Very early onset inflammatory bowel disease (VEO-IBD), diagnosed <6 years old, can be genetically and phenotypically distinct and more refractory than older-onset IBD. Identified causal monogenic defects have been targeted therapeutically in a small subset of VEO-IBD; however, for most of these children, treatment strategies, such as phenotypic profiles, are critically needed to improve outcomes.
View Article and Find Full Text PDFBackground: Gastrostomy tube (G-tube) placement is a common intervention for newborns with severe feeding difficulties. Infants with congenital diaphragmatic hernia (CDH) are at high risk for feeding problems. Prevalence of G-tube placement and consequent nutritional outcomes of infants with CDH and G-tubes has not been described.
View Article and Find Full Text PDFAlthough B cells have emerged as important contributors to chronic graft-versus-host-disease (cGVHD) pathogenesis, the mechanisms responsible for their sustained activation remain unknown. We previously showed that patients with cGVHD have significantly increased B cell-activating factor (BAFF) levels and that their B cells are activated and resistant to apoptosis. Exogenous BAFF confers a state of immediate responsiveness to antigen stimulation in normal murine B cells.
View Article and Find Full Text PDFCombination studies of histone deacetylase inhibitors (HDACi) and proteasome inhibitors are providing preclinical framework to build better strategies against hematologic malignancies. Our previous work found that a novel proteasome inhibitor, NPI-0052, and HDACi synergistically induce apoptosis in leukemia cells in a caspase-8- and oxidant-dependent manner. Here we extend those observations to primary leukemia cells and identify novel mechanisms of synergy.
View Article and Find Full Text PDF