1/19. 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) |
2/19. 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) |
3/19. Gammadelta T lymphocytosis associated with granulomatous disease in a patient with common variable immunodeficiency.common variable immunodeficiency (CVID) is a heterogeneous group of immunodeficiency syndromes that involves defective production of specific antibodies and decreased serum concentrations of > or =1 immunoglobulin isotype. We describe a patient with an atypical case of CVID who had extensive granulomatous lesions that were partially attributable to mycobacterial infection. In the peripheral blood, there was a massive increase in the number of double-negative CD3 T cells that expressed the gammadelta T cell receptor.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
4/19. 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) |
5/19. ecthyma gangrenosum and septic shock syndrome secondary to chromobacterium violaceum.chromobacterium violaceum is a gram-negative bacterium of soil and water in tropical and subtropical environments. Typically, it is considered a bacterium of low virulence although, uncommonly, it causes human infection, particularly in persons with defects in host defenses. Infection generally follows exposure of broken skin to contaminated water and soil, and is often characterized by pustules, lymphadenitis, fever, and vomiting, as well as rapid dissemination and a high mortality rate. Unfortunately, because C violaceum is ubiquitous, it is often dismissed as a contaminant when cultured. Because rapid diagnosis (by taking appropriate specimens) and treatment are vital to a good prognosis, it is imperative that physicians be aware of this organism. In addition, patients with chromobacterial infections should have an immunologic workup because infections in immunocompetent individuals are rare. Here we report an aggressive yet nonfatal case of C violaceum septicemia in an adolescent male, diagnosed through a punch biopsy of a skin lesion, and resulting in a new diagnosis of chronic granulomatous disease.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
6/19. Chronic granulomatous disease: a different pattern in hong kong?From July 1988 to December 1989, six boys with chronic granulomatous disease were diagnosed in our institutions. Their clinical features were reviewed in order to delineate the pattern of infections which seems to have both similarities and differences when compared with published reports of Caucasian patients. The most striking differences was the lack of skin sepsis and chronic lymphadenitis in our six patients. Gram-negative organisms were the commonest pathogens while Staphylococci sp. were not isolated. Clinical features which should alert one to the diagnosis were also highlighted. Prophylactic co-trimoxazole was effective in reducing the frequency of bacterial infections. early diagnosis is not only essential for optimal patient management but also for genetic counselling for the extended family.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
7/19. Infection caused by francisella philomiragia (formerly yersinia philomiragia). A newly recognized human pathogen.We evaluated the clinical characteristics of patients with francisella philomiragia (formerly yersinia philomiragia) isolated from normally sterile sites. Isolates from 14 patients were received by the Centers for disease Control between 1975 and 1987: 9 were from blood; 2 from lung biopsies; and 1 each from pleural, peritoneal, and cerebrospinal fluid. Underlying problems included chronic granulomatous disease in 5 patients, near-drowning in 5, and a myeloproliferative disease in 2. All 13 patients for whom records were available had a febrile syndrome compatible with bacterial infection. pneumonia and fever-bacteremia were the commonest clinical syndromes reported. In 7 cases, F. philomiragia was the only sterile-site isolate, and the clinical syndrome did not resolve without appropriate antibiotics. Familiarity with this organism is important because of its ability to cause serious disease in chronic granulomatous disease and near-drowning patients. Further study may yield new insights into pathogenic and host defense mechanisms.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
8/19. 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) |
9/19. Granulomatous cystitis in chronic granulomatous disease: ultrasound diagnosis.Chronic granulomatous disease (CGD) is a fatal hereditary disease of childhood characterized by chronic recurrent bacterial infections. Involvement of the genitourinary tract is uncommon. We report a child with CGD with granulomatous cystitis demonstrated by both ultrasound and computed tomography.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
10/19. Cytochrome b and FAD content in polymorphonuclear leucocytes in a family with X-linked chronic granulomatous disease.Chronic granulomatous disease (CGD), an immunodeficiency syndrome characterized by extreme susceptibility to bacterial infections, is due to a defect of the respiratory burst in human phagocytes. nadph oxidase, the enzyme that catalyzes the reduction of oxygen and the release of oxidative radicals, was studied in polymorphonuclear leucocytes (PMNs) in a family affected by an x-linked inheritance form at high penetrance of the disease. The contents of cytochrome b, suggested as the terminal component of the oxidase electron transport chain, and FAD, the hypothetical proximal component of the chain, were determined in patients and in carriers. Cytochrome b showed the typical behaviour of x-linked CGD: total absence in patients, intermediate values in carriers. FAD content evaluated on plasma membranes was less decreased than cytochrome b. Carriers also showed a decrease of this flavoprotein. Cytochrome b and FAD contents were compared to NBT test and superoxide production: a clear correlation was observed for the cytochrome b, but FAD plasma membrane evaluation could also be an interesting tool for the metabolic characterization of the disease in patients and in carriers.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
| | Next -> |