Avoidant/restrictive food intake disorder prevalence is high in children with gastroparesis and functional dyspepsia.

BACKGROUND: Avoidant/restrictive food intake disorder (ARFID) prevalence in children with gastroparesis (Gp) and/or functional dyspepsia (FD) is unknown. We aimed to identify ARFID prevalence and trajectory over 2 months in children with Gp, FD, and healthy children (HC) using two screening questionnaires. We also explored the frequency of a positive ARFID screen between those with/without delayed gastric emptying or abnormal fundic accommodation. METHODS: In this prospective longitudinal study conducted at an urban tertiary care hospital, patients ages 10-17 years with Gp or FD and age- and gender-matched HC completed two validated ARFID screening tools at baseline and 2-month follow-up: the Nine Item ARFID Screen (NIAS) and the Pica, ARFID, and Rumination Disorder Interview-ARFID Questionnaire (PARDI-AR-Q). Gastric retention and fundic accommodation (for Gp and FD) were determined from gastric emptying scintigraphy. KEY RESULTS: At baseline, the proportion of children screening positive for ARFID on the NIAS versus PARDI-AR-Q was Gp: 48.5% versus 63.6%, FD: 66.7% versus 65.2%, HC: 15.3% versus 9.7%, respectively; p < 0.0001 across groups. Of children who screened positive at baseline and participated in the follow-up, 71.9% and 53.3% were positive 2 months later (NIAS versus PARDI-AR-Q, respectively). A positive ARFID screen in Gp or FD was not related to the presence/absence of delayed gastric retention or abnormal fundic accommodation. CONCLUSIONS & INFERENCES: ARFID detected from screening questionnaires is highly prevalent among children with Gp and FD and persists for at least 2 months in a substantial proportion of children. Children with these disorders should be screened for ARFID.

The Role of Pediatric Gastroenterologists in the Evaluation of Complex Aerodigestive Disorders.

PURPOSE OF REVIEW: Children with aerodigestive disorders frequently have concerns regarding difficulty breathing, swallowing, and growing. In this review, we explored the role of pediatric gastroenterologists in the evaluation of complex aerodigestive disorders and the overall approach to these often-challenging patients. RECENT FINDINGS: Pediatric gastroenterologists evaluate children with aerodigestive concerns ranging from dysphagia and gastroesophageal reflux to complex congenital abnormalities such as esophageal atresia. Diagnostic tools, such as multichannel intraluminal impedance-pH monitoring, are used for diagnosing gastroesophageal reflux and assessing the correlation with symptoms. Endoscopic evaluation, and in some complex cases, with therapeutic dilations may also be performed. Gastrointestinal dysmotility evaluation with manometry studies are also being increasingly utilized. Multidisciplinary aerodigestive programs can provide a coordinated approach to children with complex airway, pulmonary and gastrointestinal tract disorders. A pediatric gastroenterologist's expertise and specialized skills not only offer many diagnostic tools for these complicated medical cases but are also important in long term medical management.

Pediatric Rome IV diagnosis agreement is greater than agreement on diagnostic testing.

BACKGROUND: Pediatric Rome IV criteria are used to diagnose childhood functional gastrointestinal disorders (FGIDs). This study of pediatric gastroenterology physicians measured their agreement in (1) Making a pediatric Rome IV FGID diagnosis; and (2) Diagnostic testing for patients with FGIDs. METHODS: Pediatric gastroenterologists and pediatric gastroenterology fellows at two medical centers completed a survey containing clinical FGID vignettes. For each vignette, raters identified the most likely Rome IV diagnosis(es) and selected which diagnostic test(s) (if any) they typically would obtain. The survey was re-administered within 3 months. Inter-rater and intra-rater weighted percent agreement was determined. Linear mixed modeling identified sources of variability in diagnostic testing. KEY RESULTS: Thirty-four raters completed the initial survey of whom thirty-one (91%) completed the repeat survey. Overall inter-rater agreement on Rome IV diagnoses was 68% for initial and repeat surveys whereas intra-rater agreement was 76%. In contrast, overall inter-rater agreement on diagnostic testing was <30% for both initial and repeat surveys and intra-rater agreement was only 57%. Between-physician differences accounted for 43% of the variability in the number of tests selected. Rater identified use of Rome criteria in clinical practice was associated with 1.1 fewer diagnostic tests on average (95% CI 0.2-2.0, p = 0.015). Higher intra-rater agreement was noted for diagnostic testing in faculty when compared to fellows (p = 0.009). CONCLUSIONS & INFERENCES: In a multicenter evaluation among pediatric gastroenterology physicians, pediatric Rome IV diagnostic agreement was higher than that reported for previous Rome versions, and higher than agreement on diagnostic testing.

Gastrojejunal Enteral Tube Serving as a Small Bowel Bezoar Nidus.

Gastrojejunal (GJ) tube placement is indicated in the management of gastric feeding-related intolerance. Though uncommon, GJ complications may occur. We present the case of a five-year-old male with congenital heart disease in which image-guided replacement of a GJ tube was unable to be completed due to a mass adhered to the tip of the tube. The subsequent endoscopic evaluation identified the mass as a hair-based bezoar and the tube was successfully removed. The child was subsequently diagnosed with trichotillomania, trichophagia, and pica. This case illustrates the importance of recognizing bezoar formation as a potential complication of GJ enteric tubes, particularly in children with trichophagia and pica.

Assessment of Quality and Readability of Internet Dietary Information on Irritable Bowel Syndrome.

Diet plays an important role for patients with irritable bowel syndrome (IBS). For medical conditions such as IBS, the Internet is a primary source of health information.1 However, recent evidence suggests that Internet health information may have several flaws including being extremely discrepant, of poor quality, and inaccurate.2 Therefore, our objectives were to evaluate both the quality and reading level of Internet dietary recommendations for both pediatric and adult IBS.

Use of gabapentin and pregabalin for pruritus and neuropathic pain associated with major burn injury: A retrospective chart review.

INTRODUCTION: Pruritis after burn is one of the most common chronic complaints in burn survivors. Pruritus is often indistinguishable from neuropathic pain. There is a paucity of studies reporting the use of gabapentin and pregabalin to treat both pruritus and neuropathic pain. The purpose of this current study is to explore and document the effect of gabapentin and pregabalin in children and adolescent burn survivors. METHODS: A retrospective review of charts and pharmacy records of gabapentin and pregabalin dispensed to control pruritus and/or pain was conducted for burn survivors up to 20 years of age. Data collected included medication doses, age and weight of patients, presence of neuropathic pain and pruritus, reported response to medication, and side effects of these medications. 136 individuals who received gabapentin, pregabalin, or both medications are included in the study. 112 received only gabapentin, none received only pregabalin, and 24 received both. All results are documented in mean±standard deviation (s.d.) dose/kg/day. 104 individuals experienced pruritus exclusively, two experienced neuropathic pain exclusively, and 30 experienced both. Use of medications was considered effective if the individuals reported pruritus or pain relief from the medication. The medication was considered safe if the individuals did not experience adverse side effects warranting discontinuation of the drugs. Medications were continued with dose adjustments if an individual reported minor side effects such as sedation or hyperactivity. RESULTS: The average effective dose mg/kg/day for gabapentin and pregabalin was calculated for each of the three age groups (≤5years, 6-12 years, and >12years). The average effective dose of gabapentin was 23.9±10.3mg/kg/day for children ≤5years, 27.0±15.3mg/kg/day for children 6-12 years, and 34.1±15.7mg/kg/day for children >12years. The average effective dose of pregabalin was 6.5±3.5mg/kg/day for children 6-12 years and 4.7±1.6mg/kg/day for children >12years. One 5-year-old child received 3.7mg/kg/day of pregabalin. Note that for all patients in this study, pregabalin was added after an inadequate response to gabapentin. For individuals receiving both gabapentin and pregabalin, the maximum gabapentin failure dose for pruritus was 32.8±18.0mg/kg/day and for both pain and pruritus was 28.1±18.3mg/kg/day. For individuals treated with only gabapentin, 91.4% had an adequate response for pruritus, 100% for neuropathic pain, and 43.3% for both pruritus and pain. 100% of individuals treated with both gabapentin and pregabalin had an adequate response for pruritus and 88.2% had an adequate response for both pruritus and pain. Gabapentin was associated with hyperactivity in two individuals, and sedation in one individual. One individual reported nausea, vomiting, and headaches when taking both medications; this resolved when gabapentin was discontinued. One individual reported sedation while taking both medications. CONCLUSION: Gabapentin and pregabalin are effective in relieving pruritus and neuropathic pain in most burn survivors. In some instances, these medications can be given together. Few individuals reported side effects.

Preclinical therapeutic efficacy of a novel blood-brain barrier-penetrant dual PI3K/mTOR inhibitor with preferential response in PI3K/PTEN mutant glioma.

Glioblastoma (GBM) is an ideal candidate disease for signal transduction targeted therapy because the majority of these tumors harbor genetic alterations that result in aberrant activation of growth factor signaling pathways. Loss of heterozygosity of chromosome 10, mutations in the tumor suppressor gene PTEN, and PI3K mutations are molecular hallmarks of GBM and indicate poor prognostic outcomes in many cancers. Consequently, inhibiting the PI3K pathway may provide therapeutic benefit in these cancers. PI3K inhibitors generally block proliferation rather than induce apoptosis. To restore the sensitivity of GBM to apoptosis induction, targeted agents have been combined with conventional therapy. However, the molecular heterogeneity and infiltrative nature of GBM make it resistant to traditional single agent therapy. Our objectives were to test a dual PI3K/mTOR inhibitor that may cross the blood-brain barrier (BBB) and provide the rationale for using this inhibitor in combination regimens to chemotherapy-induced synergism in GBM. Here we report the preclinical potential of a novel, orally bioavailable PI3K/mTOR dual inhibitor, DS7423 (hereafter DS), in in-vitro and in-vivo studies. DS was tested in mice, and DS plasma and brain concentrations were determined. DS crossed the BBB and led to potent suppression of PI3K pathway biomarkers in the brain. The physiologically relevant concentration of DS was tested in 9 glioma cell lines and 22 glioma-initiating cell (GIC) lines. DS inhibited the growth of glioma tumor cell lines and GICs at mean 50% inhibitory concentration values of less than 250 nmol/L. We found that PI3K mutations and PTEN alterations were associated with cellular response to DS treatment; with preferential inhibition of cell growth in PI3KCA-mutant and PTEN altered cell lines. DS showed efficacy and survival benefit in the U87 and GSC11 orthotopic models of GBM. Furthermore, administration of DS enhanced the antitumor efficacy of temozolomide against GBM in U87 glioma models, which shows that PI3K/mTOR inhibitors may enhance alkylating agent-mediated cytotoxicity, providing a novel regimen for the treatment of GBM. Our present findings establish that DS can specifically be used in patients who have PI3K pathway activation and/or loss of PTEN function. Further studies are warranted to determine the potential of DS for glioma treatment.