Diagnosis and Management of Gastroesophageal Reflux Disease in Children: Recommendations of Pediatric Gastroenterology Chapter of Indian Academy of Pediatrics, Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition (ISPGHAN).

JUSTIFICATION: Gastroesophageal reflux (GER) related symptoms are a major cause of parental concern and referrals at all ages. These guidelines have been framed to inform pediatricians regarding current diagnosis and management of gastro-esophageal reflux disease (GERD). PROCESS: A group of experts from the pediatric gastroenterology sub-specialty chapter of Indian Academy of Pediatrics (ISPGHAN) discussed various issues relating to the subject online on 25 October, 2020. A consensus was reached on most aspects and a writing committee was constituted. This committee had three meetings for a detailed discussion. The statement was sent to the entire group and their approval obtained. OBJECTIVE: To formulate a consensus statement to enable proper diagnosis and management of GERD in children. RECOMMENDATIONS: GER is physiological in most infants and it improves as age advances. The pathological form, called GERD causes distressing symptoms that affect daily activities and may result in complications. The presentation would vary from regurgitation to severe symptoms due to esophageal or respiratory tract disease. In older children, esophagitis is the commonest manifestation of GERD. A careful history and clinical examination are adequate to make a diagnosis in most patients, but judicious investigations are necessary in a few. Upper gastro intestinal tract endoscopy may be required in those with esophageal manifestations, dysphagia and hematemesis. In children with extra-esophageal symptoms, MII-pH monitoring and scintigraphy are necessary. Empirical treatment with a Proton pump inhibitor (PPI) has not been proven useful in infants, but a four-week trial is recommended in older children without complications. While positioning and feed thickening have limited benefit in infants, life-style modifications are important in older children.

Guidelines on Diagnosis and Management of Cow's Milk Protein Allergy.

JUSTIFICATION: Cow's milk protein allergy (CMPA) is increasingly being diagnosed in the West, while there is scant data on the subject from India. There is low awareness among pediatricians about its diagnosis and management; leading to improper diagnosis. PROCESS: A group of experts from the pediatric gastroenterology sub-specialty chapter of Indian Academy of Pediatrics (Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition) met at Mumbai on 26 October, 2018 and discussed various issues relating to the subject. A broad consensus was reached and a writing committee was formed. They met again on 11 August, 2019 at Chennai for a detailed discussion. The statement was sent to the entire group by e-mail and their approval obtained. OBJECTIVE: To formulate a consensus statement enable proper diagnosis and management of Cow's milk protein allergy. RECOMMENDATIONS: Cow's milk protein allergy is most common in the first year of life. Gastrointestinal manifestations are usually non-IgE mediated and therefore skin prick test and specific IgE levels are not useful in diagnosis. Clinical response to elimination diet followed by a positive oral food challenge is diagnostic. In patients with only gastrointestinal manifestations, sigmoidoscopy and rectal biopsy may be considered as an alternative. Management involves strict avoidance of all forms of bovine milk protein. For infants who are artificially fed, an extensively hydrolyzed formula is the first choice. Soy formula is an alternative in those above six months of age. Since most infants outgrow the allergy, elimination diet is only for a limited period and re-evaluation should be done periodically.

Coronavirus Disease (COVID-19) and the Gastrointestinal System in Children.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV2), though primarily a respiratory pathogen, also involves the gastrointestinal tract. Similar to the respiratory mucosa, angiotensin converting enzyme-2 (ACE-2) receptor and transmembrane serine protease 2 (TMPRSS2) co-express in the gastrointestinal tract, which facilitates viral entry into the tissue. Less than 10% of children with infection develop diarrhea and vomiting. Prolonged RT PCR positivity in the stool has raised the possibility of feco-oral transmission. Elevated transaminases are common, especially in those with severe coronavirus disease (COVID-19). Children with inflammatory bowel disease and post liver transplant patients do not have an increased risk of disease, and should remain on medications they are already on. Children with chronic liver disease should continue their medications as usual. All elective procedures like endoscopy should be postponed.

Therapeutic Enteral Formulas in Children.

PURPOSE: A variety of enteral formulas for various diseases have become available in India in the last few years. Awareness among pediatricians about the availability and indications for these therapeutic formulas is low. METHODS: A literature search was conducted in PUBMED and relevant data collected from all English language publications available. Data on the commercial preparations was sourced from the individual companies, the Diet 4 life initiative as well as FSSAI (Food safety and standards authority of India). CONCLUSIONS: Therapeutic enteral formulas, which are indicated in various disease states belong to four categories - lactose modified, hydrolyzed, MCT based and metabolic disease specific formulas. Lactose modified formulas which are used in temporary or permanent lactose intolerance and Galactosemia are either casein or soy protein based. Hydrolyzed formulas could be partially hydrolyzed, extensively hydrolyzed or amino acid based. Only extensively hydrolyzed formula should be recommended in milk protein allergy. Amino acid (elemental) formulas are mainly indicated in patients with diffuse intestinal mucosal disease. MCT formulas are used in chronic liver disease with cholestasis, and have 30 to 80% MCT. Formulas for inborn errors of metabolism are free of specific carbohydrate, amino acid or fatty acid. Proprietary formulas presently available in India with their specifications have been listed.

Corrigendum to "Wilson's Disease: Clinical Practice Guidelines of the Indian National Association for the Study of Liver (INASL), The Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition (ISPGHAN) and the Movement Disorders Society of India (MDSI)" [J Clin Exp Hepatol 9 (2019) 74-98].

[This corrects the article DOI: 10.1016/j.jceh.2018.08.009.].

Wilson's Disease: Clinical Practice Guidelines of the Indian National Association for Study of the Liver, the Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition, and the Movement Disorders Society of India.

Clinical practice guidelines for Wilson's disease (WD) have been published by the American Association for the Study of Liver Diseases and European Association for the Study of the Liver in 2008 and 2012, respectively. Their focus was on the hepatic aspects of the disease. Recently, a position paper on pediatric WD was published by the European Society of Pediatric Gastroenterology Hepatology and Nutrition. A need was felt to harmonize guidelines for the hepatic, pediatric, and neurological aspects of the disease and contextualize them to the resource-constrained settings. Therefore, experts from national societies from India representing 3 disciplines, hepatology (Indian National Association for Study of the Liver), pediatric hepatology (Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition), and neurology (Movement Disorders Society of India) got together to evolve fresh guidelines. A literature search on retrospective and prospective studies of WD using MEDLINE (PubMed) was performed. Members voted on each recommendation, using the nominal voting technique. The Grades of Recommendation, Assessment, Development and Evaluation system was used to determine the quality of evidence. Questions related to diagnostic tests, scoring system, and its modification to a version suitable for resource-constrained settings were posed. While ceruloplasmin and 24-h urine copper continue to be important, there is little role of serum copper and penicillamine challenge test in the diagnostic algorithm. A new scoring system - Modified Leipzig score has been suggested with extra points being added for family history and serum ceruloplasmin lower than 5 mg/dl. Liver dry copper estimation and penicillamine challenge test have been removed from the scoring system. Differences in pharmacological approach to neurological and hepatic disease and global monitoring scales have been included. Rising bilirubin and worsening encephalopathy are suggested as indicators predicting need for liver transplant but need to be validated. The clinical practice guidelines provide recommendations for a comprehensive management of WD which will be of value to all specialties.

Management of neonatal cholestasis: consensus statement of the Pediatric Gastroenterology Chapter of Indian Academy of Pediatrics.

JUSTIFICATION: Neonatal cholestasis is an important cause of chronic liver disease in young children. Late referral and lack of precise etiological diagnosis are reasons for poor outcome in substantial number of cases in India. There is a need to create better awareness among the pediatricians, obstetricians and primary care physicians on early recognition, prompt evaluation and referral to regional centers. PROCESS: Eminent national faculty members were invited to participate in the process of forming a consensus statement. Selected members were requested to prepare guidelines on specific issues, which were reviewed by two other members. These guidelines were then incorporated into a draft statement, which was circulated to all members. A round table conference was organized; presentations, ensuing discussions, and opinions expressed by the participants were incorporated into the final draft. OBJECTIVES: To review available published data on the subject from India and the West, to discuss current diagnostic and management practices in major centers in India, and to identify various problems in effective diagnosis and ways to improve the overall outcome. Current problems faced in different areas were discussed and possible remedial measures were identified. The ultimate aim would be to achieve results comparable to the West. RECOMMENDATIONS: Early recognition, prompt evaluation and algorithm-based management will improve outcome in neonatal cholestasis. Inclusion of stool/urine color charts in well baby cards and sensitizing pediatricians about differentiating conjugated from the more common unconjugated hyperbilirubinemia are possible effective steps. Considering the need for specific expertise and the poor outcome in sub- optimally managed cases, referral to regional centers is warranted.

Correlation of EEG, CT, and MRI Brain with Neurological Outcome at 12 Months in Term Newborns with Hypoxic Ischemic Encephalopathy.

OBJECTIVE: To correlate electroencephalogram (EEG), computed tomography (CT), and magnetic resonance imaging (MRI) brain with neurological outcome at 12 months in term neonates with hypoxic ischemic encephalopathy. DESIGN: Prospective observational study. SETTING: Neonatal intensive care unit (NICU) in a tertiary care teaching hospital. MATERIALS AND METHODS: The study was conducted between June 2010 and November 2011. Consecutive term neonates with perinatal asphyxia and hypoxic ischemic encephalopathy were the subjects. All babies were managed as per standard protocol. EEG was done as soon as the baby was stable and CT brain within 7 days. MRI was done at 3 months. Neurodevelpmental assessment was done at 12 months. RESULTS: Of the 31 babies, four died and one was lost to follow-up. Neurodevelopmental at 12 months of age was normal in 15 babies. EEG was normal in six babies and all of them had a normal neurodevelopment. Thirteen of the 14 babies with burst suppression pattern were abnormal (P<0.001). CT brain was normal in 14 and all of them had normal neurodevelopment (P<0.001), while 11 of the 12 with cerebral edema had abnormal outcome (P<0.001). Of the 16 babies with normal MRI, 14 were normal, while all six babies with abnormal signals in the cortex and thalamus had abnormal outcome (P=0.002). CONCLUSIONS: A normal EEG and CT brain in a term newborn with hypoxic ischemic encephalopathy (HIE) is associated with good neurological outcome. Burst suppression pattern in EEG, bleeds, or hypodensities in the CT and involvement of basal ganglia/thalamus in the MRI are predictors of abnormal outcome.

Pseudohypoaldosteronism type 1: a rare cause of severe dyselectrolytemia and cardiovascular collapse in neonates.

Severe hyperkalemia is a medical emergency and occurs due to a variety of underlying illnesses. We present a 7 day old neonate who presented with life threatening hyperkalemia due to pseudohypoaldosteronism type 1 (PHA1). The clinical picture resembled congenital adrenal hyperplasia (CAH). Very aggressive management including peritoneal dialysis was required to control hyperkalemia. It is important to differentiate PHA1 from CAH since the former does not respond to corticosteroid therapy and may require peritoneal dialysis for control of hyperkalemia. A discussion on the types, clinical course, and management of pseudohypoaldosteronism type 1 is presented.

Preduodenal portal vein in association with midgut malrotation and duodenal web-triple anomaly?

Preduodenal portal vein (PDPV) is a rare anomaly in which the portal vein passes anterior to the duodenum rather than posteriorly. Generally asymptomatic, PDPV may rarely cause duodenal obstruction or may coexist with other anomalies. We report a neonate who presented with duodenal obstruction and was found out to have 3 coexisting anomalies, each of which can lead to duodenal obstruction independently-PDPV, midgut malrotation, and duodenal web. A duodenoduodenostomy and a Ladd procedure were done, and the child recovered uneventfully. The mechanism of obstruction, interesting metabolic aberrations observed, outcome, and relevant literature are presented.

Leucocyte adhesion defect-1.

Leucocyte adhesion defect (LAD) is an inherited disorder of phagocytic function. It is characterized by inability of the leucocytes, in particular neutrophilis to migrate from the blood stream towards sites of inflammation. LAD -1 is characterized by the absence of b 2 integrins (CD 11 / CD18) on leucocytes. This disorder is characterized by delayed separation of the umbilical cord, recurrent severe infections, periodontitis, and delayed wound healing. We report an infant where the diagnosis was confirmed by flow cytometry.

Antenatally diagnosed ovarian cyst with torsion managed laparoscopically.

Ovarian cyst are the most common intra-abdominal cyst in female neonate. With the help of ultrasound one can make an antenatal diagnosis. We present one such neonate, she was managed by laparoscopic excision. We conclude that neonatal laparoscopy is technically feasible for management of such cysts.

Symptomatic hepatic hemangioendothelioma in a newborn.

A case of hepatic hemangioendothelioma presenting as congestive cardiac failure in a neonate is reported. There was also evidence of platelet consumption. The case was managed successfully with oral prednisolone, resulting in improvement of symptoms and tumor regression over 3 months.

Bilateral communicating bronchopulmonary foregut malformations in a child.

Communicating bronchopulmonary foregut malformations are rare anomalies. The complex anatomy requires innovative surgical techniques. We report a child with bilateral sequestrations communicating with the lower esophagus. The sequestrations were excised through a single thoracotomy incision and the esophagus was repaired. Postoperatively the child has remained asymptomatic.