Cystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorFibrosisPseudomonas InfectionsPseudomonas aeruginosaSweatSputumMice, Inbred CFTRBurkholderia InfectionsBurkholderia cepaciaChloridesEndomyocardial FibrosisIdiopathic Pulmonary FibrosisLungExocrine Pancreatic InsufficiencyLiver CirrhosisChloride ChannelsRespiratory MucosaTobramycinNasal MucosaPancreatinNeonatal ScreeningForced Expiratory VolumeRetroperitoneal FibrosisBurkholderia cepacia complexRespiratory Function TestsPancreatic ExtractsRespiratory SystemBronchiectasisMutationLung DiseasesEpithelial CellsBronchiLung TransplantationMucusAnti-Bacterial AgentsHeterozygote DetectionMeconiumExpectorantsAlginatesBleomycinPancrelipaseTracheaAchromobacter denitrificansMucociliary ClearanceBurkholderia cenocepaciaSweat GlandsDisease Models, AnimalAminophenolsAspergillosis, Allergic BronchopulmonaryIon TransportLeukocyte ElastaseOral Submucous FibrosisVital CapacityPhenotypeCell LineRespiratory Tract InfectionsLiver Cirrhosis, ExperimentalGenotypeAmilorideAdministration, InhalationGenetic TestingEpitheliumDrainage, PosturalCells, CulturedHexuronic AcidsColistinStenotrophomonas maltophiliaGlucuronic AcidHydroxyprolineChronic DiseaseExocrine GlandsMolecular Sequence DataMucinsBurkholderiaBronchoalveolar Lavage FluidColforsinRespiratory TherapyHepatic Stellate CellsTime FactorsMice, Inbred C57BLMembrane ProteinsInfant, NewbornUreteral ObstructionTrypsinogenPolymerase Chain ReactionCyclic AMPBicarbonatesMyofibroblastsGenetic CounselingTransforming Growth Factor betaHeterozygoteRNA, MessengerBiopsyBronchopneumoniaFibroblastsCollagenCarbon TetrachlorideSeverity of Illness IndexHeart-Lung Transplantation