Association of mast-cell-related conditions with hypermobile syndromes: a review of the literature.

Ehlers-Danlos syndrome (EDS) is a group of related connective tissue disorders consisting of 13 subtypes, each with its own unique phenotypic and genetic variation. The overlap of symptoms and multitude of EDS variations makes it difficult for patients to achieve a diagnosis early in the course of their disease. The most common form, hypermobile type EDS (hEDS) and its variant, hypermobile spectrum disorder (HSD), are correlated with rheumatologic and inflammatory conditions. Evidence is still needed to determine the pathophysiology of hEDS; however, the association among these conditions and their prevalence in hEDS/HSD may be explained through consideration of persistent chronic inflammation contributing to a disruption of the connective tissue. Aberrant mast cell activation has been shown to play a role in disruption of connective tissue integrity through activity of its mediators including histamine and tryptase which affects multiple organ systems resulting in mast cell activation disorders (MCAD). The overlap of findings associated with MCAD and the immune-mediated and rheumatologic conditions in patients with hEDS/HSD may provide an explanation for the relationship among these conditions and the presence of chronic inflammatory processes in these patients. It is clear that a multidisciplinary approach is required for the treatment of patients with EDS. However, it is also important for clinicians to consider the summarized symptoms and MCAD-associated characteristics in patients with multiple complaints as possible manifestations of connective tissue disorders, in order to potentially aid in establishing an early diagnosis of EDS.

Biomechanical Studies for Glenoid Based Labral Repairs With Suture Anchors Do Not Use Consistent Testing Methods: A Critical Systematic Review.

PURPOSE: The purpose of this systematic review was to investigate variability in biomechanical testing protocols for laboratory-based studies using suture anchors for glenohumeral shoulder instability and SLAP lesion repair. METHODS: A systematic review of Medline, Embase, Scopus, and Google Scholar using Covidence software was performed for all biomechanical studies investigating labral-based suture anchor repair for shoulder instability and SLAP lesions. Clinical studies, technical notes or surgical technique descriptions, or studies treating glenoid bone loss or capsulorrhaphy were excluded. Risk of bias (ROB) was assessed with the ROBINS-I tool. Study quality was assessed with the Quality Appraisal for Cadaveric Studies. Heterogeneity was assessed with the I2 statistic. RESULTS: A total of 41 studies were included. ROB was serious and critical in 27 studies, moderate in 13, and low in 1; 6 studies had high quality, 21 good quality, 10 moderate quality, 2 low quality, and 2 very low quality. Thirty-one studies used and 22 studies included cyclic loading. Angle of anchor insertion was reported by 33 studies. The force vector for displacement varied. The most common directions were perpendicular to the glenoid (n = 9), and anteroinferior or anterior (n = 8). The most common outcome measures were load to failure (n = 35), failure mode (n = 23), and stiffness (n = 21). Other outcome measures included load at displacement, displacement at failure, tensile load at displacement, translation, energy absorbed, cycles to failure, contact pressure, and elongation. CONCLUSION: This systematic review demonstrated a clear lack of consistency in those cadaver studies that investigated biomechanical properties after surgical repair with suture anchors for shoulder instability and SLAP lesions. Testing methods between studies varied substantially with no universally applied standard for preloading, load to failure and cyclic loading protocols, insertion angles of suture anchors, or direction of loading. To allow comparability between studies standardization of testing protocols is strongly recommended.

Altering Dynamics of Autonomic Processing Therapy (ADAPT) trial: a novel, targeted treatment for reducing anxiety in joint hypermobility.

BACKGROUND: Hypermobility is a poorly recognised and understood musculoskeletal disorder thought to affect around 20% of the population. Hypermobility is associated with reduced physiological and psychological functioning and quality of life and is a known risk factor for the development of an anxiety disorder. To date, no evidence-based, targeted treatment for anxiety in the context of hypermobility exists. The present intervention (ADAPT-Altering Dynamics of Autonomic Processing Therapy) is a novel therapy combining bio-behavioural training with cognitive approaches from clinical health psychology targeting the catastrophisation of internal sensations, with aim to improve autonomic trait prediction error. METHOD: Eighty individuals with diagnosed hypermobility will be recruited and the efficacy of ADAPT to treat anxiety will be compared to an Emotion-Focused Supportive Therapy (EFST) comparator therapy in a randomised controlled trial. The primary treatment target will be post therapy score on the Beck Anxiety Inventory, and secondary outcomes will also be considered in relation to interoception, depression, alexithymia, social and work adjustment, panic symptoms and dissociation. Due to COVID restrictions, the intervention will be moved to online delivery and qualitative assessment of treatment tolerance to online therapy will also be assessed. DISCUSSION: Online delivery of an intervention targeting anxiety would improve the quality of life for those experiencing anxiety disorder and help to reduce the £11.7 billion that anxiety disorders cost the UK economy annually. TRIAL REGISTRATION: World Health Organization ISRCTN17018615 . Registered on 20th February 2019; trial protocol version 2.