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Toronto Notes 2019 Respirology Pediatrics P81
• CFTRgenemutationanalysis
• diseaseoftendetectedduringnewborngeneticscreening;positiveresultrequiresDNAtestingand
subsequent sweat choride testing
Management
• nutritionalcounselling:highcaloriediet,pancreaticenzymereplacements,fatsolublevitamin supplements
• managementofchestdisease:physiotherapy,posturaldrainage,exercise,bronchodilators,aerosolized DNAase and inhaled hypertonic saline, antibiotics (e.g. cephalosporin, cloxacillin, ciprofloxacin, inhaled tobramycin depending on sputum C&S), lung transplantation
• geneticcounselling
Complications
• respiratoryfailure,pneumothorax(poorprognosticsign),corpulmonale(late),pancreaticfibrosiswith DM, gallstones, cirrhosis with portal HTN, infertility (male)
• earlydeath(currentmediansurvivalinCanadais46.6yr)
Dyspnea
Approach to Dyspnea
• determineifpatientissickornotsick;ABCs
• history:onset,previousepisodes,precipitatingevents,associatedsymptoms,pastmedical/familyhistory
of respiratory disease
• physicalexam:vitals,SpO2,evidenceofcyanosis,respiratory,cardiovascular
• investigations:CBCanddifferential,electrolytes,BUN,Cr,NPswab,ABG,CXR,ECG(basedon
clinical findings)
Upper Airway
Foreign body Croup
Laryngeal edema Epiglottitis Retropharyngeal abscess Tracheitis
Dyspnea
Figure 17. Approach to dyspnea in childhood
Pneumonia
Etiology
Pulmonary
Lower airway
Bronchiolitis Pneumonia Atelectasis Asthma
Pleura
Cardiac
CHF
Cardiac tamponade Pulmonary edema Pulmonary embolus
Other
Ó ICP Ascites Scoliosis
Pulmonary effusion Empyema Pneumothorax
• inflammationofpulmonarytissue,associatedwithconsolidationofalveolarspaces
Clinical Presentation
• incidenceisgreatestinfirstyearoflifewithviralcausesbeingmostcommoninchildren<5yr
• fever, cough, tachypnea
• CXR:diffuse,streakyinfiltratesbilaterally
• bacterialcausesmaypresentwithcough,fever,chills,dyspnea,moredramaticCXRchanges(e.g.lobar
consolidation, pleural effusion)
Management
• supportivetherapy:hydration,antipyretics,humidifiedO2