01A.05 · Mucoviscidose V — atteinte pulmonaire

Cystic fibrosis V — pulmonary involvement

Cystic fibrosis series · 39 pages

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Cystic Fibrosis: Pulmonary Involvement

1Cystic Fibrosis: Pulmonary Involvement 2Multi-System Involvement 3Improved Survival

Lung Anatomy Review

4Airway Branching and Gas Exchange 5Pulmonary Ultrastructure 6Bronchial Epithelium

CFTR and Pathophysiology

7CFTR Expression in Epithelia 8Ion Transport Defect and Mucus Dehydration 9Abnormal Ciliary Beating and Clearance Defect 10Mucus Adhesion and Mucus Plugs 11Consequences I: Infection and Inflammation Cycle 12Consequences II: Obstruction and Bronchiectasis 13Bronchiectasis

Bacterial Colonization

14From Abnormal Clearance to Colonization 15Chronic Colonization by Age 16Pseudomonas: Mucoid Conversion

17Pathophysiology Summary

Clinical Assessment

18Assessment Overview 19Clinical Signs

20Chest X-Ray 21Thoracic CT Scan

Pulmonary Function Tests

22Spirometry and Plethysmography 23Lung Volumes and Obstructive Pattern 24Longitudinal FEV1 Decline 25FEV1 Decline as Prognostic Factor

Clinical Assessment

26Sputum Microbiology 27General Impact

Respiratory Management

28Treatment Principles 29Physiotherapy: Cornerstone of Treatment 30Autogenic Drainage and Devices 31Inhaled Treatments and Antibiotics 32Daily Treatment Burden and Compliance

CFTR Modulator Therapy

33Potentiators and Correctors 34Trikafta: Clinical Trial Results 35Individual FEV1 and Sweat Test Response 361-Year Imaging Follow-Up 37Population Outcomes: IV Antibiotics and Transplant

38Conclusion 39Thank You

01A.05Page 1Cystic Fibrosis: Pulmonary Involvement

Mucoviscidose V — atteinte pulmonaire page 1

01A.05Page 2Cystic Fibrosis: Pulmonary InvolvementMulti-System Involvement

Mucoviscidose V — atteinte pulmonaire page 2

01A.05Page 3Cystic Fibrosis: Pulmonary InvolvementImproved Survival

Mucoviscidose V — atteinte pulmonaire page 3

01A.05Page 4Cystic Fibrosis: Pulmonary InvolvementLung Anatomy ReviewAirway Branching and Gas Exchange

Mucoviscidose V — atteinte pulmonaire page 4

01A.05Page 5Cystic Fibrosis: Pulmonary InvolvementLung Anatomy ReviewPulmonary Ultrastructure

Mucoviscidose V — atteinte pulmonaire page 5

01A.05Page 6Cystic Fibrosis: Pulmonary InvolvementLung Anatomy ReviewBronchial Epithelium

Mucoviscidose V — atteinte pulmonaire page 6

01A.05Page 7Cystic Fibrosis: Pulmonary InvolvementCFTR and PathophysiologyCFTR Expression in Epithelia

Mucoviscidose V — atteinte pulmonaire page 7

01A.05Page 8Cystic Fibrosis: Pulmonary InvolvementCFTR and PathophysiologyIon Transport Defect and Mucus Dehydration

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01A.05Page 9Cystic Fibrosis: Pulmonary InvolvementCFTR and PathophysiologyAbnormal Ciliary Beating and Clearance Defect

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01A.05Page 10Cystic Fibrosis: Pulmonary InvolvementCFTR and PathophysiologyMucus Adhesion and Mucus Plugs

Mucoviscidose V — atteinte pulmonaire page 10

01A.05Page 11Cystic Fibrosis: Pulmonary InvolvementCFTR and PathophysiologyConsequences I: Infection and Inflammation Cycle

Mucoviscidose V — atteinte pulmonaire page 11

01A.05Page 12Cystic Fibrosis: Pulmonary InvolvementCFTR and PathophysiologyConsequences II: Obstruction and Bronchiectasis

Mucoviscidose V — atteinte pulmonaire page 12

01A.05Page 13Cystic Fibrosis: Pulmonary InvolvementCFTR and PathophysiologyBronchiectasis

Mucoviscidose V — atteinte pulmonaire page 13

01A.05Page 14Cystic Fibrosis: Pulmonary InvolvementBacterial ColonizationFrom Abnormal Clearance to Colonization

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01A.05Page 15Cystic Fibrosis: Pulmonary InvolvementBacterial ColonizationChronic Colonization by Age

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01A.05Page 16Cystic Fibrosis: Pulmonary InvolvementBacterial ColonizationPseudomonas: Mucoid Conversion

Mucoviscidose V — atteinte pulmonaire page 16

01A.05Page 17Cystic Fibrosis: Pulmonary InvolvementPathophysiology Summary

Mucoviscidose V — atteinte pulmonaire page 17

01A.05Page 18Cystic Fibrosis: Pulmonary InvolvementClinical AssessmentAssessment Overview

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01A.05Page 19Cystic Fibrosis: Pulmonary InvolvementClinical AssessmentClinical Signs

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01A.05Page 20Cystic Fibrosis: Pulmonary InvolvementClinical AssessmentRadiologyChest X-Ray

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01A.05Page 21Cystic Fibrosis: Pulmonary InvolvementClinical AssessmentRadiologyThoracic CT Scan

Mucoviscidose V — atteinte pulmonaire page 21

01A.05Page 22Cystic Fibrosis: Pulmonary InvolvementClinical AssessmentPulmonary Function TestsSpirometry and Plethysmography

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01A.05Page 23Cystic Fibrosis: Pulmonary InvolvementClinical AssessmentPulmonary Function TestsLung Volumes and Obstructive Pattern

Mucoviscidose V — atteinte pulmonaire page 23

01A.05Page 24Cystic Fibrosis: Pulmonary InvolvementClinical AssessmentPulmonary Function TestsLongitudinal FEV1 Decline

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01A.05Page 25Cystic Fibrosis: Pulmonary InvolvementClinical AssessmentPulmonary Function TestsFEV1 Decline as Prognostic Factor

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01A.05Page 26Cystic Fibrosis: Pulmonary InvolvementClinical AssessmentSputum Microbiology

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01A.05Page 27Cystic Fibrosis: Pulmonary InvolvementClinical AssessmentGeneral Impact

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01A.05Page 28Cystic Fibrosis: Pulmonary InvolvementRespiratory ManagementTreatment Principles

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01A.05Page 29Cystic Fibrosis: Pulmonary InvolvementRespiratory ManagementPhysiotherapy: Cornerstone of Treatment

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01A.05Page 30Cystic Fibrosis: Pulmonary InvolvementRespiratory ManagementAutogenic Drainage and Devices

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01A.05Page 31Cystic Fibrosis: Pulmonary InvolvementRespiratory ManagementInhaled Treatments and Antibiotics

Mucoviscidose V — atteinte pulmonaire page 31

01A.05Page 32Cystic Fibrosis: Pulmonary InvolvementRespiratory ManagementDaily Treatment Burden and Compliance

Mucoviscidose V — atteinte pulmonaire page 32

01A.05Page 33Cystic Fibrosis: Pulmonary InvolvementCFTR Modulator TherapyPotentiators and Correctors

Mucoviscidose V — atteinte pulmonaire page 33

01A.05Page 34Cystic Fibrosis: Pulmonary InvolvementCFTR Modulator TherapyTrikafta: Clinical Trial Results

Mucoviscidose V — atteinte pulmonaire page 34

01A.05Page 35Cystic Fibrosis: Pulmonary InvolvementCFTR Modulator TherapyIndividual FEV1 and Sweat Test Response

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01A.05Page 36Cystic Fibrosis: Pulmonary InvolvementCFTR Modulator Therapy1-Year Imaging Follow-Up

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01A.05Page 37Cystic Fibrosis: Pulmonary InvolvementCFTR Modulator TherapyPopulation Outcomes: IV Antibiotics and Transplant

Mucoviscidose V — atteinte pulmonaire page 37

01A.05Page 38Cystic Fibrosis: Pulmonary InvolvementConclusion

Mucoviscidose V — atteinte pulmonaire page 38

01A.05Page 39Cystic Fibrosis: Pulmonary InvolvementThank You

Mucoviscidose V — atteinte pulmonaire page 39