Abstract Objectives Many non-musculoskeletal complaints in EDS-HT may be related to dysautonomia. This study therefore aims to investigate whether dysautonomia is present and to explore the ...underlying mechanisms. Methods A total of 39 females with EDS-HT and 35 age-matched controls underwent autonomic function testing. Resting autonomic tone was assessed using heart rate variability (frequency domain) and baroreflex sensitivity analysis (cross correlation). Autonomic reactivity was assessed using the Autonomic Reflex Screen test battery. Factors suspected to contribute to dysautonomia, e.g., neuropathy, medication use, decreased physical activity, depression, pain-induced sympathetic arousal, and connective tissue laxity, were quantified using validated questionnaires, the Beighton score, and measurement of skin extensibility. Results The EDS-HT group showed autonomic deregulation with increased sympathetic activity at rest and reduced sympathetic reactivity to stimuli. Increased resting activity was indicated by a higher LF/HF ratio compared to controls (1.7 ± 1.23 vs 0.9 ± 0.75, p = 0.002); decreased reactivity by a greater BP fall during valsalva (−19 ± 12 vs −8 ± 10, p < 0.001), and a smaller initial diastolic BP increase during tilt (7% vs 14%, p = 0.032). Orthostatic intolerance was significantly more prevalent in EDS-HT than controls (74% vs 34%) and was most frequently expressed as postural orthostatic tachycardia. Lowered QSART responses suggest that sympathetic neurogenic dysfunction is common in patients ( p < 0.013), which may explain the dysautonomia in EDS-HT. Further, connective tissue laxity and vasoactive medication use were identified as important factors in aggravating dysautonomia ( p < 0.035). Conclusion Dysautonomia consisting of cardiovascular and sudomotor dysfunction is present in EDS-HT. Neuropathy, connective tissue laxity, and vasoactive medication probably play a role in its development.
Chronic widespread pain is highly present in patients with the Ehlers–Danlos syndrome hypermobility type (EDS-HT), but up to now, evidence for generalized hyperalgesia is lacking. The aim of this ...study is to investigate whether pressure pain thresholds (PPTs) at both symptomatic and asymptomatic body areas differ in EDS-HT patients compared to healthy subjects. Twenty-three women with EDS-HT and 23 gender- and age-matched healthy controls participated. All subjects marked on Margolis Pain Diagram where they felt pain lasting longer than 24 h in the past 4 weeks. Then, they completed several questionnaires assessing pain cognitions, fatigue, disability, and general health status, in order to take the possible influence of these factors on PPTs into account. Patients also completed a form concerning the type of pain they experienced. Thereupon, a blinded researcher assessed PPTs at 14 body locations on the trunk and extremities. PPTs were compared for the two complete groups. In addition, PPTs of patients and controls who did not report pain in a respective zone were compared. PPTs of the patients were significantly lower compared to those of the control group, also when pain-free samples per zone were compared. The mean (SD) PPT was 2.9 (1.62) kg/cm
2
in the EDS-HT patients and 5.2 (1.88) kg/cm
2
in the controls (
P
< 0.001). No confounding factors responsible for the observed differences could be revealed. In half of the patient group, a predominantly neuropathic pain component was likely present. This study provides evidence for the existence of hyperalgesia even in asymptomatic areas (generalized secondary hyperalgesia). The generalized hyperalgesia may represent the involvement of a sensitized central nervous system, which inquires an adapted pain management for this patient group.
Abstract Objective The goal of this systematic literature review is to determine whether there are differences and similarities in heart rate variability (HRV) between adult patients with ...fibromyalgia (FM), chronic fatigue syndrome (CFS), and healthy pain-free control subjects. Methods To obtain relevant articles, PubMed and Web of Knowledge were searched for case–control studies. Selection of the literature was based on selection criteria ascertaining studies with adult human patient groups comparing HRV. Risk of bias and levels of evidence were determined. Results Sixteen case–control studies were included, 10 comparing FM patients to controls and 6 comparing CFS patients to controls. Methodological quality was moderate to good. Both time domain and frequency domain measurements were used. The majority of the researchers observed lower HRV in FM patients compared to healthy control persons, as well as increased sympathetic activity and a blunted autonomic response to stressors. Resistance training improved HRV in FM patients. In CFS patients HRV was only reduced during sleep. Conclusion FM patients show more HRV aberrances and indices of increased sympathetic activity. Increased sympathetic activity is only present in CFS patients at night. Since direct comparisons are lacking and some confounders have to be taken into account, further research is warranted. The role of pain and causality can be subject of further research, as well as therapy studies directed to reduced HRV.
Objective:
In this study we evaluated the effect of sprint interval training on metabolic and physical fitness in adolescents and young adults with intellectual disabilities when compared with ...continuous aerobic training and no training (control).
Methods:
Fifty-four persons with intellectual disabilities (age: 17 (3.0), body mass index: 27.7 (3.7), intelligence quotient: 59 (8.6)) were matched based on age, gender and intelligence quotient between sprint interval training (n = 17), continuous aerobic training (n = 15) and control (n = 14). Sprint interval training was composed of three blocks of 10 minutes at ventilatory threshold (blocks 1 and 3: 10 sprint bouts of 15 seconds, followed by 45 seconds relative rest; block 2: continuous training) twice a week for 15 weeks. Continuous aerobic training was composed of three blocks of 10 minutes continuous training. After eight weeks, intensity was increased to 110% of ventilatory threshold. The control group did not participate in supervised exercise training. Before and after the training period, body composition, physical and metabolic fitness were evaluated.
Results:
Sprint interval training showed a significant positive evolution for waist circumference, fat%, systolic blood pressure, lipid profile, fasting insulin, homeostasis model assessment of insulin resistance, peak VO2, peak Watt, ventilatory threshold, 6-minute walk distance and muscle fatigue resistance when compared with no training (P < 0.01). The sprint interval training group demonstrated significant improvements for fat%, systolic blood pressure, low-density lipoprotein, fasting insulin, peak VO2 and peak power and ventilatory threshold (P < 0.01) when compared with continuous aerobic training.
Conclusion:
In this study we could observe that sprint interval training has stronger beneficial effects on body composition, physical fitness and metabolic fitness compared with control. Compared with continuous aerobic training, sprint interval training seems to result in better outcome.
Abstract Aim This study provides insight into the profile and importance of autonomic symptoms in the hypermobility type (HT) of Ehlers–Danlos syndrome (EDS). The impact of these symptoms is put into ...perspective by comparing with fibromyalgia (FM) and two other EDS types. Methods Overall, 80 patients with EDS-HT participated, as well as 11 with classical EDS (cEDS), seven with vascular EDS (vEDS), 38 with FM, and 43 healthy controls. All participants filled out the autonomic symptom profile (ASP). Furthermore, they were inquired about quality of life (QOL, SF-36) and factors contributing to the EDS disease burden, e.g., hypermobility (5-point questionnaire, GHQ), fatigue (checklist individual strength, CIS), pain (pain detect questionnaire, PDQ), affective distress (hospital anxiety and depression scale, HADS), and physical activity (Baecke). Results The total autonomic symptom burden was higher in EDS-HT (57.9 ± 21.57) than in controls (11.3 ± 19.22), cEDS (32.3 ± 19.47), and vEDS (29.1 ± 19.18), but comparable to FM (53.8 ± 19.85). Especially orthostatic and gastrointestinal complaints were prevalent. The importance of autonomic symptoms in EDS-HT was emphasized by the correlation with lowered QOL ( r = −0.402), fatigue ( r = 0.304), and pain severity ( r = 0.370). Although affective distress and decreased physical activity are often suggested as possible causes for dysautonomia, the ASP did not correlate with the HADS and Baecke score. By contrast, the correlation of the GHQ ( r = 0.298) and PDQ ( r = 0.413) with the ASP supports the hypothesis that joint hypermobility and neuropathy may play a role in the development of autonomic symptoms. Conclusion Autonomic symptoms, especially orthostatic and gastrointestinal complaints, are frequent extraarticular manifestations of EDS-HT and contribute to the disease burden.
To describe medication use, surgery, and physiotherapy, and to examine the effect of these treatment modalities on functional impairment and amount of complaints among patients with the hypermobility ...type of Ehlers-Danlos syndrome (EDS-HT).
Cross-sectional study.
Physical and rehabilitation medicine department and center for medical genetics.
Patients with EDS-HT (N=79; 8 men, 71 women) were recruited for this study.
Not applicable.
Patients filled out questionnaires regarding type of complaints, medication use, surgery, physiotherapy, and outcome of treatment. Functional impairment in daily life was measured by the Sickness Impact Profile. Pain severity was assessed with visual analog scales.
Patients reported a large number of complaints, a considerable presence of severe pain, and a clinically significant impact of disease on daily functioning. Most patients (92.4%) used medications, among which analgesics were the most prevalent. Fifty-six patients (70.9%) underwent surgery, including mainly interventions of the extremities and abdomen. Forty-one patients (51.9%) are currently enrolled in a physical therapy program, mainly comprising neuromuscular exercises, massage, and electrotherapy. Patients with a high consumption of analgesics, who visited the physiotherapist, or who underwent surgery had a higher dysfunction in daily life. Only 33.9% of the patients who underwent surgery and 63.4% of patients in physical therapy reported a positive outcome.
Patients with EDS-HT have numerous complaints and an impaired functional status that strongly determine their high rate of treatment consumption. The outcome of surgical and physiotherapy treatment is disappointing in a large percentage, which illustrates a strong need for evidence-based therapy.
Although autonomic nervous system (ANS) dysfunction in Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) has been proposed, conflicting evidence makes it difficult to draw firm conclusions ...regarding ANS activity at rest in ME/CFS patients. Although severe exercise intolerance is one of the core features of ME/CFS, little attempts have been made to study ANS responses to physical exercise. Therefore, impairments in ANS activation at rest and following exercise were examined using a case-control study in 20 ME/CFS patients and 20 healthy people. Different autonomous variables, including cardiac, respiratory, and electrodermal responses were assessed at rest and following an acute exercise bout. At rest, parameters in the time-domain represented normal autonomic function in ME/CFS, while frequency-domain parameters indicated the possible presence of diminished (para)sympathetic activation. Reduced parasympathetic reactivation during recovery from exercise was observed in ME/CFS. This is the first study showing reduced parasympathetic reactivation during recovery from physical exercise in ME/CFS. Delayed HR recovery and/or a reduced HRV as seen in ME/CFS have been associated with poor disease prognosis, high risk for adverse cardiac events, and morbidity in other pathologies, implying that future studies should examine whether this is also the case in ME/CFS and how to safely improve HR recovery in this population.
Background
People with Ehlers-Danlos Syndromes and Hypermobility Spectrum Disorders are hampered in their social participation, especially in the social relationships they have.
Objective
The aim of ...this study is to research the impact of hypermobile Ehlers-Danlos Syndrome (hEDS) and Hypermobile Spectrum Disorders (HSD) on interpersonal interactions and relationships.
Methods
A phenomenological hermeneutic study was performed. Semi-structured interviews were used to explore the experiences of 11 participants.
Results
Four themes emerged from the data analysis. (1) people with hEDS or HSD can no longer do what they want to do and that affects their identity, (2) people with hEDS or HSD have to find a balance in the amount of activities they participate in, (3) having hEDS or HSD influences how to ask for, accept and give help, and (4) Relationships are affected in persons with hEDS or HSD. As well as changes in the social network, different types of relationships are influenced by the disease, including relationship with their partner, their children, their friends, strangers, fellow-sufferers and health care professionals.
Chronic fatigue in Ehlers–Danlos syndrome—Hypermobile type Hakim, Alan; De Wandele, Inge; O'Callaghan, Chris ...
American journal of medical genetics. Part C, Seminars in medical genetics,
March 2017, 2017-03-00, 20170301, Letnik:
175, Številka:
1
Journal Article
PDF
