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

Purpose:Endoscopic variceal hemostasis is a critical, life-saving intervention frequently performed by pediatric gastroenterologists. To ensure safe and effective practices, the Asian Pacific Society for Pediatric Gastroenterology, Hepatology and Nutrition Endoscopy Scientific Subcommittee developed a consensus-based position statement. Methods:A comprehensive literature review was conducted with an emphasis on pediatric evidence. Nineteen draft statements were formulated and refined through electronic votes and virtual meetings. Statements with <80% agreement were revised until consensus was reached. Levels of evidence certainty were evaluated to support the final recommendations. Results:Nineteen position statements, covering screening, acute management, and surgical options, were endorsed. Although noninvasive tools such as elastography and platelet-based indices may assist in risk stratification, endoscopy remains the diagnostic and therapeutic gold standard. Endoscopic variceal ligation was identified as the preferred modality for both prophylaxis and treatment in children ≥10 kg, outperforming sclerotherapy and non-selective beta-blockers. Standard acute management incorporates octreotide and restrictive transfusion strategies, with endoscopy performed within 24 hours. In refractory cases, the Meso-Rex stunt or transjugular intrahepatic portosystemic shunt may be considered depending on anatomy and available expertise. Conclusion:These findings provide an evidence-based framework for the management of pediatric variceal bleeding. Individualized care is emphasized while highlighting the pressing need for high-quality pediatric studies to strengthen future recommendations.

Background:Classical galactosemia (CG), if untreated, can be life threatening in early infancy; however, with early galactose restriction, survival has improved markedly. Despite early diagnosis and dietary exclusion, complications may ensue. This study aimed to identify risk factors for adverse outcomes in CG. Methods:We conducted an analysis of our prospectively maintained database. Diagnosis was based on galactose-1-phosphate uridyltransferase (GALT) enzyme levels <10u/gm hemoglobin or mutation of the GALT gene. Clinical and laboratory data were retrieved from hospital electronic records and analyzed to identify potential predictors of mortality or neuro-ophthalmic morbidity (poor neurocognition, learning disability, and new-onset or persistent cataract). Results:Fifty-nine CG patients presented with infantile cholestasis. Their median age of symptom onset and diagnosis was 16 (interquartile range [IQR]: 3-90) and 50 (IQR: 4-120) days, respectively. Among the 48 survivors, 41 had follow-up for ≥18 months and were analyzed for long-term outcomes. Complete liver recovery was documented in all 41 patients, with a median time to recovery of 5 months (IQR: 3-8) following diagnosis. On a lactose-free diet (LFD), 8 (19.5%) developed new-onset cataracts, and 21 (51.2%) patients had neurocognitive issues. The univariate analysis of non-survivors (n = 11) versus survivors (n = 48) identified risk factors: older age at diagnosis, high baseline Child-Turcotte-Pugh (CTP) and Pediatric End-Stage Liver Disease (PELD) scores, low serum sodium and albumin levels, and higher international normalized ratio values. In addition, refractory ascites, persistent coagulopathy at 4 weeks on LFD, and culture-positive sepsis were significantly associated risk factors in the non-survival group. No significant predictors were found for neurocognitive issues and cataract in follow-up. Conclusion:PELD and CTP scores at admission predict survival. Long-term neuro-ophthalmic morbidity is not associated with liver disease severity at onset in CG.

Background:As serum ceruloplasmin has ferroxidase properties, we hypothesized that Wilson disease (WD) may have greater iron accumulation than other liver diseases. We aimed to assess liver iron overload in WD by evaluating the iron metabolism biomarkers and liver iron concentration (LIC). Methods:Compensated and recompensated WD patients with ≥3 years of chelation and serum exchangeable copper (ExCu) < 1.15 μmol/L were recruited and compared to controls. All patients underwent assessment of iron metabolism biomarkers and T2∗-weighted liver magnetic resonance imaging (MRI) for LIC. High LIC was defined as >1.5 mg/g dry weight (dw). Results:Thirty-seven WD patients were compared to age, sex, and liver disease score-matched controls (n = 10). High LIC was seen in 49% WD vs. 10% controls (P = 0.027). In those with a duration of chelation ≥6 years vs. <6 years, high LIC was found in 89% vs. 58% (P = 0.03). High LIC was seen in 3/9 (30%), 7/15 (47%), and 8/13 (62%) of the WD patients in 3-5 years, 6-9 years, and 10-12 years of chelation therapy, respectively. In those with LIC >1.5 mg/g dw (n = 18) and LIC >2.0 mg/g dw (n = 10), longer duration of chelation therapy inversely correlated with serum ferroxidase activity (r = -0.7, P < 0.001; r = -0.75, P = 0.01 respectively). Serum ferritin had poor correlation with LIC (r = 0.177, P = 0.3). Mean (standard deviation) ExCu in high vs. normal LIC were 0.66 (0.27) vs. 0.94 (0.33), P = 0.01. Conclusion:High LIC is found in approximately half of WD patients, especially in those with ≥6 years of chelation therapy and low ExCu. MRI is recommended as a screening tool for iron overload in WD.

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.

Drug-resistant abdominal tuberculosis (DR-ATB) is suspected in subjects with non-response to standard therapy despite good compliance. Literature is scarce in children. We studied symptoms, yield of investigations, management and outcome of DR-ATB in children. The electronic database of children (<18 years) diagnosed with DR-ATB over last 15 years was analysed retrospectively and clinical profile, investigations, treatment and outcome were noted. Samples were subjected to microscopy, Xpert MTB/RIF Ultra assay, culture, second-line drug susceptibility test (SL-DST) by line probe assay and histology. DR-TB was classified as per guidelines. Thirteen girls (median age: 15 years) presented with anorexia (12, 92%), fever (11, 85%), weight-loss (11, 85%), abdominal pain (11, 85%), lump (6, 46%), intestinal obstruction (5, 38%) and perforation (6, 46%). Imaging showed necrotic lymph-nodes (11, 85%), bowel thickening (9, 69%), ascites (8, 62%), omental thickening (5, 38%), visceral involvement (4, 31%) and tubo-ovarian mass (3, 23%). All had multiple site involvement in abdomen and had received anti-tubercular therapy (ATT) previously. Eight (62%) cases had extra-abdominal tuberculosis. Eleven (85%) cases were rifampicin-resistant (one was pre-extensively drug resistant [pre-XDR]) and 1 (7%) case each was isoniazid mono-resistant and probable XDR-TB. ATT was given for 24 (10-42) months, 11 (85%) responded, one succumbed and one was lost to follow-up. Adverse events occurred in five (38%) cases. In conclusion, rifampicin-resistance accounted for 85% of all DR-ATB. Majority of the patients with DR-ATB were adolescent females with past history of ATT, large necrotic lymph-nodes and multiple site involvement in abdomen. Majority showed good response to therapy.

Access to quality healthcare for individuals with chronic diseases like inflammatory bowel disease (IBD) remains a global challenge. Tweens (aged 9-12 years) and teens (typically aged 13-19 years) with IBD face unique challenges compared to adults, including limited access to medications, difficulties transitioning to adult care, and barriers to clinical trial enrollment. Additional concerns include mental health, social media influence, and growth through puberty. This review article brings together current evidence reported by pediatric IBD clinicians worldwide to highlight these issues. While many challenges are universal, some are region specific and reflect geographic disparities in care.

Functional abdominal pain disorders (FAPD) are common in children and there is heterogeneity in the available literature regarding the role of fibre in FAPD and none so-far in functional abdominal pain-not otherwise specified (FAP-NOS). The study aimed to evaluate the efficacy of psyllium fiber in pediatric FAPD including Irritable bowel syndrome (IBS) and FAP-NOS. In this prospective randomized placebo-controlled trial, children (5–18 years) diagnosed as FAPD based on Rome IV criteria were randomized to psyllium fiber or placebo (maltodextrin) for 12 weeks. Post-treatment improvement of pain and quality of life (QoL) from baseline was compared between the two groups. Pain assessment was done using the pain score table, which assessed duration, frequency, and intensity of pain. The mean age of 109 children (FAP-NOS: 85 http://ctri.nic.in ).

Background/Aims:Portosystemic shunt (PSS) surgery is a well-established procedure for managing portal hypertension and associated complications of extrahepatic portal venous obstruction (EHPVO) in children. However, the long-term renal effects have not been systematically studied. We aimed to evaluate the prevalence, clinical spectrum, histopathology, treatment response, and outcomes of nephropathy developing after PSS in children with EHPVO. Methods:We conducted an ambispective observational cohort study of 315 EHPVO children undergoing non-selective PSS (August 1996-December 2024). Patients were followed with monitoring and Doppler ultrasonography to assess shunt patency. If the clinician suspected urinary protein loss, then serial measurements of serum creatinine and blood urea nitrogen, and spot/24-h urine protein quantification was done. Renal biopsy was performed if proteinuria was persistent. The clinical, biochemical, and histologic findings, treatment, and outcomes were systematically recorded. Results:Twelve of 315 children (4%) developed post-shunt nephropathy in a span of 24 months (interquartile range [IQR]: 18-36) from PSS. Renal biopsies were suggestive of membranoproliferative glomerulonephritis (n = 6), immunoglobulin A nephropathy (n = 4), focal segmental glomerulosclerosis (n = 1), and diffuse global glomerulosclerosis (n = 1). Median total cholesterol at presentation was 312 mg/dL (IQR: 268-386) and median triglycerides 196 mg/dL (IQR: 148-268). Treatment included angiotensin receptor blockers (n = 11), immunosuppressive therapy (n = 7), and renal replacement therapy (n = 2). Partial remission occurred in 7 cases; four children progressed to end-stage renal disease, and two died. One patient experienced recurrence of nephropathy in the renal graft. Conclusions:Post-shunt nephropathy is an under-recognized but serious complication following PSS in EHPVO. Renal surveillance, early biopsy, and timely therapy are critical.

Introduction: Biliary atresia (BA) is a major cause of neonatal cholestasis and the leading indication for pediatric liver transplantation. Timely and accurate diagnosis is critical to enable early surgical intervention and improve native liver survival. Percutaneous transhepatic cholecysto-cholangiography (PTCC) has emerged as a minimally invasive alternative to intraoperative cholangiography (IOC) for diagnosing BA. This prospective study aimed to evaluate the feasibility, safety, and diagnostic accuracy of PTCC in differentiating BA from other causes of neonatal cholestasis. Materials and Methods: Conducted between October 2023 and March 2025, this study enrolled infants under 6 months of age with clinical suspicion of BA. All patients underwent ultrasonography and PTCC. PTCC feasibility, diagnostic accuracy, and safety were assessed in comparison with IOC. Gallbladder (GB) parameters and laboratory findings were also analyzed. Results: Of the 45 enrolled patients, PTCC was feasible in 62.2% (28/45), diagnosing BA in 71.4% (20/28) and excluding it in 28.6% (8/28). PTCC was not feasible in 37.8% (17/45). Overall, BA was confirmed in 77.8% (35/45). Gallbladder parameters were evaluated as predictors of PTCC feasibility. Receiver operating characteristic analysis identified fasting GB volume, length, and width as significant predictors (P < 0.05) for PTCC feasibility (area under the curve 0.789, 0.716, 0.727, respectively). GB width demonstrated the highest sensitivity (82.1%) for predicting PTCC feasibility. No procedural complications were observed. Conclusion: PTCC is a safe, feasible, and reliable diagnostic modality for preoperative assessment of BA. GB parameters on ultrasound are significant predictors of PTCC feasibility, reinforcing its integration into the diagnostic workup for neonatal cholestasis.

Aims:This study aimed to evaluate the feasibility, safety, and efficacy of laparoscopic excision of choledochal cysts (CDCs) with Roux-en-Y hepaticojejunostomy (HJ) in pediatric patients at a tertiary care teaching institution in India. Methods:We conducted a retrospective review of electronic medical records from November 2019 to November 2024, involving 43 children who underwent laparoscopic CDC excision with Roux-en-Y HJ in a single surgical unit. Data collected included demographic information, clinical presentation, imaging features, CDC classification, preoperative interventions, surgical details, postoperative complications, and follow-up outcomes. Laparoscopic HJ was performed by a single surgeon with a four-port technique with extracorporeal jejunojejunostomy. Results:The cohort consisted of 43 children (26 girls), with a mean age of 5.5 (0.33-17) years and a mean weight of 16.5 (6-48) kg, including seven infants. Preoperative interventions were required in 11 (25.6%) children. The mean operative time was 406 (315-545) min, with an 11.6% conversion rate to open surgery due to difficult anatomy. Postoperative complications were observed in 34.8% of patients, primarily minor (Clavien-Dindo Grade I or II), including bile leak in 6.9% of patients, managed without further intervention. Major complications included adhesive obstruction and anastomotic stricture, requiring reoperations in two patients. The mean time to full enteral feeds was 6.5 (4-9.5) days, and the mean hospital stay was 9.3 (5-25) days. Follow-up (mean duration: 27.2 months) showed no intrahepatic biliary radical dilation or recurrent cholangitis or pancreatitis, with normal liver function tests in all patients except one who developed liver decompensation and was lost to follow-up. Conclusions:Laparoscopic excision of CDC with Roux-en-Y HJ in children is a safe, feasible, and effective approach, offering numerous benefits while presenting manageable risks and complications. With advancements in minimally invasive techniques and increased surgeon expertise, this method holds promise for becoming a widely adopted standard in pediatric surgery.

To validate the disease-specific quality of life (QoL) instrument for pediatric inflammatory bowel disease (PIBD) patients in three Indian languages (Hindi, Tamil and Bengali). Additionally, also to reveal the significant factors which effect QoL of PIBD patients in India. One hundred and two (102) PIBD patients (mean age 13 ± 2.59 y) across 6 centres were enrolled. Each child completed two questionnaires - the IMPACT-III and Paediatric Quality of Life Inventory Version 4.0 Generic Core Scale (PedsQL™) - in one of the three languages. A uniform clinico-demographic proforma was completed for each recruit to reveal factors which determine QoL. During analysis authors used Cronbach’s alpha for internal consistency, principal component analysis for factor analysis, Spearman’s correlation between the questionnaires for concurrent validity and ANOVA analysis between IMPACT-III health-related quality of life (HRQoL) scores and disease severity to establish discriminant validity. A five-domain structure was most suitable: ‘Concerns’, ‘Social acceptance’, ‘Mental disposition’, ‘Disease adjustment’ ‘Self-confidence’, with good internal reliability (Cronbach’s α = 0.73–0.94). Concurrent and discriminant validity of the new questionnaire was also statistically significant (p < 0.001). Higher monthly family income led to better QoL scores in the ‘Concerns’ (p = 0.04) and ‘Disease adjustment’ (p = 0.03) domains while children with ulcerative colitis (UC) had better ‘Social acceptance’ scores than children with Crohn’s disease (CD) (p = 0.02). Modified IMPACT-III questionnaire with a five-domain structure demonstrated good validity and reliability for Indian population. ‘Social acceptance’ was higher in patients with ulcerative colitis. There is a favourable impact of higher family income on ‘Concerns’ and ‘Disease adjustment’ in PIBD.

ABSTRACT Background/Aims Pediatric functional abdominal pain disorders (FAPDs) subtypes; functional dyspepsia (FD), irritable bowel syndrome (IBS), functional abdominal pain‐not otherwise specified (FAP‐NOS), and abdominal migraine (AM) are influenced by demographic and social factors. The study aimed to evaluate the spectrum and demographic and social factors associated with FAPD subtypes. Methods Consecutive children (< 18 years) diagnosed with FAPD subtypes according to ROME‐IV criteria between April 2018 and March 2020 were included. The clinical, demographic, and social parameters were analyzed between various subtypes of FAPD, and factors responsible for severe symptoms were studied. Results A total of 479 children (mean age 12.34 ± 3.82 years, 60% boys) were included. FAP‐NOS (63%) was the most commonly diagnosed subtype followed by IBS (17.4%) and FD (15%). The age at presentation, site of pain, duration of symptoms, and associated symptoms were significantly different among the three main subtypes (p < 0.001). Stressors could be identified in 39.3% and academic pressure (22.3%) was the most common. Family members with functional disorders (OR: 2.21, 95% CI: 1.31–3.42, p = 0.02), presence of stressors (OR: 2.03, 95% CI: 1.14–3.65, p = 0.016), and rural origin (OR: 1.75, 95% CI: 1.08–2.83, p = 0.023) predicted severe symptoms. Conclusions FAP‐NOS is the most common FAPD subtype in children in India. Children with FAP‐NOS are much younger than other subtypes of FAPD. The presence of stressors and functional disorders in family members could be associated with severe symptoms. However, it mandates more prospective studies to validate the findings.

Children diagnosed with inflammatory bowel disease (IBD) before the age of 6 years are considered to have “very early-onset IBD (VEO-IBD),” which is challenging to diagnose and treat. Notably, many children with VEO-IBD have monogenic forms of the disease, meaning that early genetic testing is useful. However, because the prevalence of genetic variants causing VEO-IBD differs globally, the diagnosis and treatment of this disease should be tailored to each region. In the present review paper, the IBD Subcommittee of the Scientific Committee of the Asia-Pacific Society of Pediatric Gastroenterology, Hepatology and Nutrition (APSPGHAN) has summarized the epidemiology, presenting features, diagnosis, and treatment of VEO-IBD in the Asia–Pacific region, with an aim to guide clinicians and researchers who work with VEO-IBD in this area. Our 3 main messages are as follows: endoscopy is essential for VEO-IBD diagnosis; all children diagnosed with VEO-IBD should be suspected of having a monogenic form; and children with suspected monogenic IBD should undergo early genetic testing. Our messages aim to improve the early diagnosis and treatment of VEO-IBD in the Asia–Pacific region, including the early detection of monogenic IBD in this area.

Congenital antral web: rare cause of gastric outlet obstruction successfully managed with endoscopic balloon dilatationRunning title: Congenital antral web: a rare cause of gastric outlet obstruction Key messageCongenital antral web, though uncommon, is an important cause of gastric outlet obstruction in children.The diagnosis can be established on upper gastrointestinal endoscopy by the presence of normal distal pylorus beyond the antral web.Treatment consists of surgery or therapeutic endoscopy.As the web lacks a muscle layer, endoscopic dilatation is effective.Here we report a case of congenital antral web that was successfully managed with endoscopic balloon dilatation.