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

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

Follow this link 

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.

The post A strategy to identify biliary atresia efficiently: a perspective from a Texas center appeared first on Childrens Liver Disease Foundation.

Title: Pediatric cholestasis: a practical approach to histological diagnosis

Source: Diagnostics 2026, 16 (6): 878

Follow this link 

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.

The post Pediatric cholestasis: a practical approach to histological diagnosis appeared first on Childrens Liver Disease Foundation.

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

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

Follow this link 

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.

The post Incidence, prevalence, medication use, and transplant rates in paediatric autoimmune hepatitis – a nationwide cohort study appeared first on Childrens Liver Disease Foundation.

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

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

Follow this link 

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.

The post Paediatric autoimmune liver disease in Europe, the prospective ERN R-Liver registry appeared first on Childrens Liver Disease Foundation.

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]

Follow this link 

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.

The post Etiology and management of cholangitis in pediatric liver transplant recipients: a systematic review appeared first on Childrens Liver Disease Foundation.

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

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

Follow this link 

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.

The post Incidence of cognitive dysfunction in children after liver transplantation: a systematic review and meta-analysis appeared first on Childrens Liver Disease Foundation.

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]

Follow this link 

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.

The post Sarcopenia and nutritional impact in pediatric patients with chronic liver disease: clinical and management strategies appeared first on Childrens Liver Disease Foundation.

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

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

Follow this link 

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.

The post Ileal Bile Acid Transport (IBAT) inhibitors as an emerging treatment for cholestatic liver disease appeared first on Childrens Liver Disease Foundation.

We’re really pleased to share Robert’s story. As far as we know, he is the longest-surviving childhood liver transplant patient in the UK, having received his second transplant in September 1989. If anyone knows differently, we’d genuinely love to hear from you. 

 

I was born in March 1979, and from the very beginning, something wasn’t quite right. My mum says the midwife had concerns but couldn’t pinpoint the problem. I fed reasonably well, but I was unsettled and clearly uncomfortable. I had a cracked shoulder bone from the birth, but it did not explain my discomfort. 

At around four weeks old, things got worse. I stopped feeding, became limp, and seemed to lose energy. A locum GP dismissed my mum as being fussy and overly worried, but she trusted her instincts and sought a second opinion. She knew something was wrong—and she was right. 

The usual family GP admitted me immediately to Southampton General Hospital. The doctors determined I was bleeding internally. What followed was chaos, confusion, and long periods of waiting. I was given a blood transfusion, but a large red bruise developed on my side, which mum worried that the doctors would believe was due to potential abuse, but this was never implied.  My condition became so critical that it was feared I would not survive the night, and my mum was asked if she would like to have me baptised, to which she answered, “he has done nothing wrong”. 

I was given a Vitamin K injection (now normal practice for newborns), and somehow, I pulled through. 

Living without answers 

After that, life became a cycle of hospital visits, tests, and biopsies. Those early biopsies were brutal, performed without the guidance of ultrasound, a core needle aimed between the ribs to hopefully gain a sample of liver tissue. Even now, the feeling still haunts me. 

Our consultant, Dr Chris Rolles, was approachable and supportive over the next 18 months as we saw numerous doctors to try and find a diagnosis. Eventually, after months of uncertainty, Professor Norman gave the diagnosis alpha-1 antitrypsin deficiency and delivered the news of a limited life expectancy, unlikely past the age of nine.  

I was too young to understand, but for my parents, it was devastating news. 

A childhood lived fully 

Despite everything, my family made a conscious decision: we were going to live as normally as possible. 

Staying within the reaches of the NHS, from rock climbing in Dorset, sailing in Cornwall, to walking in the Lake District, we spent as much time outdoors as we could. One memorable sailing trip to the Isle of Wight, was interrupted by a severe internal bleed. An emergency detour in the tender to Seaview, a taxi to St Marys Hospital, Newport, and the bleed was dealt with by cauterization. There were hospital visits, scares, and the occasional emergencies—but life carried on. 

Now as a parent myself, looking back, I realise how much stress my parents must have been under. Every bump, every fall, every illness came with an extra layer of fear for them. But as a child, I just got on with it. What I now truly appreciate, is that my parents have seen me at my best and at my worst, and I would not be where I am today without their stoic support.

When things got real 

In 1988, by the time I was eight, I was getting noticeably weaker. I had jaundice, struggled to eat, and had very little energy. I was also dealing with the normal challenges of childhood—including a bit of bullying, thanks to the bloating and jaundice—which I learned to handle in my own way. 

It was proposed that we travel to Addenbrookes Hospital, Cambridge, to meet Sir Roy Calne. Sir Roy was a leading surgeon specialising in organ transplants, also sidelining as a very proficient artist, he later painted me whilst in the ICU. Sir Roy introduced us (including our new golden retriever puppy Suki) to the transplant team, in the premise that it may, one day, be a possible route of treatment. We continued with our cycle of life, school and hospital visits, but my health was notably declining. 

Then, in early 1989, everything changed. Late one evening in February, mum received a phone call from Addenbrookes out of the blue: there was a liver available for transplant, and we needed to get to Cambridge the next morning for the major operation.  

Mum was doing her PGCE at Southampton, childcare needed to be arranged for Victoria and William, and we hadn’t even realised I was on the list being considered for a transplant, a life-threatening operation – panic logistics ensued, and by the next day, with my dad at my side, I underwent my first surgery.  

The operation took around seven hours. For my parents, the waiting must have felt endless. They opted to take a 12-mile walk back to my Grandparent’s house to distract themselves. 

 

A setback—and another chance 

Then began the recovery. I was in hospital for around six weeks, and mum and dad alternated staying with me, and being at home with my brother and sister, trying to maintain as normal a life as possible.   

After I was discharged, I swelled up with ascites and again became jaundice. There followed six months of constant check-ups and occasional panics. During the summer, we took a camping trip to the Lake District. I had such a high temperature, I ran round the campsite at night hallucinating (and William was sick all over Victoria’s bed) which resulted in a mad dash to Cambridge. After that episode calmed down, we planned a sailing trip to the Channel Islands. I struggled to walk far, but was not seasick, and coped ok. However, on our return to Addenbrookes in early September, the dreaded news was given: the transplant had failed and a second would be necessary.  

A week later, we went through it again, with another week in PICU and five weeks or so on the Children’s ward. Happily, this time, it was a success. 

 

Growing up after transplant 

After recovering, I threw myself into life. 

I competed in the Transplant Games, particularly in swimming where I excelled, which gave me a real sense of confidence and normality. But as I got older, I wanted to move beyond being “the transplant kid” and just be like everyone else. 

 

 

I’ve always been more adventurous than sporty—climbing, sailing, surfing. I didn’t want my transplant to define what I could or couldn’t do. At one point, I even played rugby and asked to be hooker in the middle of the scrum, despite being told by doctors “no contact sports”. In fact, this has been a common theme throughout my life, the doctors more nervous of my pursuits than I am. I see the holistic advantages of a healthy full life, outweighing the risks of succumbing to a more sedentary approach. 

School wasn’t easy. Dyslexia and missed lessons made things difficult, but I found my strengths elsewhere. At school, I pushed myself physically and mentally, completing endurance challenges that tested me to my limits. 

Finding My Path 

In my late teens and early twenties, I worked in outdoor education, teaching climbing and canoeing. Later, I became a qualified surfing and windsurfing instructor, working around the world—from the UK and Ireland to Greece, Turkey, and spent time in the Canary Islands and Hawaii. 

Eventually, I realised I needed a more stable career. So, at 21, I went back to education, completed my GCSEs, and went on to university. That decision changed the course of my life and today, I work in global marine transport, a career I’m proud of. 

 

In 2009, I married my wife Emma, we now have two wonderful daughters. Family life has always been incredibly important to me—especially given everything that came before. 

Ongoing challenges 

In 2011, shortly after the birth of my first daughter, I was told I had entered “chronic rejection”. Hearing that was difficult, especially just after becoming a dad for the first time. I travelled for work to Singapore with a heavy heart. However, with some amendments to my medicine, nothing significantly changed until 2020, when signs of internal bleeding led me to have an investigative endoscopy. I was recalled the following day for banding of ruptured varices, just in time for the Covid pandemic to hit and for me to receive my latest label “vulnerable” and get my shopping slot with the elderly and receive a Government supply box (just as the supermarket delivery had been) which we duly donated to the food shelter.   

In 2021 we moved from Winchester to The Witterings, to be closer to the sea and for the girls to enjoy a childhood on the beach, just as I had done. Six months in, after a dinner party with friends, during the night I suffered a massive internal bleed, vomiting blood covering the bathroom floor. We phoned for an ambulance, and I had the next few days in hospital feeling very weak and was once again jaundiced and frail. That was my last ever glass of wine and I have been without alcohol ever since.  

Since then, there have been ups and downs—internal bleeding, surprise hospital stays, ascites, biopsies, ultrasounds, varices, etc. There have even been discussions about a possible third transplant. For now, though, things are stable. 

 

Where I am now 

As a family we sail, surf, windsurf, hike, climb and more. We are incredibly lucky. I’ve had to adapt how I stay fit, but I still do the things I love. Being active and enjoying the outdoors has always been a big part of who I am. 

More than anything, I try to focus on living fully. 

What I’ve learned 

If there’s any advice, I’d give to anyone going through something similar, it’s this: 

What you go through doesn’t define you. Yes, it changes you—but it doesn’t have to limit you. You can still build a life, follow your passions, and find your own path forward. 

You don’t get to choose the cards you’re dealt. But you do get to choose how you play them, and importantly, who you play them with. 

The post Live your best life with the cards you’ve been dealt appeared first on Childrens Liver Disease Foundation.

 

Chloe has kindly shared her experience with her son, Max, which she hopes will help other families in her situation. 

Max, known as our Max in a Million, has a condition called portal cavernoma, which has led to portal hypertension. We wanted to share his story from diagnosis to now, in the hope that it might provide awareness or understanding for other families facing similar challenges. 

Max was born extremely prematurely at just over 23 weeks gestation and had to spend six months in NICU.  During this time, he faced multiple rounds of sepsis and required extensive ventilator support for eight to nine weeks. His tiny body relied entirely on umbilical cord cannulas, Hickman lines, and numerous other lines for blood transfusions, platelets, TPN, and fluids, crucial to sustain him through these fragile early stages. 

Looking back, Max had very visible veins across his chest, and we were told that his liver was scarred, likely a result of prolonged TPN, along with high bilirubin levels in his blood tests. When he was discharged after around six months, he went home with oxygen and regular follow-ups for prematurity-related issues, but we were completely unaware of the liver complications that lay ahead and had received no diagnosis. 

 

 

When he was around eight months old, Max underwent surgery for a hernia repair and as doctors were still unaware of his underlying condition, he was given ibuprofen during recovery. After being discharged home, Max wouldn’t settle, and we knew something wasn’t right, so we rushed him back to our local hospital. He had his first emergency bleed on the ward, these symptoms were completely new to our local hospital, and he was transferred to the high-dependency children’s ward at a Tier 3 hospital for specialist care. An ultrasound revealed a blockage in his portal vein.  Following a recovery period, he was referred to King’s College Hospital. 

It was at King’s that Max’s diagnosis was confirmed: he had portal hypertension, caused by a blockage in his portal vein, known as a portal cavernoma. This rare complication is suspected to have resulted from the lifesaving umbilical cord cannula he had as a premmie baby, which unfortunately caused a thrombosis (blood clot) in the portal vein.

Portal Hypertension occurs when there is increased pressure in the portal vein, which carries blood from the intestines to the liver. This can lead to enlarged veins in the oesophagus and stomach (oesophageal varices), which are prone to life-threatening bleeding.  

 

 

What followed for us was a relentless cycle. Max faced repeated emergency bleeds, sometimes just three weeks apart, requiring ambulance trips to King’s College Hospital for sclerotherapy and banding (OGD). The instability of his oesophageal varices, graded at grade 3, and the constant fear of the next bleed was overwhelming. 

Despite the challenges, the teams at King’s have been incredible. Max’s treatment now includes monthly injections, daily medications, and regular sclerotherapy and banding procedures, which have helped his condition significantly. He is now four years old and attends routine procedures every three to six months, giving us hope and glimpses of normal life amid a challenging medical journey.   

 

 

 

I believe it was during our second emergency bleed that we were admitted to Rays of Sunshine Ward, where the clinical lead nurses provided me with an information brochure about portal hypertension and the support which CLDF offers. So I subsequently made contact with Kate who has been extremely reassuring and mentioned that once Max has a school placement in place, she will be able to help advise the school and support them in understanding his condition. We also received one of your education packs, which has been very helpful. 

Max recently reached a huge milestone by coming off home oxygen. He loves spending time with his big sister Cassie (also born prematurely but thankfully in full health) dancing or cuddling up on the sofa to watch their favourite TV programmes. 

Starting school will be the next milestone – something which we would love to happen but are also nervous about at the same time.  Max does have an EHCP in place as, in addition to his liver condition, he is autistic and has chronic lung disease as a result of being premature.   

 

 

Sharing Max’s story is our way of raising awareness about portal hypertension in children, and the challenges families face with rare liver complications. We hope that by sharing our experiences, other families can find reassurance that they are not alone, and that even in the most difficult circumstances, there is hope. 

 

The post Max in a Million: Our Journey with Portal Hypertension appeared first on Childrens Liver Disease Foundation.