What is bronchiectasis and the pathophysiology of this?
Permanent dilation of the bronchi/bronchioles secondary to chronic infection/inflammation
Scarring reduces the number of cilia so decreases mucus clearance and predisposes to further infection
What are the causes of bronchiectasis in children?
What is primary cilliary dyskinesia? (A.K.A Kartagener’s syndrome)
How is PCD diagnosed?
What are the following conditions:
How may a child with bronchiectasis present?
What investigations should you do for children with suspected bronchiectasis?
Step 1: Confirm Diagnosis
Step 2: Find underlying cause
What signs are indicative of bronchiectasis on HRCT in children?
Different patterns different aetiologies
What may lung function tests show in a child with bronchiectasis?
Mild: Normal
Severe: obstructive or mixed as scarring occurs
What are the management principles in bronchiectasis in children?
How is bronchiectasis managed in children?
What are some complications of bronchiectasis in children?
What is cystic fibrosis and the epidemiology of this?
Autosomal recessive multi-system disease with predominantly respiratory symptoms. 1 in 25 are carrier, 1 in 2500 have it
Caused by mutations to CFTR gene on chromosome 7. This encodes a chloride channel.
Most diagnosed at birth due to newborn heel prick screening, others are diagnosed following characteristic presentations (e.g. failure to thrive, recurrent chest infections, steatorrhea)
What is the pathophysiology of the respiratory symptoms in CF?
Dehydration of airway fluid leading to muco-cilliary dysfunction
CFTR channels on the apical surface of epithelial cells. Defects in normal ion transport leads to dehydration and depletion of airway surface liquid so cilia dysfunction
Reduced mucus clearance, airway obstruction and a predisposition to infection. Recurrent infection leads to chronic bronchitis, damage to the bronchi and eventual bronchiectasis
What are some signs in a newborn of CF before heel prick testing is back?
What are the respiratory symptoms of CF?
Productive cough and Recurrent chest infections
In childhood Staphylococcus aureus and Haemophilus influenza are commonly isolated
In older age groups colonisation with Pseudomonas aeruginosa becomes increasingly common
Recurrent infections result in bronchiectasis secondary to damage to the bronchial walls
What are some other symptoms of CF not involving the respiratory system?
Pancreatic Disease: Acute/Chronic pancreatitis, CF related Diabetes
GI Disease: Meconium Ileus, Distal intestinal obstruction syndrome, Liver Cirrhosis,Rectal prolapse
Malignancies: Increase risk of large and small bowel, pancreas and biliary tract, Hepatocellular carcinoma
Infertility
Poor growth/failure to thrive
What are some signs on examination of CF?
What are some other causes of clubbing in children?
How is cystic fibrosis diagnosed?
What is the issue when a CF patient becomes colonised with Pseudomonas Aeruginosa?
What monitoring is done for a child with CF in general?
Annually:
What are some complications of Cystic Fibrosis?
How is the pulmonary disease in CF managed? NEED TO FINISH THIS CARD!!!
Always use MDT approach - two aims of treatment
Clearance of secretions
Managing/Preventing Infections