{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T08:23:19Z","timestamp":1781079799670,"version":"3.54.1"},"reference-count":13,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,3,12]],"date-time":"2020-03-12T00:00:00Z","timestamp":1583971200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2020,3,12]],"date-time":"2020-03-12T00:00:00Z","timestamp":1583971200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100006492","name":"Division of Intramural Research, National Institute of Allergy and Infectious Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006492","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"publisher","award":["1K08DK106463"],"award-info":[{"award-number":["1K08DK106463"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000011","name":"Howard Hughes Medical Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000011","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100002247","name":"National Marfan Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002247","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Gastroenterol"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n<jats:title>Background<\/jats:title>\n<jats:p>Loeys-Dietz syndrome (LDS) is a systemic connective tissue disease (CTD) associated with a predisposition for intestinal inflammation, food allergy, and failure to thrive, often necessitating nutritional supplementation via gastrostomy tube. Poor wound healing has also been observed in in some patients with CTD, potentially increasing the risk of surgical interventions. We undertook to determine the safety and efficacy of gastrostomy tube placement in this population.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>We performed a retrospective cohort study of 10 LDS patients who had a total of 12 gastrostomy tubes placed.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>No procedural complications occurred, although one patient developed buried bumper syndrome in the near post-procedural time period and one patient had a small abscess at a surgical stitch. Most patients exhibited improvements in growth, with a median immediate improvement in BMI Z-score of 0.2 per month following the institution of gastrostomy tube feedings. Those with uncontrolled inflammation due to inflammatory bowel disease or eosinophilic gastrointestinal disease showed the least benefit and in some cases failed to demonstrate significant weight gain despite nutritional supplementation.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>Gastrostomy tube placement (surgical or endoscopic) is a generally safe and a reasonable therapeutic option for patients with LDS despite their underlying CTD.<\/jats:p>\n<\/jats:sec>","DOI":"10.1186\/s12876-020-01213-2","type":"journal-article","created":{"date-parts":[[2020,3,12]],"date-time":"2020-03-12T11:03:21Z","timestamp":1584011001000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Safety and outcome of gastrostomy tube placement in patients with Loeys-Dietz syndrome"],"prefix":"10.1186","volume":"20","author":[{"given":"Pamela A.","family":"Frischmeyer-Guerrerio","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gretchen","family":"MacCarrick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harry C.","family":"Dietz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F. Dylan","family":"Stewart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony L.","family":"Guerrerio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,3,12]]},"reference":[{"issue":"3","key":"1213_CR1","doi-asserted-by":"publisher","first-page":"275","DOI":"10.1038\/ng1511","volume":"37","author":"BL Loeys","year":"2005","unstructured":"Loeys BL, Chen J, Neptune ER, Judge DP, Podowski M, Holm T, et al. A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2. Nat Genet. 2005;37(3):275\u201381.","journal-title":"Nat Genet"},{"issue":"8","key":"1213_CR2","doi-asserted-by":"publisher","first-page":"788","DOI":"10.1056\/NEJMoa055695","volume":"355","author":"BL Loeys","year":"2006","unstructured":"Loeys BL, Schwarze U, Holm T, Callewaert BL, Thomas GH, Pannu H, et al. Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med. 2006;355(8):788\u201398.","journal-title":"N Engl J Med"},{"issue":"195","key":"1213_CR3","doi-asserted-by":"publisher","first-page":"195ra94","DOI":"10.1126\/scitranslmed.3006448","volume":"5","author":"PA Frischmeyer-Guerrerio","year":"2013","unstructured":"Frischmeyer-Guerrerio PA, Guerrerio AL, Oswald G, Chichester K, Myers L, Halushka MK, et al. TGFbeta receptor mutations impose a strong predisposition for human allergic disease. Sci Transl Med. 2013;5(195):195ra94.","journal-title":"Sci Transl Med"},{"issue":"9","key":"1213_CR4","doi-asserted-by":"publisher","first-page":"2058","DOI":"10.1097\/MIB.0000000000000872","volume":"22","author":"AL Guerrerio","year":"2016","unstructured":"Guerrerio AL, Frischmeyer-Guerrerio PA, Huang C, Wu Y, Haritunians T, McGovern DPB, et al. Increased prevalence of inflammatory bowel disease in patients with mutations in genes encoding the receptor subunits for TGFbeta. Inflamm Bowel Dis. 2016;22(9):2058\u201362.","journal-title":"Inflamm Bowel Dis"},{"issue":"4","key":"1213_CR5","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1097\/MPG.0b013e3181aed6f1","volume":"50","author":"JE Fortunato","year":"2010","unstructured":"Fortunato JE, Troy AL, Cuffari C, Davis JE, Loza MJ, Oliva-Hemker M, et al. Outcome after percutaneous endoscopic gastrostomy in children and young adults. J Pediatr Gastroenterol Nutr. 2010;50(4):390\u20133.","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"12","key":"1213_CR6","doi-asserted-by":"publisher","first-page":"1321","DOI":"10.1007\/s00383-018-4358-6","volume":"34","author":"F Sandberg","year":"2018","unstructured":"Sandberg F, Viktorsdottir MB, Salo M, Stenstrom P, Arnbjornsson E. Comparison of major complications in children after laparoscopy-assisted gastrostomy and percutaneous endoscopic gastrostomy placement: a meta-analysis. Pediatr Surg Int. 2018;34(12):1321\u20137.","journal-title":"Pediatr Surg Int"},{"issue":"4","key":"1213_CR7","first-page":"448","volume":"85","author":"S Klein","year":"1990","unstructured":"Klein S, Heare BR, Soloway RD. The \"buried bumper syndrome\": a complication of percutaneous endoscopic gastrostomy. Am J Gastroenterol. 1990;85(4):448\u201351.","journal-title":"Am J Gastroenterol"},{"issue":"4","key":"1213_CR8","first-page":"407","volume":"16","author":"SP Schrag","year":"2007","unstructured":"Schrag SP, Sharma R, Jaik NP, Seamon MJ, Lukaszczyk JJ, Martin ND, et al. Complications related to percutaneous endoscopic gastrostomy (PEG) tubes. A comprehensive clinical review. J Gastrointestin Liver Dis. 2007;16(4):407\u201318.","journal-title":"J Gastrointestin Liver Dis"},{"issue":"3","key":"1213_CR9","doi-asserted-by":"publisher","first-page":"154","DOI":"10.1159\/000478943","volume":"25","author":"R Azevedo","year":"2018","unstructured":"Azevedo R, Caldeira A, Banhudo A. Early presentation of buried bumper syndrome. GE Port J Gastroenterol. 2018;25(3):154\u20136.","journal-title":"GE Port J Gastroenterol"},{"issue":"5","key":"1213_CR10","doi-asserted-by":"publisher","first-page":"421","DOI":"10.5811\/westjem.2013.2.15843","volume":"14","author":"W Geer","year":"2013","unstructured":"Geer W, Jeanmonod R. Early presentation of buried bumper syndrome. West J Emerg Med. 2013;14(5):421\u20133.","journal-title":"West J Emerg Med"},{"issue":"3","key":"1213_CR11","doi-asserted-by":"publisher","first-page":"580","DOI":"10.1016\/j.gie.2008.04.015","volume":"68","author":"TH Lee","year":"2008","unstructured":"Lee TH, Lin JT. Clinical manifestations and management of buried bumper syndrome in patients with percutaneous endoscopic gastrostomy. Gastrointest Endosc. 2008;68(3):580\u20134.","journal-title":"Gastrointest Endosc"},{"issue":"Suppl 2","key":"1213_CR12","doi-asserted-by":"publisher","first-page":"E85","DOI":"10.1055\/s-2007-995552","volume":"40","author":"H Kohler","year":"2008","unstructured":"Kohler H, Lang T, Behrens R. Buried bumper syndrome after percutaneous endoscopic gastrostomy in children and adolescents. Endoscopy. 2008;40(Suppl 2):E85\u20136.","journal-title":"Endoscopy"},{"issue":"4","key":"1213_CR13","doi-asserted-by":"publisher","first-page":"495","DOI":"10.1097\/00005176-200110000-00015","volume":"33","author":"D Segal","year":"2001","unstructured":"Segal D, Michaud L, Guimber D, Ganga-Zandzou PS, Turck D, Gottrand F. Late-onset complications of percutaneous endoscopic gastrostomy in children. J Pediatr Gastroenterol Nutr. 2001;33(4):495\u2013500.","journal-title":"J Pediatr Gastroenterol Nutr"}],"container-title":["BMC Gastroenterology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s12876-020-01213-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s12876-020-01213-2\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s12876-020-01213-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,3,12]],"date-time":"2021-03-12T00:33:57Z","timestamp":1615509237000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcgastroenterol.biomedcentral.com\/articles\/10.1186\/s12876-020-01213-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,3,12]]},"references-count":13,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2020,12]]}},"alternative-id":["1213"],"URL":"https:\/\/doi.org\/10.1186\/s12876-020-01213-2","relation":{},"ISSN":["1471-230X"],"issn-type":[{"value":"1471-230X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,3,12]]},"assertion":[{"value":"7 November 2019","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"3 March 2020","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"12 March 2020","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and\/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This study was approved by the Johns Hopkins University\u2019s institutional review board. Informed consent: This study was granted a waiver of consent as a retrospective chart review by the Johns Hopkins University\u2019s institutional review board (IRB00096462) for individual participants included in the study.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Patients\/Guardians have consented to publication in writing under protocol approved by the National Institutes of Health (15-I-0162).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"71"}}