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- W2914384939 abstract "Patients with diagnoses of postural orthostatic tachycardia syndrome (POTS) and hypermobile Ehlers-Danlos syndrome (hEDS) often report symptoms suggestive of mast cell activation. We evaluated the frequency of symptoms suggestive of mast cell activation syndrome (MCAS) in a cohort of patients with confirmed diagnoses of either POTS/hEDS. A retrospective chart review was performed for patients with hEDS and/or POTS from 2008 to mid-2018. Symptoms suggestive of mast cell activation were assessed using the 2018 diagnostic criteria. A total of 31 patients were diagnosed with POTS (Group 1) (2015 Heart Rhythm Society Expert Consensus Statement) and 38 patients with hEDS (Group 2) (March 2017 nosology) and of these, 23 patients had both POTS and hEDS (Group 3). In Groups 1, 2 and 3, 100% had both cutaneous and gastrointestinal involvement. Next in frequency were naso-ocular symptoms in 45 – 61%; cardiovascular 52 – 58%; respiratory 43 – 47%; and central nervous system 3 – 5%. There was very little variance in the frequency of organ system involvement in these 3 cohorts of patients. Over 95% of patients in all groups reported a significant response to H1 blockers, over 89% to H2 blockers and 80% to mast cell stabilizers. Symptoms of mast cell activation were present in patients with confirmed diagnoses of POTS alone; with hEDS alone, and both POTS and hEDS. The most dominant manifestations recurring were cutaneous and gastrointestinal. The use of H1 and H2 blockers and mast cell stabilizers were associated with reduction in these symptoms." @default.
- W2914384939 created "2019-02-21" @default.
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- W2914384939 date "2019-02-01" @default.
- W2914384939 modified "2023-10-18" @default.
- W2914384939 title "Prevalence of Symptoms of Mast Cell Activation in Patients with Postural Orthostatic Tachycardia Syndrome and Hypermobile Ehlers-Danlos Syndrome" @default.
- W2914384939 doi "https://doi.org/10.1016/j.jaci.2018.12.558" @default.
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