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)
Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury.
Infections with bacteria of the genus PSEUDOMONAS.
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.
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.
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.
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.
Inorganic compounds derived from hydrochloric acid that contain the Cl- ion.
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.
Either of the pair of organs occupying the cavity of the thorax that effect the aeration of the blood.
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.
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.
Cell membrane glycoproteins that form channels to selectively pass chloride ions. Nonselective blockers include FENAMATES; ETHACRYNIC ACID; and TAMOXIFEN.
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.
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.
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.
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.
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.
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 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.
Measurement of the various processes involved in the act of respiration: inspiration, expiration, oxygen and carbon dioxide exchange, lung volume and compliance, etc.
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.
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.
Persistent abnormal dilatation of the bronchi.
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.
Pathological processes involving any part of the LUNG.
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.
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.
The transference of either one or both of the lungs from one human or animal to another.
The viscous secretion of mucous membranes. It contains mucin, white blood cells, water, inorganic salts, and exfoliated cells.
Substances that reduce the growth or reproduction of BACTERIA.
Identification of genetic carriers for a given trait.
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.
Agents that increase mucous excretion. Mucolytic agents, that is drugs that liquefy mucous secretions, are also included here.
Salts of alginic acid that are extracted from marine kelp and used to make dental impressions and as absorbent material for surgical dressings.
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.
A preparation of hog pancreatic enzymes standardized for lipase content.
The cartilaginous and membranous tube descending from the larynx and branching into the right and left main bronchi.
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.
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.
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.
Sweat-producing structures that are embedded in the DERMIS. Each gland consists of a single tube, a coiled body, and a superficial duct.
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.
Phenols substituted in any position by an amino group.
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.
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.
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
'Oral Submucous Fibrosis' is a chronic, insidious, and potentially disabling condition, characterized by progressive stiffness and loss of elasticity of the oral mucosa, due to fibrotic changes in the lamina propria, often associated with juxta-epithelial inflammation and epithelial atrophy.
The volume of air that is exhaled by a maximal expiration following a maximal inspiration.
The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.
Established cell cultures that have the potential to propagate indefinitely.
Invasion of the host RESPIRATORY SYSTEM by microorganisms, usually leading to pathological processes or diseases.
Experimentally induced chronic injuries to the parenchymal cells in the liver to achieve a model for LIVER CIRRHOSIS.
The genetic constitution of the individual, comprising the ALLELES present at each GENETIC LOCUS.
A pyrazine compound inhibiting SODIUM reabsorption through SODIUM CHANNELS in renal EPITHELIAL CELLS. This inhibition creates a negative potential in the luminal membranes of principal cells, located in the distal convoluted tubule and collecting duct. Negative potential reduces secretion of potassium and hydrogen ions. Amiloride is used in conjunction with DIURETICS to spare POTASSIUM loss. (From Gilman et al., Goodman and Gilman's The Pharmacological Basis of Therapeutics, 9th ed, p705)
The administration of drugs by the respiratory route. It includes insufflation into the respiratory tract.
Detection of a MUTATION; GENOTYPE; KARYOTYPE; or specific ALLELES associated with genetic traits, heritable diseases, or predisposition to a disease, or that may lead to the disease in descendants. It includes prenatal genetic testing.
One or more layers of EPITHELIAL CELLS, supported by the basal lamina, which covers the inner or outer surfaces of the body.
A rehabilitation therapy for removal of copious mucus secretion from the lung of patients with diseases such as CHRONIC BRONCHITIS; BRONCHIECTASIS; PULMONARY ABSCESS; or CYSTIC FIBROSIS. The patient's head is placed in a downward incline (so the TRACHEA is inferior to the affected area) for 15- to 20-minute sessions.
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.
Term used to designate tetrahydroxy aldehydic acids obtained by oxidation of hexose sugars, i.e. glucuronic acid, galacturonic acid, etc. Historically, the name hexuronic acid was originally given to ascorbic acid.
Cyclic polypeptide antibiotic from Bacillus colistinus. It is composed of Polymyxins E1 and E2 (or Colistins A, B, and C) which act as detergents on cell membranes. Colistin is less toxic than Polymyxin B, but otherwise similar; the methanesulfonate is used orally.
A species of STENOTROPHOMONAS, formerly called Xanthomonas maltophilia, which reduces nitrate. It is a cause of hospital-acquired ocular and lung infections, especially in those patients with cystic fibrosis and those who are immunosuppressed.
A sugar acid formed by the oxidation of the C-6 carbon of GLUCOSE. In addition to being a key intermediate metabolite of the uronic acid pathway, glucuronic acid also plays a role in the detoxification of certain drugs and toxins by conjugating with them to form GLUCURONIDES.
A hydroxylated form of the imino acid proline. A deficiency in ASCORBIC ACID can result in impaired hydroxyproline formation.
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)
Glands of external secretion that release its secretions to the body's cavities, organs, or surface, through a duct.
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.
High molecular weight mucoproteins that protect the surface of EPITHELIAL CELLS by providing a barrier to particulate matter and microorganisms. Membrane-anchored mucins may have additional roles concerned with protein interactions at the cell surface.
A genus of gram-negative, aerobic, rod-shaped bacteria. Organisms in this genus had originally been classified as members of the PSEUDOMONAS genus but overwhelming biochemical and chemical findings indicated the need to separate them from other Pseudomonas species, and hence, this new genus was created.
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.
Potent activator of the adenylate cyclase system and the biosynthesis of cyclic AMP. From the plant COLEUS FORSKOHLII. Has antihypertensive, positive inotropic, platelet aggregation inhibitory, and smooth muscle relaxant activities; also lowers intraocular pressure and promotes release of hormones from the pituitary gland.
Care of patients with deficiencies and abnormalities associated with the cardiopulmonary system. It includes the therapeutic use of medical gases and their administrative apparatus, environmental control systems, humidification, aerosols, ventilatory support, bronchopulmonary drainage and exercise, respiratory rehabilitation, assistance with cardiopulmonary resuscitation, and maintenance of natural, artificial, and mechanical airways.
Perisinusoidal cells of the liver, located in the space of Disse between HEPATOCYTES and sinusoidal endothelial cells.
Elements of limited time intervals, contributing to particular results or situations.
Inbred C57BL mice are a strain of laboratory mice that have been produced by many generations of brother-sister matings, resulting in a high degree of genetic uniformity and homozygosity, making them widely used for biomedical research, including studies on genetics, immunology, cancer, and neuroscience.
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.
An infant during the first month after birth.
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.
The inactive proenzyme of trypsin secreted by the pancreas, activated in the duodenum via cleavage by enteropeptidase. (Stedman, 25th ed)
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 adenine nucleotide containing one phosphate group which is esterified to both the 3'- and 5'-positions of the sugar moiety. It is a second messenger and a key intracellular regulator, functioning as a mediator of activity for a number of hormones, including epinephrine, glucagon, and ACTH.
Inorganic salts that contain the -HCO3 radical. They are an important factor in determining the pH of the blood and the concentration of bicarbonate ions is regulated by the kidney. Levels in the blood are an index of the alkali reserve or buffering capacity.
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.
An educational process that provides information and advice to individuals or families about a genetic condition that may affect them. The purpose is to help individuals make informed decisions about marriage, reproduction, and other health management issues based on information about the genetic disease, the available diagnostic tests, and management programs. Psychosocial support is usually offered.
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.
An individual having different alleles at one or more loci regarding a specific character.
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.
Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.
Inflammation of the lung parenchyma that is associated with BRONCHITIS, usually involving lobular areas from TERMINAL BRONCHIOLES to the PULMONARY ALVEOLI. The affected areas become filled with exudate that forms consolidated patches.
Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules.
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)
Levels within a diagnostic group which are established by various measurement criteria applied to the seriousness of a patient's disorder.
The simultaneous, or near simultaneous, transference of heart and lungs from one human or animal to another.
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.
An individual in which both alleles at a given locus are identical.
Devices that cause a liquid or solid to be converted into an aerosol (spray) or a vapor. It is used in drug administration by inhalation, humidification of ambient air, and in certain analytical instruments.
Inflammation of the lung parenchyma that is caused by bacterial infections.
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.
Any tests done on exhaled air.
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.
Infections caused by bacteria that show up as pink (negative) when treated by the gram-staining method.
The excretory duct of the testes that carries SPERMATOZOA. It rises from the SCROTUM and joins the SEMINAL VESICLES to form the ejaculatory duct.
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 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 member of the CXC chemokine family that plays a role in the regulation of the acute inflammatory response. It is secreted by variety of cell types and induces CHEMOTAXIS of NEUTROPHILS and other inflammatory cells.
A condition caused by the lack of intestinal PERISTALSIS or INTESTINAL MOTILITY without any mechanical obstruction. This interference of the flow of INTESTINAL CONTENTS often leads to INTESTINAL OBSTRUCTION. Ileus may be classified into postoperative, inflammatory, metabolic, neurogenic, and drug-induced.
Determination of the nature of a pathological condition or disease in the postimplantation EMBRYO; FETUS; or pregnant female before birth.
Negatively charged atoms, radicals or groups of atoms which travel to the anode or positive pole during electrolysis.
A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.
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.
Sodium channels found on salt-reabsorbing EPITHELIAL CELLS that line the distal NEPHRON; the distal COLON; SALIVARY DUCTS; SWEAT GLANDS; and the LUNG. They are AMILORIDE-sensitive and play a critical role in the control of sodium balance, BLOOD VOLUME, and BLOOD PRESSURE.
Any impairment, arrest, or reversal of the normal flow of INTESTINAL CONTENTS toward the ANAL CANAL.
Colloids with a gaseous dispersing phase and either liquid (fog) or solid (smoke) dispersed phase; used in fumigation or in inhalation therapy; may contain propellant agents.
Inorganic binary compounds of iodine or the I- ion.
State of the body in relation to the consumption and utilization of nutrients.
Benzoic acids, salts, or esters that contain an amino group attached to carbon number 2 or 6 of the benzene ring structure.
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.
The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.
An infection caused by an organism which becomes pathogenic under certain conditions, e.g., during immunosuppression.
Encrustations, formed from microbes (bacteria, algae, fungi, plankton, or protozoa) embedding in extracellular polymers, that adhere to surfaces such as teeth (DENTAL DEPOSITS); PROSTHESES AND IMPLANTS; and catheters. Biofilms are prevented from forming by treating surfaces with DENTIFRICES; DISINFECTANTS; ANTI-INFECTIVE AGENTS; and antifouling agents.
The most common form of fibrillar collagen. It is a major constituent of bone (BONE AND BONES) and SKIN and consists of a heterotrimer of two alpha1(I) and one alpha2(I) chains.
An adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.
The phenotypic manifestation of a gene or genes by the processes of GENETIC TRANSCRIPTION and GENETIC TRANSLATION.
Body organ that filters blood for the secretion of URINE and that regulates ion concentrations.
Measurement of volume of air inhaled or exhaled by the lung.
Focal accumulations of EDEMA fluid in the NASAL MUCOSA accompanied by HYPERPLASIA of the associated submucosal connective tissue. Polyps may be NEOPLASMS, foci of INFLAMMATION, degenerative lesions, or malformations.
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 Pseudomonas aeruginosa secretory product pyocyanin inactivates alpha1 protease inhibitor: implications for the pathogenesis of cystic fibrosis lung disease. (1/5500)

Alpha1 Protease inhibitor (alpha1PI) modulates serine protease activity in the lung. Reactive oxygen species inactivate alpha1PI, and this process has been implicated in the pathogenesis of a variety of forms of lung injury. An imbalance of protease-antiprotease activity is also detected in the airways of patients with cystic fibrosis-associated lung disease who are infected with Pseudomonas aeruginosa. P. aeruginosa secretes pyocyanin, which, through its ability to redox cycle, induces cells to generate reactive oxygen species. We tested the hypothesis that redox cycling of pyocyanin could lead to inactivation of alpha1PI. When alpha1PI was exposed to NADH and pyocyanin, a combination that results in superoxide production, alpha1PI lost its ability to form an inhibitory complex with both porcine pancreatic elastase (PPE) and trypsin. Similarly, addition of pyocyanin to cultures of human airway epithelial cells to which alpha1PI was also added resulted in a loss of the ability of alpha1PI to form a complex with PPE or trypsin. Neither superoxide dismutase, catalase, nor dimethylthiourea nor depletion of the media of O2 to prevent formation of reactive oxygen species blocked pyocyanin-mediated inactivation of alpha1PI. These data raise the possibility that a direct interaction between reduced pyocyanin and alpha1PI is involved in the process. Consistent with this possibility, pretreatment of alpha1PI with the reducing agent beta-mercaptoethanol also inhibited binding of trypsin to alpha1PI. These data suggest that pyocyanin could contribute to lung injury in the P. aeruginosa-infected airway of cystic fibrosis patients by decreasing the ability of alpha1PI to control the local activity of serine proteases.  (+info)

Lipopolysaccharide (LPS) from Burkholderia cepacia is more active than LPS from Pseudomonas aeruginosa and Stenotrophomonas maltophilia in stimulating tumor necrosis factor alpha from human monocytes. (2/5500)

Whole cells and lipopolysaccharides (LPSs) extracted from Burkholderia cepacia, Pseudomonas aeruginosa, Stenotrophomonas maltophilia, and Escherichia coli were compared in their ability to stimulate tumor necrosis factor alpha (TNF-alpha) from the human monocyte cell line MonoMac-6. B. cepacia LPS, on a weight-for-weight basis, was found to have TNF-alpha-inducing activity similar to that of LPS from E. coli, which was approximately four- and eightfold greater than the activity of LPSs from P. aeruginosa and S. maltophilia, respectively. The LPS-stimulated TNF-alpha production from monocytes was found to be CD14 dependent. These results suggest that B. cepacia LPS might play a role in the pathogenesis of inflammatory lung disease in cystic fibrosis, and in some patients it might be responsible, at least in part, for the sepsis-like cepacia syndrome.  (+info)

The sialylation of bronchial mucins secreted by patients suffering from cystic fibrosis or from chronic bronchitis is related to the severity of airway infection. (3/5500)

Bronchial mucins were purified from the sputum of 14 patients suffering from cystic fibrosis and 24 patients suffering from chronic bronchitis, using two CsBr density-gradient centrifugations. The presence of DNA in each secretion was used as an index to estimate the severity of infection and allowed to subdivide the mucins into four groups corresponding to infected or noninfected patients with cystic fibrosis, and to infected or noninfected patients with chronic bronchitis. All infected patients suffering from cystic fibrosis were colonized by Pseudomonas aeruginosa. As already observed, the mucins from the patients with cystic fibrosis had a higher sulfate content than the mucins from the patients with chronic bronchitis. However, there was a striking increase in the sialic acid content of the mucins secreted by severely infected patients as compared to noninfected patients. Thirty-six bronchial mucins out of 38 contained the sialyl-Lewis x epitope which was even expressed by subjects phenotyped as Lewis negative, indicating that at least one alpha1,3 fucosyltransferase different from the Lewis enzyme was involved in the biosynthesis of this epitope. Finally, the sialyl-Lewis x determinant was also overexpressed in the mucins from severely infected patients. Altogether these differences in the glycosylation process of mucins from infected and noninfected patients suggest that bacterial infection influences the expression of sialyltransferases and alpha1,3 fucosyltransferases in the human bronchial mucosa.  (+info)

Cystic fibrosis-associated mutations at arginine 347 alter the pore architecture of CFTR. Evidence for disruption of a salt bridge. (4/5500)

Arginine 347 in the sixth transmembrane domain of cystic fibrosis transmembrane conductance regulator (CFTR) is a site of four cystic fibrosis-associated mutations. To better understand the function of Arg-347 and to learn how mutations at this site disrupt channel activity, we mutated Arg-347 to Asp, Cys, Glu, His, Leu, or Lys and examined single-channel function. Every Arg-347 mutation examined, except R347K, had a destabilizing effect on the pore, causing the channel to flutter between two conductance states. Chloride flow through the larger conductance state was similar to that of wild-type CFTR, suggesting that the residue at position 347 does not interact directly with permeating anions. We hypothesized that Arg-347 stabilizes the channel through an electrostatic interaction with an anionic residue in another transmembrane domain. To test this, we mutated anionic residues (Asp-924, Asp-993, and Glu-1104) to Arg in the context of either R347E or R347D mutations. Interestingly, the D924R mutation complemented R347D, yielding a channel that behaved like wild-type CFTR. These data suggest that Arg-347 plays an important structural role in CFTR, at least in part by forming a salt bridge with Asp-924; cystic fibrosis-associated mutations disrupt this interaction.  (+info)

beta3-adrenoceptor control the cystic fibrosis transmembrane conductance regulator through a cAMP/protein kinase A-independent pathway. (5/5500)

In human cardiac myocytes, we have previously identified a functional beta3-adrenoceptor in which stimulation reduces action potential duration. Surprisingly, in cardiac biopsies obtained from cystic fibrosis patients, beta3-adrenoceptor agonists produced no effects on action potential duration. This result suggests the involvement of cystic fibrosis transmembrane conductance regulator (CFTR) chloride current in the electrophysiological effects of beta3-adrenoceptor stimulation in non-cystic fibrosis tissues. We therefore investigated the control of CFTR activity by human beta3-adrenoceptors in a recombinant system: A549 human cells were intranuclearly injected with plasmids encoding CFTR and beta3-adrenoceptors. CFTR activity was functionally assayed using the 6-methoxy-N-(3-sulfopropyl)quinolinium fluorescent probe and the patch-clamp technique. Injection of CFTR-cDNA alone led to the expression of a functional CFTR protein activated by cAMP or cGMP. Co-expression of CFTR (but not of mutated DeltaF508-CFTR) with high levels of beta3-adrenoceptor produced an increased halide permeability under base-line conditions that was not further sensitive to cAMP or beta3-adrenoceptor stimulation. Patch-clamp experiments confirmed that CFTR channels were permanently activated in cells co-expressing CFTR and a high level of beta3-adrenoceptor. Permanent CFTR activation was not associated with elevated intracellular cAMP or cGMP levels. When the expression level of beta3-adrenoceptor was lowered, CFTR was not activated under base-line conditions but became sensitive to beta3-adrenoceptor stimulation (isoproterenol plus nadolol, SR 58611, or CGP 12177). This later effect was not prevented by protein kinase A inhibitors. Our results provide molecular evidence that CFTR but not mutated DeltaF508-CFTR is regulated by beta3-adrenoceptors expression through a protein kinase A-independent pathway.  (+info)

Comparison of flagellin genes from clinical and environmental Pseudomonas aeruginosa isolates. (6/5500)

Pseudomonas aeruginosa, an important opportunistic pathogen, was isolated from environmental samples and compared to clinically derived strains. While P. aeruginosa was isolated readily from an experimental mushroom-growing unit, it was found only rarely in other environmental samples. A flagellin gene PCR-restriction fragment length polymorphism analysis of the isolates revealed that environmental and clinical P. aeruginosa strains are not readily distinguishable. The variation in the central regions of the flagellin genes of seven of the isolates was investigated further. The strains used included two strains with type a genes (998 bp), four strains with type b genes (1,258 bp), and one strain, K979, with a novel flagellin gene (2,199 bp). The route by which flagellin gene variation has occurred in P. aeruginosa is discussed.  (+info)

Molecular analysis of the cystic fibrosis gene reveals a high frequency of the intron 8 splice variant 5T in Egyptian males with congenital bilateral absence of the vas deferens. (7/5500)

It has previously been shown that defects in the cystic fibrosis transmembrane conductance regulator (CFTR) gene are largely responsible for the condition of congenital bilateral absence of the vas deferens (CBAVD), without associated renal abnormalities, in Caucasian populations. To assess the involvement of the CFTR in CBAVD in a population with presumed low cystic fibrosis (CF) frequency, we have analysed 20 CBAVD males from Egypt for the presence of 12 common Caucasian CFTR mutations and the intron 8 5T splice variant, IVS-5T, known to be a major cause of CBAVD in Caucasian patients. In 16 of the males without associated renal abnormalities only one deltaF508 carrier was identified, but an exceptionally high frequency of the IVS-5T variant was found (14 of 32 alleles or 43.7%), confirming that this variant is involved in many cases of CBAVD, even in populations where CF is rare. CFTR mutations or the IVS-5T variant were found neither in the remaining four patients with associated renal abnormalities nor in the spouses of the 20 CBAVD patients. However, one patient was homozygous for a leucine to proline substitution at amino acid position 541 (L541P) of the CFTR. It is as yet not clear whether this change is involved in CBAVD in this male.  (+info)

Formal analysis of electrogenic sodium, potassium, chloride and bicarbonate transport in mouse colon epithelium. (8/5500)

1. The mammalian colonic epithelium carries out a number of different transporting activities simultaneously, of which more than one is increased following activation with a single agonist. These separate activities can be quantified by solving a set of equations describing these activities, provided some of the dependent variables can be eliminated. Using variations in the experimental conditions, blocking drugs and comparing wild type tissues with those from transgenic animals this has been achieved for electrogenic ion transporting activity of the mouse colon. 2. Basal activity and that following activation with forskolin was measured by short circuit current in isolated mouse colonic epithelia from normal and cystic fibrosis (CF) mice. 3. Using amiloride it is shown that CF colons show increased electrogenic sodium absorption compared to wild type tissues. CF mice had elevated plasma aldosterone, which may be responsible for part or all of the increased sodium absorbtion in CF colons. 4. The derived values for electrogenic chloride secretion and for electrogenic potassium secretion were increased by 13 and 3 fold respectively by forskolin, compared to basal state values for these processes. 5. The loop diuretic, frusemide, completely inhibited electrogenic potassium secretion, but apparently only partially inhibited electrogenic chloride secretion. However, use of bicarbonate-free solutions and acetazolamide reduced the frusemide-resistant current, suggesting that electrogenic bicarbonate secretion accounts for the frusemide-resistant current. 6. It is argued that the use of tissues from transgenic animals is an important adjunct to pharmacological analysis, especially where effects in tissues result in the activation of more than one sort of response.  (+info)

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

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

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

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

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

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

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

In medical terms, fibrosis is defined as:

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

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

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

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

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

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

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

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

Sweat, also known as perspiration, is the fluid secreted by the sweat glands in human skin. It's primarily composed of water, with small amounts of sodium chloride, potassium, and other electrolytes. Sweat helps regulate body temperature through the process of evaporation, where it absorbs heat from the skin as it turns from a liquid to a gas.

There are two types of sweat glands: eccrine and apocrine. Eccrine glands are found all over the body and produce a watery, odorless sweat in response to heat, physical activity, or emotional stress. Apocrine glands, on the other hand, are mainly located in the armpits and groin area and become active during puberty. They produce a thicker, milky fluid that can mix with bacteria on the skin's surface, leading to body odor.

It is important to note that while sweating is essential for maintaining normal body temperature and overall health, excessive sweating or hyperhidrosis can be a medical condition requiring treatment.

Sputum is defined as a mixture of saliva and phlegm that is expelled from the respiratory tract during coughing, sneezing or deep breathing. It can be clear, mucoid, or purulent (containing pus) depending on the underlying cause of the respiratory issue. Examination of sputum can help diagnose various respiratory conditions such as infections, inflammation, or other lung diseases.

'Inbred CFTR mice' refers to a strain of laboratory mice that have been selectively bred to carry a specific genetic mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The CFTR gene provides instructions for making a protein that helps regulate the movement of salt and water in and out of cells.

In humans, mutations in the CFTR gene can lead to cystic fibrosis (CF), a genetic disorder that affects multiple organs, particularly the lungs and digestive system. The most common CF-causing mutation is called ΔF508, which results in the production of a misfolded CFTR protein that does not function properly.

Inbred CFTR mice carry the same ΔF508 mutation as human CF patients and can serve as an important model for studying the disease mechanisms and testing potential therapies. These mice exhibit many of the symptoms seen in human CF, including lung inflammation, mucus accumulation, and digestive problems. By using inbred CFTR mice, researchers can control for genetic background and focus on the effects of the CFTR mutation, providing valuable insights into the pathophysiology of cystic fibrosis.

Burkholderia infections are caused by bacteria belonging to the Burkholderia genus, which includes several species that can cause various types of infection in humans. The most well-known and medically significant species include Burkholderia cepacia complex (Bcc), Burkholderia pseudomallei, and Burkholderia mallei.

1. Burkholderia cepacia Complex (Bcc): These are a group of closely related bacteria that can be found in various environments such as soil, water, and plants. They can cause respiratory infections, particularly in people with weakened immune systems or chronic lung diseases like cystic fibrosis. Bcc infections can be difficult to treat due to their resistance to many antibiotics.

2. Burkholderia pseudomallei: This species is the causative agent of melioidosis, a potentially severe and life-threatening infection endemic in tropical and subtropical regions, particularly in Southeast Asia and northern Australia. The bacteria can be found in contaminated water and soil, and people can get infected through direct contact with contaminated sources, ingestion, or inhalation of the bacteria. Melioidosis symptoms may vary widely, from mild flu-like illness to severe pneumonia, abscesses, and sepsis.

3. Burkholderia mallei: This species is responsible for glanders, a rare but serious disease primarily affecting horses, donkeys, and mules. Human infections are usually associated with occupational exposure to infected animals or their secretions. Glanders can cause severe symptoms such as fever, pneumonia, sepsis, and skin ulcers.

Treatment of Burkholderia infections typically involves the use of specific antibiotics, often in combination therapy, depending on the species and severity of infection. In some cases, surgical intervention may be necessary to drain abscesses or remove infected tissues. Preventive measures include avoiding contact with contaminated sources, practicing good hygiene, and using appropriate personal protective equipment when handling animals or working in high-risk environments.

Burkholderia cepacia is a gram-negative, motile bacillus that is commonly found in the environment, particularly in water and soil. It is a conditional pathogen, meaning it can cause infection in individuals with weakened immune systems or underlying lung conditions such as cystic fibrosis.

Infections caused by B. cepacia can be difficult to treat due to its resistance to many antibiotics. The bacteria can colonize the lungs and cause a chronic respiratory infection that can lead to decline in lung function, increased frequency of exacerbations, and even death in some cases. It is also associated with outbreaks in healthcare settings, particularly in patients receiving respiratory therapy or using contaminated medical equipment.

It's important to note that B. cepacia is not typically considered a community-acquired pathogen and is not commonly associated with typical pneumonia or other respiratory infections in healthy individuals.

Chlorides are simple inorganic ions consisting of a single chlorine atom bonded to a single charged hydrogen ion (H+). Chloride is the most abundant anion (negatively charged ion) in the extracellular fluid in the human body. The normal range for chloride concentration in the blood is typically between 96-106 milliequivalents per liter (mEq/L).

Chlorides play a crucial role in maintaining electrical neutrality, acid-base balance, and osmotic pressure in the body. They are also essential for various physiological processes such as nerve impulse transmission, maintenance of membrane potentials, and digestion (as hydrochloric acid in the stomach).

Chloride levels can be affected by several factors, including diet, hydration status, kidney function, and certain medical conditions. Increased or decreased chloride levels can indicate various disorders, such as dehydration, kidney disease, Addison's disease, or diabetes insipidus. Therefore, monitoring chloride levels is essential for assessing a person's overall health and diagnosing potential medical issues.

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

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

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

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

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

Exocrine pancreatic insufficiency (EPI) is a condition characterized by the reduced ability to digest and absorb nutrients due to a lack of digestive enzymes produced by the exocrine glands in the pancreas. These enzymes, including lipases, amylases, and proteases, are necessary for breaking down fats, carbohydrates, and proteins in food during the digestion process.

When EPI occurs, undigested food passes through the gastrointestinal tract, leading to malabsorption of nutrients, which can result in various symptoms such as abdominal pain, bloating, diarrhea, weight loss, and steatorrhea (fatty stools). EPI is often associated with chronic pancreatitis, cystic fibrosis, pancreatic cancer, or other conditions that damage the exocrine glands in the pancreas.

EPI can be diagnosed through various tests, including fecal elastase testing, fecal fat quantification, and imaging studies to assess the structure and function of the pancreas. Treatment typically involves replacing the missing enzymes with oral supplements taken with meals and snacks to improve digestion and absorption of nutrients. In addition, dietary modifications and management of underlying conditions are essential for optimal outcomes.

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

Chloride channels are membrane proteins that form hydrophilic pores or gaps, allowing the selective passage of chloride ions (Cl-) across the lipid bilayer of cell membranes. They play crucial roles in various physiological processes, including regulation of neuronal excitability, maintenance of resting membrane potential, fluid and electrolyte transport, and pH and volume regulation of cells.

Chloride channels can be categorized into several groups based on their structure, function, and mechanism of activation. Some of the major classes include:

1. Voltage-gated chloride channels (ClC): These channels are activated by changes in membrane potential and have a variety of functions, such as regulating neuronal excitability and transepithelial transport.
2. Ligand-gated chloride channels: These channels are activated by the binding of specific ligands or messenger molecules, like GABA (gamma-aminobutyric acid) or glycine, and are involved in neurotransmission and neuromodulation.
3. Cystic fibrosis transmembrane conductance regulator (CFTR): This is a chloride channel primarily located in the apical membrane of epithelial cells, responsible for secreting chloride ions and water to maintain proper hydration and mucociliary clearance in various organs, including the lungs and pancreas.
4. Calcium-activated chloride channels (CaCCs): These channels are activated by increased intracellular calcium concentrations and participate in various physiological processes, such as smooth muscle contraction, neurotransmitter release, and cell volume regulation.
5. Swelling-activated chloride channels (ClSwells): Also known as volume-regulated anion channels (VRACs), these channels are activated by cell swelling or osmotic stress and help regulate cell volume and ionic homeostasis.

Dysfunction of chloride channels has been implicated in various human diseases, such as cystic fibrosis, myotonia congenita, epilepsy, and certain forms of cancer.

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

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

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

Tobramycin is an aminoglycoside antibiotic used to treat various types of bacterial infections. According to the Medical Subject Headings (MeSH) terminology of the National Library of Medicine (NLM), the medical definition of Tobramycin is:

"A semi-synthetic modification of the aminoglycoside antibiotic, NEOMYCIN, that retains its antimicrobial activity but has less nephrotoxic and neurotoxic side effects. Tobramycin is used in the treatment of serious gram-negative infections, especially Pseudomonas infections in patients with cystic fibrosis."

Tobramycin works by binding to the 30S ribosomal subunit of bacterial cells, inhibiting protein synthesis and ultimately leading to bacterial cell death. It is commonly used to treat severe infections caused by susceptible strains of gram-negative bacteria, including Pseudomonas aeruginosa, Escherichia coli, Klebsiella pneumoniae, Proteus mirabilis, Serratia marcescens, and Enterobacter species.

Tobramycin is available in various formulations, such as injectable solutions, ophthalmic ointments, and inhaled powder for nebulization. The choice of formulation depends on the type and location of the infection being treated. As with any antibiotic, it's essential to use Tobramycin appropriately and under medical supervision to minimize the risk of antibiotic resistance and potential side effects.

Nasal mucosa refers to the mucous membrane that lines the nasal cavity. It is a delicate, moist, and specialized tissue that contains various types of cells including epithelial cells, goblet cells, and glands. The primary function of the nasal mucosa is to warm, humidify, and filter incoming air before it reaches the lungs.

The nasal mucosa produces mucus, which traps dust, allergens, and microorganisms, preventing them from entering the respiratory system. The cilia, tiny hair-like structures on the surface of the epithelial cells, help move the mucus towards the back of the throat, where it can be swallowed or expelled.

The nasal mucosa also contains a rich supply of blood vessels and immune cells that help protect against infections and inflammation. It plays an essential role in the body's defense system by producing antibodies, secreting antimicrobial substances, and initiating local immune responses.

Pancreatin is a mixture of digestive enzymes, including amylase, lipase, and proteases, naturally produced by the pancreas in humans and other mammals. These enzymes aid in the digestion of carbohydrates, fats, and proteins, respectively, in the small intestine. Pancreatin is often used as a replacement therapy for individuals with conditions like cystic fibrosis, chronic pancreatitis, or pancreatectomy, who have impaired pancreatic function and struggle to digest food properly. It can be obtained from animal pancreases, typically from pigs, and is available in various forms such as tablets, capsules, or powders for medical use.

Neonatal screening is a medical procedure in which specific tests are performed on newborn babies within the first few days of life to detect certain congenital or inherited disorders that are not otherwise clinically apparent at birth. These conditions, if left untreated, can lead to serious health problems, developmental delays, or even death.

The primary goal of neonatal screening is to identify affected infants early so that appropriate treatment and management can be initiated as soon as possible, thereby improving their overall prognosis and quality of life. Commonly screened conditions include phenylketonuria (PKU), congenital hypothyroidism, galactosemia, maple syrup urine disease, sickle cell disease, cystic fibrosis, and hearing loss, among others.

Neonatal screening typically involves collecting a small blood sample from the infant's heel (heel stick) or through a dried blood spot card, which is then analyzed using various biochemical, enzymatic, or genetic tests. In some cases, additional tests such as hearing screenings and pulse oximetry for critical congenital heart disease may also be performed.

It's important to note that neonatal screening is not a diagnostic tool but rather an initial step in identifying infants who may be at risk of certain conditions. Positive screening results should always be confirmed with additional diagnostic tests before any treatment decisions are made.

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

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

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

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

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

Symptoms of RPF may include:

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

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

The Burkholderia cepacia complex (Bcc) is a group of closely related bacterial species that are gram-negative, motile, and aerobic. These bacteria are commonly found in various environments such as soil, water, and vegetation. The Bcc organisms are known to be opportunistic pathogens, meaning they primarily cause infections in individuals with compromised immune systems or underlying lung conditions, such as cystic fibrosis (CF) patients.

Bcc infections can lead to a range of clinical manifestations, including pneumonia, bacteremia, and chronic lung colonization. The bacteria are particularly notorious for their high level of antibiotic resistance and their ability to form biofilms, making them difficult to eradicate from the lungs of CF patients. Accurate identification of Bcc species is essential for appropriate treatment and infection control measures.

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

RFTs include several types of tests, such as:

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

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

Pancreatic extracts are preparations that contain digestive enzymes derived from the pancreas. These enzymes, including amylase, lipase, and trypsin, help in the breakdown of carbohydrates, fats, and proteins, respectively, during the digestion process. Pancreatic extracts are often used in medical treatments, such as replacing deficient pancreatic enzymes in individuals with conditions like cystic fibrosis or chronic pancreatitis to improve their nutrient absorption and overall digestive health.

The Respiratory System is a complex network of organs and tissues that work together to facilitate the process of breathing, which involves the intake of oxygen and the elimination of carbon dioxide. This system primarily includes the nose, throat (pharynx), voice box (larynx), windpipe (trachea), bronchi, bronchioles, lungs, and diaphragm.

The nostrils or mouth take in air that travels through the pharynx, larynx, and trachea into the lungs. Within the lungs, the trachea divides into two bronchi, one for each lung, which further divide into smaller tubes called bronchioles. At the end of these bronchioles are tiny air sacs known as alveoli where the exchange of gases occurs. Oxygen from the inhaled air diffuses through the walls of the alveoli into the bloodstream, while carbon dioxide, a waste product, moves from the blood to the alveoli and is exhaled out of the body.

The diaphragm, a large muscle that separates the chest from the abdomen, plays a crucial role in breathing by contracting and relaxing to change the volume of the chest cavity, thereby allowing air to flow in and out of the lungs. Overall, the Respiratory System is essential for maintaining life by providing the body's cells with the oxygen needed for metabolism and removing waste products like carbon dioxide.

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

A mutation is a permanent change in the DNA sequence of an organism's genome. Mutations can occur spontaneously or be caused by environmental factors such as exposure to radiation, chemicals, or viruses. They may have various effects on the organism, ranging from benign to harmful, depending on where they occur and whether they alter the function of essential proteins. In some cases, mutations can increase an individual's susceptibility to certain diseases or disorders, while in others, they may confer a survival advantage. Mutations are the driving force behind evolution, as they introduce new genetic variability into populations, which can then be acted upon by natural selection.

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

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

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

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

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

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

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

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

Mucus is a viscous, slippery secretion produced by the mucous membranes that line various body cavities such as the respiratory and gastrointestinal tracts. It serves to lubricate and protect these surfaces from damage, infection, and foreign particles. Mucus contains water, proteins, salts, and other substances, including antibodies, enzymes, and glycoproteins called mucins that give it its characteristic gel-like consistency.

In the respiratory system, mucus traps inhaled particles such as dust, allergens, and pathogens, preventing them from reaching the lungs. The cilia, tiny hair-like structures lining the airways, move the mucus upward toward the throat, where it can be swallowed or expelled through coughing or sneezing. In the gastrointestinal tract, mucus helps protect the lining of the stomach and intestines from digestive enzymes and other harmful substances.

Excessive production of mucus can occur in various medical conditions such as allergies, respiratory infections, chronic lung diseases, and gastrointestinal disorders, leading to symptoms such as coughing, wheezing, nasal congestion, and diarrhea.

Anti-bacterial agents, also known as antibiotics, are a type of medication used to treat infections caused by bacteria. These agents work by either killing the bacteria or inhibiting their growth and reproduction. There are several different classes of anti-bacterial agents, including penicillins, cephalosporins, fluoroquinolones, macrolides, and tetracyclines, among others. Each class of antibiotic has a specific mechanism of action and is used to treat certain types of bacterial infections. It's important to note that anti-bacterial agents are not effective against viral infections, such as the common cold or flu. Misuse and overuse of antibiotics can lead to antibiotic resistance, which is a significant global health concern.

Heterozygote detection is a method used in genetics to identify individuals who carry one normal and one mutated copy of a gene. These individuals are known as heterozygotes and they do not typically show symptoms of the genetic disorder associated with the mutation, but they can pass the mutated gene on to their offspring, who may then be affected.

Heterozygote detection is often used in genetic counseling and screening programs for recessive disorders such as cystic fibrosis or sickle cell anemia. By identifying heterozygotes, individuals can be informed of their carrier status and the potential risks to their offspring. This information can help them make informed decisions about family planning and reproductive options.

Various methods can be used for heterozygote detection, including polymerase chain reaction (PCR) based tests, DNA sequencing, and genetic linkage analysis. The choice of method depends on the specific gene or mutation being tested, as well as the availability and cost of the testing technology.

Meconium is the first stool passed by a newborn infant, typically within the first 48 hours of life. It is composed of materials ingested during fetal development, including intestinal epithelial cells, lanugo (fine hair), amniotic fluid, mucus, bile, and water. The color of meconium is usually greenish-black, and its consistency can range from a thick paste to a liquid. Meconium staining of the amniotic fluid can occur when the fetus has passed meconium while still in the uterus, which may indicate fetal distress and requires careful medical attention during delivery.

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

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

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

Alginates are a type of polysaccharide derived from brown algae or produced synthetically, which have gelling and thickening properties. In medical context, they are commonly used as a component in wound dressings, dental impressions, and bowel cleansing products. The gels formed by alginates can provide a protective barrier to wounds, help maintain a moist environment, and promote healing. They can also be used to create a mold of the mouth or other body parts in dental and medical applications. In bowel cleansing, sodium alginates are often combined with sodium bicarbonate and water to form a solution that expands and stimulates bowel movements, helping to prepare the colon for procedures such as colonoscopy.

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

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

Pancrelipase is a prescription medication that contains a combination of three enzymes (lipases, proteases, and amylases) that are normally produced by the pancreas. These enzymes help break down fats, proteins, and carbohydrates in food so that they can be absorbed into the intestines.

Pancrelipase is used to replace these enzymes when a person's pancreas is not able to produce enough of them due to conditions such as cystic fibrosis, chronic pancreatitis, or pancreatectomy. By taking pancrelipase with meals and snacks, people with these conditions can improve their digestion and absorption of nutrients from food.

It is important to note that pancrelipase should be taken under the guidance of a healthcare professional, as improper use or dosage can lead to serious side effects such as nausea, vomiting, diarrhea, abdominal pain, and constipation.

The trachea, also known as the windpipe, is a tube-like structure in the respiratory system that connects the larynx (voice box) to the bronchi (the two branches leading to each lung). It is composed of several incomplete rings of cartilage and smooth muscle, which provide support and flexibility. The trachea plays a crucial role in directing incoming air to the lungs during inspiration and outgoing air to the larynx during expiration.

*Achromobacter denitrificans* is a gram-negative, aerobic, rod-shaped bacterium that is commonly found in various environments such as soil, water, and clinical samples. It is known for its ability to denitrify, which means it can convert nitrates to nitrogen gas under anaerobic conditions. This bacterium is generally considered to be non-pathogenic, but there have been rare cases of infections associated with *Achromobacter denitrificans* in immunocompromised individuals.

The medical definition of *Achromobacter denitrificans* would refer to its identification and classification as a bacterium, along with its potential role in certain types of infections. However, it is important to note that this organism is not typically associated with specific diseases or medical conditions, and its presence in clinical samples may simply reflect environmental contamination or colonization rather than active infection.

Mucociliary clearance is a vital defense mechanism of the respiratory system that involves the coordinated movement of tiny hair-like structures called cilia, which are present on the surface of the respiratory epithelium, and the mucus layer. This mechanism helps to trap inhaled particles, microorganisms, and other harmful substances and move them away from the lungs towards the upper airways, where they can be swallowed or coughed out.

The cilia beat in a coordinated manner, moving in a wave-like motion to propel the mucus layer upwards. This continuous movement helps to clear the airways of any debris and maintain a clean and healthy respiratory system. Mucociliary clearance plays an essential role in preventing respiratory infections and maintaining lung function. Any impairment in this mechanism, such as due to smoking or certain respiratory conditions, can increase the risk of respiratory infections and other related health issues.

Burkholderia cenocepacia is a species of gram-negative, motile bacteria that belongs to the family Burkholderiaceae. These bacteria are commonly found in various environments such as soil, water, and plant roots. They are known to form biofilms and can survive under a wide range of conditions, making them difficult to eradicate.

B. cenocepacia is an opportunistic pathogen that can cause serious respiratory infections in individuals with weakened immune systems, particularly those with cystic fibrosis (CF). In CF patients, B. cenocepacia infections can lead to a rapid decline in lung function and are associated with high mortality rates. The bacteria can also cause other types of infections such as bacteremia, wound infections, and urinary tract infections.

B. cenocepacia is resistant to many antibiotics, which makes treatment challenging. Infection control measures, such as contact isolation and rigorous environmental cleaning, are crucial in preventing the spread of B. cenocepacia in healthcare settings.

Sweat glands are specialized tubular structures in the skin that produce and secrete sweat, also known as perspiration. They are part of the body's thermoregulatory system, helping to maintain optimal body temperature by releasing water and heat through evaporation. There are two main types of sweat glands: eccrine and apocrine.

1. Eccrine sweat glands: These are distributed throughout the body, with a higher concentration on areas like the palms, soles, and forehead. They are responsible for producing a watery, odorless sweat that primarily helps to cool down the body through evaporation.

2. Apocrine sweat glands: These are mainly found in the axillary (armpit) region and around the anogenital area. They become active during puberty and produce a thick, milky fluid that does not have a strong odor on its own but can mix with bacteria on the skin's surface, leading to body odor.

Sweat glands are controlled by the autonomic nervous system, meaning they function involuntarily in response to various stimuli such as emotions, physical activity, or changes in environmental temperature.

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

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

Examples of animal disease models include:

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

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

Aminophenols are organic compounds that consist of an amino group (-NH2) attached to a phenol group (aromatic ring with a hydroxyl group, -OH). There are two primary aminophenols: para-aminophenol (PAP) and ortho-aminophenol (OAP), which differ in the position of the amino group on the aromatic ring.

Para-aminophenol (PAP):
Chemical formula: C6H5NOH
IUPAC name: 4-Aminophenol

Ortho-aminophenol (OAP):
Chemical formula: C6H5NOH
IUPAC name: 2-Aminophenol

These compounds have various applications, including pharmaceuticals, dyes, and chemical intermediates. However, they can also be toxic and pose health risks if not handled properly. For instance, PAP is a metabolite of the analgesic drug paracetamol (acetaminophen), and overexposure to it can lead to liver damage.

Allergic bronchopulmonary aspergillosis (ABPA) is a medical condition characterized by an hypersensitivity reaction to the fungus Aspergillus species, most commonly A. fumigatus. It primarily affects the airways and lung tissue. The immune system overreacts to the presence of the fungus, leading to inflammation and damage in the lungs.

The main symptoms of ABPA include wheezing, coughing, production of thick mucus, shortness of breath, and chest tightness. These symptoms are similar to those seen in asthma and other respiratory conditions. Some people with ABPA may also experience fever, weight loss, and fatigue.

Diagnosis of ABPA typically involves a combination of clinical evaluation, imaging studies (such as chest X-rays or CT scans), and laboratory tests (such as blood tests or sputum cultures) to detect the presence of Aspergillus species and elevated levels of certain antibodies.

Treatment for ABPA usually involves a combination of corticosteroids to reduce inflammation and antifungal medications to eradicate the Aspergillus infection. In some cases, immunomodulatory therapies may also be used to help regulate the immune system's response to the fungus.

It is important to note that ABPA can lead to serious complications if left untreated, including bronchiectasis (permanent enlargement of the airways), pulmonary fibrosis (scarring of the lung tissue), and respiratory failure. Therefore, prompt diagnosis and treatment are essential for managing this condition.

Ion transport refers to the active or passive movement of ions, such as sodium (Na+), potassium (K+), chloride (Cl-), and calcium (Ca2+) ions, across cell membranes. This process is essential for various physiological functions, including nerve impulse transmission, muscle contraction, and maintenance of resting membrane potential.

Ion transport can occur through several mechanisms, including:

1. Diffusion: the passive movement of ions down their concentration gradient, from an area of high concentration to an area of low concentration.
2. Facilitated diffusion: the passive movement of ions through specialized channels or transporters in the cell membrane.
3. Active transport: the energy-dependent movement of ions against their concentration gradient, requiring the use of ATP. This process is often mediated by ion pumps, such as the sodium-potassium pump (Na+/K+-ATPase).
4. Co-transport or symport: the coupled transport of two or more different ions or molecules in the same direction, often driven by an electrochemical gradient.
5. Counter-transport or antiport: the coupled transport of two or more different ions or molecules in opposite directions, also often driven by an electrochemical gradient.

Abnormalities in ion transport can lead to various medical conditions, such as cystic fibrosis (which involves defective chloride channel function), hypertension (which may be related to altered sodium transport), and certain forms of heart disease (which can result from abnormal calcium handling).

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

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

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

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

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

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

Respiratory tract infections (RTIs) are infections that affect the respiratory system, which includes the nose, throat (pharynx), voice box (larynx), windpipe (trachea), bronchi, and lungs. These infections can be caused by viruses, bacteria, or, less commonly, fungi.

RTIs are classified into two categories based on their location: upper respiratory tract infections (URTIs) and lower respiratory tract infections (LRTIs). URTIs include infections of the nose, sinuses, throat, and larynx, such as the common cold, flu, laryngitis, and sinusitis. LRTIs involve the lower airways, including the bronchi and lungs, and can be more severe. Examples of LRTIs are pneumonia, bronchitis, and bronchiolitis.

Symptoms of RTIs depend on the location and cause of the infection but may include cough, congestion, runny nose, sore throat, difficulty breathing, wheezing, fever, fatigue, and chest pain. Treatment for RTIs varies depending on the severity and underlying cause of the infection. For viral infections, treatment typically involves supportive care to manage symptoms, while antibiotics may be prescribed for bacterial infections.

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

Genotype, in genetics, refers to the complete heritable genetic makeup of an individual organism, including all of its genes. It is the set of instructions contained in an organism's DNA for the development and function of that organism. The genotype is the basis for an individual's inherited traits, and it can be contrasted with an individual's phenotype, which refers to the observable physical or biochemical characteristics of an organism that result from the expression of its genes in combination with environmental influences.

It is important to note that an individual's genotype is not necessarily identical to their genetic sequence. Some genes have multiple forms called alleles, and an individual may inherit different alleles for a given gene from each parent. The combination of alleles that an individual inherits for a particular gene is known as their genotype for that gene.

Understanding an individual's genotype can provide important information about their susceptibility to certain diseases, their response to drugs and other treatments, and their risk of passing on inherited genetic disorders to their offspring.

Amiloride is a medication that belongs to a class of drugs called potassium-sparing diuretics. It works by preventing the reabsorption of salt and water in the kidneys, which helps to increase urine output and decrease fluid buildup in the body. At the same time, amiloride also helps to preserve the level of potassium in the body, which is why it is known as a potassium-sparing diuretic.

Amiloride is commonly used to treat high blood pressure, heart failure, and edema (fluid buildup) in the body. It is available in tablet form and is typically taken once or twice a day, with or without food. Common side effects of amiloride include headache, dizziness, and stomach upset.

It's important to note that amiloride can interact with other medications, including some over-the-counter products, so it's essential to inform your healthcare provider of all the medications you are taking before starting amiloride therapy. Additionally, regular monitoring of blood pressure, kidney function, and electrolyte levels is necessary while taking this medication.

"Inhalation administration" is a medical term that refers to the method of delivering medications or therapeutic agents directly into the lungs by inhaling them through the airways. This route of administration is commonly used for treating respiratory conditions such as asthma, COPD (chronic obstructive pulmonary disease), and cystic fibrosis.

Inhalation administration can be achieved using various devices, including metered-dose inhalers (MDIs), dry powder inhalers (DPIs), nebulizers, and soft-mist inhalers. Each device has its unique mechanism of delivering the medication into the lungs, but they all aim to provide a high concentration of the drug directly to the site of action while minimizing systemic exposure and side effects.

The advantages of inhalation administration include rapid onset of action, increased local drug concentration, reduced systemic side effects, and improved patient compliance due to the ease of use and non-invasive nature of the delivery method. However, proper technique and device usage are crucial for effective therapy, as incorrect usage may result in suboptimal drug deposition and therapeutic outcomes.

Genetic testing is a type of medical test that identifies changes in chromosomes, genes, or proteins. The results of a genetic test can confirm or rule out a suspected genetic condition or help determine a person's chance of developing or passing on a genetic disorder. Genetic tests are performed on a sample of blood, hair, skin, amniotic fluid (the fluid that surrounds a fetus during pregnancy), or other tissue. For example, a physician may recommend genetic testing to help diagnose a genetic condition, confirm the presence of a gene mutation known to increase the risk of developing certain cancers, or determine the chance for a couple to have a child with a genetic disorder.

There are several types of genetic tests, including:

* Diagnostic testing: This type of test is used to identify or confirm a suspected genetic condition in an individual. It may be performed before birth (prenatal testing) or at any time during a person's life.
* Predictive testing: This type of test is used to determine the likelihood that a person will develop a genetic disorder. It is typically offered to individuals who have a family history of a genetic condition but do not show any symptoms themselves.
* Carrier testing: This type of test is used to determine whether a person carries a gene mutation for a genetic disorder. It is often offered to couples who are planning to have children and have a family history of a genetic condition or belong to a population that has an increased risk of certain genetic disorders.
* Preimplantation genetic testing: This type of test is used in conjunction with in vitro fertilization (IVF) to identify genetic changes in embryos before they are implanted in the uterus. It can help couples who have a family history of a genetic disorder or who are at risk of having a child with a genetic condition to conceive a child who is free of the genetic change in question.
* Pharmacogenetic testing: This type of test is used to determine how an individual's genes may affect their response to certain medications. It can help healthcare providers choose the most effective medication and dosage for a patient, reducing the risk of adverse drug reactions.

It is important to note that genetic testing should be performed under the guidance of a qualified healthcare professional who can interpret the results and provide appropriate counseling and support.

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

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

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

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

Postural drainage is a medical technique that uses gravity to help clear secretions from the airways. It involves positioning the body in various ways to promote the flow of mucus or other fluids from the lungs, sinuses, or middle ear into the upper airway, where they can be more easily cleared by coughing or suctioning. This technique is often used in patients with respiratory conditions such as cystic fibrosis, bronchiectasis, and pneumonia to help improve lung function and reduce the risk of infection. It may also be used in patients with chronic sinusitis or middle ear effusions to promote drainage and relieve symptoms. The specific position used during postural drainage depends on the location of the secretions within the airway and may involve elevating the head, trunk, or legs, or turning the body to one side.

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

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

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

Hexuronic acids are a type of uronic acid that contains six carbon atoms and is commonly found in various biological tissues and polysaccharides, such as pectins, heparin, and certain glycoproteins. The most common hexuronic acids are glucuronic acid and iduronic acid, which are formed from the oxidation of the corresponding hexoses, glucose and galactose, respectively. Hexuronic acids play important roles in various biological processes, including the detoxification and excretion of xenobiotics, the formation of proteoglycans, and the regulation of cell growth and differentiation.

Colistin is an antibiotic that belongs to a class of drugs called polymyxins. It is primarily used to treat infections caused by Gram-negative bacteria, including some that are resistant to other antibiotics. Colistin works by disrupting the bacterial cell membrane and causing the bacterium to lose essential components, leading to its death.

Colistin can be administered intravenously or inhaled, depending on the type of infection being treated. It is important to note that colistin has a narrow therapeutic index, meaning that there is a small difference between the effective dose and the toxic dose. Therefore, it must be used with caution and under the close supervision of a healthcare professional.

Common side effects of colistin include kidney damage, nerve damage, and muscle weakness. It may also cause allergic reactions in some people. Colistin should not be used during pregnancy or breastfeeding unless the benefits outweigh the risks.

Stenotrophomonas maltophilia is a gram-negative, aerobic, non-fermentative bacillus that is commonly found in moist environments such as soil and water. It has emerged as an important nosocomial pathogen, particularly in patients with compromised immune systems or underlying lung diseases.

S. maltophilia can cause a variety of infections, including pneumonia, bacteremia, urinary tract infections, and wound infections. It is inherently resistant to many antibiotics, making it difficult to treat. The bacteria produce biofilms, which can make them even more resistant to antibiotics and host defenses.

Infection with S. maltophilia is associated with high mortality rates, particularly in critically ill patients. Prompt identification and appropriate antimicrobial therapy are essential for the successful management of infections caused by this organism.

Glucuronic acid is a physiological important organic acid, which is a derivative of glucose. It is formed by the oxidation of the primary alcohol group of glucose to form a carboxyl group at the sixth position. Glucuronic acid plays a crucial role in the detoxification process in the body as it conjugates with toxic substances, making them water-soluble and facilitating their excretion through urine or bile. This process is known as glucuronidation. It is also a component of various polysaccharides, such as heparan sulfate and chondroitin sulfate, which are found in the extracellular matrix of connective tissues.

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

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

A chronic disease is a long-term medical condition that often progresses slowly over a period of years and requires ongoing management and care. These diseases are typically not fully curable, but symptoms can be managed to improve quality of life. Common chronic diseases include heart disease, stroke, cancer, diabetes, arthritis, and COPD (chronic obstructive pulmonary disease). They are often associated with advanced age, although they can also affect children and younger adults. Chronic diseases can have significant impacts on individuals' physical, emotional, and social well-being, as well as on healthcare systems and society at large.

Exocrine glands are a type of gland in the human body that produce and release substances through ducts onto an external or internal surface. These glands are responsible for secreting various substances such as enzymes, hormones, and lubricants that help in digestion, protection, and other bodily functions.

Exocrine glands can be further classified into three types based on their mode of secretion:

1. Merocrine glands: These glands release their secretions by exocytosis, where the secretory product is enclosed in a vesicle that fuses with the cell membrane and releases its contents outside the cell. Examples include sweat glands and mucous glands.
2. Apocrine glands: These glands release their secretions by pinching off a portion of the cytoplasm along with the secretory product. An example is the apocrine sweat gland found in the armpits and genital area.
3. Holocrine glands: These glands release their secretions by disintegrating and releasing the entire cell, including its organelles and secretory products. An example is the sebaceous gland found in the skin, which releases an oily substance called sebum.

Molecular sequence data refers to the specific arrangement of molecules, most commonly nucleotides in DNA or RNA, or amino acids in proteins, that make up a biological macromolecule. This data is generated through laboratory techniques such as sequencing, and provides information about the exact order of the constituent molecules. This data is crucial in various fields of biology, including genetics, evolution, and molecular biology, allowing for comparisons between different organisms, identification of genetic variations, and studies of gene function and regulation.

Mucins are high molecular weight, heavily glycosylated proteins that are the major components of mucus. They are produced and secreted by specialized epithelial cells in various organs, including the respiratory, gastrointestinal, and urogenital tracts, as well as the eyes and ears.

Mucins have a characteristic structure consisting of a protein backbone with numerous attached oligosaccharide side chains, which give them their gel-forming properties and provide a protective barrier against pathogens, environmental insults, and digestive enzymes. They also play important roles in lubrication, hydration, and cell signaling.

Mucins can be classified into two main groups based on their structure and function: secreted mucins and membrane-bound mucins. Secreted mucins are released from cells and form a physical barrier on the surface of mucosal tissues, while membrane-bound mucins are integrated into the cell membrane and participate in cell adhesion and signaling processes.

Abnormalities in mucin production or function have been implicated in various diseases, including chronic inflammation, cancer, and cystic fibrosis.

Burkholderia is a genus of gram-negative, rod-shaped bacteria that are widely distributed in the environment, including soil, water, and associated with plants. Some species of Burkholderia are opportunistic pathogens, meaning they can cause infection in individuals with weakened immune systems or underlying medical conditions.

One of the most well-known species of Burkholderia is B. cepacia, which can cause respiratory infections in people with cystic fibrosis and chronic granulomatous disease. Other notable species include B. pseudomallei, the causative agent of melioidosis, a potentially serious infection that primarily affects the respiratory system; and B. mallei, which causes glanders, a rare but severe disease that can affect humans and animals.

Burkholderia species are known for their resistance to many antibiotics, making them difficult to treat in some cases. Proper identification of the specific Burkholderia species involved in an infection is important for determining the most appropriate treatment approach.

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

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

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

Colforsin is a drug that belongs to a class of medications called phosphodiesterase inhibitors. It works by increasing the levels of a chemical called cyclic AMP (cyclic adenosine monophosphate) in the body, which helps to relax and widen blood vessels.

Colforsin is not approved for use in humans in many countries, including the United States. However, it has been used in research settings to study its potential effects on heart function and other physiological processes. In animals, colforsin has been shown to have positive inotropic (contractility-enhancing) and lusitropic (relaxation-enhancing) effects on the heart, making it a potential therapeutic option for heart failure and other cardiovascular conditions.

It is important to note that while colforsin has shown promise in preclinical studies, more research is needed to establish its safety and efficacy in humans. Therefore, it should only be used under the supervision of a qualified healthcare professional and in the context of a clinical trial or research study.

Respiratory therapy is a healthcare profession that specializes in the diagnosis, treatment, and management of respiratory disorders and diseases. Respiratory therapists (RTs) work under the direction of physicians to provide care for patients with conditions such as chronic obstructive pulmonary disease (COPD), asthma, cystic fibrosis, sleep apnea, and neuromuscular diseases that affect breathing.

RTs use a variety of techniques and treatments to help patients breathe more easily, including oxygen therapy, aerosol medication delivery, chest physiotherapy, mechanical ventilation, and patient education. They also perform diagnostic tests such as pulmonary function studies to assess lung function and help diagnose respiratory conditions.

RTs work in a variety of healthcare settings, including hospitals, clinics, long-term care facilities, and home health agencies. They may provide care for patients of all ages, from premature infants to the elderly. The overall goal of respiratory therapy is to help patients achieve and maintain optimal lung function and quality of life.

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

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

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

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

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

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

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

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

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

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

Membrane proteins are a type of protein that are embedded in the lipid bilayer of biological membranes, such as the plasma membrane of cells or the inner membrane of mitochondria. These proteins play crucial roles in various cellular processes, including:

1. Cell-cell recognition and signaling
2. Transport of molecules across the membrane (selective permeability)
3. Enzymatic reactions at the membrane surface
4. Energy transduction and conversion
5. Mechanosensation and signal transduction

Membrane proteins can be classified into two main categories: integral membrane proteins, which are permanently associated with the lipid bilayer, and peripheral membrane proteins, which are temporarily or loosely attached to the membrane surface. Integral membrane proteins can further be divided into three subcategories based on their topology:

1. Transmembrane proteins, which span the entire width of the lipid bilayer with one or more alpha-helices or beta-barrels.
2. Lipid-anchored proteins, which are covalently attached to lipids in the membrane via a glycosylphosphatidylinositol (GPI) anchor or other lipid modifications.
3. Monotopic proteins, which are partially embedded in the membrane and have one or more domains exposed to either side of the bilayer.

Membrane proteins are essential for maintaining cellular homeostasis and are targets for various therapeutic interventions, including drug development and gene therapy. However, their structural complexity and hydrophobicity make them challenging to study using traditional biochemical methods, requiring specialized techniques such as X-ray crystallography, nuclear magnetic resonance (NMR) spectroscopy, and single-particle cryo-electron microscopy (cryo-EM).

A newborn infant is a baby who is within the first 28 days of life. This period is also referred to as the neonatal period. Newborns require specialized care and attention due to their immature bodily systems and increased vulnerability to various health issues. They are closely monitored for signs of well-being, growth, and development during this critical time.

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

Trypsinogen is a precursor protein that is converted into the enzyme trypsin in the small intestine. It is produced by the pancreas and released into the duodenum, where it is activated by enterokinase, an enzyme produced by the intestinal mucosa. Trypsinogen plays a crucial role in digestion by helping to break down proteins into smaller peptides and individual amino acids.

In medical terms, an elevated level of trypsinogen in the blood may indicate pancreatic disease or injury, such as pancreatitis or pancreatic cancer. Therefore, measuring trypsinogen levels in the blood is sometimes used as a diagnostic tool to help identify these conditions.

Polymerase Chain Reaction (PCR) is a laboratory technique used to amplify specific regions of DNA. It enables the production of thousands to millions of copies of a particular DNA sequence in a rapid and efficient manner, making it an essential tool in various fields such as molecular biology, medical diagnostics, forensic science, and research.

The PCR process involves repeated cycles of heating and cooling to separate the DNA strands, allow primers (short sequences of single-stranded DNA) to attach to the target regions, and extend these primers using an enzyme called Taq polymerase, resulting in the exponential amplification of the desired DNA segment.

In a medical context, PCR is often used for detecting and quantifying specific pathogens (viruses, bacteria, fungi, or parasites) in clinical samples, identifying genetic mutations or polymorphisms associated with diseases, monitoring disease progression, and evaluating treatment effectiveness.

Cyclic adenosine monophosphate (cAMP) is a key secondary messenger in many biological processes, including the regulation of metabolism, gene expression, and cellular excitability. It is synthesized from adenosine triphosphate (ATP) by the enzyme adenylyl cyclase and is degraded by the enzyme phosphodiesterase.

In the body, cAMP plays a crucial role in mediating the effects of hormones and neurotransmitters on target cells. For example, when a hormone binds to its receptor on the surface of a cell, it can activate a G protein, which in turn activates adenylyl cyclase to produce cAMP. The increased levels of cAMP then activate various effector proteins, such as protein kinases, which go on to regulate various cellular processes.

Overall, the regulation of cAMP levels is critical for maintaining proper cellular function and homeostasis, and abnormalities in cAMP signaling have been implicated in a variety of diseases, including cancer, diabetes, and neurological disorders.

Bicarbonates, also known as sodium bicarbonate or baking soda, is a chemical compound with the formula NaHCO3. In the context of medical definitions, bicarbonates refer to the bicarbonate ion (HCO3-), which is an important buffer in the body that helps maintain normal pH levels in blood and other bodily fluids.

The balance of bicarbonate and carbonic acid in the body helps regulate the acidity or alkalinity of the blood, a condition known as pH balance. Bicarbonates are produced by the body and are also found in some foods and drinking water. They work to neutralize excess acid in the body and help maintain the normal pH range of 7.35 to 7.45.

In medical testing, bicarbonate levels may be measured as part of an electrolyte panel or as a component of arterial blood gas (ABG) analysis. Low bicarbonate levels can indicate metabolic acidosis, while high levels can indicate metabolic alkalosis. Both conditions can have serious consequences if not treated promptly and appropriately.

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

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

Genetic counseling is a process of communication and education between a healthcare professional and an individual or family, aimed at understanding, adapting to, and managing the medical, psychological, and familial implications of genetic contributions to disease. This includes providing information about the risk of inherited conditions, explaining the implications of test results, discussing reproductive options, and offering support and resources for coping with a genetic condition. Genetic counselors are trained healthcare professionals who specialize in helping people understand genetic information and its impact on their health and lives.

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

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

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

A heterozygote is an individual who has inherited two different alleles (versions) of a particular gene, one from each parent. This means that the individual's genotype for that gene contains both a dominant and a recessive allele. The dominant allele will be expressed phenotypically (outwardly visible), while the recessive allele may or may not have any effect on the individual's observable traits, depending on the specific gene and its function. Heterozygotes are often represented as 'Aa', where 'A' is the dominant allele and 'a' is the recessive allele.

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

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

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

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

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

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

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

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

Bronchopneumonia is a type of pneumonia that involves inflammation and infection of the bronchioles (small airways in the lungs) and alveoli (tiny air sacs in the lungs). It can be caused by various bacteria, viruses, or fungi and often occurs as a complication of a respiratory tract infection.

The symptoms of bronchopneumonia may include cough, chest pain, fever, chills, shortness of breath, and fatigue. In severe cases, it can lead to complications such as respiratory failure or sepsis. Treatment typically involves antibiotics for bacterial infections, antiviral medications for viral infections, and supportive care such as oxygen therapy and hydration.

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

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

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

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

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

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

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

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

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

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

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

Heart-lung transplantation is a surgical procedure where both the heart and lungs of a patient are replaced with those from a deceased donor. This complex and highly specialized surgery is typically considered as a last resort for patients suffering from end-stage lung or heart-lung diseases, such as cystic fibrosis, pulmonary fibrosis, chronic obstructive pulmonary disease (COPD), or certain forms of congenital heart disease, who have exhausted all other treatment options and face imminent death.

The procedure involves removing the patient's diseased heart and lungs en bloc, followed by implanting the donor's heart and lungs in their place. The surgery requires a skilled multidisciplinary team of cardiothoracic surgeons, anesthesiologists, perfusionists, transplant coordinators, and intensive care specialists.

Following the transplantation, patients require lifelong immunosuppressive therapy to prevent rejection of the transplanted organs. Despite the significant risks associated with this procedure, including infection, bleeding, and rejection, heart-lung transplantation can significantly improve both survival and quality of life for carefully selected patients with advanced heart-lung disease.

The pancreas is a glandular organ located in the abdomen, posterior to the stomach. It has both exocrine and endocrine functions. The exocrine portion of the pancreas consists of acinar cells that produce and secrete digestive enzymes into the duodenum via the pancreatic duct. These enzymes help in the breakdown of proteins, carbohydrates, and fats in food.

The endocrine portion of the pancreas consists of clusters of cells called islets of Langerhans, which include alpha, beta, delta, and F cells. These cells produce and secrete hormones directly into the bloodstream, including insulin, glucagon, somatostatin, and pancreatic polypeptide. Insulin and glucagon are critical regulators of blood sugar levels, with insulin promoting glucose uptake and storage in tissues and glucagon stimulating glycogenolysis and gluconeogenesis to raise blood glucose when it is low.

A homozygote is an individual who has inherited the same allele (version of a gene) from both parents and therefore possesses two identical copies of that allele at a specific genetic locus. This can result in either having two dominant alleles (homozygous dominant) or two recessive alleles (homozygous recessive). In contrast, a heterozygote has inherited different alleles from each parent for a particular gene.

The term "homozygote" is used in genetics to describe the genetic makeup of an individual at a specific locus on their chromosomes. Homozygosity can play a significant role in determining an individual's phenotype (observable traits), as having two identical alleles can strengthen the expression of certain characteristics compared to having just one dominant and one recessive allele.

Nebulizer: A nebulizer is a medical device that delivers medication in the form of a mist to the respiratory system. It is often used for people who have difficulty inhaling medication through traditional inhalers, such as young children or individuals with severe respiratory conditions. The medication is placed in the nebulizer cup and then converted into a fine mist by the machine. This allows the user to breathe in the medication directly through a mouthpiece or mask.

Vaporizer: A vaporizer, on the other hand, is a device that heats up a liquid, often water or essential oils, to produce steam or vapor. While some people use vaporizers for therapeutic purposes, such as to help relieve congestion or cough, it is important to note that vaporizers are not considered medical devices and their effectiveness for these purposes is not well-established.

It's worth noting that nebulizers and vaporizers are different from each other in terms of their purpose and usage. Nebulizers are used specifically for delivering medication, while vaporizers are used to produce steam or vapor, often for non-medical purposes.

Bacterial pneumonia is a type of lung infection that's caused by bacteria. It can affect people of any age, but it's more common in older adults, young children, and people with certain health conditions or weakened immune systems. The symptoms of bacterial pneumonia can vary, but they often include cough, chest pain, fever, chills, and difficulty breathing.

The most common type of bacteria that causes pneumonia is Streptococcus pneumoniae (pneumococcus). Other types of bacteria that can cause pneumonia include Haemophilus influenzae, Staphylococcus aureus, and Mycoplasma pneumoniae.

Bacterial pneumonia is usually treated with antibiotics, which are medications that kill bacteria. The specific type of antibiotic used will depend on the type of bacteria causing the infection. It's important to take all of the prescribed medication as directed, even if you start feeling better, to ensure that the infection is completely cleared and to prevent the development of antibiotic resistance.

In severe cases of bacterial pneumonia, hospitalization may be necessary for close monitoring and treatment with intravenous antibiotics and other supportive care.

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

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

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

A breath test is a medical or forensic procedure used to analyze a sample of exhaled breath in order to detect and measure the presence of various substances, most commonly alcohol. The test is typically conducted using a device called a breathalyzer, which measures the amount of alcohol in the breath and converts it into a reading of blood alcohol concentration (BAC).

In addition to alcohol, breath tests can also be used to detect other substances such as drugs or volatile organic compounds (VOCs) that may indicate certain medical conditions. However, these types of breath tests are less common and may not be as reliable or accurate as other diagnostic tests.

Breath testing is commonly used by law enforcement officers to determine whether a driver is impaired by alcohol and to establish probable cause for arrest. It is also used in some healthcare settings to monitor patients who are being treated for alcohol abuse or dependence.

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

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

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

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

Gram-negative bacterial infections refer to illnesses or diseases caused by Gram-negative bacteria, which are a group of bacteria that do not retain crystal violet dye during the Gram staining procedure used in microbiology. This characteristic is due to the structure of their cell walls, which contain a thin layer of peptidoglycan and an outer membrane composed of lipopolysaccharides (LPS), proteins, and phospholipids.

The LPS component of the outer membrane is responsible for the endotoxic properties of Gram-negative bacteria, which can lead to severe inflammatory responses in the host. Common Gram-negative bacterial pathogens include Escherichia coli (E. coli), Klebsiella pneumoniae, Pseudomonas aeruginosa, Acinetobacter baumannii, and Proteus mirabilis, among others.

Gram-negative bacterial infections can cause a wide range of clinical syndromes, such as pneumonia, urinary tract infections, bloodstream infections, meningitis, and soft tissue infections. The severity of these infections can vary from mild to life-threatening, depending on the patient's immune status, the site of infection, and the virulence of the bacterial strain.

Effective antibiotic therapy is crucial for treating Gram-negative bacterial infections, but the increasing prevalence of multidrug-resistant strains has become a significant global health concern. Therefore, accurate diagnosis and appropriate antimicrobial stewardship are essential to ensure optimal patient outcomes and prevent further spread of resistance.

The vas deferens is a muscular tube that carries sperm from the epididymis to the urethra during ejaculation in males. It is a part of the male reproductive system and is often targeted in surgical procedures like vasectomy, which is a form of permanent birth control.

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

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

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

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

Interleukin-8 (IL-8) is a type of cytokine, which is a small signaling protein involved in immune response and inflammation. IL-8 is also known as neutrophil chemotactic factor or NCF because it attracts neutrophils, a type of white blood cell, to the site of infection or injury.

IL-8 is produced by various cells including macrophages, epithelial cells, and endothelial cells in response to bacterial or inflammatory stimuli. It acts by binding to specific receptors called CXCR1 and CXCR2 on the surface of neutrophils, which triggers a series of intracellular signaling events leading to neutrophil activation, migration, and degranulation.

IL-8 plays an important role in the recruitment of neutrophils to the site of infection or tissue damage, where they can phagocytose and destroy invading microorganisms. However, excessive or prolonged production of IL-8 has been implicated in various inflammatory diseases such as chronic obstructive pulmonary disease (COPD), rheumatoid arthritis, and cancer.

Ileus is a condition characterized by a lack of intestinal motility or paralysis of the bowel, leading to obstruction of the digestive tract. It is not caused by a physical blockage but rather by a disruption of the normal muscular contractions (peristalsis) that move food through the intestines. This can result in abdominal distention, vomiting, and absence of bowel movements or gas passage. Ileus can be a complication of various surgical procedures, intra-abdominal infections, or other medical conditions. It is essential to diagnose and treat ileus promptly to prevent further complications such as tissue damage, sepsis, or even death.

Prenatal diagnosis is the medical testing of fetuses, embryos, or pregnant women to detect the presence or absence of certain genetic disorders or birth defects. These tests can be performed through various methods such as chorionic villus sampling (CVS), amniocentesis, or ultrasound. The goal of prenatal diagnosis is to provide early information about the health of the fetus so that parents and healthcare providers can make informed decisions about pregnancy management and newborn care. It allows for early intervention, treatment, or planning for the child's needs after birth.

An anion is an ion that has a negative electrical charge because it has more electrons than protons. The term "anion" is derived from the Greek word "anion," which means "to go up" or "to move upward." This name reflects the fact that anions are attracted to positively charged electrodes, or anodes, and will move toward them during electrolysis.

Anions can be formed when a neutral atom or molecule gains one or more extra electrons. For example, if a chlorine atom gains an electron, it becomes a chloride anion (Cl-). Anions are important in many chemical reactions and processes, including the conduction of electricity through solutions and the formation of salts.

In medicine, anions may be relevant in certain physiological processes, such as acid-base balance. For example, the concentration of anions such as bicarbonate (HCO3-) and chloride (Cl-) in the blood can affect the pH of the body fluids and help maintain normal acid-base balance. Abnormal levels of anions may indicate the presence of certain medical conditions, such as metabolic acidosis or alkalosis.

The liver is a large, solid organ located in the upper right portion of the abdomen, beneath the diaphragm and above the stomach. It plays a vital role in several bodily functions, including:

1. Metabolism: The liver helps to metabolize carbohydrates, fats, and proteins from the food we eat into energy and nutrients that our bodies can use.
2. Detoxification: The liver detoxifies harmful substances in the body by breaking them down into less toxic forms or excreting them through bile.
3. Synthesis: The liver synthesizes important proteins, such as albumin and clotting factors, that are necessary for proper bodily function.
4. Storage: The liver stores glucose, vitamins, and minerals that can be released when the body needs them.
5. Bile production: The liver produces bile, a digestive juice that helps to break down fats in the small intestine.
6. Immune function: The liver plays a role in the immune system by filtering out bacteria and other harmful substances from the blood.

Overall, the liver is an essential organ that plays a critical role in maintaining overall health and well-being.

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

Epithelial Sodium Channels (ENaC) are a type of ion channel found in the epithelial cells that line the surface of many types of tissues, including the airways, kidneys, and colon. These channels play a crucial role in regulating sodium and fluid balance in the body by allowing the passive movement of sodium ions (Na+) from the lumen or outside of the cell to the inside of the cell, following their electrochemical gradient.

ENaC is composed of three subunits, alpha, beta, and gamma, which are encoded by different genes. The channel is normally closed and opens in response to various stimuli, such as hormones, neurotransmitters, or changes in osmolarity. Once open, the channel allows sodium ions to flow through, creating a positive charge that can attract chloride ions (Cl-) and water molecules, leading to fluid absorption.

In the kidneys, ENaC plays an essential role in regulating sodium reabsorption in the distal nephron, which helps maintain blood pressure and volume. In the airways, ENaC is involved in controlling the hydration of the airway surface liquid, which is necessary for normal mucociliary clearance. Dysregulation of ENaC has been implicated in several diseases, including hypertension, cystic fibrosis, and chronic obstructive pulmonary disease (COPD).

Intestinal obstruction, also known as bowel obstruction, is a medical condition characterized by a blockage that prevents the normal flow of contents through the small intestine or large intestine (colon). This blockage can be caused by various factors such as tumors, adhesions (scar tissue), hernias, inflammation, or impacted feces.

The obstruction can be mechanical, where something physically blocks the intestinal lumen, or functional, where the normal muscular contractions of the bowel are impaired. Mechanical obstructions are more common than functional ones.

Symptoms of intestinal obstruction may include abdominal pain and cramping, nausea and vomiting, bloating, inability to pass gas or have a bowel movement, and abdominal distention. If left untreated, intestinal obstruction can lead to serious complications such as tissue death (necrosis), perforation of the intestine, and sepsis. Treatment typically involves hospitalization, intravenous fluids, nasogastric decompression, and possibly surgery to remove the obstruction.

Aerosols are defined in the medical field as suspensions of fine solid or liquid particles in a gas. In the context of public health and medicine, aerosols often refer to particles that can remain suspended in air for long periods of time and can be inhaled. They can contain various substances, such as viruses, bacteria, fungi, or chemicals, and can play a role in the transmission of respiratory infections or other health effects.

For example, when an infected person coughs or sneezes, they may produce respiratory droplets that can contain viruses like influenza or SARS-CoV-2 (the virus that causes COVID-19). Some of these droplets can evaporate quickly and leave behind smaller particles called aerosols, which can remain suspended in the air for hours and potentially be inhaled by others. This is one way that respiratory viruses can spread between people in close proximity to each other.

Aerosols can also be generated through medical procedures such as bronchoscopy, suctioning, or nebulizer treatments, which can produce aerosols containing bacteria, viruses, or other particles that may pose an infection risk to healthcare workers or other patients. Therefore, appropriate personal protective equipment (PPE) and airborne precautions are often necessary to reduce the risk of transmission in these settings.

Iodides are chemical compounds that contain iodine in the form of an iodide ion (I-). Iodide ions are negatively charged ions that consist of one iodine atom and an extra electron. Iodides are commonly found in dietary supplements and medications, and they are often used to treat or prevent iodine deficiency. They can also be used as expectorants to help thin and loosen mucus in the respiratory tract. Examples of iodides include potassium iodide (KI) and sodium iodide (NaI).

Nutritional status is a concept that refers to the condition of an individual in relation to their nutrient intake, absorption, metabolism, and excretion. It encompasses various aspects such as body weight, muscle mass, fat distribution, presence of any deficiencies or excesses of specific nutrients, and overall health status.

A comprehensive assessment of nutritional status typically includes a review of dietary intake, anthropometric measurements (such as height, weight, waist circumference, blood pressure), laboratory tests (such as serum albumin, total protein, cholesterol levels, vitamin and mineral levels), and clinical evaluation for signs of malnutrition or overnutrition.

Malnutrition can result from inadequate intake or absorption of nutrients, increased nutrient requirements due to illness or injury, or excessive loss of nutrients due to medical conditions. On the other hand, overnutrition can lead to obesity and related health problems such as diabetes, cardiovascular disease, and certain types of cancer.

Therefore, maintaining a good nutritional status is essential for overall health and well-being, and it is an important consideration in the prevention, diagnosis, and treatment of various medical conditions.

Ortho-Aminobenzoates are chemical compounds that contain a benzene ring substituted with an amino group in the ortho position and an ester group in the form of a benzoate. They are often used as pharmaceutical intermediates, plastic additives, and UV stabilizers. In medical contexts, one specific ortho-aminobenzoate, para-aminosalicylic acid (PABA), is an antibiotic used in the treatment of tuberculosis. However, it's important to note that "ortho-aminobenzoates" in general do not have a specific medical definition and can refer to any compound with this particular substitution pattern on a benzene ring.

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

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

A base sequence in the context of molecular biology refers to the specific order of nucleotides in a DNA or RNA molecule. In DNA, these nucleotides are adenine (A), guanine (G), cytosine (C), and thymine (T). In RNA, uracil (U) takes the place of thymine. The base sequence contains genetic information that is transcribed into RNA and ultimately translated into proteins. It is the exact order of these bases that determines the genetic code and thus the function of the DNA or RNA molecule.

Opportunistic infections (OIs) are infections that occur more frequently or are more severe in individuals with weakened immune systems, often due to a underlying condition such as HIV/AIDS, cancer, or organ transplantation. These infections are caused by microorganisms that do not normally cause disease in people with healthy immune function, but can take advantage of an opportunity to infect and cause damage when the body's defense mechanisms are compromised. Examples of opportunistic infections include Pneumocystis pneumonia, tuberculosis, candidiasis (thrush), and cytomegalovirus infection. Preventive measures, such as antimicrobial medications and vaccinations, play a crucial role in reducing the risk of opportunistic infections in individuals with weakened immune systems.

Biofilms are defined as complex communities of microorganisms, such as bacteria and fungi, that adhere to surfaces and are enclosed in a matrix made up of extracellular polymeric substances (EPS). The EPS matrix is composed of polysaccharides, proteins, DNA, and other molecules that provide structural support and protection to the microorganisms within.

Biofilms can form on both living and non-living surfaces, including medical devices, implants, and biological tissues. They are resistant to antibiotics, disinfectants, and host immune responses, making them difficult to eradicate and a significant cause of persistent infections. Biofilms have been implicated in a wide range of medical conditions, including chronic wounds, urinary tract infections, middle ear infections, and device-related infections.

The formation of biofilms typically involves several stages, including initial attachment, microcolony formation, maturation, and dispersion. Understanding the mechanisms underlying biofilm formation and development is crucial for developing effective strategies to prevent and treat biofilm-associated infections.

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

Adenosine Triphosphate (ATP) is a high-energy molecule that stores and transports energy within cells. It is the main source of energy for most cellular processes, including muscle contraction, nerve impulse transmission, and protein synthesis. ATP is composed of a base (adenine), a sugar (ribose), and three phosphate groups. The bonds between these phosphate groups contain a significant amount of energy, which can be released when the bond between the second and third phosphate group is broken, resulting in the formation of adenosine diphosphate (ADP) and inorganic phosphate. This process is known as hydrolysis and can be catalyzed by various enzymes to drive a wide range of cellular functions. ATP can also be regenerated from ADP through various metabolic pathways, such as oxidative phosphorylation or substrate-level phosphorylation, allowing for the continuous supply of energy to cells.

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

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

A kidney, in medical terms, is one of two bean-shaped organs located in the lower back region of the body. They are essential for maintaining homeostasis within the body by performing several crucial functions such as:

1. Regulation of water and electrolyte balance: Kidneys help regulate the amount of water and various electrolytes like sodium, potassium, and calcium in the bloodstream to maintain a stable internal environment.

2. Excretion of waste products: They filter waste products from the blood, including urea (a byproduct of protein metabolism), creatinine (a breakdown product of muscle tissue), and other harmful substances that result from normal cellular functions or external sources like medications and toxins.

3. Endocrine function: Kidneys produce several hormones with important roles in the body, such as erythropoietin (stimulates red blood cell production), renin (regulates blood pressure), and calcitriol (activated form of vitamin D that helps regulate calcium homeostasis).

4. pH balance regulation: Kidneys maintain the proper acid-base balance in the body by excreting either hydrogen ions or bicarbonate ions, depending on whether the blood is too acidic or too alkaline.

5. Blood pressure control: The kidneys play a significant role in regulating blood pressure through the renin-angiotensin-aldosterone system (RAAS), which constricts blood vessels and promotes sodium and water retention to increase blood volume and, consequently, blood pressure.

Anatomically, each kidney is approximately 10-12 cm long, 5-7 cm wide, and 3 cm thick, with a weight of about 120-170 grams. They are surrounded by a protective layer of fat and connected to the urinary system through the renal pelvis, ureters, bladder, and urethra.

Spirometry is a common type of pulmonary function test (PFT) that measures how well your lungs work. This is done by measuring how much air you can exhale from your lungs after taking a deep breath, and how quickly you can exhale it. The results are compared to normal values for your age, height, sex, and ethnicity.

Spirometry is used to diagnose and monitor certain lung conditions, such as asthma, chronic obstructive pulmonary disease (COPD), and other respiratory diseases that cause narrowing of the airways. It can also be used to assess the effectiveness of treatment for these conditions. The test is non-invasive, safe, and easy to perform.

Nasal polyps are benign (noncancerous) growths that originate from the lining of your nasal passages or sinuses. They most often occur in the area where the sinuses open into the nasal cavity. Small nasal polyps may not cause any problems. But if they grow large enough, they can block your nasal passages and lead to breathing issues, frequent infections and loss of smell.

Nasal polyps are associated with chronic inflammation due to conditions such as asthma, allergic rhinitis or chronic sinusitis. Treatment typically includes medication to reduce the size of the polyps or surgery to remove them. Even after successful treatment, nasal polyps often return.

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

Search GeneCards for genes involved in cystic fibrosis Cystic Fibrosis Mutation Database "Cystic Fibrosis". MedlinePlus. U.S. ... The name "cystic fibrosis" refers to the characteristic fibrosis and cysts that form within the pancreas. Cystic fibrosis ... The Cystic Fibrosis Foundation recommends a diagnosis of cystic fibrosis for anyone suspected of cystic fibrosis (positive ... "Orphanet: Cystic fibrosis". Retrieved 29 July 2022. Hodson M, Geddes D, Bush A, eds. (2012). Cystic Fibrosis ( ...
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- ...
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. Archived from the original on July 3, 2015. Retrieved ... "Board of Trustees". Cystic Fibrosis Foundation. Retrieved May 6, 2018. "Advisory Council". Cystic Fibrosis Foundation. ... Henderson, Wendy (July 5, 2017). "The Danger of Cross Infections for Those Living With Cystic Fibrosis - Cystic Fibrosis News ...
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 ... Since 1997, the Cystic Fibrosis Trust has invested over £10 million in the NHS to improve clinical care for the 8,000 people in ...
Cystic fibrosis (CF) is an autosomal recessive and monogenetic disorder. It is caused by mutations in the cystic fibrosis ... "About Cystic Fibrosis". Cystic Fibrosis Foundation. Retrieved 2022-11-18. Roehmel, Jobst F.; Kallinich, Tilmann; Staab, Doris; ... "CF Foundation Continues Working Toward Equity and Timeliness in Cystic Fibrosis Newborn Screening". Cystic Fibrosis Foundation ... the Cystic Fibrosis Foundation is currently[when?] striving for more equity and timeliness in cystic fibrosis newborn screening ...
... (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: ... and prognostic implications of cystic fibrosis-related diabetes: a technical review". Diabetes Care. 33 (12): 2677-83. doi: ...
... 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 ... Cystic Fibrosis (CF, Mucoviscidosis) and Congenital Absence of the Vas Deferens (CAVD) The Cystic Fibrosis Transmembrane ... "Cystic fibrosis transmembrane conductance regulator and the etiology and pathogenesis of cystic fibrosis". FASEB Journal. 6 (10 ... "Relationships between cystic fibrosis transmembrane conductance regulator, extracellular nucleotides and cystic fibrosis". ...
Cystic-L Cystic Fibrosis Canada Cystic Fibrosis Foundation Cystic Fibrosis Lifestyle Foundation Archived 2008-02-25 at the ... 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". Retrieved 2019-09-05. ... at the Wayback Machine Nederlandse Cystic Fibrosis Stichting Cystic Fibrosis Trust Deutsche CF-Hilfe European Cystic Fibrosis ...
"Actor Anton Yelchin, killed in an accident as he was about to talk publicly about his cystic fibrosis, celebrated in intimate ... The following notable people have or had cystic fibrosis. "'Real World: San Diego' Alum Frankie Abernathy Dead At 25". MTV. ... Robinson, Georgina (June 10, 2014). "Cystic fibrosis no barrier for Nathan Charles as he prepares for Test debut". Rugby Heaven ... "Lisa Bentley Triumphs Over CF". Canadian Cystic Fibrosis Foundation. July 7, 2004. Archived from the original on September 27, ...
... as is the case for cystic fibrosis carriers. If two cystic fibrosis carriers have children, they have a 25 percent chance of ... Ratjen, Felix; Döring, Gerd (February 2003). "Cystic fibrosis". Lancet. 361 (9358): 681-689. doi:10.1016/S0140-6736(03)12567-6 ...
"Cystic Fibrosis Canada". 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 is an inherited (genetic) disease that can present with symptoms within the first two years of life. The ... This predisposes patients with cystic fibrosis to repeated episodes of respiratory infection in the form of pneumonia or ... Shteinberg M, Haq IJ, Polineni D, Davies JC (June 2021). "Cystic fibrosis". The Lancet. 397 (10290): 2195-2211. doi:10.1016/ ... Common chronic illnesses in children include asthma, diabetes, cystic fibrosis, obesity and overweight, malnutrition, ...
... cystic fibrosis, pulmonary hypertension and idiopathic pulmonary fibrosis. Methods for the preservation of donor lungs are ... Cystic fibrosis is a genetic disease, caused by a mutation that results in defects in movement of salt and water in and out of ... "Cystic fibrosis". 2017-10-23. Retrieved 2023-03-28. CDC (2019-12-03). "Pulmonary Hypertension ,". Centers for ... Idiopathic pulmonary fibrosis causes the lungs to become scarred which results in a difficulty in breathing. The causes are of ...
"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 ...
Negulescu's research focuses on the therapy of cystic fibrosis. Cystic fibrosis can be caused by any of the thousands of ... Negulescu also led the discovery of another cystic fibrosis drug, lumacaftor, which is known as a "corrector" as it acts as a ... Ivacaftor was approved by the Food and Drug Administration (FDA) for cystic fibrosis patients with one specific Class III ... Morgan, David (May 13, 2015). "FDA panel recommends Vertex cystic fibrosis treatment". Reuters. Archived from the original on ...
High levels are seen in acute pancreatitis and cystic fibrosis. Three isoforms of trypsinogens may be found in human pancreatic ... Dickinson KM, Collaco JM (February 2021). "Cystic Fibrosis". Pediatr Rev. 42 (2): 55-67. doi:10.1542/pir.2019-0212. PMC 8972143 ...
Cystic fibrosis (CF), also known as mucoviscidosis, is a genetic disorder that affects mostly the lungs, but also the pancreas ... Major advances over the past few years in the management of cystic fibrosis (CF) have resulted in dramatic improvements in ... Prasad, SA; Tannenbaum, EL; Mikelsons, C (2000). "Physiotherapy in cystic fibrosis". J R Soc Med. 93 Suppl 38 (Suppl 38): 27-36 ... Miller, S; Hall, DO; Clayton, CB; Nelson, R (1995). "Chest physiotherapy in cystic fibrosis; a comparative study of autogenic ...
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 ...
In people with cystic fibrosis, evidence is insufficient about the effectiveness of long-term antibiotic treatment with ... It was first isolated in patients with cystic fibrosis (CF) in 1977, when it was known as Pseudomonas cepacia. In the 1980s, ... ISBN 978-0-19-856925-1. "Cystic Fibrosis". Mandell, Douglas, and Bennett's principles and practice of infectious diseases. John ... such as cystic fibrosis or chronic granulomatous disease). Patients with sickle-cell haemoglobinopathies are also at risk. The ...
Cystic Fibrosis. Among the thousands of ABC transporters, one member, the cystic fibrosis transmembrane conductance regulator ( ... Mutation of the CFTR gene causes cystic fibrosis (CF), a lethal disease with a prevalence of 1 in 2500 in Caucasian populations ... Nature 529 (7587), 537-540 Atomic structure of the cystic fibrosis transmembrane conductance regulator. Z Zhang, J Chen. Cell ... cystic fibrosis; and bacterial nutrition. She has also been using crystallography to visualize how the maltose transporter ...
In some cases, mutations may result in genetic diseases, such as Cystic Fibrosis, which is the result of a mutation to the CFTR ... "cystic fibrosis". Genetics Home Reference. Retrieved 16 November 2016. "What is a genetic mutation and how do mutations occur ... cystic fibrosis, and more. DNA profiling, whereby a DNA fingerprint is constructed by extracting a DNA sample from body tissue ...
Cystic fibrosis, diarrhea, liver disease, anemia or iron deficiency, Crohn's disease, and coeliac disease make it more ... Davies, Jane C; Alton, Eric W F W; Bush, Andrew (2007-12-15). "Cystic fibrosis". BMJ: British Medical Journal. 335 (7632): 1255 ... Disorders that cause difficulties absorbing or digesting nutrients, such as Crohn's disease, cystic fibrosis, or celiac disease ... and a sweat chloride test can be used to screen for cystic fibrosis. If no cause is discovered, a stool examination could be ...
2004). Cystic Fibrosis. Wellcome Witnesses to Contemporary Medicine. History of Modern Biomedicine Research Group. ISBN 978-0- ... He also chaired working parties on cystic fibrosis for the World Health Organization. Dodge was appointed Commander of the ... Dodge was chair of the Scientific and Medical Advisory Committees of the International Cystic Fibrosis (Mucoviscidosis) ... specialising in cystic fibrosis. After his retirement in 1997, he became Emeritus Professor of Child Health at the Queen's ...
"Achromobacter xylosoxidans". Cystic Fibrosis Medicine. Retrieved 11 November 2015. Jakobsen, T.H.; Hansen, M.A.; Jensen, P.Ø.; ... In 2013, the complete genome of an A. xylosoxidans strain from a patient with cystic fibrosis was sequenced. A. xylosoxidans is ... 22 July 2013). "Complete Genome Sequence of the Cystic Fibrosis Pathogen Achromobacter xylosoxidans NH44784-1996 Complies with ... Achromobacter xylosoxidans can cause infections such as bacteremia, especially in patients with cystic fibrosis. ...
CTH Cystic fibrosis; 219700; CFTR Cystinosis, late-onset juvenile or adolescent nephropathic; 219900; CTNS Cystinosis, ... PHOX2A Fibrosis of extraocular muscles, congenital, 3A; 600638; TUBB3 Fibrosis of extraocular muscles, congenital, 3B; 135700; ... ANTXR2 Fibrosis of extraocular muscles, congenital, 1; 135700; KIF21A Fibrosis of extraocular muscles, congenital, 2; 602078; ... CC2D2A Medullary cystic kidney disease 2; 603860; UMOD Medullary thyroid carcinoma; 155240; RET Medullary thyroid carcinoma, ...
"Cystic Fibrosis Foundation". Archived from the original on 2007-09-28. Retrieved 2007-05-22. "Sierra's Haven". Retrieved 2007- ... Community & school-related service activities: American Red Cross blood drives, CF (cystic fibrosis) walks, health fairs, ...
She has also worked in support of charities, in particular the Cystic Fibrosis Trust, of which she is a patron (she is also a ... She has been involved in raising awareness of the illness cystic fibrosis, which she believes was responsible for the deaths of ... Bowdler, Neil (25 June 2010). "Jenny Agutter: 'Cystic fibrosis is in my family'". BBC News. BBC. Archived from the original on ... She supports several charitable causes, mostly ones related to cystic fibrosis, a condition from which her niece suffers, and ...
He served as president of the Cystic Fibrosis Trust from 1986 to 2003; of the British Lung Foundation from 1987 to 1995; and of ... 2004). Cystic Fibrosis. Wellcome Witnesses to Contemporary Medicine. History of Modern Biomedicine Research Group. ISBN 978-0- ...
"Cystic Fibrosis Foundation". Archived from the original on September 28, 2007. Retrieved May 22, 2007. "Sierra's Haven". ... Community and school-related service activities: American Red Cross blood drives, CF (cystic fibrosis) walks, health fairs, ...
"Cystic Fibrosis Canada Calls for CF Newborn Screening in Every Province-Early CF Detection Saves Lives". Cystic Fibrosis Canada ... Wagener, J. S.; Zemanick, E. T.; Sontag, M. K. (2012). "Newborn screening for cystic fibrosis". Current Opinion in Pediatrics. ... Molecular techniques are used for the diagnosis of cystic fibrosis and severe combined immunodeficiency.[citation needed] The ... Cystic fibrosis (CF) was first added to newborn screening programs in New Zealand and regions of Australia in 1981, by ...
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 ... U.S. National Library of Medicine, Medline Plus: Cystic Fibrosis. *Cystic Fibrosis Foundation ...
Search GeneCards for genes involved in cystic fibrosis Cystic Fibrosis Mutation Database "Cystic Fibrosis". MedlinePlus. U.S. ... The name "cystic fibrosis" refers to the characteristic fibrosis and cysts that form within the pancreas. Cystic fibrosis ... The Cystic Fibrosis Foundation recommends a diagnosis of cystic fibrosis for anyone suspected of cystic fibrosis (positive ... "Orphanet: Cystic fibrosis". Retrieved 29 July 2022. Hodson M, Geddes D, Bush A, eds. (2012). Cystic Fibrosis ( ...
Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. The symptoms and severity of CF can vary. Get the ... Consider Tube Feeding (Cystic Fibrosis Foundation) * Day-to-Day Exercise and Cystic Fibrosis (CF) (Cystic Fibrosis Foundation) ... Infections and Cystic Fibrosis (Cystic Fibrosis Foundation) * Marvels of Mucus and Phlegm: The Slime That Keeps You Healthy ( ... Cystic fibrosis: MedlinePlus Genetics (National Library of Medicine) * Learning about Cystic Fibrosis (National Human Genome ...
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; ... Cystic fibrosis (CF) is the most common lethal inherited disease in white persons. [4] Cystic fibrosis is an autosomal ...
Does Medicare cover cystic fibrosis?. Medically reviewed by Ayonna Tolbert, PharmD. Medicare offers coverage for various ... The Cystic Fibrosis Foundation recommends that people with CF not live together and avoid any activities that include close ... Cystic fibrosis is a prevalent condition that people can be carriers of without knowing. Testing is available to help a person ... People with cystic fibrosis (CF) have an increased risk of contracting and passing on harmful bacteria to other people with CF ...
"The mission of the New Jersey State of Cystic Fibrosis to ease the heavy financial burdens placed on CF patients and their ... "The mission of the New Jersey State of Cystic Fibrosis to ease the heavy financial burdens placed on CF patients and their ... New Jersey State Organization of Cystic Fibrosis. Totowa, NJ. , ...
... accurate cystic fibrosis diagnosis - including sweat testing appointments within 72 hours - and expert disease management for a ... Meet our cystic fibrosis care providers Meet our cystic fibrosis care providers Cystic Fibrosis Tests and Treatments * Genetic ... Among the Best in the U.S. for Cystic Fibrosis Care. The Rush Cystic Fibrosis Center is accredited by the Cystic Fibrosis ... The Rush Approach to Cystic Fibrosis Care. If you or your child has cystic fibrosis, comprehensive care is key to managing the ...
Lung transplantation has become an excellent treatment option for patients with cystic fibrosis (CF) and bronchiectasis with ... Lung transplantation for cystic fibrosis and bronchiectasis Semin Respir Crit Care Med. 2013 Jun;34(3):297-304. doi: 10.1055/s- ... Lung transplantation has become an excellent treatment option for patients with cystic fibrosis (CF) and bronchiectasis with ... survival is more favorable than that seen in patients with chronic obstructive pulmonary disease and pulmonary fibrosis. ...
... evaluation and treatment of liver disease are increasingly important challenges in children with cystic fibrosis (CF). Liver ... Liver disease in children with cystic fibrosis Paediatr Respir Rev. 2001 Dec;2(4):340-9. doi: 10.1053/prrv.2001.0170. ... 1 Department of Respiratory Medicine and Cystic Fibrosis, Birmingham Childrens Hospital NHS Trust, Birmingham, UK. diwakar@ ... evaluation and treatment of liver disease are increasingly important challenges in children with cystic fibrosis (CF). Liver ...
Johns Hopkins All Childrens Hospital is now offering a new drug therapy to cystic fibrosis patients that will help an ... Cystic Fibrosis: Kelsies Story Cystic Fibrosis: Evelyns Story Cystic Fibrosis Liver Disease Cystic Fibrosis ... Sam at the Cystic Fibrosis Care Center.. Patient Story Highlights *Sam, a 14-year-old with cystic fibrosis, became the first ... Cystic Fibrosis Center at Johns Hopkins All Childrens Hospital The Cystic Fibrosis Center at Johns Hopkins All Childrens ...
An experimental drug that has proven effective in treating muscular dystrophy also works for cystic fibrosis, according to ... Tags: Biopharmaceutical, Compound, Cystic Fibrosis, DNA, Duchenne Muscular Dystrophy, Fibrosis, Food, Gene, Genes, Genetic, ... An experimental drug that has proven effective in treating muscular dystrophy also works for cystic fibrosis, according to ... MHRA extends the license of two cystic fibrosis drugs for children aged two to five years old ...
Epix Pharmaceuticals and Cystic Fibrosis Foundation Therapeutics (CFFT), a nonprofit affiliate of the Cystic Fibrosis ... BiomoleculesCellular, Molecular and Developmental BiologyCystic fibrosisGenetic variantsGeneticsMedicine, Diagnosis, and ... CFTR is believed to be the key protein associated with cystic fibrosis. ... virtual 3-D molecule of the full-length Cystic Fibrosis Transmembrane conductance Regulator (CFTR) in January. ...
Evaluating the Impact of Precision Medicine: How Ivacaftor Reduces Hospitalizations of Patients with Cystic Fibrosis. Precision ... cystic fibrosis - Genomics and Precision Health Blog ...
... aztreonam was an effective new treatment for life-threatening lung infections in cystic ... US FDA panel says Gilead drug aids cystic fibrosis. By Jackie Frank 12/10/09 AT 7:46 PM EST. ... While cystic fibrosis patients live longer now than 20 years ago, it is still a devastating disease, Marshall said. The average ... Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive systems of about 70,000 people worldwide, ...
A Pulse CPD article answering questions about cystic fibrosis diagnosis, management and advances in treatment ... Q: How common is cystic fibrosis (CF) in children? What are the genetics of the disease?. A: Around 200 children are diagnosed ...
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 (national office). 4550 Montgomery Ave.. Suite 1100 N. Bethesda, MD 20814 ... Cystic fibrosis is a genetic disorder that affects the lungs, pancreas, and other organs. ... could potentially benefit anyone with cystic fibrosis, including those who cant take CFTR modulators. ...
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 (national office). 4550 Montgomery Ave.. Suite 1100 N. Bethesda, MD 20814 ... Cystic fibrosis is a genetic disorder that affects the lungs, pancreas, and other organs. ... could potentially benefit anyone with cystic fibrosis, including those who cant take CFTR modulators. ...
2023 Cystic Fibrosis Canada. All Rights Reserved. Privacy Policy , Digital Accessibility Statement , Site Map ...
... [ Post a Message , Cystic Fibrosis and Glutathione Stories ]. CYSTIC FIBROSIS (CF) AND ... New website about glutathione in cystic fibrosis - Lucy October 18, 2012, 8:13 pm *Re: New website about glutathione in cystic ... New website about glutathione in cystic fibrosis - Hope November 4, 2012, 9:19 am *Re: New website about glutathione in cystic ... fibrosis - Hope October 22, 2012, 10:23 pm *Re: New website about glutathione in cystic fibrosis - Lucy November 3, 2012, 6:47 ...
Cystic fibrosis is classified to ICD-9-CM subcategory 277.0 and includes the following codes:. • 277.00, Cystic fibrosis ... ICD-10-CM Coding for Cystic Fibrosis. In ICD-10-CM, cystic fibrosis is classified to category E84. Similar to ICD-9-CM, there ... Cystic fibrosis causes abnormal mucus glands due to an abnormal function of the cystic fibrosis transmembrane conductance ... Code E84.11 is for meconium ileus in cystic fibrosis. If the meconium ileus is not due to cystic fibrosis, then assign code ...
Help us end cystic fibrosis. Now is the time. Donate to help ensure people with CF can live longer, healthier lives, free from ... 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 (national office). 4550 Montgomery Ave.. Suite 1100 N. Bethesda, MD 20814 ... Cystic fibrosis is a genetic disorder that affects the lungs, pancreas, and other organs. ...
... [ Post a Message , Cystic Fibrosis and Glutathione Stories ]. CYSTIC FIBROSIS (CF) AND ... New website about glutathione in cystic fibrosis - Lucy October 18, 2012, 8:13 pm *Re: New website about glutathione in cystic ... New website about glutathione in cystic fibrosis - Hope November 4, 2012, 9:19 am *Re: New website about glutathione in cystic ... fibrosis - Hope October 22, 2012, 10:23 pm *Re: New website about glutathione in cystic fibrosis - Lucy November 3, 2012, 6:47 ...
This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.. Edits to original content made by Denver Health. Copyright © EBSCO Information ...
Teens with cystic fibrosis have some specific nutritional needs. Heres how they can meet those needs. ... Nutritional Needs in Teens With Cystic Fibrosis. Teens with cystic fibrosis (CF) have unique nutritional needs. If you have CF ... Consejos dietéticos para adolescentes con fibrosis quística. ...
Couple with cystic fibrosis put love before medical concerns and pay the highest price as one of them enters hospice care. ...
Sign-up for Cystic Fibrosis Clinic Connection and get monthly expert tips and insights, latest news and event notifications in ... Youll be the first to get expert cystic fibrosis health care tips and insights, hear the latest news on medical treatments, ... Cystic Fibrosis Clinic Connection Cystic Fibrosis Clinic Connection Email Sign-Up Youre now subscribed. Thank you for staying ...
... and a wide variety of health care providers involved in research and care related to cystic fibrosis. ... Join Expres Scripts as we discuss solutions to making health care more affordable for those who manage cystic fibrosis every ... and a wide variety of health care providers involved in research and care related to cystic fibrosis. ...
Disability Overview Cystic fibrosis is the most common autosomal recessive disorder in Caucasians with an incidence rate of 1 ... Cystic Fibrosis : A Defect. What is Cystic Fibrosis? How does it affect people living with it? Cystic Fibrosis, also known as ... Common Genetic Disorders: Cystic Fibrosis. Every day, people die from Cystic Fibrosis. That is why Cystic Fibrosis is known as ... 70,000 people worldwide.? (Diagnosed With Cystic Fibrosis, Cystic Fibrosis Foundation) Someone who has Cystic Fibrosis have a ...
  • CF results from mutations (changes) in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene , which has instructions for making the CFTR protein. (
  • Genetic therapies - including mRNA therapy, gene therapy, and gene editing - could potentially benefit anyone with cystic fibrosis, including those who can't take CFTR modulators. (
  • Cystic fibrosis, which affects about 30,000 people in the United States, mostly Caucasians of north European origin, is an inherited disease caused by a defect in a gene called the cystic fibrosis transmembrane conductance regulator (CFTR). (
  • An individual with cystic fibrosis has a specific mutation in a gene and protein called cystic fibrosis transmembrane conductance regulator (CFTR). (
  • 5 Called the cystic fibrosis transmembrane conductance regulator (CFTR) gene, this mutation prevents the CFTR protein from working properly. (
  • The sensitivity and specificity of such testing can now be improved as a result of the recent discovery of the Cystic Fibrosis Transmembrane Conductance Regulatory (CFTR) gene. (
  • The discovery of the Cystic Fibrosis Transmembrane Conductance Regulatory (CFTR) gene (5) renewed interest in this possibility, as the sensitivity and specificity of testing could be improved through DNA-based testing. (
  • Severe liver disease distinguished by cirrhosis with portal hypertension may be developed by a small fraction about 3 - 5 percent with cystic fibrosis (CF). Prior study has claimed that genetic variability that is not related with the cystic fibrosis transmembrane conductance regulator (CFTR) gene may add to the danger for severe liver disease. (
  • Cystic fibrosis (CF) is an autosomal recessive condition caused by the mutation of the cystic fibrosis trans-membrane regulator gene (CFTR) on chromosome 7. (
  • It encodes a membrane-associated protein called the cystic fibrosis transmembrane conductance regulator (CFTR). (
  • A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. (
  • We've already seen the significant impact that disease-modifying medicines can have on patients and are incredibly pleased that there is now a third treatment option that enables more patients to benefit from CFTR modulation," Patrick Flume, MD, director of the Medical University of South Carolina Cystic Fibrosis Center and principal investigator for the EXTEND study, said in the news release. (
  • Acquired cystic fibrosis transmembrane regulator (CFTR) dysfunctions have been associated with several conditions, including myocardial infarction (MI). (
  • We have been directly working on this for many years at our center, ever since one of the drugs that now is also one-third of the medication that comprises Trikafta was first studied in 2011," says Green, who will attend the North American Cystic Fibrosis Conference this week in Nashville. (
  • citation needed] In 1989, Canadian researchers, funded by Cystic Fibrosis Canada, discovered the gene responsible for cystic fibrosis, and they continue to play a leading role in developing new treatments. (
  • Nearly 1 in every 30 Americans are a carrier of the recessive gene that leads to CF (About Cystic Fibrosis, n.d. (
  • The gene that causes cystic fibrosis is recessive. (
  • This means that in order to have cystic fibrosis, children must inherit two copies of the gene, one from each parent. (
  • Parents who carry the cystic fibrosis gene are often healthy and have no symptoms of disease, and yet are still likely to pass it on to their children. (
  • In fact, it's estimated that as many as 10 million people may be carriers of a cystic fibrosis gene and not know it. (
  • If both parents carry a defective cystic fibrosis gene, each pregnancy has a 25 percent chance of producing a child with cystic fibrosis. (
  • The cystic fibrosis gene is most common in Caucasians of northern European descent. (
  • however, it's not possible to identify every person who carries a gene for cystic fibrosis. (
  • Because there are hundreds of specific cystic fibrosis gene mutations (not all of which are known), genetic testing for cystic fibrosis is not 100 percent sensitive. (
  • How is the cystic fibrosis ( CF ) gene inherited? (
  • A genetic analysis now points out that a particular gene variation in patients suffering from cystic fibrosis could considerably add to their possibility of developing severe liver disease. (
  • The suspected diagnosis of cystic fibrosis was confirmed with a sweat chloride level of 120 mmol/L and homo-zygozity for the Delta F 508 gene on genetic studies. (
  • Diagnosis is by sweat test or identification of 2 cystic fibrosis-causing gene variants in patients with a positive newborn screening test result or characteristic clinical features. (
  • Identification of the cystic fibrosis gene: chromosome walking and jumping. (
  • The prospective study, "Early respiratory viral infections in infants with cystic fibrosis," was published in the Journal of Cystic Fibrosis. (
  • Treatment is supportive through aggressive multidisciplinary care along with small-molecule correctors and potentiators targeting the cystic fibrosis transmembrane conductance regulator protein defect. (
  • citation needed] Cystic Fibrosis Canada's mandate is to help individuals with cystic fibrosis, principally by funding cystic fibrosis research and care. (
  • Years ago, individuals with cystic fibrosis would rarely live beyond his or her childhood, but now many individuals with the disease are living well into their adulthood (Falvo, 2014). (
  • As well as general information about cystic fibrosis in Canada and resources for teachers, parents, and health care professionals, Cystic Fibrosis Canada publishes newsletters and reports covering such areas as research and training grants, clinical services and annual data on patients with cystic fibrosis. (
  • Cystic fibrosis (CF) 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. (
  • The Rush Cystic Fibrosis Center is accredited by the Cystic Fibrosis Foundation (CFF) and has been selected as a CFF partner on quality initiatives aimed at improving care for all cystic fibrosis patients. (
  • Sam, a 14-year-old with cystic fibrosis, became the first patient at Johns Hopkins All Children's Hospital to take Trikafta, a new drug therapy that will help an estimated 90% of cystic fibrosis patients. (
  • This is definitely expected to be a life-changing medication for the majority of my patients," says Deanna Green, M.D., M.H.S. , medical director of the nationally accredited Cystic Fibrosis (CF) Care Center and the CF Therapeutic Development Center at Johns Hopkins All Children's Hospital in St. Petersburg, Florida. (
  • Lung transplantation has become an excellent treatment option for patients with cystic fibrosis (CF) and bronchiectasis with very advanced lung disease. (
  • Despite the challenges that the CF patients present, survival is more favorable than that seen in patients with chronic obstructive pulmonary disease and pulmonary fibrosis. (
  • NTM have been increasingly isolated from the sputum of performed by using Microsoft Excel 2007 (Microsoft, Red- patients with cystic fibrosis (CF) ( 7,8 ). (
  • GAITHERSBURG, Md., Dec 10 - A U.S. Food and Drug Administration advisory panel on Thursday said it found Gilead Sciences Inc's (GILD.O) aztreonam was an effective new treatment for life-threatening lung infections in cystic fibrosis patients. (
  • While cystic fibrosis patients live longer now than 20 years ago, it is still a devastating disease, Marshall said. (
  • It describes a potential strategy for treating cystic fibrosis (CF) - an inherited, fatal disease that affects 30,000 Americans - by using slightly acidified sodium nitrite, a common food preservative, to kill antibiotic-resistant bacteria that thrive in the mucous-clogged lungs of CF patients. (
  • CINCINNATI-Researchers led by a University of Cincinnati (UC) scientist say they have discovered what might be the "Achilles' heel" of a dangerous organism that lives in the lungs of cystic fibrosis patients-a fatal flaw that leaves the organism vulnerable to destruction by a common food preservative. (
  • It has been known for some time that the bacterium, Pseudomonas aeruginosa, grows within the deadly, lung-clogging mucous found in the airways of cystic fibrosis patients and significantly weakens them. (
  • The new study suggests, however, that a mutation-known as mucA-in the organism also represents a fatal flaw that could help physicians clear the characteristic "goop" from the lungs of advanced cystic fibrosis patients. (
  • The lung-clogging, suffocating mucoid form of Pseudomonas aeruginosa essentially is a death sentence for cystic fibrosis patients because these bacteria are inherently antibiotic and white-cell resistant," said Dr. Hassett. (
  • I joined the Cystic Fibrosis PFAC to educate the Council and the CF adult community about the double lung transplant journey and to work together to make our experience at the Cystic Fibrosis Clinic and Lung Transplnat Clinic at Stanford Hospital a positive, emotional and physical environment for Cystic Fibrosis patients. (
  • When cystic fibrosis was first documented in 1938, the life expectancy was only a few months for the patients (O'Sullivan & Freedman, 2009). (
  • This is an observational multicentre study including 819 patients diagnosed with non-cystic fibrosis bronchiectasis using high-resolution computed tomography. (
  • Which bacteria causes the greatest infection burden in cystic fibrosis patients? (
  • Breathe deep: Which Patients Could Benefit from Inhaled Steroids in Cystic Fibrosis? (
  • Increased expression of interleukin-9, interleukin-9 receptor, and the calcium-activated chloride channel hCLCA1 in the upper airways of patients with cystic fibrosis. (
  • Today we are pleased to announce the 2021/2022 donation of $5,000 to Cystic Fibrosis Canada which is part of our Health & Welfare strategy. (
  • 1. Cystic Fibrosis Foundation Patient Registry 2021 Annual Data Report Bethesda, Maryland 2022 Cystic Fibrosis Foundation. (
  • Cystic fibrosis (CF) lung disease is characterised by chronic inflammation and infection. (
  • Interactions between secondhand smoke and genes that affect cystic fibrosis lung disease. (
  • You'll be the first to get expert cystic fibrosis health care tips and insights, hear the latest news on medical treatments, read exclusive patient stories and much more. (
  • Today, thanks to improved treatments, people with cystic fibrosis live an average of 35 years. (
  • Our study shows strong pre-clinical evidence that PTC124 is capable of suppressing 'nonsense mutations' that cause cystic fibrosis," said David Bedwell, Ph.D., professor in the UAB Department of Microbiology and lead author on the study. (
  • Disability Overview Cystic fibrosis is the most common autosomal recessive disorder in Caucasian's with an incidence rate of 1 in every 2,500 births. (
  • Cystic fibrosis is an existence restricting autosomal recessive disorder that influences 70,000 people around the world. (
  • With this study, we aimed to create and validate an easy-to-use multidimensional grading system that could classify the severity of non-cystic fibrosis (CF) bronchiectasis more accurately than any single variable, according to its capacity to predict the 5-year risk of all-cause mortality. (
  • Nasal mucociliary clearance and ciliary beat frequency in cystic fibrosis compared with sinusitis and bronchiectasis. (
  • Rush offers fast, accurate cystic fibrosis diagnosis - including sweat testing appointments within 72 hours - and expert disease management for a lifetime. (
  • Early diagnosis for cystic fibrosis (CF) can make a big difference in improving the quality of life for people with. (
  • If your baby has a positive newborn screening for cystic fibrosis, you can schedule a sweat test at Rush within 72 hours by calling (312) 942-3034. (
  • Since the development of the immunoreactive trypsinogen test (IRT) for cystic fibrosis (CF), experts in the field of CF have considered adding this test to the newborn screening panel. (
  • The preclinical and clinical data on PTC124 support our hope that this drug will be an important disease-modifying therapy for cystic fibrosis," said Robert J. Beall, Ph.D., president and chief executive officer of the Cystic Fibrosis Foundation in Bethesda, Md. "We look forward to the next stage of clinical development to demonstrate the benefits of this promising therapy. (
  • Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive systems of about 70,000 people worldwide, according to the Cystic Fibrosis Foundation. (
  • Cystic fibrosis is a genetic disorder that affects the lungs, pancreas, and other organs. (
  • During the chronic form of cystic fibrosis," Dr. Hassett said, "the mutated form of the organism, combined with the immune system's attempts to fight it off, wreaks havoc in the lungs. (
  • Cystic Fibrosis, commonly known as CF, is a life-threatening, lethal genetic disease that mostly affects the lungs and digestive system (O'Sullivan & Freedman, 2009). (
  • 12] Unusually viscous emissions in the airway of the lungs and in ducts of the pancreas in people with cystic fibrosis cause hindrances that prompt aggravation, tissue harm and destruction of both organ systems. (
  • Cystic Fibrosis is a life threatening condition that causes severe damage to the lungs and digestive system. (
  • Cystic fibrosis (CF) is an inherited life-threatening disorder that damages the lungs and digestive system. (
  • The organs most often affected by cystic fibrosis are the lungs and the pancreas, which can lead to breathing and digestive problems. (
  • In a person with cystic fibrosis, mucus is still able to trap bacteria, but it has trouble moving out of the lungs. (
  • Cystic fibrosis is a disease affecting the lungs and digestive system. (
  • Over time, mucus buildup and infections can lead to permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs. (
  • When two channels that are supposed to move chloride and sodium ions out of cells in the lungs fail to function properly, it leads to the mucus buildup seen in cystic fibrosis. (
  • Shinerama List of cystic fibrosis organizations Cystic Fibrosis Foundation Cystic Fibrosis Trust "Annual Report 2009: Keeping a Promise" (PDF). (
  • According to the Cystic Fibrosis Foundation , people with CF need to keep a distance of 6 feet between each other and between those with an infection, including a cold or flu. (
  • The Cystic Fibrosis Foundation notes that if people with CF are having difficulty speaking with their partner about their condition, they may find it beneficial to speak with a member of their healthcare team or a therapist. (
  • At the Cystic Fibrosis Foundation-accredited Rush Cystic Fibrosis Center at Rush University Medical Center, our multidisciplinary team consists of pulmonologists, gastroenterologists, nurse practitioners and palliative care experts, along with nurses, dietitians, genetic counselors, physical therapists, respiratory therapists and even a dedicated psychologist. (
  • The Johns Hopkins All Children's program is nationally accredited by the Cystic Fibrosis Foundation as a Cystic Fibrosis Care Center. (
  • The program is also a Cystic Fibrosis Foundation-designated Therapeutic Development Center and specializes in clinical trials to evaluate the safety and efficacy of new therapies. (
  • Michael Boyle, M.D. , is an internationally recognized cystic fibrosis expert with Johns Hopkins Medicine in Baltimore and senior vice president of therapeutics development of the Cystic Fibrosis Foundation. (
  • Dr. Bruce Marshall of the Cystic Fibrosis Foundation urged FDA to approve the drug swiftly and told the panel: There is a desperate need for additional inhaled antibiotics. (
  • The Cystic Fibrosis Foundation is the world's leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles. (
  • The research was funded by the U.S. National Institutes of Health, the Cystic Fibrosis Foundation and the U.S. Department of Energy. (
  • Nature Coast APA member Lori Hartt knows firsthand what the Cystic Fibrosis Foundation means to someone with the disease her granddaughter, Lexi, has it. (
  • The workshop planning committee comprised representatives from CDC, the Cystic Fibrosis Foundation, the National Institutes of Health, and the University of Wisconsin. (
  • CRESTVIEW - Organizers are hoping thousands of people will pack Main Street Nov. 2 for the annual Great Strides Race for Lace, raising at least $62,000 for the Cystic Fibrosis Foundation. (
  • LeGrys VA, Yankaskas JR, Quittell LM, Marshall BC, Mogayzel PJ Jr. Diagnostic sweat testing: the Cystic Fibrosis Foundation guidelines. (
  • In a person with cystic fibrosis, the thick, sticky mucus blocks ducts (or paths) between the pancreas and the intestines . (
  • Because the pancreas controls the level of sugar in the blood, a small percentage of people with cystic fibrosis may also develop type-one diabetes (formerly called juvenile or insulin-dependent diabetes). (
  • Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. (
  • Cystic fibrosis is an inherited disease of the exocrine glands affecting primarily the gastrointestinal and respiratory systems. (
  • Symptoms may include fatigue, salty-tasting skin, persistent cough with phlegm, wheezing and shortness of breath, lung infections, and poor growth and weight loss (Cystic Fibrosis Symptoms, Causes & Risk Factors, 2018). (
  • Cite this: FDA Clears Tezacaftor/Ivacaftor Combo for Cystic Fibrosis - Medscape - Feb 13, 2018. (
  • The 14-year-old from Sarasota has cystic fibrosis , a genetic disease that can cause persistent lung infections and limits the ability to breathe over time. (
  • Cystic fibrosis affects the body by producing thick mucus in varying organ systems which can cause chronic sinus tract infections, respiratory infections, malabsorption disorders, increased metabolic demand, pancreatic dysfunction and potential sterility in males. (
  • How are viral infections related to cystic fibrosis? (
  • Such organisms have emerged pdf) was sent to the lead CF physician in all UK pediat- as pathogens in immunocompetent adults, for example, af- ric and adult CF centers identified by the principal UK CF ter traumatic limb injuries sustained during the 2004 tsu- charity, the Cystic Fibrosis Trust, in 2009. (
  • Behavioral Health Screening in Military Cystic Fibrosis Centers: A Survey. (
  • Cystic Fibrosis Canada's primary objective is to fund CF research and care: the organization annually funds approximately 50 research projects, in addition to scholars, post-doctoral fellows, and students undertaking CF investigations. (
  • 1 Department of Respiratory Medicine and Cystic Fibrosis, Birmingham Children's Hospital NHS Trust, Birmingham, UK. (
  • Cystic Fibrosis - "Our children's lives are worth the cost. (
  • Each year, Cystic Fibrosis Canada awards more than $7 million in grants to CF researchers, and approximately $2 million in grants to the 42 CF clinics, and five transplant centres across the country. (
  • Canadian researchers are viewed as leaders in the global effort to find a cure or control for cystic fibrosis. (
  • An experimental drug that has proven effective in treating muscular dystrophy also works for cystic fibrosis, according to researchers at the University of Alabama at Birmingham (UAB) . (
  • Amelioration of cystic fibrosis intestinal mucous disease in mice by restoration of mCLCA3. (
  • Identification, evaluation and treatment of liver disease are increasingly important challenges in children with cystic fibrosis (CF). Liver disease usually presents at puberty and is receiving more attention with improved life expectancy. (
  • In some people with cystic fibrosis, the intestines, liver , sweat glands, and reproductive organs are also harmed. (
  • Is it safe for people with cystic fibrosis to be together? (
  • People with cystic fibrosis (CF) have an increased risk of contracting and passing on harmful bacteria to other people with CF. This means people with CF need to avoid close physical contact with other people with the condition. (
  • In people with cystic fibrosis, the secretions are abnormally thick and sticky, so that they don't move as easily. (
  • As a result, people with cystic fibrosis have trouble digesting food and getting the vitamins and nutrients they need from it. (
  • How many people are affected by cystic fibrosis? (
  • In the UAB tests performed on mice, PTC124 restored to normal function up to 29 percent of the cases of abnormal cystic-fibrosis (CF) protein. (
  • The condition is known to affect principally those of European descent, though cystic fibrosis has been accounted for in all races and ethnicities. (
  • Does your letter state "This infant's newborn screen for cystic fibrosis (CF) was abnormal and follow-up testing is required. (
  • Research shows that children and adults with cystic fibrosis do best when treated in a multidisciplinary clinic. (
  • More than 30,000 children and adults in the United States have Cystic Fibrosis. (
  • Cystic Fibrosis and Stem Cell Therapy Cystic fibrosis is a genetic disease that leads to the destruction and damage to the digestive system. (
  • To provide a network and a forum for communication among adults who have cystic fibrosis, their families, and medical professionals and to publish a quarterly publication to facilitate the exchange of information among cystic fibrosis adults. (
  • is one of the world's largest social media networks dedicated exclusively to the cystic fibrosis community. (
  • The US Food and Drug Administration (FDA) has approved a third drug from Vertex Pharmaceuticals directed at an underlying cause of cystic fibrosis (CF), the company has announced. (
  • A genetic disorder which is known to be a hereditary disease of the secretory glands, as well as the glands that makes mucus and sweat is called as cystic fibrosis. (
  • Cystic fibrosis (CF) is a life-threatening genetic disease, which in the United States occurs in about 1/3,300 White births, 1/15,300 Black births, and 1/32,000 Asian American births. (
  • Cystic fibrosis (CF) is the most common life -threatening genetic illness in the United States . (
  • It is often difficult to determine whether isolation mycobacteria (NTM) is increasing among persons with cys- of NTM represents colonization or disease that requires tic fibrosis (CF). We assessed prevalence and management treatment. (
  • We believe that we have discovered the Achilles' heel of the formidable mucoid form of Pseudomonas aeruginosa, which could lead to improved treatment for cystic fibrosis airway disease," said Dr. Hassett. (
  • Semi-directive interviews were realized with three adolescents with cystic fibrosis to understand theirs perceptions about puberty, and their future planning and treatment adherence. (
  • To help educate and to help find a cure for Cystic Fibrosis. (