ABSTRACT Background Visceral myopathy (VSCM) is an ultra‐rare life‐threatening condition characterized by severe impairment of gastrointestinal (GI), genitourinary, and uterine smooth muscle. This disorder represents a significant clinical challenge due to variable presentation and the lack of standardized diagnostic and therapeutic protocols. Methods To discuss advances in the field, scientists and clinicians with a special interest in VSCM met in Arenzano, Genova, Italy in October 2024 for the second International Forum on Visceral Myopathy 2024 (IFVM2024) ( https://ifvm2024.ge.ibf.cnr.it/ ). Key Results As in the previous edition of the event ( https://poic‐e‐dintorni.org/efvm‐2022/ ), attendees included clinicians and researchers from around the world who study this disease, representatives from support organizations, patients affected by VSCM and their families, and companies that co‐funded the event. The present manuscript aims to summarize knowledge shared during the IFVM2024 conference, thus providing an updated state‐of‐the‐art summary of VSCM biology and disease management. Conclusions Here, we pay particular attention to the epidemiology of the disease, histopathology, genetics, novel treatments, advances in molecular and cell biology, experimental models, and the lived experiences and impact of this disorder on families.
Adolescent Normative Intervals for Body Surface Gastric Mapping: Spectral Analysis
ABSTRACT Background Body surface gastric mapping (BSGM) non‐invasively assesses gastric myoelectrical activity along with real‐time symptom reporting. In adults, the development of normative intervals has underpinned new explanatory phenotypes, aiding clinical decision‐making. This study established normative reference intervals for adolescent BSGM metrics. Methods Healthy adolescents aged 12–17 years with a BMI ≤ 35 kg/m 2 were recruited from New Zealand, Australia, and the United States. BSGM using Gastric Alimetry (Alimetry, New Zealand) involved a 30‐min fast, followed by a 480‐kcal meal, and a 4‐h postprandial recording. Reference intervals were calculated for four validated metrics: Principal Gastric Frequency (PGF), body mass index (BMI)‐adjusted amplitude, Gastric Alimetry Rhythm Index (GA‐RI, indicating rhythm stability), and the fed: fasted amplitude ratio (ff‐AR). Results were reported at the median and 5th and/or 95th percentiles as appropriate. Key Results A total of 107 participants (52.8% female, median age 14 [IQR 13–16], median BMI 20.1 [IQR 18.75–22.40]) with mixed ethnicities were included. No substantive correlations were observed between BSGM metrics and demographics or anthropometric data. Therefore, a single set of normative reference intervals was established. Median PGF was 3.06 cycles per minute; reference interval 2.72–3.37. Median BMI‐adjusted amplitude was 37.80 μV; reference interval 20.0–72.0. Median GA‐RI was 0.51; reference interval ≥ 0.22. Median ff‐AR was 2.12; reference interval ≥ 1.0. Conclusions and Inferences This study presents normative reference intervals for BSGM spectral metrics in adolescent populations, informing the interpretation of tests in research and clinical practice.
Pediatric Aerodigestive Medicine: Advancing Collaborative Care for Children With Oropharyngeal Dysphagia
Objectives:Aerodigestive disorders encompass various pathological conditions affecting the lungs, upper airway, and gastrointestinal tract in children. While advanced care has primarily occurred in specialty centers, many children first present to general pediatric gastroenterologists with aerodigestive symptoms necessitating awareness of these conditions. At the 2021 Annual North American Society for Pediatric Gastroenterology, Hepatology and Nutrition meeting, the aerodigestive Special Interest Group held a full‐day symposium entitled, Pediatric Aerodigestive Medicine: Advancing Collaborative Care of Children with Aerodigestive Disorders. The symposium aimed to underline the significance of a multidisciplinary approach to achieve better outcomes for these complex patients.Methods:The symposium brought together leading experts to highlight the growing aerodigestive field, promote new scientific and therapeutic strategies, share the structure and benefits of a multidisciplinary approach in diagnosing common and rare aerodigestive disorders, and foster multidisciplinary discussion of complex cases while highlighting the range of therapeutic and diagnostic options. In this article, we showcase the diagnostic and therapeutic approach to oropharyngeal dysphagia (OPD), one of the most common aerodigestive conditions, emphasizing the role of a collaborative model.Conclusions:The aerodigestive field has made significant progress and continues to grow due to a unique multidisciplinary, collaborative model of care for these conditions. Despite diagnostic and therapeutic challenges, the multidisciplinary approach has enabled and greatly improved efficient, high‐quality, and evidence‐based care for patients, including those with OPD.