Flashcards in CF - L4 Deck (14):
1
Describe how the sweat test works
pilocarpine is administered by iontophoresis, pilocarpine acts on cholinergic receptors
sweat is collected in filter paper
2
What does CFTR do to ENaC in sweat duct?
Activates, not inhibit!
so Na is transported with Cl in order to maintain electrical neutrality
3
What are the exocrine enzymes that the pancreas makes?
lipase
amylase
protease
4
What are the endocrine enzymes that the pancreas makes?
insulin
glucagon
lack of these relates to diabetes
5
How does a defect in CFTR affect the pancreas?
regulation of Cl secretion, lack of luminal liquid
regulation of bicarbonate secretion, acidic luminal
6
What happens in the duct of the pancreas in CF?
Cl & bicarbonate not transported
affects sodium transport
affects bicarbonate transport on other side
duct secretion becomes sticky
enzymes get activated in pancreas due to low pH
7
What happens in the acinus of the pancreas in CF?
makes enzymes and is unaffected
8
What is PERT?
pancreatic enzyme replacement therapy
lipase, amylase, protease
9
Describe the pathogenesis of the liver in CF
CFTR is in intra-hepatic bile ducts
increased viscosity of bile
plugging of bile ducts leads to cirrhosis
10
Clinical features of liver in CF?
prolonged neonatal jaundice
cirrhosis & portal hypertension
11
What is cirrhosis?
a chronic disease of the liver marked by degeneration of cells, inflammation, and fibrous thickening of tissue
12
What is meconium ileus?
Meconium ileus is a bowel obstruction that occurs when the meconium in your child's intestine is even thicker and stickier than normal meconium, creating a blockage in a part of the small intestine called the ileum
13
What are the clinical features in the GIT?
meconium ileus
distal intestinal obstruction syndrome
gastro-esophageal reflux (chest and stomach compete for same space)
other (prolapse, malignancy)
14