[Bronchial hyperreactivity and cystic fibrosis].
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Asthma↗
Biomedical subjects
G Bellon
Publications and source records attributed to G Bellon.
[Aberrant left pulmonary artery and tracheal abnormality].
A case of left pulmonary artery sling and tracheobronchial tree malformation is reported in a 3 month-old infant. Acute respiratory presenting symptoms required mechanical ventilatory support. The malformation was suspected because of ventilatory support difficulties. Barium oesophagogram showed an anterior indentation, fiberoptic bronchoscopy and tracheobronchography showed a severe tracheal stenosis from a right bronchus lobaris superior to the carina. This was worsened by the right side compression of trachea end part, due to the abnormal left pulmonary artery as demonstrated by pulmonary angiography. A surgical left artery transposition relieved lateral compression and allowed weaning of ventilatory support. After a 27 month-follow-up, the girl's respiratory status is satisfactory.
Female↗
Activation of human neutrophils by type I collagen. Requirement of two different sequences.
Contact between type I collagen purified from several species and human polymorphonuclear neutrophils (PMNs) triggers the production of O2.- by these cells. The activity of collagen is located in the alpha 1(I)-CB6 cyanogen bromide-cleaved (CB)-peptide, which is the C-terminal CB-peptide of the alpha 1(I) chain. Experiments based on the competitive inhibition of O2.- production by simultaneous incubation of PMNs with type I collagen and synthetic peptides identical to the conserved sequences of this collagen demonstrated that the binding of collagen to PMNs and the subsequent activation of these cells depend on the simultaneous presence of two sequences: Arg-Gly-Asp [residues 915, 916 and 917 of the complete alpha 1(I) chain, located in the helical part] Asp-Gly-Gly-Arg-Tyr-Tyr (residues 1034-1039, located in the C-terminal non-helical telopeptide).
Amino Acid Sequence↗
Human type VI collagen: isolation and characterization of alpha 1 and alpha 2 chains by two-dimensional preparative gel electrophoresis.
Two 140 kDa collagenous glycoproteins were isolated from 5 M guanidinium chloride extracts of human uterine leiomyoma by two-dimensional preparative gel electrophoresis. The glycoproteins represented the major concanavalin A binding fraction of the extract and were also present in adult human skin. On two-dimensional gel electrophoresis the glycoproteins appeared as elongated spots, indicating variations of their isoelectric points from 5 to 6. These glycoproteins were disulfide-bonded components of high molecular mass protein and, after reduction, became sensitive to collagenase treatment that generated peptides corresponding in size to those of the noncollagenous domains of type VI collagen. Antisera raised against these purified glycoproteins reacted with either pepsin-derived alpha 1(VI) or pepsin-derived alpha 2(VI) chains but not with alpha 3(VI) chain of human type VI collagen. Reciprocally, these glycoproteins reacted with monoclonal antibodies against type VI collagen. These results indicate that the glycoproteins represent the integral alpha 1 and alpha 2 chains of type VI collagen. The globular domains of alpha 1(VI) and alpha 2(VI) chains remaining after collagenase treatment appeared on two-dimensional gel electrophoresis as elongated spots, suggesting that the noncollagenous portions determine the well known microheterogeneity of the molecule. The differences in isoelectric points between and within alpha chains may facilitate the formation of microfibrillar network.
Amino Acids↗
[Cystic fibrosis: choices of treatment of respiratory manifestations (except Pseudomonas aeruginosa infections)].
A knowledge of the natural history of cystic fibrosis is the basis for a treatment which, so far, has only been symptomatic. The three fundamental elements of our therapeutic possibilities are: prevention and treatment of bronchial obstruction, administration of antibiotics active against staphylococci and Haemophilus influenza (Pseudomonas aeruginosa infections will be dealt with in another article of this journal), and control of bronchial inflammatory processes. Some complications of cystic fibrosis regarded as serious are no longer beyond our resources. Many works of fundamental research are needed, concerning the local conditions that are necessary to the selective implantation and the development of Staphylococcus aureus in the lung, and the virus Staphylococcus and Staphylococcus-Pseudomonas relations. The real hope of an absolute treatment justifies the new, aggressive therapeutic approach but the precise indications of an antistaphylococcal treatment must still be evaluated carefully. Finally, the complexity of a treatment which must constantly be adjusted is one of the reasons for the existence of "specialized consultations", the organization of which is summarized.
Airway Obstruction↗
Effect of prostaglandin E2 on proline uptake and protein synthesis by cultured human mesangial cells.
PGE2 production by glomeruli is increased in a variety of glomerular diseases. Potentially, this process may affect mesangial cell protein synthesis and mesangial cell growth. Thus studies have been undertaken, using cultured human mesangial cells, to assess the effects of PGE2 on proline uptake, protein synthesis and cell proliferation. In the presence of 140 mM NaCl, incubation of mesangial cells with 0.01 to 1 microM PGE2 for 72 hours resulted in a marked decrease of 14C proline uptake, but did not modify 14C leucine uptake. Substitution of choline to sodium inhibited 14C proline uptake by 85% which became independent of PGE2, indicating that this PG specifically altered sodium-dependent proline uptake. Inhibition of this component reached 35 to 50% with 1 microM PGE2. The inhibitory effect of PGE2 on sodium-dependent proline uptake required a lag time of 48 hours, and was suppressed by ouabain, an inhibitor of Na+, K+ ATPase activity. PGE2 did not modify the Vmax of the transport system (1.007 vs. 1.023 nmol/mg/min) but increased (P less than 0.01) its Km (1.179 vs. 0.823 mM). 8-bromo-cyclic AMP also inhibited sodium-independent proline uptake, and PGE2 markedly increased cyclic AMP production. Taken together, these results suggested that PGE2 acted via cyclic AMP stimulation. PGE2 under identical conditions (1 microM, 72 hr incubation) produced a decrease in collagen synthesis estimated by the relative rate of collagen production after incubation of mesangial cells with 14C proline (percentage of 14C radioactivity in collagenase-sensitive proteins over total proteins). PGE2 also diminished the intracellular free proline pool. More generally, PGE2 inhibited cell proliferation and cell total proteins.(ABSTRACT TRUNCATED AT 250 WORDS)
Cell Division↗
Stimulation of collagen synthesis in fibroblast cultures by a triterpene extracted from Centella asiatica.
The drug "Titrated Extract from Centella asiatica" (TECA), used for its stimulating properties on the healing of wounds, is a mixture of 3 terpenes extracted from a tropical plant: asiatic acid (30%, w/w), madecassic acid (30%, w/w) and asiaticoside (40%, w/w). The effects of TECA and its individual components were checked on human foreskin fibroblast monolayer cultures. TECA increased the collagen synthesis in a dose-dependent fashion whereas a simultaneous decrease in the specific activity of neosynthesized collagen was observed. Asiatic acid was found to be the only component responsible for collagen synthesis stimulation. TECA and all three terpenes increased the intracellular free proline pool. This effect was independent of the stimulation of collagen synthesis.
Cell Count↗
[Antibiotic therapy in cystic fibrosis: uncertainty and reality].
Antibiotics inspite of certain contradictions remain an important part of the treatment of mucoviscidosis (MCV). Despite this the current practice of their extensive use does not always rest on firmly placed criteria. Before colonisation with pseudomonas (PA), the systemic prophylaxis of infection by Staphylococcus (SA), or Hemophilus (HI) is debatable. At the time of exacerbations, even if this may be caused by a virus, the prescription of antibiotics (ATB) active against SA and HI should often be continued for 2 or 3 weeks to maintain a durable clinical benefit. While awaiting the results of complimentary multi-centre trials at present underway it remains uncertain whether eradication of the germ at all costs remains indispensable. Systemic anti-PA antibiotic therapy given quarterly from the time of the implantation of the germ has not yet proved to have definitive advantages when compared to the treatment of exacerbations. An appreciation of the benefit of therapy should take count not only of survival curves but also of the quality of life. The indications for treatment should not neglect the possibility of therapeutic progress, sometimes unexpected. Intensive therapy may be justifiable in certain cases awaiting a lung transplant. ATB is only one part of treatment aimed strictly at the broncho-pulmonary system. Drainage and account of infectious agents other than classical bacterial infections or pathogenic mechanisms other than infections (nutritional problems, bronchial hyper-reactivity, immunological phenomenon, or primary or secondary inflammatory changes) should not be ignored.
Anti-Bacterial Agents↗
[Ultrastructural heterogeneity in primary ciliary dyskinesia syndrome. A case report].
One of the causes of middle lobe syndrome is primary ciliary dyskinesia. This diagnosis was established in an eight-year-old girl by functional studies of the cilia on bronchial and nasal biopsy specimens. The clinical approach to this disease is emphasized and the technical difficulties raised by the diagnosis are discussed.
Biopsy↗
[The preventive treatment of recurring respiratory infections using RU 41740 in 3,008 children].
Biostim is an immunomodulating agent of biologic origin indicated for the prophylactic treatment of recurrent respiratory tract infections in adults and children over one year of age. It has been proved effective by double-blind placebo-controlled trials. This study was undertaken to evaluate the value and tolerance of Biostim in a large number of patients under the conditions of actual use. Three thousand and eight patients were included in the study. At inclusion, 93.7% of patients had a history of recurrent ENT infections (6.27 +/- 3.04 episodes during the previous year). After three months follow-up, results were considered good by the investigator in 75.8% of cases; 75.3% of the children had either no infection or only one infection during the three months follow-up. Results were not influenced by age and/or a history of allergy.
Adjuvants, Immunologic↗
Quantification of collagen synthesis by cultured human glomerular cells.
This study examines the amount of total collagen and its different fractions synthesized by cultured human glomerular epithelial and mesangial cells. Two quantitative techniques were used, namely estimation of proline (Pro) plus hydroxyproline (Hyp) present in the collagenase-sensitive proteins and ELISA or RIA of the different types of collagen. In addition, the pattern of collagen synthesis for both cell types was further examined using immunofluorescence methods and polyacrylamide gel electrophoresis. Glomerular epithelial cells synthesized mainly type IV collagen and it was, for the better part, cell-associated. Mesangial cells synthesized approx. 4-times more collagen than epithelial cells. Type I collagen was predominant, but there were also type IV and III collagens. Secreted and cell-associated collagens were present in roughly equivalent amounts. In both cell lines 10-14% of the newly synthesized collagen had been degraded within the cells. These results provide quantitative data on collagen synthesis by human glomerular cells in vitro and represent the first necessary stage before studying which factors mediate the development of glomerular sclerosis.
Cells, Cultured↗
Cystic fibrosis with and without meconium ileus.
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Cystic Fibrosis↗
Cultures of fibroblasts in fibrin lattices: models for the study of metabolic activities of the cells in physiological conditions.
Two techniques for fibroblast culture in three-dimensional fibrin matrices (fibrin lattices) were used to study the behavior and metabolism of cells in this physiological support. When the fibrin lattices were prepared in silicone-coated glass petri dishes, fibroblasts induced retraction of lattices to an extent dependent on both cell density and serum concentration, the cells stopped dividing, and their protein and collagen syntheses proceeded at a lower rate, like that in collagen lattices. When fibrin lattices were prepared in plastic petri dishes, the matrix attached to the walls, there was no retraction, and the protein synthesis was very active even as regards collagen. The optimal concentration of ascorbic acid in nonretracting fibrin lattices was lower (10 micrograms/ml) than in monolayers (50 micrograms/ml). The comparison of collagen and fibrin lattices showed that the collagenic nature of the lattice was not compulsory for supporting the phenomenon of retraction and that fibroblasts, when exposed to a stress in a fibrin matrix prevented from retracting, secreted far more collagen.
Ascorbic Acid↗
Effects of thyroid-stimulating hormone and phorbol ester on glycosaminoglycan synthesis in porcine thyroid epithelial cells in primary culture.
The effects of thyroid-stimulating hormone (TSH) and a tumor promoter: 12-0-tetradecanoyl-phorbol-13-acetate on glycosaminoglycan (GAG) synthesis were studied in porcine thyroid epithelial cells in primary culture. TSH is known to involve cyclic AMP mechanism and phorbol ester to act by protein kinase C pathway. Chronic treatment of cells with TSH increased the synthesis of heparan sulphate associated with the cell layer and hyaluronic acid in the culture medium. Phorbol ester increased the radioactivity of total GAGs in the culture medium but had no effect on GAGs associated with the cell layer. It inhibited the positive effect of TSH on heparan sulphate synthesis. These results suggest that in thyroid epithelial cells the synthesis of the GAGs associated with the cell layer and those secreted into the culture medium are regulated by different intracellular mechanisms.
Animals↗
[Which treatment for primary tuberculosis?].
Primary tuberculosis should be treated in all cases. In adults, the total treatment course for pulmonary tuberculosis can be shortened to 6 months. If the disease is not symptomatic, the rifampicin-isoniazid combination (10 mg/kg/day each) appears to be preferable to single drug therapy. In patients with symptomatic disease, pyrazinamide (30 mg/kg/day) should be added during the first 6-8 weeks and in some cases, corticosteroid therapy is also required. The drugs are usually well tolerated, but hepatic function should be monitored.
Acquired Immunodeficiency Syndrome↗
[Broncho-alveolar lavage by fibroscopy in immunodepressed children].
Thirty broncho-alveolar lavage (BAL) were performed in order to investigate 30 infectious episodes in immunocompromised children. Twenty patients were previously treated by high-dose chemotherapy and autologous bone marrow transplantation and 6 other patients by conventional methods. A specific etiologic diagnosis was obtained in 16 of 30 episodes (56%), 22 microorganisms were identified by BAL. The most frequently involved microorganism was Candida albicans and the other agents were as follows: 3 cytomegalovirus, 2 Pneumococcus, 2 Pneumocystis carinii, 1 Aspergillosis, 1 syncytial respiratory virus, 1 myxovirus, 1 Pseudomonas aeruginosa, 1 Mycoplasma pneumoniae, 1 Haemophilus influenzae and 1 Escherichia coli. In 5 cases, more than 2 agents were involved. This study emphasizes the diagnostic interest of BAL for infectious diseases of the immunocompromised child. BAL appears to be a non invasive, rapid and reproducible method, and a useful therapeutic approach in the treatment of infectious episodes occurring in grafted children where several microorganisms could be involved at the same time.
Bacterial Infections↗
Quantitative evaluation of collagen fractions separated by acrylamide gel electrophoresis: its application for collagen typing in normal and pathological tissues.
A method for the quantitation of collagen chains or cyanogen bromide peptides separated by sodium dodecyl sulphate polyacrylamide gel electrophoresis is described. After electrophoresis, the bands, slightly stained by Coomassie brilliant blue, are cut and hydrolysed in 6 M hydrochloric acid. Proline and hydroxyproline are measured by a fluorometric procedure after thin-layer separation. When the method is applied to the fractions solubilized by pepsin digestion, it provides a measurement of type I, III, IV and V collagens. When it is applied to cyanogen bromide peptides, its permits the calculation of the proportions of type I and III collagens. Applied to polycystic kidney, this method indicates a significant increase of type I and a limited increase of type IV collagen in this abnormal tissue compared with normal kidney.
Animals↗
A parallel between two techniques of extraction of connective tissue macromolecules.
A method of extraction of the collagen and noncollagen proteins from deep dermis of young adult rabbits using a 0.1 M tartaric acid solution was set up. The tartaric acid extraction, together with the preliminary neutral salt extraction, solubilized 95% of the total collagen and 98% of the noncollagen proteins, far more than the 6 M guanidinium Cl solution used for comparison. Elastin was not extracted. Studies on the fibrillation of the extracted collagen in neutral solution at 25 degrees C or on the results of pepsin digestion in acidic solution at +4 degrees C showed that the tartaric acid-extracted collagen was in a nondenatured form, whereas that extracted by guanidinium Cl was largely denatured. Polyacrylamide gel electrophoresis (PAGE) indicated that most of the collagen was of type I and that many noncollagen proteins were present, mostly in the molecular weight range of 40 kDa. Bidimensional PAGE gave a reproducible pattern of these noncollagen proteins, showing that several additional proteins were present in tartaric acid extracts and not in guanidinium chloride extracts.
Animals↗