The Ehlers-Danlos syndromes (EDS) are known to be associated with increased bleeding symptoms, including easy bruising, gum bleeding, and abnormal uterine bleeding. This bleeding is due to modifications in collagen that cause fragility of vessel walls, decreased support of those vessels, and affect the function of platelets (found in the blood, supporting healing, and important in helping to stop bleeding).
However, bleeding and bruising in people with generalized joint hypermobility (GJH), hypermobility spectrum disorders, and hypermobile Ehlers-Danlos syndrome has not been well-defined, particularly within the pediatric and adolescent populations.
As excessive bleeding symptoms could significantly impact overall health and quality of life, defining the general incidence and natural course of bleeding symptoms in this population are important. Early recognition in childhood or adolescence may help prevent bleeding and bruising concerns such as excessive bleeding during surgery or after trauma, and even incorrect diagnosis of physical abuse.
Led by Dr. Nicole Kendel, member of the International Consortium on Ehlers-Danlos Syndromes and Hypermobility Spectrum Disorders Hematology Working Group, a team from Akron and Columbus, Ohio, USA recently carried out research to assess bleeding symptoms in young people with generalized/benign joint hypermobility (GJH), compare bleeding scores to healthy historical pediatric controls, and determine whether a correlation exists between Beighton scores and bleeding scores.
The research team assessed eighty-one children aged between 10 and 16 years old with GJH. Seventy-eight percent of participants had abnormal bleeding scores, with a mean bleeding score significantly elevated compared to healthy controls. Commonly observed bleeding symptoms were oral bleeding (74%), easy bruising (59%), and bleeding with minor wounds (42%).
Bleeding scores did not depend on biological sex or nonsteroidal anti-inflammatory drug use, although there was a positive correlation with increasing age. Also, no correlation was found between a participant’s bleeding tendency and their Beighton score.
Subsequently, the researchers propose that screening for bleeding symptoms should be integrated into routine care for people with GJH, with referral to hematology clinics for people with bleeding concerns.
The research insights from this study were presented as a poster at The Ehlers-Danlos Society’s International Scientific Symposium on the Ehlers-Danlos Syndromes and Hypermobility Spectrum Disorders, Rome, Italy, September 2022.
The Ehlers-Danlos Society funded the Open Access of this work to enable everyone around the world to view the research paper for free.
Assessing Bleeding Symptoms in Pediatric Patients With Generalized Joint Hypermobility
Nicole E. Kendel, Joseph R. Stanek, Bethanne B. Thomas, Stacy P. Ardoin, Sarah H. O’Brien
https://doi.org/10.1002/acr.25074
