This inpatient and outpatient Clinical Research Center proposes to: 1) facilitate the transfer of basic knowledge generated in research laboratories to the clinical arena by elucidating pathogenic mechanisms of disease and evaluating new modes of patient management, and 2) augment investigative collaborations among the clinicians and researchers at SCRF. Twenty-eight research projects are proposed for studies on the Center. Among these protocols are studies to examine the therapeutic role and effective regimen for 2-Chlorodeoxyadenosine, a new lymphocyte-selective antineoplastic agent, in patients with chronic lymphocytic leukemia, lymphoma, hairy cell leukemia, mycosis fungoides and several autoimmune syndromes. The molecular genetic abnormalities underlying Gaucher Disease and its clinical variants are also being studied with the ultimate aim of correcting these hereditary enzyme deficiencies. The pathogenetic roles of active bronchial tissue kallikrein and associated generation of kinin in asthmatic disease are also under study. The degree and consequences of activation of the contact and complement systems of plasma proteins in asthma and bronchitis, arthritis, and angioedema are under study with the intent to establish their relative roles in pathogenesis in the various syndromes. Regulation of superoxide production in human neutrophils will be examined in patients with chronic granulomatous disease or adult respiratory distress syndrome. Studies are also underway investigating the roles of analgesics and food additives in the induction of attacks of bronchial asthma and in mechanisms for suppressing these sensitivities. Other studies include investigations of the neurological markers of familial alcoholism, the pathogenetic mechanisms responsible for hepatocellular injury and malignant transformation during hepatitis B virus infection, and molecular defects in von Willebrand disease and Bernard-Soulier Syndrome.
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