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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

Symptoms from other systems?

osteoporosis
infertility
renal?
ENT blockage
heart strain
skin rash