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Cystic Fibrosis

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benryan95's version from 2016-01-13 16:14

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Question Answer
What inheritance pattern does Cystic Fibrosis have?Autosomal recessive
How many live births have cystic fibrosis?1 in 2000
What 3 clinical features do neonates with cystic fibrosis have?Failure to thrive, meconium ileus, rectal prolapse
What 8 respiratory clinical features can children and young adults with CF have?cough, wheeze, recurrent infections, bronchiectasis, pneumothorax, haemoptysis, respiratory failure, cor pulmonale
What 4 GI clinical features can children and young adults with CF have?Pancreatic insufficiency (diabetes mellitus, steatorrhoea), distal intestinal obstruction syndrome (meconium ileus equivalent), gallstones, cirrhosis
What 7 other clinical features can children and young adults with CF have?Male infertility, osteoporosis, arthritis, vasculitis, nasal polyps, sinusitis and hypertrophic pulmonary osteoarthropathy
What are 3 signs of CF in children and young adults?cyanosis, finger clubbing and bilateral coarse crackles
Name 3 investigations used in diagnosis of CF?Sweat test, genetics, faecal elastase (exocrine pancreatic dysfunction)
Name 6 blood tests important in CFFBC, U&E, LFT, clotting, vitamin A, D and E, glucose tolerance
What can CF show on CXR?hyperinflation, bronchiectasis
What pattern is observed in CF on spirometry?obstructive
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