{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T22:45:30Z","timestamp":1788561930700,"version":"build-2803163510"},"reference-count":35,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100012688","name":"North American Society for Pediatric Gastroenterology, Hepatology and Nutrition","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100012688","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Pediatric intestinal pseudo\u2010obstruction (PIPO) is a severe bowel motility disorder characterized by impaired propulsion of gastrointestinal contents without mechanical obstruction. PIPO encompasses congenital and acquired disorders, including neuropathies, myopathies, and mesenchymopathies. PIPO presents with abdominal distension, bilious vomiting, and severe constipation. Diagnosis is based on objective measures of neuromuscular dysfunction, dilated bowel on imaging, parenteral and\/or enteral nutrition dependence, and genetic or metabolic testing. Antroduodenal manometry permits objective assessment of proximal bowel neuromuscular function. Genetic testing is increasingly valuable although causes of PIPO often remain incompletely defined. Understanding genotype\u2010phenotype correlations is essential for clarifying disease mechanisms and guiding therapies. This study aimed to characterize the clinical and genetic profiles of children with PIPO, utilizing manometric data for subtype classification.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A retrospective chart review was conducted at a tertiary care pediatric medical center, with inclusion criteria of PIPO diagnosis, completed manometry testing, and genetic evaluation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Nineteen children met inclusion criteria. Antroduodenal manometry classified 59% as neuropathic, 35% as myopathic, and one with mixed neuropathic and myopathic dysfunction. Genetic testing revealed pathogenic\n                      <jats:italic>ACTG2<\/jats:italic>\n                      mutations in all myopathic cases, while neuropathic PIPO exhibited more genetic variability. Histopathology was inconsistent and often nonspecific. Therapeutic approaches focused on nutritional support and promotility agents, with surgical intervention more common in myopathic cases.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      This study highlights the association of\n                      <jats:italic>ACTG2<\/jats:italic>\n                      mutations with a myopathic phenotype, and genetic diversity in neuropathic PIPO, emphasizing the need for further research to improve phenotyping to enhance diagnosis and treatment.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jpn3.70334","type":"journal-article","created":{"date-parts":[[2026,1,2]],"date-time":"2026-01-02T04:49:25Z","timestamp":1767329365000},"page":"660-671","source":"Crossref","is-referenced-by-count":2,"title":["Clinical, manometric, genetic, and histologic associations in pediatric intestinal pseudo\u2010obstruction: A case series"],"prefix":"10.1002","volume":"82","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5442-4533","authenticated-orcid":false,"given":"Sharon","family":"Wolfson","sequence":"first","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology and Nutrition Children\u2032s Hospital of Philadelphia Philadelphia Pennsylvania USA"},{"name":"Department of Pediatrics, Perelman School of Medicine University of Pennsylvania Philadelphia Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Myra","family":"Butrviengpunt","sequence":"additional","affiliation":[{"name":"Touro College of Osteopathic Medicine Middletown New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naomi","family":"Tjaden","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology and Nutrition Children\u2032s Hospital of Philadelphia Philadelphia Pennsylvania 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