A quality improvement initiative to improve primary care referral rates for penicillin allergy delabeling.

Background: Of the US population, 10% reports a penicillin allergy but more than 90% can ultimately tolerate penicillin. Confirmation of these allergies in the pediatric population may improve future health outcomes and decrease costs. Referring patients for confirmatory testing is the first step in clarifying penicillin allergies.

Economic burden of congenital athymia in the United States for patients receiving supportive care during the first 3 years of life.

Aims: Congenital athymia is an ultra-rare pediatric condition characterized by the lack of thymus in utero and the naïve T cells critical for infection defense and immune regulation. Patients with congenital athymia receive supportive care to minimize and treat infections, autoimmune phenomena, and autologous graft-versus-host disease (aGVHD) manifestations, but historically, die within the first 3 years of life with supportive care only. We estimated the healthcare resource utilization and economic burden of supportive care over patients' first 3 years of life in the United States.

Defining the Clinical, Emotional, Social, and Financial Burden of Congenital Athymia.

Introduction: Characterize the burden of illness in pediatric patients with congen̄ital athymia who were receiving supportive care.

Methods: This cross-sectional study of adult caregivers of patients with congenital athymia used both a quantitative survey and qualitative interviews. Caregivers of patients currently receiving supportive care responded to questions about the past 12 months and completed the parent proxy version of the Pediatric Quality of Life Inventory Generic instrument (PedsQL) for patients aged 2-4 years.

Congenital Athymia: Genetic Etiologies, Clinical Manifestations, Diagnosis, and Treatment.

Congenital athymia is an ultra-rare disease characterized by the absence of a functioning thymus. It is associated with several genetic and syndromic disorders including FOXN1 deficiency, 22q11.2 deletion, CHARGE Syndrome (Coloboma, Heart defects, Atresia of the nasal choanae, Retardation of growth and development, Genitourinary anomalies, and Ear anomalies), and Complete DiGeorge Syndrome.

Abnormal SCID Newborn Screening and Spontaneous Recovery Associated with a Novel Haploinsufficiency IKZF1 Mutation.

Purpose: IKAROS, encoded by IKZF1, is a member of the IKAROS family of zinc-finger transcription factors playing critical roles in lymphocyte development, differentiation, and tumor suppression. Several studies demonstrated that IKZF1 mutations affecting DNA binding or homo-/hetero-dimerization are mostly associated with common variable immunodeficiency, combined immunodeficiency, or hematologic manifestations. Herein we report a likely de novo, nonsense IKZF1 mutation (p.

Provider recommendations and maternal practices when providing breast milk to children with immunoglobulin E-mediated food allergy.

Background: There is limited research investigating maternal dietary practices and health care provider recommendations when providing breast milk (BM) to children with immunoglobulin (Ig) E-mediated food allergy.

Objective: To explore health care provider recommendations and maternal practices when providing BM to children with IgE-mediated food allergy and to assess for possible IgE-mediated reactions to BM while the mother consumed the food to which her child has allergy.

Methods: A web-based survey was distributed to breastfeeding (BF) mothers of children with IgE-mediated food allergies.

When Screening for Severe Combined Immunodeficiency (SCID) with T Cell Receptor Excision Circles Is Not SCID: a Case-Based Review.

Newborn screening efforts focusing on the quantification of T cell receptor excision circles (TRECs), as a biomarker for abnormal thymic production of T cells, have allowed for the identification and definitive treatment of severe combined immunodeficiency (SCID) in asymptomatic neonates. With the adoption of TREC quantification in Guthrie cards across the USA and abroad, typical, and atypical SCID constitutes only ~ 10% of cases identified with abnormal TRECs associated with T cell lymphopenia. Several other non-SCID-related conditions may be identified by newborn screening in a term infant.

Pediatric nurse practitioners' attitudes/beliefs and knowledge/perceived competence in caring for transgender and gender-nonconforming youth.

Purpose: There is a growing number of youth and their parents seeking assistance from care providers related to gender dysphoria and transitional care for transgender and gender-nonconforming (TGNC) youth. The purpose of this study was to determine pediatric nurse practitioners' (PNP) attitudes/beliefs and knowledge/competence in caring for TGNC youth.

Design And Methods: This cross-sectional descriptive study surveyed PNPs (N = 93) from eight states using two survey instruments to measure attitudes and beliefs and knowledge and perceived competence related to caring for TGNC youth.

Measles Maternal Antibodies With Low Avidity Do Not Interfere With the Establishment of Robust Quantity and Quality Antibody Responses After the Primary Dose of Measles, Mumps, and Rubella Vaccine Administered at 12-Months of Age.

In this study, we illustrate, for the first time, that preexisting low-avidity neutralizing measles maternal antibodies do not interfere with the development of high concentrations of high-avidity measles antibodies in children immunized at age 12 months. This suggests that the quality of measles maternal antibodies, rather than the quantity, impacts immunogenicity of primary measles immunization.

Evaluation of long-term course in children with eosinophilic esophagitis reveals distinct histologic patterns and clinical characteristics.

Background: Eosinophilic esophagitis (EoE) is a chronic and increasingly prevalent antigen-driven disease. There is a paucity of information on long-term course in children.

Objective: We sought to understand the longitudinal trajectory of pediatric EoE during routine clinical care.

Evaluation of a Clinical Cancer Trial Research Training Workshop: Helping Nurses Build Capacity in Southwest Virginia.

Residents of Southwest Virginia (SWVA) face significant barriers in accessing the most advanced forms of cancer care, cancer risk reduction, and clinical trials involvement. A collaboration between the University of Virginia (UVA) Cancer Center and UVA School of Nursing was forged with oncology caregivers in this region to build community capacity to support Cancer Clinical trials (CCT) by strengthening the workforce, and thus improving health outcomes for this underserved region of Appalachia. The UVA School of Nursing designed an educational workshop focusing on the basics of CCT to facilitate the development of a skilled nursing workforce in the SWVA region that could provide care to patients on protocol and/or to encourage residents to participate in trials.

Predictors of student outcomes on perceived knowledge and competence of genetic family history risk assessment.

The publication of the human genome project has launched a number of discoveries set to change the landscape of healthcare. Unfortunately, nursing faculty across the United States report they are unprepared to teach students who will be practicing in the genomic era. The purpose of this study, utilizing Rogers' (2003) Diffusion of Innovation theory as a framework, was to determine the degree to which nursing school characteristics predict graduating undergraduate nursing students' perceived knowledge and competence of genetic family history risk assessment.

Protein tyrosine phosphatase SHP-1 positively regulates TLR-induced IL-12p40 production in macrophages through inhibition of phosphatidylinositol 3-kinase.

SHP-1 is a cytoplasm protein tyrosine phosphatase expressed primarily in hematopoietic cells. In the immune system, SHP-1 plays critical roles in regulation of many receptor-mediated signaling cascades, and SHP-1 deficiency in mice causes spontaneous inflammation and autoimmunity. Here, we report a unique requirement for SHP-1 in interleukin-12/23 p40 (IL-12p40) production in response to Toll-like receptor (TLR) stimulation in macrophages.

Cytosol as battleground: ubiquitin as a weapon for both host and pathogen.

Ubiquitin was first described as a tag allowing cells to degrade and recycle their own proteins. Recent research has shown ubiquitin to be central for immune system recognition of invading bacteria. This review describes a set of complex host-pathogen interactions that are dependent on ubiquitination.

Atg5-independent sequestration of ubiquitinated mycobacteria.

Like several other intracellular pathogens, Mycobacterium marinum (Mm) escapes from phagosomes into the host cytosol where it can polymerize actin, leading to motility that promotes spread to neighboring cells. However, only approximately 25% of internalized Mm form actin tails, and the fate of the remaining bacteria has been unknown. Here we show that cytosolic access results in a new and intricate host pathogen interaction: host macrophages ubiquitinate Mm, while Mm shed their ubiquitinated cell walls.

Reinventing practice and education partnerships for capacity expansion.

Two alternative models offering solutions to the nursing shortage in Texas enhance communication between schools of nursing and acute care service agencies to strengthen the partnership between education and practice. These initiatives use the valuable asset of onsite nurses in practice to clinically instruct nursing students, thus replenishing the number of clinical instructors and freeing clinical space to overcome the obstacles in increasing capacity. Forming practice-education partnerships and integrating technology support, including using simulations and online courseware to train qualified nurses, reinvent the combining resources to address a common need.