Long-term Durability Between Parent and Child Patient Reported Outcomes in Eosinophilic Esophagitis.

BACKGROUND: Because young children cannot self-report symptoms, there is a need for parent surrogate reports. While early work suggested parent child alignment for eosinophil esophagitis (EoE) patient reported outcomes (PROs), the longitudinal alignment is unclear. OBJECTIVE: To assess the agreement and longitudinal stability of PROs between children with EoE and their parents. METHODS: 292 parent-child respondents completed 723 completed questionnaires over 5 years in an observational trial in the Consortium of Eosinophilic Gastrointestinal Disease Researchers. The change in and agreement between parent and child Pediatric Eosinophilic Esophagitis Symptom Score version 2 (PEESSv2.0) and Pediatric Quality of Life Eosinophilic Esophagitis Module (PedsQL-EoE) PROs over time were assessed using Pearson correlation and Bland-Altman analyses. Clinical factors influencing PROs and their agreement were evaluated using linear mixed models. RESULTS: The cohort had a median disease duration equalling 3.7 years and was predominantly male (73.6%) and white (85.3%). Child and parent PEESSv2.0 response groups were identified and were stable over time. There was strong correlation between child and parent report (PEESSv2.0 0.83, PedsQL-EoE 0.74) with minimal pairwise differences for symptoms. Longitudinally, parent-reported PedsQL-EoE scores were stable (p ≥ 0.32), whereas child-reported PedsQL-EoE scores improved (p = 0.026). A larger difference in parent and child PedsQL-EoE reports was associated with younger age (p < 0.001) and differences were driven by psychosocial PRO domains. CONCLUSION: There is strong longitudinal alignment between child and parent report using EoE PROs. These data provide evidence that parent report is a stable proxy for objective EoE symptoms in their children.

International Consensus Recommendations for Eosinophilic Gastrointestinal Disease Nomenclature.

BACKGROUND & AIMS: Substantial heterogeneity in terminology used for eosinophilic gastrointestinal diseases (EGIDs), particularly the catchall term "eosinophilic gastroenteritis," limits clinical and research advances. We aimed to achieve an international consensus for standardized EGID nomenclature. METHODS: This consensus process utilized Delphi methodology. An initial naming framework was proposed and refined in iterative fashion, then assessed in a first round of Delphi voting. Results were discussed in 2 consensus meetings, and the framework was updated and reassessed in a second Delphi vote, with a 70% threshold set for agreement. RESULTS: Of 91 experts participating, 85 (93%) completed the first and 82 (90%) completed the second Delphi surveys. Consensus was reached on all but 2 statements. "EGID" was the preferred umbrella term for disorders of gastrointestinal (GI) tract eosinophilic inflammation in the absence of secondary causes (100% agreement). Involved GI tract segments will be named specifically and use an "Eo" abbreviation convention: eosinophilic gastritis (now abbreviated EoG), eosinophilic enteritis (EoN), and eosinophilic colitis (EoC). The term "eosinophilic gastroenteritis" is no longer preferred as the overall name (96% agreement). When >2 GI tract areas are involved, the name should reflect all of the involved areas. CONCLUSIONS: This international process resulted in consensus for updated EGID nomenclature for both clinical and research use. EGID will be the umbrella term, rather than "eosinophilic gastroenteritis," and specific naming conventions by location of GI tract involvement are recommended. As more data are developed, this framework can be updated to reflect best practices and the underlying science.

Esophageal Food Impaction and Eosinophilic Esophagitis: A Retrospective Study, Systematic Review, and Meta-Analysis.

BACKGROUND: Esophageal food impaction (EFI) can be the initial presentation of eosinophilic esophagitis (EoE). EoE is characterized by persistent esophageal eosinophilia (EE). Both EFI and EE are related to a variety of conditions. To date, the relationship between EFI, EE, and EoE remains unclear. AIMS: To review our institutional experience with EFIs and combine our knowledge with the existing literature to conduct a systematic review and meta-analysis for delineating the relationship between EFI, EE, and EoE. METHODS: We reviewed medical records of 72 children with EFI presenting to our emergency center between 2007 and 2013. PubMed, EMBASE, and Scopus databases were screened from inception until July 2014 to identify studies linking EFI and EoE. Included studies were methodically assessed for the quality and strength of association between EFI and EoE. RESULTS: Our institutional experience highlighted the possibility of proton-pump inhibitor therapy-responsive EE (PPI-REE) as an underrecognized risk factor for EFI. A systematic review of 14 studies, including ours, revealed that most studies did not eliminate other causes of EFI or EE. The meta-analysis revealed that esophageal biopsies were obtained from 54% (40-68) of individuals presenting with EFI, and the overall EoE-attributable EFI among those who were biopsied was 54% (43-65). Substantial heterogeneity was noted among the studies. DISCUSSION: PPI-REE is an underestimated risk factor for EFI. The quality of existing evidence linking EFI and EoE is limited by several important factors. Future studies with robust design are warranted to delineate the relationship between EFI, EE, and EoE.

Reproducibility of high-resolution optical coherence tomography in multiple sclerosis.

Optical coherence tomography (OCT) is a non-invasive method to quantify neurodegeneration as an outcome in multiple sclerosis clinical trials; however, no data exist on Cirrus spectral domain optical coherence tomography (SD-OCT) reproducibility in patients with multiple sclerosis. The objective of this study was to determine the protocol for achieving optimal inter-visit, inter-rater, and intra-rater reproducibility for studies performed on healthy controls and multiple sclerosis patients utilizing novel high-definition SD-OCT. This is a prospective study of inter-visit, inter-rater, and intra-rater reproducibility in multiple sclerosis patients (n = 58) and healthy controls (n = 32) on Cirrus-HD SD-OCT. Excellent reproducibility of average and quadrantic retinal nerve fiber layer (RNFL) thickness values, average macular thickness (AMT), and total macular volume (TMV) [measured by intraclass correlation coefficient (ICC)] was found for inter-visit (healthy controls: mean RNFL = 0.97, quadrant range = 0.92-0.97, AMT = 0.97, TMV = 0.92), inter-rater (MS: mean RNFL = 0.97, quadrant = 0.94-0.98, AMT = 0.99, TMV = 0.96; healthy controls: mean RNFL = 0.97, quadrant = 0.94-0.97, AMT = 0.98, TMV = 0.99), and intra-rater (MS patients: mean RNFL = 0.99, quadrant = 0.83-0.99, AMT = 0.97, TMV = 0.98) reproducibility. The reproducibility of retinal measures derived by Cirrus HD-OCT, especially quadrantic values, is excellent. Specific procedures for OCT acquisition and analysis of retinal imaging metrics using SD-OCT technology may improve the application of this novel technology in multiple sclerosis.

Impact of Homeland Security Alert level on calls to a law enforcement peer support hotline.

The Homeland Security Advisory System (HSAS) was established by the Department of Homeland Security to communicate the risk of a terrorist event. In order to explore the potential psychological impacts of HSAS we analyzed the effects of terror alerts on the law enforcement community. We used data from the New Jersey Cop 2 Cop crisis intervention hotline. Incidence Rate Ratios--interpreted as average relative increases in the daily number of calls to the Cop 2 Cop hotline during an increased alert period--were computed from Poisson models. The hotline received a total of 4,145 initial calls during the study period. The mean daily number of calls was higher during alert level elevation compared to prior 7 days (7.68 vs. 8.00). In the Poisson regression analysis, the Incidence Rate Ratios of number of calls received during elevated alert levels compared to the reference period of seven days preceding each change in alert were close to 1, with confidence intervals crossing 1 (i.e. not statistically significant) for all lag periods evaluated. This investigation, in the context of New Jersey law enforcement personnel, does not support the concern that elevating the alert status places undue stress upon alert recipients.

Beyond the Pediatric end-stage liver disease system: solutions for infants with biliary atresia requiring liver transplant.

Biliary atresia (BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the Kasai portoenterostomy; however most patients still require a liver transplant, with up to one half of BA children needing a transplant by age two. In the current pediatric end-stage liver disease system, children with BA face the risk of not receiving a liver in a safe and timely manner. In this review, we discuss a number of possible solutions to help these children. We focus on two general approaches: (1) preventing/delaying need for transplantation, by optimizing the success of the Kasai operation; and (2) expediting transplantation when needed, by performing techniques other than the standard deceased-donor, whole, ABO-matched organ transplant.

A meta-analysis of studies comparing the respiratory route with the subcutaneous route of measles vaccine administration.

The respiratory tract has been suggested as an optimal alternative site to target measles vaccine delivery. We performed a meta-analysis to evaluate the efficacy of measles vaccine administered through the respiratory route compared to the subcutaneous route. We analyzed 20 reported results from 16 eligible studies. Seroresponse was 4% higher amongst vaccinees in the respiratory group compared to the subcutaneous group (M-H pooled RR = 1.04; 95% CI = 0.98-1.10). For vaccinees over 9 months of age, seroresponse was 15% higher in the respiratory group (M-H pooled RR = 1.15; 95% CI = 1.08 to 1.17). When Edmonston Zagreb (EZ) strain was used, the vaccine was found to be neither more nor less efficacious when administered through respiratory route (M-H pooled RR=1.00; 95% CI = 0.94 to 1.08). Seroresponse in the vaccinees receiving aerosol measles vaccine was 10% higher (M-H pooled RR = 1.10, 95% CI = 1.04-1.17) compared to those who received measles vaccine through the subcutaneous route. Seroresponse due to aerosolized EZ vaccine was 9% higher than subcutaneous EZ vaccine (M-H pooled RR = 1.09; 95% CI = 1.02-1.16). The seroresponse among seropositive vaccinees was 60% higher (M-H RR = 1.60; 95% CI = 1.40 to 1.80). The results suggest that the respiratory route of delivery of measles vaccine is at least as efficacious as measles vaccine administered through the subcutaneous route. However, more research is required on standardization of dosage, administration equipments, efficacy, and safety of respiratory (aerosol) route of administration of measles vaccine.