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Safety and outcome of gastrostomy tube placement in patients with Loeys-Dietz syndrome

BACKGROUND: 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 i...

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Autores principales: Frischmeyer-Guerrerio, Pamela A., MacCarrick, Gretchen, Dietz, Harry C., Stewart, F. Dylan, Guerrerio, Anthony L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7066767/
https://www.ncbi.nlm.nih.gov/pubmed/32164578
http://dx.doi.org/10.1186/s12876-020-01213-2
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author Frischmeyer-Guerrerio, Pamela A.
MacCarrick, Gretchen
Dietz, Harry C.
Stewart, F. Dylan
Guerrerio, Anthony L.
author_facet Frischmeyer-Guerrerio, Pamela A.
MacCarrick, Gretchen
Dietz, Harry C.
Stewart, F. Dylan
Guerrerio, Anthony L.
author_sort Frischmeyer-Guerrerio, Pamela A.
collection PubMed
description BACKGROUND: 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. METHODS: We performed a retrospective cohort study of 10 LDS patients who had a total of 12 gastrostomy tubes placed. RESULTS: 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. CONCLUSIONS: Gastrostomy tube placement (surgical or endoscopic) is a generally safe and a reasonable therapeutic option for patients with LDS despite their underlying CTD.
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spelling pubmed-70667672020-03-18 Safety and outcome of gastrostomy tube placement in patients with Loeys-Dietz syndrome Frischmeyer-Guerrerio, Pamela A. MacCarrick, Gretchen Dietz, Harry C. Stewart, F. Dylan Guerrerio, Anthony L. BMC Gastroenterol Research Article BACKGROUND: 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. METHODS: We performed a retrospective cohort study of 10 LDS patients who had a total of 12 gastrostomy tubes placed. RESULTS: 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. CONCLUSIONS: Gastrostomy tube placement (surgical or endoscopic) is a generally safe and a reasonable therapeutic option for patients with LDS despite their underlying CTD. BioMed Central 2020-03-12 /pmc/articles/PMC7066767/ /pubmed/32164578 http://dx.doi.org/10.1186/s12876-020-01213-2 Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Research Article
Frischmeyer-Guerrerio, Pamela A.
MacCarrick, Gretchen
Dietz, Harry C.
Stewart, F. Dylan
Guerrerio, Anthony L.
Safety and outcome of gastrostomy tube placement in patients with Loeys-Dietz syndrome
title Safety and outcome of gastrostomy tube placement in patients with Loeys-Dietz syndrome
title_full Safety and outcome of gastrostomy tube placement in patients with Loeys-Dietz syndrome
title_fullStr Safety and outcome of gastrostomy tube placement in patients with Loeys-Dietz syndrome
title_full_unstemmed Safety and outcome of gastrostomy tube placement in patients with Loeys-Dietz syndrome
title_short Safety and outcome of gastrostomy tube placement in patients with Loeys-Dietz syndrome
title_sort safety and outcome of gastrostomy tube placement in patients with loeys-dietz syndrome
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7066767/
https://www.ncbi.nlm.nih.gov/pubmed/32164578
http://dx.doi.org/10.1186/s12876-020-01213-2
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