Bikrant Bihari Lal

Background and Objective: ABO‐incompatible (ABO‐i) liver transplantation (LT) in children is increasingly used to address organ scarcity, but concerns about immunological, vascular, and biliary complications remain. With evolving desensitization strategies and emerging pediatric data, an updated meta‐analysis comparing ABO‐i and ABO‐compatible (ABO‐c) LT is warranted. Methods: PubMed, Embase, and Scopus were searched from inception to June 2026 to include studies evaluating ABO‐i versus ABO‐c LT in recipients ≤ 18 years of age. Primary outcomes were comparison of 1‐, 3‐, and 5‐year patient and graft survival between the two groups. Secondary outcomes included comparisons of vascular and biliary complications, rejection, cytomegalovirus (CMV) and Epstein–Barr virus (EBV) infections, and post‐transplant lymphoproliferative disease (PTLD). Results: Seventeen studies were included. Patient survival was comparable between the two groups at 1 year (risk ratio [RR]: 0.97, 95% confidence interval [CI]: 0.94–1.0, p = 0.07), 3 years (RR: 0.98, 95% CI: 0.94–1.01, p = 0.22) and 5 years (RR: 0.97, 95% CI: 0.88–1.07, p = 0.50). Likewise, graft survival showed no significant differences. Incidence of vascular and biliary complications, acute rejection, CMV and EBV infection, and PTLD also did not differ significantly between groups. Antibody‐mediated rejection (AMR) was significantly higher in ABO‐i recipients (RR: 20.04, 95% CI: 3.25–123.56, p = 0.001), although this finding was based on only four studies. Overall, heterogeneity was low. Certainty of evidence was rated as very low, primarily due to the observational design of included studies and risk of bias. Conclusions: ABO‐i LT in pediatric recipients demonstrates survival and complication outcomes comparable to those of ABO‐c LT. However, very low certainty of evidence highlights the need for future well‐designed prospective studies.

Wilson disease (WD) and autoimmune hepatitis (AIH) are leading causes of chronic liver disease in children and often present with overlapping clinical and immunological features, complicating early diagnosis. This retrospective study evaluated serum immunoglobulin G (IgG) and autoantibody profiles in 79 genetically confirmed, treatment‐naive pediatric WD patients compared with 112 AIH controls. Elevated IgG (>1.1× upper limit of normal) was observed in 82.3% of WD patients, while 31.5% tested positive for at least one autoantibody, including anti‐smooth muscle antibody, anti‐nuclear antibody, and anti‐liver kidney microsomal. Notably, 20.2% of WD patients met the simplified diagnostic criteria for AIH. Compared to AIH, WD patients had significantly lower IgG levels and less frequent autoantibody positivity. Histology revealed distinguishing features, with WD showing more steatosis and ballooning but less interface hepatitis and plasma cell infiltration. These findings highlight the potential for diagnostic misclassification. Comprehensive evaluation, including histology and genetic testing, remains critical to differentiate WD from AIH and guide appropriate therapy.

Autoimmune hepatitis (AIH) in children presents significant diagnostic and therapeutic challenges requiring a multidisciplinary and evidence-based approach. The Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition (ISPGHAN) convened a consensus meeting on February 23, 2025, bringing together national and international experts to address key clinical and research questions. The deliberations spanned epidemiology, clinical presentation, diagnosis including autoantibodies, immunoglobulin G (IgG), histology and diagnostic scores, therapeutic strategies, management of difficult-to-treat AIH, long-term monitoring, and special scenarios, such as seronegative AIH (SN-AIH), autoimmune sclerosing cholangitis/overlap syndromes and post-transplant complications like recurrence and de novo-AIH. Recommendations were formulated using standard GRADE system and finalized through structured consensus. These guidelines aim to standardize care, assist clinicians in decision-making, and improve outcomes in children with AIH across diverse healthcare settings.

Objectives Cirrhotic cardiomyopathy (CCM) is an under recognized entity in children. The present study aimed to explore the prevalence and natural history of CCM and its impact on outcomes among children with biliary atresia (BA) and genetic intrahepatic cholestasis (GIC). Methods This was a prospective observational study. All children aged 0–18 years and diagnosed with either BA or GIC were included. Relevant clinical and biochemical parameters were recorded and transthoracic echocardiography was done. Left ventricular mass index (LVMI) ≥ 95 g/m^2.7 and/or relative wall thickness of left ventricle ≥ 0.42 was used for defining CCM. Results Eighty nine children were included in the study, 64 with BA and 25 with GIC. Prevalence of CCM in children with BA and GIC were 53.13% and 48% respectively. LVMI (g/m^2.7) was significantly higher in children with unsuccessful KPE (p = 0.05 and p = 0.024) and unoperated BA (p < 0.001 and p = 0.002) compared to those with successful KPE and GIC. Serum bile acid levels were significantly (p = 0.026) higher among children with CCM. CCM was associated with a lower native liver survival (NLS) (p = 0.0039) and overall survival (OS) (p = 0.006) at 12 months. There were no significant associations between presence of CCM and post-LT mortality, time to extubation, duration of inotrope requirement or length of ICU stay. 80% children with CCM at baseline had resolution of CCM within 6 months post-LT. Conclusions CCM is prevalent in almost half of patients with BA and GIC and is associated with decreased NLS and OS. Serum bile acids were significantly higher in children with CCM.