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1.
Chronic granulomatous disease (CGD) is a rare inherited disease of childhood, characterized by recurrent bacterial or fungal infections. The underlying defect is a dysfunction of neutrophil granulocytes interfering with their ability to kill phagocytosed microorganisms. Genitourinary tract involvement has been reported in 38% of these patients. We report a case of CGD in whom the most important findings were urinary bladder tumors at different locations and a subsequent obstruction of the left ureter. A review of the pathogenesis of the disease, potential involvement of the urinary tract and treatment is presented.  相似文献   

2.
Chronic granulomatous disease (CGD) is a rare genetic disorder in which phagocytes fail to produce superoxide because of defects in one of several components of the NADPH oxidase complex. As a result, patients develop recurrent life-threatening bacterial and fungal infections. The organisms to which CGD patients are most susceptible produce catalase, regarded as an important factor for microbial pathogenicity in CGD. To test the role of pathogen-derived catalase in CGD directly, we have generated isogenic strains of Aspergillus nidulans in which one or both of the catalase genes (catA and catB), have been deleted. We hypothesized that catalase negative mutants would be less virulent than the wild-type strain in experimental animal models. CGD mice were produced by disruption of the p47(phox) gene which encodes the 47-kD subunit of the NADPH oxidase. Wild-type A. nidulans inoculated intranasally caused fatal infection in CGD mice, but did not cause disease in wild-type littermates. Surprisingly, wild-type A. nidulans and the catA, catB, and catA/catB mutants were equally virulent in CGD mice. Histopathological studies of fatally infected CGD mice showed widely distributed lesions in the lungs regardless of the presence or absence of the catA and catB genes. Similar to the CGD model, catalase-deficient A. nidulans was highly virulent in cortisone-treated BALB/c mice. Taken together, these results indicate that catalases do not play a significant role in pathogenicity of A. nidulans in p47(phox)-/- mice, and therefore raise doubt about the central role of catalases as a fungal virulence factor in CGD.  相似文献   

3.
OBJECTIVE: Cerebrospinal fluid (CSF) diagnoses encompass a wide spectrum of conditions. The authors review one institution's CSF cytology results over an 11-year period. DESIGN AND SETTING: A retrospective study of 5951 CSF specimens generated between 1985 and 1995. Specimens from pediatric patients (<19 years of age) from the same time period were separately identified. RESULTS: A total of 5561 adult and 390 pediatric CSF specimens were interpreted. A diagnosis of "negative for malignant cells" was assigned in 5171 (93%) of the adult cases and in 351 (90%) of the pediatric cases. Specific infectious organisms were identified in 26 adult specimens and one pediatric specimen. Cryptococcus was the most common infectious agent observed (n = 23 adults), and Toxoplasma was the sole pediatric infectious agent. Two hundred seventy-six (5%) adult cases and 31 (8%) pediatric cases were positive for malignant cells. Diagnoses included metastatic tumors (adult, 140 [51%]; pediatric, 0); lymphoma/leukemia (adult, 112 [41%]; pediatric, 4 [13%]); malignant unclassified neoplasms (adult, 9 [3%]; pediatric, 0); and primary central nervous system neoplasms (adult, 12 [4%]; pediatric, 27 [87%]). Medulloblastoma was the most common pediatric neoplasm (n = 21). There were 105 (2%) adult cases and 8 (2%) pediatric cases with atypical cells present. Atypical lymphoid cells were the most common type in adult cases (53%). CONCLUSIONS: In our experience, infectious agents were rarely identified in pediatric CSF specimens. In adult specimens, the most commonly identified organisms was Cryptococcus. Primary central nervous system neoplasms accounted for a higher percentage of CSF specimens in the pediatric population than in the adult population. The most commonly identified malignancy in adults was metastatic neoplasms, and in children, medulloblastoma.  相似文献   

4.
The cytologic examination of fine-needle aspirates and fluid specimens is plagued by a persistent false negative rate. The rate of false negative results will be decreased if sensitive molecular assays can be developed to detect cytologically malignant cells. The current study investigated telomerase expression as a potential marker of malignancy, using the telomeric repeat amplification protocol (TRAP) in fine-needle aspirates and fluid specimens. TRAP was performed on 24 fine-needle aspirate and 24 fluid specimens from different body sites and of different histological diagnoses. We found that 6 of 12 fine-needle aspirate specimens that were cytologically positive for malignant cells expressed telomerase activity, while no specimens that were cytologically suspicious for malignancy, atypical, or negative tested positive for telomerase activity. Of the fluid specimens, 4 of 6 cytologically positive cases and 1 of 18 cytologically negative cases expressed telomerase. Seven of eight telomerase negative, cytologically positive specimens contained only rare malignant cells in a very bloody background. Peripheral blood contamination is a possible pitfall in the TRAP assay, as applied in the current study, because the assay is standardized to protein concentration that may be derived from lysed red blood cells. We conclude that with further technical refinement, the TRAP assay could become a useful adjunct in the cytologic examination of fine-needle aspirates and fluid samples.  相似文献   

5.
OBJECTIVE: To determine the pathologic outcome in human immunodeficiency virus (HIV)-seropositive individuals with nonspecific bronchoalveolar lavage (BAL) cytology. STUDY DESIGN: The study group consisted of 126 cytologically negative or nonspecific BAL specimens from HIV-seropositive adults. Concurrent microbial cultures and transbronchial biopsies, as well as subsequent pulmonary cytology, lung biopsy or autopsy results were reviewed. Additionally, the cytologic morphology of specimens from patients found to have a potential bacterial pathogen was reviewed. RESULTS: In the 126 cases with nonspecific BAL cytology, a potential pulmonary pathogen was identified from a concurrent or subsequent pathologic specimen in 27% of cases, while no pathogen was identified in 73% of cases. Bacteria and fungi were the most common pathogens identified. Microbial cultures alone identified the pathogen in 59% of cases, while transbronchial biopsy added information in only 9%. Specimens with marked acute inflammation often yielded bacterial pathogens on microbial culture. CONCLUSION: A potential pulmonary pathogen can be identified in 27% of HIV-seropositive individuals with negative BAL cytology using other diagnostic modalities. Bacterial pathogens are most common and are usually identified by microbial culture. Marked acute inflammation in a BAL specimen is often associated with bacterial pneumonia.  相似文献   

6.
BACKGROUND/AIMS: Despite the high frequency of benign hepatic cysts, they rarely cause symptoms. Large cysts, however, may produce clinical manifestations. In these cases, ultrasonography-guided therapy has been proposed. We report the results of this therapy in patients with symptomatic benign hepatic cysts. METHODOLOGY: Seven patients with non-parasitic, non-neoplastic benign hepatic cysts were submitted to fine-needle ultrasonography-guided aspiration, followed by injection of diluted tetracycline hydrochloride (1 g). In all cases, cytology, tumour markers and microbiology analysis of aspirates were performed. One patient required two sessions. The grade of patient satisfaction and ultrasonography changes were assessed 1, 3 and 6 months after the procedure. RESULTS: Cyst size decreased in all patient, with total collapse in three. No major complications occurred. Cytology, alpha fetoprotein, CA19.9, CEA and microbiology of the cyst fluid confirmed the initial ultrasonographic diagnosis of simple biliary cysts. Clinical complaints improved in 2 cases, and 5 patients became asymptomatic. Ultrasonography evaluation 3 months after the procedure was more reliable in predicting successful treatment. CONCLUSION: Intracystic instillation of tetracycline hydrochloride is an effective and safe technique and may become the first choice therapy for benign hepatic cysts.  相似文献   

7.
To estimate the prevalence of chronic granulomatous disease (CGD) in Sweden, an inquiry asking for known and possible CGD cases was mailed to paediatric, internal medicine and infectious disease departments all over Sweden. The detected patients were characterized as to genetics and the clinical presentation. Twenty-one patients (belonging to 16 different families) were found, corresponding to a prevalence of approximately 1/450,000 individuals. The patients with X-linked disease, lacking a functional gp91phox protein (n = 12), comprised 57% and 43% of the patients had an autosomal recessive (AR) disease lacking p47phox (n = 7) or p67phox (n = 1), respectively. All unrelated patients with X-linked disease displayed different gene abnormalities such as point mutations predicting nonsense (n = 3), missense (n = 1) or splice site mutations (n = 2), but also a total deletion and a unique 40 base pair duplicature insertion. The patients with p47phox-deficiency showed a GT deletion at a GTGT tandem repeat, and the p67phox-deficient patient displayed a heterozygous in-frame deletion of AAG combined with a large deletion in the other allele. Three patients died during the study period, two from pseudomonas cepacia infections. Patients with X-linked disease had more frequent infections (mean of 1.7 per year), than the patients with AR inheritance (0.5 infections per year). The most common infections were dermal abscesses (n = 111), followed by lymphadenitis (n = 82) and pneumonias (n = 73). Inflammatory bowel disease-like symptoms, mimicking Crohn's disease of the colon, was seen in three CGD patients.  相似文献   

8.
DB Safran  E Dawson 《Canadian Metallurgical Quarterly》1997,132(11):1184-8; discussion 1188-9
OBJECTIVE: To assess the effect of aggressive antifungal prophylaxis and empiric antifungal therapy using fluconazole on the mycotic microbiology and associated infectious complications in a surgical intensive care unit. DESIGN: Retrospective review of a cohort of critically ill surgical patients treated during an 11-month period. SETTING: Surgical intensive care unit, university hospital, state-designated level I trauma center. PATIENTS: All patients treated with fluconazole during the study. MAIN OUTCOME MEASURES: Positive fungal cultures obtained after commencement of antifungal prophylaxis or antifungal treatment with fluconazole. Overall and infectious mortality rates for patients with positive cultures were also measured. RESULTS: Of 72 surgical patients who were treated with fluconazole; 16 (22%) had secondary mycoses. Fourteen (88%) of these patients were receiving fluconazole as antifungal prophylaxis or as empiric treatment of suspected but unproved infection. The predominant organisms isolated from these 16 patients were Candida glabrata (41%) and Candida parapsilosis (41%). Overall mortality for this group was 44%, and infectious mortality was 38%. The infectious mortality rate was significantly higher than the rate found in patients who were successfully treated with fluconazole for primary mycoses, and who did not have secondary infections with resistant organisms (mortality, 9%; P < .01, chi 2). CONCLUSIONS: Emergence of resistant species after treatment with fluconazole does occur in surgical patients, and suggests that the development of a secondary fungal infection with a resistant organism may be associated with a poor prognosis.  相似文献   

9.
In chronic granulomatous disease (CGD), diminished or absent neutrophil NADPH oxidase function leads to recurrent pyogenic infections and granuloma formation. In a recent randomized, placebo-controlled trail, short-term prophylactic use of recombinant human interferon gamma (rIFN-gamma 1b) reduced the risk of serious infection in CGD patients by 67%, The current study evaluated the safety and effectiveness of long-term rIFN-gamma therapy in CGD patients. Patients were treated three times weekly with rIFN-gamma and evaluated semiannually. Serious infections (requiring hospitalization and parenteral antibiotic therapy), adverse clinical events, and measures of growth and development were noted. Thirty patients were evaluated for 12 months. The total average duration of rIFN-gamma therapy was 2.5 years. Three patients developed a total of four serious infections (0.13 infections per patient year). This rate compare favorably with rates of 1.10 and 0.38 infections per patient year found in the placebo and rIFN-gamma groups, respectively, during a previous study. Common adverse events were fever (23%), diarrhea (13%), and flu-like illness (13%). No serious adverse event was attributable to rIFN-gamma therapy and no obvious effects on growth and development were observed. rIFN-gamma is a safe and effective adjunctive therapy for reducing the frequency and severity of serious infections in CGD patients.  相似文献   

10.
BACKGROUND: The purpose of this study was to assess the capability of magnetic resonance imaging (MRI) and cytology and flow cytometric (FCM) deoxyribonucleic acid (DNA) analysis in fine-needle aspiration biopsy (FNAB)-derived materials for diagnosing malignancy of the parotid lesions and the efficacy of FCM analysis in FNAB. METHODS: Magnetic resonance imaging findings and FCM results (ploidy and S + G2 + M phases [S + G2M] fraction) and cytology in FNAB-derived materials in 26 patients with 26 parotid lesions (12 benign lesions, 14 malignancies) were assessed for predicting malignancy. Flow cytometric results in aspirates were compared with those in surgically resected tissues. RESULTS: When a single predictor was used, cytology (92% accuracy) was most accurate for malignancy, followed by ill-defined margin (88% accuracy) and aneuploidy (88% accuracy). The combination of FCM and cytology raised the rate of sufficient materials from 92% to 100% and accuracy from 92% to 96% compared with cytology alone. The same highest accuracy (96%) was obtained with the combination of the ill-defined margin or other findings such as cytology, aneuploidy, or a high (S + G2M) fraction (6% <). Deoxyribonucleic acid ploidy in the FNAB showed full agreement with that in the surgical specimens. Receiver operating characteristic curves showed that the diagnosis of malignancy with (S + G2M) fraction in FNAB was superior to that in surgical specimens, but no significant difference was noted. CONCLUSIONS: A combination of MRI findings, cytology, and FCM results is optimal for diagnosing malignancies of the parotid lesions, and FNAB may replace the surgical specimens in FCM analysis.  相似文献   

11.
We reviewed the results of microscopic Gram stain examination and routine culture for 2,635 cerebrospinal fluid (CSF) samples processed in an adult hospital microbiology laboratory during 55 months. There were 56 instances of bacterial or fungal meningitis (16 associated with central nervous system [CNS] shunt infection), four infections adjacent to the subarachnoid space, four cases of sepsis without meningitis, and an additional 220 CSF specimens with positive cultures in which the organism isolated was judged to be a contaminant. Because 121 of these contaminants were isolated in broth only, elimination of the broth culture would decrease unnecessary work. However, 25% of the meningitis associated with CNS shunts would have been missed by this practice. The most common cause of meningitis was Cryptococcus neoformans, followed by Streptococcus pneumoniae and Neisseria meningitidis. In 48 of 56 (88%) of cases, examination of the Gram-stained specimen revealed the causative organism. If patients who had received effective antimicrobial therapy prior to lumbar puncture are excluded, the CSF Gram stain is 92% sensitive. Microscopic examination incorrectly suggested the presence of organisms in only 3 of 2,635 (0.1%) CSF examinations. Thus, microscopic examination of Gram-stained, concentrated CSF is highly sensitive and specific in early diagnosis of bacterial or fungal meningitis.  相似文献   

12.
We describe 3 cases of Hodgkin's disease (HD) of unusual suppurative type, which were diagnosed on fine-needle aspirates. The smears were dominated by neutrophils, macrophages, and cellular debris. Only a few large, atypical cells of Hodgkin and Reed-Sternberg type were observed. The differential diagnoses of such smears include infectious mononucleosis, tuberculosis, metastatic lymph node involvement, non-Hodgkin's large-cell anaplastic Ki-1-positive lymphomas, T-cell-rich B-cell lymphomas, and peripheral T-cell lymphomas of mixed type. Immunocytochemistry identified the large atypical cells as CD 30 (BerH2)-positive and negative for CD 45 (LCA) in cytospin material from 2 patients, which allowed a conclusive diagnosis of HD.  相似文献   

13.
We reviewed our experience with 9,726 cases of fine-needle aspiration cytology of the breast that were done from January 1983 to February 1992. During our review, we found that 214 aspirates had been submitted from pregnant and lactating women for the investigation of breast mass(es). Despite a variable clinical presentation and spectrum of cytologic findings, we considered the application of aspiration cytology in these women as useful as in the nonpregnant-nonlactating women for management decision. A team approach between the clinician and cytopathologist was always maintained; with this approach, the false-positive or -negative diagnoses were reduced to almost nil. In all cases in which the cytodiagnosis of carcinoma of breast was made, the findings corresponded with subsequent cell blocks from the aspirate and tissue examination. Benign lesions were diagnosed cytologically with minimal difficulty, and all aspirates that were less than optimal for cytodiagnosis were repeated to minimize the chance of missing an abnormality. All the benign lesions were followed throughout pregnancy, postpartum, and thereafter; if the mass persisted, the aspiration was repeated. With this protocol of follow-up, the need for a customary liberal surgical biopsy was reduced to a minimum.  相似文献   

14.
OBJECTIVE: To establish the aerobic and anaerobic microbiology of perianal cellulitis in children, comparing skin swab and needle aspirate methodology. METHOD: Swabs of involved skin and needle aspirates of cellulitis were studied for aerobic and anaerobic bacteria. RESULTS: Specimens obtained from 10 patients with perianal cellulitis showed bacterial growth. Polymicrobial aerobic-anaerobic flora was found in all skin surface cultures, where the predominate isolates were Peptostreptococcus spp., Escherichia coli, and alpha hemolytic streptococci. The number of isolates in needle aspirates varied between one and two. The predominant ones were E. coli (3), Peptostreptococcus spp. (3), Staphylococcus aureus (2), and Bacteroides fragilis group (2). Complete or partial concordance in microbiology between skin swabs and needle aspirates was present in six instances. In four instances, isolates recovered from needle aspirates were not isolated from the skin surface. CONCLUSIONS: This study demonstrates the diversity of aerobic and anaerobic organisms isolated from perianal cellulitis, and the superiority of needle aspirates in establishing the microbiology of the infection.  相似文献   

15.
The microbiology of needle aspirates from 40 inflamed cervical lymph glands was studied for aerobic and anaerobic bacteria, fungi and mycobacteria. Forty-two bacterial, 11 mycobacterial and six fungal isolates were isolated. Aerobic bacteria only were recovered in 11 (27.5%), anaerobes alone in five (12.5%) and mixed aerobic and anaerobic bacteria in seven (17.5%). Mycobacterium sp. were recovered in 11 (27.5%) and fungi in six (15%). The recovery of anaerobes was associated with dental infection. Eighteen aerobic bacteria were isolated and the predominant ones were Staphylococcus aureus (eight isolates) and group A streptococci (four). Twenty-four anaerobic bacteria were recovered and the predominant ones were: Prevotella sp. (six), Peptostreptococcus sp. (five), Propionibacterium acnes (four) and Fusobacterium sp. (three). These findings demonstrate the role of anaerobic organisms in cervical lymphadenitis and the need to culture aspirated material from the glands for both aerobic and anaerobic microorganisms.  相似文献   

16.
The incidence of indections requiring hospitalization was determined in 122 patients with sarcoidosis. The group was remarkably free of infection except for three patients with Aspergillus mycetoma occurring in areas of long-standing parenchymal involvement with cystic degeneration. There was a single instance of complicating pulmonary tuberculosis, and the only extrathoracic infection was a single instance of disseminated herpes zoster. This study confirms that aspergillosis, not tuberculosis, is currently the most common infectious complication of sarcoidosis. Although previous case reports have suggested an increased incidence of invasive fungal infection in patients with sarcoidosis, there is little to support this concept. None of the patients in the present study group developed these fungal infections during a mean 7.2-year follow-up. The clinical presentation of many of the previously reported cases suggests that the entire course of the granulomatous illness was infectious in nature rather than sarcoidosis with complicating infection.  相似文献   

17.
The telomerase enzyme is capable of replacing telomeric DNA sequences that are lost at each cell division. It has been suggested that the function of this enzyme is necessary for cells to become immortal, and in concordance with this hypothesis, telomerase activity has been detected in malignant tumor cells, whereas the enzyme is inactive in normal somatic cells. The measurement of this activity in human tissue samples may have diagnostic value, and in this study, we examined whether such a measurement may be useful for the detection of malignant cells within the thyroid. Telomerase activity was assayed using the telomeric repeat amplification protocol and related to the histological diagnosis of thyroid biopsy tissue samples and of cells obtained from the thyroid by fine-needle aspiration (FNA). Extracts from 9 of 11 (82%) carcinoma biopsy tissue samples contained telomerase activity, whereas enzyme activity was detected in only 2 of 14 (14%) benign tissue sample extracts. These two positive cases were subsequently diagnosed as Graves' disease with severe lymphocytic infiltration. Five of six (83.3%) histologically confirmed carcinoma FNA samples were identified by using the telomeric repeat amplification protocol assay, and two samples considered to be suspicious by FNA cytology were also positive. Conversely, only 4 of 48 (8.3%) benign FNA samples had telomerase. These promising data indicate that this sensitive assay could become a useful adjunct to microscopic cytopathology in the detection of cancer cells in small tissue biopsies and in fine-needle aspirates of the thyroid.  相似文献   

18.
AIMS: For the diagnosis of pulmonary alveolar proteinosis from bronchoalveolar lavage specimens it is normally necessary to make an ultrastructural examination. However, this is thought to be impractical for bronchoalveolar lavage specimens that have been routinely fixed in ethanol. In the present study, bronchoalveolar lavage cytology smears on slide glasses were examined directly ultrastructurally to make a diagnosis of pulmonary alveolar proteinosis. METHODS: Bronchoalveolar lavage smears from three pulmonary alveolar proteinosis patients were stained with Papanicolaou and periodic acid-Schiff (PAS) for identification of amorphous globular structures. Subsequently, they were refixed with glutaraldehyde and osmium tetroxide, and embedded in epoxy resin. Ultrathin sections were cut and examined ultrastructurally. RESULTS: Papanicolaou stained specimens from pulmonary alveolar proteinosis patients contained scattered amorphous or granular globules, 20-50 microns in diameter, which were PAS positive. Ultrastructural examination of the globules revealed multilamellated structures, characteristic of pulmonary alveolar proteinosis, in all cases. CONCLUSIONS: In general, it is thought that the morphological diagnosis of pulmonary alveolar proteinosis from bronchoalveolar lavage specimens requires both cytological and ultrastructural examination. However, the amorphous globules evident on cytology smears proved to contain multilamellated structures so that they can themselves be used as diagnostic evidence.  相似文献   

19.
This retrospective review of infectious complications was undertaken at two craniofacial centers (Dallas and Philadelphia). Fourteen infections were identified over a 6.5-year period in 567 intracranial procedures primarily for craniosynostosis. There were no infections in infants under 13 months of age and no cases of meningitis. The overall infection rate was 2.5 percent, and 85 percent of infections occurred in secondary reoperative cases. Tracheostomies were not identified as a risk factor for infection. No difference was found in infection rates between patients with shaved and unshaved scalps. Candida and Pseudomonas were the two most common organisms identified, and 28 percent of our infections involved yeast. The average time to diagnose infection was 11.5 days (excluding three patients who averaged 5 months). Thirteen of the fourteen infections were treated surgically with placement of a subgaleal irrigation/drainage system. Initial bony debridement was kept to a minimum. Based on our findings, recommendations are made to further lower infection rates, particularly those caused by opportunistic organisms.  相似文献   

20.
Toward the completion of elective colorectal operations, 75 patients had qualitative aerobic and anaerobic cultures of specimens obtained from peritoneal irrigation fluid, anastomoses sites, and abdominal wound irrigation fluid to determine if a correlation exists between intraoperative flora and postoperative infectious complications. Patients enrolled in this prospective study received a mechanical bowel prep and a 12-18 hour course of perioperative intravenous antibiotics. Comparisons were made between the 60 (80%) patients who had no postoperative infections and the 15 (20%) who developed postoperative infectious complications (9 wound infections, 6 intraabdominal infections). There were significantly more low anterior resections in patients who developed postoperative infection compared to those who had no postoperative infection (26% vs 2%), while there were more colocolostomies in the group with no infections (38% vs 7%). Streptococcus spp., Bacteroides fragilis group, and Escherichia coli were the most commonly isolated organisms from each of the three sites sampled. Isolation of > or = 3 organisms from incisional wound cultures (P = 0.017) and < or = 4 organisms from peritoneal irrigation (P = 0.009) or anastomotic culture (P = 0.004) correlated with development of postoperative infectious complications. Thus, patients with infectious complications had significantly more isolates than those without infectious complications, and were more likely to have had a low anterior resection. These data suggest that future clinical studies should reexamine the duration of perioperative antimicrobials based on early laboratory reports of qualitative and quantitative operative site bacteriology.  相似文献   

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