642 results about "Cystic fibrosis lungs" patented technology

A pharmaceutical composition and formulations comprise preventative, prophylactic or therapeutic amounts of an oligo(s) anti-sense to a specific gene(s) or its corresponding mRNA(s), and a glucocorticoid and / or non-glucocorticoid steroid or a ubiquinone or their salts. The agents, composition and formulations are used for treatment of ailments associated with impaired respiration, bronchoconstriction, lung allergy(ies) or inflammation, and abnormal levels of adenosine, adenosine receptors, sensitivity to adenosine, lung surfactant and ubiquinone, such as pulmonary fibrosis, vasoconstriction, inflammation, allergies, allergic rhinitis, asthma, impeded respiration, lung pain, cystic fibrosis, bronchoconstriction, COPD, RDS, ARDS, cancer, and others. The present treatment is effectively administered by itself for conditions without known therapies, as a substitute for therapies exhibiting undesirable side effects, or in combination with other treatments, e.g. before, during and after other respiratory system therapies, radiation, chemotherapy, antibody therapy and surgery, among others. Each of the agents of this invention may be administered directly into the respiratory system so that they gain direct access to the lungs, or by other effective routes of administration. A kit comprises a delivery device, the agents and instructions for its use.

Embodiments of the present invention provide a method for treatment of respiratory disorders such as asthma, chronic obstructive pulmonary disease, and chronic sinusitis, including cystic fibrosis, interstitial fibrosis, chronic bronchitis, emphysema, bronchopulmonary dysplasia and neoplasia. The method involves administration, preferably oral, nasal or pulmonary administration, of anti-inflammatory and anti-proliferative drugs (rapamycin or paclitaxel and their analogues).

The present invention relates to modulators of ATP-Binding Cassette (“ABC”) transporters or fragments thereof, including Cystic Fibrosis Transmembrane Regulator (“CFTR”), compositions thereof, and methods therewith. The present invention also relates to methods of treating ABC transporter mediated diseases using such modulators.

The present invention relates to peptides, particularly human monoclonal antibodies, that bind specifically to P. aeruginosa mucoid exopolysaccharide. The invention further provides methods for using these peptides in the diagnosis, prophylaxis and therapy of P. aeruginosa infection and related disorders (e.g., cystic fibrosis). Some antibodies of the invention enhance opsonophagocytic killing of multiple mucoid strains of P. aeruginosa. Compositions of these peptides, including pharmaceutical compositions, are also provided, as are functionally equivalent variants of such peptides.

This invention relates to coated digestive enzyme preparations and enzyme delivery systems and pharmaceutical compositions comprising the preparations. This invention further relates to methods of preparation and use of the systems, pharmaceutical compositions and preparations to treat persons having ADD, ADHD, autism, cystic fibrosis and other behavioral and neurological disorders.

The present invention relates to modulators of ATP-Binding Cassette (“ABC”) transporters or fragments thereof, including Cystic Fibrosis Transmembrane Regulator (“CFTR”), compositions thereof, and methods therewith. The present invention also relates to methods of treating ABC transporter mediated diseases using such modulators.

The present invention relates to therapeutic combinations and kits useful in treating CFTR-related diseases, such as cystic fibrosis.

The invention provides compositions and methods for treating pancreatic enzyme insufficiency, such as the pancreatic enzyme insufficiency associated with cystic fibrosis. The invention also provides compositions comprising lipase from Candida cylindracea, alone or in combination with amylase or amyloglucosidase, protease and / or lactase. Furthermore, the invention discloses methods for treating pancreatic enzyme insufficiency comprising administering compositions to patients in need thereof.

A pharmaceutical composition comprising Compound 1, (R)-1-(2,2-difluorobenzo[d][1,3]dioxol-5-yl)-N-(1-(2,3-dihydroxypropyl)-6-fluoro-2-(1-hydroxy-2-methylpropan-2-yl)-1H-indol-5-yl)cyclopropanecarboxamide, and at least one excipient selected from: a filler, a diluent, a disintegrant, a surfactant, a glidant and a lubricant, the composition being suitable for oral administration to a patient in need thereof to treat a CFTR mediated disease such as Cystic Fibrosis. Methods for treating a patient in need thereof include administering the pharmaceutical composition of Compound 1 are also disclosed.

The present invention relates to modulators of cystic fibrosis transmembrane conductance regulator (“CFTR”), compositions thereof, and methods therewith. The present invention also relates to pharmaceutical compositions comprising a compound of Formula I with one or both of a Compound of Formula II and / or a Compound of Formula III. Further, the present invention relates to methods of treating CFTR mediated diseases, particularly cystic fibrosis, using modulators of CFTR, and compositions and combinations thereof.

The invention relates to improvements in or relating to medicament delivery devices. In particular, this invention relates to a communications device which may be fitted to an electronic medicament delivery device. The invention may be particularly suitable for use with electronic medicament inhalers such as those used for the treatment of diabetes, or respiratory diseases such as asthma, COPD, cystic fibrosis, and bronchiectasis.

Provided are electrokinetically-altered fluids (gas-enriched (e.g., oxygen-enriched) electrokinetic fluids) comprising an ionic aqueous solution of charge-stabilized oxygen-containing nanostructures in an amount sufficient to provide, upon contact with a cell, modulation of at least one of cellular membrane potential and cellular membrane conductivity, and therapeutic compositions and methods for using same in treating cystic fibrosis or a symptom thereof. The electrokinetically-altered fluid compositions and methods include electrokinetically-altered fluids optionally in combination with other therapeutic agents (e.g., antibiotics, albuterol, budesonide, etc.). Particular embodiments comprise use and / or synergy with tobramycin for treating bacterial infection, and use and / or synergy with a bronchiodilator. In certain aspects, the methods comprise regulating intracellular signal transduction by modulation of at least one of cellular membranes, membrane potential, membrane proteins (like, membrane receptors, including but not limited to G protein coupled receptors, and intercellular junctions).

The present invention relates to the use of N-[2,4-bis(1,1-dimethylethyl)-5-hydroxyphenyl]-1,4-dihydro-4-oxoquinoline-3-carboxamide and pharmaceutical compositions thereof for the treatment of cystic fibrosis, in patients, including kits and / or products thereof.

The present invention relates to the use of carbon monoxide (CO) as a biomarker and therapeutic agent of heart, lung, liver, spleen, brain, skin and kidney diseases and other conditions and disease states including, for example, asthma, emphysema, bronchitis, adult respiratory distress syndrome, sepsis, cystic fibrosis, pneumonia, interstitial lung diseases, idiopathic pulmonary diseases, other lung diseases including primary pulmonary hypertension, secondary pulmonary hypertension, cancers, including lung, larynx and throat cancer, arthritis, wound healing, Parkinson's disease, Alzheimer's disease, peripheral vascular disease and pulmonary vascular thrombotic diseases such as pulmonary embolism. CO may be used to provide anti-inflammatory relief in patients suffering from oxidative stress and other conditions especially including sepsis and septic shock. In addition, carbon monoxide may be used as a biomarker or therapeutic agent for reducing respiratory distress in lung transplant patients and to reduce or inhibit oxidative stress and inflammation in transplant patients.

The present invention relates to modulators of ATP-Binding Cassette (“ABC”) transporters or fragments thereof, including Cystic Fibrosis Transmembrane Conductance Regulator, compositions thereof, and methods therewith. The present invention also relates to methods of treating ABC transporter mediated diseases using such modulators.

A stable preparation of digestive / pancreatic enzymes which can be readily formed into a dosage formulation is provided as a treatment of pancreatic insufficiency in persons having cystic fibrosis. The dosage formulation can be administered either by an oral preparation including, but not limited to, a microcapsule, mini-capsule, time released capsule, sprinkle or other methodology. A further object of this invention is to provide a stabilized preparation of a combination medicant which resists degradation by light, heat, humidity or association with commonly used excipients.

Particular aspects provide electrokinetically-generated fluids (e.g., electrokinetically-generated gas-enriched fluids and solutions), and therapeutic compositions and methods comprising use thereof in treating at least one symptom of cystic fibrosis. In particular embodiments, at least one symptom of cystic fibrosis treated by the present invention include inhibition of Pseudomonas infection, synergy with tobramycin (including TOBI) for use against bacterial infection, and synergy with a bronchiodilator. In particular embodiments, the electrokinetically-generated fluids or therapeutic compositions and methods comprise combination with other therapeutic agents (e.g., antibiotics, albuterol, budesonide, etc.). In certain aspects, the methods comprise regulating or modulating intracellular signal transduction by modulation of at least one of cellular membranes, membrane potential, membrane proteins such as membrane receptors, including but not limited to G protein coupled receptors, and intercellular junctions (e.g., tight junctions, gap junctions, zona adherins and desmasomes).

Provided are electrokinetically-altered fluids (gas-enriched (e.g., oxygen-enriched) electrokinetic fluids) comprising an ionic aqueous solution of charge-stabilized oxygen-containing nanostructures in an amount sufficient to provide, upon contact with a cell, modulation of at least one of cellular membrane potential and cellular membrane conductivity, and therapeutic compositions and methods for using same in treating cystic fibrosis or a symptom thereof. The electrokinetically-altered fluid compositions and methods include electrokinetically-altered fluids optionally in combination with other therapeutic agents (e.g., antibiotics, albuterol, budesonide, etc.). Particular embodiments comprise use and / or synergy with tobramycin for treating bacterial infection, and use and / or synergy with a bronchiodilator. In certain aspects, the methods comprise regulating intracellular signal transduction by modulation of at least one of cellular membranes, membrane potential, membrane proteins (like, membrane receptors, including but not limited to G protein coupled receptors, and intercellular junctions).

The present invention relates to pharmaceutical compositions comprising a compound of Formula I in combination with one or both of a Compound of Formula II and / or a Compound of Formula III. The invention also relates to solid forms and to pharmaceutical formulations thereof, and to methods of using such compositions in the treatment of CFTR mediated diseases, particularly cystic fibrosis.

The present invention relates to pharmaceutical compositions containing a solid dispersion of N—[2,4-Bis(1,1-dimethylethyl)-5-hydroxyphenyl]-1,4-dihydro-4-oxoquinoline-3-carboxamide including formulations of the solid dispersions into powders, granules and mini-tablets, methods for manufacturing and processing the powders, granules and mini-tablets, and methods for treating cystic fibrosis employing the pharmaceutical composition.

Compositions and methods are disclosed for inhibiting the release of a proinflammatory cytokine from a vertebrate cell, and for inhibiting an inflammatory cytokine cascade in a patient. The compositions comprise, for example, high affinity antibodies that specifically bind HMG1 and antigenic fragments thereof. The high affinity antibodies of the present invention and pharmaceutical compositions comprising the same are useful for many purposes, for example, as therapeutics against a wide range of inflammatory diseases and disorders such as sepsis, rheumatoid arthritis, peritonitis, Crohn's disease, reperfusion injury, septicemia, endotoxic shock, cystic fibrosis, endocarditis, psoriasis, psoriatic arthritis, arthritis, anaphylactic shock, organ ischemia, reperfusion injury, and allograft rejection. In addition, the high affinity antibodies of the present inventions are useful as diagnostic antibodies.

The present invention relates to certain novel dinucleotides and formulations thereof which are highly selective agonists of the P2Y2 and / or P2Y4 purinergic receptor. They are useful in the treatment of chronic obstructive pulmonary diseases such as chronic bronchitis, PCD, cystic fibrosis, as well as prevention of pneumonia due to immobility. Furthermore, because of their general ability to clear retained mucus secretions and stimulate ciliary beat frequency, the compounds of the present invention are also useful in the treatment of sinusitis, otitis media and nasolacrimal duct obstruction. They are also useful for treatment of dry eye disease and retinal detachment as well as facilitating sputum induction and expectoration.

The invention is a composition administrable orally to provide systemic glutathione (reduced) and a method for providing systemic glutathione by oral administration of glutathione (reduced) in a liposome encapsulation. The administration of a therapeutically effective amount of oral liposomal glutathione (reduced) results in improvement of symptoms in disease states related to glutathione deficiency such as Parkinson's disease and cystic fibrosis. Compounds enhancing the effect of the liposomal glutathione are contemplated such as Selenium, EDTA, carbidopa, and levodopa.

The present invention relates to therapeutic nanoemulsion compositions and to methods of utilizing the same. In particular, nanoemulsion compositions are described herein that find use in the treatment and / or prevention of infection (e.g., respiratory infection (e.g., associated with cystic fibrosis)), in burn wound management, and in immunogenic compositions (e.g., comprising a Burkholderia antigen) that generate an effective immune response (e.g., against a bacterial species of the genus Burkholderia) in a subject administered the immunogenic composition. Compositions and methods of the present invention find use in, among other things, clinical (e.g. therapeutic and preventative medicine), industrial, and research applications.

The claimed invention relates to methods of modulating mucin synthesis and the therapeutic application of compounds of Formula II in controlling mucin over-production associated with diseases such as chronic obstructive pulmonary diseases (COPD) including asthma and chronic bronchitis, inflammatory lung diseases, cystic fibrosis and acute or chronic respiratory infectious diseases.whereinX is S, N, O or CR;Y is CRR′, O, NR6, CRR′—CRR′ or CR═CR;Z is NR6, O, S, CRR′ or CRR′—CRR′;R1-R3 are independently selected from the group consisting of H, C1-C8 alkyl, C1-C8 alkoxy, amino, hydroxy, halosubstituted alky and halo;R4 isQ is CR, NR6 orR5 is H or benzyl;R6 is H, C1-C8 alkyl, C1-C8 alkoxy, OH or halo; andR and R′ are independently H, C1-C8 alkyl, C1-C8 alkoxy, OH or halo,or a pharmaceutically acceptable salt thereof.

The present invention relates to methods and compositions to treat subjects having cystic fibrosis. These compositions comprise the class of isothiocyanates. Isothiocyanates, absorbed by a cell are conjugated with glutathione GSH by glutathione-s-tranferase (GST). The conjugates are substrates of the multi-drug resistance associated (MRP) / multi-drug resistance (MDR) proteins. These proteins are functionally redundant to the cystic fibrosis transmembrane conductance regulator (CFTR), allowing for the substrate conjugates to be exported from the cell. The export of GSH conjugates restores intracellular and extracellular levels of GSH to normal levels. Normalizing both extracellular and intracellular GSH via the increased conjugation of isothiocyanates with GSH, and subsequent export, can significantly rectify numerous enzymatic processes and correct the pathologies that are typical of patients suffering from cystic fibrosis.

The present invention relates to a method of treatment of cachexia in a subject in need of treatment. More specifically, the present invention relates to the use of retinoid compounds that act on retinoid X receptors (RXRs) for the treatment of cachexia in a subject in need of treatment. The cachexia is associated with, in other words a complication of, a primary disease, condition or disorder. Primary diseases, conditions and disorders include, but are not limited to, cancer, AIDS, liver cirrhosis, diabetes mellitus, chronic renal failure, chronic obstructive pulmonary disease, chronic cardiac failure, immune system diseases (e.g., rheumatoid arthritis and systemic lupus erythematosus), tuberculosis, cystic fibrosis, gastrointestinal disorders (e.g., irritable bowel syndrome and inflammatory bowel disease), Parkinson's disease, anorexia nervosa, dementia, major depression, an aged condition and sarcopenia.

Provided are lipid antiinfective formulations substantially free of anionic lipids with a lipid to antiinfective ratio is about 1:1 to about 4:1, and a mean average diameter of less than about 1 μm. Also provided is a method of preparing a lipid antiinfective formulation comprising an infusion process. Also provided are lipid antiinfective formulations wherein the lipid to drug ratio is about 1:1 or less, about 0.75:1 or less, or about 0.50:1 or less prepared by an in line fusion process. The present invention also relates to a method of treating a patient with a pulmonary infection comprising administering to the patient a therapeutically effective amount of a lipid antiinfective formulation of the present invention. The present invention also relates to a method of treating a patient for cystic fibrosis comprising administering to the patient a therapeutically effective amount of a lipid antiinfective formulation of the present invention.

The invention describes novel nitrosated and / or nitrosylated compounds of the invention and pharmaceutically acceptable salts thereof, and novel compositions comprising at least one nitrosated and / or nitrosylated compound of the invention, and, optionally, at least one nitric oxide donor and / or at least one therapeutic agent. The invention also provides novel compositions comprising at least one compound of the invention, and at least one nitric oxide donor and / or at least one therapeutic agent. The invention also provides novel kits comprising at least one compound of the invention, that is optionally nitrosated and / or nitrosylated, and, optionally, at least one nitric oxide donor and / or at least one therapeutic agent. The invention also provides methods for (a) treating bacterial infections; (b) treating viral infections; (c) treating fungal infections; and (d) treating lesions. The nitrosated and / or nitrosylated compounds of the invention are preferably nitrosated and / or nitrosylated antimicrobial compounds, nitrosated and / or nitrosylated adenosine antagonists, nitrosated and / or nitrosylated LTB4 antagonists, nitrosated and / or nitrosylated mucoregulators and nitrosated and / or nitrosylated purine agonists. The methods of the invention are preferably for the treatment of bacterial infections associated with pulmonary diseases such as cystic fibrosis.