Bronchiectasis and Cystic Fibrosis Flashcards
What is the most common genetic cause for bronchiectasis?
Cystic Fibrosis
Define bronchiectasis and what is it characterised by?
What is the definition of Cystic Fibrosis and what is it characterized by?
Re - it is one of the major causes of bronchiectasis.
The thickened secretions in each of those areas are how you should organise symptoms and signs
What chromosome is the CFTR gene on?
Ch 7
What are the 2 most common classes of CFTR mutations? Differentiate between them.
Between these 2 which is more severe?
Then state the other rarer forms
What is the most common variation of CF in ireland?
What is the most severe?
How many people are CF carriers in Ireland?
What are the causes of bronchiectasis?
RFs for bronchiectasis
Symptoms of CF can be divided into Respiratory, GI, Genitourinary and in a history, you would ask about features in infancy.
Pancreatic insufficiency and sx of cirrhosis make up the symptoms to ask under GI.
State the symptoms of pancreatic insufficiency. How are they managed?
What about the things to ask about in terms of infancy?
Steatorrhoea (oily stool) -> Creon + Vit. ADEK (fat soluble vitamins)
Constipation (Straining, tenesmus, frequency, Pellet-like stools) -> Laxatives
Sx of Anaemia (malabsoption) -> Based on deficiency (e.g. Iron -> iron supplements/Galfer)
Polyurea/Polydipsia (Diabetes from pancreatic insufficiency) -> Insulin for glycemic control)
(For cirrhosis, things to ask in a hx are jaundice, ascites, oedema, fatigue, easy bruising)
Infancy: Meconium ileus, failure to thrive, rectal prolapse, volvulus
Why do patients with Cf have both constipation and steatorrhoea?
Steatorrhoea in CF is due to malabsorption of fats both from the biliary disease and cirrhosis but more importantly from the thick mucous preventing absorption.
Constipation on the other hand is due to distal intestinal obstruction
What symptoms would you like to ask about in the history of a CF patient?
What symptoms would you like to ask about in the history of a bronchiectasis patient?
What exam findings would you expect to see in a patient with CF but not bronchiectasis? (AKA specific signs for CF)
What exam findings would you expect to see in both a CF and a non CF Bronchiectasis patient?
What are the diagnostic investigations for Bronchiectasis and what will it show?
1) Broncho-arterial ratio >1.5
2) Lack of tapering => bronchi remain dilated as we move to the peripheries and are visible (normally not visible)
3) Bronchial wall thickening
What grading will you use for Bronchiectasis? (just the name)
FACED
Is the FACED grading in bronchiectasis used for all patients with bronchiectasis?
What are the components?
How are the results interpreted?
How would you investigate a patient with Bronchiectasis? Justify each.
Note: IgE/RAST for ADPA - Allergic Bronchopulmonary Aspergillosis
You are investigating a suspected CF patient and you request a nasal potential difference. What does this test used for? Describe how the test is done.
You are investigating a CF patient and you request a PFT and OGTT. What is the importance of each of these tests.
What is the nasal potential difference test for? Explain the procedure and interpretation of results.
What are the diagnostic investigations for CF?
How would you investigate a patient with CF? Justify each.
What is the ABLE grading in CF used for?
What are the components?
How are the results interpretd?
Outline the acute management of CF and Bronchiectasis.
Outline the conservative management of CF and Bronchiectasis.
Outline the long-term resp management of CF.
Outline the long-term GI management of CF.
Outline the FULL long-term management of CF.
Outline the full management of CF.
What are some complications of CF and Bronchiectasis?
What does this image show?
incentive spirometry
List some ddx for haemoptysis.
What is the mean survival of a CF patient.
What does this image show
Incentive spirometry
