Fibrosis
Cystic Fibrosis
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.
Cystic Fibrosis Transmembrane Conductance Regulator
Endomyocardial Fibrosis
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).
Idiopathic Pulmonary Fibrosis
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 Cirrhosis
Retroperitoneal Fibrosis
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.
Bleomycin
Liver Cirrhosis, Experimental
Lung
Hydroxyproline
Hepatic Stellate Cells
Ureteral Obstruction
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.
Disease Models, Animal
Myofibroblasts
Collagen
Carbon Tetrachloride
Transforming Growth Factor beta
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.
Pseudomonas aeruginosa
Peritoneal Fibrosis
Collagen Type III
Fibroblasts
Collagen Type I
Biopsy
Liver
Sweat
Sputum
Nephritis, Interstitial
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.
Scleroderma, Systemic
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.
Mice, Inbred CFTR
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.
Myocardium
Disease Progression
Liver Diseases, Parasitic
Kidney
Hepatitis C, Chronic
Burkholderia cepacia
Nephrogenic Fibrosing Dermopathy
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.
Epithelial Cells
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.
Mice, Knockout
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.
Connective Tissue Growth Factor
Elasticity Imaging Techniques
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.
Biological Markers
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, Cultured
Fatty Liver
Respiratory Mucosa
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.
Bronchoalveolar Lavage Fluid
Respiratory Function Tests
RNA, Messenger
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.
Immunohistochemistry
Lung Diseases, Interstitial
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.
Smad3 Protein
Inflammation
Extracellular Matrix
Exocrine Pancreatic Insufficiency
Actins
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.
Rats, Sprague-Dawley
Severity of Illness Index
Alanine Transaminase
Signal Transduction
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.
Thioacetamide
Chronic Disease
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)
Kidney Tubules
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.
Chloride Channels
Tissue Inhibitor of Metalloproteinase-1
Lung Transplantation
Forced Expiratory Volume
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.
Mutation
Cardiomegaly
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.
Ventricular Remodeling
Capillaria
Aspartate Aminotransferases
Nasal Mucosa
Bile Ducts
Tobramycin
Rats, Wistar
Gene Expression
Phenotype
Cardiomyopathies
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).
Procollagen
Pancreatin
Neonatal Screening
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.
Bronchi
Smad2 Protein
Respiratory System
Asbestosis
Gene Expression Regulation
Liver Function Tests
Reverse Transcriptase Polymerase Chain Reaction
Burkholderia cepacia complex
Mice, Transgenic
Pulmonary Alveoli
Schistosomiasis mansoni
Mucus
Pancreatic Extracts
Hyaluronic Acid
Expectorants
Vital Capacity
Cell Transdifferentiation
Genotype
Silicosis
Primary Myelofibrosis
Epithelium
Blotting, Western
Smad7 Protein
Treatment Outcome
Hepatocytes
Retrospective Studies
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.
Hepatitis C
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.
Pneumoconiosis
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.
Fibronectins
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.
Dimethylnitrosamine
Trachea
Cytokines
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.
Smad Proteins
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.
Up-Regulation
Apoptosis
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.
Pancreas
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.
Nephrosclerosis
Macrophages
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.)
Epithelial-Mesenchymal Transition
Antibiotics, Antineoplastic
Oxidative Stress
Meconium
Skin
Hepatitis B, Chronic
Alginates
Collagen Type IV
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.
Administration, Inhalation
Lung Injury
HSP47 Heat-Shock Proteins
Bronchoalveolar Lavage
Gadolinium
Polymerase Chain Reaction
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.
Leukocyte Elastase
Pleura
Matrix Metalloproteinase 2
Angiotensin II
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.
Case-Control Studies
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.
Aspergillosis, Allergic Bronchopulmonary
Asbestos
Drugs, Chinese Herbal
Sensitivity and Specificity
Radiation Pneumonitis
Silicon Dioxide
Mucociliary Clearance
Tomography, X-Ray Computed
Enzyme-Linked Immunosorbent Assay
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.
Achromobacter denitrificans
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.
Alveolitis, Extrinsic Allergic
Prospective Studies
Predictive Value of Tests
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.
Burkholderia cenocepacia
Cholestasis
Peritoneum
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 Glands
Extracellular Matrix Proteins
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).
Intercellular Signaling Peptides and Proteins
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.
ROC Curve
Membrane Proteins
Liver Cirrhosis, Alcoholic
Myocytes, Cardiac
Dose-Response Relationship, Drug
Hypertension, Portal
Molecular Sequence Data
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.
Matrix Metalloproteinase 9
Genetic Therapy
Ion Transport
Interleukin-13
Gadolinium DTPA
Statistics, Nonparametric
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)
Macrophages, Alveolar
Respiratory Tract Infections
Hypertrophy, Left Ventricular
Chemokine CCL2
Body Weight
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)
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INABILITY TO REGULATE FATTY ACID OXIDATION OR GLYCOLYSIS INCREASES RENAL FIBROSIS
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Cystic Fibrosis
Fibrosis
Fibrothorax Pulmonary fibrosis Cystic fibrosis Idiopathic pulmonary fibrosis (idiopathic meaning the cause is unknown) ... Glial scar Myocardial fibrosis has mainly two forms: Interstitial fibrosis, which has been described in congestive heart ... Replacement fibrosis, which indicates an older myocardial infarction. Healthy myocardium versus interstitial fibrosis in ... Progressive massive fibrosis (lungs); a complication of coal workers' pneumoconiosis Retroperitoneal fibrosis (soft tissue of ...
Mediastinal fibrosis
... most common cause is idiopathic mediastinal fibrosis; less commonly histoplasmosis tuberculosis or unknown ... It is characterized by invasive, calcified fibrosis centered on lymph nodes that block major vessels and airways. In Europe, ... Mitchell IM, Saunders NR, Maher O, Lennox SC, Walker DR (1986). "Surgical treatment of idiopathic mediastinal fibrosis: report ... Mediastinitis Davis AM, Pierson RN, Loyd JE (2001). "Mediastinal fibrosis". Seminars in Respiratory Infections. 16 (2): 119-30 ...
Retroperitoneal fibrosis
Malignant retroperitoneal fibrosis usually gives uneven MRI signals, is bulky, extends above the origins of renal arteries, or ... Biopsy should also be done if the location of fibrosis is atypical or if there is an inadequate response to initial treatment. ... Retroperitoneal fibrosis or Ormond's disease is a disease featuring the proliferation of fibrous tissue in the retroperitoneum ... Additionally, malignant retroperitoneal fibrosis less frequently displaces the ureters medially when compared to other causes ...
Cardiac fibrosis
Replacement fibrosis, which indicates an older infarction. Micrograph of healthy myocardium versus interstitial fibrosis in ... The most obvious treatment for cardiac valve fibrosis or fibrosis in other locations, consists of stopping the stimulatory drug ... Following are types of myocardial fibrosis: Interstitial fibrosis, which is unspecific, and has been described in congestive ... Cardiac fibrosis is common in non-human great apes in human care. The term idiopathic myocardial fibrosis was coined to ...
Injection fibrosis
Fibrosis Mukherjee PK, Das AK (1980). "Injection fibrosis in the quadriceps femoris muscle in children". J Bone Joint Surg Am. ... Injection fibrosis is a complication of intramuscular injection, occurring especially often in infants and children. Injections ...
Cystic fibrosis
The name "cystic fibrosis" refers to the characteristic fibrosis and cysts that form within the pancreas. Cystic fibrosis ... Search GeneCards for genes involved in cystic fibrosis Cystic Fibrosis Mutation Database "Cystic Fibrosis". MedlinePlus. U.S. ... "Orphanet: Cystic fibrosis". www.orpha.net. Retrieved 29 July 2022. Hodson M, Geddes D, Bush A, eds. (2012). Cystic Fibrosis ( ... Cochrane Cystic Fibrosis and Genetic Disorders Group) (March 2021). "Dornase alfa for cystic fibrosis". The Cochrane Database ...
Pulmonary fibrosis
"Pulmonary Fibrosis". MedicineNet, Inc. Archived from the original on 19 July 2014. Retrieved 26 July 2014. "Pulmonary fibrosis ... Misdiagnosis is common because, while overall pulmonary fibrosis is not rare, each individual type of pulmonary fibrosis is ... "Pulmonary Fibrosis". medlineplus.gov. Retrieved 20 December 2019. "Pulmonary fibrosis - Symptoms and causes". Mayo Clinic. ... and the resulting stiffness or decreased compliance makes pulmonary fibrosis a restrictive lung disease. Pulmonary fibrosis is ...
Bauxite fibrosis
Basic and Clinical Aspects of Pulmonary Fibrosis. CRC Press. pp. 391-. ISBN 978-0-8493-8927-6. WYATT JP, RIDDELL AC (1949). " ...
Cystic Fibrosis Canada
Shinerama List of cystic fibrosis organizations Cystic Fibrosis Foundation Cystic Fibrosis Trust "Annual Report 2009: Keeping a ... Cystic Fibrosis Canada's mandate is to help individuals with cystic fibrosis, principally by funding cystic fibrosis research ... Cystic Fibrosis Canada raises funds in order to promote public awareness and support research and high-quality cystic fibrosis ... "Cystic Fibrosis Canada: About us: What we do: Mission". Cystic Fibrosis Canada. 2009-08-06. Archived from the original on 2011- ...
Cystic Fibrosis Foundation
The Cystic Fibrosis Foundation has been a pioneer of cystic fibrosis treatment, having played a major role in the development ... "About the Cystic Fibrosis Foundation". Cystic Fibrosis Foundation. Retrieved 2013-02-19. "FDA Approves Drug That Could Treat 90 ... "The Danger of Cross Infections for Those Living With Cystic Fibrosis - Cystic Fibrosis News Today". Retrieved 2020-09-29. ... "Board of Trustees". Cystic Fibrosis Foundation. Retrieved May 6, 2018. "Advisory Council". Cystic Fibrosis Foundation. ...
Nephrogenic systemic fibrosis
... is a rare syndrome that involves fibrosis of skin, joints, eyes, and internal organs. NSF is ... "nephrogenic systemic fibrosis" to better describe its systemic nature. The term "gadolinium-associated systemic fibrosis" has ... Kalb RE, Helm TN, Sperry H, Thakral C, Abraham JL, Kanal E (March 2008). "Gadolinium-induced nephrogenic systemic fibrosis in a ... Daftari Besheli L, Aran S, Shaqdan K, Kay J, Abujudeh H (July 2014). "Current status of nephrogenic systemic fibrosis". ...
Oral submucous fibrosis
... is clinically divided into three stages: Stage 1: Stomatitis Stage 2: Fibrosis a- Early lesions, ... Severe oral submucous fibrosis is irreversible. Moderate oral submucous fibrosis is reversible with cessation of habit and ... Oral Submucous Fibrosis at eMedicine Donoghue M, Basandi PS, Adarsh H, Madhushankari GS, Selvamani M, Nayak P (2015). "Habit- ... Group IVA: Trismus is severe, with an interincisal distance of less than 15 mm and extensive fibrosis of all the oral mucosa. ...
Cystic Fibrosis Trust
List of cystic fibrosis organizations Cystic Fibrosis Foundation Canadian Cystic Fibrosis Foundation UK Cystic Fibrosis Gene ... The aim of the Cystic Fibrosis Trust research is to understand, treat and cure cystic fibrosis. The Cystic Fibrosis Trust is ... The Cystic Fibrosis Trust (stylised as Cystic Fibrosis), is a UK-based national charity founded in 1964, dealing with all ... The Cystic Fibrosis Trust sets the national standard on clinical care; provides and funds a UK CF Clinical Database; and ...
Idiopathic pulmonary fibrosis
Wikimedia Commons has media related to Idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis at Curlie (All articles ... December 2011). "Familial pulmonary fibrosis is the strongest risk factor for idiopathic pulmonary fibrosis". Respiratory ... "Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical ... "Mucin 5B promoter polymorphism is associated with idiopathic pulmonary fibrosis but not with development of lung fibrosis in ...
Progressive massive fibrosis
... (PMF), characterized by the development of large conglomerate masses of dense fibrosis (usually in ... Progressive massive fibrosis increased during the period 1970-2016 among coal miners in central Appalachia who filed for black ... Primary Source: Annals of the American Thoracic Society Source Reference: Almberg KS, et al "Progressive massive fibrosis ... fibrosis, and emphysema.[citation needed] There are also some mechanical factors involved in the pathogenesis of Complex ...
Radiation Fibrosis Syndrome
"Síndrome de fibrosis por radiación". OncoLink. Trustees of the University of Pennsylvania. Retrieved 2 May 2022. "Fibrosis ... "Pulmonary fibrosis". Cleveland Clinic. Cleveland Clinic. 2021. Retrieved 2 May 2022. "Idiopathic pulmonary fibrosis". AMBOSS. ... Radiation fibrosis syndrome is a human illness. It occurs as a result of cell death, and can be caused by radiotherapy. It is ... Hematic biometry Imaging tests such as X-rays or tomography Pulmonary function tests Biopsy Pulmonary fibrosis is not currently ...
Congenital hepatic fibrosis
... usually presents in adolescent or young adulthood, but onset of signs and symptoms can range from ... The fibrosis would affect resistance in portal veins leading to portal hypertension.[citation needed] The condition is usually ... Embryogenically, congenital hepatic fibrosis is due to malformation of the duct plate, a round structure appearing in the ... Congenital hepatic fibrosis is an inherited fibrocystic liver disease associated with proliferation of interlobular bile ducts ...
Equine multinodular pulmonary fibrosis
... is a chronic lung disease of horses. There is evidence that the disease is caused by ... The distribution between two patterns can be seen as a large, whitish until tan and firm nodules of fibrosis. There are noticed ... Poth T, Niedermaier G, Hermanns W (January 2009). "Equine multinodular pulmonary fibrosis in association with an EHV-5 ... Signs of equine multinodular pulmonary fibrosis are mainly weight loss, fever, respiratory distress and depression. ...
Cystic fibrosis-related diabetes
... (CFRD) is diabetes specifically caused by cystic fibrosis, a genetic condition. Cystic ... Cystic fibrosis Lek N, Acerini CL (January 2010). "Cystic fibrosis related diabetes mellitus - diagnostic and management ... Kayani K, Mohammed R, Mohiaddin H (2018-02-20). "Cystic Fibrosis-Related Diabetes". Frontiers in Endocrinology. 9: 20. doi: ... Articles with short description, Short description matches Wikidata, Diabetes, Pulmonology, Endocrinology, Cystic fibrosis). ...
Non-cirrhotic portal fibrosis
... (NCPF) is a chronic liver disease and type of non-cirrhotic portal hypertension (NCPH). It is ... Sarin, SK (December 2002). "Non-cirrhotic portal fibrosis". Journal of Gastroenterology and Hepatology. 17 Suppl 3: S214-23. ... "Non-cirrhotic portal fibrosis: one disease with many names? An analysis from morphological study of native explant livers with ...
Cystic fibrosis transmembrane conductance regulator
... in one copy of the CFTR gene and a cystic fibrosis-causing mutation in the other copy of CFTR. Cystic fibrosis: More than 1,800 ... The Cystic Fibrosis Transmembrane Conductance Regulator Protein The Human Gene Mutation Database - CFTR Records Cystic Fibrosis ... "Cystic fibrosis transmembrane conductance regulator and the etiology and pathogenesis of cystic fibrosis". FASEB Journal. 6 (10 ... Wong LJ, Alper OM, Wang BT, Lee MH, Lo SY (July 2003). "Two novel null mutations in a Taiwanese cystic fibrosis patient and a ...
Combined pulmonary fibrosis and emphysema
... (CPFE), describes a medical syndrome involving both pulmonary fibrosis and emphysema ... Wand, O; Kramer, MR (January 2018). "The Syndrome of Combined Pulmonary Fibrosis and Emphysema - CPFE". Harefuah. 157 (1): 28- ... Jankowich, MD; Rounds, SI (January 2012). "Combined pulmonary fibrosis and emphysema syndrome: a review". Chest. 141 (1): 222- ... Cottin, V; Cordier JF (June 2005). "Combined pulmonary fibrosis and emphysema: an experimental and clinically relevant ...
List of cystic fibrosis organizations
Cystic Fibrosis Foundation (CFF), a US non-profit providing the means to cure and control cystic fibrosis. Cystic Fibrosis ... Cystic Fibrosis Australia Cystic Fibrosis Community Care "CFWA - Cystic Fibrosis WA". www.cfwa.org.au. Retrieved 2019-09-05. ... Cystic-L Cystic Fibrosis Canada Cystic Fibrosis Foundation Cystic Fibrosis Lifestyle Foundation Archived 2008-02-25 at the ... Cystic Fibrosis Canada (CCFF), a Canada-wide health charity, which funds cystic fibrosis research and care. ...
Congenital fibrosis of the extraocular muscles
GeneReviews/NCBI/NIH/UW entry on Congenital Fibrosis of the Extraocular Muscles OMIM entries on Congenital Fibrosis of the ... Congenital fibrosis of the extraocular muscles is a class of rare genetic disorders affecting one or more of the muscles that ... Individuals with congenital fibrosis of the extraocular muscles (CFEOM) have varying degrees of ophthalmoplegia (an inability ... PMID 20074521.{{cite journal}}: CS1 maint: multiple names: authors list (link) Laughlin, R.C. Congenital fibrosis of the ...
List of people diagnosed with cystic fibrosis
The following notable people have or had cystic fibrosis. "'Real World: San Diego' Alum Frankie Abernathy Dead At 25". MTV. ... "Kerrang!". Robinson, Georgina (June 10, 2014). "Cystic fibrosis no barrier for Nathan Charles as he prepares for Test debut". ... "Lisa Bentley Triumphs Over CF". Canadian Cystic Fibrosis Foundation. July 7, 2004. Archived from the original on September 27, ... Travis Flores Webster, Philip; Coates, Sam (November 29, 2006). "Gordon Brown's baby son diagnosed with cystic fibrosis". The ...
Hereditary fibrosing poikiloderma with tendon contractures, myopathy, and pulmonary fibrosis
Muscle biopsy shows fibrosis and fatty infiltration. Skin biopsy shows fibrosis and alterations of the elastic network. This ... Hereditary fibrosing poikiloderma with tendon contractures, myopathy and pulmonary fibrosis is a rare genetic syndrome ... The main characteristics of this condition are poikiloderma, tendon contractures and progressive pulmonary fibrosis. Other ... and pulmonary fibrosis. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, (editors). GeneReview ...
Fibrothorax
... 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 ...
Lethal allele
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 ...
M. S. Valiathan
Endomyocardial Fibrosis. The National Medical Journal of India. Vol. 6. Oxford University Press. pp. 212-216. ISBN 978-0-19- ...
Germline mutation
"Cystic Fibrosis Canada". www.cysticfibrosis.ca. Retrieved 2017-11-30. O'Sullivan BP, Freedman SD (May 2009). "Cystic fibrosis ... Cystic fibrosis is an autosomal recessive disorder that causes a variety of symptoms and complications, the most common of ... If both parents have a mutated CFTR (cystic fibrosis transmembrane conductance regulator) protein, then their children have a ... Many Mendelian disorders stem from dominant point mutations within genes, including cystic fibrosis, beta-thalassemia, sickle- ...
Cystic Fibrosis | CDC
Cystic fibrosis (CF) is a genetic disorder that causes problems with breathing and digestion. CF affects about 35,000 people in ... CF results from mutations (changes) in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene, which has ... National Institutes of Health, National Heart, Lung, and Blood Institute: Cystic Fibrosis ...
Congenital fibrosis of the extraocular muscles: MedlinePlus Genetics
Congenital fibrosis of the extraocular muscles (CFEOM) is a disorder of the nervous system that affects use of the muscles that ... FIBROSIS OF EXTRAOCULAR MUSCLES, CONGENITAL, 2. *FIBROSIS OF EXTRAOCULAR MUSCLES, CONGENITAL, 3A, WITH OR WITHOUT EXTRAOCULAR ... Congenital fibrosis of the extraocular muscles (CFEOM) is a disorder of the nervous system that affects use of the muscles that ... Congenital Fibrosis of the Extraocular Muscles Overview. 2004 Apr 27 [updated 2021 Aug 12]. In: Adam MP, Mirzaa GM, Pagon RA, ...
Home | Cystic Fibrosis Foundation
The Cystic Fibrosis Foundation is the worlds leader in the search for a cure for CF and supports a broad range of research ... Cystic Fibrosis Foundation Announces Irena Barisic as Next Chief Operating and Financial Officer ... Newly Expanded Cystic Fibrosis Foundation Lab Will Enable More Research Into Genetic Therapies ... Cystic Fibrosis Foundation (national office). 4550 Montgomery Ave.. Suite 1100 N. Bethesda, MD 20814 ...
Cystic Fibrosis Canada Blog
Bonnell Foundation: Living with Cystic Fibrosis - National Organization for Rare Disorders
About Bonnell Foundation: Living with Cystic Fibrosis. Our mission is to give emotional and/or financial support to parents who ... Bonnell Foundation: Living with Cystic Fibrosis. PO Box 1215. Royal Oak, MI, USA ... have a loved one with cystic fibrosis. Our hope is one day no CF parent will experience the pain of losing a child to this ...
Asbestos May Trigger Pulmonary Fibrosis
Gadolinium: nephrogenic systemic fibrosis
Nephrogenic Systemic Fibrosis Medication
... is a disease of fibrosis of the skin and internal organs reminiscent but distinct from scleroderma or scleromyxedema. It is ... Nephrogenic systemic fibrosis (NSF), also known as nephrogenic fibrosing dermopathy (NFD), ... Role of endothelin-1/endothelin receptor signaling in fibrosis and calcification in nephrogenic systemic fibrosis. Exp Dermatol ... encoded search term (Nephrogenic Systemic Fibrosis) and Nephrogenic Systemic Fibrosis What to Read Next on Medscape ...
The Pulmonary Fibrosis Trust - JustGiving
Fundraise or donate to The Pulmonary Fibrosis Trust with JustGiving, the worlds leading online fundraising platform, helping ... The Pulmonary Fibrosis Trust. We provide personal support to people affected by Pulmonary Fibrosis. ... About The Pulmonary Fibrosis Trust. We offer practical, emotional and financial support where there is a need. We also raise ...
Antiinflammatory Therapies for Cystic Fibrosis
Review the use of anti-inflammatory medications for cystic fibrosis. ... The clinical features of cystic fibrosis occur as a result of mutations in the cystic fibrosis trans-membrane conductance ... Cystic fibrosis is the most common lethal, genetically inherited disorder among Caucasians.[4,6,8,11] ... Clearing the Air in Cystic Fibrosis: Airway Clearance Approaches for Patients 0.5 CME / CE / ABIM MOC Credits ...
Postural drainage in cystic fibrosis: is there a link with gastro-oesophageal reflux?
Cystic fibrosis*
Vertex May Make History With Cystic Fibrosis Drug
Cystic fibrosis is a symbol of how difficult it is to move from genetic discoveries to new medicines. The gene for the disorder ... In a potentially historic victory, a drug targeted to a specific genetic defect has improved the ability of cystic fibrosis ... The result is a big win for developer Vertex Pharmaceuticals and its partner, the Cystic Fibrosis Foundation, a patient group. ... Another measure of the drugs effect: patients with cystic fibrosis have elevated levels of chloride ions in their sweat, ...
Targeting cardiac fibrosis with engineered T cells | Nature
Excessive cardiac fibrosis is an important factor in the progression of various forms of cardiac disease and heart failure2. ... Adoptive transfer of CAR T cells against the fibroblast marker FAP reduces cardiac fibrosis and restores function after cardiac ... Here we demonstrate the efficacy of redirected T cell immunotherapy to specifically target pathological cardiac fibrosis in ... However, clinical interventions and therapies that target fibrosis remain limited3. ...
cystic fibrosis
Cystic Fibrosis and Nutrition (for Parents) - HackensackUMC
... kids with cystic fibrosis have some specific nutritional needs. Find out ways to help your child with CF grow healthy and ... Fibrosis quística y nutrición. What Is Cystic Fibrosis?. Cystic fibrosis (CF) is a genetic disorder that affects the bodys ... How Does Cystic Fibrosis Affect Nutrition?. The sticky mucus from cystic fibrosis can block normal absorption of key nutrients ... Kids with cystic fibrosis have some specific nutritional needs:. Salt. Kids with CF lose more salt in their sweat than their ...
fibrosis Clinical Research Trials | CenterWatch
cystic fibrosis
Cystic Fibrosis: Sample Menu (for Teens) - Cook Children's
PTC124 shows activity in cystic fibrosis; Pha | EurekAlert!
ABOUT CYSTIC FIBROSIS Cystic fibrosis (CF) is a life-threatening genetic disease that causes serious lung infections and ... the Cystic Fibrosis Foundation Therapeutics Inc. (the nonprofit affiliate of the Cystic Fibrosis Foundation), which recently ... PTC124 shows activity in cystic fibrosis; Phase 2 proof-of-concept data published in Lancet 1 of the first therapies to address ... Life sciences/Genetics/Human genetics/Medical genetics/Genetic disorders/Cystic fibrosis * /Life sciences/Genetics/Molecular ...
Cystic Fibrosis (CF) History
A history on the medical condition cystic fibrosis (CF) ... Cystic Fibrosis: How CF Affects the Body * Cystic Fibrosis: ... The earliest reference to cystic fibrosis is in medical texts around 1595. These texts linked salty skin and damage to the ... In particular, the Cystic Fibrosis Foundation (United States) has played a significant role in developing the current model of ... "cystic fibrosis of the pancreas" based on her autopsy findings of children who died of malnutrition. Other physicians of the ...
Citrullinated Vimentin Mediates Development and Progression of Lung Fibrosis
Raising awareness of covert killer: pulmonary fibrosis
Cystic Fibrosis Clinical Presentation: History, Physical Examination
Cystic fibrosis is an autosomal recessive disorder, and most carriers of the gene are asymptomatic. ... Cystic fibrosis (CF) is the most common lethal inherited disease in white persons. ... Cystic Fibrosis Foundation. Fibrosis Foundation Patient registry Annual Report 2008. Bethesda, MD: Cystic Fibrosis Foundation; ... Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation. J Pediatr. 2017 Feb. 181S:S4-S15.e1. [ ...
Canon Novallele Cystic Fibrosis Genotyping Assays | GenomeWeb
How Cystic Fibrosis Is Treated
Cystic fibrosis can be treated with airway clearance, antibiotics, mucolytics, lung transplant, and a promising new class of ... A Phase 2 study of the safety, pharmacokinetics, and efficacy of anabasum (JBT-101) in cystic fibrosis (CF). J Cystic Fibrosis ... Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines. J Cyst ... Can you cure cystic fibrosis with a lung transplant? It wont completely cure you. After a lung transplant, you wont have ...
Cystic Fibrosis Week
Cystic Fibrosis Research, Inc. (CFRI) was formed in 1975 by a group of parents whose children were not expected to survive ... We hope that you enjoy Cystic Fibrosis week at the ATS and find these resources helpful in advancing your understanding of this ... This is an incredible improvement from the 1950s, when an infant born with cystic fibrosis was not expected to survive to ... Our vision is Inform, engage and empower the cystic fibrosis community to reach the highest possible quality of life, as we ...