Medical status of 219 children with biliary atresia surviving long-term with their native livers: results from a North American multicenter consortium
Vicky Lee Ng, Barbara H Haber, John C Magee, Alexander Miethke, Karen F Murray, Sonia Michail, Saul J Karpen, Nanda Kerkar, Jean P Molleston, Rene Romero, Philip Rosenthal, Kathleen B Schwarz, Benjamin L Shneider, Yumirle P Turmelle, Estella M Alonso, Averell H Sherker, Ronald J Sokol, Childhood Liver Disease Research and Education Network (CHiLDREN), Kathleen Schwarz, Robert Anders, Paul Colombani, Kim Kafka, Wikrom Karnsakul, Laura Wachter, Ann Lurie, Robert H Lurie, Peter Whitington, Estella Alonso, Lee Bass, Elizabeth Kaurs, Sue Kelly, Hector Melin-Aldana, Denise Rizzo, Ricardo Superina, Jorge Bezerra, Kevin Bove, Julie Denlinger, Andrea Ferris, James Heubi, Pinky Jha, Denise LaGory, Alexander Miethke, Joseph Palermo, Stacey Reed, Kenneth Setchell, Melissa Stamper, Greg Tiao, Ronald Sokol, Tim Byrne, Joan Hines, Michelle Hite, Frederick Karrer, Mark Lovell, Cara Mack, Todd Miller, Michael Narkewicz, Timothy Schardt, Frederick Suchy, Shikha Sundaram, Tracy Urban, Johan Van Hove, Ronen Arnon, Jamie Chu, Ivy Cohen, Brandy Haydel, Kishore Iyer, Margret Magid, Sheetal Ramnath, Kathleen Loomes, Lindsay Brown, Timothy Crisci, Jessi Erlichman, Alex Falsey, Alan Flake, David Piccoli, Elizabeth Rand, Pierre Russo, Nancy Spinner, Eileen Wu, Benjamin Shneider, Feras Alissa, Amy Bookser, A'Delbert Bowen, Kathy Bukauskas, Ronald Jaffe, Douglas Lindblad, George Mazariegos, Roberto Ortiz-Aguayo, David Perlmutter, Sarangarajan Ranganathan, Erin Sandene, Stefan Scholz, Rakesh Sindhi, Donna Smith, Robert Squires Jr, Veena Venkat, Jerry Vockley, Philip Rosenthal, Laura Bull, Scott Fields, Shannon Fleck, Melvin Heyman, Shinjiro Hirose, Grace Kim, Camille Langlois, John Roberts, Yumirle Turmelle, Laura Bontemps, Pat Dillon, Kathleen Harris, Jeffrey Lowell, Stacey Postma, Jonathan Rider, David Rudnick, Janis Stoll, Alexander Weymann, Frances White, Paula Hertel, Zoe Apted, Mary Brandt, Jameisha Brown, Beth Carter, Milton Finegold, Richard Gibbs, Sanjiv Harpavat, Tara McCartney, Jeffery Teckman, Vikki Kociela, Jean Molleston, Molly Bozic, Beth Byam, Oscar Cummings, Ann Klipsch, Cindy Sawyers, Girish Subbarao, Karen West, Karen Murray, Kara Cooper, Laura Finn, Patrick Healey, Simon Horslen, Susan Jacob, Melissa Young, Vicky Ng, Catherine Chung, Maria DeAngelis, Annie Fecteau, Wardah Hannan, Kelsey Hunt, Binita Kamath, Jacob Langer, Constance O'Connor, Claudia Quammie, Stephanie Rankin, Krista Van Roestel, Kasper Wang, Henri Ford, Evelyn Franco, Catherine Goodhue, Nanda Kerker, Sonia Michail, John Pech, Danny Thomas, Larry Wang, Saul Karpen, Carlos Abramowsky, Matthew Clifton, Liezl de la Cruz-Tracey, Nikita Gupta, Dana Hankerson-Dyson, Jim Rhodes, Rene Romero, Bahig Shehata, Rita Tory, Taieshia Turner-Green, Mariam Vos, Allison Wellons, Richard Thompson, Sherry Brown, Edward Doo, Jay Hoofnagle, Averell Sherker, Rebecca Torrance, John Magee, Donna DiFranco, Karen Jones, Trivellore Raghunathan, Cathie Spino, Wen Ye, Vicky Lee Ng, Barbara H Haber, John C Magee, Alexander Miethke, Karen F Murray, Sonia Michail, Saul J Karpen, Nanda Kerkar, Jean P Molleston, Rene Romero, Philip Rosenthal, Kathleen B Schwarz, Benjamin L Shneider, Yumirle P Turmelle, Estella M Alonso, Averell H Sherker, Ronald J Sokol, Childhood Liver Disease Research and Education Network (CHiLDREN), Kathleen Schwarz, Robert Anders, Paul Colombani, Kim Kafka, Wikrom Karnsakul, Laura Wachter, Ann Lurie, Robert H Lurie, Peter Whitington, Estella Alonso, Lee Bass, Elizabeth Kaurs, Sue Kelly, Hector Melin-Aldana, Denise Rizzo, Ricardo Superina, Jorge Bezerra, Kevin Bove, Julie Denlinger, Andrea Ferris, James Heubi, Pinky Jha, Denise LaGory, Alexander Miethke, Joseph Palermo, Stacey Reed, Kenneth Setchell, Melissa Stamper, Greg Tiao, Ronald Sokol, Tim Byrne, Joan Hines, Michelle Hite, Frederick Karrer, Mark Lovell, Cara Mack, Todd Miller, Michael Narkewicz, Timothy Schardt, Frederick Suchy, Shikha Sundaram, Tracy Urban, Johan Van Hove, Ronen Arnon, Jamie Chu, Ivy Cohen, Brandy Haydel, Kishore Iyer, Margret Magid, Sheetal Ramnath, Kathleen Loomes, Lindsay Brown, Timothy Crisci, Jessi Erlichman, Alex Falsey, Alan Flake, David Piccoli, Elizabeth Rand, Pierre Russo, Nancy Spinner, Eileen Wu, Benjamin Shneider, Feras Alissa, Amy Bookser, A'Delbert Bowen, Kathy Bukauskas, Ronald Jaffe, Douglas Lindblad, George Mazariegos, Roberto Ortiz-Aguayo, David Perlmutter, Sarangarajan Ranganathan, Erin Sandene, Stefan Scholz, Rakesh Sindhi, Donna Smith, Robert Squires Jr, Veena Venkat, Jerry Vockley, Philip Rosenthal, Laura Bull, Scott Fields, Shannon Fleck, Melvin Heyman, Shinjiro Hirose, Grace Kim, Camille Langlois, John Roberts, Yumirle Turmelle, Laura Bontemps, Pat Dillon, Kathleen Harris, Jeffrey Lowell, Stacey Postma, Jonathan Rider, David Rudnick, Janis Stoll, Alexander Weymann, Frances White, Paula Hertel, Zoe Apted, Mary Brandt, Jameisha Brown, Beth Carter, Milton Finegold, Richard Gibbs, Sanjiv Harpavat, Tara McCartney, Jeffery Teckman, Vikki Kociela, Jean Molleston, Molly Bozic, Beth Byam, Oscar Cummings, Ann Klipsch, Cindy Sawyers, Girish Subbarao, Karen West, Karen Murray, Kara Cooper, Laura Finn, Patrick Healey, Simon Horslen, Susan Jacob, Melissa Young, Vicky Ng, Catherine Chung, Maria DeAngelis, Annie Fecteau, Wardah Hannan, Kelsey Hunt, Binita Kamath, Jacob Langer, Constance O'Connor, Claudia Quammie, Stephanie Rankin, Krista Van Roestel, Kasper Wang, Henri Ford, Evelyn Franco, Catherine Goodhue, Nanda Kerker, Sonia Michail, John Pech, Danny Thomas, Larry Wang, Saul Karpen, Carlos Abramowsky, Matthew Clifton, Liezl de la Cruz-Tracey, Nikita Gupta, Dana Hankerson-Dyson, Jim Rhodes, Rene Romero, Bahig Shehata, Rita Tory, Taieshia Turner-Green, Mariam Vos, Allison Wellons, Richard Thompson, Sherry Brown, Edward Doo, Jay Hoofnagle, Averell Sherker, Rebecca Torrance, John Magee, Donna DiFranco, Karen Jones, Trivellore Raghunathan, Cathie Spino, Wen Ye
Abstract
Objectives: To examine the medical status of children with biliary atresia (BA) with their native livers after hepato- portoenterostomy (HPE) surgery.
Study design: The Childhood Liver Disease Research and Education Network database was utilized to examine subjects with BA living with their native livers 5 or more years after HPE and to describe the prevalence of subjects with BA with an "ideal" outcome, defined as no clinical evidence of chronic liver disease, normal liver biochemical indices (aspartate aminotransferase, alanine aminotransferase, γ-glutamyl transpeptidase, platelet count, total bilirubin, international normalized ratio, and albumin), and normal health-related quality of life 5 or more years after HPE.
Results: Children with BA (n = 219; 43% male) with median age 9.7 years were studied. Median age at HPE was 56 (range 7-125) days. Median age- and sex-adjusted height and weight z-scores at 5-year follow-up were 0.487 (IQR -0.27 to 1.02) and 0.00 (IQR -0.74 to 0.70), respectively. During the 12 preceding months, cholangitis and bone fractures occurred in 17% and 5.5%, respectively. Health-related quality of life was reported normal by 53% of patients. However, only 1.8% met the study definition of "ideal" outcome. Individual tests of liver synthetic function (total bilirubin, albumin, and international normalized ratio) were normal in 75%, 85%, and 73% of the study cohort.
Conclusion: Cholangitis and fractures in long-term survivors underscore the importance of ongoing medical surveillance. Over 98% of this North American cohort of subjects with BA living with native livers 5 or more years after HPE have clinical or biochemical evidence of chronic liver disease.
Trial registration: ClinicalTrials.gov NCT00345553.
Conflict of interest statement
The authors declare no conflicts of interest.
Copyright © 2014 Elsevier Inc. All rights reserved.
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Source: PubMed