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Pulmonary scintigraphy in fibrosing mediastinitis due to histoplasmosis.

The pulmonary scintigraphic findings from four patients with fibrosing mediastinitis due to histoplasmosis are reported. The ventilation/perfusion (V/Q) mismatch mimicked pulmonary emboli. However, in these cases the chest radiographs and/or gallium-67 scintigraphy were abnormal, suggesting mediastinal or hilar disease. Awareness of the nonembolic conditions that can result in V/Q mismatches is important in the interpretation of lung scans.

Adolescent↗

Evidence of increased histamine levels of lung lavage fluids from patients with cryptogenic fibrosing alveolitis.

In this study we report a significant increase in histamine in lung lavage fluids from a group of 33 patients with lone cryptogenic fibrosing alveolitis (lone CFA), and from a group of 13 patients having CFA in association with other connective tissue disorders, when compared with findings for 13 smoking patient controls without peripheral lung disease (P less than 0.001, P less than 0.05 respectively). The increases were independent of smoking or treatment. Significant correlations were obtained between the raised histamine levels in CFA and increased levels of albumin and increased counts of neutrophils and eosinophils in the lavage fluids, and with more pronounced fibrosis in CFA lung biopsies.. Thus histamine is associated with features of inflammation relating to progressive or more severe disease. No significant increase in histamine was observed in a group of 22 patients with sarcoidosis, although 21 had evidence of disease involving the lung parenchyma. There were, however, significantly higher levels in the patients with X-ray evidence of upper lobe contraction, suggestive of "fibrosis" (P less than 0.025). The levels also showed a correlation with increasing counts of lavage neutrophils (P less than 0.005), a feature also associated with X-ray evidence of contraction in this group. Mast cells were readily identified in biopsies from 12 CFA patients suggesting that these cells may provide one possible source of histamine in CFA lungs. These observations raise the question whether histamine, and/or possibly other substances derived from mast cells, plays any role in amplifying inflammation associated with pulmonary fibrosis.

Adult↗

Tracheal stenosis: an unusual presenting complication of idiopathic fibrosing mediastinitis.

Idiopathic fibrosing mediastinitis is a rare, obliterative, inflammatory process which is characterized by an abnormally exuberant proliferation of fibrous tissue within the superior mediastinum, resulting in the encroachment and occlusion of its contained structures. Usually, this is one of the low-pressure vascular structures, most often the superior vena cava. Airway obstruction as a primary presenting complication of this condition is rare with past treatment results usually being palliative at best. In this article, a patient with this complication is presented in whom emergent surgical treatment and tracheal decortication afforded permanent relief. The clinial presentation, morphology of the disease, and treatment are reviewed.

Adult↗

[Therapy of chronic fibrosing alveolitis (author's transl)].

The treatment with steroids, spironolactone, immunosuppressive drugs and D-Penicillamin is studied in 25 patients suffering from fibrosing alveolitis. A clinical improvement and an improvement of vital-capacity and total-capacity is seen, but no change in atrerial blood gas tensions.

Drug Therapy, Combination↗

The prognostic significance of functional tests in cryptogenic fibrosing alveolitis.

Fifty-six patients with kryptogenic fibrosing alveolitis were followed up during 6.3 years (from 1.5 to 19.3 years) after their first functional examination including cardiac catheterization. Twenty-two out of 56 patients died during the follow-up. The significance of individual functional tests as predictors of the survival was evaluated by means of actuarial survival probability curves. Four variables were predictive for nonsurvival: a mean pulmonary arterial pressure exceeding 30 mmHg, a vital capacity of 60% of the predicted value or lower, a CO diffusing capacity at rest less than 40% of the predicted value, an age at first symptoms over 30 years. Other variables were not significantly predictive for a given time of follow-up. A simple prognostic score derived from predictive variables was able to separate the subgroup of patients with unfavourable short-term prognosis. Among significantly predictive variables, a resting pulmonary arterial pressure over 30 mmHg appears to be the most important.

Adult↗

Long-term development of pulmonary hypertension in cryptogenic fibrosing alveolitis.

Values characterizing the central haemodynamics in patients with cryptogenic fibrosing alveolitis tend to vary considerably in the individual subjects over the years, but the long-term average indicates a trend towards a gradual deterioration. In most patients, the development of pulmonary hypertension is rather slow, amounting to 0.712 kPa (1.3 mmHg) per year on the average. This development is due both to the increase of the transpulmonary driving pressure gradient and to an increase in the pulmonary artery wedge pressure.

Adolescent↗

[Variants and stages in the course of idiopathic fibrosing alveolitis].

Clinicomorphological analysis covered 41 cases of idiopathic fibrosing alveolitis (IFA). The comparison of the degree of respiratory insufficiency, hypoxia, survival of the patients with cytogram of the bronchoalveolar lavage, morphological findings, generation of active oxygen forms by alveolar macrophages and leukocytes has provided evidence on an important role of alveolar macrophage in interstitial alveolitis and fibrosis in IFA. Three variants of chronic IFA are distinguished: aggressive, persistent and slowly progressive. The variants have different prognosis and need individual therapeutic approach.

Adult↗

Fibrosing necrotic nodule of the liver.

A fibrosing necrotic nodule of the liver is described in a 35 year old man suffering from malaria. This non-tumorous lesion is considered to be an entity of diverse pathogenesis.

Adult↗

Idiopathic fibrosing pancreatitis causing obstructive jaundice in young adults: two case reports and literature review.

Chronic pancreatitis is uncommon in children and adolescents. A rare syndrome of idiopathic fibrosing pancreatitis has been reported in 28 patients, ages 4 months to 17 yr. We report two young adults with this syndrome who presented with obstructive jaundice. Both were seen in adult gastroenterology practice, and one is the oldest reported patient at age 20. We review the clinical features, diagnosis, and treatment of this disorder, which merits attention by adult gastroenterologists.

Adolescent↗

Plasma lactate dehydrogenase: a marker of disease activity in cryptogenic fibrosing alveolitis and extrinsic allergic alveolitis?

Total plasma lactate dehydrogenase (LDH) activity may be elevated in cryptogenic fibrosing alveolitis (CFA) and extrinsic allergic alveolitis (EAA), and may be a useful monitor of disease progress. In a retrospective, primary referral centre study, we compared LDH at presentation, prior to bronchoalveolar lavage BAL, and after treatment and follow-up with changes in pulmonary function, in patients with CFA, EAA and pulmonary sarcoidosis. Plasma levels of LDH at presentation in CFA (n = 47) and EAA (n = 10) were significantly higher than in patients with sarcoidosis (n = 36). LDH activity decreased in patients with improving lung function (EAA, p = 0.008; CFA, p = 0.02), whereas it increased in CFA patients with deteriorating lung function (p = 0.015). Total plasma LDH is a simple, though nonspecific test, which appears to reflect changes of disease activity in patients with CFA and EAA.

Adult↗

[Icterus as an initial symptom in a young man with idiopathic fibrosing chronic pancreatitis].

Painless idiopathic fibrosing chronic pancreatitis as a cause of obstructive jaundice in childhood and adolescence is extremely rare. Only four patients have been reported earlier in the paediatric literature. We report a case story of a young man with this disease and with jaundice as the primary manifestation. Our report demonstrates the need to consider chronic pancreatitis as cause of jaundice in young patients.

Adult↗

[Treatment of idiopathic fibrosing alveolitis. Therapeutic experiences with azathioprine-prednisolone and D-penicillamine-prednisolone combination therapy].

Lung function data during three different therapies for idiopathic fibrosing alveolitis are presented. Corticosteroid monotherapyis without effect. A combination of prednisolone and azathioprin improves vital capacity by more than 15% in half of cases. Under this treatment the resting PO2 improves in a third of patients. No improvement in PO2 under exercise is observable in any patient. Combined therapy with D-penicillamine and prednisolone improves vital capacity and PO2 both at rest and under exercise; this therapy thus appears to be superior to the other two. The effectiveness of D-penicillamine-prednisolone therapy can be enhanced by adding azathioprin, as has been found in a pilot study thus far covering 4 patients.

Adult↗

[Fibrosing alveolitis--current aspects of the problem].

Review of the literature and author's data on the etiology, pathogenesis and morphogenesis of fibrosing alveolitis (FA) which is a stereotype manifestation of the majority of lung interstitial diseases is presented. FA is characterized by an acute or chronic, focal or diffuse non-purulent inflammation of the interstitium of respiratory lung areas resulting in interstitial fibrosis. FA early stage is characterized by an exudative-productive inflammation, the late stage -by sclerotic changes resulting in a block of the aero-hematic barrier and development of the respiratory failure and hypoxia. A leading role in FA morphogenesis belongs to the cell cooperation including alveolar macrophages, T-lymphocytes, fibroblasts and not infrequently polynuclear leukocytes. Alveolar macrophage is able not only to participate in the cell defence of lung tissue but to exert damaging sclerogenic effect as well. Nosological features of different interstitial lung diseases are better presented at an early stage of FA and are levelled in lung fibrosis progression.

Acute Disease↗

Idiopathic fibrosing pancreatitis: a rare cause of obstructive jaundice in children.

Idiopathic fibrosing pancreatitis is a rare cause of obstructive jaundice in children and young adults. Only 30 cases have been previously reported. Four children with this entity have been treated at Egleston Children's Hospital at Emory University since 1970. Diagnosis was established by imaging studies and open pancreatic biopsy. Treatment was by bilioenteric bypass, and excellent results have been achieved with follow-up periods up to 25 years.

Adolescent↗

Prolonged survival in fibrosing cholestatic hepatitis with long-term ganciclovir therapy.

A 45-yr-old man underwent liver transplantation for cirrhosis due to hepatitis B and developed recurrent infection. Serial liver biopsies revealed fibrosing cholestatic hepatitis, an entity that is associated with rapid graft failure, and this was treated with long-term intravenous ganciclovir therapy. The patient is alive and well 2 yr after transplantation, despite the presence of well-established cirrhosis and a marked accumulation of intrahepatic hepatitis B surface and core antigens. It is postulated that partial reduction of viral replication resulted in an incomplete syndrome in which rapid graft failure did not occur, but progressive fibrosis developed. Our case suggests that newer nucleoside analogues that provide a greater degree of inhibition to hepatitis B virus replication may greatly improve the outcome of patients with recurrent infection after liver transplantation.

Antiviral Agents↗

[Fibrosing cholestatic hepatitis by B virus reactivation in AIDS].

We report an Hepatitis B Virus reactivation, in a patient with an end-stage Human Immunodeficiency Virus infection who developed a rapidly progressive liver failure in four months. The main histological features include ballooning of hepatocytes and ground glass transformation, without significant inflammation. Immunohistochemical staining showed a high expression of viral antigens. This case can be related to "Fibrosing Cholestatic Hepatitis", first described after liver transplantation for cirrhosis B. The mechanism of hepatocellular damage is likely to be a direct cytotoxicity of hepatitis B virus.

Acquired Immunodeficiency Syndrome↗

Fulminant hepatic failure in a renal transplant recipient with positive hepatitis B surface antigens: a case report of fibrosing cholestatic hepatitis.

This 28-year-old male, a hepatitis B virus (HBV) carrier, received cadaveric renal transplantation and was maintained on cyclosporin A and prednisolone. Jaundice occurred 8 months after the transplantation and he died 2 weeks later due to hepatic failure. The liver histologic findings were compatible with fibrosing cholestatic hepatitis (FCH), which is caused by HBV and has only been reported in liver allografts of orthotopic liver transplantations. This is the first case of FCH developing in a renal transplant recipient. The report illustrates that (1) FCH is also a unique histologic entity in renal transplantations; (2) FCH might occur in a liver chronically infected by HBV without co-existing hepatitis D virus; and (3) FCH can cause fulminant hepatic failure within one year after transplantation while the patient is still in an immunosuppressed state.

Adult↗