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  1. Book ; Online ; E-Book: Symptomatic

    Francomano, Clair A. / Hakim, Alan J. / Henderson, Lansdale G. S. / Henderson Sr., Fraser C.

    the symptom-based handbook for Ehlers-Danlos syndromes and hypermobility spectrum disorders

    2023  

    Title variant Symptom-based handbook for Ehlers-Danlos syndromes and hypermobility spectrum disorders
    Author's details edited by Clair A. Francomano [and more]
    MeSH term(s) Ehlers-Danlos Syndrome ; Joint Instability
    Keywords Ehlers-Danlos syndrome ; Joints/Hypermobility
    Subject code 616.77
    Language English
    Size 1 online resource (662 pages)
    Edition 1st ed.
    Publisher Elsevier
    Publishing place San Diego
    Document type Book ; Online ; E-Book
    Remark Zugriff für angemeldete ZB MED-Nutzerinnen und -Nutzer
    ISBN 0-323-95083-3 ; 9780323950824 ; 978-0-323-95083-1 ; 0323950825
    Database ZB MED Catalogue: Medicine, Health, Nutrition, Environment, Agriculture

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  2. Book: Hypermobility, fibromyalgia and chronic pain

    Hakim, Alan J. / Keer, Rosemary / Grahame, Rodney

    2010  

    Author's details ed. by Alan J. Hakim, Rosemary Keer and Rodney Grahame
    Keywords Joint Instability ; Pain / therapy ; Chronic Disease ; Fibromyalgia / therapy ; Joints--Hypermobility ; Fibromyalgia ; Chronic pain
    Subject code 616.72
    Language English
    Size XXI, 310 S. : Ill., graph. Darst.
    Publisher Churchill Livingstone Elsevier
    Publishing place Edinburgh u.a.
    Publishing country Great Britain
    Document type Book
    HBZ-ID HT016586403
    ISBN 978-0-7020-3005-5 ; 0-7020-3005-8
    Database Catalogue ZB MED Medicine, Health

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  3. Article ; Online: Severity classes in adults with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorder.

    Hakim, Alan J

    Rheumatology (Oxford, England)

    2019  Volume 58, Issue 10, Page(s) 1705–1706

    MeSH term(s) Adult ; Ehlers-Danlos Syndrome ; Humans ; Italy ; Joint Instability ; Pilot Projects
    Language English
    Publishing date 2019-07-15
    Publishing country England
    Document type Editorial ; Comment
    ZDB-ID 1464822-2
    ISSN 1462-0332 ; 1462-0324
    ISSN (online) 1462-0332
    ISSN 1462-0324
    DOI 10.1093/rheumatology/kez134
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  4. Article ; Online: Ehlers-Danlos syndromes, hypermobility spectrum disorders, and associated co-morbidities: Reports from EDS ECHO.

    Hakim, Alan J / Tinkle, Brad T / Francomano, Clair A

    American journal of medical genetics. Part C, Seminars in medical genetics

    2021  Volume 187, Issue 4, Page(s) 413–415

    MeSH term(s) Comorbidity ; Databases, Genetic ; Ehlers-Danlos Syndrome/epidemiology ; Humans ; Joint Instability/epidemiology ; Morbidity
    Language English
    Publishing date 2021-11-18
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2108622-9
    ISSN 1552-4876 ; 0148-7299 ; 1552-4868
    ISSN (online) 1552-4876
    ISSN 0148-7299 ; 1552-4868
    DOI 10.1002/ajmg.c.31954
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  5. Article: Self-reported throat symptoms in Ehlers-Danlos syndromes and hypermobility spectrum disorders: A cross-sectional survey study.

    Williams, Hannah R / Wood, Gary / Hakim, Alan J / Birchall, Martin / Hirani, Shashivadan P

    Laryngoscope investigative otolaryngology

    2023  Volume 8, Issue 5, Page(s) 1259–1264

    Abstract: Objectives: This study identified the frequency and severity of dysphagia, dysphonia, and laryngopharyngeal reflux symptoms in people with Ehlers-Danlos syndromes (EDS) or hypermobility spectrum disorders (HSD) and explored differences between ... ...

    Abstract Objectives: This study identified the frequency and severity of dysphagia, dysphonia, and laryngopharyngeal reflux symptoms in people with Ehlers-Danlos syndromes (EDS) or hypermobility spectrum disorders (HSD) and explored differences between diagnostic groups.
    Methods: Participants were recruited via non-probability convenience sampling. Information was gathered via online survey, including the Reflux Symptom Index (RSI; Belafsky et al.,
    Results: There were 1620 participants (96.6% female, 2.8% male) that met the inclusion criteria. The mean age was 38.09 (SD 12.22). 75.51% had hypermobile EDS (hEDS), 17.83% had HSD and 3.33% had classic EDS (cED). The cohort's mean scores were RSI = 22.95 (SD 9.01), EAT-10 = 11.91 (SD 9.66), and VHI score = 31.99 (SD 24.36). The hEDS group had significantly higher mean scores than the HSD group on RSI score and on some RSI items, on EAT-10 score and on all EAT-10 items, and on one VHI item.
    Conclusion: People with EDS/HSD experience symptoms of acid reflux, dysphagia, and dysphonia to varying degrees with significant differences between hEDS than HSD. Awareness of the impact of EDS/HSD on throat symptoms will enable health care professionals to anticipate throat symptoms more readily in this population, providing individualized and effective management plans.
    Level of evidence: IV.
    Language English
    Publishing date 2023-09-18
    Publishing country United States
    Document type Journal Article
    ISSN 2378-8038
    ISSN 2378-8038
    DOI 10.1002/lio2.1120
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  6. Article ; Online: Initial description and evaluation of EDS ECHO: An international effort to improve care for people with the Ehlers-Danlos syndromes and hypermobility spectrum disorders.

    Schubart, Jane R / Bascom, Rebecca / Francomano, Clair A / Bloom, Lara / Hakim, Alan J

    American journal of medical genetics. Part C, Seminars in medical genetics

    2021  Volume 187, Issue 4, Page(s) 609–615

    Abstract: The Ehlers-Danlos Society Extension for Community Health Care Outcomes (EDS ECHO) is a portfolio of teleconferencing programs developed around the principles and practices of Project ECHO®, aimed at increasing awareness of Ehlers-Danlos syndromes (EDS) ... ...

    Abstract The Ehlers-Danlos Society Extension for Community Health Care Outcomes (EDS ECHO) is a portfolio of teleconferencing programs developed around the principles and practices of Project ECHO®, aimed at increasing awareness of Ehlers-Danlos syndromes (EDS) and hypermobility spectrum disorders (HSD) among clinicians, enhancing their confidence in the assessment and management of these complex conditions, and generating networks of clinicians across specialties. We assessed the outcomes of the first EDS ECHO program, launched in April 2019, with two hub locations: Indiana University Health, Indianapolis, Indiana, USA, and The Royal Society of Medicine, London, UK. Clinicians were surveyed before and 6 months after their participation. We describe the initial outcomes of the first four EDS ECHO programs, each comprising nine sessions. Participants reported increased levels of knowledge and confidence in providing care (93% and 95%, respectively) and an increase in referral network participation (65%). Additionally, 80% reported that their interest in EDS and HSD increased; 57% reporting a great increase. Also, 59% reported a decrease in feeling overwhelmed by EDS and HSD, and nearly half reported a decrease in their level of frustration because of participating in EDS ECHO. Clinicians participating in EDS ECHO programs know more, have more confidence in their ability to provide care, and feel energized by their participation.
    MeSH term(s) Ehlers-Danlos Syndrome/therapy ; Humans ; Joint Instability/diagnostic imaging ; Surveys and Questionnaires
    Language English
    Publishing date 2021-11-25
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2108622-9
    ISSN 1552-4876 ; 0148-7299 ; 1552-4868
    ISSN (online) 1552-4876
    ISSN 0148-7299 ; 1552-4868
    DOI 10.1002/ajmg.c.31960
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  7. Article ; Online: Recognizing the scale of joint hypermobility burden: comment on the article by Mulvey et al.

    Hakim, Alan J / Grahame, Rodney

    Arthritis care & research

    2014  Volume 66, Issue 3, Page(s) 496

    MeSH term(s) Female ; Humans ; Joint Instability/epidemiology ; Male ; Musculoskeletal Pain/epidemiology ; Population
    Language English
    Publishing date 2014-03
    Publishing country United States
    Document type Comment ; Letter
    ZDB-ID 645059-3
    ISSN 2151-4658 ; 0893-7524 ; 2151-464X
    ISSN (online) 2151-4658
    ISSN 0893-7524 ; 2151-464X
    DOI 10.1002/acr.22221
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  8. Article ; Online: Pediatric joint hypermobility: a diagnostic framework and narrative review.

    Tofts, Louise Jane / Simmonds, Jane / Schwartz, Sarah B / Richheimer, Roberto M / O'Connor, Constance / Elias, Ellen / Engelbert, Raoul / Cleary, Katie / Tinkle, Brad T / Kline, Antonie D / Hakim, Alan J / van Rossum, Marion A J / Pacey, Verity

    Orphanet journal of rare diseases

    2023  Volume 18, Issue 1, Page(s) 104

    Abstract: Background: Hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD) are debilitating conditions. Diagnosis is currently clinical in the absence of biomarkers, and criteria developed for adults are difficult to use in ... ...

    Abstract Background: Hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD) are debilitating conditions. Diagnosis is currently clinical in the absence of biomarkers, and criteria developed for adults are difficult to use in children and biologically immature adolescents. Generalized joint hypermobility (GJH) is a prerequisite for hEDS and generalized HSD. Current literature identifies a large proportion of children as hypermobile using a Beighton score ≥ 4 or 5/9, the cut off for GJH in adults. Other phenotypic features from the 2017 hEDS criteria can arise over time. Finally, many comorbidities described in hEDS/HSD are also seen in the general pediatric and adolescent population. Therefore, pediatric specific criteria are needed. The Paediatric Working Group of the International Consortium on EDS and HSD has developed a pediatric diagnostic framework presented here. The work was informed by a review of the published evidence.
    Observations: The framework has 4 components, GJH, skin and tissue abnormalities, musculoskeletal complications, and core comorbidities. A Beighton score of ≥ 6/9 best identifies children with GJH at 2 standard deviations above average, based on published general population data. Skin and soft tissue changes include soft skin, stretchy skin, atrophic scars, stretch marks, piezogenic papules, and recurrent hernias. Two symptomatic groups were agreed: musculoskeletal and systemic. Emerging comorbid relationships are discussed. The framework generates 8 subgroups, 4 pediatric GJH, and 4 pediatric generalized hypermobility spectrum disorders. hEDS is reserved for biologically mature adolescents who meet the 2017 criteria, which also covers even rarer types of Ehlers-Danlos syndrome at any age.
    Conclusions: This framework allows hypermobile children to be categorized into a group describing their phenotypic and symptomatic presentation. It clarifies the recommendation that comorbidities should be defined using their current internationally accepted frameworks. This provides a foundation for improving clinical care and research quality in this population.
    MeSH term(s) Adult ; Adolescent ; Humans ; Child ; Joint Instability/diagnosis ; Ehlers-Danlos Syndrome/diagnosis ; Connective Tissue Diseases ; Skin
    Language English
    Publishing date 2023-05-04
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 2225857-7
    ISSN 1750-1172 ; 1750-1172
    ISSN (online) 1750-1172
    ISSN 1750-1172
    DOI 10.1186/s13023-023-02717-2
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  9. Article ; Online: Arachnodactyly--a key to diagnosing heritable disorders of connective tissue.

    Grahame, Rodney / Hakim, Alan J

    Nature reviews. Rheumatology

    2013  Volume 9, Issue 6, Page(s) 358–364

    Abstract: Arachnodactyly literally means spidery fingers, and describes the long, slender fingers typical of patients with Marfan syndrome (MFS). Many clinicians regard arachnodactyly as pathognomonic of MFS; however, this view is misleading as arachnodactyly is a ...

    Abstract Arachnodactyly literally means spidery fingers, and describes the long, slender fingers typical of patients with Marfan syndrome (MFS). Many clinicians regard arachnodactyly as pathognomonic of MFS; however, this view is misleading as arachnodactyly is a key element of the marfanoid habitus, which is present in several heritable disorders of connective tissue (HDCTs). Other features of the marfanoid habitus include long hands and feet, increased skin stretch, joint hypermobility and characteristic changes in the physiology of the pectum. Here, we focus on the differential diagnosis of diseases with features of the marfanoid habitus. Ectopia lentis (lens dislocation) and aortic root dilation or dissection are cardinal features of MFS. Distinguishing MFS from other HCDTs has important implications for treatment, as cardiovascular and ocular complications commonly seen in patients with MFS are not seen in all HDCTs. Joint hypermobility syndrome and Ehlers-Danlos syndrome are also HDCTs, neither of which is associated with ectopia lentis or aortic changes. Some of the rarer forms of Ehlers-Danlos syndrome are associated with severe vascular, dental and skin pathologies. This Review serves as a guide for correctly diagnosing members of the HDCT family.
    MeSH term(s) Arachnodactyly/etiology ; Connective Tissue Diseases/congenital ; Connective Tissue Diseases/diagnosis ; Humans
    Language English
    Publishing date 2013-03-12
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2491532-4
    ISSN 1759-4804 ; 1759-4790
    ISSN (online) 1759-4804
    ISSN 1759-4790
    DOI 10.1038/nrrheum.2013.24
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  10. Article ; Online: Use of Cluster Analysis to Delineate Symptom Profiles in an Ehlers-Danlos Syndrome Patient Population.

    Schubart, Jane R / Schaefer, Eric / Hakim, Alan J / Francomano, Clair A / Bascom, Rebecca

    Journal of pain and symptom management

    2019  Volume 58, Issue 3, Page(s) 427–436

    Abstract: Context: The Ehlers-Danlos Syndromes (EDSs) are a set of rare heritable disorders of connective tissue, characterized by defects in the structure and synthesis of extracellular matrix elements that lead to a myriad of problems including joint ... ...

    Abstract Context: The Ehlers-Danlos Syndromes (EDSs) are a set of rare heritable disorders of connective tissue, characterized by defects in the structure and synthesis of extracellular matrix elements that lead to a myriad of problems including joint hypermobility and skin abnormalities. Because EDS affects multiple organ systems, defining clear boundaries and recognizing overlapping clinical features shared by disease phenotypes is challenging.
    Objectives: The objective of this study was to seek evidence of phenotypic subgroups of patients with distinctive symptom profiles and describe these resulting subgroups.
    Methods: Data were extracted from a repository assembled 2001-2013 by the National Institute on Aging Intramural Research Program. Agglomerative hierarchical clustering was used to form distinct subgroups of patients with respect to the domains of pain, physical and mental fatigue, daytime sleepiness, and nighttime sleep. Domains were selected based on literature review, clinician expertise, and guidance from patient advisors.
    Results: One hundred seventy-five patients met all inclusion criteria. Three subgroups were identified. The Pain Dominant subgroup (39 patients) had the highest mean pain values, but lowest mean values of other symptoms. The High Symptom Burden subgroup (71 patients) had high mean values in all domains. The Mental Fatigue subgroup (65 patients) had a high mean value for mental fatigue and daytime sleepiness, but a lower mean value for pain.
    Conclusion: The subgroups aligned with clinical observation of the heterogeneous nature of EDS, with overlapping symptoms between subtypes and a wide divergence in degree of symptoms within subtypes. This exploratory study helps characterize the various phenotypes and comorbidities of patients with EDS.
    MeSH term(s) Adult ; Cluster Analysis ; Databases, Factual ; Ehlers-Danlos Syndrome/diagnosis ; Ehlers-Danlos Syndrome/physiopathology ; Female ; Humans ; Male ; Middle Aged ; Pain/diagnosis ; Pain/physiopathology ; Phenotype ; Surveys and Questionnaires ; Symptom Assessment ; Young Adult
    Language English
    Publishing date 2019-05-31
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 639142-4
    ISSN 1873-6513 ; 0885-3924
    ISSN (online) 1873-6513
    ISSN 0885-3924
    DOI 10.1016/j.jpainsymman.2019.05.013
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