Title: Impact of graft size matching on graft survival in pediatric whole liver transplantations in recipients with biliary atresia weighing less than 10 kg  

Source: Transplantation Proceedings 2026, Feb 27. [E–publication]

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Date of publication: February 2026

Publication type: Retrospective study

Abstract: Background: The size mismatch between a liver graft and the recipient can result in complications and poor survival after pediatric whole liver transplantation (WLT).

Methods: A retrospective study was designed, which included 114 recipients. Multiple variable predictors of graft loss suggested the graft-to-native-liver weight ratio (GNLWR), and then the GNLWR was calculated and categorized into 2 groups. The demographic, operation, complications, and survival analysis data were collected and compared.

Results: Group 1 with a GNLWR < 0.41 had a higher incidence of hepatic artery thrombosis (HAT) and lower graft survival in the first 3 months (70.4% vs 96.6%, P < .001). Patient survival at 3 months was significantly different between groups (85.2% vs 97.7%, P = .010). As for the graft survival rate, it was 70.4% for group 1 at both 1 and 3 years, whereas it was 94.2% for group 2 at both 1 and 3 years. Additionally, the patient survival rate for group 1 was 85.2% at both 1 and 3 years, whereas for group 2, it was 95.4% at both 1 and 3 years. Further analysis showed the PELD score was the only independent risk factor for graft loss in the group with GNLWR < 0.41. It indicated a worse prognosis when the PELD score was more than 23.5.

Conclusions: A GNLWR of less than 0.41 suggested a poor prognosis for grafts in pediatric WLT recipients with biliary atresia weighing less than 10 kg. Reducing blood transfusions may help improve graft survival.

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Title: Unveiling hepatic protein alterations in neonatal and infant biliary atresia

Source: Clinical Pharmacology and Therapeutics 2026, Mar 4. [E–publication]

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Date of publication: March 2026

Publication type: Article

Abstract: Pediatric populations differ from adults in drug elimination capacity. While current scaling methods account for enzyme and transporter maturation, they overlook comorbidities, such as biliary atresia (BA), a liver disease appearing within the first 2-8 weeks of life that can progress to cirrhosis. Such conditions may impair hepatic drug clearance, requiring dose adjustments. Physiologically based pharmacokinetic (PBPK) tools aim to address such cases and have been advocated to fill gaps in clinical data instead of less formalized and evidence-based guesswork. However, the paucity of systems data in rare disease populations has hindered the development of robust PBPK models. This study used global liquid chromatography and tandem mass spectrometry (LC-MS/MS) proteomics to quantify drug-metabolizing enzymes and transporters in diseased neonatal (n = 13) and infant (n = 12) liver samples, revealing significant expression changes in biliary atresia (BA) livers vs. controls (n = 19). Based on cohort means, CYP2A6, CYP2B6, and CYP2E1 levels were 6-17-fold higher in BA livers compared to controls, while CYP4F11 and CYP20A1 were reduced. UGT1A1, UGT2B4, and UGT2B7 showed up to 16-fold higher abundance in neonates with BA. Among transporters, ABCF1 abundance increased dramatically (46-fold), whereas B3AT/SLC4A1, ADT1/SLC25A4, and S27A5/SLC27A5 were decreased. The observed alterations suggest that assuming similar liver function in BA and non-BA patients has implications, with impact varying by drug clearance pathway. While in silico models can explore this, clinical pharmacokinetic studies in BA are essential for verification. To our knowledge, such studies are absent. Our observations underscore the urgent need for dedicated pharmacokinetic studies in BA patients to improve precision dosing.

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Title: Demographic factors and biliary atresia: a Childhood Liver Disease Research Network study  

Source: Pediatrics 2026, 157 (3): e2025071520

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Date of publication: March 2026

Publication type: Article

Abstract: Objective: To test the hypothesis that community deprivation, race, and ethnicity lead to decreased likelihood of undergoing hepatoportoenterostomy, older age at surgery, decreased likelihood of achieving successful bile drainage, and lower rates of native liver survival for infants with biliary atresia.

Methods: We analyzed a prospectively enrolled cohort of infants with biliary atresia from the Childhood Liver Disease Research Network (ChiLDReN) that reflects the demographics of the US population. We tested the association between demographic, clinical, and anatomic variables and the probability of undergoing hepatoportoenterostomy, age at surgery, success of surgical intervention, and native liver survival using linear and logistic regression.

Results: Seven hundred nineteen infants with biliary atresia from 15 centers met study inclusion criteria and 672 (93.5%) underwent hepatoportoenterostomy. After adjusting for potential confounders, Asian race (odds ratio [OR] = 0.21, 0.06-0.77), Hispanic ethnicity (OR = 0.33, 0.14-0.76), and community deprivation (0.71 per 0.1 increase, 0.52-0.97) were independently associated with decreased probability of undergoing hepatoportoenterostomy. Each 10% increase in community deprivation increased the age at hepatoportoenterostomy by approximately two-and-one-third days (estimate 2.31; P = .48). Black/African American infants were approximately 9 days older than white infants at the time of operation (estimate 9.19; P = .01), while age at hepatoportoenterostomy (OR = 0.90, P = .01) and successful bile drainage at 3 months (OR = 26.15, P < .01) were independently associated with native liver survival.

Conclusions: Community deprivation, race, and ethnicity are associated with both lower hepatoportoenterostomy rates and older age at the time of operation, whereas clinical and anatomic variables are associated with successful biliary drainage and native liver survival.

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Title: Breastmilk and medium-chain triglyceride supplementation: retrospective study on outcomes in biliary atresia infants after Kasai  

Source: Journal of Pediatric Gastroenterology and Nutrition 2026, Feb 23. [E–publication]

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Date of publication: February 2026  

Publication type: Retrospective study

Abstract: Objective: To relate post-surgical feeding regimens to growth and surgical outcomes in children with biliary atresia (BA) after hepato-portoenterostomy (Kasai).

Methods: Retrospectively, all children with BA from Denmark and the Netherlands who underwent Kasai from 2014 to 2022 were included. The effect of breastmilk intake on anthropometry after Kasai was evaluated using jointed modeling of longitudinal and survival data, removing children from the analysis at transplantation. Mixed linear models were used to assess surgical outcomes in relation to breastmilk intake and anthropometry in relation to medium-chain triglycerides (MCT) intake, adjusted for calorie intake.

Results: We included 116 children. After Kasai, breastmilk with MCT supplementation was received in 22% for at least 1 month, 9% for 1 week, and 68% did not receive breastmilk. Those receiving breastmilk after Kasai had stable length-for-age z-score (LAZ) over 12 months whereas LAZ decreased in infants without breastmilk feeding (-0.48; 95% confidence interval [CI] 0.24, 0.72). Infants receiving breastmilk for at least 1 month had lower total bilirubin at 3 months (79 vs. 116 µmol/L, p = 0.028) and a lower incidence of cholangitis compared with infants not receiving breastmilk after Kasai. Of those only receiving formula after Kasai, 4% received <40% MCT, 72% received 40-59% MCT and 24% received ≥60% MCT. LAZ was lower in children with MCT intake <40% than in children with MCT intake ≥40%.

Conclusion: Feeding regimens in children with BA after Kasai are related to growth and surgical outcomes. Further studies with infants randomized to receive breast milk and to different MCT levels are needed.

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Title: Children with biliary atresia have substantial morbidity in early childhood and a high risk of liver transplantation  

Source: Birth Defects Research 2026, 118 (2): e70024

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Date of publication: January 2026 

Publication type: Cohort study 

Abstract: Background: Biliary atresia is a rare but severe congenital anomaly associated with substantial morbidity and mortality in early childhood. Population-based estimates of survival, surgical management, and liver transplantation across Europe remain limited. This study aimed to describe mortality and morbidity among children born with biliary atresia using multinational population-based data.

Methods: We investigated children diagnosed with biliary atresia across nine registries from five countries within the European surveillance of congenital anomalies network (EUROCAT), covering births from 1995 to 2014. The data were linked to hospital databases and adjusted for regional differences and follow-up length.

Results: Our cohort included 171 children, with an infant mortality rate of 12.3% (95% CI: 7.8-17.6) and a mortality rate before age five of 18.5% (95% CI: 10.7-27.7). Among these children, 151 had undergone surgery, including 133 who received the Kasai procedure by the age of 1 year at a median age of 57 days (95% CI: 51-62 days). By age five, 37% (adjusted percentage, 95% CI: 30-44) had undergone liver transplantation, with the median age at transplantation being 318 days (95% CI: 244-391 days). Median age at death in the first year was over 6 months and was not immediately after surgery.

Conclusion: The high mortality and the substantial need for liver transplantation within the first year of life underline the severity of biliary atresia. This highlights the urgent need for further research into pregnancy exposures that may contribute to this rare but severe congenital anomaly to develop primary prevention strategies. 

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Title: The physical and psychosocial adaptation experiences of youths diagnosed with biliary atresia and treated using the Kasai procedure at infancy: a qualitative descriptive study  

Source: Gastroenterology Nursing 2026, 49 (1): 30-38  

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Date of publication: January 2026  

Publication type: Qualitative descriptive study

Abstract: Biliary atresia (BA) is a disease of the liver and bile ducts. Newborns with BA must undergo the Kasai procedure immediately after birth to survive. Postoperative nutrition, portal hypertension, and splenomegaly affect prognosis on an ongoing basis. With recent advancements in medicine and health care quality, children treated for BA can survive into adolescence and even early adulthood. This qualitative descriptive study explores how these youths have adapted, and their concerns as they continue to age. A total of 13 patients with BA aged 16-24 years were recruited using purposive sampling at a gastroenterology outpatient clinic in northern Taiwan. In-depth semi-structured interviews were conducted with each participant. The participants’ life experiences were extracted into three themes via inductive qualitative content analysis: (1) being responsible for one’s dietary regimen, (2) adjusting one’s mindset about the illness, and (3) facing long-term challenges. Healthcare professionals can provide care recommendations tailored to the developmental needs of this patient group during regular follow-ups, assist them with self-care management and with accepting their uniqueness, provide information, and discuss their future careers and family plans.

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Title: Growth in children with biliary atresia before and after liver transplantation: a retrospective analysis  

Source: Frontiers in Pediatrics 2026, Jan 12. [E–publication]

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Date of publication: January 2026

Publication type: Retrospective study 

Abstract: Background: Little is known about the long-term survival and growth outcomes of children following liver transplantation in China. Therefore, we investigated the long-term survival and growth status of children with biliary atresia (BA) before and after liver transplantation and attempted to identify specific influencing factors.

Methods: We included children who underwent liver transplantation for BA at Tianjin First Central Hospital between January 2014 and December 2018. Clinical data, indicators of liver function, growth data, and ascites status were collected for analysis from all patients. Postoperative complications were systematically assessed using a combination of clinical, laboratory, histological, and imaging evaluations. Complications were categorized and graded according to the Clavien-Dindo classification system.

Results: This study included a total of 93 children under 2 years of age who underwent liver transplantation for BA. No significant increase in height was observed six months post-transplantation when compared to pre-transplantation (P = 0.126). A significant increase in the height-for-age Z-score occurred by year 2 (P = 0.001). Preoperative growth was negatively correlated with growth improvement at 2- and 5-years after transplantation (P < 0.001, r = -0.674 and r = -0.774, respectively). The occurrence of biliary complications was significantly associated with impaired catch-up growth post-transplantation (P = 0.008). The weight-for-age Z-score increased significantly over the first two years post-transplantation when compared with that before transplantation (P < 0.001); subsequently, this parameter plateaued and exhibited only minimal changes.

Conclusions: Growth retardation is common in children with BA prior to liver transplantation, with catch-up growth commencing six months post-surgery and peaking after two years. Children with more severe preoperative growth delays exhibited faster postoperative growth. Preoperative growth status exhibited only minimal impact on early liver function recovery post-transplantation.

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Title: Gut microbiome in biliary atresia

Source: World Journal of Pediatric Surgery 2026, 9 (1): e001068

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Date of publication: January 2026 

Publication type: Review article

Abstract: Biliary atresia (BA) is a progressive cholangiopathy of infancy and the leading cause of pediatric liver transplantation. Despite surgical intervention with the Kasai portoenterostomy, long-term outcomes remain poor, with many patients progressing to cirrhosis. Emerging evidence implicates the gut microbiota-a dynamic ecosystem crucial to immune development and liver homeostasis-in BA pathogenesis and clinical progression. This review synthesizes current literature on gut microbiota composition in BA before and after the Kasai procedure, highlighting consistent patterns of dysbiosis, including pathobiont expansion and depletion of beneficial microbes such as Bifidobacterium. The review explores associations between microbial profiles and clinical outcomes-highlighting potential mechanisms involving bile acid metabolism, microbial translocation, and immune modulation. Further understanding of gut-liver-microbiota interactions in BA may inform microbiome-targeted therapies to improve native liver survival.

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Title: Nutrition support adequacy in children with biliary atresia after liver transplant  

Source: Nutrients 2025, 18 (1): 133  

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Date of publication: December 2025 

Publication type: Retrospective cohort study 

Abstract: Background: The nutrition support of children with biliary atresia after liver transplant is affected by multiple factors, and a connection between these factors and conditions present before transplant can potentially make the nutrition support more challenging. We aim to assess the adequacy of nutrition support, specifically energy and protein, during the first week of admission to the Pediatric Intensive Care Unit (PICU) in children after liver transplant secondary to biliary atresia. 

Methods: We performed a retrospective cohort study of 138 patients [13.9 median (9-33.4) IQR months; 62% female] with a diagnosis of biliary atresia admitted to the PICU after liver transplantation at Texas Children’s Hospital over a 14-year study period. We obtained nutrition adequacy of enteral and parenteral nutrition support for the first week after transplant during their PICU admission. 

Results: Goal adequacy was reached at the end of the first week of admission when combined enteral and parenteral nutrition support was provided (median 98% for energy and 101% for protein). Infants achieved significantly higher adequacies than older children during the first week (136% < 1 year vs. 0% > 1 year, p < 0.001 for calories, and 157% < 1 year vs. 0% > 1 year for protein; p < 0.01). 

Conclusions: These findings highlight the complex nutritional challenges faced by this population, and strategies are needed to meet the unique needs of children after liver transplantation.

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Title: The immunological profile of children with portal hypertension

Source: Digestive and Liver Disease 2025, Dec 20. [E–publication]

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Date of publication: December 2025

Publication type: Review article

Abstract: Portal hypertension is a clinical syndrome with potentially life-threatening complications. Diagnosis and management in children are complex due to the invasiveness of hepatic venous pressure gradient measurement and the limited number of definitive treatment options, so that liver transplantation often remains the only definitive treatment. The influence of the immune system on the development of portal hypertension has recently received attention; however, the connection between portal hypertension, impaired immune response, and the development of liver changes has not yet been fully elucidated. This review provides an overview of the main current knowledge on the role of cytokines, immune cells, and other molecules involved in the inflammation and vascular changes associated with portal hypertension. A better understanding of the pathogenesis of portal hypertension could address the need to identify non-invasive markers for the diagnosis of portal hypertension and predictors of its complications in children. Furthermore, understanding the strong interaction between the immune system and the development of portal hypertension could be useful for identifying new potential therapeutic options, orienting therapeutic management towards immunomodulatory approaches.

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