ArticleThe Journal of pediatrics2014
Medical status of 219 children with biliary atresia surviving long-term with their native livers: results from a North American multicenter consortium.
Article in The Journal of pediatrics, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 34 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
34 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Prophylactic oral antibiotic use for the prevention of cholangitis after Kasai portoenterostomy in patients with biliary atresia: a systematic review and meta-analysis.Frontiers in pediatrics · 2026Pooled it
- Cyclooxygenase-2 Inhibitor Reduces Hepatic Stiffness in Pediatric Chronic Liver Disease Patients Following Kasai Portoenterostomy.Yonsei medical journal · 2016Trial
- Spleen and liver shear wave elastography for assessment of portal hypertension in children with biliary atresia.Scientific reports · 2026Article
- Taurocholic acid induces intrahepatic cholangiocyte cell proliferation via activating NRAS and YAP1.PloS one · 2026Article
- Does Gamma-Glutamyl Transpeptidase Serum Level Play a Role as a Prognostic Marker in Biliary Atresia, an Immune-Mediated Cholangiopathy in Children?Journal of clinical medicine · 2025Article
- Portal hypertension in biliary atresia: a Japanese biliary atresia registry study.Pediatric surgery international · 2025Article
- Establishment of Biliary Atresia Prognostic Classification System via Survival-Based Forward Clustering - A New Biliary Atresia Classification.Indian journal of pediatrics · 2025Article
- Biliary atresia.Nature reviews. Disease primers · 2024Review
- Diseases of bile duct in children.World journal of gastroenterology · 2024Review
- Long-term clinical and socioeconomic outcomes of children with biliary atresia.JGH open : an open access journal of gastroenterology and hepatology · 2023Article
- Clinical Characteristics and Outcome of Infants with Biliary Atresia in Bahrain.Oman medical journal · 2023Article
- MRI Liver Imaging Integrated with Texture Analysis in Native Liver Survivor Patients with Biliary Atresia after Kasai Portoenterostomy: Correlation with Medical Outcome after Surgical Treatment.Bioengineering (Basel, Switzerland) · 2023Article
- Native liver survivors of portoenterostomy for biliary atresia with excellent outcome: redefining "successful" portoenterostomy.Pediatric surgery international · 2022Article
- Risk of variceal hemorrhage and pretransplant mortality in children with biliary atresia.Hepatology (Baltimore, Md.) · 2022Article
- Cholangitis Definition and Treatment after Kasai Hepatoportoenterostomy for Biliary Atresia: A Delphi Process and International Expert Panel.Journal of clinical medicine · 2022Article
- The outcome of Kasai portoenterostomy after day 70 of life.Frontiers in pediatrics · 2022Article
- Machine Learning Evaluation of Biliary Atresia Patients to Predict Long-Term Outcome after the Kasai Procedure.Bioengineering (Basel, Switzerland) · 2021Article
- Factors influencing the quality of life in children after biliary atresia treatment.Translational pediatrics · 2021Article
- Prepregnancy Assessment of Liver Function to Predict Perinatal and Postpregnancy Outcomes in Biliary Atresia Patients with Native Liver.Journal of clinical medicine · 2021Article
- Imaging prediction with ultrasound and MRI of long-term medical outcome in native liver survivor patients with biliary atresia after kasai portoenterostomy: a pilot study.Abdominal radiology (New York) · 2021Article
Corrections and comments
- Erratum issued
Authors and funding
18 authors.
Funding
Abstract
objectivesTo examine the medical status of children with biliary atresia (BA) with their native livers after hepato- portoenterostomy (HPE) surgery. STUDY
designThe 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.
resultsChildren 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.
conclusionCholangitis 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.
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