Inflamed Esophagus Cause of Oral Aversion for 3 Children With CF

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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Difficulties with eating and proper nutrition for cystic fibrosis (CF) patients may be due to an inflammation of the esophagus known as eosinophilic esophagitis (EoE), according to a report on three young children in the U.S.

Shared traits, particularly gastrointestinal issues like gastroesophageal reflux disease (GERD) and abdominal pain that leave children food averse, can make it “challenging for clinicians to diagnose the coexistent EoE in patients with CF,” its researchers wrote, noting their report is thought to be the “second case series” connecting the two disorders.

The children were treated with oral corticosteroids. Their symptoms eased and they were doing well at a mean follow-up of almost four years (46 months).

The report, “Eosinophilic esophagitis in cystic fibrosis: A case series with long-term follow-up,” was published as a letter in the journal Pediatric Pulmonology.

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Symptoms of CF often begin in infancy and can affect various organs, including those of the digestive system. This happens because CFTR — the protein that does not work properly or is missing in patients — is present in the cells lining organs of the digestive system.

Common digestive system symptoms of CF in children include GERD or acid reflux, when the acid contents of the stomach flow back into the esophagus, constipation, and pancreatic insufficiency, which occurs when the pancreas does not release enough of the enzymes needed to break up food in the intestines. As such, children with CF can show poor weight gain and delayed growth.

These symptoms can also be common to other gastrointestinal disorders. EoE, for instance, is caused by the buildup of immune cells called eosinophils in the esophagus (the tube connecting the stomach and mouth), and it can cause damage, inflammation, pain, and trouble swallowing.

Though EoE is commonly marked by difficulty swallowing, in children gastrointestinal symptoms such as heartburn, regurgitation, abdominal pain, failure-to-thrive, and oral aversion — a strong dislike or fear of anything touching the mouth — are usually evident. These can be due to various disorders.

“This case series focuses on three toddlers with CF who presented with oral aversion and challenging mealtime behaviors,” the researchers, all based in Cleveland, Ohio, wrote.

The children, all boys, were given a CF diagnosis through newborn screening. One had two copies — one in each CTFR gene copy — of the common disease-causing F508del mutation, and the other two boys had an F508del mutation in one gene copy and a less common mutation in the other. CTFR is the gene that provides instructions for making the protein of the same name.

Each had pancreatic insufficiency and GERD, which was treated with acid suppression medication. One boy had started experiencing EoE at 16 months of age, and had a history of poor weight gain and oral aversion to both formula milk and solid foods. The other two began showing EoE symptoms at 14 months and 4 years old, and had relatively better weight gain. But both received their nutrition mostly through oral supplements or cow’s milk, with a restricted intake of solid foods. 

EoE was diagnosed after an esophagogastroduodenoscopy, a procedure that uses a thin tube with a small camera to allow doctors to view the esophagus, the stomach, and the duodenum (a part of the small intestine that connects it to the stomach), was given the children.

A biopsy also confirmed the presence of eosinophils in the wall of the esophagus, and revealed swelling and an increased number of the round cells that form the deeper layer of the digestive tube.

“The esophagus may appear near normal in active disease so it is important to obtain biopsies … when suspecting EoE,” the researchers noted.

All three children improved after starting on “topical (swallow) corticosteroids,” resulting in “successful management of esophageal inflammation,” the team wrote.

“As poor growth and suboptimal weight gain are recognized risk factors for worse pulmonary disease and shorter lifespan in CF, early identification of treatable pathologies such as EoE will help optimize their nutritional status,” the researchers concluded.