kma annual meeting 2010 - allergy - wheeze, crackle, pop: when its not asthma/copd

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Presentation by Rodney J. Folz, MD, PhD

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  • 1. KYSocietyofAllergy,Asthma&ClinicalImmunology 2010KMAAnnualMeeting Sept22,2010Wheeze,Crackle,Pop:Whenitsnot Asthma/COPD RodneyJ.Folz,MD,PhD Chief,DivisionofPulmonary,CriticalCareand SleepDisordersMedicine UniversityofLouisvilleSchoolofMedicine Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 2. Disclosures FundingFrom: NIH AmericanHeartAssociation Pfizer Merck BoehringerIngelheim BioMarcks GlaxoSmithKline CysticFibrosisFoundation Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 3. Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 4. Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 5. Case1 60yoWFreferredfor2nd opinion Allergicrhinits withchroniccough,asthma,andmild Significantlyimprovedwithallergyshots bronchiectasis. andmedications CC Mildasthmadx15yearsago Wellcontrolled,improvedwithallergy chroniccough shotsandICS/LABA. Waxedandwaned Minimallyproductive Mildrecurrentsinusitis NormalsinusCT recurrentpneumoniaeverycoupleof years,bronchitisover34yrs Allergies: allergicrhinosinusitisandplacedon PCN,Sulfa,Ceftin,flagyl immunotherapy Lactoseintolerant Ink,cats,dust,moldallergies bronchoscopy6yearsago Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 6. Case1 Meds: montelukast ICS/LABA Albuterol FH:+recurrentbronchitis(mother) SH: Homemaker hobbies:printmakeupwithsomeexposuretosolventsacetones/solvents PE:+bibasilarexpiratorycrackles Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 7. AdultCombinedSkinTests Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 8. Case1 MildobstructionUof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 9. TypicalFlowVolumeCurveNormalvs severeobstruction PatientNormal Severeobstruction Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 10. Case1 Findings: Coarselungmarkings withinthebase Prominentright cardiophrenicfatpadUof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 11. Case1 Findings: Scatteredparenchymal abnormalities Mildleftbase bronchiectasisUof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 12. Case1 Immunoglobulinlevels Fungalserologies:neg IGG 1180 A1AT:170 IGA 152 IGM 100 IGE 246 (High) CBC,CMP,TSH,UAnormal Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 13. Whatwouldyoudoatthispoint?ActiveDiagnoses: DDxforBronchiectasis Allergicrhinosinusitis Pneumonia,recurrent Acquiredairwayobstruction(foreign Asthma/cough body,TB,airwayadenoma,amyloid, Priorpneumonia ABPA,impaction,etc) Congenitalairwayobstruction Recurrentbronchitis mild (bronchialcyst,sequestration, Mildbronchiectasis Immunodeficiencies Chronicgranulomatousdisease Ciliarydefects Recurrentaspiration Inhalationtoxicfumes/dusts Cysticfibrosis Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 14. PilocarpineIontophoresis SweatChloride Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 15. CFTRGenotypingUof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 16. OverviewofCysticFibrosis MostcommonlethalgeneticdiseaseinCaucasian population 1:3,300 Caucasian 1:29 CarriersmutantCFTRgene(moreinothercountries e.g.NIreland,Australia) Lowerincidenceinotherpopulations 1:9,500 Hispanic 1:15,300 AfricanAmerican 1:32,100Asian ~30,000patientsinU.S. Closeto50%ofthosewithCFarenowadults Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 17. HistoryandEpidemiology 1938:CysticFibrosisofthePancreasdescribedby Andersen 1950s:Excessivesaltlossinsweatrecognizedinheat wavebydiSantAgnese 1980s:Specificiontransportabnormalities described(Boucher,Knowles,others) 1989:CFTRclonedbyCollins,Tsuigroups 1990s:Newtreatmentstrategies,genetherapy 2000s:Pathophysiology,genemodifiers,standards ofcare,qualityimprovement,noveltreaments Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 18. Median Survival CFF registry dataUof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 19. Adult with CF are increasing CFF registry report 2007 Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 20. Frequencyhistogramforageat diagnosisforthose>40years Median age at diagnosis = 13 years Rodman et al., AJRCCM 171:621-626, 2005 Uof LDivisionofPulmonary,CriticalCare,andSleepDisordersMedicine
  • 21. CFGenetics Monogenetic,autosomal recessive Carriersareasymptomatic Affectedgene CysticFibrosisTransmembrane conductanceRegulatororCFTR.

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