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

Objective: Intestinal lymphangiectasia (IL; primary or secondary) is an important cause of protein-losing enteropathy. We evaluated the clinicolaboratory profile, response to therapy, complications, and outcome of children with primary IL (PIL). Methods: Consecutive children (≤18 years) diagnosed with PIL (clinical setting, typical small bowel histopathology, and exclusion of secondary causes) from 2007 to 2017 were evaluated. Results: Twenty-eight children with PIL (16 boys, age at symptom onset-12 [1–192] months and at diagnosis 8 [1–18] years) were studied. Pedal edema (93%), chronic diarrhea (78.6%), and recurrent anasarca (64%) were the common presentations. Ascites, pleural, and pericardial effusion were seen in 64 (n-18; chylous-5, non-chylous-13), 18, and 18% cases, respectively. Hypoproteinemia, hypoalbuminemia, hypocalcemia, and lymphopenia were present in 82, 82, 75 and 39% cases, respectively. Duodenal biopsy established the diagnosis in 86% cases, while 14% required distal small bowel biopsies. Dietary therapy was given in all and 6 cases required additional therapy (octreotide-6, tranexamic acid-3, and total parenteral nutrition-1). Lymphedema (3/5 vs. 1/23), pleural effusion (4/5 vs. 1/23), and the need for additional therapy (4/5 vs. 2/23) were significantly more in patients with chylous ascites (n = 5) than those without chylous ascites (n = 23). Twenty-four cases in follow-up (39 [6–120] months) showed improvement; however, 8 required readmission (symptom recurrence-6 [25%], complication-2 [8.3%], Budd Chiari Syndrome-1, and abdominal B cell lymphoma-1). Conclusion: Presence of chylous ascites suggests severe disease in children with PIL. Majority of PIL children respond to dietary therapy; only 20% need additional therapy. Long-term follow-up is essential to monitor for symptoms relapse and complications.

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Background Portal cavernoma cholangiopathy (PCC), a surgical-endoscopic dilemma, has not been studied comprehensively, more so in children. Our study aimed to evaluate PCC in children using a combination of magnetic resonance cholangiography-portovenography (MRC-MRPV) and endoscopic ultrasonography (EUS). Methods In this prospective cross-sectional study, recruited children with extrahepatic portal venous obstruction (EHPVO) underwent MRC-MRPV and radial array EUS. PCC was categorized as asymptomatic PCC, symptomatic and no-PCC. Modified Llop grading was used to grade the MRC changes.Results Sixty-six of 72 (92%) children had PCC (85% asymptomatic; 7% symptomatic) on MRC. Age at study and duration of disease had significant correlation (r = 0.588, P < 0.001). 63% had grade III MRC changes. MRC grades and superior mesenteric vein block (64%) on MRPV significantly corresponded with EUS changes (intracholedochal varices, choledochal perforators, intramural cholecystic collaterals and biliary calculi). Superior mesenteric vein non-patency was a strong predictor of MRC biliary changes (P = 0.003, odds ratio 46.4, 95% confidence interval 4.91-623.6). Conclusions A majority of EHPVO children have asymptomatic cholangiopathy and should be routinely evaluated for PCC at the time of first presentation by MRC-MRPV. Additional superior mesenteric vein block with portal cavernoma results in significantly higher changes of cholangiopathy on MRC and EUS.

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Objectives: Solitary rectal ulcer syndrome (SRUS) is said to be rare in children (largest series so far; 55 in children, 116 in adults). We analyzed our experience to look at its clinical presentations, endoscopic appearance, and treatment outcome in a large cohort of children. Methods: Clinical and endoscopic data were collected between 2000 and 2018. Children (18 years or younger) diagnosed to have SRUS on colonoscopy and confirmed by histopathology were included. All children with SRUS were treated with behavioral modification, bulk laxative. Most with ulcer received steroid enema and some sulfasalazine or sucralfate enema. Results: The median age of 140 children was 12 (interquartile range [IQR]: 10–14) years, 79% were boys. The median symptom duration was 21 (IQR: 9–36) months. Rectal bleeding was the presenting feature in 131 (93.6%); constipation in 38 (27%); and small, frequent stools in 79 (56%). Most children had features of dyssynergic defecation such as prolonged sitting in the toilet (131, 93.6%), excessive straining (138, 98.6%), a feeling of incomplete evacuation (130, 92.8%), and rectal digitation (71, 50.7%). Rectal prolapse was noted in 24 (17%) cases. Colonoscopy documented rectal ulcer in 101 (72%) [Single: 84]. Over a median follow-up of 6 (IQR: 4–18) months, 27 patients were lost to follow-up and of the remaining 113 cases, 71 (62.8%) showed clinical improvement (healing of ulcer documented in 36/82, 44%). Conclusions: The majority of cases of SRUS presented in second decade with rectal bleeding and features of dyssynergic defecation. Ulcer was noted in three fourths of cases. The outcome of medical treatment with behavioral modification and local therapy was modest.

Objectives: Polyethylene glycol (PEG) is the most effective colon-cleansing agent but volume-related adverse effects are common. Though split-dose PEG is used in adults, no pediatric study so-far has compared split-dose with single-dose PEG. We aimed at comparing the efficacy and tolerability of split-dose versus single-dose PEG for bowel preparation in children. Methods: Consecutive children (1–18 years) were randomized into either single-dose or split-dose PEG. Single-dose group received 4000 mL/1.73 m2 PEG solution day before colonoscopy whereas split-dose group received half dose day before and the remaining half on the day of colonoscopy. Effectiveness of bowel preparation was assessed on Aronchik scale, by the endoscopist who was blinded to the type of preparation. Interobserver variability was analyzed by comparing with independent scoring by the blinded trained endoscopy-nurse. The trial was registered with Clinical Trials Registry of India (Trail number 2017/08/009303). Results: Of the 220 randomized children, 179 completed the study (split-dose: 93, single-dose: 86). The mean age of the study population was 11.51 (4.82) years (72.6% boys). The efficacy of bowel preparation was better with split-dose (satisfactory preparation:76.34% vs 43.02%, P < 0.001) with almost perfect inter-observer agreement (k = 0.803). Nausea, vomiting, and sleep disturbance were significantly less in split-dose than single-dose group (P < 0.05). Split-dose patients were able to drink PEG solution faster (P = 0.002). Total sleep duration and uninterrupted sleep duration was also better in split-dose group as compared with single-dose (P = 0.001). Conclusions: Split-dose PEG is more effective than single-dose regimen for bowel preparation with better tolerability and improved sleep quality in pediatric population.

Background/Objective: Percutaneous biopsy (PB) and transjugular liver biopsy (TJLB) are 2 main ways of obtaining liver tissue. We evaluated the indications, success rate, tissue yield, and complications of TJLB in comparison to PB in children. Methods: Electronic records of children undergoing liver biopsy (LB) were reviewed. Clinico laboratory data including indication, type of biopsy, complications, and tissue yield (length and number of complete portal tracts [CPT]) were noted. Results: Five hundred forty LB (indication: neonatal cholestasis [42.9%], chronic liver disease [43.7%], liver failure [3.7%], focal lesions [3.3%] and others [6.3%]) were done. Four hundred seventy-three were PB (317 boys, 14 [1--216] months) done by percussion (322 [68%]), real-time ultrasound guidance (125 [26.4%]), or plugged method [26 (5.5%)]. Sixty-seven (12.4%) were TJLB [38 boys, 140 (24--216) months], done in patients with contraindications for PB. Technical success (67/68 vs 473/473; P = 0.7) and complications (4 [6%]; vs 15 [3.3%]; P = 0.2) of TJLB and PB were similar. Major complications (0.5%) included supraventricular tachycardia (n = 1) in TJLB and hemoperitoneum (n = 2) in PB. Tissue yield of TJLB was poorer in terms of length (1.0 [0.2--2.0] vs 1.1 [0.4--2.1] cm; P < 0.001), CPT (4 [0--9] vs 5 [2--17]; P < 0.001) and adequacy for reporting (56/67 vs 459/473; P < 0.001). Biopsy yield of <6 CPT was predicted by cirrhosis at histology and TJLB. No factor identified risk of complications with LB. Conclusions: LB is a safe procedure and only 12% children require TJLB because of contraindications of PB. Technical success and complications are similar but tissue yield is poorer in TJLB than PB. Presence of cirrhosis and TJLB adversely affected tissue yield.