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PubMed1996

Inhibitory effects of the dietary antioxidants butylated hydroxyanisole and butylated hydroxytoluene on bronchioloalveolar cell proliferation during the bleomycin-induced pulmonary fibrosing process in hamsters.

The effects of dietary antioxidants on bleomycin (BLM)-induced pulmonary fibrosis were investigated in Syrian golden hamsters. In addition, the influence on cell proliferative activity in bronchioloalveolar hyperplastic lesions during the lung fibrosing process was evaluated in terms of argyrophil nucleolar organizer regions (AgNORs) and proliferating cell nuclear antigen (PCNA). Male 6-wk-old hamsters were divided into six groups. Groups 1-3 were intratracheally instilled with BLM at a dose of 2.5 U/kg body weight on days 0 and 14, and then given a diet supplemented with 1% butylated hydroxyanisole (BHA), or 1% butylated hydroxytoluene (BHT), or basal diet alone for the following 41 days. Groups 4-6 were given 1% BHA, 1% BHT or basal diet without BLM treatment for the same time period as that in those of groups 1-3. The mortality rate of animals in group 1 (BLM/BHA) (one in 20; 5%) was lower than in those of groups 2 (BLM/BHT) (three in 20; 15%) and 3 (BLM alone) (four in 20; 20%). BHA and BHT treatments significantly inhibited lung weight gains by BLM (P < 0.05). Histopathologically, both BHA and BHT reduced BLM-induced pulmonary histopathological changes such as fibrosis, macrophage aggregation and epithelial proliferation, with a tendency for correlation with accumulation of type III collagen. In addition, antioxidant treatment significantly lowered the mean numbers of AgNORs (P < 0.01) and PCNA-labelling indices (P < 0.05) in the hyperplastic bronchioloalveolar lesions. The results thus indicate that these antioxidants exert inhibitory effects on proliferation of hyperplastic lesions associated with lung fibrosis.

Animals↗
PubMed1995

Fibrosing cholestatic hepatitis and HBV after bone marrow transplantation.

Liver failure caused by reactivation of hepatitis B virus (HBV) is an uncommon complication of bone marrow transplantation. Fibrosing cholestatic hepatitis is a recently described liver lesion that develops in some patients undergoing liver transplantation for chronic HBV infection. The lesion is characterised by peri portal fibrosis, ballooning degeneration of hepatocytes, prominent cholestasis and paucity of inflammation. Recent data suggests it is a cytopathic effect of the pre-core mutant form of HBV with over-expression of viral antigens. Although only one case has so far been described associated with bone marrow transplantation (BMT) it is likely that increasing use of BMT in people with chronic HBV infection will lead to further patients being recognised.

Bone Marrow Transplantation↗
PubMed1994

Atopy as a risk factor for cryptogenic fibrosing alveolitis.

In a case-control study of occupational and environmental causes of cryptogenic fibrosing alveolitis we recently observed a higher prevalence of self-reported symptoms of wheeze, rhinitis or conjunctivitis in cases than in controls. To determine objectively whether this was due to a higher prevalence of atopy amongst cases of CFA, we have measured skin sensitivity to common allergens (D. Pteronyssinus, grass pollen, and cat fur), eosinophil counts and total Immunoglobulin E (IgE) in venous blood in the 22 surviving CFA cases from that study who were willing to provide a blood sample, and in one matched control per case. At least one positive skin test was recorded in 13 cases and six controls, the matched odds of one or more positive test being significantly increased in cases by a ratio of 8.0 (95% confidence interval 1.01-64, P = 0.05). Geometric mean eosinophil counts and IgE levels were also increased amongst cases by factors of 1.6 (95% CI 0.96-2.6) and 1.9 (0.9-3.8) respectively, although these differences were not significant. Differences in skin sensitivity, eosinophil counts, and IgE levels all tended to be more obvious in the 13 case control pairs in which the case was not taking steroids or other immunosuppressive treatment. We conclude that cases of CFA showed increased skin sensitivity to common allergens, and had evidence of increased IgE and eosinophilia in peripheral blood. These findings suggest that atopy may be an important determinant of susceptibility to CFA.

Aged↗
PubMed2006

Unilateral pulmonary edema due to pulmonary venous obstruction from fibrosing mediastinitis.

An unusual case of fibrosing mediastinitis with obstruction of the inferior and superior left pulmonary veins and severe narrowing of the right pulmonary artery, disclosed after unilateral pulmonary edema, is described. The 18-year-old male patient had a long history of cough, progressive dyspnea and recurrent hemoptysis and the possible diagnosis of "interstitial fibrosis" from a previous lung biopsy. The diagnosis and the pulmonary vessels involvement were suspected after right heart catheterization combined with transesophageal echocardiography and confirmed during urgent thoracotomy and at postmortem examination.

Adolescent↗
PubMed2006

Nephrogenic fibrosing dermopathy: a series in a non-Western population.

BACKGROUND: Nephrogenic fibrosing dermopathy (NFD) is a newly proposed clinical entity resembling scleromyxedema. This is mainly found in patients with chronic renal failure who have undergone hemodialysis. Nearly all cases have been reported in the Western population. OBJECTIVE: We sought to assess whether NFD cases occurred in a non-Western population and to ascertain the frequency, clinical characteristics, histopathology, and natural history of the condition in the event of its occurrence. METHODS: Patients postdialysis in the nephrology department of a tertiary referral hospital in Kolkata, India, were evaluated clinically and histopathologic assessment was done on the basis of a clinical scoring to ascertain the frequency of the condition and its characteristics. This report is based on the findings of the first 3 years of an ongoing study. RESULTS: NFD was diagnosed in 6 of 2146 patients postdialysis 0.28%). In transplant recipients undergoing dialysis, NFD was diagnosed in 2 of 43 patients (4.65%). The average number of dialyses undergone by the patients was 7.2 (range: 2-16). Of the 6 cases of NFD, 5 conformed to the diagnosis of chronic kidney disease-V. Among them, 3 were noninsulin-dependent diabetes mellitus and one each were cases of chronic glomerulonephritis and systemic lupus erythematosus. One case followed dialysis in acute tubular necrosis. All cases clinically showed circumscribed thickening, roughened texture, hardening, xerosis, and infiltrated papules, plaques, or both. One case showed rippled pigmentation, four showed varied hyperpigmentation, two demonstrated localized fibrosis, and one resembled papular mucinosis. Increased thickness of dermis, increased number of plump spindle cells, abundance of thick collagen bundles with prominent clefts, and sparse small, multinucleated histiocytes were the hallmark of microscopic findings. LIMITATIONS: The inherent nature of the study precluded the inclusion of NFD cases without any background of dialysis, which have now been reported. No statistical conclusions may be established from our small series. CONCLUSION: To our knowledge, this is the first reported series of NFD outside North America and Europe.

Acute Kidney Injury↗
PubMed2006

Gadolinium deposition in nephrogenic fibrosing dermopathy.

There is growing recognition of the association between the use of gadolinium-containing radiocontrast agents for magnetic resonance imaging and the serious dermal and systemic disease nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis (NFD/NSF). The pathogenesis of this entity remains unclear; however, our recent observations suggest a likely mechanism for the initial dermal manifestations of this gadolinium toxicity.

Acidosis↗
PubMed2004

Hepatic failure due to fibrosing cholestatic hepatitis in a patient with pre-surface mutant hepatitis B virus and mixed connective tissue disease treated with prednisolone and chloroquine.

Fibrosing cholestatic hepatitis (FCH) is a severe variant of hepatitis B infection that has until recently been described almost exclusively in the setting of organ transplantation and HIV infection. This case report describes a patient with pre-surface (pre-S) mutant hepatitis B virus (HBV) infection who developed a fatal form of FCH after high dose prednisolone for mixed connective tissue disease (MCTD). The role of corticosteroids and pre-S viral mutation in the pathogenesis of the disease is discussed, and the importance of early diagnosis is emphasised. This report alerts the physician to the need for close monitoring of LFTs and HBV DNA of hepatitis B carriers during immunosuppressive therapy regardless of the indication. As in the transplantation setting, viral DNA levels should be kept to undetectable if viral replication or recurrence is to be prevented.

Adult↗
PubMed2007

Eosinophilic fibrosing gastritis and toxoplasmosis in a cat.

A 3-year-old, neutered male Tiffany cat was presented to the Animal Health Trust for investigation of pyrexia and a gastric lesion. Radiography and ultrasound showed severe thickening of the gastric wall and regional lymphadenopathy. There was altered gastric wall layering, predominately due to muscularis thickening. Histopathology confirmed eosinophilic fibrosing gastritis. The cat also had evidence of generalised Toxoplasma gondii infection, which may have been responsible for the gastric changes.

Animals↗
PubMed2006

Description of 12 cases of nephrogenic fibrosing dermopathy and review of the literature.

OBJECTIVES: To review the clinical and laboratory features of 12 cases of nephrogenic fibrosing dermopathy (NFD) studied at our institution and of 70 previously described cases in the literature. METHODS: Clinical evaluation and laboratory studies of 12 patients with NFD associated with chronic hemodialysis or peritoneal dialysis for end-stage renal disease and a review of 23 previous publications describing 70 patients with this disease. RESULTS: Eleven patients undergoing chronic hemodialysis and 1 patient undergoing chronic peritoneal dialysis for end-stage renal failure developed a severe and progressive cutaneous fibrotic process with woody induration of legs, thighs, hands, and forearms, and severe loss of motion and flexion contractures in multiple joints. Several patients displayed systemic involvement including fibrosis of muscles, myocardium, and lungs and marked elevations of the erythrocyte sedimentation rate and/or C-reactive protein. Three patients died within 2 years of symptom onset. A review of previously published reports of this disorder confirmed the presence of systemic involvement and a poor prognosis with a high mortality rate. CONCLUSIONS: NFD is a severe and usually progressive systemic fibrotic disease affecting the dermis, subcutaneous fascia, and striated muscles. It also appears that the disease can cause fibrosis of lungs, myocardium, and other organs.

Adult↗
PubMed2005

Nephrogenic fibrosing dermopathy: a case study.

Nephrogenic fibrosing dermopathy (NFD) is a rare disease which has only recently been described histopathologically. In the literature only about 100 cases of this disease have been described. NFD occurs most frequently in patients with renal insufficiency or after kidney transplantation. We present a case study of a patient who showed manifestations of NFD in the abdomen after a second transplantation of a cadaver kidney. The diagnosis was determined by an experienced histopathologist after excision of the focus. The authors discuss the etiology and genesis, which is not yet known, diagnosis of this disease and its treatment.

Collagen↗
PubMed1995

Serial changes of cystic air spaces in fibrosing alveolitis: a CT-pathological study.

In patients with cryptogenic fibrosing alveolitis (CFA), subpleural large cysts are sometimes seen within honeycombing on computed tomography (CT). These cysts may be interpreted as bullae and the coexistence of emphysema with CFA might be assumed. We evaluated whether cystic spaces in honeycombing become larger in patients with CFA on serial CT scans. CT scans were obtained in 16 patients with CFA, who had undergone two CT examinations (6 to 43 months, mean 25.1 months apart), were reviewed focusing on the changes in size of the cystic spaces in honeycombing. The mechanism of enlargement of cystic spaces was investigated using microscopic serial sections of autopsied specimens. In 15 of 16 patients, the cystic spaces in honeycombing were larger on the second examination. Histologically, stenosis of bronchioles and slit-like structures between cysts and bronchioles were detected in addition to alveolar septal dissolution and bronchiolectasis. Both the bronchiolar stenosis and the slit-like gaps have the potential to work as check-valves and increase the volume of cystic spaces. Therefore, in the evaluation of CFA, large cysts should be interpreted with caution to avoid misinterpretation of the cysts as bullae associated with emphysema. From the results of this correlative study, we suggest that a check-valve effect is an important mechanism in the formation of large cysts in honeycomb lung.

Aged↗
PubMedDate not supplied

Vascular chronic-fibrosing pancreatitis of the rat after 5/6-nephrectomy.

The pancreases of 77 uremic rats of a Wistar strain were investigated light-microscopically in groups of 5 animals at 11 different times between one day and ten weeks after 5/6-nephrectomy. The serum concentration of urea reached a maximum mean value of 375 mg% after three days; during the stage of chronic uremia the mean value was found to be 175 mg%. The blood pressure increased in the third week after 5/6-nephrectomy and reached in the course of the experiment values of up to 200 mm Hg and more. Histologically, after an initial acute interstitial pancreatitis with focal dystrophies of the exocrine parenchyma which was virtually cured by the 14th day of the experiment, an increasing proliferative-exudative and granulomatous vasculopathy appeared at the end of the third week. This was followed by a perivascular transmitted chronic-fibrosing pancreatitis which may be regarded as a model of vascularly induced pancreatitis. Early damage by uremia and initial pancreatitis, the rapidly developing hypertension and allergical and autoallergical reactions are discussed as aetiological complex for the vascular alterations. The typical findings of the uremic pancreatopathy, well-known in human pathology, are not reproducible.

Animals↗
PubMed1997

Segmental pericholangial fibrosis: a peculiar benign fibrosing disease at the hepatic hilum.

The authors report on a 9-year-old child who underwent surgery to remove a tumor of the hepatic hilum with preoperative radiographic studies suggestive of malignancy, but whose surgical specimens showed a peculiar fibrosing disease. The lesion was localized to the bifurcation of the hepatic duct, where the bile duct wall and the surrounding tissue was markedly fibrotic. No malignant cells or epithelial destruction were seen. The patient's postoperative course was uneventful, and he is without any sign of recurrence 2 years after surgery. Because the histological features of this case do not correspond to any established disease, including primary sclerosing cholangitis, the authors believe it represents a new entity, segmental pericholangial fibrosis. Local resection resulted in a good outcome. A review of the literature disclosed a few similar cases with a benign clinical course.

Bile Duct Neoplasms↗
PubMed1990

Tension myalgia as a diagnosis at the Mayo Clinic and its relationship to fibrositis, fibromyalgia, and myofascial pain syndrome.

Tension myalgia is a diagnosis that has been in use at the Mayo Clinic for more than 40 years. The term describes a common muscle pain disorder that is conceptually similar to other muscle pain disorders such as fibrositis, fibromyalgia, and myofascial pain syndrome. This article outlines the history of these disorders and proposes "tension myalgia" as a term that unifies these separate diagnoses under one conceptual framework. Because the diagnostic criteria for tension myalgia have been vague, the Department of Physical Medicine and Rehabilitation at the Mayo Clinic has developed specific criteria for generalized, regional, and localized forms of this disorder. The recommended treatment approach includes reassurance, elimination of contributing factors, physical therapy to restore normal neuromuscular function, conditioning, and medications.

Algorithms↗
PubMed1997

The pathology of fibrosing colonopathy of cystic fibrosis: a study of 12 cases and review of the literature.

The authors studied eight colectomy and eight biopsy specimens from 12 patients with cystic fibrosis who had developed fibrosing colonopathy, a complication observed in patients receiving high-strength enzyme replacement. The colectomies originated from five male and three female patients ranging in age from 18 months to 6 years. Five individuals had localized strictures of the right colon and three had stenosing fibrosis of the entire colon. The affected colon had a cobblestone appearance, submucosal fibrosis, thickening of the muscularis propria and chronic mucosal inflammation in all patients, with active cryptitis in four. Moderate to severe infiltration by eosinophils, with increase in the number of mast cells, and widespread interruption of the muscularis mucosa were present in every case. Four colectomies were preceded by endoscopic biopsies; four patients who have not undergone surgery also underwent biopsy. All the biopsies showed evidence of active or chronic inflammation, and all had increased mucosal eosinophils. Prolonged colonic mucosal contact with either the enzymes and/or the enteric coating itself may lead to mucosal colonic ulceration and inflammation. Topical allergy may then promote the stenosing fibroplasia.

Biopsy↗