Author:
Frischmeyer-Guerrerio Pamela A.,MacCarrick Gretchen,Dietz Harry C.,Stewart F. Dylan,Guerrerio Anthony L.
Abstract
Abstract
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.
Funder
Division of Intramural Research, National Institute of Allergy and Infectious Diseases
National Institute of Diabetes and Digestive and Kidney Diseases
Howard Hughes Medical Institute
National Marfan Foundation
Publisher
Springer Science and Business Media LLC
Subject
Gastroenterology,General Medicine
Cited by
1 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献