Publications by authors named "C S Zerbe"

Background: The transition to adult health care is challenging for adolescents and young adults (AYA) with Chronic Granulomatous Disease (CGD). This pilot study aimed to facilitate the learning of AYA with CGD about their health care and to aid in the development of life skills to enhance self-care.

Methods: AYA and caregivers (for participants <18 years of age) completed an adapted Transition Readiness Assessment.

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Chronic granulomatous disease (CGD) is a disorder of immunity characterized by phagocyte dysfunction. Mold infections in patients with CGD are often severe and disseminated. We present patient characteristics, microbiological data, and outcomes for 26 patients with CGD who received hematopoietic cell transplantation (HCT) or gene therapy-modified cells (GT) between 2008 and 2019, with proven fungal infection either before or during their transplant.

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Article Synopsis
  • - Patients with GATA2 haploinsufficiency face higher risks for HPV-related anogenital cancers, with the study showing that HPV appeared at a median age of 18.9 in females and 25.6 in males, often leading to significant health issues.
  • - The research conducted a thorough review of 68 patients over 17 years, revealing that 76% had HPV-related squamous intraepithelial lesions (SIL), with females showing a higher prevalence and severity of high-grade lesions compared to males.
  • - Among patients receiving hematopoietic stem cell transplants for GATA2-linked diseases, some still experienced persistent HPV issues post-treatment; however, those who received vaccinations afterward showed minimal evidence
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Neutrophils collectively migrate to sites of injury and infection. How these swarms are coordinated to ensure the proper level of recruitment is unknown. Using an ex vivo model of infection, we show that human neutrophil swarming is organized by multiple pulsatile chemoattractant waves.

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Immunological health has been challenging to characterize but could be defined as the absence of immune pathology. While shared features of some immune diseases and the concept of immunologic resilience based on age-independent adaptation to antigenic stimulation have been developed, general metrics of immune health and its utility for assessing clinically healthy individuals remain ill defined. Here we integrated transcriptomics, serum protein, peripheral immune cell frequency and clinical data from 228 patients with 22 monogenic conditions impacting key immunological pathways together with 42 age- and sex-matched healthy controls.

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