Paediatric Respiratory & ENT Flashcards
How is CF inherited?
Autosomal recessive
The gene affected by CF is the CFTR gene- this affects the movement of which ion across epithelial surfaces?
Chloride
In Northern Europe, 1 in every how many individuals are a carrier for CF?
1 in 25
Nasal polyps are a feature of which chronic lung condition seen in children?
CF
What gastrointestinal problem is seen in 20% of neonates with CF?
Meconium ileus
Children with CF can become deficient in which vitamins?
Fat soluble vitamins (ADEK)
What happens to the levels of sodium and chloride ions in the blood of individuals with CF?
Both low
When screening for CF, the serum concentration of what is measured?
Trypsinogen (will be raised in CF)
What is the gold standard investigation for CF?
Chloride sweat test
In CF patients, chronic infection with what organism is associated with a deterioration in lung function and a poorer prognosis?
Pseudomonas aeruginosa
What kind of diet is recommended for children with CF?
High calorie, high fat diet
Because of impaired pancreatic function, there is a risk that children with CF may develop what other chronic condition?
Diabetes mellitus
What fungal organism colonises up to 60% of CF patients?
Aspergillus fumigatus
What is the median lifespan of an individual with CF?
40 years
What medication is used to treat cystic fibrosis patients who are homozygous for the delta F508 mutation?
Lumacaftor/Ivacaftor
Most children with CF are on what prophylactic antibiotic?
Flucloxacillin
Which organism is responsible for > 90% of childhood URTIs?
Rhinovirus
How are viral URTIs treated in children?
Paracetamol and/or ibuprofen and adequate fluid intake
What can sometimes be the only sign of pneumonia in children?
Abdominal pain
The peak incidence of bronchiolitis is less than how old?
18 months
What organism is responsible for causing 80% of cases of bronchiolitis?
Respiratory syncytial virus (RSV)
What is the natural history of bronchiolitis infection?
Worsening of symptoms until days 5-7, followed by a gradual improvement and resolution by around day 10
A diagnosis of bronchiolitis is usually made clinically. However, the RSV virus can be detected on what investigation?
Nasopharyngeal aspirate
Which children with bronchiolitis are admitted to hospital?
If they are hypoxic or if they are unable to take half of their normal feeds
How is bronchiolitis managed?
Supportive care with oxygen therapy and feeding (possible NG)
What treatments may be indicated in babies with bronchiolitis who have worsening hypoxia, exhaustion or apnoea?
CPAP or mechanical ventilation