[Chronic septic granulomatous disease in two brothers (author's transl)].
Explore the source record for details and available documents.
SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Explore the source record for details and available documents.
Two cases in adults with recurrent staphylococcal infections associated with abnormal granulocytic chemotaxis and hyperimmunoglobulinaemia E (Job's syndrome) are described. The pathophysiological mechanisms seems to consist of an abnormal IgE reaction against staphylococcal antigens causing secondary abnormality of granulocyte function. Abnormal cellular immune function was demonstrated in vitro and in vivo. Corticosteroid administration at first proved effective in both patients. One patient developed Hodgkin's disease of the mixed type in the course of the disease.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Particulate fractions from normal human granulocytes preactivated with opsonized zymosan were found to catalyze superoxide production in the presence of reduced pyridine nucleotides. Similar preparations from three patients with X-linked chronic granulomatous disease produced no detectable superoxide. The failure to produce superoxide was not due to an inhibitor, since cell-free preparations from the patients' granulocytes had no effect on superoxide production by normal particles. Particles from the mothers of two of the patients produced superoxide at diminished rates; superoxide production by particles from the third mother was normal. These findings suggest that chronic granulomatous disease represents either a defect in a pyridine nucleotide-dependent superoxide-forming oxidase or a lesion in the apparatus responsible for activating the oxidase.
Explore the source record for details and available documents.
Explore the source record for details and available documents.