CF, Cystic Disorders, Pancreatic Cancer Flashcards
Cystic fibrosis pathophys?
Genetic Disorder of defective chloride secretion
Main symptoms of cystic Fibrosis?
Lung Infections, chronic pancreatitis, hypertonic sweat
Diagnosis
Sweat Chloride test
Treatment of CF?
Antibiotics and Pancreatic enzymes
WHat is the most common lethal genetic mutation in caucasians?
CF
Most CF is caused by what three base pair deletion?
DeltaF508
This three basepair deletion affects what processes?
folding, processing, and functioning of the CF gene product, CFTR (Cystic Fibrosis Transmembrane conductance regulator, which is a cAMP activated chloride channel
More specifically the pathophysiology of CF equals
- Defective chloride secretion through the CFTR
- Thickened secretory product
- Obstruction of ducts and lumens
- Infection, Inflammation, tissue destruction
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Clinical symptoms of CF
- Frequent Pulmonary Infections (pseudomonas)
- Diarrhea, malabsorption, failure to thrive
- Frequent episodes of dehydration
Cystic diseases of the pancreas
may be benign of malignant
Most common cystic structure in the pancreas
psuedocyst associated with chronic pancreatitis
Of the neoplastic cysts, which type has high malignancy potential and which type doesn’t?
Serous cysts have very little malignant potential
Mucinous cysts have high malignant potential
What are the three types of benign cystic lesions
- Psuedocysts- very common….chronic pancreatitis
- Non-neoplastic cysts: rare
- Serous cystic neoplasms: very little malignant potential
What are the three types of malignant cystic lesions
- Mucinous cysts
- Intraductal Papillary Mucinous Neoplasm
- Solid psuedopapillary neoplasm
The vast majority of pancreatic cancers are?
Adenocarcinoma of ductal origin