Cystic Fibrosis. Cystic Fibrosis. Cystic Fibrosis 5/01/2011 CYSTIC FIBROSIS OF THE PANCREAS AND ITS RELATION TO CELIAC DISEASE. D ANDERSEN.
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1 1938 OF THE PANCREAS AND ITS RELATION TO CELIAC DISEASE. D ANDERSEN. American Journal Diseases Children. : The beginning May 1938: 49 cases Nos of cases Age at death Commonest lethal inherited condition in WW Life limiting and incurable. 600 patients t in Victoria i with CF. One in every 2,500 live births About new born pts per year Median age survival late 30 s 95% survive till 20 years 80% till 30 years 1
2 New Born screening: Hits All states of Australia Day 3 2 part test: IRT and CF gene Positive results by 4-6 weeks New Born screening 2 part test: IRT top 1% CF genotype CF gene: Over 1500 different mutations Δ508 commonest - present in 90% pts in Australia Current Vic program screens for 13 commonest Mutations screened for differ between states 5 Classes Class 1: Premature termination of mrna translation 2
3 Class 1: Premature termination of mrna translation due to base substitution causing stop codons Class 1: Premature termination of mrna translation due to base substitution causing stop codons or mutations that shift the reading frame Producing defective proteins Class 1: Premature termination of mrna translation due to base substitution causing stop codons or mutations that shift the reading frame Producing defective proteins G542X and W1282X Class 2: Defects in protein processing CFTR is degraded in ER and does not reach apical membrane 508 and N1303K 3
4 Class 3: Regulatory mutations CFTR reaches apical membrane but fails respond to activation signals G551D Class 4: Defective protein conduction Reaches apical membrane but has altered channel properties R117H and R347P Class 5: Reduced levels of RNA for CFTR 4
5 New Born screening: Misses Chronic Cough New Born screening: Misses Chronic Cough Failure to thrive 5
6 New Born screening: Misses Chronic Cough Failure to thrive Chronic Diarrhoea Underlying defect in CF 6
7 Mechanisms of lung damage in CF Infection CFTR dysfunction Viscous secretions Inflammation Aim to minimise/ delay onset disease thru new born screening Lung damage Abnormal lung function Death Early intervention in CF patients prevents severe malnutrition Wisconsin CF Neonatal Screening Study Randomised controlled trial with 650,000 newborns. Split into two groups: an early-diagnosis screened cohort a standard diagnosis control group Newborns screened for CF by immunoreactive trypsinogen (IRT) test ( ) IRT test + DNA-based detection of ΔF508 ( ) t SDS Weight Effect of screening on weight Screened Unscreened p<0.05 Aim to minimise thru NBS Regular assessments: BAL CT Scans PFT s Regular assessments: BAL 30% infants will have Pseudomonas by age 3 Linnane B 7
8 Regular assessments: BAL 30% infants will have Pseudomonas by age 3 despite aggressive cross infection policies Regular assessments: BAL 30% infants will have Pseudomonas by age 3 despite aggressive cross infection policies Aggressive eradication programs 85% successful CF update +ve sample culture for P. aeruginosa? IV s 3 months Nebulised tobramycin Cipro Cipro POSITIVE Reculture: Sputum or BAL NEGATIVE Regular assessments: BAL 30% infants will have Pseudomonas by age 3 despite aggressive cross infection policies Aggressive eradication programs 85% successful Pseudomonas EVER is a bad prognostic factor 2 weeks of IV antipseudomonal antibiotics and then repeat No further treatment or back to prophylactic antibiotics If following second attempt of eradication sputum or BAL is still positive is likely chronic Pa infection Regular assessments: CT scans 90% infants will show signs of bronchiectasis on CT at age 5 years Regular assessments: PFT s Changes occur very early and are progressive Wainwright C 8
9 Linnane B Future management? Trials anti inflammatory Medication Future management? Trials anti inflammatory Medication - AZT CF update Antibiotic therapy in CF Targeted Non Pseudomonal: Staph/ H Inf Incidence airway pathogens by age tures (%) Total cult S. aureus H. influenzae >35 Age group (years) 9
10 B. CEPACIA Genomovars (1-9) Aggressive lung disease (cenocepacia type 3) Non 3 mild disease Bactrim Some clearance ASPERGILLUS ABPA Airway colonisation Aspergilloma Infection in pre existing cyst Incidence airway pathogens by age tures (%) Total cult P. aeruginosa S. aureus H. influenzae >35 Age group (years) 10
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