Notes in Cystic Fibrosis LECTURE 12

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Published	01/07/2025	Cystic fibrosis - inheritance (types of conditon){{c1::commonest inherited genetic condition}}{{c1::autosomal recessive condition}}
Published	01/07/2025	how many people in the UK have CF?{{c1::9000}}
Published	01/07/2025	which protein is affected in CF?{{c1::CFTR - Cystic Fibrosis Transmembrane Regulator Protein}}
Published	01/07/2025	why does CF occur?:- where is mutation?- which protein does it result in the defect of?{{c1::due to mutation in gene on long arm of chromosome 7}}{{c1…
Published	01/07/2025	CFTR protein (physiologically):- where does it sit and what does it regulate?-  what ions does it inhibit?{{c1::CFTR sits in membrane of epitheli…
Published	01/07/2025	CFTR protein: (pathologically)  - ∆F508 mutationwhat does it affect and cause?{{c1::affects chloride channel}}{{c1::chloride builds up in cell}}{…
Published	01/07/2025	how is CFTR protein mutated?{{c1::due to many different ways}}
Published	01/07/2025	impact of abnormal CFTR?:in terms of reaborptionhigh or low viscocity?{{c1::decreased chloride reabsorption and increased sodium reabsorption}}{{c1::s…
Published	01/07/2025	what kind of condition is CF?{{c1::multisystem}}
Published	01/07/2025	what organs are affected by CF?{{c1::respiratory system: bronchiectasis, RRIs, RF}}{{c1::gastrointestinal system: exocrine pancreatic insufficiency, d…
Published	01/07/2025	symptoms of respiratory tract in CF: upper{{c1::chronic sinus disease}}{{c1::nasal polyps}}
Published	01/07/2025	symptoms of respiratory tract in CF: lower{{c1::chronic cough and sputum}}{{c1::bronchiectasis (dilated and inflammed bronchi)}}{{c1::chronic suppurat…
Published	01/07/2025	what is bronchiectasis?what does it lead to?destruction and dilation of {{c1::distal bronchioles}} which are inflamedlots of {{c1::mucus}} w…
Published	01/07/2025	organisms involved in frequent respiratory infections:- what does it change with?{{c1::staphylococcus aureus}}{{c1::haemophilus influenza}}{{c1::pseud…
Published	01/07/2025	what is important about respiratory infections by bacteria?{{c1::colonisation is difficult to eradicate}}
Published	01/07/2025	what does this show?{{c1::arrow show dilated bronchi}}{{c1::images show bronchiectasis}}
Published	01/07/2025	CF effect on GI tract:1. Pancreas:{{c1::sticky secretions}}{{c1::blockage of ducts}}{{c1::failure to deliver digestive enzymes}}2. Intestine{{c1::stic…
Published	01/07/2025	what does destruction of pancreas and exocrine insufficiency cause?decreased {{c1::lipase activity}}{{c1::decreased absorption of fat}} in small …
Published	01/07/2025	how is thick faecal material a symptom of CF?what is it called in neonates?{{c1::decreased water content in intestines}}{{c1::causes intestinal o…
Published	01/07/2025	CF presentation in newbown:{{c1::meconium ileus}}{{c1::failure to thrive (FTT)}}{{c1::recurrent respiratory infections}}
Published	01/07/2025	other impacts of CF:infertility{{c1::azoospermia}}{{c1::failure of vas deferens to canalize}}{{c1::sub-fertility}}{{c1::psychosocial}}
Published	01/07/2025	screening for CF:{{c1::antenatal: carrier testing, genetic counselling}}{{c1::prenatal: DNA analysis, amniocentesis}}{{c1::neonatal: Guthrie test, swe…
Published	01/07/2025	CYSTIC FIBROSIS:which protein did blood spots test for in neonates?{{c1::immunoreactive trypsin (IRT)}}
Published	01/07/2025	diagnosis of CF:{{c1::clinical presentation}} (RRIs, MI, FTT){{c1::positive sweat test}}: chloride content > 60mmol/L = abnormal{{c1::abnormal…
Published	01/07/2025	how does sweat test show CF?{{c1::high chloride levels in sweat}}
Published	01/07/2025	management of CF:multisystem condition so:{{c1::specialist centre}}{{c1::MDT}}{{c1::CFT trust}}
Published	01/07/2025	treatment of respiratory disease{{c1::prompt antibiotics for RIs}}{{c1::intense chest physiotherapy (to clear phlegm)}}{{c1::nebulised DNAse - decreas…
Published	01/07/2025	management of pancreatic disease1. {{c1::exocrine:}}{{c1::creon (pancreatic enzymes) - to increase fat absorption}}{{c1::multivitamins}}{{c1::nutritio…
Published	01/07/2025	new potential treatments for CF {{c1::}}
Published	01/07/2025	what is the gene therapy for CF?what does it improve?
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