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PubMed1983

CT of fibrosing mediastinitis: findings and their utility.

The computed tomographic (CT) manifestations of fibrosing mediastinitis were assessed in seven patients with pathologically proven disease. Computed tomography had been done to evaluate further a mediastinal or hilar mass seen on the conventional chest radiograph or to define extent of disease preoperatively. Findings included a mediastinal or hilar mass (7/7), calcifications of the central mass or in associated lymph nodes (6/7), tracheobronchial narrowing (5/7), and pulmonary infiltrates (4/7). In six of the seven patients, CT demonstrated masses or calcifications that were not evident with conventional radiography. The CT findings often were sufficient to suggest or corroborate the diagnosis of fibrosing mediastinitis, and the extent of the disease process was well depicted. In selected patients the CT findings may be sufficient to exclude the need for diagnostic tissue sampling.

Adult↗
PubMed1990

A case of idiopathic fibrosing pancreatitis.

We experienced a case of chronic fibrosing pancreatitis in an 18/12-year-old girl, which was idiopathic because there were no familial back ground, no cystic fibrosis of pancrease, no ductal anomalies and obstruction. The patient presented intermittent colicky abdominal pain and progressive obstructive jaundice, but T-tube drainage and removal of the lymph nodes around the common bile duct relieved her symptoms and disease process. This seems to be the first case reported in a Korean child. Idiopathic fibrosing pancreatitis should be considered in the differential diagnosis of abdominal pain with obstructive jaundice in children.

Abdominal Pain↗
PubMed2000

Fibrosing cholestatic hepatitis: a report of three cases.

Fibrosing cholestatic hepatitis is an aggressive and usually fatal form of viral hepatitis in immunosuppressed patients. We report three cases of fibrosing cholestatic hepatitis in various clinical situations. Case 1 was a 50-year-old man who underwent a liver transplant for hepatitis B virus (HBV)-associated liver cirrhosis. Two and a half years after the transplant, he complained of fever and jaundice, and liver enzymes were slightly elevated. Serum HBsAg was positive. Case 2 was a 30-year-old man in an immunosuppressed state after chemotherapy for acute lymphoblastic leukemia. He was a HBV carrier. Liver enzymes and total bilirubin were markedly elevated. Case 3 was a 50-year-old man who underwent renal transplantation as a known HBV carrier. One year after the transplant, jaundice developed abruptly, but liver enzymes were not significantly elevated. Microscopically lobules were markedly disarrayed, showing ballooning degeneration of hepatocytes, prominent pericellular fibrosis, and marked canalicular or intracytoplasmic cholestasis. Portal inflammation was mild, but interphase activity was definite and cholangiolar proliferation was prominent. Hepatocytes were diffusely positive for HBsAg and HBcAg in various patterns. Patients died of liver failure within 1 to 3 months after liver biopsy in spite of anti-viral treatment.

Adult↗
PubMedDate not supplied

Fibrosing mediastinitis causing rapidly progressive dyspnea, pulmonary edema and death in a 16 yr old male.

Idiopathic fibrosing mediastinitis is a rare entity involving more severely the more compliant structures within the mediastinum. In this report a rare case of simultaneous involvement of both the superior vena cava (SVC) and pulmonary veins is described in a 16--year old male with progressive dyspnea on exertion, cough and a three months' history of blood--tinged sputum. Physical examination and imaging studies revealed signs of pulmonary venous hypertension (PVH) and SVC stenosis. Fibrosing mediastinitis was confirmed by multiple biopsy samples.

Adolescent↗
PubMedDate not supplied

Longitudinal study of peritoneal membrane function in continuous ambulatory peritoneal dialysis: relationship with peritonitis and fibrosing factors.

BACKGROUND: The peritoneal equilibration test (PET) is a useful assessment of peritoneal function in continuous ambulatory peritoneal dialysis (CAPD) patients. However, the natural course of longitudinal change in peritoneal transport is not well defined. PATIENTS: We studied 105 unselected CAPD patients. Average age at enrollment was 50.7 +/- 11.3 years. METHODS: A PET was performed at enrollment. Peritoneal transport was expressed as dialysate-to-plasma creatinine ratio at 4 hours (DIP). Fibrosing factors and mesothelial cell markers, including TGFbeta, epidermal growth factor (EGF), platelet-derived growth factor (PDGF), hyaluronan, and cancer antigen 125 (CA125), were measured in overnight peritoneal dialysate effluent (PDE). Patients were followed for two years. Peritonitis episodes were recorded. Severe peritonitis was defined as an episode that required catheter removal or antibiotic therapy for more than 3 weeks. After two years, 75 patients were still alive and on CAPD. RESULTS: The PET was repeated in 64 patients, of whom 35 were male and 9 had diabetes. The change in D/P over two years was represented as AD/P. No significant change in peritoneal transport was seen after two years (D/P: 0.56 +/- 0.12 vs 0.55 +/- 0.13). A centripetal pattern of change in D/P was observed. The deltaD/P had normal distribution and was inversely correlated with D/P at baseline (r = -0.427, p < 0.005). Both results suggest a regression-to-mean phenomenon. The deltaD/P had no significant correlation with the total number of peritonitis episodes (Spearman r = 0.052, p = 0.74), but after severe peritonitis, affected patients had higher deltaD/P than patients who experienced no severe infection (0.040 +/- 0.136 vs -0.032 +/- 0.120, p < 0.05). For patients with no episodes of severe peritonitis (n = 47), deltaD/P was weakly correlated with baseline TGFbeta level (r = -0.506, p < 0.01). No correlation was seen between the levels of other fibrosing factors and change in peritoneal transport. CONCLUSIONS: Our findings suggest that the centripetal change of peritoneal transport probably reflects a regression-to-mean phenomenon. Peritoneal transport increases after severe peritonitis. The role of TGFbeta levels in PDE with regard to longitudinal change in peritoneal transport requires further study.

Adolescent↗
PubMed2001

Cholestatic fibrosing hepatitis and hepatitis B after bone marrow transplantation.

In the setting of transplantation and chronic hepatitis B viral infection there is a unique histological feature termed cholestatic fibrosing hepatitis. The use of nucleoside analogues in the treatment of this condition has been successful. We describe a case of cholestatic fibrosing hepatitis, which occurred after intense immunosuppression for graft versus host disease in a patient with bone marrow transplantations. She was commenced on lamivudine therapy and showed good clinical, biochemical and virological response. However she succumbed due to sepsis.

Adult↗
PubMed2002

[A case of fibrosing mediastinitis manifesting recurrent nerve palsy].

A 68 year-old man was referred and admitted to our hospital because of hoarseness due to recurrent nerve palsy and an anterior mediastinal tumor detected on chest CT. Chest CT showed a low-density tumor surrounding the aorta and branching arteries. Chest MRI revealed a low-intensity tumor in both T1- and T2-weighted images. The tumor was adjacent to the heart and aorta in the upper anterior mediastinum. A biopsy was performed under minimally invasive video-assisted thoracic surgery (VATS) and a diagnosis of fibrosing mediastinitis was made histopathologically. Oral prednisolone treatment markedly reduced the tumor and improved not only abnormal laboratory data such as C-reactive protein, but also the hoarseness. We report herein that steroid monotherapy was successful in treating a case of fibrosing mediastinitis manifesting recurrent nerve palsy.

Aged↗
PubMed2002

[Clinical and histological features of fibrosing cholestatic hepatitis].

OBJECTIVE: To investigate the clinical and histological characteristics of fibrosing cholestatic hepatitis (FCH) and the therapeutic effect of lamivudine. METHODS: By retrospective analysis, 17 cases developed severe jaundice in 794 renal-transplanted recipients, and of them, FCH was clinically suggested in 11 and confirmed by liver biopsy in 6 cases. RESULTS: The prevalence of chronic HBV infection in renal transplantation patients was 9.3%, of whom the FCH occurred in 22.9%. In 6 liver-biopsied cases, the onset was within 1.5-22 months. Two cases remitted who had early received lamivudine and 4 cases who were treated with the drug before transplantation did not develop the disease. All patients received large amounts of multiple immuno-suppressors after transplantation. About one fifth of HBV-infected cases gradually developed cholestatic hepatitis and some of them rapidly proceeded to hepatic failure. All had very high serum level of HBV DNA. The histology revealed unique lesion combination. The hepatocytes had widespread ballooning change and some ground-glass appearance. There were liver cytolysis and focal cell loss, bile stasis, periportal fibrosis, while only mild lymphocytic infiltration. CONCLUSIONS: Fibrosing cholestatic hepatitis may happen following renal transplantation. Lamivudine has marked therapeutic effect for FCH.

Adult↗
PubMedDate not supplied

Frontal fibrosing alopecia.

Two cases of frontal fibrosing alopecia in post menopausal women, one of them biopsy-proven, are reported. Both women showed a progressive marginal alopecia and none had multifocal areas of involvement typical of lichen planopilaris nor the mucosal or cutaneous lesions of lichen planus. However, scalp biopsy specimens from the frontal hair margin showed perifollicular fibrosis and lymphocytic inflammation. Because the histologic findings are indistinguishable from those seen in lichen planopilaris, frontal fibrosing alopecia is considered a variant of lichen planus pilaris with a particular specific localization and usually onset in the postmenopausal age.

Aged↗
PubMed2003

[Nephrogenic fibrosing dermopathy].

A 58-year-old man with renal insufficiency, who was being treated by haemodialysis, developed progressive skin lesions. He had thickening and hardening of the skin at the extremities and swelling of the toes and fingers with flexion contractures. His face was not affected. Laboratory evaluation was unremarkable and a skin biopsy [table: see text] showed an increase of collagen and mucin, without an inflammatory infiltrate. These clinical features resemble a recently reported new disorder: nephrogenic fibrosing dermopathy. This disorder manifests as scleromyxedema-like cutaneous skin lesions without associated paraproteinemia, occurring in the setting of renal disease. The histopathologic features of nephrogenic fibrosing dermopathy, i.e. thickened collagen and mucin deposition, are unique. The incidence, prevalence and cause of the disease are unknown and there is currently no effective treatment. The Centers for Disease Control and Prevention (CDC) in the USA are calling on physicians who have encountered patients suffering from this type of lesions to contact the CDC for an intended control study.

Fibrosis↗
PubMed2005

[The hemostatic system and correction of its impairments, by using the disaggregatory agent ticlopidine and low-molecular-weight heparin in patients with idiopathic fibrosing alveolitis].

The study was undertaken to examine the mechanisms of changes in the plasma and platelet links of hemostasis in patients with idiopathic fibrosing alveolitis in relation to the course of the disease and the possibilities of correcting the detected disorders with antiaggregatory and anticoagulative agents. Sixty-five patients were examined. All the patients were found to have the signs of hypercoagulation and a drastic change in the functional status of platelets. There was a relationship of the severity of impairments of hemocoagulation and platelet aggregation to the nature of a course of the disease. Addition of the antiaggregatory agent ticlopidine to the standard therapy exerted a normalizing impact on the functional status of platelets, which was more pronounced in the progressive course of the disease. The use of the low molecular-weight heparin fraxiparine had a beneficial effect on both components of the hemostatic system, which was more marked in the progressive course of idiopathic fibrosing alveolitis.

Drug Therapy, Combination↗
PubMed1991

[Idiopathic fibrosing alveolitis and bronchioloalveolar cancer].

A case of bronchiolo-alveolar carcinoma (BAC) developing in the presence of idiopathic fibrosing alveolitis (IFA) in a man of 67 with a long history of pulmonary lesion is described. A honeycomb bung was found at the autopsy. Histologically, multiple foci of BAC of mixed-cell structure and those of mucus-producing cells against the background of fibrosing alveolitis at the stage of honeycomb lung were observed. An increased content of collagen type III, IV and V was found immunohistochemically. The role of sclerotic changes and defected immunological surveillance over the epithelial regeneration in the IFA-affected lung in relevant carcinoma genesis is suggested. The origin of the lung carcinoma in IFA is considered to be the regenerating epithelium of the low respiratory tract.

Adenocarcinoma, Bronchiolo-Alveolar↗
PubMed1991

[Treatment of fibrosing interstitial lung diseases].

In fibrosing pneumonitis, respiratory insufficiency is due to both fibrosis and inflammation induced pulmonary fibrosis. There is no treatment that can suppress fibrosis, so the current treatment of fibrosing pneumonitis--corticosteroid and/or immunosuppressive drugs--aims at reducing pulmonary inflammation and thus at slowing down the development of fibrosis which cannot regress. Therefore, it is necessary to determine the respective parts of inflammation, potentially reversible, and of fibrosis which is not. Most of the time, respiratory insufficiency cannot be prevented and requires long-term oxygen therapy. In a few patients, lung transplantation must be discussed.

Follow-Up Studies↗
PubMed1990

Value of enumerating cellular constituents of bronchoalveolar lavage fluid in differentiating sarcoidosis and cryptogenic fibrosing alveolitis.

Based on the observations of cellular constituents in the BAL fluid in 73 patients with sarcoidosis and 18 patients with cryptogenic fibrosing alveolitis, various diagnostic criteria for differentiating these two disorders were examined. Receiver operator characteristics curve was constructed using different levels of lymphocyte percent (L) in the BAL fluid. Discriminant accuracy was improved if the percent of polymorphs (P) was also taken into account. A log transformation of the ratio L/P + 1 was normally distributed and most useful in differentiating sarcoidosis from cryptogenic fibrosing alveolitis. A formal analysis of results may be helpful in assigning likelihood ratios for the observations on cellular constituents of BAL in patients suspected to have sarcoidosis.

Adult↗
PubMed1990

Fibrosing cholehepatitis in broiler chickens induced by bile duct ligations or inoculation of Clostridium perfringens.

The pathogenesis of fibrosing hepatitis causing condemnations in broiler chickens was investigated. Three to four week old broilers were inoculated via the hepatoenteric bile duct with saline washed suspensions of Clostridium perfringens (10(7) and 10(8) organisms). In another group of broilers, both bile ducts were ligated. The sequential development of liver and gall bladder lesions was studied at intervals ranging from 1-28 days postsurgery. The lesions were similar in both experiments in that the liver became mottled and swollen by five to seven days. Fibrinoid necrosis, heterophil and lymphocyte infiltration, bile duct hyperplasia and fibrosis with reticulin fiber proliferation occurred. By 14-17 days, the liver was enlarged, tan colored and firm with red and white foci. By 28 days, bile duct proliferation and fibrosis were massive with only a few hepatocytes remaining. The liver capsule was not involved. Jaundice was not present but the birds with ligated bile ducts excreted intensely yellow stained droppings after six to seven days. The gall bladder in inoculated birds was edematous and distended with flocculent or inspissated material. Clostridium perfringens was reisolated from gall bladder and/or liver of inoculated birds up to 28 days postsurgery. It is suggested that this organism plays a role in the pathogenesis of fibrosing cholehepatitis by inducing septic intrahepatic cholestasis.

Animals↗
PubMed1988

Cerebrospinal fluid levels of beta-endorphin in patients with fibromyalgia (fibrositis syndrome).

Cerebrospinal fluid (CSF) samples from 18 female patients with fibromyalgia (fibrositis syndrome) were analyzed for beta-endorphin. The mean CSF level of beta-endorphin was 20.7 +/- 0.7 fmol/ml in the patients compared to 20.5 +/- 2.0 fmol/ml (mean +/- SEM) in healthy controls (p greater than 0.05). Thus, patients with fibromyalgia (fibrositis syndrome) seem to have normal CSF levels of the pain modulatory neuropeptide beta-endorphin.

Adult↗
PubMed1989

A comparative study of pain, sleep quality and pain responsiveness in fibrositis and myofascial pain syndrome.

Twenty patients with fibrositis and 19 patients with myofascial pain syndrome were compared with regard to pain levels, sleep quality, general pain threshold and localized pain responsiveness at fibrositic tender points. Patients with fibrositis had significantly lower pain responsiveness (p less than 0.01), lower pain threshold (p less than 0.05) and higher pain levels (p less than 0.05) than patients with myofascial pain syndrome when differences in age between the groups were controlled. No significant difference was found for sleep quality. Regional pain levels influenced local measures of pain sensitivity. A discriminant function, developed on the 4 main study variables, resulted in an almost 80% correct classification to groups.

Adult↗