Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury.
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.
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 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).
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.
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.
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 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.
Experimentally induced chronic injuries to the parenchymal cells in the liver to achieve a model for LIVER CIRRHOSIS.
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.
Perisinusoidal cells of the liver, located in the space of Disse between HEPATOCYTES and sinusoidal endothelial cells.
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.
Infections with bacteria of the genus PSEUDOMONAS.
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.
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.
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).
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 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 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.
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.
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.
Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules.
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.
Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.
A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.
The fluid excreted by the SWEAT GLANDS. It consists of water containing sodium chloride, phosphate, urea, ammonia, and other waste products.
Material coughed up from the lungs and expectorated via the mouth. It contains MUCUS, cellular debris, and microorganisms. It may also contain blood or pus.
Inflammation of the interstitial tissue of the kidney. This term is generally used for primary inflammation of KIDNEY TUBULES and/or surrounding interstitium. For primary inflammation of glomerular interstitium, see GLOMERULONEPHRITIS. Infiltration of the inflammatory cells into the interstitial compartment results in EDEMA, increased spaces between the tubules, and tubular renal dysfunction.
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 strain of mice widely studied as a model for cystic fibrosis. These mice are generated from embryonic stem cells in which the CFTR (cystic fibrosis transmembrane conductance regulator) gene is inactivated by gene targeting. As a result, all mice have one copy of this altered gene in all their tissues. Mice homozygous for the disrupted gene exhibit many features common to young cystic fibrosis patients, including failure to thrive, meconium ileus, and alteration of mucous and serous glands.
The muscle tissue of the HEART. It is composed of striated, involuntary muscle cells (MYOCYTES, CARDIAC) connected to form the contractile pump to generate blood flow.
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.
Liver diseases caused by infections with PARASITES, such as tapeworms (CESTODA) and flukes (TREMATODA).
Body organ that filters blood for the secretion of URINE and that regulates ion concentrations.
INFLAMMATION of the LIVER in humans that is caused by HEPATITIS C VIRUS lasting six months or more. Chronic hepatitis C can lead to LIVER CIRRHOSIS.
Infections with bacteria of the genus BURKHOLDERIA.
A species of BURKHOLDERIA considered to be an opportunistic human pathogen. It has been associated with various types of infections of nosocomial origin.
A chronic, acquired, idiopathic, progressive eruption of the skin that occurs in the context of RENAL FAILURE. It is sometimes accompanied by systemic fibrosis. The pathogenesis seems to be multifactorial, with postulated involvement of circulating fibrocytes. There is a strong association between this disorder and the use of gadolinium-based contrast agents.
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.
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.
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.
Non-invasive imaging methods based on the mechanical response of an object to a vibrational or impulsive force. It is used for determining the viscoelastic properties of tissue, and thereby differentiating soft from hard inclusions in tissue such as microcalcifications, and some cancer lesions. Most techniques use ultrasound to create the images - eliciting the response with an ultrasonic radiation force and/or recording displacements of the tissue by Doppler ultrasonography.
Inorganic compounds derived from hydrochloric acid that contain the Cl- ion.
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.
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.
Lipid infiltration of the hepatic parenchymal cells resulting in a yellow-colored liver. The abnormal lipid accumulation is usually in the form of TRIGLYCERIDES, either as a single large droplet or multiple small droplets. Fatty liver is caused by an imbalance in the metabolism of FATTY ACIDS.
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.
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.
Measurement of the various processes involved in the act of respiration: inspiration, expiration, oxygen and carbon dioxide exchange, lung volume and compliance, etc.
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.
Pathological processes of the KIDNEY or its component tissues.
Pathological processes involving any part of the LUNG.
Histochemical localization of immunoreactive substances using labeled antibodies as reagents.
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.
Persistent abnormal dilatation of the bronchi.
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.
Elements of limited time intervals, contributing to particular results or situations.
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 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.
A malabsorption condition resulting from greater than 10% reduction in the secretion of pancreatic digestive enzymes (LIPASE; PROTEASES; and AMYLASE) by the EXOCRINE PANCREAS into the DUODENUM. This condition is often associated with CYSTIC FIBROSIS and with chronic PANCREATITIS.
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.
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.
Levels within a diagnostic group which are established by various measurement criteria applied to the seriousness of a patient's disorder.
An enzyme that catalyzes the conversion of L-alanine and 2-oxoglutarate to pyruvate and L-glutamate. (From Enzyme Nomenclature, 1992) EC
The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.
A crystalline compound used as a laboratory reagent in place of HYDROGEN SULFIDE. It is a potent hepatocarcinogen.
Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care. (Dictionary of Health Services Management, 2d ed)
Long convoluted tubules in the nephrons. They collect filtrate from blood passing through the KIDNEY GLOMERULUS and process this filtrate into URINE. Each renal tubule consists of a BOWMAN CAPSULE; PROXIMAL KIDNEY TUBULE; LOOP OF HENLE; DISTAL KIDNEY TUBULE; and KIDNEY COLLECTING DUCT leading to the central cavity of the kidney (KIDNEY PELVIS) that connects to the URETER.
Cell membrane glycoproteins that form channels to selectively pass chloride ions. Nonselective blockers include FENAMATES; ETHACRYNIC ACID; and TAMOXIFEN.
A member of the family of TISSUE INHIBITOR OF METALLOPROTEINASES. It is a N-glycosylated protein, molecular weight 28 kD, produced by a vast range of cell types and found in a variety of tissues and body fluids. It has been shown to suppress metastasis and inhibit tumor invasion in vitro.
The transference of either one or both of the lungs from one human or animal to another.
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.
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
Enlargement of the HEART, usually indicated by a cardiothoracic ratio above 0.50. Heart enlargement may involve the right, the left, or both HEART VENTRICLES or HEART ATRIA. Cardiomegaly is a nonspecific symptom seen in patients with chronic systolic heart failure (HEART FAILURE) or several forms of CARDIOMYOPATHIES.
The geometric and structural changes that the HEART VENTRICLES undergo, usually following MYOCARDIAL INFARCTION. It comprises expansion of the infarct and dilatation of the healthy ventricle segments. While most prevalent in the left ventricle, it can also occur in the right ventricle.
A genus of trichuroid nematodes parasitic in the liver and intestines of many mammals and birds. Two species, C. hepatica and C. philippinensis, produce often fatal infections in man.
Enzymes of the transferase class that catalyze the conversion of L-aspartate and 2-ketoglutarate to oxaloacetate and L-glutamate. EC
The mucous lining of the NASAL CAVITY, including lining of the nostril (vestibule) and the OLFACTORY MUCOSA. Nasal mucosa consists of ciliated cells, GOBLET CELLS, brush cells, small granule cells, basal cells (STEM CELLS) and glands containing both mucous and serous cells.
The channels that collect and transport the bile secretion from the BILE CANALICULI, the smallest branch of the BILIARY TRACT in the LIVER, through the bile ductules, the bile ducts out the liver, and to the GALLBLADDER for storage.
An aminoglycoside, broad-spectrum antibiotic produced by Streptomyces tenebrarius. It is effective against gram-negative bacteria, especially the PSEUDOMONAS species. It is a 10% component of the antibiotic complex, NEBRAMYCIN, produced by the same species.
A strain of albino rat developed at the Wistar Institute that has spread widely at other institutions. This has markedly diluted the original strain.
The phenotypic manifestation of a gene or genes by the processes of GENETIC TRANSCRIPTION and GENETIC TRANSLATION.
The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.
A group of diseases in which the dominant feature is the involvement of the CARDIAC MUSCLE itself. Cardiomyopathies are classified according to their predominant pathophysiological features (DILATED CARDIOMYOPATHY; HYPERTROPHIC CARDIOMYOPATHY; RESTRICTIVE CARDIOMYOPATHY) or their etiological/pathological factors (CARDIOMYOPATHY, ALCOHOLIC; ENDOCARDIAL FIBROELASTOSIS).
A biosynthetic precursor of collagen containing additional amino acid sequences at the amino-terminal and carboxyl-terminal ends of the polypeptide chains.
A mammalian pancreatic extract composed of enzymes with protease, amylase and lipase activities. It is used as a digestant in pancreatic malfunction.
The identification of selected parameters in newborn infants by various tests, examinations, or other procedures. Screening may be performed by clinical or laboratory measures. A screening test is designed to sort out healthy neonates (INFANT, NEWBORN) from those not well, but the screening test is not intended as a diagnostic device, rather instead as epidemiologic.
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 receptor-regulated smad protein that undergoes PHOSPHORYLATION by ACTIVIN RECEPTORS, TYPE I. It regulates TRANSFORMING GROWTH FACTOR BETA and ACTIVIN signaling.
The tubular and cavernous organs and structures, by means of which pulmonary ventilation and gas exchange between ambient air and the blood are brought about.
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.
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.
Blood tests that are used to evaluate how well a patient's liver is working and also to help diagnose liver conditions.
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.
A group of phenotypically similar but genotypically distinct species (genomovars) in the genus BURKHOLDERIA. They are found in water, soil, and the rhizosphere of crop plants. They can act as opportunistic human pathogens and as plant growth promoting and biocontrol agents.
Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.
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.
Schistosomiasis caused by Schistosoma mansoni. It is endemic in Africa, the Middle East, South America, and the Caribbean and affects mainly the bowel, spleen, and liver.
Infections with nematodes of the order ENOPLIDA.
The viscous secretion of mucous membranes. It contains mucin, white blood cells, water, inorganic salts, and exfoliated cells.
Established cell cultures that have the potential to propagate indefinitely.
Extracts prepared from pancreatic tissue that may contain the pancreatic enzymes or other specific uncharacterized factors or proteins with specific activities. PANCREATIN is a specific extract containing digestive enzymes and used to treat pancreatic insufficiency.
A natural high-viscosity mucopolysaccharide with alternating beta (1-3) glucuronide and beta (1-4) glucosaminidic bonds. It is found in the UMBILICAL CORD, in VITREOUS BODY and in SYNOVIAL FLUID. A high urinary level is found in PROGERIA.
Agents that increase mucous excretion. Mucolytic agents, that is drugs that liquefy mucous secretions, are also included here.
The volume of air that is exhaled by a maximal expiration following a maximal inspiration.
A naturally occurring phenomenon where terminally differentiated cells dedifferentiate to the point where they can switch CELL LINEAGES. The cells then differentiate into other cell types.
Pathological processes of the LIVER.
The genetic constitution of the individual, comprising the ALLELES present at each GENETIC LOCUS.
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.
A de novo myeloproliferation arising from an abnormal stem cell. It is characterized by the replacement of bone marrow by fibrous tissue, a process that is mediated by CYTOKINES arising from the abnormal clone.
One or more layers of EPITHELIAL CELLS, supported by the basal lamina, which covers the inner or outer surfaces of the body.
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.
Substances that reduce the growth or reproduction of BACTERIA.
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.
The main structural component of the LIVER. They are specialized EPITHELIAL CELLS that are organized into interconnected plates called lobules.
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.
INFLAMMATION of the LIVER in humans caused by HEPATITIS C VIRUS, a single-stranded RNA virus. Its incubation period is 30-90 days. Hepatitis C is transmitted primarily by contaminated blood parenterally, and is often associated with transfusion and intravenous drug abuse. However, in a significant number of cases, the source of hepatitis C infection is unknown.
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.
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 nitrosamine derivative with alkylating, carcinogenic, and mutagenic properties. It causes serious liver damage and is a hepatocarcinogen in rodents.
The cartilaginous and membranous tube descending from the larynx and branching into the right and left main bronchi.
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.
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 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.
Identification of genetic carriers for a given trait.
One of the mechanisms by which CELL DEATH occurs (compare with NECROSIS and AUTOPHAGOCYTOSIS). Apoptosis is the mechanism responsible for the physiological deletion of cells and appears to be intrinsically programmed. It is characterized by distinctive morphologic changes in the nucleus and cytoplasm, chromatin cleavage at regularly spaced sites, and the endonucleolytic cleavage of genomic DNA; (DNA FRAGMENTATION); at internucleosomal sites. This mode of cell death serves as a balance to mitosis in regulating the size of animal tissues and in mediating pathologic processes associated with tumor growth.
A nodular organ in the ABDOMEN that contains a mixture of ENDOCRINE GLANDS and EXOCRINE GLANDS. The small endocrine portion consists of the ISLETS OF LANGERHANS secreting a number of hormones into the blood stream. The large exocrine portion (EXOCRINE PANCREAS) is a compound acinar gland that secretes several digestive enzymes into the pancreatic ductal system that empties into the DUODENUM.
Hardening of the KIDNEY due to infiltration by fibrous connective tissue (FIBROSIS), usually caused by renovascular diseases or chronic HYPERTENSION. Nephrosclerosis leads to renal ISCHEMIA.
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.)
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.
Application of a ligature to tie a vessel or strangulate a part.
Chemical substances, produced by microorganisms, inhibiting or preventing the proliferation of neoplasms.
Infection of the lung often accompanied by inflammation.
A disturbance in the prooxidant-antioxidant balance in favor of the former, leading to potential damage. Indicators of oxidative stress include damaged DNA bases, protein oxidation products, and lipid peroxidation products (Sies, Oxidative Stress, 1991, pxv-xvi).
All of the processes involved in increasing CELL NUMBER including CELL DIVISION.
The thick green-to-black mucilaginous material found in the intestines of a full-term fetus. It consists of secretions of the INTESTINAL GLANDS; BILE PIGMENTS; FATTY ACIDS; AMNIOTIC FLUID; and intrauterine debris. It constitutes the first stools passed by a newborn.
The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
INFLAMMATION of the LIVER in humans caused by HEPATITIS B VIRUS lasting six months or more. It is primarily transmitted by parenteral exposure, such as transfusion of contaminated blood or blood products, but can also be transmitted via sexual or intimate personal contact.
Salts of alginic acid that are extracted from marine kelp and used to make dental impressions and as absorbent material for surgical dressings.
A non-fibrillar collagen found in the structure of BASEMENT MEMBRANE. Collagen type IV molecules assemble to form a sheet-like network which is involved in maintaining the structural integrity of basement membranes. The predominant form of the protein is comprised of two alpha1(IV) subunits and one alpha2(IV) subunit, however, at least six different alpha subunits can be incorporated into the heterotrimer.
The administration of drugs by the respiratory route. It includes insufflation into the respiratory tract.
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.
Basic glycoprotein members of the SERPIN SUPERFAMILY that function as COLLAGEN-specific MOLECULAR CHAPERONES in the ENDOPLASMIC RETICULUM.
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.
The fibrous tissue that replaces normal tissue during the process of WOUND HEALING.
Gadolinium. An element of the rare earth family of metals. It has the atomic symbol Gd, atomic number 64, and atomic weight 157.25. Its oxide is used in the control rods of some nuclear reactors.
In vitro method for producing large amounts of specific DNA or RNA fragments of defined length and sequence from small amounts of short oligonucleotide flanking sequences (primers). The essential steps include thermal denaturation of the double-stranded target molecules, annealing of the primers to their complementary sequences, and extension of the annealed primers by enzymatic synthesis with DNA polymerase. The reaction is efficient, specific, and extremely sensitive. Uses for the reaction include disease diagnosis, detection of difficult-to-isolate pathogens, mutation analysis, genetic testing, DNA sequencing, and analyzing evolutionary relationships.
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
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 secreted endopeptidase homologous with INTERSTITIAL COLLAGENASE, but which possesses an additional fibronectin-like domain.
An octapeptide that is a potent but labile vasoconstrictor. It is produced from angiotensin I after the removal of two amino acids at the C-terminal by ANGIOTENSIN CONVERTING ENZYME. The amino acid in position 5 varies in different species. To block VASOCONSTRICTION and HYPERTENSION effect of angiotensin II, patients are often treated with ACE INHIBITORS or with ANGIOTENSIN II TYPE 1 RECEPTOR BLOCKERS.
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.
A preparation of hog pancreatic enzymes standardized for lipase content.
Hypersensitivity reaction (ALLERGIC REACTION) to fungus ASPERGILLUS in an individual with long-standing BRONCHIAL ASTHMA. It is characterized by pulmonary infiltrates, EOSINOPHILIA, elevated serum IMMUNOGLOBULIN E, and skin reactivity to Aspergillus antigen.
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.
Chinese herbal or plant extracts which are used as drugs to treat diseases or promote general well-being. The concept does not include synthesized compounds manufactured in China.
Binary classification measures to assess test results. Sensitivity or recall rate is the proportion of true positives. Specificity is the probability of correctly determining the absence of a condition. (From Last, Dictionary of Epidemiology, 2d ed)
Inflammation of the lung due to harmful effects of ionizing or non-ionizing radiation.
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.
A non-specific host defense mechanism that removes MUCUS and other material from the LUNGS by ciliary and secretory activity of the tracheobronchial submucosal glands. It is measured in vivo as mucus transfer, ciliary beat frequency, and clearance of radioactive tracers.
Tomography using x-ray transmission and a computer algorithm to reconstruct the image.
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 type species of gram negative, aerobic bacteria in the genus ACHROMOBACTER. Previously in the genus ALCALIGENES, the classification and nomenclature of this species has been frequently emended. The two subspecies, Achromobacter xylosoxidans subsp. denitrificans and Achromobacter xylosoxidans subsp. xylosoxidans are associated with infections.
The measurement of an organ in volume, mass, or heaviness.
Pyridine derivatives with one or more keto groups on the ring.
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.
Observation of a population for a sufficient number of persons over a sufficient number of years to generate incidence or mortality rates subsequent to the selection of the study group.
In screening and diagnostic tests, the probability that a person with a positive test is a true positive (i.e., has the disease), is referred to as the predictive value of a positive test; whereas, the predictive value of a negative test is the probability that the person with a negative test does not have the disease. Predictive value is related to the sensitivity and specificity of the test.
A species of gram-negative bacteria that causes disease in plants. It is found commonly in the environment and is an opportunistic pathogen in humans.
Impairment of bile flow due to obstruction in small bile ducts (INTRAHEPATIC CHOLESTASIS) or obstruction in large bile ducts (EXTRAHEPATIC CHOLESTASIS).
Restoration of integrity to traumatized tissue.
A membrane of squamous EPITHELIAL CELLS, the mesothelial cells, covered by apical MICROVILLI that allow rapid absorption of fluid and particles in the PERITONEAL CAVITY. The peritoneum is divided into parietal and visceral components. The parietal peritoneum covers the inside of the ABDOMINAL WALL. The visceral peritoneum covers the intraperitoneal organs. The double-layered peritoneum forms the MESENTERY that suspends these organs from the abdominal wall.
Sweat-producing structures that are embedded in the DERMIS. Each gland consists of a single tube, a coiled body, and a superficial duct.
Macromolecular organic compounds that contain carbon, hydrogen, oxygen, nitrogen, and usually, sulfur. These macromolecules (proteins) form an intricate meshwork in which cells are embedded to construct tissues. Variations in the relative types of macromolecules and their organization determine the type of extracellular matrix, each adapted to the functional requirements of the tissue. The two main classes of macromolecules that form the extracellular matrix are: glycosaminoglycans, usually linked to proteins (proteoglycans), and fibrous proteins (e.g., COLLAGEN; ELASTIN; FIBRONECTINS; and LAMININ).
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.
Proteins prepared by recombinant DNA technology.
A graphic means for assessing the ability of a screening test to discriminate between healthy and diseased persons; may also be used in other studies, e.g., distinguishing stimuli responses as to a faint stimuli or nonstimuli.
Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors.
FIBROSIS of the hepatic parenchyma due to chronic excess ALCOHOL DRINKING.
Striated muscle cells found in the heart. They are derived from cardiac myoblasts (MYOBLASTS, CARDIAC).
Phenols substituted in any position by an amino group.
The relationship between the dose of an administered drug and the response of the organism to the drug.
Abnormal increase of resistance to blood flow within the hepatic PORTAL SYSTEM, frequently seen in LIVER CIRRHOSIS and conditions with obstruction of the PORTAL VEIN.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
An endopeptidase that is structurally similar to MATRIX METALLOPROTEINASE 2. It degrades GELATIN types I and V; COLLAGEN TYPE IV; and COLLAGEN TYPE V.
Techniques and strategies which include the use of coding sequences and other conventional or radical means to transform or modify cells for the purpose of treating or reversing disease conditions.
The movement of ions across energy-transducing cell membranes. Transport can be active, passive or facilitated. Ions may travel by themselves (uniport), or as a group of two or more ions in the same (symport) or opposite (antiport) directions.
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.
A complex of gadolinium with a chelating agent, diethylenetriamine penta-acetic acid (DTPA see PENTETIC ACID), that is given to enhance the image in cranial and spinal MRIs. (From Martindale, The Extra Pharmacopoeia, 30th ed, p706)
A class of statistical methods applicable to a large set of probability distributions used to test for correlation, location, independence, etc. In most nonparametric statistical tests, the original scores or observations are replaced by another variable containing less information. An important class of nonparametric tests employs the ordinal properties of the data. Another class of tests uses information about whether an observation is above or below some fixed value such as the median, and a third class is based on the frequency of the occurrence of runs in the data. (From McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed, p1284; Corsini, Concise Encyclopedia of Psychology, 1987, p764-5)
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.
Invasion of the host RESPIRATORY SYSTEM by microorganisms, usually leading to pathological processes or diseases.
Enlargement of the LEFT VENTRICLE of the heart. This increase in ventricular mass is attributed to sustained abnormal pressure or volume loads and is a contributor to cardiovascular morbidity and mortality.
A chemokine that is a chemoattractant for MONOCYTES and may also cause cellular activation of specific functions related to host defense. It is produced by LEUKOCYTES of both monocyte and lymphocyte lineage and by FIBROBLASTS during tissue injury. It has specificity for CCR2 RECEPTORS.
The mass or quantity of heaviness of an individual. It is expressed by units of pounds or kilograms.
A system of vessels in which blood, after passing through one capillary bed, is conveyed through a second set of capillaries before it returns to the systemic circulation. It pertains especially to the hepatic portal system.

Enhanced Th1 and dampened Th2 responses synergize to inhibit acute granulomatous and fibrotic responses in murine schistosomiasis mansoni. (1/5330)

In murine schistosomiasis mansoni, CD4(+) Th1 and Th2 cells participate in the ovum-induced granulomatous inflammation. Previous studies showed that the interleukin-12 (IL-12)-induced Th1 response strongly suppressed the Th2-cell-mediated pulmonary granuloma development in naive or primed mice. However, liver granulomas were only moderately suppressed in egg-vaccinated, recombinant IL-12 (rIL-12)-treated infected mice. The present study shows that repeated rIL-12 injections given during early granuloma development at 5 to 7 weeks after infection prolonged the Th1 phase and resulted in gamma interferon-mediated suppression of liver granulomas. The timing is crucial: if given at 6 to 8 weeks, during the Th2-dominated phase of florid granuloma growth, the treatment is ineffective. Daily injections of rIL-12 given between 5 and 7.5 weeks during the period of granuloma growth achieved a somewhat-stronger diminution in granuloma growth with less deposition of collagen but caused 60% mortality and liver pathology. In contrast, combined treatment with rIL-12 and anti-IL-4-anti-IL-10 monoclonal antibody (MAb) injections given during the Th2 phase strongly inhibited liver granuloma growth without mortality. The diminished inflammatory response was accompanied by less deposition of collagen in the liver. Moreover, neutralization of endogenous IL-12 by anti-IL-12 MAbs effectively decreased the early Th1 phase (between 5 and 6 weeks after infection) but not the developing Th2 phase (5 to 7 weeks) of granuloma development. These studies indicate that the granulomatous response in infected mice can be manipulated by utilizing the Th1-Th2-subset antagonism with potential salutary results in the amelioration of fibrous pathology.  (+info)

Elevated carboxy terminal cross linked telopeptide of type I collagen in alcoholic cirrhosis: relation to liver and kidney function and bone metabolism. (2/5330)

BACKGROUND: The carboxy terminal cross linked telopeptide of type I collagen (ICTP) has been put forward as a marker of bone resorption. Patients with alcoholic liver disease may have osteodystrophy. AIMS: To assess circulating and regional concentrations of ICTP in relation to liver dysfunction, bone metabolism, and fibrosis. METHODS: In 15 patients with alcoholic cirrhosis and 20 controls, hepatic venous, renal venous, and femoral arterial concentrations of ICTP, and bone mass and metabolism were measured. RESULTS: Circulating ICTP was higher in patients with cirrhosis than in controls. No overall significant hepatic disposal or production was found in the patient or control groups but slightly increased production was found in a subset of patients with advanced disease. Significant renal extraction was observed in the controls, whereas only a borderline significant extraction was observed in the patients. Measurements of bone mass and metabolism indicated only a mild degree of osteodystrophy in the patients with cirrhosis. ICTP correlated significantly in the cirrhotic patients with hepatic and renal dysfunction and fibrosis, but not with measurements of bone mass or metabolism. CONCLUSIONS: ICTP is highly elevated in patients with cirrhosis, with no detectable hepatic net production or disposal. No relation between ICTP and markers of bone metabolism was identified, but there was a relation to indicators of liver dysfunction and fibrosis. As the cirrhotic patients conceivably only had mild osteopenia, the elevated ICTP in cirrhosis may therefore primarily reflect liver failure and hepatic fibrosis.  (+info)

3D MRI of the membranous labyrinth. An age related comparison of MR findings in patients with labyrinthine fibrosis and in persons without inner ear symptoms. (3/5330)

PURPOSE: We compared MRI of the membranous labyrinth in patients with chronic non-neoplastic inner ear disease and MR signs of labyrinthine fibrosis and controls depending on their age, in order to establish whether there were any MR differences regarding patient age groups, control age groups and between the patients and controls themselves. MATERIALS AND METHODS: Clinical ENT examinations as well as a T2* weighted 3D CISS (Constructive Interference in Steady State) sequence with a slice thickness of 0.7 mm were performed. Our collective was subdivided as follows: 0-19 years (10 controls, 3 patients with chronic non-neoplastic inner ear disease), 20-49 years (55 controls, 8 patients), 50 years and older (40 controls, 22 patients). Detectability of labyrinthine structures (e.g. cochlea, vestibule, semicircular canals) and filling defects were evaluated. RESULTS: In the 3 age-groups of the control collective no significant differences were observed in the membranous labyrinth. However differences concerning labyrinthine detectability emerged between controls and patients in both the 20-49 years and 50 years and older age groups. In the patient collective the 3 age groups showed no significant discrepancy in the mean number of lesions. CONCLUSION: Filling defects of the membranous labyrinth on 3D CISS MR images are pathological even in older persons. We would therefore recommend high resolution T2* weighted MRI in the case of suspected labyrinthine fibrosis.  (+info)

Ultramicroscopic structures of the leptomeninx of mice with communicating hydrocephalus induced by human recombinant transforming growth factor-beta 1. (4/5330)

An experimental model of communicating hydrocephalus was developed based on intrathecal injection of human recombinant transforming growth factor-beta 1 (hrTGF-beta 1) in the mouse. To clarify the mechanism of this hydrocephalus model, the ultrastructure of the leptomeninx in the process of ventricular dilation was examined in C57/BL6 mice injected intrathecally with 60 ng of hrTGF-beta 1. The leptomeninx was examined at various periods after injection by light and electron microscopy. Immunostaining for fibroblasts and macrophages was also performed. Leptomeninx within a week after injection showed that the thin cytoplasmic processes of leptomeningeal cells formed a laminated structure with a meshwork, which was almost the same as the controls. In the second week, many cells with a round nucleus appeared in the leptomeninx. Immunohistochemically, these cells were positive for anti-fibroblast antibody and negative for anti-Mac-1 and anti-macrophage BM-8 antibodies. Three weeks later, the laminated structure was disrupted and abundant deposition of collagen fibers was found in the inter-cellular space of the leptomeninx. Such inter-meningeal fibrosis would disturb cerebrospinal fluid flow in the mouse leptomeninx and cause slowly progressive ventricular dilation.  (+info)

Irradiation induces upregulation of CD31 in human endothelial cells. (5/5330)

Radiation-induced vascular injury is believed to be a major factor contributing to parenchymal atrophy, fibrosis and necrosis in normal tissue after radiotherapy. In this study irradiation of human umbilical vein endothelial cells (HUVECs) significantly increased adherence of U-937 cells in a time-dependent manner. Given the potential multifunctional role of CD31 in the vasculature we have examined the possible effects of irradiation on levels of CD31 expression in HUVECs. Irradiation upregulated CD31 expression on HUVECs, independently of initial plating density and radiation-induced changes such as cell number, cell cycle stage, or cell size. CD31 mRNA levels were raised in irradiated HUVECs relative to controls. Both CD31 mRNA and surface protein showed similar changes, suggesting that the increase in mRNA in irradiated HUVECs is responsible for the elevation in cell surface protein. A semi-quantitative study of tissue specimens from patients who had received radiotherapy indicated that CD31 staining in the blood vessels from irradiated tissues was increased compared with controls. Endothelial CD31 is important in the transmigration of leukocytes. We have demonstrated that the incorporation of monoclonal antibody to CD31 significantly inhibited the transmigration of human peripheral blood leukocytes through a monolayer of irradiated HUVECs. Taken together these data strongly suggest that irradiation induces a marked increase in CD31 expression on endothelial cells as part of a general response to irradiation. Its upregulation may play an important role in the development of radiation-induced normal tissue damage and thus is a possible target for therapeutic intervention.  (+info)

Pancreatic stellate cells are activated by proinflammatory cytokines: implications for pancreatic fibrogenesis. (6/5330)

BACKGROUND: The pathogenesis of pancreatic fibrosis is unknown. In the liver, stellate cells play a major role in fibrogenesis by synthesising increased amounts of collagen and other extracellular matrix (ECM) proteins when activated by profibrogenic mediators such as cytokines and oxidant stress. AIMS: To determine whether cultured rat pancreatic stellate cells produce collagen and other ECM proteins, and exhibit signs of activation when exposed to the cytokines platelet derived growth factor (PDGF) or transforming growth factor beta (TGF-beta). METHODS: Cultured pancreatic stellate cells were immunostained for the ECM proteins procollagen III, collagen I, laminin, and fibronectin using specific polyclonal antibodies. For cytokine studies, triplicate wells of cells were incubated with increasing concentrations of PDGF or TGF-beta. RESULTS: Cultured pancreatic stellate cells stained strongly positive for all ECM proteins tested. Incubation of cells with 1, 5, and 10 ng/ml PDGF led to a significant dose related increase in cell counts as well as in the incorporation of 3H-thymidine into DNA. Stellate cells exposed to 0.25, 0.5, and 1 ng/ml TGF-beta showed a dose dependent increase in alpha smooth muscle actin expression and increased collagen synthesis. In addition, TGF-beta increased the expression of PDGF receptors on stellate cells. CONCLUSIONS: Pancreatic stellate cells produce collagen and other extracellular matrix proteins, and respond to the cytokines PDGF and TGF-beta by increased proliferation and increased collagen synthesis. These results suggest an important role for stellate cells in pancreatic fibrogenesis.  (+info)

Cyclosporin exerts a direct fibrogenic effect on human tubulointerstitial cells: roles of insulin-like growth factor I, transforming growth factor beta1, and platelet-derived growth factor. (7/5330)

To assess the direct fibrogenic effects of cyclosporin A (CyA) on the human tubulointerstitium, primary cultures of human renal proximal tubule cells (PTC) and renal cortical fibroblasts (CF) were incubated for 24 h with various concentrations of CyA. Cytotoxicity was confirmed in both cell populations by dose-dependent inhibition of thymidine incorporation, viability, and PTC apical sodium-hydrogen exchange activity (ethylisopropylamiloride-sensitive apical 22Na+ uptake). Compared with controls, both 500 and 1000 ng/ml CyA significantly stimulated CF collagen synthesis (proline incorporation 4.6 +/- 0.4, 6.5 +/- 0.8, and 7.1 +/- 1.0%, respectively; p <.05) and inhibited matrix metalloproteinase-2 (100%, 85.7 +/- 10.0%, and 38.8 +/- 9.2%) and matrix metalloproteinase-9 activity (100%, 110.6 +/- 19.0%, and 49.9 +/- 12.8%). CyA did not affect CF secretion of transforming growth factor beta1, but markedly stimulated insulin-like growth factor-I (IGF-I) secretion and inhibited secretion of both IGF-I binding protein-(IGFBP)-3 and IGFBP-2. CyA-induced CF collagen synthesis was abrogated by 5 microgram/ml anti-IGF-I receptor antibody, but not by 5 microgram/ml murine nonimmune globulin. Increasing concentrations of CyA progressively augmented PTC secretion of the fibrogenic cytokines transforming growth factor-beta1 and platelet-derived growth factor. These results indicate that clinically relevant concentrations of CyA are directly toxic to PTC and CF, irrespective of hemodynamic effects, and promote interstitial fibrosis by inhibiting matrix degradation and stimulating cortical fibroblast collagen synthesis via induction of autocrine IGF-I action. The latter effect may be further accentuated by the ability of CyA to augment secretion of transforming growth factor beta1 and platelet-derived growth factor by PTCs.  (+info)

Prognostic significance of bone marrow biopsy in essential thrombocythemia. (8/5330)

BACKGROUND AND OBJECTIVE: The diagnostic and prognostic value of bone marrow biopsy (BMB) has been widely investigated in patients with chronic myeloproliferative disorders (CMPD). The present study is based on a review of the results of routine BMBs taken from 93 essential thrombocythemia (ET) patients at the time of diagnosis. DESIGN AND METHODS: The common BMB histologic parameters and clinico-hematologic variables were considered for diagnostic and prognostic purposes. Clinico-pathologic correlations were looked for univariately. Moreover, the diagnostic significance of the histologic findings was tested by means of cluster analysis. Overall survival and event-free survival were considered as prognostic endpoints. RESULTS: There were no correlations between the clinic and pathologic findings, and none of the histologic and clinical parameters was predictive of survival or the occurrence of major clinical events. Cluster analysis of the BMB findings revealed two distinct morphologic patterns: one was clearly myeloproliferative; the other had somewhat dysplastic features. The event-free and overall survival rates in the latter group were significantly worse (p = 0.0377 and p = 0.0162 respectively), with major ischemic events accounting for most of the difference in event-free survival. INTERPRETATION AND CONCLUSIONS: These results have no clearcut counterpart in the literature, but we feel that dysplastic BMB findings could be included in the definition of ET prognostic scores in order to allow therapeutic strategies to be adapted to the level of risk.  (+info)

The present PhD proposes a rigorous evaluation and quantification of pediatric liver allograft fibrosis in 595 liver biopsies of 139 patients from 6 months to 10 years following liver transplantation, analyzing the influence of clinical variables and the immunosuppression on fibrosis progression, as well as the relevance of activated Hepatic Stellate cells (HSCs) in predicting allograft fibrosis development. A novel fibrosis scoring system, the Liver Allograft Fibrosis Score, was designed and validated specifically for allograft fibrosis assessment, categorizing separately sinusoidal, centrilobular and portal fibrosis. Progressive fibrosis was found in 74% of the liver biopsies reviewed. Clinical factors were identified to promote fibrosis in a specific area of the liver parenchyma. The steroid therapy did not contribute to reduce fibrosis. Finally, the high proportion of activated-HSCs found in this population was useful as a predictor of long-term allograft fibrosis development.. [fre] Cette ...
Liver organ fibrosis is the final stage of liver diseases that lead to liver failure and malignancy. gene manifestation was inhibited in the advanced stage of liver fibrosis while transient but prominent MMP13 gene manifestation occurs during the early stage of recovery from experimental rat liver organ fibrosis. It is therefore acceptable to consider that upregulation of MMP13 gene appearance can lead to a consequent upsurge in collagenase activity in fibrotic livers and provide a procedure for antifibrotic therapy in preventing liver organ fibrosis. Since comprehensive regression from advanced cirrhosis is normally both doubtful and controversial it really is even more feasible to build up a secure and efficient method of preventing the development of fibrosis in liver organ disease. In regards to towards the transfer from the MMP gene in to the liver organ youll be able to selectively inject nude DNA in to the liver organ via either the portal vein or the hepatic artery. Gene delivery ...
Background: Cardiac fibrosis contributes to heart failure progression following myocardial infarction (MI) and chronic kidney disease (uremic cardiomyopathy). We examined the effect of the anti-fibrotic agent, tranilast, on cardiac fibrosis in both heart and kidney failure.. Methods: MI was induced by coronary artery ligation in Sprague Dawley (SD) rats, and animals randomized to receive tranilast (300mg/kg/day, p.o.) or vehicle for 28 days. Renal failure was induced by 5/6 nephrectomy (STNx) and animals randomized to receive tranilast (300mg/kg/day, p.o.) or vehicle for 12 weeks. Myocardial tissues were harvested for histological analysis. To determine direct effects of tranilast, cardiac fibrosis independent of any hemodynamic influence, neonatal cardiac fibroblasts (NCF) were stimulated with TGFβ1 in the presence and absence of tranilast and examined for proline incorporation, profibrotic gene and phospho-Smad2 protein expression.. Results: Tranilast treatment in MI animals attenuated the ...
TY - JOUR. T1 - Novel anti-fibrotic therapies. AU - McVicker, Benita L.. AU - Bennett, Robert G.. PY - 2017/5/31. Y1 - 2017/5/31. N2 - Fibrosis is a major player in cardiovascular disease, both as a contributor to the development of disease, as well as a post-injury response that drives progression. Despite the identification of many mechanisms responsible for cardiovascular fibrosis, to date no treatments have emerged that have effectively reduced the excess deposition of extracellular matrix associated with fibrotic conditions. Novel treatments have recently been identified that hold promise as potential therapeutic agents for cardiovascular diseases associated with fibrosis, as well as other fibrotic conditions. The purpose of this review is to provide an overview of emerging antifibrotic agents that have shown encouraging results in preclinical or early clinical studies, but have not yet been approved for use in human disease. One of these agents is bone morphogenetic protein-7 (BMP7), which ...
TY - JOUR. T1 - Review: Endothelial-myofibroblast transition, a new player in diabetic renal fibrosis. AU - Li, Jinhua. AU - Bertram, John Frederick. PY - 2010. Y1 - 2010. N2 - This review considers the evidence for endothelialmesenchymal transition (EndoMT) as a mechanism contributing to progressive renal fibrosis in experimental kidney disease, with an emphasis on diabetic nephropathy. Recent findings using genetically tagged endothelial cells are critically discussed, and directions for future studies proposed.. AB - This review considers the evidence for endothelialmesenchymal transition (EndoMT) as a mechanism contributing to progressive renal fibrosis in experimental kidney disease, with an emphasis on diabetic nephropathy. Recent findings using genetically tagged endothelial cells are critically discussed, and directions for future studies proposed.. UR - U2 - 10.1111/j.1440-1797.2010.01319.x. DO - ... Researchers at the University of Birmingham in the U.K. say they have shown that a novel low molecular weight dextran-sulphate, ILB®, could play a key role in treating open-angle glaucoma (OAG), a neurodegenerative disease that affects over 70 million people worldwide and causes irreversible blindness. OAG develops slowly over many years. Fibrosis within the eye’s main fluid drainage site can lead to increased intraocular pressure (IOP), resulting in damage to the optic nerve. The team’s study “ILB resolves inflammatory scarring and promotes functional tissue repair,” reported in npj Regenerative Medicine, has shown that that ILB can normalize matrix deposition inside the eye and lower IOP in a preclinical model used to mimic these aspects of human glaucoma, paving the way for new anti-fibrotic therapies to be developed for the disease. OAG is a complex disease and it has proved difficult to develop effective therapeutics to target the biochemical pathways
Background: Prognostic value of myocardial fibrosis in patients with non-ischemic idiopathic cardiomyopathy (NICM) is not well-defined. We sought to assess the association of focal and diffuse myocardial fibrosis to left ventricular reversed remodeling.. Methods: Patients with NICM who underwent cardiac MRI and baseline and subsequent follow-up echocardiographic were included in the study. Post-contrast T1 times were measured using Look-Locker gradient echo, and was adjusted for renal function, body size, gadolinium dose and delayed time after Gadolinium injection. Patients were followed over a median time of 29 months (20,37) to evaluate changes of left ventricular end-systolic volume index (LVESVi) over time. Linear Mixed Model was used to assess the relationship between the LVESVi during follow-up,T1 value, and delayed hyperenhancement (DHE).. Results: A total of 103 patients (mean age 51±15 years, 62% male) were included in the analysis. Mean LVEF 32±10%, LVESVi 62±39 ml/m2, and T1 time ...
Cardiac fibrosis as a result of excessive extracellular matrix deposition leads to stiffening of the heart, which can eventually lead to heart failure. An important event in cardiac fibrosis is the transformation of fibroblasts into myofibroblasts, which secrete large amounts of extracellular matrix proteins. While the function of protein-coding genes in myofibroblast activation and fibrosis has been a topic of investigation for a long time, it has become clear that non-coding RNAs also play key roles in cardiac fibrosis. This review discusses the involvement of microRNAs and lncRNAs in cardiac fibrosis and summarizes the issues related to translating these findings into real life therapies. ...
Fibrosis contributes to the development of many diseases and many target molecules are involved in fibrosis. Currently, the majority of fibrosis treatment strategies are limited to specific diseases or organs. However, accumulating evidence demonstrates great similarities among fibroproliferative diseases, and more and more drugs are proved to be effective anti-fibrotic therapies across different diseases and organs. Here we comprehensively review the current knowledge on the pathological mechanisms of fibrosis, and divide factors mediating fibrosis progression into extracellular and intracellular groups. Furthermore, we systematically summarize both single and multiple component drugs that target fibrosis. Future directions of fibrosis drug discovery are also proposed.
Renal fibrosis is a serious clinical problem forming the utmost cause of need for renal replacement therapy. No adequate preventive or curative therapy is available that can be clinically used to specifically target renal fibrosis. The search for new efficacious treatment strategies is therefore warranted. Although in vitro models using homogeneous cell populations have contributed to the understanding of the pathogenetic mechanisms involved in renal fibrosis, these models poorly mimic the complex in vivo milieu. Therefore, here we evaluated a precision-cut kidney slice (PCKS) model as a new, multicellular ex vivo model to study development of fibrosis and the prevention thereof using anti-fibrotic compounds.. Precision-cut slices (200-300 µm thickness) were prepared from healthy C57BL/6 mouse kidneys using a Krumdieck tissue slicer. To induce changes mimicking the fibrotic process, slices were incubated with TGFβ1 (5 ng/ml) for 48 hours in the presence or absence of the anti-fibrotic cytokine ...
Results Nintedanib dose-dependently reduced platelet-derived growth factor-induced and transforming growth factor-β-induced proliferation and migration as well as myofibroblast differentiation and collagen release of dermal fibroblasts from patients with and healthy individuals. Nintedanib also inhibited the endogenous activation of SSc fibroblasts. Nintedanib prevented bleomycin-induced skin fibrosis in a dose-dependent manner and was also effective in the treatment of established fibrosis. Moreover, treatment with nintedanib ameliorated fibrosis in the chronic graft-versus-host disease model and in tight-skin-1 mice in well-tolerated doses. ...
Both groups survived long-term (,100 days). The allogeneic grafts were infiltrated by significantly increased numbers of CD4+ (P ,0.0001), CD8+ (P ,0.0001), and CD11b+ cells (P = 0.0065) by day 100. Furthermore, elevated IL-13 levels (P = 0.0003) and numbers of infiltrating IL-13+ cells (P = 0.0037), together with an expression of IL-13Rα2, were detected only within allografts. The expression of IL-13 and IL-13Rα2 resulted in significantly increased TGF-β1 levels (P ,0.0001), higher numbers of CD11bhighGr1intermediateTGF-β1+ cells, and elevated cardiac collagen deposition (P = 0.0094). The allograft fibrosis found in these experiments was accompanied by upregulation of multiple profibrotic genes, which was confirmed by immunohistochemical stainings of allograft tissue. Blockage of the IL-13/TGF-β1 interaction by IL-13Rα2 siRNA led to lower numbers of CD11bhighGr1intermediateTGF-β1+, CD4+, CD8+, and CD11b+ cells, and prevented collagen deposition (P = 0.0018) within these allografts ...
Renal fibrosis is a main cause of end-stage renal disease. Clinically, there is no beneficial treatment that can effectively reverse the progressive loss of renal function. We recently synthesized a novel proteolysis-resistant cyclic helix B peptide (CHBP) that exhibits promising renoprotective effects. In this study, we evaluated the effect of CHBP on renal fibrosis in an in vivo ischemia reperfusion injury (IRI) model and in vitro TGF-β-stimulated tubular epithelial cells (TCMK-1 and HK-2) model. In the IRI in vivo model, mice were randomly divided into sham (sham operation), IR and IR + CHBP groups (n = 6). CHBP (8 nmol/kg) was administered intraperitoneally at the onset of reperfusion, and renal fibrosis was evaluated at 12 weeks post-reperfusion. Our results showed that CHBP markedly attenuated the IRI-induced deposition of collagen I and vimentin. In the in vitro model, CHBP reversed the TGF-β-induced down-regulation of E-cadherin and up-regulation of α-SMA and vimentin. Furthermore, CHBP
Fibrosis is defined by the deposition of pathological fibrillar collagen, inflammatory cells, loss of capillaries, and dedifferentiation of epithelial cells. Fibrosis occurs as a part of normal wound healing. However, excessive or dysregulated fibrosis can lead to severe organ dysfunction and is a feature of a variety of diseases. Due to its insidious onset, fibrosis tends to go undetected in its early stages. This is in part why these diseases remain so poorly understood. Animal models have provided a means to examine these early stages and to isolate and understand the effect of perturbations in signaling pathways, chemokines, and cytokines. Here, we summarize recent progress in the understanding of the molecular pathogenesis of fibrosis, both its initiation and its maintenance phases, from animal models of fibrosis in the skin and liver. Due to these organs properties, modeling fibrosis in them poses unique challenges. Elegant solutions have therefore been developed for modeling fibrosis in
Fibrosis is characterized by persistent deposition of extracellular matrix (ECM) by fibroblasts. Fibroblast mechanosensing of a stiffened ECM is hypothesized to drive the fibrotic program; however, the spatial distribution of ECM mechanics and their derangements in progressive fibrosis are poorly ch …
Oct. 19, 2017 /PRNewswire/ -- SummaryGlobal Markets Directs latest Pharmaceutical and Healthcare disease pipeline guide Kidney Fibrosis - Pipeline Review, H2 2017, provides an overview of the Kidney Fibrosis (Genito Urinary System And Sex Hormones) pipeline landscape.Download the full.....
Summary Latest Pharmaceutical and Healthcare disease pipeline guide Kidney Fibrosis Pipeline Review, H1 2017, provides an overview of the Kidney Fibrosis (
The global kidney fibrosis treatment market is expected to register growth over the forecast period, driven by the increasing global geriatric population base, rising disposable income of the patients, and growing awareness about renal fibrosis treatment
TY - JOUR. T1 - Intractable diarrhea caused by intestinal fibrosis. AU - Ferretti, F.. AU - Bella, S.. AU - Boldrini, R.. AU - Gambarara, M.. AU - Capuano, L.. AU - Papadatou, B.. AU - Bosman, C.. AU - Diamanti, A.. AU - Castro, M.. PY - 1998. Y1 - 1998. UR - UR - U2 - 10.1016/S0041-1345(98)00717-9. DO - 10.1016/S0041-1345(98)00717-9. M3 - Article. C2 - 9745478. AN - SCOPUS:0031710426. VL - 30. SP - 2541. EP - 2542. JO - Transplantation Proceedings. JF - Transplantation Proceedings. SN - 0041-1345. IS - 6. ER - ...
Tubulointerstitial fibrosis invariably accompanies the course of chronic renal failure towards end-stage renal disease. Tubular epithelial cells, the predominant cell type in the tubulointerstitium, are increasingly being recognized for playing a dominant role as mediators of renal fibrogenesis. Tub …
Fibrosis occurs when transforming growth factor-β (TGF-β) signaling is not attenuated after wound healing and fibroblasts remain activated, resulting in tissue scarring. The mechanisms that turn off TGF-β signaling after wound healing and inhibit this pathway in general are not well-understood. Palumbo-Zerr et al. noted that the abundance of the orphan receptor NR4A1 (nuclear receptor 4A1) was increased at the mRNA and protein levels in the fibrotic skin of patients with systemic sclerosis and in the skin of mice overexpressing a constitutively active TGF-β receptor type I (TBRI), which are a model for fibrosis. TGF-β stimulated the increased expression of NR4A1 through a complex containing the transcriptional effectors SMAD3, SMAD4, and SP1. Global Nr4a1 deficiency enhanced the expression of TGF-β target genes and fibrosis in mice overexpressing TBRI, as well as in other mouse models of fibrosis, including pulmonary fibrosis induced by bleomycin. Nr4a1-/- fibroblasts deposited more ...
Myofibroblas adalah sumber utama komponen matriks ekstraseluler yang terakumulasi selama fibrosis jaringan, dan hepatic stellate cells (HSC) dipercaya menjadi sumber utama myofibroblas di dalam liver. Sampai saat ini, sistem yang kuat untuk memanipulasi sel-sel ini secara genetik belum dikembangkan. Dilaporkan bahwa Cre di bawah kontrol promoter Pdgfrb (Pdgfrb-Cre) menginaktivasi gen loxP-flanked di dalam tikus HSC…
Alterations in Titin Properties and Myocardial Fibrosis Correlate With Clinical Phenotypes in Hemodynamic Subgroups of Severe Aortic Stenosis: Titin-isoform exp
Alterations in Titin Properties and Myocardial Fibrosis Correlate With Clinical Phenotypes in Hemodynamic Subgroups of Severe Aortic Stenosis: Titin-isoform exp
Progression of renal fibrosis: the underestimated role of endothelial alterations. . Biblioteca virtual para leer y descargar libros, documentos, trabajos y tesis universitarias en PDF. Material universiario, documentación y tareas realizadas por universitarios en nuestra biblioteca. Para descargar gratis y para leer online.
Causes of Muscular fibrosis, multifocal - obstructed vessels including triggers, hidden medical causes of Muscular fibrosis, multifocal - obstructed vessels, risk factors, and what causes Muscular fibrosis, multifocal - obstructed vessels.
Fibrosis refers to a phenomenon of development of excessive connective tissue as a result of some injury or some disease. It results in formation of a laye
Objective: To analyze the CT imaging results of patients with COVID-19 who previously received several follow-up visits and to explain the changes in pulmonary inflammation. Methods: Cases of 15 patients with COVID-19 were retrospectively analyzed: their epidemiology, clinical history, laboratory tests, and multiple CT chest scans obtained during the disease period were studied. Results: The CT scans of the 15 patients showed different results. Four patients had no abnormal findings in their chest CT scans. The first scan of 1 patient revealed right lower lobe inflammation, while the lesion had been completely absorbed in follow-up. Two patients showed bilateral pulmonary inflammation in the first scan which had been absorbed by follow-up but the last examination showed extensive fibrosis. Two patients had no abnormalities in their first CT scans, while pulmonary inflammation was found in the second scan and this had not been completely absorbed by the last follow-up. One patient had pulmonary ...
One of the most difficult problems to tackle is the development of fibrosis (scarring) which is a direct consequence of the kidneys limited capacity to regenerate after injury. Such fibrosis can develop into a life-threatening condition. At present we may be able to halt the progression of renal scarring, but thats all. Early detection of CKD would allow improved disease management.. One thing to remember is that the aetiology of CKD is diverse. It includes immunological, mechanical, metabolic and toxic origins amongst others. To complicate the matter further it will affect three functional compartments of the kidney: the vasculature, glomerulus and tubulointerstitium . Therefore, it is extremely challenging to choose the optimal therapy. At present, renal biopsy offers one of the best methods to test for the presence of fibrosis but it requires surgical intervention, is painful, and it is very costly. A non-invasive method to detect the early incidence of such fibrosis would be ...
Main Zone Tuner Frequency Sirius 001,@TFQ:000001. Main Zone Tuner Frequency Sirius 002,@TFQ:000002. Main Zone Tuner Frequency Sirius 003,@TFQ:000003. Main Zone Tuner Frequency Sirius 004,@TFQ:000004. Main Zone Tuner Frequency Sirius 005,@TFQ:000005. Main Zone Tuner Frequency Sirius 006,@TFQ:000006. Main Zone Tuner Frequency Sirius 007,@TFQ:000007. Main Zone Tuner Frequency Sirius 008,@TFQ:000008. Main Zone Tuner Frequency Sirius 009,@TFQ:000009. Main Zone Tuner Frequency Sirius 010,@TFQ:000010. Main Zone Tuner Frequency Sirius 011,@TFQ:000011. Main Zone Tuner Frequency Sirius 012,@TFQ:000012. Main Zone Tuner Frequency Sirius 013,@TFQ:000013. Main Zone Tuner Frequency Sirius 014,@TFQ:000014. Main Zone Tuner Frequency Sirius 015,@TFQ:000015. Main Zone Tuner Frequency Sirius 016,@TFQ:000016. Main Zone Tuner Frequency Sirius 017,@TFQ:000017. Main Zone Tuner Frequency Sirius 018,@TFQ:000018. Main Zone Tuner Frequency Sirius 019,@TFQ:000019. Main Zone Tuner Frequency Sirius 020,@TFQ:000020. Main Zone ...
Fibrosis pulmoner (pulmonary fibrosis) adalah kondisi di mana jaringan parut yang berlebihan terbentuk di paru-paru. Kondisi ini membuat paru-paru menjadi kaku dan mengurangi jumlah luas permukaan yang tersedia untuk pertukaran udara.. ...
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Liver Session Role of allo-antibodies in the development of progressive fibrosis in pediatric livery allografts and need for a unique grading system ...
Table of Contents. Table of Contents 2. List of Tables 7. List of Figures 8. Introduction 9. Global Markets Direct Report Coverage 9. Fibrosis Overview 10. Therapeutics Development 11. Pipeline Products for Fibrosis-Overview 11. Pipeline Products for Fibrosis-Comparative Analysis 12. Fibrosis-Therapeutics under Development by Companies 13. Fibrosis-Therapeutics under Investigation by Universities/Institutes 15. Fibrosis-Pipeline Products Glance 16. Clinical Stage Products 16. Early Stage Products 17. Unknown Stage Products 18. Fibrosis-Products under Development by Companies 19. Fibrosis-Products under Investigation by Universities/Institutes 21. Fibrosis-Companies Involved in Therapeutics Development 22. Abeome Corporation 22. Acceleron Pharma, Inc. 23. Complexa, Inc. 24. Digna Biotech, S.L. 25. F. Hoffmann-La Roche Ltd. 26. FibroGen, Inc. 27. GlycoMimetics, Inc. 28. HanAll Biopharma Co., Ltd. 29. Isarna Therapeutics GmbH 30. Lycera Corp. 31. miRagen Therapeutics, Inc. 32. Neumedicines Inc. ...
A team of scientists has developed a playbook for ending the devastating impact of fibrotic diseases of the liver, lung, kidney, and other organs, which are responsible for as many as 45 percent of all deaths in the industrialized ...
Evento no competitivo en el que la solidaridad, el compañerismo y las ganas de practicar la natación en las aguas de Formentera se unen para ayudar a los niños y jóvenes con Fibrosis Quística
Evento no competitivo en el que la solidaridad, el compañerismo y las ganas de practicar la natación en las aguas de Formentera se unen para ayudar a los niños y jóvenes con Fibrosis Quística
We are utilising the power of our i-body technology platform to develop a pipeline of i-bodies, with an initial focus on treating fibrotic diseases.
Fibrotic Diseases | Scientific research info incl meetings, conferences, seminars, symposia,tradeshows,jobs,jobfairs, professional tips and more.
纤维化程度 在 消化系统疾病 分类中 的翻译结果:fibrosis;fibrosis degree;degree of fibrosis||双语例句|英文例句|相关文摘
Systemic sclerosis (SSc) is a connective tissue disorder characterised by the development of skin fibrosis. Our current understanding of the disease pathogenesis is incomplete and the study of SSc is hindered, at least partially, by a lack of animal models that fully replicate the complex state of human disease. Murine model of bleomycin-induced dermal fibrosis encapsulates important events that take place early in the disease course. To characterise the optimum in vivo parameters required for the successful induction of dermal fibrosis we subjected three commonly used mouse strains to repeated subcutaneous bleomycin injections. We aimed to identify the effects of genetic background and gender on the severity of skin fibrosis. We used male and female Balb/C, C57BL/6, and DBA/2 strains and assessed their susceptibility to bleomycin-induced fibrosis by measuring dermal thickness, hydroxyproline/collagen content and number of resident myofibroblasts, all of which are important indicators of the severity of
Adipose tissue fibrosis development blocks adipocyte hypertrophy and favors ectopic lipid accumulation. Here, we show that adipose tissue fibrosis is associated with obesity and insulin resistance in humans and mice. Kinetic studies in C3H mice fed a high-fat diet show activation of macrophages and progression of fibrosis along with adipocyte metabolic dysfunction and death. Adipose tissue fibrosis is attenuated by macrophage depletion. Impairment of Toll-like receptor 4 signaling protects mice from obesity-induced fibrosis. The presence of a functional Toll-like receptor 4 on adipose tissue hematopoietic cells is necessary for the initiation of adipose tissue fibrosis. Continuous low-dose infusion of the Toll-like receptor 4 ligand, lipopolysaccharide, promotes adipose tissue fibrosis. Ex vivo, lipopolysaccharide-mediated induction of fibrosis is prevented by antibodies against the profibrotic factor TGFβ1. Together, these results indicate that obesity and endotoxemia favor the development of adipose
Fibroproliferative disorders such as idiopathic pulmonary fibrosis and systemic sclerosis have no effective therapies and result in significant morbidity and mortality due to progressive organ fibrosis. We examined the effect of peptides derived from endostatin on existing fibrosis and fibrosis triggered by two potent mediators, transforming growth factor-β (TGF-β) and bleomycin, in human and mouse tissues in vitro, ex vivo, and in vivo. We identified one peptide, E4, with potent antifibrotic activity. E4 prevented TGF-β-induced dermal fibrosis in vivo in a mouse model, ex vivo in human skin, and in bleomycin-induced dermal and pulmonary fibrosis in vivo, demonstrating that E4 exerts potent antifibrotic effects. In addition, E4 significantly reduced existing fibrosis in these preclinical models. E4 amelioration of fibrosis was accompanied by reduced cell apoptosis and lower levels of lysyl oxidase, an enzyme that cross-links collagen, and Egr-1 (early growth response gene-1), a transcription ...
Interstitial fibrosis can be an inevitable outcome of all kinds of progressive chronic kidney disease (CKD). suppressed the immunoreactivity of mTOR signaling, which decreased the inflammatory responses and ECM accumulation in the obstructed kidneys. Isolated macrophages from rapamycin-treated obstructed kidneys presented less inflammatory activity than vehicle groups. In vitro study confirmed that rapamycin significantly inhibited the fibrogenic activation of cultured fibroblasts (NIH3T3 cells), which was induced by the stimulation of TGF-1. Further experiment revealed that rapamycin did not directly inhibit the fibrogenesis of HK2 cells with aristolochic acid treatment. Our findings clarified that rapamycin can ameliorate kidney fibrosis by blocking the mTOR signaling in interstitial macrophages and myofibroblasts. Introduction Tubulointerstitial fibrosis is the final common pathway of a wide variety of chronic progressive kidney diseases. Intense studies have focused on the molecular and ...
TY - JOUR. T1 - Associations of electrocardiographic P-wave characteristics with left atrial function, and diffuse left ventricular fibrosis defined by cardiac magnetic resonance. T2 - The PRIMERI study. AU - Win, Theingi Tiffany. AU - Venkatesh, Bharath Ambale. AU - Volpe, Gustavo J.. AU - Mewton, Nathan. AU - Rizzi, Patricia. AU - Sharma, Ravi K.. AU - Strauss, David G.. AU - Lima, Joao A.. AU - Tereshchenko, Larisa G.. PY - 2015/1/1. Y1 - 2015/1/1. N2 - BACKGROUND: Abnormal P-terminal force in lead V1 (PTFV1) is associated with an increased risk of heart failure, stroke, atrial fibrillation, and death. OBJECTIVE: Our goal was to explore associations of left ventricular (LV) diffuse fibrosis with left atrial (LA) function and electrocardiographic (ECG) measures of LA electrical activity. METHODS: Patients without atrial fibrillation (n = 91; mean age 59.5 years; 61.5% men; 65.9% white) with structural heart disease (spatial QRS-T angle ≥105° and/or Selvester QRS score ≥5 on ECG) but LV ...
Background: Using cardiovascular magnetic resonance imaging (CMR), it is possible to detect diffuse fibrosis of the left ventricle (LV) in patients with atrial fibrillation (AF), which may be independently associated with recurrence of AF after ablation. By conducting CMR, clinical, electrophysiology and biomarker assessment we planned to investigate LV myocardial fibrosis in patients undergoing AF ablation. Methods: LV fibrosis was assessed by T1 mapping in 31 patients undergoing percutaneous ablation for AF. Galectin-3, coronary sinus type I collagen C terminal telopeptide (ICTP), and type III procollagen N terminal peptide were measured with ELISA. Comparison was made between groups above and below the median for LV extracellular volume fraction (ECV), followed by regression analysis. Results: On linear regression analysis LV ECV had significant associations with invasive left atrial pressure (Beta 0.49, P = 0.008) and coronary sinus ICTP (Beta 0.75, P , 0.001), which remained significant on ...
Renal fibrosis is characterized by activation and proliferation of fibroblasts, which continually produce and deposit ECM proteins, leading to progressive fibrosis. Herein we demonstrate that GSK3β is expressed in myofibroblasts, and GSK3β expression and activity are increased in mouse kidneys following I/R and in cultured fibroblasts following TGF-β1 treatment. Pharmacological inhibition of GSK3 using TDZD significantly reduced pro-inflammatory and pro-fibrotic cytokines, macrophage infiltration and ECM deposition, thereby reducing fibrosis. GSK3 inhibition reduced the myofibroblast population in vivo and fibroblast-to-myofibroblast differentiation in vitro by a TGF-β-SMAD signaling-dependent mechanism. Thus, GSK3β plays a pro-fibrotic role in the kidney following I/R, and its inhibition, even after the injury has occurred, could prevent the future development of fibrosis.. GSK3β is expressed in proximal tubules (Nørregaard et al., 2015), and increased GSK3β has been detected in renal ...
In the pathogenesis of renal fibrosis, oxidative stress (OS) enhances the production of reactive oxygen species (ROS) leading to sustained cell growth, inflammation, excessive tissue remodelling and accumulation, which results in the development and acceleration of renal damage. In our previous work (128) we established protein DJ-1 (PARK7) as an important ROS scavenger and key player in renal cell response to OS. In the present study we investigated the impact of profibrogenic agonists on DJ-1 and shed light on the role of this protein in renal fibrosis. Treatment of renal fibroblasts and epithelial cells with the profibrogenic agonist ANG II or PDGF resulted in a significant up-regulation of DJ-1 expression parallel to an increase in the expression of fibrosis markers. Monitoring of DJ-1 expression in kidney extract and tissue sections from renal fibrosis mice model (Col4a3-deficient) revealed a disease grad dependent regulation of the protein. Overexpression of DJ-1 prompted cell resistance ...
A total of 32 patients (36.4%) had hypertension, 21 (23.9%) with hyperlipidemia, 9 (10.2%) with diabetes, 6 (6.8%) patients had with a history of tobacco abuse and 10 (11.4%) with coronary artery disease. A total of 38 (43.2%) patients were on statin, 25 (28.4%) were on angiotensin converting enzyme inhibitor, 36 (40.9%) were on a beta-°©-blocker, 31 (35.2%) were on anticoagulant, and 13 (14.8%) were on antiarrhythmic medication. Subsequent follow-°©-up MRIs were performe on 32 (36%) patients that exhibited progression of atrial fibrosis.. Using univariate logistic regression models we were not able to identify significant predictors of fibrosis progression. The majority of patients (64%) did not reveal any significant changes from the initial quality and quantity of atrial fibrosis at 1 year MRI follow-up. ...
The objective of our study was to investigate the effect of Aliskiren, a renin inhibitor, on the deoxycorticosterone (DOCA) induced myocardial fibrosis in a rat model and its underlying mechanism. A total of 45 Sprague-Dawley (SD) rats underwent right nephrectomy and were randomly assigned into 3 groups: control group (CON group: silicone tube was embedded subcutaneously); DOCA treated group (DOC group: 200 mg of DOCA was subcutaneously administered); DOCA and Aliskiren (ALI) treated group (ALI group: 200 mg of DOCA and 50 mg/kg/d ALI were subcutaneously and intragastrically given, respectively). Treatment was done for 4 weeks. Sirius red staining was employed to detect the expression of myocardial collagen, and the myocardial collagen volume fraction (CVF) and perivascular collagen volume area (PVCA) were calculated. Radioimmunoassay was carried out to measure the renin activity (RA) and content of angiotensin II (Ang II) in the plasma and ventricle. Western blot assay was done to detect the ...
The objective of our study was to investigate the effect of Aliskiren, a renin inhibitor, on the deoxycorticosterone (DOCA) induced myocardial fibrosis in a rat model and its underlying mechanism. A total of 45 Sprague-Dawley (SD) rats underwent right nephrectomy and were randomly assigned into 3 groups: control group (CON group: silicone tube was embedded subcutaneously); DOCA treated group (DOC group: 200 mg of DOCA was subcutaneously administered); DOCA and Aliskiren (ALI) treated group (ALI group: 200 mg of DOCA and 50 mg/kg/d ALI were subcutaneously and intragastrically given, respectively). Treatment was done for 4 weeks. Sirius red staining was employed to detect the expression of myocardial collagen, and the myocardial collagen volume fraction (CVF) and perivascular collagen volume area (PVCA) were calculated. Radioimmunoassay was carried out to measure the renin activity (RA) and content of angiotensin II (Ang II) in the plasma and ventricle. Western blot assay was done to detect the ...
Liver fibrosis is the excessive accumulation of extracellular matrix proteins including collagen that occurs in most types of chronic liver diseases. Advanced liver fibrosis results in cirrhosis, liver failure, and portal hypertension and often requires liver transplantation. Our knowledge of the cellular and molecular mechanisms of liver fibrosis has greatly advanced. Activated hepatic stellate cells, portal fibroblasts, and myofibroblasts of bone marrow origin have been identified as major collagen-producing cells in the injured liver. These cells are activated by fibrogenic cytokines such as TGF-β1, angiotensin II, and leptin. Reversibility of advanced liver fibrosis in patients has been recently documented, which has stimulated researchers to develop antifibrotic drugs. Emerging antifibrotic therapies are aimed at inhibiting the accumulation of fibrogenic cells and/or preventing the deposition of extracellular matrix proteins. Although many therapeutic interventions are effective in ...
Cysteine-rich protein 61 (Cyr61) is a secreted matrix-associated protein that regulates a broad spectrum of biological and cellular activities. This study aimed to investigate the role of Cyr61 in progressive kidney fibrosis induced by unilateral ureteral obstruction (UUO) surgery in mice. The expression of Cyr61 transcripts and proteins in the obstructed kidneys were increased from day 1 and remained high until day 10 after surgery. Immunohistochemistry indicated that Cyr61 was expressed mainly in renal tubular epithelial cells. The upregulated Cyr61 in UUO kidneys was reduced in mice treated with pan-transforming growth factor-β (TGF-β) antibody. The role of TGF-β in tubular Cyr61 upregulation after obstructive kidney injury was further supported by experiments showing that TGF-β1 stimulated Cyr61 expression in cultured tubular epithelial cells. Notably, the upregulation of Cyr61 in UUO kidneys was followed by a marked increase in monocyte chemoattractant protein 1 (MCP-1) ...
Finding a gene for familial cardiac fibrosis (NHS grant 2000.130) Summary of results. A large multi-generation family with an autosomal dominantly inherited form of cardiac fibrosis with a highly malignant clinical outcome is presented and used to identify the gene causing this disease. We consider it a hereditary form of cardiac fibrosis since it was shown that the myocardial fibrosis in this family preceded the clinical and echocardiographic signs.. Twenty-four individuals from this family were clinically evaluated and 18 of them were used for a genome wide linkage analysis giving the highest LOD score (2.6) in the region of the lamin AC (LMNA) gene. Mutation analyses of this candidate gene failed to show a point mutation. Subsequent Southern blot and multiplex ligation-dependent probe amplification analyses, however, revealed a deletion of the start-codon containing exon. The up- and downstream flanking exons proved not to be deleted.. Furthermore, we demonstrate in-vitro that the deletion ...
The master cytokine TGF-β mediates tissue fibrosis associated with inflammation and tissue injury. TGF-β induces fibroblast activation and differentiation into myofibroblasts that secrete extracellular matrix proteins. Canonical TGF-β signaling mobilizes Smad2 and Smad3 transcription factors that control fibrosis by promoting gene expression. However, the importance of TGF-β-Smad2/3 signaling in fibroblast-mediated cardiac fibrosis has not been directly evaluated in vivo. Here, we examined pressure overload-induced cardiac fibrosis in fibroblast- and myofibroblast-specific inducible Cre-expressing mouse lines with selective deletion of the TGF-β receptors Tgfbr1/2, Smad2, or Smad3. Fibroblast-specific deletion of Tgfbr1/2 or Smad3, but not Smad2, markedly reduced the pressure overload-induced fibrotic response as well as fibrosis mediated by a heart-specific, latency-resistant TGF-β mutant transgene. Interestingly, cardiac fibroblast-specific deletion of Tgfbr1/2, but not Smad2/3, ...
2015 The Authors. Uncontrolled extracellular matrix (ECM) production by fibroblasts in response to injury contributes to fibrotic diseases, including idiopathic pulmonary fibrosis (IPF). Reactive oxygen species (ROS) generation is involved in the pathogenesis of IPF. Transforming growth factor-β1 (TGF-β1) stimulates the production of NADPH oxidase 4 (NOX4)-dependent ROS, promoting lung fibrosis (LF). Dysregulation of microRNAs (miRNAs) has been shown to contribute to LF. To identify miRNAs involved in redox regulation relevant for IPF, we performed arrays in human lung fibroblasts exposed to ROS. miR-9-5p was selected as the best candidate and we demonstrate its inhibitory effect on TGF-β receptor type II (TGFBR2) and NOX4 expression. Increased expression of miR-9-5p abrogates TGF-β1-dependent myofibroblast phenotypic transformation. In the mouse model of bleomycin-induced LF, miR-9-5p dramatically reduces fibrogenesis and inhibition of miR-9-5p and prevents its anti-fibrotic effect both in ...
The Role of Toll-Like Receptor 2 in Inflammation and Fibrosis during Progressive Renal Injury. Leemans, Jaklien C.; Butter, Loes M.; Pulskens, Wilco P. C.; Teske, Gwendoline J. D.; Claessen, Nike; van der Poll, Tom; Florquin, Sandrine // PLoS ONE;2009, Vol. 4 Issue 5, p1 Tissue fibrosis and chronic inflammation are common causes of progressive organ damage, including progressive renal disease, leading to loss of physiological functions. Recently, it was shown that Toll-like receptor 2 (TLR2) is expressed in the kidney and activated by endogenous danger signals.... ...
The biology of heart failure is complex and diverse (2), posing challenges for developing efficacious therapies. All but one recent phase III heart failure trial failed to reduce mortality (3). Unlike oncology, we do not split heart failure into subtypes on the basis of disease pathways (4). We need a more targeted approach, not only for drug development, but also for drug response monitoring. Myocardial fibrosis is an attractive biomarker-fibrosis is already an established marker in the liver, kidneys, and lung-and likely a causal disease pathway mediating outcomes. Cardiac fibrosis can be measured on myocardial biopsy and tracks disease severity and outcome (5,6). But biopsy is invasive and impractical for routine clinical diagnosis and monitoring (7). Many of our current measurements are partial surrogates for fibrosis (e.g., imaging for cardiac remodeling, systolic and diastolic function), but more are needed, particularly circulating blood biomarkers. Cardiology quantifies only 2 myocardial ...
TY - JOUR. T1 - A DDX5 S480A polymorphism is associated with increased transcription of fibrogenic genes in hepatic stellate cells. AU - Guo, Jinsheng. AU - Hong, Feng. AU - Loke, Johnny. AU - Yea, Steven. AU - Lim, Chooi Ling. AU - Lee, Ursula. AU - Mann, Derek A.. AU - Walsh, Martin J.. AU - Sninsky, John J.. AU - Friedman, Scott L.. PY - 2010/2/19. Y1 - 2010/2/19. N2 - We recently identified a missense single nucleotide polymorphism (SNP) in DDX5 (rs1140409, p.S480A) that enhances the risk of developing cirrhosis. DDX5 is an ATP-dependent RNA helicase and transcriptional modulator. We hypothesized that the activity of DDX5 in regulating fibrogenic gene transcription in hepatic stellate cells (HSCs) is altered by the S480A SNP. To test this, we employed two approaches: 1) transient overexpression of DDX5 cDNA or siRNA knockdown of endogenous DDX5, with replacement by either DDX5 wild type (WT) or SNP cDNA, or 2) stable expression of exogenous DDX5 WT and SNP in HSC lines. WT DDX5 mRNA in HSCs ...
Renal tubular atrophy and interstitial fibrosis are common hallmarks of etiologically different progressive chronic kidney diseases (CKD) that eventually result in organ failure. Even though these pathological manifestations constitute a major public health problem, diagnostic tests, as well as therapeutic options, are currently limited. Members of the dickkopf (DKK) family, DKK1 and -2, have been associated with inhibition of Wnt signaling and organ fibrosis. Here, we identify DKK3 as a stress-induced, tubular epithelia-derived, secreted glycoprotein that mediates kidney fibrosis. Genetic as well as antibody-mediated abrogation of DKK3 led to reduced tubular atrophy and decreased interstitial matrix accumulation in two mouse models of renal fibrosis. This was facilitated by an amplified, antifibrogenic, inflammatory T cell response and diminished canonical Wnt/β-catenin signaling in stressed tubular epithelial cells. Moreover, in humans, urinary DKK3 levels specifically correlated with the ...
Results Females in all three groups had significantly increased septal myocardial ECV compared with males (0.29±0.03 vs 0.25±0.03, p,0.01). Septal myocardial ECV was higher in ALMS than hypertensive and controls (0.28±0.02 vs 0.25±0.03 vs 0.24±0.03, p,0.05). Three male older ALMS patients (mean 43±5 years vs 27±10 years) without a history of infantile CM had patchy diffuse LE in non-coronary artery territories with an increased ECV compared to remote normal myocardium (ECV 0.41±0.08 vs 0.27±0.03, p,0.05). MAPSE was reduced in patients with ALMS and hypertension compared to controls (13±2 cm/s vs 12±3 cm/s vs 17±2 cm/s, p,0.01. There were no differences in LV ejection fraction, LV mass or LA volumes. Septal myocardial ECV was negatively correlated with a MAPSE in patients with ALMS (r=−0.64, p,0.05). NT-BNP was not correlated with septal ECV but was increased in patients with LGE (median 178 pmol/l vs 44 pmol/l).. ...
The master cytokine TGF-β mediates tissue fibrosis associated with inflammation and tissue injury. TGF-β induces fibroblast activation and differentiation into myofibroblasts that secrete extracellular matrix proteins. Canonical TGF-β signaling mobilizes Smad2 and Smad3 transcription factors that control fibrosis by promoting gene expression. However, the importance of TGF-β-Smad2/3 signaling in fibroblast-mediated cardiac fibrosis has not been directly evaluated in vivo. Here, we examined pressure overload-induced cardiac fibrosis in fibroblast- and myofibroblast-specific inducible Cre-expressing mouse lines with selective deletion of the TGF-β receptors Tgfbr1/2, Smad2, or Smad3. Fibroblast-specific deletion of Tgfbr1/2 or Smad3, but not Smad2, markedly reduced the pressure overload-induced fibrotic response as well as fibrosis mediated by a heart-specific, latency-resistant TGF-β mutant transgene. Interestingly, cardiac fibroblast-specific deletion of Tgfbr1/2, but not Smad2/3, ...
The master cytokine TGF-β mediates tissue fibrosis associated with inflammation and tissue injury. TGF-β induces fibroblast activation and differentiation into myofibroblasts that secrete extracellular matrix proteins. Canonical TGF-β signaling mobilizes Smad2 and Smad3 transcription factors that control fibrosis by promoting gene expression. However, the importance of TGF-β-Smad2/3 signaling in fibroblast-mediated cardiac fibrosis has not been directly evaluated in vivo. Here, we examined pressure overload-induced cardiac fibrosis in fibroblast- and myofibroblast-specific inducible Cre-expressing mouse lines with selective deletion of the TGF-β receptors Tgfbr1/2, Smad2, or Smad3. Fibroblast-specific deletion of Tgfbr1/2 or Smad3, but not Smad2, markedly reduced the pressure overload-induced fibrotic response as well as fibrosis mediated by a heart-specific, latency-resistant TGF-β mutant transgene. Interestingly, cardiac fibroblast-specific deletion of Tgfbr1/2, but not Smad2/3, ...
Abstract. Tubulointerstitial renal fibrosis, characterized as a progressive detrimental connective tissue deposition on the kidney parenchyma, appears to be a harmful process leading inevitably to renal function deterioration, independently of the primary renal disease which causes the original kidney injury. Epithelial to Mesenchymal Transition (EMT) of tubular epithelial cells which are transformed to mesenchymal fibroblasts migrating to adjacent interstitial parenchyma constitutes the principal mechanism of renal fibrosis along with local and circulating cells. Proteinuria as well as hypoxia is included among the main mechanisms of EMT stimulation. TGFβ-1 through the SMAD pathway is considered as the main modulator regulating the EMT molecular mechanism, probably in cooperation with hypoxia inducible factors. Hepatocyte Growth Factor (HGF) and Bone Morphogenetic Factor-7 (BMF-7) are inhibitory to EMT molecules which could prevent in experimental and clinical level the catastrophic process of ...
A major pathophysiological component of cardiac remodeling during heart failure (HF), cardiac fibrosis has become a target for therapeutic intervention. Additionally, compelling evidence indicates a key role of cardiac fibrosis in myocardial malfunctioning during aging. Cardiac fibrosis is a complex phenomenon resulting from aberrant activation of various cell types and signaling pathways as a consequence of injury or damage to tissue. It develops over a time course that also depends upon the type of noxa activation of tissue-specific repair programs, resulting in the subsequent activation of proliferation and migration of fibroblasts from different myocardial locations to the injury site, where they synthesize extracellular matrix (ECM) (1,2).. Tissue repair through the synthesis of new ECM by fibroblasts is beneficial, particularly after myocardial infarction. However, prolonged activation of this process results in excess scar tissue formation, increased ECM deposition, and therefore, bad ...
Most pro-fibrogenic polypeptides are produced by infiltrating immune, inflammatory, mesenchymal and tissue-specific cells, thereby facilitating paracrine pro-fibrogenic effects that perpetuate inflammation-driven fibrosis (see below) (Figure 2, Figure 3) [12]. One of the most potent pro-fibrogenic cytokines in vivo is TGFβ. Three TGFβ isoforms have been described to date (TGFβ1, TGFβ2 and TGFβ3), all of which are initially generated as latent precursors [70]. When active TGFβ is liberated, it binds to a heterodimeric receptor complex consisting of one TGFβ type I receptor molecule, termed activin-linked kinase (ALK)5, and one TGFβ type II receptor. In the canonical TGFβ pathway in normal fibroblasts, ligand binding leads ALK5 to phosphorylate Smad2 and 3, which in turn bind to Smad4 to form a complex that is translocated to the nucleus, which activates transcription [71]. However, TGFβ has also been shown to signal via several additional pathways, including p38 mitogen-activated ...
Results TRB3 expression was increased in fibroblasts of patients with SSc and in murine models of SSc in a transforming growth factor-β (TGF-β)/Smad-dependent manner. Overexpression of TRB3 stimulated canonical TGF-β signalling and induced an activated phenotype in resting fibroblasts. In contrast, knockdown of TRB3 reduced the profibrotic effects of TGF-β and decreased the collagen synthesis. Moreover, siRNA-mediated knockdown of TRB3 exerted potent antifibrotic effects and ameliorated bleomycin as well as constitutively active TGF-β receptor I-induced fibrosis with reduced dermal thickening, decreased hydroxyproline content and impaired myofibroblast differentiation. ... Eur Heart J Cardiovasc Imaging. 2014 May 12. [Epub ahead of print] A comprehensive evaluation of myocardial fibrosis in hypertrophic cardiomyopathy with cardiac magneticresonance imaging: linking genotype with fibrotic phenotype. Ellims AH1, Iles LM, Ling LH, Chong B, Macciocca I, Slavin GS, Hare JL, Kaye DM, Marasco SF, McLean CA, James PA, du Sart…
The European Union commits funds to the FIBRO-TARGETS (Targeting cardiac fibrosis for heart failure treatment) research consortium, to be coordinated by Inserm over a four-year period. The objective of the project is to determine the underliying mechanisms in myocardial interstitial fibrosis contributing to the development of heart failure.
Dr Peter Millett chats about arthrofibrosis, and why patients with established arthrofibrosis need to seek out a surgeon who is expert in this potentially devastating complication.
TY - JOUR. T1 - Predictive Value of Precision-Cut Kidney Slices as an Ex Vivo Screening Platform for Therapeutics in Human Renal Fibrosis. AU - Bigaeva, Emilia. AU - Puerta Cavanzo, Nataly. AU - Stribos, Elisabeth G D. AU - de Jong, Amos J. AU - Biel, Carin. AU - Mutsaers, Henricus A M. AU - Jensen, Michael S. AU - Nørregaard, Rikke. AU - Leliveld, Anna M. AU - de Jong, Igle J. AU - Hillebrands, Jan-Luuk. AU - van Goor, Harry. AU - Boersema, Miriam. AU - Bank, Ruud A. AU - Olinga, Peter. PY - 2020/5/18. Y1 - 2020/5/18. N2 - Animal models are a valuable tool in preclinical research. However, limited predictivity of human biological responses in the conventional models has stimulated the search for reliable preclinical tools that show translational robustness. Here, we used precision-cut kidney slices (PCKS) as a model of renal fibrosis and investigated its predictive capacity for screening the effects of anti-fibrotics. Murine and human PCKS were exposed to TGF beta or PDGF pathway inhibitors ...
Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system. A defective gene and its protein product cause the body to produce unusually thick, sticky mucus.. Cystic fibrosis Symptoms:. Clogs the lungs and leads to life-threatening lung infections;. Obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.. Now researchers are discovering that cystic fibrosis can be found among adults also and it is more complicated than what the doctors earlier assumed.. In fact many people have this disease since childhood but it is not identified till they grows adults .Many patients are identified as having a mild version of the disease but in its classic form, can quickly attack lungs.. In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect ...
In this work, we present an automatic method for liver segmentation and fibrosis classification in liver computed-tomography (CT) portal phase scans. The input is a full abdomen CT scan with an unknown number of slices, and the output is a liver volume segmentation mask and a fibrosis grade. A multi-stage analysis scheme is applied to each scan, including: volume segmentation, texture features extraction and SVM based classification. Data contains portal phase CT examinations from 80 patients, taken with different scanners. Each examination has a matching Fibroscan grade. The dataset was subdivided into two groups: first group contains healthy cases and mild fibrosis, second group contains moderate fibrosis, severe fibrosis and cirrhosis. Using our automated algorithm, we achieved an average dice index of 0.93 ± 0.05 for segmentation and a sensitivity of 0.92 and specificity of 0.81for classification. To the best of our knowledge, this is a first end to end automatic framework for liver ...
The relentless progression of renal disease is closely linked to the process of fibrosis, which is triggered by initial or ongoing injury. Although it is still a point of debate, there is literature to support a mechanistic rather than merely an associative role of fibrosis in progression of kidney disease [40]. This systematic review is a comprehensive evaluation of renal biomarkers that can be used in the detection of fibrosis as well as in the prediction of progression of renal disease. However, the development of a clinically useful biomarker is a sequential process that usually requires five phases; phase 1 identifies promising directions in preclinical studies, phase 2 is clinical assay validation and detection of established disease, phase 3 is biomarker prediction of clinical disease in longitudinal studies, phase 4 is prospective screening and finally phase 5 is impact of screening on disease burden [41]. This systematic review aimed to identify fibrosis biomarkers that achieved phase 2 ...
Ophthotech Corporation (New York, NY) has initiated the first of several planned expansion trials to investigate the potential role of Fovista (anti-platelet derived growth factor, anti-PDGF) combination therapy in reducing subretinal fibrosis, addressing suboptimal treatment response, and reducing treatment burden in wet-AMD patients receiving anti-VEGF monotherapy. The first expansion trial is a phase 2a open-label study investigating the potential role of anti-PDGF therapy in combination with anti-VEGF therapy in reducing subretinal fibrosis in wet-AMD patients.. The anti-PDGF phase 3 program consists of 3 clinical trials to evaluate the safety and efficacy of anti-PDGF for use in combination with anti-VEGF agents for the treatment of wet AMD. Ophthotech expects to enroll up to 1866 patients in the 3 trials in more than 225 centers worldwide and to have initial, topline data from the phase 3 clinical trial available in 2016.. Updated August 5, 2014. ...
The liver organ lymphocyte population is enriched with organic killer (NK) cells, which play a key part in sponsor protection against viral infection and tumor transformation. IL-4, IL-13, hedgehog ligands, and osteopontin; nevertheless, NKT cells may also attenuate liver organ fibrosis under particular circumstances by eliminating HSCs and by generating IFN-. Finally, the potential for NKT and NK cells to be used as therapeutic targets for anti-fibrotic therapy is talked about. proof for the contact between NK cells and early turned on HSCs. (that NK cells eliminate early turned on, but not really quiescent or turned on completely, HSCs. Initial, the amount of early turned on desmin positive HSCs with an oval form was considerably reduced in DDC-fed MLN9708 rodents after administration of the NK cell activator poly I:C (Radaeva and Gao, MLN9708 unpublished data). Second, immunohistochemistry MLN9708 studies present that early turned on HSCs and NK cells possess very similar distributions ...
Fibrosis is the excessive accumulation of extracellular matrix that often occurs as a wound healing response to repeated or chronic tissue injury, and may lead to the disruption of organ architecture and loss of function. Although fibrosis was previously thought to be irreversible, recent evidence indicates that certain circumstances permit the resolution of fibrosis when the underlying causes of injury are eradicated. The mechanism of fibrosis resolution encompasses degradation of the fibrotic extracellular matrix as well as elimination of fibrogenic myofibroblasts through their adaptation of various cell fates, including apoptosis, senescence, dedifferentiation, and reprogramming. In this Review, we discuss the present knowledge and gaps in our understanding of how matrix degradation is regulated and how myofibroblast cell fates can be manipulated, areas that may identify potential therapeutic approaches for fibrosis.. ...
14. Chen, C.A., Tseng, W.Y.I., Wang, J.K., Chen, S.Y., Ni, Y.H., Huang, K.C., Ho, Y.L., Chang, C.I., Chiu, I.S., Su, M.Y.M., Yu, H.Y., Lin, M.T., Lu, C.W., Wu, M.H.*. 2013. Circulating biomarkers of collagen type I metabolism mark the right ventricular fibrosis and adverse markers of clinical outcome in adults with repaired tetralogy of Fallot. International Journal of Cardiology; 167(6):2963-8. ...
A serious complication of radiotherapy in the treatment of cancer patients is the late onset of fibrosis in normal tissues. Transforming growth factor beta (TGF-beta) is emerging as a key mediator of the fibrotic process through its effects on stimulation of fibroblast proliferation, migration and extracellular matrix (ECM) synthesis. The fact that radiation-induced vascular injury tends to precede the development of fibrosis has led to the suggestion that vascular damage is crucial in its pathogenesis. CD105, the specific type III vascular receptor for TGF-beta1 and -beta3, modulates cell proliferation and ECM production in response to TGF-beta in vitro. In this study, we have quantified the levels of TGF-beta1 and soluble CD105-TGF-beta1 complex in 91 pre-radiotherapy plasma samples from early-stage (T1 or T2) breast cancer patients utilising an enhanced chemiluminescence ELISA system. During the follow-up period, 24 patients had developed moderate and one severe fibrosis of the breast. The ...
Cystic fibrosis is a progressive genetic disease wherein the body produces thick and sticky mucus that clogs the lungs and obstructs the pancreas. Cystic fibrosis can be life-threatening, and patients who suffer from it tend to have a short life span. Cystic fibrosis affects some 30,000 people in the United States, and around 1,000 new cases […]
What is cystic fibrosis? Cystic fibrosis is a genetic disease that causes mucus in the body to become thick and sticky. This glue-like mucus builds up and causes problems in many of the bodys organs, especially the lungs and the pancreas. People who have cystic fibrosis can have serious breathing problems and lung...
What is cystic fibrosis? Cystic fibrosis is a genetic disease that causes mucus in the body to become thick and sticky. This glue-like mucus builds up and causes problems in many of the bodys organs, especially the lungs and the pancreas. People who have cystic fibrosis can have serious breathing problems and lung...
Cystic fibrosis can bring its own financial burden, so we provide a range of grants for people with cystic fibrosis and their families. These grants range from our Breath4CF physical activity grant, hospital and transplant allowances and personalised grants to meet individual needs.. We also provide awards and endowments to people with cystic fibrosis, to celebrate amazing achievements and support future opportunities.. Find out more about financial assistance ...
LAVAL, QUEBEC--(Marketwired - June 1, 2015) - ProMetic Life Sciences Inc. (TSX:PLI) (OTCQX:PFSCF), (ProMetic or the Corporation) presented new data at the European Renal Association (ERA) annual meeting in London, UK. The new data confirms that PBI-4050s anti-fibrotic effect demonstrated in the kidney in several different animal models has been...
Cystic fibrosis[edit]. CASS4 has been reported to play a modifying role in cystic fibrosis severity, progression and comorbid ... Cystic fibrosis Possible correlation with severity of the lung manifestation of the disease (according to GWAS).[22] ... clinical value is likely to be as a predictive variant for severity and onset of Alzheimer's disease and cystic fibrosis. ... "Genome-wide association and linkage identify modifier loci of lung disease severity in cystic fibrosis at 11p13 and 20q13.2" ...
a b c Shen, X, Yang, Y, Xiao, T, Peng, J, Liu, X. (2011) Protective effect of oxymatrine on myocardial fibrosis induced by ... Role in cardiac fibrosis[edit]. See also: TGF beta signaling pathway. Recent research has shown that oxymatrine prevents ... The mechanism by which oxymatrine may inhibit fibrosis is still unidentified. One theory that has been proposed is that ... preventing the formation of cardiac fibrosis.[8] Huang and Chen (2013) claim that oxymatrine may even be involved in inhibiting ...
"Johns Hopkins Cystic Fibrosis Center. Retrieved 23 September 2018.. *^ a b c d e f Sander JD, Joung JK (April 2014). "CRISPR- ... Cystic Fibrosis[edit]. Cystic Fibrosis is an autosomal recessive disorder that causes a variety of symptoms and complications, ... "Cystic Fibrosis Canada". Retrieved 2017-11-30.. *^ O'Sullivan BP, Freedman SD (May 2009). "Cystic ... Many Mendelian disorders stem from dominant point mutations within genes, for example Cystic Fibrosis, B-Thalassemia, Sickle- ...
Cystic fibrosis[edit]. Many studies have concluded that pyocyanin has a derogatory effect in cystic fibrosis which enables P. ... aeruginosa to persist in the cystic fibrosis lung; it is often detected in the sputum from cystic fibrosis patients. Pyocyanin ... This allows P. aeruginosa to have a competitive advantage as it may dominate over other microorganisms in the cystic fibrosis ... In the cystic fibrosis lung, intracellular pyocyanin converts molecular oxygen to the superoxide free radical by oxidizing ...
Radiation-induced fibrosis, vascular cell damage and oxidative stress can lead to these and other late side effect symptoms.[20 ... Fibrosis. Tissues which have been irradiated tend to become less elastic over time due to a diffuse scarring process.. ... The gastrointestinal tract can be damaged following abdominal and pelvic radiotherapy.[22] Atrophy, fibrosis and vascular ... myocardial fibrosis, valvular heart disease, coronary artery disease, heart arrhythmia and peripheral artery disease. ...
Ankylosing spondylitis; osteoarthritis; rheumatoid arthritis; fibrosis; capsulitis; soft-tissue disorders.. As per diclofenac. ...
Lacertus fibrosis. *Proximal arch of the FDS. *Rare causes such as following tendon transfers for radial palsy ...
... she developed a serious illness called pulmonary fibrosis- a type of condition that causes hardening of the lungs.[5] ...
pulmonary fibrosis. *fatal asymmetric interstitial lung disease[13]. Reported[edit]. *It has also been suggested that erlotinib ...
Submucous fibrosis. Fracture of the zygomatic arch. Acute osteomyelitis Ankylosis of the TMJ (fibrous or bony) Condylar ... When muscles of mastication are within the field of radiation, it leads to fibrosis and result in decreased mouth opening. ... fibrosis, etc. Acute closed locked conditions - displaced meniscus Rarely, trismus is a symptom of nasopharyngeal or ... Needle prick to the medial pterygoid muscle Oral submucous fibrosis. Radiation therapy to the head and neck. Tetanus, also ...
Cystic fibrosis. Large amount of abnormally thick mucus in the lungs and intestines; leads to congestioni, pneumonia, diarrhea ... There is a variety of DTC tests, ranging from tests for breast cancer alleles to mutations linked to cystic fibrosis. Benefits ... the test can provide information about a couple's risk of having a child with a genetic condition like cystic fibrosis. ...
increased (e.g., Langerhans cell histiocytosis, lymphangioleiomyomatosis, cystic fibrosis, allergic bronchopulmonary ... Edges, e.g. apices for fibrosis, pneumothorax, pleural thickening or plaques ...
"FDA approves new breakthrough therapy for cystic fibrosis". U.S. Food and Drug Administration (FDA) (Press release). October 21 ...
Examples of this type of disorder are Albinism, Medium-chain acyl-CoA dehydrogenase deficiency, cystic fibrosis, sickle-cell ...
Alprostadil is sold in the United States as urethral suppositories and in injectable form. The suppositories are sold under the brand name Muse.[6] The injectable forms are Edex[7] and Caverject.[8] Muse delivers alprostadil as a penile suppository, inserted into the urethra, at least ten minutes before the erection is needed. Caverject and Edex are similarly fast-acting, but instead are injected by syringe directly into the corpus cavernosum of the penis. Alprostadil is also available as a generic. The major cost is that it must be mixed by a compounding pharmacy and supplies may be difficult to obtain. The different formulations, including Bimix and Trimix, may include papaverine and/or phentolamine. A typical mix might be 30 mg of papaverine, 2 mg of phentolamine, and 20 μg alprostadil. As a generic, it is much less expensive than the packaged injectables. It is premixed and must be kept refrigerated and the user must load a syringe with the quantity needed. Most recently, the compound has ...
Cystic fibrosis research #1 (New Zealand) July 2002 "Your Song" Elton John and Alessandro Safina Sport Relief 4 (UK), 88 ( ...
Drug induced hepatic fibrosis. Drug induced hepatic granuloma. Toxic liver disease with hepatitis. Toxic liver disease with ...
These include pulmonary fibrosis which can occur when the lung is inflamed for a long period of time. Fibrosis in the lung ... "Cystic Fibrosis News Today. 3 August 2018.. *^ a b Walter F., PhD. Boron (2004). Medical Physiology: A Cellular And Molecular ... Congenital disorders include cystic fibrosis, pulmonary hypoplasia (an incomplete development of the lungs)[68]congenital ... and common causes of bronchiectasis include severe infections and cystic fibrosis. The definitive cause of asthma is not yet ...
Risk factors associated with Stenotrophomonas infection include HIV infection, malignancy, cystic fibrosis, neutropenia, ... maltophilia colonization rates in individuals with cystic fibrosis have been increasing.[7] ...
Lu, D.; Insel, P. A. (18 December 2013). "Cellular Mechanisms of Tissue Fibrosis. 6. Purinergic signaling and response in ... The bronchoalveolar lavage (BAL) fluid of patients with idiopathic pulmonary fibrosis contains a higher concentration of ATP ... Adenosine receptors affect bronchial reactivity, endothelial permeability, fibrosis, angiogenesis and mucus production. ... fibroblasts and tissue fibrosis". AJP: Cell Physiology. 306 (9): C779-C788. doi:10.1152/ajpcell.00381.2013. Karmouty-Quintana, ...
"Brown's son has cystic fibrosis". BBC News. 26 November 2006. Archived from the original on 27 January 2007.. ... and was diagnosed with cystic fibrosis that November.[79] ...
... though it is unclear exactly how this results in fibrosis. The precise mechanisms producing the fibrosis are not entirely clear ... Fibrosis can affect one or both of the two layers of tissue forming the pleura-the visceral pleura adjacent to the lung and the ... Fibrosis in the pleura may be produced intentionally using a technique called pleurodesis to prevent recurrent punctured lung ( ... Exposure to certain substances, such as asbestos, can cause generalised fibrosis of the lungs, which may involve the pleura and ...
If two cystic fibrosis carriers have children, they have a 25 percent chance of producing offspring having two copies of the ... Not all heterozygotes for recessive lethal alleles will show a mutant phenotype, as is the case for cystic fibrosis carriers. ... Ratjen, Felix; Döring, Gerd (February 2003). "Cystic fibrosis". Lancet. 361 (9358): 681-689. doi:10.1016/S0140-6736(03)12567-6 ...
Endomyocardial Fibrosis. Oxford University Press. ISBN 978-0-19-563237-8. Valiathan, M. S. (2003). The Legacy of Caraka. Orient ...
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 ... "Pulmonary Fibrosis". Mayo Clinic. Retrieved 13 December 2013. Gurujeyalakshmi G, Giri SN (Sep-Oct 1995). "Molecular mechanisms ... and cystic fibrosis. COPD is characterized by goblet cell hyperplasia and mucus hypersecretion. Mucus secretion was found to be ... of antifibrotic effect of interferon gamma in bleomycin-mouse model of lung fibrosis: downregulation of TGF-beta and ...
"Cystic fibrosis - Symptoms and causes". Mayo Clinic. Retrieved 2020-08-02. Reference, Genetics Home. "Cystic fibrosis". ... Cystic fibrosis, a condition caused by a genetic mutation that can lead to injury to the body's organs including the lungs and ... cystic fibrosis, and celiac disease Physical examination involves: Abdominal examination (tenderness, distention, and/or bowel ...
Bethesda, Cystic Fibrosis Foundation 4550 Montgomery Ave Suite 1100 N. "Coughing and Huffing". Retrieved 19 June ... Bethesda, Cystic Fibrosis Foundation 4550 Montgomery Ave Suite 1100 N. "Active Cycle of Breathing Technique (ACBT)". www.cff. ... Bethesda, Cystic Fibrosis Foundation 4550 Montgomery Ave Suite 1100 N. "Chest Physical Therapy". Homnick, DN ( ... Bethesda, Cystic Fibrosis Foundation 4550 Montgomery Ave Suite 1100 N. "Airway Clearance". Retrieved 21 June 2020 ...
Davies investigates cystic fibrosis. She was involved with a major UK trial of gene therapy for cystic fibrosis. Davies leads ... "Tackling Cystic Fibrosis". Retrieved 2019-02-26. Bush, Andrew; Alton, Eric W. F. W.; Davies, Jane C. (2007 ... Greisenbach, U.; Alton, E.W.F.W.; Davies, J.C. (2006). "CF Modifier Genes". In Bush, Andrew (ed.). Cystic Fibrosis in the 21st ... She began to work at the Royal Brompton Hospital on cystic fibrosis. She earned her MD[when?][where?] on host-pathogen ...
Endomyocardial fibrosis in Africa. Postgraduate Medical Journal. 1983 Mar 1;59(689):170-8. Falase AO, Ogah OS. Cardiomyopathies ... Endomyocardial fibrosis. Problems in differential diagnosis. Heart. 1976 Apr 1;38(4):369-74. "Ayodele Olajide Falaise's ...
We trained our model from steady-state and time-dependent experimental measurements made using normal and cystic fibrosis (CF) ... Marsico Lung Institute/University of North Carolina Cystic Fibrosis Center, School of Medicine, University of North Carolina at ... model reveals role of nucleotide signaling in airway surface liquid homeostasis and its dysregulation in cystic fibrosis.. ... model reveals role of nucleotide signaling in airway surface liquid homeostasis and its dysregulation in cystic fibrosis ...
Cystic Fibrosis. Fibrosis. Pathologic Processes. Pancreatic Diseases. Digestive System Diseases. Lung Diseases. Respiratory ... Safety and Tolerability Study of Allogeneic Mesenchymal Stem Cell Infusion in Adults With Cystic Fibrosis (CEASE-CF). The ... This study is being done to test if it is safe to give stem cells to adult patients with Cystic Fibrosis (CF). The kind of stem ... Diary reports (Cystic Fibrosis Respiratory Symptom Diary (CFRSD)) [ Time Frame: 35 days ]. *Changes in subject reported ...
... (CF) is an inherited genetic condition, which mainly affects the lungs, digestive system and ... Cystic Fibrosis Victoria - 65 Red Roses. Cystic Fibrosis Index of On-line Resources. CF Information. Information on Sweat ... Living With Cystic Fibrosis (CF). 9 November 2003: I went to the Drs because my left side has been that sore. I am phelm still ... Cystic Fibrosis Index of On-Line Resources. chest Physical Therapy Sessions. CF Yahoo CLUB. Respiratory Self Support Group. Got ...
Cystic Fibrosis and COVID-19. People with cystic fibrosis (CF) are among those who might be at an increased risk for severe ... Cystic fibrosis (CF) is a genetic disorder that causes problems with breathing and digestion. CF affects about 35,000 people in ... Learn more about steps to take for people with cystic fibrosis and those who have had lung or other solid organ transplants. ... CF results from mutations (changes) in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) geneexternal icon, which ...
... (CF) is a genetic disorder that particularly affects the lungs and digestive system. Kids who ... What Is Cystic Fibrosis?. CF affects more than 30,000 kids and young adults in the United States. It disrupts the normal ... Some of the symptoms of SDS are similar to those of CF, so it may be confused with cystic fibrosis. However, in kids with SDS, ... Cystic fibrosis is the most common cause of pancreatic insufficiency in children, but a condition called Shwachman-Diamond ...
fibrosis (countable and uncountable, plural fibroses) *(medicine) The formation of (excess) fibrous connective tissue in an ... "fibrosis" in Diccionario de la lengua española, Vigésima tercera edición, Real Academia Española, 2014. ... Retrieved from "" ...
... (CF) is an inherited disease that causes the body to produce mucus thats extremely thick and sticky. It mainly ... What Is Cystic Fibrosis?. Cystic fibrosis (CF) is an inherited disease that causes the body to produce mucus thats extremely ... Cystic fibrosis is an inherited disease caused by mutations (changes) in a gene on chromosome 7, one of the 23 pairs of ... Cystic fibrosis occurs most frequently in Caucasians of northern European descent, in whom the CF gene is most common - ...
Nephrogenic systemic fibrosis (NSF) is a rare syndrome that involves fibrosis of skin, joints, eyes, and internal organs. NSF ... "nephrogenic systemic fibrosis" to better describe its systemic nature.[12] The term "gadolinium-associated systemic fibrosis" ... "Nephrogenic Systemic Fibrosis". Emedicine.. *^ Bhawan J, Perez-Chua TA, Goldberg L (September 2013). "Sclerotic bodies beyond ... Thomsen HS (September 2009). "Nephrogenic systemic fibrosis: history and epidemiology". Radiologic Clinics of North America. 47 ...
cystic fibrosis *(pathology) An inherited condition in which the exocrine glands produce abnormally viscous mucus, causing ... Retrieved from "" ...
Cystic fibrosis is an inherited condition in which the lungs and digestive system can become clogged with thick, sticky mucus. ... Find out more about treatments for cystic fibrosis.. Complications of cystic fibrosis. People with cystic fibrosis also have a ... How cystic fibrosis is inherited. To be born with cystic fibrosis, a child has to inherit a copy of the faulty gene from both ... Diagnosing cystic fibrosis. In the UK, all newborn babies are screened for cystic fibrosis as part of the newborn blood spot ...
... fibrosis) occur in the back of the abdominal cavity, behind (retro-) the membrane that surrounds the organs of the digestive ... Retroperitoneal fibrosis is a disorder in which inflammation and extensive scar tissue ( ... Retroperitoneal fibrosis is a disorder in which inflammation and extensive scar tissue (fibrosis) occur in the back of the ... No genes associated with retroperitoneal fibrosis have been identified.. Retroperitoneal fibrosis occasionally occurs with ...
Cystic fibrosis is an inherited disease characterized by the buildup of thick, sticky mucus that can damage many of the bodys ... Update in cystic fibrosis 2005. Am J Respir Crit Care Med. 2006 May 1;173(9):944-7. Review. Citation on PubMed or Free article ... Cystic fibrosis is a common genetic disease within the white population in the United States. The disease occurs in 1 in 2,500 ... Mutations in the CFTR gene cause cystic fibrosis. The CFTR gene provides instructions for making a channel. that transports ...
Whats the Treatment for Cystic Fibrosis?. Whats the Treatment for Cystic Fibrosis? Whats the Treatment for Cystic Fibrosis? ... Mayo Clinic: "Cystic Fibrosis -- Treatment," "Cystic Fibrosis -- Symptoms and Causes.". Cystic Fibrosis Foundation: "Life with ... Cystic fibrosis is caused by a defect on a gene known as CFTR. New medicines called "CFTR modulators" can fix this gene so it ... Theres no cure for cystic fibrosis (CF). But many treatments can reduce your symptoms and improve your quality of life. Heres ...
fibrosis (es); Fibrosi (eu); fibrosi (ca); Fibrose (de); فیبروز (fa); 纤维化 (zh); 線維化 (ja); Fibros (sv); Фіброз (uk); Fibrosis ( ... fibrosis (en); تليف (ar); Fibrosis (ms); Fibroz (uz) formation of excess fibrous connective tissue in an organ or tissue in a ... Fibrosis formation of excess fibrous connective tissue in an organ or tissue in a reparative or reactive process ... Media-media dalam kategori "Fibrosis". Yang berikut ialah 2 daripada 2 buah fail dalam kategori ini. ...
Join us to raise money towards research for a cure for cystic fibrosis (CF). CF is a life-threatening genetic disease that ... One young man had cystic fibrosis and talked about how his medications and treatment almost bankrupted him until The Affordable ... The Avon Police Department has organized the Avon Mountain Challenge to benefit the Cystic Fibrosis Inpatient Fund, a charity ... The Connecticut chapter of the Cystic Fibrosis Foundation is hosting its annual Hartfords Finest fundraiser on July 26, where ...
Asbestos and retroperitoneal fibrosis. Original title. Asbest und Retroperitonealfibrose [in German]. Amiante et fibrose r trop ... This article discusses one of the diseases caused by asbestos, namely retroperitoneal fibrosis, recently recognized as an ... peritoneal diseases; occupational diseases; fibrosis; asbestos; Switzerland. Descriptors (secondary). compensation of ...
Many forms of liver injury are marked by fibrosis, which is defined as an excess deposition of the components of the ... What is the liver fibrosis?. Updated: Jul 30, 2018 * Author: David C Wolf, MD, FACP, FACG, AGAF, FAASLD; Chief Editor: BS Anand ... Hepatic fibrosis. Schiff ER, Sorrell MF, Maddrey WC, eds. Schiffs Diseases of the Liver. 8th ed. Philadelphia, Pa: Lippincott- ... Many forms of liver injury are marked by fibrosis, which is defined as an excess deposition of the components of the ...
Cystic Fibrosis: UXL Encyclopedia of Diseases and Disorders dictionary. ... Cystic Fibrosis. New York: Rosen Publishing Group, 2007. Thomson, Anne H., and Ann Harris. Cystic Fibrosis: The Facts, 2nd ed. ... As with cystic fibrosis, there is no cure for TSD.. TSD is inherited in the same pattern as cystic fibrosis; that is, both ... Fibrosis: The medical term for the formation of scar tissue.. Gene therapy: The insertion of normal genes into a persons cells ...
Cystic fibrosis is an autosomal recessive disorder, and most carriers of the gene are asymptomatic. Cystic fibr... more ... Cystic fibrosis (CF) is the most common lethal inherited disease in white persons.{ref4} ... Cystic Fibrosis Foundation. Fibrosis Foundation Patient registry Annual Report 2008. Bethesda, MD: Cystic Fibrosis Foundation; ... encoded search term (What is cystic fibrosis (CF)?) and What is cystic fibrosis (CF)? What to Read Next on Medscape. Related ...
Adult cystic fibrosis.. Boyle MP1.. Author information. 1. Johns Hopkins Adult Cystic Fibrosis Program, Division of Pulmonary ... As it is estimated that within the next decade more than half of all individuals with cystic fibrosis will be aged 18 years or ... Cystic fibrosis is a multisystem disease characterized primarily by chronic pulmonary infection and bronchiectasis, pancreatic ... with median predicted survival in cystic fibrosis now to older than 35 years. This increase in predicted survival has also been ...
fibrosis quística (es); Муковисцидоз (kk-kz); Penyakit Sistik Fibrosis (ms); مۋكوۆىيستسىيدوز (kk-cn); Kistik fibrozis (tr); 囊腫性 ... cystic fibrosis (en); تليف كيسي (ar); Cisztás fibrózis (hu); Fibrosi kistiko (eu); Fibrosis quística (ast); Муковисцидоз (ru); ... CYSTIC FIBROSIS, CYSTIC FIBROSIS; CF (en); kista fibrozo pankreata (eo); mukoviscidóza (cs); mucoviscidosi (ca) ... Fibrosis cystica, Cystische fibrose, Mucoviscidose, Cystic fibrosis, Cystische fybrose, CF (nl); zwłóknienie torbielowate (pl ...
Cystic fibrosis is one of the most common hereditary diseases occurring in the UK. These findings will be of interest to the ... Cystic fibrosis is the most common inherited life-threatening disease that occurs among Caucasian people in the UK. In an ... The gene mutation in cystic fibrosis is well known to affect a cell membrane transporter of salt and water that leads to ... Lung infections are a major cause of death in people who have cystic fibrosis. Only further research will reveal whether these ...
Idiopathic retroperitoneal fibrosis Br Med J (Clin Res Ed) 1981; 282 :1873 ... Idiopathic retroperitoneal fibrosis. Br Med J (Clin Res Ed) 1981; 282 doi: ( ...
Pulmonary fibrosis, end result of a variety of inflammatory diseases of the lungs in which dense fibrous connective tissue ... More About Pulmonary fibrosis. 1 reference found in Britannica articles. Assorted References. *idiopathic pulmonary fibrosis* ... respiratory disease: Idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis is also known as cryptogenic fibrosing ... Pulmonary fibrosis, end result of a variety of inflammatory diseases of the lungs in which dense fibrous connective tissue ...
Cystic Fibrosis Cystic Fibrosis Transmembrane Conductance Regulator Rectal Prolapse Pancreatic Insufficiency Nephrogenic ... Kollberg H: Cystic fibrosis and physical activity: An introduction. Int J Sports Med. 1988;9(Suppl):2-5.PubMedCrossRefGoogle ... Buchwald M, Tsui LC, Riordan J: The search for the cystic fibrosis gene. Am J Physiol. 1989;257:L47-L52.PubMedGoogle Scholar ... Penketh ARL, Wise A, Mearns MB, et al: Cystic fibrosis in adolescents and adults. Thorax. 1987;42:526-532.PubMedCrossRefGoogle ...
Today the median life expectancy for the 30,000 Americans living with cystic fibrosis is closing in on 40, and expected to ... Emily Schaller is one of the estimated 30,000 Americans living with cystic fibrosis. (Cystic Fibrosis Foundation). It started ... Beating Cystic Fibrosis. In the 1970s, most children with CF never made it through elementary school. Today the median life ... But with the development of new drugs and airway clearance techniques that are helping to make cystic fibrosis a manageable ...
... in Ballindaggin National School recently when it celebrated Friendship Week and also raised money for Cystic Fibrosis Ireland. ... volunteering in the Market Square for Cystic Fibrosis Awareness Day. Cystic Fibrosis Awareness Day in Ballindaggan School on ... Cystic Fibrosis fundraiser. TY students, Niamh Lambert and Cayleigh OBrien-Doyle (Colaiste Bride) and Lily Byrne (Enniscorthy ... Local people and some secondary school students also helped raise money for cystic fibrosis as part of the fundraising drive. ...
Definition Cystic fibrosis (CF) is a chronic, progressive and genetic (inherited) disease of the bodys exocrine (mucus ... Cystic fibrosis is transmitted to a child when both parents carry the recessive gene but do not have the disease. When such a ... Cystic fibrosis is the most common fatal hereditary disorder for Caucasians in the U.S. About 1 in 2500 Caucasians are affected ... Cystic fibrosis (CF) is a chronic, progressive and genetic (inherited) disease of the bodys exocrine (mucus producing) glands ...
Liver fibrosis is the excessive accumulation of extracellular matrix proteins including collagen that occurs in most types of ... Advanced liver fibrosis results in cirrhosis, liver failure, and portal hypertension and often requires liver transplantation. ... This review summarizes recent progress in the study of the pathogenesis and diagnosis of liver fibrosis and discusses current ... Our knowledge of the cellular and molecular mechanisms of liver fibrosis has greatly advanced. Activated hepatic stellate cells ...
Cystic fibrosis is often diagnosed at birth, through the routine screening of newborns. The disease may also be suspected early ... Newborn screening for cystic fibrosis All newborn babies are screened for cystic fibrosis. A small amount of the babys blood ... If cystic fibrosis is suspected, genetic testing is performed to look for the mutation that causes this disease. The mutation ... Cystic fibrosis is often diagnosed at birth, through the routine screening of newborns. The disease may also be suspected early ...
  • We all wore purple into the school as that is the colour associated with cystic fibrosis,' said Mr McDonald. (
  • Mutations associated with cystic fibrosis can be detected in screening tests. (
  • Approximately 1 in 4000 children born in the United States is affected with cystic fibrosis. (
  • Li KP, Zhu J, Zhang JL, Huang F. Idiopathic retroperitoneal fibrosis (RPF): clinical features of 61 cases and literature review. (
  • However, in many cases the cause is unknown, and thus the disease is referred to as idiopathic pulmonary fibrosis. (
  • The diagnosis of idiopathic pulmonary fibrosis (IPF) typically involves questioning about medical history and examinations to classify the condition and eliminate other possible conditions. (
  • Retrieved on September 16, 2019 from (
  • Idiopathic pulmonary fibrosis is the most common diagnosis among patients presenting with interstitial lung disease. (
  • The cause of idiopathic pulmonary fibrosis is unknown. (
  • The outlook for recovery is generally poor for occupational dust diseases and idiopathic (no-known cause) interstitial pulmonary fibrosis in which the lungs progressively stiffen. (
  • The most common form of PF is idiopathic pulmonary fibrosis (IPF), for which there is no known cause. (
  • A panel discussion with physicians and patients with familial idiopathic pulmonary fibrosis will address genetics and environmental factors in interstitial lung disease. (
  • Idiopathic pulmonary fibrosis (IPF) is a condition in which, over a period of time, lung tissue becomes thickened, stiff and scarred. (
  • When the cause of the fibrosis is unknown (and certain pathologic or radiographic criteria are met), the disease is called idiopathic pulmonary fibrosis or IPF. (
  • Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic and progressive fibrosing interstitial pneumonia of unknown cause. (
  • An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. (
  • Raghu G, Chen S-Y, Hou Q, Yeh W-S, Collard HR. Incidence and prevalence of idiopathic pulmonary fibrosis in US adults 18-64 years old. (
  • A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. (
  • Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. (
  • An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis. (
  • Does current knowledge explain the pathogenesis of idiopathic pulmonary fibrosis? (
  • Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. (
  • The detection of Epstein-Barr virus DNA in lung tissue from patients with idiopathic pulmonary fibrosis. (
  • Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: a genome-wide association study. (
  • Prevalence of hiatal hernia by blinded multidetector CT in patients with idiopathic pulmonary fibrosis. (
  • Progression of idiopathic pulmonary fibrosis: lessons from asymmetrical disease. (
  • Tobin RW, Pope CE, Pellegrini CA, Emond MJ, Sillery J, Raghu G. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. (
  • High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis. (
  • Idiopathic pulmonary fibrosis (IPF), previously known as cryptogenic fibrosing alveolitis (CFA) in Europe, is a chronic, relentlessly progressive fibrotic disorder of the lower respiratory tract that typically affects adults over the age of 40 [ 1 ]. (
  • See 'Approach to the adult with interstitial lung disease: Clinical evaluation' and 'Approach to the adult with interstitial lung disease: Diagnostic testing' and 'Treatment of idiopathic pulmonary fibrosis' . (
  • The characteristic histopathologic features of usual interstitial pneumonia (UIP) in patients with idiopathic pulmonary fibrosis (IPF) include abnormal proliferation of mesenchymal cells, varying degrees of fibrosis, overproduction and disorganized deposition of collagen and extracellular matrix, distortion of pulmonary architecture and subpleural cystic airspaces (3 to 10 mm diameter) called honeycomb cysts. (
  • Pulmonary fibrosis (Idiopathic pulmonary fibrosis or IPF) is a condition where the tissue deep in the lungs becomes scarred over time. (
  • Idiopathic Pulmonary Fibrosis primarily affects people in their early 70s. (
  • Idiopathic pulmonary fibrosis is a chronic, progressive lung disease. (
  • Idiopathic pulmonary fibrosis is a chronic, progres. (
  • Idiopathic pulmonary fibrosis (IPF, also called cryptogenic fibrosing alveolitis) is specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause, occurring in adults and limited to the lungs. (
  • Diagnosis and management of suspected idiopathic pulmonary fibrosis, 2013. (
  • Commonly used three-drug regimen for idiopathic pulmonary fibrosis found harmful. (
  • Fibrosis can occur in many tissues within the body, typically as a result of inflammation or damage, and examples include: Lungs Fibrothorax Pulmonary fibrosis Cystic fibrosis Idiopathic pulmonary fibrosis (idiopathic meaning the cause is unknown) Radiation-induced lung injury (following treatment for cancer) Liver Bridging fibrosis An advanced stage of liver fibrosis seen in the progressive form of chronic liver diseases. (
  • When this is the case, the condition is called idiopathic pulmonary fibrosis (IPF). (
  • Active epithelial Hippo signaling in idiopathic pulmonary fibrosis. (
  • Kerrebijn KF, Veentjer R, Bonjet VD, et al: The immediate effect of physiotherapy and aerosol treatment on pulmonary function in children with cystic fibrosis. (
  • In a study of 22 children with cystic fibrosis (CF), the researchers have found new insights into "a spectrum of aerobic and anaerobic lifestyles" of pathogens involved in the disease. (
  • Seattle Children's takes a proactive approach to caring for children with cystic fibrosis (CF) so they can grow up as healthy and active as possible. (
  • Going a step further, the team tested the newborns' ability to fight off infection by introducing Staphylococcus aureus into their lungs, a bacteria commonly found in infants and children with cystic fibrosis. (
  • In the 1950s, few children with cystic fibrosis lived to attend elementary school. (
  • Marsico Lung Institute/University of North Carolina Cystic Fibrosis Center, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599. (
  • Learn more about steps to take for people with cystic fibrosis and those who have had lung or other solid organ transplants . (
  • People with cystic fibrosis may need to take different medicines to treat and prevent lung problems. (
  • Over time, mucus buildup and infections result in permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs. (
  • Yankaskas JR, Mallory GB Jr. Lung transplantation in cystic fibrosis: consensus conference statement. (
  • Interactions between secondhand smoke and genes that affect cystic fibrosis lung disease. (
  • Lung infections are a major cause of death in people who have cystic fibrosis. (
  • They also compared the ceramide content in lung samples from three people with cystic fibrosis who had had lung transplants (i.e. they compared the ceramide in their diseased lungs with ceramide in their healthy transplanted lungs). (
  • The researchers found that mutant mice treated with amitriptyline had fewer bacteria ( Pseudomonas aeruginosa , which commonly causes lung infection in people with cystic fibrosis) in their lungs and had reduced mortality from lung infection after seven days. (
  • Pulmonary fibrosis , end result of a variety of inflammatory diseases of the lungs in which dense fibrous connective tissue replaces lung tissue. (
  • CHICAGO , June 27, 2018 /PRNewswire-USNewswire/ -- For more than one million people living with serious lung conditions, such as pulmonary fibrosis (PF), a prescription for supplemental oxygen therapy can seem overwhelming. (
  • Pulmonary fibrosis is a group of lung diseases that cause scarring in the lungs, which limits the oxygen intake necessary for the brain, heart, and other organs to function. (
  • Cystic fibrosis is an inherited disease that causes breathing problems, lung disease, nutrition problems, and problem in digestive system. (
  • Cystic fibrosis (CF) is a genetic disorder that affects the lungs and digestive system Kids who have it can get lung infections often and have trouble breathing. (
  • In general, the presence of a honeycomb effect in the lung tissue often reflects scarring in the area and is indicative of fibrosis. (
  • Interstitial Pulmonary Fibrosis is the scarring and thickening of the lung tissues, a form of interstitial lung disease. (
  • The response to corticosteroids is better in patients with more inflammation and less fibrosis noted on lung biopsy. (
  • Lung transplantation has been suggested for selected patients with end-stage pulmonary fibrosis. (
  • Informative and collaborative sessions will highlight the most current developments in the research and treatment of pulmonary fibrosis, a deadly lung disease. (
  • Cathleen Morrison, chief executive officer of the Canadian Cystic Fibrosis Foundation, agrees that chronic lung infection with the mutated bug is the leading cause of death among CF patients. (
  • Cystic fibrosis patients produce abnormally viscous mucus in their lungs, which leads to chronic lung damage. (
  • The problem is, after receiving a lung transplant, the new lungs do not have CF, but Cystic Fibrosis still exists in the sinuses, pancreas, intestines, sweat glands and reproductive tract, which may find their way to the new lungs eventually. (
  • The second annual Investors Ball, sponsored by the Cystic Fibrosis Guild of Orange County, raised more than $80,000 toward finding a cure for the disease, a lung ailment that causes repeated infections and eventual suffocation in children. (
  • Ben, who was diagnosed with cystic fibrosis at birth, believes exercise has been an important factor for him in achieving and maintaining his healthy lung function. (
  • According to Cystic Fibrosis Ireland, exercise and physical fitness is important to people with cystic fibrosis as it is a natural form of chest physiotherapy which increases lung capacity and helps keep airways clear. (
  • Pulmonary fibrosis is a lung disease that occurs when scars form in the lung. (
  • Pulmonary fibrosis, which is also known as scarring in the lungs, is a respiratory disease in which scars are formed in the lung tissues. (
  • Pulmonary fibrosis is a condition that causes lung scarring and stiffness. (
  • The purpose of this study is to determine how two medications, azithromycin and inhaled tobramycin, work when taken together to treat cystic fibrosis (CF) patients with chronic pseudomonas aeruginosa (PA) lung infections. (
  • Chronic cough, recurrent pneumonia , and the progressive loss of lung function are the major manifestations of lung disease, which is the most common cause of death of persons with cystic fibrosis. (
  • Pigs bred with a mutation for cystic fibrosis exhibit classic symptoms found in humans with the disease, including a thickened layer of mucus (top, in magenta) lining the membranes of organs like the lung and pancreas. (
  • For years, scientists have tried to track the disease in mice engineered with the genetic mutation for cystic fibrosis, but the mice have not developed the trademark symptoms, including chronic lung disease. (
  • Part of the motivation for the research is that, while the genetic root cystic fibrosis has long been known, it's still unclear how this leads to lung disease. (
  • It's important to answer that question because if you now know the sequence of events of lung disease in cystic fibrosis, that could dictate how you treat it. (
  • Researches currently believe that a combination of exposure to lung irritants like certain chemicals, smoking, and infections, along with genetics and immune system activity, play key roles in pulmonary fibrosis. (
  • The formation of significant lung scarring eventually becomes pulmonary fibrosis. (
  • According to the National Heart, Lung, and Blood Institute, most people with pulmonary fibrosis have IPF. (
  • Pulmonary fibrosis is one of more than 200 types of lung diseases that exist. (
  • In the gene therapy trials, patients receive copies of the cystic fibrosis gene they lack, packaged in a type of cold virus able to infect lung cells and deliver the essential genes. (
  • Many cystic fibrosis patients die of chronic lung infections before the age of 30. (
  • By giving patients working versions of the cystic fibrosis gene, researchers hope to forestall the mucus buildup, prevent lung damage and essentially cure the disease, rather than simply treat the symptoms as is now done. (
  • CF results from mutations (changes) in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene external icon , which has instructions for making the CFTR protein. (
  • The inherited CF gene directs the body's epithelial cells to produce a defective form of a protein called CFTR (or cystic fibrosis transmembrane conductance regulator) found in cells that line the lungs, digestive tract, sweat glands, and genitourinary system. (
  • CF occurs because of mutations in the gene that makes a protein called CFTR (cystic fibrosis transmembrane regulator). (
  • Mutations in the CFTR gene cause cystic fibrosis. (
  • For example, mutations in genes other than CFTR might help explain why some people with cystic fibrosis are more severely affected than others. (
  • Cystic fibrosis is caused by a defect on a gene known as CFTR. (
  • Wayne Grody, of the UCLA School of Medicine, Los Angeles, CA, who is not affiliated with these studies, states "Taken together, these three papers demonstrate how the widespread and thoughtful experience with [cystic fibrosis] mutation testing and screening continues to reveal new insights about the mutational alleles of the CFTR gene and further refinements in how best to detect them and assure appropriate quality control while doing so. (
  • Cystic fibrosis is caused by a mutation in the CFTR gene located on chromosome 7. (
  • A child must inherit the defective gene from both parents in order to develop CF. If either parent has a normal CFTR gene, the child may be a carrier for the disease but will not have any symptoms of cystic fibrosis . (
  • If two carriers marry, they have a 25 percent chance of having a child with cystic fibrosis, and a 50 percent chance of having a child who will be a carrier of the defective CFTR gene. (
  • A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. (
  • In 1989, researchers found the defective gene (called delta F508) that causes CF. This gene disrupts a protein (called cystic fibrosis transmembrane conductance regulator - CFTR) that causes the symptoms of CF. (
  • The mutation occurs in the gene that codes for a protein called the cystic fibrosis transmembrane conductance regulator (CFTR). (
  • CFTR modulators, antibiotics and anti-inflammatory medications are the mainstay of therapy for treating the disease , according to Joan K. DeCelie-Germana M.D ., CF Center Director, TDC Co-Director Pediatric Cystic Fibrosis Research, Division of Pediatric Pulmonary And Cystic Fibrosis Center, Cohens Children's Medical Center, Northwell Health. (
  • When your child has cystic fibrosis, the CFTR protein that causes thick mucous in the lungs also causes thick mucus in the gut that hinders appropriate digestion. (
  • The gene, called cystic fibrosis transmembrane conductance regulator , or CFTR , lies in the middle of chromosome 7 and encodes a protein of the same name, designated CFTR. (
  • Most cases of cystic fibrosis are caused by a mutation that corresponds to the production of a CFTR protein that lacks the amino acid phenylalanine . (
  • In addition, mutations in the CFTR gene are associated with degeneration of the ductus deferens and sterility in adult males who have cystic fibrosis. (
  • The protein coded by the CF gene is known as the cystic fibrosis transmembrane regulator (CFTR). (
  • Ivacaftor affects a genetic mutation in 4% to 5% of cystic fibrosis patients that prevents a protein called CFTR from allowing enough chloride ions to exit a certain type of cell. (
  • Cystic Fibrosis (CF) is an inherited genetic condition, which mainly affects the lungs, digestive system and the sweat glands. (
  • Cystic fibrosis (CF) is a genetic disorder that particularly affects the lungs and digestive system . (
  • Cystic fibrosis is an inherited condition that causes sticky mucus to build up in the lungs and digestive system. (
  • Cystic fibrosis is a hereditary disease that affects mucus secretions in the lungs, liver, pancreas, and intestines. (
  • Cystic fibrosis, or CF, is a fatal inherited disease caused by a mutation in a gene that leads to the buildup of thick, sticky mucus in the lungs and pancreas. (
  • Most patients eventually die in late adolescence or their early adult years from damage to the lungs caused by the buildup of scar tissue (fibrosis). (
  • The report is based on the findings from a study in mice which has shed further light on the biological processes involved in the changes to the lungs caused by the cystic fibrosis mutation. (
  • The researchers were trying to get a better understanding about the biological processes that take place in the lungs when an organism has cystic fibrosis. (
  • Pulmonary fibrosis is scarring throughout the lungs . (
  • Cystic fibrosis is an inherited, life-threatening disease caused by a genetic flaw that leads to the buildup of mucus in the lungs and other vital organs. (
  • A stethoscope can be used to listen to the sound of breathing in the lungs, as a crackling sound can be suggestive of fibrosis. (
  • Over the three-day conference, prominent physicians and researchers will present the most current information about pulmonary fibrosis, a devastating and deadly disease resulting from progressive scarring of the lungs. (
  • Cystic fibrosis is an inherited, chronic disease that affects primarily the lungs and digestive tract. (
  • Cystic Fibrosis (CF) is a fatal and progressive genetic disorder that affects the lungs, pancreas and additional organs. (
  • 22 years after the cystic fibrosis gene was discovered, some patients are about to receive a drug called Kalydeco to treat the defect that causes their lungs to clog up with sticky mucus. (
  • a complication of coal workers' pneumoconiosis Retroperitoneal fibrosis (soft tissue of the retroperitoneum) Scleroderma/systemic sclerosis (skin, lungs) Some forms of adhesive capsulitis (shoulder) Myocardial fibrosis has mainly two forms: Interstitial fibrosis, which has been described in congestive heart failure, hypertension, and normal aging. (
  • Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). (
  • THE BIG PICTURE: Cystic fibrosis causes sticky mucus buildup in the lungs and other organs, leading to infections, digestive problems and usually early death. (
  • Symptoms of cystic fibrosis mainly involve the lungs, pancreas, sex organs, intestines, and sinuses and arise from abnormal mucus that is unusually thick and sticky. (
  • THE first effort to install healthy genes in the lungs of cystic fibrosis patients has hit a few bumpy spots, forcing researchers in the United States to redesign their projects and sharply reduce the dose of the experimental therapy they give to people taking part in the trials. (
  • Cystic fibrosis (CF) is an inherited disease that causes the body to produce mucus that's extremely thick and sticky. (
  • Cystic fibrosis is an inherited disease characterized by the buildup of thick, sticky mucus that can damage many of the body's organs. (
  • In people with cystic fibrosis, the body produces mucus that is abnormally thick and sticky. (
  • In people with cystic fibrosis, mucus often damages the pancreas, impairing its ability to produce insulin and digestive enzymes. (
  • Most men with cystic fibrosis have congenital bilateral absence of the vas deferens (CBAVD), a condition in which the tubes that carry sperm (the vas deferens) are blocked by mucus and do not develop properly. (
  • Frates RC, Kaizu T, Last JA: Mucus glycoproteins secreted by respiratory epithelial tissue from cystic fibrosis patients. (
  • Cystic fibrosis (CF) is a chronic, progressive and genetic (inherited) disease of the body's exocrine (mucus producing) glands which affects approximately 30,000 children and adults in the United States. (
  • Cystic fibrosis is a disease of the mucus and sweat glands. (
  • Cystic fibrosis affects the glands in the body that produce mucus and sweat. (
  • Identified as a progressive genetic disease, cystic fibrosis is a disorder that affects the mucus and sweat glands. (
  • Cystic fibrosis (CF) , also called mucoviscidosis , formerly cystic fibrosis of the pancreas , an inherited metabolic disorder , the chief symptom of which is the production of a thick, sticky mucus that clogs the respiratory tract and the gastrointestinal tract . (
  • Cystic fibrosis affects the functioning of the body's exocrine glands -e.g., the mucus-secreting and sweat glands -in the respiratory and digestive systems. (
  • To help people struggling with supplemental oxygen issues, the Pulmonary Fibrosis Foundation has launched the Oxygen Information Line, 844-825-5733. (
  • Oxygen can provide a lifeline for people living with pulmonary fibrosis,' said William T. Schmidt , President and Chief Executive Officer of the Pulmonary Fibrosis Foundation. (
  • The Pulmonary Fibrosis Foundation provides a downloadable Quick Start Guide for Supplemental Oxygen and a Traveling with Supplemental Oxygen Guide . (
  • The Pulmonary Fibrosis Foundation (PFF) mobilizes people and resources to provide access to high quality care and leads research for a cure so people with pulmonary fibrosis will live longer, healthier lives. (
  • The PFF Summit is patient-centric and presenters will address the individuality of pulmonary fibrosis and its progression,' said Bill Schmidt , President and CEO of the Pulmonary Fibrosis Foundation. (
  • Some key CPF programs will be moved to the Pulmonary Fibrosis Foundation, a non-profit organization with a similar mission, based in Chicago. (
  • The Board of Directors of the CPF believes the time could not be better for the CPF to merge our key programs into the Pulmonary Fibrosis Foundation (PFF). (
  • This new, unified voice is one the PF community has long wanted and we are pleased efforts the CPF started will continue through the Pulmonary Fibrosis Foundation," added Michon. (
  • According to the Pulmonary Fibrosis Foundation, about 10 to 15 percent of people with IPF have another family member with pulmonary fibrosis. (
  • In a survey by the Pulmonary Fibrosis Foundation, 55 percent of respondents reported being misdiagnosed at some point. (
  • The diagnosis of cystic fibrosis is based on typical pulmonary manifestations, GI tract manifestations, a family history, and positive sweat chloride test results (see Workup). (
  • This review summarizes recent progress in the study of the pathogenesis and diagnosis of liver fibrosis and discusses current antifibrotic strategies. (
  • If you or a loved one recently received a diagnosis of pulmonary fibrosis, you may have questions about the disease. (
  • Detailed information on cystic fibrosis, including symptoms, diagnosis, treatment, and geneticsture to Product Detail Pages: POST LAUNCH ( retainer ) We continue to monitor COVID-1. (
  • The Liver Fibrosis SIG is an interactive group of investigators focusing on the pathogenesis, diagnosis, development of therapeutics for liver fibrosis and outcomes. (
  • Newborn screening is not a definitive diagnostic test for cystic fibrosis, but, if positive, indicates the need for additional testing to rule out or confirm a CF diagnosis. (
  • Jonathan's father, Paul, took an early and active role in combating cystic fibrosis after his son's diagnosis. (
  • The diagnosis was cystic fibrosis , an incurable genetic disease that, at the time, meant a life expectancy of 18 years. (
  • Almost 1,400 different mutations of the CF gene can lead to cystic fibrosis (some mutations cause milder symptoms than others). (
  • Cystic fibrosis is an inherited disease caused by mutations (changes) in a gene on chromosome 7, one of the 23 pairs of chromosomes that children inherit from their parents. (
  • Cystic fibrosis occurs most frequently in Caucasians of northern European descent, in whom the CF gene is most common - although people of other heritages can get the disease, too. (
  • To be born with cystic fibrosis, a child has to inherit a copy of the faulty gene from both of their parents. (
  • This can happen if the parents are "carriers" of the faulty gene, which means they don't have cystic fibrosis themselves. (
  • cystic fibrosis patients must inherit a mutated gene from each parent. (
  • Genetic screening for cystic fibrosis carrier mutations (one copy of a mutated gene) is universally recommended for the reproductive-age population. (
  • [ 4 ] Cystic fibrosis is an autosomal recessive disorder, and most carriers of the gene are asymptomatic. (
  • Identification of the cystic fibrosis gene: chromosome walking and jumping. (
  • Buchwald M, Tsui LC, Riordan J: The search for the cystic fibrosis gene. (
  • Rommens JM, Iannuzzi MC, Kerem B, et al: Identification of the cystic fibrosis gene: Chromosome walking and jumping. (
  • Riordan JR, Rommens JM, Kerem B, et al: Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA. (
  • Kerem B, Rommens JM, Buchanan JA, et al: Identification of the cystic fibrosis gene: genetic analysis. (
  • Cystic fibrosis is transmitted to a child when both parents carry the recessive gene but do not have the disease. (
  • Cystic Fibrosis (CF) is when a gene in the body has mutated, and it can no longer control the fluids and salts that go in and out of the cells. (
  • To have the disease, you must inherit the faulty cystic fibrosis gene from both parents. (
  • And you're only at risk of cystic fibrosis if both of your parents carry the defective gene. (
  • It's possible to have the carrier gene for cystic fibrosis, but not the condition itself. (
  • This means that if you carry the faulty gene and you produce an offspring with another person who also has the faulty gene, there's a 1 in 4 chance that your child will be born with cystic fibrosis. (
  • How can I get tested to see if I am a carrier of the cystic fibrosis gene? (
  • Cystic fibrosis is an autosomal recessive disorder, which means that you have to have two faulty copies of the gene to suffer from the disease. (
  • In 1989 the defective gene responsible for cystic fibrosis was isolated. (
  • At the same time, scientists in Britain have begun a human gene therapy trial of their own, using a very different and theoretically gentler method of inserting new genes into the diseased airways of cystic fibrosis patients. (
  • Two weeks ago, Dr. James Wilson of the University of Pennsylvania in Philadelphia also began a gene therapy trial using the adenovirus to shepherd in copies of the cystic fibrosis gene, treating a 32-year-old woman from New Jersey. (
  • Adults with cystic fibrosis experience health problems affecting the respiratory, digestive, and reproductive systems. (
  • Cystic fibrosis is a disease of exocrine gland function that involves multiple organ systems but chiefly results in chronic respiratory infections, pancreatic enzyme insufficiency, and associated complications in untreated patients (see Clinical). (
  • Boat T: Cystic fibrosis, in Murray J, Nadel J (eds): Textbook of Respiratory Medicine. (
  • Knowles MR, Stutts MJ, Spock A et al: Abnormal ion permeation through cystic fibrosis respiratory epithelium. (
  • Gaskin K, Gurwitz D, Durie P, et al: Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption. (
  • Cystic fibrosis (CF) primarily affects the respiratory, digestive and reproductive systems, as well as the sweat glands. (
  • P. aeruginosa is the most common virulent respiratory pathogen in cystic fibrosis," she says, adding that over 48% of children and young adults with cystic fibrosis in Canada are infected with the bug. (
  • The Travel Bug Item you have found contains information about Cystic Fibrosis, which is a genetic disease that affects mainly respiratory and digestive system. (
  • This is an open-label, multi-center interventional trial in Cystic Fibrosis (CF) patients with new MRSA isolated from the respiratory tract (oropharyngeal (OP) = OP swab, sputum, or bronchoscopy) at a clinical encounter. (
  • Cystic fibrosis is a chronic disease that affects approximately 30,000 children and young adults in the United States. (
  • Cystic fibrosis is a lifelong (chronic) disease that affects glands throughout the body. (
  • Cystic fibrosis is a fatal genetic disease that affects 30,000 children and adults in the United States. (
  • The Food and Drug Administration approved Kalydeco Jan. 31 to treat a rare form of the disease that affects just 1,200 people in the U.S., or 4 percent of the population with cystic fibrosis. (
  • I've been a part of it the past two or three years now and to be able to come out here and raise money for cystic fibrosis, and see how it affects the families and the kids, we're trying to help. (
  • In the UK, all newborn babies are screened for cystic fibrosis as part of the newborn blood spot test (heel prick test) carried out shortly after they're born. (
  • All newborn babies are screened for cystic fibrosis. (
  • Does weight history affect fibrosis in the setting of chronic liver disease? (
  • Cystic fibrosis is a multisystem disease characterized primarily by chronic pulmonary infection and bronchiectasis, pancreatic exocrine impairment, and elevated sweat chloride. (
  • Liver fibrosis is the excessive accumulation of extracellular matrix proteins including collagen that occurs in most types of chronic liver diseases. (
  • Pulmonary fibrosis can be caused by many conditions including chronic inflammatory processes, infections, environmental agents, exposure to ionizing radiation , chronic conditions, and certain medications. (
  • Cystic fibrosis is a chronic, progressive, life-threatening disease. (
  • Hepatic fibrosis is usually associated with chronic liver diseases caused by infection, drugs, metabolic disorders, or autoimmune imbalances. (
  • As hepatic fibrosis is a common outcome of a variety of chronic liver diseases, this review will highlight and summarize recent progresses of the role of autophagy in hepatic fibrosis. (
  • Repeated injuries, chronic inflammation and repair are susceptible to fibrosis where an accidental excessive accumulation of extracellular matrix components, such as the collagen is produced by fibroblasts, leading to the formation of a permanent fibrotic scar. (
  • A discussion about chronic infections, their relevance in cystic fibrosis, and why they are difficult to treat with standard antibiotics. (
  • Oral submucous fibrosis is a chronic debilitating disease of the oral cavity characterized by inflammation and progressive fibrosis of the submucosal tissues (lamina propria and deeper connective tissues). (
  • In one article, Pratt et al describes a project coordinated by the Centers for Disease Control and Prevention's Genetic Testing Reference Material (GeT-RM) Program to develop a set of reference materials for the expanded cystic fibrosis panel of mutations. (
  • About the Galapagos - AbbVie collaboration in cystic fibrosis In September 2013 Galapagos signed an agreement with AbbVie in which they will work collaboratively to develop and commercialize oral drugs that address the main mutations in CF patients, including F508del and G551D. (
  • Oh behalf of the cystic fibrosis community, I want to thank members of Congress for their commitment to fight cystic fibrosis. (
  • A Canadian teenager has scooped a CAN$5,000 prize and deserved glory after successfully wielding the power of a scientific supercomputing network to develop a mix of drugs which could be used to fight cystic fibrosis. (
  • These tests can also be used to diagnose cystic fibrosis in older children and adults who didn't have the newborn test. (
  • Learn about topics such as How to Diagnose Cystic Fibrosis , How to Be Vegan with Cystic Fibrosis , How to Cope with Stress As a Cystic Fibrosis Caregiver , and more with our helpful step-by-step instructions with photos and videos. (
  • Retroperitoneal fibrosis is a disorder in which inflammation and extensive scar tissue (fibrosis) occur in the back of the abdominal cavity, behind (retro-) the membrane that surrounds the organs of the digestive system (the peritoneum). (
  • In the past, treatment was aimed at minimizing inflammation and slowing the progression from inflammation to fibrosis. (
  • Unfortunately with pulmonary fibrosis there is a limited amount of adjunct therapies, seeing as their alveolar tissue is essentially scar tissue now with maybe a hint of underlying inflammation. (
  • Galapagos (Euronext & NASDAQ: GLPG) is a clinical-stage biotechnology company specialized in the discovery and development of small molecule medicines with novel modes of action, with a pipeline comprising three Phase 2 programs, two Phase 1 trials, five pre-clinical studies, and 20 discovery small-molecule and antibody programs in cystic fibrosis, inflammation, and other indications. (
  • The mission of the Cystic Fibrosis Foundation is to cure cystic fibrosis and to provide all people with the disease the opportunity to lead full, productive lives by funding research and drug development, promoting individualized treatment, and ensuring access to high-quality, specialized care. (
  • The mission of the Cystic Fibrosis Foundation, a nonprofit donor-supported organization, is to cure cystic fibrosis and to provide all people with the disease the opportunity to lead full, productive lives by funding research and drug development, promoting individualized treatment, and ensuring access to high-quality, specialized care. (
  • To think we're going to cure cystic fibrosis in a year is naive," Dr. Crystal said. (
  • Although the pathogenesis of pulmonary fibrosis remains incompletely understood, identification and understanding the role of genetic risk factors helps provide novel insights into the pathophysiology of the disease and identify molecular regulators of inflammatory and fibrotic processes. (
  • The pathogenesis of the disease is not well established, but the cause of oral submucous fibrosis is believed to be multifactorial. (
  • The areca nut component of betel quid plays a major role in the pathogenesis of oral submucous fibrosis. (
  • The development of the scar tissue is called fibrosis. (
  • Fibrosis is the replacement of normal tissue with scar tissue, and it can affect a joint's range of motion and/or be a cause of pain Fibrosis is the replacement of normal tissue wi. (
  • Fibrosis, also known as fibrotic scarring, is a pathological wound healing in which connective tissue replaces normal parenchymal tissue to the extent that it goes unchecked, leading to considerable tissue remodelling and the formation of permanent scar tissue. (
  • Penketh ARL, Wise A, Mearns MB, et al: Cystic fibrosis in adolescents and adults. (
  • CF has really transformed from a childhood disease to one in which adults are planning and living out their futures," said Seth Walker, a pulmonologist who heads up the Adult Cystic Fibrosis Center at Emory University at Atlanta. (
  • The event, held annually since 1986, has raised over $4.87 million to help find a cure for cystic fibrosis, a fatal disease afflicting children and young adults. (
  • Retroperitoneal fibrosis can occur at any age but appears most frequently between the ages of 40 and 60. (
  • Retroperitoneal fibrosis occurs in 1 in 200,000 to 500,000 people per year. (
  • No genes associated with retroperitoneal fibrosis have been identified. (
  • Retroperitoneal fibrosis occasionally occurs with autoimmune disorders, which result when the immune system malfunctions and attacks the body's own organs and tissues. (
  • Researchers suggest that the immune system may be involved in the development of retroperitoneal fibrosis. (
  • Most cases of retroperitoneal fibrosis are sporadic, which means that they occur in people with no apparent history of the disorder in their family. (
  • Brandt AS, Kamper L, Kukuk S, Haage P, Roth S. Associated findings and complications of retroperitoneal fibrosis in 204 patients: results of a urological registry. (
  • Two brothers with mediastinal-retroperitoneal fibrosis. (
  • Pipitone N, Vaglio A, Salvarani C. Retroperitoneal fibrosis. (
  • This article discusses one of the diseases caused by asbestos, namely retroperitoneal fibrosis, recently recognized as an occupational disease in Switzerland. (
  • Johns Hopkins Adult Cystic Fibrosis Program, Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. (
  • Dr. Allada is an associate professor of medicine and the Director of the Adult Cystic Fibrosis program. (
  • What is the liver fibrosis? (
  • Many forms of liver injury are marked by fibrosis, which is defined as an excess deposition of the components of the extracellular matrix (ie, collagens, glycoproteins, proteoglycans) in the liver. (
  • Advanced liver fibrosis results in cirrhosis, liver failure, and portal hypertension and often requires liver transplantation. (
  • Our knowledge of the cellular and molecular mechanisms of liver fibrosis has greatly advanced. (
  • Reversibility of advanced liver fibrosis in patients has been recently documented, which has stimulated researchers to develop antifibrotic drugs. (
  • Although many therapeutic interventions are effective in experimental models of liver fibrosis, their efficacy and safety in humans is unknown. (
  • Cellular mechanisms of liver fibrosis. (
  • If the liver injury persists, accumulation of activated HSCs and portal myofibroblasts occurs, synthesizing large amounts of ECM proteins and leading to tissue fibrosis. (
  • If the cause of the liver injury is removed, fibrosis is resolved. (
  • Besides that, autophagy can induce some liver diseases to develop while it may play a protective role in hepatocellular abnormal aggregates related liver diseases and reduces fibrosis. (
  • Hepatic fibrosis can develop into cirrhosis within 1-10 years with a 7- to 10-year liver-related mortality of 12% to 25% [ 1 ]. (
  • The goals of our SIG are to broaden our knowledge of the disease and to foster collaborations in basic and translational / clinical studies of liver fibrosis. (
  • The Liver Fibrosis SIG sponsors Programs at the AASLD Annual Meeting, identifies emerging topics in fibrosis research and develops educational webinar programs. (
  • I am wondering about the regression of liver fibrosis on all oral treatment. (
  • If SVR is attained and depending on the initial degree of liver damage most studies show fibrosis regression can and does occur and can be as much as much as one or two stages. (
  • A cirrhotic liver or those with advance fibrosis will never revert back to stage 0 but the fibrosis will diminish with most and the overall health of the liver will be greatly improved. (
  • Quantitative results of 6 biochemical tests are analyzed using a computational algorithm to provide a quantitative surrogate marker (0.0-1.0) for liver fibrosis (METAVIR F0-F4) and for necroinflammatory activity (METAVIR A0-A3). (
  • They're more likely to pick up infections, and more vulnerable to complications if they do develop an infection, which is why people with cystic fibrosis shouldn't meet face to face. (
  • [ 12 , 13 ] This suggests that the muscle is abnormal initially and is especially susceptible to injury and the development of fibrosis. (
  • In the UK, most cases of cystic fibrosis are picked up at birth using the newborn screening heel prick test. (
  • A person with cystic fibrosis is born with the condition. (
  • This is a pancreatic enzyme precursor that is elevated in the blood of babies with cystic fibrosis due to blocked pancreatic ducts failing to drain adequately. (
  • Some babies with cystic fibrosis may also appear fine at birth, but then develop breathing difficulties or do not put on weight in their first four to six weeks. (
  • Some people with pulmonary fibrosis become ill very quickly. (
  • Marshall Zhang, a Grade 11 student at Bayview Secondary School in Richmond Hill near Toronto, used Canada's collaborative SCINET super-net to model the effects of different compounds on the mutant proteins responsible for cystic fibrosis. (
  • A study of the frequency of hiatal hernia and gastroesophageal reflux in interstitial pulmonary fibrosis of obscure etiology. (
  • LeGrys VA, Yankaskas JR, Quittell LM, Marshall BC, Mogayzel PJ Jr. Diagnostic sweat testing: the Cystic Fibrosis Foundation guidelines. (
  • Quinton PM, Bigman J: Higher bioelectric potentials due to decreased chloride absorption in the sweat glands of patients with cystic fibrosis. (
  • Sato K, Sato F: Defective beta adrenergic response of cystic fibrosis sweat glands in vivo and in vitro. (
  • Another test used to check for cystic fibrosis is the sweat test, which measures the amount of chloride present in a patient's sweat. (
  • High levels of chloride in the sweat indicates cystic fibrosis. (
  • Sweat tests must be performed by trained technicians and evaluated in an experienced, reliable laboratory, or at a Cystic Fibrosis Foundation-accredited Care Center where strict guidelines help ensure accurate results. (
  • The high salt content in perspiration is the basis for the "sweat test," which is the definitive diagnostic test for the presence of cystic fibrosis. (
  • In an experiment conducted in several different parts, the researchers used strains of mice with a mutation that gave them cystic fibrosis. (
  • The researchers were testing the theory that at the higher cell-pH associated with the cystic fibrosis mutation, the enzyme that normally breaks down ceramide does not work and sometimes produces more ceramide. (
  • To see whether their findings were relevant to humans, the researchers compared the ceramide content in certain cells taken from the noses of 18 people with cystic fibrosis with nasal cells taken from 17 healthy controls. (
  • The researchers say their study sheds light on the conditions under which microbes involved in cystic fibrosis can survive. (
  • More studies on cystic fibrosis should help researchers find better and more effective treatments for this serious condition. (
  • Physicians, researchers and patients will gather in Nashville for the Pulmonary Fibrosis Foundation's biennial global summit, Nov. 9-11. (
  • After nearly 15 years of service to the Pulmonary Fibrosis (PF) community of patients, families, researchers, physicians and advocates, the Coalition for Pulmonary Fibrosis (CPF) announced today it will be closing. (
  • Researchers at the University of Iowa and the University of Missouri have developed a better animal model of cystic fibrosis. (
  • While the pigs reproduce clear clinical trademarks of the human disease, the researchers found that, like humans with cystic fibrosis, the piglets are born with a bowel obstruction that, without surgery, is 100 percent fatal. (
  • Cystic fibrosis used to be considered a fatal disease of childhood. (
  • With improved treatments and better ways to manage the disease, many people with cystic fibrosis now live well into adulthood. (
  • Cystic fibrosis is a common genetic disease within the white population in the United States. (
  • Cystic fibrosis (CF) is the most common lethal inherited disease in white persons. (
  • Amelioration of cystic fibrosis intestinal mucous disease in mice by restoration of mCLCA3. (
  • Cystic fibrosis is the most common inherited life-threatening disease that occurs among Caucasian people in the UK. (
  • But with the development of new drugs and airway clearance techniques that are helping to make cystic fibrosis a manageable disease, she's a drummer, a triathlete, and the CEO of Rock CF, a Denver-based non-profit organization aimed at improving quality of life for people with CF through exercise. (
  • Cystic fibrosis is an inherited disease. (
  • There is no cure for cystic fibrosis, treatment of symptoms is used to manage the disease. (
  • Inhalation of certain toxins, autoimmune disease, and radiation may cause pulmonary fibrosis. (
  • Cystic fibrosis is a type of genetic disease that many people struggle with today. (
  • Cystic Fibrosis: The Facts provides a much needed simple and understandable source book about this disease. (
  • A dozen white chocolate roses (a symbol for cystic fibrosis because children tend to pronounce the disease "65 roses") were given to Emma Jane. (
  • A fitness trainer from Belfast who has cystic fibrosis says a new online workout programme could be 'massive' for people living with the disease. (
  • Treatments for people with cystic fibrosis can vary depending on the severity of the disease but can include the use of inhalers, nebulisers, antibiotic therapy and a high calorie diet. (
  • I think it's important for people to know that cystic fibrosis is a complex, multi-system disease. (
  • Although Kalydeco has so far been approved only for 4 per cent of cystic fibrosis patients, who carry a specific mutation, the three drugs together could treat more than 90 per cent of people with the disease. (
  • A pair of teenagers with cystic fibrosis meet in a hospital and fall in love, though their disease means they must avoid close physical contact. (
  • Cystic fibrosis is considered a life-shortening disease, but advances in care have increased the median (middle of the age range) of survival to more than 38 years. (
  • Our team of experts believes that effectively treating cystic fibrosis means focusing on both the pulmonary aspects of the disease and the resulting digestive tract complications. (
  • Cystic fibrosis was not recognized as a separate disease until 1938 and was then classified as a childhood disease because mortality among afflicted infants and children was high. (
  • WASHINGTON, July 15 /PRNewswire-USNewswire/ -- The U.S. House of Representatives today passed a resolution to encourage public awareness and understanding of cystic fibrosis, and support research to find a cure for the disease. (
  • The group found no major differences between the disease and control groups before the disease group began showing symptoms, suggesting that people born with cystic fibrosis are not necessarily born with a hypersensitive immune response. (
  • Since the Mariners first partnered with the Cystic Fibrosis Foundation nearly 28 years ago, the life expectancy for someone struggling with the disease has nearly doubled, from approximately 18 years old, to nearly 40. (
  • Cystic fibrosis (CF) is the most frequent autosomal recessive inheritable disease in the Caucasian population in Denmark, affecting 1 in 4,700 children. (
  • Cystic fibrosis, the most common lethal hereditary disease among whites, afflicts about 30,000 Americans. (
  • He maintained a "Neutral" rating on the stock and said he thinks Vertex will have about $5 billion in total sales of cystic fibrosis drugs by 2017. (
  • A mutated type of these bacteria, called the "mucoid" form, can contribute to fatal pneumonia in people with the genetic disorder, cystic fibrosis. (
  • Cystic Fibrosis is a serious genetic disorder caused by the inheritance of a defective transporter protein . (
  • In adolescence or adulthood, a shortage of insulin can cause a form of diabetes known as cystic fibrosis-related diabetes mellitus (CFRDM). (
  • In those with what is known as cystic fibrosis, this could be the case - as more often than not, individuals are born with this condition. (
  • and various ducts, causing the characteristic signs and symptoms of cystic fibrosis. (
  • What are the symptoms of cystic fibrosis? (
  • Find out more about treatments for cystic fibrosis . (
  • What Are the Treatments for Cystic Fibrosis? (
  • However, research and treatments for cystic fibrosis have improved vastly in the last several decades. (
  • NEW YORK (AP) -- Shares of Vertex Pharmaceuticals Inc. surged Friday after the company reported positive clinical trial results for a drug regimen designed to treat cystic fibrosis. (