A phase II study of multiparametric MR-guided high-dose response-adaptive radiotherapy with concurrent temozolomide in patients with newly diagnosed glioblastoma: Results from an interim analysis.

Background: Biologically-informed radiotherapy (RT) targeting an adversely prognostic hypercellular/hyperperfused imaging phenotype in patients with newly diagnosed glioblastoma (GBM) may improve outcomes by identifying emerging regions of treatment resistance associated with survival (OS), and is under investigation in an ongoing Phase II trial (XXX) of individualized, response-adaptive RT.

Methods: In this single-arm phase II study, patients with newly diagnosed GBM following resection undergo dose-intensified chemoradiation (chemoRT) targeting the residual hypercellular (TV, 2 SD above mean intensity contralateral normal brain) and hyperperfused tumor volume (TV, 1 SD above contralateral normal frontal lobe grey matter) identified using high b-value diffusion-weighted and dynamic contrast-enhanced perfusion MRI. The combination of TV and TV (TV/TV) is treated to 50 Gy in 20 fractions (2.

Illness understanding and religiousness in patients with recurrent glioblastoma.

Background: Patients with glioblastoma (GBM) often have inaccurate perceptions of prognosis. Strong religious beliefs have been associated with limited illness understanding (IU) in patients with advanced cancer, but IU and religiousness have not been investigated in patients with GBM. The aim of this study was to evaluate the association between religiousness and spirituality and IU in patients with GBM.

Prospective assessment of end-of-life symptoms and quality of life in patients with high-grade glioma.

Background: Glioblastoma and high-grade glioma (HGG) remain non-curable diseases. Symptoms and Quality-of-life (QoL) in the end-of-life (EoL) phase have not been prospectively studied with validated instruments. Therefore, we prospectively assessed symptom progression, symptom management, and hospice utilization in patients with treatment-refractory progressive HGG.

Somatic, emotional, and gastrointestinal symptom severity are increased among children and adolescents with COVID-19.

Background: Post-infectious disorders of gut-brain interaction (PI-DGBI) have significant impact on children and adolescents. The effect of COVID-19 on PI-DGBI-associated symptoms in this population, however, is unknown.

Methods: We performed electronic medical record searches to identify patients 8-17 years old with a SARS-CoV2 PCR test at Lurie Children's Hospital between November 2020 and March 2021 (cohort 1) and April-October 2021 (cohort 2).

Intrapyloric Botulinum Toxin Injection for Refractory Nausea and Vomiting in Pediatric Patients.

Objectives: Chronic nausea and vomiting may be associated with gastroparesis or other conditions. Poor mechanistic understanding of symptoms often precludes targeted therapy. Numerous case series suggest that intrapyloric botulinum toxin injection (IPBI) may be beneficial in treating gastroparesis and dyspepsia in children.

Assessment of comorbid symptoms in pediatric autonomic dysfunction.

Purpose: Pediatric patients with autonomic dysfunction and orthostatic intolerance (OI) often present with co-existing symptoms and signs that might or might not directly relate to the autonomic nervous system. Our objective was to identify validated screening instruments to characterize these comorbidities and their impact on youth functioning.

Methods: The Pediatric Assembly of the American Autonomic Society reviewed the current state of practice for identifying symptom comorbidities in youth with OI.

Impact of Sleep Disturbance on Fatigue, Nausea, and Pain: Mediating Role of Depressive Symptoms Among Youth With Disorders of Gut-Brain Interaction.

Objectives: A high degree of sleep disturbance is reported among youth with disorders of gut-brain interaction (DGBIs). Given that sleep quality impacts a range of pediatric health outcomes including somatic sensations (eg, pain) and depressive mood occurs relatively frequently among youth with DGBIs, there is a dire need to disentangle the unique contributions of sleep and depressive mood on the somatic sensations experienced by youth with DGBIs. We aimed to examine whether depressive mood mediates the relations among sleep disturbance and pain intensity, nausea, and fatigue among youth with DGBIs.

Creating a data dictionary for pediatric autonomic disorders.

Purpose: Whether evaluating patients clinically, documenting care in the electronic health record, performing research, or communicating with administrative agencies, the use of a common set of terms and definitions is vital to ensure appropriate use of language. At a 2017 meeting of the Pediatric Section of the American Autonomic Society, it was determined that an autonomic data dictionary comprising aspects of evaluation and management of pediatric patients with autonomic disorders would be an important resource for multiple stakeholders.

Methods: Our group created the list of terms for the dictionary.

Use of Cluster Analysis to Identify Subtypes of Pediatric Functional Nausea.

Objective: The aim of the study was to evaluate whether there are clinical subtypes in children with functional nausea based on comorbidities and responses to the Nausea Profile questionnaire.

Methods: Patients from the Neurointestinal and Motility Program clinical registry at Lurie Children's Hospital were included if they met Rome IV criteria for functional nausea. Patients completed the Nausea Profile, a multidimensional measure of nausea with gastrointestinal, emotional, and somatic subscales.

Children with chronic nausea and orthostatic intolerance have unique brain network organization: A case-control trial.

Background: Determine whether subjects with chronic nausea and orthostatic intolerance share common alterations in key brain networks associated with central autonomic control: default mode, salience, and central executive networks, and the insula, a key component of the salience network.

Methods: Ten subjects (ages 12-18 years; 8 females, 2 males) with nausea predominant dyspepsia, orthostatic intolerance, and abnormal head-upright tilt test were consecutively recruited from pediatric gastroenterology clinic. These subjects were compared with healthy controls (n = 8) without GI symptoms or orthostatic intolerance.

Caregiver perceptions of end-of-life care in patients with high-grade glioma.

Background: Patients dying from high-grade gliomas (HGG) suffer from high symptom burden in the end-of-life (EoL) phase. Family caregivers are most informed about the patient's symptoms and disease course. The aim of this study is to assess caregiver perception on quality of EoL care of HGG patients.

Food protein-induced enterocolitis syndrome: Dynamic relationship among gastrointestinal symptoms, immune response, and the autonomic nervous system.

Objective: To explore the relationship among gastrointestinal (GI) symptoms, immune response, and autonomic nervous system (ANS) in food protein-induced enterocolitis syndrome (FPIES) in relation to the current understanding of disease phenotype and pathogenesis.

Data Sources: Relevant studies related to FPIES, GI symptomatology, and ANS were reviewed. Literature search was performed using PubMed, with keyword combinations including but not limited to FPIES, allergic GI disorders, ANS, autonomic dysfunction, dysautonomia, GI, diarrhea, vomiting, neuroimmune, and clinical phenotyping tools.

The Emerging Importance of High-Resolution Manometry in the Evaluation and Treatment of Deglutition in Infants, Children, and Adults: New Opportunities for Speech-Language Pathologists.

Purpose Diagnostic precision and prolonged testing before, during, and after deglutition is lacking across the age spectrum. Conventional clinical evaluation and radiologic methods are widely used but are reliant on human perception, carrying the risk of subjectivity. High-resolution manometry (HRM) is an emerging clinical and research tool and has the capability to objectively measure the dynamics, kinetics, regulatory, and correlation aspects of deglutition.

Children with Functional Nausea-Comorbidities outside the Gastrointestinal Tract.

Objective: To detail common comorbidities and procedures performed to evaluate functional nausea in children.

Study Design: In total, 63 children age 7-18 years seen in a tertiary care pediatric clinic who met Rome IV criteria for functional nausea prospectively completed an Intake Questionnaire, the Pediatric and Parent-Proxy PROMIS-25 Profile v 2.0, the Pediatric and Parent-Proxy Pediatric Sleep Disturbance-Short Form 4a, and the COMPASS 31 orthostatic intolerance scale to assess comorbidities.

Proceedings of the 2018 Advances In Motility and In NeuroGastroenterology: AIMING for the Future Single Topic Symposium.

Objectives: Motility and functional disorders are common in children and often debilitating, yet these disorders remain challenging to treat effectively. At the 2018 Annual North American Society for Pediatric Gastroenterology, Hepatology and Nutrition meeting, the Neurogastroenterology and Motility Committee held a full day symposium entitled, 2018 Advances In Motility and In NeuroGastroenterology - AIMING for the future. The symposium aimed to explore clinical paradigms in pediatric gastrointestinal motility disorders and provided a foundation for advancing new scientific and therapeutic research strategies.

Children with orthostatic intolerance exhibit elevated markers of inflammation in the dorsal medulla.

Children with orthostatic intolerance (OI) have exaggerated decreases in heart rate variability (HRV) and suppression of baroreflex sensitivity (BRS) with standing. Accompanying brain transmitter and metabolite profiles are unknown. In this study, we used proton (H) magnetic resonance spectroscopy (H-MRS) to quantify markers of neuronal and glial integrity in a pilot study of children with OI compared with asymptomatic controls.

Evaluating the yield of gastrointestinal testing in pediatric patients in aerodigestive clinic.

Objective: To improve understanding of the interrelatedness of airway and esophageal diagnoses by evaluating the yield of procedural and radiographic testing of the gastrointestinal tract in children with airway conditions by their referring diagnoses in a pediatric aerodigestive clinic.

Methods: A retrospective chart review of all 325 patients seen in the aerodigestive program from 2010 to 2013 was performed in a single academic medical center. Demographics and results from esophagogastroduodenoscopies with biopsies (EGD), upper gastrointestinal fluoroscopy studies (UGI), and pH multichannel intraluminal impedance probe (pH-MII) performed within 30 days of the clinic visit were evaluated according to presenting diagnoses.

Chronic nausea and orthostatic intolerance: Diagnostic utility of orthostatic challenge duration, Nausea Profile Questionnaire, and neurohumoral measures.

Background: Chronic nausea in pediatrics is a debilitating condition with unclear etiology. We aimed to define hemodynamic and neurohumoral characteristics of chronic nausea associated with orthostatic intolerance in order to improve identification and elucidate mechanism.

Methods: Children (10-18 years) meeting Rome III criteria for functional dyspepsia with nausea and symptoms of orthostatic intolerance (OI) completed a Nausea Profile Questionnaire followed by prolonged (45 minutes rather than the traditional 10 minutes) head-upright tilt (HUT) (70° tilt up) test.

Brainstem Glioblastoma Multiforme in a Patient with NF1.

This case report presents the first known case of a brainstem glioblastoma multiforme (GBM) in a patient with neurofibromatosis type 1 (NF1). While research has proposed that larger germ-line mutations in NF1 may be the driving factor that predisposes patients with NF1 to high-grade astrocytomas, this patient had a nonsense mutation in the NF1 gene, suggesting a variant tumorigenesis. Limited data on targeted immunotherapy for NF1 patients with a GBM have been reported and more data are required before targeted therapies could be proven as second-line treatment options.

Pediatric Disorders of Orthostatic Intolerance.

Orthostatic intolerance (OI), having difficulty tolerating an upright posture because of symptoms or signs that abate when returned to supine, is common in pediatrics. For example, ∼40% of people faint during their lives, half of whom faint during adolescence, and the peak age for first faint is 15 years. Because of this, we describe the most common forms of OI in pediatrics and distinguish between chronic and acute OI.