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Timely diagnosis and management of pediatric acute liver failure (PALF) is of paramount importance to improve survival. The Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition invited national and international experts to identify and review important management and research questions. These covered the definition, age appropriate stepwise workup for the etiology, non-invasive diagnosis and management of cerebral edema, prognostic scores, criteria for listing for liver transplantation (LT) and bridging therapies in PALF. Statements and recommendations based on evidences assessed using the modified Grading of Recommendations Assessment, Development and Evaluation (GRADE) system were developed, deliberated and critically reappraised by circulation. The final consensus recommendations along with relevant published background information are presented here. We expect that these recommendations would be followed by the pediatric and adult medical fraternity to improve the outcomes of PALF patients., (© 2024. Asian Pacific Association for the Study of the Liver.)

Purpose: This single-center retrospective study explores the safety and efficacy of 177 Lu-DOTATATE in children and young adult population with metastatic/inoperable neuroendocrine tumors (NETs)., Patients and Methods: This study is a retrospective analysis of all children and young adult patients (≤29 years) with advanced inoperable/metastatic epithelial or nonepithelial NETs who were administered a median of 4 cycles of 177 Lu-DOTATATE therapy and low-dose oral capecitabine as a radiosensitizer every 8-12 weeks, except 2 patients who received CAPTEM chemotherapy. The radiological response was assessed using RECIST 1.1 on interim and end-of-treatment 68 Ga-DOTANOC PET/CT. The primary endpoint was objective response rate, whereas disease control rate, toxicity profile, progression-free survival, and overall survival were secondary endpoints., Results: Nineteen biopsy-proven NET patients (median age, 22 ± 10 years) with 8 of them adolescents (10-18 years) and the remaining young adults (19-29 years) were included. Fourteen patients had gastroenteropancreatic neuroendocrine tumor (pancreas being most common primary site), whereas the rest had non-gastroenteropancreatic neuroendocrine tumor. A total of 65 cycles of 177 Lu-DOTATATE (range, 1-6 cycles) were administered with a median cumulative activity of 600 mCi (range, 100-1000 mCi). The objective response rate and disease control rate were 41% and 94%, respectively. Grade 1 and 2 adverse events were observed in 14 (74%) and 5 (26%) of 19 patients, respectively. In a total of 8 events (42%), 4 events each of disease progression and death occurred during a median follow-up of 80.1 months with an estimated 5-year progression-free survival and overall survival of 54% (95% confidence interval, 30-78) and 63% (95% confidence interval, 39-87), respectively., Conclusions: 177 Lu-DOTATATE appears safe and effective in children and young adults with metastatic/inoperable NETs. Large prospective trials are required to validate these results., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction  The native liver survival in biliary atresia (BA) depends on various factors, and one of the crucial factors is the rate of progression of liver fibrosis after portoenterostomy, but there is no reliable investigation to assess it. This study evaluated shear wave elastography (SWE) to detect liver fibrosis in BA patients and assess its utility during follow-up. Materials and Methods  This was an observational study; SWE was done preoperatively and postoperatively at 3 and 6 months. The SWE values were analyzed to determine their correlations with preoperative liver histology as well as with postoperative SWE variation between different postoperative outcomes. Results  Twenty-one patients were included in the study; the preoperative SWE values were strongly correlated with liver biopsy grading ( p  < 0.001). At the 3 months postoperatively, SWE was done for 18 children: 12 in group A (patent bilioenteric drainage on hepatobiliary iminodiacetic acid scan) and 6 (nonpatent) in group B; mean SWE value was 12.8 and 17.3 kPa, respectively ( p  < 0.001). Ten children from group A underwent SWE 6 months postoperatively, and the mean value was 13.23 kPa. Conclusion  The SWE values correlate with liver histology grading, suggesting a reliable alternative to biopsy. Additionally, the baseline SWE values and their trend during follow-up can provide information on the disease's progression., Competing Interests: Conflict of Interest None declared., (Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. ( https://creativecommons.org/licenses/by-nc-nd/4.0/ ).)

Abstract: Gastrinomas with predilection for the adult male population are located in the gastrinoma triangle (>90%). Primary hepatic gastrinoma especially in pediatric population is very rare. Peptide receptor radionuclide therapy has shown benefit in metastatic gastroenteropancreatic neuroendocrine tumors (NETs) with an increasing interest in expanding its role as neoadjuvant treatment modality to improve the surgical candidature in inoperable NETs. There is currently no literature supporting its role in the pediatric NET patients. We present a rare case of a young boy with primary hepatic gastrinoma where 177Lu-based peptide receptor radionuclide therapy in the neoadjuvant setting contributed to his final disease-free status., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To compare the acquisition time, diagnostic efficacy, and image quality of the newer compressed SENSE 3D MRCP (CS-3D MRCP) with conventional 3D MRCP (C-3D MRCP) in children with pancreatitis., Methods: A total of 24 children (2-17 y) diagnosed with pancreatitis were included in this study. The children underwent CS-3D MRCP and C-3D MRCP sequences. C-3D MRCP and CS-3D MRCP images were evaluated for the acquisition time duration, visualization of the pancreaticobiliary ducts, background suppression, image quality degradation by artifacts, and overall image quality by the two radiologists independently. Paired sample t-test was used to compare the acquisition time, the McNemar test for the image quality features, and the kappa coefficient was used for interobserver agreement., Results: A two-fold decrease in the acquisition time of CS-3D MRCP (~148 ± 61 s) was seen, compared to C-3D MRCP (~310 ± 98 s), p < 0.001. The median scores for overall image quality on CS-3D MRCP and C-3D MRCP, respectively, were 2.05 ± 0.52 and 2.21 ± 0.53 (p = 0.18) for both radiologists. No significant difference was seen for the visibility of ducts, background suppression, and artifacts between the two radiologists, with substantial to almost perfect agreement seen for the different findings., Conclusion: The application of compressed SENSE 3D MRCP in children with pancreatitis results in a two-fold reduction in acquisition time with acceptable image quality. This may help in reducing the need for long sedation in children requiring anesthesia support for the MRCP and potentially help in reducing motion artifacts., (© 2022. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation.)

Background: GCSF may improve the prognosis of severe liver disease by promoting liver regeneration and immune restoration. Our Aim was to investigate its controversial efficacy in decompensated cirrhosis, acute alcoholic hepatitis (AAH), or acute-on-chronic liver failure (ACLF) through meta-analysis., Methods: Meta-analysis of proportions (random effect model) including 19 RCTs (1287 patients from 16 Asian and 3 European studies including 487 ACLF, 231 AAH and 569 cirrhotic patients) evaluating survival at day-28, day-90, 6 months, one year, and/or occurrence of sepsis as major outcomes., Results: In patients with decompensated cirrhosis, G-CSF administration was associated with a reduction in the weight-adjusted risk of mortality of 9% at day-90 (OR=0.33; 95%CI: 0.18-0.58; p = 0.0002), 16% at 6 months (OR=0.31; 95%CI: 0.15-0.62; p = 0.0009), 26% at one year (OR=0.21; 95%CI:0.12-0.38, p<0.0001) and a weight-adjusted 28% risk reduction for sepsis (OR=0.28; 95%CI: 0.16-0.49; p<0.0001). Only Asian studies were positive. In AAH, G-CSF was associated with an 18% reduction in weight-adjusted mortality risk at day-28 (OR=0.31; 95%CI:0.11-0.83, p = 0.021), 32% at day-90 (OR=0.20; 95%CI:0.09-0.46, p<0.0001) and a weight-adjusted 42% risk reduction for sepsis (OR=0.17; 95%CI: 0.08-0.38; p<0.0001). Only Asian studies, in which corticosteroids were not given systematically in case of severe AAH, were positive. In patients with ACLF, the results on mortality at day-28 were heterogeneous, and GCSF had no beneficial effect on sepsis or survival at day-90., Conclusion: G-CSF may be effective in patients with decompensated cirrhosis or AAH by reducing the occurrence of sepsis and mortality. Further meta-analyses of individual data, or new, powerful and methodologically flawless therapeutic trials, are warranted to confirm these results, which harbor wide divergences between Asian and European RCTs., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023. Published by Elsevier Masson SAS.)

Background: Celiac disease (CD) is frequently associated with type I diabetes mellitus (T1D), where its diagnosis may be a challenging task. This study aims to test the usefulness of the double staining immunofluorescence (dsIF) technique for the detection of intestinal anti-tissue transglutaminase specific IgA antibody (tTG-IgA) deposits in CD and T1D children with coexisting CD., Methods: A total of 46 patients (30 cases of CD and 16 cases of T1D with CD) and 16 non-diabetic, non-celiac children were recruited. Endoscopic biopsies were taken and analyzed by light microscopy, quantitative histology (QH), and a dsIF technique., Results: Histologically, villous atrophy was most severe in CD, followed by T1D with CD, while all control biopsies except 1 were normal. QH showed a statistically significant difference in villous height (Vh), crypt depth (CrD), and Vh:CrD ratio between diabetic and non-diabetic patients with CD. dsIF technique could detect tTG-IgA deposits in 85.7% of cases of CD alone and 93.8% of biopsies from diabetic children. Surprisingly, deposits were more extensive in biopsies with minimal villous shortening. Also, all 5 biopsies from T1D patients with normal histology were dsIF positive., Conclusion: In-situ analysis of tTG-IgA immune deposits facilitates the detection of positive serology early-onset CD. Quantitative analysis may be used as an ancillary tool to increase the reliability of histological findings in these patients., Competing Interests: Declaration of competing interest None of the authors has any conflict of interest to disclose., (Copyright © 2023 Taiwan Pediatric Association. Published by Elsevier B.V. All rights reserved.)

Background: Kasai portoenterostomy (KPE) is the initial treatment for biliary atresia (BA). Even after initial jaundice clearance, a significant number of children presented with the reappearance of symptoms due to ongoing fibrosis involving porta and intrahepatic ducts. Mitomycin-C (MMC) is an antifibrotic agent, and the study hypothesized that local application of MMC at porta can decrease fibrosis, which can improve jaundice clearance and lead to better native liver survival (NLS)., Materials and Methods: This prospective randomized control trial included children with BA, who were allocated to groups A or B. The patients in both groups underwent standard KPE; in addition, a 5 French infant feeding tube (IFT) was placed near the porta through the Roux limb in Group B children. During the postoperative period, MMC was locally instilled over the porta in Group B children through IFT. Postoperative jaundice clearance and NLS were assessed and compared., Results: A total of 27 children were enrolled in the study, 16 in Group A and 11 in Group B. Both groups were comparable preoperatively. Although the NLS was not statistically significant in Group B, the survival was quite higher, that was 91%, 81%, and 73% at 6 months, 1 year, and 2 years, respectively, compared to 63%, 50%, and 38% in Group A., Conclusion: Children in Group B clinically showed an early jaundice clearance and a better trend of serial bilirubin levels as well as longer NLS than Group A, but it was not statistically significant. The procedure was technically easy, and no complication was encountered related to surgical technique or MMC instillation., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Journal of Indian Association of Pediatric Surgeons.)

Background: A strict lifelong gluten-free diet (GFD) remains the only treatment of celiac disease (CD). Adherence to gluten-free diet is best reflected by mucosal healing. Noninvasive tools capable of predicting mucosal recovery in CD patients need to be identified., Aims: To compare the ability of various modalities used to assess compliance to GFD, for predicting persistent mucosal damage in children with CD., Methods: A prospective, single-center, observational study on children with CD on a GFD was conducted between January 2020 and April 2021. Children with CD on GFD were consecutively enrolled and various modalities used to assess adherence to GFD were compared., Results: One hundred and fifty children (Mean age 12.2 ± 3.6 years, 58% Boys) on GFD (Mean duration 6 ± 3.1 years) were enrolled in the study. Persistent mucosal damage was seen in 88% of the enrolled. Fecal gluten immunogenic peptide (GIP) was positive in 87.8% (129/147). Antibodies to tissue transglutaminase (TGA-IgA) and/or deamidated gliadin peptide (DGP) were positive in 32% (48/150) whereas antibody to synthetic neoepitopes of TGA-IgA was positive in 24.8% (37/149). Non-compliance as assessed by local questionnaire, Biagi score, and dietitian detailed interview were 62.7%, 60%, and 75.3%, respectively. Serology had the highest specificity (83%) and fecal GIP had the highest sensitivity (89%). On logistic regression analysis, only non-compliance by Biagi score predicted poor mucosal recovery., Conclusion: Fecal GIP may be sensitive to detect only "one-point dietary transgression." None of the existing modalities used to assess compliance to GFD accurately predict persistent mucosal damage. A subset of patients may develop gluten tolerance., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: Pediatric irritable bowel syndrome (IBS) is a common functional gastrointestinal disorder with variable response to various therapeutic agents. Psyllium has been proven to be effective in adults; however, there is no study in children. The objective of this study is to evaluate the efficacy of psyllium husk as compared to placebo in pediatric IBS patients., Methods: In this double-blind randomized controlled trial, 43 children were assigned to psyllium arm (Group A) and 38 into placebo arm (Group B). Severity is assessed at baseline and after 4 weeks of treatment using IBS severity scoring scale (IBS-SSS) and classified into mild, moderate, and severe categories. Categorical data was compared with chi-square test and paired categorical variable was compared with McNemer test., Results: Mean ages (±SD; in years) of Groups A and B were 9.87 (2.7) and 9.82 (3.17), respectively, with median duration of illness of 12 months. At baseline, type, severity, and parameters (IBS-SSS) of IBS were equally distributed in 2 groups. There was a significant reduction in median interquartile range (IQR) of total IBS-SSS in psyllium versus placebo [75 (42.5-140) vs 225 (185-270); P < 0.001] at 4 weeks. Similarly 43.9% in Group A versus 9.7% in Group B attained remission [IBS-SSS < 75 ( P < 0.0001)]. The mean difference in IBS-SSS between Group A and Group B was -122.85 with risk ratio of 0.64 (95% CI; 0.42-0.83; P = 0.001) and absolute risk reduction of 32% (NNT = 3)., Conclusions: Psyllium husk is effective for the therapy of pediatric IBS when compared with placebo in short term., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background & Objectives: Celiac disease (CD) is a genetic immune mediated disorder characterised by gluten intolerance. This single centre study, from north India was aimed to assess the clinical, serological and histological profile of CD in a large cohort of children and the changing trends in its presentation., Methods: A review of clinical details of CD children diagnosed between 2000 and 2019 and currently on follow up was performed. Information on demography, symptoms, associated conditions, serology, biopsy findings and gluten-free diet were analyzed., Results: The mean age (±standard deviation) of 891 children included in the study, at onset and at diagnosis was 4.0±2.7 and 6.2±3.1 yr, respectively. Growth faltering, abdominal pain, abdominal distension and diarrhoea were presenting symptoms in 70, 64.2, 61.2 and 58.2 per cent, respectively. A positive family history of CD was present in 14 per cent and autoimmune conditions in 12.3 per cent of children. Thyroid disorders were seen in 8.5 per cent of children and Type 1 diabetes mellitus (T1DM) in 5.7 per cent. The duration of breastfeeding had a weak positive correlation with age at onset and diagnosis of CD (P<0.001). Non-classical CD was significantly more common in children aged >10 yr and in those presenting after 2010 (P<0.01). T1DM and hypothyroidism occurred more frequently in non-compliant children., Interpretation & Conclusions: This was the largest single centre study, pertaining to the presentation and follow up of CD in children. Infants and young children were more likely to present with classical symptoms of diarrhoea, abdominal distension and growth failure while older children presented with non-classical CD. There was a trend towards non-classical forms of CD in recent years.

Background: The functional and morphological recovery following an episode of acute pancreatitis (AP) in children still remains ill understood as research exploring this is limited. We aimed to characterize the morphological and functional changes in pancreas following AP and ARP (acute recurrent pancreatitis) in children., Methods: Children with AP were followed prospectively and assessed at two time points at least 3 months apart, with the first assessment at least 3 months after the AP episode. Exocrine and endocrine functions were measured using fecal elastase and fasting blood sugar/HbA1c levels respectively. Morphological assessment was done using endoscopic ultrasound (EUS) and magnetic resonance imaging and cholangiopancreatography (MRI/MRCP)., Results: Seventy-three children (boys:59%; mean age:8.4 ± 3.2years) were studied and 21 of them (29%) progressed to ARP. Altered glucose homeostasis was seen in 19 (26%) at first and 16 (22%) at second assessment and it was significantly more in ARP group than the AP group at first (42.8%vs19.2%; p = 0.03) as well as second assessment (38.1%vs15.3%; p = 0.03). Twenty-one children (28.7%) at first and 24 (32.8%) at second assessment developed biochemical exocrine pancreatic insufficiency. EUS detected indeterminate and suggestive changes of chronic pancreatitis in 21% at first (n = 38) and 27.6% at second assessment (n = 58). On MRCP, main pancreatic duct and side branch dilatation were seen in 15 (20.5%) and 2 (2.7%) children respectively., Conclusions: More than one-quarter of children have evidence of altered glucose homeostasis and biochemical exocrine pancreatic insufficiency following an episode of AP. Similarly, morphological features of chronicity seen in some of the children suggest that a fraction of subjects may develop chronic pancreatitis on longer follow-up., Competing Interests: Declaration of competing interest None., (Copyright © 2022 IAP and EPC. Published by Elsevier B.V. All rights reserved.)

Purpose: To evaluate the shear wave elastography (SWE) values of the spleen in healthy children using Elastography Point Quantification (ElastPQ)., Methods: In this IRB approved prospective study, spleen stiffness was measured in 146 healthy children (2-15 years) using ElastPQ. SWE values were recorded in upper pole, mid pole, and lower pole of the spleen in all the children., Results: The mean (± SD) SWE values of the spleen in children in ≤ 5 years age group, > 5-10 years age group, and > 10-15 years age group were 5.6 (± 4.2) kPa, 6.5 (± 3.2) kPa, and 5.9 (± 3.6) kPa, respectively. No statistically significant difference was seen in SWE values of the spleen between these three groups (p > 0.5). The mean (± SD) SWE values in ≤ 10 years and > 10 years age group were 6.1 (± 3.6) kPa and 5.9 (± 2.6) kPa, respectively. No statistically significant difference was seen in SWE values of the spleen between these two groups (p > 0.5). There was no significant difference in the SWE values for boys and girls. Statistically significant difference was seen between the mean SWE values in the two groups based on the median splenic length, which was 5.5 (± 3.8) kPa in the group with a length of ≤ 7.6 cm and 6.7 (± 2.8) kPa in the group with a length of > 7.6 cm., Conclusion: SWE values of the spleen in healthy children do not correlate with age, and no significant difference is there in the SWE values for boys and girls. There was a statistically significant difference in the SWE values of the spleen while comparing the groups based on the median splenic length., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Portal cavernoma cholangiopathy (PCC) refers to abnormalities of the extra- and intrahepatic bile ducts in patients with portal cavernoma. The literature on PCC in children is very scarce. This study aimed at characterizing PCC in children with extrahepatic portal venous obstruction (EHPVO) using endoscopic ultrasound (EUS) and magnetic resonance cholangiography/portovenography (MRC/MRPV)., Methods: A total of 53 consecutive children diagnosed with EHPVO were prospectively evaluated for PCC using MRC/MRPV and EUS. Chandra classification was used for type of involvement and Llop classification for grading of severity., Results: All 53 children (100%) had PCC changes on MRC/EUS, but none were symptomatic. Extrahepatic ducts (EHDs) and intrahepatic ducts were involved in majority (85%), and 58.5% had severe changes. Periductal thickening/irregularity (71%) was the commonest change in intrahepatic ducts, whereas irregular contour of the duct with scalloping (68%); common bile duct (CBD) angulation (62.3%) were the frequent changes in the EHDs. Increased CBD angulation predisposed to CBD strictures ( P  = 0.004). Both left and right branches of portal vein were replaced by collaterals in all children. Among the EUS biliary changes, para-pericholedochal, intrapancreatic, and intramural gall bladder collaterals had significant association with severity, with higher frequency of occurrence in children with the most severe Llop Grade., Conclusions: PCC develops early in the disease course of EHPVO, in children, but is asymptomatic despite severe changes. EUS biliary changes are more likely to be observed with increasing severity of PCC., (© 2021 Indian National Association for Study of the Liver. Published by Elsevier B.V. All rights reserved.)

Competing Interests: Competing interests: None declared.

Purpose: To evaluate role of 3-T magnetic resonance portovenography (MRPV) in children with extra hepatic portal venous obstruction (EHPVO) and compare unenhanced and contrast-enhanced sequences., Methods: 20 Children with EHPVO underwent MRPV using unenhanced [T2, T1 pre-contrast, Balanced turbo field echo (BTFE)] and contrast-enhanced (CE) modified DIXON (mDIXON) sequences. The images were evaluated for the patency of abdominal veins, hepatic and splenic parenchyma by two radiologists for interobserver agreement., Results: BTFE and post-contrast mDIXON sequences performed best for evaluation of abdominal veins. Concordance between the BTFE and CE sequences was good to very good for both the radiologists for splenic vein (SV) evaluation. The concordance between the two sequences for evaluation of superior mesenteric vein and its confluence with the SV was less remarkable and varied from fair to good, while the interobserver agreement was very good to perfect. The interobserver agreement between the BTFE and CE sequence for the evaluation of left renal vein was very good, while that for IVC was perfect. The agreement between BTFE and CE sequence was perfect for the evaluation of right and middle hepatic veins, while the interobserver agreement was good to perfect. The interobserver agreement was poor for evaluation of hepatic parenchyma on BTFE sequence as compared to CE sequence, and moderate for splenic parenchyma., Conclusion: BTFE sequence is the single best unenhanced MR pulse sequence to detect all the vascular structures in children with EHPVO. CE-MRI is not superior to BTFE sequence and should be used at the discretion of the radiologist., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Introduction:  Minimal access surgical approach to choledochal cyst (CC) is becoming a standard of care in pediatric age group. Robotic-assisted excision of CC is increasingly being practiced at centers which have access to the system. We present our experience and technique of hepaticoduodenostomy (HD). Over all initial experience, short-term outcomes and complications are also presented and discussed., Materials and Methods:  Patients with CC and undergoing robotic excision were retrospectively studied. Patients with active cholangitis, liver dysfunction, and perforated CC were excluded for robotic procedures. All included patients were preoperatively evaluated as per the defined protocol. They underwent excision of CC with HD. The duodenal anastomosis was done after limited mobilization and emphasis was laid on anastomosing the distal D2 part to the common hepatic duct. This prevents bile reflux into stomach. The follow-up evaluation was done for these patients. Hepatobiliary iminodiacetic acid (HIDA) scan for duodenogastric reflux (DGR) was done only if patients reported symptoms related to it., Results:  A total of 19 patients (10 females) were studied. The mean age was 84 months. Type 1b was present in 12 patients and the rest were type IVb. Complete cyst excision with HD was done in all patients except conversion to open in one patient. The mean surgical time was 170 ± 40 minutes with console time of 140 ± 20 minutes. Median follow-up duration is 2.5 years (range: 3.5-0.5 years). HIDA scan was done in five patients who had reported epigastric pain. Of these five, one patient had a positive DGR. He is on conservative management., Conclusion:  Robot-assisted CC excision with HD is feasible as proven by the outcome of 19 patients presented in this series. HD is to be done away from pylorus in distal part of down curving D2. This particular step prevents DGR and is the most important point of technique in doing HD. The presented series is the first report of robotic excision of CC with HD. The robot is a facilitator for complex and difficult operations as CC excision and HD., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Liver abscess (LA), a common problem in children in the tropics, is believed to be mostly pyogenic (PLA), sometimes amebic (ALA). We aimed to analyze the clinical profile, etiology, risk factors for complications, management and outcomes of LA in children., Methods: The details of 81 children with LA managed in a tertiary set up over a period of 3 years were analyzed. A comparison of different parameters was performed with respect to etiology and complications., Results: ALA, PLA and mixed infection LA were diagnosed in 40 (49.4%), 32 (39.5%) and 9 (11.1%) children. The triad of fever, hepatomegaly and right upper quadrant tenderness was seen in 65 (80.2%). Coagulopathy was observed in 60 (77%) and jaundice in 12 (14.8%). Majority (71.6%) had a single LA in the right lobe (69%). Conservative, percutaneous needle aspiration, percutaneous catheter drainage and surgical drainage were done in 11.1%, 3.7%, 82.7% and 2.5%, respectively. Forty-three (53.1%) had complicated LA with rupture in 55.8% and vascular thrombosis in 16.2%. Children with complicated LA had higher alanine transaminase, prolonged prothrombin time/international normalized ratio, low serum protein and albumin levels (P < 0.05). Median duration of follow-up was 2 months and mean time to resolution of LA was 48.5 ± 18 days., Conclusions: ALA is the commonest cause of pediatric LA in endemic regions and is difficult to differentiate from PLA clinically. Percutaneous catheter drainage is safe and effective modality for the management of LA in children. A higher alanine transaminase, prolonged prothrombin time/international normalized ratio and low serum albumin levels (<3 g/dL) at presentation identify complicated LA., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Necrotizing enterocolitis is one of the most common and worrying diseases in neonates, commonly shown in premature neonates, and is associated with significant mortality and morbidity. Necrotizing enterocolitis is characterized by intestinal mucosal injury that can progress to transmural bowel necrosis, and radiologically it can present with either pneumatosis intestinalis or portal venous gas. It is postulated to develop in an immunocompromised host in the setting of bacterial colonization, usually after administration of non-breast milk feed. Cow's milk allergy association with necrotizing enterocolitis has not been well determined, and the pathophysiology is still not clear. Necrotizing enterocolitis is very rare following living donor liver transplant. In our case, a 6-year-old boy who was doing well in the postoperative period had sudden worsening of general condition after he was started on milk feed. On evaluation and reexploration, he was diagnosed with necrotizing enterocolitis and later succumbed to death.

The immunological response to hepatitis B virus (HBV) vaccine may be suboptimal in children with celiac disease (CD), but the reasons for this are not well defined., Objectives: This study was undertaken to assess the immune response to HBV vaccine in CD children and to explore the possible factors affecting the immune response., Methods: The study population consisted of 3 groups-50 newly diagnosed CD children (group 1), 50 previously diagnosed CD children who were on gluten free diet (GFD) >3 months (group 2), and 100 age and gender matched healthy controls (group 3). The patient characteristics were recorded, and the blood samples were analyzed for HBsAg and anti-HBs titers. The nonresponders were given a booster dose of HBV vaccine and reevaluated after 6 weeks., Results: Positive anti-HBs response was found in 46% in newly diagnosed CD children, 60% in CD children on GFD, and 83% in healthy controls ( P  < 0.001). The immune response to HBV vaccine in CD children was inferior to that in healthy children (53% vs 83%, P  < 0.001). The immune response was found to be significantly affected by age at diagnosis, delay in diagnosis, type of presentation, and compliance to GFD. 44 out of 45 (97.77%) nonresponders from CD group seroconverted after a single booster dose., Conclusion: Early diagnosis and good compliance to GFD may improve the immune response to HBV vaccine in CD children. Single additional booster dose is sufficient to attain optimal immune response., Competing Interests: The author has no funding and conflicts of interest to report., (Copyright © 2021 The Author(s). Published by Wolters Kluwer on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Competing Interests: Conflict of Interest: None

Background: Gallbladder (GB) hypomotility has been reported in adults with celiac disease (CD), but there is no literature on GB dysfunction in children with CD. We aimed to study GB motility in children with CD, before and after a gluten-free diet (GFD), using ultrasonography (USG) and technetium-99 labeled mebrofenin hepatobiliary scintigraphy (HBS)., Methods: Children with newly diagnosed CD were enrolled and evaluated for GB ejection fraction (GBEF) using HBS and USG. Those with reduced GBEF on initial HBS were again evaluated after 6 months of strict GFD and the results were compared., Results: Of the 50 children with CD (mean age 9±2.1 years, 54% boys), 16% (n=8) had a low GBEF at baseline (19±13%). These children had a significantly greater delay in diagnosis compared to those with normal GBEF (6.5±2.0 vs. 2.3±1.2 years, P<0.001). A significant improvement in GBEF was noted on HBS post GFD (74±12%1 vs. 9±13%, P<0.001). GBEF also improved significantly as assessed by USG parameters after GFD (P<0.001). The fasting GB volume decreased (5.7±1.4 vs. 10.2±2.6, P=0.002), with a significant improvement in percentage postprandial GB volume change (52±10.5% vs. 24±16.5%, P=0.007) compared to baseline pre-GFD values. Orocecal transit time was also increased in children with reduced GBEF., Conclusions: GB function is impaired in at least 16% of children with CD at diagnosis and is reversible with GFD. GB dysfunction is significantly associated with a delayed diagnosis and may be a part of general gastrointestinal dysmotility., Competing Interests: Conflict of interest: None, (Copyright: © 2021 Hellenic Society of Gastroenterology.)

Background: Pancreatic ascites (PA) and pleural effusion (PPE) are rarely encountered in children. They develop due to disruption of the pancreatic duct (PD) or leakage from an associated pancreatic fluid collection (PFC). The literature on childhood PA/PPE and its management is scarce., Methods: A retrospective review of children with PA/PPE diagnosed and managed at our center over the last 4 years was performed. The clinical, biochemical, radiological and management profiles were analyzed. Conservative management included nil per oral, octreotide and drainage using either percutaneous catheter or repeated paracentesis. Endotherapy included endoscopic retrograde cholangiopancreatography (ERCP) and transpapillary stenting., Results: Of the 214 children with pancreatitis, 15 (7%) had PA/PPE. Median age was 9 years with a third under 2 years. Median ascitic fluid amylase was 8840 U/L and all had elevated protein (>2.5 g/dl) and low serum ascites-albumin gradient ascites (<1.1). While PA/PPE was the first manifestation of underlying chronic pancreatitis (CP) in 10 children (67%), trauma was seen in 4 (26%) and hypertriglyceridemia in 1 (7%). On imaging, PD disruption could be identified in 10 (67%) children. ERCP and stenting was done in 10 children. Conservative management alone (n = 4) and endotherapy (n = 10) was successful in 93% with only one requiring surgery. The younger children (n = 4), were managed conservatively and only 1 of them required surgery. Resolution of PA/PPE was achieved in all with no recurrences., Conclusions: Conservative management and ERCP plus transpapillary stenting results in resolution of majority of pediatric PA/PPE. Children presenting with PA/PPE needs to be evaluated for CP., Competing Interests: Declaration of competing interest None., (Copyright © 2020 IAP and EPC. Published by Elsevier B.V. All rights reserved.)

Bilateral renal cortical necrosis (RCN) as a cause of acute kidney injury is very rare in the pediatric population. Progression to end-stage renal disease is seen virtually in every patient with RCN. There are many causes for the occurrence of cortical necrosis in children, with severe pancreatitis being a rarity. In this report, we describe a child with severe acute pancreatitis complicated by bilateral RCN.

Background: Noninvasive tools (NITs) for predicting varices in children with portal hypertension (PHTN) are infrequently used., Methods: Eighty-five consecutive, treatment-naïve children with PHTN and 97 controls were enrolled study from July 2017 to November 2018. Each case was evaluated by esophagogastroduodenoscopy (EGD) and various NITs: platelet spleen size Z (PSZ), clinical prediction rule (CPR), King's variceal prediction rule (KVaPS), Splenic stiffness (SSM) and liver stiffness measurement (LSM) by point shear wave elastrography (pSWE)., Results: Had PHTN due to extra hepatic portal vein obstruction (EHPVO) and 70% due to cirrhosis [chronic liver disease (CLD)]. Sixty-five percent of PHTN cases had varices. Children with varices had lower platelet counts, lower albumin and larger spleens. SSM and LSM were significantly higher in cases as compared with controls. SSM was significantly higher in cases with varices than those without. SSM and LSM, at cutoffs of 3.8 and 3.2 kPa, respectively, discriminated PHTN cases from controls with an area under the curve (AUROC) of 0.67 (0.59-0.74). Both SSM and LSM predicted varices in CLD, but in EHPVO, only SSM predicted varices. SSM of 5.2 and 12.8 kPa, in CLD and EHPVO subgroups, respectively, had AUROC of 0.73 and 0.94 for variceal prediction. Blood-based NITs performed better in the CLD subgroup: aspartate aminotransferase platelet ratio index, CPR and KVPS predicted severity of PHTN with AUROC of 0.81, 0.92 and 0.93, respectively., Conclusions: Blood-based NITs outperform elastography for prediction of PHTN/varices in children with CLD. SSM by pSWE is a better predictor of varices than LSM, especially in the EHPVO subgroup.

Eosinophilic ascites, owing to serosal involvement, is a very rare manifestation of eosinophilic gastroenteritis in children, especially when it occurs with muscular involvement in the absence of mucosal disease, which may be confirmed by endoscopic ultrasonography. An 11-year-old girl, presenting with massive eosinophilic ascites and colicky abdominal pain with peripheral eosinophilia, raised IgE levels and positive skin prick test, had such investigation which confirmed the presence of muscle layer thickening of both stomach and small bowel. She responded well to steroids and montelukast.

Background & Aims: Acute Pancreatitis (AP) tends to have a benign course in children. However there is a paucity of information with respect to severity of AP in children, the categorization of collections viz., walled off necrosis (WON)& pseudocyst and their natural history & outcomes., Methods: A retrospective medical record review of 187 children with pancreatitis diagnosed and managed at our centre was performed., Results: 101 children (59% boys, Median age 9yrs) had AP of which 37.6%, 60.4% and 2% had mild, moderately severe and severe AP. 61.4%(62) had PFC at diagnosis; 34%(21) acute pancreatic fluid collections (APFC) and 66%(41) acute necrotic collections (ANC). 52.3%(11of21) of APFC evolved into pseudocysts & 68.2%(28of41) of ANC into WON's. Drainage was required in 31%(12of39) of persisting collections, more frequently in children with traumatic AP. Percutaneous catheter drainage (PCD) was done in 6 children and endoscopic ultrasound (EUS) guided cystogastrostomy with placement of plastic or self expanding metal stents (SEMS) in 6 children., Conclusions: Moderately severe AP is common in hospitalized children with AP with PFC developing in 61.4%, majority being APFC. 48% of APFC and 32% of ANC will resolve and the rest evolve into pseudocyst or WON. Spontaneous resolution is more likely in children with non -traumatic AP having pseudocysts rather than WON's., Competing Interests: Declaration of competing interest None., (Copyright © 2020 IAP and EPC. Published by Elsevier B.V. All rights reserved.)

Background: Acute-on-chronic liver failure (ACLF) results in very high mortality in children. We aimed to evaluate the role of granulocyte colony-stimulating factor (GCSF) on short-term outcome of children with ACLF in a nontransplant unit., Methods: Children (aged > 1 year) diagnosed with ACLF over a 15 month period were randomised. Group A was given GCSF therapy along with standard medical care (SMC - details in supplementary data) and group B was given only SMC. The outcome was evaluated as survival at 30 and 60 days of therapy., Result: Thirty-one children with ACLF were enrolled, with a mean age of 6.92 ± 4.3yrs. A total of 15 patients were randomised to group A and 16 to group B. The overall mortality was 54.83%. The intervention group showed survival rates of 80%, 66.67% and 53.3%, whereas the control group had survival rates of 43.75%, 37.5% and 37.5% at 14, 30 and 60 days, respectively. A significant survival benefit was noted on day 14 (p = 0.043) of therapy in group A with significant difference in Child-Turcotte-Pugh (CTP) and pediatric end-stage liver disease (PELD) scores in the two groups. After an initial rise in group A, the granulocyte counts fell to become comparable in the two groups by day 30 and 60, indicating that the effect of GCSF therapy wears off over time. There was no significant difference in the overall survival, median/mean CTP, PELD and MCS (Modified Cliff sequential organ failure assesment (SOFA)) scores on day 30 and 60. Mean (%) CD 34 + cells level showed a rise on day 7 in group A but was statistically insignificant., Conclusion: The present study shows that GCSF therapy at 5 mcg/kg/day for 5 days seems to be ineffective in improving the survival outcome on day 30 and 60 of therapy. Studies with larger number of children enrolled and longer duration of therapy are required. (CTRI/2017/11/010420)., Competing Interests: The authors have none to declare., (© 2019 Indian National Association for Study of the Liver. Published by Elsevier B.V. All rights reserved.)

We discuss the case of a two-month-old girl admitted with complaints of progressive pallor, generalized body swelling and pale colored stool since the neonatal period. On examination, severe pallor, chubby cheeks and moderate hepatomegaly were noted. Investigations revealed isolated anemia, transaminitis, conjugated hyperbilirubinemia, prolonged prothrombin time and hyperlipidemia. She died due to severe sepsis, shock, and pulmonary hemorrhage. An autopsy revealed characteristic histopathology findings of cystic fibrosis in the liver, lungs, and pancreas. Genetic analysis performed on autopsy tissue was positive for F508del compound heterozygous (WT/F508del) mutation, confirming the diagnosis of cystic fibrosis.

We encountered an eight-year-old boy who was subsequently diagnosed with a retroperitoneal ganglioneuroma. In view of the rarity of this tumour and its presentation, we are prompted to report this case.

Celiac disease (CD) is known to be associated with several autoimmune disorders. We studied the prevalence of subclinical CD among patients with immune thrombocytopenia (ITP) as compared to general population. Cases of primary ITP between the age group of 18-60 years were studied. Besides clinical examination, all patients underwent serology testing for tissue transglutaminase antibody (tTG) IgA and anti-endomysial antibodies IgA. The diagnosis of CD was made if both antibodies were positive. Healthy subjects acted as controls and underwent serological testing for tTG IgA. Seventy-nine primary ITP and 316 healthy subjects underwent serology testing for CD. Four patients of primary ITP (4/79) were positive for both serology as compared to 2 (2/316) healthy controls [odds ratio 8.37 (CI 1.50-46.47, p  < 0.005)]. Among the ITP cases only one had clinical symptoms of CD while none of the healthy controls had symptoms of CD. There is a significantly higher prevalence of subclinical CD in patients with ITP. Since the prevalence of CD is known to vary among different geographical zones, we suggest further studies on screening of ITP patients for CD in areas of high prevalence., Competing Interests: Conflict of interestAll authors declare no conflict of interest in writing this manuscript., (© Indian Society of Hematology and Blood Transfusion 2019.)

Background and Aim: Abdominal tuberculosis (ATB) in children poses a diagnostic challenge because of its nonspecific clinical features, which often delay the diagnosis. Our aim was to present our real-world experience and provide an insight into the presentation, pattern of distribution, and diagnosis of the disease., Methods: A retrospective review was conducted of case records of all children ≤12 years of age diagnosed with ATB from January 2007 to January 2018. Clinical details and investigations were recorded and analyzed., Results: A total of 218 children (110 boys), with a median age of 10 (0.25-12) years, were included. There was a median delay of 4 (0.5-36) months in establishing the diagnosis. Abdominal pain, fever, and loss of weight were the most common presenting features, with the triad of symptoms present in 54%. Multiple intra-abdominal sites were involved in 118 (54%) patients, with a combination of the gastrointestinal tract (I) and abdominal lymph nodes (L) being the most common (53/118). Among children with single-site involvement ( n = 100), the most commonly involved was L in 39 (39%), followed by I in 35(35%). Loss of weight was more common in children with involvement of multiple sites (85/118 vs 60/100, P = 0.03). Overall, a confirmed diagnosis was possible in 94 participants (43.1%). Suggestive imaging had the highest diagnostic yield of 85%. Nine (4.1%) patients needed surgical management., Conclusion: A triad of abdominal pain, fever, and weight loss is suggestive of ATB. Multiple intra-abdominal sites are frequently involved. Microbiological confirmation is possible in only one-third of the cases., (© 2019 The Authors. JGH Open: An open access journal of gastroenterology and hepatology published by Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.)

Paediatric chylous ascites in tropics is commonly caused by infections and trauma. We describe the clinical characteristics of an uncommon inherited cause of chylous ascites, Hennekam syndrome, treated by nutritional modification., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Primary lymph node gastrinoma has been defined as gastrin-producing tumor present in lymph nodes and predominantly found in well-defined anatomical region known as gastrinoma triangle. They are usually localized preoperatively with imaging, and their surgical resection results in long-term relief. The authors report a case of unresectable primary lymph nodal gastrinoma with liver metastases in a 14-year-old adolescent boy with proven histopathology detected on Ga-DOTANOC whole-body PET/CT scan followed by preoperative multiple Lu-DOTATATE cycles for cytoreduction. Subsequent surgical resection of residual mass resulted in complete response with a follow-up of around 4 years in this unusual case of Zollinger-Ellison syndrome.

Background and Aim: There is lack of data on functional and morphological recovery after an attack of acute pancreatitis (AP) or acute recurrent pancreatitis (ARP) in children. This study aims to evaluate the functional impairment and morphological changes in the pancreas after recovery., Methods: All consecutive patients presenting with AP (n = 61) or ARP (n = 35), as per standard diagnostic criteria, were enrolled. After 2 months of pancreatitis, fecal elastase-1 (FE-1) (μg/g) and 2-h oral glucose tolerance test to calculate oral disposition index (DI o ) (mmol/L) (β-cell function) were performed. Morphological changes were assessed by endoscopic ultrasound and transabdominal ultrasound. Patients with chronic pancreatitis (CP) (n = 27) and healthy children (HC) (n = 26) were included as controls for functional parameters., Results: At a median follow up of 12 (4-44) and 11 (2-108) months, 66.7% and 75.9% (P = 0.57) of AP and ARP demonstrated exocrine insufficiency (FE-1 < 200), respectively. Mean (SD) FE-1 was 183.64 ± 150.94 (AP), 135.70 ± 103.80 (ARP), 46.56 ± 30.20 (CP), and 240.00 ± 181.83 (HC) (P < 0.001; anova) (AP vs CP, ARP vs CP, and CP vs HC; P < 0.001). Prediabetes due to insulin resistance was seen in 16.6% and 22.6% (P = 0.56) of AP and ARP. Median (interquartile range) DI o (mmol/L) was comparable between AP (4.20 [2.36, 8.3]) and HC (5.20 [2.89, 8.68]), but was low in ARP (2.97 [1.80, 5.12]), which was comparable with CP (1.91 [1.20, 2.83]). Endoscopic ultrasound demonstrated morphological changes in 25% and 37% (P = 0.34) of AP and ARP, respectively., Conclusion: There was high frequency of biochemical evidence of exocrine insufficiency. β-Cell function (DI o ) was preserved among AP but was poor in ARP. Nearly one-third showed morphological changes in imaging., (© 2018 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.)

Bertiella is a common parasite seen in non-human primates. It is rarely seen in humans. We present the case of a 2-year-old child with bertiellosis. He had recurrent abdominal pain, and worm fragments were found in stool, which were refractory to albendazole therapy.

The study was carried out to study expression of Toll like receptors 2 (TLR2), natural/inducible Treg and Interferon-γ alongside oxidative stress and understand their significance in pediatric samples. Influence of oxidative stress on Celiac Disease was analysed by evaluating lipid peroxidation, reduced glutathione, Glutathione peroxidase, etc. A comparison was performed among CD patients, CD patients on gluten free diet (GFD), and healthy controls. Peripheral nTregs exhibited a similar pattern of reduced numbers in CD and GFD cases when compared to healthy controls. On the other hand, inducible Tregs were much lower in GFD patients as compared to CD patients. Expression of TLR2 on iTregs was elevated in CD and GFD, however, expression on nTregs was unaltered in all the three groups. The inflammatory cytokine IFN-γ positive Treg cells were found to be elevated in CD as compared to control group. Oxidative stress was elevated in CD as compared to healthy controls while that in GFD samples was lower in comparison to CD. The levels of LPO, activities of enzymes SOD and Catalase were higher in CD and GFD samples when compared to controls. However, enzyme Glutathione peroxidase and reduced glutathione levels declined in both CD and GFD groups as compared to controls. This report highlights the effect of elevated oxidative stress in CD on reduced traffic of iTregs toward periphery. A strong correlation was observed between the cytokine IFN-γ and TLR2 expression in movement of iTregs in CD patients., (© 2017 APMIS. Published by John Wiley & Sons Ltd.)

Background: Liver abscess is a common deep seated abscess in children; amebic liver abscess is associated with more local complications., Case Characteristics: We report two preschool children presenting with short history of pain, fever and right upper quadrant pain. The abscess communicated with gastro-intestinal tract (ascending colon in case 1 and duodenum in case 2), and diagnosis of amebic liver abscess was confirmed by DNA PCR., Outcome: Both children were successfully managed with intravenous antibiotics and catheter drainage., Message: Gastrointestinal fistulization may be rarely seen in amebic liver abscess. Conservative management with antibiotics, catheter drainage and supportive care may suffice.