Bronchiectasis And Cystic Fibrosis Flashcards
What is bronchiectasis?
It is the permanent abnormal dilation in one or more of the lungs bronchi. Extra mucus is secreted and pools in the areas of the airways that are dilated making the person more prone to infection
What is the patho genesis of bronchiectasis?
Impaired mucocilliary clearance leads to the accumulation of secretions
Accumulation of secretions leads to infection by bacteria.
Infection by bacteria leads to increased mucus production, impaired ciliary performance and increased inflammatory response. An excessive inflammatory response causes tissue damage.
Tissue damage eventually produces dilated bronchi including loss of ciliates epithelium and impaired mucociliary clearance
What are the clinical features of bronchiectasis?
All patients have cough and chronic sputum production
Dyspnoea and wheeze
Chest pain
Recurrent exacerbations are common
What are the different types of bronchiectasis?
Saccular (occurs in large bronchi that become large and balloon like)
Cylindrical (involves medium sized bronchi which usually are symmetrically dilated)
Varicose (constrictions and dilation deform the bronchi)
The above may be localised or widespread
What are the symptoms of an acute exacerbation of bronchiectasis?
Change in sputum production Increased dyspnoea Inicreased cough Temperature >38.0 degrees Increased wheezing Malaise, fatigue, lethargy or decreased exercise tolerance Reduced pulmonary function Change in chest sounds
What does treatment and management of bronchiectasis look like?
Bronchodilater
Steroids
Nasal sprays
What is cystic fibrosis?
An inherited autosomal recessive disease. It is the result of a gene mutation on chromosome 7. Chromosome 7 is responsible for encoding the CFRT protein. The defect leads to a compromise in ion transport- this impairs transport of chloride ions and also affects levels of sodium and water in the cell.
It affects several organs causing ducts to become obstructed with mucus causing inflammation and replacement of damaged cells with connective tissue
What does the CFTR protein do?
It controls the flow of water and chloride ions between membranes, when the structure is changed, passage of molecules and ions is blocked.
A defect in this ion causes respiratory disease, high sodium sweat, pancreatic insufficiency, biliary disease etc
What are the common symptoms of cystic fibrosis?
Persistent cough
Inflammation of the lungs, wheezing, sob, difficulty breathing, recurring chest and lung infections, cross infection, impaired diaphragm from enlarged liver
What are the treatment options possible for cystic fibrosis?
PD PERCUSSIONS/ vibrations and shakes ACBT AD ADJUNCTS MOBILISATION/Exercise Suction (ITU)
