We sought to determine the prevalence of adrenal suppression (AS) in children with eosinophilic esophagitis treated with oral viscous budesonide (OVB). This was a retrospective review of a quality assurance initiative, whereby all children in our center treated with OVB for ≥3 months were referred over an 18-month time frame for endocrine assessment including 1 μg adrenocorticotropic hormone stimulation test. Fourteen of 19 children complied with the referral; of these 14 children, 6 (43%) had suboptimal stimulated cortisol (range 343-497 nmol/L, mean [±SD] 424.7 nmol/L [±52.4], normal ≥500 nmol/L). There was no significant association to treatment duration, dose, or concomitant use of inhaled/nasal corticosteroids. This study suggests that children treated with OVB may be at risk for AS.

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We demonstrate that active esophagitis in pediatric EoE patients is associated with elevated levels of IgG4-positive plasma cells, which was more significant in EoE patients with a documented food allergy. Our study also adds to the growing literature that EoE may involve more than just an exaggerated TH2 immune response.

Renal impairment is frequently compromised in patients with end-stage liver disease and is associated with increased long-term mortality post-LT. In contrast to CNI, basiliximab is an immunosuppressive agent with minimal nephrotoxic potential. This study reviews the experience of a single pediatric liver transplant center's renal-sparing approach with the use of basiliximab and MMF to compensate for delayed entry of CNI in children with renal impairment at the time of organ availability. There were no differences in renal function between pediatric patients with and without pre-LT renal impairment within the first year (cGFR: 135 mL/min/1.73 m2 vs. 144 mL/min/1.73 m2 ; p = 0.56) or at 5-8 yr following LT, (129 mL/min/1.73 m2 vs. 130 mL/min/1.73 m2 ; p = 0.97). In addition, there was no difference in ACR rates (50% vs. 43%, p = 0.62) between patients in the basiliximab group and those patients receiving standard CNI and steroid strategies. The utilization of a renal-sparing approach with basiliximab alongside delayed entry and lower early target trough levels of CNI in children with renal impairment at the time of LT is safe and maintains excellent long-term kidney function.

Objectives Cow’s milk is a commonly implicated trigger in eosinophilic esophagitis (EoE). Exclusive cow’s milk avoidance has been reported previously, but the degree of elimination required for remission is unclear. Strict food avoidance may confer a risk of developing immunoglobulin E (IgE)-mediated allergy. The goal of this study was to evaluate the effectiveness of cow’s milk elimination (CME) in children with EoE and compare responses of strict and liberalized CME diets. Methods Children (≤16 years) diagnosed with EoE who were treated with exclusive CME diets were evaluated clinically and histologically. Strict diets eliminated all milk products, including ‘may-contain’ and baked milk goods. Liberalized diets eliminated obvious sources including milk, cheese, yogurt, cream-based products but permitted foods with traces of milk and baked goods. Results Cow’s milk elimination induced histological remission of <15 eosinophils per high-powered field in 18 of 31 children (58%) and complete remission in 23%. Overall, 77% had decreased eosinophils with this single intervention. Symptoms were improved in 90% of patients, regardless of histologic response. A liberalized (n=7) CME diet was associated with a nonsignificantly lower response compared with strict (n=24) elimination (29% versus 67%, P =0.099). Eight responders to strict elimination were transitioned to a liberalized diet; 63% maintained remission. Conclusion Cow’s milk elimination induced clinicopathological remission in a majority of patients with EoE, supporting its use as a first-line intervention. Liberalized CME allows dietary freedom and may prevent subsequent development of anaphylactic milk allergy but may be inferior to strict CME for improving EoE.

Recently, genetic associations have been described in intestinal transplants. Namely, Crohn's disease susceptibility gene NOD2 polymorphisms have been reported to be more prevalent in patients with graft failure following intestinal transplantation (IT). Therefore, we sought to determine if polymorphisms in the NOD2 signaling cascade, including NOD2, CARD9, RAC1 and ATG16L1 are associated with intestinal failure (IF) or its complications. We carried out a cross-sectional study of 59 children with IF and 500 healthy Caucasian controls. Using the Taqman platform we determined the prevalence of NOD2 as well as ATG16L1, RAC1 and CARD9 SNPs. NOD2 pathway polymorphisms were evaluated in relation to outcomes of episodes of sepsis, ICU admissions, hyperbilirubinemia and need for IT. We found that the minor allele of a CARD9 SNP was associated with protection from developing IF when compared to healthy controls and was also associated with decreased odds of sustained conjugated hyperbilirubinemia. Therefore, IF patients with CARD9 polymorphism are less likely to develop progressive liver disease and suggests that host innate immunity may play a role in IF associated liver disease.

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