Grothe, Rayna

Objectives:Nutrition support is essential in improving outcome and survival in children on extra corporal membranous support (ECMO). We aim to evaluate the association between the timing of enteral nutrition (EN) initiation and its impact on outcome.Methods:We retrospectively reviewed the electronic health records of children (≤18 years) from November 2014 to November 2019 who were on veno‐arterial ECMO for ≥48 hours. Abstracted data included demographics, ECMO indication and duration, timing of EN initiation, change in weight‐for‐age z score (WAZ), and survival rate. The vasoactive‐inotropic score (VIS) was calculated to assess illness acuity.Results:We identified 76 children with median age (interquartile range [IQR]) of 0.3 years (0–2.6), 46 of which were infants (59%) who required ECMO for a median (IQR) of 10 days (6–22). Thirty‐six (47%) survived to hospital discharge. EN was initiated in 55 (72%) of patients while on ECMO. EN initiation by day 3 of ECMO was positively associated with survival (P = 0.0438). VIS at the time of EN initiation was lower in surviving infants (P = 0.022). Children who achieved enteral autonomy were more likely to survive (P = 0.0024). Survivors had greater WAZs at ECMO completion (P = 0.0004).Conclusions:Initiation of EN by day 3 of ECMO and at a lower VIS is associated with greater likelihood of survival.

Mutations in the hepatocyte nuclear factor‐1‐beta (HNF1B) gene cause a variety of diseases in different organ systems. Mutations have been described as causing neonatal cholestasis, maturity‐onset diabetes of the young (type 5), cortical renal cysts, urogenital abnormalities, liver dysfunction, and atrophy of the pancreas. We describe a male patient who presented with cholestatic liver disease in infancy which progressed by age 14 to end‐stage liver disease due to HNF1B disease. He subsequently underwent liver transplantation at age 15 and then developed diabetes requiring insulin which did not resolve after cessation of corticosteroids. To our knowledge, this is the first case reported of liver transplantation for decompensated cirrhosis secondary to HNF1B disease.

ABSTRACTBackground:The association between celiac disease (CD) and eosinophilic esophagitis (EoE) has been the focus of multiple studies with variable results. Both diseases are immune mediated, and dietary triggers play a role in their pathogenesis.Objectives:The aim of the study was to analyze the risk of EoE in children with CD, assess the magnitude of association between CD and EoE in children, and report the characteristics and outcomes of children with both conditions.Methods:We conducted a retrospective study of the Mayo Clinic Electronic medical records between January 1, 1998 and December 31, 2015. Systematic review and meta‐analysis of multiple databases was conducted to include studies reporting on the same association. Random‐effects model was used to report pooled odds ratio (OR) and 95% confidence interval (CI).Results:In this cohort study, of 10,201 children who underwent at least 1 endoscopy, 595 had EoE, and 546 had CD. The risk of having EoE was not increased in children with CD compared to those without CD (OR, 0.29; 95% CI, 0.154–0.545). Nine of 10 children improved with gluten‐free diet, topical glucocorticosteroid, and/or elimination diet. One child lost to follow‐up.Meta‐analysis of 5 studies showed similar results (OR, 0.525; 95% CI, 0.364–0.797). A total of 45 cases in the literature had both CD and EoE (mean age, 10 years; 64% boys; majority presenting with abdominal pain, vomiting, and diarrhea).Conclusions:Based on our cohort and the observational data, the diagnosis of CD in children is not associated with increased risk of EoE.

Abstract Background: The existence, etiology, diagnosis, and treatment of median arcuate ligament syndrome (MALS) have long been subjects of debate. To our knowledge, there have not been any studies assessing the effectiveness of surgical treatment in improving physical and psychological quality of life in pediatric patients. Subjects and Methods: This is an Institutional Review Board–approved prospective study including all patients undergoing surgical treatment of MALS between 2009 and 2012 at our institution. Demographic information, presenting symptoms, radiological imaging, procedure duration, hospital length of stay, and perioperative complications were gathered for analysis. Patients and their parents were asked to complete the Child Health Questionnaire, a physical and psychological health survey, both within 1 week prior to and at least 3 months following their surgery. Results: Six patients underwent laparoscopic release for MALS. The majority of patients were female ( n =5 [83.3%]), with an average age of 15.7±1.5 years. Presenting symptoms lasted on average 16.5±12.7 months prior to treatment. Average pre- and postsurgical ultrasound celiac artery peak velocities with inspiration were 332.0±34.1 cm/second and 224.3±31.2 cm/second, respectively, with a statistically significant decrease of 107.67 cm/second ( P =.03). The average follow-up period from time of surgery to time of quality of life survey completion was 13±11.3 months, with a range of 3–29 months. A significant improvement from pre- to postsurgical scores was observed in the physical functioning ( P =.03), mental health ( P =.03), and self-esteem categories ( P =.03) of the child assessment. Similarly, there was a significant postsurgical improvement in all categories pertaining to the parent's quality of life ( P =.03). Improvement was also seen in the parents' perception of their child's physical functioning ( P =.03), bodily pain/discomfort ( P =.03), mental health ( P =.03), and general health perceptions ( P =.03). No intraoperative or postoperative complications occurred. Conclusions: Our preliminary results demonstrate that laparoscopic median arcuate ligament release for MALS in the pediatric population is safe and effective and improves overall quality of life for the patients and their parents. In carefully selected patients, laparoscopic release for MALS without additional celiac artery reconstruction normalizes blood flow in the celiac artery and improves physical and psychosocial quality of life for the child and his or her parents.

ABSTRACTObjective:To describe the relationships between gastric emptying, autonomic function, and postural tachycardia in adolescent patients with nausea and/or abdominal discomfort. It was hypothesized that patients with both gastrointestinal symptoms and symptoms of orthostatic intolerance are more likely to show abnormal tilt table results and delayed gastric emptying.Patients and Methods:A retrospective review was conducted of adolescent patients who came to a pediatric referral center because of nausea and dyspepsia and who subsequently underwent both autonomic reflex screening and gastric emptying testing. Patients with a heart rate change of 30 or more beats per minute on the heads‐up tilt table test were assigned to the postural orthostatic tachycardia syndrome (POTS) group (n = 21), and those with a heart rate change of fewer than 30 beats per minute on the heads‐up tilt table test were assigned to the non‐POTS group (n = 10).Results:There was no significant difference between the POTS and non‐POTS groups with regard to presenting symptoms (P > 0.05). Overall, 13 (42%) individuals had abnormal gastric emptying results (delayed in 6, accelerated in 7), but gastric emptying scores were similar between the POTS and non‐POTS groups. Furthermore, there was no correlation between an individual's gastric emptying results at 1, 2, and 4 hours and that person's heart rate change on HUT (r = −0.05, −0.15, and −0.19).Conclusions:Although altered gastric emptying and postural tachycardia are common in a referral population of adolescents with nausea and/or abdominal discomfort, the clinical presentation was not predictive of test results. Furthermore, delayed gastric emptying was not correlated with the current definition of postural tachycardia.