Medical Status of 219 Children with Biliary Atresia Surviving Long-Term with their Native Livers: Results from a North American Multicenter Consortium
Identifieur interne : 000113 ( Canada/Analysis ); précédent : 000112; suivant : 000114Medical Status of 219 Children with Biliary Atresia Surviving Long-Term with their Native Livers: Results from a North American Multicenter Consortium
Auteurs : Vicky Lee Ng [Canada] ; Barbara H. Haber [États-Unis] ; John C. Magee [États-Unis] ; Alexander Miethke [États-Unis] ; Karen F. Murray [États-Unis] ; Sonia Michail [États-Unis] ; Saul J. Karpen [États-Unis] ; Nanda Kerkar [États-Unis] ; Jeanp. Molleston [États-Unis] ; Rene Romero [États-Unis] ; Philip Rosenthal [États-Unis] ; Kathleen B. Schwarz [États-Unis] ; Benjamin L. Shneider [États-Unis] ; Yumirle P. Turmelle [États-Unis] ; Estella M. Alonso [États-Unis] ; Averell H. Sherker [États-Unis] ; Ronald J. Sokol [États-Unis]Source :
- The Journal of pediatrics [ 0022-3476 ] ; 2014.
Descripteurs français
- KwdFr :
- MESH :
- Wicri :
- geographic : Canada, États-Unis.
English descriptors
- KwdEn :
- MESH :
- geographic : Canada, United States.
- surgery : Biliary Atresia, Liver.
- Child, Enterostomy, Female, Health Status, Humans, Male, Quality of Life, Survivors, Time Factors.
Abstract
To examine the medical status of children with biliary atresia (BA) with their native livers after hepatic portoenterostomy (HPE) surgery.
The Childhood Liver Disease Research and Education Network (ChiLDREN) 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, gamma glutamyl transpeptidase, platelet count, total bilirubin, International Normalized Ratio, and albumin) and normal Health-Related Quality of Life (HRQOL) 5 or more years after HPE.
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 (interquartile range [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. HRQOL was reported normal by 53% of patients. However, only 1.8% met the study definition of “ideal” outcome. Individual tests of liver synthetic function (TB, Alb, and INR) were normal in 75%, 85% and 73% of the study cohort.
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.
Url:
DOI: 10.1016/j.jpeds.2014.05.038
PubMed: 25015575
PubMed Central: 4144331
Affiliations:
- Canada, États-Unis
- Californie, Colorado, Géorgie (États-Unis), Illinois, Indiana, Maryland, Michigan, Missouri (État), Ohio, Ontario, Pennsylvanie, Washington (État), État de New York
- Toronto
- Université de Toronto
Links toward previous steps (curation, corpus...)
- to stream Pmc, to step Corpus: 000E82
- to stream Pmc, to step Curation: 000E57
- to stream Pmc, to step Checkpoint: 000D16
- to stream PubMed, to step Corpus: 001E08
- to stream PubMed, to step Curation: 001D99
- to stream PubMed, to step Checkpoint: 001D99
- to stream Ncbi, to step Merge: 002972
- to stream Ncbi, to step Curation: 002972
- to stream Ncbi, to step Checkpoint: 002972
- to stream Main, to step Merge: 002277
- to stream Main, to step Curation: 002189
- to stream Main, to step Exploration: 002189
- to stream Canada, to step Extraction: 000113
Links to Exploration step
PMC:4144331Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Medical Status of 219 Children with Biliary Atresia Surviving Long-Term with their Native Livers: Results from a North American Multicenter Consortium</title><author><name sortKey="Ng, Vicky Lee" sort="Ng, Vicky Lee" uniqKey="Ng V" first="Vicky Lee" last="Ng">Vicky Lee Ng</name><affiliation wicri:level="4"><nlm:aff id="A1">Division of Pediatric Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada</nlm:aff><country xml:lang="fr">Canada</country><wicri:regionArea>Division of Pediatric Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, University of Toronto, Toronto, ON</wicri:regionArea><orgName type="university">Université de Toronto</orgName><placeName><settlement type="city">Toronto</settlement><region type="state">Ontario</region></placeName></affiliation></author><author><name sortKey="Haber, Barbara H" sort="Haber, Barbara H" uniqKey="Haber B" first="Barbara H." last="Haber">Barbara H. Haber</name><affiliation wicri:level="2"><nlm:aff id="A2">Division of Gastroenterology & Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Pennsylvanie</region></placeName><wicri:cityArea>Division of Gastroenterology & Nutrition, Children's Hospital of Philadelphia, Philadelphia</wicri:cityArea></affiliation></author><author><name sortKey="Magee, John C" sort="Magee, John C" uniqKey="Magee J" first="John C." last="Magee">John C. Magee</name><affiliation wicri:level="2"><nlm:aff id="A3">Department of Surgery, University of Michigan Medical School, Ann Arbor, MI</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Michigan</region></placeName><wicri:cityArea>Department of Surgery, University of Michigan Medical School, Ann Arbor</wicri:cityArea></affiliation></author><author><name sortKey="Miethke, Alexander" sort="Miethke, Alexander" uniqKey="Miethke A" first="Alexander" last="Miethke">Alexander Miethke</name><affiliation wicri:level="2"><nlm:aff id="A4">Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Ohio</region></placeName><wicri:cityArea>Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati</wicri:cityArea></affiliation></author><author><name sortKey="Murray, Karen F" sort="Murray, Karen F" uniqKey="Murray K" first="Karen F." last="Murray">Karen F. Murray</name><affiliation wicri:level="2"><nlm:aff id="A5">Hepatobiliary Program, Seattle Children'sHospital and University of Washington, Seattle, WA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Washington (État)</region></placeName><wicri:cityArea>Hepatobiliary Program, Seattle Children'sHospital and University of Washington, Seattle</wicri:cityArea></affiliation></author><author><name sortKey="Michail, Sonia" sort="Michail, Sonia" uniqKey="Michail S" first="Sonia" last="Michail">Sonia Michail</name><affiliation wicri:level="2"><nlm:aff id="A6">Department of Gastroenterology and Nutrition, Children's Hospital Los Angeles, Keck School of Medicine University of Southern California, Los Angeles, CA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Californie</region></placeName><wicri:cityArea>Department of Gastroenterology and Nutrition, Children's Hospital Los Angeles, Keck School of Medicine University of Southern California, Los Angeles</wicri:cityArea></affiliation></author><author><name sortKey="Karpen, Saul J" sort="Karpen, Saul J" uniqKey="Karpen S" first="Saul J." last="Karpen">Saul J. Karpen</name><affiliation><nlm:aff id="A7">Texas Children's Hospital, Houston, Tx</nlm:aff><wicri:noCountry code="subfield">Tx</wicri:noCountry></affiliation><affiliation wicri:level="2"><nlm:aff id="A8">Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta, Emory University, Atlanta, GA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Géorgie (États-Unis)</region></placeName><wicri:cityArea>Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta, Emory University, Atlanta</wicri:cityArea></affiliation></author><author><name sortKey="Kerkar, Nanda" sort="Kerkar, Nanda" uniqKey="Kerkar N" first="Nanda" last="Kerkar">Nanda Kerkar</name><affiliation wicri:level="2"><nlm:aff id="A6">Department of Gastroenterology and Nutrition, Children's Hospital Los Angeles, Keck School of Medicine University of Southern California, Los Angeles, CA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Californie</region></placeName><wicri:cityArea>Department of Gastroenterology and Nutrition, Children's Hospital Los Angeles, Keck School of Medicine University of Southern California, Los Angeles</wicri:cityArea></affiliation><affiliation wicri:level="2"><nlm:aff id="A9">Division of Pediatric Hepatology, Mount Sinai School of Medicine, New York, NY</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">État de New York</region></placeName><wicri:cityArea>Division of Pediatric Hepatology, Mount Sinai School of Medicine, New York</wicri:cityArea></affiliation></author><author><name sortKey="Molleston, Jeanp" sort="Molleston, Jeanp" uniqKey="Molleston J" first="Jeanp." last="Molleston">Jeanp. Molleston</name><affiliation wicri:level="2"><nlm:aff id="A10">Pediatric Gastroenterology, Hepatology and Nutrition, Riley Hospital for Children, Indiana University, Indianapolis, IN</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Indiana</region></placeName><wicri:cityArea>Pediatric Gastroenterology, Hepatology and Nutrition, Riley Hospital for Children, Indiana University, Indianapolis</wicri:cityArea></affiliation></author><author><name sortKey="Romero, Rene" sort="Romero, Rene" uniqKey="Romero R" first="Rene" last="Romero">Rene Romero</name><affiliation wicri:level="2"><nlm:aff id="A8">Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta, Emory University, Atlanta, GA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Géorgie (États-Unis)</region></placeName><wicri:cityArea>Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta, Emory University, Atlanta</wicri:cityArea></affiliation></author><author><name sortKey="Rosenthal, Philip" sort="Rosenthal, Philip" uniqKey="Rosenthal P" first="Philip" last="Rosenthal">Philip Rosenthal</name><affiliation wicri:level="2"><nlm:aff id="A11">Pediatrics Gastroenterology, Hepatology and Nutrition, UCSF Benioff Children's Hospital, University of California, San Francisco, CA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Californie</region></placeName><wicri:cityArea>Pediatrics Gastroenterology, Hepatology and Nutrition, UCSF Benioff Children's Hospital, University of California, San Francisco</wicri:cityArea></affiliation></author><author><name sortKey="Schwarz, Kathleen B" sort="Schwarz, Kathleen B" uniqKey="Schwarz K" first="Kathleen B." last="Schwarz">Kathleen B. Schwarz</name><affiliation wicri:level="2"><nlm:aff id="A12">Division of Pediatric Gastroenterology and Nutrition, Johns Hopkins Medical Institutions,Baltimore, MD</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Maryland</region></placeName><wicri:cityArea>Division of Pediatric Gastroenterology and Nutrition, Johns Hopkins Medical Institutions,Baltimore</wicri:cityArea></affiliation></author><author><name sortKey="Shneider, Benjamin L" sort="Shneider, Benjamin L" uniqKey="Shneider B" first="Benjamin L." last="Shneider">Benjamin L. Shneider</name><affiliation wicri:level="2"><nlm:aff id="A13">Department of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital Pittsburgh of UPMC, Pittsburgh, PA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Pennsylvanie</region></placeName><wicri:cityArea>Department of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital Pittsburgh of UPMC, Pittsburgh</wicri:cityArea></affiliation></author><author><name sortKey="P Turmelle, Yumirle" sort="P Turmelle, Yumirle" uniqKey="P Turmelle Y" first="Yumirle" last="P. Turmelle">Yumirle P. Turmelle</name><affiliation wicri:level="2"><nlm:aff id="A14">Division of Gastroenterology and Nutrition, Washington University, St. Louis, MO</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Missouri (État)</region></placeName><wicri:cityArea>Division of Gastroenterology and Nutrition, Washington University, St. Louis</wicri:cityArea></affiliation></author><author><name sortKey="Alonso, Estella M" sort="Alonso, Estella M" uniqKey="Alonso E" first="Estella M." last="Alonso">Estella M. Alonso</name><affiliation wicri:level="2"><nlm:aff id="A15">Division of Pediatric Gastroenterology, Hepatology and Nutrition, Ann and Robert H. Lurie Children's Hospital and Northwestern University, Chicago, IL</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Illinois</region></placeName><wicri:cityArea>Division of Pediatric Gastroenterology, Hepatology and Nutrition, Ann and Robert H. Lurie Children's Hospital and Northwestern University, Chicago</wicri:cityArea></affiliation></author><author><name sortKey="Sherker, Averell H" sort="Sherker, Averell H" uniqKey="Sherker A" first="Averell H." last="Sherker">Averell H. Sherker</name><affiliation wicri:level="2"><nlm:aff id="A16">Liver Diseases Research Branch, NIDDK, NIH, Bethesda, MD</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Maryland</region></placeName><wicri:cityArea>Liver Diseases Research Branch, NIDDK, NIH, Bethesda</wicri:cityArea></affiliation></author><author><name sortKey="Sokol, Ronald J" sort="Sokol, Ronald J" uniqKey="Sokol R" first="Ronald J." last="Sokol">Ronald J. Sokol</name><affiliation wicri:level="2"><nlm:aff id="A17">Derpatment of Gastroenterology, Hepatology and Nutrition, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Colorado</region></placeName><wicri:cityArea>Derpatment of Gastroenterology, Hepatology and Nutrition, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora</wicri:cityArea></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">25015575</idno><idno type="pmc">4144331</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4144331</idno><idno type="RBID">PMC:4144331</idno><idno type="doi">10.1016/j.jpeds.2014.05.038</idno><date when="2014">2014</date><idno type="wicri:Area/Pmc/Corpus">000E82</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">000E82</idno><idno type="wicri:Area/Pmc/Curation">000E57</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">000E57</idno><idno type="wicri:Area/Pmc/Checkpoint">000D16</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">000D16</idno><idno type="wicri:source">PubMed</idno><idno type="wicri:Area/PubMed/Corpus">001E08</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">001E08</idno><idno type="wicri:Area/PubMed/Curation">001D99</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">001D99</idno><idno type="wicri:Area/PubMed/Checkpoint">001D99</idno><idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">001D99</idno><idno type="wicri:Area/Ncbi/Merge">002972</idno><idno type="wicri:Area/Ncbi/Curation">002972</idno><idno type="wicri:Area/Ncbi/Checkpoint">002972</idno><idno type="wicri:doubleKey">0022-3476:2014:Ng V:medical:status:of</idno><idno type="wicri:Area/Main/Merge">002277</idno><idno type="wicri:Area/Main/Curation">002189</idno><idno type="wicri:Area/Main/Exploration">002189</idno><idno type="wicri:Area/Canada/Extraction">000113</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Medical Status of 219 Children with Biliary Atresia Surviving Long-Term with their Native Livers: Results from a North American Multicenter Consortium</title><author><name sortKey="Ng, Vicky Lee" sort="Ng, Vicky Lee" uniqKey="Ng V" first="Vicky Lee" last="Ng">Vicky Lee Ng</name><affiliation wicri:level="4"><nlm:aff id="A1">Division of Pediatric Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada</nlm:aff><country xml:lang="fr">Canada</country><wicri:regionArea>Division of Pediatric Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, University of Toronto, Toronto, ON</wicri:regionArea><orgName type="university">Université de Toronto</orgName><placeName><settlement type="city">Toronto</settlement><region type="state">Ontario</region></placeName></affiliation></author><author><name sortKey="Haber, Barbara H" sort="Haber, Barbara H" uniqKey="Haber B" first="Barbara H." last="Haber">Barbara H. Haber</name><affiliation wicri:level="2"><nlm:aff id="A2">Division of Gastroenterology & Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Pennsylvanie</region></placeName><wicri:cityArea>Division of Gastroenterology & Nutrition, Children's Hospital of Philadelphia, Philadelphia</wicri:cityArea></affiliation></author><author><name sortKey="Magee, John C" sort="Magee, John C" uniqKey="Magee J" first="John C." last="Magee">John C. Magee</name><affiliation wicri:level="2"><nlm:aff id="A3">Department of Surgery, University of Michigan Medical School, Ann Arbor, MI</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Michigan</region></placeName><wicri:cityArea>Department of Surgery, University of Michigan Medical School, Ann Arbor</wicri:cityArea></affiliation></author><author><name sortKey="Miethke, Alexander" sort="Miethke, Alexander" uniqKey="Miethke A" first="Alexander" last="Miethke">Alexander Miethke</name><affiliation wicri:level="2"><nlm:aff id="A4">Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Ohio</region></placeName><wicri:cityArea>Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati</wicri:cityArea></affiliation></author><author><name sortKey="Murray, Karen F" sort="Murray, Karen F" uniqKey="Murray K" first="Karen F." last="Murray">Karen F. Murray</name><affiliation wicri:level="2"><nlm:aff id="A5">Hepatobiliary Program, Seattle Children'sHospital and University of Washington, Seattle, WA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Washington (État)</region></placeName><wicri:cityArea>Hepatobiliary Program, Seattle Children'sHospital and University of Washington, Seattle</wicri:cityArea></affiliation></author><author><name sortKey="Michail, Sonia" sort="Michail, Sonia" uniqKey="Michail S" first="Sonia" last="Michail">Sonia Michail</name><affiliation wicri:level="2"><nlm:aff id="A6">Department of Gastroenterology and Nutrition, Children's Hospital Los Angeles, Keck School of Medicine University of Southern California, Los Angeles, CA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Californie</region></placeName><wicri:cityArea>Department of Gastroenterology and Nutrition, Children's Hospital Los Angeles, Keck School of Medicine University of Southern California, Los Angeles</wicri:cityArea></affiliation></author><author><name sortKey="Karpen, Saul J" sort="Karpen, Saul J" uniqKey="Karpen S" first="Saul J." last="Karpen">Saul J. Karpen</name><affiliation><nlm:aff id="A7">Texas Children's Hospital, Houston, Tx</nlm:aff><wicri:noCountry code="subfield">Tx</wicri:noCountry></affiliation><affiliation wicri:level="2"><nlm:aff id="A8">Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta, Emory University, Atlanta, GA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Géorgie (États-Unis)</region></placeName><wicri:cityArea>Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta, Emory University, Atlanta</wicri:cityArea></affiliation></author><author><name sortKey="Kerkar, Nanda" sort="Kerkar, Nanda" uniqKey="Kerkar N" first="Nanda" last="Kerkar">Nanda Kerkar</name><affiliation wicri:level="2"><nlm:aff id="A6">Department of Gastroenterology and Nutrition, Children's Hospital Los Angeles, Keck School of Medicine University of Southern California, Los Angeles, CA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Californie</region></placeName><wicri:cityArea>Department of Gastroenterology and Nutrition, Children's Hospital Los Angeles, Keck School of Medicine University of Southern California, Los Angeles</wicri:cityArea></affiliation><affiliation wicri:level="2"><nlm:aff id="A9">Division of Pediatric Hepatology, Mount Sinai School of Medicine, New York, NY</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">État de New York</region></placeName><wicri:cityArea>Division of Pediatric Hepatology, Mount Sinai School of Medicine, New York</wicri:cityArea></affiliation></author><author><name sortKey="Molleston, Jeanp" sort="Molleston, Jeanp" uniqKey="Molleston J" first="Jeanp." last="Molleston">Jeanp. Molleston</name><affiliation wicri:level="2"><nlm:aff id="A10">Pediatric Gastroenterology, Hepatology and Nutrition, Riley Hospital for Children, Indiana University, Indianapolis, IN</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Indiana</region></placeName><wicri:cityArea>Pediatric Gastroenterology, Hepatology and Nutrition, Riley Hospital for Children, Indiana University, Indianapolis</wicri:cityArea></affiliation></author><author><name sortKey="Romero, Rene" sort="Romero, Rene" uniqKey="Romero R" first="Rene" last="Romero">Rene Romero</name><affiliation wicri:level="2"><nlm:aff id="A8">Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta, Emory University, Atlanta, GA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Géorgie (États-Unis)</region></placeName><wicri:cityArea>Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta, Emory University, Atlanta</wicri:cityArea></affiliation></author><author><name sortKey="Rosenthal, Philip" sort="Rosenthal, Philip" uniqKey="Rosenthal P" first="Philip" last="Rosenthal">Philip Rosenthal</name><affiliation wicri:level="2"><nlm:aff id="A11">Pediatrics Gastroenterology, Hepatology and Nutrition, UCSF Benioff Children's Hospital, University of California, San Francisco, CA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Californie</region></placeName><wicri:cityArea>Pediatrics Gastroenterology, Hepatology and Nutrition, UCSF Benioff Children's Hospital, University of California, San Francisco</wicri:cityArea></affiliation></author><author><name sortKey="Schwarz, Kathleen B" sort="Schwarz, Kathleen B" uniqKey="Schwarz K" first="Kathleen B." last="Schwarz">Kathleen B. Schwarz</name><affiliation wicri:level="2"><nlm:aff id="A12">Division of Pediatric Gastroenterology and Nutrition, Johns Hopkins Medical Institutions,Baltimore, MD</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Maryland</region></placeName><wicri:cityArea>Division of Pediatric Gastroenterology and Nutrition, Johns Hopkins Medical Institutions,Baltimore</wicri:cityArea></affiliation></author><author><name sortKey="Shneider, Benjamin L" sort="Shneider, Benjamin L" uniqKey="Shneider B" first="Benjamin L." last="Shneider">Benjamin L. Shneider</name><affiliation wicri:level="2"><nlm:aff id="A13">Department of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital Pittsburgh of UPMC, Pittsburgh, PA</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Pennsylvanie</region></placeName><wicri:cityArea>Department of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital Pittsburgh of UPMC, Pittsburgh</wicri:cityArea></affiliation></author><author><name sortKey="P Turmelle, Yumirle" sort="P Turmelle, Yumirle" uniqKey="P Turmelle Y" first="Yumirle" last="P. Turmelle">Yumirle P. Turmelle</name><affiliation wicri:level="2"><nlm:aff id="A14">Division of Gastroenterology and Nutrition, Washington University, St. Louis, MO</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Missouri (État)</region></placeName><wicri:cityArea>Division of Gastroenterology and Nutrition, Washington University, St. Louis</wicri:cityArea></affiliation></author><author><name sortKey="Alonso, Estella M" sort="Alonso, Estella M" uniqKey="Alonso E" first="Estella M." last="Alonso">Estella M. Alonso</name><affiliation wicri:level="2"><nlm:aff id="A15">Division of Pediatric Gastroenterology, Hepatology and Nutrition, Ann and Robert H. Lurie Children's Hospital and Northwestern University, Chicago, IL</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Illinois</region></placeName><wicri:cityArea>Division of Pediatric Gastroenterology, Hepatology and Nutrition, Ann and Robert H. Lurie Children's Hospital and Northwestern University, Chicago</wicri:cityArea></affiliation></author><author><name sortKey="Sherker, Averell H" sort="Sherker, Averell H" uniqKey="Sherker A" first="Averell H." last="Sherker">Averell H. Sherker</name><affiliation wicri:level="2"><nlm:aff id="A16">Liver Diseases Research Branch, NIDDK, NIH, Bethesda, MD</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Maryland</region></placeName><wicri:cityArea>Liver Diseases Research Branch, NIDDK, NIH, Bethesda</wicri:cityArea></affiliation></author><author><name sortKey="Sokol, Ronald J" sort="Sokol, Ronald J" uniqKey="Sokol R" first="Ronald J." last="Sokol">Ronald J. Sokol</name><affiliation wicri:level="2"><nlm:aff id="A17">Derpatment of Gastroenterology, Hepatology and Nutrition, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO</nlm:aff><country xml:lang="fr">États-Unis</country><placeName><region type="state">Colorado</region></placeName><wicri:cityArea>Derpatment of Gastroenterology, Hepatology and Nutrition, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora</wicri:cityArea></affiliation></author></analytic><series><title level="j">The Journal of pediatrics</title><idno type="ISSN">0022-3476</idno><idno type="eISSN">1097-6833</idno><imprint><date when="2014">2014</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Biliary Atresia (surgery)</term><term>Canada</term><term>Child</term><term>Enterostomy</term><term>Female</term><term>Health Status</term><term>Humans</term><term>Liver (surgery)</term><term>Male</term><term>Quality of Life</term><term>Survivors</term><term>Time Factors</term><term>United States</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Atrésie des voies biliaires ()</term><term>Canada</term><term>Enfant</term><term>Entérostomie</term><term>Facteurs temps</term><term>Femelle</term><term>Foie ()</term><term>Humains</term><term>Mâle</term><term>Qualité de vie</term><term>Survivants</term><term>État de santé</term><term>États-Unis d'Amérique</term></keywords><keywords scheme="MESH" type="geographic" xml:lang="en"><term>Canada</term><term>United States</term></keywords><keywords scheme="MESH" qualifier="surgery" xml:lang="en"><term>Biliary Atresia</term><term>Liver</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Child</term><term>Enterostomy</term><term>Female</term><term>Health Status</term><term>Humans</term><term>Male</term><term>Quality of Life</term><term>Survivors</term><term>Time Factors</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Atrésie des voies biliaires</term><term>Canada</term><term>Enfant</term><term>Entérostomie</term><term>Facteurs temps</term><term>Femelle</term><term>Foie</term><term>Humains</term><term>Mâle</term><term>Qualité de vie</term><term>Survivants</term><term>État de santé</term><term>États-Unis d'Amérique</term></keywords><keywords scheme="Wicri" type="geographic" xml:lang="fr"><term>Canada</term><term>États-Unis</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><sec id="S1"><title>Objectives</title><p id="P1">To examine the medical status of children with biliary atresia (BA) with their native livers after hepatic portoenterostomy (HPE) surgery.</p></sec><sec id="S2"><title>Study design</title><p id="P2">The Childhood Liver Disease Research and Education Network (ChiLDREN) 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, gamma glutamyl transpeptidase, platelet count, total bilirubin, International Normalized Ratio, and albumin) and normal Health-Related Quality of Life (HRQOL) 5 or more years after HPE.</p></sec><sec id="S3"><title>Results</title><p id="P3">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 (interquartile range [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. HRQOL was reported normal by 53% of patients. However, only 1.8% met the study definition of “ideal” outcome. Individual tests of liver synthetic function (TB, Alb, and INR) were normal in 75%, 85% and 73% of the study cohort.</p></sec><sec id="S4"><title>Conclusion</title><p id="P4">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.</p></sec></div></front></TEI><affiliations><list><country><li>Canada</li><li>États-Unis</li></country><region><li>Californie</li><li>Colorado</li><li>Géorgie (États-Unis)</li><li>Illinois</li><li>Indiana</li><li>Maryland</li><li>Michigan</li><li>Missouri (État)</li><li>Ohio</li><li>Ontario</li><li>Pennsylvanie</li><li>Washington (État)</li><li>État de New York</li></region><settlement><li>Toronto</li></settlement><orgName><li>Université de Toronto</li></orgName></list><tree><country name="Canada"><region name="Ontario"><name sortKey="Ng, Vicky Lee" sort="Ng, Vicky Lee" uniqKey="Ng V" first="Vicky Lee" last="Ng">Vicky Lee Ng</name></region></country><country name="États-Unis"><region name="Pennsylvanie"><name sortKey="Haber, Barbara H" sort="Haber, Barbara H" uniqKey="Haber B" first="Barbara H." last="Haber">Barbara H. Haber</name></region><name sortKey="Alonso, Estella M" sort="Alonso, Estella M" uniqKey="Alonso E" first="Estella M." last="Alonso">Estella M. Alonso</name><name sortKey="Karpen, Saul J" sort="Karpen, Saul J" uniqKey="Karpen S" first="Saul J." last="Karpen">Saul J. Karpen</name><name sortKey="Kerkar, Nanda" sort="Kerkar, Nanda" uniqKey="Kerkar N" first="Nanda" last="Kerkar">Nanda Kerkar</name><name sortKey="Kerkar, Nanda" sort="Kerkar, Nanda" uniqKey="Kerkar N" first="Nanda" last="Kerkar">Nanda Kerkar</name><name sortKey="Magee, John C" sort="Magee, John C" uniqKey="Magee J" first="John C." last="Magee">John C. Magee</name><name sortKey="Michail, Sonia" sort="Michail, Sonia" uniqKey="Michail S" first="Sonia" last="Michail">Sonia Michail</name><name sortKey="Miethke, Alexander" sort="Miethke, Alexander" uniqKey="Miethke A" first="Alexander" last="Miethke">Alexander Miethke</name><name sortKey="Molleston, Jeanp" sort="Molleston, Jeanp" uniqKey="Molleston J" first="Jeanp." last="Molleston">Jeanp. Molleston</name><name sortKey="Murray, Karen F" sort="Murray, Karen F" uniqKey="Murray K" first="Karen F." last="Murray">Karen F. Murray</name><name sortKey="P Turmelle, Yumirle" sort="P Turmelle, Yumirle" uniqKey="P Turmelle Y" first="Yumirle" last="P. Turmelle">Yumirle P. Turmelle</name><name sortKey="Romero, Rene" sort="Romero, Rene" uniqKey="Romero R" first="Rene" last="Romero">Rene Romero</name><name sortKey="Rosenthal, Philip" sort="Rosenthal, Philip" uniqKey="Rosenthal P" first="Philip" last="Rosenthal">Philip Rosenthal</name><name sortKey="Schwarz, Kathleen B" sort="Schwarz, Kathleen B" uniqKey="Schwarz K" first="Kathleen B." last="Schwarz">Kathleen B. Schwarz</name><name sortKey="Sherker, Averell H" sort="Sherker, Averell H" uniqKey="Sherker A" first="Averell H." last="Sherker">Averell H. Sherker</name><name sortKey="Shneider, Benjamin L" sort="Shneider, Benjamin L" uniqKey="Shneider B" first="Benjamin L." last="Shneider">Benjamin L. Shneider</name><name sortKey="Sokol, Ronald J" sort="Sokol, Ronald J" uniqKey="Sokol R" first="Ronald J." last="Sokol">Ronald J. Sokol</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Amérique/explor/PittsburghV1/Data/Canada/Analysis
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 000113 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Canada/Analysis/biblio.hfd -nk 000113 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien
|wiki= Wicri/Amérique
|area= PittsburghV1
|flux= Canada
|étape= Analysis
|type= RBID
|clé= PMC:4144331
|texte= Medical Status of 219 Children with Biliary Atresia Surviving Long-Term with their Native Livers: Results from a North American Multicenter Consortium
}}
Pour générer des pages wiki
HfdIndexSelect -h $EXPLOR_AREA/Data/Canada/Analysis/RBID.i -Sk "pubmed:25015575" \
| HfdSelect -Kh $EXPLOR_AREA/Data/Canada/Analysis/biblio.hfd \
| NlmPubMed2Wicri -a PittsburghV1
| This area was generated with Dilib version V0.6.38. |  |