Described herein are compounds, compositions, and methods of their use for the treatment of cystic fibrosis.

This invention provides compounds of the formula I: ##STR00001##
and their pharmaceutically acceptable salts, useful as sodium channel blockers, compositions containing the same, therapeutic methods and uses for the same and processes for preparing the same.

The present invention relates to the compound of the formula: ##STR00001##
or pharmaceutically acceptable salts thereof, as well as compositions containing the same, processes for the preparation of the same, and therapeutic methods of use therefore in promoting hydration of mucosal surfaces and the treatment of diseases including chronic obstructive pulmonary disease (COPD), asthma, bronchiectasis, acute and chronic bronchitis, cystic fibrosis, emphysema, and pneumonia.

The present invention relates to the compound of the formula: ##STR00001##
or pharmaceutically acceptable salts thereof, as well as compositions containing the same, processes for the preparation of the same, and therapeutic methods of use therefore in promoting hydration of mucosal surfaces and the treatment of diseases including chronic obstructive pulmonary disease (COPD), asthma, bronchiectasis, acute and chronic bronchitis, cystic fibrosis, emphysema, and pneumonia.

The present invention relates to ENaC inhibitors (e.g., compounds of Formula (I), and pharmaceutically acceptable salts, stereoisomers, tautomers, isotopically labeled derivatives, solvates, hydrates, polymorphs, co-crystals, and prodrugs thereof). Also disclosed are compositions, methods of preparation, combination therapies, kits, uses, and methods. Exemplary uses include promoting hydration of mucosal surfaces and treating diseases and disorders including chronic obstructive pulmonary disease (COPD), asthma, bronchiectasis, acute and chronic bronchitis, cystic fibrosis, primary ciliary dyskinesia, idiopathic pulmonary fibrosis, and pneumonia.

##STR00001##

The present invention relates to ENaC inhibitors (e.g., compounds of Formula (I), and pharmaceutically acceptable salts, stereoisomers, tautomers, isotopically labeled derivatives, solvates, hydrates, polymorphs, co-crystals, and prodrugs thereof). Also disclosed are compositions, methods of preparation, combination therapies, kits, uses, and methods. Exemplary uses include promoting hydration of mucosal surfaces and treating diseases and disorders including chronic obstructive pulmonary disease (COPD), asthma, bronchiectasis, acute and chronic bronchitis, cystic fibrosis, primary ciliary dyskinesia, idiopathic pulmonary fibrosis, and pneumonia.

##STR00001##

The present invention provides methods for the purification of the compound of Formula (I) from the precipitated reaction product comprising the compound of Formula (I), impurities, and/or residual or entrained solvents.

The present invention provides methods for the purification of the compound of Formula (I) from the precipitated reaction product comprising the compound of Formula (I), impurities, and/or residual or entrained solvents.