David S. Grenda

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Severe congenital neutropenia (SCN) is an inborn disorder of granulopoiesis. Mutations of the ELA2 gene encoding neutrophil elastase (NE) are responsible for most cases of SCN and cyclic neutropenia (CN), a related but milder disorder of granulopoiesis. However, the mechanisms by which these mutations disrupt granulopoiesis are unclear. We hypothesize that(More)
Severe congenital neutropenia (SCN) is a syndrome characterized by an isolated block in granulocytic differentiation and an increased risk of developing acute myeloid leukemia (AML). Recent studies have demonstrated that the majority of patients with SCN and cyclic neutropenia, a related disorder characterized by periodic oscillations in the number of(More)
Tetracycline and quinolone antibiotics have for many years served as important classes of veterinary drugs. Two representatives of both classes: doxycycline from tetracyclines and flumequine from quinolones are often administered together. When the withdrawal periods are not obeyed, the antibiotic residues may be present in edible products, e.g., in meat,(More)
Severe congenital neutropenia (SCN) is an inborn disorder of granulopoiesis that in many cases is caused by mutations of the ELANE gene, which encodes neutrophil elastase (NE). Recent data suggest a model in which ELANE mutations result in NE protein misfolding, induction of endoplasmic reticulum (ER) stress, activation of the unfolded protein response(More)
Neutrophils are critical components of the innate immune response, and persistent neutropenia is associated with a marked susceptibility to infection. There are a number of inherited clinical syndromes in which neutropenia is a prominent feature. A study of these rare disorders has provided insight into the mechanisms regulating normal neutrophil(More)
Extracorporeal membrane oxygenation (ECMO) is an established therapy in the management of patients with refractory cardiogenic shock or acute respiratory failure. In this report, we describe the rapid development and implementation of an organized ECMO program at a facility that previously provided ad hoc support. The program provides care for patients(More)
Extracorporeal membrane oxygenation (ECMO) has become an increasingly utilized modality for the support of patients with severe cardiac or pulmonary dysfunction. Unfortunately, the costs and expertise required to maintain a formal ECMO program preclude the vast majority of hospitals from employing such technology routinely. These barriers to implementation(More)
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