Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI.
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ABSTRACT:
SUBMITTER: Ermarth AK
PROVIDER: S-EPMC5226192 | biostudies-literature | 2016 Aug
REPOSITORIES: biostudies-literature
Publications
Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI.
Ermarth Anna K AK Pohl John J Esty Brittany B Sempler Jessica K JK Carey John C JC O'Gorman Molly A MA
ACG case reports journal 20160801 4
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 associati ...[more]
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