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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± Are North Indians Caucasian? ± 1:2500 White Americans
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Cystic Fibrosis - Cystic Fibrosis Advances and Asian...

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