Urethral diverticulum: an unusual presentation of Wegener's granulomatosis.
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Biomedical subjects
Publications and source records attributed to E E Volk.
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BACKGROUND: Endothelial cells that line microvascular blood vessels have an important role in inflammation through their ability to bind and recruit circulating leucocytes. Endothelial cells from the intestines of patients with chronically inflamed Crohn's disease and ulcerative colitis--the two forms of inflammatory bowel disease--display an increased leucocyte-binding capacity in vitro. We investigated whether this enhanced leucocyte binding is a primary or an acquired defect. METHODS: We cultured human intestinal microvascular endothelial cells (HIMEC) from the uninvolved intestine and chronically inflamed bowel of three patients with inflammatory bowel disease (two Crohn's disease, one ulcerative colitis). We assessed HIMEC binding to polymorphonuclear leucocytes and U937 cells by means of an adhesion assay. FINDINGS: After activation with interleukin-1beta or lipopolysaccharide, HIMEC from the chronically inflamed tissue in all three patients with inflammatory bowel disease bound twice as many polymorphonuclear leucocytes and U937 cells as endothelial cells from uninvolved tissue. INTERPRETATION: Enhanced leucocyte binding by HIMEC from chronically inflamed tissue in patients with inflammatory bowel disease is an acquired defect since it is not found in the uninvolved intestinal segments from the same individuals. Because interaction between endothelial cells and leucocytes is a key regulatory step in the inflammatory process, this enhanced binding may contribute to the pathophysiology of chronic intestinal inflammation.
Bone marrow cultures (BMCs) and blood cultures (BCs) are frequently obtained in the evaluation of fever of unknown origin (FUO). However, the low yield of clinically significant isolates leads to questions about their cost-effectiveness. We retrospectively compared BMC with BC and studied the usefulness of bone marrow trephine biopsy (BMTB) histopathology in detecting infection in an unselected population of 61 patients with FUO, among whom 215 BMCs had been performed. For patients who had undergone BMTB, the histopathology was evaluated for granulomas and microorganisms. Only 1 BMC had a clinically significant isolate, Mycobacterium avium complex (MAC), which was also identified by BC. Rhodotorula rubra was found in the BMC of another patient and classified as a contaminant. Both patients had HIV infection. No growth occurred in BCs for the other 59 patients. Culture results for all 26 BMTB specimens were negative; 4 contained nonnecrotizing granulomas, including the case with MAC. BMCs are probably not justified for routine initial evaluation of FUO, but may be valuable after culture results for blood and easily obtainable tissues have been negative. Bone marrow histopathology and special stains for microorganisms in the absence of granulomas were noncontributory.
Focal active colitis (FAC) is a common pattern of injury in colorectal biopsy specimens. Recently, FAC was found to be a marker of an infectious colitis-like diarrheal illness and ischemic colitis but not of Crohn's disease. We evaluated 31 cases of FAC at the Cleveland Clinic Foundation, Cleveland. Ohio, between 1982 and 1992, to assess the clinical significance of this histologic finding. We evaluated the degree of neutrophil-mediated crypt epithelial injury, the degree of neutrophil-mediated surface epithelial injury, and the lamina propria cellularity and cell type. In each biopsy specimen, all of the above features were scored as 1+ (involving < 10%), 2+ (10-25%), or 3+ (26-50%) of the specimen. Clinical follow-up for the 31 patients ranged from 1 to 51 months (mean, 26 mo). Clinical diagnoses included infectious-type colitis (15 cases, 48%); incidental FAC (9 cases, 29%), occurring in asymptomatic patients undergoing screening for colonic neoplasia; ischemic colitis (3 cases, 10%); and Crohn's disease (4 cases, 13%). Histologically, all of the cases had some degree of cryptitis, and 24 (77%) of the 31 had neutrophil-mediated surface epithelial injury. In 13 (42%) of the 31, there was an expansion of the lamina propria by neutrophils, in 12 (39%) by eosinophils, and in 11 (35%) by plasma cells. None of the histologic features correlated with specific clinical diagnostic categories (Fisher's exact test). In conclusion, FAC most commonly correlates clinically with an infectious-type of colitis. On occasion, FAC might be a harbinger of Crohn's disease. Histologic features are not useful in predicting specific clinical diagnoses as a correlate to FAC.
Hamartomas are a poorly defined group of lesions and a rare cause of chronic epilepsy. We studied 13 patients, nine males and four females, whose cause of seizures was attributed to a hamartoma. The patients ranged in age from 6 to 33 years (mean, 21 years). Seizure duration before surgery ranged from 1.5 to 22 years (mean, 10 years). Seven hamartomas were located on the right side and six on the left. Six were located in the frontal lobe, five in the temporal lobe, and two in the occipital lobe. Twelve patients underwent gross total resection of the lesion and one a partial resection. All consisted of a circumscribed, disorganized collection of glial cells, primarily astrocytes. Rarely a neuronal component was admixed. One lesion contained an increased number of small blood vessels. Eight (62%) hamartomas contained eosinophilic granular bodies, and focal microcalcification was observed in three lesions (23%). Adjacent cortical architectural abnormalities (cortical dysplasia) were identified in eight (62%) resection specimens. Necrosis, mitoses, and prominent cytological atypia were absent in all lesions. Differential diagnostic considerations include low grade astrocytoma, ganglioglioma, dysembryoplastic neuroepithelial tumor, and cortical dysplasia. Postoperatively, 10 patients (77%) had complete resolution or greater than 90% reduction of seizure frequency. Two patients (16%) developed recurrent seizures 8 and 13 months postoperatively. One patient who underwent a partial resection showed no decrease in seizure frequency. No lesion recurrence on imaging studies has been observed in the 12 patients who underwent gross total resection of their hamartoma during 1 to 51 months (mean, 14 months) follow-up. We conclude that hamartomas seen in the setting of chronic epilepsy are generally low-grade lesions that respond well to gross total resection. Circumscription and lack of significant cytological atypia help distinguish these lesions from other neoplastic causes of epilepsy. Hamartomas that arise in the setting of chronic epilepsy appear to be associated with increased incidence of cortical architectural abnormalities (cortical dysplasia) and represent maldevelopmental lesions.
Percutaneous transluminal angioplasty is a technique widely used to treat stenotic atherosclerotic lesions of the coronary arteries. This technique is currently gaining acceptance for the treatment of cerebral atherosclerotic disease. Autopsy findings of fatal complications of percutaneous transluminal angioplasty of the posterior cerebral circulation have, to our knowledge, not yet been published. We report the case of a patient with severe diffuse atherosclerosis of vertebral and basilar arteries, whose symptoms were not ameliorated with standard medical therapy. Transfemoral percutaneous transluminal angioplasty was performed in an attempt to reestablish blood flow in the posterior cerebral circulation by angioplasty of a severely stenotic basilar artery. The patient suffered a fatal complication during the procedure due to rupture of the basilar artery by the percutaneous transluminal angioplasty guidewire apparatus, leading to massive subarachnoid hemorrhage. We report the clinical and autopsy findings of this case.
BACKGROUND & AIMS: Treatment for invasive adenocarcinoma in colorectal polyps (malignant polyps) is controversial. The aim of this study was to evaluate our institutional treatment strategy for malignant polyps. METHODS: Malignant polyps were designated as having favorable histology (grade I or II carcinoma with at least a 2-mm free margin) or unfavorable histology (grade III invasive adenocarcinoma, invasive adenocarcinoma with an unassessable margin, or a margin of < 2 mm). Malignant polyps with favorable histology were considered treated adequately by endoscopic polypectomy, whereas further therapy was recommended for malignant polyps with unfavorable histology. Recurrence, residual adenocarcinoma in a follow-up resection specimen, or metastasis during follow-up were considered adverse outcomes. RESULTS: Of the 47 patients identified, 17 (36%) had favorable histology. Sixteen patients (94%) were treated with polypectomy alone. None had an adverse outcome (median follow-up, 70 months). Thirty patients (64%) had unfavorable histology, and 21 patients (70%) underwent colectomy. Five patients underwent radiation therapy alone. Four patients underwent no additional therapy. Ten of 30 patients with unfavorable histology had adverse outcomes that differed significantly from the favorable histology group (P = 0.03). CONCLUSIONS: Endoscopic polypectomy alone is adequate therapy for malignant polyps with favorable histology.
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