Annals of Pediatric Gastroenterology and Hepatology ISPGHAN
Volume 6 | Issue 1 | Year 2024

Publications by the Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition Members in PubMed-indexed Journals (1st December 2023 to 29th February 2024)

Arghya Samanta

Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Corresponding Author: Arghya Samanta, Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India, Phone: +91 9818847696, e-mail:

How to cite this article Samanta A. Publications by the Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition Members in PubMed-indexed Journals (1st December 2023 to 29th February 2024). Ann Pediatr Gastroenterol Hepatol 2024;6(1):5–6.

Source of support: Nil

Conflict of interest: Dr Arghya Samanta is associated as the Associate Editorial Board member of this journal.

Samanta A, Srivastava A, Yadav R, et al. Budd-Chiari syndrome in children: radiological intervention and role of shear wave elastography in monitoring response. J Pediatr Gastroenterol Nutr 2024;78(1):17–26.

In this ambispective single-center study, the authors evaluated the comparative long-term outcome of children with chronic Budd–Chiari syndrome (BCS), with and without radiological interventions (RI). They also assessed the role of liver and splenic stiffness measurement (LSM, SSM) by two-dimensional shear wave elastography (2D-SWE) in assessing response. A total of 60 children [40 boys, median age 10.5 (6.5–15.25) years] with BCS (29 newly diagnosed, 31 follow-up) were evaluated. The majority (78%) had isolated hepatic vein block. A total of 44 (73%) cases underwent RI, while 16 (27%) were managed conservatively. Post-RI, patients showed significant improvement clinically and biochemically, as well as features of portal hypertension, while those managed conservatively did not improve. LSM [33 (32–34.5) to 19.2 (18–20.67) kPa] and SSM [54.5 (52.3–57.6) to 28.9 (27.6–30.25) kPa] showed a significant decline from baseline value over a follow-up of 12 (6–13) months. The gradual reduction was seen in the LSM and SSM over 1–5 years, with near-normal LSM [10.2 (9.2–11.5) kPa] and SSM [22.3 (20.5–24.3) kPa] values in patients (n = 16) with > 5 years follow-up. A total of four children developed hepatopulmonary syndrome in follow-up; interestingly, two of them even after undergoing successful RI. The authors concluded that RI leads to clinical recovery and reduction with near-normalization of LSM and SSM over long-term follow-up(> 5 years) in children with BCS. 2D-SWE is a promising tool to monitor outcomes.

McLin VA, Franchi-Abella S, Heaton N, et al. Expert management of congenital portosystemic shunts and their complications. JHEP Rep 2023;6(1):100933. DOI: 10.1016/j.jhepr.2023.100933

Congenital portosystemic shunts (CPSS) are often associated with systemic complications, such as hepatic nodules, pulmonary hypertension, endocrine abnormalities, and neurocognitive dysfunction. A panel of international experts drafted their recommendations based on their review of the literature, presentations, and discussions. The key recommendations are as follows:

Hansen BE, Vandriel SM, Vig P, et al. Event-free survival of maralixibat-treated patients with Alagille syndrome compared to a real-world cohort from GALA. Hepatology 2023. DOI: 10.1097/HEP.0000000000000727

This international, multi-centric study compared the 6-year outcomes of patients with Alagille syndrome treated with maralixibat (n = 84) vs patients from the natural history cohort from the Global ALagille Alliance (GALA) study (n = 1438). Age, total bilirubin, γ-glutamyl transferase, and alanine aminotransferase were comparable between groups with no statistical differences. Event-free survival (EFS) in the maralixibat cohort was significantly better than the GALA cohort (hazard ratio 0.305; 95% CI, 0.189–0.491; p < 0.0001). Results from this comparison study suggest that maralixibat improves EFS in patients with Alagille syndrome.

Samanta A, Sarma MS, Jain M, Srivastava A, Poddar U. Ulcerated polypoidal lesion in rectum: not always a malignancy. Gastrointest Endosc 2024;99(2):300–301. DOI: 10.1016/j.gie.2023.07.036

In this case report, the authors describe the case of a 10-year-old boy who had an ulcerated polypoidal lesion at the rectum, which resembled a malignant lesion. Computed tomography imaging of the abdomen showed an asymmetric cystic lesion without tissue invasion or regional lymph node involvement. Histopathology confirmed the diagnosis, showing cysts in the rectal submucosa without mucosal layer involvement. The lesion healed completely with steroid enema over the next 1 year. This case is an excellent example of the importance of keeping a broad differential diagnosis when a colorectal tumor is encountered.

Sodhi KS, Maralakunte M, Bhatia A, et al. Utility of the new faster compressed SENSE MRCP at 3 Tesla MRI in children with pancreatitis. Indian J Pediatr 2023;90(12):1210–1215. DOI: 10.1007/s12098-022-04403-w

In this prospective study, the authors compared the diagnostic efficacy and image quality of the newer compressed SENSE three-dimensional (3D) magnetic resonance cholangiopancreatography (MRCP) (CS-3D MRCP) with conventional 3D MRCP (C-3D MRCP) in 24 children with pancreatitis. The recently developed compressed-sensing accelerated MRCP (CS-MRCP) has shown great potential in shortening the acquisition time, which may help in reducing the need for long sedation in children requiring anesthesia support for the MRCP and potentially help in reducing motion artifacts. In this study, the mean acquisition time of CS-3D MRCP was significantly lower as compared to C-3D MRCP (148 ± 61 s vs ~310 ± 98 s, p < 0.001), while the median scores for overall image quality were comparable. The authors concluded that the use of compressed SENSE 3D MRCP in children with pancreatitis results in a two-fold reduction in acquisition time with acceptable image quality.

Seetharaman J, Srivastava A, Yadav RR, et al. Visceral fat indices: do they help differentiate Crohn’s disease and intestinal tuberculosis in children? J Crohn’s Colitis 2023;17(12):2026–2032. DOI: 10.1093/ecco-jcc/jjad109

In this single-center retrospective study, the authors evaluated the utility of fat indices [visceral fat (VF) and subcutaneous fat (SF)] in differentiating Crohn’s disease (CD) and intestinal tuberculosis (ITB) in children. A total of 34 [age 14 years (10.8–17.0), 14 boys] children were recruited: 12 had CD (seven boys, age 13.0 years), and 22 had ITB (seven boys, age 14.5 years). VF area was higher in CD compared to ITB [18.34 cm2 [15.62–40.01] vs 6.48 cm2 (2.65–21.96); p = 0.012]. The SF and TF areas were similar in ITB and CD. The ratios of VF/SF [0.82 (0.57–1.5) vs 0.33 (0.16–0.48); p = 0.004] and VF/TF [0.45 (0.36–0.60) vs 0.25 (0.13–0.32); p =0.004] were significantly higher in CD. On comparing CD and ITB in boys and girls separately, the difference was significant for boys but not for girls. A VF/SF ratio of 0.609 predicted CD with good sensitivity (75%) and specificity (86.4%) (area under the curve 0.795, 95% confidence interval 0.636–0.955; p = 0.005). The authors concluded that the VF/SF ratio is a simple, noninvasive, objective parameter to differentiate CD and ITB in children, particularly boys. Larger studies are needed to validate this in girls.

Pamecha V, Patil NS, Falari S, et al. Live donor liver transplantation for pediatric acute liver failure: challenges and outcomes. Hepatol Int 2023;17(6):1570–1586. DOI: 10.1007/s12072-023-10571-4

In this study, the authors described the challenges and outcomes of 41 children undergoing live-donor liver transplantation (LDLT) for pediatric acute liver failure (PALF). PALF constituted 38% of all pediatric transplants during this duration. A large majority (75.6%) had grade 3/4 hepatic encephalopathy, and one-third required hemodynamic support with vasopressors. Post-LDLT 1- and 5-year patient and graft survival were 75.6 and 70.9%, respectively. The vascular and biliary complication rates were 2.4 and 4.8%, respectively. No graft loss occurred because of acute rejection. In multivariate analysis, pre-LT culture positivity and cerebral edema, the persistence of brain edema after transplantation, and resultant pulmonary complications were significantly associated with post-LT death. A total of 13 (32%) children who underwent plasmapheresis prior to LT had better post-LT neurological recovery, as evidenced by early extubation. The authors concluded that early referral and better selection can save more lives through timely transplantation.

Bolia R. Diagnosing appendicitis on the basis of clinical prediction rules: are we there yet? Indian J Pediatr 2023;90(12):1173–1174. DOI: 10.1007/s12098-023-04771-x

In this editorial, the authors discussed the role of the Pediatric Appendicitis Score (PAS) in diagnosing acute appendicitis. Recent literature suggests that PAS is a very useful tool for both ”ruling out” and ”ruling in” appendicitis. However, the presence of patients with a higher pretest probability and the presence of a more specialized evaluator in those studies could have inflated the yield of the score. However, no score on the PAS could completely eliminate the need for an ultrasound abdomen, which is a noninvasive and cost-effective imaging tool.

Vasudevan AK, Shanmugam N, Rammohan A, et al. Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis. Pediatr Transplant 2023;27(8):e14600.

In this prospective study, the authors described their experience of 60 children with progressive familial intrahepatic cholestasis (PFIC) type 1 (n = 13), 2 (n = 11), 3 (n = 31), 4 (n = 5) who underwent liver transplantation (LT) for each type of PFIC and compared their early, and long-term outcomes between 2011 and 2022. They compared PFIC 1–4 types with regard to early and long-term outcomes, including attainment of catch-up growth. There were no significant differences in gender, pediatric end-stage liver disease (PELD) scores, body mass index, type of grafts, cold and warm ischemia times, intraoperative blood loss, and morbidity among the groups. Catch-up growth was significantly poorer in the PFIC1 group (44.4 vs 88%, 90 and 100% p < 0.001). Overall, 1- and 5-year patient survival of the four PFIC groups (I–IV) was 69.2, 81.8, 96.8, and 100%, and 69.2, 81.8, 96.8, and 100%, respectively. Post-LT chronic diarrhea was observed in 6 (46.1%) children with PFIC-I, and 3 (23%) developed graft steatohepatitis. These three children with steatohepatitis underwent total internal biliary diversion, and on 1-year follow-up, their graft steatosis resolved. Although the overall post-LT outcome was good for the whole cohort, the authors found that those after LT for PFIC1 were relatively poorer regarding catch-up growth, graft steatosis, and post-LT diarrhea, and these issues can be improved by making biliary diversion.

Menon J, Shanmugam N, Vij M, et al. Liver Transplantation for Zellweger Syndrome. Indian J Pediatr 2023. DOI: 10.1007/s12098-023-04937-7

In this case report, the authors have described an interesting case of an 11-year-old boy diagnosed with Zellweger syndrome who presented with decompensated cirrhosis along with bilateral proptosis and who underwent an ABO incompatible liver transplantation with his mother being the donor. He had an uneventful postoperative period and had normal graft function and normal cognition, along with resolution of proptosis at the end of the 24-month follow-up. This case highlights the good outcome of liver transplantation in a group of carefully selected patients with Zellweger syndrome.

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