Objectives: Distension of the gastrointestinal lumen is crucial for visualization and advancement during endoscopic procedures. An increasing number of pediatric centers now use carbon dioxide (CO2) preferentially over air as many adult studies and a few pediatric studies have concluded that CO2 is better tolerated than air, especially for colonoscopy. Aims: The aim of the study was to determine if CO2 is as safe and as effective as air and if it reduces abdominal discomfort and distension in children undergoing upper endoscopy and colonoscopy. Methods: Double blinded, prospective, randomized clinical study. Patient- and nursing-reported outcomes of pain and distension were recorded. End tidal CO2 (EtCO2) was monitored continuously with a CO2-sampling nasal cannula for patients undergoing procedural sedation and via the endotracheal tube for those who were intubated. Results: One hundred seventy-eight patients with 180 procedures were enrolled, 91 procedures were randomized to receive CO2, and 89 to air. Groups did not differ significantly with respect to nursing-assessed abdominal discomfort, change in girth from baseline, or endoscopist-perceived ease of inflation. Use of CO2 was associated with transient spikes in the EtCO2 (≥60 mmHg) in a significant number of patients during sedated upper endoscopy without endotracheal intubation. There was a reduction of bloating and flatulence for all procedures in the CO2 group. Conclusions: The benefits of using CO2 for insufflation were minimal in our patients. The observed transient elevations of EtCO2 during sedated upper endoscopy raise concerns of possible systemic hypercarbia. The wisdom of its routine use for all pediatric endoscopic procedures is questioned.
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Objective: The aim of the study was to determine whether clinical characteristics and management of pediatric acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) differ across INSPPIRE (INternational Study Group of Pediatric Pancreatitis: In Search for a cuRE) sites. Study Design: Data were collected from INSPPIRE and analyzed per US regions and “non-US” sites. Between-group differences were compared by Pearson chi-square test. Differences in disease burden were compared by Kruskal-Wallis test. Results: Out of the 479 subjects, 121 (25%) were enrolled in West, 151 (32%) Midwest, 45 Northeast (9%), 78 (16%) South, and 84 (18%) at non-US sites. Hispanic ethnicity was more common in South (P < 0.0001); white race in Northeast (P = 0.009). CP was less common and time from diagnosis of first acute pancreatitis to CP was longer in children at non-US sites (P = 0.0002 and P = 0.011, respectively). Genetic mutations were most common among all groups; PRSS1 variants predominated in Midwest (P = 0.002). Gallstones were more frequent in South (P = 0.002). Endoscopic retrograde cholangiopancreatography (ERCP) and computed tomography (CT) imaging were more commonly utilized in United States compared with non-United States (P < 0.0001), but there were no differences in the use of MRI/MRCP. Disease burden was highest in the West and Midwest, possibly as total pancreatectomy and islet autotransplantation (TPIAT) referral sites were located in these regions. All therapies were less commonly administered in non-US sites (P < 0.0001). Conclusions: This is the first study to describe geographical variations in the INSPPIRE cohort, which possibly reflect variations in practice and referral patterns. The underlying reason behind the lower frequency of CP and fewer treatments in non-United States sites need to be further explored.
Niemann-Pick disease type C (NPC) has been reported in association with inflammatory bowel disease. In cases where colitis has been reported in association with NPC, the neurological manifestations of NPC often precede the development of colitis. We report a rare case of a child who presented at age 2 with perianal Crohn’s disease. Initial imaging studies to characterise the disease revealed an incidental finding of splenomegaly. Extensive workup for splenomegaly revealed NPC1 mutations consistent with NPC disease. He did not have any typical neurological symptoms at the time of his diagnosis. He is currently doing well on biweekly adalimumab injections for his Crohn’s disease and biweekly intrathecal injections of 2-hydroxypropyl-β-cyclodextrin (VTS-270) for the NPC.
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