title: Assessment of oral ascorbate in three children with chronic granulomatous disease and defective neutrophil motility over a 2-year period.
abstract: Two brothers and their sister with chronic granulomatous disease, elevated levels of serum IgE and defective neutrophil motility were treated with a single oral daily dose of 1 g sodium ascorbate as a supplement to prophylactic trimethoprim--sulphamethoxazole therapy for 2 years. Laboratory tests of neutrophil functions were performed prior to ascorbate therapy and repeated at 1-monthly intervals for 6 months and at 6-monthly intervals thereafter. Introduction of ascorbate to the therapeutic regimen was accompanied by slight increases in neutrophil hexose monophosphate shunt activity and staphylocidal activity and good improvement of neutrophil motility in all three children. The improved staphylocidal activity was not due to ascorbate-mediated inhibition of neutrophil or serum catalase activities or to detectable increases in superoxide and H2O2 production or activity of the MPO/H2O2/halide system. Both male children have remained free from obvious infection since ascorbate was added to their therapeutic regimen; their sister has experienced one urinary tract infection during a period when treatment with prophylactic co-trimoxazole and ascorbate was inadvertently stopped. All three children have gained weight.
mesh_terms: D001205:Ascorbic Acid / Q000627:therapeutic use*; D001770:Blood Bactericidal Activity; D002374:Catalase / Q000097:blood; D002634:Chemotaxis, Leukocyte / Q000187:drug effects; D002648:Child; D002675:Child, Preschool; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D006801:Humans; D006861:Hydrogen Peroxide / Q000097:blood; D007073:Immunoglobulin E / Q000032:analysis; D008297:Male; D009504:Neutrophils / Q000187:drug effects / Q000276:immunology* / Q000378:metabolism; D013481:Superoxides / Q000096:biosynthesis
chemical_list: D013481:Superoxides; D007073:Immunoglobulin E; D006861:Hydrogen Peroxide; D002374:Catalase; D001205:Ascorbic Acid