Paediatric respiratory Flashcards
What are the classic clinical features of cystic fibrosis?
Suppurative lung disease, pancreatic exocrine insufficiency manifesting as steatorrhea and failure to thrive, male infertility, elevated sweat electrolytes
What are the management priorities for cystic fibrosis
Nutrition: high-fat high calorie diet, pancreatic enzyme replacement, vitamin supplementation
Salt supplementation
Pulmonary care, chest physiotherapy, mx of infections with aggressive antibiotics
what are the main features of mycoplasma pneumonia?
subacute course
prominent cough
+/- headache
+/- sore throat
why do children with cerebral palsy frequently present to hospital with respiratory issues like pneumonia?
higher risk of aspiration
Poor cough
immobility and scoliosis
increased secretions (due to benzo use)
which cough is typically heard in the morning?
bronchiectasis cough because secretions pool over night
what is important to clarify with parents about the relationship between cough and vomit?
ask the parents- does vomiting occur AFTER episodes of coughing or does it occur WITHOUT coughing?
remember that children don’t expectorate well and often will swallow their mucous
at what age group do we normally expect inhaled foreign bodies?
between 12 months (when neat pincer grip develops) and to 2-3 yrs old (toddler phase)
what is the most common cause of chronic wet cough in children?
bronchitis- persistent infection in the large conducting airways
how long do normal viral respiratory infections usually last in children?
1-2 weeks but in some children it can last up to a month
what the radiological features of bronchiectasis on a CXR?
tram tracking
hyperinflation
patchy opacities
what does a sweat test for CF involve for a child?
Put pilocarpine in the forearm and then put an electric current across it to induce sweat glands.
The sweat will be analysed for Chloride concentration.
> 60 mmol/L of Cl- is diagnostic of CF
what are some common causes of chronic suppurative lung disease in children?
Cystic fibrosis Primary ciliary dyskinesia primary or secondary immunodeficiency chronic severe pneumonia TB missed foreign body
what are some causes of acquired/secondary immunodeficiency in children?
HIV/AIDs
malignancy + treatment
malnutrition
steroids use
what are the clinical features of primary ciliary dyskinesia?
chronic rhinitis and sinusitis chronic suppurative lung disease OM males are infertility 50% of patients have dextrocardia (kartagener's syndrome)
what is a good screening test for primary ciliary dyskinesia
nasal nitric oxide- low measurement of NO can indicate PCD
what does DIOS refer to in cystic fibrosis?
DIOS= distal ileum obstruction syndrome, a complication of CF.
essentially equivalent to meconium ileus
faecal matter becomes impacted in ileum causing obstruction
how many classes of mutations relating to CFTR gene are there?
5 classes
what are the complications/disease manifestations of CF?
pancreatic exocrine insufficiency–> malabsorption–> poor growth + poor bone health (decreased vitamin D) + other nutritional deficiencies
pancreatic destruction can lead to insulin requiring diabetes
prone to bowel obstruction (DIOS)
concurrent respiratory infections–> bronchiectasis–> failure to thrive –> chronic lung disease
biliary obstruction–> liver damage–> cirrhosis
infertility in males
chronic sinusitis–> nasal polyps–> obstructed nasal passage
what are some general management considerations for patients with CF?
• Key aim is to improve longevity and QOL
• Daily chest physiotherapy to clear secretions
• Postural drainage (ending up sitting upright)
• Aggressive antibiotics for respiratory infections
• Good infection control
• Replacing pancreatic enzymes
Replacing nutrient deficiency such as vitamin D
• High calorie diet and optimising nutrition for growth
• Salt replacement
• –> lung transplantation if end-stage
• Encourage active play/exercise and PEP games
Psychosocial support
what are the types of medications often used for CF patients?
- Bronchodilators- ventolin
- Mucolytics e.g. dornase alfa or hypertonic saline
- Corticosteroids post physiotherapy
- Antibiotics (prophylactic)
- Creon
- Salt supplements
CF patients are managed by a multidisciplinary team. Can you list the members of this team?
• CF physician/general paediatrician, GP • Dietician • Physiotherapist • Community nurse • Genetic counsellor • Pharmacist • CF educator Psychosocial supports--> CF Victoria
what are some key routine ix required for children with CF?
- Bronchoalveolar lavage to culture sputum if the child cannot expectorate- dictates antibiotic regimen
- Or sputum culture
- HRCT- looking at disease progression
- Spirometry in children > 5 yrs old
FBE/UEC/LFTs/vitamin D- ongoing monitoring
what is the ratio of H2Co3 to NaHCo3 to maintain normal physiological pH in the body?
1 H2Co3 (Co2) : 20 NaHCo3 (bicarb)
so if co2 drops, bicarb must also drop to maintain the 1:20 ratio
if co2 increases, bicarb must also increase
when do we start to see compensation for respiratory/metabolic electrolyte derangement on ABGs?
If the lungs compensate for a metabolic abnormality, compensation occurs within hours (e.g. increase/decrease respiratory rate), but the kidneys take 2-4 days to compensate for respiratory abnormality.
Any acute respiratory problem will always be uncompensated, as the kidneys would not have had time to compensate.