Cystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorFibrosisPseudomonas InfectionsPseudomonas aeruginosaSweatSputumMice, Inbred CFTRBurkholderia InfectionsBurkholderia cepaciaChloridesEndomyocardial FibrosisIdiopathic Pulmonary FibrosisLungExocrine Pancreatic InsufficiencyLiver CirrhosisChloride ChannelsRespiratory MucosaTobramycinNasal MucosaADP-Ribosylation Factor 1Neonatal ScreeningForced Expiratory VolumeRetroperitoneal FibrosisBurkholderia cepacia complexRespiratory Function TestsPancreatic ExtractsRespiratory SystemBronchiectasisMutationLung DiseasesEpithelial CellsBronchiLung TransplantationMucusAnti-Bacterial AgentsHeterozygote DetectionMeconiumExpectorantsAlginatesBleomycinPancrelipaseKyphoplastyAchromobacter denitrificansMucociliary ClearanceBurkholderia cenocepaciaSweat GlandsDisease Models, AnimalAminophenolsAspergillosis, Allergic BronchopulmonaryIon TransportLeukocyte ElastaseOral Submucous FibrosisVital CapacityAppointments and SchedulesCell 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