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
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?
-Tx of infections
-high dose pancreatic enzymes
What are some benign pancreatic cysts?
–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?
Describe mucinous pancreatic cysts
These are common in women, found in the tail, and are precursors in invasive carcinomas. Need to be removed
Describe intraductal papillary mucinous neoplasms (IPMNs)
These are mucin producing growths found in larger pancreatic ducts, more commonly in men
More common in the HEAD of the pancreas and can progress to cancer (need to remove)
Describe solid pseudopapillary neoplasm
These are found in young women, often cause abdominal discomfort because of their typical large size, and can be locally aggressive, so they need to be removed (most pts. are cured following complete surgical resection)
What are the most common pancreatic cancer? Presentation?
The vast majority are adenocarcinomas of ductal origin
•Slow onset abdominal pain and weight loss are common presentations (pretty vague).
•Prognosis is poor unless caught very early.
What are the risk factors for pancreatic cancer?
•Alcohol in the setting of chronic pancreatitis (alcohol alone is not)
•Family history of pancreatic cancer
What are the symptoms of pancreatic adenocarcinoma?
– abdominal pain
– jaundice (if the head is involved)
What serum marker is elevated in pancreatic adenocarcinoma?
What is Migratory thrombophlebitis?
also known as the Trousseau sign, this occurs in about 10% of patients with pancreatic adenocarcinoma and is attributable to the elaboration of platelet-activating factors and procoagulants from the carcinoma or its necrotic products. Pts. might get DVT or pulmonary emboli
Not specific to pancreatic cancer
You shouldnt see the main pancreatic duct in a CT like this! Or dilations in the liver
PanIN is a progression!