ISPGHAN LITERATURE FESTIVAL


https://doi.org/10.5005/jp-journals-11009-0106A
Annals of Pediatric Gastroenterology and Hepatology
Volume 4 | Issue 3 | Year 2022

Event Brief

The 3rd Pediatric Gastroenterology Literature Festival was held on 20th and 21st of August in Chennai at Hotel Ramada Plaza Guindy. The event was organized by Gleneagles Global Health City, Chennai under the banner of ISPGHAN.

Literature fest is unique conference where high-quality literature pertaining to the field of pediatric gastroenterology, hepatology, and nutrition is reviewed and discussed. The quality evidence is subsequently applied in clinical practice.

The first Lit fest was organized by Dr Yogesh Waikar in 2018 at Nagpur and 2nd fest by Dr Rimjhim Shrivastava in Raipur in 2019. The program was inaugurated by Dr Neelam Mohan, President ISPGHAN, Dr VS Shankaranarayanan, senior pediatric gastroenterologist, Chennai, and Dr Malathi Sathiyasekaran, senior pediatric gastroenterologist, Chennai. The event was well attended by more than 70 delegates. The delegates included pediatric gastroenterologists from all over India, ISPGHAN fellowship students, and also DNB/DM (medical gastroenterology) residents. The high-quality academics were enjoyed by all the delegates.

The unique feature of this festival was that the two best presentations each from Hepato-Pancreato-Biliary and Luminal GI section were awarded best presentation certificates.

Dr Rimjhim Shrivastava and Dr Prasanth K Sobhan won the best presentation in Luminal GI section and Dr Rajeev Khanna and Dr Jagadeesh Menon in Hepatology section.

Somashekara Hosaagrahara Ramakrishna
Consultant, Pediatric GI & Liver, Gleneagles Global Hospitals, Chennai, Tamil Nadu, India

Treatment of Mitochondrial Hepatopathy

Remya R Pai, Lisie Hospital, Kochi, Kerala

Mitochondria, powerhouse of the cell, are closely linked to the pathophysiology of various common as well as uncommon disorders of liver and beyond. Although new therapeutic approaches have been introduced with encouraging preclinical and clinical outcome, there is no definitive cure for primary mitochondrial hepatopathy (PMH). The treatment is still in infancy. Current treatment is aimed at circumventing acute metabolic crisis and long-term management including nutritional rehabilitation. While case studies have reported treatment effects, there is scant evidence to support most pharmacological interventions, except Co-enzymeQ supplementation in Primary Co-enzyme Q10 synthesis defect and L-Arginine for Metabolic stroke. With recent advances in molecular diagnosis, PMH are now being increasingly identified and more controlled trials are expected. Treatment also focuses on maintaining optimal health, using preventive measures to mitigate symptoms worsening during times of stress and avoiding mitotoxic substances. Role of liver transplant (LT) in PMH is controversial. Multisystem involvement often precludes performing LT. Overall post LT survival is less with PMH. The translation of modern genomic technology into clinical practice and research has facilitated major advances in the development of treatment of rare genetic diseases. Other than the mitochondrial replacement therapy utilized to prevent the inherited mitochondrial mutations, genomic editing therapy can be considered the ultimate treatment strategy. Significant breakthroughs in developing new therapeutic approaches will continue in coming decades through further advances in gene therapy and screening assays to improve mitochondrial functions.

Cytomegalovirus Infection of Gut and Liver

Bhaswati C Acharyya DCH, MD (Paed), DNB (Paed), DNB (Gastro), MRCPCH (UK), FRCPCH

Dhanasekhar Kesavelu MD, MRCPCH, FRCPCH

Cytomegalovirus (CMV) is a ubiquitous virus, the largest of the Herpesviridae that commonly infects people of all ages throughout the world. In children, it can cause an asymptomatic or mild disease in immunologically normal hosts. But It can result in a severe and potentially life-threatening disease in newborns and immunocompromised children. Infection with CMV is worrying because in addition to their direct effects of tissue injury and clinical illness it establishes latency and intermittently reactivates. In India about 1.2% newborns are infected with CMV. Neonates with symptomatic congenital CMV disease with or without central nervous system (CNS) involvement have improved audiologic and neurodevelopmental outcomes at 2 years of age when treated with oral valganciclovir (16 mg/kg/dose, administered orally twice daily) for 6 months. CMV status may influence the prognosis of biliary atresia. Clinicians should be able to routinely identify the subset of biliary atresia patients who are also CMV-positive, in order to improve native liver survival. Universal screening of all pregnant women to assist in the diagnosis of primary CMV infection is currently not recommended but treatment of pregnant mother and antenatal diagnosis are possible if a mother asks for it. Acquired CMV infection in healthy children and adolescents is most often asymptomatic. However, approximately 10% of acquired CMV infections produce symptoms1 causing a mononucleosis-like syndrome. In children with ulcerative colitis, during relapse if tissue viral load is more than 250 copies/mg of tissue or >4 cells/section on immunohistochemistry (IH); treatment of CMV should be initiated. It is unclear what to do with a patient’s immunosuppression in the event of initiation of antiviral therapy.2 The ECCO guidelines recommend that in patients, in addition to starting antiviral therapy, immunomodulators could be discontinued. Immunomodulators, however, must be discontinued in cases of systemic CMV disease.3 Antiviral prophylaxis and pre-emptive therapy are intended to decrease CMV infections and disease in LT patients. Oral valganciclovir and intravenous ganciclovir are the mainstays of treatment, with variable duration depending on CMV manifestations, viral load, histopathology, and clinical response.

Eosinophilic Esophagitis: Pathophysiology, Management and Newer Insights Including Newer Therapies

Dr Rimjhim Shrivastava

Eosinophilic esophagitis (EoE)is a chronic, Th2 immune and antigen-mediated esophageal disease which is characterized clinically by symptoms related to esophageal dysfunction, and histologically by eosinophil-predominant inflammation ( >15 eso/hpf). So, far there is no approved drug for EoE, however, the common practice to handle this disease is the use of proton pump inhibitors or swallowed topical corticosteroids and/or specific diet. The clinical presentation of EoE is age dependent in most of the cases. Infants and younger children present with vomiting, food refusal or growth failure whereas older children and adolescents present with heartburn, dysphagia and food impaction. This may be due to the progressive nature of the disease and the subsequent tissue remodeling which makes it range from an inflammatory disease to fibro stenotic disease. Diagnosis is by typical endoscopic features (longitudinal furrow, edema, exudate, fragile mucosa, pseudorings, stricture, etc.) and mucosal eosinophilia ( >15 eso/hpf), after ruling out other causes of mucosal eosinophilia. Treatment includes: dietary modifications (elimination diet or elemental diet); drugs as proton pump inhibitors or topical corticosteroids and endoscopic dilatation in cases of low caliber esophagus or stricture. Elimination diet (efficacy 74%) includes either targeted diet after allergic testing or empirical elimination of six commonly allergic foods ([six food elimination diet] SFED) as dairy, wheat, eggs, soy, peanuts/tree nuts and seafood; or a step-up approach with four food elimination diet as dairy products, gluten-containing grains, eggs, and legumes or two-foods elimination diet excluding dairy products and wheat. Elemental diet has an efficacy of 97% but it should be reserved for multiple food allergies, failure to thrive, or severe disease owing to its high cost, poor palatability, lack of adherence, the need for nasogastric tubes for younger children, and frequent endoscopy for reintroduction of food. Proton pump inhibitors (efficacy: 30–60%) and topical corticosteroids (fluticasone propionate and budesonide) (efficacy: 60–90%) are required to be given in high doses for 6–12 weeks until remission and then should be continued in low maintenance dose for 1–3 years or more. The main drawback of drugs is the relapse of the disease after stopping the drug in most of the patients. Recently interleukin (IL)-13 and interleukin (IL)-14 inhibitor dupilumab has been approved by FDA for EoE in more than 12 years of age-group.

Gastrointestinal Manometry in Children

Yogesh Waikar https://orcid.org/0000-0001-7394-5150, Consultant Pediatric Gastroenterologist & Hepatologist, Superspecialty GI Clinics & Endoscopy Center, Dhantoli, Nagpur, Maharashtra, India, e-mail:
pedgihep@yahoo.com

Ano rectal manometry is one of the commonly used motility test in children. It evaluates the motility function of anorectal tract. It is helpful for children with constipation, rectal hypersensitivity, fecal incontinence, Hirschsprung’s disease, anal achalasia, and anorectal malformations. The most common indication of anorectal manometry is to establish recto-anal inhibitory Reflex to rule out Hirschsprung’s disease. It is the safe procedure.

ORCID

Yogesh Waikar https://orcid.org/0000-0001-7394-5150

(Images taken post consent from patient and subject to copy right Superspecialty Clinics & Endoscopy Center, Nagpur, Dr Yogesh Waikar).

Normal values for children are now available. Both solid state and water perfused catheter can be used for anorectal manometry (ARM). It can help in diagnosis of anorectal motor dysfunction. It can also be used in biofeedback therapy for treatment of defecation dyssynergia in children. More studies are needed before using advanced ARM-metrics in children. London the international anorectal physiology working group (IAPWG) protocol should be followed in future studies for comparative analysis.

Structural disorders of esophagus are well managed by endoscopy. Functional or motor disorder of esophagus are called as esophageal dysmotility disorders. These can be assessed by manometry in children. Esophageal manometry (HREM) can study disorders of swallowing, feeding problem in infants, nonobstructive dysphagia, and lower esophagus dysfunction. Lower esophageal dysfunction induced reflux disease and esophageal gastric junction outlet obstruction syndrome can also be evaluated by HREM. Normal metric values are now available.

Esophageal manometry (HREM) is safe in children. High resolution HREM is an important tool to diagnose esophageal dysmotility disorders in children. Normal values are now available in children. Standard and newer biometrics can help in classification of esophageal dysmotility. Chicago 4.0 classification provides frame work and should be used in children judiciously with age appropriate adjustments. Manometry with impedance studies can guide proper management in dysmotility. HREM can be used preoperatively, intraoperatively and postoperatively to differentiate and classify dysphagia for optimum results. HREM can help to diagnose and localize feeding problems in children. Unnecessary prokinetics, proton pump inhibitor and antidepressant can be avoided. Motility disorder of esophagus can be secondary to underlying systemic disease or drugs. Clinical diagnosis and radiological interpretative correlation and personal drug history is must before analysis of HREM.

Diagnosis of Mitochondrial Hepatopathy

Dr Aashay Abhay Shah, Paediatric Gastroenterologist & Hepatologist, Prism Paediatric Gastro, Ahmedabad, Gujarat, India

Mitochondrial hepatopathy is difficult to diagnose as signs and symptoms are nonspecific and symptom mimic sepsis. Biochemical laboratory includes amino, organic acids, lactate: pyruvate and acylcarnitine profiles can strongly increase suspicion for mitochondrial disease. The blood lactate: pyruvate ratio is most reliable in differentiating electron transport chain disease from disorders of pyruvate metabolism. A combination of lactate: pyruvate ratio, oral glucose challenge & urine gas chromatography/mass spectrometry (GCMS) has good sensitivity. Tissue biopsy plays a vital role in diagnosing mitochondrial disorders. Typical findings in liver biopsy are acinar disarray, ballooning degeneration, micro-macro vesicular steatosis, foci of lobular inflammation, and spotty necrosis. mtDNA length mutations are best detected by southern blot analysis in total mtDNA extracts from blood lymphocytes. Next-generation sequencing (NGS) is optimally suited for heterogeneous disorders. It improved cost efficacy vs sequential single gene or candidate gene panels. An NGS methodology providing complete coverage of known mitochondrial disease genes is preferred. Single-gene testing should usually be avoided because mutations in different genes can produce the same phenotype. If no known mutation is identified via known NGS gene panels, then whole exome sequencing should be considered. Muscle liver biopsies should be performed in the routine analysis for mitochondrial disease when the diagnosis cannot be confirmed with DNA testing. Emerging biomarkers like fibroblast growth factor 21 (FGF21) growth differentiation factor 15 (GDF15), glutathione, and cerebrospinal fluid (CSF) neopterin further research are needed and ongoing for diagnosing mitochondrial hepatopathy.

Fig. 1: Anorectal manometry images during rest, squeeze and push.

Third Space Endoscopy Utility in Pediatrics

Dr Priyanka Udawat, Pediatric Gastroenterologist & Hepatologist, Institute of Digestive & Liver Care (IDL Care), S L Raheja Hospital, Mumbai, Maharashtra, India

Third space or submucosal space is a potential space which on expansion allows the endoscopist to execute a multitude of therapeutic procedures where deeper layers of the gastrointestinal tract can be accessed by tunneling in the submucosal space without compromising the integrity of the overlying mucosa.

Third space endoscopy is less invasive emerging technique for various treatments in pediatric population. Peroral endoscopic myotomy (POEM) is an effective alternative to pneumatic dilatation and Laparoscopic Heller’s Myotomy (LHM) in pediatric achalasia. It is less invasive with minimal recovery time and has low complication rates. POEM may be the preferred option for patients with type III achalasia. Randomized controlled trials comparing POEM to another modality such as LHM or balloon dilatation, are warranted in order to recommend POEM as a first-line procedure for pediatric achalasia. Case reports shows gastric POEM (G-POEM) is technically feasible, safe and successful procedure for congenital hypertrophied pyloric stenosis in newborn and infants. Comparative studies are needed to compare the advantages of G-POEM with laparoscopic pyloromyotomy. Per-rectal endoscopic myotomy (PREM) is safe and effective minimally invasive procedure appears promising in cases with short segment Hirschsprung’s disease. Quality studies are required before PREM can be recommended in routine clinical practice. Endoscopic submucosal dissection (ESD) is safe and effective in resection of polyps in pediatric population. Repair of esophageal fistula by endoscopic closure after ESD of the surrounding mucosa may allow a definitive resolution of the endotracheal fistulas. Local expertise is crucial component in choosing between these options.

REFERENCES

1. Kimberlin DW, Jester PM, Sánchez PJ,et al. Valganciclovir for symptomatic congenital cytomegalovirus disease. N Engl J Med 2015;372(10):933–943. DOI: 10.1056/NEJMoa1404599

2. Mourad FH, Hashash JG, Kariyawasam VC, et al. Ulcerative colitis and cytomegalovirus infection: from A to Z. J Crohns Colitis 2020;14(8):1162–1171. DOI: 10.1093/ecco-jcc/jjaa036

3. Rahier JF, Magro F, Abreu C, et al. European Crohn’s and Colitis Organisation [ECCO]. Second European evidence-based consensus on the prevention, diagnosis and management of opportunistic infections in inflammatory bowel disease. J Crohns Colitis 2014;8(6):443–468. DOI: 10.1016/j.crohns.2013.12.013

________________________
© The Author(s). 2022 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by-nc/4.0/), which permits unrestricted use, distribution, and non-commercial reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.