17 Cystic Fibrosis
- name from fibrous cyst found in pancreas
- abnormal: sticky mucosal surfaces because not enough water
- abnormal folding: protein targeted for ubiquitin protease. Result: no protein on cell surface
- protein is made just didn't make it to the membrane
Symptoms
- testing for recurrent infections leads to CF diagnosis
- meconium: bowel obstructions
- no bowel movement in first few days
- air fluid: when bowel obstructed, fluid backs up in obstruction, mix up with air = air fluid levels
- no pancreatic enzymes to absorb fat
- stable for a while then develop acute exacerbation
- pseudomonas both for acute and chronic exacerbation
- clog up pancreatic duct
- recurrent pancreatitis: burn out islet cells
- vitamin E: antioxidant. Deficient leads to anemia, acanthocytosis, muscle weakness, spinocerebellar tract demylelination (ataxia). Neurologic symptoms without megaloblastic anemia
- thickened mucous can develop into polyps inside nose
Diagnosis
- iontophoresis: electric field for test
- pilocarpine: muscarinic, parasympathetic, induce sweating (sweat induced by sympathetic but Ach NT)
- nasal potential difference: Na abnormality, not Cl abnormality
Treatment
- dying neutrophils spill DNA, polymerized in mucous
- saline: moisten secretions
- N-acetylcysteine: cleaves disulfide bonds in mucous, but causes bronchospasm
- transplant: have to be relatively healthy (kids)
- not always done
- trypsinogen: pancreatic zymogen
- heel stick testing
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