Paediatric Respiratory Medicine - Distress, cough, apnoea and CF Flashcards
What are the signs of respiratory distress seen in a child?
Accessory muscles Tachypnoea Grunting Nasal flaring Recession Head bobbing Tripoding - optimise use of accessory muscles Cyanosis
What is grunting caused by?
Exhaling against a partially closed glottis to keep airway open
What is the difference between a wheeze and stridor?
Wheeze is an expiratory sound caused by lower airway obstruction. Intrathoracic airway collapse
Stridor is a high pitched inspiratory sound caused by upper airway obstruction. Extra thoracic airways collapse
Name some causes of wheeze
Asthma
Bronchiolitis
Viral wheeze
Foreign body
Name some causes of stridor
Croup Foreign body Epiglottitis Laryngomalacia Vocal cord dysfunction Abscess - peritonsillar or retropharyngeal Anaphylaxis
What causes of respiratory distress should you consider? (broadly speaking)
Cardiac problems?
Structural issues?
Infective cause?
Gas exchange problem?
What duration of cough corresponds to being acute, subacute and chronic respectively?
Acute <3 weeks
Subacute 3-8 weeks
Chronic >8 weeks
What generally causes acute coughs?
Infection
What generally causes subacute coughs?
Post viral cough
What about a cough could indicate it is due to asthma?
Night time
Wheeze
Has a trigger
What features of a cough could indicate it is due to a recurrent infection?
Start with additional respiratory signs
What feature of a cough would indicate a post nasal drip or GORD
Worse on lying down
What would suggest a cough is one of habit?
Won’t cough at night
Otherwise well
What would suggest a cough has an environmental cause?
History of smoking/parental smoking
Live in polluted area
How would a cough present in a child with Cystic Fibrosis?
Begin at young age
Systemically unwell
What is a classical Tuberculosis cough?
Systemically unwell
Cough progress
What features of a cough would suggest a child has inhaled a foreign body?
Acute onset
Haemoptysis?
What are the red flag signs for a cough?
Sudden onset Haemoptysis Dysphagia Moist productive cough Night sweats Weight loss Worsening cough Failure to thrive
What investigations would you consider if a child had a chronic cough?
CXR
Lung function
Skin prick
Sputum sample for culture and microscopy
A 12 year old obese boy comes in with feeling tired at school. His mum says he is always grumpy in the morning and has missed school because of headaches. She often hears him snoring loudly at night. Upon examination his tonsils appear swollen. What is the likely diagnosis?
Obstructive sleep apnoea
What is obstructive sleep apnoea?
Intermittent airway obstruction during sleep
How does obstructive sleep apnoea present?
Sleep disturbance
Snoring
Waking grumpy - headache, refuse breakfast
Dry mouth and cracked lips - mouth breathing
Repeated Ent infections - adenoidal hypertrophy
What are the causes of obstructive sleep apnoea?
Adenotonsillar hypertrophy
Obesity
Craniofacial abnormalities
Achondroplasia
How is a diagnosis of obstructive sleep apnoea made?
Generally clinical
Can do overnight polysomnography
How is obstructive sleep apnoea managed?
Remove tonsils/adenoids
CPAP
Weight loss
Orthodontic/maxillary surgery
What should you not give to help treat obstructive sleep apnoea and why?
DONT GIVE CODEINE
Causes respiratory depression
What is cystic fibrosis?
Autosomal recessive disease leading to mutation in CFTR gene which leads to defective Cl- secretion and increased Na+ absorption
What systems can cystic fibrosis affect?
Respiratory system Pancreas Sweat Liver Intestines Bones Male fertility
How does Cystic Fibrosis present in a newborn?
Meconium ileus
Failure to thrive
Prolonged jaundice
How does meconium ileum happen in a newborn with Cystic Fibrosis?
Bowel blocked by sticky secretions leading to signs of intestinal blockage:
Bilious vomit
Abdo distention
Delay in passing meconium
Why do children with Cystic Fibrosis fail to thrive?
Malnutrition due to poor gut absorption
What is the pathophysiology behind Distal Intestinal Obstruction Syndrome in children with Cystic Fibrosis?
Insufficient pancreatic enzymes and thick mucus –> thick dehydrated contents –> Faecal obstruction in ileocaecum
What signs of Distal Intestinal Obstruction Syndrome would you find on examination/imaging?
Palpable RIF mass
AXR show faecal loading and junction between small and large bowel
How does Cystic Fibrosis affect the respiratory system?
Abnormal ion transport leads to thickening of mucus
Inadequate mucociliary clearance
Chronic bacterial colonization
Lung injury
What respiratory problems can be seen in Cystic Fibrosis?
Recurrent chest infections
Chronic sinusitis
Nasal polyps
Bronchiectasis
What pattern of breathing would you see on a lung function test in cystic fibrosis
?
Obstructive pattern
What changes would you see on CXR in children with Cystic Fibrosis?
Hyperinflation - flat diaphragm Bronchial dilation Bronchiectasis Pulmonary artery dilation RV hypertrophy
How is Cystic Fibrosis diagnosed?
Postive history in sibling OR positive newborn screen result
AND
> 60mmol/L Cl- on sweat test OR 2 CF genetic mutations OR abnormal nasal epithelium ion transport
2 positive sweat tests confirm diagnosis
What bacteria are children with Cystic Fibrosis particularly susceptible to?
Staph Aureus H Influenzae Pseudomonas aeruginosa Burkholderia cepacia Aspergillus
What signs would you see on examination of a child with Cystic Fibrosis?
Hyperinflation
Wheeze
Crackles
Clubbing
Why do children with Cystic Fibrosis get steatorrhea?
Deficiency in pancreatic enzymes –> pale greasy offensive stools
How are the chest symptoms of Cystic Fibrosis managed?
Physio - postural drainage and airway clearance 2x per day
Prophylactic antibiotics
Mucolytics - DNase or hypertonic saline
What physiotherapy devices are used in Cystic Fibrosis?
PEP masks
Flutter devices
Acapella device
How are gastro symptoms managed in Cystic Fibrosis?
Creon - pancreatic enzyme replacement
High calorie diet
Fat soluble vitamines - ADEK
What do you screen for in children with Cystic Fibrosis?
Diabetes - annually
Osteoporosis
What lifestyle changes are suggested for children with Cystic Fibrosis?
No smoking Avoid other patients with CF Avoid people with colds No Jacuzzi (pseudomonas) Annual flu vaccine Clean and dry nebulisers fully NaCl tablets in hot weather Exercise!
What is the prognosis for Cystic Fibrosis?
50% live beyond 40yo
What screening methods are used to find if children have Cystic Fibrosis?
IRT - immunoreactive trypsin
Sweat test
CFTR gene
What are nasal polyps in a child indicative of?
Cystic Fibrosis or Primary ciliary dyskinesia
When do chest problems start to affect children with CF?
Generally seen once they get a little bit older
What can cause false positive and false negative sweat tests
False positive
Malnutrition, G6PD, hypothyroidism, adrenal insufficiency
False negative
Skin oedema due to hypoalbuminaemia due to pancreatic exocrine insufficiency
