Cystic Fibrosis Flashcards
What is Cystic Fibrosis (CF)?
Defective chloride transport channel on CM
CM is composed of ______ and ______ making up the _______ layer. Heads of CM are _______ and tails are _______.
lipids and proteins
mosaic
hydrophobic
hydrophyllic
What are the 2 types of protein found on CM? Are they on outside or inside of CM?
- Extrinsic proteins–> outside of CM
2. Intrinsic proteins –>inner part of CM
Extrinsic and intrinsic proteins make up the _____ which move ___ and _______ in and out of cells.
channels
ion
substances
Etiology of CF (3)
- CFTR gene mutation on Chr. 7
- autosomal recessive
- increase rate in caucasian population
What does the CFTR gene stand for and function?
CFTR gene = cystic fibrosis transmembrane regulated genes
Fxn: Code for protein that regulate the passage of ions across the membrane
How does CFTR gene mutation result in CF?
transmembrane exchange is altered specifically to the exchange of Cl- ions —> causing defective Cl- ion channels
What does autosomal recessive mean?
req two defective alleles
If Cl channels are defective, there are secretions of _____ ions not ____ ions. This affects ______ glands and is not restricted to the _________ system. The problem is _____ sepcific
sodium
Cl-
exocrine
respiratory
site
Patho of CF (3)
- gene code Cl- protein channel on CM
- mutations –> channel permeability altered
- abn exocrine sec (tissue specific)
Patho: Gene code for Cl- protein channels are mutated.
protein that is responsible for transporting Cl- ions are defective
Patho: How does the mutation affect the protein channels? What happens to the transport of Cl- ion?
Cl- transport channels are impaired –> abnormal secretion of Cl- (xs, minimal, or no transport of Cl- ions)
T or F: CF can affect exocrine and endocrine galnds
WRONGGGG
affects EXOCRINE gland ONLY
Patho: What does it mean by abn exocrine secretion is tissue specific?
What happens in one area may not occur in another area
What organ system does it affect (3)?
- GIT
- resp system
3, reproductive system
Fig. 29. 14: What is needed for mucus to from? (3)
What is the consistency with these in place?
- chloride
- sodium
- H2O
normal consistency: not too thick and not too sticky
Fig. 29. 14: In the respiratory tract, what happens if the Cl- ions are defective? Na and H2O?
no chloride ions secretion and accum in the cytoplasm –> increase in conc.–>Na and H2O secrete as normal BUT reabsorb into the cytoplasm–> leaves a thick, sticky, and viscous mucus
Fig. 29. 14: thick and sticky mucus overwhelms the _________ ________ and _____ is unable to clear mucus
mucociliary blanket
cilia
Fig. 29. 14: abn mucus composition creates a ______________ that protects _________ _________ such as ________ resulting in infection and ______.
microenvironment
foreign microbes
bacteria
inflm –> release proteases which cause tissue destruction
Fig. 29. 14: What can result with decrease mucus clearance and abn mucus composition?
- chronic a/w obstruction
2. bacterial infection
Complications of CF (3)
- chronic bronchitis
- bronchiectasis
- respiratory failure
Respiratory Tract in CF: How does thick mucus lead to impaired breathing? What else can occur? What causes death?
- thick mucus –> compromised ciliary function–> a/w obstruction–> impaired ventilation—> no GE —> impaired breathing
- bacterial infection
- death from severe pulm complication
Respiratory Tract in CF: Why is breathing impaired?
no GE—> hypoxemia—> systemic hypoxia and hypercapnia –> dyspnea
Respiratory Tract in CF: abn composition of mucus create an ideal ________ for _______ to establish and cause _________ b/c inhaled microbe cannot be _________
medium
bacteria
pneumonia
expelled
