Cystic Fibrosis - Cystic Fibrosis Advances and Asian...

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Cystic Fibrosis Advances and Asian Perspective Dr AS Paul 13 October 06
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Cystic Fibrosis An Overview Asian Perspective Advances
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Cystic Fibrosis An Overview
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Epidemiology and Pathogenesis Most common severe AR disorder in Caucasians Carrier rate of 1:25/Incidence of 1:2500 Mutation affecting gene on long arm of chromosome 7 CFTR - Cystic fibrosis transmembrane conductance regulator – a chloride channel- essential for salt and water movement across membranes.
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Δ F508 most common mutation-67% UK Numerous others in the same region Increased sweat sodium chloride Increased potential difference across respiratory epithelium detected in the nose Increased viscosity of secretions/ciliary dysfunction/chronic infection and bronchiectasis
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Disorders in the gut epithelium/ pancreas/liver (malabsorption/DM/cirrhosis) Infertility in most men Lung fn normal at birth but deteriorates. Staph and Pseudomonas predominate
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Why is it so common? Carrier advantage in E coli diarrhea and cholera.
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Management Principles Lung Disease Maintaining function Monitoring bacteriology Transplant when appropriate Nutrition Nutritional support/ weight/enzymes GI Portal HT/Biliary cirrhosis
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Management Endocrine CFRD Osteporosis Fertility advice Psychosocial well being
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CF Pulmonary Sepsis Causes much mortality/morbidity Chronic colonization at an early age with bronchiectasis by 5 years FEV 1 best marker of deterioration/ improves with antibiotics To assess decline/ exacerbation/ antibiotic response.
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Organisms Colonization changes over time. Goals Prevent Eradicate Control H influenzae/ S aureus/ P aeruginosa(non mucoid/ mucoid) Burkholderia cepacia/MRSA/NTM
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Antibiotics Higher doses and durations Clinical response guided by cultures Macrolides At home IV antibiotics/ usual course 2 wks
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Other pulmonary interventions Physiotherapy DNAse Oxygen and NIV Steroids: ABPA/ exacerbations/ terminally Immunization: influenza/ pneumococcal
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Transplant Life expectancy less than 2 years FEV 1 less than 30% Hypoxia and hypercapnia Young female patients deteriorate faster Infection/ acute rejection Bronchiolitis obliterans 40% 1 year survival 58%
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Other pulmonary problems Pneumothorax ABPA Non-tuberculous mycobacteria CF asthma Haemoptysis
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Prognosis improving with better control of bronchial sepsis Median survival is about 40 years for those born in the nineties Gene therapy
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Asian Perspective Are North Indians Caucasian? 1:2500 White Americans 1:15000 Black Americans 1:31000 Asian Americans Unknown in Indians Low index of suspicion and therefore late diagnosis
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Cystic fibrosis in North Indian Children Singh M et al; Indian J Pediatr 2002; 69(7):627-629 Bhakoo 1968 Chandigarh 1 Mehta 1969 Chandigarh 13 Reddy 1970
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