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At least 19 recordsLinked to original sources
PubMed1977

Studies on cystic fibrosis using isoelectric focusing. IV. Distinction between ciliary dyskinesia activity in cystic fibrosis and asthmatic sera and association of cystic fibrosis protein with the activity in cystic fibrosis serum.

The cystic fibrosis protein (CFP) and ciliary dyskinesia activities (CDA) in sera from CF homozygotes, heterozygote carriers, and individuals with bronchial asthma have been partially purified. Concurrently, the CDA's in sera from patients with cystic fibrosis (CF) or bronchial asthma were shown to be different substances by ion-exchange or gel permeation chromatographic procedures. Sephadex G-200 chromatography indicated that the CF-CDA eluted with a protein fraction of molecular weight (MW) 68,000-150,000 and that the asthma CDA was found in a protein fraction of MV greater than 150,000. The two activities could also be separated by DEAE-cellulose chromatography. Prior acidification of whole normal, CF homozygote, obligate heterozygote, or asthmatic sera to pH 3.7 using EDTA, followed by fractionation of Sephadex G-200 removed all the CDA's from fractions of highermolecular weight and shifted the activities to a protein fraction of MW 1,100-13,700. This procedure afforded a 200-fold purification of the CDA's in sera from patients with asthma or CF. EDTA treatment, however, also generated a CDA in previously nonreactive normal sera. Subsequent fractionation of the various active G-200 fractions on Bio-Gel P10 allowed for the separation of three separate activities (Bio-Gel Fractions I, II-IV, and V). Fraction I was shown to represent the activity in sera from patients with asthma and was determined to be C3a (MW 9,000). Fraction I was also found in normal, CF, and carrier sera and therefore is not a specific CDA. Fraction II-IV is thought to represent a CF-specific CDA (MW 5,000) since it could not be demonstrated in either normal or asthmatic sera but was found in sera of obligate heterozygotes. Fraction III-IV also did not react with antisera to human C3a. Fraction V was generated from all serum types upon acidification of the serum with EDTA and is thought to be a nonspecific CDA. Bio-Gel P10 filtration of Sephadex G-200 fractions provided 823-fold and 650-fold purification of the asthmatic and CF CDA's, respectively. Concurrent analysis of column fractions for CDA's by bioassay and for CFP by electrofocusing showed CFP only in fractions that contained the CF-CDA. Combined analyses employing acid disc gel electrophoresis, isoelectric focusing, and EDTA treatment of active CF-immunoglobulin (Ig) G and Sephadex G-200 column fractions, followed by Bio-Gel P10 chromatography, provided evidence that CFP was associated with the CF-CDA. It is unknown as yet whether CFP itself is responsible for the CF-CDA activity.

Asthma↗
PubMed1977

Pseudomonas aeruginosa infection in cystic fibrosis. Bactericidal effect of serum from normal individuals and patients with cystic fibrosis on P. aeruginosa strains from patients with cystic fibrosis or other diseases.

P. aeruginosa strains originating from the respiratory tract of patients with cystic fibrosis (CF) and patients with other diseases (non-CF) were analysed with regard to their sensitivity to the bactericidal activity of human serum. P. aeruginosa strains isolated from CF patients were more sensitive than strains from non-CF patients to the bactericidal activity of normal human serum. The bactericidal activity was heatlabile. As regards the sensitivity to normal human serum, mucoid and non-mucoid variants were not found to differ. Strains originating from chronically infected CF patients with many precipitins against these bacteria did not differ with respect to serum sensitivity from strains originating from intermittently colonized CF patients without P. aeruginosa precipitins. Compared with normal sera, CF sera showed similar or higher bactericidal activity against a panel of P. aeruginosa strains. In this respect, any difference between CF sera with precipitins and CF sera without precipitins against P. aeurginosa was not found. Sera from three CF patients chronically infected with P. aeruginosa, and with many precipitins against these bacteria, showed a selective inability in bactericidal activity against the patients' own P. aeruginosa isolate possibly reflecting the presence of "bactericidal blocking" antibodies.

Adolescent↗
PubMed1979

Morphologic-physiologic correlates of the severity of fibrosis and degree of cellularity in idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a progressive disease of lung parenchyma characterized by a chronic inflammatory cellular infiltration and varying degrees of interstitial fibrosis. Current data indicate that the severity of fibrosis and the degree of cellularity determine, in part, the prognosis of IPF and the response to therapy. Whereas lung biopsy gives the best assessement of fibrosis and cellularity, physiologic studies are used to stage and monitor the disease process. To determine which physiologic studies correlate best with severity of fibrosis and degree of cellularity, these parameters were graded in lung biopsies of 23 patients with IPF and compared with a variety of physiologic studies. Although vital capacity, total lung capacity, and diffusing capacity are commonly used as objective monitors of the disease process, none of these parameters correlated with either the severity of fibrosis or the degree of cellularity in biopsy specimens. In contrast, almost all parameters of lung distensibility correlated with the morphologic assessment of degree of fibrosis; compliance had the best correlation. Parameters of distensibility, however, correlated poorly with the degree of cellularity. In comparison, gas exchange during exercise correlated with both morphologic parameters; the exercise-induced changes in arterial oxygen pressure per liter of oxygen consumed had a high correlation with the degree of fibrosis (r = 0.89; P less than 0.001) and correlated to a lesser extent with the degree of cellularity (r = 0.56; P = 0.009). In contrast, neither the resting arterial oxygen tension nor the arterial oxygen tension at maximal exercise correlated with the morphologic assessment of degree of fibrosis or the degree of cellularity. These morphologic-physiologic comparisons suggest that (a) lung volumes and diffusing capacity are poor monitors of both the degree of fibrosis and the degree of cellularity; (b) the fibrotic process contributes, at least in part, to parameters of lung distensibility, and both fibrosis and cellularity contribute to gas exchange alterations during exercise; and (c) parameters of lung distensibility and exercise-induced gas exchange alterations may be useful in staging the severity of disease in IPF.

Adult↗
PubMed1990

Effects of inflammation and fibrosis on pulmonary function in diffuse lung fibrosis.

To investigate the relation between lung function and inflammation and fibrosis in patients with diffuse lung fibrosis, a study was made of untreated patients without appreciable airway obstruction (14 patients with cryptogenic fibrosing alveolitis and seven with pneumoconiosis). Quantitative assessment of inflammatory infiltration and fibrosis was carried out on open lung biopsy specimens and compared with lung volumes, carbon monoxide transfer factor (TLCO), TLCO corrected for alveolar volume (TLCO/VA), and arterial blood gases at rest and during exercise. The degree of fibrosis and the degree of cellular infiltration were positively correlated. Lung volumes and TLCO were correlated with the grades of fibrosis and cellular infiltration of alveoli; arterial blood gases during exercise tended to correlate with both fibrosis and infiltration (p less than 0.06). In contrast, morphological data were not correlated with gas exchange at rest or with TLCO/VA. It is concluded that, in untreated patients with diffuse lung fibrosis, lung volumes, TLCO, and arterial blood gases during exercise reflect the lung lesions, and that the pulmonary function tests used cannot discriminate between fibrosis and infiltration of the lung by inflammatory cells.

Adult↗
PubMed1991

Bleomycin-induced pulmonary fibrosis in genetically mast cell-deficient WBB6F1-W/Wv mice and mechanism of the suppressive effect of tranilast, an antiallergic drug inhibiting mediator release from mast cells, on fibrosis.

It has been well known that the number of mast cells increases during the development of fibrosis in various tissues including the lung. However, the role of mast cells in fibrosis still remains obscure. In the present paper, we evidenced that pulmonary fibrosis could be induced in genetically mast cell-deficient WBB6F1-W/Wv mice as well as WBB6F1-(+/+) mice having mast cells normally by the treatment with bleomycin (BLM, 5 mg/kg, i.v., 10 days), and there was not much difference in the histological changes of lungs between the two strains. An increase in the hydroxyproline content of the lung of WBB6F1-W/Wv mice was rather higher than that of WBB6F1-(+/+) mice. Previously, we reported that tranilast, an antiallergic drug inhibiting chemical mediator release from mast cells, suppressed the development of BLM-induced pulmonary fibrosis in ICR mice, suggesting the possibility that mast cells play certain roles in fibrosis. However, it was evidenced in the present report that tranilast suppressed BLM-induced fibrosis in WBB6F1-W/Wv mice. Tranilast neither suppressed the cytotoxic activity of BLM against KB cells and L-929 cells in vitro, nor inhibited the antitumor activity of BLM against Sarcoma-180 transplanted subcutaneously into ICR mice. Tranilast may act through suppressing BLM-induced activation of lymphoid cells including macrophage and neutrophil. These results indicate an inconsequential role of mast cells in the development of fibrosis. Increases in the number of mast cells and in histamine content of the lung, which were widely reported in the lungs of BLM-treated mice, may be the result of fibrosis.

Analysis of Variance↗
PubMed1989

Plasma and serum lactoferrin levels in cystic fibrosis. Relationship with the presence of cystic fibrosis protein.

Plasma lactoferrin concentrations were measured in blood of cystic fibrosis patients, heterozygotes and controls using a specific and sensitive enzyme immunoassay. 67 plasmas were studied (26 controls, 23 heterozygotes, 18 cystic fibrosis patients) and the results showed a statistically significant increase (p less than 0.05) of the level of plasma lactoferrin in cystic fibrosis patients (265 +/- 224 micrograms/l) compared to controls (168 +/- 100 micrograms/l) and heterozygotes (150 +/- 72 micrograms/l). Since it is well established that plasma lactoferrin level could be influenced by the number of neutrophils, a second set of experiments was performed on 20 cystic fibrosis patients on whom leukocyte counts were also made. When the 15 plasmas with normal neutrophils (in the range 2 to 6 giga/l) were considered, the mean lactoferrin level was 318 +/- 116 micrograms/l, still far above the normal values. For serum, a similar significant increase of lactoferrin concentration was observed in 33 cystic fibrosis patients (610 +/- 551 micrograms/l) compared to the values observed for 25 controls (237 +/- 155 micrograms/l) and 37 heterozygotes (272 +/- 231 micrograms/l). Cystic fibrosis protein (CFP) was identified in the same sera by isoelectric focusing and the intensity of the band was closely related to the increase of lactoferrin concentration in cystic fibrosis patients. In contrast, no difference in serum lactoferrin concentrations was observed between heterozygotes with or without CFP, indicating that the increased CFP concentration cannot be due only to altered granulocyte function.

Blood Proteins↗
PubMed1991

Cystic fibrosis. 4. Abnormalities of airway epithelial function and the implications of the discovery of the cystic fibrosis gene.

Details of ion transporting abnormalities in cystic fibrosis airway epithelium are now known. The central hypothesis, that excessive drying of the airway surfaces is a primary event that leads to all the manifestations of the respiratory insufficiency in cystic fibrosis, is not proved. The hypothesis is, however, consistent with the known transporting abnormalities and is strengthened by the modest clinical improvement produced by a strategy designed to correct the transporting abnormalities. The discovery of the cystic fibrosis gene, together with the presumed structure of the protein product, provides a focal point that must eventually connect the functional abnormalities with the genetic defect. The cellular function of the cystic fibrosis transmembrane regulator must now be the major target in research on cystic fibrosis. Strategies for treatment based on known cellular and molecular abnormalities are beginning to emerge but will be undoubtedly more focused once the responsibility of the cystic fibrosis transmembrane regulator is known.

Biological Transport, Active↗
PubMed1976

Pulmonary fibrosis associated with tracheobronchial aspiration. A study of the frequency of hiatal hernia and gastroesophageal reflux in interstitial pulmonary fibrosis of obscure etiology.

Tracheobronchial aspiration of gastric secretions has been suggested in published reports as a possible cause for idiopathic pulmonary fibrosis. Forty-eight of 131 patients with roentgenographic evidence of pulmonary fibrosis had no established etiologic diagnosis after individualized evaluations. They were prospectively studied by upper gastrointestinal series to determine the incidence of gastroesophageal reflux. The incidence of both hiatal hernia and reflux were statistically higher in the study group than in a group of 270 age-matched controls who had upper gastrointestinal series for the usual indications; (2) a subgroup of 15 patients who had pulmonary fibrosis and serologic evidence which suggested immune-mediated diseases; and (3) a subgroup of 23 patients with pulmonary fibrosis of established etiology. The patients in the study group could be further characterized by clinical and roentgenographic presentations, low maximum-mid-expiratory flow rates, and lung biopsies compatible with interstitial fibrosis. These observations and other cited evidence are supportive of the concept that repeated, small tracheobronchial aspirations of gastric acid secretions over a long period of time may cause interstitial pulmonary fibrosis.

Adult↗
PubMed1979

The pineal and regulation of fibrosis: pinealectomy as a model of primary biliary cirrhosis: roles of melatonin and prostaglandins in fibrosis and regulation of T lymphocytes.

Pinealectomy leads to increased formation of fibrous tissue in the abdominal cavity, increased skin pigmentation and elevated cholesterol and alkaline phosphatase levels. It also leads to reduced formation and/or action of prostaglandin (PG) E1 and thromboxane (TX) A2. PGE1 plays an important role in enhancing function of T suppressor lymphocytes which control overactive antibody-producing B lymphocytes. In primary biliary cirrhosis there are increased skin pigmentation, hepatic fibrosis, elevated cholesterol and alkaline phosphatase levels, defective T lymphocytes and hyperactive B lymphocytes. Primary biliary cirrhosis may be a pineal deficiency disease. Serotonin is important in the pineal and the serotonin antagonist methysergide may cause retroperitoneal fibrosis by interfering with pineal function. There is a good deal of other evidence which suggests that melatonin PGE1 and TXA2 are important in the regulation of fibrosis in other situations such as "collagen" diseases, lithium-induced fibrosis and cardiomyopathies. This suggests that enhancement of formation of PGE1 and TXA2 may be of value in diseases associated with excess fibrosis and defective T suppressor cell function. PGE1 levels may be raised by zinc, penicillin, penicillamine and essential fatty acids. TXA2 levels may be raised by low dose colchicine. These new approaches to treatment may prove safer and more effective than existing ones. They may be of value in disorders such as cardiomyopathy, Hodgkin's disease and other lymphomas, multiple sclerosis, Crohn's disease, atopy and other diseases in which defective T cell function is suspected.

Animals↗
PubMed1975

Studies on the inhibition of experimental liver fibrosis. 2. The mechanism of the inhibition of liver fibrosis of rats due to carbon tetrachloride by elastase.

In the previous experiments, it was demonstrated that high purity elastase extracted from porcine pancreas remarkably inhibits liver fibrosis of rats having chronic liver injury caused by carbon tetrachloride. This time, with the purpose to clarify the mechanism of inhibition of liver fibrosis by elastase, comparative study was made on the activity of lysosomal enzymes by measuring beta-glucuronidase, cathepsin and collagenolytic activity, with the rats administered with elastase and with those untreated, during the period of development of liver fibrosis and the recovery from it. In addition to it, in vitro experiments were made by having elastase act on the substrate comprising mixed collagen of acid soluble and neutral soluble collagens extracted from the skin of guinea pigs and by observing collagen components by disc electrophoresis. With any lysosomal enzymes, no marked difference was noticed between elastase group and non-administered group and thus the possibility of inhibition of liver fibrosis through activation of lysosomal enzyme by elastase was denied. The results of disc electrophoretic observation of the performance of elastase on collagen revealed that beta-component of collagen is disappeared but alpha-component remained. From the above, inhibition of liver fibrosis by elastase may be due to direct affection of elastase to telopeptide portion of collagen.

Animals↗
PubMed1992

Cystic fibrosis mutations in French Canadians: three CFTR mutations are relatively frequent in a Quebec population with an elevated incidence of cystic fibrosis.

The French-Canadian population in the Saguenay-Lac St. Jean region of northeastern Quebec has an elevated frequency of cystic fibrosis (CF). The average incidence of cystic fibrosis was 1 in 891 births and the prevalence of CF carriers was estimated to be 1 in 15. We tested for 10 mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene in 133 French-Canadian CF families from Quebec. Ninety-one families were from the Saguenay-Lac St. Jean region and 42 families were referred from other regions of Quebec. We detected the CFTR mutation in 93 and 92% of the CF chromosomes in the Saguenay-Lac St. Jean and the major-urban Quebec families, respectively. The two groups of French-Canadian families were significantly different for the proportions of CFTR mutations. The three most common mutations in the Saguenay-Lac St. Jean families were delta F508 (58%), 621 + 1G----T (23%), and A455E (8%); and in the major-urban Quebec families were delta F508 (71%), 711 + 1G----T (9%), and 621 + 1G----T (5%). These results provide evidence for the role of founder effect in the elevated incidence of cystic fibrosis in the Saguenay-Lac St. Jean population.

Cystic Fibrosis↗
PubMed1990

[Procollagen-III-peptide in bronchoalveolar lavage fluid as an index of fibrosis in sarcoidosis and idiopathic lung fibrosis?].

In order to answer the question whether in sarcoidosis and idiopathic pulmonary fibrosis there is a relationship between the activity of alveolitis (T4/T8 ratio in sarcoidosis, number of granulocytes in idiopathic pulmonary fibrosis) and the activity of connective tissue formation (type III procollagen peptide in the BAL fluid) BAL was performed in 12 healthy subjects, 33 patients with type II sarcoidosis, and 26 patients with idiopathic pulmonary fibrosis. In the unconcentrated BAL fluid of the healthy subjects, P3P was not measurable. On the basis of the T4/T8 ratio and P3P in type II sarcoidosis, three groups of patients with possibly different risks of progression were found: 1) T4/T8 normal and P3P not or only mildly elevated, 2) T4/T8 elevated and P3P normal or only mildly elevated, 3) T4/T8 elevated and P3P greatly increased. In patients with idiopathic pulmonary fibrosis, the concentration of P3P correlated significantly with the number of granulocytes and the clinical activity parameters. On the basis of these results, we conclude that P3P levels in the BAL fluid, as a direct measure of connective tissue neogenesis, may be a valuable addition to cellular and immunocytological BAL findings.

Bronchoalveolar Lavage Fluid↗
PubMed1992

Dependence of ultrasonic attenuation of liver on pathologic fat and fibrosis: examination with experimental fatty liver and liver fibrosis models.

To clarify the effect of the pathological state of the liver on ultrasonic attenuation, we produced two experimental rabbit models. The influence of fat on ultrasonic attenuation was examined using a fatty liver model without liver fibrosis, and that of fibrosis on attenuation using a liver fibrosis model without fatty infiltration. Ultrasonic data were obtained in vivo directly from the liver, and an acoustic attenuation coefficient slope was obtained by the spectral difference method. Tissue components of the liver, namely the total lipid, hydroxyproline and water contents, were measured precisely by quantitative methods. We revealed that ultrasonic attenuation depends mainly on fatty infiltration of the liver and to a lesser extent on fibrosis, but not on the water content.

Adipose Tissue↗