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Prevalence of Atopic Disease in Pediatric Patients with Hypermobile Ehlers-Danlos Syndrome
Jordan T Jones1, Kathleen G Click2, Ashley Lytch1
1Children's Mercy Kansas City, Kansas City, Missouri, USA.
Insights
Pediatric patients with hypermobile Ehlers-Danlos syndrome (hEDS) show a higher prevalence of atopic conditions like allergic rhinitis and asthma compared to the general US pediatric population. Healthcare providers should monitor for these allergies in children with hEDS.
Area of Science:
- Pediatric Allergy and Immunology
- Genetics and Rare Diseases
- Connective Tissue Disorders
Background:
- Ehlers-Danlos syndromes (EDS) are connective tissue disorders causing skin laxity and joint hypermobility.
- EDS is linked to various comorbidities requiring multidisciplinary care.
- While adults with EDS have higher rates of atopic conditions, pediatric prevalence remains understudied.
Purpose of the Study:
- To determine the prevalence of atopic conditions (allergies, asthma, atopic dermatitis) in pediatric patients with hypermobile EDS (hEDS).
- To compare these prevalences to the general United States pediatric population.
Main Methods:
- A convenience sample of 149 pediatric patients (aged 8-22) diagnosed with hEDS and atopic disease was analyzed.
- Data on atopic conditions and medication use were systematically reviewed.
Main Results:
- The study included predominantly female (85%) and Caucasian (89%) patients with a mean age of 15.5 years.
- Prevalence rates were higher for allergic rhinitis (46%), medication allergies (44%, notably antibiotics), and asthma (39%, mostly mild intermittent) compared to the general US pediatric population.
- Atopic dermatitis prevalence was not significantly different from the general population.
Conclusions:
- Pediatric patients with hEDS exhibit an increased prevalence of atopic conditions compared to their non-hEDS counterparts in the US.
- Healthcare providers should proactively screen for and monitor atopic and allergic conditions in pediatric hEDS patients.
- Consultation with allergy and pulmonology specialists is recommended for symptomatic pediatric patients with hEDS.
Abstract:
Background:The Ehlers-Danlos syndromes (EDSs) are a group of connective tissue disorders characterized by skin laxity and hypermobile joints. EDS is associated with many comorbidities that often necessitate multidisciplinary care. Adults with EDS report more atopic conditions such as allergic rhinitis and asthma; however, gaps exist around the prevalence of atopic conditions in pediatric patients with EDS. The objective of this study was to determine the prevalence of atopic conditions (allergies, asthma, and atopic dermatitis) in pediatric patients with hypermobile EDS (hEDS) and compare it to the general pediatric population in the United States.Methods:A convenience sample of 149 patients aged 8-22 years, with a diagnosis of hEDS and atopic disease was evaluated. Atopic conditions and medication use were reviewed.Results:Patients were mostly females (85%) and Caucasian (89%) with an average age of 15.5 years (standard deviation: 3.1). Many patients had allergic rhinitis (46%); medication allergies (44%), with antibiotics as the most common; and asthma (39%), mild intermittent as the most common severity. All prevalences were higher compared to the general United States pediatric population except for atopic dermatitis.Conclusions:There was an increased prevalence of atopic conditions in pediatric patients with hEDS compared to the United States pediatric population. Due to the increased prevalence of atopic conditions, health care providers should be aware of and monitor for signs of atopic and allergic conditions in pediatric patients with hEDS. Allergy and pulmonology specialists should be considered in the management of pediatric patients with hEDS if symptoms are present.
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