Congential/childhood resp diseases Flashcards
Why do CF patients suffer from failure to thrive and poor weight gain?
Thick mucus in GI tract prevents efficent absorption of nutrients
How is CF managed?
CREON
Bronchodilators
Pulmonary rehab
Nutritional support
What condition is associated with salty sweat?
CF
Due to increased Cl- levels
What is meconium ileus and what condition is associated with it?
Medical emergency
V thick black first stool - v difficult to pass
CF
In the LONG ARM of what chromsome is the mutation in CF found?
What does this mutuation cause?
chromosome 7
abnormality in CFTR
A Cl- transporter
CF can cause infertility in men. Why?
Absence of Van Deferens
What must be noted about antibiotic treatment of bacterial infections in CF?
ALWAYS give 2 antibiotics
- Reduces resistance
What is the first line test for CF?
Sweat test - >60mmol -> immediately refer to CF clinic
What is the most common microorganism cause of bronchiolitis?
Respiratory syncytial virus (RSV)
Alpha-1 antitrypsin deficiency is caused by what?
What kind of inheritance is associated with it?
Failure to breakdown neutrophil elastase
Autosomal recessive
Most congenital abnormalities are caused by what?
Unknown cause
“Barking seal cough”
How would it present on CXR?
Croup
Church steeple sign
How can croup be managed?
Supportively + ORAL DEXLEMETHASONE
Describe the differences between adult and foetal Hb?
Foetal - higher affinity for O2
Adult - has beta subunits unlike foetal (beta = better)
What are the 8 signs of resp distress syndrome?
V IMPORTANT
- Subcostal/intercostal recession
- High resp rate
- Low O2 sats
- Evidence of use of accessory muscles
- Tracheal tapping
- Head bobbing
- Cyanosis
- Abnormal airway noises
- Nasal flaring
