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PubMed1980

Dendriform pulmonary ossification.

Four cases of dendriform pulmonary ossification are reported from postmortem roentgenography of isolated lungs. All showed pathoanatomical evidence of chronic fibrosing, mostly interstitial, rarely intraalveolar pneumonia, besides the typical manifestations of dendriform pulmonary ossification. Microscopy revealed fluid transition from proliferating, interstitially increased connective tissue to fully developed osseous structures. These dendriform structures can be seen in some analogy to the structural pattern of interstitial, bronchovascular as well as septal connective tissue; however, actual topographical correlation between bone structures and blood or lymph vessels could not be safely determined. Our observations suggest that dendriform pulmonary ossification may be interpreted as a rare complication, or special manifestation, of chronic fibrosing interstitial inflammation of the lung.

Aged↗
PubMedDate not supplied

The role of MRI in the intestinal complications in cystic fibrosis.

Fibrosing colonopathy is a complication recently detected in children with cystic fibrosis (CF), and is thought to be associated with the use of high-strength pancreatic enzymes. The goal of this study was to evaluate the effectiveness of magnetic resonance (MR) in detecting possible pathologic gut findings in patients with CF under pancreatic enzyme treatment. Twenty-five patients with CF and pancreatic insufficiency, all under treatment with high-dose pancreatic enzymes, were studied by MR. MR was performed on a 1.5-T magnet by T1-weighted, 2D-FLASH fat-suppression, breath-hold sequences before and after intravenous administration of gadolinium, and by T2-weighted Turbo Spin-Echo (TSE) and Half-Fourier Acquisition Single-Shot Turbo Spin Echo (HASTE) fat-suppression, breath-hold sequences. A superparamagnetic negative oral contrast agent was given 1.5 h before the examination. MR showed a wall thickening of the terminal ileum and the ascending colon (>4-12 mm) in 22 patients; nine of them (wall thickness >4-6 mm) showed both a moderate hyperintensity of the bowel wall on T2-weighted sequences and an enhancement after intravenous gadolinium on T1-weighted sequences; 13 patients (wall thickness >6-12 mm) showed both a great wall enhancement after intravenous gadolinium and an increased signal intensity of the bowel wall on T2-weighted sequences. Fecal impaction without bowel wall involvement was detected in three patients. MR proved to be a useful, noninvasive, diagnostic tool for the evaluation of patients with CF and fibrosing colonopathy. The signal hyperintensity on T2-weighted sequences and the great wall enhancement after intravenous gadolinium administration indicating an acute edematous condition, provide the clinicians useful information for the therapeutic adjustment.

Adolescent↗
PubMed1989

Pulmonary involvement in systemic sclerosis: the detection of early changes by thin section CT scan, bronchoalveolar lavage and 99mTc-DTPA clearance.

Systemic sclerosis is frequently complicated by fibrosing alveolitis although clinical and radiological abnormalities are not usually apparent until the lung disease is well established. The aim of this study was to investigate pulmonary involvement in systemic sclerosis by thin section CT scan, bronchoalveolar lavage (BAL) and 99mTc-DTPA clearance studies, and assess the value of these tests in defining pulmonary abnormalities in patients with a normal chest radiograph. Patients were divided into those with an abnormal chest radiograph (Group I, n = 14) and those with a normal chest radiograph (Group II, n = 16). CT scans were abnormal in all patients in Group I and 7 of 16 (44%) in Group II. BAL inflammatory cell counts were raised in all 12 (100%) patients studied in Group I and 11 of 15 (73%) in Group II. There was no difference in the type of inflammatory cells observed between the two groups. 99mTc-DTPA clearance was faster than normal controls in ten of 14 patients (71%) in Group I and seven of 15 (47%) in Group II and correlated with carbon monoxide transfer factor (P less than 0.05). Lung biopsies were performed on nine patients in Group I and three in Group II all of whom had abnormal CT scans. Fibrosing alveolitis was confirmed in every case. Group II biopsies could not be distinguished from Group I biopsies; both showed fibrosis as well as inflammation suggesting that pulmonary fibrosis is an early abnormality in systemic sclerosis. Our results indicate that CT scans, BAL and 99mTc-DTPA are frequently abnormal in asymptomatic patients with systemic sclerosis who have normal chest radiographs. When the CT scan is normal abnormalities of BAL and/or 99mTc-DTPA (99mTechnetium diethylenetriamine pentacetate) clearance may indicate lung disease at a still earlier stage. This observation requires further investigation.

Adult↗
PubMed1986

Effect of denervation on primate laryngeal muscles: a morphologic and morphometric study.

Primate intrinsic laryngeal muscles studied consisted of Type I and Type II fibres. Type II fibres were larger than Type I fibres. The coefficient of variance of these muscles suggested that the fibre-size variation was similar in all of these muscles. Denervation resulted in shrinking of Type II fibres at two weeks in all the muscles, while at four weeks the thyroarytenoid showed an inflammatory reaction and far greater reduction in size than the other muscles. At eight weeks, however, all the muscles were fibrosed. We conclude that laryngeal muscles begin to show evidence of denervation at two weeks and are fibrosed in eight weeks. The thyroarytenoid muscle showed inflammatory degeneration, while the posterior cricoarytenoid and cricothyroid muscles showed classical changes of denervation. These findings should have clinical significance in the timing of reinnervation procedures.

Animals↗
PubMed1999

Remodeling of vascular wall in Crohn's disease.

To clarify the changes in vascular resistance in Crohn's disease, we studied changes in the thickness of arterial media histometrically. The resected specimens of mesenterium and intestine from 18 patients with Crohn's disease were used. Each intestinal lesion was classified into three grades: no inflammation, exudative change, and fibrosing change. In the ileum, significant atrophy in the media of the submucosal peripheral arteries was noted during exudative and fibrosing changes, while in the submucosal larger vessels or in the mesenterium, the thickness of the arterial media did not change. In the colon, atrophy in the media of the submucosal arteries was noted in all stages except in the larger vessel during exudative change, while no significant changes were noted in the mesenterium. In conclusion, it is suggested that circulatory disturbance occurs even in the early stage in Crohn's disease, and there seems to be increased vascular resistance in the area between the deep submucosal and the distal mesenteric arteries.

Adult↗
PubMed1991

Mast cell tryptase and histamine concentrations in bronchoalveolar lavage fluid from patients with interstitial lung disease.

1. Mast cell activation in the lung was investigated by measuring concentrations of mast cell tryptase and histamine in the bronchoalveolar lavage fluid from patients with bronchial carcinoma, sarcoidosis, extrinsic allergic alveolitis or cryptogenic fibrosing alveolitis and from normal subjects. 2. Histamine concentrations in bronchoalveolar lavage fluid supernatants were elevated in the bronchial carcinoma and cryptogenic fibrosing alveolitis groups, and were correlated with the histamine content of the cells recovered. 3. An avidin-biotin-enhanced antigen-capture e.l.i.s.a., using polyclonal rabbit antibody specific for tryptase, and mouse monoclonal antibody AA5, allowed the quantification of tryptase in all samples of bronchoalveolar lavage fluid. Tryptase concentrations were increased in the bronchial carcinoma and extrinsic allergic alveolitis groups and in some of the patients with sarcoidosis, and the levels correlated with mast cell numbers and also with concentrations of albumin. 4. There was no significant correlation between levels of tryptase and histamine, suggesting differences in the rates of metabolism or different cellular sources. 5. The tryptase and histamine concentrations measured suggest that there is continuous degranulation of mast cells within the normal lung, but that this process is more pronounced in patients with bronchial carcinoma or interstitial lung disease.

Adult↗
PubMed1998

Translocation 2;11 in a fibroma of tendon sheath.

AIMS: To determine whether fibromas of tendon sheath represent a reactive fibrosing process or whether they are neoplastic, we investigated the chromosomes of the proliferating cells. A clonally occurring karyotypic abnormality would be an argument in favour of these fibromas being neoplastic. METHODS AND RESULTS: A biopsy specimen of a fibroma of tendon sheath was cultured after collagenase exposure leading to dissociation of the tissue. The cultured cells were harvested after 4 days and chromosome analysis done according to standard procedures. Ten out of 20 karyotyped cells showed an identical chromosome abnormality characterized by a t(2;11)(q31-32;q12). The other 10 cells showed a normal 46,XX karyotype. CONCLUSION: The clonally occurring chromosome abnormality found in this fibroma of tendon sheath suggests that this proliferation is neoplastic and not a reactive fibrosing process.

Chromosomes, Human, Pair 11↗
PubMed2000

Pentoxifylline inhibits human peritoneal mesothelial cell growth and collagen synthesis: effects on TGF-beta.

BACKGROUND: Prevention or treatment of peritoneal fibrosing syndrome has become an important issue in patients on continuous ambulatory peritoneal dialysis (CAPD). Recent evidence has suggested that mesothelial stem cell proliferation and matrix over-production predispose the development of peritoneal fibrosis. We investigated whether pentoxifylline (PTX) affects human peritoneal mesothelial cell (HPMC) growth and collagen synthesis. METHODS: HPMC was cultured from human omentum by an enzymic disaggregation method. Cell proliferation was assayed using a methyltetrazolium uptake method. Cell cycle analysis was performed by flow cytometry. Collagen synthesis was measured by 3H-proline incorporation into pepsin-resistant, salt-precipitated collagen. Prostaglandins and cAMP were determined by enzyme immunoassay. Northern blot analysis was used to determine mRNA expression. RESULTS: Our data show that PTX inhibited serum-stimulated HPMC growth and collagen synthesis in a dose-dependent manner. Cell cycle analysis showed that PTX arrested the HPMCs in the G1 phase. PTX decreased the procollagen alpha1 (I) mRNA expression either stimulated by serum or transforming growth factor-beta (TGF-beta). PTX did not alter prostaglandins synthesis but dose-dependently increased intracellular cAMP level. PTX, the same as 3-isobutyl-l-methylxanthine, could potentiate prostaglandin E1 (PGE1) increased cAMP levels of HPMC. The antimitogenic and antifibrogenic effects of PTX on HPMC were reversed by N-[2]-((p-Bromocinnamyl)amino)ethyl]-5-isoquinolinesulfonamide (H-89). Therefore, the mechanism of these effects may be due to the phospodiesterase inhibitory property of PTX. CONCLUSIONS: These data suggest that PTX may have a role in treating peritoneal fibrosing syndrome.

Cell Division↗
PubMed2001

Nuclear factor-kappa B in the liver of patients with chronic hepatitis C: decreased RelA expression is associated with enhanced fibrosis progression.

The mechanisms of liver damage in chronic hepatitis C virus (HCV) infection are poorly understood. The transcription factor, nuclear factor-kappa B (NF-kappa B), regulates the expression of genes involved in apoptosis, inflammation, and antiviral response. It plays a protective role in several forms of liver damage. In this study, we analyzed NF-kappa B by gel mobility shift assay and immunohistochemistry in liver biopsies from HCV-infected patients, and we have determined the hepatic levels of the components of the NF-kappa B system by semiquantitative polymerase chain reaction (PCR). We found that NF-kappa B was activated in the liver of patients with chronic hepatitis C. Neither NF-kappa B activity nor the RNA levels of NF-kappa B subunits showed correlation with liver inflammatory activity, viral load, or HCV genotype. By contrast, hepatic mRNA values of RelA, the main element of active NF-kappa B, correlated inversely with apoptosis (r = -.68; P <.05) and with the rate of fibrosis progression (r = -.51; P <.04). In intermediate/rapid fibrosers, RelA mRNA levels were significantly decreased as compared with slow fibrosers (P <.003) and with normal livers (P <.03). In conclusion, we found that NF-kappa B is activated in chronic HCV-infected livers, and that the expression of RelA is inversely correlated with liver cell apoptosis and with the rate of fibrosis progression. Our data thus suggest that RelA expression may protect against liver fibrosis and hepatocellular damage.

Adult↗
PubMed2002

Cellular vacuolization and apoptosis induced by hepatitis B virus large surface protein.

Fibrosing cholestatic hepatitis (FCH) is a rapidly progressive form of viral hepatitis B that occurs in severely immunosuppressed patients. Pathologically, the liver in FCH is characterized by widespread hepatocyte vacuolization and apoptosis, which, in contrast to more common forms of hepatitis B, is only rarely associated with significant inflammation. Therefore, it has been proposed that, in FCH, hepatocytes may be injured by a direct cytopathic effect of the virus rather than by the host immune response. In support of this hypothesis, we present evidence that cultured hepatoma cells that had been transfected with a plasmid selectively expressing the viral large surface protein form numerous large vacuoles and undergo apoptosis. The similarity of the cytopathology in FCH in vivo and in these transfected cells in vitro strongly implicates the large surface protein as the direct cause of this acute liver disease. This conclusion is further supported by the published demonstration that hepatocytes tend to accumulate large surface protein in FCH, which may reflect its overexpression by the virus. In conclusion, our data implicate the large surface protein as a major cause of hepatocyte injury in fibrosing cholestatic hepatitis.

3T3 Cells↗
PubMed1979

[Cytological diagnosis of thyroiditis (author's transl)].

In 2347 patients fine needle aspiration of the thyroid gland was performed. In 49 cases (2.1%), a final diagnosis of thyroiditis was established. Non-specific granulomatous thyroiditis occurred most frequently (n = 24), followed by lymphocytic hypertrophic thyroiditis Hashimoto (n = 18), focal lymphocytic (n = 5), atrophic lymphocytic (n = 1) and chronic fibrosing thyroiditis (n = 1). Fine needle aspiration biopsy is most suited to diagnose granulomatous thyroiditis. In Hashimoto's thyroiditis cytological investigations are superior to estimation of thyroglobulin antibodies. The cytological method is of little value for the diagnosis of atrophic lymphocytic and chronic fibrosing thyroiditis.

Biopsy, Needle↗
PubMed2006

High-resolution computed tomography of interstitial pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF), also referred to as cryptogenic fibrosing alveolitis (CFA), is the clinical-radiological-pathological syndrome associated with the most common form of fibrosing interstitial pneumonia, usual interstitial pneumonia (UIP). Unfortunately, the diagnosis of IPF carries with it a relatively poor prognosis. The characteristic features on high-resolution computed tomography (HRCT), consisting of reticular abnormality and honeycombing with basal and peripheral predominance, are virtually diagnostic within the correct clinical context. The extent of fibrosis on HRCT is an important prognostic indicator. When ground-glass attenuation is seen in patients with IPF, it commonly progresses to fibrosis and honeycombing. Complications of IPF include accelerated progression and deterioration, lung cancer, spontaneous air leak, and secondary infection. This article discusses the key imaging features as correlated with histopathology, differential diagnosis, complications, and follow-up evaluation of idiopathic pulmonary fibrosis.

Diagnosis, Differential↗
PubMed1996

[Experimental results of excimer-laser-PTA in an experimental ex vivo vascular model].

PURPOSE: To evaluate parameters and characteristics of excimer-laser-angioplasty on ex-vivo vessel models. MATERIAL AND METHODS: 450 tissue ablations were performed on pig aortas, human arteries and fibrin-fixed plaque material. A pulsed 308nm-XeCl-laser delivering 120 ns pulses was tested. Ablations were analysed depending on frequency (10-40 Hz), energy density (30-70 ml/mm2), catheter-material-angle (0-90 degrees) and object-catheter-distance (0-40 mm). RESULTS: Vessel recanalisation speeds of up to 0.5 mm/s were achieved on fibrosed to slightly calcified human vessel models. In perforation tests on normal to slightly fibrosed human vessel walls and vessels of pigs ablation speeds of 0.01-0.5 mm/s were measured. Perforations on calcified vessels were successful in 50% of cases after two minutes application time. Vessel perforations were not detected without the support of mechanical power. At catheter-material angles < or = 30 degrees a minor damage of the intima could be found. There was no evidence of necrosis or coagulation in our studies. CONCLUSION: In-vitro tests of human plaque material yield reproducible vessel recanalisation distances of up to 25 mm at a variable speed from 0.1-0.2 mm/s.

Angioplasty, Laser↗
PubMed1990

[High resolution CT of interstitial lung diseases].

High-resolution CT images were assessed in 39 patients with confirmed interstitial lung disease. 22 patients with fibrosing alveolitis, 12 patients with lymphangitic spread of tumour and 5 patients with lymphangioleiomyomatosis of the lung were included. In fibrosing alveolitis the dominant features on HR-CT were honeycomb cysts with concomitant fibrosis in a predominantly peripheral subpleural distribution. In contrast to this the patients with LAM showed cystic lesions without any predominance--throughout the lung fields and without any fibrotic changes. In lymphangitic spread of tumour, polygonal lines and septal lines as well as nodules were found along the bronchovascular bundles. The patterns seen on HR-CT of the thorax correlate extremely well with the underlying pathology and make it possible to differentiate between the three disease entities by means of characteristic features.

Humans↗
PubMed1988

[Differentiation of cicatricial fibrosis and recurrence of gynecological tumors by MRI].

In a retrospective analysis the relative signal intensities in recurrent tumours of gynaecological origin (n = 16) and in areas of pelvic fibrosis (n = 24) were evaluated. MRI was able to assess biopsy-proven recurrences in 15/16 patients. In one female MRI was false-negative (submucosal tumour spreading), in another case false-positive (reactive lymphnode hyperplasia). Early fibroses (1-12 months following tumour treatment) were of higher signal intensity that late fibroses (time interval since treatment more than 12 months). Differentiation of recurrent disease from post-treatment fibrosis was optimized by long TR (2500 msec), long TE (80-100 msec) spinechopulse sequences.

Biopsy↗
PubMed1990

[Persisting alveolitis after Legionella pneumonia].

Pneumonia was diagnosed radiologically in three patients (43, 54 and 58 years old, respectively), presenting with temperatures between 39 degrees and 40 degrees C, cough and weight loss. These signs persisted for 6, 7 and 13 weeks, respectively, but the pathogens could not be cultivated. Lung function analysis showed partial respiratory insufficiency with extensive restrictive impairment of ventilation. Samples of lung tissue were obtained in all three cases and histology revealed fibrosing alveolitis. In two patients serology yielded antibody titres of 1:512 and in one patient of 1:128, against Legionella pneumophila. Treatment with 1 g erythromycin three times daily was unsuccessful. Therefore, the patients were given prednisone at an initial dosage of 50-100 mg which was subsequently reduced. Lung function normalised during this treatment course, radiological findings and antibody titres receded. Hence, treatment with corticosteroids should be attempted if there is an urgent suspicion of fibrosing alveolitis caused by Legionella pneumophila, after having excluded a florid infectious pneumonia and after failure of erythromycin treatment.

Adult↗
PubMed1989

[Immunocytochemical studies in the diagnosis of pulmonary histiocytosis X].

The diagnostic value of immunocytochemical tests was analysed for 19 cases of pulmonary histiocytosis X (PHX) and eight of other types of fibrosing pulmonary disease (sarcoidosis, 3; exogenous allergic alveolitis, 3; chronic pneumonia, 1; fibrosing alveolitis, 1). The cellular, proliferative-fibroblastic and fibrocystic stages in the course of pulmonary changes were differentiated. PHX cells reacted with anti-S 100 protein in all stages. In three cases for which unfixed tissue was available, all PHX cells reacted with antibody Leu-6, and 35% of these cells also reacted with the proliferation marker Ki-67. The few S-100 positive cells from the eight controls were limited to peribronchial tissue. Thus the antibodies Leu-6 and S-100 are useful aids in the diagnosis of PHX.

Antibodies↗
PubMed2002

Fibre-years, pulmonary asbestos burden and asbestosis.

The relations between cumulative asbestos fibre doses at the work-places and asbestos burden of the lung evaluated by lung dust analyses have been tested on 3 different groups of patients of the German Mesothelioma Register: 1. total collective (n = 366), 2. collective without elevated asbestos burden of the lungs (n = 193), 3. collective with asbestoses/minimal asbestoses (n = 64). The relations between the above mentioned parameters are in general only weak. The limit value of > 25 fibre-years is found in 19.6% of persons without increased pulmonary asbestos burden. In spite of reaching or exceeding the cumulative doses of 25 fibre-years, 24% of the whole collective also show no elevated asbestos-concentrations in their lung tissues. By contrast, 42% of patients with asbestos-associated lung fibroses do not attain 25 fibre-years at their work-places. Considering our data it is doubtful that the postulated limit value of 25 fibre-years can be an adequate parameter for the evaluation of asbestos-associated lung fibroses.

Adult↗