Notes in Cystic Fibrosis

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Published	10/08/2024	Cystic fibrosis is screened for at birth with the {{c1::newborn bloodspot test. }}
Published	10/08/2024	{{c1::Meconium ileus}} is often the first sign of cystic fibrosis.
Published	10/08/2024	If cystic fibrosis is not diagnosed shortly after birth it can present later with recurrent {{c1::lower}} respiratory tract infections, {{c1::failu…
Published	10/08/2024	Signs of Cystic Fibrosis • {{c1::Low}} weight or height on growth charts • Nasal {{c1::polyps}} • Finger {{c1::clubbing}} • {{c1::Crackles}} a…
Published	10/08/2024	The {{c1::sweat test}} is the key investigation for cystic fibrosis
Published	10/08/2024	The Sweat Test for CFpatch of skin is chosen & {{c2::Pilocarpine}} is applied Electrodes placed either side of the patch & a smal…
Published	10/08/2024	Patients with cystic fibrosis take long term prophylactic {{c1::flucloxacillin}} to prevent staph aureus infection.
Published	10/08/2024	{{c1::Pseudomonas}} should be remembered as a particularly troublesome coloniser that is hard to treat and worsens the prognosis of patients wi…
Published	10/08/2024	Other Treatment Options for CF • {{c1::Lung transplantation}} is an option in end stage respiratory failure• {{c1::Liver transplan}}t in liver failu…
Published	10/08/2024	Patients with cystic fibrosis are managed and followed up in specialist clinics, typically every {{c1::6 months.  }}
Published	10/08/2024	Cystic fibrosis is an autosomal {{c1::recessive}} genetic condition affecting the {{c1::mucus glands}}.  A genetic mutation of the&nbsp…
Published	10/08/2024	The key consequences of the cystic fibrosis mutation are:{{c1::Thick pancreatic and biliary}} secretions that cause blockage of the duc…
Published	10/08/2024	In about 20% of babies with CF, the meconium is {{c1::thick}} and {{c1::sticky}}, causing it to get stuck and obstruct t…
Published	10/08/2024	Symptoms of Cystic Fibrosis Lungs: Chronic {{c1::cough}} and {{c1::Thick sputum}} productionRecurrent {{c1::respiratory tract infection…
Published	10/08/2024	Causes of Clubbing in Children• Hereditary clubbing• {{c1::Cyanotic heart}} disease • Infective endocarditis • {{c1::Cystic fibrosis}}• {{c1…
Published	10/08/2024	Diagnosis of CFPostnatal:• {{c1::Newborn blood spot}} testing is performed on all children shortly after birth• {{c1::sweat test}} is the go…
Published	10/08/2024	Patients with cystic fibrosis struggle to clear the secretions in their airways. plenty of moisture and oxygen for colonies of bacteria…
Published	10/08/2024	Pseudomonas colonisation in Cystic Fibrosis can be treated with long term {{c1::nebulised::method?}} antibiotics e.g. {{c1::tobramycin}…
Published	10/08/2024	Management of Cystic Fibrosis Conservative/lifestyle:{{c1::Chest physiotherapy}}: clear mucus & reduce risk of infection {{c1::Exer…
Published	10/08/2024	Patients with cystic fibrosis require regular monitoring of their {{c1::sputum}} for colonisation of bacteria like pseudomonas.&nb…
Published	10/08/2024	Prognosis of CF• {{c1::90}}% of patients with CF develop {{c1::pancreatic}} insufficiency• {{c1::50}}% of adults with CF develop cystic fibr…
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