1/26. 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, infection (Clic here for more details about this article) |
2/26. Recurrent Pseudomonas infection associated with neutrophil dysfunction.A 72-year-old male is described with a history of 4 episodes of pseudomonas aeruginosa sepsis and chronic otitis media caused by pseudomonas species. in vitro testing of the patient's polymorphonuclear leukocytes (PMNs) revealed profoundly abnormal chemotactic responses and defective intracellular killing of Ps. aeruginosa, staphylococcus aureus and escherichia coli. Chemiluminescence production by the patient's PMNs in response to opsonized zymosan as well as endotoxin stimulated nitroblue tetrazolium dye reduction were markedly depressed. These data indicate the presence of a profound, apparently acquired, defect in PMN function in an elderly male. Detailed evaluation of adult patients with recurrent infections may reveal similar, apparently acquired defects in PMN function.- - - - - - - - - - ranking = 0.11137112774556keywords = infection (Clic here for more details about this article) |
3/26. 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 = 1.0222742255491keywords = bacterial infection, infection (Clic here for more details about this article) |
4/26. Respiratory syncytial virus infection in patients with phagocyte defects.patients with phagocyte defects frequently develop bacterial or fungal pneumonias, but they are not considered to be at increased risk for viral infections. We describe 3 patients with known phagocyte immunodeficiencies who developed lower respiratory tract infections (LRTI) caused by respiratory syncytial virus (RSV). All 3 patients had dense pneumonias as indicated by computed tomography scan of the lungs and RSV was recovered. We conclude that RSV can present as a dense pneumonia in patients with phagocyte defects. Along with common pathogens causing LRTI, RSV should be considered in the differential diagnosis. Viral cultures as well as rapid antigen detection assays for respiratory viruses should be included in the evaluation of LRTI in patients with phagocyte defects. respiratory syncytial virus, phagocyte, immunodeficiency, pneumonia.- - - - - - - - - - ranking = 0.13364535329467keywords = infection (Clic here for more details about this article) |
5/26. association of Pseudomonas cepacia with chronic granulomatous disease.Pseudomonas cepacia was recovered from a number of infected sites in three patients with chronic granulomatous disease of childhood. The organisms were identified on the basis of their oxidative utilization of a variety of carbohydrates and their positive beta-galactosidase and oxidase activities. They were resistant to most antimicrobial agents and moderately susceptible to chloramphenicol. Peripheral blood leukocytes isolated from two siblings with chronic granulomatous disease, including one of the patients in this series, failed to kill P. cepacia in vitro. Prolonged prophylactic and antimicrobial therapy may well have played a significant role in the colonization and infection of these patients with P. cepacia.- - - - - - - - - - ranking = 0.022274225549112keywords = infection (Clic here for more details about this article) |
6/26. A case of impaired chemotaxis and lymphocyte transformation.The report describes the clinical syndroms of a 14-year-old boy which suffered from recurrent infections since early infancy. The clinical and general laboratory findings were similar to "the granulomatous disease of childhood" as described by Bridges et al. (8). The following serum factors were determined: immunoglobulins, complement factors, isoagglutinins. The following assays with normal or patient's granulocytes were done: Chemotactic activity, nitroblue-tetrazolium test, bactericidal assay, fungicidal assay, myeloperoxidase, monocytes fungicidal assay. Immunological studies include kinetics of phytohaemagglutinin response, effect of serum of the patient on lymphocytic reactivity in vitro and skin tests. The following results were obtained: 1) Assays with normal or patient's granulocytes showed an impaired chemotatic activity, when serum of the patient was added. There was strong indication by treatment of the patient with plasma infusions, that the chemotactic defect is a serum dependent factor. 2) It could be demonstrated that the patient's serum also inhibited the response of lymphocytes to tuberculin and phytohaemagglutinin. Therefore the patient report focuses attention upon the possibility of serum related abnormalities that may influence granulocytic as well as lymphocytic functions leading to recurrent bacterial, fungal and viral infections.- - - - - - - - - - ranking = 0.044548451098223keywords = infection (Clic here for more details about this article) |
7/26. Diffuse chronic granulomatous mucocutaneous candidiasis.A 3-year-old Thai boy with diffuse chronic granulomatous mucocutaneous candidiasis, recurrent bacterial skin infection and adrenal insufficiency is reported. candida albicans was demonstrated in the dermal granuloma. He had a defect in cell-mediated immunity and was anemic. Although therapy with topical clotrimazole, oral iron, systemic antibiotic and low-dose of prednisone gave a dramatic result, he died of disseminated cryptococcosis.- - - - - - - - - - ranking = 0.022274225549112keywords = infection (Clic here for more details about this article) |
8/26. An inherited defect in granulocyte function: impaired chemotaxis, phagocytosis and intracellular killing of microorganisms.The neutrophil granulocytes of a 1-year-old boy with severe recurrent infections were found to have almost no chemotactic responsiveness, impaired phagocytosis and reduced intracellular killing of candida albicans "in vitro". During the course of a febrile illness of unknown etiology which was accompanied by a leukemoid reaction, phagocytic activity became normal; the chemotactic response was also increased but still remained slightly subnormal. family studies suggested that the defect was an inherited autosomal recessive one.- - - - - - - - - - ranking = 0.022274225549112keywords = infection (Clic here for more details about this article) |
9/26. 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, infection (Clic here for more details about this article) |
10/26. 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, infection (Clic here for more details about this article) |
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