Children with the inherited disorder known as Ehlers Danlos type 3 syndrome may be at high risk for severe food allergy, British researchers reported here.

Among a cohort of 19 children with this syndrome, which is characterized by joint hypermobility and cardiovascular involvement, all had gastrointestinal symptoms found to be allergic in origin, Rebecca M. Cutts, MBBS, of Great Ormond Street Children’s Hospital in London, and colleagues reported at the annual meeting of the American Academy of Allergy, Asthma, and Immunology.

Ehlers Danlos syndrome is a group of connective tissue disorders caused by mutations in genes that regulate collagen deposition, with type 3 being the most common.

Type 3 results from mutations in at least one of two genes, and can be inherited either in an autosomal dominant or recessive manner.

Along with joint laxity, the condition often manifests skin elasticity, bruising, inadequate wound healing, and, in adults, commonly causes abdominal symptoms including pain and reflux.

Little is known about the specific gastrointestinal manifestations in young children, however, so Cutts and colleagues conducted a retrospective study at their institution, where members of the gastroenterology department have special expertise in food allergy.

The children, 11 of whom were boys, averaged slightly over 8 years-old and had confirmed Ehlers Danlos syndrome as measured clinically on the Beighton hypermobility scoring system.

Food elimination testing found that 74% were allergic to milk, egg, wheat, and soy, with small numbers also becoming symptomatic on exposure to dairy, nuts, gluten, and bananas.

Elimination of the offending foods from the diet was insufficient for symptom control, however, Cutts said in a poster session.

“This is a population that is extremely difficult to manage,” she said.

Artificial nutrition was needed for 13 of the children either via gastrostomy or parenterally.

Symptoms included abdominal pain (90%) and constipation (90%), and the most common food allergic diagnoses were eosinophilic colitis and allergic dysmotility.

Multiple medications were needed, including antihistamines and immunosuppressants in 100% and proton pump inhibitors in two-thirds.

“Severity of this syndrome can range from mild to life-threatening,” the researcher explained.

In this cohort, urologic complications were present in 28% and cardiovascular comorbidities in 39%.

The children needed care from multiple healthcare sources, including physiotherapists and dietitians along with gastroenterologists, cardiologists, and urologists.

“We don’t know why this genetic disorder is associated with food allergies, or even if there is causality involved,” Cutt told MedPage Today.

Further prospective studies are warranted to more fully explore these associations, she said.

“This is brand new to us - it’s a work in progress,” she said.

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Primary source: American Academy of Allergy, Asthma, and Immunology
Source reference: Cutts R, et al “Gastrointestinal food allergies in children with Ehlers Danlos type 3 syndrome” AAAAI 2012; Abstract 129.