Assessment of gastrointestinal function and its' effect on bone mineral density and body composition in hypermobility spectrum disorder and hypermobile Ehlers-Danlos syndrome.

BACKGROUND: Hypermobile Ehlers-Danlos Syndrome (hEDS) and Hypermobility Spectrum Disorders (HSD) are associated with hypermobility, musculoskeletal pain, a decreased bone mineral density (BMD) and gastrointestinal (GI) complications. The role of GI symptoms and diet in BMD has not been established in this population. The GI complications can lead to an energy deficit due to lack of essential macronutrients. The primary objective of this study was to determine the severity of GI symptoms compared to body composition and BMD in individuals with hEDS/HSD. The secondary objective is to examine GI symptoms on energy balance, body composition and strength. METHODOLOGY: This study was IRB approved. Eighteen female participants (aged 28.2 ± 4.9; BMI 22.5 ± 4.9) with a diagnosis of hEDS or HSD and 18 female healthy control participants (aged 28.1 ± 3.8; BMI 22.8 ±3.9) signed consent to participate. Participants were matched by sex, age, and BMI. The Gastrointestinal Symptom Rating Scale (GSRS) was used to investigate severity of GI symptoms. Dual X-ray absorptiometry was used to determine body composition (body fat%, lean body mass (LBM). BMD was measured by Z- scores of both femurs and lumbar spine. Resting metabolic rate (RMR) was measured using indirect calorimetry and strength was determined using a hand grip dynamometer. RESULTS: All hEDS/HSD participants reported GI symptoms. There was no difference in body composition between hEDS/HSD and controls. Participants with hEDS/HSD had lower BMD both femoral z scores (p=0.02,0.004) and spine z scores (p= 0.04). There was no difference in caloric intake between groups; yet both groups demonstrated caloric deficits. Additionally, hEDS/HSD consumed less protein and more carbohydrates (p=0.03, p=0.03). There were no differences in grip strength. CONCLUSIONS: This study identified that pre-menopausal women with hEDS/HSD presented with significant GI complications and lower BMD than age matched controls. The GI complications and the reduced protein intake long-term may have a lasting impact on bone health. This study found that the GSRS identified and quantified GI symptoms in persons with hEDS/HSD. Future studies are needed for the longitudinal effects of a caloric/protein deficit in this population and to help guide future preventive and nutritional treatment approaches in individuals with hEDS/HSD.

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.

The psychological burden associated with Ehlers-Danlos syndromes: a systematic review.

CONTEXT: Ehlers-Danlos syndromes (EDS) are disorders of connective tissue that lead to a wide range of clinical presentations. While we are beginning to understand the association between EDS and psychological manifestations, it is critical that we further elucidate the relationship between the two. Understanding the correlation between EDS and mental health will better ensure swift diagnosis and effective treatment for patients. OBJECTIVES: This study aims to systematically examine and report the prevalence of psychiatric disorders in the EDS population. METHODS: The PubMed database was searched on June 14, 2021 for articles published from January 2011 to June 2021. We included original, evidence-based, peer-reviewed journal articles in English that reported information on psychiatric disorders among EDS patients. Psychiatric disorders and psychological conditions were limited to those included in the "psychology" and "mental disorders" Medical Subject Headings (MeSH) search terms defined by the National Library of Medicine. Publications identified utilizing this search strategy by M.K. were imported into the Covidence system, where they first underwent a title and abstract screening process by three independent reviewers (M.K., K.L., H.G.). During the full-text review, two independent reviewers read the full text of the questionable articles to assess their eligibility for inclusion. Studies were excluded if they did not meet our target objective or if they were not in English or if they were opinion pieces, conference abstracts, or review articles. Data were extracted from the shortlisted studies by reviewers. During the data extraction phase, the quality and risk of publication bias were assessed by two independent reviewers utilizing the National Institutes of Health (NIH) Study Quality Assessment Tools. Any disagreements in study selection, data extraction, or quality assessment were adjudicated via discussion between the two reviewers, utilizing a third reviewer as a decider if necessary. RESULTS: Out of 73 articles identified, there were no duplicates. A total of 73 records were screened, but only 40 articles were assessed in full text for eligibility. A total of 23 articles were ultimately included, which collectively discussed 12,298 participants. Ten (43.5%) of the included studies were cross-sectional in design, three (13.0%) were case reports, and three (13.0%) were retrospective chart reviews. The remaining seven (30.4%) articles were either case-control, cohort, qualitative, controlled observational, or validation studies. Twelve (52.2%) of the studies reported data on depression disorders, six of which reported prevalence data. Nine (39.1%) of the studies reported data on anxiety disorders, five of which reported prevalence data. Studies that reported nonprevalence data presented odds-ratio, mean scores on psychiatric evaluations, and other correlation statistics. Psychiatric disorders that were most reported in these articles were mood disorders (n=11), anxiety disorders (n=9), and neurodevelopmental disorders (n=7). Although the reports varied, the highest psychiatric prevalence reports in EDS patients involved language disorders (63.2%), attention-deficit/hyperactivity disorder (ADHD) (52.4%), anxiety (51.2%), learning disabilities (42.4%), and depression (30.2%). CONCLUSIONS: Although mood disorders were cited in more articles, the highest reported prevalence was for language disorders and ADHD. This discrepancy highlights the importance of performing more research to better understand the relationship between EDS and psychiatric disorders.

A Global Perspective of Mentorship in Medical Schools: Systematic Review from 2014 to 2019.

Formal mentorship programs afford medical students research involvement, career guidance, and personal and professional development. We reviewed a total of 36 studies on mentorship in medical schools, 17 of which are from the United States (US) and 19 from outside the US. This descriptive study has determined the most common mentorship models used in medical schools both in the United States and internationally. Despite variance between programs, medical students in the US and worldwide prefer highly invested mentors with strong interpersonal skills. We concluded that both US and international mentorship provide crucial professional development for students; however, US-based mentorship is more focused on providing experience within individual specialties. More studies on current mentorship practices in medical schools are required to ensure that the diverse international medical education community is better represented.

The dermatological aspects of hEDS in women.

Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders that may present with a wide range of multisystemic symptoms. Hypermobile EDS, one of 13 identified subtypes of EDS, is the only variant without a known associated genetic mutation. A review of the literature suggests the five primary dermatological changes associated with hypermobile EDS are soft skin, atrophic cutaneous scars, piezogenic papules, hyperextensive stretchability, and hematomas. Our paper will address these cutaneous manifestations and delve into how they affect patients (primarily women). Possible consequences and treatment options for these different dermatological changes, as well as other skin manifestations such as livedo reticularis and elastosis perforans serpiginosa, will also be further explored.

Erratum to "The Dermatological Aspects of hEDS in Women". [International Journal of Women's Dermatology 7/3 (2021) 285- 289].

[This corrects the article DOI: 10.1016/j.ijwd.2021.01.020.].

The Many Facets of Hypermobile Ehlers-Danlos Syndrome.

Of the 13 subtypes of Ehlers-Danlos Syndromes (EDSs) identified in the 2017 international classification of EDSs, 12 have a recognized, associated genetic mutation. However, hypermobile EDS (hEDS) currently has no identifiable associated gene. Therefore, patients with hEDS are identified through a set of clinical diagnosis guidelines and criteria, which are meant to differentiate hEDS from other hypermobile joint conditions and other EDSs subtypes. In this article, the authors provide an overview of hEDS symptoms and comborbidities, current treatment options, and the clinical criteria currently guiding the standard of care.

Incorporating a Standardized Online Professionalism Curriculum in Osteopathic Medical School.

Online social media platforms increase patients' access to physicians, thus potentially blurring the boundaries between physicians' professional and private lives. Although many medical organizations have established guidelines on how physicians should proceed on social media and social networking sites (SNSs), there has not been a mandated standardized curriculum on the use of social media, SNSs, and online professionalism for physicians or medical students. With the increase in physician involvement on social media and SNSs, professionalism issues can emerge. Online professionalism complaints have been increasing. Therefore, osteopathic medical students need to be taught the dangers and benefits of engaging with online media. This article seeks to address the need for mandating a standardized online professionalism curriculum for osteopathic medical students and show how a simulation-based medical education curriculum can help accomplish this goal.

Using the Flipped Classroom With Simulation-Based Medical Education to Engage Millennial Osteopathic Medical Students.

With advances in technology and the advent of social media and social networking sites, the way medical education is taught to millennials must evolve. The "flipped classroom," which occurs when students are exposed to learning materials before their assigned face-to-face class or didactic session, can help engage all learning styles. A flipped classroom paired with simulation-based medical education (SBME) engages millennial learners to acquire the large amount of information required in osteopathic medical school. Using this paired approach in osteopathic medical education can help students meet the requirements outlined by the Accreditation Council for Graduate Medical Education (ACGME). With the single accreditation system for graduate medical education, finalizing in 2020, this article proposes mandating a flipped classroom with SBME to better prepare osteopathic medical students for their training in ACGME-accredited programs.

Using Simulation-Based Medical Education to Meet the Competency Requirements for the Single Accreditation System.

Simulation-based medical education can provide medical training in a nonjudgmental, patient-safe, and effective environment. Although simulation has been a relatively new addition to medical education, the aeronautical, judicial, and military fields have used simulation training for hundreds of years, with positive outcomes. Simulation-based medical education can be used in a variety of settings, such as hospitals, outpatient clinics, medical schools, and simulation training centers. As the author describes in the present article, residencies currently accredited by the American Osteopathic Association can use a simulation-based medical education curriculum to meet training requirements of the 6 competencies identified by the Accreditation Council for Graduate Medical Education. The author also provides specific guidance on providing training and assessment in the professionalism competency.