Mesenteric plexiform neurofibroma is a subtype of plexiform neurofibroma that involves the mesentery and causes a variety of gastrointestinal complaints. Plexiform neurofibroma is classically found in patients with neurofibromatosis type 1, although genetic contributions to plexiform neurofibroma pathogenesis are heterogeneous. We report the first case of mesenteric plexiform neurofibroma in a patient with a YPEL3 pathogenic variant. This patient presented with growth failure, generalized abdominal pain and chronic diarrhea. She was confirmed to have mesenteric plexiform neurofibroma on histopathology and targeted sequencing on affected tissue confirmed that there were no neurofibromatosis type 1 variants present. Given that this patient's mesenteric plexiform neurofibroma is associated with YPEL3 dysfunction, she is unlikely to benefit from MEK inhibitors, which are the newly approved treatment for inoperable plexiform neurofibroma in patients with neurofibromatosis type 1.

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Background: Pain is the most common symptom of acute pancreatitis (AP), and opioids have been utilized as the cornerstone of treatment. Despite the adverse effects of opioids, data on effective analgesia in children with AP is lacking. We aimed to evaluate analgesia prescribing patterns in pediatric AP, identify factors associated with opioid administration, and test the associations between opioid administration and hospital length of stay (LOS). Methods: This is a retrospective cohort study of pediatric AP hospitalizations in a single institution from 2010 to 2020. Opioid administration was calculated for the first 48 hours of admission (morphine milligram equivalent; MME48). Data on multimodal analgesia [defined as the administration of acetaminophen and nonsteroidal anti-inflammatory drugs (NSAIDs)] during hospitalization was captured. Results: The sample included 224 patients, mean age 12.0 years (standard deviation = 4.9) and 58.9% female. Median LOS was 4 days (interquartile range 2-9). Most patients (71.4%) were prescribed opioids, 77.7% acetaminophen, 40.2% NSAIDs, and 37.5% multimodal analgesia. Opioid administration decreased over the study period; in contrast, there was an increase in multimodal analgesia administration. Opioid administration did not differ by sex, age, biliary versus non-biliary etiology, or race/ethnicity. In a multivariate regression model, lower albumin values (P < 0.01) and younger age (P < 0.05) were significant predictors of increased LOS, while MME48 was not associated with increased LOS. Conclusions: Opioids were commonly administered; only 37.5% of children were administered multimodal analgesia during their hospitalization for AP. Opioid administration was not associated with increased LOS. Prospective studies are needed to determine optimal pain management for pediatric AP.

Background Pain is a predominant symptom of inflammatory bowel disease (IBD), and is influenced by cognitive, emotional and behavioral factors. The cognitive-affective model of symptom appraisal (CAMSA) has been used to understand how youth view symptoms in chronic conditions. We sought to 1) determine how youth with IBD and their parents appraise pain, and how their perspectives fit within CAMSA, 2) explore health care providers’ understanding and communication about pain. Methods Participants included 19 youth ages 10-17 years with chronic IBD pain and their parents, and 5 IBD providers from a gastroenterology clinic. Separate semi-structured qualitative interviews with youth, parents, and providers were conducted. Interview prompts were adapted from CAMSA, previous studies of pediatric pain and symptom monitoring, and a qualitative study in adults with IBD pain. Interviews were analyzed according to principles of reflexive thematic analysis. Results Three key components of CAMSA (IBD Threat, Fear/Worry, Biased Attending) were identified in youth and parent dyads. Some youth showed Biased Attending, including difficulty disengaging, while other youth simply monitored pain. The overarching theme for provider interviews was Gastroenterologists view pain as a secondary (rather than primary) treatment issue. Conclusions CAMSA is potentially applicable to pain appraisal in youth with IBD and their parents. When health care providers communicate about pain, they should consider how symptom uncertainty may be influenced by threat, fear/worry, and biased attending. Further studies are needed to develop and test psychosocial interventions to reduce fear and threat of pain in youth with IBD in partnership with families and providers.