Title: Donor type, social deprivation, and long‑term outcomes in pediatric liver transplantation: a 30‑year population‑based cohort

Source: Liver Transplantation 2026, Apr 10. [E–publication]

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Date of publication: April 2026

Publication type: Population-based cohort study

Abstract: Background & aims: Living donor liver transplantation (LDLT) reduces wait‑list mortality in children, but its long‑term advantages over deceased donor liver transplantation (DDLT) and how socioeconomic context shapes outcomes in a universal healthcare system remain uncertain. We compared long‑term outcomes after pediatric LDLT versus DDLT and evaluated modification by socioeconomic status (SES).

Approach & results: We linked clinical data for pediatric liver transplants in Ontario, Canada from 1991-2021 to provincial health administrative data, yielding 449 recipients (189 LDLT, 260 DDLT) who underwent first transplant. Over the 30-year period, LDLT recipients had superior patient and graft survival. After adjustment, DDLT was associated with a higher risk of mortality (adjusted hazard ratio [aHR] 2.1, 95% CI 1.0-4.3), graft failure (aHR 2.1, 95% CI 1.0-4.3), and chronic kidney disease (adjusted subdistribution HR 5.3, 95% CI 1.4-15.3), compared to LDLT. SES profoundly modified long-term outcomes: among DDLT recipients, lower neighborhood income and higher material deprivation were strongly linked to worse survival and increased graft loss. In contrast, LDLT moderated these socioeconomic disadvantages, with recipients showing comparable outcomes regardless of their SES (P for interaction <0.01).

Conclusions: In this population-based cohort study, LDLT was associated with significantly better long-term patient and graft survival and a lower risk of chronic kidney disease compared to DDLT. Socioeconomic disadvantage negatively impacted outcomes primarily among DDLT recipients, highlighting the need to improve equitable access to LDLT and to strengthen targeted post-transplant support for socioeconomically vulnerable families.

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Title: A strategy to identify biliary atresia efficiently: a perspective from a Texas center

Source: World Journal of Pediatric Surgery 2026, 9: e001142

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Date of publication: March 2026 

Publication type: Article 

Abstract: Infants with biliary atresia are often diagnosed after 60 days of life because the disease is difficult to detect in its early stages. However, infants treated before 30–45 days of life have the best long-term outcomes. To help accelerate the biliary atresia diagnosis, we have developed a streamlined strategy that involves two sequential tests: (1) direct or conjugated bilirubin measurements and (2) a feeding abdominal ultrasound exam. In this review, the strategy is shared to encourage others to provide feedback as well as to consider incorporating portions into their own clinical workflows.

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Title: Pediatric cholestasis: a practical approach to histological diagnosis

Source: Diagnostics 2026, 16 (6): 878

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Date of publication: March 2026

Publication type: Review article

Abstract: Pediatric (neonatal and infantile) jaundice resulting from underlying cholestasis (caused by conjugated hyperbilirubinemia) is always pathological and requires prompt evaluation. Pediatric cholestasis can be caused by medical or surgical factors and, if left untreated, can lead to irreversible liver damage. Timely recognition of pediatric cholestasis and identification of the underlying etiology are paramount to improve outcomes. The broad spectrum of causes potentially underlying pediatric cholestasis requires a multidisciplinary diagnostic approach, and each aspect must be interpreted in the concomitant clinical picture. A liver biopsy is one component of a complex diagnostic puzzle. However, interpreting a liver biopsy performed on a newborn/infant with conjugated/direct hyperbilirubinemia can be a challenging task, as these biopsies are rarely encountered in general hospitals. The aim of this review is to provide a practical and simplified approach to pediatric cholestasis with examples of real clinical cases we have encountered and discuss key features, both histological and clinical, that can help narrow the differential diagnosis and identify treatable causes.

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Title: Incidence, prevalence, medication use, and transplant rates in paediatric autoimmune hepatitis – a nationwide cohort study  

Source: Liver International 2026, 46 (5): e70625  

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Date of publication: April 2026 

Publication type: Cohort study 

Abstract: Background and aims: Despite the aggressive nature of paediatric autoimmune hepatitis (P-AIH), population-based epidemiology is poorly described. We aimed to validate an algorithm to identify patients with P-AIH in the Danish National Patient Registry (DNPR) and report incidence rates, prevalence, and describe medication exposure and liver transplantation rates.

Methods: This was a nationwide, register-based study. We used a smaller population-based P-AIH cohort as true positives for validation. The best-performing algorithm was then used to identify all patients in Denmark with P-AIH (1978-2022). Incidence rates and prevalence for the period 1996-2022 are presented. We used the National Prescription Register and DNPR to report on the use of prednisolone, thiopurine, and tacrolimus as well as liver transplantation.

Results: Based on the best-performing algorithm (sensitivity 0.94, specificity 1.0), we identified 222 incident patients. The incidence rate rose from 0.7/100000 person-years in 1999-2001 to the highest observed incidence rate in 2014-2016 (4.5/100000 person-years). The prevalence rose from 2.2/100.000 persons in 1999-2001 to the highest observed in the 2017-2019 period (8.8/100.000 persons). During the first year, 212 (95%) were treated with prednisolone, 159 (72%) with thiopurines, and 23 (10%) with tacrolimus. After five years, 219 (99%) had been treated with prednisolone, 178 (80%) with thiopurines, and 44 (20%) with tacrolimus. Native liver survival was 97% and 93% at 5 and 10 years after diagnosis.

Conclusion: The incidence and prevalence of paediatric autoimmune hepatitis in Denmark have increased over the past two decades. Most patients receive corticosteroids and thiopurines, and native liver survival remains high.

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Title: Paediatric autoimmune liver disease in Europe, the prospective ERN R-Liver registry

Source: JHEP Reports 2026, Apr 9. [E–publication]

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Date of publication: April 2026 

Publication type: Article

Abstract: Background & aims: Most literature on paediatric autoimmune liver disease (p-AILD) comprises single-centre, retrospective studies. This study aims to describe robust, real-world data for p-AILD during the first year following diagnosis, utilising data from the prospective European Reference Network (ERN) R-LIVER Registry.

Methods: All patients younger than 18 years with autoimmune hepatitis (AIH) or autoimmune sclerosing cholangitis (ASC) enrolled in the ERN R-LIVER Registry from January 2017 to October 2023 and with >12 months of follow-up were included. Each participating centre recorded data from three time points: diagnosis, six and twelve months.

Results: A total of 116 p-AILD patients were enrolled. 71 patients had AIH1, 8 had AIH2, and 37 had ASC. At diagnosis, 27% had cirrhosis. Large duct disease was diagnosed in 45% of ASC. Inflammatory bowel disease was present in 14% at diagnosis. No differences were found in presentation and outcome between AIH1 and 2. Most patients (94%) began treatment with standard therapy (prednisolone with/without thiopurines), and 80% were kept on it during the first year. Complete biochemical remission was achieved by 44 (42%) at six months and by 45 (42%) at 1 year, while normal ALT (<45) and IgG (age dependent) levels were observed in 81 (72%) and 53 (51%), respectively, at 12 months. All patients were alive at the end of follow-up, and two required liver transplantation.

Conclusions: Short-term survival in p-AILD is excellent. However, less than half of p-AILD patients achieved complete biochemical remission at one year, with ASC and cirrhosis as main predictors of failure. These findings emphasise the need for improved therapeutic strategies.

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Title: Etiology and management of cholangitis in pediatric liver transplant recipients: a systematic review 

Source: Current Opinion in Organ Transplantation 2026, Apr 7. [E–publication]

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Date of publication: April 2026  

Publication type: Systematic review  

Abstract: Purpose of review: Children undergoing liver transplantation are highly vulnerable to infections. Cholangitis is a potential post-transplant complication requiring broad-spectrum anti-infective therapy, raising concerns about antimicrobial resistance in this immunosuppressed population. We conducted a systematic review to evaluate antimicrobial management of post-transplant cholangitis in pediatric patients.

Recent findings: Nine heterogeneous studies were included. Definitions of cholangitis varied widely, combining clinical, laboratory, imaging, and microbiological criteria, highlighting the need for standardization. Gram-negative bacteria predominated, particularly Klebsiella spp., Pseudomonas aeruginosa, and Escherichia coli. Prophylaxis commonly relied on broad-spectrum antibiotics, and initial treatment was empirical in all studies, with occasional adjustment based on microbiological results. First-line therapies included piperacillin-tazobactam and third-generation cephalosporins. Second-line regimens involved agents from various antibiotic classes, including glycopeptides, lipopeptides, aminoglycosides, and fluoroquinolones, as well as antifungals. Meropenem was used as either first-line or second-line therapy.

Summary: Improved characterization and standardized reporting of pathogens and treatments are needed to guide targeted antimicrobial strategies and limit multidrug-resistant organisms. More detailed and harmonized data reporting is essential to optimize management of post-transplant cholangitis in children.

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Title: Incidence of cognitive dysfunction in children after liver transplantation: a systematic review and meta-analysis

Source: Pediatric Transplantation 2026, 30 (4): e70307

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Date of publication: April 2026  

Publication type: Systematic review

Abstract: This study aimed to systematically review the incidence and factors associated with cognitive dysfunction in children after liver transplantation. Four electronic databases (PubMed, Embase, Web of Science, and ProQuest) were searched from inception to October 22, 2024. Study quality and risk of bias were assessed using the Newcastle-Ottawa Scale (NOS). The pooled incidence was calculated using R software (version 4.3.1). We performed a narrative review to summarize the factors associated with cognitive dysfunction in children after liver transplantation. The protocol of this study was registered with the International Prospective Register of Systematic Reviews (PROSPERO) database, registration number: CRD42025630498. This study included 38 articles involving 7494 participants. The pooled incidence of cognitive dysfunction following pediatric liver transplantation was estimated at 24% (95% CI: 19.0%-30.0%). Highest rates were observed in Asia (30.6%) and among children transplanted < 1 year-old (39.4%). Disease-related factors, treatment-related factors, individual factors, cognitive-behavioral factors and social factors were summarized. Collected evidence showed that the overall incidence of cognitive dysfunction in children after liver transplantation was high. A multifactorial approach to risk assessment and intervention is needed to optimize long-term cognitive outcomes in this vulnerable population.

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Title: Sarcopenia and nutritional impact in pediatric patients with chronic liver disease: clinical and management strategies

Source: Journal of Pediatric Gastroenterology and Nutrition 2026, Apr 2. [E–publication]

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Date of publication: April 2026

Publication type: Review article 

Abstract: Sarcopenia, a progressive skeletal muscle disorder characterized by the decline in muscle strength, mass, and function, is increasingly recognized in pediatric chronic liver disease (CLD) as a marker of poor clinical outcomes. Its pathogenesis is multifactorial, involving malnutrition, systemic inflammation, hormonal imbalances, hypermetabolism, impaired protein synthesis, and micronutrient deficiencies. A related entity, sarcopenic obesity notably observed in metabolic-associated steatotic liver disease adds further complexity by combining muscle loss with excess adiposity, insulin resistance, and inflammation. Assessment of sarcopenia in children currently relies on a combination of imaging modalities and functional evaluations. Nutritional strategies to mitigate sarcopenia include tailored macronutrient intake (e.g., adequate protein, branched-chain amino acids, and medium-chain triglycerides), supplementation with key micronutrients (such as vitamin D and zinc), and structured physical activity programs. Emerging evidence supports integrating sarcopenia screening into routine clinical care, particularly during liver transplant evaluation. Given the limited consolidated evidence on the interplay between sarcopenia, liver dysfunction, and nutrition in children, the North American Society for Pediatric Gastroenterology, Hepatology & Nutrition Hepatology and Nutrition Committees have conducted a comprehensive review to provide updated guidance on the diagnosis and nutritional management of sarcopenia in pediatric CLD.

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Title: Ileal Bile Acid Transport (IBAT) inhibitors as an emerging treatment for cholestatic liver disease

Source: Alimentary Pharmacology & Therapeutics 2026, Apr 9. [E–publication]

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Date of publication: April 2026

Publication type: Review article  

Abstract: Background: Cholestatic liver diseases such as Alagille syndrome (ALGS), progressive familial intrahepatic cholestasis (PFIC), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) cause significant morbidity and mortality. Chronic pruritus is common, debilitating, and impairs health-related quality of life. Recently, pharmacological inhibition of the ileal bile acid transporter (IBAT) has emerged as a therapeutic target.

Aims: To review the current landscape of IBAT inhibitors, summarise emerging clinical data, discuss their role in the treatment of cholestatic liver diseases and future directions for their development and use.

Methods: This narrative review summarises current data on IBAT inhibitors, exploring their mechanisms, efficacy, and safety across ALGS, PFIC, PBC and PSC. References were identified through searches of PubMed from January 2000 to August 2025.

Results: In phase 2 and 3 trials involving paediatric patients with ALGS and PFIC, IBAT inhibitors (odevixibat and maralixibat) significantly reduced pruritus and serum bile acid concentrations. In post hoc analysis, responders demonstrated improved event-free and transplant-free survival compared to historic control cohorts. In PBC and PSC, early phase trials have shown modest pruritus reduction with linerixibat and maralixibat. Mild to moderate gastrointestinal side effects, most commonly diarrhoea and abdominal discomfort, are common with IBAT inhibition, particularly in PBC and PSC.

Conclusions: IBAT inhibitors represent the first upstream pharmacotherapy targeting enterohepatic bile acid recirculation and are effective at reducing pruritus in ALGS and PFIC. Their role in PBC and PSC is promising yet undefined. Long-term studies are needed to assess effects on fibrosis progression, hepatocellular carcinoma risk and transplant-free survival.

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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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