Lecture 11 Flashcards
The increase in life expectancy has lead to..
new CF complication emerging
105ml/L is the normal _____ concentration
Cl
Sweat moves up the duct, during which sodium and chloride are _____
resorbed
doesn’t happen in CF
In the sweat gland CFTR _____ eNaC
stimulates
True or False
ENaC is the only channel through which Sodium is reabsorbed
False
What are the clinical implications of high salt sweat
Hypoatremic dehydration
Pseudo-Bartter’s syndrome
Headaches or irritability Muscle cramps Nausea and vomiting Fatigue Poor concentration
855 of CF patients are Pancreatic ______-
insufficient
lack of exocrine function
Which classes of mutations are more likely to have pancreatic insufficiency
Class 1,2,3, 6
CFTR is in the ____ membrane of the pancreatic ductal epithelial cell
It’s role there includes:
apical
Regulation of Cl secretion - reduced luminal liquid
Regulation of bicarbonate secretion (acidity controller)
CF related reduction inthe pancreas’ ability to producce insulin is called
what does this mean for the patients prognosis?
CF related diabetes mellitus
a significance increase in mortality, greater lung function decline
What is the pathogenesis of CF related Liver disease
(CFTR is in epithelial cells lining intra-hepatic bile ducts)
- Increase in viscosity of bile
- Plugging of intra-hepatic bile ducts
- Cirrhosis, initially multi-focal, in monority
CFTR incompetence in the GIT leads to…
Dehydration of luminal contents
constipation
Gastro-oesophageal reflux
meconium is the..
stool that the fetus produces in utero
Meconium illeus is when…
thick intraluminal meconium causes a bowel obstruction
Meconium illeus can lead to Distal Intestinal Obstruction Syndrome (DIOS)
what does this entail?
Abdominal pain
A palpable mass in the illiac fossa (RIF)
constipation
