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NURS 203
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PANCREAS Cystic Fibrosis Cystic fibrosis – growth alteration b/c mucous in ducts of the pancreas. See atrophy b/c block lumen of exocrine ducts, and pressure goes back to the glands and that pressure atrophies the glands, leading to malabsorption. Can cystic fibrosis also lead to diabetes? Yes – b/c eventually fibrose off the islet cells, leading to type I diabetes, too. Molecular bio: c’some 7 with 3 nucleotide deletion, and those 3 nucleotides codes for phenylalanine. So, you are def of phenylalanine in the cystic fibrosis transmembrane regulator protein (CFTR). So, all its missing is phenylalanine. Most things, after they are made in the ribosome in the RER, have posttranslational modifications in the Golgi apparatus, which is where the real defect is. The real problem is when it gets to the Golgi apparatus – it’s supposed to be modified and secreted to the cell surface. It ends up being degraded in the cell, and you end up having the CFTR. So, the prob is in the Golgi apparatus – it screws it up, and never makes it to the surface, therefore has no function. So, what does it do? In the sweat glands, normally, it would reabsorb Na and Cl out of the sweat gland. B/c they are def in this, they are losing salt, which is the basis of the sweat test. 3 y/o kid, failure to thrive, chronic diarrhea, resp infection, mom states that the baby taste’s salty when she kisses the baby. This is the give away for CF, b/c they lose considerable salt and become salt depleted when they are overheated. Why are all the secretions so thick in the lungs, pancreas, and bile ducts? CFTR regulator – what does it do? – In lungs, need to have salt and secretions in the lumens of the resp tract to keep it viscous (to keep it nice and loose); if you are missing CFTR, Na is reabsorbed OUT of the secretions in the airway (therefore a lil dehydrated). And, chloride cannot be pumped into the lumen of the airway – so you are taking away the 2 imp ingredients with this pump: taking Na out and not putting Cl in. Therefore these secretions are thick like concrete. The same is true for secretions in the pancreas (Na pumped out and Cl not put in). MCC death = pseudomonas aeruginosa. Fertility: what is chance of male with cystic fibrosis having children? 0- 5% (most are infertile); for females, they can get pregnant, but onl
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