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
Respiratory Tract in CF: _____ from severe pulm complications.
death
Diagnosis for CF (4)
- sweat test
- newborn screen (trypsinogen)
- look for resp and GI mnfst
- sibling hx
How is the sweat test used?
Sweat test–> analyze the perspiration of the sweat —>measures the conc. of sodium chloride concentration
In CF, conc. of NaCl is ___-___ time higher b/c most NaCl remain in the _____ b/c there are _____ to ___ withdrawal of Cl- ions moving out of the _____ ___
2-5x higher
duct
little to no
secretory cell
In a normal individual: Na and Cl move from _______ into the _______ cells, as secretion moves down, certain quantity of Cl- ions is _______ out of secretory cells and Na _____.
circulation
secretory cells
removed
follows
In a normal individual: most NaCl are ______ from secretion and some _____ in perspiration
removed
remain
What is the newborn screen? Trypsinogen increase or decrease?
It is a blood test screening in newborns to detect increase levels of trypsinogen d/t secretory obstruction in pancreas
Trypsinogen is a ______ for ____ which break down ______
precursor
trypsin
proteins
GI Manifestations (3)
- diarrhea
- abdominal pain
- abn pancreatic secretion
Screening test are done esp if newborns’s ________ have _____ _______. Why?
siblings
CF
strong possibility that newborn have it too
Treatment of CF (8)
- no cure
- manage prog
- antibiotics and gamma gobulins for infection
- diet
- enzyme supplement
- mucolytic and anti-inflm
- chest physio
- DNAase
There is no cure b/c it is a _____ prob
genetic
__________ management
symptomatic
What are gamma gobulins? How does it tx infection?
antibodies
use to prophylactically tx infection
What is supplemented in diet? What is limited and increased in diet?
supplement NaCl in diet
limit fats
ensure high protein and calorie diet
Why supplement enzymes?
d/t deficiency of pancreatic enzymes
Why is chest physiotherapy used to tx CF?
to help dislodge and expectorate mucus
Explain DNAase
an enzyme that breaks up the DNA strands present in mucus within the a/w making mucus more mobile to expectorate
