A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death.
A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change.
Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury.
A complex of related glycopeptide antibiotics from Streptomyces verticillus consisting of bleomycin A2 and B2. It inhibits DNA metabolism and is used as an antineoplastic, especially for solid tumors.
An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.
Either of the pair of organs occupying the cavity of the thorax that effect the aeration of the blood.
A hydroxylated form of the imino acid proline. A deficiency in ASCORBIC ACID can result in impaired hydroxyproline formation.
A diverse group of lung diseases that affect the lung parenchyma. They are characterized by an initial inflammation of PULMONARY ALVEOLI that extends to the interstitium and beyond leading to diffuse PULMONARY FIBROSIS. Interstitial lung diseases are classified by their etiology (known or unknown causes), and radiological-pathological features.
Washing liquid obtained from irrigation of the lung, including the BRONCHI and the PULMONARY ALVEOLI. It is generally used to assess biochemical, inflammatory, or infection status of the lung.
Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules.
Spindle-shaped cells with characteristic CONTRACTILE PROTEINS and structures that contribute to the WOUND HEALING process. They occur in GRANULATION TISSUE and also in pathological processes such as FIBROSIS.
Small polyhedral outpouchings along the walls of the alveolar sacs, alveolar ducts and terminal bronchioles through the walls of which gas exchange between alveolar air and pulmonary capillary blood takes place.
A chloride channel that regulates secretion in many exocrine tissues. Abnormalities in the CFTR gene have been shown to cause cystic fibrosis. (Hum Genet 1994;93(4):364-8)
A common interstitial lung disease caused by hypersensitivity reactions of PULMONARY ALVEOLI after inhalation of and sensitization to environmental antigens of microbial, animal, or chemical sources. The disease is characterized by lymphocytic alveolitis and granulomatous pneumonitis.
A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of SKIN; CONNECTIVE TISSUE; and the organic substance of bones (BONE AND BONES) and teeth (TOOTH).
Historically, a heterogeneous group of acute and chronic diseases, including rheumatoid arthritis, systemic lupus erythematosus, progressive systemic sclerosis, dermatomyositis, etc. This classification was based on the notion that "collagen" was equivalent to "connective tissue", but with the present recognition of the different types of collagen and the aggregates derived from them as distinct entities, the term "collagen diseases" now pertains exclusively to those inherited conditions in which the primary defect is at the gene level and affects collagen biosynthesis, post-translational modification, or extracellular processing directly. (From Cecil Textbook of Medicine, 19th ed, p1494)
A factor synthesized in a wide variety of tissues. It acts synergistically with TGF-alpha in inducing phenotypic transformation and can also act as a negative autocrine growth factor. TGF-beta has a potential role in embryonal development, cellular differentiation, hormone secretion, and immune function. TGF-beta is found mostly as homodimer forms of separate gene products TGF-beta1, TGF-beta2 or TGF-beta3. Heterodimers composed of TGF-beta1 and 2 (TGF-beta1.2) or of TGF-beta2 and 3 (TGF-beta2.3) have been isolated. The TGF-beta proteins are synthesized as precursor proteins.
A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.
A condition characterized by the thickening of the ventricular ENDOCARDIUM and subendocardium (MYOCARDIUM), seen mostly in children and young adults in the TROPICAL CLIMATE. The fibrous tissue extends from the apex toward and often involves the HEART VALVES causing restrictive blood flow into the respective ventricles (CARDIOMYOPATHY, RESTRICTIVE).
An antineoplastic agent derived from BLEOMYCIN.
Measurement of the various processes involved in the act of respiration: inspiration, expiration, oxygen and carbon dioxide exchange, lung volume and compliance, etc.
A subtype of transforming growth factor beta that is synthesized by a wide variety of cells. It is synthesized as a precursor molecule that is cleaved to form mature TGF-beta 1 and TGF-beta1 latency-associated peptide. The association of the cleavage products results in the formation a latent protein which must be activated to bind its receptor. Defects in the gene that encodes TGF-beta1 are the cause of CAMURATI-ENGELMANN SYNDROME.
Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.
The transference of either one or both of the lungs from one human or animal to another.
Sarcoidosis affecting predominantly the lungs, the site most frequently involved and most commonly causing morbidity and mortality in sarcoidosis. Pulmonary sarcoidosis is characterized by sharply circumscribed granulomas in the alveolar, bronchial, and vascular walls, composed of tightly packed cells derived from the mononuclear phagocyte system. The clinical symptoms when present are dyspnea upon exertion, nonproductive cough, and wheezing. (Cecil Textbook of Medicine, 19th ed, p431)
Enlargement of air spaces distal to the TERMINAL BRONCHIOLES where gas-exchange normally takes place. This is usually due to destruction of the alveolar wall. Pulmonary emphysema can be classified by the location and distribution of the lesions.
Chemical substances, produced by microorganisms, inhibiting or preventing the proliferation of neoplasms.
Liver disease in which the normal microcirculation, the gross vascular anatomy, and the hepatic architecture have been variably destroyed and altered with fibrous septa surrounding regenerated or regenerating parenchymal nodules.
Washing out of the lungs with saline or mucolytic agents for diagnostic or therapeutic purposes. It is very useful in the diagnosis of diffuse pulmonary infiltrates in immunosuppressed patients.
Syndrome characterized by the triad of oculocutaneous albinism (ALBINISM, OCULOCUTANEOUS); PLATELET STORAGE POOL DEFICIENCY; and lysosomal accumulation of ceroid lipofuscin.
Pyridine derivatives with one or more keto groups on the ring.
A form of pneumoconiosis caused by inhalation of asbestos fibers which elicit potent inflammatory responses in the parenchyma of the lung. The disease is characterized by interstitial fibrosis of the lung, varying from scattered sites to extensive scarring of the alveolar interstitium.
A slowly progressive condition of unknown etiology, characterized by deposition of fibrous tissue in the retroperitoneal space compressing the ureters, great vessels, bile duct, and other structures. When associated with abdominal aortic aneurysm, it may be called chronic periaortitis or inflammatory perianeurysmal fibrosis.
A pulmonary surfactant associated protein that plays a role in alveolar stability by lowering the surface tension at the air-liquid interface. It is a membrane-bound protein that constitutes 1-2% of the pulmonary surfactant mass. Pulmonary surfactant-associated protein C is one of the most hydrophobic peptides yet isolated and contains an alpha-helical domain with a central poly-valine segment that binds to phospholipid bilayers.
Round, granular, mononuclear phagocytes found in the alveoli of the lungs. They ingest small inhaled particles resulting in degradation and presentation of the antigen to immunocompetent cells.
Epithelial cells that line the PULMONARY ALVEOLI.
Asbestos. Fibrous incombustible mineral composed of magnesium and calcium silicates with or without other elements. It is relatively inert chemically and used in thermal insulation and fireproofing. Inhalation of dust causes asbestosis and later lung and gastrointestinal neoplasms.
A fibrillar collagen consisting of three identical alpha1(III) chains that is widely distributed in many tissues containing COLLAGEN TYPE I. It is particularly abundant in BLOOD VESSELS and may play a role in tissues with elastic characteristics.
A group of interstitial lung diseases with no known etiology. There are several entities with varying patterns of inflammation and fibrosis. They are classified by their distinct clinical-radiological-pathological features and prognosis. They include IDIOPATHIC PULMONARY FIBROSIS; CRYPTOGENIC ORGANIZING PNEUMONIA; and others.
Pathological processes involving any part of the LUNG.
The volume of air that is exhaled by a maximal expiration following a maximal inspiration.
Damage to any compartment of the lung caused by physical, chemical, or biological agents which characteristically elicit inflammatory reaction. These inflammatory reactions can either be acute and dominated by NEUTROPHILS, or chronic and dominated by LYMPHOCYTES and MACROPHAGES.
The most common form of fibrillar collagen. It is a major constituent of bone (BONE AND BONES) and SKIN and consists of a heterotrimer of two alpha1(I) and one alpha2(I) chains.
An interstitial lung disease of unknown etiology, occurring between 21-80 years of age. It is characterized by a dramatic onset of a "pneumonia-like" illness with cough, fever, malaise, fatigue, and weight loss. Pathological features include prominent interstitial inflammation without collagen fibrosis, diffuse fibroblastic foci, and no microscopic honeycomb change. There is excessive proliferation of granulation tissue within small airways and alveolar ducts.
The amount of a gas taken up, by the pulmonary capillary blood from the alveolar gas, per minute per unit of average pressure of the gradient of the gas across the BLOOD-AIR BARRIER.
The mucous membrane lining the RESPIRATORY TRACT, including the NASAL CAVITY; the LARYNX; the TRACHEA; and the BRONCHI tree. The respiratory mucosa consists of various types of epithelial cells ranging from ciliated columnar to simple squamous, mucous GOBLET CELLS, and glands containing both mucous and serous cells.
Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.
Infection of the lung often accompanied by inflammation.
The worsening of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis.
Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.
Transparent, tasteless crystals found in nature as agate, amethyst, chalcedony, cristobalite, flint, sand, QUARTZ, and tridymite. The compound is insoluble in water or acids except hydrofluoric acid.
An idiopathic systemic inflammatory granulomatous disorder comprised of epithelioid and multinucleated giant cells with little necrosis. It usually invades the lungs with fibrosis and may also involve lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands.
Cells that line the inner and outer surfaces of the body by forming cellular layers (EPITHELIUM) or masses. Epithelial cells lining the SKIN; the MOUTH; the NOSE; and the ANAL CANAL derive from ectoderm; those lining the RESPIRATORY SYSTEM and the DIGESTIVE SYSTEM derive from endoderm; others (CARDIOVASCULAR SYSTEM and LYMPHATIC SYSTEM) derive from mesoderm. Epithelial cells can be classified mainly by cell shape and function into squamous, glandular and transitional epithelial cells.
A form of pneumoconiosis resulting from inhalation of dust containing crystalline form of SILICON DIOXIDE, usually in the form of quartz. Amorphous silica is relatively nontoxic.
Basic glycoprotein members of the SERPIN SUPERFAMILY that function as COLLAGEN-specific MOLECULAR CHAPERONES in the ENDOPLASMIC RETICULUM.
Drugs used for their effects on the respiratory system.
Measurable and quantifiable biological parameters (e.g., specific enzyme concentration, specific hormone concentration, specific gene phenotype distribution in a population, presence of biological substances) which serve as indices for health- and physiology-related assessments, such as disease risk, psychiatric disorders, environmental exposure and its effects, disease diagnosis, metabolic processes, substance abuse, pregnancy, cell line development, epidemiologic studies, etc.
Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES.
A receptor-regulated smad protein that undergoes PHOSPHORYLATION by ACTIVIN RECEPTORS, TYPE I. Activated Smad3 can bind directly to DNA, and it regulates TRANSFORMING GROWTH FACTOR BETA and ACTIVIN signaling.
Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.
Histochemical localization of immunoreactive substances using labeled antibodies as reagents.
The volume of air contained in the lungs at the end of a maximal inspiration. It is the equivalent to each of the following sums: VITAL CAPACITY plus RESIDUAL VOLUME; INSPIRATORY CAPACITY plus FUNCTIONAL RESIDUAL CAPACITY; TIDAL VOLUME plus INSPIRATORY RESERVE VOLUME plus functional residual capacity; or tidal volume plus inspiratory reserve volume plus EXPIRATORY RESERVE VOLUME plus residual volume.
A CCN protein family member that regulates a variety of extracellular functions including CELL ADHESION; CELL MIGRATION; and EXTRACELLULAR MATRIX synthesis. It is found in hypertrophic CHONDROCYTES where it may play a role in CHONDROGENESIS and endochondral ossification.
Tomography using x-ray transmission and a computer algorithm to reconstruct the image.
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
A gel-forming mucin that is predominantly expressed by submucosal glands of airway tissues and the SUBLINGUAL GLAND. It is one of the principal components of high molecular weight salivary mucin.
Inflammation of the lung due to harmful effects of ionizing or non-ionizing radiation.
A meshwork-like substance found within the extracellular space and in association with the basement membrane of the cell surface. It promotes cellular proliferation and provides a supporting structure to which cells or cell lysates in culture dishes adhere.
An abundant pulmonary surfactant-associated protein that binds to a variety of lung pathogens, resulting in their opsinization. It also stimulates MACROPHAGES to undergo PHAGOCYTOSIS of microorganisms. Surfactant protein A contains a N-terminal collagen-like domain and a C-terminal lectin domain that are characteristic of members of the collectin family of proteins.
Filamentous proteins that are the main constituent of the thin filaments of muscle fibers. The filaments (known also as filamentous or F-actin) can be dissociated into their globular subunits; each subunit is composed of a single polypeptide 375 amino acids long. This is known as globular or G-actin. In conjunction with MYOSINS, actin is responsible for the contraction and relaxation of muscle.
The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.
A metabolite of BROMHEXINE that stimulates mucociliary action and clears the air passages in the respiratory tract. It is usually administered as the hydrochloride.
A heterogeneous group of disorders, some hereditary, others acquired, characterized by abnormal structure or function of one or more of the elements of connective tissue, i.e., collagen, elastin, or the mucopolysaccharides.
Phenotypic changes of EPITHELIAL CELLS to MESENCHYME type, which increase cell mobility critical in many developmental processes such as NEURAL TUBE development. NEOPLASM METASTASIS and DISEASE PROGRESSION may also induce this transition.
Elements of limited time intervals, contributing to particular results or situations.
Difficult or labored breathing.
Experimentally induced chronic injuries to the parenchymal cells in the liver to achieve a model for LIVER CIRRHOSIS.
A receptor-regulated smad protein that undergoes PHOSPHORYLATION by ACTIVIN RECEPTORS, TYPE I. It regulates TRANSFORMING GROWTH FACTOR BETA and ACTIVIN signaling.
Inhaling liquid or solids, such as stomach contents, into the RESPIRATORY TRACT. When this causes severe lung damage, it is called ASPIRATION PNEUMONIA.
A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function.
A biosynthetic precursor of collagen containing additional amino acid sequences at the amino-terminal and carboxyl-terminal ends of the polypeptide chains.
Agents that increase mucous excretion. Mucolytic agents, that is drugs that liquefy mucous secretions, are also included here.
Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control (induction or repression) of gene action at the level of transcription or translation.
Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons.
Levels within a diagnostic group which are established by various measurement criteria applied to the seriousness of a patient's disorder.
A diffuse parenchymal lung disease caused by inhalation of dust and by tissue reaction to their presence. These inorganic, organic, particulate, or vaporized matters usually are inhaled by workers in their occupational environment, leading to the various forms (ASBESTOSIS; BYSSINOSIS; and others). Similar air pollution can also have deleterious effects on the general population.
Non-antibody proteins secreted by inflammatory leukocytes and some non-leukocytic cells, that act as intercellular mediators. They differ from classical hormones in that they are produced by a number of tissue or cell types rather than by specialized glands. They generally act locally in a paracrine or autocrine rather than endocrine manner.
The phenotypic manifestation of a gene or genes by the processes of GENETIC TRANSCRIPTION and GENETIC TRANSLATION.
Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.
A family of proteins that are involved in the translocation of signals from TGF-BETA RECEPTORS; BONE MORPHOGENETIC PROTEIN RECEPTORS; and other surface receptors to the CELL NUCLEUS. They were originally identified as a class of proteins that are related to the mothers against decapentaplegic protein, Drosophila and sma proteins from CAENORHABDITIS ELEGANS.
A rapid onset form of SYSTEMIC SCLERODERMA with progressive widespread SKIN thickening over the arms, the legs and the trunk, resulting in stiffness and disability.
A poisonous dipyridilium compound used as contact herbicide. Contact with concentrated solutions causes irritation of the skin, cracking and shedding of the nails, and delayed healing of cuts and wounds.
A cytokine synthesized by T-LYMPHOCYTES that produces proliferation, immunoglobulin isotype switching, and immunoglobulin production by immature B-LYMPHOCYTES. It appears to play a role in regulating inflammatory and immune responses.
An essential ribonucleoprotein reverse transcriptase that adds telomeric DNA to the ends of eukaryotic CHROMOSOMES.
A subfamily of HERPESVIRIDAE characterized by variable reproductive cycles. The genera include: LYMPHOCRYPTOVIRUS and RHADINOVIRUS.
Perisinusoidal cells of the liver, located in the space of Disse between HEPATOCYTES and sinusoidal endothelial cells.
The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.
The relatively long-lived phagocytic cell of mammalian tissues that are derived from blood MONOCYTES. Main types are PERITONEAL MACROPHAGES; ALVEOLAR MACROPHAGES; HISTIOCYTES; KUPFFER CELLS of the liver; and OSTEOCLASTS. They may further differentiate within chronic inflammatory lesions to EPITHELIOID CELLS or may fuse to form FOREIGN BODY GIANT CELLS or LANGHANS GIANT CELLS. (from The Dictionary of Cell Biology, Lackie and Dow, 3rd ed.)
One or more layers of EPITHELIAL CELLS, supported by the basal lamina, which covers the inner or outer surfaces of the body.
A pathological accumulation of air in tissues or organs.
Measure of the maximum amount of air that can be expelled in a given number of seconds during a FORCED VITAL CAPACITY determination . It is usually given as FEV followed by a subscript indicating the number of seconds over which the measurement is made, although it is sometimes given as a percentage of forced vital capacity.
Blockage in any part of the URETER causing obstruction of urine flow from the kidney to the URINARY BLADDER. The obstruction may be congenital, acquired, unilateral, bilateral, complete, partial, acute, or chronic. Depending on the degree and duration of the obstruction, clinical features vary greatly such as HYDRONEPHROSIS and obstructive nephropathy.
A positive regulatory effect on physiological processes at the molecular, cellular, or systemic level. At the molecular level, the major regulatory sites include membrane receptors, genes (GENE EXPRESSION REGULATION), mRNAs (RNA, MESSENGER), and proteins.
Infections with bacteria of the genus PSEUDOMONAS.
An abundant pulmonary surfactant-associated protein that binds to a variety of lung pathogens and enhances their opsinization and killing by phagocytic cells. Surfactant protein D contains a N-terminal collagen-like domain and a C-terminal lectin domain that are characteristic of members of the collectin family of proteins.
Studies which start with the identification of persons with a disease of interest and a control (comparison, referent) group without the disease. The relationship of an attribute to the disease is examined by comparing diseased and non-diseased persons with regard to the frequency or levels of the attribute in each group.
The administration of therapeutic agents drop by drop, as eye drops, ear drops, or nose drops. It is also administered into a body space or cavity through a catheter. It differs from THERAPEUTIC IRRIGATION in that the irrigate is removed within minutes, but the instillate is left in place.
The washing of a body cavity or surface by flowing water or solution for therapy or diagnosis.
The least progressive form of SYSTEMIC SCLERODERMA with skin thickening restricted to the face, neck and areas distal to the elbows and/or knees, sparing the trunk. The CREST SYNDROME is a form of limited scleroderma.
A variation of the PCR technique in which cDNA is made from RNA via reverse transcription. The resultant cDNA is then amplified using standard PCR protocols.
An enzyme that catalyzes the hydrolysis of proteins, including elastin. It cleaves preferentially bonds at the carboxyl side of Ala and Val, with greater specificity for Ala. EC 3.4.21.37.
Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states.
All of the processes involved in increasing CELL NUMBER including CELL DIVISION.
An immunoassay utilizing an antibody labeled with an enzyme marker such as horseradish peroxidase. While either the enzyme or the antibody is bound to an immunosorbent substrate, they both retain their biologic activity; the change in enzyme activity as a result of the enzyme-antibody-antigen reaction is proportional to the concentration of the antigen and can be measured spectrophotometrically or with the naked eye. Many variations of the method have been developed.
The larger air passages of the lungs arising from the terminal bifurcation of the TRACHEA. They include the largest two primary bronchi which branch out into secondary bronchi, and tertiary bronchi which extend into BRONCHIOLES and PULMONARY ALVEOLI.
A solvent for oils, fats, lacquers, varnishes, rubber waxes, and resins, and a starting material in the manufacturing of organic compounds. Poisoning by inhalation, ingestion or skin absorption is possible and may be fatal. (Merck Index, 11th ed)
A strain of albino rat used widely for experimental purposes because of its calmness and ease of handling. It was developed by the Sprague-Dawley Animal Company.
Glycoproteins found on the surfaces of cells, particularly in fibrillar structures. The proteins are lost or reduced when these cells undergo viral or chemical transformation. They are highly susceptible to proteolysis and are substrates for activated blood coagulation factor VIII. The forms present in plasma are called cold-insoluble globulins.
A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations.
A CC-type chemokine that is found at high levels in the THYMUS and has specificity for CCR4 RECEPTORS. It is synthesized by DENDRITIC CELLS; ENDOTHELIAL CELLS; KERATINOCYTES; and FIBROBLASTS.
The smallest member of the MATRIX METALLOPROTEINASES. It plays a role in tumor progression.
A species of gram-negative, aerobic, rod-shaped bacteria commonly isolated from clinical specimens (wound, burn, and urinary tract infections). It is also found widely distributed in soil and water. P. aeruginosa is a major agent of nosocomial infection.
Established cell cultures that have the potential to propagate indefinitely.
A type of asbestos that occurs in nature as the dihydrate of magnesium silicate. It exists in two forms: antigorite, a plated variety, and chrysotile, a fibrous variety. The latter makes up 95% of all asbestos products. (From Merck Index, 11th ed, p.893)
Disorder characterized by a wide range of structural changes in PERITONEUM, resulting from fibrogenic or inflammatory processes. Peritoneal fibrosis is a common complication in patients receiving PERITONEAL DIALYSIS and contributes to its gradual decrease in efficiency.
The N-acetyl derivative of CYSTEINE. It is used as a mucolytic agent to reduce the viscosity of mucous secretions. It has also been shown to have antiviral effects in patients with HIV due to inhibition of viral stimulation by reactive oxygen intermediates.
Regulatory proteins and peptides that are signaling molecules involved in the process of PARACRINE COMMUNICATION. They are generally considered factors that are expressed by one cell and are responded to by receptors on another nearby cell. They are distinguished from HORMONES in that their actions are local rather than distal.
The thin serous membrane enveloping the lungs (LUNG) and lining the THORACIC CAVITY. Pleura consist of two layers, the inner visceral pleura lying next to the pulmonary parenchyma and the outer parietal pleura. Between the two layers is the PLEURAL CAVITY which contains a thin film of liquid.
A CXC chemokine that is predominantly expressed in EPITHELIAL CELLS. It has specificity for the CXCR2 RECEPTORS and is involved in the recruitment and activation of NEUTROPHILS.
Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.
Persistent abnormal dilatation of the bronchi.

The integrin alpha v beta 6 binds and activates latent TGF beta 1: a mechanism for regulating pulmonary inflammation and fibrosis. (1/2241)

Transforming growth factor beta (TGF beta) family members are secreted in inactive complexes with a latency-associated peptide (LAP), a protein derived from the N-terminal region of the TGF beta gene product. Extracellular activation of these complexes is a critical but incompletely understood step in regulation of TGF beta function in vivo. We show that TGF beta 1 LAP is a ligand for the integrin alpha v beta 6 and that alpha v beta 6-expressing cells induce spatially restricted activation of TGF beta 1. This finding explains why mice lacking this integrin develop exaggerated inflammation and, as we show, are protected from pulmonary fibrosis. These data identify a novel mechanism for locally regulating TGF beta 1 function in vivo by regulating expression of the alpha v beta 6 integrin.  (+info)

Idiopathic pulmonary haemosiderosis. Epidemiology, pathogenic aspects and diagnosis. (2/2241)

Idiopathic pulmonary haemosiderosis (IPH) is a rare clinical entity characterized by recurrent episodes of diffuse alveolar haemorrhage, often presenting with haemoptysis. Many patients have iron deficiency anaemia due to deposition of haemosiderin iron in the alveoli, and eventually develop moderate pulmonary fibrosis. Typically, intensive search for an aetiology ends up negative. There is no evidence of pulmonary vasculitis or capillaritis. The aetiology is obscure, but may be an immunological or toxic mechanism causing a defect in the basement membrane of the pulmonary capillary. IPH affects both children and adults. During an acute episode, a chest X-ray demonstrates bilateral, alveolar infiltrates. Sputum examination discloses haemosiderin-laden alveolar macrophages. Diagnosis is established by lung biopsy (fiber-optic or thoracoscopic), showing large numbers of haemosiderin-laden macrophages in the alveoli and without evidence of capillaritis or deposition of immunoglobulins. Corticosteroids and/or immunosuppressive drugs may be effective during an acute bleeding episode, and may in some patients improve symptoms and prognosis on the long-term, but the response to treatment displays great interindividual variation.  (+info)

Pulmonary expression of interleukin-13 causes inflammation, mucus hypersecretion, subepithelial fibrosis, physiologic abnormalities, and eotaxin production. (3/2241)

Interleukin (IL)-13 is a pleiotropic cytokine produced in large quantities by activated CD4(+) Th2 lymphocytes. To define further its potential in vivo effector functions, the Clara cell 10-kDa protein promoter was used to express IL-13 selectively in the lung, and the phenotype of the resulting transgenic mice was characterized. In contrast to transgene-negative littermates, the lungs of transgene-positive mice contained an inflammatory response around small and large airways and in the surrounding parenchyma. It was mononuclear in nature and contained significant numbers of eosinophils and enlarged and occasionally multinucleated macrophages. Airway epithelial cell hypertrophy, mucus cell metaplasia, the hyperproduction of neutral and acidic mucus, the deposition of Charcot-Leyden-like crystals, and subepithelial airway fibrosis were also prominently noted. Eotaxin protein and mRNA were also present in large quantities in the lungs of the transgene-positive, but not the transgene-negative, mice. IL-4, IL-5, granulocyte-macrophage colony-stimulating factor, and monocyte chemoattractant protein-5 were not similarly detected. Physiological evaluations revealed significant increases in baseline airways resistance and airways hyperresponsiveness (AHR) to methacholine in transgene-positive animals. Thus, the targeted pulmonary expression of IL-13 causes a mononuclear and eosinophilic inflammatory response, mucus cell metaplasia, the deposition of Charcot-Leyden-like crystals, airway fibrosis, eotaxin production, airways obstruction, and nonspecific AHR. IL-13 may play an important role in the pathogenesis of similar responses in asthma or other Th2-polarized tissue responses.  (+info)

Reduced tumor necrosis factor-alpha and transforming growth factor-beta1 expression in the lungs of inbred mice that fail to develop fibroproliferative lesions consequent to asbestos exposure. (4/2241)

Tumor necrosis factor (TNF)-alpha and transforming growth factor (TGF)-beta mRNA and protein expression and the degree of fibroproliferative response to inhaled asbestos fibers are clearly reduced in the 129 inbred mouse strain as compared with typical fibrogenesis observed in the C57BL/6 inbred strain. The C57BL/6 mice showed prominent lesions at bronchiolar-alveolar duct (BAD) junctions where asbestos fibers deposit and responding macrophages accumulate. The 129 mice, however, were generally indistinguishable from controls even though the numbers of asbestos fibers deposited in the lungs of all exposed animals were the same. Quantitative morphometry of H&E-stained lung sections comparing the C57BL/6 and 129 mice showed significantly less mean cross-sectional area of the BAD junctions in the 129 animals, apparent at both 48 hours and 4 weeks after exposure. In addition, fewer macrophages had accumulated at these sites in the 129 mice. Nuclear bromodeoxyuridine immunostaining demonstrated that the number of proliferating cells at first alveolar duct bifurcations and in adjacent terminal bronchioles was significantly reduced in the 129 strain compared with C57BL/6 mice at 48 hours after exposure (P < 0.01). TNF-alpha and TGF-beta1 gene expression, as measured by in situ hybridization, was reduced in the 129 mice at 48 hours after exposure, and expression of TNF-alpha and TGF-beta1 protein, as measured by immunohistochemistry, was similarly reduced or absent in the 129 animals. We postulate that the protection afforded the 129 mice is related to reduction of growth factor expression by the bronchiolar-alveolar epithelium and lung macrophages.  (+info)

Effects of pirfenidone on procollagen gene expression at the transcriptional level in bleomycin hamster model of lung fibrosis. (5/2241)

A time course study was carried out to elucidate the mechanisms for antifibrotic effect of pirfenidone (PD). Hamsters were intratracheally (i.t.) instilled with saline (SA) or bleomycin (BL) (7.5 units/kg/5 ml). The animals were fed a diet containing 0.5% PD or the same control diet (CD) without the drug 2 days before and throughout the study. The animals were sacrificed at various times after instillation. The lung hydroxyproline level in BL + CD groups was gradually increased and peaked at 21 days to 181% of the SA + CD control. The BL + PD-treated groups showed a gradual decrease in their lung collagen content, showing a maximum reduction of 40% at day 21. The lung malondialdehyde levels of the BL + CD groups were increased by several-fold of the corresponding SA + CD groups at various times. The lung prolyl hydroxylase (PH) activities in the BL + CD groups were also increased by several-fold of the corresponding SA + CD groups at these time points. The hamsters in the BL + PD showed a gradual decrease in the lung malondialdehyde levels from 10 to 21days compared with their corresponding BL + CD groups. Treatment with PD also reduced the lung PH activities in the BL + PD groups compared with the corresponding BL + CD groups. However, PD failed to manifest any direct inhibitory effect on PH activity in vitro. 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. Studies evaluating the regulation of these genes at the transcriptional level revealed PD significantly reduced the transcription of PC I at 14 days. 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.  (+info)

T cell independence of bleomycin-induced pulmonary fibrosis. (6/2241)

The role of T cells and cytokines in bleomycin (BLM)-induced fibrosis was evaluated in susceptible and resistant strains of normal and SCID mice. Histology and hydroxyproline analysis showed that BLM induced pulmonary fibrosis in C57BL/6 and (C57BL/6 x BALB/c)F1 mice, whereas BALB/c mice were resistant to the disease. To test whether lymphocytes were required for the induction of BLM-induced pulmonary fibrosis, SCID mice were injected intratracheally with BLM and evaluated for the development of pulmonary inflammation and fibrosis. Similar morphological changes and increases in hydroxyproline were observed in both C57BL/6 SCID and (C57BL/6 x CB.17)F1 SCID animals compared to those seen in wild-type C57BL/6 and (C57BL/6 x BALB/c)F1 mice. In contrast, CB.17 SCID mice, which are genetically similar to BALB/c mice, were resistant to disease induction. Analysis of the cellular infiltrate in BLM-treated C57Bl/6 SCID mice confirmed a lack of T cells in the lungs of SCID mice and demonstrated a pronounced accumulation of eosinophils in areas of developing pulmonary fibrosis. NK cells were significantly elevated in untreated SCID mice and did not increase further after BLM treatment. Analysis of selected cytokines 1 day after initiation of BLM-induced pulmonary fibrosis indicated that the levels of TNF-alpha and IFN-gamma appeared to segregate with fibrosis in both the SCID and wild-type mice. The data demonstrate that T cells are not required for the induction of fibrosis by BLM and suggest that responses by non-lymphoid cells may be sufficient for the induction of fibrosis.  (+info)

Structural elucidation of a novel exopolysaccharide produced by a mucoid clinical isolate of Burkholderia cepacia. Characterization of a trisubstituted glucuronic acid residue in a heptasaccharide repeating unit. (7/2241)

The structure of the exopolysaccharide (EPS) produced by a clinical isolate of Burkholderia cepacia isolated from a patient with fibrocystic lung disease has been investigated. By means of methylation analyses, carboxyl reduction, partial depolymerization by fuming HCl and chemical degradations such as Smith degradation, lithiumethylenediamine degradation and beta-elimination, supported by GC/MS and NMR spectroscopic analyses, the repeat unit of the EPS has been identified and was shown to correspond to the acidic branched heptasaccharide with the following structure: [formula: see text]. This partially acetylated acidic polymer, distinguished by the presence of the less usual D-isomer of rhamnose and of a trisubstituted glucuronic acid residue, could represent the main EPS produced by this bacterial species.  (+info)

In search of a cause of cryptogenic fibrosing alveolitis (CFA): one initiating factor or many? (8/2241)

The history of patients with idiopathic pulmonary fibrosis (IPF) shows that the disease may be preceded by a viral-like illness. Although viruses have not been demonstrated, it is possible that viruses were not detected in culture because they do not replicate during latency. We investigated the presence of adenovirus in IPF and interstitial pneumonia associated with collagen vascular disease (CVD-IP), using the nested polymerase chain reaction (PCR) and in situ hybridization (ISH) for the E1A region of the adenovirus genome. Studies were performed on lung tissues obtained by transbronchial lung biopsy from 19 patients with IPF, 10 patients with CVD-IP and, for comparison, 20 patients with sarcoidosis. The E1A DNA was present in 3 out of 19 (16%) cases of IPF, in 5 of 10 (50%) cases of CVD-IP, and in 2 of 20 (10%) cases of sarcoidosis. The incidence of E1A DNA in CVD-IP was significantly higher than that in sarcoidosis (p < 0.05). In patients with IPF and CVD-IP, E1A DNA was more prevalent in patients treated with corticosteroids (6 out of 9 cases; 67%) than in those without it (2 out of 20 cases; 10%) (p < 0.01). ISH studies showed that 1 out of 8 cases of IPF and CVD-IP, in which E1A DNA was detected by PCR, was positive for E1A DNA. We conclude that adenovirus E1A is unlikely to be aetiologically involved in the pathogenesis of idiopathic pulmonary fibrosis or interstitial pneumonia associated with collagen vascular disease. However, a latent adenovirus infection may be reactivated or may newly infect the host following corticosteroid administration.  (+info)

A patient with cryptogenic fibrosing alveolitis, with both mural and desquamative features, had two lung biopsies at the times of coronary artery surgery. These lung specimens were studied, using light and electron microscopy, with immunofluorescence techniques and electron microanalysis. In addition to the typical changes of cryptogenic fibrosing alveolitis previously reported, we found blue-staining bodies within alveolar macrophages and giant cells. These bodies were 15--25 micrometer in diameter with an iron rich outer rim and core of connective tissue mucin--possibly chondroitin sulphate or dermatan sulphate. It seems unlikely that these blue bodies were due to fibreglass dust to which the patients had had a trivial exposure, but their exact nature and significance remains unclear.. ...
Relief is when you and the right researcher find each other Finding the right clinical trial for Familial interstitial pulmonary fibrosis can be challenging. However, with TrialsFinder (which uses the Reg4ALL database and privacy controls by Private Access), you can permit researchers to let you know opportunities to consider - all without revealing your identity. ...
Interstitial Pulmonary fibrosis Def. group of diseases characterized by infiltration of the interstitial tissue of the lung by abnormal tissue. Site of pathology = Interstitial tissue (in between alveoli) = Peri-alveolar tissue. Pathogenesis Exposure to dust (for example) ► Trigger immune system Or ► Inflammatory reaction Or ► Direct toxicity
SUMMARY. Twenty patients with unexplained pulmonary fibrosis and circulating antinuclear factor were identified. Diseases commonly associated with antinuclear factor, such as systemic lupus erythematosus or scleroderma, were excluded. Half of the patients showed typical restrictive ventilatory defects and X-ray evidence of severe fibrotic changes. The remaining patients predominantly had evidence of obstructive airway diseases.. Four lung biopsies were available. Three showed histological evidence of interstitial pulmonary fibrosis. Immunofluorescent study of one of the biopsy sections with pulmonary fibrosis from a patient with only IgM serum antinuclear factor showed IgM in the alveolar septa. Relatively little fibrosis was present in immunoglobulin-laden areas whereas no immunoglobulin could be demonstrated in the adjacent alveolar septa showing marked fibrosis. Seven out of 10 patients with X-ray evidence of severe interstitial pulmonary fibrosis and typical restrictive ventilatory defects ...
Anti-Jo-1 antibody: a marker for myositis with interstitial lung disease. Bernstein, R.M.; Morgan, S.H.; Chapman, J.; Bunn, C.C.; Mathews, M.B.; Turner-Warick, M.; Hughes, G.R.V. // British Medical Journal (Clinical Research Edition);7/21/1984, Vol. 289 Issue 6438, p151 Discusses the function of anti-jo-1 antibody in patients with myositis and cryptogenic fibrosing alveolitis in Great Britain. Definition of myositis; Identification of the antibody by counterimmunoelectrophoresis; Usage of cytotoxic drugs. ...
PubMed journal article Lung cancer and cryptogenic fibrosing alveolitis. A population-based cohort stud were found in PRIME PubMed. Download Prime PubMed App to iPhone or iPad.
TY - JOUR. T1 - Abatement of bleomycin-induced increases in vascular permeability, inflammatory cell infiltration, and fibrotic lesions in hamster lungs by combined treatment with taurine and niacin. AU - Wang, Q.. AU - Hyde, D. M.. AU - Giri, S. N.. PY - 1992. Y1 - 1992. N2 - BACKGROUND: The bleomycin (BL) hamster model of interstitial pulmonary fibrosis has been widely used to study the pathogenesis of interstitial pulmonary fibrosis and to screen potentially desirable antifibrotic agents. We have recently shown that taurine and niacin in combination, diminished BL- induced increases in lung lipid peroxidation and hydroxyproline content in hamsters. In the present study, we have evaluated the effects of taurine and niacin on the bronchoalveolar lavage (BAL) cells, and morphologic and morphometric features of the lung in the same model of pulmonary fibrosis. EXPERIMENTAL DESIGN: The hamsters were divided into 4 groups: saline; taurine + niacin + saline; BL; and taurine + niacin + BL. Treatment ...
The mechanisms underlying the pathogenesis of idiopathic pulmonary fibrosis (IPF) involve multiple pathways, such as inflammation, epithelial mesenchymal transition, coagulation, oxidative stress, and developmental processes. The small GTPase, RhoA, and its target protein, Rho-kinase (ROCK), may interact with other signaling pathways known to contribute to pulmonary fibrosis. This study aimed to determine the beneficial effects and mechanisms of fasudil, a selective ROCK inhibitor, on bleomycin-induced pulmonary fibrosis in mice. Our results showed that the Aschcroft score and hydroxyproline content of the bleomycin-treated mouse lung decreased in response to fasudil treatment. The number of infiltrated inflammatory cells in the bronchoalveolar lavage fluid (BALF) was attenuated by fasudil. In addition, fasudil reduced the production of transforming growth factor-β1 (TGF-β1), connective tissue growth factor (CTGF), alpha-smooth muscle actin (α-SMA), and plasminogen activator inhibitor-1 (PAI-1)
Individuals who are 18 years of age or older with any of the following:. Idiopathic pulmonary fibrosis (defined by either an open lung biopsy demonstrating pulmonary fibrosis and/or HRCT scan findings consistent with idiopathic pulmonary fibrosis as outlined by the American Thoracic Society/European Respiratory Society guidelines),. Familial pulmonary fibrosis (defined as idiopathic pulmonary fibrosis in two or more first-degree relatives). Relatives of patients with hereditary pulmonary fibrosis,. Hermansky-Pudlak syndrome (diagnosed by paucity or deficiency of platelet dense bodies on whole mount electron microscopy),. Pulmonary fibrosis associated with rheumatoid arthritis [defined by 1987 American College of Rheumatology Revised Criteria for the Classification of RA], or. Healthy research volunteers by history and indicated tests (individuals without history of chronic pulmonary disorder, collagen vascular disease, or bleeding disorder).. EXCLUSION CRITERIA:. Individuals with any of the ...
Individuals who are 18 years of age or older with any of the following:. Idiopathic pulmonary fibrosis (defined by either an open lung biopsy demonstrating pulmonary fibrosis and/or HRCT scan findings consistent with idiopathic pulmonary fibrosis as outlined by the American Thoracic Society/European Respiratory Society guidelines),. Familial pulmonary fibrosis (defined as idiopathic pulmonary fibrosis in two or more first-degree relatives). Relatives of patients with hereditary pulmonary fibrosis,. Hermansky-Pudlak syndrome (diagnosed by paucity or deficiency of platelet dense bodies on whole mount electron microscopy),. Pulmonary fibrosis associated with rheumatoid arthritis [defined by 1987 American College of Rheumatology Revised Criteria for the Classification of RA], or. Healthy research volunteers by history and indicated tests (individuals without history of chronic pulmonary disorder, collagen vascular disease, or bleeding disorder).. EXCLUSION CRITERIA:. Individuals with any of the ...
The mortality analysis of the members of the BTS CFA cohort identified that there were 46 deaths from lung cancer among 488 people who died (9.4%) and 23 (6.9%) deaths with both idiopathic fibrosing alveolitis and lung cancer mentioned on the death certificates, a figure only marginally higher than the UK co-mortality estimate (5.6%).3. It is plausible that the population-based co-mortality studies2 3 were limited by incomplete listings of pulmonary fibrosis on the death certificates, and diagnostic misclassification may have also contributed to the lack of association between IPF and lung cancer. A far higher prevalence of lung cancer was reported in other studies. The highest were those by Matsushita et al,1 Hironaka and Fukayama6 and Qunn,7 with a lung cancer prevalence of 48.2%, 45.7% and 43.1%, respectively. All of these studies were autopsy studies conducted in Japan and none was blinded. These estimates may be elevated by the increased probability of the presence of lung cancer being ...
TY - JOUR. T1 - A new model of progressive pulmonary fibrosis in rats. AU - Last, Jerold A. AU - Gelzleichter, T. R.. AU - Pinkerton, Kent E. AU - Walker, R. M.. AU - Witschi, H.. PY - 1993. Y1 - 1993. N2 - Sprague-Dawley rats were exposed for 6 h daily to 0.8 ppm of ozone and 14.4 ppm of nitrogen dioxide. Approximately 7 to 10 wk after the initiation of exposure, animals began to demonstrate respiratory insufficiency and severe weight loss. About half of the rats died between Days 55 and 78 of exposure; no overt ill effects were observed in animals exposed to filtered air, to ozone alone, or to nitrogen dioxide. Biochemical findings in animals exposed to ozone and nitrogen dioxide included increased lung content of DNA, protein, collagen, and elastin, which was about 300% higher than the control values. The collagen-specific crosslink hydroxypyridinium, a biomarker for mature collagen in the lung, was decreased by about 40%. These results are consistent with extensive breakdown and remodeling ...
Synonyms: fibrosing alveolitis, cryptogenic fibrosing alveolitis Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease characterised initially...
Circulating fibrocytes had been reported to migrate into the injured lungs, and contribute to fibrogenesis via chemokine-chemokine receptor systems including CXCL12-CXCR4 axis. Here we hypothesized that blockade of CXCR4 might inhibit the migration of fibrocytes to the injured lungs and the subsequent pulmonary fibrosis. To explore the antifibrotic effects of blockade of CXCR4, we used a specific antagonist for CXCR4, AMD3100, in bleomycin-induced pulmonary fibrosis model in mice. Administration of AMD3100 significantly improved the loss of body weight of mice treated with bleomycin, and inhibited the fibrotic lesion in subpleural areas of the lungs. The quantitative analysis demonstrated that treatment with AMD3100 reduced the collagen content and fibrotic score (Aschcroft score) in the lungs. Although AMD3100 did not affect cell classification in bronchoalveolar lavage fluid on day 7, the percentage of lymphocytes was reduced by AMD3100 on day 14. AMD3100 directly inhibited the migration of ...
Chronic lung diseases including asthma and idiopathic pulmonary fibrosis (IPF) are characterized by the airway inflammation, airway remodeling, subepithelial fibrosis, and hypoxia. Previous study indicated that hypoxia plays a critical role in tissue fibrosis. In chronic asthma and IPF, the CXCR4/CXCL12 (stromal cell-derived factor-1, SDF-1) axis plays important role in pulmonary fibrosis. CXCL12 is a potent chemokine for homing of CXCR4+ fibrocytes to sites of lung tissue injury, which directly contribute to pulmonary fibrosis. Circulating CXCR4+ fibrocytes and CXC12 were found to be significantly increases in both plasma and lung of the patient with pulmonary fibrosis. Moreover, an anti-CXCL12 neutralizing antibody attenuated bleomycin-induced pulmonary fibrosis in mice. In addition, CXCL12 plays an important role in carcinoma-associated fibroblast differentiation. These results suggest that interfering with CXCL12 network may help to block pulmonary fibrosis. There is increasing evidence ...
TY - JOUR. T1 - Pharmacologic Inhibition of HIF-1α Attenuates Radiation-Induced Pulmonary Fibrosis in a Preclinical Image Guided Radiation Therapy. AU - Nam, Jae Kyung. AU - Kim, A. Ram. AU - Choi, Seo Hyun. AU - Kim, Ji Hee. AU - Han, Su Chul. AU - Park, Seungwoo. AU - Lee, Yong Jin. AU - Kim, Joon. AU - Cho, Jaeho. AU - Lee, Hae June. AU - Lee, Yoon Jin. N1 - Funding Information: This work was supported by grants from the National Research Foundation ( NRF-2017M2A2A7A02019482/2020M2D9A2093964 and NRF-2020R1A2B5B02002709 ), and a grant from the Korea Institute of Radiologic and Medical Sciences (KIRAMS, 50531-2020 ) funded by the Ministry of Science and ICT (MSIT), Republic of Korea. PY - 2021/2/1. Y1 - 2021/2/1. N2 - Purpose: Radiation-induced pulmonary fibrosis (RIPF) is a long-term side effect of thoracic radiation therapy. Hypoxia-induced vascular endothelial mesenchymal transition (EndMT) can occur during the development of RIPF. Here, we examined the direct contribution of endothelial ...
The specific role of Toll-like receptor 4 (TLR4) in bleomycin-induced lung fibrosis of mice, a model of human idiopathic pulmonary fibrosis, has not been characterized. We injected bleomycin intratracheally into TLR4 knockout (TLR4-/-) and wild-type (WT) mice. Twenty-one days after injection, mice were sacrificed and their lungs were harvested for pathological, hydroxyproline, mRNA expression, and collagen I analyses. Body weight changes and mortality were observed.
Idiopathic pulmonary fibrosis (IPF, also called cryptogenic fibrosing alveolitis) is specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause, occurring in adults and limited to the lungs. It is associated with the his
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Idiopathic pulmonary fibrosis (IPF), previously known as cryptogenic fibrosing alveolitis (CFA) in Europe, is a chronic, relentlessly progressive fibrotic disorder of the lower respiratory tract that typically affects adults over the age of 40. Idiop
TY - JOUR. T1 - Role of fibronectin in fibrotic lung disease. A growth factor for human lung fibroblasts. AU - Bitterman, P.. AU - Rennard, S.. AU - Adelberg, S.. AU - Crystal, R. G.. PY - 1983. Y1 - 1983. N2 - The presence of increased amounts of the matrix component fibronectin together with alveolar macrophage-derived growth factor within the alveolar structures of patients with fibrotic lung disease may play a significant role in the fibrosis characteristic of these disorders, by amplifying the numbers of fibroblasts in the alveolar structures of these patients.. AB - The presence of increased amounts of the matrix component fibronectin together with alveolar macrophage-derived growth factor within the alveolar structures of patients with fibrotic lung disease may play a significant role in the fibrosis characteristic of these disorders, by amplifying the numbers of fibroblasts in the alveolar structures of these patients.. UR - ...
Pulmonary fibrosis may be a secondary effect of other diseases. Most of these are classified as interstitial lung diseases. Examples include autoimmune disorders, viral infections and bacterial infection like tuberculosis which may cause fibrotic changes in both lungs upper or lower lobes and other microscopic injuries to the lung. However, pulmonary fibrosis can also appear without any known cause. In this case, it is termed idiopathic. Most idiopathic cases are diagnosed as idiopathic pulmonary fibrosis. This is a diagnosis of exclusion of a characteristic set of histologic/pathologic features known as usual interstitial pneumonia (UIP). In either case, there is a growing body of evidence which points to a genetic predisposition in a subset of patients. For example, a mutation in surfactant protein C (SP-C) has been found to exist in some families with a history of pulmonary fibrosis. Autosomal dominant mutations in the TERC or TERT genes, which encode telomerase, have been identified in ...
Canadian Pulmonary Fibrosis Foundation (CPFF) seeks an Executive Director who is passionate, mission-driven and a self-directed individual. The ideal candidate has experience in the nonprofit sector, has excellent relationship and management skills, is innovative and effective in fundraising, has prior experience working with a volunteer Board of Directors, and can develop and execute a vision for CPFF.. CPFF is a national patient-led and patient-focused organization dedicated to the needs of pulmonary fibrosis patients, their families, caregivers, and health care professionals. CPFF is recognized globally for being a leader in pulmonary fibrosis awareness and advocacy.. CPFFs mission includes patient and caregiver support and education, public education about pulmonary fibrosis and support for pulmonary fibrosis research. It works closely with the Medical Advisory Board comprised of some of Canadas top respirologists and with a network of sponsors and partners. Over the past seven years the ...
Here, we show the pivotal role of PDGF signaling in the pathogenesis of radiation-induced pulmonary fibrosis. Importantly, our findings suggest that direct inhibition of fibrogenesis by PDGF RTKIs is an effective strategy to attenuate lung fibrosis. We found that three distinct inhibitors of PDGF signaling (Imatinib/Gleevec, SU9518, and SU11657) markedly attenuated pulmonary fibrosis in a mouse model of radiation-induced lung fibrosis (C57/Bl6).. We could demonstrate that treatment of irradiated mice with PDGF RTKIs markedly attenuates the development of fibroblast foci, the hallmark of pulmonary fibrosis, and the subsequent remodeling of the lung architecture. The morphological results were in agreement with qualitative and quantitative high resolution CT scans of mouse lungs, demonstrating that PDGFR inhibition could dramatically attenuate the typical radiological features of lung fibrosis induced by thoracic irradiation.. Interestingly, with respect to the time course of fibrosis development, ...
A recent market study published by the company Pulmonary Fibrosis Treatment Market: Global Industry Analysis 2014-2018 & Forecast, 2019-2029 consists of a comprehensive assessment of the most important market dynamics. After conducting thorough research on the historic as well as current growth parameters of the pulmonary fibrosis treatment market, the growth prospects of the market can be obtained with maximum precision. The report features the unique and salient factors that are likely to significantly impact the development of the pulmonary fibrosis treatment market during the forecast period. It can help market players modify their manufacturing and marketing strategies to envisage maximum growth in the pulmonary fibrosis treatment market in the upcoming years. The report provides detailed information about the current and future growth prospects of the pulmonary fibrosis treatment market in the most comprehensive manner for the better understanding of readers.. Chapter 01 - Executive ...
Jo 1 (histidyl tRNA synthetase) is a member of the amino acyl-tRNA synthetase family of enzymes found in all nucleated cells. Jo 1 antibodies in patients with polymyositis bind to conformational epitopes of the enzyme protein and inhibit its catalytic activity in vitro.(1). Jo 1 antibodies are a marker for the disease polymyositis, and occur most commonly in myositis patients who also have interstitial lung disease. The antibodies occur in up to 50% of patients with interstitial pulmonary fibrosis and symmetrical polyarthritis.(2). See Connective Tissue Disease Cascade (CTDC) in Special Instructions.. ...
Jo 1 (histidyl tRNA synthetase) is a member of the amino acyl-tRNA synthetase family of enzymes found in all nucleated cells. Jo 1 antibodies in patients with polymyositis bind to conformational epitopes of the enzyme protein and inhibit its catalytic activity in vitro.(1). Jo 1 antibodies are a marker for the disease polymyositis, and occur most commonly in myositis patients who also have interstitial lung disease. The antibodies occur in up to 50% of patients with interstitial pulmonary fibrosis and symmetrical polyarthritis.(2). See Connective Tissue Disease Cascade (CTDC) in Special Instructions.. ...
1 Answer - Posted in: lipitor, idiopathic pulmonary fibrosis - Answer: Havent heard that before, but that doesnt mean anything at all. My ...
Feifukang (FFK) is a traditional Chinese medicine composed of herbs that protect lung function. However, difficulty arises regarding the clinical application of FFK due to the complex mechanism of Chinese medicines. This study aimed to investigate the efficacy of FFK and explore its targeted genes and pathways. Histopathological changes and collagen deposition were measured to evaluate the effect of FFK on bleomycin-induced pulmonary fibrosis in mice. The differentially expressed targeted genes and pathways were first screened using RNA sequencing. Then network pharmacology and other experiments were conducted to confirm RNA sequencing data. FFK treatment reduced the pathological score and collagen deposition, with a decrease in α-SMA and collagen. RNA sequencing and network pharmacology results all showed that FFK can ameliorate pulmonary fibrosis through multi-genes and multi-pathways. The targeted genes in JAK-STAT signaling pathway are some of the most notable components of these multi-genes and
Singer Robert Goulet is gravely ill at the sickbay to the stars, Cedars-Sinai Medical Center in Los Angeles.Goulets family says he has Interstitial Pulmonary Fibrosis, a rare but rapidly progressive and fatal condition. Goulet is hoping for a lung transplant if a donor can be found.
Ludhiana, 6th April, 2012 (Shalu Arora and Rector Kathuria) Mrs Amrit Kaur-a 65 year old lady - W/o S. Gurcharan Singh of Janakpuri was in a serious condition. She had developed a large clot in her left leg which was in imminent danger of getting loose and blocking her lungs. She was seen in the Emergency by Dr Harinder Singh Bedi - head of Cardio Vascular Endovascular & Thoracic Surgery at the Christian Medical College & Hospital in Ludhiana. On examination and investigation Dr Bedi found that she already had previous episodes of pulmonary embolism. As it is - she suffered from a lung disease called Interstitial Pulmonary Fibrosis for which she was already under treatment. Her lung functions were very low because of the ILD and any further pulmonary embolism would have been potentially fatal. After a thorough check up she underwent a new procedure called catheter directed thrombolysis (CDT) with a strong blood thinner - tissue plasminogen activator (TPA) . Dr Bedi explained that this clot ...
Familial Pulmonary Fibrosis webinar presented by Janet Talbert, MS, CGC, Advisory for the National Jewish Health Interstitial Lung Disease Program. View the entire full length webinar here today. Please contact us for further detail!
Idiopathic pulmonary fibrosis is an inexorably fatal disorder characterized by connective tissue deposition within the terminal air spaces resulting in loss of lung function and eventual respiratory failure. Previously, we demonstrated that foci of activated fibroblasts expressing high levels of fibronectin, procollagen, and smooth muscle actin and thus resembling those found in healing wounds are responsible for the connective tissue deposition and scarring in idiopathic pulmonary fibrosis. Using in situ hybridization and immunohistochemistry, we now demonstrate the presence of transforming growth factor beta 1 (TGF-beta 1), a potent profibrotic cytokine, in the foci containing these activated fibroblasts. These results suggest that matrix-associated TGF-beta 1 may serve as a stimulus for the persistent expression of connective tissue genes. One potential source of the TGF-beta 1 is the alveolar macrophage, and we demonstrate the expression of abundant TGF-beta 1 mRNA in alveolar macrophages in ...
About the Familial Pulmonary Fibrosis Genetic Counseling Program and the affiliation between National Jewish and the Coalition for Pulmonary Fibrosis.
Patients with Pulmonary Fibrosis are not alone. Watch inspiring stories of those living with Pulmonary Fibrosis, including their challenges and triumphs, here.
Pulmonary fibrosis is more prevalent among men than it is among women. Adults between the ages of 40 and 70 have the highest risk, and approximately 65% of those affected by the condition are over the age of 60. Certain chemotherapy drugs and cardiac medications are potential risk factors, and these drugs are most commonly used by individuals in this age category. Some medical researchers believe that pulmonary fibrosis may begin as an inflammatory response. In tissues like the lungs that dont have the capacity to regenerate, inflammation can lead to scarring. Scar tissue cant support specialized cells, so functional impairment occurs. This theory appears to be supported by the fact that pulmonary fibrosis often arises in conjunction with autoimmune diseases like lupus erythematosus, rheumatoid arthritis, and scleroderma. There are also higher incidences of pulmonary fibrosis among individuals whove been exposed to environmental toxicants like cigarette smoke, asbestos fibers, radiation, and ...
Interleukin-8 (IL-8) is a potent chemo-attractant cytokine responsible for neutrophil infiltration in lungs with idiopathic pulmonary fibrosis (IPF). The IL-8 protein and mRNA expression are increased in the lung with IPF. We evaluated the effect of single nucleotide polymorphisms (SNPs) of the IL-8 gene on the risk of IPF. One promoter (rs4073T>A) and two intronic SNPs (rs2227307T>G and rs2227306C>T) of the IL-8 genes were genotyped in 237 subjects with IPF and 456 normal controls. Logistic regression analysis was applied to evaluate the association of these SNPs with IPF. IL-8 in BAL fluids was measured using a quantitative sandwich enzyme immunoassay, and promoter activity was assessed using the luciferase reporter assay. The minor allele frequencies of rs4073T>A and rs2227307T>G were significantly lower in the 162 subjects with surgical biopsy-proven IPF and 75 subjects with clinical IPF compared with normal controls in the recessive model (OR = 0.46 and 0.48, p = 0.006 and 0.007, respectively). The
Our studies demonstrate that signaling via the CCR2 receptor leads to the generation of pro-fibrotic signals following FITC or bleomycin inoculation. In particular, our studies yield several important points: 1) in the absence of CCR2, FITC-induced pulmonary fibrosis is diminished both histologically and quantitatively; 2) the protection is related to the absence of this specific chemokine receptor; deletion of the CCR5 receptor had no effect on the severity of pulmonary fibrosis; 3) the protection conferred by the absence of CCR2 is a generalized phenomenon; CCR2−/− mice are protected from pulmonary fibrosis induced by either FITC or bleomycin; 4) the protection from pulmonary fibrosis seen in CCR2−/− mice is not due to differences in early lung injury caused by the FITC inoculation; 5) despite the absence of the CCR2 receptor, CCR2−/− mice develop an inflammatory cellular response following FITC inoculation that is similar in magnitude and composition compared with the response in ...
Despite the restrictions of the pandemic, this years virtual Pulmonary Fibrosis Awareness Month campaign raised more than $73,000 (as of December 5,2020) and drew thousands to our education forums, Virtual Bubbles event and the Clarke Walk for Pulmonary Fibrosis.. Thanks to all of you, participation and connections were made across the country. More than 500 of you shared tributes and stories and almost 1,100 photos. You walked 16, 107 km, well beyond a return journey across the country (which is 6,818 km one way) in the Clarke Walk for Pulmonary Fibrosis.. Thousands of you took part in our Virtual Bubbles event. And we all appreciated the more than 20 experts from across the country who shared their knowledge and answered your questions during 16 live, online, educational forums.. The Wrap up and Applause event was both touching and inspirational. To the hundreds of people across the country who helped show people with pulmonary fibrosis and their families that they are not alone, thank ...
Participants can build a network of support, learn more about Pulmonary Fibrosis, receive practical information on living with the disease, and share common experiences. This support group is open to anyone dealing with Pulmonary Fibrosis and their care partners.. This months topic is Update on Research for Pulmonary Fibrosis by Rebecca Bascom, MD, MPH from Hershey Medical Center Pulmonary Fibrosis Clinic. No registration is necessary. For more information, call (717) 262-8447.. ...
December 26, 2017. To date, the molecular basis of pulmonary fibrosis has been poorly understood. Scientists from the Max Planck Institute for Heart and Lung Research in Bad Nauheim have now shown that reduced activity of the transcription factor FoxO3 plays a key role in the development of the disease. In research on mice, the progress of the disease was able to be halted using drugs that boost FoxO3 activity. The researchers are hoping they may have found a possible approach to treatment.. Idiopathic pulmonary fibrosis is currently an incurable lung disease, in which sufferers lose the ability to absorb adequate oxygen. Although the word idiopathic means that the cause is unknown, the disease primarily affects former and active heavy smokers from the age of 50.. An important role in idiopathic pulmonary fibrosis is played by connective tissue cells called fibroblasts. These cells provide structure to the air sacs (alveoli) in the lungs. During development of the disease, characteristic ...
Background Weve previously explored a therapeutic strategy for specifically targeting the profibrotic activity of IL-13 during experimental pulmonary fibrosis using a fusion protein comprised of human IL-13 and a mutated form of exotoxin A (IL13-PE) and observed that the intranasal delivery of IL13-PE reduced bleomycin-induced pulmonary fibrosis through its elimination of IL-13-responsive cells in the lung. of whole lung samples were performed at day 28 after bleomycin. Intrapulmonary infection promoted a neutralizing IgG2A and IgA antibody response in BALF and serum. Surprisingly, histological analysis showed a prior disease attenuated the introduction of bleomycin-induced pulmonary fibrosis, that was further attenuated from the intranasal administration of IL13-PE modestly. Although prior intranasal administration CGI1746 of IL13-PE didnt elicit an antibody response, the systemic administration of IL13-PE induced a solid neutralizing antibody response. Nevertheless, the last systemic ...
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Several cases of fatal exacerbations of fibrosing alveolitis associated with rheumatoid arthritis after infliximab treatment have recently been reported.1 We report here a case of fatal exacerbation of pulmonary fibrosis after adalimumab treatment in a patient with polyarthritis related to systemic sclerosis (SSc).. The patient, a 74 year old woman, had been diagnosed 5 years previously with severe Raynauds syndrome, sclerodactyly, pulmonary fibrosis, abnormal oesophageal peristalsis, and non-specific antinuclear antibodies (1/100; indirect immunofluorescence on Hep 2 cells). She also had severe distal polyarthritis affecting the wrists, metacarpophalangeal and proximal interphalangeal joints, with demineralisation but no erosion on x ray examination. She tested negative for rheumatoid factor and anti-cyclic citrullinated peptide antibodies. Successive treatments with low dose prednisone associated with methotrexate, leflunomide or azathioprine were ineffective ...
The study is a major step toward understanding and one day treating pulmonary fibrosis, which affects about 100,000 people in the U.S. The disease often is called idiopathic pulmonary fibrosis because, in most cases, the cause cannot be found. While the prognosis is unpredictable, patients typically survive only three to five years after diagnosis, according to the U.S. National Library of Medicine.. Pulmonary fibrosis slowly robs patients of breath and finally life, said Paul W. Noble, MD, professor and chair of the Department of Medicine and director of the Womens Guild Lung Institute at Cedars-Sinai. In our study, we identified novel potential pathways to finding treatments for this relentless disease. Noble was the studys principal investigator.. The investigators focused on alveoli, the small air sacs at the ends of lung airways. In the alveoli, oxygen and carbon dioxide are exchanged with blood during respiration. Epithelial cells that line the alveoli also make a substance that ...
Mesenchymal stromal/stem cell (MSC) therapy has shown promise in experimental models of idiopathic pulmonary fibrosis (IPF). The aim of this study was to test the therapeutic effects of extracellular vesicles produced by human BM MSCs (MEx) in a bleomycin-induced pulmonary fibrosis model and investigate mechanisms of action. Adult C57BL/6 mice were challenged with endotracheal instillation of bleomycin and treated with MEx concurrently, or for reversal models, at day 7 or 21. Experimental groups were assessed at day 7, 14, or 28. Bleomycin-challenged mice presented with severe septal thickening and prominent fibrosis, and this was effectively prevented or reversed by MEx treatment. MEx modulated lung macrophage phenotypes, shifting the proportions of lung proinflammatory/classical and nonclassical monocytes and alveolar macrophages toward the monocyte/macrophage profiles of control mice. A parallel immunomodulatory effect was demonstrated in the BM. Notably, transplantation of MEx-preconditioned ...
Pulmonary fibrosis (scarring throughout the lungs) symptoms are shortness of breath, coughing, and diminished exercise tolerance. Idiopathic pulmonary fibrosis describes a condition in which the cause is unknown. Pulmonary fibrosis has many causes such as exposure to asbestos, infections, lupus, RA, and medication.
In the present study, TGF-β1 was used to induce the proliferation of lung fibroblasts and generate an in vitro model of pulmonary fibrosis. In a preliminary experiment, treatment with 50 ng/ml TGF-β1 for 48 h induced the proliferation of lung fibroblasts, indicating that this was a suitable concentration to establish the in vitro model. This model has also been used in foreign and domestic studies (31-33). In addition, as lung fibroblasts are considered to participate in pulmonary inflammation and fibrosis in numerous autoimmune diseases, including rheumatoid arthritis (4,34), the model has also been used to investigate pulmonary fibrosis secondary to numerous other diseases in vitro. The present study suggested that TGF-β1 also induced lung fibroblasts to secrete inflammatory cytokine IL-6 and synthesize ColIα and ColIII, which are among the deposited ECM materials (35). It is likely that in lung tissue affected by pulmonary fibrosis, fibroblasts were affected by inflammation for a long ...
Before Its News). Idiopathic Pulmonary Fibrosis Market report 2016-2020 focuses on the major drivers and restraints for the key players. Idiopathic Pulmonary Fibrosis research report also provides granular analysis of the market share, segmentation, revenue forecasts and geographic regions of the market. The Idiopathic Pulmonary Fibrosis market research report is a professional and in-depth study on the current state of Idiopathic Pulmonary Fibrosis Industry.. Analysts forecast the global Idiopathic Pulmonary Fibrosis market to grow at a CAGR of 23.97% during the period 2016-2020.. Browse Detailed TOC, Tables, Figures, Charts and Companies Mentioned in Global Idiopathic Pulmonary Fibrosis market research report @ http://www.360marketupdates.com/global-idiopathic-pulmonary-fibrosis-2016-2020-10289800. The Idiopathic Pulmonary Fibrosis Market research report covers the present scenario and the growth prospects of the global Idiopathic Pulmonary Fibrosis industry for 2016-2020.. Idiopathic ...
Idiopathic Pulmonary Fibrosis Market. The Idiopathic Pulmonary Fibrosis market is expected to increase during the forecast period owing to the increasing prevalent population of Idiopathic Pulmonary Fibrosis (IPF) patients in the 7MM.. The Idiopathic Pulmonary Fibrosis market outlook section of the report helps to build the detailed comprehension of the historic, current and forecasted Idiopathic Pulmonary Fibrosis market trends by analyzing the impact of current therapies on the market, unmet needs, drivers and barriers and demand for better technology. The report gives a thorough detail of Idiopathic Pulmonary Fibrosis market trend of each marketed drug and late-stage pipeline therapy by evaluating their impact based on the annual cost of therapy, inclusion and exclusion criteria, mechanism of action, increasing patient pool, covered patient segment, expected launch year, competition with other therapies, and view of the key opinion leaders. Idiopathic Pulmonary Fibrosis Epidemiology. The ...
Idiopathic Pulmonary Fibrosis Market. The Idiopathic Pulmonary Fibrosis market is expected to increase during the forecast period owing to the increasing prevalent population of Idiopathic Pulmonary Fibrosis (IPF) patients in the 7MM.. The Idiopathic Pulmonary Fibrosis market outlook section of the report helps to build the detailed comprehension of the historic, current and forecasted Idiopathic Pulmonary Fibrosis market trends by analyzing the impact of current therapies on the market, unmet needs, drivers and barriers and demand for better technology. The report gives a thorough detail of Idiopathic Pulmonary Fibrosis market trend of each marketed drug and late-stage pipeline therapy by evaluating their impact based on the annual cost of therapy, inclusion and exclusion criteria, mechanism of action, increasing patient pool, covered patient segment, expected launch year, competition with other therapies, and view of the key opinion leaders. Idiopathic Pulmonary Fibrosis Epidemiology. The ...
Looking for desquamative interstitial pneumonia? Find out information about desquamative interstitial pneumonia. in agriculture, method of treating animals infested with skin parasites such as mites, ticks, and warbles. The animal is dipped into or forced to swim... Explanation of desquamative interstitial pneumonia
Idiopathic pulmonary fibrosis is a chronic, progressive lung disease. This condition causes scar tissue (fibrosis) to build up in the lungs, which makes the lungs unable to transport oxygen into the bloodstream effectively. The disease usually affects people between the ages of 50 and 70. Idiopathic pulmonary fibrosis belongs to a group of conditions called interstitial lung diseases (also known as ILD), which describes lung diseases that involve inflammation or scarring in the lung.. The most common signs and symptoms of idiopathic pulmonary fibrosis are shortness of breath and a persistent dry, hacking cough. Many affected individuals also experience a loss of appetite and gradual weight loss. Some people with idiopathic pulmonary fibrosis develop widened and rounded tips of the fingers and toes (clubbing) resulting from a shortage of oxygen. These features are relatively nonspecific; not everyone with these health problems has idiopathic pulmonary fibrosis. Other respiratory diseases, some of ...
Looking for online definition of diffuse fibrosing alveolitis in the Medical Dictionary? diffuse fibrosing alveolitis explanation free. What is diffuse fibrosing alveolitis? Meaning of diffuse fibrosing alveolitis medical term. What does diffuse fibrosing alveolitis mean?
Bleomycin-induced lung fibrosis in mice reproduces some key features of pulmonary fibrosis in humans including alveolar inflammation, myofibroblast proliferation, and collagen deposition. Glucocorticoids have been used as first-line therapy for the treatment of lung fibrosis, although their clinical efficacy is equivocal. We examined the effect of the glucocorticoid, methylprednisolone (MP), and the estrogen metabolite, 2-methoxyestradiol (2MEO) on bleomycin-induced bronchoalveolar inflammation, fibrosis, and changes in lung function. The characterization of the time-course of the bleomycin-induced fibrosis indicated that lung dry mass and hydroxyproline content showed less variance than histopathological assessment of fibrosis. The bleomycin-induced increases in bronchoalveolar lavage (BAL) fluid cell number and protein levels were not significantly influenced by treatment with either MP (1 mg.(kg body mass)(-1).day(-1), i.p.) or 2MEO (50 mg.(kg body mass)(-1).day(-1), i.p.). Lung fibrosis, ...
We evaluated 500 consecutive cases, 426 of which were included: 266 had TBLC and 160 had SLB. 189 patients had idiopathic pulmonary fibrosis, 143 had other fibrotic interstitial lung diseases, and 94 had non-fibrotic interstitial lung diseases. Patients undergoing TBLC had more comorbidities and better preserved lung function compared with those undergoing SLB; among patients with a final MDT diagnosis of idiopathic pulmonary fibrosis, patients undergoing TBLC were older, had more comorbidities, and had a different post-biopsy treatment profile than those who received SLB. The distinction between idiopathic pulmonary fibrosis and other interstitial lung diseases made by MDT diagnosis on the basis of TBLC biopsy had clear prognostic significance, with a 5-year transplant-free survival of 68% (95% CI 57-76) in patients with an MDT idiopathic pulmonary fibrosis diagnosis based on TBLC compared with 93% (87-96) in patients without an idiopathic pulmonary fibrosis diagnosis based on TBLC (hazard ...
Description of disease Idiopathic pulmonary fibrosis. Treatment Idiopathic pulmonary fibrosis. Symptoms and causes Idiopathic pulmonary fibrosis Prophylaxis Idiopathic pulmonary fibrosis
Real-World Study Analysing Progression and Survival of Idiopathic Pulmonary Fibrosis Patients with Preserved Lung Function on Antifibrotic Treatment
Background The pathogenesis of pulmonary fibrosis remains poorly understood. therefore theoretically avoiding ligand-receptor discussion. Frizzled-related proteins (FRZB) was the founding person in this family members [16-18] and verified to bind xWNT8 and antagonize its activity in and versions, including the impact caused by lack of endogenous SFRP1 and FRZB in the bleomycin-induced lung fibrosis model. We display that both and so are upregulated during bleomycin-induced lung fibrosis. to review their powerful profile in the bleomycin-induced pulmonary fibrosis model. and mRNA amounts had been 2 log-scales even more abundant than those of and may not be recognized. amounts were significantly improved at all period factors after bleomycin treatment however, not different between period points (Shape?1C) (2-method ANOVA PBS, 0.05 for period and connections). amounts were considerably and consistently elevated as time passes after bleomycin treatment (2-method ANOVA 0.0001 for bleomycin PBS, and ...
Latest industry research report on: Global Idiopathic Pulmonary Fibrosis Therapeutic Market , Industry Size, Share, Research, Reviews, Analysis, Strategies, Demand, Growth, Segmentation, Parameters, Forecasts. A rare lung disease often terminal, idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease affecting the lung interstitium. Patients diagnosed with IPF suffer an extreme lung deterioration resulting a decline in the lung functioning. IPF causes pulmonary fibrosis, which basically means damaging of the lung tissues. Breathing distress is the most common intricacy involved with idiopathic pulmonary fibrosis.. The cause of idiopathic pulmonary fibrosis still remains unknown, however, it is mostly linked with excessive inhalation of smoke or dust, exposure to unhealthy gases and chemicals and smoking of cigarettes. IPF may also be caused due to genetic predisposition or develop from other lung condition.. Some of the medical conditions, which may develop in a patient with ...
Looking for Idiopathic pulmonary fibrosis? Find out information about Idiopathic pulmonary fibrosis. the formation of an abnormal amount of fibrous tissue in an organ or part as the result of inflammation, irritation, or healing Growth of fibrous connective... Explanation of Idiopathic pulmonary fibrosis
TY - JOUR. T1 - Inhibition of serine palmitoyltransferase delays the onset of radiation-induced pulmonary fibrosis through the negative regulation of sphingosine kinase-1 expression. AU - Gorshkova, Irina. AU - Zhou, Tong. AU - Mathew, Biji. AU - Jacobson, Jeffrey R.. AU - Takekoshi, Daisuke. AU - Bhattacharya, Palash. AU - Smith, Brett. AU - Aydogan, Bulent. AU - Weichselbaum, Ralph R.. AU - Natarajan, Viswanathan. AU - Garcia, Joe G.N.. AU - Berdyshev, Evgeny V.. PY - 2012/8. Y1 - 2012/8. N2 - The enforcement of sphingosine-1-phosphate (S1P) signaling network protects from radiation-induced pneumonitis. We now demonstrate that, in contrast to early postirradiation period, late postirradiation sphingosine kinase-1 (SphK1) and sphingoid base-1-phosphates are associated with radiation-induced pulmonary fibrosis (RIF). Using the mouse model, we demonstrate that RIF is characterized by a marked upregulation of S1P and dihydrosphingosine-1-phosphate (DHS1P) levels in the lung tissue and in ...
Idiopathic Pulmonary Fibrosis (IPF) Market Research Report present a detailed analysis of the market listing Idiopathic Pulmonary Fibrosis (IPF) Epidemiology, Drug therapies and pipeline for study period from 2016-2018.
Previously, we have shown that heparan sulfate (HS) 6-O-endosulfatase 1 (Sulf1) is a transforming growth factor-β1 (TGF-β1)-responsive gene in normal human lung fibroblasts and functions as a negative feedback regulator of TGF-β1 and that TGF-β1 induces the expression of Sulf1 as well as that of the closely related Sulf2 in a murine model of pulmonary fibrosis. In this study, we focused on the role of Sulf2 in modulating TGF-β1 function and the development of pulmonary fibrosis. We found that Sulf2 mRNA was overexpressed in lung samples from human patients with idiopathic pulmonary fibrosis (IPF), and Sulf2 protein was specifically localized to the hyperplastic type II alveolar epithelial cells (AECs). In vitro, TGF-β1 induced the expression of Sulf2 with accompanied HS 6-O-desulfation in A549 cells, adenocarcinoma cells derived from the type II alveolar epithelium. Using small interference RNA to block Sulf2 expression, we observed a biphasic TGF-β1 response with early enhanced Smad ...
Common intrinsic or interstitial lung diseases include interstitial pulmonary fibrosis, sarcoidosis, and hypersensitivity pneumonitis. The autoimmune (collagen) diseases can affect any joint in the body, including the costochondral and costovertebral joints. Restrictive lung diseases are much less common than asthma; thus, it is not surprising that there are not enough hard data to assess whether such processes increase a divers risk of pulmonary barotrauma. If your lungs cant hold as much air as they used to, you may have a restrictive lung disease. In severe cases, the lung tissue, heart and major vessels may be compromised by the deformity and altered mechanics. The AP and transverse diameters of the chest should increase with inspiration, but do not increase to normal levels in these conditions. Symptoms consist of non-productive cough, wheezing and dyspnea; chest radiography is normal in most cases. This can occur when tissue in the chest wall becomes stiffened, or due to weakened muscles ...
TY - JOUR. T1 - Diagnostic criteria for idiopathic pulmonary fibrosis. T2 - A Fleischner Society White Paper. AU - Lynch, David A.. AU - Sverzellati, Nicola. AU - Travis, William D.. AU - Brown, Kevin K.. AU - Colby, Thomas V.. AU - Galvin, Jeffrey R.. AU - Goldin, Jonathan G.. AU - Hansell, David M.. AU - Inoue, Yoshikazu. AU - Johkoh, Takeshi. AU - Nicholson, Andrew G.. AU - Knight, Shandra L.. AU - Raoof, Suhail. AU - Richeldi, Luca. AU - Ryerson, Christopher J.. AU - Ryu, Jay H. AU - Wells, Athol U.. PY - 2017/1/1. Y1 - 2017/1/1. N2 - This Review provides an updated approach to the diagnosis of idiopathic pulmonary fibrosis (IPF), based on a systematic search of the medical literature and the expert opinion of members of the Fleischner Society. A checklist is provided for the clinical evaluation of patients with suspected usual interstitial pneumonia (UIP). The role of CT is expanded to permit diagnosis of IPF without surgical lung biopsy in select cases when CT shows a probable UIP pattern. ...
Objectives Reduced caveolin-1 levels in lung fibroblasts from patients with scleroderma and the lungs of bleomycin-treated mice promote collagen overexpression and lung fibrosis. This study was undertaken to determine whether caveolin-1 is deficient in leucocytes from bleomycin-treated mice and patients with scleroderma and to examine the consequences of this deficiency and its reversal.. Methods Mice or cells received the caveolin-1 scaffolding domain (CSD) peptide to reverse the pathological effects of reduced caveolin-1 expression. In bleomycin-treated mice, the levels of caveolin-1 in leucocytes and the effect of CSD peptide on leucocyte accumulation in lung tissue were examined. To validate the results in human disease and to identify caveolin-1-regulated molecular mechanisms, monocytes and neutrophils were isolated from patients with scleroderma and control subjects and caveolin-1, extracellular signal-regulated protein kinase (ERK), c-Jun N-terminal kinase (JNK), p38, CXC chemokine ...
Idiopathic Pulmonary Fibrosis is a respiratory disease characterized by the formation of scar tissue in the lungs called fibrosis. IPF patients exhibit a rapid increase in fibrosis leading to severe breathing difficulties, a persistent cough, and difficulty in performing daily tasks. It usually affects people between 50 and 70 years old, particularly men and smokers. The disease is fatal and approximately two-thirds of patients succumb within five years of diagnosis. Most IPF patients die of lung failure while waiting for a lung donor.. IPF is estimated to affect over 130,000 Americans. Every year, 48,000 new cases are diagnosed and 40,000 patients die from the disease. Although there are two FDA-approved therapies for IPF, Esbriet (Roche) and OFEV (Boehringer-Ingelheim), there is no cure.. AEOL 10150 in Idiopathic Pulmonary Fibrosis. Data generated in pre-clinical studies performed under the BARDA contract for Lung-ARS suggest that 10150 may prevent the development of fibrosis in diseases such ...
Idiopathic Pulmonary Fibrosis (IPF) is a rare, chronic, progressive fibrosing interstitial pneumonia which is found to affect the middle-aged and older adults; and affects lung tissue (alveoli in particular) by either thickening, stiffening, or persistent and progressive scarring (fibrosis) which increases irreversibly over time. If an individual has IPF, scarring affects the air sacs, limiting the amount of oxygen that gets into the blood. With less oxygen in the blood, one can get breathlessness from everyday activities, like walking. This group of lung disorders is also known as Diffuse Parenchymal Lung Diseases, which is characterized by a broader umbrella of Interstitial Lung Diseases (IDLs).. According to DelveInsight, the total Idiopathic Pulmonary Fibrosis (IPF) prevalent cases in 7 MM was found to be 229,170, in the year 2017.. Request for a detailed sample copy of the report: https://www.delveinsight.com/sample-request/idiopathic-pulmonary-fibrosis-market. Idiopathic Pulmonary ...
Idiopathic Pulmonary Fibrosis (IPF) is a rare, chronic, progressive fibrosing interstitial pneumonia which is found to affect the middle-aged and older adults; and affects lung tissue (alveoli in particular) by either thickening, stiffening, or persistent and progressive scarring (fibrosis) which increases irreversibly over time. If an individual has IPF, scarring affects the air sacs, limiting the amount of oxygen that gets into the blood. With less oxygen in the blood, one can get breathlessness from everyday activities, like walking. This group of lung disorders is also known as Diffuse Parenchymal Lung Diseases, which is characterized by a broader umbrella of Interstitial Lung Diseases (IDLs).. According to DelveInsight, the total Idiopathic Pulmonary Fibrosis (IPF) prevalent cases in 7 MM was found to be 229,170, in the year 2017.. Request for a detailed sample copy of the report: https://www.delveinsight.com/sample-request/idiopathic-pulmonary-fibrosis-market. Idiopathic Pulmonary ...
Idiopathic Pulmonary Fibrosis (IPF) is a rare, chronic, progressive fibrosing interstitial pneumonia which is found to affect the middle-aged and older adults; and affects lung tissue (alveoli in particular) by either thickening, stiffening, or persistent and progressive scarring (fibrosis) which increases irreversibly over time. If an individual has IPF, scarring affects the air sacs, limiting the amount of oxygen that gets into the blood. With less oxygen in the blood, one can get breathlessness from everyday activities, like walking. This group of lung disorders is also known as Diffuse Parenchymal Lung Diseases, which is characterized by a broader umbrella of Interstitial Lung Diseases (IDLs).. According to DelveInsight, the total Idiopathic Pulmonary Fibrosis (IPF) prevalent cases in 7 MM was found to be 229,170, in the year 2017.. Request for a detailed sample copy of the report: https://www.delveinsight.com/sample-request/idiopathic-pulmonary-fibrosis-market. Idiopathic Pulmonary ...
Pirfenidone in the treatment of idiopathic pulmonary fibrosis: an evidence-based review of its place in therapy George A Margaritopoulos,1 Eirini Vasarmidi,2 Katerina M Antoniou2 1Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK; 2Department of Thoracic Medicine and Laboratory of Molecular and Cellular Pneumonology, Interstitial Lung Disease Unit, University Hospital of Heraklion, Heraklion, Greece Abstract: The landscape of idiopathic pulmonary fibrosis (IPF) has changed. The significant progress regarding our knowledge on the pathogenesis of the disease together with the experience achieved after a series of negative trials has led to the development of two drugs for the treatment of IPF. Both pirfenidone and nintedanib can slow significantly the rate of disease progression. They are safe with side effects that can be either prevented by close collaboration between health care professionals and patients or treated successfully when they occur, rarely leading to treatment
TY - JOUR. T1 - Mechanisms of neutrophil accumulation in the lungs of patients with idiopathic pulmonary fibrosis. AU - Hunninghake, G. W.. AU - Gadek, J. E.. AU - Lawley, T. J.. AU - Crystal, R. G.. PY - 1981/1/1. Y1 - 1981/1/1. N2 - Neutrophils are a characteristic feature of the alveolitis of idiopathic pulmonary fibrosis (IPF), a chronic disorder limited to lung. One mechanism by which neutrophils may be selectively attracted to lung and not other tissues is via the secretion of a neutrophil-specific chemotactic factor by alveolar macrophages. To evaluate the role of alveolar macrophages in modulating the migration of neutrophils to the lung in IPF, alveolar macrophages, obtained by bronchoalveolar lavage of patients with IPF, were evaluated for their ability to release a chemotactic factor for neutrophils. Unstimulated alveolar macrophages from normal individuals did not release the factor. In patients with IPF, there was a significant correlation between the proportions of neutrophils in ...
Idiopathic pulmonary fibrosis biomarkers: clinical utility and a way of understanding disease pathogenesis Matthew Flynn, Elisabeth S Baker, Daniel J Kass Dorothy P and Richard P Simmons Center for Interstitial Lung Disease, Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA, USA Abstract: Idiopathic pulmonary fibrosis (IPF) is a typically fatal disease that remains incompletely understood despite intense study and the arrival of drugs that may alter the natural history of the disease. Rendering an accurate diagnosis and predicting prognosis remain challenging problems to clinicians. One potential solution to these clinical problems is the identification of IPF biomarkers, easily measured factors that can be employed to predict clinical behavior. Candidate biomarkers have been identified by research in the laboratory on potential culprit cells or genes that may contribute to the pathogenesis of IPF. In this review, we present the current data on a
Idiopathic pulmonary fibrosis (IPF), the prototype of interstitial lung diseases, has the worst prognosis and is the only interstitial lung disease for which approved pharmacological treatments are available. Despite being considered a rare disease, IPF patients pose major challenges to both physicians and healthcare systems. It is estimated that a large number of IPF patients reside in BRIC countries (Brazil, Russia, India, and China) given their overall total population of approximately 3 billion inhabitants. Nevertheless, the limited availability of chest imaging in BRIC countries is considered a chief obstacle to diagnosis, since high-resolution computed tomography of the chest is the key diagnostic test for IPF. Further, obtaining reliable lung function tests and providing treatment access is difficult in the more rural areas of these countries. However, IPF might represent an opportunity for BRIC countries: the exponentially increasing demand for the enrollment of IPF patients in clinical ...
TY - JOUR. T1 - Idiopathic pulmonary fibrosis. T2 - Pathogenesis and management. AU - Sgalla, Giacomo. AU - Iovene, Bruno. AU - Calvello, Mariarosaria. AU - Ori, Margherita. AU - Varone, Francesco. AU - Richeldi, Luca. PY - 2018/2/22. Y1 - 2018/2/22. N2 - Background: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive disease characterized by the aberrant accumulation of fibrotic tissue in the lungs parenchyma, associated with significant morbidity and poor prognosis. This review will present the substantial advances achieved in the understanding of IPF pathogenesis and in the therapeutic options that can be offered to patients, and will address the issues regarding diagnosis and management that are still open. Main body: Over the last two decades much has been clarified about the pathogenic pathways underlying the development and progression of the lung scarring in IPF. Sustained alveolar epithelial micro-injury and activation has been recognised as the trigger of several biological ...
Idiopathic pulmonary fibrosis (IPF) is a chronic fibrosing interstitial lung disease with a poor prognosis. The impact of malnutrition, especialy fat-free mass (FFM), on mortality is scarcely assessed in IPF patients. This study aimed at identifying factors associated with higher mortaliy in IPF. Nutritional status of 185 consecutive IPF patients from a referal Centre were prospectively evaluated by bioelectrical impedancemeter analysis (BIA) to measure FFM, body mass index (BMI), mid-arm circumference, triceps skinfold thickness, food intake analogue visual scale and biology. Malnutrition was defined as a FFM index (FFMI) |15 in women and |17 in men. Stepwise logistic regressions were performed to identify the factors associated with mortality. Malnutrition prevalence was 28% (52/185). IPF patients with low FFMI had higher mortality compared to patients with normal FFMI (p=0.015). In multivariable analyses, factors independently associated with mortality were active cancer, FFMI and BMI.
Introduction: Pulmonaryhypertension (PH) is a common complication of idiopathic pulmonary fibrosis (IPF) that is associated with poor prognosis. Screening for PH in IPF patients proves to be challenging.. Hypothesis: We hypothesized that combining several non-invasive studies can predict the presence of PH in IPF patients.. Methods: We included 235 IPF patients who underwent right heart catheterization (RHC) as part of the lung transplant evaluation. We measured ECG and echocardiographic variables as well as the pulmonary artery (PA) diameter and ascending aorta (AA) diameters on chest CT. We recorded results of arterial blood gases (ABG), pulmonary function (PFT) and 6-min walk tests (6MWT).. Results: Several variables were predictors of PH in IPF patients in univariate models including arterial oxygenation, 6MWT distance, worse right ventricular (RV) function, the QRS axis, FVC/DLCOc ratio, PA/AA diameter ratio and estimated RV systolic pressure. In multivariate analysis, RV function and PA/AA ...
There are no data regarding the late toxicity of trastuzumab (T) administration with radiotherapy (RT). In this experimental study, we aimed to asses if concurrent or sequential administration of T has any impact for the development of radiation-induced pulmonary fibrosis in rats. Fifty-four female Wistar-albino rats were divided into 6 groups. First group of rats (Group 1; concurrent T) had irradiation to whole thoracic region concurrently with T. Second group (Group 2: sequential T-RT) received thoracic irradiation, 1 week after T. Third group (Group 3: sequential RT-T) had thoracic irradiation first and they had T injection 1 week after RT. Fourth group (Group 4: T only) had only T application. Fifth group (Group 5: RT) had only RT. The last group (Group 6: sham) of rats were observed without any application. A single dose of 12 Gy was given to both lungs with an anterior field at 2 cm depth. T dose which was equivalent to 6 mg/kg adult dose was calculated for each rat, and injected by the ...
The term idiopathic means unknown, making it a good name for a disorder that many are unfamiliar with. Its also unclear why idiopathic pulmonary fibrosis (IPF) develops. Idiopathic Pulmonary Fibrosis (IPF) is a disease of the lungs that causes stiffness of the tissue of the lungs. This makes it harder for you to natur
Brussels, Belgium, 22 February 2016 - Patient groups from nine European countries have come together to establish the European Idiopathic Pulmonary Fibrosis & Related Disorders Federation (EU-IPFF) in order to defend the interests of European patients with idiopathic pulmonary fibrosis (IPF) through one unified voice. IPF is a progressive, irreversible chronic disease of the lungs,1 with currently no known cure. EU-IPFF will push forward the IPF agenda at European level by improving patient access to treatment, increasing disease awareness, and advocating for research into new treatment options.. The newly formed federation represents an important landmark for the independent IPF patient groups and is the culmination of their continued fruitful collaboration building on initiatives like the IPF World Week Awareness Campaign and the IPF Patient Charter (www.ipfcharter.org). EU-IPFF will continue to collaborate with relevant medical organisations and bodies to ensure that the Charter ...
MODERN TIMES HELP LINE PHARMA - Exporter, Distributor & Supplier of IPF (idiopathic pulmonary fibrosis) Medicine based in Delhi, India
The Lancet Editors (July 25, p 312)1 highlight the publication of a new international clinical guideline for the treatment of idiopathic pulmonary fibrosis.2 This updated guideline provides conditional recommendations for use of nintedanib and pirfenidone to treat patients with idiopathic pulmonary fibrosis and emphasises the role of individual patients preferences and values in treatment decisions.2 According to the Editors, this guideline suggests that nintedanib and pirfenidone might be suitable for some patients, whereas the conditional recommendation, according to the guideline, is when the majority of individuals in this situation would want the suggested course of action, but many would not.. ...
Doctor will diagnose idiopathic pulmonary fibrosis based on your medical history, a physical exam, and the results from tests. Tests can help rule out other causes of your symptoms and show how badly your lungs are damaged.
We are a charity that focuses on supporting those with pulmonary fibrosis and IPF. We educate healthcare professionals about pulmonary fibrosis and IPF, give funds towards research and set up pulmonary fibrosis support groups. You can read more about symptoms of IPF and pulmonary fibrosis, fundraising for charity and where to find help about diagnosis.
This guideline is mainly useful to us in terms of when to consider Idiopathic Pulmonary Fibrosis , but there was some information in here that I was not aware of that is important. When to consider Idiopathic Pulmonary Fibrosis: • Patients over age 45 • Persistent breathlessness on exertion •…
Books contemporary approaches to idiopathic pulmonary fibrosis full online in PDF, ePub format, this books, Books contemporary approaches to idiopathic pulmonary fibrosis in PDF, ePub format. ...
In this study, after doses of 1 g/kg/day of the traditional Chinese formula Ma-Xing-Shi-Gan-Tang (MXSGT), significant reversal of pneumatocysts was achieved in Spague-Dawley rats with bleomycin-induced lung fibrosis. We found that MXSGT can improve the damaged condition of general cellular membranes. Greatly increased levels of lung NO were found in the bleomycin-induced group, which were then significantly reduced by MXSGT. MXSGT drastically prevented depletion of superoxide dismutase (SOD) and reduced the myeloperoxidase (MPO) activities and malondialdehyde (MDA) levels in lung tissue of rats treated with bleomycin. On the basis of the results presented in this paper, MXSGT prevents bleomycin-induced lung fibrosis, and the mechanism may be due to the inhibitory effect on nitric oxide generation in the bleomycin-induced lung-fibrosis model of rats ...
Idiopathic pulmonary fibrosis is a progressive disease that causes unremitting extracellular matrix deposition with resulting distortion of pulmonary architecture and impaired gas exchange. β-Arrestins regulate G protein (heterotrimeric guanine nucleotide-binding protein)-coupled receptors through receptor desensitization while also acting as signaling scaffolds to facilitate numerous effector pathways. Here, we examine the role of β-arrestin1 and β-arrestin2 in the pathobiology of pulmonary fibrosis. In the bleomycin-induced mouse lung fibrosis model, loss of either β-arrestin1 or β-arrestin2 resulted in protection from mortality, inhibition of matrix deposition, and protected lung function. Fibrosis was prevented despite preserved recruitment of inflammatory cells and fibroblast chemotaxis. However, isolated lung fibroblasts from bleomycin-treated β-arrestin-null mice failed to invade extracellular matrix and displayed altered expression of genes involved in matrix production and ...
Idiopathic pulmonary fibrosis (IPF) is a chronic irreversible and fatal disease caused by a progressive decline in lung function.[1][2] The term pulmonary fibrosis means scarring of lung tissue and is the cause of worsening dyspnea (shortness of breath). Fibrosis is usually associated with a poor prognosis.[1][2][3] IPF belongs to a large group of more than 200 lung diseases known as interstitial lung diseases (ILDs), characterized by the involvement of lung interstitium. IPF usually occurs in adults of between 50 and 70 years of age, particularly those with a history of cigarette smoking, and affects more men than women. Treatment to slow down the progression of the disease may include nintedanib or pirfenidone.[4] ...
... she developed a serious illness called pulmonary fibrosis- a type of condition that causes hardening of the lungs.[5] ...
Pulmonary toxicity *interstitial pneumonitis. *bronchiolitis obliterans with organizing pneumonia (BOOP). *pulmonary fibrosis ...
al-Masalkhi, A.; Walton, S.P. (1994). "Pulmonary fibrosis and occupational exposure to aluminum". The Journal of the Kentucky ... Exposure to powdered aluminium or aluminium welding fumes can cause pulmonary fibrosis.[153] Fine aluminium powder can ignite ...
"Pulmonary Fibrosis". Mayo Clinic. Retrieved 13 December 2013. Gurujeyalakshmi G, Giri SN (Sep-Oct 1995). "Molecular mechanisms ... such as chronic obstructive pulmonary disease (COPD) and cystic fibrosis. COPD is characterized by goblet cell hyperplasia and ... As a result, when interferon (IFN)-γ was used to knock down TGF-β, fibrosis of the lungs, caused by damage and scarring to lung ... Rennard SI (November 1999). "Inflammation and repair processes in chronic obstructive pulmonary disease". American Journal of ...
... in fibroblasts from the lungs of mice with bleomycin-induced pulmonary fibrosis and humans with Idiopathic pulmonary fibrosis ... pulmonary fibrosis; certain forms of malignant disease such as colon cancer including those that arise from Adenomatous ... Butaprost for the treatment of pulmonary fibrosis and certain neurological diseases CP533,536 for the stimulation of bone ... forms of inflammation such NMDA receptor-related neurotoxicity and the rodent model of Bleomycin-induced pulmonary fibrosis. ...
Cándido Pérez). January 22 José Ángel García, 69-70, actor and television director (La rosa de Guadalupe); pulmonary fibrosis. ... Cándido Pérez" (in Spanish) Fallece el director José Ángel García, padre de Gael García, de fibrosis (in Spanish) "Matan al ...
"Pulmonary Fibrosis". The Mayo Clinic. Retrieved July 25, 2018. Semedo D (June 5, 2016). "Phase 2 Study of Molecule Inhibitor ... idiopathic pulmonary fibrosis (IPF), and moderate to severe psoriasis. It is an inhibitor of Rho-associated coiled-coil kinase ... for Idiopathic Pulmonary Fibrosis Begins". Lung Disease News. BioNews Services, LLC. Retrieved 25 July 2018. Zanin-Zhorov A, ... Treatment with belumosudil reduced lung fibrosis in a bleomycin mouse model study. Belumosudil may have a therapeutic benefit ...
The most serious complication of bleomycin is pulmonary fibrosis and impaired lung function. It has been suggested that ... Due to the oxygen sensitive nature of bleomycin, and the theorised increased likelihood of developing pulmonary fibrosis ... Bleomycin is used in research to induce pulmonary fibrosis in mice.[15] ... "Vagotomy attenuates bleomycin-induced pulmonary fibrosis in mice". Scientific Reports. 5: 13419. doi:10.1038/srep13419. PMC ...
Maqbool A, Stallings VA (November 2008). "Update on fat-soluble vitamins in cystic fibrosis". Current Opinion in Pulmonary ... Fat-soluble vitamin deficiency due to malabsorption is of particular significance in cystic fibrosis.[26] ...
Benchley died of pulmonary fibrosis on February 12, 2006 in Princeton, New Jersey.[1] ...
Swigris JJ, Brown KK (2018-07-25). Idiopathic pulmonary fibrosis. St. Louis. ISBN 978-0-323-54432-0. OCLC 1053744041. Liu Y, ... GeneReviews/NCBI/NIH/UW entry on Dyskeratosis Congenita GeneReviews/NCBI/NIH/UW entry on Pulmonary Fibrosis, Familial ... Mutations in TERC have been associated with dyskeratosis congenita, idiopathic pulmonary fibrosis, aplastic anemia, and ...
Chronic activation of fibroblasts can result in diseases such as pulmonary fibrosis, where the hardening and thickening of the ... King TE, Pardo A, Selman M (December 2011). "Idiopathic pulmonary fibrosis". Lancet. 378 (9807): 1949-61. doi:10.1016/S0140- ... April 2016). "Reduced Ets Domain-containing Protein Elk1 Promotes Pulmonary Fibrosis via Increased Integrin αvβ6 Expression". ... Thus high αvβ6 expression in fibrosis and cancer is usually associated with a poorer prognosis. Fibrosis occurs in response to ...
Goulet died from pulmonary fibrosis on October 30, 2007, at Cedars-Sinai Medical Center while awaiting a lung transplant. He ... On September 30, 2007, Goulet was hospitalized in Las Vegas, where he was diagnosed with idiopathic pulmonary fibrosis, a rare ... "Prominent People Lost to IPF/PF: Robert Goulet". Pulmonary Fibrosis Foundation. Retrieved December 9, 2011. "Robert Goulet-2006 ...
He died on December 1, 2007 of pulmonary fibrosis. Newman was 88 years old. New York Times, Section E, Page 2, Column 3, ...
Daba MH, El-Tahir KE, Al-Arifi MN, Gubara OA (June 2004). "Drug-induced pulmonary fibrosis". Saudi Medical Journal. 25 (6): 700 ... Interstitial pneumonitis can progress to pulmonary fibrosis and can be fatal. Interstitial pneumonitis with bicalutamide is ... An assessment done prior to the publication of the aforementioned study estimated the rates of pulmonary toxicity with ...
... idiopathic pulmonary fibrosis; 14% cystic fibrosis; 12% idiopathic (formerly known as "primary") pulmonary hypertension; 5% ... Pulmonary Hypertension: A Patient's Survival Guide 3rd ed. p.134. Pulmonary Hypertension: A Patient's Survival Guide 3rd ed. p ... Pulmonary Hypertension: A Patient's Survival Guide 3rd edition p. 126 Rundle, Rhonda L. (2 January 2003). "New Blood Procedure ... Lung transplantation, or pulmonary transplantation, is a surgical procedure in which a patient's diseased lungs are partially ...
He had pulmonary fibrosis. He was survived by his wife, Frieda. The ART of Euphonium Playing, Volumes I and II, published by ...
Daba MH, El-Tahir KE, Al-Arifi MN, Gubara OA (June 2004). "Drug-induced pulmonary fibrosis". Saudi Medical Journal. 25 (6): 700 ... Interstitial pneumonitis can potentially progress to pulmonary fibrosis and may be fatal. Symptoms that may indicate lung ...
CS1 maint: discouraged parameter (link) "Prominent People who have died from Pulmonary Fibrosis". Pulmonary Fibrosis Foundation ... He was suffering from pulmonary fibrosis. His funeral was held at the London Central Mosque, Regent Park, and he was laid to ...
Scadding, J. G. (1974). "Diffuse pulmonary alveolar fibrosis". Thorax. 29 (3): 271-281. doi:10.1136/thx.29.3.271. PMC 470146. ... Diffuse pulmonary alveolar fibrosis 1974 George Algernon Smart, Monitoring in medicine 1975 Allan George Williams Whitfield, ... Gee, S. (1899). "The Lumleian Lectures on Bronchitis, Pulmonary Emphysema, and Asthma". The British Medical Journal. 1 (1995): ... Pulmonary Emphysema and Asthma 1900 Walter Butler Cheadle, On Some Cirrhoses of the Liver 1901 Joseph Frank Payne, On Cancer, ...
He had pulmonary fibrosis. Cooper, Hal (October 30, 1970), Call Me Irresponsible, Robert Reed, Florence Henderson, Ann B. Davis ... Jump died on September 22, 2003 from pulmonary fibrosis, leading to respiratory failure at his home near Los Angeles, ...
Cystic fibrosis is also sometimes included in obstructive pulmonary disease. Diagnosis of obstructive disease requires several ... Chronic obstructive pulmonary disease is mainly a combination of chronic bronchitis and emphysema, but may be more or less ... Chronic obstructive pulmonary disease (COPD), also known as chronic obstructive airways disease (COAD) or chronic airflow ... The diagnosis of COPD is established through spirometry although other pulmonary function tests can be helpful. A chest x-ray ...
It was not approved to treat idiopathic pulmonary fibrosis (IPF). In 2002, the manufacturer InterMune issued a press release ... pulmonary rheumatoid nodules, and aspiration of food and other particulate material into the lung. The infectious ...
pulmonary fibrosis, Infant Respiratory Distress Syndrome, weak respiratory muscles, pneumothorax. volumes are decreased. often ... These are the basic elements of a ventilatory pulmonary function test. Determination of the residual volume is more difficult ... The results (in particular FEV1/FVC and FRC) can be used to distinguish between restrictive and obstructive pulmonary diseases ... giving an increase in pulmonary ventilation. This is necessary to meet the increased oxygen requirement of the body, which ...
He eventually developed pulmonary fibrosis, an eventually-terminal lung disease. He retired in 2013. Public officers of the ...
Williams, KJ (March 2014). "Gammaherpesviruses and Pulmonary Fibrosis: Evidence From Humans, Horses, and Rodents". Veterinary ...
Pian died of pulmonary fibrosis. After her death, she was widely eulogized in various obituaries. A memorial at Harvard was ...
Ramin died of pulmonary fibrosis. At his Sage Chapel memorial service, June 18, 1995, the overflow of mourners attested to the ...
Pneumoconiosis Silicosis Asbestosis Idiopathic pulmonary fibrosis Pulmonary fibrosis Emphysema Travis WD, King TE, Bateman ED, ... In case of idiopathic pulmonary fibrosis, certain medications like nintedanib and pirfenidone can help slow the progression. ... "Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline". American Journal of ... the clinical term for UIP of unknown cause is idiopathic pulmonary fibrosis (IPF). Examples of known causes of UIP include ...
Tucci E, Verdiani P, Di Carlo S, Sforza V (1986). "Lomustine (CCNU)-induced pulmonary fibrosis". Tumori. 72 (1): 95-8. doi: ...
In addition to inflammation, chronic graft-versus-host disease may lead to the development of fibrosis, or scar tissue, similar ... The most common severe adverse reactions were pulmonary edema/deep vein thrombosis, splenic rupture, and myocardial infarction ...
Examples include pulmonary function testing (e.g. nitrogen washout test, diffusion capacity testing (carbon monoxide, helium, ... Medication for asthma, croup, cystic fibrosis and some other conditions. Mechanism[edit]. Inhalation begins with the ...
fibrosis). External agents/. occupational. lung disease. Pneumoconiosis (Asbestosis, Baritosis, Bauxite fibrosis, Berylliosis, ... ARDS · Pulmonary edema · Löffler's syndrome/Eosinophilic pneumonia · Respiratory hypersensitivity (Allergic bronchopulmonary ...
Marik, PE (May 2011). "Pulmonary aspiration syndromes". Current Opinion in Pulmonary Medicine. 17 (3): 148-54. doi:10.1097/MCP. ... at cystic fibrosis sa Pseudomonas aeruginosa at Staphylococcus aureus.[15] Ang Streptococcus pneumoniae ay mas karaniwan sa ... pulmonary edema (pagkakaroon ng tubig sa baga), bronchiectasis (paglapad ng mga bronchi), kanser sa baga, at pulmonary emboli ( ... George, Ronald B. (2005). Chest medicine : essentials of pulmonary and critical care medicine (ika-5th ed. (na) edisyon). ...
... membranous nephropathy is known to predispose affected individuals to develop blood clots such as pulmonary emboli. Membranous ...
... pulmonary fibrosis, cystic fibrosis, and COPD. It is also helpful as part of a system of health surveillance, in which ... This test will pick up diffusion impairments, for instance in pulmonary fibrosis.[19] This must be corrected for anemia (a low ... such as pulmonary fibrosis) the FEV1 and FVC are both reduced proportionally and the value may be normal or even increased as a ... "Chapter 14.1 Pulmonary Function Testing". ACP Medicine. Decker Intellectual Properties. Retrieved 29 April 2011.. ...
fibrosis). External agents/. occupational. lung disease. Pneumoconiosis Aluminosis. Asbestosis. Baritosis. Bauxite fibrosis. ... Wikimedia Commons has media related to Chronic obstructive pulmonary disease.. *Chronic obstructive pulmonary disease at Curlie ... "Chronic obstructive pulmonary disease (COPD)". WHO. Retrieved 5 June 2019. Chronic Obstructive Pulmonary Disease (COPD) is not ... pulmonary rehabilitation appears to improve the overall quality of life and the ability to exercise.[109][110] If pulmonary ...
A minority of these patients develop late pulmonary fibrosis. Also at enhanced risk are persons with co-morbidities. Several ... 2007) Transforming growth factor (TGF)-beta1 stimulates pulmonary fibrosis and inflammation via a Bax-dependent, bid-activated ... 2007) Transforming growth factor (TGF)-beta1 stimulates pulmonary fibrosis and inflammation via a Bax-dependent, bid-activated ... 2008) Trial of prophylactic inhaled steroids to prevent or reduce pulmonary function decline, pulmonary symptoms, and airway ...
SOD may reduce free radical damage to skin-for example, to reduce fibrosis following radiation for breast cancer. Studies of ... or Chronic obstructive pulmonary disease (COPD).[42][43][44] ... Superoxide dismutase is known to reverse fibrosis, possibly ... "Topical superoxide dismutase reduces post-irradiation breast cancer fibrosis". Journal of Cellular and Molecular Medicine. 8 (1 ...
He lived in Piedmont, California and died in April 2019 at the age of 78 from complications of pulmonary fibrosis.[3] ...
鐵釩土纖維化(英语:Bauxite fibrosis). 鈹肺病(英语:Berylliosis). 類風濕塵肺綜合症(英语:Caplan's syndrome). 石末肺(英语:Chalicosis). 煤礦工人肺塵病(英语:Coalworker's ... 滑石肺(英语:Pulmonary talcosis). 綿屑肺(英语:Byssinosis). 過敏性肺炎(英语:Hypersensitivity pneumonitis) 蔗塵肺(英语:Bagassosis). 養鳥人肺(英语:Bird ... 慢性阻塞性肺病急性發作(英语:Acute
The resulting inadequate blood flow produces tubular atrophy, interstitial fibrosis, and glomerular alterations (smaller ... Light micrograph showing signs of hypertensive nephropathy: interstitial fibrosis, tubular atrophy with thickened tubular ... periglomerular fibrosis). In advanced stages, kidney failure will occur. Functional nephrons[5] have dilated tubules, often ...
A 1993-94 case study based on cases of pulmonary hemorrhage in infants in Cleveland, Ohio originally concluded there was causal ... Müller FM, Seidler M (August 2010). "Characteristics of pathogenic fungi and antifungal therapy in cystic fibrosis". Expert Rev ... August 1998). "Acute pulmonary hemorrhage in infants associated with exposure to Stachybotrys atra and other fungi". Arch ... cystic fibrosis, depression, seizure disorders, Sickle Cell disease, kidney failure, and liver failure.[13] ...
Remillard CV, Yuan JX (May 2005). "PGE2 and PAR-1 in pulmonary fibrosis: a case of biting the hand that feeds you?". American ... in pulmonary fibrosis". Biochemical Society Transactions. 30 (2): 211-6. doi:10.1042/BST0300211. PMID 12023853. Tellez C, Bar- ... "Regulation of neutrophilic inflammation by proteinase-activated receptor 1 during bacterial pulmonary infection". Journal of ...
The liver and pancreas of the fetus may also show fibrosis and/or a cystic change. ... Thomas, IT; Smith, DW (June 1974). "Oligohydramnios, cause of the nonrenal features of Potter's syndrome, including pulmonary ... These infants will eventually die as either a result of pulmonary hypoplasia or renal failure. ... pulmonary hypoplasia and cranial anomalies related to the oligohydramnios.[clarification needed] Oligohydramnios is the ...
The word tachycardia came to English from New Latin as a neoclassical compound built from the combining forms tachy- + -cardia, which are from the Greek ταχύς tachys, "quick, rapid" and καρδία, kardia, "heart". As a matter both of usage choices in the medical literature and of idiom in natural language, the words tachycardia and tachyarrhythmia are usually used interchangeably, or loosely enough that precise differentiation is not explicit. Some careful writers have tried to maintain a logical differentiation between them, which is reflected in major medical dictionaries[7][8][9] and major general dictionaries.[10][11][12] The distinction is that tachycardia be reserved for the rapid heart rate itself, regardless of cause, physiologic or pathologic (that is, from healthy response to exercise or from cardiac arrhythmia), and that tachyarrhythmia be reserved for the pathologic form (that is, an arrhythmia of the rapid rate type). This is why five of the previously referenced ...
Idir, 70, Algerian folk singer-songwriter ("A Vava Inouva") and guitarist, pulmonary fibrosis.[18] ...
Combined pulmonary fibrosis and emphysema. *Pulmonary edema. *Löffler's syndrome/Eosinophilic pneumonia. *Respiratory ... fibrosis). External agents/. occupational. lung disease. Pneumoconiosis Aluminosis. Asbestosis. Baritosis. Bauxite fibrosis. ...
Examples of single gene disorders include achondroplasia, cystic fibrosis, Duchenne muscular dystrophy, hereditary breast ... pulmonary, ophthalmologic, renal, psychiatric, and dermatologic conditions. ...
For example, rare specialized cells in the lung called pulmonary ionocytes that express the Cystic Fibrosis Transmembrane ... "A single-cell atlas of the airway epithelium reveals the CFTR-rich pulmonary ionocyte". Nature. 560 (7718): 377-381. Bibcode ...
This elevation may be caused by congenital heart disease, cor pulmonale, pulmonary fibrosis, too much erythropoietin, or ... This process occurs in the pulmonary capillaries adjacent to the alveoli of the lungs. The oxygen then travels through the ...
... idiopathic pulmonary haemosiderosis, lung abscesses, and pulmonary arteriovenous malformations. Chopin's biographers have often ... Majka, L; Goździk, J; Witt, M (2003). "Cystic fibrosis--a probable cause of Frédéric Chopin's suffering and death". Journal of ... The hypothesis that Chopin suffered from cystic fibrosis was first presented by O'Shea in 1987. Chopin possibly was sexually ... Kubba and Young pointed out a number of other conceivable, if unlikely, diagnoses, besides cystic fibrosis and alpha 1- ...
Micrograph of a heart with fibrosis (yellow) and amyloidosis (brown). Movat's stain. ...
The primary pathologic change seen in atrial fibrillation is the progressive fibrosis of the atria. This fibrosis is due ... pulmonary embolism, pneumonia, or another acute pulmonary disease ... Fibrosis is not limited to the muscle mass of the atria and may ... Mutation of the lamin AC gene is also associated with fibrosis of the atria that can lead to atrial fibrillation. ... There are also SNPs associated with loss of function of the Pitx2c gene (involved in cellular development of pulmonary valves ...
2 partial pressure more than 60 kPa can eventually lead to permanent pulmonary fibrosis.[123] Exposure to a O. 2 partial ... some disorders that cause increased pulmonary artery pressure, and any disease that impairs the body's ability to take up and ...
People with severe pulmonary fibrosis and severe emphysema with a life expectancy ,1 year should be considered poor candidates ... small-cell lung carcinoma and broncho-pulmonary carcinoid tumors.[30] With TNM staging, the cancer is classified based on the ...
2 partial pressure more than 60 kPa can eventually lead to permanent pulmonary fibrosis.[123] Exposure to a O. 2 partial ... some disorders that cause increased pulmonary artery pressure, and any disease that impairs the body's ability to take up and ...
Hypertonic saline is currently recommended by the Cystic Fibrosis Foundation as a primary part of a cystic fibrosis treatment ... may be used in perioperative fluid management protocols to reduce excessive intravenous fluid infusions and lessen pulmonary ... Reeves EP et al (2011). "Nebulized hypertonic saline decreases IL-8 in sputum of patients with cystic fibrosis." Am J Respir ... "Nebulized hypertonic saline via positive expiratory pressure versus via jet nebulizer in patients with severe cystic fibrosis ...

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