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At least 541 records · Page 30Linked to original sources
PubMed2002

Unique pattern of fibrosing cholestatic hepatitis after liver transplantation.

OBJECTIVE: To explore the pathological features and the differential diagnosis of recurrent HBV after liver transplantation. METHODS: One case of liver transplantation for HBV cirrhosis was subjected to liver biopsies on time postoperatively. RESULTS: 25 days after liver transplantation, serologic HBsAg, HBeAg and HBV-DNA of the patient became negative, but HBsAg was positive again on day 58 after liver transplantation. Histopathological examination showed balloon-like changes of hepatocytes with fragmental necrosis, fibrosis in the portal areas and around the portal veins, cholestasis in some hepatocytes and canaliculi, and positive HBsAg and HBcAg with immunohistochemical staining. clinically hepatic enzyme levels progressively increased, maintained for some time, and decreased rapidly at last. Stubborn hypoproteinemia was associated with the aggregation of general condition of the patient. CONCLUSIONS: Fibrosing cholestatic hepatitis (FCH) is a special type in recurrent infection of HBV after liver transplantation. It has a serious clinical process and specific pathological changes different from those of the usual HBV.

Biopsy↗
PubMedDate not supplied

[Atypical adenomatous hyperplasia and dysplasia in squamous epithelium of the honeycomb lung in outcome of idiopathic fibrosing alveolitis].

Relationships between sclerosis and carcinogenesis in the honeycomb lung were studied in the outcome of two variants of idiopathic fibrosing alveolitis (IFA)-common interstitial pneumonia (CIP) and desquamative interstitial pneumonia (DIP) which may be a background for lung carcinoma development. The material was obtained from 43 patients with the diagnosis of IFA. Immunohistochemically were studied: TNF-alpha (DAKO, Denmark, 1:100), pancytokeratines (Immunotech, Germany, concentration 1:100), Ki67 (DAKO, Denmark, 1:40), TGF-beta (Biosource international, USA, 1:100), CD34 (Novocastra, Great Britain, 1:100), EMA (DAKO, Denmark, 1:100). Differences in morphogenesis of CIP and DIP were found. CIP is characterised by primary pronounced lung interstitium damage with stroma vascularisation already at early stages with secondary involvement of the epithelium with development of adenomatous hyperplasia with or without atypia which is usually observed at the stage of lung honeycomb. Pronounced primary damage of alveolar epithelium as a result of action of activated alveolar macrophages with subsequent proliferation, desquamation and squamous epithelium metaplasia were more typical for DIP. The presence of squamous meta- and dysplasia of the epithelium is characteristic for DIP outcome in the honeycomb lung.

Adenomatosis, Pulmonary↗
PubMed2003

Clinical experience with tamoxifen in peritoneal fibrosing syndromes.

Peritoneal sclerosis is one of the most important complications of peritoneal dialysis (PD) treatment. Encapsulating peritoneal sclerosis (EPS) represents the most advanced stage of that disease and has a high mortality. No therapy of choice has been established for sclerosing peritonitis, although many have been proposed, with variable results. Tamoxifen has been successfully used in the treatment of patients with fibrosing diseases, mainly retroperitoneal fibrosis. Our purpose in the present study was to investigate whether treatment with tamoxifen in PD patients with peritoneal sclerosis has a beneficial effect. Among more than 450 patients treated in our program since 1980, 23 were diagnosed with peritoneal sclerosis. Of those 23.9 were treated with tamoxifen [20 mg every 12 hours: tamoxifen group (TG)] for a mean period of 14.5 +/- 7 months (range: 6-30 months). The other 14 patients received no treatment and were considered the control group (CG). Both groups were similar in demography and peritoneal antecedents. Follow-up was longer in CG than in TG (mean: 47 months vs. 29 months), but the difference did not reach statistical significance. Mild thrombopenia in 1 patient was the only toxic effect observed with the use of tamoxifen. In CG, 4 patients developed EPS and died--3 of them during the first 6 months after diagnosis. No patient treated with tamoxifen developed EPS. Overall mortality was significantly higher in CG (71% vs. 22%, p = 0.03). Although follow-up was longer in CG, half the patients in that group died during the first 2 years after diagnosis. Our experience suggests that treatment with tamoxifen of patients diagnosed with peritoneal sclerosis diminishes the related complications and significantly reduces mortality, at least in the short- to mid-term. However, a prospective therapeutic trial is required to confirm our results.

Adult↗
PubMed1992

[A case of chronic fibrosing mediastinitis].

This case report describes a 32-year-old male patient with superior vena cava syndrome due to chronic fibrosing mediastinitis involving superior vena cava, bilateral brachiocephalic, subclavian, and internal jugular veins. Although the ringed ePTFE graft was placed between left internal jugular vein and right atrium twice, it occluded each time. Pathological examination of the removed specimen showed invasion of the fibrous tissue into the lumen of the graft at the anastomotic site and severe fibroelastosis in the intima of the jugular vein. The third reconstruction of the venous system with the ringed ePTFE substitute was done in the same manner. Postoperative venography demonstrated the patent graft and the interruption at the midportion of the left internal jugular vein. The patient remains free from the symptoms one year and nine months after the last operation.

Adult↗
PubMed2004

[Fibrosing mediastinitis: a rare cause of fever].

A 47-year-old woman was examined due to fever of unknown origin. She had been on holiday in Southeast Asia. Routine laboratory investigations confirmed the presence of inflammation. Serology for Hepatitis B virus, HIV, Borrelia, Cytomegalovirus, toxoplasmosis, lues, Epstein Barr virus, brucellosis, bartonellosis, histoplasmosis and auto-immune factors was negative. CT-scans of the chest and abdomen failed to reveal the cause. Finally, gallium-67 scintigraphy showed an increased uptake in the left mediastinum and the left side of the neck. This led to the discovery of an infraclavicular mass. On histological examination of the surgical excision biopsy the diagnosis 'fibrosing mediastinitis' was made. The patient was treated successfully with corticosteroids.

Adrenal Cortex Hormones↗
PubMed2004

[Exogenous fibrosing alveolitis due to the condensation aerosol (smoke) of zinc oxide].

Clinical-and-biological, biochemical, immunological, histomorphological; X-ray and functional examinations of workers of an electric-melting shop manufacturing brass alloys, who had contacts with condensation aerosol with a high zinc oxide concentrations, were used to detect in them pneumoconiosis with the exogenous fibrosing alveolitis (ZEFA). Some workers had acute conditions, i.e. "foundry fever" speaking in clinical terms, which was followed by a period of "visible improvement" lasting on the average for 8.6 +/- 0.8 years. The latter was described by the autoimmune activation of B-lymphocytes accompanied by an intensified formation of circulating immune complexes with a sharp reduction of the DR-cell content. The disease onset is gradual with the below signs: increasing dyspnea, cough and cyanosis of the lips due to the developing hypoxemia with decreasing PO2 (below 80 mm Hg); it can also be displayed through a mixed type of respiratory insufficiency with a lower PO2 and a higher PCO2 (above 40 mm Hg)-X-ray showed reticular changes in the pulmonary pattern. Generation of a high-above-norm quantity of active forms of oxygen and nitrogen by alveolar macrophages and neutrophils in their contacts with cellular membranes is the key mechanism triggering the pathological process like it happens in all cases of pneumoconiosis. Transformation of the mentioned products of free-radical oxygenation into hydroxyl radicals in the catalytic centers of the dust-particle borders containing zinc (which is, like iron, a metal with transient valence) is ZEFA specificity. The factor draws together ZEFA with pathological processes caused by asbestos-fiber dust, which have iron ions in their catalytic centers.

Aerosols↗
PubMed2004

[Vasaprostan treatment of fibrosing alveolitis in patients with pulmonary hypertension].

AIM: To study clinical efficacy of vasaprostan in patients with fibrosing alveolitis (FA) complicated by pulmonary hypertension (PH), its effect on functional activity of platelets and endothelium, intensity of free radical processes. MATERIAL AND METHODS: Seven FA patients were examined. They had either idiopathic FA or FA with diffuse diseases of the connective tissues. The following methods were used to assess the effect: standard clinical tests, high resolution computer tomography, Doppler echocardiography, definition of the complex thrombin-antithrombin (TAT) and thrombocytic factor 4 (TF-4). Generation of oxygen active forms by leukocytes was measured by luminol-dependent chemiluminescence. Morphological verification of the diagnosis was made by the results of open pulmonary biopsies. RESULTS: Vasaprostan reduced pressure in the pulmonary artery from 31.6 +/- 2.31 to 19.58 +/- 3.90 mm Hg (p < 0.05) and coagulation parameters. TAT decreased after 2 and 8 weeks of treatment from 15.25 +/- 4.5 to 5.1 +/- 0.33 and 2.4 +/- 0.31 pg/ml (p < 0.05). Initially low TF-4 (2.11 +/- 0.39 pg/ml) elevated to the end of the treatment and reached values close to control (4.37 +/- 0.25 pg/ml, p < 0.05). Moreover, vasaprostan enhanced the ability of platelets to inhibit generation of active oxygen forms (from 0.9 +/- 0.18 to 1.23 +/- 0.16 r. u., p < 0.05) and thus depressed activity of lipid peroxidation. CONCLUSION: Good effect of vasaprostan on platelet activity, free radical processes validates its use in combined treatment of various FA forms for correction of PH, its complications and as an antifibrogenic agent.

Adult↗
PubMedDate not supplied

[Specific features of humoral immunity in cryptogenic fibrosing alveolitis patients].

Cryptogenic fibrosing alveolitis (CFA) is a severe autoimmune disease of unclear etiology and prognostically unfavorable. The complexity of the diagnostics of this disease makes it necessary to search for new methods; for this reason immunity in CFA patients must be studied. The study of humoral organ-specific, organ-unspecific and antibacterial immunity of CFA patients revealed that the latter differed from the members of the groups used for comparison by a higher frequency of positive reactions in EIA determinations of IgG antibodies to cytokeratin-8 and Moraxella catarrhalis antigens. In addition, only in CFA patients a high degree of correlation (r=0.88) between these results was established. This made it possible to propose to use these reactions for confirming the diagnosis of CFA and suggested the probable role of M. catarrhalis in triggering autoimmune reactions characteristic of this disease.

Antibodies, Bacterial↗
PubMed2004

Non-neoplastic, degenerative brain pathologies and fibroses diagnosed on the basis of ultra-small samples obtained by stereotactic biopsy.

Brain biopsy and other stereotactic procedures have evolved over the last decades. Recently, the morbidity and mortality decreased radically along with an increase in the number of successful histopathological diagnoses. Therefore, applications of appropriate treatments in neoplastic brain pathologies are now possible, especially of those located in deep regions. Stereotactic biopsy may also be used as a diagnostic method followed by appropriate management in conditions where a non-neoplastic pathology is suspected. Between December 2000 and February 2004, we performed 116 stereotactic procedures based on the system of stereotactic planning and Brain-Lab treatment, which was equipped with automatic CT/MR image fusion software. In this report, we have focused on 10 cases of non-neoplastic brain pathologies diagnosed on the basis of ultra-small samples obtained from stereotactic biopsy. Among them there were 4 cases of gliosis, 3 cases of brain degenerative disorders, 2 cases of hippocampal fibroses, and 1 case of normal brain tissue. We have presented all these cases in detail by discussing their histology, clinical manifestations, localisation, management and follow-up.

Adult↗
PubMed2005

Therapeutic targets for prevention and regression of progressive fibrosing renal diseases.

Renal fibrosis complicates most chronic renal diseases, leading to a progressive loss of function and ultimately resulting in terminal renal failure. Molecular mechanisms underlying the development and progression of renal fibrosis have been increasingly identified, and much progress has been made towards a better understanding of the roles of different growth factors/cytokines and regulators of matrix turnover, as well as of the interactions between renal inflammation and fibrosis. This review focuses on recent advances in the identification of novel targets, as well as the development of new therapeutic tools for use in the treatment of progressive fibrosing renal diseases. Using our growing knowledge, treatment strategies need to be identified that prevent progression more effectively, as well as inducing regression of developed renal fibrosis. This is likely by combining compounds that interfere with a variety of targets simultaneously.

Angiotensin-Converting Enzyme Inhibitors↗
PubMedDate not supplied

Management of fibrosing alveolitis.

Fibrosing alveolitis kills half of those who suffer from it within 5 years of diagnosis. Its incidence appears to be increasing and the response to treatment is disappointing. This article explores the reasons why the response to treatment is poor and ways in which new approaches to treatment might be developed.

Age Factors↗
PubMed2005

Frontal fibrosing alopecia.

A 75-year-old woman presented with a 3-year history of progressive loss of her eyebrow hair and with frontal-parietal hairline recession. Multiple biopsy specimens supported a histopathologic diagnosis of lichen planopilaris. With these histolopathologic findings, and the patient's clinical presentation, a diagnosis of frontal fibrosing alopecia was made. Treatment to date with topical glucocorticoid preparations, intralesional triamcinolone injections, and tacrolimus ointment have been unsuccessful.

Aged↗
PubMed1991

[The role of phagocytic cells in the pathogenesis of idiopathic fibrosing alveolitis].

The paper deals with the examination of the role of phagocytes--alveolar macrophages and neutrophils--and peripheral monocytes, in the pathogenesis of idiopathic fibrosing alveolitis (IFA). As the disease aggravates, activation of the absorbing capacity of monocyte-macrophagal cells corresponds to a sharp rise in the level of circulating immune complexes in the blood of IFA patients. Higher activities of elastase and collagenase are observed in the IFA patients' bronchial lavage fluid.

Adult↗
PubMedDate not supplied

[Nephrogenic fibrosing dermopathy].

Nephrogenic fibrosing dermopathy is a rare disease with focal dermal thickening. A case is described of a male of 51 years after two renal transplantations for glomerulonephritis. He had a node in the anterior abdominal wall which was removed and studied. Immunohistochemically, there were positive dendritic cells and positive mononuclears, proliferation of dermal fibroblasts.

Dendritic Cells↗
PubMed2006

Interstitial lung disease in patients with rheumatoid arthritis: comparison with cryptogenic fibrosing alveolitis over 5 years.

OBJECTIVE: There is little information on the natural history of patients with rheumatoid arthritis (RA) and associated interstitial lung disease (ILD). Cryptogenic fibrosing alveolitis (CFA) is known to have a poor longterm prognosis, and we compared the 2 conditions through a longitudinal prospective study. METHODS: We previously compared baseline clinical, physiological, and radiological characteristics in 18 RA-ILD patients with 18 case controls with CFA. Clinical, physiological, and radiological assessment was repeated in all survivors at 5 years, and data on treatment and mortality were collected. RESULTS: The median age in each group was 77 years and 10 patients in each group were male. More patients with RA-ILD survived to 5 years (8 RA-ILD vs 2 CFA; p = 0.03), and median survival was significantly longer for patients with RA-ILD (60 mo) compared to CFA (27 mo; p <or= 0.05). Death was due to respiratory failure in half the patients with CFA, but was more often due to other causes in patients with RA. Clubbing and reduced baseline gas transfer were predictors of poor prognosis, while normal technetium clearance enhanced survival in nonsmokers. CONCLUSION: Patients with RA-ILD did better than those with CFA, and died less often from respiratory failure. Patients with finger clubbing and/or low gas transfer declined more rapidly.

Aged↗
PubMed2006

[Respiratory mechanics in patients with stage III pulmonary sarcoidosis and idiopathic fibrosing alveolitis: similarities and differences].

Impaired function of the lung was studied in 28 patients with Stage 111 pulmonary sarcoidosis and 17 patients with idiopathic fibrosing alveolitis (IFA). The study involved spirography, body plethysmography, and esophageal probing of respiratory mechanics. In patients with Stage III pulmonary sarcoidosis, the mechanics of respiration has the following similarities: restrictive ventilation disorder is revealed in IFA; the functional syndrome with a predominance of restriction is also detectable in some patients with Stage III sarcoidosis; significant differences are found in patients with IFA and Stage III pulmonary sarcoidosis: there is no marked impaired patency of peripheral bronchi in IFA; and all the functional changes found suggest that there is an increase in lung tissue rigidity and a decrease in the capacity of the lung to straighten; in Stage III sarcoidosis, of great importance is a marked impairment of patency of peripherally located bronchi, which is associated with the increase of this or that degree of lung compliance and in the retraction index.

Acute Disease↗
PubMed2006

High resolution computed tomography in fibrosing alveolitis associated with systemic sclerosis.

OBJECTIVE: To investigate the use of high resolution computed tomography (HRCT) in diagnosis of patients with fibrosing alveolitis associated with systemic sclerosis (FA-SSc), and to determine predictors of disease progression. METHODS: We retrospectively studied 90 patients with SSc who had undergone an initial (Time 1) and followup (Time 2) clinical and HRCT evaluation, with a mean +/- SD interval of 5.14 +/- 2.98 years between T1 and T2. RESULTS: At T1, HRCT was normal in 40 patients; at T2, 34/40 (85%) continued to have a normal HRCT. For the 50 patients with FA-SSc on HRCT scan at T1, the overall disease progression comprised extension of lung changes toward the apices with worsening of lung fibrosis at T2. Among the 37 patients who had areas of isolated ground-glass opacities at T1, 25 (68%) had progression of lung fibrosis at T2. These 25 patients were mostly men, who showed a more marked decrease of diffusing capacity and a longer interval between T1 and T2. CONCLUSION: The results emphasize the good longterm prognosis indicated by a normal initial HRCT in SSc. Patients with FA-SSc with abnormal HRCT experienced progressive replacement of ground-glass opacities by honeycombing and/or traction bronchiectasis/bronchiolectasis. Ground-glass opacity is probably the first step of lung fibrosis in SSc, and treatment should be discussed even at this early stage.

Adult↗
PubMed1990

[Fibrosing cholangiolitis after administration of methyltestosterone].

A case of intrahepatic cholestasis of great intensity was observed in a patient taking methyltestosterone. In histological examination of liver biopsy specimen evidence was found of fibrosing intralobular cholangiolitis. The histological findings and the clinical course are discussed considering the disease as an atypical liver reaction to methyltestosterone.

Bile Ducts, Intrahepatic↗