{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,20]],"date-time":"2025-11-20T13:05:04Z","timestamp":1763643904283,"version":"3.41.2"},"reference-count":34,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2023,7,1]],"date-time":"2023-07-01T00:00:00Z","timestamp":1688169600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100005006","name":"Asan Institute for Life Sciences, Asan Medical Center","doi-asserted-by":"publisher","award":["2019\u2010261"],"award-info":[{"award-number":["2019\u2010261"]}],"id":[{"id":"10.13039\/501100005006","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"publisher","award":["2021R1A6A1A03040260"],"award-info":[{"award-number":["2021R1A6A1A03040260"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neurogastroenterology Motil"],"published-print":{"date-parts":[[2023,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>In this prospective cohort study, we evaluated features of \u201cadult\u2010onset megacolon with focal hypoganglionosis.\u201d<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We assessed the radiologic, endoscopic, and histopathologic phenotyping and treatment outcomes of 29 patients between 2017 and 2020. Data from community controls, consisting of 19,948 adults undergoing health screenings, were analyzed to identify risk factors. Experts reviewed clinical features and pathological specimens according to the London Classification for gastrointestinal neuromuscular pathology.<\/jats:p><\/jats:sec><jats:sec><jats:title>Key results<\/jats:title><jats:p>The median age of the patients with adult\u2010onset megacolon with focal hypoganglionosis at symptom onset was 59\u2009years (range, 32.0\u201374.9\u2009years), with mean symptom onset only 1\u2009year before diagnosis. All patients had focal stenotic regions with proximal bowel dilatation (mean diameter, 78.8\u2009mm; 95% confidence interval [CI], 72\u201386). The comparison with community controls showed no obvious risk factors. Ten patients underwent surgery, and all exhibited significant hypoganglionosis: 5.4 myenteric ganglion cells\/cm (interquartile range [IQR], 3.7\u201316.4) in the stenotic regions compared to 278 cells\/cm (IQR, 190\u2013338) in the proximal and 95 cells\/cm (IQR, 45\u2013213) in the distal colon. Hypoganglionosis was associated with CD3+ T cells along the myenteric plexus. Colectomy was associated with significant symptom improvement compared to medical treatment [change in the Global Bowel Satisfaction score, \u22125.4 points (surgery) vs. \u20100.3 points (medical treatment); <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001].<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions and inferences<\/jats:title><jats:p>Adult\u2010onset megacolon with focal hypoganglionosis has distinct features characterized by hypoganglionosis due to inflammation. Bowel resection appears to benefit these patients.<\/jats:p><\/jats:sec>","DOI":"10.1111\/nmo.14630","type":"journal-article","created":{"date-parts":[[2023,7,1]],"date-time":"2023-07-01T18:24:21Z","timestamp":1688235861000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Adult\u2010onset megacolon with focal hypoganglionosis: A detailed phenotyping and prospective cohort study"],"prefix":"10.1111","volume":"35","author":[{"given":"Jiyoung","family":"Yoon","sequence":"first","affiliation":[{"name":"Department of Gastroenterology University of Ulsan College of Medicine, Asan Medical Center  Seoul Korea"},{"name":"Department of Gastroenterology Eulji University School of Medicine, Uijeongbu Eulji Medical Center  Uijeoungbu Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3771-3691","authenticated-orcid":false,"given":"Kee Wook","family":"Jung","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology University of Ulsan College of Medicine, Asan Medical Center  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nam Seok","family":"Ham","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology University of Ulsan College of Medicine, Asan Medical Center  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jihun","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Ulsan College of Medicine, Asan Medical Center  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoon Suh","family":"Do","sequence":"additional","affiliation":[{"name":"Health Screening and Promotion Center University of Ulsan College of Medicine, Asan Medical Center  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seon Ok","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Clinical Epidemiology and Biostatistics Asan Medical Center  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sang Hyun","family":"Choi","sequence":"additional","affiliation":[{"name":"Department of Radiology and the Research Institute of Radiology University of Ulsan College of Medicine, Asan Medical Center  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dong Wook","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Radiology and the Research Institute of Radiology University of Ulsan College of Medicine, Asan Medical Center  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sung Wook","family":"Hwang","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology University of Ulsan College of Medicine, Asan Medical Center  Seoul 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