Quality Improvement to Identify and Address Food Insecurity During Pediatric Hospitalizations.

Objectives: Hospitalized children represent a vulnerable population with high rates of unidentified food insecurity (FI). We aimed to improve FI screening for eligible families from 0% to 60%. Secondarily, we sought to provide location-based food resources to families that screened positive.

Increase in the level of β-HCG after uterine artery embolization in the context of partial molar pregnancy: A case report.

This report documents the case of a patient with a previously diagnosed partial molar pregnancy evacuated by dilation and suction curettage with appropriately declining post-operative levels of beta-human chorionic gonadotropin (beta-hCG), who, one month later, underwent uterine artery embolization in the setting of acute bleeding and imaging concerning for arteriovenous malformation. After embolization, beta-hCG levels increased, prompting concern for gestational trophoblastic neoplasia and referral to gynecologic oncology. With further workup, the elevation was found to be transient and benign - a phenomenon not previously described.

Lymphocytes Utilize Somatic Mutations, Epigenetic Silencing, and the Proteasome to Escape Truncated WASP Expression.

Wiskott-Aldrich Syndrome Protein (WASP) deficiency causes Wiskott-Aldrich Syndrome (WAS), a sex-linked disorder characterized by combined immunodeficiency, microthrombocytopenia, and eczema. Like WASP-deficient humans, WASP-deficient mice produce normal numbers of functionally defective T cells. Here, we report a WAS patient with a novel germline frameshifting WAS mutation encoding a truncated form of WASP lacking the C-terminal cofilin homology (C) and the acidic region (A) domains (WASPΔCA).

Constrained chromatin accessibility in PU.1-mutated agammaglobulinemia patients.

The pioneer transcription factor (TF) PU.1 controls hematopoietic cell fate by decompacting stem cell heterochromatin and allowing nonpioneer TFs to enter otherwise inaccessible genomic sites. PU.

The genes regulate the sphingolipid synthesis pathway to ensure proper myelination and neurologic function in mice.

Sphingolipids are membrane and bioactive lipids that are required for many aspects of normal mammalian development and physiology. However, the importance of the regulatory mechanisms that control sphingolipid levels in these processes is not well understood. The mammalian ORMDL proteins (ORMDL1, 2 and 3) mediate feedback inhibition of the de novo synthesis pathway of sphingolipids by inhibiting serine palmitoyl transferase in response to elevated ceramide levels.

Common variable immunodeficiency-associated endotoxemia promotes early commitment to the T follicular lineage.

Background: Although chiefly a B-lymphocyte disorder, several research groups have identified common variable immunodeficiency (CVID) subjects with numeric and/or functional T cell alterations. The causes, interrelationships, and consequences of CVID-associated CD4 T-cell derangements to hypogammaglobulinemia, autoantibody production, or both remain unclear.

Objective: We sought to determine how circulating CD4 T cells are altered in CVID subjects with autoimmune cytopenias (AICs; CVID+AIC) and the causes of these derangements.