Impact of Atopic Status on Clinical Presentation and Treatment Response in Pediatric Patients With Eosinophilic Esophagitis.

BACKGROUND: Nearly 80% of patients with eosinophilic esophagitis (EoE) have coexisting atopic disease, yet a subset do not. It is unclear if this lack of atopy impacts presentation or response to therapy. OBJECTIVES: To characterize the presentation and response to therapy in atopic versus nonatopic pediatric patients with EoE. METHODS: A case-control study of patients with EoE aged 6 months to 18 years (between 2018 and 2021) was performed. Patients were eligible if they had allergy testing, assessment of atopic history, and at least 1 endoscopy after initiation of treatment. Patients were considered nonatopic if they had negative allergy testing and no history of significant atopy. Response to therapy was classified as complete (peak eosinophils [eos] <15/high power field [hpf]), partial (≥15 eos/hpf but at least a 50% reduction in peak eos), or nonresponse. RESULTS: A total of 168 participants were enrolled. The majority were White (n = 141, 84%), male (n = 124, 74%), and non-Hispanic (n = 158, 95%). The mean age at diagnosis was 9.4 years (standard deviation: ±4.8 years). A total of 123 participants (73.2%) were atopic, and 45 (26.8%) were nonatopic. There was no significant difference between atopic and nonatopic for most demographics or presenting symptoms. Nonatopic participants were younger than atopic participants (8.14 vs 9.8 years, P = .046). Swallowed topical corticosteroids (STC) and food elimination diets (FED) were used at a similar rate. There were no differences in treatment response between atopic/nonatopic participants in regard to STC, FED, or STC+FED. CONCLUSIONS: Atopic status does not significantly impact presentation or response to treatment in pediatric EoE, but a lack of atopy may be a risk for earlier onset of disease.

Rectal suction biopsy to exclude the diagnosis of Hirschsprung disease.

OBJECTIVES: Hirschsprung disease (HD) is defined as the absence of ganglion cells in the Meissner and Auerbach plexuses. Diagnosis depends on demonstrating the absence of ganglion cells in rectal biopsy specimens. Rectal suction biopsy is widely employed as the method of choice in obtaining such specimens. Classically, the diagnosis was made until the 1990s by using the Multipurpose Suction Biopsy Kit, or Rubin Tube. This device was replaced by the Model SBT-100 Suction Biopsy Kit as the exclusive device used to procure rectal tissue. Because the suction devices are known to occasionally yield tissue that is insufficient, the present study was undertaken to determine the efficacy of using this technique to make or exclude the diagnosis of HD. METHODS: The last 50 biopsy sessions using the Multipurpose Suction Biopsy Kit and the first 46 sessions using the Model SBT-100 were included for review. RESULTS: Both groups had similar yields (24%) of biopsy sessions with insufficient tissue to allow meaningful interpretation. The predictive value of rectal suction biopsy in excluding HD at the first biopsy session was 65%. CONCLUSIONS: We conclude that HD can be excluded with a single rectal suction biopsy 65% of the time. A second biopsy session will exclude the diagnosis in an additional 11% of patients. Both devices yield biopsies of comparable quality and are equally useful in excluding the diagnoses of HD.

Feasibility of using MR enterography for the assessment of terminal ileitis and inflammatory activity in children with Crohn disease.

BACKGROUND AND OBJECTIVE: Radiation exposure increases cancer risk in children with Crohn disease (CD). Magnetic resonance enterography (MRE) can image the gastrointestinal tract without exposure to radiation. The aim of the present study was to determine whether our MRE protocol could diagnose terminal ileitis and the degree of inflammatory activity in children with CD. METHODS: Retrospective review of patients 18 years of age or younger who underwent MRE for known or suspected CD from June 15, 2007 to April 1, 2010. MRE was performed with Volumen and water as oral contrast and gadolinium-based intravenous contrast. No antiperistaltic agent was used. Each MRE was compared with ileal biopsies obtained within 90 days. Severity of inflammation on MRE was scored and compared with the Pediatric Crohn Disease Activity Index (PCDAI). RESULTS: Seventy-two patients underwent 80 MREs during the study period. Forty-two of the 72 patients (58.3%) underwent colonoscopy within 90 days of MRE, and the terminal ileum was intubated in 33. Compared with histology, MRE had a sensitivity of 71.4% and a specificity of 100% for terminal ileitis. The positive and negative predictive values were 100% and 70%, respectively. PCDAI was calculated in 39 of the 72 patients (54.2%) and had a statistically significant positive correlation with MRE score of 0.37 (P = 0.020426). CONCLUSIONS: In children with known or suspected CD, our MRE protocol has a high specificity and positive predictive value for terminal ileitis. Severity of inflammation on MRE had a statistically significant positive correlation with PCDAI.

Factors that determine risk for surgery in pediatric patients with Crohn's disease.

BACKGROUND & AIMS: We examined the incidence of Crohn's disease (CD)-related surgery in a multi-center, inception cohort of pediatric patients with CD. We also examined the effect of starting immunomodulator therapy within 30 days of diagnosis. METHODS: Data from 854 children with CD from the Pediatric Inflammatory Bowel Disease Collaborative Research Group who were diagnosed with CD between 2002 and 2008 were analyzed. RESULTS: Overall, 76 (9%) underwent a first CD-related surgery, 57 (7%) underwent a first bowel surgery (bowel resection, ostomy, strictureplasty, or appendectomy), and 19 (2%) underwent a first non-bowel surgery (abscess drainage or fistulotomy). The cumulative risks for bowel surgery, non-bowel surgery, and all CD-related surgeries were 3.4%, 1.4%, and 4.8%, respectively, at 1 year after diagnosis and 13.8%, 4.5%, and 17.7%, respectively, at 5 years after diagnosis. Older age at diagnosis, greater disease severity, and stricturing or penetrating disease increased the risk of bowel surgery. Disease between the transverse colon and rectum decreased the risk. Initiation of immunomodulator therapy within 30 days of diagnosis, sex, race, and family history of inflammatory bowel disease did not influence the risk of bowel surgery. CONCLUSIONS: In an analysis of pediatric patients with CD, the 5-year cumulative risk of bowel surgery was lower than that reported in recent studies of adult and pediatric patients but similar to that of a recent retrospective pediatric study. Initiation of immunomodulator therapy at diagnosis did not alter the risk of surgery within 5 years of diagnosis.