Respiratory Flashcards
What Is cystic fibrosis
AR disorder causing increased viscocity of secretions
due to defect in CFTR gene
Most common mutation is delta F508
Name 4 organisms that normally colonise CF patients
S aureus
Psudomonas aeruginosa
Burkholderia cepacia
Give 4 features of CF in childhood and early adulthood
Neonatal
- Meconium ileus
- Recurrent chest infections
- Steatorrhoea
- FTT
Short stature
DM
Delayed puberty
Nasal polyps
Male infertility
Pancreatitis
How is CF diagnosed
Newborn blood spot test
High sweat chloride test - >60
Amniocentesis - Genetic testing
Give 4 causes of a false positive sweat test
malnutrition
adrenal insufficiency
G6PD
Hypothyroidism
How is CF managed
MDT
Conservative
Chest physio
Postural drainage
High-calorie diet
minimize contact with others
medical
CREON tablets
Ivacaftor
Surgical
Lung transplant
How many people are carriers of CF gene
1 in 25
How many people have CF
1 in 2500
How does a meconium ileus present
Failure to pass meconium in 24 hours
vomiting
abdominal distension
Give sx of cystic fibrosis
chronic cough
thick sputum production
recurrent respiratory infections
Steatorrhoea
abdominal pain and bloating
FTT
Give 4 signs of CF
Low birth weight
nasal polyps
clubbing
crackles and wheeze
abdominal distension
Give features of CF
Thick pancreatic and biliary secretions that cause blockage of the ducts, resulting in a lack of digestive enzymes such as pancreatic lipase in the digestive tract
Low volume thick airway secretions that reduce airway clearance, resulting in bacterial colonisation and susceptibility to airway infections
Congenital bilateral absence of the vas deferens in males
Who requires the pneumococcal vaccine every 5 years
asplenism
splenic dyfunction
CKD
What is pulmonary hypertension and how is it managed
Raised resistance and pressure in pulmonary arteries
IV Prostanoids - Epoprostenol
Phosphodiesterase 5 inhibitors - Sildenafil
