso EM, Bendarek J, Bezerra JA, Goodhue C, Karpen SJ, Loomes KM, Magee JC, Ng VL, Sherker AH, Smith C, Spino C, Venkat VL, Wang K, Sokol RJ, Mack CL, ChiLDReN
Hepatology Communications (2019)
Kim SJ, Moore J, Alonso EM, Bendarek J, Bezerra JA, Goodhue C, Karpen SJ, Loomes KM, Magee JC, Ng VL, Sherker AH, Smith C, Spino C, Venkat VL, Wang K, Sokol RJ, Mack CL, ChiLDReN Hepatology Communications2019
A genome wide association study was performed to identify genes that could be related to biliary atresia susceptibility. The top gene candidate was EFEMP1, which encodes the protein fibulin-3, found in the extracellular matrix around bile ducts. EFEMP1 expression is increased in biliary atresia liver and also in other cholestatic liver disease samples compared to normal liver. The protein is expressed in blood vessels around the ducts in both normal and diseased liver, and expressed in bile ducts only in BA and other diseased liver samples. Further studies will be required to understand how EFEMP1 function might contribute to BA disease susceptibility.
Chen Y, Gilbert MA, Grochowski CM, McEldrew D, Llewellyn J, Waisbourd-Zinman O, Hakonarson H, Bailey-Wilson JE, Russo P, Wells RG, Loomes KM, Spinner N, Devoto M
PLOS Genetics (2018)
Chen Y, Gilbert MA, Grochowski CM, McEldrew D, Llewellyn J, Waisbourd-Zinman O, Hakonarson H, Bailey-Wilson JE, Russo P, Wells RG, Loomes KM, Spinner N, Devoto MPLOS Genetics2018
We hypothesized that if infection is the proximate cause of congenital biliary atresia, an appropriate response to antigen would occur in lymph nodes contiguous with the biliary remnant. We compared the number of follicular germinal centers (GC) in 79 surgically excised hilar lymph nodes (LN) and 27 incidentally discovered cystic duct LNs in 84 subjects at the time of hepatic portoenterostomy (HPE) for biliary atresia (BA) to autopsy controls from the pancreaticobiliary region of non-septic infants >3 months old at death. All 27 control LN lacked GC, a sign in infants of a primary response to antigenic stimulation. GC were found in 53% of 106 LN in 56 of 84 subjects. Visible surgically excised LN contiguous with the most proximal biliary remnants had 1 or more well-formed reactive GC in only 26/51 subjects. Presence of GC and number of GC/LN was unrelated to age at onset of jaundice or to active fibroplasia in the biliary remnant but was related to older age at HPE. Absent GC in visible and incidentally removed cystic duct LNs predicted survival with the native liver at 2 and 3 years after HPE, P = .03, but significance was lost at longer intervals. The uncommon inflammatory lesions occasionally found in remnants could be secondary either to bile-induced injury or secondary infection established as obstruction evolves. The absence of consistent evidence of antigenic stimulation in LN contiguous with the biliary remnant supports existence of at least 1 major alternative to infection in the etiology of biliary atresia.
Bove KE, Sheridan R, Fei L, Anders RA, Chung C, Cummings OW, Finegold MJ, Finn LS, Ranganathan S, Kim G, Lovell MA, Magid MS, Melin-Aldana H, Russo P, Shehata BM, Wang L, White FV, Chen Z, Spino C, Magee JC
Pediatric and Developmental Pathology (2018)
Bove KE, Sheridan R, Fei L, Anders RA, Chung C, Cummings OW, Finegold MJ, Finn LS, Ranganathan S, Kim G, Lovell MA, Magid MS, Melin-Aldana H, Russo P, Shehata BM, Wang L, White FV, Chen Z, Spino C, Magee JCPediatric and Developmental Pathology2018
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 DW, Sherker AH, Sokol RJ
Hepatology Communications (2018)
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 DW, Sherker AH, Sokol RJHepatology Communications2018
Objectives: Cirrhosis occurs in 5-10% of CF (cystic fibrosis) patients, often accompanied by portal hypertension. We analyzed three adverse liver outcomes, variceal bleeding (VB), liver transplant (LT), and liver death (LD), and risk factors for these in CF Foundation Patient Registry (CFFPR) subjects with reported cirrhosis.
Ye W, Narkewicz MR, Leung DH, Karnsakul W, Murray KF, Alonso EM, Magee JC, Schwarzenberg SJ, Weymann A, Molleston JP, CFLD-NET
Journal of Pediatric Gastroenterology and Nutrition (2018)
Ye W, Narkewicz MR, Leung DH, Karnsakul W, Murray KF, Alonso EM, Magee JC, Schwarzenberg SJ, Weymann A, Molleston JP, CFLD-NET Journal of Pediatric Gastroenterology and Nutrition2018
In murine sclerosing cholangitis, intrahepatic regulatory T cell (Treg) responses control hepatic CD8+ T cell proliferation which drive biliary injury and fibrosis. The authors show that IL-2c mediated intrahepatic Treg expansion improves phenotype and conclude that low-dose IL-2 treatment represents a promising new therapy for sclerosing cholangitis.
Taylor AE, Carey AN, Kudira R, Lages CS, Shi T, Lam S, Karns R, Simmons JR, Shanmukhappa K, Almanan M, Chougnet C, Miethke A
Hepatology (2018)
Taylor AE, Carey AN, Kudira R, Lages CS, Shi T, Lam S, Karns R, Simmons JR, Shanmukhappa K, Almanan M, Chougnet C, Miethke AHepatology2018
Bone density in children with chronic liver disease correlates with growth and cholestasis
Loomes KM, Spino C, Goodrich NP, Hangartner TN, Marker AE, Heubi J, Kamath BM, Shneider BL, Rosenthal P, Hertel PM, Karpen SJ, Molleston JP, Murray KF, Schwarz KB, Squires RH, Teckman JH, Turmelle YP, Alonso EM, Sherker AH, Magee JC, Sokol RJ
Hepatology (2018)
Loomes KM, Spino C, Goodrich NP, Hangartner TN, Marker AE, Heubi J, Kamath BM, Shneider BL, Rosenthal P, Hertel PM, Karpen SJ, Molleston JP, Murray KF, Schwarz KB, Squires RH, Teckman JH, Turmelle YP, Alonso EM, Sherker AH, Magee JC, Sokol RJHepatology2018
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