1/14. Primary pulmonary botryomycosis. A manifestation of chronic granulomatous disease.A 19-year-old woman with a childhood history of cavitating left upper lobe pneumonia presented with persistent weight loss, fever, cough and roentgenographic evidence of right upper lobe pneumonia resistant to antibiotic therapy. An open lung biopsy led to the diagnosis of botryomycosis. Neutrophil function studies including flow cytometric evaluation of oxidative burst, bacterial killing and evaluation of neutrophil cytosolic proteins required for oxidase activation were consistent with chronic granulomatous disease. This is the first case report of primary pulmonary botryomycosis as a clinical manifestation of CGD. Other recent cases of immunodeficiency states associated with botryomycosis are reviewed.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
2/14. Interpretation of granulomatous lesions in malignancy.In patients with malignancy the most frequent granuloma-associated conditions are tumor-related sarcoid reactions, sarcoidosis, tuberculosis and other infections. Quite often, the finding of granulomatous lesions in patients with cancer may lead to difficulties of interpretation resulting in inappropriate treatment of both granulomatous disease and malignancy. This problem is reviewed and exemplified by a number of typical case histories. A systematic diagnostic approach must integrate anamnestic, clinical, histomorphological, immunohistological and laboratory information. In addition, prolonged follow-up may be necessary in order to establish the true nature of a granuloma-associated condition.- - - - - - - - - - ranking = 0.2keywords = granulomatous disease (Clic here for more details about this article) |
3/14. A novel bacterium associated with lymphadenitis in a patient with chronic granulomatous disease.Chronic granulomatous disease (CGD) is a rare inherited disease of the phagocyte nadph oxidase system causing defective production of toxic oxygen metabolites, impaired bacterial and fungal killing, and recurrent life-threatening infections. We identified a novel gram-negative rod in excised lymph nodes from a patient with CGD. Gram-negative rods grew on charcoal-yeast extract, but conventional tests could not identify it. The best 50 matches of the 16S rRNA (using BLAST) were all members of the family acetobacteraceae, with the closest match being gluconobacter sacchari. Patient serum showed specific band recognition in whole lysate immunoblot. We used mouse models of CGD to determine whether this organism was a genuine CGD pathogen. Intraperitoneal injection of gp91(phox -/-) (X-linked) and p47 (phox -/-) (autosomal recessive) mice with this bacterium led to larger burdens of organism recovered from knockout compared with wild-type mice. Knockout mouse lymph nodes had histopathology that was similar to that seen in our patient. We recovered organisms with 16S rRNA sequence identical to the patient's original isolate from the infected mice. We identified a novel gram-negative rod from a patient with CGD. To confirm its pathogenicity, we demonstrated specific immune reaction by high titer antibody, showed that it was able to cause similar disease when introduced into CGD, but not wild-type mice, and we recovered the same organism from pathologic lesions in these mice. Therefore, we have fulfilled Koch's postulates for a new pathogen. This is the first reported case of invasive human disease caused by any of the acetobacteraceae. Polyphasic taxonomic analysis shows this organism to be a new genus and species for which we propose the name Granulobacter bethesdensis.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
4/14. Defective initiation of the metabolic stimulation in phagocytizing granulocytes: a new congenital defect.Two patients suffering from recurrent bacterial infections were studied: a boy and a girl from one family, children of apparently healthy parents. The granulocytes of these patients were capable of normal ingestion of latex particles and dna-anti-dna immune complexes. When the metabolic changes in these granulocytes during phagocytosis of latex particles were studied, however, no stimulation of oxygen consumption, superoxide production, or hexose monophosphate shunt activity could be observed. Moreover, zymosan particles were not iodinated. These findings are comparable to those found in chronic granulomatous disease. In sharp contrast to the observations in this latter disease, however, a completely normal stimulation of cell metabolism was found after phagocytosis of IgG-coated latex particles or IgG aggregates. Since latex and IgG-coated latex were equally well ingested, this means that the absence of metabolic stimulation after uptake of tatexf metabolic stimulation after uptake of latex must be due to a defect in the triggering of the oxidative burst. As far as we know, this is the first time that a defect in the triggering of the metabolic stimulation during phagocytosis could be demonstrated. Moreover, these finding suggest that adherence and subsequent ingestion of particles are in themselves not sufficient for the metabolic stimulation of granulocytes.- - - - - - - - - - ranking = 0.2keywords = granulomatous disease (Clic here for more details about this article) |
5/14. 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 = granulomatous disease (Clic here for more details about this article) |
6/14. 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 = 0.4keywords = granulomatous disease (Clic here for more details about this article) |
7/14. Limitations in the use of indium-111-oxine-labeled leucocytes for the diagnosis of occult infection in children.Fifteen children underwent scintigraphy with indium 111 (111In)-labeled white blood cells (WBC) for the detection of a local suppuration. The procedure generally contributed to a correct diagnosis. False negative results were observed in 5 children, but in two of them positive foci were also present. The missed lesions were 2 liver abscesses, 1 lung abscess, foci of osteomyelitis and 1 pericarditis. Two cases of chronic granulomatous disease are presented in which increased leucocyte accumulation was not observed in proven instances of infection.- - - - - - - - - - ranking = 0.2keywords = granulomatous disease (Clic here for more details about this article) |
8/14. Chronic granulomatous disease in pregnancy.The ability of phagocytes to kill certain bacteria is impaired in chronic granulomatous disease. This results in frequent infection in the childhood period and frequent death in the early teens. survival beyond this period is rare and often occurs in patients who have a mild variant of the disease. Reported herein is the obstetric course of a patient with chronic granulomatous disease.- - - - - - - - - - ranking = 1.2keywords = granulomatous disease (Clic here for more details about this article) |
9/14. Intrinsic polymorphonuclear chemotactic defect in a boy with chronic granulomatous disease.A six year old boy is described who suffured from recurrent and protracted infections of multiple organs by various catalase positive bacteria. A severe episode of osteomyelitis involving several bones was caused by aspergillus fumigatus. Studies of his PMNs revealed impaired metabolic as well as microbicidal functions characteristic of CGD. Chemiluminescence in response to both opsonized zymosan and sodium fluoride was markedly depressed, while control PMNs showed significant responses. Control leukocytes suspended in patient's serum likewise evoked normal chemiluminescence. Microbicidal activity against staphylococcus aureus 502A was also decreased using patient's PMNs, whereas control PMNs were able to reduce the number of colony forming bacteria by 2 logs in 120 minutes. Viable intracellular bacteria after lysis of extracellular bacteria formed 3 X 10(7) colonies from patient's PMNs and less than 2 X 10(5) colonies from the control. NBT dye reduction studies of the family members suggested an x-linked recessive mode of inheritance. The extraordinary nature of this case lies in the discovery of an associated intrinsic cellular defect of chemotaxis involving his polymorphonuclear leukocytes. Specifically, the Rebuck skin window showed predominantly mononuclear cells from 4 up to 24 hours. In addition, the patient's PMNs failed to migrate in response to cultured filtrates of E. coli as the chemoattractant. This abnormality persisted in the presence of autologous plasma or serum as well as in control plasma or serum. Control PMNs showed normal chemotaxis in the presence of the patient's plasma or serum. The extent to which the rare coexistence of these two phenomena influence the clinical disease is not known and remains to be elucidated.- - - - - - - - - - ranking = 0.8keywords = granulomatous disease (Clic here for more details about this article) |
10/14. chromobacterium violaceum infection of the eye. A report of two cases.We report what we believe to be the first two known cases of chromobacterium violaceum infection of the eye. One patient had conjunctivitis as the initial manifestation of an ultimately fatal infection. In the second patient, who suffered from chronic granulomatous disease of childhood, orbital cellulitis developed as part of a disseminated C violaceum infection from which he subsequently recovered. Infections caused by C violaceum may involve the eye and orbit. These infections are frequently fatal, but early recognition and aggressive treatment may result in a higher cure rate.- - - - - - - - - - ranking = 0.2keywords = granulomatous disease (Clic here for more details about this article) |
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