Analysis of eosinophilic esophagitis in children with repaired congenital esophageal atresia.

BACKGROUND: A high prevalence of eosinophilic esophagitis (EoE) has been preliminarily reported in patients after repair of esophageal atresia (EA), but the basis of this association is unknown. OBJECTIVES: We aimed to (1) characterize the EoE transcriptome in patients with EA, (2) compare the EoE transcriptome in patients with EoE and EA with that in patients with EoE alone, and (3) identify transcripts that could predispose patients with EA to EoE. METHODS: This single-center, population-based, retrospective study identified 4 EoE study cohorts: healthy control subjects, patients with EA and EoE (EA+EoE+), patients with EA without EoE (EA+EoE-), and patients with EoE without EA (EA-EoE+). Molecular signatures were assessed by using the EoE diagnostic panel, a 94-gene expression quantitative PCR array. RESULTS: In a cohort of 110 pediatric patients with surgically repaired EA, 20 (18%) patients were given a diagnosis of EoE, representing a 364-fold enrichment of EoE in patients with EA compared with the general pediatric population. EoE diagnostic panel analyses revealed a major overlap between the EA+EoE+ and EA-EoE+ cohorts. A proportion (approximately 25%) of EoE signature genes were dysregulated in patients with EA+EoE- compared with healthy control subjects, including those involved in epithelial barrier function and type 2-associated inflammatory responses. Patients with EA+EoE+ exhibit a more severe EoE clinical phenotype than those with EA-EoE+ in terms of dysphagia and dilation need. CONCLUSIONS: Patients with EA have increased risk of EoE. Patients with EoE with EA have a similar molecular profile compared with that of patients with EoE without EA. Dysregulated baseline epithelial barrier and type 2-associated genes in EA monomorbidity might explain the higher EoE prevalence in patients with EA.

Latex allergy in children with oesophageal atresia.

BACKGROUND: Latex allergy is frequently found in children and patients with spina bifida and urogenital abnormalities and have been considered at risk for latex sensitization. The aim of the study was to evaluate the incidence of latex sensitization in patients with oesophageal atresia and undergoing three or more surgical procedures and to identify possible risk factors in the process of latex sensitization. METHODS: A total of 20 patients were analysed: 19 boys and one girl. The oesophageal atresias were as follows: type I in three children, type II in two and type III in 15 children. Surgical and anaesthetic procedures, intensive care management, age, type of oesophageal atresia, associated congenital malformations, Waterston and Montreal prognostic classifications were considered as risk factors that may be implicated in the process of sensitization. RESULTS: Five patients (25%) were considered sensitized to latex (group 1) and 15 (75%) nonsensitized (group 2). Among the five sensitized patients, three reported clinical reactions to latex, while the other two presented only specific IgE sensitization. The number of operations, the total hours of surgery, the number of drainages, the total days of drainage, the total days of central venous catheter were significantly greater in group 1 than in group 2. Both of the highest risk oesophageal atresia classes (Waterston C and Montreal II) were related to latex allergy. CONCLUSIONS: Oesophageal atresia, especially in cases of prolonged management, must be considered as a risk for the development of latex allergy.

The adaptive immune response to major surgery in the neonate.

The effect of major surgery on components of the adaptive immune response in babies has not previously been reported. In a prospective study, eight neonates undergoing uncomplicated surgery for repair of esophageal atresia were investigated. They were compared with ten age-matched normal babies not undergoing surgery. The parameters of the immune response investigated were: total leukocytes (WBC), lymphocytes and their subsets (T-helper, T-suppressor, natural killer [NK], B-lymphocytes), monocytes, immunoglobulins (Ig) G and M, the cytokines tumor necrosis factor-alpha (TNF-alpha) and interleukin 1-beta (Il-1beta), and C-reactive protein (CRP), an acute-phase protein. When compared to the normal controls, the operated group showed a fall in all types of WBC following surgery, but only the falls in B-lymphocytes and NK cells were significant on postoperative day 3 (P < 0.05). The suppression in WBC was temporary, and by day 7 the operated group had significantly higher numbers of total WBC and T-helper cells than the controls (P < 0.05), who were undergoing their physiological postpartum fall in WBC. Within the operated group, there was a significant fall in the numbers of total lymphocytes, T-suppressors, and B-lymphocytes compared to preoperative levels (P < 0.01). The ratio of T-helper/T-suppressor cells increased significantly following surgery. There was a vigorous immune response in terms of the humoral factors: CRP, TNF-alpha, and Il-1beta all rose significantly postoperatively (P < 0.02).