CF - L4 Flashcards
Describe how the sweat test works
pilocarpine is administered by iontophoresis, pilocarpine acts on cholinergic receptors
sweat is collected in filter paper
What does CFTR do to ENaC in sweat duct?
Activates, not inhibit!
so Na is transported with Cl in order to maintain electrical neutrality
What are the exocrine enzymes that the pancreas makes?
lipase
amylase
protease
What are the endocrine enzymes that the pancreas makes?
insulin
glucagon
lack of these relates to diabetes
How does a defect in CFTR affect the pancreas?
regulation of Cl secretion, lack of luminal liquid
regulation of bicarbonate secretion, acidic luminal
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
What happens in the acinus of the pancreas in CF?
makes enzymes and is unaffected
What is PERT?
pancreatic enzyme replacement therapy
lipase, amylase, protease
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
Clinical features of liver in CF?
prolonged neonatal jaundice
cirrhosis & portal hypertension
What is cirrhosis?
a chronic disease of the liver marked by degeneration of cells, inflammation, and fibrous thickening of tissue
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
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)
Symptoms from other systems?
osteoporosis infertility renal? ENT blockage heart strain skin rash
