Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI

We report the first observation of a patient with contgenital chylous ascites (CCA) and Ehlers-Danlos syndrome type VI due to primary lymphatic defect with additional vascular anomaly. CCA is a rare condition, and there is limited understanding of its pathophysiology and treatment options. We also r...

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Autores principales: Ermarth, Anna K., Pohl, John, Esty, Brittany, Sempler, Jessica K., Carey, John C., O’Gorman, Molly A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American College of Gastroenterology 2016
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5226192/
https://www.ncbi.nlm.nih.gov/pubmed/28119937
http://dx.doi.org/10.14309/crj.2016.159
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author Ermarth, Anna K.
Pohl, John
Esty, Brittany
Sempler, Jessica K.
Carey, John C.
O’Gorman, Molly A.
author_facet Ermarth, Anna K.
Pohl, John
Esty, Brittany
Sempler, Jessica K.
Carey, John C.
O’Gorman, Molly A.
author_sort Ermarth, Anna K.
collection PubMed
description We report the first observation of a patient with contgenital chylous ascites (CCA) and Ehlers-Danlos syndrome type VI due to primary lymphatic defect with additional vascular anomaly. CCA is a rare condition, and there is limited understanding of its pathophysiology and treatment options. We also review the patient’s treatment course mitigated with octreotide and total parenteral nutritional support, as there are no current established guidelines for CCA. Early recognition of possible association with Ehlers-Danlos syndrome is important for quick intervention and successful management of pediatric patients.
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spelling pubmed-52261922017-01-24 Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI Ermarth, Anna K. Pohl, John Esty, Brittany Sempler, Jessica K. Carey, John C. O’Gorman, Molly A. ACG Case Rep J Case Report We report the first observation of a patient with contgenital chylous ascites (CCA) and Ehlers-Danlos syndrome type VI due to primary lymphatic defect with additional vascular anomaly. CCA is a rare condition, and there is limited understanding of its pathophysiology and treatment options. We also review the patient’s treatment course mitigated with octreotide and total parenteral nutritional support, as there are no current established guidelines for CCA. Early recognition of possible association with Ehlers-Danlos syndrome is important for quick intervention and successful management of pediatric patients. American College of Gastroenterology 2016-12-21 /pmc/articles/PMC5226192/ /pubmed/28119937 http://dx.doi.org/10.14309/crj.2016.159 Text en Copyright © Ermarth et al. This is an open-access article. This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-nd/4.0/
spellingShingle Case Report
Ermarth, Anna K.
Pohl, John
Esty, Brittany
Sempler, Jessica K.
Carey, John C.
O’Gorman, Molly A.
Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI
title Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI
title_full Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI
title_fullStr Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI
title_full_unstemmed Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI
title_short Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI
title_sort congenital chylous ascites and ehlers-danlos syndrome type vi
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5226192/
https://www.ncbi.nlm.nih.gov/pubmed/28119937
http://dx.doi.org/10.14309/crj.2016.159
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