Abstract: Methods are disclosed for removing retained mucus secretions from the lungs of a human subject. The method includes (1) generating an aerosol suspension of respirable solid, dry particulate amiloride, and (2) delivering by inhalation the aerosol suspension to the respiratory system of the subject. The solid particulate amiloride is comprised of particles of about 1 to 5 microns and is free of liquid amiloride particles. The aerosol suspension of respirable solid particulate amiloride is administered in an amount sufficient to achieve concentrations of amiloride on the airway surfaces of from about 10.sup.-7 to 10.sup.-3 Moles/liter. As a result of the treatment, the secretions are hydrated and more easily transported from the lung via mucociliary action. Methods are also disclosed for the treatment of cystic fibrosis in humans.

Abstract: Substituted sulfonimidamides, processes for their preparation, their use as a medicament or diagnostic, and medicament comprising themSulfonimidamides of the formula I ##STR1## in which at least one of the three substituents R(1), R(2) and R(3) is a benzoylguanidine, and in which the other substituents have the meanings indicated in the claims, are outstandingly suitable as medicaments having action on the cardiovascular system; that is as antiarrhythmic pharmaceuticals having a cardioprotective component as well as for treating ischemically induced damage; also in operative interventions, such as organ transplantation.

Abstract: The use of phospholipids of the following formula to reduce the viscosity of mucus in a patient is described: ##STR1## wherein one of X, Y, or Z represent: ##STR2## in which each R represents hydrogen or methyl, and each of the other two of X, Y, or Z represents --CO--R.sup.1 in which R.sup.1 represents linear or branched C.sub.11-21 alkyl or C.sub.11-21 alkenyl, unsubstituted or substituted with one or more substituents selected from the group consisting of halo, C.sub.1-6 linear or branched alkoxy or cyano.

Abstract: Pharmaceutical compositions comprising an inorganic pyrophosphate (PPi) in a form that renders the PPi accessible to ABC proteins when administered to a subject in vivo, as well as use of the compositions for treating subjects having a disease or disorder associated with inappropriate or inadequate ABC protein activity (e.g., cystic fibrosis, multi drug resistance, Zellweger's Syndrome) is disclosed.

Abstract: Methods and compositions for treating Cystic Fibrosis by mobilizing mutant forms of CFTR, which retain at least some functional activity, to the plasma membrane where they can mediate chloride ion transport are disclosed.

Abstract: The present invention relates to a method to protect an animal from a disease involving inflammation by treating that animal with an effective amount of IL-12. The present invention also relates to a method for prescribing treatment for a respiratory disease involving an inflammatory response and a method for monitoring the success of a treatment for a respiratory disease involving an inflammatory response in an animal. Also included in the present invention is a formulation comprising IL-12 and a compound capable of enhancing the effectiveness of the IL-12 at protecting an animal from a disease involving inflammation.

Abstract: Method of hydrating lung mucous secretions in the lungs of a subject are disclosed. The methods involve administering benzamil or phenamil to the lungs of the subject in an amount effective to hydrate lung mucous secretions. The administering step is preferably carried out by inhalation administration. The method is useful in the treatment of diseases such as cystic fibrosis and chronic bronchitis.

Abstract: A compound of Formula (I), or a pharmaceutically acceptable salt thereof, is disclosed: ##STR1## wherein: n is from 1 to 6;X is --OH or --SH;A and B are each independently selected from the group consisting of: ##STR2## wherein R is H or Br. The compounds are useful in the treatment of airway diseases such as cystic fibrosis. Pharmaceutical formulations comprising a compound of Formula (I), and methods of hydrating mucous secretions in the lungs of a subject in need of such treatment, comprising administering to the lungs of the subject a compound of Formula I as given above, are also disclosed.

Abstract: A method and composition for treating cystic fibrosis comprising administering to a patient a first component, a second component, and preferably a third component. The first component is an inhibitor which is specific for a cGMP-inhibited type III cAMP phosphodiesterase, preferably milrinone or amrinone; the second component is an adenylate cyclase activator, preferably forskolin, isoproterenol or albuterol; the third component is cAMP or a cAMP analog which activates protein kinase A.

Abstract: A substantially homogeneous protein having cystic fibrosis transmembrane conductance regulator activity is provided. Also provided is a therapeutically effective composition for treating a subject having cystic fibrosis.

Abstract: In a preferred embodiment, drugs having pharmacological properties which are useful in the medicinal therapy of fibrotic disease for the reparation and prevention of fibrotic lesional tissues, such drugs including as the active ingredient one or more N-substituted 2-(1H) pyridone(s). The composition of this invention is novel as an anti-fibrotic drug, namely, as an agent for treating fibrosis. Any existing compounds have not been shown to be effective for the reparation and prevention of fibrotic lesions.

Abstract: Methods for reducing the risk of or preventing infections by bacterial pathogens in vivo. In particular, a method for reducing the risk of P. aeruginosa infection in vivo in compromised hosts such as cystic fibrosis patients. The methods involve the use of dextran or dextran sulphate as the active ingredient.

Abstract: A method of facilitating the clearance of retained pulmonary secretions in a subject using lantibiotics is disclosed. The method comprises administering to the lungs of the subject an effective amount of a lantibiotic. The lantibiotic is preferably administered by topically applying it to the respiratory epithelia, such as by generating an aerosol thereof which is then inhaled by the subject. A preferred lantibiotic for carrying out the present invention is duramycin. The method may be used in treating disorders such as cystic fibrosis, chronic bronchitis, and asthma. Also disclosed is a method of combatting tuberculosis comprising administering a lantibiotic to a subject in need of such treatment.

Abstract: O-derivatized alginic acid antigens capable of eliciting opsonizing antibodies in vivo are described. The O-derivatized antigens show enhanced antigenicity and immunogenicity relative to native, non-O-derivatized alginic acid antigens, particularly the MEP antigen of P. aeruginosa. Pharmaceutical compositions containing the O-derivatized antigens of the invention are also described. The invention also pertains to use of the compositions as vaccines, in immunodiagnostic assays, and in methods for producing monoclonal antibodies reactive against the antigen.Methods for forming O-derivatized alginic acid antigens of the invention are described. A non-O-derivatized starting material is reacted in a solvent with an alkyl anhydride derived from a fatty acid.

Abstract: Stable, cold water-dispersible, liquid or pulverous compositions of fat-soluble substances which contain fish gelatin as the protective colloid and a method of making same are described.

Abstract: A method and medicament for the inhibition of oxidants comprising administering a treatment effective amount of alkylaryl polyether alcohol polymers to a chemical or biologic system in need thereof. The medicament is preferably administered by aerosolization into the mammalian respiratory system. The medicament may also be applied to the mammalian skin. Preferably, the medicament includes a physiologically acceptable carrier which may be selected from the group consisting of physiologically buffered saline, isotonic saline, normal saline, petrolatum based ointments and U.S.P. cold cream.

Abstract: The invention is generally directed to methods of promoting normal respiratory tract airflow in subjects with restricted airflow and ciliary clearance caused by the presence of pathological airway contents, and particularly mucoid contents. Actin-binding proteins are administered into the respiratory tract of a subject with a pathological respiratory condition involving the presence of such contents. The actin-binding protein binds to actin polymers in the contents and decreases the viscosity. The actin binding proteins also prevent actin from binding to exogenous or endogenous DNase, thus increasing the degradation of DNA polymers in the contents.

Abstract: A novel therapy for Pseudomonas aeruginosa infections, in particular in cystic fibrosis patients, is described. Macrophages provide the first line of defence in protecting the lung against bacterial infections. Nonetheless, Pseudomonas aeruginosa infections continue to be problematic in cystic fibrosis patients and are the leading cause of death in these patients. Previously, little was known regarding the factors which regulate the capacity of macrophages to mediate phagocytosis of Pseudomonas aeruginosa. The inventors have now shown that phagocytosis of nonopsonized Pseudomonas aeruginosa by macrophages is dependent upon the presence of glucose. They have also shown that the action of glucose is on the macrophage rather than the bacteria. Glucose therapy can therefore be used to prevent or combat Pseudomonas aeruginosa infections.

Abstract: Oligomers of the formula ##STR1## have demonstrated effectiveness in preventing connective tissue damage.

Abstract: In a preferred embodiment, drugs having heretofore unknown pharmacological properties which are useful in the medicinal therapy of fibrotic disease for the reparation and prevention of fibrotic lesional tissues, such drugs including as the active ingredient 5-methyl-1-phenyl-2-(1H)-pyridone. The pharmaceutical composition of this invention is novel as an anti-fibrotic drug, namely, as an agent for treating fibrosis. Any existing compounds have not been shown to be effective for the reparation and prevention of fibrotic lesions.

Abstract: The present invention provides a new medical use for the phenethanolamine compound 4-hydroxy-.alpha..sup.1 -[[[6-(4-phenylbutoxy)hexyl]amino]methyl]-1,3-benzenedimethanol and physiologically acceptable salts and solvates thereof in the treatment of inflammation, allergy and allergic reaction.

Abstract: A method of using an imidazoline compound, which is a vasodilator and an alpha-adrenergic blocking agent, for the treatment of symptoms of chronic obstructive pulmonary diseases (COPD), including cystic fibrosis, chronic bronchitis and emphysema, or COPD where it is associated with asthma, comprises administering the imidazoline to a patient by inhalation. Preferably, the imidazoline compound employed is tolazoline. The presentation of an imidazoline compound in a device for the administration of an aerosol is disclosed.

Abstract: The present invention relates to a method of treating diseases associated with elevated levels of interleukin 1 (IL-1) including inflammatory diseases such as arthritis, skin hypersensitivity and endotoxemia. More specifically, the invention relates to a method for the treatment of such diseases in warm-blooded animals, such as humans, which comprises the administration of a therapeutically effective amount of an aromatic diamidine, sufficient to inhibit IL-1 release from IL-1 producing cells. The aromatic diamidine can also be used to block interleukin 6 (IL-6) and tumor necrosis factor from cells producing these cytokines.

Abstract: 4-R.sup.4 -R.sup.5 -2-Saccharinylmethyl aryl carboxylates, useful in the treatment of degenerative diseases, are prepared by reacting a 4-R.sup.4 -R.sup.5 -2-halomethylsaccharin with an arylcarboxylic acid in the presence of an acid-acceptor.

Abstract: New substituted azetidinones of the general formula (A), which have been found to be potent elastase inhibitors and thereby useful as anti-inflammatory and antidegenerative agents, are described.

Abstract: New substituted azetidinones of the general formula (A), which have been found to be potent elastase inhibitors and thereby useful as anti-inflammatory and antidegenerative agents, are described.

Abstract: Methods for the therapy of cystic fibrosis, Bartter's syndrome, and secretory diarrheas, and for diuretic treatment, by administering to a patient dodecahydro-7,14-methano-2H,6H-di-pyrido[1,2-a:1',2'-e][1,5]diazocine or a pharmaceutically acceptable derivative thereof are disclosed. The formulations include an aerosol formulation comprising the active ingredient in association with an aerosol propellant.

Abstract: A method of using an imidazoline compound, which is a vasodilator and an alpha-adrenergic blocking agent, for the treatment of symptoms of chronic obstructive pulmonary diseases (COPD), including cystic fibrosis, chronic bronchitis and emphysema, or COPD where it is associated with asthma, comprises administering the imidazoline to a patient by inhalation. Preferably, the imidazoline compound employed is tolazoline. The presentation of an imidazoline compound in a device for the administration of an aerosol is disclosed.

Abstract: A method of treatment of cystic fibrosis which comprises administration to a patient suffering from that condition of a therapeutically effective quantity of 9-ethyl-6,9-dihydro-4,6-dioxo-10-propyl-4H-pyrano(3,2-g)quinoline-2,8-dica rbo xylic acid or a pharmaceutically acceptable derivative thereof.Pharmaceutically acceptable salts of the active ingredient which may be used include alkali metal salts, in particular the di-sodium salt which is known as nedocromil sodium.

Abstract: The invention relates to an oral composition for alleviating digestive manifestations in patients afflicted with cystic fibrosis, which comprises a therapeutic amount of taurine in association with a pharmaceutically acceptable carrier, and to a method of alleviating digestive manifestations in patients afflicted with cystic fibrosis by the administration of said oral composition.

Abstract: The present invention provides certain novel substituted naphthalenes, indoles, benzofurans, and benzothiophenes of Formula I which are useful as inhibitors of leukotriene biosynthesis and as inhibitors of lipoxygenase. They are thus employed wherever it is medically necessary or desirable to inhibit these systems.