1/7. Chronic granulomatous disease of childhood.Two boys and one girl suffering from recurrent severe bacterial infections were investigated. All 3 exhibited normal cellular and humoral immunity, normal neutrophil phagocytic ability, and defective neutrophil bacterial capacity. The clinical features and laboratory findings in these patients are diagnostic of chronic granulomatous disease. A sex-linked inheritance pattern was confirmed in 1 patient by the demonstration of a heterozygous carrier state in the mother.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
2/7. Chronic granulomatous disease: an inherited disorder of phagocytosis in a Maori ancestry.Chronic granulomatous disease, in which abnormal susceptibility to infection is caused by an inherited defect in phagocytic cells, has been diagnosed in three brothers. Two brothers had repeated bacterial infections of the skin, superficial lymph nodes and lungs from infancy and died aged 27 months and 13 months. Characteristic suppurating granulomata were found in many organs. The diagnosis was established in both during life, and in the third asymptomatic brother shortly after birth, by studies of phagocytic function which included tests for nitroblue-tetrazolium reduction, hexose monophosphate shunt activity and bactericidal capacity. Their mother and a maternal aunt, both Maoris with no known Caucasian ancestry, were identified as carriers of the presumed sex-linked recessive gene. The clinical features of the disease and the laboratory methods for diagnosis are described.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
3/7. Multiple leukocyte abnormalities in chronic granulomatous disease: a familial study.A variety of leukocyte enzyme activities were studied in an 11-year-old female with chronic granulomatous disease (CGD) and several members of her family. Leukocyte glucose-6-phosphate dehydrogenase (G-6-PD) activity was 17 nmol/min/mg protein in the patient; two brothers with symptoms of recurrent bacterial infections have G-6-PD activities of 58 and 37 nmol/min/mg protein; the activites of this enzyme in both parents, maternal grandmother, and one additional brother were within normal limits. Storage at 4 degrees or heating at 37 degrees over a 120-min period revealed a marked lability of G-6-PD activity in the patient's cells which could not be stabilized by the addition of nadp and 2-mercaptoethanol; this lability was not seen in other family members tested. Activities of leukocyte glutathione reductase were reduced in both parents and the two affected male siblings with values of 18, 23, 23, and 24 nmol/min/mg protein, respectively. Activities of leukocyte glutathione peroxidase were reduced in all of the immediate family members tested, with values ranging from 11.2 to 43 nmol/min/mg protein; the activity of this enzyme in the patient was 38.5. Leukocyte nadp content in the patient, father, and two affected male siblings were 16.5, 23.4, 22.2, and 28.2 nmol/15 min/10(7) leukocytes, respectively.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
4/7. Chronic myelogenous leukemia simulating chronic granulomatous disease.A 5-year illness of a child, characterized by recurrent bacterial infections and abnormal results of nitroblue tetrazolium dye reduction tests, was suggestive of chronic granulomatous disease but the illness terminated in overt myeloid leukemia. During this progression studies of leukocyte structure and metabolic activity revealed abnormalities that suggested the existence of a "preleukemic" state.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
5/7. Increased susceptibility to infection in hypothermic children: possible role of acquired neutrophil dysfunction.The addition of hypothermia to regimens to control cerebral edema in children at our institution has been associated with a substantial incidence of infectious complications. Of the 13 children maintained at 30 degrees C to prevent cerebral edema, 3 developed haemophilus influenzae pneumonia and 2 developed streptococcus pneumoniae sepsis (one with pneumonia). The importance of neutrophil (PMN) function for elimination of bacterial pathogens prompted in vitro studies of PMN function at clinically attainable hypothermic temperatures. neutrophils at 30 degrees C had significantly less ability to migrate towards a chemotactic stimulus (45 /- 10% inhibition; P less than 0.02), to ingest staphylococci (22 /- 5% inhibition; P less than 0.01) and to be metabolically activated as measured by superoxide production (35 /- 10% inhibition; P less than 0.01) or by chemiluminescence (18 /- 8% inhibition; P less than 0.05). These in vitro findings support the clinical observation that persons with decreased body temperature may be at an increased risk for bacterial infections secondary to PMN dysfunction.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
6/7. Granulomatous hepatitis and pleuritis after ileal bypass for obesity.A patient is presented who developed a granulomatous hepatitis and pleuritis approximately 7 months after an ileal bypass procedure for morbid obesity. Although the etiological agent was presumed to be mycobacterium tuberculosis no pathogenic organism was grown from the liver, pleura, bone marrow, sputum, or gastric aspirate. The possibly increased susceptibility of these patients to mycobacterial infections is discussed. The value of obtaining serum levels of ethambutol, isoniazid, and rifampin, in patients with malabsorption is stressed. Although this patient seemed to respond to antituberculous therapy, other possible causes for the granulomatous process are explored.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
7/7. Job's syndrome.Job's syndrome is characterized by the clinical features of fair skin, red hair, recurrent cold staphylococcal skin abscesses with concurrent other bacterial infections and skin lesions. This case report chronicles a classic presentation of Job's syndrome. A brief review of the otolaryngological presentations of immune deficiencies is presented. Other than its relationship to the manifestations of other immunological defects, Job's syndrome is of interest to the otolaryngologist because of the head and neck infections represented in its clinical expression. Familiarity with phenotypic and laboratory manifestations of such diseases will enable earlier recognition and treatment of this syndrome.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |