AnatomyOrganismsDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentPsychiatry and PsychologyPhenomena and ProcessesDisciplines and OccupationsInformation ScienceHealth Care
Pulmonary FibrosisBleomycinLungFibrosisCystic FibrosisIdiopathic Pulmonary FibrosisHydroxyprolineLung DiseasesBronchoalveolar Lavage FluidSilicosisLung Diseases, InterstitialLung InjuryMyofibroblastsRadiation PneumonitisScleroderma, SystemicFibroblastsMice, Inbred C57BLCollagenPulmonary AlveoliLung NeoplasmsSilicon DioxideTransforming Growth Factor beta1Antibiotics, AntineoplasticDisease Models, AnimalTransforming Growth Factor betaCystic Fibrosis Transmembrane Conductance RegulatorAsbestosisLung TransplantationPneumoniaEndomyocardial FibrosisCarcinoma, Non-Small-Cell LungHermanski-Pudlak SyndromeMice, KnockoutInstillation, DrugMacrophages, AlveolarLiver CirrhosisRespiratory MucosaCollagen Type IPleural DiseasesEpithelial CellsRespiratory Function TestsAcute Lung InjuryRetroperitoneal FibrosisAsbestosCells, CulturedAlveolitis, Extrinsic AllergicPulmonary Surfactant-Associated Protein CRNA, MessengerCytokinesConnective Tissue Growth FactorActinsCathepsin KSignal TransductionPyridonesRadiography, ThoracicImmunohistochemistryAirway RemodelingInterleukin-13Lung Volume MeasurementsGene ExpressionMice, TransgenicReverse Transcriptase Polymerase Chain ReactionInflammationTomography, X-Ray ComputedGene Expression RegulationCell ProliferationSmad3 ProteinInhalation ExposureDisease ProgressionMesocricetusSmad ProteinsRats, Sprague-DawleyApoptosisRadiation Injuries, ExperimentalMice, Inbred BALB CSkinSmad2 ProteinTime FactorsOral Submucous FibrosisExtracellular MatrixSpecific Pathogen-Free OrganismsEpithelial-Mesenchymal TransitionFibronectinsBlotting, WesternIntubation, IntratrachealLiver Cirrhosis, ExperimentalHypertension, PulmonaryPseudomonas InfectionsTotal Lung CapacityUp-RegulationCell MovementOrgan SizeBiological MarkersReceptors, Transforming Growth Factor betaCell LineReal-Time Polymerase Chain ReactionAnti-Inflammatory AgentsDose-Response Relationship, DrugPseudomonas aeruginosaRats, Wistar
Idiopathic pulmonarTransplantationChildren with cystic fibrosisModel of lung fibrosisTissueCure for pulmonary fibrosisDiseasesEpithelial cellsTransplantInfectionsRespiratoryCFTRPatientsBiopsyClinicalVivoDiagnosed with pulmonary fibrosisCystic Fibrosis FDiagnosis of cystic fibrosisTherapy for pulmonary fibrosisDisease with poor prognosis and limitedPulmonary hypertensionDevelopment of fibrosisComplicationsMurine modelInflammation and fibrosisProgressesHumansPirfenidoneDeterioration in lung functionHuman lungOrganProgressionMutationsTreatmentMucusIrreversibleBacterialTherapeuticAlveolar epithelialChestSignificantly2002InflammatoryCauses pulmonaryFunctionAbnormalitiesPathologyUntreatableFibrotic disorders
Idiopathic pulmonar50
Transplantation19
  • Lung transplantation has become an excellent treatment option for patients with cystic fibrosis (CF) and bronchiectasis with very advanced lung disease. (nih.gov)
  • The limiting factor in lung transplantation remains the number of organs available. (nih.gov)
  • This permits not only the ability to measure the function of the lungs, something of great value for lungs from donors with circulatory death (donation after cardiac death), but also the potential to introduce lung repair and convert a nonusable lung to one that can be safely used for transplantation. (nih.gov)
  • Researchers from the University of Washington School of Medicine dissected 10 pairs of lungs from cystic fibrosis patients - obtained after lung transplantation - and examined some 12,000 isolates of Pseudomona aeruginosa inhabiting various lung regions. (genomeweb.com)
  • Tissue fibrosis is the primary cause of long-term graft failure after organ transplantation. (jci.org)
  • Two main challenges in the process of lung transplantation evaluation are to be considered. (medscape.com)
  • The inability to accurately predict an event means that future exacerbations cannot be considered when referring for lung transplantation. (medscape.com)
  • 9 Previous animal and human studies have shown that high TGF-β 1 producers develop significantly more lung fibrosis in response to a number of inflammatory triggers such as radiation, 10 chemotherapy, 11 and lung transplantation. (bmj.com)
  • There are no European suggestions on issues explicitly connected with lung transplantation (LTX) in Cystic Fibrosis (CF). The primary objective of this paper is to furnish CF care colleagues with clinically significant CF-explicit data on all parts of LTX, featuring areas of agreement and contention all through Europe. (alliedacademies.org)
  • Reciprocal lung transplantation has been demonstrated to be a significant helpful choice for end-stage CF pneumonic sickness. (alliedacademies.org)
  • This audit utilizes explicit proof in the field of lung transplantation in CF patients and resolves all issues of pragmatic significance. (alliedacademies.org)
  • The incidence of invasive fungal infections (IFIs) is significantly high in patients following lung transplantation. (pitt.edu)
  • There have been no investigations on the pharmacokinetics of posaconazole in patients with CF. In this study, 7 patients with CF and 13 patients without CF were enrolled in a pharmacokinetic study upon initiation of posaconazole prophylaxis following lung transplantation. (pitt.edu)
  • Pediatric lung transplantation has advanced over the years, providing a potential life-prolonging therapy to patients with cystic fibrosis. (umn.edu)
  • Despite this, many challenges in lung transplantation remain and result in worse outcomes than other solid organ transplants. (umn.edu)
  • As CF lung disease progresses, children and their caregivers are often simultaneously preparing for lung transplantation and end of life. (umn.edu)
  • In this article, we will discuss the current barriers to success in pediatric CF lung transplantation as well as approaches to end of life care in this population. (umn.edu)
  • Pulmonary rehabilitation, lung transplantation, and drugs such as pirfenidone and nintedanib , are treatments. (msdmanuals.com)
  • Lung transplantation may occasionally be an option. (wikipedia.org)
Children with cystic fibrosis4
  • Passive smoking was measured in 57 children with cystic fibrosis and in 51 controls using a questionnaire and a measurement of urinary cotinine concentration. (bmj.com)
  • BACKGROUND: The impact of Aspergillus on lung disease in young children with cystic fibrosis is uncertain. (edu.au)
  • CONCLUSION: In children with cystic fibrosis, positive Aspergillus BAL cultures at age 5 years were associated contemporaneously with air trapping but not bronchiectasis. (edu.au)
  • We report an outbreak of Corynebacterium pseudodiphtheriticum infection in children with cystic fibrosis (CF). To identify 18 C. pseudodiphtheriticum strains isolated from 13 French children with CF, we used molecular methods (partial rpoB gene sequencing) and matrix-assisted laser desorption ionization time-of-flight (MALDI-TOF) mass spectrometry. (cdc.gov)
Model of lung fibrosis3
  • Treatment with Aramchol resulted in statistically significant fibrosis improvement in a validated bleomycin model of lung fibrosis (IPF), comparable to Pirfenidone which is the gold standard treatment. (prnewswire.com)
  • In experiments using lung tissues from patients with IPF, mouselung fibroblasts and a murine model of lung fibrosis, a team led by Zmijewski showed the reversal of lung fibrosis and the underlying cellular mechanisms affected by the drug treatment. (uab.edu)
  • These initial results open an exciting possibility to test the effects of metformin, an AMPK activator, in a model of lung fibrosis in mice. (uab.edu)
Tissue23
  • In pulmonary fibrosis, the normally thin lung tissue is replaced with thick, coarse scar tissue that impairs the flow of oxygen into the blood and leads to a loss of lung function. (innovations-report.com)
  • In human lung tissue TGF-β 1 produced by bronchial epithelial cells stimulates fibroblasts to proliferate in vitro. (bmj.com)
  • Examination of lung tissue in two patients with cystic fibrosis showed strong immunohistological staining of inflammatory tissue using an antibody against this cytokine. (bmj.com)
  • The lung alveolar epithelium represents a major site of tissue injury in IPF and senescence of this cell population is probably detrimental to lung repair. (ersjournals.com)
  • Here we demonstrate that lung epithelial cells exhibit increased P16 and P21 expression as well as senescence-associated β-galactosidase activity in experimental and human lung fibrosis tissue and primary cells. (ersjournals.com)
  • Nintedanib, a kinase inhibitor that blocks multiple pathways involved in scarring of lung tissue, is not recommended in people with moderate to severe liver problems nor in pregnant women. (the-rheumatologist.org)
  • Pirfenidone, which works on multiple pathways involved in scarring of lung tissue, is not recommended for people with severe liver problems or end-stage kidney disease, or those who require dialysis. (the-rheumatologist.org)
  • Dr. Blackwell also spoke about a possible overlap in treatments for IPF and connective tissue lung disease and idiopathic interstitial lung diseases based on lessons learned from familial interstitial pneumonia (FIP), a disease course that is similar to IPF. (the-rheumatologist.org)
  • In this two-part explorative proteomic study, we demonstrate how proteins associated with tissue remodeling, inflammation and chemotaxis such as MMP7, CXCL13 and CCL19 are released in response to aberrant extracellular matrix (ECM) in IPF lung. (lu.se)
  • We used a novel ex vivo model where decellularized lung tissue from IPF patients and healthy donors were repopulated with healthy fibroblasts to monitor locally released mediators. (lu.se)
  • In a mouse model of lung injury, KIN001 significantly reduced lung weights and tissue fibrosis score vs. controls. (swissbiotech.org)
  • In order to understand the mechanisms of action of KIN001, global gene expression changes in lung tissue were measured by the RNAseq method. (swissbiotech.org)
  • Pulmonary fibrosis is characterized by the permanent scarring of lung tissue. (newswire.com)
  • The diagnostic capabilities of CT scans in detecting subtle structural changes in the lung tissue and bronchial tree are underlined, which allows to diagnose light and atypical forms of CF in adults, determinate the stage and activity of the pathological process and the effectiveness of the treatment. (dntb.gov.ua)
  • Pulmonary fibrosis is a serious lung disease that causes the lung tissue to become scarred and stiff. (chestnet.org)
  • IPF is a type of interstitial lung disease (ILD), which means that it affects the interstitium, or the tissue around the air sacs of the lung. (chestnet.org)
  • Your provider may also take a biopsy of your lung tissue-that is, a small amount of tissue that is removed to look at under a microscope. (chestnet.org)
  • An open lung biopsy is surgery to remove a small piece of tissue from the lung. (medlineplus.gov)
  • Tissue is taken from the lung and sent to a laboratory for examination. (medlineplus.gov)
  • The lungs and lung tissue will be normal. (medlineplus.gov)
  • OGG1 and SMAD7 interact to induce fibroblast proliferation and differentiation and display roles in fibrotic murine and IPF patient lung tissue. (lu.se)
  • Such scar tissue causes an irreversible decrease in oxygen diffusion capacity, and the resulting stiffness or decreased compliance makes pulmonary fibrosis a restrictive lung disease. (wikipedia.org)
  • Common genes implicated in fibrosis are Transforming Growth Factor-Beta (TGF-β), Connective Tissue Growth Factor (CTGF), Epidermal Growth Factor Receptor (EGFR), Interleukin-13 (IL-13), Platelet-Derived Growth Factor (PDGF), Wnt/β-catenin signaling pathway. (wikipedia.org)
Cure for pulmonary fibrosis1
Diseases14
Epithelial cells2
Transplant12
  • By the time he was 11, physical exertion left him struggling for breath due to the severity of his lung infection and his condition had deteriorated to the point that his doctors referred him for heart/lung transplant assessment. (selfgrowth.com)
  • In an in vivo mouse orthotopic lung transplant model of BOS, antagonism of the LPA receptor (LPA1) or ATX inhibition decreased allograft fibrosis and was associated with lower active β-catenin and dephosphorylated NFAT1 expression. (jci.org)
  • Since there is no cure to this disease, a lung transplant is the only solution to keep him alive. (gofundme.com)
  • The PFF Lung Transplant Community Support Group is intended for those who have had, or are awaiting lung transplant due to pulmonary fibrosis. (pulmonaryfibrosis.org)
  • The relevance of this genetic diagnosis for lung transplant management has not been examined. (ersjournals.com)
  • We gathered an international series of telomerase mutation carriers who underwent lung transplant in the USA, Australia and Sweden. (ersjournals.com)
  • While these findings need to be expanded to other cohorts, caution should be exercised when approaching the transplant evaluation and management of this subset of pulmonary fibrosis patients. (ersjournals.com)
  • The significance of the genetic diagnosis of telomerase-mediated pulmonary fibrosis to lung transplant management has not been examined. (ersjournals.com)
  • Counselling can also help patients and carers to cope with the prospect of a lung transplant. (ilfa.ie)
  • Twelve years post-double lung transplant, Donna DiCosta is beating the odds in a big way! (jerrycahill.com)
  • It's easier to just sit and let life take its course until you end up on the transplant list, get new lungs, and are able to run marathons. (cffatboy.com)
  • How do you feel about the "your lungs vs new lungs" decision or working as hard as you can now so working hard after transplant isn't so rough? (cffatboy.com)
Infections12
  • DOI 10.1258/jrsm.2008.s18012)we see some very strong evidence indicating that colloidal silver may turn out to be a successful treatment for the debilitating chronic lung infections associated with Cystic Fibrosis (CF). (selfgrowth.com)
  • Because doctors had documented serious infections in the boy's lungs, and because aggressive IV antibiotic therapy administered by the boy's doctors had proven to be futile, his parents began to look into alternative remedies for the infection. (selfgrowth.com)
  • The study was published in The American Journal of Respiratory and Critical Care Medicine on February 21, 2017, " Restoring CFTR Function Reduces Airway Bacteria and Inflammation in People With Cystic Fibrosis and Chronic Lung Infections . (cysticfibrosis.com)
  • New findings presented at the North American Cystic Fibrosis conference in Orlando, US, showed that the treatment reduced the rate of lung decline by 42% and caused a 35% drop in the number of serious infections. (huffingtonpost.co.uk)
  • The condition clogs the lungs up with thick, sticky mucus, leading to repeated infections and ultimately shortening life. (huffingtonpost.co.uk)
  • I was mentally and physically exhausted from years of training, and I was repeatedly let down due to debilitating lung infections. (cff.org)
  • Thus, infections of the lung in patients with CF are now considered as polymicrobial infections. (cdc.gov)
  • The first difficulty in studying infections in the lungs of patients with CF is that many bacteria present in the lung cannot be isolated from sputum samples either because of their fastidious growth requirements or because of the presence of other more common CF-related pathogens, including P. aeruginosa , S. aureus , H. influenzae, and Branhamella catarrhalis , that might ordinarily overgrow other bacteria in culture. (cdc.gov)
  • This collection of sticky mucus results in life-threatening lung infections and serious digestion problems. (masslung.com)
  • Examples include autoimmune disorders, viral infections and bacterial infection like tuberculosis which may cause fibrotic changes in both lung's upper or lower lobes and other microscopic injuries to the lung. (wikipedia.org)
  • currently, largely reflected the prevalence of tuberculosis, pneumonia, lower respiratory tract infections and opportunist lung infections in those with HIV. (who.int)
  • The common reasons for prolonged hospitalization were mandatory isolation 28% (n = 62), hospital-acquired infections 17% (n = 37), acute respiratory distress syndrome 15% (n = 32), myopathy/neuropathy 14% (n = 31), pulmonary fibrosis requiring oxygen supplementation 14% (n = 31), and completion of COVID-19 treatment 12% (n = 25). (who.int)
Respiratory6
  • Despite advances in the medical management of cystic fibrosis over the last three decades, there are still a number of unexplained differences in its clinical course, especially in the rate of development of respiratory failure of patients who appear to have the same CFTR genotype, degree of bacterial colonisation, and compliance with medication. (bmj.com)
  • Cystic fibrosis (CF) is an autosomal recessive disease characterized by defective ion channels, resulting in multiorgan dysfunction, most notably affecting the respiratory tract. (cdc.gov)
  • We provide a team approach to care that includes medical, nurse, registered dietician, physical therapy, social worker, and respiratory therapy cystic fibrosis specialists. (masslung.com)
  • Full range of treatments for all complications related to cystic fibrosis, including those involving the respiratory, sinus, digestive, endocrine, and reproductive systems. (masslung.com)
  • It is critical to obtain a complete history, including medication history, drug use, social history, occupational, recreational, and environmental respiratory exposure history, risk factors for human immunodeficiency virus infection, and review of systems, to ensure other causes of interstitial lung disease are excluded. (medscape.com)
  • Pulmonary fibrosis is a well-known consequence of acute respiratory distress syndrome and affects the life of survivors (5). (who.int)
CFTR9
  • Ivacaftor has been shown to increase CFTR activity and lung function in people with Cystic Fibrosis and the G551D mutation, but until a recent study, scientists were not sure if ivacaftor changed the level of bacteria in the lungs. (cysticfibrosis.com)
  • METHODS Polymorphisms in the TGF-β 1 gene defining amino acids of codons 10 and 25 were determined by ARMS-PCR using DNA stored on 171 Caucasian patients who were homozygous for the ΔF508 mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. (bmj.com)
  • A malfunctioning CFTR channel fails to deliver enough chloride ions to the lungs. (rtmagazine.com)
  • The diagnosis of cystic fibrosis (CF) is based on the occurrence of two mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and on assays that measure the basic defect of abnormal chloride transport in the affected organs. (eur.nl)
  • Genetic analysis showed that the patient was a symptomatic heterozygote, which suggests that one lesion in the CFTR gene may be sufficient to cause CF-like lung disease. (eur.nl)
  • We recently demonstrated that triple-combination CFTR (cystic fibrosis transmembrane conductance regulator) modulator therapy with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) improves CFTR function in airway and intestinal epithelia to 40-50% of normal in patients with cystic fibrosis (CF) with one or two F508del alleles. (asthmafoundation.org.nz)
  • Our data demonstrate that improvement of CFTR function by ELX/TEZ/IVA improves lung ventilation and abnormalities in lung morphology, including airway mucus plugging and wall thickening, in adolescent and adult patients with CF and one or two F508del alleles in a real-world, postapproval setting. (asthmafoundation.org.nz)
  • Cholon D.M., Quinney N.L., Fulcher M.L., Esther CR Jr, Das J, Dokholyan NV, Randell SH, Boucher RC, Gentzsch M. Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis // Sci Transl Med. (dntb.gov.ua)
  • Cystic fibrosis (CF) is an inherited condition caused by a defective gene (CFTR - Cystic Fibrosis Transmembrane Conductance Regulator) which causes the body to produce abnormally thick and sticky fluid, called mucus. (masslung.com)
Patients32
  • Functional exercise tolerance and quality of life have been shown to be significantly affected in patients with lung fibrosis. (nih.gov)
  • So, in patients with lung fibrosis there seems a clear indication to refer them for comprehensive pulmonary rehabilitation programmes and to initiate palliative care in an early phase of the disease. (nih.gov)
  • In the current review we will present a rationale for pulmonary rehabilitation in patients with lung fibrosis and the effects of this type of non-pharmacological intervention on exercise capacity and quality of life. (nih.gov)
  • The doctors, of course, are duly cautious about recommending colloidal silver usage for Cystic Fibrosis patients, even though it was they who documented the child's startling recovery from the severe symptoms of the disease. (selfgrowth.com)
  • Despite the challenges that the CF patients present, survival is more favorable than that seen in patients with chronic obstructive pulmonary disease and pulmonary fibrosis. (nih.gov)
  • The new findings, together with the robust anti-fibrotic effects demonstrated in clinical studies of patients with NASH and advanced fibrosis could potentially enable Galmed to quickly transition to Phase 2/3 clinical studies with Aramchol in indications with unmet need and faster development pathways. (prnewswire.com)
  • Bleomycin, an anti-neoplastic agent that causes lung fibrosis in human patients, has been used extensively in rodent models to mimic IPF and serves as the standard agent for induction of experimental pulmonary fibrosis in animals. (prnewswire.com)
  • NEW YORK (GenomeWeb) - Isolation of bacteria within different regions of the lungs of cystic fibrosis patients drives their diversification, according to a phylogenetic study appearing in Cell Host & Microbe today . (genomeweb.com)
  • Jorth and his colleagues sampled both left and right lungs from 10 cystic fibrosis patients at three sites: the upper, middle, and lower lobe airways. (genomeweb.com)
  • Based on their finding of strong signals of genetic compartmentalization, regional isolates with similar protein expression profiles, and spatially distinct distribution of bacterial phenotypes, the researchers said that their study indicates that isolation in different lung regions helped drive the diversification of P. aeruginosa in the lungs of cystic fibrosis patients. (genomeweb.com)
  • Additionally, patients taking GER medications had a lower fibrosis score on HRCT. (medscape.com)
  • In addition, so-called "real world" data on this drug, drawn from patient registries, showed that it roughly halved the number of UK patients having to receive lung transplants. (huffingtonpost.co.uk)
  • However, the contribution of this cytokine to organ fibrosis in patients with cystic fibrosis is presently unclear. (bmj.com)
  • This study was undertaken to examine the association between TGF-β 1 gene polymorphisms and the development of pulmonary dysfunction in patients with cystic fibrosis. (bmj.com)
  • RESULTS Patients with cystic fibrosis of a TGF-β 1 high producer genotype for codon 10 had more rapid deterioration in lung function than those with a TGF-β 1 low producer genotype. (bmj.com)
  • DISCUSSION TGF-β 1 genotypes may have a role in mediating pulmonary dysfunction in patients with cystic fibrosis. (bmj.com)
  • Breathe deep: Which Patients Could Benefit from Inhaled Steroids in Cystic Fibrosis? (rtmagazine.com)
  • Joseph is one of many Lung Institute patients who has experienced an improvement in his quality of life after receiving adult stem cell therapy. (newswire.com)
  • According to the Lung Institute's in-house pilot study, 82 percent of patients saw an improvement in their quality of life. (newswire.com)
  • To examine the effect of ELX/TEZ/IVA on LCI and lung MRI scores in patients with CF and one or two F508del alleles aged ⩾12 years. (asthmafoundation.org.nz)
  • The viability of patients is determined by the degree of lung injury despite the fact that cystic fibrosis (CF) is a multiple organ disease. (dntb.gov.ua)
  • Early diagnosis and adequate treatment complex of cystic fibrosis prolong the lives of patients and improves its quality. (dntb.gov.ua)
  • To refine the radiology's capabilities in detecting lung changes in case of cystic fibrosis in adult patients and to determine the role of the computed tomography (CT) in the diagnostic process. (dntb.gov.ua)
  • The most typical radiographic and CT signs of lung damage were determined in adult CF patients. (dntb.gov.ua)
  • It has been shown that the radiographic changes in the lungs are non-specific for CF patients and are determined by the secondary inflammatory process. (dntb.gov.ua)
  • Clinical manifestations of cystic fibrosis among patients with diagnosis in adulthood // Chest 2004;126 (4):1215-24. (dntb.gov.ua)
  • Second, correct identification of bacteria in patients with CF remains challenging because phenotype variation is a common feature during chronic infection of the lung ( 4 , 9 ). (cdc.gov)
  • Management of cystic fibrosis lung disease, including mucus clearance techniques, mucolytics, and preventive therapy for patients infected with pseudomonas. (masslung.com)
  • grin Here is the biggest take-away from the findings for those who can't read the jargon: for patients who lost more than 5% of their weight over the course of a year experienced a 16.5% loss in lung function . (cffatboy.com)
  • Longitudinal follow up showed that malnourished patients of all ages and those with P aeruginosa infection had significantly worse lung function than their normally nourished counterparts and a greater yearly loss of FEV 1 % predicted. (cffatboy.com)
  • Influence of genetic variation of the ß2-adrenergic receptor on lung diffusion in patients with cystic fibrosis. (bvsalud.org)
  • Autosomal dominant mutations in the TERC or TERT genes, which encode telomerase, have been identified in about 15 percent of pulmonary fibrosis patients. (wikipedia.org)
Biopsy3
Clinical4
  • Many doctors are skeptical of the clinical application of stem cells for lung disease, simply because advancements in the field have been so recent. (newswire.com)
  • Diagnosis of Cystic Fibrosis in Adults // Clinical Pulmonary Medicine. (dntb.gov.ua)
  • Clinical symptoms were exhibited by 10 children (76.9%), including cough, rhinitis, and lung exacerbations. (cdc.gov)
  • These data emphasise the close relationship between nutrition, lung function, and clinical course in CF. Normal body weight and absence of P aeruginosa infection was associated with better preservation of lung function. (cffatboy.com)
Vivo3
  • Perhaps the most encouraging development, however, is that of ex vivo lung perfusion. (nih.gov)
  • Lung allografts from β-catenin reporter mice demonstrated reduced β-catenin transcriptional activation in the presence of LPA1 antagonist, confirming an in vivo role for LPA signaling in β-catenin activation. (jci.org)
  • PAT-048 reduces dermal fibrosis in vivo. (targetmol.com)
Diagnosed with pulmonary fibrosis1
Cystic Fibrosis F1
Diagnosis of cystic fibrosis2
Therapy for pulmonary fibrosis1
  • Online, August 25, 2016 (Newswire.com) - Joseph O. visited the Lung Institute in Tampa, Fla., to receive adult stem cell therapy for pulmonary fibrosis in March of 2015, and his results surprised his pulmonologist. (newswire.com)
Disease with poor prognosis and limited1
  • Moreover, interstitial pulmonary fibrosis is a progressive disease with poor prognosis and limited response to conventional pharmacological treatment like immunosuppressive agents. (nih.gov)
Pulmonary hypertension1
  • Cor Pulmonale Cor pulmonale is enlargement and thickening of the ventricle on the right side of the heart resulting from an underlying lung disorder that causes pulmonary hypertension (high pressures in the. (msdmanuals.com)
Development of fibrosis1
  • It is a potent stimulator of fibrosis, and increased TGF-β signaling is associated with the development of fibrosis in various organs. (wikipedia.org)
Complications1
  • We report the case of a 12-year-old boy with severe CF lung complications who was treated by his family with colloidal silver. (selfgrowth.com)
Murine model1
  • In addition, TH5487 decreases levels of pro-inflammatory mediators, inflammatory cell infiltration, and lung remodeling in a murine model of bleomycin-induced pulmonary fibrosis conducted in male C57BL6/J mice. (lu.se)
Inflammation and fibrosis1
  • Aramchol's ability to modulate hepatic lipid metabolism was discovered and validated in animal models, demonstrating downregulation of the three key pathologies of NASH: steatosis, inflammation and fibrosis. (prnewswire.com)
Progresses1
  • As the fibrosis progresses, it may become harder to breathe, and you may need oxygen to help you breathe. (chestnet.org)
Humans1
Pirfenidone1
  • The combination of KIN001 with pirfenidone demonstrated greater reduction in lung fibrosis, indicating the potential for additional benefit of KIN001 in combination with the current standard of care. (swissbiotech.org)
Deterioration in lung function1
  • Progressive deterioration in lung function results in increasing dyspnea, chronic cough, and frequent hospitalizations. (medscape.com)
Human lung1
  • Research Objective This proposal will design new models to study behavior that is specific to human lung stem cells, and screen for drugs that can target abnormal stem cells in fibrotic disease. (ca.gov)
Organ2
  • In most European nations, they are then advised to an organ obtainment association which appoints accessible contributor lungs as indicated by foreordained models. (alliedacademies.org)
  • This mucus builds up in the breathing passages of the lungs and in the pancreas, the organ that helps to break down and absorb food. (masslung.com)
Progression2
  • Here, we have identified an autocrine pathway linking nuclear factor of activated T cells 2 (NFAT1), autotaxin (ATX), lysophosphatidic acid (LPA), and β-catenin that contributes to progression of fibrosis in lung allografts. (jci.org)
  • A non-linear mixed-effects disease progression model was developed using FEV1% predicted measurements at age 5 years from the ACFBAL study and at ages 6-14 years for these subjects from the Australian Cystic Fibrosis Data Registry. (edu.au)
Mutations2
Treatment11
  • In this particular case, a young boy was first diagnosed with Cystic Fibrosis at age two, and over the ensuing nine years grew worse and worse in spite of intensive medical treatment. (selfgrowth.com)
  • Our lead compound, Aramchol™, a backbone drug candidate for the treatment of NASH and fibrosis is currently in a Phase 3 registrational study. (prnewswire.com)
  • Aramchol (arachidyl amido cholanoic acid) is a novel fatty acid bile acid conjugate, liver targeted SCD1 modulator, developed as an oral therapy for the treatment of nonalcoholic steatohepatitis ("NASH") and fibrosis. (prnewswire.com)
  • Phylogenetic analysis indicated that all P. aeruginosa infecting a lung were related, but it also showed that as the bacteria became isolated in different lung regions, they began to evolve differences in treatment resistance, nutritional requirements, and more. (genomeweb.com)
  • I hypothesis that manipulation of lung macrophage phenotype by treatment with azithromycin may limit fibrosis in the lung. (europa.eu)
  • These findings are important because despite significant advances to reveal the pathological mechanisms of persistent fibrosis, effective treatment interventions are lacking. (uab.edu)
  • Treatment with PD also reduced the lung PH activities in the BL + PD groups compared with the corresponding BL + CD groups. (aspetjournals.org)
  • BL treatment increased the lung procollagen I and III gene expressions in the BL + CD groups by several-fold at varying times compared with the corresponding SA + CD, and treatment with PD in the BL + PD groups significantly down-regulated the BL-induced overexpression of these genes. (aspetjournals.org)
  • These preclinical data strongly support the potential of KIN001 to be an effective treatment for IPF and fibrotic disorders of the lung and other organs, both in single or combination therapy. (swissbiotech.org)
  • When pulmonary fibrosis made Joseph's life a daily struggle, he knew he had to find another treatment option. (newswire.com)
  • There are a number of potential causes of pulmonary fibrosis, including exposure to airborne toxins, undergoing radiation treatment, genetics and taking some specific medications. (newswire.com)
Mucus2
  • When two channels that are supposed to move chloride and sodium ions out of cells in the lungs fail to function properly, it leads to the mucus buildup seen in cystic fibrosis. (rtmagazine.com)
  • Japanese researchers have discovered that the channel dysfunctions also reduce the amount of zinc ions going into the lungs, further contributing to the thick mucus accumulation. (rtmagazine.com)
Irreversible1
Bacterial3
  • What made this so important to us is that the bacterial populations inhabiting different lung regions varied dramatically in terms of their antibiotic resistance and virulence," first author Peter Jorth from the University of Washington School of Medicine said in a statement. (genomeweb.com)
  • The researchers used phenotypic data to classify the isolates into subpopulations, and found that each patient harbored between 12 and 40 bacterial subpopulations in their lungs and that each lobar region contained between one and three of those sub-populations. (genomeweb.com)
  • Recent advances in bacterial taxonomy and improved microbial identification methods have led to increasing recognition of the complexity of microbial ecology of the CF lung ( 3 - 5 ). (cdc.gov)
Therapeutic3
  • To date, therapies to limit/reverse fibrosis in the lung have failed to provide long-term beneficial effects and new therapeutic targets are desperately needed. (europa.eu)
  • The work proposed in this application will allow me to investigate the role of the immune system in general, and more specifically the macrophage, in fibrosis and identify new therapeutic targets. (europa.eu)
  • A: In spite of significant advances in understanding mechanisms involved in development of lung injury and lung fibrosis, therapeutic interventions are very limited. (uab.edu)
Alveolar epithelial1
  • However, the potential pathomechanisms of alveolar epithelial cell senescence and the impact of senolytic drugs on senescent lung cells and fibrosis remain unknown. (ersjournals.com)
Chest3
  • METHODS: A cross-sectional analysis of association between Aspergillus positive bronchoalveolar lavage (BAL) cultures and chest high-resolution CT (HRCT) scan findings at age 5 years in subjects from the Australasian Cystic Fibrosis Bronchoalveolar Lavage (ACFBAL) study was performed. (edu.au)
  • A chest x-ray may show lung damage, mostly in the lower parts of both lungs. (msdmanuals.com)
  • Symptoms of pulmonary fibrosis are mainly: Shortness of breath, particularly with exertion Chronic dry, hacking coughing Fatigue and weakness Chest discomfort including chest pain Loss of appetite and rapid weight loss Pulmonary fibrosis is suggested by a history of progressive shortness of breath (dyspnea) with exertion. (wikipedia.org)
Significantly2
  • In the cystic fibrosis group, cotinine was significantly lower than in the controls. (bmj.com)
  • KIN001 significantly reduced the upregulation of multiple key inflammatory cytokines and chemokines implicated in the pathology of lung fibrosis. (swissbiotech.org)
20021
  • The Irish Lung Fibrosis Association (ILFA) is the national patient organisation for lung fibrosis and was founded in 2002. (ilfa.ie)
Inflammatory2
  • Our results indicate that the antifibrotic effect of PD was partly due to suppression of the BL-induced inflammatory events and partly due to down-regulation of BL-induced overexpression of lung procollagen I and III genes. (aspetjournals.org)
  • This reaction may appear to resolve prior to the onset of late fibrosis and/or represent a continuum, leading to the suggestion that late fibrosis is a consequence of the initial inflammatory reaction. (radioprotection.org)
Causes pulmonary2
Function10
  • Research Objective Understanding regulators of human alveolar lung stem cell function will promote more normal lung regeneration after injury and avoid the nearly untreatable problem of advanced pulmonary fibrosis. (ca.gov)
  • I will investigate whether specific macrophage phenotypes contribute to loss of function by driving aberrant epithelial repair and fibrotic remodeling in the lung. (europa.eu)
  • The major focus of our lab is to establish whether metabolic "switches" and modulators of mitochondrial function are viable targets to promote of fibrosis resolution. (uab.edu)
  • Passive smoking and impaired lung function in cystic fibrosis. (bmj.com)
  • 2) accelerated lung function decline between ages 5 years and 14 years and (3) to identify explanatory variables. (edu.au)
  • Lung function demonstrated a non-linear decline in this population. (edu.au)
  • However, no association was observed between positive Aspergillus BAL cultures on FEV1% predicted at age 5 years or with lung function decline between ages 5 years and 14 years. (edu.au)
  • This decrease in lung ability can be seen on breathing tests, also known as lung function tests . (chestnet.org)
  • These tests may include blood tests, imaging tests, and lung function tests . (chestnet.org)
  • Here I was assuming that I had better lung function back in the day simply because I was younger, but remembering that I weighed 118 back then and never got close to that ever since had me wondering if a better life was around the corner just by gaining weight. (cffatboy.com)
Abnormalities1
  • however, effects on the lung clearance index (LCI) determined by multiple-breath washout and abnormalities in lung morphology and perfusion detected by magnetic resonance imaging (MRI) have not been studied. (asthmafoundation.org.nz)
Pathology1
  • As an example, our discovery is possible only because of comprehensive collaboration with Dr. Victor J. Thannickal (PACCM) and Dr. Victor Darley-Usmar (Pathology)-leading experts in lung fibrosis and mitochondrial bioenergetics, respectively. (uab.edu)
Untreatable1
Fibrotic disorders1