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PURPOSE: Postural tachycardia syndrome (POTS) is a disorder featured by orthostatic intolerance. The purpose of this study was to investigate the severity of quality of life issues in POTS patients. METHODS: Online surveys for health related quality of life, sleep quality, fatigue, pain, and suicidal ideation were completed by 624 POTS patients and 139 controls. RESULTS: People with POTS have significantly more days of poor physical health (p < 0.001), fewer days with good energy (p < 0.001), and significantly more days with activity limitations (p < 0.001) than controls. Pain severity was significantly higher for those with POTS (p < 0.001) while feelings of control over life was lower than controls (p < 0.001). Sleep quality and daytime fatigue were also significantly worse for those with POTS than controls (p < 0.001). Finally, those with POTS have a significantly higher risk of suicide compared with controls (p < 0.001). INTERPRETATION: The myriad of symptoms from which many POTS patients suffer is associated with a decreased quality of life. Nearly half of our sample with POTS was at high risk for suicide. More work needs to be done to determine the underlying issues surrounding suicide in POTS so that an appropriate treatment regimen can be developed.
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Inquéritos Epidemiológicos , Síndrome da Taquicardia Postural Ortostática/complicações , Síndrome da Taquicardia Postural Ortostática/psicologia , Qualidade de Vida/psicologia , Ideação Suicida , Adulto , Feminino , Inquéritos Epidemiológicos/métodos , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome da Taquicardia Postural Ortostática/diagnóstico , Fatores de Risco , Autorrelato , Suicídio/psicologiaRESUMO
Dysautonomia is an abnormal clinical state with multiple etiologies, including autoimmunity. Antiphospholipid antibodies (aPL) are among the autoantibodies that have been associated with autonomic dysfunction. We have observed that an elevated total serum IgM appears to be associated with the presence of aPL in dysautonomia patients. This is a retrospective study analyzing the clinical characteristics of 45 consecutive patients with cardiac autonomic dysfunction and a persistently elevated total serum IgM. 93% of patients were female with a mean age of 32.7 years. Most patients had severely disabling disease, with a mean Karnofsky-like functional ability score of 42% (normal 100%). 93% of patients tested persistently positive for one or more aPL and all patients tested persistently positive for aPL and/or Sjogren's antibodies. No patient had lupus specific antibodies. One third of patients experienced one or more thrombotic events and 58% of patients attempting pregnancy experienced pregnancy morbidity. Lastly, 78% of aPL-positive patients treated with antithrombotic therapy experienced 50 to 100% improvement in one or more symptoms (e.g., migraine, cognitive dysfunction) recognized to be responsive to antithrombotic therapy in a subset of aPL-positive patients and 73% of patients treated with and tolerating immune modulatory therapy experienced a positive response. We propose total serum IgM as a reliable and inexpensive test that can be used to identify dysautonomia patients at risk for persistent aPL-positivity. These patients are important to identify as they have a significant risk for thrombosis and pregnancy morbidity and often experience significant symptomatic improvement with antithrombotic therapy and/or immune modulatory therapy.
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Excipients, or inactive ingredients, are a frequent cause of medication intolerance and allergy. Patients and clinicians concerned about medication allergies and sensitivities rely on the U.S. National Library of Medicine's DailyMed for accurate lists of excipients. Based on our anecdotal discovery of several examples of excipient omissions, we wished to examine the accuracy of DailyMed's listings more systematically in a sample of commonly prescribed medications. The objective of the study is to identify the frequency of inconsistency of excipient reporting within the DailyMed website. We performed a database audit of the Structured Product Labeling XML file provided by the drug manufacturer to the Food and Drug Administration and DailyMed for two randomly selected formulations of each of 50 commonly prescribed medications. For each of the 100 formulations, we compared the excipients listed in the "Description" to those in the "Ingredients and Appearance" sections in DailyMed. The Structured Product Labeling data file provided by the drug manufacturer contained internal inconsistencies of excipients in 39% of the formulations examined. Despite the use of Structured Product Labeling, the drug manufacturer's medication labels provided to the FDA and reported by DailyMed often contain conflicting information about inactive ingredients. Patients with allergies and excipient sensitivity should be aware of these discrepancies and consult multiple sections of the label to identify potential allergy-inducing inactive ingredients.
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Background: We aimed to determine whether patients with postural orthostatic tachycardia syndrome (POTS) have sexual dysfunction compared to age-matched healthy controls. Methods: Utilizing online COMPASS-31 to evaluate dysautonomia symptom severity, Beck's Depression Inventory Second Edition (BDII), Female Sexual Function (FSF), and International Index of Erection Function (IIEF) questionnaires, we compared sexual function scores in patients with POTS to scores obtained from sex- and age-matched healthy controls via a cross-sectional case-control study. Results: A total of 160 women with POTS, mean age 30.2 ± 7.9 (range 21-50 years), had lower FSF scores than 62 healthy age-matched female controls. IIEF scores in 29 male patients with POTS with a mean age of 30.1 ± 6.0 (range 21-47) were significantly lower than in 27 healthy age-matched male controls. Female POTS patients had significantly lower scores in the sub-domains of desire, arousal, and satisfaction, while male POTS patients had significantly lower scores in erectile and orgasmic function, desire, and satisfaction than healthy controls. Predictive factors of sexual dysfunction were depression in women and age in men. The severity of autonomic symptoms correlated with sexual dysfunction in women, but this effect disappeared after controlling for depression. Conclusions: Compared to healthy controls, women and men with POTS have significant sexual dysfunction, which needs to be considered in the diagnostic and therapeutic approaches as part of comprehensive patient care.
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OBJECTIVES: Postural orthostatic tachycardia syndrome (POTS) is a chronic disorder of the autonomic nervous system that is associated with orthostatic intolerance (OI) and gastrointestinal (GI) symptoms. We aimed to determine if a gluten-free diet (GFD) may be an effective treatment in patients with POTS. METHODS: Patients with confirmed POTS and without celiac disease who followed GFD for 4 weeks retrospectively completed pre- and post- GFD COMPASS-31 questionnaires. Paired-samples t-tests were conducted to compare COMPASS-31 scores before and after adopting GFD. RESULTS: All 20 patients (all females, age 16-62 years (mean age 33)), had POTS symptoms for 1-30 years (mean 10.8, SD = 10.23 years). Eleven patients had co-morbid mast cell activation syndrome, and 8 had hypermobile Ehlers-Danlos syndrome. Pre-GFD COMPASS-31 scores (mean 57.8) were significantly higher than post-GFD scores (mean 38.2, t(19) = -8.92, p < 0.00001), with the largest improvement noted in the OI (p < 0.00001), vasomotor, (p = 0.0034), and GI (p = 0.0004) domains and with a mean reduction in total COMPASS-31 score by 33.9% after implementing GFD. All patients reported improved symptoms on GFD with mean self-assessed improvement of 50.5% (range 10-99%). DISCUSSION: GFD may be effective in reducing the symptom burden in patients with POTS, particularly in the OI, vasomotor, and GI symptom domains. Large prospective studies are necessary to confirm whether GFD is an effective long-term treatment option for patients.
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Intolerância Ortostática , Síndrome da Taquicardia Postural Ortostática , Feminino , Humanos , Adulto , Adolescente , Adulto Jovem , Pessoa de Meia-Idade , Síndrome da Taquicardia Postural Ortostática/complicações , Síndrome da Taquicardia Postural Ortostática/diagnóstico , Síndrome da Taquicardia Postural Ortostática/terapia , Estudos Retrospectivos , Estudos Prospectivos , Dieta Livre de Glúten , Intolerância Ortostática/complicações , Intolerância Ortostática/diagnósticoRESUMO
Determine efficacy and adverse events (AEs) of hydroxyurea (HU) in mast cell activation syndrome (MCAS) patients who were refractory to standard medical therapy. An electronic chart review was performed to find MCAS patients who received HU in a MCAS medical practice. Diagnosis of MCAS was established on the basis of mast cell (MC) activation symptoms in ≥ 5 systems plus ≥ 1 abnormal MC mediators and/or ≥ 20 MC/high power field on duodenal biopsies. Medicines not providing significant clinical improvement prior to HU were tabulated. The following symptoms were evaluated by patients on a 0-10 scale prior to and at the study conclusion: bone pain, abdominal pain, diarrhea, bloating, and nausea. Safety labs were obtained on a regular basis. Twenty out of three hundred ten (8.4%) MCAS patients received HU. Patients included 22 females, average age 42.4 years. Dysautonomia was present in 60%. An average of 10.6 (SD 1.7, range 8-13) medications were used prior to adding HU to various concomitant medications. Average dose of HU was 634 mg. In 20 patients who continued therapy for ≥ 2 months, there was statistically significant reduction of bone pain, abdominal pain, diarrhea, bloating, and nausea. Fourteen patients noted prolonged success with therapy. Six patients stopped HU within 6 weeks owing to AEs. Four patients treated ≥ 2 months had AEs and 2 led to HU cessation. All AEs were reversible. Refractory MCAS patients showed clear significant improvement in bone pain and gastrointestinal symptoms on HU. Systematic monitoring was effective in preventing the occurrence of severe HU-induced adverse events.
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Hidroxiureia , Síndrome da Ativação de Mastócitos , Dor Abdominal/induzido quimicamente , Dor Abdominal/tratamento farmacológico , Adulto , Diarreia/induzido quimicamente , Diarreia/tratamento farmacológico , Feminino , Humanos , Hidroxiureia/efeitos adversos , Mastócitos , Náusea/induzido quimicamente , Náusea/tratamento farmacológico , Estudos RetrospectivosRESUMO
STUDY OBJECTIVES: Sleep disturbance is common in long-COVID (LC). Restless legs syndrome (RLS) is characterized by sleep disturbance and has been reported after viral infections. Therefore, we evaluated RLS symptoms cross-sectionally in individuals with LC at both current and pre-coronavirus disease 2019 (pre-COVID-19) time points. METHODS: Adults on LC-focused Facebook pages were recruited for an online assessment of symptoms before COVID-19 infection and during their present LC state in a cross-sectional manner. The LC group documented baseline symptoms retrospectively. Questions were included about the presence/severity of RLS symptoms and assessments of fatigue, quality of life, and sleep apnea. A control group was recruited and included individuals ≥ 18 years of age who never had overt symptoms of COVID-19. Pregnancy was an exclusion criterion for both groups. RESULTS: There were 136 participants with LC (89.7% females, age 46.9 ± 12.9 years) and 136 controls (65.4% females, age 49.2 ± 15.5). RLS prevalence in females with LC was 5.7% pre-COVID-19 and 14.8% post-COVID-19 (P < .01) vs 6.7% in control females. Severity of RLS was moderate in both groups. Logistic regression predicting post-COVID-19 RLS among females with LC failed to find significant effects of hospitalization, sleep apnea, neuropathic pain severity, or use of antihistamines and antidepressants. CONCLUSIONS: The baseline prevalence of RLS in females with LC was similar to the general population group as well as to patients in epidemiological studies. The prevalence significantly increased in the LC state. Postinfectious immunological mechanisms may be at play in the production for RLS symptoms. CITATION: Weinstock LB, Brook JB, Walters AS, Goris A, Afrin LB, Molderings GJ. Restless legs syndrome is associated with long-COVID in women. J Clin Sleep Med. 2022;18(5):1413-1418.
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COVID-19 , Complicações Infecciosas na Gravidez , Síndrome das Pernas Inquietas , Síndromes da Apneia do Sono , Transtornos do Sono-Vigília , Adulto , COVID-19/complicações , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Gravidez , Qualidade de Vida , Síndrome das Pernas Inquietas/complicações , Síndrome das Pernas Inquietas/diagnóstico , Síndrome das Pernas Inquietas/epidemiologia , Estudos Retrospectivos , Síndromes da Apneia do Sono/complicações , Transtornos do Sono-Vigília/complicações , Transtornos do Sono-Vigília/epidemiologia , Síndrome de COVID-19 Pós-AgudaRESUMO
OBJECTIVES: Hyper-inflammation caused by COVID-19 may be mediated by mast cell activation (MCA) which has also been hypothesized to cause Long-COVID (LC) symptoms. We determined prevalence/severity of MCA symptoms in LC. METHODS: Adults in LC-focused Facebook support groups were recruited for online assessment of symptoms before and after COVID-19. Questions included presence and severity of known MCA and LC symptoms and validated assessments of fatigue and quality of life. General population controls and mast cell activation syndrome (MCAS) patients were recruited for comparison if they were ≥18 years of age and never had overt COVID-19 symptoms. RESULTS: There were 136 LC subjects (89.7% females, age 46.9 ±12.9 years), 136 controls (65.4% females, age 49.2 ±15.5), and 80 MCAS patients (85.0% females, age 47.7 ±16.4). Pre-COVID-19 LC subjects and controls had virtually identical MCA symptom and severity analysis. Post-COVID-19 LC subjects and MCAS patients prior to treatment had virtually identical MCA symptom and severity analysis. CONCLUSIONS: MCA symptoms were increased in LC and mimicked the symptoms and severity reported by patients who have MCAS. Increased activation of aberrant mast cells induced by SARS-CoV-2 infection by various mechanisms may underlie part of the pathophysiology of LC, possibly suggesting routes to effective therapy.
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COVID-19 , Síndrome da Ativação de Mastócitos , Adulto , COVID-19/complicações , Feminino , Humanos , Masculino , Mastócitos , Pessoa de Meia-Idade , Qualidade de Vida , SARS-CoV-2 , Síndrome de COVID-19 Pós-AgudaRESUMO
The concept that disease rooted principally in chronic aberrant constitutive and reactive activation of mast cells (MCs), without the gross MC neoplasia in mastocytosis, first emerged in the 1980s, but only in the last decade has recognition of "mast cell activation syndrome" (MCAS) grown significantly. Two principal proposals for diagnostic criteria have emerged. One, originally published in 2012, is labeled by its authors as a "consensus" (re-termed here as "consensus-1"). Another sizable contingent of investigators and practitioners favor a different approach (originally published in 2011, newly termed here as "consensus-2"), resembling "consensus-1" in some respects but differing in others, leading to substantial differences between these proposals in the numbers of patients qualifying for diagnosis (and thus treatment). Overdiagnosis by "consensus-2" criteria has potential to be problematic, but underdiagnosis by "consensus-1" criteria seems the far larger problem given (1) increasing appreciation that MCAS is prevalent (up to 17% of the general population), and (2) most MCAS patients, regardless of illness duration prior to diagnosis, can eventually identify treatment yielding sustained improvement. We analyze these proposals (and others) and suggest that, until careful research provides more definitive answers, diagnosis by either proposal is valid, reasonable, and helpful.
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Mastocitose , Consenso , Humanos , Mastócitos , Mastocitose/diagnósticoRESUMO
STUDY OBJECTIVES: Mast cell activation syndrome (MCAS) is an inflammatory and allergic disorder. We determined the prevalence of restless legs syndrome (RLS) in MCAS because each common syndrome may be inflammatory in nature and associated with dysautonomia. METHODS: Individuals with MCAS were evaluated for RLS by two standard questionnaires. Prevalence comparisons included spouse control patients and two prevalence publications. MCAS diagnosis required mast cell (MC) symptoms in ≥ 2 organs plus ≥ 1 elevated MC mediators, improvement with MC therapy, and/or increased intestinal MC density. Clinical variables were studied. RESULTS: There were 174 patients with MCAS (146 female, 28 male, mean age 44.8 years) and 85 spouse control patients (12 female, 73 male, mean age 50.9 years). Patients with MCAS as a whole had a higher prevalence of RLS (40.8%) than spouse control (12.9%) (P < .0001) Male patients with MCAS had a higher prevalence of RLS (32.1%) than male controls (12.3%, odds ratio [OR] 3.4, confidence interval [CI] 1.2-9.7, P = .025), American men (8.4%, OR 5.2, CI 2.2-12.0, P < .001), and French men (5.8%, OR 7.7, CI 3.4-17.1, P < .001). Female patients with MCAS also had a higher prevalence of RLS (42.5%) than female controls (16.7%) but this did not reach statistical significance perhaps because of the sample size of the female controls. However, female patients with MCAS had a statistically higher prevalence of RLS than American women (10.0%, OR 6.7, CI 4.5-9.7, P < .0001) and French women (10.8%, OR 6.1, CI 4.4-8.6, P < .0001). CONCLUSIONS: RLS appears to be associated with MCAS. Effects of mast cell mediators, inflammation, immune mechanisms, dysautonomia, or hypoxia may theoretically activate RLS in MCAS.
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Mastocitose , Síndrome das Pernas Inquietas , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Razão de Chances , Prevalência , Síndrome das Pernas Inquietas/epidemiologia , Inquéritos e QuestionáriosRESUMO
A patient with severe postural orthostatic tachycardia syndrome (POTS) and mast cell activation syndrome (MCAS) received immunotherapy with low-dose naltrexone (LDN) and intravenous immunoglobulin (IVIg) and antibiotic therapy for small intestinal bacterial overgrowth (SIBO). A dramatic and sustained response was documented. The utility of IVIg in autoimmune neuromuscular diseases has been published, but clinical experience with POTS is relatively unknown and has not been reported in MCAS. As a short-acting mu-opioid antagonist, LDN paradoxically increases endorphins which then bind to regulatory T cells which regulate T-lymphocyte and B-lymphocyte production and this reduces cytokine and antibody production. IVIg is emerging as a promising therapy for POTS. Diagnosis and treatment of SIBO in POTS is a new concept and appears to play an important role.
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Antibacterianos/administração & dosagem , Síndrome da Alça Cega/tratamento farmacológico , Imunoglobulinas Intravenosas/administração & dosagem , Mastocitose/tratamento farmacológico , Naltrexona/administração & dosagem , Antagonistas de Entorpecentes/administração & dosagem , Síndrome da Taquicardia Postural Ortostática/tratamento farmacológico , Adulto , Quimioterapia Combinada , Feminino , Humanos , Intestino Delgado/microbiologiaRESUMO
We describe a young woman who developed POTS with positive serum anti-NMDA receptor antibodies and no evidence of encephalitis after vaccination with HPV vaccine, Cervarix. Her symptoms improved significantly with immunomodulatory therapy and re-occurred after immunomodulatory therapy was stopped, suggesting an autoimmune etiology of POTS after vaccination.
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Anticorpos/sangue , Vacinas contra Papillomavirus/efeitos adversos , Síndrome da Taquicardia Postural Ortostática/induzido quimicamente , Receptores de N-Metil-D-Aspartato/imunologia , Adolescente , Feminino , Humanos , Fatores Imunológicos/uso terapêutico , Plasmaferese , Síndrome da Taquicardia Postural Ortostática/sangue , Síndrome da Taquicardia Postural Ortostática/imunologia , Síndrome da Taquicardia Postural Ortostática/terapia , Vacinação/efeitos adversosRESUMO
OBJECTIVE: We investigated the prevalence of suicidal ideation in relationship with symptoms of sleep disruption in people with postural orthostatic tachycardia syndrome (POTS). METHODS: Online surveys (including the Pittsburgh Sleep Quality Index and the Suicide Behaviors Questionnaire - Revised) were completed by 705 POTS patients and 170 non-POTS controls. RESULTS: Poor sleep quality was reported in 98.4% of POTS patients with a calculated subjective sleep efficiency of 65.4%. The POTS group's sleep efficiency was significantly lower (t[873]= -11.32; p<0.001) and sleep disturbances because of pain were significantly higher (t[873]=15.36; p<0.001) than controls. Chi-square testing showed a larger proportion of individuals at high-risk for suicide among POTS patients than controls (c2 [1, n=875]=55.6; p<0.001). Multiple linear regression analysis showed that sleep scores (ß=0.23, p<0.001), age (ß=-0.03, p<0.001), and illness with POTS (ß=0.68, p=0.05) were significantly associated with suicide ideation scores (F[4, 870]=38.34, p<0.001). This model explained 15% of variance (R2=0.15) in suicidal ideation scores. CONCLUSION: Patients with POTS may suffer from increased sleep disturbance and suicidal ideation compared with the general population. Treatment to improve sleep efficiency and sleep quality is an important step toward better quality of life for POTS patients.