Other Causes of Chronic Diarrhea in Children.

Chronic diarrhea is still an important cause of morbidity and mortality in children. There are several causes of chronic diarrhea which may be due to intestinal, extra-intestinal or underlying systemic diseases. The etiology varies depending on the age of onset and may include both common and uncommon disorders. In this article some of the uncommon disorders such as immune deficiencies, intestinal lymphangiectasias, drug induced diarrhea, eosinophilic gastrointestinal disorders, endocrinopathies, neuroendocrine secretory tumors, malignancy and factitious diarrhea have been included. Though these disorders are uncommon it is essential that they are considered in select situations as detailed below and evaluated so that definitive therapy may be offered.

A Rare Cause of Rectovaginal Fistula in Early Infancy: It is in the Genes!

Acquired Rectovaginal Fistula (RVF) is rare in infants. Interleukin10/ Interleukin 10 receptor deficiencies are monogenic disorders presenting as aggressive forms of infantile onset inflammatory bowel disease with perianal abscess and fistula. Genetic studies assist in confirming the diagnosis. We present a two month old infant with rectovaginal fistula, severe colitis, failure to thrive and recurrent infections in whom colonoscopy revealed irregular colonic ulcers, and genetic studies confirmed an IL10RB mutation. Hematopoietic Stem cell transplantation is the definitive therapy for this disorder which the child underwent. We report this infant with an acquired RVF with extraintestinal features due to IL10RB mutation to highlight the importance of thinking beyond the local anatomy and looking into the genetic domain.

Inflammatory bowel diseases in Tamil Nadu: A survey of demographics, clinical profile, and practices.

BACKGROUND: Inflammatory bowel disease (IBD) is increasingly diagnosed in South Asia. This survey by the Tamil Nadu Chapter of the Indian Society of Gastroenterology (TNISG) documents the demography, clinical profile, and therapeutic practices related to IBD in Tamil Nadu. METHODS: TNISG members from 32 institutions completed an online cross-sectional questionnaire on IBD patients from March 2020 to January 2021. RESULTS: Of 1295 adult IBD patients, 654 had Crohn's disease (CD), 499 ulcerative colitis (UC), and 42 IBD-unclassified (IBD-U). CD and UC showed a unimodal age distribution. A total of 55% were graduates or postgraduates. A positive family history was noted in 30, other risk factors were uncommon. In CD, the pattern of involvement was ileocolonic (42.8%), ileal (34.7%), colonic (18.9%), and upper gastrointestinal (3.5%); while in UC, disease was characterized as extensive (44.9%), left-sided (41.7%), or proctitis (13.4%). Perineal disease, perianal fistulae, and bowel obstruction were noted in 4.3, 14.0, and 23.5%, respectively, of CD. The most widely used drugs were mesalamine, azathioprine, and corticosteroids. Surgery was undertaken in 141 patients with CD and 23 patients with UC. Of the 138 patients with pediatric IBD (≤16 years), 23 were characterized as very early onset IBD (VEO-IBD), 27 as early-onset, and 88 as adolescent IBD. VEO-IBD were more likely to have a positive family history of IBD and were more likely to have perineal disease and to have the IBD-U phenotype. Among pediatric IBD patients, corticosteroids, mesalamine, and azathioprine were the most commonly used medications, while 25 pediatric patients received biologics. CONCLUSION: This study provides important information on demography, clinical profile, and treatment practices of IBD in India.

Management of Helicobacter pylori infection: The Bhubaneswar Consensus Report of the Indian Society of Gastroenterology.

The Indian Society of Gastroenterology (ISG) felt the need to organize a consensus on Helicobacter pylori (H. pylori) infection and to update the current management of H. pylori infection; hence, ISG constituted the ISG's Task Force on Helicobacter pylori. The Task Force on H. pylori undertook an exercise to produce consensus statements on H. pylori infection. Twenty-five experts from different parts of India, including gastroenterologists, pathologists, surgeons, epidemiologists, pediatricians, and microbiologists participated in the meeting. The participants were allocated to one of following sections for the meeting: Epidemiology of H. pylori infection in India and H. pylori associated conditions; diagnosis; treatment and retreatment; H. pylori and gastric cancer, and H. pylori prevention/public health. Each group reviewed all published literature on H. pylori infection with special reference to the Indian scenario and prepared appropriate statements on different aspects for voting and consensus development. This consensus, which was produced through a modified Delphi process including two rounds of face-to-face meetings, reflects our current understanding and recommendations for the diagnosis and management of H. pylori infection. These consensus should serve as a reference for not only guiding treatment of H. pylori infection but also to guide future research on the subject.

Current practices in the management of corrosive ingestion in children: A questionnaire-based survey and recommendations.

BACKGROUND AND AIM: Corrosive ingestion causes significant morbidity in children with no standard guidelines regarding management. This survey aimed to understand practices adopted by gastroenterologists, identify lacunae in evaluation and management and suggest a practical algorithm. METHODS: Indian gastroenterologists participated in an online survey (65 questions) on managing corrosive ingestion. When ≥ 50% of respondents agreed on a management option, it was considered as 'agreement'. RESULTS: Ninety-eight gastroenterologists (72 pediatric) who had managed a total of ~ 2600 corrosive ingestions in the last 5 years responded. The commonest age group affected was 2-5 years (61%). Majority of ingestion was accidental (89%) with 80% due to improper corrosive storage. Ingestion of alkali and acid was equally common (alkali 41%, acid 39%, unknown 20%). History of inducing-vomiting after ingestion by community physicians was present in 57%. There was an agreement on 77% of questions. The respondents agreed on endoscopy (70%) and chest X-ray (67%) in all, irrespective of symptoms. Endoscopy was considered safe on days 1-5 after ingestion (91%) and relatively contraindicated thereafter. The consensus was to use acid suppression, always (59%); steroids, never (68%) and antibiotics, if indicated (59%). Feeding was based on endoscopic findings: oral in mild injuries and nasogastric (NG) in others. Eighty percent placed a NG tube under endoscopic guidance. Stricture dilatation was considered safe after 4 weeks of ingestion. Agreement on duration of acid suppression and stricture management (dilatation protocol and refractory strictures) was lacking. CONCLUSION: Corrosive ingestion mostly affects 2-5-year olds and is accidental in majority. It can be potentially prevented by proper storage and labelling of corrosives. An algorithm for management is proposed.

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.

A 6-year-old boy with Wilson disease-A diagnostic dilemma.

A 6-year-old boy presented with 2 months history of progressive abdominal distension and jaundice. He was deeply icteric with ascites, hepatosplenomegaly, hyperbilirubinemia, raised transaminases, and coagulopathy. Viral markers and slit lamp examination for Kayser-Fleischer ring were negative. Serum ceruloplasmin and 24-h urinary copper post-D-pencillamine challenge were normal. Anti-smooth muscle antibody was positive 1:20, and liver biopsy showed micronodular cirrhosis with abundant Mallory hyaline and stainable copper deposits. The liver histology was indicative of Indian childhood cirrhosis, whereas the presence of autoantibodies, elevated transaminases, and increased globulin was suggestive of autoimmune hepatitis. Gene studies identified p.R969Q mutation in ATP7B gene, which solved the dilemma and confirmed the diagnosis of Wilson disease (WD). We report a clinicopathological conference of this boy to highlight the challenges faced by pediatricians in the diagnosis of Wilson disease. ᅟ.

Pancreatitis in Children.

Pancreatic disease in children has a wide clinical spectrum and may present as Acute pancreatitis (AP), Acute recurrent pancreatitis (ARP), Chronic pancreatitis (CP) and Pancreatic disease without pancreatitis. This article highlights the etiopathogenesis and management of pancreatitis in children along with clinical data from five tertiary care hospitals in south India [Chennai (3), Cochin and Pune].

Two Uncommon Paraneoplastic Neurological Syndromes in a Child With Hodgkin Lymphoma.

Paraneoplastic neurological syndromes (PNS) are rare, remote effects of cancer that are usually caused by an altered immune response to the tumor and not due to the tumor mass, metastasis, infection, ischemia, or metabolic derangements. PNSs can affect any area of the central, the peripheral, and the autonomic nervous systems. These are rare in lymphomas compared with solid tumors attributed to their presentation even in late stages and the absence of onconeural antibodies. We present a child with stage IIB Hodgkin lymphoma who presented with dual PNS, achalasia cardia, and Holmes Adie pupil occurring synchronously with the cancer.

A questionnaire survey of pediatric inflammatory bowel disease in India.

BACKGROUND: Inflammatory bowel disease (IBD) is not uncommon in children and is an important cause of morbidity. Since information on IBD in Indian children is sparse, the study aimed at highlighting the salient features in them. MATERIALS AND METHODS: A questionnaire survey was done among 221 children and adolescents with IBD [ulcerative colitis (UC) 93 (42.1 %); Crohn's disease (CD) 122 (55.2 %); unclassified (IBD-U) 6 (2.7 %)] across seven centers in India. The cut-off age was 18 years and below. RESULTS: The mean age of presentation for UC and CD was 10.2 ± 4.4 and 11.0 ± 4.5 years, respectively, with no gender difference. Diarrhea (69.9 %, p = 0.001) and blood in the stools (90.3 %, p = 0.0001) were common in UC, whereas abdominal pain (73.8 %, p = 0.01), fever (39.3 %, p = 0.0001), anemia (64.7 %, p = 0.001), and growth failure (76.2 %, p = 0.0001) were common in CD. Extraintestinal manifestations (EIM) were a feature in 23.6 % and 36.1 % of UC and CD, respectively. Pancolitis (E3) was predominant in UC (70.9 %) and 88 % required steroids. Ileocolonic CD (L3) was common in 72.9 %; 76.2 % required azathioprine for maintenance. Of the children with UC, 11.8 % had complications like massive hemorrhage and toxic megacolon, while 27 % of CD had fistulae, perianal abscess, stricture, and perforation. Biologicals were used in 0.8 % of severe UC and in 12.2 % of CD. In UC, 4.3 % required surgical intervention. CONCLUSION: Pediatric inflammatory bowel disease (P-IBD) in India shares similarities with adult-onset IBD. Distinctive features were growth failure and more severe forms of the disease necessitating immunomodulators.

Hepatic artery Doppler indices in children with extra hepatic portal vein obstruction.

UNLABELLED: Doppler measurement provides information on the hemodynamics in the hepatic artery and the portal venous system. AIM: To study the hepatic artery hemodynamics in children with extra hepatic portal vein obstruction. MATERIALS AND METHODS: Hepatic artery indices were studied using Doppler indices in 15 children (<12 years) with extra hepatic portal hypertension (EHPVO) and obliterated esophageal varices. The hepatic artery resistive index, the arterial acceleration time and the acceleration index were used to determine the flow pattern within the hepatic artery. Controls were 15 healthy age-sex matched children, belonging to the same socioeconomic strata in absence of liver disease. RESULTS: The mean age of the children was 8.43 ± 3.2 years and male female ratio was 4:1. All the children had obliterated esophageal varices. The hepatic artery resistive index in the children with EHPVO was normal and similar to controls. The hepatic arterial early systolic acceleration index was significantly higher in cases compared to controls (436 ± 290 vs 214 ± 100; P value <0.004). The hepatic arterial acceleration time though low in the cases (86 ± 35 cm/s) was not statistically different from the controls (128 ± 14 cm/s). CONCLUSION: There was a significant increase in hepatic arterial early systolic acceleration in children with chronic EHPVO. The latter may be responsible for an increase in hepatic arterial in flow velocity in a slow flow system despite a normal resistive index.

Solitary rectal ulcer syndrome: a case series.

A retrospective analysis of the clinical profile, endoscopic features and management of 22 children (age 18 months-18 years) diagnosed as solitary rectal ulcer syndrome is presented. The majority (81.8%) were ≥8 years of age. Rectal bleeding was the presenting feature in all the children. Mucorrhea, constipation, tenesmus and rectal prolapse were observed in 77.3%, 63.6%, 59% and 13.6% children, respectively. Colonoscopy showed classical single rectal ulcer in 68.2% and multiple ulcers in 22.7%. Polypoidal and erosive lesions were documented in 4.5% each. The medical management comprised of bowel training and high fibre diet for all children. The other modalities included oral 5-amino salicylate (59%), sucralfate enema (4.5%) and rectal mesalamine in 9%. 64% children recovered and 13.6% had recurrence of symptoms.

Primary intestinal lymphangiectasia.

Primary intestinal lymphangiectasia (PIL) is a rare disease of intestinal lymphatics presenting with hypoproteinemia, bilateral lower limb edema, ascites, and protein losing enteropathy. We report a series of 4 children from Chennai, India presenting with anasarca, recurrent diarrhea, hypoproteinemia and confirmatory features of PIL on endoscopy and histopathology.

Profile of viral hepatitis A in Chennai.

This retrospective study compares the clinical features, laboratory profile and complications of anti HAV IgM positive acute viral hepatitis A in 138 children between 1-15 year (1-5 year: n=31; 5-10 year: n=85; and 10-15 year: n=22). We found that older children presented with HAV (hepatitis A virus) infection with more atypical manifestations (ascites and splenomegaly) and complications.

Immunogenicity and safety of live attenuated hepatitis A vaccine: a multicentric study.

OBJECTIVE: To evaluate immunogenicity and tolerability of single dose live attenuated injectable hepatitis A vaccine in four metropolitan cities of India. METHODS: Live attenuated hepatitis A vaccine was administered to 505 children aged 18 to 60 months in four centers across India. Immunogenicity of the vaccine was assessed by estimation of anti-HAV antibody titer at 6 weeks and 6 months following administration of the vaccine. Safety evaluation of the vaccine was also done during the visits. RESULTS: At 6 weeks, 480 subjects (95%) came for the follow-up and 411 (81.4%) subjects reported at the end of 6 months. The geometric mean titer (GMT) of anti-HAV antibody of the subjects who did not have the seroprotective titer at the baseline were assessed at 6 weeks and 6 months which was 81.04 mIU/ml and 150.66 mIU/ml respectively. At 6 weeks, 95.1 % seroconverted and at the end of 6 months, 97.9 % had seroconverted. Both solicited and unsolicited vaccine-induced local and systemic adverse events were insignificant at all the centers, except swelling and induration in a few. CONCLUSION: Live attenuated injectable hepatitis A vaccine was immunogenic and tolerable with minimal reactogenecity, in this study of single dose schedule. Safety profile was also satisfactory in the study population.