Pancreas II Flashcards
Why do pts. with CF get dehydrated?
Normally we put salt into our secretory ducts and then reabsorb it but CF pts. can reabsorb it leading to loss of salt and fluids to produce a hypertonic sweat compared to the noraml hypotonic sweat
What is Cystic fibrosis?
a genetic disorder of defectve chloride secretion that presents pediatrically with the main symptoms of lung infections, chronic pancreatitis, and hypertonic sweat as diagnosed via a sweat chloride test
What causes CF?
An AR disorder common in caucasians (1:25 gene frequency) that is caused mostly caused by a three BP deletion (F508) that affects the folding, processing, and functioning of the CF gene product, the cystic fibrosis transmembrane conductance regulator (CFTR), a cAMP-activated chloride channel
What is the pathophys of CF?
Defective chloride secretion causes abnormal, thickened secretory products that obstruct ducts and lumens leading to infection, inflammation, and tissue destruction
CFTR is involved in biliary secretions
Male infertility
How does CF present?
frequent pediatric pulmonary infections marked by pseudomomas, diarrhea, malabsorption, and commonly failure to thrive, and frequent episodes of dehydration
How is CF treated?
- Pulmonary decongestants/expectorants/oscillators
- Tx of infections
- high dose pancreatic enzymes
What are some benign pancreatic cysts?
–Pseudocysts
–Rare non-neoplastic cysts
What are some NEOPLASTIC pancreatic cysts?
–Serous cystic tumors
–Mucinous cyst neoplasms
–Intraductal papillary mucinous neoplasms
–Solid Pseudopapillary Neoplasms
Most pancreatic growths are found incidentally via CT scans when a pt. presents with abdominal pain. Then how are they managed?
Pts. will receive an endoscopic ultrasound to assess structure and/or obtain fluid and tissue for analysis AND/or
get serum markers (e.g. CEA for mucinous cystic neoplasms)
•ERCP for intraductal papillary mucinous neoplasms.
Describe serous pancreatic cysts
These are more common in women and represent 25% of cystic neoplasms. They are benign, multicystic, small (1-3mm), and commonly found in the tail of the pancreas
These are typically left alone, besides analysis
Serous pancreatic cysts have an association with what?
VHL
Describe mucinous pancreatic cysts
These are common in women, found in the tail, and are precursors in invasive carcinomas. Need to be removed