Cyclical vomiting syndrome (CVS) is a functional gastrointestinal disorder marked by recurrent vomiting. Lack of awareness and symptom overlap often leads to incorrect diagnosis. There is limited data from the developing world, especially on the natural history of the disease in children. Thus, the aim of our study was to evaluate the clinical presentation, natural history, treatment and outcomes of CVS in children. Retrospective audit of children (≤ 18 years) diagnosed with CVS between January 2008 to December 2024. Clinical data was retrieved from hospital records and telephonic interviews. Seventy-one patients (age of onset 7[IQR 4–9] years, boys [39, 54.9

BACKGROUND:Chronic pancreatitis (CP) complications in adults are successfully managed with endoscopic retrograde cholangiopancreatography (ERCP). Data are limited on the success of ERCP in children with CP. We reviewed our experience to ascertain the success, safety, and effectiveness of ERCP for relieving pain and its effect on exocrine and endocrine insufficiency. METHODS:Retrospective analysis of a prospectively kept database of children (≤18 y) with CP who underwent therapeutic ERCP between 2010 and 2020 was reviewed. Clinical details, laboratory and radiologic investigations, procedural details, and follow-up data were retrieved from manual and electronic records. Categorical and numerical data were expressed as proportion and mean ± SD. RESULTS:Sixty-five children (60% males), age 12.8 ± 2.9years, were included. The most common etiology was idiopathic (n = 45, 69%). Technical, endoscopic, and clinical success rates were 95.3%, 92.3%, and 85.6%, respectively. Only 4 patients (6.1%) had major complications. Over a median follow-up of 24 months, therapeutic ERCP resulted in significant pain relief, with nonprogression of exocrine and endocrine insufficiency. Recurrence of symptoms was noted in 10%, which were managed successfully with repeat ERCP. CONCLUSION:Therapeutic ERCP is a safe and effective therapy for the management of CP in children, with significant improvement in pain, and could influence stabilization of exocrine and endocrine functions.

Acute kidney injury (AKI) is present in 30%-40% of hospitalized patients with cirrhosis. Its incidence is higher in patients with severe alcoholic hepatitis, spontaneous bacterial peritonitis, and acute-on-chronic-liver failure (ACLF). Kidney injury is an important landmark event in the natural history of cirrhosis as it is associated with higher mortality. Overwhelming systemic vasodilation, cardiac dysfunction, hypoperfusion, endotoxemia, and direct nephrotoxicity predispose patients with cirrhosis to kidney injury. Infection is present in 25% of patients with decompensated cirrhosis and 35%-40% of patients with ACLF. Advanced cirrhosis with portal hypertension leads to a sluggish portal flow, leading to increased gut congestion, altered gut permeability and bacterial translocations. They drive infection and endotoxemia in such patients. Pathogen-associated molecular patterns activate inflammatory cascades, which leads to further deterioration in hemodynamics and reduced glomerular filtration rate. Infections and pro-inflammatory cytokines like interleukin 6 (IL-6), IL-1, and tumor necrosis factor alpha may directly cause kidney parenchymal injury. The combined effect of dysfunctional albumin and systemic and splanchnic vasodilatation leads to low effective blood volume, activating the renin-angiotensin-aldosterone system. This causes renal vasoconstriction, water retention, and ascites, which progresses to hepatorenal physiology and AKI development. Vasoconstriction and volume expansion effectively improve arterial blood volume and systemic hemodynamics, thereby improving renal blood flow. It is of paramount importance to predict, detect, and treat AKI in its early state, as progressive renal dysfunction is invariably associated with higher mortality in patients with decompensated cirrhosis and ACLF. This comprehensive review will focus on the recent evolving concepts of the pathophysiology, diagnosis, and management of AKI in patients with cirrhosis.

Pediatric inflammatory bowel disease (PIBD) is a chronic inflammatory disorder of the gastrointestinal tract, with rising global incidence and prevalence. Over the past two decades, biologics have added to the therapeutic armamentarium and revolutionized the approach to treatment of inflammatory bowel disease. The available biologics include monoclonal antibodies which target inflammatory cytokines (anti-tumor necrosis factor alpha, anti-interleukin 12/23) or recruitment of leucocytes to the gastrointestinal tract (anti-alpha4beta7 integrin) and small molecules (Janus kinase inhibitors, sphingosine 1-phosphate-inhibitors) which modify the proinflammatory signaling. Considering their potential disease-modifying ability, recent pediatric guidelines from the West have advocated upfront use of biologics in appropriate clinical scenarios as a top-down approach rather than the conventional step-up approach. Although real-world studies are available regarding the clinical efficacy of biologics in PIBD, there is paucity of long-term outcome and safety data in children. Also, little information is available about the best approach in the newly industrialized - developing countries where PIBD is rising but at the same time, infections are prevalent and resources are limited. In this review, we summarize the efficacy and safety profile of biologics and small molecule drugs and discuss the challenges in the management of PIBD, especially in the developing world, and future directions.

Objectives: Paucity of data and concerns about potential lower effectiveness and more adverse events limit the use of endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic cystogastrostomy in younger children even in high-volume centers. We retrospectively analyzed indications, success rates, and adverse events of all the children (<18 years) who underwent ERCP and endoscopic cystogastrostomy between January 2010 to May 2024 at our center. Methods: Data, including patient demographics, indications for the procedure, technical details, and adverse events, were collected from our prospectively kept database and compared according to age groups (<1 year, 1-5 years, 5-10 years, and 10-18 years). Results: A total of 286 ERCP (273 therapeutic and 13 diagnostic) and 57 endoscopic cystogastrostomy were performed in 222 (138 boys) and 55 children (32 boys), respectively, during the study period with 20% ERCP procedures in under-five children. In children <5 years, the majority of the ERCPs were for biliary diseases (87%), while pancreatic duct procedures (39.5%) were done in higher numbers in children >5 years. For biliary ERCP, choledochal cyst (15, 33%) was the most common etiology in under-five children and choledocholithiasis (60, 34%) in children >5 years. Cannulation and technical success rates were 95% and 92%, respectively with no significant difference across age groups. Adverse events were noted in 36 (16%) with post-ERCP pancreatitis (8%) being the most common. All adverse events were managed conservatively with no mortality. Conclusion: ERCP can safely be performed in all children, including those under five with various hepato-pancreato-biliary diseases with high technical success rates.

The European Society of Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) 2024 guidelines on eosinophilic esophagitis in children provide a systematic approach to the diagnosis and management of this rising disease entity in children. We present a concise update of the guideline to simplify management protocols, thus improving patient outcome.

Background/Aims: Acute liver injury is a common manifestation of parvovirus B19 (PVB19) infection in immunocompromised patients. However, literature in immunocompetent children is scarce. We aimed to study the clinicolaboratory features and outcome of hepatic involvement by PVB19 infection in hospitalized children. Methods: We retrospectively analyzed our prospectively kept database of all children (<18 years old) admitted with acute viral hepatitis (AVH), acute liver failure (ALF) or acute-on-chronic liver failure (ACLF), and PVB19 infection between January 2010 and December 2023. Clinical features, laboratory parameters, and complications were evaluated. Poor outcome was defined as death or liver transplantation. Results: A total of 35 children (19 boys [54%j, median age: 7.25 [interquartile range: 4-10.8j years) with PVB19-related hepatitis were studied (28 [80%j isolated PVB19 infection and 7 [20%j coinfections [3 with Epstein-Barr virus, 2 with hepatitis A, and 1each with hepatitis- E and cytomegalovirusj). AVH (17, 49%) was the most common presentation, followed by ALF (13, 37%) and acute insult in ACLF (5, 14%). Patients with coinfection had significantly higher bilirubin (14.6 [9.4-21.5j vs 6.8 [4.3- 10.9j mg/dl; P =0.004) and transaminases (ALT: 697 [428-1296j vs. 277 [157-478j U/L; P =0.02) but similar mortality (1/7 vs 6/23; P =1.0) than PVB19 alone. Nine cases (25.7%) had extrahepatic complications (hemophagocytic lymphohistiocytosis [HLHj: 3, acute kidney injury: 3, aplastic anemia: 2, and myocarditis: 1). Poor outcome occurred in 38% (5/13) ALF, 11.7% (2/17) AVH (HLH: 1, myocarditis: 1), and none (0/5) of the ACLF cases. Conclusion: PVB19 should be considered in children presenting with indeterminate acute liver injury, especially in younger children or those with complications such as aplastic anemia, HLH, or myocarditis. ( J C LIN E XP H EP- ATOL 2025;15:102416)

Objectives:Strict gluten-free diet (GFD) is the mainstay of therapy in celiac disease (CD), however, data on factors determining long-term adherence is limited. We aimed to compare serology, dietician detailed interview (DDI) and various questionnaires (Biagi, modified Leffler) to assess GFD adherence, to identify determinants of adherence and to assess the impact of counseling. Methods:In this prospective study, consecutive children (<18 years) with CD on GFD were enrolled between November 2022 and May 2024. GFD adherence was assessed by anti-tissue transglutaminase (tTG), DDI and two questionnaires at baseline and after 6 months of follow-up. Results:A total of 103 children (64 boys, median age 8 years [interquartile range [IQR]: 4-12.5]) on GFD (median duration 35 months [IQR: 11-52]) were enrolled. Adherence as assessed by serology, DDI, Biagi score and modified Leffler score were 73.8%, 48.5%, 66.2%, and 60.3%, respectively. Upon regression analysis, having a sibling with CD, and higher parental knowledge about CD influenced positively to adherence to GFD, while age >10 years and longer duration of follow-up influenced negatively to adherence. After dietary counseling, GFD-adherence improved on follow-up (p < 0.05 in all). Conclusions:Almost a third of children have adherence issue. Having a sibling with CD, and higher parental awareness about CD positively and older age and longer follow-up negatively influenced GFD-adherence. Adherence improved significantly with dietary counseling on follow-up.

A 16-year-old boy presented with a history of painless, fresh bleeding from the rectum and a prolapsing mass during defecation for the previous 6 months. On digital rectal examination, multiple polyps were felt. A full colonoscopy revealed a large (2 cm) multilobulated polypoidal growth with an ulcerated surface just above the dentate line covering >50% of the lumen (A). Contrast-enhanced CT showed an eccentric, asymmetrical polypoidal lesion with enhancing wall thickening in the rectum, without any locoregional or distant lymph node involvement (B).

Cholestatic liver diseases in children often have an underlying genetic defect. Genetic testing by next-generation sequencing has become a crucial part of the diagnostic armamentarium in such clinical scenarios. Here, we report three children who presented with early-onset cholestatic jaundice and pruritus. All of them had low gamma-glutamyl transferase and high serum bile acid levels. Symptoms were alleviated with ursodeoxycholic acid and cholestyramine in all 3 children with normal LFT at follow-up. They were detected to have novel pathogenic USP53 mutations (2 homozygous, 1 compound heterozygous) on next-generation sequencing which have previously not been reported.

ARTICLEde novo HCC is also seen in children. 1 Hemangioendothelioma presents as massive hepatomegaly with severe anemia and cardiac failure.In addition, there may be features of hypothyroidism (constipation and lethargy).There is a high expression of type III iodothyronine deiodinase activity within the tumor.This enzyme is responsible for the conversion of T4 and T3 to inactive metabolites.Sudden onset of pain in the right upper quadrant and recurring ascites with prominent dilated abdominal veins can be seen in acute BCS.

Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare entity that mimics various inflammatory strictures of the small intestine. Pediatric literature is scarce. We analyzed the clinical, radiological, endoscopic and histopathological features of children with CMUSE that differentiate it from small bowel Crohn’s disease (SBCD) and gastrointestinal tuberculosis (GITB). CMUSE was diagnosed by the following criteria: (1) unexplained small bowel strictures with superficial ulcers, (2) chronic/relapsing ulcers of small bowel after resection, (3) no signs of systemic inflammation, (4) absence of other known etiologies of small bowel ulcers. SBCD and GITB were diagnosed based on standard criteria. The clinical features, laboratory parameters, radioimaging, endoscopy (including video capsule endoscopy [VCE], intra-operative endoscopy), histopathological features and treatment outcome were noted. Out of 48, CMUSE was diagnosed in 13 (27

The worldwide epidemiology of non-alcoholic fatty liver disease (NAFLD) is showing an upward trend, parallel to the rising trend of metabolic syndrome, owing to lifestyle changes. The pathogenesis of NAFLD has not been fully understood yet. Therefore, NAFLD has emerged as a public health concern in the field of hepatology and metabolisms worldwide. Recent changes in the nomenclature from NAFLD to metabolic dysfunction-associated steatotic liver disease have brought a positive outlook changes in the understanding of the disease process and doctor-patient communication. Lifestyle changes are the main treatment modality. Recently, clinical trial using drugs that target ‘insulin resistance’ which is the driving force behind NAFLD, have shown promising results. Further translational research is needed to better understand the underlying pathophysiological mechanism of NAFLD which may open newer avenues of therapeutic targets. The role of gut dysbiosis in etiopathogenesis and use of fecal microbiota modification in the treatment should be studied extensively. Prevention of this silent epidemic by spreading awareness and early intervention should be our priority.

BACKGROUND:Post-endoscopic retrograde cholangio-pancreatography pancreatitis (PEP) is seen in 3% to 16% of children undergoing therapeutic endoscopic retrograde cholangio-pancreatography (ERCP). We evaluated the risk factors of PEP and utility of 4-hour post-ERCP amylase and lipase for early prediction of PEP in children with chronic pancreatitis (CP). MATERIALS AND METHODS:Thirty children with CP (boys 20, 14.3 [interquartile range, 9.3-16] years) who underwent 62 ERCP procedures were studied. Clinical and procedural details with outcome were noted. Serum amylase and lipase were measured before, 4 hours, and 24 hours after ERCP. Multivariate analysis was done to identify risk factors for PEP. Cutoff scores of 4-hour amylase and lipase were identified. RESULTS:PEP occurred in 14.5% (9/62) of ERCP procedures (mild, 8; moderate, 1) with no mortality. On univariate analysis, endoscopic sphincterotomy ( P = 0.04), difficult cannulation ( P = 0.004), and prior PEP ( P = 0.036) were risk factors, while prior ERCP ( P = 0.04) was protective. Difficult cannulation (odds ratio, 5.83; 95% confidence interval, 1.329-25.592) was the independent risk factor on multivariate analysis overall and for first ERCP session alone. Amylase >3.3 times upper limit of normal (ULN) and lipase of >5 times ULN at 4 hours had best sensitivity and specificity for diagnosis of PEP. All cases with PEP were symptomatic by 6 hours and none had amylase/lipase <3 ULN at 4 hours. Amylase/lipase of <3 ULN at 4 hours could exclude PEP with good sensitivity (100%) and specificity (76% and 81%, respectively). CONCLUSIONS:PEP occurred in 14.5% of procedures in children with CP, with difficult cannulation being the independent risk factor. Asymptomatic patients with 4-hour amylase/lipase <3 times ULN can be safely discharged.

Pediatric acute liver failure (PALF) is a catastrophic clinical condition with very high morbidity and mortality without early detection and intervention. It is characterized by the acute onset of massive hepatocellular injury that releases circulating inflammatory mediators, resulting in metabolic disturbances, coagulopathy, hepatic encephalopathy and multi-organ failure. The etiological spectrum is dominated by hepatotropic viruses, drug-induced liver injury, metabolic and genetic disorders and immune-mediated diseases. Unlike adults, indeterminate causes for acute liver failure constitute a considerable proportion of cases of acute liver failure in children in the west. The heterogeneity of age and etiology in PALF has led to difficulties in developing prognostic scoring. The recent guidelines emphasize prompt identification of PALF, age-appropriate evaluation for hepatic encephalopathy and laboratory evaluation with careful monitoring. Current therapy focuses on supporting the failing liver and other organs, pending either spontaneous recovery or liver transplantation. Targeted therapy is available for a select group of etiologies. Liver transplantation can be lifesaving and a plan for the same should be organized, whenever indicated. The aim of this review is to define PALF, understand its etiopathogenesis, address the challenges encountered during the management and update the latest advances in liver transplantation and non-transplant treatment options in PALF.

Due to its complex pathogenesis, treatment of hepatic encephalopathy (HE) continues to be a therapeutic challenge. Of late, gut microbiome has garnered much attention for its role in the pathogenesis of various gastrointestinal and liver diseases and its potential therapeutic use. New evidence suggests that gut microbiota plays a significant role in cerebral homeostasis. Alteration in the gut microbiota has been documented in patients with HE in a number of clinical and experimental studies. Research on gut dysbiosis in patients with HE has opened newer therapeutic avenues in the form of probiotics, prebiotics and the latest fecal microbiota transplantation (FMT). Recent studies have shown that FMT is safe and could be effective in improving outcomes in advanced liver disease patients presenting with HE. However, questions over the appropriate dose, duration and route of administration for best treatment outcome remains unsettled.

Hepatic pseudotumors are rare lesions of unknown origin, characterized by the proliferation of fibrous connective tissue and inflammatory cell infiltrates. They mimic malignant lesions clinically, and radiologically, given their non-specific clinical and imaging features. The pathophysiology of hepatic pseudotumor is incompletely understood and there are no standardized criteria for diagnosis. Pseudotumors have been reported to develop in various organs in the body with the lung and liver being the most common site. Hepatic pseudotumors develop in patients with underlying triggers of liver inflammation and injury, including infections, autoimmune liver diseases, bile duct injury, or surgery. Hepatic pseudotumors respond well to conservative treatment with antibiotics, and steroids and some may regress spontaneously, thus avoiding unnecessary resection. This condition is rewarding to treat. It is important to recognize pseudotumor as a distinct clinical entity and include it in the differential of liver masses with atypical imaging features.