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Pulmonary responses to atmospheric pollutants. I: an ultrastructural study of fibrosing alveolitis evoked by petrol vapour.

Rats exposed to an atmosphere contaminated with petrol vapour at a concentration of 100 parts per million for up to 12 weeks exhibit a high incidence of electron microscopic changes in the lung parenchyma characterized by interstitial fibrosis with associated alveolar collapse. Initial changes appearing after 6 weeks include degeneration of endothelium and interstitial fibroblasts followed by hypertrophy of Type 2 pneumocytes. Subsequent degeneration of surfactant organelles of the hypertrophied Type 2 pneumocytes correlates with the appearance of focal alveolar collapse and associated interstitial fibrosis. Because of the rapidity with which lesions are induced in the rat lung, this experimental technique provides an economical and reproducible model for an integrated study of the sequential morphological and biochemical events preceding pulmonary fibrosis which might well lead to a better understanding of the enigmatic human syndrome of fibrosing alveolitis.

Animals↗

The pathology of experimental chronic fibrosing pancreatitis--light microscopic and ultrastructural observations.

The light microscopic and ultrastructural changes seen in a canine model of chronic fibrosing pancreatitis are described. The distribution of the experimental lesion is similar to that of human chronic obstructive pancreatitis. The canine lesion is also compared to that of human chronic calcifying pancreatitis. It is suggested that the common denominator in all these conditions is an increased pressure in the terminal intercalated ducts which produces pressure atrophy of the acinar cells in the periphery of the obstructed lobules.

Animals↗

Idiopathic fibrosing alveolitis: a review with emphasis on ultrastructural and immunohistochemical features.

Fibrosing alveolitis is a usually chronic pulmonary disease affecting middle-aged men and women and causing progressive dyspnea. This review discusses the nosologic, etiologic, immunopathogenic, histologic, immunohistochemical and ultrastructural features of this condition. A hypothesis is presented suggesting microvascular damage as the primary pathologic change in cases associated with collagen vascular diseases and viral pneumonia.

Acquired Immunodeficiency Syndrome↗

The effects of steroid therapy on pulmonary hypertension secondary to fibrosing mediastinitis.

Mediastinal fibrosis, a rare cause of pulmonary hypertension, may produce cough, dyspnea, and hemoptysis. Steroid therapy has been suggested for individuals with progressive symptoms, but data demonstrating the efficacy of such therapy are lacking. We present a case of pulmonary hypertension secondary to fibrosing mediastinitis. Hemodynamic and scintigraphic studies performed before and after a trial of corticosteroid therapy were unable to demonstrate any therapeutic benefit from the corticosteroids. In order to achieve better use of steroids for the treatment of this disease, we suggest that similar determinations be made on other patients with mediastinal fibrosis who receive such treatment.

Adult↗

Short report: a case of fibrosing mediastinitis caused by Wuchereria bancrofti.

Wuchereria bancrofti is a mosquito-borne filarial nematode that commonly invades lymphatic vessels. Common clinical manifestations include elephantiasis, orchitis, epididymitis, and chyluria. This report concerns an Egyptian man who developed superior vena cava syndrome secondary to a mediastinal mass that was found to contain a filarial nematode consistent with W. bancrofti. This is the first case, to our knowledge, of this parasite causing fibrosing mediastinitis.

Aged↗

Case report. The nonspecific rheumatoid subcutaneous nodule: its presence in fibrositis and scleroderma.

"Rheumatoid" subcutaneous nodules that were at one time considered diagnostic of rheumatoid arthritis or rheumatic fever have also been found in individuals withoug known disease, in patients with systemic lupus erythematosus, and in other conditions. In this report, subcutaneous nodules were described in one patient with fibrositis and in another scleroderma. Multiple pathogenic mechanisms are probably responsible for development of these nodules.

Adult↗

[Fibrosing alveolitis following Legionella pneumonia].

We reported a case of fibrosing alveolitis following Legionella pneumonia. A 62-year-old man was admitted to our hospital with fever after a visit to a hot spring. Chest X-ray films on admission demonstrated air-space consolidation in the right lower lung. Legionella pneumonia was diagnosed because the patient had elevated serum antibody to Legionella pneumophila serogroup Ia and tested positive for urinary antigen. Although he was initially treated with rifampicin and erythromycin, he experienced drug-induced eruptions. Antibiotic therapy was accordingly changed to clarithromycin, levofloxacin, and minocycline, which together alleviated the patient's clinical symptoms but delayed radiologic regression. Chest X-ray films 2 months after the onset of illness revealed diffuse ground-glass opacities and progressive reduction of volume in the right lung. Long-term corticosteroid treatment was required. Three and half months after disease onset, fever recurred with the appearance of interstitial shadows in the left lung and positive tests for urinary antigen. Increasing the corticosteroid dose resolved the patient's symptoms.

Anti-Inflammatory Agents↗

The spectrum of peritoneal fibrosing syndromes in peritoneal dialysis.

A variable degree of diffuse peritoneal fibrosis has been documented in all patients who have been on long-term peritoneal dialysis. Peritoneal dialysis-induced diffuse peritoneal fibrosis varies from opacification and "tanning" of the peritoneum, which may have only a moderate detrimental effect on peritoneal transport kinetics, to a progressive, sclerosing encapsulating peritonitis (SEP), which may lead to cessation of peritoneal dialysis and to death. Fewer than 1% of peritoneal dialysis patients develop overt SEP as manifested by combinations of intestinal obstruction, weight loss, and ultrafiltration failure. The diagnosis of SEP depends on a combination of laparotomy and radiological features in suspected cases and consequently the true incidence of SEP is most likely underestimated. Several predisposing, interrelated risk factors for both peritoneal fibrosis and sclerosing encapsulating peritonitis have been identified: prolonged duration of peritoneal dialysis, history of severe or recurrent episodes of peritonitis, and higher exposure to hypertonic glucose-based dialysis solutions. Nevertheless, the etiology of SEP is unknown and several causal factors may simultaneously or sequentially initiate and maintain a low-grade serositis that leads to uncontrolled fibroneogenesis. The high mortality rate of SEP has emphasized the need to develop preventive strategies. These strategies include early peritoneal catheter removal to avoid refractory peritonitis, the development of more biocompatible dialysis solutions, restriction of the use of hypertonic glucose-based dialysis solutions during and after episodes of peritonitis, and, perhaps, limiting the duration of peritoneal dialysis in at-risk patients. This approach was followed in a Japanese unit where a subgroup of all patients who had been on peritoneal dialysis for more than 5 years and who had poor ultrafiltration and peritoneal calcification on computed tomography (CT) scan were shown to have peritoneal sclerosis on peritoneal biopsy and were therefore electively transferred to hemodialysis. This acquired spectrum of peritoneal fibrosing syndromes leads to long-term complications in peritoneal dialysis, whereas localized fibrous adhesions secondary to prior abdominal surgery may prevent the successful initiation of peritoneal dialysis.

Fibrosis↗

Pulmonary mechanics in diffuse fibrosing alveolitis.

The mechanical and the diffusing lung properties have been measured in 19 cases of diffuse fibrosing alveolitis of unknown etiology (proved by histology in 17 cases). The characteristic findings are: a) a striking reduction of DLCO in all the cases; b) a marked reduction of the static and dynamic compliance, in correlation with the reduction of vital capacity; c) an increase in P1max in correlation with the decrease of FRC; d) an increase of the elastic recoil when it is considered at various percentages of the predicted lung volume, but in some cases a normal shape of the V-P curve if the measured lung volume is taken into account; e) a reduction of the maximum expiratory flows suggesting bronchiolar stenosis; f) alterations of DL more marked than those of CL suggesting that DL is a more sensitive test.

Adult↗

[Fibrosing mediastinitis].

Up to date aspects of fibrosing mediastinitis are discussed in the review. The most common changes in the organs of mediastinum have been described with regard to surgical possibilities of their treatment, particularly superior caval vein syndrome, strictures of bronchial tree and pulmonary vessels and changes of the esophagus. The approach to rich literature has been pointed out.

Fibrosis↗

[Fatal fibrosing cholestatic hepatitis following renal transplantation].

A 65-year-old HBsAg positive man developed progressive cholestatic liver enzyme abnormalities with histopathological portoportal septum formation, cholestasis, limited mixed infiltrate and hepatocellular ballooning with a ground glass aspect after renal transplantation. Both clinical and pathological features were characteristic of fibrosing cholestatic hepatitis (FCH), a histological variant of hepatitis-B-virus (HBV) infection with a high mortality rate which affects immunocompromised patients. The diagnosis was made about 9 months after transplantation, after retrospective analysis had shown a postoperative increase in HBV replication. Discontinuation of prednisone treatment and starting antiviral lamivudine therapy reduced HBV DNA load immediately. However due to renal failure caused by hepatorenal syndrome, lamivudine therapy had to be interrupted. The patient died following subacute liver failure with progressive FCH. This case illustrates the importance of early diagnosis and treatment with reduction of immunosuppression and institution of antiviral therapy to prevent progression of FCH in immunocompromised HBsAg positive patients.

Aged↗

[Postmenopausal frontal fibrosing alopecia. Report of 3 cases].

Postmenopausal frontal fibrosing alopecia is a rare aspect of scarring alopecia concerning elderly women. It appears as a receding anterior hair line localised in the frontal and temporal regions. It is a particular pathologic and clinical form of lichen planopilaris. The histologic aspect is that of a lichenoïd inflammatory infiltrate affecting the dermal follicular junction, accompanied by a fibrous scarring aspect, the latter contributing to the diagnosis and individualization of this entity. Discoïd lupus erythematous is the main histologic differential diagnosis. Postmenopausal period is the only associated condition found in affected women. Evolution is unpredictable and does not seem to be modified by treatment.

Aged↗

[Idiopathic fibrosing alveolitis in therapeutic practice].

Three cases of idiopathic fibrosing alveolitis (IFA) are reported. An algorithm of differential diagnosis with pneumonia is provided. IFA suspects should be referred to specialized pulmonological centers where they should be examined with performance of lung biopsy, high-resolution computed tomography with subsequent pathogenetic therapy with glucocorticoids.

Adult↗

Chronic focal fibrosing pancreatitis: detection by MRI.

The study of a 70-year-old woman with fibrosing pancreatitis, an uncommon variety of chronic pancreatitis, presenting as a discrete solid mass in the head of the pancreas, is reported. CT and US were non-diagnostic while ERCP and MR detected a focal anomaly. This case report stresses the sensitivity of MR in some pancreatic pathologies.

Aged↗

Fibrosing cholestatic hepatitis-like syndrome in a hepatitis B virus and hepatitis C virus-negative renal transplant recipient: a case report with autopsy findings.

We report a patient with fibrosing cholestatic hepatitis (FCH)-like syndrome in renal transplant recipient, who was negative for hepatitis-B and C-virus infection. The patient presented initially with extrahepatic biliary obstruction due to stricture at the lower end of the common bile duct. Cholestasis persisted inspite of effective biliary drainage. He was operated for empyema of the gallbladder and histological examination showed the presence of cytomegalovirus inclusions in the wall of the gallbladder. The patient died inspite of aggressive management; autopsy examination of the liver revealed evidence of FCH-like changes.

Autopsy↗

Benign fibrosing disease at the hepatic confluence mimicking Klatskin tumors.

BACKGROUND: Hilar obstructions remain a challenge with regard to diagnosis and treatment. METHODS: In the period from 1984 to 1990, 82 patients underwent resective surgery under the presumptive diagnosis of hilar cholangiocarcinoma (Klatskin tumor). The diagnosis was based on the combined appearances on direct cholangiography and ultrasonography in all cases, with the use of various other imaging modalities in some cases. RESULTS: The perioperative findings from an experienced surgical team were usually thought to be compatible with bile duct carcinoma. However, histologic examination of the resected specimens revealed benign fibrosing or localized sclerosing lesions in 11 patients (13.4%). CONCLUSIONS: The current state of diagnostic imaging fails as yet to discriminate reliably between benign and malignant hilar lesions. Whereas the immediate therapeutic consequences may be equal (resection followed by hepaticojejunostomy), the late consequences differ in a major way because benign disease has a much better prognosis. In the presence of suspicious hilar obstruction, operable lesions should not be treated by "palliative" intubational techniques and radiation therapy without a firm diagnosis of malignancy. However, overtreatment (extended liver resection, vascular reconstruction, and liver transplantation) should be avoided as well when a benign lesion has not been ruled out.

Adenoma, Bile Duct↗

Proline-oxidase and hydroxypyroline-2-epimerase activities in experimental fibrosing granulomatous processes of the lung.

Three experimental fibrosing granulomatous processes of the lung (pulmonary granulomas induced by complete Freund adjuvant, viral pneumonitis induced by the A2 influenza virus, and tuberculosis induced in animals exposed to tobacco smoke) were produced in rabbits and the results confronted in view of establishing some relationships between mesenchymal cell accumulations, reticulin fibrillogenesis, and the enzymic activities of proline-oxidase (PO) and of hydroxyproline-2-epimerase (HEP), enzymes intervening in the control of hydroxyproline incorporation in the procollagen molecule. Histopathologic, histochemical and histoenzymic methods, quantification of cells and fibrils, statistical analyses, including the regression lines method, were used. The confrontation of the three processes made obvious that the cell accumulation invariably preceded the fibrillogenetic process, that the immune nature of the process was accompanied by larger cell accumulations than the non-immune one, that between cell accumulations and fibrillogenesis there existed highly significant correlations, and that the increase of the enzymic activities of proline-oxidase and of hydroxyproline-2-epimerase always accompanied these tissular changes, being topographically coincident with them (interlobular areas, alveolar walls).

Amino Acid Oxidoreductases↗