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BACKGROUND Paediatric inflammatory bowel disease (PIBD) is increasing across the world. However, information from India is sparse. This multicentre study evaluated the demographics, clinical phenotype and outcome of PIBD from India. METHODS Data of children (≤18 years) with PIBD were collected using a proforma containing details of demographics, clinical profile, extraintestinal manifestations (EIM), investigations, disease extent and treatment. RESULTS Three hundred twenty-five children [Crohn's disease: 65.2%, ulcerative colitis: 28.0%, IBD unclassified (IBDU): 6.7%, median age at diagnosis: 11 (interquartile range 6.3) years] were enrolled. 6.9% children had family history of IBD. Pancolitis (E4) was predominant in ulcerative colitis (57.8%) and ileocolonic (L3, 55.7%) in Crohn's disease. Perianal disease was present in 10.9% and growth failure in 20.9% of Crohn's disease cases. Steroids were the initial therapy in 84.2%, 5-amino salicylic acid in 67.3% and exclusive enteral nutrition (EEN) in 1.3% cases. Overall, immunomodulators and biologics were given to 84.3 and 17.9% cases, respectively, and 2.9% cases underwent surgery. Very early onset IBD (VEOIBD) was seen in 60 (19.2%) children. IBDU was commoner in the VEOIBD than the older-PIBD (18/60 vs 4/253; P < 0.001). VEOIBD-Crohn's disease patients more often had isolated colonic disease than the older Crohn's disease (45.4% vs 11.8%; P < 0.001). Prevalence of perianal disease, EIM, therapeutic requirements and outcome were not different between VEOIBD and older-PIBD. CONCLUSION Disease location and phenotype of PIBD in Indian children is similar to the children from the west. However, the therapeutic options of EEN, biologics and surgery are underutilized. VEOIBD accounted for 19.2% of PIBD.

OBJECTIVES: Gamma-glutamyl transferase levels (GGT) are typically elevated in biliary atresia (BA), but normal GGT levels have been observed. This cohort of "normal GGT" BA has neither been described nor has the prognostic value of GGT level on outcomes in BA. We aimed to describe outcomes of a single-centre Australian cohort of infants with BA and assess the impact of GGT level at presentation on outcomes in BA. METHODS: Infants diagnosed with BA between 1991 and 2017 were retrospectively analysed. Outcomes were defined as survival with native liver, liver transplantation (LT), and death. Patients were categorized into normal (

Objectives A significant proportion of children with Crohn disease develop a secondary loss of response (LOR) to infliximab. Our aim was to study the impact of initial treatment strategies on secondary LOR. Methods We reviewed the medical records of children with Crohn disease who received scheduled maintenance infliximab therapy for at least 12 months. We compared children who developed LOR with those who did not; with regards to their clinical and laboratory parameters, disease phenotype, and treatment strategy before developing LOR. Results A total of 73 children (median age at diagnosis 11 (2-16) years, 41 boys) who had received a median duration of 33 (13-110) months of infliximab therapy were included in the final analysis. LOR was seen in 25(34.2%). Demographic variables, disease phenotype (age, disease location, and behavior), inflammatory parameters, and pediatric Crohn disease activity index at induction with infliximab were similar between both groups. Children with LOR had a significantly greater number of flares of the disease when compared to those who did not have LOR (4 [1-8] vs 2 [1-5] P = 0.03). The choice of the concomitant immunomodulator-methotrexate (11/29 [37.9%]) versus azathioprine (11/36 [30.5%]) (P = 0.6) did not affect LOR rates. The median time-lag between diagnosis and induction with infliximab was significantly longer in children with LOR as compared to those who did not have an LOR (28 [4-90] months vs 12.5 [1-121] months, P = 0.004). Conclusion Early use of infliximab in pediatric Crohn disease is associated with a decrease in secondary LOR. The type of concomitant immunomodulator used does not make a difference to LOR rates.

Liver biopsy is a valuable tool. Even though outpatient liver biopsies are routinely performed in adults, there are no clear recommendations on its safety and feasibility in children. We reviewed the records of children who underwent a percutaneous liver biopsy at our institution between January 2005 and June 2015. A total of 626 biopsies were performed on 497 patients (250 boys, median age 6 [0.2-19.3] years). Abnormal liver function tests for investigation 288 (46%) was the most common indication. Thirty (4.8%) biopsies had complications, most common being subcapsular hematoma 14 of 30 (46.7%). Complications were identified within 8 hours of the biopsy in all patients. Approach, needle size, or number of passes did not affect the complication rate. Bleeding-related complications were not related to the international normalized ratio. Performing liver biopsies on an outpatient basis would have saved 60 beds/AU$ 80,000/year. The present study suggests that if an observation period of 8 hours is instituted, outpatient liver biopsies can be performed safely in children.

Recent years have witnessed an increase in acute pancreatitis (AP) in children; however, the natural history of acute fluid collection (AFC) and pseudocyst is largely unknown. We evaluated the frequency, clinical characteristics, and natural history of pseudocysts in children with AP.Children with AP admitted at Sanjay Gandhi Postgraduate Institute of Medical Sciences from 2001 to 2011 were enrolled and studied until complete resolution. Subjects with inadequate follow-up, recurrent AP, and chronic pancreatitis were excluded.Of the 58 children (43 boys, median age 14 [1-18] years) with AP, 34 (58.6%) and 22 (38%) developed AFC and pseudocyst, respectively. No difference in age (12 [4-18] vs 13 [1-16] years), etiology (idiopathic 64% vs 47% and traumatic 27.2% vs 22.2%), and systemic complications (pulmonary [18% vs 11%], renal [22.7% vs 11%], and shock [13.6% vs 10%]) was observed between children with and without pseudocyst. A total of 11 of the 22 subjects with pseudocyst underwent drainage, the commonest symptom requiring drainage being gastric outlet obstruction [n = 5] and infection [n = 2]. The 11 of the 22 children with AP and pseudocyst (size 6.4 [3-14.4] cm) showed spontaneous resolution (disappearance [n = 9] and significant reduction in size [n = 2]) during 110 (25-425) days. Symptomatic pseudocysts requiring drainage were more often secondary to traumatic AP (6/6 vs 2/14 [idiopathic], P = 0.0007) than asymptomatic pseudocysts resolving spontaneously. Overall, only 26.4% (9/34) children with AFC required drainage because of symptomatic pseudocyst.Among children with AP, 58.6% developed AFC and 38% developed pseudocysts. Only patients with symptomatic pseudocyst need drainage, and asymptomatic pseudocyst can be safely observed irrespective of size and duration of collection.

We report 3 children who presented with fever and abdominal pain, deranged liver function tests, and on abdominal ultrasound were found to have an enlarged pancreas, substantial abdominal lymphadenopathy, and extrahepatic biliary duct dilatation. After ruling out malignancy, probable immunoglobulin G4-related disease (IgG4RD) associated with autoimmune pancreatitis was considered. This condition was first described in the adults and often mimics pancreatic cancer. It can involve multiple organs, either synchronously or metachronously, and is rarely reported in children. The disorder mostly responds to corticosteroid therapy and other immune suppression. We highlight the difficulty in diagnosing autoimmune pancreatitis/IgG4-related disease in children and illustrate the difference between pediatric and adult presentation.