Constipation, digestive symptoms common in young hEDS patients

Gastrointestinal screening for issues ‘should be routine,’ researchers say

Patricia Inácio, PhD avatar

by Patricia Inácio, PhD |

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A person seated against pillows is seen hunched over in pain with both arms folded over the abdomen.

Constipation and trouble swallowing are common digestive symptoms in children and young adults with joint hypermobility syndrome or hypermobile Ehlers-Danlos syndrome (hEDS), according to a single-center study in the U.S.

Also, about 20% of patients had eosinophilic esophagitis, an allergic reaction in the esophagus — the muscular tube that carries food and liquid into the stomach — that is triggered by eosinophils, a type of immune cells.

Given the high prevalence of such digestive symptoms, screening for gastrointestinal issues in these patient populations “should be routine, with further investigations and referrals guided by identified symptoms,” the researchers wrote.

Their study, “High prevalence of gastrointestinal disorders in a large cohort of patients with joint hypermobility,” was published in the Journal of Pediatric Gastroenterology and Nutrition.

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Joint hypermobility syndrome, called JHS, and hypermobile EDS share similar manifestations. Collectively, they affect the body’s connective tissue and are characterized by hypermobile joints —that is, joints that move past the expected range of motion. While these patients also may have digestive tract symptoms, such manifestations are poorly characterized in affected children.

Here, researchers at Cincinnati Children’s Hospital Medical Center, in Ohio, and the Children’s Hospital of Pittsburgh, in Pennsylvania, reviewed clinical data from 435 patients diagnosed with JHS or hEDS who had gastrointestinal symptoms. The patients’ median age was 16, and 66% were female. As hEDS and JHS both are under the umbrella term hypermobility spectrum disorders, no distinction was made between these two diagnoses in the analysis.

According to the study, constipation was the most commonly reported symptom, affecting 61% of these children and young adults, followed by dysphagia, or difficulty swallowing, which affected 32%.

A quarter of the patients reported abdominal pain, nausea after having a meal, vomiting, early satiety or feeling full, or weight loss.

Future prospective trials to determine the natural history of symptom progression, treatment outcomes, and translational studies analyzing connective tissue dynamics involved in GI [gastrointestinal] disorders are needed in this population.

Based on their symptoms, 79% underwent further tests, such as an endoscopy — an exam to visualize the upper part of the digestive tract — and/or motility tests that assess how well food travels through the digestive tract.

Among the 293 patients who underwent an upper gastrointestinal tract endoscopy, 34% had abnormal results in biological tissues. Eosinophilic esophagitis was the most common diagnosis, followed by celiac disease, which is an immune reaction to eating gluten, then acid reflux and chronic gastritis, or an inflamed stomach lining.

A total of 111 patients underwent a colonoscopy, an exam to look at the large intestine and the rectum, with 24% showing abnormal findings. The most common was eosinophilic colitis, or eosinophil-triggered injury and irritation in the colon, followed by microscopic colitis, or an inflamed colon, and inflammatory bowel disease.

Impaired swallowing was found for 14 patients, according to an appropriate test. Esophageal dysmotility, meaning dysfunction of the esophagus, was the most reported symptom.

Additionally, 50 participants were found to have a longer than normal digestion period, needing four hours to have food passing down the stomach.

Motility studies revealed abnormal results in 25 of 80 patients (31%). The most common symptoms included acid reflex, as well as poor motility in the esophagus, small intestine, and colon.

Eosinophilic esophagitis was significantly more common in patients who reported swallowing problems compared with those who did not (33% vs. 16%.) This means that the odds of eosinophilic esophagitis were 2.15 times higher in patients with swallowing issues than in those without such problems.

The researchers noted that “a high prevalence of constipation [and] dysphagia … in our cohort of patients with hEDS/JHS.”

Overall, “future prospective trials to determine the natural history of symptom progression, treatment outcomes, and translational studies analyzing connective tissue dynamics involved in GI [gastrointestinal] disorders are needed in this population,” the team concluded.