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2.
Proc Natl Acad Sci U S A ; 116(34): 16750-16759, 2019 08 20.
Article in English | MEDLINE | ID: mdl-31350352

ABSTRACT

In October 2017, most European countries reported unique atmospheric detections of aerosol-bound radioruthenium (106Ru). The range of concentrations varied from some tenths of µBq·m-3 to more than 150 mBq·m-3 The widespread detection at such considerable (yet innocuous) levels suggested a considerable release. To compare activity reports of airborne 106Ru with different sampling periods, concentrations were reconstructed based on the most probable plume presence duration at each location. Based on airborne concentration spreading and chemical considerations, it is possible to assume that the release occurred in the Southern Urals region (Russian Federation). The 106Ru age was estimated to be about 2 years. It exhibited highly soluble and less soluble fractions in aqueous media, high radiopurity (lack of concomitant radionuclides), and volatility between 700 and 1,000 °C, thus suggesting a release at an advanced stage in the reprocessing of nuclear fuel. The amount and isotopic characteristics of the radioruthenium release may indicate a context with the production of a large 144Ce source for a neutrino experiment.

4.
J Antimicrob Chemother ; 73(9): 2396-2399, 2018 09 01.
Article in English | MEDLINE | ID: mdl-29757406

ABSTRACT

Background: The spore is the virulence factor identified to be involved in the recurrence of the disease caused by Clostridium difficile. Objectives: To demonstrate that lethal antibiotic concentrations induce the appearance of C. difficile persister-like non-spore cells. Methods: C. difficile and derivative spo0A mutant strains were tested for their susceptibility to antibiotics, as determined using an agar dilution method. Persister-cell generation was determined for all strains using up to 10â€Š× the MIC of every antibiotic for up to 6 days. Results: Using up to 10â€Š× the MIC of every antibiotic, we were able to induce the appearance of persister-like behaviour since biphasic killing curves could be observed in response to treatment antibiotics. Conclusions: To the best of our knowledge, this work provides, for the first time, experimental evidence of the appearance of C. difficile persister-like cells, opening a new research avenue in the pathogenesis of this nosocomial pathogen.


Subject(s)
Anti-Bacterial Agents/pharmacology , Clostridioides difficile/drug effects , Clostridioides difficile/growth & development , Microbial Viability/drug effects , Microbial Sensitivity Tests
5.
J Environ Radioact ; 151 Pt 1: 264-274, 2016 Jan.
Article in English | MEDLINE | ID: mdl-26547362

ABSTRACT

The radon prone areas definition is matter of many researches in radioecology, since radon is considered a leading cause of lung tumours, therefore the authorities ask for support to develop an appropriate sanitary prevention strategy. In this paper, we use geostatistical tools to elaborate a definition accounting for some of the available information about the dwellings. Co-kriging is the proper interpolator used in geostatistics to refine the predictions by using external covariates. In advance, co-kriging is not guaranteed to improve significantly the results obtained by applying the common lognormal kriging. Here, instead, such multivariate approach leads to reduce the cross-validation residual variance to an extent which is deemed as satisfying. Furthermore, with the application of Monte Carlo simulations, the paradigm provides a more conservative radon prone areas definition than the one previously made by lognormal kriging.


Subject(s)
Air Pollutants, Radioactive/analysis , Air Pollution, Indoor/analysis , Radiation Monitoring/methods , Radon/analysis , Housing , Spatial Analysis
6.
J Environ Radioact ; 138: 208-19, 2014 Dec.
Article in English | MEDLINE | ID: mdl-25261867

ABSTRACT

Studying the geographical distribution of indoor radon concentration, using geostatistical interpolation methods, has become common for predicting and estimating the risk to the population. Here we analyse the case of Friuli Venezia Giulia (FVG), the north easternmost region of Italy. Mean value and standard deviation are, respectively, 153 Bq/m(3) and 183 Bq/m(3). The geometric mean value is 100 Bq/m(3). Spatial datasets of indoor radon concentrations are usually affected by clustering and apparent non-stationarity issues, which can eventually yield arguable results. The clustering of the present dataset seems to be non preferential. Therefore the areal estimations are not expected to be affected. Conversely, nothing can be said on the non stationarity issues and its effects. After discussing the correlation of geology with indoor radon concentration It appears they are created by the same geologic features influencing the mean and median values, and can't be eliminated via a map-based approach. To tackle these problems, in this work we deal with multiple definitions of RPA, but only in quaternary areas of FVG, using extensive simulation techniques.


Subject(s)
Air Pollutants, Radioactive/analysis , Air Pollution, Indoor/analysis , Radiation Monitoring/methods , Radon/analysis , Geographic Mapping , Italy , Models, Statistical
7.
Clin Exp Dermatol ; 39(3): 385-90, 2014 Apr.
Article in English | MEDLINE | ID: mdl-24772485

ABSTRACT

The differences in systemic T-cell responses between patients with psoriatic arthritis (PsA) and patients with cutaneous psoriasis (Ps) are still largely unknown. To determine differential features that could be used to distinguish PsA from Ps, we compared the cytokine secretion profile of circulating T cells in patients with PsA, patients with cutaneous Ps and control subjects. We determined Th1, Th2 and Th17 cytokine secretion of anti-CD3-stimulated peripheral blood mononuclear cells (PBMCs) using a cytokine bead array. Normality of data distribution was assessed by the Shapiro-Wilk test, and statistical significance was calculated by the Mann-Whitney test. Phenotypic characterization of circulating T cells was performed by fluorescence-activated cell sorting analysis. We found that the major systemic differences distinguishing PsA from cutaneous Ps were the increased secretion of interleukin (IL)-2 by α-CD3-stimulated PBMCs and a higher percentage of circulating CD3+ T cells expressing the proliferation marker CD71 in PsA. These results indicate IL-2 as a possible biomarker of PsA, and suggest a role of circulating T cells with high proliferative capacity in the pathogenesis of PsA.


Subject(s)
Arthritis, Psoriatic/metabolism , CD3 Complex/immunology , Interleukin-2/metabolism , Leukocytes, Mononuclear/metabolism , Psoriasis/metabolism , Adolescent , Adult , Cytokines/metabolism , Female , Flow Cytometry , Humans , Leukocytes, Mononuclear/immunology , Male , Middle Aged , T-Lymphocytes, Helper-Inducer/metabolism , Young Adult
8.
Minerva Anestesiol ; 79(10): 1132-9, 2013 Oct.
Article in English | MEDLINE | ID: mdl-23698547

ABSTRACT

BACKGROUND: An increase in serum lactate can occur in patients undergoing craniotomy. We hypothesized that prolonged craniotomy for brain tumor resection leads to inadequate tissue perfusion as demonstrated by increased level of lactate. This study attempts to determine the mechanism and identify any modifiable risk factors. METHODS. Prospective, observational study of 18 patients undergoing craniotomy for brain tumor resection. The primary outcome was that peak serum lactate would correlate with length of surgery. Secondary outcomes included lactate at 3, 6 and 9 hours, creatine kinase (CK) and myoglobinuria overtime. These values were correlated with expected risk factors for lactatemia including length of surgery, Body Mass Index (BMI), hypotension, hemoglobin and mannitol therapy. RESULTS. Serum lactate consistently increased in the first 3 hours in all patients (2.21±1.22 mmol/L) with a peak increase at 9 hours (3.73±1.62 mmol/L) (P<0.05 for both). The peak serum lactate did not correlate with length of surgery (P=0.799). However, the change in lactate over 3 hours (Δ3hrLactate) did correlate with BMI (P=0.010). Serum CK was increased at 12 hours (P<0.05) and reached a peak level greater than 1000 U/L in 8 of 18 patients. Six of these patients experienced myoglobinuria. No other parameters correlated with increased lactate. CONCLUSION: We observed a consistent and early increase in serum lactate in patients undergoing craniotomy, which correlated with BMI, but not length of surgery. Associated increases in CK and myoglobinuria support the hypothesis that elevated BMI contributed to muscle ischemia and tissue breakdown during craniotomy. Future studies are required to establish the overall clinical significance and mechanism of hyperlactatemia during neurosurgery.


Subject(s)
Body Mass Index , Craniotomy/adverse effects , Lactic Acid/blood , Adult , Aged , Blood Gas Analysis , Brain Neoplasms/surgery , Female , Humans , Male , Middle Aged , Oximetry , Pilot Projects , Prospective Studies , Risk Factors , Young Adult
9.
G Ital Med Lav Ergon ; 34(3 Suppl): 753-5, 2012.
Article in Italian | MEDLINE | ID: mdl-23405770

ABSTRACT

Our study has examined the valuation of exposure to dust, noise and hand-arm vibrations (HAVs) during "extra-professional" activities of do-it-yourself, like wood smoothing, considering a group of 8 heterogeneous volunteer subjects (not expert of the sector), to find any kind of variability among the subjects. The results have shown a moderate risk for dust exposure and a realer one for HAVs, also higher noise exposure levels when an aspiration system is added to the sander. It's important that the exposure time considered in this study is not comparable to professional time exposure, cause of the "domestic" feature of this activities. Moreover, data could be influenced by different use conditions, grip and material grounds. It's also significant that there are not controls, formation and information of the subjects about the health risks, as well as ipersusceptibility.


Subject(s)
Occupational Exposure , Adult , Aged , Female , Humans , Male , Middle Aged , Risk Factors , Young Adult
10.
J Biomater Sci Polym Ed ; 23(6): 823-32, 2012.
Article in English | MEDLINE | ID: mdl-21396177

ABSTRACT

A collagen membrane with microscopic order is presented. The membranes were produced with acid-soluble collagen, using two different methods to obtain orientation. The product was characterized by mean of UV and IR spectra, scanning electronic microscopy, optical microscopy and laser diffractometry. The results clearly show a high level of order in the membranes obtained by both techniques. Permeability for rifamycin, ascorbic acid and NaCl was also measured. Due to the characteristics of the membranes, they have a potential application for treatment of surface injuries.


Subject(s)
Collagen/chemistry , Collagen/chemical synthesis , Membranes, Artificial , Achilles Tendon , Animals , Ascorbic Acid/chemistry , Bandages , Cattle , Lasers , Materials Testing , Microscopy, Electron, Scanning , Optical Imaging , Permeability , Rifamycins/chemistry , Scattering, Radiation , Sodium Chloride/chemistry , Spectrum Analysis , Temperature
11.
Int J Immunopathol Pharmacol ; 23(3): 965-9, 2010.
Article in English | MEDLINE | ID: mdl-20943071

ABSTRACT

Treatment of patients with psoriasis and/or psoriatic arthritis and concomitant hepatitis C infection remains difficult. Except for cyclosporine, other drugs have proved unacceptable because of hepatotoxicity in patients with HCV. With the advent of anti-TNF-alpha drugs, including etanercept, new therapeutic options have become available. Our study population was five patients with psoriasis and/or psoriatic arthritis and concomitant chronic HCV infection undergoing etanercept therapy. Serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), and viral load were used as markers for liver damage and disease progression, respectively. The Psoriasis Area Severity Index (PASI) was used as a reference parameter for evaluating the therapeutic efficacy of etanercept therapy in improving the clinical skin picture. AST, ALT, viral load and PASI were monitored at 3-month intervals starting from the beginning of therapy up to two years after initiation of etanercept therapy. In four out of five patients, liver enzyme levels and viral load remained substantially unchanged during the course of therapy. In the one remaining patient, viral load and liver enzyme levels increased during etanercept therapy, and then decreased following the initiation of Peg-IFN/ribavirin in combination with anti-TNF-alpha therapy. PASI scores decreased in all five patients. Our data suggest that etanercept therapy is safe and provides an efficacious therapeutic alternative in patients with psoriasis and concomitant HCV infection.


Subject(s)
Hepatitis C/drug therapy , Immunoglobulin G/therapeutic use , Immunosuppressive Agents/therapeutic use , Psoriasis/drug therapy , Receptors, Tumor Necrosis Factor/therapeutic use , Aged , Alanine Transaminase/blood , Antiviral Agents/therapeutic use , Arthritis, Psoriatic/complications , Arthritis, Psoriatic/drug therapy , Aspartate Aminotransferases/blood , Etanercept , Female , Hepatitis C/complications , Hepatitis C/virology , Humans , Male , Middle Aged , Psoriasis/complications , RNA, Viral/blood , Ribavirin/therapeutic use , Tumor Necrosis Factor-alpha/metabolism , Viral Load
12.
Radiat Prot Dosimetry ; 137(3-4): 318-23, 2009 Dec.
Article in English | MEDLINE | ID: mdl-19919960

ABSTRACT

The study of spatial distribution of the indoor radon has assumed in the last years a lot of interest. The geostatistical techniques turn out to be particularly promising. The present work presents the results of a study where around 4000 indoor radon data from Veneto, Friuli Venezia-Giulia and Alto Adige, collected during the sampling campaigns performed in dwellings and in schools, have been analyzed. After the definition of the common data set, the study of the spatial distribution of the phenomenon has been performed by examining the experimental variograms. Declustering techniques have been applied. Predictive maps were defined by using simulation techniques; they allow to determine the probabilities of exceeding defined concentration levels, the 'radon-prone' areas. Systematic results regarding the validation of these maps are reported. This methodological study indicates how it is possible to understand the geographical variability of the phenomenon, trying to find out correlations among indoor radon, geological characteristics (i.e. lithology, morphology, tectonics, soil gas) and building-specific features, which can significantly influence radon concentrations.


Subject(s)
Air Pollution, Indoor/analysis , Air Pollution, Indoor/statistics & numerical data , Air Pollution/analysis , Air Pollution/statistics & numerical data , Data Interpretation, Statistical , Radiation Monitoring/statistics & numerical data , Radon/analysis , Italy , Radiation Dosage
13.
Int J Immunopathol Pharmacol ; 22(1): 243-6, 2009.
Article in English | MEDLINE | ID: mdl-19309573

ABSTRACT

Amicrobial pustulosis associated with autoimmune diseases (APAD) is a clinical entity which was described only recently and few cases are reported in the literature. This condition is characterized by recurrent acute onset with pustular lesions predominantly involving skin folds, genitals, scalp and external auditory canals of young women. The etiopathogenesis of APAD is unknown and the most effective therapeutic treatment seems to be systemic corticosteroids. We describe the case of a 16-year old female patient suffering from APAD successfully treated with cyclosporine A.


Subject(s)
Autoimmune Diseases/drug therapy , Cyclosporine/therapeutic use , Immunosuppressive Agents/therapeutic use , Skin Diseases, Vesiculobullous/drug therapy , Adolescent , Female , Humans
14.
J Radiol Prot ; 28(3): 369-78, 2008 Sep.
Article in English | MEDLINE | ID: mdl-18714133

ABSTRACT

This work deals with the measurement of radioactivity and radon exhalation rate from building materials manufactured in Douala city from geological materials. Nine types of building material were surveyed for their natural radioactivity contents using high-resolution gamma-ray spectrometry. The activity concentrations for (226)Ra, (232)Th and (40)K varied from 11.5 to 49 Bq kg(-1), 16 to 37 Bq kg(-1) and 306 to 774 Bq kg(-1), respectively. The absorbed dose rate in the samples investigated at 1 m above ground level ranged from 28.5 to 66.6 nGy h(-1). External and internal hazard indices were also estimated as defined by the European Commission. The Ra equivalents of the materials studied ranged from 57.5 to 133 Bq kg(-1) and are much smaller than the recommended limit of 370 Bq kg(-1) for construction materials for dwellings. Polycarbonate nuclear track detectors (NTDs), type CR-39, were used for measuring the radon concentration from different materials. In fact, knowledge of the radon exhalation rate from building materials is important for understanding the individual contribution of each material to the total indoor radon exposure. Samples were hermetically closed in glass vessels and the radon growth was followed as a function of time. The radon exhalation rate was therefore derived from the experimental measurement of alpha-track densities. The radon exhalation varied from (5.77 +/- 0.06) x 10(-5) to (7.61 +/- 0.07) x 10(-5) Bq cm(-2) h(-1) in bricks, from (5.79 +/- 0.05) x 10(-5) to (11.6 +/- 0.12) x 10(-5) in tiles, and was (6.95 +/- 0.03) x 10(-5) Bq cm(-2) h(-1) in concrete. A correlation (correlation coefficient approximately 0.8) was found between radium concentration measured with a HPGe detector and the radon exhalation rate obtained using nuclear track detectors.


Subject(s)
Construction Materials , Potassium Radioisotopes/analysis , Radium/analysis , Radon/analysis , Thorium/analysis , Cameroon
15.
J Environ Radioact ; 99(7): 1056-60, 2008 Jul.
Article in English | MEDLINE | ID: mdl-18272267

ABSTRACT

The radioactivity level of soils in a volcanic area in Cameroon was determined and discussed. Thirty soils samples were collected from Buea and Limbé cities located in the south-western Cameroon. These two regions are known for theirs volcanic grounds due to the presence of Mount Cameroon Mountain. The activity concentrations of natural radionuclides as well as that of the fission product were evaluated by gamma-ray spectrometry using a hyper-purity germanium detector (HPGe). The ranges of concentrations in the surveyed soils were 11-17 Bq kg(-1), 22-36 Bq kg(-1) and 43-201 Bq kg(-1) for (226)Ra, (232)Th and (40)K, respectively. The radioisotope (137)Cs was also found but in a very small amount. The outdoor absorbed dose rate 1m above ground with the corresponding annual effective dose rate, assuming a 20% occupancy factor was estimated. The radium equivalent and the external hazard index were also evaluated and results are compared with available data from other studies and with the world average value [United Nations Scientific Committee on the Effects of Atomic Radiation (UNSCEAR), 1988. Sources, Effects and Risks of Ionizing Radiation. Report to the General Assembly on the Effects of Atomic Radiation. United Nations, New York; UNSCEAR, 2000. Sources and Effects of Ionizing Radiations. Report to the General Assembly with Scientific Annexes. United Nations, New York]. A solid state nuclear track detector (SSNTD), LR-115 was used for soil radon measurements at a depth of 50 cm. The ranges of soil radon concentrations were 6.7-10.8 kBq m(-3) and 5.5-8.7 kBq m(-3) in Buea and Limbé, respectively. A positive correlation was found between concentrations of radium measured with gamma-spectrometry and the soil radon concentrations measured with the nitrate cellulose detectors. The results of this study provide the radioactivity level in soil of a volcanic area, which has been found to be within the safety limits. The south-western Cameroon can be considered as having normal natural background radiation.


Subject(s)
Radiation Monitoring/methods , Soil Pollutants, Radioactive/analysis , Volcanic Eruptions/analysis , Cameroon , Geography , Radium/analysis , Radon/analysis
16.
Phys Rev E Stat Nonlin Soft Matter Phys ; 75(2 Pt 1): 021115, 2007 Feb.
Article in English | MEDLINE | ID: mdl-17358321

ABSTRACT

Efficient tools to characterize stochastic processes are discussed. Quantifiers originally proposed within the framework of information theory, like entropy and statistical complexity, are translated into wavelet language, which renders the above quantifiers into tools that exhibit the important "localization" advantages provided by wavelet theory. Two important and popular stochastic processes, fractional Brownian motion and fractional Gaussian noise, are studied using these wavelet-based informational tools. Exact analytical expressions are obtained for the wavelet probability distribution. Finally, numerical simulations are used to validate our analytical results.

17.
Phys Rev E Stat Nonlin Soft Matter Phys ; 76(6 Pt 1): 061114, 2007 Dec.
Article in English | MEDLINE | ID: mdl-18233821

ABSTRACT

By recourse to appropriate information theory quantifiers (normalized Shannon entropy and Martín-Plastino-Rosso intensive statistical complexity measure), we revisit the characterization of Gaussian self-similar stochastic processes from a Bandt-Pompe viewpoint. We show that the ensuing approach exhibits considerable advantages with respect to other treatments. In particular, clear quantifiers gaps are found in the transition between the continuous processes and their associated noises.

18.
J Colloid Interface Sci ; 303(2): 388-403, 2006 Nov 15.
Article in English | MEDLINE | ID: mdl-16987521

ABSTRACT

The role of the interfacial forces was emphasized in interactive processes, involving membrane surface and penetrating molecules. The surface character controlling the dissolution process of some species (CO2, H2O, C3H6O2, C4H8O2, C5H10O2) was evaluated in relation to the supra-molecular chemistry of membranes based on 80PTMO/PA12. Infrared analyses combined with the estimation of the hydrophilic and hydrophobic domains of the membrane surface yielded useful information about the distribution, availability and accessibility of the polar moieties responsible for the penetrant sorption. At the interface, attractive Lewis acid/base interactions such as H-bonding directed the sorption of vapor species into the membranes, whereas quadrupolar CO2 participated in specific Lifshitz-van der Waals interactions with the modified polymers. In both the cases, the presence of additional polar moieties such as carbonyl, sulfonamide, and hydroxyl groups enhanced the affinity of the Pebax-based membranes for the penetrating species considered in this work. As a result, the quantification of the reactivity of a membrane surface for specific molecules may allow predictive models to be constructed and selective membranes to be designed.


Subject(s)
Membranes, Artificial , Polymers/chemistry , 1-Butanol/chemistry , 1-Propanol/chemistry , Citric Acid/analogs & derivatives , Citric Acid/chemistry , Hydrophobic and Hydrophilic Interactions , Solutions/chemistry , Spectroscopy, Fourier Transform Infrared , Sucrose/analogs & derivatives , Sucrose/chemistry , Sulfonamides/chemistry , Surface Properties , Thermodynamics
19.
Acta Physiol (Oxf) ; 187(1-2): 43-9, 2006.
Article in English | MEDLINE | ID: mdl-16734741

ABSTRACT

The many different functional phenotypes described in mammalian cells can only be explained by an intense interaction of the underlying proteins, substantiated by the fact that the number of independently expressed proteins in living cells seems not to exceed 25 K, a number way too small to explain the >250 K different phenotypes on a one-protein-one-function base. Therefore, the study of the interactome of the different proteins is of utmost importance. Here, we describe the present knowledge of the ICln interactome. ICln is a protein, we cloned and whose function was reported to be as divers as (i) ion permeation, (ii) cytoskeletal organization, and (iii) RNA processing. The role of ICln in these different functional modules can be described best as being a 'connector hub' with 'date hub' function.


Subject(s)
Cells/metabolism , Ion Channel Gating , Ion Channels/metabolism , Signal Transduction/physiology , Binding Sites , Cell Membrane/metabolism , Humans , Proteomics , Structure-Activity Relationship
20.
J Mol Med (Berl) ; 84(1): 97-107, 2006 Jan.
Article in English | MEDLINE | ID: mdl-16283140

ABSTRACT

Glutathione (GSH) is one of the most important defense mechanisms against oxidative stress in the respiratory epithelial lining fluid. Considering that GSH secretion in respiratory cells has been postulated to be at least partially electrogenic, and that the mucoregulator S-carbocysteine lysine salt monohydrate (S-CMC-Lys) can cause an activation of epithelial Cl(-) conductance, the purpose of this study was to verify whether S-CMC-Lys is able to stimulate GSH secretion. Experiments have been performed by patch-clamp technique, by high-performance liquid chromatography (HPLC) assay, and by Western blot analysis on cultured lines of human respiratory cells (WI-26VA4 and CFT1-C2). In whole-cell configuration, after cell exposure to 100 microM S-CMC-Lys, a current due to an outward GSH flux was observed, which was inhibitable by 5-nitro-2-(3-phenylpropylamino)-benzoate and glibenclamide. This current was not observed in CFT1-C2 cells, where a functional cystic fibrosis transmembrane conductance regulator (CFTR) is lacking. Inside-out patch-clamp experiments (GSH on the cytoplasm side, Cl(-) on the extracellular side) showed the activity of a channel, which was able to conduct current in both directions: the single channel conductance was 2-4 pS, and the open probability (P(o)) was low and voltage-independent. After preincubation with 100 microM S-CMC-Lys, there was an increase in P(o), in the number of active channels present in each patch, and in the relative permeability to GSH vs Cl(-). Outwardly directed efflux of GSH could also be increased by protein kinase A, adenosine 5'-triphosphate, and cyclic adenosine monophosphate (cAMP) added to the cytoplasmic side (whole-cell configuration). The increased secretion of GSH observed in the presence of S-CMC-Lys or 8-bromoadenosine-3',5'-cyclic monophosphate was also confirmed by HPLC assay of GSH on a confluent monolayer of respiratory cells. Western blot analysis confirmed the presence of CFTR in WI-26VA4 cells. This study suggests that S-CMC-Lys is able to stimulate a channel-mediated GSH secretion by human respiratory cells: electrophysiological and pharmacological characteristics of this channel are similar to those of the CFTR channel.


Subject(s)
Carbocysteine/analogs & derivatives , Glutathione/metabolism , Respiratory Mucosa/drug effects , Respiratory Mucosa/metabolism , Carbocysteine/pharmacology , Cell Line , Chlorides/metabolism , Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Cystic Fibrosis Transmembrane Conductance Regulator/metabolism , Humans , Patch-Clamp Techniques , Respiratory Mucosa/cytology
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