Paediatric Respiratory Medicine - Distress, cough, apnoea and CF Flashcards

1
Q

What are the signs of respiratory distress seen in a child?

A
Accessory muscles
Tachypnoea
Grunting 
Nasal flaring
Recession 
Head bobbing
Tripoding - optimise use of accessory muscles
Cyanosis
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2
Q

What is grunting caused by?

A

Exhaling against a partially closed glottis to keep airway open

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3
Q

What is the difference between a wheeze and stridor?

A

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

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4
Q

Name some causes of wheeze

A

Asthma
Bronchiolitis
Viral wheeze
Foreign body

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5
Q

Name some causes of stridor

A
Croup
Foreign body
Epiglottitis
Laryngomalacia
Vocal cord dysfunction
Abscess - peritonsillar or retropharyngeal
Anaphylaxis
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6
Q

What causes of respiratory distress should you consider? (broadly speaking)

A

Cardiac problems?
Structural issues?
Infective cause?
Gas exchange problem?

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7
Q

What duration of cough corresponds to being acute, subacute and chronic respectively?

A

Acute <3 weeks
Subacute 3-8 weeks
Chronic >8 weeks

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8
Q

What generally causes acute coughs?

A

Infection

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9
Q

What generally causes subacute coughs?

A

Post viral cough

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10
Q

What about a cough could indicate it is due to asthma?

A

Night time
Wheeze
Has a trigger

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11
Q

What features of a cough could indicate it is due to a recurrent infection?

A

Start with additional respiratory signs

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12
Q

What feature of a cough would indicate a post nasal drip or GORD

A

Worse on lying down

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13
Q

What would suggest a cough is one of habit?

A

Won’t cough at night

Otherwise well

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14
Q

What would suggest a cough has an environmental cause?

A

History of smoking/parental smoking

Live in polluted area

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15
Q

How would a cough present in a child with Cystic Fibrosis?

A

Begin at young age

Systemically unwell

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16
Q

What is a classical Tuberculosis cough?

A

Systemically unwell

Cough progress

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17
Q

What features of a cough would suggest a child has inhaled a foreign body?

A

Acute onset

Haemoptysis?

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18
Q

What are the red flag signs for a cough?

A
Sudden onset
Haemoptysis
Dysphagia
Moist productive cough
Night sweats
Weight loss
Worsening cough
Failure to thrive
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19
Q

What investigations would you consider if a child had a chronic cough?

A

CXR
Lung function
Skin prick
Sputum sample for culture and microscopy

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20
Q

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?

A

Obstructive sleep apnoea

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21
Q

What is obstructive sleep apnoea?

A

Intermittent airway obstruction during sleep

22
Q

How does obstructive sleep apnoea present?

A

Sleep disturbance
Snoring
Waking grumpy - headache, refuse breakfast
Dry mouth and cracked lips - mouth breathing
Repeated Ent infections - adenoidal hypertrophy

23
Q

What are the causes of obstructive sleep apnoea?

A

Adenotonsillar hypertrophy
Obesity
Craniofacial abnormalities
Achondroplasia

24
Q

How is a diagnosis of obstructive sleep apnoea made?

A

Generally clinical

Can do overnight polysomnography

25
Q

How is obstructive sleep apnoea managed?

A

Remove tonsils/adenoids
CPAP
Weight loss
Orthodontic/maxillary surgery

26
Q

What should you not give to help treat obstructive sleep apnoea and why?

A

DONT GIVE CODEINE

Causes respiratory depression

27
Q

What is cystic fibrosis?

A

Autosomal recessive disease leading to mutation in CFTR gene which leads to defective Cl- secretion and increased Na+ absorption

28
Q

What systems can cystic fibrosis affect?

A
Respiratory system
Pancreas
Sweat
Liver
Intestines
Bones
Male fertility
29
Q

How does Cystic Fibrosis present in a newborn?

A

Meconium ileus
Failure to thrive
Prolonged jaundice

30
Q

How does meconium ileum happen in a newborn with Cystic Fibrosis?

A

Bowel blocked by sticky secretions leading to signs of intestinal blockage:

Bilious vomit
Abdo distention
Delay in passing meconium

31
Q

Why do children with Cystic Fibrosis fail to thrive?

A

Malnutrition due to poor gut absorption

32
Q

What is the pathophysiology behind Distal Intestinal Obstruction Syndrome in children with Cystic Fibrosis?

A

Insufficient pancreatic enzymes and thick mucus –> thick dehydrated contents –> Faecal obstruction in ileocaecum

33
Q

What signs of Distal Intestinal Obstruction Syndrome would you find on examination/imaging?

A

Palpable RIF mass

AXR show faecal loading and junction between small and large bowel

34
Q

How does Cystic Fibrosis affect the respiratory system?

A

Abnormal ion transport leads to thickening of mucus

Inadequate mucociliary clearance
Chronic bacterial colonization
Lung injury

35
Q

What respiratory problems can be seen in Cystic Fibrosis?

A

Recurrent chest infections
Chronic sinusitis
Nasal polyps
Bronchiectasis

36
Q

What pattern of breathing would you see on a lung function test in cystic fibrosis
?

A

Obstructive pattern

37
Q

What changes would you see on CXR in children with Cystic Fibrosis?

A
Hyperinflation - flat diaphragm
Bronchial dilation
Bronchiectasis
Pulmonary artery dilation
RV hypertrophy
38
Q

How is Cystic Fibrosis diagnosed?

A

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

39
Q

What bacteria are children with Cystic Fibrosis particularly susceptible to?

A
Staph Aureus
H Influenzae
Pseudomonas aeruginosa
Burkholderia cepacia
Aspergillus
40
Q

What signs would you see on examination of a child with Cystic Fibrosis?

A

Hyperinflation
Wheeze
Crackles
Clubbing

41
Q

Why do children with Cystic Fibrosis get steatorrhea?

A

Deficiency in pancreatic enzymes –> pale greasy offensive stools

42
Q

How are the chest symptoms of Cystic Fibrosis managed?

A

Physio - postural drainage and airway clearance 2x per day

Prophylactic antibiotics

Mucolytics - DNase or hypertonic saline

43
Q

What physiotherapy devices are used in Cystic Fibrosis?

A

PEP masks
Flutter devices
Acapella device

44
Q

How are gastro symptoms managed in Cystic Fibrosis?

A

Creon - pancreatic enzyme replacement
High calorie diet
Fat soluble vitamines - ADEK

45
Q

What do you screen for in children with Cystic Fibrosis?

A

Diabetes - annually

Osteoporosis

46
Q

What lifestyle changes are suggested for children with Cystic Fibrosis?

A
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!
47
Q

What is the prognosis for Cystic Fibrosis?

A

50% live beyond 40yo

48
Q

What screening methods are used to find if children have Cystic Fibrosis?

A

IRT - immunoreactive trypsin
Sweat test
CFTR gene

49
Q

What are nasal polyps in a child indicative of?

A

Cystic Fibrosis or Primary ciliary dyskinesia

50
Q

When do chest problems start to affect children with CF?

A

Generally seen once they get a little bit older

51
Q

What can cause false positive and false negative sweat tests

A

False positive
Malnutrition, G6PD, hypothyroidism, adrenal insufficiency

False negative
Skin oedema due to hypoalbuminaemia due to pancreatic exocrine insufficiency