Publications by the Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition Members in PubMed-indexed Journals (28^th August to 30^th November 2023)

Prasanth K Sobhan

Division of Pediatric Gastroenterology; Department of Pediatrics, Sree Avittom Thirunal Hospital for Women and Children (SAT Hospital), Government Medical College, Thiruvananthapuram, Kerala, India

Corresponding Author: Prasanth K Sobhan, Division of Pediatric Gastroenterology; Department of Pediatrics, Sree Avittom Thirunal Hospital for Women and Children (SAT Hospital), Government Medical College, Thiruvananthapuram, Kerala, India, e-mail: drprasanthksobhan@gmail.com

How to cite this article: Sobhan PK. Publications by the Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition Members in PubMed-indexed Journals (28^th August to 30^th November 2023). Ann Pediatr Gastroenterol Hepatol 2023;5(4):75–77.

Source of support: Nil

Conflict of interest: Dr Prasanth K Sobhan is associated as the Associate Editorial Board member of this journal and this manuscript was subjected to this journal’s standard review procedures, with this peer review handled independently of this editorial board member and his research group.

Source: Samanta A, Poddar U, Sarma MS, et al. Endoscopic band ligation in blue rubber bleb nevus syndrome: a report of two children. JPGN Rep 2023;4(4):e344. DOI: 10.1097/PG9.0000000000000344

In this case series, authors have reported on two children with cutaneous lesions who presented with gastrointestinal (GI) bleeding one of them with obscure GI bleeding and the other with overt GI bleeding. Colonoscopy and upper GI endoscopy revealed bluish polypoidal lesions in the GI tract and video capsule endoscopy helped in disease mapping in the small bowel clinching the diagnosis of blue rubber bleb nevus syndrome (BRBNS). Both of them were successfully treated with endoscopic band ligation and nonselective β-blockers, which resulted in an improvement in their hemoglobin levels. The authors have concluded by highlighting the successful use of endoscopic band ligation of GI lesions as a therapeutic modality in BRBNS.

Source: Vasudevan AK, Shanmugam N, Rammohan A, et al. Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis. Pediatr Transplant 2023;27(8):e14600. DOI: 10.1111/petr.14600

In this retrospective analysis of prospective data from a single center over a decade (2011–2022) authors have aimed to present their data on pediatric liver transplantation (PLT) for progressive familial intrahepatic cholestasis (PFIC) and compared their early, intermediate, and long-term outcomes, highlighted their differences, and management strategies. A total of 580 consecutive PLT during the study period, 60 (10.3%) were performed for PFIC, (21% (n = 13), 18% (n = 11) 52% (n = 31), and 8% (n = 5) PFIC1, bile salt exporter pump (BSEP) (PFIC2), multidrug-resistant protein-3 (MDR3) (PFIC3) and transjunctional protein-2 (TJP2) (PFIC4) deficiencies, respectively. There were no significant differences in gender, PELD scores, body mass index, type of grafts, cold and warm ischemia times, intraoperative blood loss, and morbidity among the groups. Post-LT chronic diarrhea was observed in six (46.1%) children with PFIC-I, and of them, three (23%) developed graft steatohepatitis. Three of these children underwent total internal biliary diversion (TIBD) and on 1-year follow-up, their graft steatosis resolved and they attained catchup growth. Catchup growth was significantly poorer in the PFIC1 group (44.4 vs 88%, 90%, 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%, 100% & 69.2%, 81.8%, 96.8%, and 100%, respectively. Authors have concluded that early post-LT morbidity and 1- and 5-year post-LT survival are excellent and comparable across the different types of PFIC in this largest to-date cohort. The outcome after LT for PFIC1 was relatively poorer as reflected by catchup growth, graft steatosis, and post-LT diarrhea, which can be optimized by the addition of TIBD during LT.

Source: Hosaagrahara Ramakrishna S, Shah N, Acharyya BC, et al. The need for culturally appropriate food allergy management strategies: the Indian milk ladder. Nutrients 2023;15(18):3921. DOI: 10.3390/nu15183921

Authors have aimed to develop a milk ladder based on food cultures in India, considering the sugar and fat content in the Indian foods included in the ladder and calculating and testing the amount of cooked milk protein in the different Indian foods included in the ladder. The group was composed of a dietitian and Indian pediatric gastroenterologists (from all four regions of India—North, South, East, and West) as well as an international pediatric gastroenterologist and pediatric allergy specialist dietician. They have developed the first milk ladder based on the unique features of Indian food habits through the consensus of Indian experts along with international collaboration. One limitation was that they only measured the total milk protein content and not the whey and casein fractions. This is the world’s first milk ladder that provides information on the timing and temperature of cooking, with validated milk protein content. Authors have concluded that “the Indian milk ladder” will be a very helpful tool for pediatricians helping manage cow’s milk allergy in children as well as their parents and caregivers, not only in India but in countries worldwide where these foods are commonly consumed.

Source: Hosaagrahara Ramakrishna S, Hassan A, Kasala MB, et al. Pediatric combined living donor liver and kidney transplantation for primary hyperoxaluria type 2. Am J Transplant 2023;23(10):1622–1625. DOI: 10.1016/j.ajt.2023.05.006

In this case report authors have described a 12-year-old boy with primary hyperoxaluria type 2 (PH2) who presented with end-stage renal disease (ESRD) and systemic oxalosis who underwent a combined living donor liver and kidney transplant (CKLT) from two donors, one of whom was a heterozygous carrier of the mutation. Surgery was uneventful, and he had immediate liver and renal graft function. He was continued on continuous renal replacement therapy intraoperatively and postoperatively for 72 hours. A total of 18 months after the transplant, he remains well with serum oxalate levels of 17 μmol/L and normal renal and liver graft functions. Through this illustrative case report authors have recommended that CLKT should be the treatment of choice for children with PH2 and early-onset ESRD.

Source: Ghosh U, Sen Sarma M, Samanta A. Challenges and dilemmas in pediatric hepatic Wilson’s disease. World J Hepatol 2023;15(10):1109–1126. DOI: 10.4254/wjh.v15.i10.1109

Diagnosis of Wilson’s disease is made with a constellation of clinical-laboratory parameters that have significant overlap with other liver diseases and often pose a significant dilemma for clinicians. In this review article, the authors have described the following challenges and dilemmas in pediatric Wilson disease in great detail with an extensive review of the literature. Though there are difficulties in making the correct diagnosis, with the help of nonceruloplasmin bound copper, relative exchangeable copper, and newer methods like anterior segment-optical coherence tomography to detect early Kayser–Fleischer ring, diagnosis can be made in resource-limited conditions, where mutational analysis is not cost-effective. Chelation remains the mainstay of treatment and is to be preferred in active disease whether symptomatic or asymptomatic. While D-penicillamine is a potent drug, its side effects lead to drug discontinuation. Trientine is cost-prohibitive in developing countries. There is no single test to assess the adequacy of chelation. Exchangeable urinary copper is an essential upcoming diagnostic and prognostic tool. Regarding the transition to maintenance therapy, the exact timeline is not yet defined but depends on the liver function. Hypersplenism clouds the assessment of myelosuppression of drugs especially in the presence of cirrhosis. It may be difficult to distinguish disease tubulopathy from drug-induced glomerulonephritis. Neurological worsening due to chelators may appear similar to disease progression. Presentation as fulminant hepatic failure requires rapid workup. There is a limited window of opportunity to salvage these patients with the help of plasmapheresis and other liver-assisted devices.

Source: Mohan N, Deswal S, Bhardwaj A. Spectrum and trend of pediatric inflammatory bowel disease: a two-decade experience from northern India. Indian J Gastroenterol 2023. DOI: 10.1007/s12664 - 023 - 01440-x

In this single-center study, authors have aimed to retrospectively analyze the clinical spectrum of pediatric inflammatory bowel disease (P-IBD) in northern India during the period from January to December 2019. A total of 126 children enrolled, ulcerative colitis (UC) was diagnosed in 76 (60.3%), Crohn’s disease (CD) in 44 (34.9%), and IBD-unclassified (IBD-U) in six (4.76%) patients. The mean age at diagnosis was 11.3 years; 38.8% were < 10 years with a male:female ratio of 1.6:1. A total of 16 children (12.7%) had very early onset IBD (VEOBD). Overall, the median time to diagnosis in IBD was 12 months [interquartile range (IQR): 3.25–24], which was as high as 52.5 months (IQR: 11–98) in CD. Pancolitis with bleeding per rectum and ileocolonic involvement with pain in the abdomen were the most common presentations in UC and CD, respectively. Structuring disease was seen in 27% of CD cases. Relapses were seen in 46% (35/76) of UC and 23% (10/44) of CD kids. The step-up treatment protocol was employed in them with the use of biologicals in 12% of cases. There was a 2.75-fold rise in IBD cases in the last 10 years (2010–2020). There was a reduction in time to diagnosis (21 months vs 90 months; p-value of 0.012) and empirical antitubercular therapy use (90 vs 5.8%) in CD over two decades. Authors have concluded that P-IBD is on the rise; UC is more common than CD; pancolitis and ileocolonic disease are the most common disease sites in UC and CD, respectively and there is a significant delay in the time to diagnosis in CD.

Source: Bhagat P, Vij M, Raju LP, et al. Update on the pathology of pediatric liver tumors: a pictorial review. Diagnostics (Basel) 2023;13(23):3524. DOI: 10.3390/diagnostics13233524

Benign and malignant pediatric liver tumors show a wide variety of morphologic features and pose a significant challenge to a surgical pathologist. In this extensive pictorial review, authors have discussed the clinical presentation, imaging findings, pathology, and relevant molecular features that can help in the correct identification of these tumors. There is a continuous evolution in the pathologic classification of pediatric liver tumors with the advent of molecular characterization. The proper utilization of immunohistochemistry markers helps in the accurate characterization of these tumors in daily practice.

Source: Samanta A, Sen Sarma M, Yadav R. Budd–Chiari syndrome in children: Challenges and outcome. World J Hepatol 2023;15(11):1174–1187. DOI: 10.4254/wjh.v15.i11.1174

In this review article, the authors have aimed to discuss the various aspects of Budd–Chiari syndrome (BCS), including recent updates on diagnostic and treatment modalities for BCS in children as well as the challenges them with special focus on chronic BCS. Compared to adults with BCS, children have primary BCS as the predominant etiology, earlier clinical presentation, and hence better treatment outcomes. Underlying prothrombotic conditions play a key role in the etiopathogenesis of BCS, though workup for the same is often unyielding in children. The use of next-generation sequencing in addition to conventional tests for thrombophilia leads to better diagnostic yield. However, unlike in adults, established treatment guidelines have not been developed in children with BCS. In recent years, advances in radiological endovascular intervention techniques (angioplasty and stenting), surgical shunting, and transjugular intrahepatic portosystemic shunt have revolutionized the treatment and outcome of BCS. Better patient and procedure selection, choice of appropriate size and type of stent, and mandatory follow-up assessment are of utmost importance for better long-term outcomes in radiological interventions. Long-standing congestion and fibrosis of hepatic parenchyma in patients with chronic BCS are known to give rise to hepatic nodules and diagnosis of hepatocellular cancer can be challenging. Long-term follow-up studies including the prevalence of hepatopulmonary syndrome and hepatocellular carcinoma in this patient population are lacking in children.

Source: Panda K, Sood V, Lal BB, et al. Liver histology and hepatic progenitor cell activity in pediatric acute liver failure: implications for clinical outcome. Pediatr Transplant 2023:e14662. DOI: 10.1111/petr.14662

In this single-center study, authors have aimed to study the correlation of liver histology and hepatic progenitor cell (HPC) activity with outcomes in pediatric acute liver failure (PALF). A total of 94 children with PALF with available hepatic histological specimens were enrolled. Specimens were analyzed for hepatocyte loss, HPC activity [using cytokeratin 7, cytokeratin 19, sex-determining region Y-related high mobility group box (SOX)9 and epithelial cell adhesion molecule (EpCAM)], hepatocyte proliferation (using Ki67), and hepatocyte senescence (using p53 and p21). A total of 22 (23.4%) survived with native liver (SNL) (good outcome group) while the rest (poor outcome group) either died (33 and 35.1%) or received liver transplants (LT) (39 and 41.5%). When compared to subjects with poor outcomes, those in the SNL group exhibited significantly less severe hepatocyte loss, fewer HPC/HPF, more proliferating hepatocytes, and less senescent hepatocytes. Increasing severity of hepatocyte loss (adjusted OR—9.95, 95% CI—4.22–23.45, p < 0.001) was identified as an independent predictor of poor outcome. Almost 80% of children with >50% native hepatocyte loss had poor outcomes within 10 days of hospitalization. Authors have concluded that in PALF, more severe hepatocyte loss, higher number of HPC activation, lesser number of proliferating hepatocytes, and greater number of senescent hepatocytes are associated with a poor outcome. Loss of >50% hepatocytes was identified as an independent predictor of poor outcome in PALF.

Source: Das P, Malik R, Kaul S, et al. Tufting enteropathy: a rare anatomical cause of small bowel diarrhea in infants with mild or no villous abnormality. Gastroenterol Hepatol Bed Bench 2023;16(2):225–229. DOI: 10.22037/ghfbb.v16i1.2731

In this case, the report authors have described a case of congenital tufting enteropathy (CTE) in a 1-year-old female child who presented with intractable diarrhea soon after birth, progressive abdominal distension, and failure to thrive. Histological examination revealed mild villous abnormality with the presence of epithelial tufts both in the villous and crypt surface, in the duodenum, and in rectal biopsies supported by complete loss of MOC31 staining. Deep sequencing revealed a homozygous 3’ splice mutation at intron 5 of the EPCAM gene (c.556-14A>G) confirming the diagnosis of CTE. She was given total parenteral nutrition (TPN) support and discharged with weight gain under a home-based parenteral nutrition supplement. This case illustrates the need for a multidisciplinary team approach to identify the etiology of infantile intractable diarrhea and highlights favorable outcomes with adequate nutritional interventions.

Source: Ramakrishna SH, Nayak SP, Rao S, et al. Kasai portoenterostomy at a slightly delayed age and native liver survival in children with biliary atresia: single center experience. Indian Pediatr 2023;60(8):655–658

In this single-center study, authors have aimed to study modifiable prognostic factors that extend/increase native liver survival in children with biliary atresia. Hospital records of patients with neonatal cholestasis, with a focus on infants diagnosed with biliary atresia between January 2014 and May 2021 were extracted and analyzed to determine the association of outcome with clinical and laboratory variables. They identified 142 infants with neonatal cholestasis, with 78 diagnosed with biliary atresia and 64 with other causes. Of the 78 babies (61.5% boys) with biliary atresia analyzed, 11 (14.1%) did not return for treatment after a confirmed diagnosis or were lost to follow-up. They observed that infants who underwent Kasai portoenterostomy (KPE) at a median (IQR) age of 76 (72–79) days had the best outcomes, with minimal severe post-KPE complications and a 2-year survival rate of 84.6%, compared to other infants (younger and older age at KPE). The median (IQR) weight at KPE in this group was 4.66 (4.2, 5.0) kg. Authors have concluded that in contrast to traditional recommendations, babies with a median age at KPE of 76 days had superior native liver survival (84.6%) and reduced post-KPE complications, as compared to earlier KPE age. Authors have suggested that this observation needs confirmation through multicentric prospective studies in different settings.

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Publications by the Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition Members in PubMed-indexed Journals (28th August to 30th November 2023)

Publications by the Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition Members in PubMed-indexed Journals (28^th August to 30^th November 2023)