News at CHOP: Summer 2021

Measuring pandemic stress on children and families

The Center for Pediatric Traumatic Stress (CPTS) at CHOP has published a paper in the Journal of Pediatric Psychology that reports on the COVID-19 Exposure and Family Impact Scales (CEFIS) it had created in the first few weeks of the pandemic. Initial analysis indicates the CEFIS to be a psychometrically sound, clinically useful instrument that measures families’ exposure to potentially traumatic aspects of the COVID-19 pandemic.

The CPTS, a joint effort of CHOP and Nemours A.I. duPont Hospital for Children, seeks to better understand the pandemic’s impact on daily life for pediatric patients and their families. Understanding the extent to which families are exposed to COVID-19-related events and how they appraise their impact could help guide efforts to assure delivery of high-quality pediatric healthcare.

CEFIS is available for use, for free, in both English and Spanish. Registration is required and registrants agree to share de-identified CEFIS data monthly so CPTS can continue its work fine-tuning the measure and establishing its psychometric properties. For more information, go here.

CHOP finds blood assay to identify Eosinophilic Esophagitis-triggering foods

By finding how a typical blood assay can pinpoint foods that cause eosinophilic esophagitis (EoE) early in the course of disease, children can eliminate those foods and suffer less from symptoms.

CHOP researchers used EoE milk allergy as a proxy and looked for immune signatures that would identify EoE milk allergy when compared to controls. They found that patients with EoE milk allergy had elevated milk-dependent memory T helper cells, and that these cells produced more of a cytokine known as interleukin-4 that contributes to allergy. The elevation of these biomarkers occurred whether the patient had stopped consuming milk or was still actively consuming it.

This finding, if applied to a regularly used assay, could help determine not only what foods an individual should avoid, but also guide timing on reintroducing causal foods, since emerging evidence shows that some children can outgrow EoE after long periods of food avoidance. The study was published in Allergy.

CHOP researchers discover new disease that prevents formation of antibodies

Neil D. Romberg, MD, and researchers in his lab at CHOP discovered a new genetic disease while trying to figure out why a young patient was continually getting repeat infections. The discovery is the first description of a human congenital PU.1 disorder, which prevents B cell development. The researchers named this condition PU.1 Mutated Agammaglobulinemia (PU.MA), and the finding was published in the Journal of Experimental Medicine.

The patient underwent whole exome sequencing, which revealed a mutation in the SPI1 gene, which encodes the PU.1 protein, which is critical for B cell development in the bone marrow.

A total of 6 PU.MA patients, who ranged in age from 15 months to 37 years, have been identified.

Three CHOP Physicians honored at PAS

Lisa R. Young, MD, Chief of the Division of Pulmonary and Sleep Medicine at CHOP and an international leader and expert on rare pediatric lung diseases, received the 2021 Norman J. Siegel Outstanding Science Award for her considerable contributions to pediatric science. The award was presented during the Pediatric Academic Societies 2021 Virtual Meeting.

Susan Furth, MD, PhD, Chief of CHOP’s Division of Nephrology, received the Society for Pediatric Research annual Maureen Andrew Mentor Award, which recognizes pediatricians with outstanding records of mentor leadership. The award was presented during PAS.

Joseph W. St. Geme III, MD, Chair of the Department of Pediatrics and Physician-in-Chief at CHOP, received the Joseph W. St. Geme Jr. Leadership Award, an honor bestowed by the Federation of Pediatric Organizations and named after his father, a renowned and celebrated pediatrician. St. Geme was named the 2020 recipient, but the recognition was postponed until this year’s PAS Meeting.

(L to R) Lisa R. Young, MD, Susan Furth, MD, PhD, and Joseph W. St. Geme III, MD