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COPD (EMPHYSEMA (PATHOPHYSIOLOGY (LIVER CIRRHOSIS Alpha-1AT protein…
COPD
EMPHYSEMA
FEATURES
Destruction of Alveolar Air Sacs
Loss of Elastic Recoil
Collapse of Small Airways
Air Trapping
AIR TRAPPING
'Drag' on Wall
Elastic Cartilage bw Alveoli are Destroyed
Decrease in Wall strength
Walls Collapse c incr. Turbulence
PATHOPHYSIOLOGY
LIVER CIRRHOSIS
Alpha-1AT protein synthesised in Liver
Mutation -> misfolded protein
Accumulation in Hepatocytes
Damages cells
CENTRIACINAR vs PANACINAR
Centriacinar (SMOKING) are more prox.
More damage
Most common
Upper lobes (smoke rises)
Panacinar is Alpha-1AT deficiency (GENETIC)
EMPHYSEMA HAS EITHER
Incr. P = Smoking (massive inflammation)
Decr. A = Alpha-1AT
Can also result in Liver Cirrhosis
Alpha-1AT Protein synthesised in Liver
Mutation -> misfolded protein
Accumulation in Hepatocytes
Damages cells
P:A RATIO
Balace of Proteases & Antiproteases req.
P:A Ratio
P = Protease
A = Antiprotease = Alpha-1AT
Alveolar Macrophages engulf particles
Inflammation
Incr. Proteases
Disrupts A:P Ratio
Alpha-1AT GENETICS
PiM = Normal Allele
PiZ = Most common clin. relevant mutation
PiMZ heterozygous -> Smoking maj. Risk Factor
PiZZ homozygous -> Sig. Risk for panacinar emphysema & cirrhosis
CLINICAL FEATURES
Cough without/min. sputum
Dyspnea
Prolonged expiration c pursed lips (Pink Puffer)
Barrel chested
Loss of elastic recoil
Wt loss
Incr. effort in breathing
ASTHMA
FEATURES
REVERSIBLE
airway bronchoconstriction
Most often due to allergic stimuli
T1 HSR
PATHOGENSESIS
CD4 bearing Th2 cells
Must be genetically susceptible
IL-4, IL-5, IL-10 secreted by Th2 cells
IL-4 -> Class switching to IgE
IL-5 -> Recruits eosinophils
IL-10 -> Inhibits Th1 cells
Promotes Th2 cells
IgE Mediated activation of
Mast Cells
Release histamine
Vasodilation
Incr. permeability
CLINICAL FEATURES
Episodic
Dyspnea
Wheezing
Productive Cough
Curschmann Spirals
Charcot-Leyden Crystals
Eosinophilic protein
TREATMENT
Review Spacer Technique @ Each Step
Step up to achieve control
RELIEVER = SABA (Salbutamol = Ventolin)
ADD PREVENTER = ICS (Budesonide = Pulmicort)
INCREASE PREVENTER OR + LABA (Salmeterol)
Available as combination = Symbicort
CHRONIC BRONCHITIS
FEATURES
Productive Cough >3mo over a min. of 2yrs
Strongly assoc. c SMOKING
CLINICAL FEATURES
Productive Cough
Cyanosis (Incr. PaCO2, Decr. PaO2)
Incr. Risk of:
Infection
Cor Pulmonale
Incr. Pressure in pulmonary circ.
RV hypertrophy
RV Heart Failure
PATHOPHYSIOLOGY
Submucosa Produces
Serous - Humidify
Mucous - Protective
Hyperplasia and Hypertrophy of Submucosa
Due to incr. irritant (smoke)
Incr. protection req.
Incr. Sputum Prodcution
Narrowing of Lumen = SOB
Reid Index b/c >50%
(Epi - LP - SM - Cartilage)
Indicates the proportion w/ is represented by SM
BRONCHIECTASIS
FEATURES
Permanent DILATION
Bronchioles
Bronchi
Loss of airway tone
Air trapping
PATHOGENESIS
Necrotising inflammation
Damage to airway walls
Px GROUPS
CF
Tumour
Necrotising Infx
Kartagener Syndrome
Ciliary defect of
dynein arm
GENERAL FEATURES
Obstruction getting air out of lungs
Decr. FEV1:FVC
Incr. TLC (Air Trapping)