{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T10:01:37Z","timestamp":1785578497208,"version":"3.56.0"},"reference-count":9,"publisher":"SAGE Publications","issue":"8","license":[{"start":{"date-parts":[[2025,12,22]],"date-time":"2025-12-22T00:00:00Z","timestamp":1766361600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["J Child Neurol"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:p>Trofinetide, a synthetic analog of glycine-proline-glutamate (GPE), is the first food and drug administration\u2013approved treatment for Rett syndrome. Gastrointestinal side effects (primarily diarrhea) are common, but severe immune-mediated reactions have not been described. We report a case of a young girl with genetically confirmed Rett syndrome who developed emesis, pallor, and lethargy after 2 months of treatment with trofinetide. An attempt was made to restart the medication after being held for a week, but the patient again experienced adverse symptoms and the drug was held. After 3 months without the medication, she experienced recurrence of initial symptoms within hours of rechallenge, consistent with food protein\u2013induced enterocolitis syndrome (FPIES). The medication was not continued after the re-challenge. The concept of drug-induced enterocolitis syndrome (DIES) has been reported with other agents; however, to our knowledge, this represents the first reported case of FPIES\/DIES triggered by trofinetide. This case expands the spectrum of trofinetide-related adverse reactions and emphasizes the need to distinguish immune-mediated enterocolitis from dose-dependent osmotic diarrhea and highlights the importance of clinician vigilance when initiating similar therapies in children with complex neurodevelopmental disorders.<\/jats:p>","DOI":"10.1177\/08830738251404099","type":"journal-article","created":{"date-parts":[[2025,12,22]],"date-time":"2025-12-22T18:38:23Z","timestamp":1766428703000},"page":"1190-1192","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":1,"title":["Trofinetide-Induced Enterocolitis Syndrome in a Child with Rett Syndrome"],"prefix":"10.1177","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5316-9964","authenticated-orcid":false,"given":"Stephen G","family":"Jones","sequence":"first","affiliation":[{"name":"University of Arkansas for Medical Sciences"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Alberty","sequence":"additional","affiliation":[{"name":"University of Arkansas for Medical Sciences"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew","family":"Bell","sequence":"additional","affiliation":[{"name":"University of Arkansas for Medical Sciences"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2025,12,22]]},"reference":[{"issue":"10","key":"e_1_3_2_2_2","first-page":"918","article-title":"Rett syndrome: clinical characteristics and management","volume":"16","author":"Leonard H","year":"2017","unstructured":"Leonard H, Cobb S, Downs J. 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