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.
Inbred C57BL mice are a strain of laboratory mice that have been produced by many generations of brother-sister matings, resulting in a high degree of genetic uniformity and homozygosity, making them widely used for biomedical research, including studies on genetics, immunology, cancer, and neuroscience.
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.
'Pleural diseases' is a broad term referring to various medical conditions that affect the pleura, the thin, double-layered membrane surrounding the lungs, including inflammation (pleurisy), effusions (excess fluid buildup), thickening, or tumors, which may cause chest pain, coughing, and breathing difficulties.
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.
'Oral Submucous Fibrosis' is a chronic, insidious, and potentially disabling condition, characterized by progressive stiffness and loss of elasticity of the oral mucosa, due to fibrotic changes in the lamina propria, often associated with juxta-epithelial inflammation and epithelial atrophy.
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.
F344 rats are an inbred strain of albino laboratory rats (Rattus norvegicus) that have been widely used in biomedical research due to their consistent and reliable genetic background, which facilitates the study of disease mechanisms and therapeutic interventions.
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)

Pulmonary fibrosis is a specific type of lung disease that results from the thickening and scarring of the lung tissues, particularly those in the alveoli (air sacs) and interstitium (the space around the air sacs). This scarring makes it harder for the lungs to properly expand and transfer oxygen into the bloodstream, leading to symptoms such as shortness of breath, coughing, fatigue, and eventually respiratory failure. The exact cause of pulmonary fibrosis can vary, with some cases being idiopathic (without a known cause) or related to environmental factors, medications, medical conditions, or genetic predisposition.

Idiopathic Pulmonary Fibrosis (IPF) is a specific type of chronic, progressive, and irreversible fibrotic lung disease of unknown cause, characterized by scarring (fibrosis) in the lungs that thickens and stiffens the lining of the air sacs (alveoli). This makes it increasingly difficult for the lungs to transfer oxygen into the bloodstream, leading to shortness of breath, cough, decreased exercise tolerance, and, eventually, respiratory failure.

The term "idiopathic" means that the cause of the disease is unknown. The diagnosis of IPF requires a combination of clinical, radiological, and pathological findings, excluding other known causes of pulmonary fibrosis. It primarily affects middle-aged to older adults, with a higher prevalence in men than women.

The progression of IPF varies from person to person, but the prognosis is generally poor, with a median survival time of 3-5 years after diagnosis. Currently, there are two FDA-approved medications for the treatment of IPF (nintedanib and pirfenidone), which can help slow down disease progression but do not cure the condition. Lung transplantation remains an option for select patients with advanced IPF.

Fibrosis is a pathological process characterized by the excessive accumulation and/or altered deposition of extracellular matrix components, particularly collagen, in various tissues and organs. This results in the formation of fibrous scar tissue that can impair organ function and structure. Fibrosis can occur as a result of chronic inflammation, tissue injury, or abnormal repair mechanisms, and it is a common feature of many diseases, including liver cirrhosis, lung fibrosis, heart failure, and kidney disease.

In medical terms, fibrosis is defined as:

"The process of producing scar tissue (consisting of collagen) in response to injury or chronic inflammation in normal connective tissue. This can lead to the thickening and stiffening of affected tissues and organs, impairing their function."

Bleomycin is a type of chemotherapeutic agent used to treat various types of cancer, including squamous cell carcinoma, testicular cancer, and lymphomas. It works by causing DNA damage in rapidly dividing cells, which can inhibit the growth and proliferation of cancer cells.

Bleomycin is an antibiotic derived from Streptomyces verticillus and is often administered intravenously or intramuscularly. While it can be effective in treating certain types of cancer, it can also have serious side effects, including lung toxicity, which can lead to pulmonary fibrosis and respiratory failure. Therefore, bleomycin should only be used under the close supervision of a healthcare professional who is experienced in administering chemotherapy drugs.

Cystic fibrosis (CF) is a genetic disorder that primarily affects the lungs and digestive system. It is caused by mutations in the CFTR gene, which regulates the movement of salt and water in and out of cells. When this gene is not functioning properly, thick, sticky mucus builds up in various organs, leading to a range of symptoms.

In the lungs, this mucus can clog the airways, making it difficult to breathe and increasing the risk of lung infections. Over time, lung damage can occur, which may lead to respiratory failure. In the digestive system, the thick mucus can prevent the release of digestive enzymes from the pancreas, impairing nutrient absorption and leading to malnutrition. CF can also affect the reproductive system, liver, and other organs.

Symptoms of cystic fibrosis may include persistent coughing, wheezing, lung infections, difficulty gaining weight, greasy stools, and frequent greasy diarrhea. The severity of the disease can vary significantly among individuals, depending on the specific genetic mutations they have inherited.

Currently, there is no cure for cystic fibrosis, but treatments are available to help manage symptoms and slow the progression of the disease. These may include airway clearance techniques, medications to thin mucus, antibiotics to treat infections, enzyme replacement therapy, and a high-calorie, high-fat diet. Lung transplantation is an option for some individuals with advanced lung disease.

A lung is a pair of spongy, elastic organs in the chest that work together to enable breathing. They are responsible for taking in oxygen and expelling carbon dioxide through the process of respiration. The left lung has two lobes, while the right lung has three lobes. The lungs are protected by the ribcage and are covered by a double-layered membrane called the pleura. The trachea divides into two bronchi, which further divide into smaller bronchioles, leading to millions of tiny air sacs called alveoli, where the exchange of gases occurs.

Hydroxyproline is not a medical term per se, but it is a significant component in the medical field, particularly in the study of connective tissues and collagen. Here's a scientific definition:

Hydroxyproline is a modified amino acid that is formed by the post-translational modification of the amino acid proline in collagen and some other proteins. This process involves the addition of a hydroxyl group (-OH) to the proline residue, which alters its chemical properties and contributes to the stability and structure of collagen fibers. Collagen is the most abundant protein in the human body and is a crucial component of connective tissues such as tendons, ligaments, skin, and bones. The presence and quantity of hydroxyproline can serve as a marker for collagen turnover and degradation, making it relevant to various medical and research contexts, including the study of diseases affecting connective tissues like osteoarthritis, rheumatoid arthritis, and Ehlers-Danlos syndrome.

Interstitial lung diseases (ILDs) are a group of disorders characterized by inflammation and scarring (fibrosis) in the interstitium, the tissue and space around the air sacs (alveoli) of the lungs. The interstitium is where the blood vessels that deliver oxygen to the lungs are located. ILDs can be caused by a variety of factors, including environmental exposures, medications, connective tissue diseases, and autoimmune disorders.

The scarring and inflammation in ILDs can make it difficult for the lungs to expand and contract normally, leading to symptoms such as shortness of breath, cough, and fatigue. The scarring can also make it harder for oxygen to move from the air sacs into the bloodstream.

There are many different types of ILDs, including:

* Idiopathic pulmonary fibrosis (IPF): a type of ILD that is caused by unknown factors and tends to progress rapidly
* Hypersensitivity pneumonitis: an ILD that is caused by an allergic reaction to inhaled substances, such as mold or bird droppings
* Connective tissue diseases: ILDs can be a complication of conditions such as rheumatoid arthritis and scleroderma
* Sarcoidosis: an inflammatory disorder that can affect multiple organs, including the lungs
* Asbestosis: an ILD caused by exposure to asbestos fibers

Treatment for ILDs depends on the specific type of disease and its underlying cause. Some treatments may include corticosteroids, immunosuppressive medications, and oxygen therapy. In some cases, a lung transplant may be necessary.

Bronchoalveolar lavage (BAL) fluid is a type of clinical specimen obtained through a procedure called bronchoalveolar lavage. This procedure involves inserting a bronchoscope into the lungs and instilling a small amount of saline solution into a specific area of the lung, then gently aspirating the fluid back out. The fluid that is recovered is called bronchoalveolar lavage fluid.

BAL fluid contains cells and other substances that are present in the lower respiratory tract, including the alveoli (the tiny air sacs where gas exchange occurs). By analyzing BAL fluid, doctors can diagnose various lung conditions, such as pneumonia, interstitial lung disease, and lung cancer. They can also monitor the effectiveness of treatments for these conditions by comparing the composition of BAL fluid before and after treatment.

BAL fluid is typically analyzed for its cellular content, including the number and type of white blood cells present, as well as for the presence of bacteria, viruses, or other microorganisms. The fluid may also be tested for various proteins, enzymes, and other biomarkers that can provide additional information about lung health and disease.

Fibroblasts are specialized cells that play a critical role in the body's immune response and wound healing process. They are responsible for producing and maintaining the extracellular matrix (ECM), which is the non-cellular component present within all tissues and organs, providing structural support and biochemical signals for surrounding cells.

Fibroblasts produce various ECM proteins such as collagens, elastin, fibronectin, and laminins, forming a complex network of fibers that give tissues their strength and flexibility. They also help in the regulation of tissue homeostasis by controlling the turnover of ECM components through the process of remodeling.

In response to injury or infection, fibroblasts become activated and start to proliferate rapidly, migrating towards the site of damage. Here, they participate in the inflammatory response, releasing cytokines and chemokines that attract immune cells to the area. Additionally, they deposit new ECM components to help repair the damaged tissue and restore its functionality.

Dysregulation of fibroblast activity has been implicated in several pathological conditions, including fibrosis (excessive scarring), cancer (where they can contribute to tumor growth and progression), and autoimmune diseases (such as rheumatoid arthritis).

Myofibroblasts are specialized cells that are present in various tissues throughout the body. They play a crucial role in wound healing and tissue repair, but they can also contribute to the development of fibrosis or scarring when their activation and proliferation persist beyond the normal healing process. Here is a medical definition of myofibroblasts:

Medical Definition of Myofibroblasts:
Myofibroblasts are modified fibroblasts that exhibit features of both smooth muscle cells and fibroblasts, including the expression of alpha-smooth muscle actin stress fibers. They are involved in the contraction of wounds, tissue remodeling, and the production of extracellular matrix components such as collagen, elastin, and fibronectin. Myofibroblasts can differentiate from various cell types, including resident fibroblasts, epithelial cells (epithelial-mesenchymal transition), endothelial cells (endothelial-mesenchymal transition), and circulating fibrocytes. Persistent activation of myofibroblasts can lead to excessive scarring and fibrosis in various organs, such as the lungs, liver, kidneys, and heart.

Pulmonary alveoli, also known as air sacs, are tiny clusters of air-filled pouches located at the end of the bronchioles in the lungs. They play a crucial role in the process of gas exchange during respiration. The thin walls of the alveoli, called alveolar membranes, allow oxygen from inhaled air to pass into the bloodstream and carbon dioxide from the bloodstream to pass into the alveoli to be exhaled out of the body. This vital function enables the lungs to supply oxygen-rich blood to the rest of the body and remove waste products like carbon dioxide.

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) is a protein that functions as a chloride channel in the membranes of various cells, including those in the lungs and pancreas. Mutations in the gene encoding CFTR can lead to Cystic Fibrosis, a genetic disorder characterized by thick, sticky mucus in the lungs and other organs, leading to severe respiratory and digestive problems.

CFTR is normally activated by cyclic AMP-dependent protein kinase (PKA) and regulates the movement of chloride ions across cell membranes. In Cystic Fibrosis, mutations in CFTR can result in impaired channel function or reduced amounts of functional CFTR at the cell surface, leading to an imbalance in ion transport and fluid homeostasis. This can cause the production of thick, sticky mucus that clogs the airways and leads to chronic lung infections, as well as other symptoms associated with Cystic Fibrosis.

Extrinsic allergic alveolitis is a type of lung inflammation that occurs in response to inhaling organic dusts or mold spores that contain allergens. It is also known as hypersensitivity pneumonitis. This condition typically affects people who have been repeatedly exposed to the allergen over a period of time, such as farmers, bird fanciers, and workers in certain industries.

The symptoms of extrinsic allergic alveolitis can vary but often include cough, shortness of breath, fever, and fatigue. These symptoms may develop gradually or suddenly, depending on the frequency and intensity of exposure to the allergen. In some cases, the condition may progress to cause permanent lung damage if it is not treated promptly.

Diagnosis of extrinsic allergic alveolitis typically involves a combination of medical history, physical examination, imaging studies such as chest X-rays or CT scans, and pulmonary function tests. In some cases, blood tests or bronchoscopy with lavage may also be used to help confirm the diagnosis.

Treatment for extrinsic allergic alveolitis typically involves avoiding further exposure to the allergen, as well as using medications such as corticosteroids to reduce inflammation and relieve symptoms. In severe cases, hospitalization and oxygen therapy may be necessary. With prompt and appropriate treatment, most people with extrinsic allergic alveolitis can recover fully and avoid long-term lung damage.

Collagen is the most abundant protein in the human body, and it is a major component of connective tissues such as tendons, ligaments, skin, and bones. Collagen provides structure and strength to these tissues and helps them to withstand stretching and tension. It is made up of long chains of amino acids, primarily glycine, proline, and hydroxyproline, which are arranged in a triple helix structure. There are at least 16 different types of collagen found in the body, each with slightly different structures and functions. Collagen is important for maintaining the integrity and health of tissues throughout the body, and it has been studied for its potential therapeutic uses in various medical conditions.

Collagen diseases, also known as collagen disorders or connective tissue diseases, refer to a group of medical conditions that affect the body's connective tissues. These tissues provide support and structure for various organs and systems in the body, including the skin, joints, muscles, and blood vessels.

Collagen is a major component of connective tissues, and it plays a crucial role in maintaining their strength and elasticity. In collagen diseases, the body's immune system mistakenly attacks healthy collagen, leading to inflammation, pain, and damage to the affected tissues.

There are several types of collagen diseases, including:

1. Systemic Lupus Erythematosus (SLE): This is a chronic autoimmune disease that can affect various organs and systems in the body, including the skin, joints, kidneys, heart, and lungs.
2. Rheumatoid Arthritis (RA): This is a chronic inflammatory disease that primarily affects the joints, causing pain, swelling, and stiffness.
3. Scleroderma: This is a rare autoimmune disorder that causes thickening and hardening of the skin and connective tissues, leading to restricted movement and organ damage.
4. Dermatomyositis: This is an inflammatory muscle disease that can also affect the skin, causing rashes and weakness.
5. Mixed Connective Tissue Disease (MCTD): This is a rare autoimmune disorder that combines symptoms of several collagen diseases, including SLE, RA, scleroderma, and dermatomyositis.

The exact cause of collagen diseases is not fully understood, but they are believed to be related to genetic, environmental, and hormonal factors. Treatment typically involves a combination of medications, lifestyle changes, and physical therapy to manage symptoms and prevent complications.

Transforming Growth Factor-beta (TGF-β) is a type of cytokine, which is a cell signaling protein involved in the regulation of various cellular processes, including cell growth, differentiation, and apoptosis (programmed cell death). TGF-β plays a critical role in embryonic development, tissue homeostasis, and wound healing. It also has been implicated in several pathological conditions such as fibrosis, cancer, and autoimmune diseases.

TGF-β exists in multiple isoforms (TGF-β1, TGF-β2, and TGF-β3) that are produced by many different cell types, including immune cells, epithelial cells, and fibroblasts. The protein is synthesized as a precursor molecule, which is cleaved to release the active TGF-β peptide. Once activated, TGF-β binds to its receptors on the cell surface, leading to the activation of intracellular signaling pathways that regulate gene expression and cell behavior.

In summary, Transforming Growth Factor-beta (TGF-β) is a multifunctional cytokine involved in various cellular processes, including cell growth, differentiation, apoptosis, embryonic development, tissue homeostasis, and wound healing. It has been implicated in several pathological conditions such as fibrosis, cancer, and autoimmune diseases.

Systemic Scleroderma, also known as Systemic Sclerosis (SSc), is a rare, chronic autoimmune disease that involves the abnormal growth and accumulation of collagen in various connective tissues, blood vessels, and organs throughout the body. This excessive collagen production leads to fibrosis or scarring, which can cause thickening, hardening, and tightening of the skin and damage to internal organs such as the heart, lungs, kidneys, and gastrointestinal tract.

Systemic Scleroderma is characterized by two main features: small blood vessel abnormalities (Raynaud's phenomenon) and fibrosis. The disease can be further classified into two subsets based on the extent of skin involvement: limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc).

Limited cutaneous systemic sclerosis affects the skin distally, typically involving fingers, hands, forearms, feet, lower legs, and face. It is often associated with Raynaud's phenomenon, calcinosis, telangiectasias, and pulmonary arterial hypertension.

Diffuse cutaneous systemic sclerosis involves more extensive skin thickening and fibrosis that spreads proximally to affect the trunk, upper arms, thighs, and face. It is commonly associated with internal organ involvement, such as interstitial lung disease, heart disease, and kidney problems.

The exact cause of Systemic Scleroderma remains unknown; however, it is believed that genetic, environmental, and immunological factors contribute to its development. There is currently no cure for Systemic Scleroderma, but various treatments can help manage symptoms, slow disease progression, and improve quality of life.

Endomyocardial fibrosis is a rare heart condition characterized by the thickening and scarring (fibrosis) of the inner layer of the heart muscle (endocardium) and the muscular walls of the lower chambers of the heart (ventricles). This process can restrict the heart's ability to fill properly with blood, leading to symptoms such as shortness of breath, fatigue, and fluid retention. The exact cause of endomyocardial fibrosis is not fully understood, but it is believed to involve an abnormal immune response or inflammation. It is more commonly found in tropical regions of Africa and Asia. Treatment typically involves medications to manage symptoms and improve heart function, as well as potentially surgical interventions to remove the scar tissue and restore normal heart function.

I'm sorry for any confusion, but "Peplomycin" is not a widely recognized or established medical term. It appears that it might be a term related to a specific type of antibiotic drug called "Pleuromutilin." Pleuromutilins are a class of antibiotics derived from certain types of fungi. Peplomycin could potentially be a specific formulation, brand name, or experimental version of a pleuromutilin antibiotic, but without more context, it is difficult to provide a precise definition. I would recommend consulting the original source or seeking additional information for clarification.

C57BL/6 (C57 Black 6) is an inbred strain of laboratory mouse that is widely used in biomedical research. The term "inbred" refers to a strain of animals where matings have been carried out between siblings or other closely related individuals for many generations, resulting in a population that is highly homozygous at most genetic loci.

The C57BL/6 strain was established in 1920 by crossing a female mouse from the dilute brown (DBA) strain with a male mouse from the black strain. The resulting offspring were then interbred for many generations to create the inbred C57BL/6 strain.

C57BL/6 mice are known for their robust health, longevity, and ease of handling, making them a popular choice for researchers. They have been used in a wide range of biomedical research areas, including studies of cancer, immunology, neuroscience, cardiovascular disease, and metabolism.

One of the most notable features of the C57BL/6 strain is its sensitivity to certain genetic modifications, such as the introduction of mutations that lead to obesity or impaired glucose tolerance. This has made it a valuable tool for studying the genetic basis of complex diseases and traits.

Overall, the C57BL/6 inbred mouse strain is an important model organism in biomedical research, providing a valuable resource for understanding the genetic and molecular mechanisms underlying human health and disease.

Respiratory Function Tests (RFTs) are a group of medical tests that measure how well your lungs take in and exhale air, and how well they transfer oxygen and carbon dioxide into and out of your blood. They can help diagnose certain lung disorders, measure the severity of lung disease, and monitor response to treatment.

RFTs include several types of tests, such as:

1. Spirometry: This test measures how much air you can exhale and how quickly you can do it. It's often used to diagnose and monitor conditions like asthma, chronic obstructive pulmonary disease (COPD), and other lung diseases.
2. Lung volume testing: This test measures the total amount of air in your lungs. It can help diagnose restrictive lung diseases, such as pulmonary fibrosis or sarcoidosis.
3. Diffusion capacity testing: This test measures how well oxygen moves from your lungs into your bloodstream. It's often used to diagnose and monitor conditions like pulmonary fibrosis, interstitial lung disease, and other lung diseases that affect the ability of the lungs to transfer oxygen to the blood.
4. Bronchoprovocation testing: This test involves inhaling a substance that can cause your airways to narrow, such as methacholine or histamine. It's often used to diagnose and monitor asthma.
5. Exercise stress testing: This test measures how well your lungs and heart work together during exercise. It's often used to diagnose lung or heart disease.

Overall, Respiratory Function Tests are an important tool for diagnosing and managing a wide range of lung conditions.

Transforming Growth Factor-beta 1 (TGF-β1) is a cytokine that belongs to the TGF-β superfamily. It is a multifunctional protein involved in various cellular processes, including cell growth, differentiation, apoptosis, and extracellular matrix production. TGF-β1 plays crucial roles in embryonic development, tissue homeostasis, and repair, as well as in pathological conditions such as fibrosis and cancer. It signals through a heteromeric complex of type I and type II serine/threonine kinase receptors, leading to the activation of intracellular signaling pathways, primarily the Smad-dependent pathway. TGF-β1 has context-dependent functions, acting as a tumor suppressor in normal and early-stage cancer cells but promoting tumor progression and metastasis in advanced cancers.

Animal disease models are specialized animals, typically rodents such as mice or rats, that have been genetically engineered or exposed to certain conditions to develop symptoms and physiological changes similar to those seen in human diseases. These models are used in medical research to study the pathophysiology of diseases, identify potential therapeutic targets, test drug efficacy and safety, and understand disease mechanisms.

The genetic modifications can include knockout or knock-in mutations, transgenic expression of specific genes, or RNA interference techniques. The animals may also be exposed to environmental factors such as chemicals, radiation, or infectious agents to induce the disease state.

Examples of animal disease models include:

1. Mouse models of cancer: Genetically engineered mice that develop various types of tumors, allowing researchers to study cancer initiation, progression, and metastasis.
2. Alzheimer's disease models: Transgenic mice expressing mutant human genes associated with Alzheimer's disease, which exhibit amyloid plaque formation and cognitive decline.
3. Diabetes models: Obese and diabetic mouse strains like the NOD (non-obese diabetic) or db/db mice, used to study the development of type 1 and type 2 diabetes, respectively.
4. Cardiovascular disease models: Atherosclerosis-prone mice, such as ApoE-deficient or LDLR-deficient mice, that develop plaque buildup in their arteries when fed a high-fat diet.
5. Inflammatory bowel disease models: Mice with genetic mutations affecting intestinal barrier function and immune response, such as IL-10 knockout or SAMP1/YitFc mice, which develop colitis.

Animal disease models are essential tools in preclinical research, but it is important to recognize their limitations. Differences between species can affect the translatability of results from animal studies to human patients. Therefore, researchers must carefully consider the choice of model and interpret findings cautiously when applying them to human diseases.

Lung transplantation is a surgical procedure where one or both diseased lungs are removed and replaced with healthy lungs from a deceased donor. It is typically considered as a treatment option for patients with end-stage lung diseases, such as chronic obstructive pulmonary disease (COPD), cystic fibrosis, idiopathic pulmonary fibrosis, and alpha-1 antitrypsin deficiency, who have exhausted all other medical treatments and continue to suffer from severe respiratory failure.

The procedure involves several steps, including evaluating the patient's eligibility for transplantation, matching the donor's lung size and blood type with the recipient, and performing the surgery under general anesthesia. After the surgery, patients require close monitoring and lifelong immunosuppressive therapy to prevent rejection of the new lungs.

Lung transplantation can significantly improve the quality of life and survival rates for some patients with end-stage lung disease, but it is not without risks, including infection, bleeding, and rejection. Therefore, careful consideration and thorough evaluation are necessary before pursuing this treatment option.

Sarcoidosis, pulmonary is a specific form of sarcoidosis, which is a multisystem inflammatory disorder characterized by the formation of noncaseating granulomas (small clusters of immune cells) in one or more organs. In pulmonary sarcoidosis, these granulomas primarily affect the lungs, but can also involve the lymph nodes within the chest. The condition is often asymptomatic, but some individuals may experience symptoms such as cough, shortness of breath, chest pain, and fatigue. Pulmonary sarcoidosis can lead to complications like pulmonary fibrosis (scarring of lung tissue) and chronic interstitial lung disease, which can impact lung function and quality of life. The exact cause of sarcoidosis is unknown, but it is believed to involve an abnormal immune response triggered by exposure to certain antigens, such as environmental particles or infectious agents.

Pulmonary emphysema is a chronic respiratory disease characterized by abnormal, permanent enlargement of the airspaces distal to the terminal bronchioles, accompanied by destruction of their walls and without obvious fibrosis. This results in loss of elastic recoil, which leads to trappling of air within the lungs and difficulty exhaling. It is often caused by cigarette smoking or long-term exposure to harmful pollutants. The disease is part of a group of conditions known as chronic obstructive pulmonary disease (COPD), which also includes chronic bronchitis.

Antibiotics are a type of medication used to treat infections caused by bacteria. They work by either killing the bacteria or inhibiting their growth.

Antineoplastics, also known as chemotherapeutic agents, are a class of drugs used to treat cancer. These medications target and destroy rapidly dividing cells, such as cancer cells, although they can also affect other quickly dividing cells in the body, such as those in the hair follicles or digestive tract, which can lead to side effects.

Antibiotics and antineoplastics are two different classes of drugs with distinct mechanisms of action and uses. It is important to use them appropriately and under the guidance of a healthcare professional.

Liver cirrhosis is a chronic, progressive disease characterized by the replacement of normal liver tissue with scarred (fibrotic) tissue, leading to loss of function. The scarring is caused by long-term damage from various sources such as hepatitis, alcohol abuse, nonalcoholic fatty liver disease, and other causes. As the disease advances, it can lead to complications like portal hypertension, fluid accumulation in the abdomen (ascites), impaired brain function (hepatic encephalopathy), and increased risk of liver cancer. It is generally irreversible, but early detection and treatment of underlying causes may help slow down its progression.

Bronchoalveolar lavage (BAL) is a medical procedure in which a small amount of fluid is introduced into a segment of the lung and then gently suctioned back out. The fluid contains cells and other materials that can be analyzed to help diagnose various lung conditions, such as inflammation, infection, or cancer.

The procedure is typically performed during bronchoscopy, which involves inserting a thin, flexible tube with a light and camera on the end through the nose or mouth and into the lungs. Once the bronchoscope is in place, a small catheter is passed through the bronchoscope and into the desired lung segment. The fluid is then introduced and suctioned back out, and the sample is sent to a laboratory for analysis.

BAL can be helpful in diagnosing various conditions such as pneumonia, interstitial lung diseases, alveolar proteinosis, and some types of cancer. It can also be used to monitor the effectiveness of treatment for certain lung conditions. However, like any medical procedure, it carries some risks, including bleeding, infection, and respiratory distress. Therefore, it is important that the procedure is performed by a qualified healthcare professional in a controlled setting.

Hermanski-Pudlak Syndrome (HPS) is a rare genetic disorder characterized by the triad of albinism, bleeding disorders, and lysosomal storage disease. It is caused by mutations in any one of several genes involved in biogenesis of lysosome-related organelles (LROs), such as melanosomes in melanocytes, platelet dense granules, and lung lamellar bodies.

The albinism in HPS results from abnormal melanosome biogenesis, leading to decreased pigmentation in the skin, hair, and eyes. The bleeding disorder is due to defective platelet dense granules, which are necessary for normal clotting function. This can result in prolonged bleeding times and easy bruising.

The lysosomal storage disease component of HPS is characterized by the accumulation of ceroid lipofuscin within LROs, leading to progressive damage to affected tissues. The most common form of HPS (HPS-1) also involves pulmonary fibrosis, which can lead to respiratory failure and death in the third or fourth decade of life.

There are currently seven known subtypes of HPS, each caused by mutations in different genes involved in LRO biogenesis. The clinical features and severity of HPS can vary widely between subtypes and even within families with the same genetic mutation.

Pyridones are a class of organic compounds that contain a pyridone ring, which is a heterocyclic ring consisting of a six-membered ring with five carbon atoms and one nitrogen atom, with one oxygen atom attached to the nitrogen atom by a double bond. Pyridones can be found in various natural sources, including plants and microorganisms, and they also have important applications in the pharmaceutical industry as building blocks for drug design and synthesis. Some drugs that contain pyridone rings include antihistamines, anti-inflammatory agents, and antiviral agents.

Asbestosis is a chronic lung disease that is caused by the inhalation of asbestos fibers. It is characterized by scarring (fibrosis) of the lung tissue, which can lead to symptoms such as shortness of breath, coughing, and chest pain. The severity of the disease can range from mild to severe, and it is often progressive, meaning that it tends to worsen over time. Asbestosis is not a malignant condition, but it can increase the risk of developing lung cancer or mesothelioma, which are forms of cancer that are associated with asbestos exposure. The disease is typically diagnosed through a combination of medical history, physical examination, and imaging tests such as chest X-rays or CT scans. There is no cure for asbestosis, but treatment can help to manage the symptoms and slow the progression of the disease.

Retroperitoneal fibrosis (RPF) is a rare and progressive condition characterized by the abnormal growth of fibrous tissue in the retroperitoneal space, which is the area behind the peritoneum (the lining that covers the abdominal cavity). This fibrous tissue can encase and compress vital structures such as the ureters, blood vessels, and nerves, leading to various symptoms.

RPF can be idiopathic (without a known cause) or secondary to other conditions like infections, malignancies, autoimmune diseases, or medications. The exact pathogenesis of RPF is not fully understood, but it's believed that an abnormal immune response and inflammation play significant roles in its development.

Symptoms of RPF may include:

1. Flank pain or back pain
2. Renal insufficiency or kidney failure due to ureteral compression
3. Hydronephrosis (dilatation of the renal pelvis and calyces)
4. Deep vein thrombosis (DVT) or pulmonary embolism (PE) due to vascular compression
5. Neurological symptoms due to nerve compression
6. Weight loss, fatigue, and fever (in some cases)

Diagnosis of RPF typically involves imaging studies such as computed tomography (CT) scans or magnetic resonance imaging (MRI), along with laboratory tests and sometimes biopsy for confirmation. Treatment options depend on the underlying cause but generally involve immunosuppressive medications, corticosteroids, and surgical intervention in severe cases.

Pulmonary surfactant-associated protein C (SP-C) is a small hydrophobic protein that is a component of pulmonary surfactant. Surfactant is a complex mixture of lipids and proteins that reduces surface tension in the alveoli of the lungs, preventing collapse during expiration and facilitating lung expansion during inspiration. SP-C plays a crucial role in maintaining the structural integrity and stability of the surfactant film at the air-liquid interface of the alveoli.

Deficiency or dysfunction of SP-C has been associated with several pulmonary diseases, including respiratory distress syndrome (RDS) in premature infants, interstitial lung diseases (ILDs), and pulmonary fibrosis. Mutations in the gene encoding SP-C (SFTPC) can lead to abnormal protein processing and accumulation, resulting in lung injury and inflammation, ultimately contributing to the development of these conditions.

Alveolar macrophages are a type of macrophage (a large phagocytic cell) that are found in the alveoli of the lungs. They play a crucial role in the immune defense system of the lungs by engulfing and destroying any foreign particles, such as dust, microorganisms, and pathogens, that enter the lungs through the process of inhalation. Alveolar macrophages also produce cytokines, which are signaling molecules that help to coordinate the immune response. They are important for maintaining the health and function of the lungs by removing debris and preventing infection.

Pneumocytes are specialized epithelial cells that line the alveoli, which are the tiny air sacs in the lungs where gas exchange occurs. There are two main types of pneumocytes: type I and type II.

Type I pneumocytes are flat, thin cells that cover about 95% of the alveolar surface area. They play a crucial role in facilitating the diffusion of oxygen and carbon dioxide between the alveoli and the bloodstream. Type I pneumocytes also contribute to maintaining the structural integrity of the alveoli.

Type II pneumocytes are smaller, more cuboidal cells that produce and secrete surfactant, a substance composed of proteins and lipids that reduces surface tension within the alveoli, preventing their collapse and facilitating breathing. Type II pneumocytes can also function as progenitor cells, capable of differentiating into type I pneumocytes to help repair damaged lung tissue.

In summary, pneumocytes are essential for maintaining proper gas exchange in the lungs and contributing to the overall health and functioning of the respiratory system.

Asbestos is a group of naturally occurring mineral fibers that are resistant to heat, chemical reactions, and electrical currents. There are six types of asbestos, but the most common ones are chrysotile, amosite, and crocidolite. Asbestos has been widely used in various construction materials, such as roofing shingles, ceiling and floor tiles, paper products, and cement products.

Exposure to asbestos can cause serious health problems, including lung cancer, mesothelioma (a rare form of cancer that affects the lining of the lungs, heart, or abdomen), and asbestosis (a chronic lung disease characterized by scarring of the lung tissue). These health risks are related to the inhalation of asbestos fibers, which can become lodged in the lungs and cause inflammation and scarring over time.

As a result, the use of asbestos has been heavily regulated in many countries, and its use is banned in several others. Despite these regulations, asbestos remains a significant public health concern due to the large number of buildings and products that still contain it.

Collagen Type III, also known as Collagen III Alpha 1 (COL3A1), is a type of collagen that is found in various connective tissues throughout the body. It is a fibrillar collagen that is produced by fibroblasts and is a major component of reticular fibers, which provide structural support to organs such as the liver, spleen, and lymph nodes. Collagen Type III is also found in the walls of blood vessels, the skin, and the intestinal tract.

Mutations in the COL3A1 gene can lead to a rare genetic disorder called Ehlers-Danlos syndrome type IV, which is characterized by fragile and elastic skin, easy bruising, and spontaneous rupture of blood vessels. Collagen Type III has been studied for its potential role in various other medical conditions, including fibrosis, cancer, and cardiovascular disease.

Idiopathic interstitial pneumonias (IIPs) are a group of rare lung diseases with no known cause, characterized by inflammation and scarring (fibrosis) of the lung tissue. The term "idiopathic" means that the cause is unknown, and "interstitial" refers to the spaces between the air sacs in the lungs where the inflammation and scarring occur.

IIPs are classified into several subtypes based on their clinical, radiological, and pathological features. These include:

1. Idiopathic Pulmonary Fibrosis (IPF): This is the most common and aggressive form of IIP, characterized by progressive scarring of the lung tissue, which leads to difficulty breathing and decreased lung function over time.
2. Nonspecific Interstitial Pneumonia (NSIP): This subtype is characterized by varying degrees of inflammation and fibrosis in the lung tissue. NSIP can be idiopathic or associated with connective tissue diseases.
3. Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD): This subtype primarily affects smokers and is characterized by inflammation of the small airways and surrounding lung tissue.
4. Desquamative Interstitial Pneumonia (DIP): This subtype is also more common in smokers and is characterized by accumulation of pigmented macrophages in the lung tissue.
5. Cryptogenic Organizing Pneumonia (COP): This subtype is characterized by the formation of fibrous masses in the small airways and alveoli, leading to cough and shortness of breath.
6. Acute Interstitial Pneumonia (AIP)/Acute Respiratory Distress Syndrome (ARDS): This subtype is a severe form of IIP that can rapidly progress to respiratory failure and requires immediate medical attention.

The diagnosis of IIPs typically involves a combination of clinical evaluation, imaging studies, and lung biopsy. Treatment options may include corticosteroids, immunosuppressive medications, and oxygen therapy, depending on the severity and subtype of the disease.

Lung diseases refer to a broad category of disorders that affect the lungs and other structures within the respiratory system. These diseases can impair lung function, leading to symptoms such as coughing, shortness of breath, chest pain, and wheezing. They can be categorized into several types based on the underlying cause and nature of the disease process. Some common examples include:

1. Obstructive lung diseases: These are characterized by narrowing or blockage of the airways, making it difficult to breathe out. Examples include chronic obstructive pulmonary disease (COPD), asthma, bronchiectasis, and cystic fibrosis.
2. Restrictive lung diseases: These involve stiffening or scarring of the lungs, which reduces their ability to expand and take in air. Examples include idiopathic pulmonary fibrosis, sarcoidosis, and asbestosis.
3. Infectious lung diseases: These are caused by bacteria, viruses, fungi, or parasites that infect the lungs. Examples include pneumonia, tuberculosis, and influenza.
4. Vascular lung diseases: These affect the blood vessels in the lungs, impairing oxygen exchange. Examples include pulmonary embolism, pulmonary hypertension, and chronic thromboembolic pulmonary hypertension (CTEPH).
5. Neoplastic lung diseases: These involve abnormal growth of cells within the lungs, leading to cancer. Examples include small cell lung cancer, non-small cell lung cancer, and mesothelioma.
6. Other lung diseases: These include interstitial lung diseases, pleural effusions, and rare disorders such as pulmonary alveolar proteinosis and lymphangioleiomyomatosis (LAM).

It is important to note that this list is not exhaustive, and there are many other conditions that can affect the lungs. Proper diagnosis and treatment of lung diseases require consultation with a healthcare professional, such as a pulmonologist or respiratory therapist.

Vital capacity (VC) is a term used in pulmonary function tests to describe the maximum volume of air that can be exhaled after taking a deep breath. It is the sum of inspiratory reserve volume, tidal volume, and expiratory reserve volume. In other words, it's the total amount of air you can forcibly exhale after inhaling as deeply as possible. Vital capacity is an important measurement in assessing lung function and can be reduced in conditions such as chronic obstructive pulmonary disease (COPD), asthma, and other respiratory disorders.

Lung injury, also known as pulmonary injury, refers to damage or harm caused to the lung tissue, blood vessels, or air sacs (alveoli) in the lungs. This can result from various causes such as infection, trauma, exposure to harmful substances, or systemic diseases. Common types of lung injuries include acute respiratory distress syndrome (ARDS), pneumonia, and chemical pneumonitis. Symptoms may include difficulty breathing, cough, chest pain, and decreased oxygen levels in the blood. Treatment depends on the underlying cause and may include medications, oxygen therapy, or mechanical ventilation.

Collagen Type I is the most abundant form of collagen in the human body, found in various connective tissues such as tendons, ligaments, skin, and bones. It is a structural protein that provides strength and integrity to these tissues. Collagen Type I is composed of three alpha chains, two alpha-1(I) chains, and one alpha-2(I) chain, arranged in a triple helix structure. This type of collagen is often used in medical research and clinical applications, such as tissue engineering and regenerative medicine, due to its excellent mechanical properties and biocompatibility.

Cryptogenic organizing pneumonia (COP) is a type of lung disorder that is characterized by the presence of inflammation and scarring in the lungs. The term "cryptogenic" means that the cause of the condition is unknown or unclear.

Organizing pneumonia is a specific pattern of injury to the lungs that can be caused by various factors, including infections, medications, and autoimmune disorders. However, in cases of COP, there is no clear underlying cause that can be identified.

The main symptoms of COP include cough, shortness of breath, fever, and fatigue. The condition can also cause crackles or wheezing sounds when listening to the lungs with a stethoscope. Diagnosis of COP typically involves a combination of imaging studies, such as chest X-rays or CT scans, and lung biopsy.

Treatment for COP usually involves the use of corticosteroids, which can help to reduce inflammation and improve symptoms. In some cases, other medications may also be used to manage the condition. The prognosis for people with COP is generally good, with most individuals responding well to treatment and experiencing improvement in their symptoms over time. However, recurrence of the condition is possible, and long-term monitoring may be necessary.

Pulmonary diffusing capacity, also known as pulmonary diffusion capacity, is a measure of the ability of the lungs to transfer gas from the alveoli to the bloodstream. It is often used to assess the severity of lung diseases such as chronic obstructive pulmonary disease (COPD) and pulmonary fibrosis.

The most common measurement of pulmonary diffusing capacity is the diffusing capacity for carbon monoxide (DLCO), which reflects the transfer of carbon monoxide from the alveoli to the red blood cells in the capillaries. The DLCO is measured during a spirometry test, which involves breathing in a small amount of carbon monoxide and then measuring how much of it is exhaled.

A reduced DLCO may indicate a problem with the lung's ability to transfer oxygen to the blood, which can be caused by a variety of factors including damage to the alveoli or capillaries, thickening of the alveolar membrane, or a decrease in the surface area available for gas exchange.

It is important to note that other factors such as hemoglobin concentration, carboxyhemoglobin level, and lung volume can also affect the DLCO value, so these should be taken into account when interpreting the results of a diffusing capacity test.

Respiratory mucosa refers to the mucous membrane that lines the respiratory tract, including the nose, throat, bronchi, and lungs. It is a specialized type of tissue that is composed of epithelial cells, goblet cells, and glands that produce mucus, which helps to trap inhaled particles such as dust, allergens, and pathogens.

The respiratory mucosa also contains cilia, tiny hair-like structures that move rhythmically to help propel the mucus and trapped particles out of the airways and into the upper part of the throat, where they can be swallowed or coughed up. This defense mechanism is known as the mucociliary clearance system.

In addition to its role in protecting the respiratory tract from harmful substances, the respiratory mucosa also plays a crucial role in immune function by containing various types of immune cells that help to detect and respond to pathogens and other threats.

A "knockout" mouse is a genetically engineered mouse in which one or more genes have been deleted or "knocked out" using molecular biology techniques. This allows researchers to study the function of specific genes and their role in various biological processes, as well as potential associations with human diseases. The mice are generated by introducing targeted DNA modifications into embryonic stem cells, which are then used to create a live animal. Knockout mice have been widely used in biomedical research to investigate gene function, disease mechanisms, and potential therapeutic targets.

Pneumonia is an infection or inflammation of the alveoli (tiny air sacs) in one or both lungs. It's often caused by bacteria, viruses, or fungi. Accumulated pus and fluid in these air sacs make it difficult to breathe, which can lead to coughing, chest pain, fever, and difficulty breathing. The severity of symptoms can vary from mild to life-threatening, depending on the underlying cause, the patient's overall health, and age. Pneumonia is typically diagnosed through a combination of physical examination, medical history, and diagnostic tests such as chest X-rays or blood tests. Treatment usually involves antibiotics for bacterial pneumonia, antivirals for viral pneumonia, and supportive care like oxygen therapy, hydration, and rest.

Disease progression is the worsening or advancement of a medical condition over time. It refers to the natural course of a disease, including its development, the severity of symptoms and complications, and the impact on the patient's overall health and quality of life. Understanding disease progression is important for developing appropriate treatment plans, monitoring response to therapy, and predicting outcomes.

The rate of disease progression can vary widely depending on the type of medical condition, individual patient factors, and the effectiveness of treatment. Some diseases may progress rapidly over a short period of time, while others may progress more slowly over many years. In some cases, disease progression may be slowed or even halted with appropriate medical interventions, while in other cases, the progression may be inevitable and irreversible.

In clinical practice, healthcare providers closely monitor disease progression through regular assessments, imaging studies, and laboratory tests. This information is used to guide treatment decisions and adjust care plans as needed to optimize patient outcomes and improve quality of life.

A biopsy is a medical procedure in which a small sample of tissue is taken from the body to be examined under a microscope for the presence of disease. This can help doctors diagnose and monitor various medical conditions, such as cancer, infections, or autoimmune disorders. The type of biopsy performed will depend on the location and nature of the suspected condition. Some common types of biopsies include:

1. Incisional biopsy: In this procedure, a surgeon removes a piece of tissue from an abnormal area using a scalpel or other surgical instrument. This type of biopsy is often used when the lesion is too large to be removed entirely during the initial biopsy.

2. Excisional biopsy: An excisional biopsy involves removing the entire abnormal area, along with a margin of healthy tissue surrounding it. This technique is typically employed for smaller lesions or when cancer is suspected.

3. Needle biopsy: A needle biopsy uses a thin, hollow needle to extract cells or fluid from the body. There are two main types of needle biopsies: fine-needle aspiration (FNA) and core needle biopsy. FNA extracts loose cells, while a core needle biopsy removes a small piece of tissue.

4. Punch biopsy: In a punch biopsy, a round, sharp tool is used to remove a small cylindrical sample of skin tissue. This type of biopsy is often used for evaluating rashes or other skin abnormalities.

5. Shave biopsy: During a shave biopsy, a thin slice of tissue is removed from the surface of the skin using a sharp razor-like instrument. This technique is typically used for superficial lesions or growths on the skin.

After the biopsy sample has been collected, it is sent to a laboratory where a pathologist will examine the tissue under a microscope and provide a diagnosis based on their findings. The results of the biopsy can help guide further treatment decisions and determine the best course of action for managing the patient's condition.

Silicon dioxide is not a medical term, but a chemical compound with the formula SiO2. It's commonly known as quartz or sand and is not something that would typically have a medical definition. However, in some cases, silicon dioxide can be used in pharmaceutical preparations as an excipient (an inactive substance that serves as a vehicle or medium for a drug) or as a food additive, often as an anti-caking agent.

In these contexts, it's important to note that silicon dioxide is considered generally recognized as safe (GRAS) by the U.S. Food and Drug Administration (FDA). However, exposure to very high levels of respirable silica dust, such as in certain industrial settings, can increase the risk of lung disease, including silicosis.

Sarcoidosis is a multi-system disorder characterized by the formation of granulomas (small clumps of inflammatory cells) in various organs, most commonly the lungs and lymphatic system. These granulomas can impair the function of the affected organ(s), leading to a variety of symptoms. The exact cause of sarcoidosis is unknown, but it's thought to be an overactive immune response to an unknown antigen, possibly triggered by an infection, chemical exposure, or another environmental factor.

The diagnosis of sarcoidosis typically involves a combination of clinical evaluation, imaging studies (such as chest X-rays and CT scans), and laboratory tests (including blood tests and biopsies). While there is no cure for sarcoidosis, treatment may be necessary to manage symptoms and prevent complications. Corticosteroids are often used to suppress the immune system and reduce inflammation, while other medications may be prescribed to treat specific organ involvement or symptoms. In some cases, sarcoidosis may resolve on its own without any treatment.

Epithelial cells are types of cells that cover the outer surfaces of the body, line the inner surfaces of organs and glands, and form the lining of blood vessels and body cavities. They provide a protective barrier against the external environment, regulate the movement of materials between the internal and external environments, and are involved in the sense of touch, temperature, and pain. Epithelial cells can be squamous (flat and thin), cuboidal (square-shaped and of equal height), or columnar (tall and narrow) in shape and are classified based on their location and function.

Silicosis is a lung disease caused by inhalation of crystalline silica dust. It is characterized by the formation of nodular lesions and fibrosis (scarring) in the upper lobes of the lungs, which can lead to symptoms such as shortness of breath, cough, and fatigue. The severity of the disease depends on the duration and intensity of exposure to silica dust. Chronic silicosis is the most common form and develops after prolonged exposure, while acute silicosis can occur after brief, intense exposures. There is no cure for silicosis, and treatment is focused on managing symptoms and preventing further lung damage.

HSP47 (Heat Shock Protein 47) is a type of molecular chaperone that assists in the proper folding and assembly of collagen molecules within the endoplasmic reticulum (ER) of eukaryotic cells. It is also known as SERPINH1, which stands for serine protease inhibitor, clade H (heat shock protein 47).

HSP47 binds to procollagen molecules in a highly specific manner and helps facilitate their correct folding and assembly into higher-order structures. Once the collagen molecules are properly assembled, HSP47 dissociates from them and allows for their transport out of the ER and further processing in the Golgi apparatus.

HSP47 is upregulated under conditions of cellular stress, such as heat shock or oxidative stress, which can lead to an accumulation of misfolded proteins within the ER. This upregulation helps to enhance the protein folding capacity of the ER and prevent the aggregation of misfolded proteins, thereby maintaining cellular homeostasis.

Defects in HSP47 function have been implicated in various connective tissue disorders, such as osteogenesis imperfecta and Ehlers-Danlos syndrome, which are characterized by abnormal collagen structure and function.

Respiratory system agents are substances that affect the respiratory system, which includes the nose, throat (pharynx), voice box (larynx), windpipe (trachea), bronchi, and lungs. These agents can be classified into different categories based on their effects:

1. Respiratory Stimulants: Agents that increase respiratory rate or depth by acting on the respiratory center in the brainstem.
2. Respiratory Depressants: Agents that decrease respiratory rate or depth, often as a side effect of their sedative or analgesic effects. Examples include opioids, benzodiazepines, and barbiturates.
3. Bronchodilators: Agents that widen the airways (bronchioles) in the lungs by relaxing the smooth muscle around them. They are used to treat asthma, chronic obstructive pulmonary disease (COPD), and other respiratory conditions. Examples include albuterol, ipratropium, and theophylline.
4. Anti-inflammatory Agents: Agents that reduce inflammation in the airways, which can help relieve symptoms of asthma, COPD, and other respiratory conditions. Examples include corticosteroids, leukotriene modifiers, and mast cell stabilizers.
5. Antitussives: Agents that suppress coughing, often by numbing the throat or acting on the cough center in the brainstem. Examples include dextromethorphan and codeine.
6. Expectorants: Agents that help thin and loosen mucus in the airways, making it easier to cough up and clear. Examples include guaifenesin and iodinated glycerol.
7. Decongestants: Agents that narrow blood vessels in the nose and throat, which can help relieve nasal congestion and sinus pressure. Examples include pseudoephedrine and phenylephrine.
8. Antimicrobial Agents: Agents that kill or inhibit the growth of microorganisms such as bacteria, viruses, and fungi that can cause respiratory infections. Examples include antibiotics, antiviral drugs, and antifungal agents.

A biological marker, often referred to as a biomarker, is a measurable indicator that reflects the presence or severity of a disease state, or a response to a therapeutic intervention. Biomarkers can be found in various materials such as blood, tissues, or bodily fluids, and they can take many forms, including molecular, histologic, radiographic, or physiological measurements.

In the context of medical research and clinical practice, biomarkers are used for a variety of purposes, such as:

1. Diagnosis: Biomarkers can help diagnose a disease by indicating the presence or absence of a particular condition. For example, prostate-specific antigen (PSA) is a biomarker used to detect prostate cancer.
2. Monitoring: Biomarkers can be used to monitor the progression or regression of a disease over time. For instance, hemoglobin A1c (HbA1c) levels are monitored in diabetes patients to assess long-term blood glucose control.
3. Predicting: Biomarkers can help predict the likelihood of developing a particular disease or the risk of a negative outcome. For example, the presence of certain genetic mutations can indicate an increased risk for breast cancer.
4. Response to treatment: Biomarkers can be used to evaluate the effectiveness of a specific treatment by measuring changes in the biomarker levels before and after the intervention. This is particularly useful in personalized medicine, where treatments are tailored to individual patients based on their unique biomarker profiles.

It's important to note that for a biomarker to be considered clinically valid and useful, it must undergo rigorous validation through well-designed studies, including demonstrating sensitivity, specificity, reproducibility, and clinical relevance.

Pulmonary hypertension is a medical condition characterized by increased blood pressure in the pulmonary arteries, which are the blood vessels that carry blood from the right side of the heart to the lungs. This results in higher than normal pressures in the pulmonary circulation and can lead to various symptoms and complications.

Pulmonary hypertension is typically defined as a mean pulmonary artery pressure (mPAP) greater than or equal to 25 mmHg at rest, as measured by right heart catheterization. The World Health Organization (WHO) classifies pulmonary hypertension into five groups based on the underlying cause:

1. Pulmonary arterial hypertension (PAH): This group includes idiopathic PAH, heritable PAH, drug-induced PAH, and associated PAH due to conditions such as connective tissue diseases, HIV infection, portal hypertension, congenital heart disease, and schistosomiasis.
2. Pulmonary hypertension due to left heart disease: This group includes conditions that cause elevated left atrial pressure, such as left ventricular systolic or diastolic dysfunction, valvular heart disease, and congenital cardiovascular shunts.
3. Pulmonary hypertension due to lung diseases and/or hypoxia: This group includes chronic obstructive pulmonary disease (COPD), interstitial lung disease, sleep-disordered breathing, alveolar hypoventilation disorders, and high altitude exposure.
4. Chronic thromboembolic pulmonary hypertension (CTEPH): This group includes persistent obstruction of the pulmonary arteries due to organized thrombi or emboli.
5. Pulmonary hypertension with unclear and/or multifactorial mechanisms: This group includes hematologic disorders, systemic disorders, metabolic disorders, and other conditions that can cause pulmonary hypertension but do not fit into the previous groups.

Symptoms of pulmonary hypertension may include shortness of breath, fatigue, chest pain, lightheadedness, and syncope (fainting). Diagnosis typically involves a combination of medical history, physical examination, imaging studies, and invasive testing such as right heart catheterization. Treatment depends on the underlying cause but may include medications, oxygen therapy, pulmonary rehabilitation, and, in some cases, surgical intervention.

Smad3 protein is a transcription factor that plays a crucial role in the TGF-β (transforming growth factor-beta) signaling pathway. When TGF-β binds to its receptor, it activates Smad3 through phosphorylation. Activated Smad3 then forms a complex with other Smad proteins and translocates into the nucleus where it regulates the transcription of target genes involved in various cellular processes such as proliferation, differentiation, apoptosis, and migration.

Mutations in the SMAD3 gene or dysregulation of the TGF-β/Smad3 signaling pathway have been implicated in several human diseases, including fibrotic disorders, cancer, and Marfan syndrome. Therefore, Smad3 protein is an important target for therapeutic interventions in these conditions.

"Cells, cultured" is a medical term that refers to cells that have been removed from an organism and grown in controlled laboratory conditions outside of the body. This process is called cell culture and it allows scientists to study cells in a more controlled and accessible environment than they would have inside the body. Cultured cells can be derived from a variety of sources, including tissues, organs, or fluids from humans, animals, or cell lines that have been previously established in the laboratory.

Cell culture involves several steps, including isolation of the cells from the tissue, purification and characterization of the cells, and maintenance of the cells in appropriate growth conditions. The cells are typically grown in specialized media that contain nutrients, growth factors, and other components necessary for their survival and proliferation. Cultured cells can be used for a variety of purposes, including basic research, drug development and testing, and production of biological products such as vaccines and gene therapies.

It is important to note that cultured cells may behave differently than they do in the body, and results obtained from cell culture studies may not always translate directly to human physiology or disease. Therefore, it is essential to validate findings from cell culture experiments using additional models and ultimately in clinical trials involving human subjects.

Immunohistochemistry (IHC) is a technique used in pathology and laboratory medicine to identify specific proteins or antigens in tissue sections. It combines the principles of immunology and histology to detect the presence and location of these target molecules within cells and tissues. This technique utilizes antibodies that are specific to the protein or antigen of interest, which are then tagged with a detection system such as a chromogen or fluorophore. The stained tissue sections can be examined under a microscope, allowing for the visualization and analysis of the distribution and expression patterns of the target molecule in the context of the tissue architecture. Immunohistochemistry is widely used in diagnostic pathology to help identify various diseases, including cancer, infectious diseases, and immune-mediated disorders.

Total Lung Capacity (TLC) is the maximum volume of air that can be contained within the lungs at the end of a maximal inspiration. It includes all of the following lung volumes: tidal volume, inspiratory reserve volume, expiratory reserve volume, and residual volume. TLC can be measured directly using gas dilution techniques or indirectly by adding residual volume to vital capacity. Factors that affect TLC include age, sex, height, and lung health status.

Connective Tissue Growth Factor (CTGF) is a cysteine-rich peptide growth factor that belongs to the CCN family of proteins. It plays an important role in various biological processes, including cell adhesion, migration, proliferation, and extracellular matrix production. CTGF is involved in wound healing, tissue repair, and fibrosis, as well as in the pathogenesis of several diseases such as cancer, diabetic nephropathy, and systemic sclerosis. It is expressed in response to various stimuli, including growth factors, cytokines, and mechanical stress. CTGF interacts with a variety of signaling molecules and integrins to regulate cellular responses and tissue homeostasis.

X-ray computed tomography (CT or CAT scan) is a medical imaging method that uses computer-processed combinations of many X-ray images taken from different angles to produce cross-sectional (tomographic) images (virtual "slices") of the body. These cross-sectional images can then be used to display detailed internal views of organs, bones, and soft tissues in the body.

The term "computed tomography" is used instead of "CT scan" or "CAT scan" because the machines take a series of X-ray measurements from different angles around the body and then use a computer to process these data to create detailed images of internal structures within the body.

CT scanning is a noninvasive, painless medical test that helps physicians diagnose and treat medical conditions. CT imaging provides detailed information about many types of tissue including lung, bone, soft tissue and blood vessels. CT examinations can be performed on every part of the body for a variety of reasons including diagnosis, surgical planning, and monitoring of therapeutic responses.

In computed tomography (CT), an X-ray source and detector rotate around the patient, measuring the X-ray attenuation at many different angles. A computer uses this data to construct a cross-sectional image by the process of reconstruction. This technique is called "tomography". The term "computed" refers to the use of a computer to reconstruct the images.

CT has become an important tool in medical imaging and diagnosis, allowing radiologists and other physicians to view detailed internal images of the body. It can help identify many different medical conditions including cancer, heart disease, lung nodules, liver tumors, and internal injuries from trauma. CT is also commonly used for guiding biopsies and other minimally invasive procedures.

In summary, X-ray computed tomography (CT or CAT scan) is a medical imaging technique that uses computer-processed combinations of many X-ray images taken from different angles to produce cross-sectional images of the body. It provides detailed internal views of organs, bones, and soft tissues in the body, allowing physicians to diagnose and treat medical conditions.

Messenger RNA (mRNA) is a type of RNA (ribonucleic acid) that carries genetic information copied from DNA in the form of a series of three-base code "words," each of which specifies a particular amino acid. This information is used by the cell's machinery to construct proteins, a process known as translation. After being transcribed from DNA, mRNA travels out of the nucleus to the ribosomes in the cytoplasm where protein synthesis occurs. Once the protein has been synthesized, the mRNA may be degraded and recycled. Post-transcriptional modifications can also occur to mRNA, such as alternative splicing and addition of a 5' cap and a poly(A) tail, which can affect its stability, localization, and translation efficiency.

Mucin-5B, also known as MUC5B, is a type of mucin protein that is heavily glycosylated and found in the respiratory tract. It is one of the major components of airway mucus, which helps to trap and remove inhaled particles and microorganisms from the lungs.

Mucin-5B is a large molecular weight gel-forming mucin that is produced by goblet cells and submucosal glands in the respiratory epithelium. It has a complex structure, consisting of a protein backbone with numerous oligosaccharide side chains that give it its gel-like properties.

Mutations in the MUC5B gene have been associated with several lung diseases, including chronic obstructive pulmonary disease (COPD), bronchiectasis, and idiopathic pulmonary fibrosis (IPF). In particular, a common genetic variant in the MUC5B promoter region has been identified as a significant risk factor for developing IPF.

Radiation pneumonitis is a inflammatory reaction in the lung tissue that occurs as a complication of thoracic radiation therapy. It usually develops 1-3 months following the completion of radiation treatment. The symptoms can range from mild to severe and may include cough, shortness of breath, fever, and chest discomfort. In severe cases, it can lead to fibrosis (scarring) of the lung tissue, which can cause permanent lung damage. Radiation pneumonitis is diagnosed through a combination of clinical symptoms, imaging studies such as chest X-ray or CT scan, and sometimes through bronchoscopy with lavage. Treatment typically involves corticosteroids to reduce inflammation and supportive care to manage symptoms.

The extracellular matrix (ECM) is a complex network of biomolecules that provides structural and biochemical support to cells in tissues and organs. It is composed of various proteins, glycoproteins, and polysaccharides, such as collagens, elastin, fibronectin, laminin, and proteoglycans. The ECM plays crucial roles in maintaining tissue architecture, regulating cell behavior, and facilitating communication between cells. It provides a scaffold for cell attachment, migration, and differentiation, and helps to maintain the structural integrity of tissues by resisting mechanical stresses. Additionally, the ECM contains various growth factors, cytokines, and chemokines that can influence cellular processes such as proliferation, survival, and differentiation. Overall, the extracellular matrix is essential for the normal functioning of tissues and organs, and its dysregulation can contribute to various pathological conditions, including fibrosis, cancer, and degenerative diseases.

Pulmonary Surfactant-Associated Protein A (SP-A) is a protein that is a major component of pulmonary surfactant, which is a complex mixture of lipids and proteins found in the alveoli of the lungs. SP-A is produced by specialized cells called type II alveolar epithelial cells and has several important functions in the lung.

SP-A plays a role in innate immunity by binding to pathogens, such as bacteria and viruses, and facilitating their clearance from the lungs. It also helps to regulate surfactant homeostasis by participating in the reuptake and recycling of surfactant components. Additionally, SP-A has been shown to have anti-inflammatory effects and may help to modulate the immune response in the lung.

Deficiencies or mutations in SP-A have been associated with various respiratory diseases, including acute respiratory distress syndrome (ARDS), pulmonary fibrosis, and chronic obstructive pulmonary disease (COPD).

Pleural diseases refer to conditions that affect the pleura, which is the thin, double-layered membrane that surrounds the lungs and lines the inside of the chest wall. The space between these two layers contains a small amount of fluid that helps the lungs move smoothly during breathing. Pleural diseases can cause inflammation, infection, or abnormal collections of fluid in the pleural space, leading to symptoms such as chest pain, cough, and difficulty breathing.

Some common examples of pleural diseases include:

1. Pleurisy: Inflammation of the pleura that causes sharp chest pain, often worsened by breathing or coughing.
2. Pleural effusion: An abnormal accumulation of fluid in the pleural space, which can be caused by various underlying conditions such as heart failure, pneumonia, cancer, or autoimmune disorders.
3. Empyema: A collection of pus in the pleural space, usually resulting from a bacterial infection.
4. Pleural thickening: Scarring and hardening of the pleura, which can restrict lung function and cause breathlessness.
5. Mesothelioma: A rare form of cancer that affects the pleura, often caused by exposure to asbestos.
6. Pneumothorax: A collection of air in the pleural space, which can result from trauma or a rupture of the lung tissue.

Proper diagnosis and treatment of pleural diseases require a thorough evaluation by a healthcare professional, often involving imaging tests such as chest X-rays or CT scans, as well as fluid analysis or biopsy if necessary.

Actin is a type of protein that forms part of the contractile apparatus in muscle cells, and is also found in various other cell types. It is a globular protein that polymerizes to form long filaments, which are important for many cellular processes such as cell division, cell motility, and the maintenance of cell shape. In muscle cells, actin filaments interact with another type of protein called myosin to enable muscle contraction. Actins can be further divided into different subtypes, including alpha-actin, beta-actin, and gamma-actin, which have distinct functions and expression patterns in the body.

Signal transduction is the process by which a cell converts an extracellular signal, such as a hormone or neurotransmitter, into an intracellular response. This involves a series of molecular events that transmit the signal from the cell surface to the interior of the cell, ultimately resulting in changes in gene expression, protein activity, or metabolism.

The process typically begins with the binding of the extracellular signal to a receptor located on the cell membrane. This binding event activates the receptor, which then triggers a cascade of intracellular signaling molecules, such as second messengers, protein kinases, and ion channels. These molecules amplify and propagate the signal, ultimately leading to the activation or inhibition of specific cellular responses.

Signal transduction pathways are highly regulated and can be modulated by various factors, including other signaling molecules, post-translational modifications, and feedback mechanisms. Dysregulation of these pathways has been implicated in a variety of diseases, including cancer, diabetes, and neurological disorders.

Oral Submucous Fibrosis (OSF) is a chronic, progressive, and potentially disabling disease that affects the oral soft tissues. It is characterized by inflammation and fibrosis (excessive deposition of collagen) of the submucosal tissues, leading to stiffness and limitation of mouth opening, tongue movement, and occasionally swallowing or speaking difficulties. The condition primarily affects individuals with a history of areca nut (betel nut) chewing, although other factors such as smoking, alcohol consumption, and genetic predisposition may also contribute to its development. Symptoms can include burning sensation in the mouth, dryness, and pain during speaking, eating, or swallowing. In severe cases, OSF can lead to significant functional impairment and require surgical intervention.

Ambroxol is a medication that belongs to the class of drugs known as mucolytic agents or expectorants. It works by thinning and loosening mucus in the airways, making it easier to cough up and clear the airways. This can help reduce symptoms such as chest congestion and shortness of breath in conditions such as chronic bronchitis, bronchiectasis, and cystic fibrosis.

Ambroxol also has some additional properties that make it useful in treating respiratory conditions. It can help to reduce inflammation in the airways, reduce the production of reactive oxygen species (which can damage cells), and increase the activity of certain immune cells that help to fight infection. These effects may contribute to the overall benefits of ambroxol in managing respiratory diseases.

It is important to note that ambroxol should only be used under the guidance of a healthcare professional, as it can have side effects and interactions with other medications. The dosage and duration of treatment will depend on various factors, including the underlying condition being treated, the patient's age and overall health status, and any other medical conditions or medications they may be taking.

Connective tissue diseases (CTDs) are a group of disorders that involve the abnormal production and accumulation of abnormal connective tissues in various parts of the body. Connective tissues are the structural materials that support and bind other tissues and organs together. They include tendons, ligaments, cartilage, fat, and the material that fills the spaces between cells, called the extracellular matrix.

Connective tissue diseases can affect many different systems in the body, including the skin, joints, muscles, lungs, kidneys, gastrointestinal tract, and blood vessels. Some CTDs are autoimmune disorders, meaning that the immune system mistakenly attacks healthy connective tissues. Others may be caused by genetic mutations or environmental factors.

Some examples of connective tissue diseases include:

* Systemic lupus erythematosus (SLE)
* Rheumatoid arthritis (RA)
* Scleroderma
* Dermatomyositis/Polymyositis
* Mixed Connective Tissue Disease (MCTD)
* Sjogren's syndrome
* Ehlers-Danlos syndrome
* Marfan syndrome
* Osteogenesis imperfecta

The specific symptoms and treatment of connective tissue diseases vary depending on the type and severity of the condition. Treatment may include medications to reduce inflammation, suppress the immune system, or manage pain. In some cases, surgery may be necessary to repair or replace damaged tissues or organs.

Epithelial-mesenchymal transition (EMT) is a biological process that involves the transformation of epithelial cells into mesenchymal cells. This process is characterized by distinct changes in cell shape, behavior, and molecular markers.

Epithelial cells are typically tightly packed together and have a polarized structure with distinct apical and basal surfaces. In contrast, mesenchymal cells are elongated, spindle-shaped cells that can migrate and invade surrounding tissues.

During EMT, epithelial cells lose their polarity and cell-to-cell adhesion molecules, such as E-cadherin, and acquire mesenchymal markers, such as vimentin and N-cadherin. This transition enables the cells to become more motile and invasive, which is critical for embryonic development, wound healing, and cancer metastasis.

EMT is a complex process that involves various signaling pathways, including TGF-β, Wnt, Notch, and Hedgehog, among others. Dysregulation of EMT has been implicated in several diseases, particularly cancer, where it contributes to tumor progression, metastasis, and drug resistance.

In the field of medicine, "time factors" refer to the duration of symptoms or time elapsed since the onset of a medical condition, which can have significant implications for diagnosis and treatment. Understanding time factors is crucial in determining the progression of a disease, evaluating the effectiveness of treatments, and making critical decisions regarding patient care.

For example, in stroke management, "time is brain," meaning that rapid intervention within a specific time frame (usually within 4.5 hours) is essential to administering tissue plasminogen activator (tPA), a clot-busting drug that can minimize brain damage and improve patient outcomes. Similarly, in trauma care, the "golden hour" concept emphasizes the importance of providing definitive care within the first 60 minutes after injury to increase survival rates and reduce morbidity.

Time factors also play a role in monitoring the progression of chronic conditions like diabetes or heart disease, where regular follow-ups and assessments help determine appropriate treatment adjustments and prevent complications. In infectious diseases, time factors are crucial for initiating antibiotic therapy and identifying potential outbreaks to control their spread.

Overall, "time factors" encompass the significance of recognizing and acting promptly in various medical scenarios to optimize patient outcomes and provide effective care.

Dyspnea is defined as difficulty or discomfort in breathing, often described as shortness of breath. It can range from mild to severe, and may occur during rest, exercise, or at any time. Dyspnea can be caused by various medical conditions, including heart and lung diseases, anemia, and neuromuscular disorders. It is important to seek medical attention if experiencing dyspnea, as it can be a sign of a serious underlying condition.

Experimental liver cirrhosis refers to a controlled research setting where various factors and substances are intentionally introduced to induce liver cirrhosis in animals or cell cultures. The purpose is to study the mechanisms, progression, potential treatments, and prevention strategies for liver cirrhosis. This could involve administering chemicals, drugs, alcohol, viruses, or manipulating genes associated with liver damage and fibrosis. It's important to note that results from experimental models may not directly translate to human conditions, but they can provide valuable insights into disease pathophysiology and therapeutic development.

Smad2 protein is a transcription factor that plays a critical role in the TGF-β (transforming growth factor-beta) signaling pathway, which regulates various cellular processes such as proliferation, differentiation, and apoptosis. Smad2 is primarily localized in the cytoplasm and becomes phosphorylated upon TGF-β receptor activation. Once phosphorylated, it forms a complex with Smad4 and translocates to the nucleus where it regulates the transcription of target genes. Mutations in the Smad2 gene have been associated with various human diseases, including cancer and fibrotic disorders.

Respiratory aspiration is defined as the entry of foreign materials (such as food, liquids, or vomit) into the lower respiratory tract during swallowing, which includes the trachea and lungs. This can lead to respiratory complications such as pneumonia, bronchitis, or lung abscesses. Aspiration can occur in individuals with impaired swallowing function due to various conditions like neurological disorders, stroke, or anesthesia.

Inflammation is a complex biological response of tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. It is characterized by the following signs: rubor (redness), tumor (swelling), calor (heat), dolor (pain), and functio laesa (loss of function). The process involves the activation of the immune system, recruitment of white blood cells, and release of inflammatory mediators, which contribute to the elimination of the injurious stimuli and initiation of the healing process. However, uncontrolled or chronic inflammation can also lead to tissue damage and diseases.

Procollagen is the precursor protein of collagen, which is a major structural protein in the extracellular matrix of various connective tissues, such as tendons, ligaments, skin, and bones. Procollagen is synthesized inside the cell (in the rough endoplasmic reticulum) and then processed by enzymes to remove specific segments, resulting in the formation of tropocollagen, which are the basic units of collagen fibrils.

Procollagen consists of three polypeptide chains (two alpha-1 and one alpha-2 chain), each containing a central triple-helical domain flanked by non-helical regions at both ends. These non-helical regions, called propeptides, are cleaved off during the processing of procollagen to tropocollagen, allowing the individual collagen molecules to align and form fibrils through covalent cross-linking.

Abnormalities in procollagen synthesis or processing can lead to various connective tissue disorders, such as osteogenesis imperfecta (brittle bone disease) and Ehlers-Danlos syndrome (a group of disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility).

Expectorants are a type of medication that help to thin and loosen mucus in the airways, making it easier to cough up and clear the airways. They work by increasing the production of fluid in the respiratory tract, which helps to moisten and soften thick or sticky mucus. This makes it easier for the cilia (tiny hair-like structures that line the airways) to move the mucus out of the lungs and into the throat, where it can be swallowed or spit out.

Expectorants are often used to treat respiratory conditions such as bronchitis, pneumonia, and chronic obstructive pulmonary disease (COPD), which can cause excessive mucus production and difficulty breathing. Some common expectorants include guaifenesin, iodinated glycerol, and potassium iodide.

It is important to follow the dosage instructions carefully when taking expectorants, as taking too much can lead to side effects such as nausea, vomiting, and diarrhea. It is also important to drink plenty of fluids while taking expectorants, as this can help to thin the mucus and make it easier to cough up.

'Gene expression regulation' refers to the processes that control whether, when, and where a particular gene is expressed, meaning the production of a specific protein or functional RNA encoded by that gene. This complex mechanism can be influenced by various factors such as transcription factors, chromatin remodeling, DNA methylation, non-coding RNAs, and post-transcriptional modifications, among others. Proper regulation of gene expression is crucial for normal cellular function, development, and maintaining homeostasis in living organisms. Dysregulation of gene expression can lead to various diseases, including cancer and genetic disorders.

Retrospective studies, also known as retrospective research or looking back studies, are a type of observational study that examines data from the past to draw conclusions about possible causal relationships between risk factors and outcomes. In these studies, researchers analyze existing records, medical charts, or previously collected data to test a hypothesis or answer a specific research question.

Retrospective studies can be useful for generating hypotheses and identifying trends, but they have limitations compared to prospective studies, which follow participants forward in time from exposure to outcome. Retrospective studies are subject to biases such as recall bias, selection bias, and information bias, which can affect the validity of the results. Therefore, retrospective studies should be interpreted with caution and used primarily to generate hypotheses for further testing in prospective studies.

A Severity of Illness Index is a measurement tool used in healthcare to assess the severity of a patient's condition and the risk of mortality or other adverse outcomes. These indices typically take into account various physiological and clinical variables, such as vital signs, laboratory values, and co-morbidities, to generate a score that reflects the patient's overall illness severity.

Examples of Severity of Illness Indices include the Acute Physiology and Chronic Health Evaluation (APACHE) system, the Simplified Acute Physiology Score (SAPS), and the Mortality Probability Model (MPM). These indices are often used in critical care settings to guide clinical decision-making, inform prognosis, and compare outcomes across different patient populations.

It is important to note that while these indices can provide valuable information about a patient's condition, they should not be used as the sole basis for clinical decision-making. Rather, they should be considered in conjunction with other factors, such as the patient's overall clinical presentation, treatment preferences, and goals of care.

Pneumoconiosis is a group of lung diseases caused by inhaling dust particles, leading to fibrosis or scarring of the lungs. The type of pneumoconiosis depends on the specific dust inhaled. Examples include coal worker's pneumoconiosis (from coal dust), silicosis (from crystalline silica dust), and asbestosis (from asbestos fibers). These diseases are generally preventable by minimizing exposure to harmful dusts through proper engineering controls, protective equipment, and workplace safety regulations.

Cytokines are a broad and diverse category of small signaling proteins that are secreted by various cells, including immune cells, in response to different stimuli. They play crucial roles in regulating the immune response, inflammation, hematopoiesis, and cellular communication.

Cytokines mediate their effects by binding to specific receptors on the surface of target cells, which triggers intracellular signaling pathways that ultimately result in changes in gene expression, cell behavior, and function. Some key functions of cytokines include:

1. Regulating the activation, differentiation, and proliferation of immune cells such as T cells, B cells, natural killer (NK) cells, and macrophages.
2. Coordinating the inflammatory response by recruiting immune cells to sites of infection or tissue damage and modulating their effector functions.
3. Regulating hematopoiesis, the process of blood cell formation in the bone marrow, by controlling the proliferation, differentiation, and survival of hematopoietic stem and progenitor cells.
4. Modulating the development and function of the nervous system, including neuroinflammation, neuroprotection, and neuroregeneration.

Cytokines can be classified into several categories based on their structure, function, or cellular origin. Some common types of cytokines include interleukins (ILs), interferons (IFNs), tumor necrosis factors (TNFs), chemokines, colony-stimulating factors (CSFs), and transforming growth factors (TGFs). Dysregulation of cytokine production and signaling has been implicated in various pathological conditions, such as autoimmune diseases, chronic inflammation, cancer, and neurodegenerative disorders.

Gene expression is the process by which the information encoded in a gene is used to synthesize a functional gene product, such as a protein or RNA molecule. This process involves several steps: transcription, RNA processing, and translation. During transcription, the genetic information in DNA is copied into a complementary RNA molecule, known as messenger RNA (mRNA). The mRNA then undergoes RNA processing, which includes adding a cap and tail to the mRNA and splicing out non-coding regions called introns. The resulting mature mRNA is then translated into a protein on ribosomes in the cytoplasm through the process of translation.

The regulation of gene expression is a complex and highly controlled process that allows cells to respond to changes in their environment, such as growth factors, hormones, and stress signals. This regulation can occur at various stages of gene expression, including transcriptional activation or repression, RNA processing, mRNA stability, and translation. Dysregulation of gene expression has been implicated in many diseases, including cancer, genetic disorders, and neurological conditions.

Transgenic mice are genetically modified rodents that have incorporated foreign DNA (exogenous DNA) into their own genome. This is typically done through the use of recombinant DNA technology, where a specific gene or genetic sequence of interest is isolated and then introduced into the mouse embryo. The resulting transgenic mice can then express the protein encoded by the foreign gene, allowing researchers to study its function in a living organism.

The process of creating transgenic mice usually involves microinjecting the exogenous DNA into the pronucleus of a fertilized egg, which is then implanted into a surrogate mother. The offspring that result from this procedure are screened for the presence of the foreign DNA, and those that carry the desired genetic modification are used to establish a transgenic mouse line.

Transgenic mice have been widely used in biomedical research to model human diseases, study gene function, and test new therapies. They provide a valuable tool for understanding complex biological processes and developing new treatments for a variety of medical conditions.

Smad proteins are a family of intracellular signaling molecules that play a crucial role in the transmission of signals from the cell surface to the nucleus in response to transforming growth factor β (TGF-β) superfamily ligands. These ligands include TGF-βs, bone morphogenetic proteins (BMPs), activins, and inhibins.

There are eight mammalian Smad proteins, which are categorized into three classes based on their function: receptor-regulated Smads (R-Smads), common mediator Smads (Co-Smads), and inhibitory Smads (I-Smads). R-Smads include Smad1, Smad2, Smad3, Smad5, and Smad8/9, while Smad4 is the only Co-Smad. The I-Smads consist of Smad6 and Smad7.

Upon TGF-β superfamily ligand binding to their transmembrane serine/threonine kinase receptors, R-Smads are phosphorylated and form complexes with Co-Smad4. These complexes then translocate into the nucleus, where they regulate the transcription of target genes involved in various cellular processes, such as proliferation, differentiation, apoptosis, migration, and extracellular matrix production. I-Smads act as negative regulators of TGF-β signaling by competing with R-Smads for receptor binding or promoting the degradation of receptors and R-Smads.

Dysregulation of Smad protein function has been implicated in various human diseases, including fibrosis, cancer, and developmental disorders.

Diffuse scleroderma is a medical condition that falls under the systemic sclerosis category of autoimmune rheumatic diseases. It is characterized by thickening and hardening (sclerosis) of the skin and involvement of internal organs. In diffuse scleroderma, the process affects extensive areas of the skin and at least one internal organ.

The disease process involves an overproduction of collagen, a protein that makes up connective tissues in the body. This excessive collagen deposition leads to fibrosis (scarring) of the skin and various organs, including the esophagus, gastrointestinal tract, heart, lungs, and kidneys.

Diffuse scleroderma can present with a rapid progression of skin thickening within the first few years after onset. The skin involvement may extend to areas beyond the hands, feet, and face, which are commonly affected in limited scleroderma (another form of systemic sclerosis). Additionally, patients with diffuse scleroderma have a higher risk for severe internal organ complications compared to those with limited scleroderma.

Early diagnosis and appropriate management of diffuse scleroderma are crucial to prevent or slow down the progression of organ damage. Treatment typically involves a multidisciplinary approach, focusing on symptom management, immunosuppressive therapy, and addressing specific organ involvement.

Paraquat is a highly toxic herbicide that is used for controlling weeds and grasses in agricultural settings. It is a non-selective contact weed killer, meaning it kills any green plant it comes into contact with. Paraquat is a fast-acting chemical that causes rapid desiccation of plant tissues upon contact.

In a medical context, paraquat is classified as a toxicological emergency and can cause severe poisoning in humans if ingested, inhaled, or comes into contact with the skin or eyes. Paraquat poisoning can lead to multiple organ failure, including the lungs, kidneys, and liver, and can be fatal in severe cases. There is no specific antidote for paraquat poisoning, and treatment typically focuses on supportive care and managing symptoms.

It's important to note that paraquat is highly regulated and its use is restricted to licensed professionals due to its high toxicity. Proper protective equipment, including gloves, goggles, and respiratory protection, should be used when handling paraquat to minimize the risk of exposure.

Interleukin-13 (IL-13) is a cytokine that plays a crucial role in the immune response, particularly in the development of allergic inflammation and hypersensitivity reactions. It is primarily produced by activated Th2 cells, mast cells, basophils, and eosinophils. IL-13 mediates its effects through binding to the IL-13 receptor complex, which consists of the IL-13Rα1 and IL-4Rα chains.

IL-13 has several functions in the body, including:

* Regulation of IgE production by B cells
* Induction of eosinophil differentiation and activation
* Inhibition of proinflammatory cytokine production by macrophages
* Promotion of mucus production and airway hyperresponsiveness in the lungs, contributing to the pathogenesis of asthma.

Dysregulation of IL-13 has been implicated in various diseases, such as allergic asthma, atopic dermatitis, and chronic rhinosinusitis. Therefore, targeting IL-13 with biologic therapies has emerged as a promising approach for the treatment of these conditions.

Telomerase is an enzyme that adds repetitive DNA sequences (telomeres) to the ends of chromosomes, which are lost during each cell division due to the incomplete replication of the ends of linear chromosomes. Telomerase is not actively present in most somatic cells, but it is highly expressed in germ cells and stem cells, allowing them to divide indefinitely. However, in many types of cancer cells, telomerase is abnormally activated, which leads to the maintenance or lengthening of telomeres, contributing to their unlimited replicative potential and tumorigenesis.

Gammaherpesvirinae is a subfamily of herpesviruses, which are double-stranded DNA viruses that can establish lifelong infections in their hosts. Gammaherpesvirinae includes two genera: Lymphocryptovirus and Rhadinovirus.

Lymphocryptovirus genus contains the human herpesvirus 4 (HHV-4), also known as Epstein-Barr virus (EBV), which is a major cause of infectious mononucleosis and is associated with several malignancies, including Burkitt's lymphoma, Hodgkin's lymphoma, nasopharyngeal carcinoma, and gastric cancer.

Rhadinovirus genus contains the human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus (KSHV), which is associated with several malignancies, including Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman's disease.

Gammaherpesviruses primarily infect B cells and epithelial cells, and they can establish latency in their host cells, allowing them to evade the immune system and persist for the lifetime of the host. Infection with these viruses has been linked to various diseases, ranging from benign conditions such as infectious mononucleosis to malignancies such as lymphomas and carcinomas.

Hepatic stellate cells, also known as Ito cells or lipocytes, are specialized perisinusoidal cells located in the space of Disse in the liver. They play a crucial role in maintaining the normal architecture and function of the liver. In response to liver injury or disease, these cells can become activated and transform into myofibroblasts, which produce extracellular matrix components and contribute to fibrosis and scarring in the liver. This activation process is regulated by various signaling pathways and mediators, including cytokines, growth factors, and oxidative stress. Hepatic stellate cells also have the ability to store vitamin A and lipids, which they can release during activation to support hepatocyte function and regeneration.

A phenotype is the physical or biochemical expression of an organism's genes, or the observable traits and characteristics resulting from the interaction of its genetic constitution (genotype) with environmental factors. These characteristics can include appearance, development, behavior, and resistance to disease, among others. Phenotypes can vary widely, even among individuals with identical genotypes, due to differences in environmental influences, gene expression, and genetic interactions.

Macrophages are a type of white blood cell that are an essential part of the immune system. They are large, specialized cells that engulf and destroy foreign substances, such as bacteria, viruses, parasites, and fungi, as well as damaged or dead cells. Macrophages are found throughout the body, including in the bloodstream, lymph nodes, spleen, liver, lungs, and connective tissues. They play a critical role in inflammation, immune response, and tissue repair and remodeling.

Macrophages originate from monocytes, which are a type of white blood cell produced in the bone marrow. When monocytes enter the tissues, they differentiate into macrophages, which have a larger size and more specialized functions than monocytes. Macrophages can change their shape and move through tissues to reach sites of infection or injury. They also produce cytokines, chemokines, and other signaling molecules that help coordinate the immune response and recruit other immune cells to the site of infection or injury.

Macrophages have a variety of surface receptors that allow them to recognize and respond to different types of foreign substances and signals from other cells. They can engulf and digest foreign particles, bacteria, and viruses through a process called phagocytosis. Macrophages also play a role in presenting antigens to T cells, which are another type of immune cell that helps coordinate the immune response.

Overall, macrophages are crucial for maintaining tissue homeostasis, defending against infection, and promoting wound healing and tissue repair. Dysregulation of macrophage function has been implicated in a variety of diseases, including cancer, autoimmune disorders, and chronic inflammatory conditions.

Epithelium is the tissue that covers the outer surface of the body, lines the internal cavities and organs, and forms various glands. It is composed of one or more layers of tightly packed cells that have a uniform shape and size, and rest on a basement membrane. Epithelial tissues are avascular, meaning they do not contain blood vessels, and are supplied with nutrients by diffusion from the underlying connective tissue.

Epithelial cells perform a variety of functions, including protection, secretion, absorption, excretion, and sensation. They can be classified based on their shape and the number of cell layers they contain. The main types of epithelium are:

1. Squamous epithelium: composed of flat, scalelike cells that fit together like tiles on a roof. It forms the lining of blood vessels, air sacs in the lungs, and the outermost layer of the skin.
2. Cuboidal epithelium: composed of cube-shaped cells with equal height and width. It is found in glands, tubules, and ducts.
3. Columnar epithelium: composed of tall, rectangular cells that are taller than they are wide. It lines the respiratory, digestive, and reproductive tracts.
4. Pseudostratified epithelium: appears stratified or layered but is actually made up of a single layer of cells that vary in height. The nuclei of these cells appear at different levels, giving the tissue a stratified appearance. It lines the respiratory and reproductive tracts.
5. Transitional epithelium: composed of several layers of cells that can stretch and change shape to accommodate changes in volume. It is found in the urinary bladder and ureters.

Epithelial tissue provides a barrier between the internal and external environments, protecting the body from physical, chemical, and biological damage. It also plays a crucial role in maintaining homeostasis by regulating the exchange of substances between the body and its environment.

Emphysema is a chronic respiratory disease characterized by abnormal, permanent enlargement of the airspaces called alveoli in the lungs, accompanied by destruction of their walls. This results in loss of elasticity and decreased gas exchange efficiency, causing shortness of breath and coughing. It is often caused by smoking or exposure to harmful pollutants. The damage to the lungs is irreversible, but quitting smoking and using medications can help alleviate symptoms and slow disease progression.

Forced Expiratory Volume (FEV) is a medical term used to describe the volume of air that can be forcefully exhaled from the lungs in one second. It is often measured during pulmonary function testing to assess lung function and diagnose conditions such as chronic obstructive pulmonary disease (COPD) or asthma.

FEV is typically expressed as a percentage of the Forced Vital Capacity (FVC), which is the total volume of air that can be exhaled from the lungs after taking a deep breath in. The ratio of FEV to FVC is used to determine whether there is obstruction in the airways, with a lower ratio indicating more severe obstruction.

There are different types of FEV measurements, including FEV1 (the volume of air exhaled in one second), FEV25-75 (the average volume of air exhaled during the middle 50% of the FVC maneuver), and FEV0.5 (the volume of air exhaled in half a second). These measurements can provide additional information about lung function and help guide treatment decisions.

Ureteral obstruction is a medical condition characterized by the partial or complete blockage of the ureter, which is the tube that carries urine from the kidney to the bladder. This blockage can be caused by various factors such as kidney stones, tumors, blood clots, or scar tissue, leading to a backup of urine in the kidney (hydronephrosis). Ureteral obstruction can cause pain, infection, and potential kidney damage if not treated promptly.

Up-regulation is a term used in molecular biology and medicine to describe an increase in the expression or activity of a gene, protein, or receptor in response to a stimulus. This can occur through various mechanisms such as increased transcription, translation, or reduced degradation of the molecule. Up-regulation can have important functional consequences, for example, enhancing the sensitivity or response of a cell to a hormone, neurotransmitter, or drug. It is a normal physiological process that can also be induced by disease or pharmacological interventions.

Pseudomonas infections are infections caused by the bacterium Pseudomonas aeruginosa or other species of the Pseudomonas genus. These bacteria are gram-negative, opportunistic pathogens that can cause various types of infections, including respiratory, urinary tract, gastrointestinal, dermatological, and bloodstream infections.

Pseudomonas aeruginosa is a common cause of healthcare-associated infections, particularly in patients with weakened immune systems, chronic lung diseases, or those who are hospitalized for extended periods. The bacteria can also infect wounds, burns, and medical devices such as catheters and ventilators.

Pseudomonas infections can be difficult to treat due to the bacteria's resistance to many antibiotics. Treatment typically involves the use of multiple antibiotics that are effective against Pseudomonas aeruginosa. In severe cases, intravenous antibiotics or even hospitalization may be necessary.

Prevention measures include good hand hygiene, contact precautions for patients with known Pseudomonas infections, and proper cleaning and maintenance of medical equipment.

Pulmonary Surfactant-Associated Protein D, also known as SP-D or surfactant protein D, is a protein that belongs to the collectin family. It is produced by specialized cells called type II alveolar epithelial cells and is found in the lungs, where it plays an important role in maintaining lung homeostasis and host defense.

SP-D has several functions in the lungs, including:

1. Reducing surface tension: SP-D helps to reduce surface tension in the alveoli, which facilitates breathing by preventing the collapse of the lungs during expiration.
2. Host defense: SP-D plays a crucial role in innate immunity by recognizing and binding to pathogens such as bacteria, viruses, and fungi. This helps to neutralize and clear these microorganisms from the lungs.
3. Inflammation regulation: SP-D has anti-inflammatory properties and can help to regulate the immune response in the lungs. It does this by modulating the activation of immune cells such as macrophages and neutrophils.
4. Tissue repair: SP-D may also play a role in tissue repair and remodeling in the lungs, although its exact mechanisms are not fully understood.

Abnormalities in SP-D have been implicated in several lung diseases, including respiratory distress syndrome, asthma, chronic obstructive pulmonary disease (COPD), and interstitial lung diseases.

A case-control study is an observational research design used to identify risk factors or causes of a disease or health outcome. In this type of study, individuals with the disease or condition (cases) are compared with similar individuals who do not have the disease or condition (controls). The exposure history or other characteristics of interest are then compared between the two groups to determine if there is an association between the exposure and the disease.

Case-control studies are often used when it is not feasible or ethical to conduct a randomized controlled trial, as they can provide valuable insights into potential causes of diseases or health outcomes in a relatively short period of time and at a lower cost than other study designs. However, because case-control studies rely on retrospective data collection, they are subject to biases such as recall bias and selection bias, which can affect the validity of the results. Therefore, it is important to carefully design and conduct case-control studies to minimize these potential sources of bias.

Instillation, in the context of drug administration, refers to the process of introducing a medication or therapeutic agent into a body cavity or onto a mucous membrane surface using gentle, steady pressure. This is typically done with the help of a device such as an eyedropper, pipette, or catheter. The goal is to ensure that the drug is distributed evenly over the surface or absorbed through the mucous membrane for localized or systemic effects. Instillation can be used for various routes of administration including ocular (eye), nasal, auricular (ear), vaginal, and intra-articular (joint space) among others. The choice of instillation as a route of administration depends on the drug's properties, the desired therapeutic effect, and the patient's overall health status.

Therapeutic irrigation, also known as lavage, is a medical procedure that involves the introduction of fluids or other agents into a body cavity or natural passageway for therapeutic purposes. This technique is used to cleanse, flush out, or introduce medication into various parts of the body, such as the bladder, lungs, stomach, or colon.

The fluid used in therapeutic irrigation can be sterile saline solution, distilled water, or a medicated solution, depending on the specific purpose of the procedure. The flow and pressure of the fluid are carefully controlled to ensure that it reaches the desired area without causing damage to surrounding tissues.

Therapeutic irrigation is used to treat a variety of medical conditions, including infections, inflammation, obstructions, and toxic exposures. It can also be used as a diagnostic tool to help identify abnormalities or lesions within body cavities.

Overall, therapeutic irrigation is a valuable technique in modern medicine that allows healthcare providers to deliver targeted treatment directly to specific areas of the body, improving patient outcomes and quality of life.

Limited scleroderma, also known as limited cutaneous systemic sclerosis (lcSSc), is a subtype of scleroderma, a chronic autoimmune connective tissue disease. In this form, the fibrosis or hardening and thickening of the skin is generally limited to areas below the elbows and knees, as well as the face and neck.

The limited cutaneous form often involves the hands, causing a tightening of the skin on the fingers, known as "sclerodactyly." It can also affect the internal organs, but this is usually less severe and occurs later in the disease course compared to diffuse scleroderma.

A common characteristic of limited scleroderma is the presence of CREST syndrome, an acronym for Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. These are specific symptoms associated with this subtype.

However, it is important to note that the manifestations of scleroderma can vary significantly from person to person, and not everyone with limited scleroderma will develop all the features of CREST syndrome.

Reverse Transcriptase Polymerase Chain Reaction (RT-PCR) is a laboratory technique used in molecular biology to amplify and detect specific DNA sequences. This technique is particularly useful for the detection and quantification of RNA viruses, as well as for the analysis of gene expression.

The process involves two main steps: reverse transcription and polymerase chain reaction (PCR). In the first step, reverse transcriptase enzyme is used to convert RNA into complementary DNA (cDNA) by reading the template provided by the RNA molecule. This cDNA then serves as a template for the PCR amplification step.

In the second step, the PCR reaction uses two primers that flank the target DNA sequence and a thermostable polymerase enzyme to repeatedly copy the targeted cDNA sequence. The reaction mixture is heated and cooled in cycles, allowing the primers to anneal to the template, and the polymerase to extend the new strand. This results in exponential amplification of the target DNA sequence, making it possible to detect even small amounts of RNA or cDNA.

RT-PCR is a sensitive and specific technique that has many applications in medical research and diagnostics, including the detection of viruses such as HIV, hepatitis C virus, and SARS-CoV-2 (the virus that causes COVID-19). It can also be used to study gene expression, identify genetic mutations, and diagnose genetic disorders.

Leukocyte elastase is a type of enzyme that is released by white blood cells (leukocytes), specifically neutrophils, during inflammation. Its primary function is to help fight infection by breaking down the proteins in bacteria and viruses. However, if not properly regulated, leukocyte elastase can also damage surrounding tissues, contributing to the progression of various diseases such as chronic obstructive pulmonary disease (COPD), acute respiratory distress syndrome (ARDS), and cystic fibrosis.

Leukocyte elastase is often measured in clinical settings as a marker of inflammation and neutrophil activation, particularly in patients with lung diseases. Inhibitors of leukocyte elastase have been developed as potential therapeutic agents for these conditions.

Western blotting is a laboratory technique used in molecular biology to detect and quantify specific proteins in a mixture of many different proteins. This technique is commonly used to confirm the expression of a protein of interest, determine its size, and investigate its post-translational modifications. The name "Western" blotting distinguishes this technique from Southern blotting (for DNA) and Northern blotting (for RNA).

The Western blotting procedure involves several steps:

1. Protein extraction: The sample containing the proteins of interest is first extracted, often by breaking open cells or tissues and using a buffer to extract the proteins.
2. Separation of proteins by electrophoresis: The extracted proteins are then separated based on their size by loading them onto a polyacrylamide gel and running an electric current through the gel (a process called sodium dodecyl sulfate-polyacrylamide gel electrophoresis or SDS-PAGE). This separates the proteins according to their molecular weight, with smaller proteins migrating faster than larger ones.
3. Transfer of proteins to a membrane: After separation, the proteins are transferred from the gel onto a nitrocellulose or polyvinylidene fluoride (PVDF) membrane using an electric current in a process called blotting. This creates a replica of the protein pattern on the gel but now immobilized on the membrane for further analysis.
4. Blocking: The membrane is then blocked with a blocking agent, such as non-fat dry milk or bovine serum albumin (BSA), to prevent non-specific binding of antibodies in subsequent steps.
5. Primary antibody incubation: A primary antibody that specifically recognizes the protein of interest is added and allowed to bind to its target protein on the membrane. This step may be performed at room temperature or 4°C overnight, depending on the antibody's properties.
6. Washing: The membrane is washed with a buffer to remove unbound primary antibodies.
7. Secondary antibody incubation: A secondary antibody that recognizes the primary antibody (often coupled to an enzyme or fluorophore) is added and allowed to bind to the primary antibody. This step may involve using a horseradish peroxidase (HRP)-conjugated or alkaline phosphatase (AP)-conjugated secondary antibody, depending on the detection method used later.
8. Washing: The membrane is washed again to remove unbound secondary antibodies.
9. Detection: A detection reagent is added to visualize the protein of interest by detecting the signal generated from the enzyme-conjugated or fluorophore-conjugated secondary antibody. This can be done using chemiluminescent, colorimetric, or fluorescent methods.
10. Analysis: The resulting image is analyzed to determine the presence and quantity of the protein of interest in the sample.

Western blotting is a powerful technique for identifying and quantifying specific proteins within complex mixtures. It can be used to study protein expression, post-translational modifications, protein-protein interactions, and more. However, it requires careful optimization and validation to ensure accurate and reproducible results.

Prednisolone is a synthetic glucocorticoid drug, which is a class of steroid hormones. It is commonly used in the treatment of various inflammatory and autoimmune conditions due to its potent anti-inflammatory and immunosuppressive effects. Prednisolone works by binding to specific receptors in cells, leading to changes in gene expression that reduce the production of substances involved in inflammation, such as cytokines and prostaglandins.

Prednisolone is available in various forms, including tablets, syrups, and injectable solutions. It can be used to treat a wide range of medical conditions, including asthma, rheumatoid arthritis, inflammatory bowel disease, allergies, skin conditions, and certain types of cancer.

Like other steroid medications, prednisolone can have significant side effects if used in high doses or for long periods of time. These may include weight gain, mood changes, increased risk of infections, osteoporosis, diabetes, and adrenal suppression. As a result, the use of prednisolone should be closely monitored by a healthcare professional to ensure that its benefits outweigh its risks.

Cell proliferation is the process by which cells increase in number, typically through the process of cell division. In the context of biology and medicine, it refers to the reproduction of cells that makes up living tissue, allowing growth, maintenance, and repair. It involves several stages including the transition from a phase of quiescence (G0 phase) to an active phase (G1 phase), DNA replication in the S phase, and mitosis or M phase, where the cell divides into two daughter cells.

Abnormal or uncontrolled cell proliferation is a characteristic feature of many diseases, including cancer, where deregulated cell cycle control leads to excessive and unregulated growth of cells, forming tumors that can invade surrounding tissues and metastasize to distant sites in the body.

An Enzyme-Linked Immunosorbent Assay (ELISA) is a type of analytical biochemistry assay used to detect and quantify the presence of a substance, typically a protein or peptide, in a liquid sample. It takes its name from the enzyme-linked antibodies used in the assay.

In an ELISA, the sample is added to a well containing a surface that has been treated to capture the target substance. If the target substance is present in the sample, it will bind to the surface. Next, an enzyme-linked antibody specific to the target substance is added. This antibody will bind to the captured target substance if it is present. After washing away any unbound material, a substrate for the enzyme is added. If the enzyme is present due to its linkage to the antibody, it will catalyze a reaction that produces a detectable signal, such as a color change or fluorescence. The intensity of this signal is proportional to the amount of target substance present in the sample, allowing for quantification.

ELISAs are widely used in research and clinical settings to detect and measure various substances, including hormones, viruses, and bacteria. They offer high sensitivity, specificity, and reproducibility, making them a reliable choice for many applications.

"Bronchi" are a pair of airways in the respiratory system that branch off from the trachea (windpipe) and lead to the lungs. They are responsible for delivering oxygen-rich air to the lungs and removing carbon dioxide during exhalation. The right bronchus is slightly larger and more vertical than the left, and they further divide into smaller branches called bronchioles within the lungs. Any abnormalities or diseases affecting the bronchi can impact lung function and overall respiratory health.

Carbon tetrachloride is a colorless, heavy, and nonflammable liquid with a mild ether-like odor. Its chemical formula is CCl4. It was previously used as a solvent and refrigerant, but its use has been largely phased out due to its toxicity and ozone-depleting properties.

Inhalation, ingestion, or skin contact with carbon tetrachloride can cause harmful health effects. Short-term exposure can lead to symptoms such as dizziness, headache, nausea, and vomiting. Long-term exposure has been linked to liver and kidney damage, as well as an increased risk of cancer.

Carbon tetrachloride is also a potent greenhouse gas and contributes to climate change. Its production and use are regulated by international agreements aimed at protecting human health and the environment.

Sprague-Dawley rats are a strain of albino laboratory rats that are widely used in scientific research. They were first developed by researchers H.H. Sprague and R.C. Dawley in the early 20th century, and have since become one of the most commonly used rat strains in biomedical research due to their relatively large size, ease of handling, and consistent genetic background.

Sprague-Dawley rats are outbred, which means that they are genetically diverse and do not suffer from the same limitations as inbred strains, which can have reduced fertility and increased susceptibility to certain diseases. They are also characterized by their docile nature and low levels of aggression, making them easier to handle and study than some other rat strains.

These rats are used in a wide variety of research areas, including toxicology, pharmacology, nutrition, cancer, and behavioral studies. Because they are genetically diverse, Sprague-Dawley rats can be used to model a range of human diseases and conditions, making them an important tool in the development of new drugs and therapies.

Fibronectin is a high molecular weight glycoprotein that is found in many tissues and body fluids, including plasma, connective tissue, and the extracellular matrix. It is composed of two similar subunits that are held together by disulfide bonds. Fibronectin plays an important role in cell adhesion, migration, and differentiation by binding to various cell surface receptors, such as integrins, and other extracellular matrix components, such as collagen and heparan sulfate proteoglycans.

Fibronectin has several isoforms that are produced by alternative splicing of a single gene transcript. These isoforms differ in their biological activities and can be found in different tissues and developmental stages. Fibronectin is involved in various physiological processes, such as wound healing, tissue repair, and embryonic development, and has been implicated in several pathological conditions, including fibrosis, tumor metastasis, and thrombosis.

Prognosis is a medical term that refers to the prediction of the likely outcome or course of a disease, including the chances of recovery or recurrence, based on the patient's symptoms, medical history, physical examination, and diagnostic tests. It is an important aspect of clinical decision-making and patient communication, as it helps doctors and patients make informed decisions about treatment options, set realistic expectations, and plan for future care.

Prognosis can be expressed in various ways, such as percentages, categories (e.g., good, fair, poor), or survival rates, depending on the nature of the disease and the available evidence. However, it is important to note that prognosis is not an exact science and may vary depending on individual factors, such as age, overall health status, and response to treatment. Therefore, it should be used as a guide rather than a definitive forecast.

Chemokine (C-C motif) ligand 17 (CCL17), also known as thymus and activation-regulated chemokine (TARC), is a small signaling protein that belongs to the CC chemokine family. Chemokines are a group of cytokines, or cell signaling molecules, that play an important role in immune function by recruiting immune cells to sites of infection or inflammation.

CCL17 is produced by various types of cells, including dendritic cells, macrophages, and endothelial cells, in response to stimulation by pro-inflammatory cytokines such as interleukin (IL)-4 and IL-13. CCL17 binds to its receptor, CCR4, which is expressed on the surface of Th2 cells, regulatory T cells, and some other immune cells.

CCL17 plays a role in the recruitment of these cells to sites of inflammation, and has been implicated in the pathogenesis of various diseases, including allergies, asthma, atopic dermatitis, and certain types of cancer. In particular, CCL17 has been shown to promote the migration of Th2 cells, which are involved in the immune response to parasites and allergens, to sites of inflammation.

In addition to its role in immune function, CCL17 has also been found to have angiogenic properties, meaning it can stimulate the growth of new blood vessels. This has led to interest in its potential as a therapeutic target for diseases characterized by abnormal blood vessel formation, such as cancer and diabetic retinopathy.

Matrix metalloproteinase 7 (MMP-7), also known as matrilysin, is a type of enzyme that belongs to the matrix metalloproteinase family. These enzymes are capable of degrading various components of the extracellular matrix, which is the structural framework of tissues in the body. MMP-7 has a broad range of substrates and can break down proteins such as collagens, gelatins, and caseins, as well as other matrix proteins. It plays important roles in tissue remodeling, wound healing, and cell migration, among other processes.

MMP-7 is synthesized and secreted by various cells, including epithelial cells, fibroblasts, and immune cells. It is a small enzyme with a molecular weight of around 28 kDa and is secreted in an active form, unlike many other MMPs that are secreted as inactive proenzymes and require activation by other proteases.

Increased expression of MMP-7 has been implicated in several pathological conditions, including cancer, where it can contribute to tumor invasion and metastasis by degrading the extracellular matrix and releasing growth factors. It has also been associated with inflammatory diseases such as rheumatoid arthritis and periodontitis.

"Pseudomonas aeruginosa" is a medically important, gram-negative, rod-shaped bacterium that is widely found in the environment, such as in soil, water, and on plants. It's an opportunistic pathogen, meaning it usually doesn't cause infection in healthy individuals but can cause severe and sometimes life-threatening infections in people with weakened immune systems, burns, or chronic lung diseases like cystic fibrosis.

P. aeruginosa is known for its remarkable ability to resist many antibiotics and disinfectants due to its intrinsic resistance mechanisms and the acquisition of additional resistance determinants. It can cause various types of infections, including respiratory tract infections, urinary tract infections, gastrointestinal infections, dermatitis, and severe bloodstream infections known as sepsis.

The bacterium produces a variety of virulence factors that contribute to its pathogenicity, such as exotoxins, proteases, and pigments like pyocyanin and pyoverdine, which aid in iron acquisition and help the organism evade host immune responses. Effective infection control measures, appropriate use of antibiotics, and close monitoring of high-risk patients are crucial for managing P. aeruginosa infections.

A cell line is a culture of cells that are grown in a laboratory for use in research. These cells are usually taken from a single cell or group of cells, and they are able to divide and grow continuously in the lab. Cell lines can come from many different sources, including animals, plants, and humans. They are often used in scientific research to study cellular processes, disease mechanisms, and to test new drugs or treatments. Some common types of human cell lines include HeLa cells (which come from a cancer patient named Henrietta Lacks), HEK293 cells (which come from embryonic kidney cells), and HUVEC cells (which come from umbilical vein endothelial cells). It is important to note that cell lines are not the same as primary cells, which are cells that are taken directly from a living organism and have not been grown in the lab.

'Asbestos, serpentine' is a type of asbestos mineral that belongs to the serpentine group of minerals. The serpentine group of minerals is characterized by its sheet or layered structure, in which each silicate tetrahedron shares three oxygen atoms with adjacent tetrahedra, forming a continuous two-dimensional sheet.

The most common type of asbestos mineral in the serpentine group is chrysotile, also known as white asbestos or serpentine asbestos. Chrysotile fibers are curly and flexible, which makes them easier to weave into textiles and other materials. As a result, chrysotile has been widely used in a variety of industrial and commercial applications, such as insulation, roofing, flooring, and cement products.

However, exposure to chrysotile fibers has been linked to several serious health problems, including lung cancer, mesothelioma, and asbestosis. As a result, the use of chrysotile and other types of asbestos has been banned or restricted in many countries around the world.

Peritoneal fibrosis is a chronic condition characterized by the formation of excessive scar tissue (fibrosis) in the peritoneum, which is the thin, transparent membrane that lines the inner wall of the abdomen and covers the abdominal organs. This fibrotic process can lead to thickening and shrinkage of the peritoneum, resulting in impaired function of the affected organs and various complications.

Peritoneal fibrosis often occurs as a result of long-term exposure to dialysis solutions used during peritoneal dialysis, a type of renal replacement therapy for patients with end-stage kidney disease. The inflammatory response triggered by the dialysate can cause progressive fibrosis and thickening of the peritoneum, ultimately leading to reduced efficiency of the dialysis process and potential complications such as fluid leakage, hernias, or bowel obstruction.

In addition to peritoneal dialysis-induced fibrosis, other causes of peritoneal fibrosis include previous abdominal surgeries, intra-abdominal infections, autoimmune diseases, and certain medications. The diagnosis of peritoneal fibrosis typically involves a combination of clinical evaluation, imaging studies (such as ultrasound or CT scan), and direct examination of the peritoneum during diagnostic laparoscopy. Treatment options may include modifying the underlying cause, optimizing dialysis techniques, using anti-fibrotic medications, or considering alternative renal replacement therapies such as hemodialysis or transplantation.

Acetylcysteine is a medication that is used for its antioxidant effects and to help loosen thick mucus in the lungs. It is commonly used to treat conditions such as chronic bronchitis, emphysema, and cystic fibrosis. Acetylcysteine is also known by the brand names Mucomyst and Accolate. It works by thinning and breaking down mucus in the airways, making it easier to cough up and clear the airways. Additionally, acetylcysteine is an antioxidant that helps to protect cells from damage caused by free radicals. It is available as a oral tablet, liquid, or inhaled medication.

Intercellular signaling peptides and proteins are molecules that mediate communication and interaction between different cells in living organisms. They play crucial roles in various biological processes, including cell growth, differentiation, migration, and apoptosis (programmed cell death). These signals can be released into the extracellular space, where they bind to specific receptors on the target cell's surface, triggering intracellular signaling cascades that ultimately lead to a response.

Peptides are short chains of amino acids, while proteins are larger molecules made up of one or more polypeptide chains. Both can function as intercellular signaling molecules by acting as ligands for cell surface receptors or by being cleaved from larger precursor proteins and released into the extracellular space. Examples of intercellular signaling peptides and proteins include growth factors, cytokines, chemokines, hormones, neurotransmitters, and their respective receptors.

These molecules contribute to maintaining homeostasis within an organism by coordinating cellular activities across tissues and organs. Dysregulation of intercellular signaling pathways has been implicated in various diseases, such as cancer, autoimmune disorders, and neurodegenerative conditions. Therefore, understanding the mechanisms underlying intercellular signaling is essential for developing targeted therapies to treat these disorders.

F344 is a strain code used to designate an outbred stock of rats that has been inbreeded for over 100 generations. The F344 rats, also known as Fischer 344 rats, were originally developed at the National Institutes of Health (NIH) and are now widely used in biomedical research due to their consistent and reliable genetic background.

Inbred strains, like the F344, are created by mating genetically identical individuals (siblings or parents and offspring) for many generations until a state of complete homozygosity is reached, meaning that all members of the strain have identical genomes. This genetic uniformity makes inbred strains ideal for use in studies where consistent and reproducible results are important.

F344 rats are known for their longevity, with a median lifespan of around 27-31 months, making them useful for aging research. They also have a relatively low incidence of spontaneous tumors compared to other rat strains. However, they may be more susceptible to certain types of cancer and other diseases due to their inbred status.

It's important to note that while F344 rats are often used as a standard laboratory rat strain, there can still be some genetic variation between individual animals within the same strain, particularly if they come from different suppliers or breeding colonies. Therefore, it's always important to consider the source and history of any animal model when designing experiments and interpreting results.

The pleura is the medical term for the double-layered serous membrane that surrounds the lungs and lines the inside of the chest cavity. The two layers of the pleura are called the parietal pleura, which lines the chest cavity, and the visceral pleura, which covers the surface of the lungs.

The space between these two layers is called the pleural cavity, which contains a small amount of lubricating fluid that allows the lungs to move smoothly within the chest during breathing. The main function of the pleura is to protect the lungs and facilitate their movement during respiration.

Chemokine (C-X-C motif) ligand 5 (CXCL5), also known as epithelial neutrophil-activating peptide 78 (ENA-78) or liver-activated peptide (LAP), is a small signaling protein belonging to the CXC chemokine family. Chemokines are a group of cytokines, or cell signaling molecules, that play important roles in immune responses and inflammation by recruiting various immune cells to sites of infection or injury through specific receptor-mediated interactions.

CXCL5 is primarily produced by epithelial cells, macrophages, and neutrophils in response to bacterial infections, tissue damage, or proinflammatory cytokines. This chemokine exerts its functions by binding to its receptor CXCR2, which is expressed on the surface of various immune cells, including neutrophils, monocytes, and lymphocytes. The primary role of CXCL5 is to attract neutrophils to the site of inflammation or infection, where they can help eliminate pathogens and promote tissue repair.

Apart from its involvement in immune responses and inflammation, CXCL5 has been implicated in several physiological and pathological processes, such as embryonic development, wound healing, cancer progression, and metastasis. Dysregulation of CXCL5 signaling has been associated with various diseases, including chronic inflammatory disorders, autoimmune diseases, and cancer.

Treatment outcome is a term used to describe the result or effect of medical treatment on a patient's health status. It can be measured in various ways, such as through symptoms improvement, disease remission, reduced disability, improved quality of life, or survival rates. The treatment outcome helps healthcare providers evaluate the effectiveness of a particular treatment plan and make informed decisions about future care. It is also used in clinical research to compare the efficacy of different treatments and improve patient care.

Bronchiectasis is a medical condition characterized by permanent, abnormal widening and thickening of the walls of the bronchi (the airways leading to the lungs). This can lead to recurrent respiratory infections, coughing, and the production of large amounts of sputum. The damage to the airways is usually irreversible and can be caused by various factors such as bacterial or viral infections, genetic disorders, immune deficiencies, or exposure to environmental pollutants. In some cases, the cause may remain unknown. Treatment typically includes chest physiotherapy, bronchodilators, antibiotics, and sometimes surgery.

"Pulmonary Fibrosis". MedicineNet, Inc. Archived from the original on 19 July 2014. Retrieved 26 July 2014. "Pulmonary fibrosis ... Misdiagnosis is common because, while overall pulmonary fibrosis is not rare, each individual type of pulmonary fibrosis is ... Lung sound in idiopathic pulmonary fibrosis velcro crackles on auscultation in a person with idiopathic pulmonary fibrosis ... "Pulmonary Fibrosis". medlineplus.gov. Retrieved 20 December 2019. "Pulmonary fibrosis - Symptoms and causes". Mayo Clinic. ...
December 2011). "Familial pulmonary fibrosis is the strongest risk factor for idiopathic pulmonary fibrosis". Respiratory ... Wikimedia Commons has media related to Idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis at Curlie (All articles ... "Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical ... The number of published studies on the role of pulmonary rehabilitation in idiopathic pulmonary fibrosis is small, but most of ...
... is a chronic lung disease of horses. There is evidence that the disease is caused by ... Poth T, Niedermaier G, Hermanns W (January 2009). "Equine multinodular pulmonary fibrosis in association with an EHV-5 ... Signs of equine multinodular pulmonary fibrosis are mainly weight loss, fever, respiratory distress and depression. ... 2007), there are significant pathomorphological findings in lungs with equine multinodular pulmonary fibrosis. In gross ...
... (CPFE), describes a medical syndrome involving both pulmonary fibrosis and emphysema ... February 2006). "Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity". European Respiratory Journal. ... Wand, O; Kramer, MR (January 2018). "The Syndrome of Combined Pulmonary Fibrosis and Emphysema - CPFE". Harefuah. 157 (1): 28- ... Jankowich, MD; Rounds, SI (January 2012). "Combined pulmonary fibrosis and emphysema syndrome: a review". Chest. 141 (1): 222- ...
Hereditary fibrosing poikiloderma with tendon contractures, myopathy and pulmonary fibrosis is a rare genetic syndrome ... The main characteristics of this condition are poikiloderma, tendon contractures and progressive pulmonary fibrosis. Other ... Muscle biopsy shows fibrosis and fatty infiltration. Skin biopsy shows fibrosis and alterations of the elastic network. This ... and pulmonary fibrosis. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, (editors). GeneReview ...
"Pulmonary fibrosis". Cleveland Clinic. Cleveland Clinic. 2021. Retrieved 2 May 2022. "Idiopathic pulmonary fibrosis". AMBOSS. ... Hematic biometry Imaging tests such as X-rays or tomography Pulmonary function tests Biopsy Pulmonary fibrosis is not currently ... "Síndrome de fibrosis por radiación". OncoLink. Trustees of the University of Pennsylvania. Retrieved 2 May 2022. "Fibrosis ... Radiation fibrosis syndrome is a human illness. It occurs as a result of cell death, and can be caused by radiotherapy. It is ...
"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. ... Heraldo, El (January 22, 2021). "Fallece el director José Ángel García, padre de Gael García, de fibrosis". EL HERALDO. "Matan ...
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 ... Idiopathic pulmonary fibrosis. St. Louis. ISBN 978-0-323-54432-0. OCLC 1053744041.{{cite book}}: CS1 maint: location missing ...
... pulmonary hypertension and idiopathic pulmonary fibrosis. Methods for the preservation of donor lungs are imperative in order ... Idiopathic pulmonary fibrosis causes the lungs to become scarred which results in a difficulty in breathing. The causes are of ... "Chronic Obstructive Pulmonary Disease (COPD) , CDC". www.cdc.gov. 2023-03-20. Retrieved 2023-03-28. "Cystic fibrosis". nhs.uk. ... "Idiopathic pulmonary fibrosis". nhs.uk. 2017-10-23. Retrieved 2023-03-28. 3 Minute Papers: Lobectomy vs Segmentectomy for Lung ...
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". ... High αvβ6 expression in fibrosis and cancer is usually associated with a poorer prognosis. Fibrosis occurs in response to ...
On September 30, 2007, Goulet was hospitalized in Las Vegas, where he was diagnosed with idiopathic pulmonary fibrosis, a rare ... While awaiting a lung transplant, Goulet died from pulmonary fibrosis on the evening of October 30, 2007, at Cedars-Sinai ... "Prominent People Lost to IPF/PF: Robert Goulet". Pulmonary Fibrosis Foundation. Retrieved December 9, 2011. "Robert Goulet-2006 ... Deaths from pulmonary fibrosis, Fellows of the Royal Conservatory of Music, Grammy Award winners, Lost Canadians, Male actors ...
It is thought to be the cause of a chronic lung disease of adult horses; equine multinodular pulmonary fibrosis. "ICTV Taxonomy ... Williams, KJ (March 2014). "Gammaherpesviruses and Pulmonary Fibrosis: Evidence From Humans, Horses, and Rodents". Veterinary ...
Daba MH, El-Tahir KE, Al-Arifi MN, Gubara OA (June 2004). "Drug-induced pulmonary fibrosis". Saudi Medical Journal. 25 (6): 700 ...
... 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 ... Lung transplantation, or pulmonary transplantation, is a surgical procedure in which one or both lungs are replaced by lungs ... Pulmonary Hypertension: A Patient's Survival Guide 3rd edition p. 126 Rundle RL (2 January 2003). "New Blood Procedure Helps ...
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 ...
"Prominent People who have died from Pulmonary Fibrosis". Pulmonary Fibrosis Foundation, Colorado. Archived from the original on ... He was suffering from pulmonary fibrosis. His funeral was held at the London Central Mosque, Regent Park, and he was laid to ...
Fonseca C, Abraham D, Renzoni EA (January 2011). "Endothelin in pulmonary fibrosis". American Journal of Respiratory Cell and ... pulmonary hypertension or 'pulmonary venous hypertension'). However, in some patients, the raised pressure in the pulmonary ... Pulmonary Hypertension Wikimedia Commons has media related to Pulmonary hypertension. Scholia has a topic profile for Pulmonary ... Signs of pulmonary hypertension on CT scan of the chest are: Enlargement of the pulmonary trunk (measured at its bifurcation). ...
While HRCT may be able to identify pulmonary fibrosis, it may not always be able to further categorize the fibrosis to a ... HRCT is used for diagnosis and assessment of interstitial lung disease, such as pulmonary fibrosis, and other generalized lung ... ISBN 978-1-60795-024-0. Gotway MB, Freemer MM, King TE (June 2007). "Challenges in pulmonary fibrosis. 1: Use of high ... such as asbestosis and idiopathic pulmonary fibrosis. HRCT temporal bone is used to determine the anatomy of chronic otitis ...
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. News, Deseret (January 23, 1993). "'TEACH THEM CORRECT PRINCIPLES': GOSPEL INFLUENCES LIFE, OUTLOOK ... Jump died on September 22, 2003, from pulmonary fibrosis that led to respiratory failure at his home near Los Angeles, ... Deaths from pulmonary fibrosis, Kansas State University alumni, Male actors from Dayton, Ohio, Converts to Mormonism, Harold B ...
Oh, CK; Murray, LA; Molfino, NL (February 2012). "Smoking and Idiopathic Pulmonary Fibrosis". Pulmonary Medicine. Hindawi ... chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF), emphysema, and various types and subtypes of ... Patel, Mit P.; Khangoora, Vikramjit S.; Marik, Paul E. (October 2019). "A Review of the Pulmonary and Health Impacts of Hookah ... Laniado-Laborín, Rafael (January 2009). "Smoking and Chronic Obstructive Pulmonary Disease (COPD). Parallel Epidemics of the ...
Pian died of pulmonary fibrosis. After her death, she was widely eulogized in various obituaries. A memorial at Harvard was ...
Oh CK, Murray LA, Molfino NA (February 2012). "Smoking and idiopathic pulmonary fibrosis". Pulmonary Medicine. Hindawi ... chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF), emphysema, and various types and subtypes of ... A study from Erasmus University Rotterdam limited to people with chronic obstructive pulmonary disease found that the cost- ... Laniado-Laborín R (January 2009). "Smoking and chronic obstructive pulmonary disease (COPD). Parallel epidemics of the 21 ...
Pneumoconiosis Silicosis Asbestosis Idiopathic pulmonary fibrosis Pulmonary fibrosis Emphysema Travis WD, King TE, Bateman ED, ... The scarring (fibrosis) involves the pulmonary interstitium (the supporting framework of the lung). UIP is thus classified as a ... 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 ...
Tucci E, Verdiani P, Di Carlo S, Sforza V (1986). "Lomustine (CCNU)-induced pulmonary fibrosis". Tumori. 72 (1): 95-8. doi: ...
... there is generally pulmonary fibrosis. Examples are: Idiopathic pulmonary fibrosis Idiopathic interstitial pneumonia, of which ... such as interstitial lung disease or pulmonary fibrosis, or from having the alveoli air spaces filled with external material ... Pulmonary function test demonstrates a decrease in the forced vital capacity. Due to the chronic nature of this disease, the ... PULMONARY FUNCTION TESTS A Workshop on Simple Spirometry & Flow Volume Loops. Archived 2010-03-31 at the Wayback Machine Dr. S ...
In November, Roche acquired Promedior and its lead treatment - PRM-151 - for the treatment of idiopathic pulmonary fibrosis, ... Pulmozyme (dornase alfa), for the improvement of pulmonary function in cystic fibrosis. Raptiva (efalizumab), for psoriasis, ... Esbriet (pirfenidone), for idiopathic pulmonary fibrosis. Fansidar (sulfadoxine/pyrimethamine), for malaria and toxoplasmosis. ... "Roche to Acquire Fibrosis-Focused Promedior for up to $1.4 Billion". "Promedior Enters into Definitive Merger Agreement to be ...
Drawing from nearly a decade ago when the first antifibrotic medication was approved for idiopathic pulmonary fibrosis (IPF), ... Cite this: Progressive Pulmonary Fibrosis: Treatment and Support - Medscape - Sep 19, 2023. ... MILAN ― Numerous unresolved questions surround progressive pulmonary fibrosis (PPF) treatment, according to Elisabeth Bendstrup ... The co-chair of the session, Marlies S. Wijsenbeek, MD, PhD, pulmonary physician and head of the ILD Centre at the Erasmus ...
"Pulmonary Fibrosis". MedicineNet, Inc. Archived from the original on 19 July 2014. Retrieved 26 July 2014. "Pulmonary fibrosis ... Misdiagnosis is common because, while overall pulmonary fibrosis is not rare, each individual type of pulmonary fibrosis is ... Lung sound in idiopathic pulmonary fibrosis velcro crackles on auscultation in a person with idiopathic pulmonary fibrosis ... "Pulmonary Fibrosis". medlineplus.gov. Retrieved 20 December 2019. "Pulmonary fibrosis - Symptoms and causes". Mayo Clinic. ...
Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of ... Where Is Idiopathic Pulmonary Fibrosis Treatment Going? * The Relationship Between Lung Fibrosis and Idiopathic Pulmonary ... encoded search term (Idiopathic Pulmonary Fibrosis (IPF)) and Idiopathic Pulmonary Fibrosis (IPF) What to Read Next on Medscape ... Pulmonary hypertension in idiopathic pulmonary fibrosis. Chest. 2007 Sep. 132(3):998-1006. [QxMD MEDLINE Link]. ...
... and pulmonary fibrosis (abbreviated POIKTMP), is a disorder that affects many parts of the body, particularly the skin, muscles ... Adults with POIKTMP can develop a condition called pulmonary fibrosis, in which scar tissue forms in the lungs. . Pulmonary ... Poikiloderma, tendon contracture and pulmonary fibrosis: a new autosomal dominant syndrome? Br J Dermatol. 2006 Nov;155(5):1057 ... Mutations in FAM111B cause hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis. Am J ...
... Respir Res. 2021 Jul 7;22(1):197. doi: 10.1186/s12931-021- ... Background: Idiopathic pulmonary fibrosis (IPF) is a progressive debilitating lung disease with considerable morbidity. ... Keywords: Case definition; Epidemiology; Idiopathic pulmonary fibrosis; Interstitial lung disease; Modeling; Rare disease. ...
Hence, the patient fulfilled the criteria for a diagnosis of an acute exacerbation of pulmonary fibrosis in his native lung. ... differential diagnosis of progressive respiratory deterioration following single lung transplantation for pulmonary fibrosis. ... Exacerbation of Pulmonary Fibrosis Following Single Lung Transplantation. Dawei Yang. ,1,2Jennifer M Wilson. ,1,3Chunxue Bai. , ... Hence, the patient fulfilled the criteria for a diagnosis of an acute exacerbation of pulmonary fibrosis in his native lung. ...
The Pulmonary Fibrosis Foundation Registry considers the protection of your privacy to be one of our most important ... PRECISIONS is a multicenter study that will evaluate biomarkers to aid in the diagnosis of pulmonary fibrosis. Studies such as ... Ayodeji Adegunsoye of the University of Chicago used PFF Registry data to study outcomes in patients with pulmonary fibrosis, ... Subscribe to receive the most current news and updates from the Pulmonary Fibrosis Foundation. ...
After a string of failed clinical trials in idiopathic pulmonary fibrosis, the results from studies of two experimental ... Clinical Trial Results Signal an Advance for Idiopathic Pulmonary Fibrosis May 29, 2014. Denver, CO - Pulmonary Fibrosis ... Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal scarring of the lungs. Researchers do not understand what causes ... National Jewish Health has one of the largest programs for the treatment and study of idiopathic pulmonary fibrosis. Dr. Brown ...
Pulmonary fibrosis is a disease that can lead to scarring in air sacs within the lungs. This can make breathing difficult. ... Tips for Living with Pulmonary Fibrosis Pulmonary fibrosis is a disease that can lead to scarring in air sacs within the lungs ... HealthDay News)-Pulmonary fibrosis is a disease that can lead to scarring in air sacs within the lungs. This can make breathing ... About 140,000 Americans have been diagnosed with pulmonary fibrosis, the American Lung Association says. ...
Pretreatment with VPA both increased the survival rate and prevented the loss of body weight in mice with pulmonary fibrosis. ... Overall, pretreatment with VPA attenuated the development of pulmonary fibrosis by inhibiting EMT in mice, which was associated ... In vivo, pretreatment with VPA attenuates pulmonary fibrosis development through EMT inhibition in mice, which was associated ... In vivo, the effects of VPA on bleomycin-induced lung fibrosis were evaluated by assessing variables such as survival rate, ...
Are you up-to-date on the management and treatment options available to patients at different stages of idiopathic pulmonary ... Where Is Idiopathic Pulmonary Fibrosis Treatment Going? * The Relationship Between Lung Fibrosis and Idiopathic Pulmonary ... Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease of unknown etiology, characterized by progressive ... Fast Five Quiz: Idiopathic Pulmonary Fibrosis: Management and Treatment - Medscape - Oct 17, 2023. ...
Mycobacterium chimaera pulmonary infection complicating cystic fibrosis: a case report. J Med Case Reports. 2011;5:473. DOI ... Mycobacterium chimaera Pulmonary Disease in Cystic Fibrosis Patients, France, 2010-2017 On This Page ... Mycobacterium chimaera Pulmonary Disease in Cystic Fibrosis Patients, France, 2010-2017. Emerging Infectious Diseases. 2019;25( ... Mycobacterium chimaera Pulmonary Disease in Cystic Fibrosis Patients, France, 2010-2017. Emerg Infect Dis. 2019;25(3):611-613. ...
Idiopathic pulmonary fibrosis is more strongly associated with coronary artery disease than chronic obstructive pulmonary ... "Idiopathic pulmonary fibrosis is more strongly associated with coronary artery disease than chronic obstructive pulmonary ... CAD Linked More to Idiopathic Pulmonary Fibrosis than COPD. Apr 25, 2023 ... lifetime non-smokers with no lung disease and participants with the chronic obstructive pulmonary disease had high-resolution ...
Idiopathic Pulmonary Fibrosis (IPF) now has two licensed treatments available. Pirfenidone was the first drug to be licensed ... Idiopathic Pulmonary Fibrosis (IPF) is a debilitating life limiting condition with a median survival ranging from 2.5 to 3.5 ... Idiopathic Pulmonary Fibrosis (IPF) now has two licensed treatments available. Pirfenidone was the first drug to be licensed ... Idiopathic pulmonary fibrosis in adults: Diagnosis and management. In NICE Guideline (CG163); National Institute for Health and ...
Play popular tracks tagged Pulmonary Fibrosis News on SoundCloud desktop and mobile. ...
Persistent Pulmonary Fibrosis, Migration to the Pleura, and Other Preliminary New Findings after Subchronic Exposure to Multi- ... Persistent Pulmonary Fibrosis, Migration to the Pleura, and Other Preliminary New Findings after Subchronic Exposure to Multi- ... Porter is a pulmonary toxicologist, and Dr. Mercer is a bio-engineer in PPRB/HELD. The authors all have expertise in pulmonary ... Such effects are similar to the interstitial pulmonary fibrosis reported previously by NIOSH researchers using single-walled ...
Abstract Idiopathic pulmonary fibrosis (IPF) is characterized by progressive scarring of the lung parenchyma, leading to ... 2018). Inflammatory pathways are upregulated in the nasal epithelium in patients with idiopathic pulmonary fibrosis. figshare. ... These findings support the concept that fibrosis is associated with upregulation of inflammatory pathways across the ...
Auxotrophy of pulmonary isolates of P aeruginosa has previously been shown to be a feature of cystic fibrosis and chronic lung ... Adult cystic fibrosis: association of acute pulmonary exacerbations and increasing severity of lung disease with auxotrophic ... Adult cystic fibrosis: association of acute pulmonary exacerbations and increasing severity of lung disease with auxotrophic ... CONCLUSIONS--The majority of adults with cystic fibrosis infected with P aeruginosa harbour auxotrophs in the sputum. A ...
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an unmet need of biomarkers that can aid in the ... Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an unmet need of biomarkers that can aid in the ... Biomarkers, Extracellular matrix, Fibroblast, Idiopathic pulmonary fibrosis. in International Journal of Molecular Sciences. ... article{6d4eb194-b010-4a77-acf7-1c1404a54769, abstract = {{,p,Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic ...
Idiopathic Pulmonary Fibrosis - Etiology, pathophysiology, symptoms, signs, diagnosis & prognosis from the MSD Manuals - ... Pathology of Idiopathic Pulmonary Fibrosis The key histologic findings of idiopathic pulmonary fibrosis are subpleural fibrosis ... Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical ... Symptoms and Signs of Idiopathic Pulmonary Fibrosis Symptoms and signs of idiopathic pulmonary fibrosis typically develop over ...
Pulmonary hypertension in idiopathic pulmonary fibrosis. Source: Annual Congress 2010 - Clinical aspects in pulmonary fibrosis ... Pathogenesis of pulmonary fibrosis. Year: 2009. Pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis (IPF) ... Clinical aspects in pulmonary fibrosis. Year: 2010. Pulmonary rehabilitation in idiopathic pulmonary fibrosis: Predictors of ... Pulmonary hypertension in idiopathic pulmonary fibrosis: correlation to pulmonary function and exercise parameters. Source: Eur ...
keywords = "honeycombing, idiopathic pulmonary fibrosis, multidisciplinary, registry",. author = "Jo, {Helen E.} and Ian ... Implications of the diagnostic criteria of idiopathic pulmonary fibrosis in clinical practice: Analysis from the Australian ... Implications of the diagnostic criteria of idiopathic pulmonary fibrosis in clinical practice: Analysis from the Australian ... Implications of the diagnostic criteria of idiopathic pulmonary fibrosis in clinical practice : Analysis from the Australian ...
Pulmonary Fibrosis and Arava. April 5, 2007. By Arthritis Center Question. I have Pulmonary Fibrosis and have recently been ... In a warning it states that Arava should not be used in patients with Pulmonary Fibrosis. This was in 2004 and would like to ... To my knowledge, Arava has not been associated with pulmonary fibrosis and thus it is frequently used instead of methotrexate ...
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disorder of unknown etiology for which there is no effective therapy ... Azithromycin: A New Treatment Hope for Idiopathic Pulmonary Fibrosis? - Medscape - Feb 23, 2018. ... Medscape Pulmonary Medicine © 2018 WebMD, LLC. Any views expressed above are the authors own and do not necessarily reflect ... Professor, Department of Medicine; Physician, Department of Pulmonary Medicine, St. Francis Hospital, Hartford, Connecticut. ...
encoded search term (Interstitial (Nonidiopathic) Pulmonary Fibrosis) and Interstitial (Nonidiopathic) Pulmonary Fibrosis What ... Interstitial (Nonidiopathic) Pulmonary Fibrosis. Updated: Sep 20, 2013 * Author: Eleanor M Summerhill, MD, FACP, FCCP; Chief ... Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med. 1998 Apr. 157(4 Pt 1 ... Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2001 Jul 15. 164(2):193 ...
Pulmonary Fibrosis Partners is not responsible or liable for any content you post. You will indemnify Pulmonary Fibrosis ... Pulmonary Fibrosis Partners, Inc. (PFP) , P.O. Box 342 , Newburgh, Indiana , 47629-0342 , [email protected]. © Pulmonary ... Pulmonary Fibrosis Partners does not warrant that this website, its servers, or email sent from this URL are free of viruses or ... Pulmonary Fibrosis Partners provides its online services to you subject to the following terms of use. By visiting this website ...
Idiopathic pulmonary fibrosis answers are found in the Diagnosaurus powered by Unbound Medicine. Available for iPhone, iPad, ... Idiopathic Pulmonary Fibrosis [Internet]. In: Diagnosaurus. McGraw-Hill Education; 2014. [cited 2024 April 16]. Available from ... Zeiger, Roni F.. "Idiopathic Pulmonary Fibrosis." Diagnosaurus, 4th ed., McGraw-Hill Education, 2014. Medicine Central, im. ... unboundmedicine.com/medicine/view/Diagnosaurus/114754/all/Idiopathic_pulmonary_fibrosis. Zeiger RFR. Idiopathic pulmonary ...
... is a chronic progressive pulmonary disease of unknown etiology. It is primarily diagnosed on the basis of clinical, physiologic ... encoded search term (Idiopathic Pulmonary Fibrosis Imaging) and Idiopathic Pulmonary Fibrosis Imaging What to Read Next on ... Idiopathic pulmonary fibrosis (IPF) is a chronic progressive pulmonary disease of unknown etiology. It is primarily diagnosed ... 18F-Fluorodeoxyglucose positron emission tomography pulmonary imaging in idiopathic pulmonary fibrosis is reproducible: ...
  • Exercise training in idiopathic pulmonary fibrosis: is it of benefit? (ers-education.org)
  • Drawing from nearly a decade ago when the first antifibrotic medication was approved for idiopathic pulmonary fibrosis (IPF), Kreuter noted its effectiveness in slowing disease progression, although it does not reverse it. (medscape.com)
  • In vivo, the effects of VPA on bleomycin-induced lung fibrosis were evaluated by assessing variables such as survival rate, body weight and histopathological changes, whilst the expression of E-cadherin and vimentin in lung tissue was also evaluated. (nature.com)
  • Background: The aim of the present study was to investigate the effect of the Chamomile hydroalcoholic extract on bleomycin-induced pulmonary fibrosis in rat. (tanaffosjournal.ir)
  • In this study we show Ogg1-targeting siRNA mitigates bleomycin-induced pulmonary fibrosis in male mice, highlighting OGG1 as a tractable target in lung fibrosis. (lu.se)
  • In addition, TH5487 decreases levels of pro-inflammatory mediators, inflammatory cell infiltration, and lung remodeling in a murine model of bleomycin-induced pulmonary fibrosis conducted in male C57BL6/J mice. (lu.se)
  • Pulmonary hypertension in idiopathic pulmonary fibrosis. (medscape.com)
  • Pulmonary Hypertension Pulmonary hypertension is increased pressure in the pulmonary circulation. (msdmanuals.com)
  • In pulmonary hypertension, pulmonary vessels may become constricted. (msdmanuals.com)
  • Cor Pulmonale Cor pulmonale is right ventricular (RV) enlargement secondary to a lung disorder that causes pulmonary artery hypertension. (msdmanuals.com)
  • Pulmonary hypertension and right heart failure often develops with any severe, chronic lung disease. (petmd.com)
  • Pulmonary fibrosis is a condition in which the lungs become scarred over time. (wikipedia.org)
  • Adults with POIKTMP can develop a condition called pulmonary fibrosis, in which scar tissue forms in the lungs . (medlineplus.gov)
  • Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal scarring of the lungs. (nationaljewish.org)
  • Pulmonary fibrosis is a disease that can lead to scarring in air sacs within the lungs. (healthywomen.org)
  • HealthDay News)-Pulmonary fibrosis is a disease that can lead to scarring in air sacs within the lungs. (healthywomen.org)
  • The research showed inflammation in the lungs of the mice, and fibrosis in their lungs, which persisted following exposure. (cdc.gov)
  • My husband has pulmonary fibrosis and 70% of his lungs are gone. (healthlinkusa.com)
  • Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive systems of about 30,000 children and adults in the United States and 70,000 people worldwide. (ufl.edu)
  • Martinez FJ, Flaherty K. Pulmonary function testing in idiopathic interstitial pneumonias. (medscape.com)
  • Hence, the patient fulfilled the criteria for a diagnosis of an acute exacerbation of pulmonary fibrosis in his native lung. (hindawi.com)
  • Clinicians should consider acute exacerbation of parenchymal lung disease of the native lung in the differential diagnosis of progressive respiratory deterioration following single lung transplantation for pulmonary fibrosis. (hindawi.com)
  • All patients had M. chimaera -positive expectorated sputum specimens, clinical symptoms of pulmonary exacerbation, or a decrease in spirometry test results that improved after specific treatment. (cdc.gov)
  • The association of an increased cough and sputum production, breathlessness, and fatigue with a reduction in forced expiratory volume in 1 s (FEV1) or forced vital capacity (FVC) was diagnosed as pulmonary exacerbation ( 7 ) for all patients ( Figure ). (cdc.gov)
  • Idiopathic pulmonary fibrosis/usual interstitial pneumonia: imaging diagnosis, spectrum of abnormalities, and temporal progression. (medscape.com)
  • Autosomal dominant mutations in the TERC or TERT genes, which encode telomerase, have been identified in about 15 percent of pulmonary fibrosis patients. (wikipedia.org)
  • Giménez A, Duch P, Puig M, Gabasa M, Xaubet A, Alcaraz J. Dysregulated Collagen Homeostasis by Matrix Stiffening and TGF-β1 in Fibroblasts from Idiopathic Pulmonary Fibrosis Patients: Role of FAK/Akt. (medscape.com)
  • Dr. Ayodeji Adegunsoye of the University of Chicago used PFF Registry data to study outcomes in patients with pulmonary fibrosis, specifically among diverse racial groups. (pulmonaryfibrosis.org)
  • With the steadfast commitment of the entire pulmonary fibrosis community - the patients, their families, the physicians, researchers and funding organizations - we are seeing measurable progress, progress that keeps the search for a cure alive," said Dr. Brown. (nationaljewish.org)
  • We report Mycobacterium chimaera pulmonary disease in 4 patients given a diagnosis of cystic fibrosis in a university hospital in Montpellier, France. (cdc.gov)
  • We report M. chimaera pulmonary disease in 4 patients with cystic fibrosis. (cdc.gov)
  • Evolution of lung function for 4 cystic fibrosis patients with Mycobacterium chimaera pulmonary disease, France, 2010-2017. (cdc.gov)
  • The 4 case-patients were Caucasian, age 8-21 years, and who had a newborn diagnosis of cystic fibrosis, preexisting respiratory impairment, and digestive malabsorption. (cdc.gov)
  • Diagnosis of idiopathic pulmonary fibrosis is suspected in patients with subacute dyspnea, nonproductive cough, and Velcro crackles on chest examination. (msdmanuals.com)
  • In a warning it states that Arava should not be used in patients with Pulmonary Fibrosis. (hopkinsarthritis.org)
  • To my knowledge, Arava has not been associated with pulmonary fibrosis and thus it is frequently used instead of methotrexate in patients with RA and lung disease. (hopkinsarthritis.org)
  • Recent animal research suggests that azithromycin , a therapy that has shown benefit for acute exacerbations of chronic obstructive pulmonary disease (COPD) in humans, may also be beneficial in patients with acute exacerbations of IPF. (medscape.com)
  • Of patients referred to a pulmonary disease specialist, an estimated 10-15% have a DPLD. (medscape.com)
  • The pulmonary function testing room and all patient rooms are disinfected between patients. (ufl.edu)
  • The clinical symptoms of COVID-19 include fever , cough , and respiratory tract inflammation , with some severe patients developing an acute and chronic lung injury , such as acute respiratory distress syndrome (ARDS) and pulmonary fibrosis (PF). (bvsalud.org)
  • reported increased odds of prolonged hospital stay among women, and among patients with fever and chronic kidney or liver disease, increased creatinine levels, or bilateral pulmonary infiltration (11). (who.int)
  • Jump passed away in 2003 at the age of 71 due to complications from pulmonary fibrosis. (patheticfacts.com)
  • A number of researchers, including PhD students and post-docs, are now compiling data and performing experiments looking on various aspects of pulmonary complications in adult age due to prematurity. (ki.se)
  • Fast Five Quiz: Idiopathic Pulmonary Fibrosis: Management and Treatment - Medscape - Oct 17, 2023. (medscape.com)
  • Triptolide suppresses paraquat induced idiopathic pulmonary fibrosis by inhibiting TGFB1-dependent epithelial mesenchymal transition. (medscape.com)
  • Epithelial-mesenchymal transition (EMT) plays a crucial role in the development of pulmonary fibrosis. (nature.com)
  • It is a potent stimulator of fibrosis, and increased TGF-β signaling is associated with the development of fibrosis in various organs. (wikipedia.org)
  • Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease of unknown etiology, characterized by progressive lung scarring. (medscape.com)
  • It is thought to begin with acute injury to the pulmonary parenchyma, leading to chronic interstitial inflammation, then to fibroblast activation and proliferation, and finally progressing to the common endpoint of pulmonary fibrosis and tissue destruction. (medscape.com)
  • Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial pneumonia, causes progressive pulmonary fibrosis. (msdmanuals.com)
  • DPLD may be idiopathic, a classic illustration of which is idiopathic interstitial fibrosis (IPF), which is discussed in another article (see Pulmonary Fibrosis, Idiopathic ). (medscape.com)
  • This type of lung fibrosis is characterized by various pathological hallmarks, including chronic pulmonary epithelial injury and the proliferation and activation of fibroblasts, which subsequently promote myofibroblast formation and extracellular matrix (ECM) accumulation [ 3 ]. (nature.com)
  • The key histologic findings of idiopathic pulmonary fibrosis are subpleural fibrosis with sites of fibroblast proliferation (fibroblast foci) and dense scarring, alternating with areas of normal lung tissue (heterogeneity). (msdmanuals.com)
  • MILAN ― Numerous unresolved questions surround progressive pulmonary fibrosis (PPF) treatment, according to Elisabeth Bendstrup, MD, PhD, a researcher and clinical professor in the Department of Clinical Medicine ― Department of Respiratory Diseases and Allergy, Aarhus University, Denmark. (medscape.com)
  • Abstract Idiopathic pulmonary fibrosis (IPF) is characterized by progressive scarring of the lung parenchyma, leading to respiratory failure and death. (figshare.com)
  • These findings support the concept that fibrosis is associated with upregulation of inflammatory pathways across the respiratory epithelium with possible implications for disease detection and pathobiology. (figshare.com)
  • The common reasons for prolonged hospitalization were mandatory isolation 28% (n = 62), hospital-acquired infections 17% (n = 37), acute respiratory distress syndrome 15% (n = 32), myopathy/neuropathy 14% (n = 31), pulmonary fibrosis requiring oxygen supplementation 14% (n = 31), and completion of COVID-19 treatment 12% (n = 25). (who.int)
  • Pulmonary fibrosis is a well-known consequence of acute respiratory distress syndrome and affects the life of survivors (5). (who.int)
  • Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. (medscape.com)
  • After a string of failed clinical trials in idiopathic pulmonary fibrosis , the results from studies of two experimental medications signal progress in the treatment of this deadly disease, according to researchers at National Jewish Health and around the nation. (nationaljewish.org)
  • The diagnosis of IPF is made on the basis of the patient's history, clinical findings, pulmonary physiology, and imaging results. (medscape.com)
  • Pulmonary fibrosis is perpetuated by aberrant wound healing, rather than chronic inflammation. (wikipedia.org)
  • Instead of chronic inflammation, physicians and scientists began to see the progressive fibrosis as the primary therapeutic target. (nationaljewish.org)
  • Occupational inhalation of hard metal (HM) induces several disorders including asthma and pulmonary fibrosis. (cdc.gov)
  • HRCT of advanced stage of pulmonary fibrosis demonstrating reticular opacities with honeycombing, with predominant subpleural distribution. (medscape.com)
  • Pulmonary fibrosis may be a secondary effect of other diseases. (wikipedia.org)
  • Diseases and conditions that may cause pulmonary fibrosis as a secondary effect include: Inhalation of environmental and occupational pollutants, such as metals in asbestosis, silicosis and exposure to certain gases. (wikipedia.org)
  • Interstitial lung diseases such as idiopathic pulmonary fibrosis (IPF) are caused by persistent micro-injuries to alveolar epithelial tissues accompanied by aberrant repair processes. (lu.se)
  • Treatment is directed towards efforts to improve symptoms and may include oxygen therapy and pulmonary rehabilitation. (wikipedia.org)
  • Ask your doctor if you may benefit from a pulmonary rehabilitation program. (healthywomen.org)
  • An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. (medscape.com)
  • pirfenidone is recommended as an option for treating idiopathic pulmonary fibrosis only if the person has a forced vital capacity (FVC) between 50% and 80% predicted. (primarycarenotebook.com)
  • Idiopathic pulmonary fibrosis (IPF) is a progressive lung disorder of unknown etiology for which there is no effective therapy. (medscape.com)
  • Recent Findings on Cell-Based Therapies for COVID19-Related Pulmonary Fibrosis. (bvsalud.org)
  • Symptoms of pulmonary fibrosis are mainly: Shortness of breath, particularly with exertion Chronic dry, hacking coughing Fatigue and weakness Chest discomfort including chest pain Loss of appetite and rapid weight loss Pulmonary fibrosis is suggested by a history of progressive shortness of breath (dyspnea) with exertion. (wikipedia.org)
  • Pulmonary fibrosis eventually causes difficulty breathing and can be life-threatening within a few years after symptoms begin. (medlineplus.gov)
  • Symptoms and signs of idiopathic pulmonary fibrosis typically develop over 6 months to several years and include dyspnea on exertion and nonproductive cough. (msdmanuals.com)
  • The biggest problem in diagnosing and treating pulmonary fibrosis is that the disease may be far along before symptoms begin to appear. (petmd.com)
  • Cats can suffer from many forms of pneumonia, pulmonary fibrosis being one of them. (petmd.com)
  • The first chest radiograph shows prominent reticulonodular pattern in both lower and middle lung fields due to fibrosis. (mudr.org)
  • I have Pulmonary Fibrosis and have recently been diagnosed as also having Rheumatoid Arthritis. (hopkinsarthritis.org)
  • Although virulence and pathogenicity of M. chimaera in lung disease are currently debated, several cases of M. chimaera lung infections have been reported in settings of chronic obstructive pulmonary disease, malignancy, or immunosuppression ( 3 - 5 ). (cdc.gov)
  • People with asthma, chronic obstructive pulmonary disease, or heart failure may experience worsening of their conditions and have difficulty breathing. (cdc.gov)
  • Nitric oxide (NO) is a multi-functional messenger in the lung while the role of NO in HM induced pulmonary pathophysiology is unknown. (cdc.gov)
  • Pretreatment with VPA both increased the survival rate and prevented the loss of body weight in mice with pulmonary fibrosis. (nature.com)
  • International guidelines for the selection of lung transplant candidates: 2006 update--a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. (medscape.com)
  • Such scar tissue causes an irreversible decrease in oxygen diffusion capacity, and the resulting stiffness or decreased compliance makes pulmonary fibrosis a restrictive lung disease. (wikipedia.org)
  • PRECISIONS is a multicenter study that will evaluate biomarkers to aid in the diagnosis of pulmonary fibrosis. (pulmonaryfibrosis.org)
  • Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an unmet need of biomarkers that can aid in the diagnostic and prognostic assessment of the disease and response to treatment. (lu.se)
  • Such effects are similar to the interstitial pulmonary fibrosis reported previously by NIOSH researchers using single-walled carbon nanotubes. (cdc.gov)
  • In contrast, quadriplegia and kyphosis are examples of causes of restrictive lung disease that do not necessarily involve pulmonary fibrosis. (wikipedia.org)
  • Idiopathic pulmonary fibrosis (IPF) is a progressive debilitating lung disease with considerable morbidity. (nih.gov)
  • Common genes implicated in fibrosis are Transforming Growth Factor-Beta (TGF-β), Connective Tissue Growth Factor (CTGF), Epidermal Growth Factor Receptor (EGFR), Interleukin-13 (IL-13), Platelet-Derived Growth Factor (PDGF), Wnt/β-catenin signaling pathway. (wikipedia.org)
  • In an adult, normal wound healing requires the knitting together of damaged tissue, which involves some fibrosis or scarring. (nationaljewish.org)
  • Overall, pretreatment with VPA attenuated the development of pulmonary fibrosis by inhibiting EMT in mice, which was associated with Smad2/3 deactivation but without Akt cellular signal involvement. (nature.com)
  • The Foundation accredits and provides partial funding for interdisciplinary pediatric and adult cystic fibrosis care centers nationwide. (ufl.edu)