2017 EDS International Classification

The new nosology for the Ehlers-Danlos syndromes (EDS) was published in 2017. It identifies 13 different types of EDS, and highlights the need for an updated and more reliable criteria. For the first time management and care guidelines were also produced for the co-morbidities that can occur within EDS and HSD.

As part of this work, the diagnostic term hypermobility spectrum disorders was introduced for symptomatic joint hypermobility in the absence of signs of a heritable disorder of connective tissue such as EDS or any other condition responsible for hypermobility-related disease.

For further information, please visit the links below:

American Journal of Medical Genetics Part C: Seminars in Medical Genetics
Supplement to the American Journal of Genetics

Issue Information

Table of Contents, Volume 175C, Number 1, March 2017 (pages 1–2)

Introduction

The international consortium on the Ehlers–Danlos syndromes (pages 5–7)
Lara Bloom, Peter Byers, Clair Francomano, Brad Tinkle, Fransiska Malfait and on behalf of the Steering Committee of The International Consortium on the Ehlers-Danlos Syndromes

Research Article

The 2017 international classification of the Ehlers–Danlos syndromes (pages 8–26)
Fransiska Malfait, Clair Francomano, Peter Byers, John Belmont, Britta Berglund, James Black, Lara Bloom, Jessica M. Bowen, Angela F. Brady, Nigel P. Burrows, Marco Castori, Helen Cohen, Marina Colombi, Serwet Demirdas, Julie De Backer, Anne De Paepe, Sylvie Fournel-Gigleux, Michael Frank, Neeti Ghali, Cecilia Giunta, Rodney Grahame, Alan Hakim, Xavier Jeunemaitre, Diana Johnson, Birgit Juul-Kristensen, Ines Kapferer-Seebacher, Hanadi Kazkaz, Tomoki Kosho, Mark E. Lavallee, Howard Levy, Roberto Mendoza-Londono, Melanie Pepin, F. Michael Pope, Eyal Reinstein, Leema Robert, Marianne Rohrbach, Lynn Sanders, Glenda J. Sobey, Tim Van Damme, Anthony Vandersteen, Caroline van Mourik, Nicol Voermans, Nigel Wheeldon, Johannes Zschocke and Brad Tinkle

Research Review

Ehlers–Danlos syndrome, classical type (pages 27–39)
Jessica M. Bowen, Glenda J. Sobey, Nigel P. Burrows, Marina Colombi, Mark E. Lavallee, Fransiska Malfait and Clair A. Francomano

Research Articles

Diagnosis, natural history, and management in vascular Ehlers–Danlos syndrome (pages 40–47)
Peter H. Byers, John Belmont, James Black, Julie De Backer, Michael Frank, Xavier Jeunemaitre, Diana Johnson, Melanie Pepin, Leema Robert, Lynn Sanders and Nigel Wheeldon

Hypermobile Ehlers–Danlos syndrome (a.k.a. Ehlers–Danlos syndrome Type III and Ehlers–Danlos syndrome hypermobility type): Clinical description and natural history (pages 48–69)
Brad Tinkle, Marco Castori, Britta Berglund, Helen Cohen, Rodney Grahame, Hanadi Kazkaz and Howard Levy

Research Reviews

The Ehlers–Danlos syndromes, rare types (pages 70–115)
Angela F. Brady, Serwet Demirdas, Sylvie Fournel-Gigleux, Neeti Ghali, Cecilia Giunta, Ines Kapferer-Seebacher, Tomoki Kosho, Roberto Mendoza-Londono, Michael F. Pope, Marianne Rohrbach, Tim Van Damme, Anthony Vandersteen, Caroline van Mourik, Nicol Voermans, Johannes Zschocke and Fransiska Malfait

Measurement properties of clinical assessment methods for classifying generalized joint hypermobility—A systematic review (pages 116–147)
Birgit Juul-Kristensen, Karoline Schmedling, Lies Rombaut, Hans Lund and Raoul H. H. Engelbert

Research Articles

A framework for the classification of joint hypermobility and related conditions (pages 148–157)
Marco Castori, Brad Tinkle, Howard Levy, Rodney Grahame, Fransiska Malfait and Alan Hakim

The evidence-based rationale for physical therapy treatment of children, adolescents, and adults diagnosed with joint hypermobility syndrome/hypermobile Ehlers Danlos syndrome (pages 158–167)
Raoul H.H. Engelbert, Birgit Juul-Kristensen, Verity Pacey, Inge de Wandele, Sandy Smeenk, Nicoleta Woinarosky, Stephanie Sabo, Mark C. Scheper, Leslie Russek and Jane V. Simmonds

Cardiovascular autonomic dysfunction in Ehlers–Danlos syndrome—Hypermobile type (pages 168–174)
Alan Hakim, Chris O’Callaghan, Inge De Wandele, Lauren Stiles, Alan Pocinki and Peter Rowe

Chronic fatigue in Ehlers–Danlos syndrome—Hypermobile type (pages 175–180)
Alan Hakim, Inge De Wandele, Chris O’Callaghan, Alan Pocinki and Peter Rowe

Gastrointestinal involvement in the Ehlers–Danlos syndromes (pages 181–187)
Asma Fikree, Gisela Chelimsky, Heidi Collins, Katcha Kovacic and Qasim Aziz

Orthopaedic management of the Ehlers–Danlos syndromes (pages 188–194)
William B. Ericson Jr. and Roger Wolman

Research Reviews

Neurological and spinal manifestations of the Ehlers–Danlos syndromes (pages 195–211)
Fraser C. Henderson Sr., Claudiu Austin, Edward Benzel, Paolo Bolognese, Richard Ellenbogen, Clair A. Francomano, Candace Ireton, Petra Klinge, Myles Koby, Donlin Long, Sunil Patel, Eric L. Singman and Nicol C. Voermans

Pain management in the Ehlers–Danlos syndromes (pages 212–219)
Pradeep Chopra, Brad Tinkle, Claude Hamonet, Isabelle Brock, Anne Gompel, Antonio Bulbena and Clair Francomano

Research Article

Oral and mandibular manifestations in the Ehlers–Danlos syndromes (pages 220–225)
John Mitakides and Brad T. Tinkle

Research Reviews

Mast cell disorders in Ehlers–Danlos syndrome (pages 226–236)
Suranjith L. Seneviratne, Anne Maitland and Lawrence Afrin

Psychiatric and psychological aspects in the Ehlers–Danlos syndromes (pages 237–245)
Antonio Bulbena, Carolina Baeza-Velasco, Andrea Bulbena-Cabré, Guillem Pailhez, Hugo Critchley, Pradeep Chopra, Nuria Mallorquí-Bagué, Charissa Frank and Stephen Porges

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