Characterization of Eosinophilic Esophagitis From the European Pediatric Eosinophilic Esophagitis Registry (pEEr) of ESPGHAN.

OBJECTIVES: Few pediatric data on phenotypic aspects of eosinophilic esophagitis (EoE) are available. The pEEr registry was developed to prospectively characterize children with EoE from Europe and Israel. METHODS: pEEr is an ongoing prospective registry enrolling children with esophageal eosinophilia (≥15 eos/HPF). Anonymized data were collected from 19 pediatric centers. Data regarding demographics, clinical manifestations, endoscopy, histology, and therapies were collected. RESULTS: A total of 582 subjects (61% male) were analyzed. The median age at diagnosis was 10.5 years [interquartile range (IQR): 5.7-17.7], whereas the age at symptom onset was 9.2 years (IQR: 4.3-16.4), resulting in a median diagnostic delay of 1.2 years (IQR: 0.7-2.3). The diagnostic delay was longer below age <6 years. Shorter diagnostic delays were associated with the presence of food allergy or a family history for EoE. Symptoms varied by age with dysphagia and food impaction more common in adolescents, while vomiting and failure to thrive more common in younger children ( P < 0.001). Among endoscopic findings, esophageal rings were more common in adolescents, whereas exudates were more frequent in younger children( P < 0.001). Patients who responded to proton pump inhibitors (PPIs) were more likely to be older, males, and less often presented severe endoscopic findings. Patients unresponsive to PPIs received topical steroids (40%), elimination diet (41%), or a combined therapy (19%). CONCLUSIONS: EoE findings vary according to age in pediatric EoE. Young children are commonly characterized by non-specific symptoms, atopic dermatitis, food allergy, and inflammatory endoscopic lesions. Adolescents usually have dysphagia or food impaction, fibrostenotic lesions, and a better PPI response.

Eosinophils in the gastrointestinal tract: how much is normal?

The normal density of eosinophils in the digestive mucosa of children has been rarely addressed despite being important to provide baseline counts for the diagnosis of eosinophilic gastrointestinal disorders (EGID). Histopathological criteria for EGID remain undefined and there has been little consistency of results in different populations. We aimed to establish the eosinophil density of the normal digestive mucosa in a paediatric population submitted to endoscopic procedures with normal histological features. Biopsies from endoscopies of 33 patients were evaluated. Quantification of eosinophils was performed manually. Review of the pathology reports confirmed absence of abnormality in the biopsy specimens. Counts were expressed in eosinophils per high power field and per mm2. Oesophagus (n = 33): eosinophils were uniformly absent in all biopsies. Stomach: counting was performed, separately, in the superficial and deep lamina propria of the fundus (n = 13), corpus (n = 13) and antrum (n = 16). Mean eosinophilic density was higher in the deep lamina propria. Small intestine: eosinophil counts revealed 18.1 ± 17.0, 14.4 ± 12.0, and 51.5 ± 35.3 in the lamina propria of the bulb (n = 13), D2 (n = 13), and ileum (n = 16), respectively. Large intestine: the highest peak count was observed in the caecum (125 mm2; n = 16) with a mean of 51.8 ± 33.5. The eosinophil counts were lower in the ascending (n = 16; 40.9 ± 27.4), transverse (n = 14; 34.3 ± 21.9), descending (n = 15; 40.0 ± 26.6), and sigmoid (n = 17; 25.8 ± 17.8) colon and in the rectum (n = 17; 13.9 ± 10.1). These data provide a baseline count and distribution of eosinophils in the gastrointestinal tract of paediatric patients with normal mucosa, thus expanding the scarce published data.