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Year 4 PAEDS > Respiratory and ENT > Flashcards

Respiratory and ENT Flashcards

1
Q

How can asthma be broadly split into two types?

A

1) Extrinsic - definitive external cause identified, most common in atopic individuals
2) Intrinsic / cryptogenic - no causative agent identified, usually late onset (middle aged)

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2
Q

What is the pathophysiology of asthma?

A

Increase in circulating IgE causes increased allergic atopic reactions

Reversible inflammatory disease of airways that responds to bronchodilators

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3
Q

List three features of asthma

A

1) Bronchial muscle contraction
2) Mast cell and basophil degranulation = inflammation
3) Increased mucus sectetion

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4
Q

List some triggers for asthma (5)

A

1) Drugs
- Beta-blockers = vasodilation and bronchoconstriction
- NSAIDS
2) Pollution / environmental allergens / dust
3) Cold air
4) Viruses
5) Physical exertion

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5
Q

List some risk factors for asthma (7)

A

1) Personal hx atopy
2) FH atopy
3) Triggers (eg pollen), dust, exercise, viruses, chemicals etc
4) Prematurity and low birth weight
5) Viral infections in early childhood
6) Maternal smoking
7) Early exposure to broad-spectrum abx

Breast feeding = protective

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6
Q

What is the triad of asthma symptoms?

A

Recurrent episodes of

1) Cough = worse at night
2) SOB
3) Wheeze

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7
Q

Ddx for wheeze:

Present from birth (3)

Shortly after birth (5)

Sudden onset in a previously well child (4)

A

Wheeze present from birth:

1) Presents immediately, constant wheeze without variation = structural abnormality eg bronchogenic cyst, vascular ring
2) Weak cry, stridor = laryngeal abnormality
3) Signs of HF = congenital heart disease

Wheeze shortly after birth:

4) Hx of prematurity or ventilation = bronchopulmonary dysplasia
5) Recurrent bacterial infections and FTT = immunodeficiency
6) Persistent cough and FTT, FH of chest disease = CF
7) Persistent nasal discharge and OM = ciliary dyskinesia
8) Vomitting and aspiration = GORD

Sudden onset in a previously well child:

9) Hx choking, unilateral reduced breath sounds = foreign body
10) Persistent wet cough = CF, bronchiectasis, recurrent aspiration, immunodeficiency, GORD
11) Finger clubbing, purulent sputum = CF, bronchiectasis
12) Focal signs in chest = developmental anomaly, post-infection, bornchiectasis, TB, foreign body

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8
Q

List the features of moderate acute asthma in a child <2yr

A

Moderate acute asthma:

  • Able to talk in sentences
  • SpO2 =/> 92%
  • Peak flow =/> 50% best or predicted
  • HR =/> 140bpm aged 2-5yr or 125 over 5yr
  • RR =/> 40 aged 2-5yr or 30 over 5yr
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9
Q

List the features of severe acute asthma in a child <2yr

A

Severe acute asthma:

  • Can’t complete sentences in one breath or too breathless to talk / feed
  • SpO2 <92%
  • Peak flow 33-55% best or predicted
  • HR >140bpm aged 2-5yr or >125bpm over 5yr
  • RR >40 aged 2-5yr or >30 over 5yr
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10
Q

List the features of life-threatening asthma in a child <2yr

A

Any one of the following in a child with severe asthma:

  • SpO2 <92%
  • Peak flow <33% best or predicted
  • Silent chest
  • Cyanosis
  • Poor response effort
  • Hypotension
  • Exhaustion
  • Confusion
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11
Q

What are the normal vital signs for:

1) Infant
2) 1-2yrs
3) 2-5yrs
4) 5-12yrs
5) >12yrs

A

1) Infant
HR: 110-160bpm
Systolic BP: 80-90mmHg
RR: 30-40

2) 1-2yrs
HR: 100-150bpm
Systolic BP: 85-95mmHg
RR: 25-35

3) 2-5yrs
HR: 95-140
Systolic BP: 85-100mmHg
RR: 25-30

4) 5-12yrs
HR: 80-120bpm
Systolic BP: 90-100mmHg
RR: 20-25

5) >12yrs
HR: 60-90bpm
Systolic BP: 100-140mmHg
RR: 14-18

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12
Q

What investigations should be performed for asthma? (6)

A

1) PEFR
2) Spirometry
3) Allergy testing
4) Exercise test
5) Bloods - Raised IgE and eosinophils
6) CXR

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13
Q

What is the stepwise management of asthma in children <12yrs?

A

1) Inhaled SABA
2) Inhaled corticosteroid (ICS): 200mcg / day
3) Add inhaled LABA (5yrs or over)

Add leukotreine receptor antagonist (LRTA) (<5yrs)

4) If only partial benefit: inc ICS to 400mcg / day

If no response: stop LABA and increase ICS to 400mcg / day

If control still inadequate: try LTRA

5) Increase ICS to 800mcg / day

OR consider addition of 4th drug eg theophylline

  • Refer to respiratory paediatrician
    6) Daily oral steroids = refer to respiratory paediatrician

Consider moving up ladder if using 3 or more doses of SABA per week

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14
Q

What is the stepwise management of asthma in children 12 yrs or over?

A 
1) Inhaled SABA
Inhaled corticosteroid (ICS) - 400mcg / day

2) Add inhaled LABA (usually combined inhaler with ICS and LABA)

If only partial benefit: increase ICS to 800mcg / day

If no response: stop LABA and increase ICS to 800mcg / day

If control still inadequate, try LRTA

3) Consider increasing ICS to 2000mcg

Or addition of 5th drug: LRTA, theophylline

4) Daily oral steroid and refer to specialist

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15
Q

What is the management of acute asthma in children?

A

O SHIT ME

Oxygen 15L/min
Salbutamol  NEB b2b
Hydrocortison IV 4mg / kg
Ipatropium NEB b2b 
Theophylline PO (or aminophylline IV)
Magnesium sulphate IV
Escalate
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16
Q

List types of bronchodilators and examples

A

1) Beta 2 agonist
- Salbutamol = Short acting
- Salmeterol = long acting

2) Anticholinergics
- Ipatropium
- Triotropium

3) Leukotriene receptor agonist
- Montelukast

4) Xanthines
- Theophylline = PO
[aminopyhlline is theophylline + ethylenediamine, given IV]

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17
Q

What is bronchiolitis? What age does It affected?

A

Acute viral infection of LRTI

Mostly ages 3-6 months (1/3rd have before age 1)

M>F

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18
Q

What is the cause of bronchiolitis?

A

80% due to respiratory syncytial virus (RSV)

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19
Q

What is the pathophysiology of bronchiolitis?

A

Initial URTI spreads to LRT = infection of bronchiolar respiratory cells and ciliated epithelial cells causes increased mucus secretion, cell death, lymphocytic infiltration and submucosal oedema

Mucus and oedema causes narrowed small airways and V/Q mismatch - leading to hypoxia

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20
Q

What are some risk factors for bronchiolitis? (9)

A

Un/under developed lungs + immune system most common:

1) Premature birth
2) Chronic lung disease eg CF or bronchopulmonary dysplasia
3) Congenital heart disease
4) Neurological disease with hypotonia and pharyngeal disco ordination
5) Immunocompromise
6) Tobacco smoke exposure
7) Non-breast fed
8) Contact with multiple children eg nursery, older siblings
9) Down’s syndrome

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21
Q

How may bronchiolitis present?

A

<2yr with 1-3 day hx of coryzal symptoms followed by:

1) Persistent cough AND
2) Either tachypnoea or chest recession (or both); AND
3) Either wheeze or crackles (or both)

Other features include low grade fever (under 39 degrees Celsius) and poor feeding

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22
Q

What are some other signs of hypoxia / increased work of breathing?

A

1) Tracheal tug
2) Cyanosis
3) Tachypnoea
4) Sub-costal recession
5) Nasal flare

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23
Q

What features may make you consider a pneumonia over bronchiolitis?

A

Higher temperature and focal crackles

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24
Q

Describe features of bronchiolitis vs viral induced wheeze

A

Bronchiolitis:

  • Younger = 2-12mnths
  • Hx of a viral illness followed by a wheeze
  • Salbutamol ineffective (too young)
  • Treated symptomatically
  • Chest sounds of wheeze and crackles

Viral wheeze:

  • Older = 1-3yrs
  • Shorter hx, wheeze develops in hours-day
  • Salbutamol effective
  • Chest sounds clear cut wheeze
  • Treated same as asthma

NB said that asthma cannot be accurately diagnosed before age 5 (but viral wheeze treated as asthma)

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25
Q

What features may make you consider a viral induced wheeze over bronchiolitis?

A

Wheeze with no crackles, episodic symptoms and/or a FH of atopy

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26
Q

How may young infants present with bronchiolitis?

A

May be lone apnoea

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27
Q

What investigations are performed for bronchiolitis? (4)

A

1) Full examination inc CRT, RR, HR, chest signs
2) Pulse oximetry - hypoxia
3) Nasopharyngeal swab fluorescent antibody test - RSV +ve
4) CXR - non specific: maybe hyperinflation, atelectasis, consolidation

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28
Q

What features require immediate referral to secondary care for bronchiolitis? (5)

A

1) Apnoea (observed or reported)
2) Marked chest recession or grunting
3) RR >70
4) Central cyanosis
5) O2 sats <92%

Or clinical judgement that the child looks severely unwell

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29
Q

What treatment is given for moderate acute bronchiolitis?

A

Usually mild, self-limiting illness that can be managed at home

Supportive treatment focussing on fluid input, nutrition and temperature control

Advise parents that symptoms peak between 2-5 days of onset

If awaiting referral to hospital, give supplementary oxygen if sats are persistently <92%

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30
Q

What treatment is given for severe bronchiolitis?

A

Admission in severe cases, symptomatic treatment:

1) Oxygen - achieve >92%
2) CPAP / intubation and ventilation
3) Fluids - limit oral needs, use NG tube or IV depending on severity (may need intermittent feeding as may vomit)

NB salbutamol ineffective = too young to have adrengergic receptors

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31
Q

What prophylactic treatment can be given for bronchiolitis?

A

Palivizumab = monoclonal antibody to RSV can be given to high risk groups my monthly IM injection

ie those with bronchopulmonary dysplasia due to premature birth, congenital heart disease or immunodeficiencies

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32
Q

What is croup also known as?

A

Acute laryngotracheitis

Acute laryngotracheobronchitis

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33
Q

Which ages are mostly affected by croup?

A

Typically 6 months - 6 years

Peak incidence at 2 years

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34
Q

What is the aetiology of croup?

A

Most commonly: Parainfluenza viruses types I, II, III and IV (approx 80%)

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35
Q

What is the pathophysiology of croup?

A

Viral URTI causes nasopharyngeal inflammation that may spread to the larynx and trachea, causing sub glottal inflammation, oedema and compromise of the airway at is narrowest portion

Movement of vocal chord impaired = characteristic cough

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36
Q

How may croup present?

A

Initially nonspecific symptoms of viral URTI eg coryzal symptoms, fever, cough (12-72hrs)

This progresses to characteristic seal barking cough and hoarse cry (worse at night)

+/- stridor
Decreased chest sounds if severe airflow limitation
+/- respiratory distress - tachypnoea, intercostal recession etc

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37
Q

What is the natural course of croup?

A

3-7 days but can persist for up to 2 weeks

Usually mild and self limiting, but the distressing symptoms can mean parents present to GP/A&E

Severe cases can compromise upper airway

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38
Q

What is the scoring system for the severity of croup?

A

Westley scoring system

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39
Q

What features to the Westley scoring system involve?

A

1) Inspiratory stridor
Not present = 0
When agitated/active = 1
At rest = 2

2) Intercostal recession
Not present = 0
Mild = 1
Miderate = 2
Severe = 3

3) Air entry
Normal = 0
Mildly decreased = 1
Severely decreased = 2

4) Cyanosis
None = 0
When agitated/active = 4
At rest = 5

5) Level of consciousness
Normal = 0
Altered = 5

Scoring / 17
0-3 = mild
4-6 = moderate
>6 = severe

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40
Q

Most children will have mild croup which can be managed at home. When is immediate assessment in hospital required?

A

Moderate or severe croup, or impending respiratory failure

Any suspicion of epiglottis, bacterial tracheitis, quinsy, retrophayngeal abscess or laryngeal diptheria eg suspicion of severe infection

Also if <6months, immunocompromised etc

41
Q

What investigations may be performed for croup? (5)

A

1) Pulse oximetry (<95% = significant respiratory impairment)

Risk vs benefit of distressing child for:

2) CXR
3) Bloods
4) Rapid influenza A throat swab

NB DO NOT EXAMINE THROAT

42
Q

What is the management of croup?

A

Single dose PO dexamethasone 0.15mg / kg ASAP

Adrenaline NEB:
For patients in moderate to severe distress

43
Q

What are some complications of croup?

A

Complications rare

Bacterial superinfection may result in pneumonia or bacterial tracheitis = S aureus (most common)

Pulmonary oedema
Pneumothorax
Lymphadenitis
OM

44
Q

What is “spasmodic croup?”

A

Barking cough with no apparent respiratory symptoms

45
Q

How common is epiglottitis? What ages are affected

A

Rare since introduction of Haemophilus Influenza type B (Hib) vaccine in 1992

2-5yrs

46
Q

What is the aetiology of epiglotttis?

A

Mostly strep

HSV

Candida and aspergillus in immunocompromised

Non-infectious causes include thermal eg steam / crack cocaine smoking, foreign bodies etc

Reactive epiglottitis can occur as a result of head and neck chemo

47
Q

How may epiglottitis present? (10)

A

1) Sore throat
2) Odynophagia
3) Inability to swallow secretions = drooling in children
4) Muffled ‘hot potato’ voice
5) Fever

6) Tachycardia
7) Anterior neck tenderness over hyoid bone
8) Ear pain
9) Cervical lymphadenopathy
10) Tripod sign = moves inflamed structures forward, thus easing upper airway obstruction

48
Q

What is odynophagia?

A

Painful swallowing

49
Q

How can epiglottitis be distinguished from croup in children?

A

Rare for a child to have a cough with epiglottitis

Croup has seal barking cough

50
Q

What investigations should be done for epilglottitis?

A

DO NOT EXAMINE THROAT WITH TONGUE DEPRESSOR = risk of laryngeal obstruction, they should be urgently referred for laryngoscopy

Fibre-optic laryngoscopy = gold standard

  • Should only be done in theatre as may need to intubate or perform tracheostomy in the event of an upper airway obstruction
  • If not possible, lateral neck x-ray can show ‘thumbprint sign’

Throat swabs when airway secure

Blood cultures if systemically unwell

CT or MRI if abscess suspected

51
Q

How is epilglottitis managed?

A

IV cefotaxime 25-50mg/kg/8hr

Surgical tracheostomy may be required in pt with severe airway obstruction where intubation is not possible

Drainage of abscess if present

52
Q

What are some complications of epiglottitis? (5)

A

1) Abscess formation (25%)
2) Meningitis
3) Sepsis
4) Pneumothorax
5) Pneumo-mediastinitis (v rare)

53
Q

Differentiate between croup and epiglottitis

A 
Croup:
Time course - days
Prodrome - coryza
Cough - barking
Feeding - can drink
Mouth - closed
Toxic - no
Fever - <38.5
Stridor - rasping
Voice - hoarse
Epiglottitis:
Time course - hours
Prodrome - none
Cough - none (or slight)
Feeding - none
Mouth - drooling saliva
Toxic - yes
Fever - >38.5
Stridor - soft
Voice - weak / silent
54
Q

Failure for croup to improve on steroids/nebulisers indicates what?

A

Bacterial tracheitis

55
Q

What is bacterial tracheitis?

A

Thick mucopurulent exudate and tracheal mucosal sloughing that is not cleared by coughing, risks include occluding the airway

Often hx of viral infection (like croup) with an acute deterioration

Pronounced tracheal tenderness may be present

56
Q 
What is the aetiology of childhood pneumonia?
<2yrs
Neonates
Infants
School age
A

Viral more common in <2yrs:

  • RSV
  • Adenovirus
  • Influenza
  • Parainfluenza
  • Hib in immunocompromised

Bacterial (most are strep peumoniae):
- Neonates = GBS, E coli, kiebsiella, s aureus

  • Infants = s pneumoniae (lobar pneumonia), chlamydia
  • School age = s pneumoniae, s aureus, GAS, Bordetella peruses, mycoplasma pneumoniae
57
Q

What are some risk factors for child pneumonia?

A

Immunodeficiency
CF
Sickle cell disease
Tracheostomy in situ

58
Q

What are some signs for childhood pneumonia?

A

1) Fever
2) Cough
3) Malaise
4) Respiratory distress eg tachypnoea, cyanosis, intercostal recession, use of accessory muscles

Hx of preceding URTI very common

59
Q

What is defined as tachypnoea in:

1) 0-5 months
2) 6-12 months
3) Over 12 months

A

1) 0-5 months = >60/min
2) 6-12 months = 50/min
3) Over 12 months >40/min

60
Q

What are some complications of childhood pneumonia?

A

Persistent pneumonia after 48hr treatment could be effusion or empyema

A fluid sample should be taken by chest drain:

  • Empyema diagnosed if acidic pH and protein +++
  • Fluid should be allowed to drain from chest drain and not removed until dry for 48hr
61
Q

What may childhood pneumonia show on examination?

A

1) Dullness to percussion
2) Inspiratory crackles
3) Diminished breath sounds`

62
Q

Audible wheezing is not seen very often in LRTI, however when is it more common?

A

Diffuse infections such as mycoplasma pneumoniae and bronchiolitis

63
Q

What pneumonia may be accompanied by conjunctivitis in the newborn? When?

A

Chlamydial pneumonia may be accompanied by chlamydial conjunctivitis

Second or third week

64
Q

Which pneumonia is particularly concerning in first 24 hours of life?

A

Streptococcal sepsis and pneumonia

65
Q

What type of pneumonia may present with abdominal pain in toddlers?

A

Lower lobe pneumonia

67
Q

What investigations may be done in pneumonia?

A

Not required in a CAP with child going home

1) FBC - raised WCC
1) Blood cultures - rarely positive in pneumonia (fewer than 10% are bacteraemic in pneumococcal disease)
2) Sputum culture / nasopharyngeal aspirate
3) CXR

Often hard to distinguish between viral / bacterial

67
Q

What should be tested for in recurrent childhood pneumonia? How?

A

CF - Sweat test

68
Q

When should admission be considered for a childhood pneumonia? (9)

A

1) O2 sats <92%
2) RR >70 (or 50 in older child)
3) Significant tachycardia
4) Prolonged CRT
5) Difficulty breathing shown by intermittent apnoea, grunting, not feeding
6) Presence of comorbidity eg congenital heart disease / CF
7) Age <6 months
8) Those in which abx have failed - should see improvement within 48hrs PO abx
9) Pleuritic pain

69
Q

What is the treatment of childhood pneumonia?

A

Mostly managed at home with fluids, antipyretics and abx if bacterial

<5yrs = amoxicillin
>5yrs = amoxicillin / macrolide eg clarithromycin
Severe = Coamoxiclav / IV cephaloposporin (cefotaxime, cefuroxime)
70
Q

What is the Centor Criteria?

A

Give up to 50% predictive power for bacterial cause of URTI

71
Q

What features are included in the Centor Criteria?

A

One point for each criterion met:

1) Fever >38 degrees celcius
2) Tonsillar exudate
3) Absent cough
4) Anterior cervical lymphadenopathy
5) Age 3-14
- Bacterial cause <3 is rare
- >45 = -1

72
Q

What is mesenteric adenitis?

A

Intermittent abdo pain caused by inflamed lymph glands usually due to viral infection

Adenitis = inflamed lymph glands

73
Q

What should be included on examination of URTI?

A

ENT examination:

  • Ear discharge
  • Bulging ear drum

Cervical lymphadeopathy

Purulent tonsillar exudate
- Seen in GABH or EBV

Check there is no difficulty breathing in infants / neonates

74
Q

What is the treatment of an URTI?

A

Mostly symptomatic

Sale drops/gentle suction can remove obstructing mucus

Abx only if swab +ve for GABH:
- Penicillin V
or erythromycin for 10 days

75
Q

Why should you avoid amoxicillin when treating an URTI?

A

Can cause an itchy maculopapular rash if the cause is EBV

76
Q

What may an URTI precipitate in a child?

A

Febrile convulsions

77
Q

What is a viral induced wheeze?

A

Wheeze related to viral UTI

Reversible obstruction = responds to bronchodilators

Admit if needing inhalers more
than 4hrly or needing oxygen

Often “grow out of it” but may develop into asthma

No role for steroids

Occasionally use montelukast or ICS if recurrent

Beware of foreign body !

78
Q

What genes are involved in CF

A

Autosomal recessive disease caused by mutation of CFTR on Chr7

79
Q

List the features of CF

A

1) High sodium sweat
2) Pancreatic insufficiency
3) Biliary disease
4) GI disease
5) Respiratory disease

80
Q

When does CF present? What is the most common presentation?

A

Normal digestive function possible with <5% pancreatic function = CF can present at any age

Most common presentation its recurrent LRTI with chronic sputum production

81
Q

What screening is performed for CF?

A

Immunoreactive trypsinogen (IRT) measured on a dried spot of blood obtained on the Guthrie card at day 6 of life

Samples with raised IRT will undergo CFTR mutation screening

82
Q

How is CF diagnosed antenatally?

A

Amniocentesis / CVS

US demonstration of bowel perforation / hyperchogenic bowel (bright bowel) = 4% due to CF

83
Q

How is CF diagnosed perinatally?

How may it present?

A

Screening

Bowel obstruction with meconium ileus (bowel atresia)

Haemorrhagic disease of the newborn
- Vit K deficiency due to malabsorption of fat soluble vitamins due to pancreatic insufficiency (but rare as vit K given after birth)

Prolonged jaundice
- Extrahepatic biliary obstruction from bile of increased density with secondary intrahepatic bile stasis

84
Q

What is meconium ileus?

A

Obstruction of the terminal ileum by abnormally thick / adherent meconium

Symptoms include bilious vomiting, abdo distention and failure to pass meconium in the first few days of life

85
Q

How may CF present in infancy and childhood?

A

1) Recurrent RTI
2) Diarrhoea
3) FTT
4) Rectal prolapse
- Frequent coughing or hard-to-pass stools
5) Nasal polyps = in children this is nearly always due to CF
6) Acute pancreatitis
7) Portal HTN and variceal haemorrhage
8) Electrolyte abnormality
- Can develop Pseudo-Bartter’s syndrome
9) Hypoproteinaemia and oedema

86
Q

What signs may be observed with CF?

A

1) Finger clubbing
2) Cough with purulent sputum
3) Crackles
4) Wheeze - mainly upper lobes
5) FEV1 shows obstruction

NB babies with CF usually have no signs / symptoms

87
Q

What investigations are done for CF?

A

Sweat test = 98% sensitive
- Chloride >60mmol/L with sodium concentration lower than that of chloride on 2 separate occasions

Molecular genetic testing for CFTR gene

Sinus X-ray / CT scan = opacification of sunless

CXR / CT thorax

Lung funcitoning test - spirometry unreliable <6yr

Sputum microbiology

88
Q

What is most of the morbidity and mortality with CF in respiratory disease related to?

A

Chronic infection and inflammation lead to bronchiectasis, progressive airflow obstruction, cor pulmonate and death

+ Inc bacterial resistance esp p aeruginosa

89
Q

What interventions are used to prevent and treat respiratory problems related to CF?

A

Mucus clearance, preventing infection nd maintaining good lung function are main aims

Chest physiotherapy twice daily (inc with infective exacerbations)

Regular sputum samples sent for culture

Prophylactic abx to reduce s aureus in children and to prevent secondary bacterial infections when pt has presumed acute viral RTI

Patient with P. aeruginosa have 2/3 inc risk of death >8yr:
- Given combination of PO, NEB and IV abx

Bronchodilators:
- LABA / SABA

Dornase alfa NEB
- Reduces sputum viscosity

High-dose ibuprofen may slow progression of lung diseases

Azithromycin may reduce inflammation

Hypertonic saline by NEB given for osmotic action

90
Q

How are nasal polyps treated in CF? What is recurrence rate?

A

Steroids initially

Polypectomy if fails

50% require repeat within 2 yrs

91
Q

How may pancreatic insufficiency present in CF?

A

Meconium ileus
FTT
Steatorrhoea
Malnutrition

Which can lead to:
Anaemia
Vit deficiency
Sometimes obstruction

Which can cause:
Rectal prolapse
Intussusception
Volvulus
Obstruction
92
Q

How is pancreatic insufficiency diagnosed in CF?

A

Confirm with stool elastase = presence of unsplit fat globule in stool or 2-3 days stool collection of fatal fat

93
Q

How is adequate weight maintained in CF?

A

High energy + protein

Enteric coated enzymes before meals + adjust to achieve normal stools

Vitamin supplements

?acid lowering meds

94
Q

What proportion of children and adolescents with CF develop diabetes?

A

2% children
19% adolescents

(40-50% adults)

95
Q

What is the treatment of CF-related DM?

A

Insulin replacement usually required with dose adjusted to match high dietary intake

96
Q

What other systems are affected by CF?

A

Liver disease - 30% of adults will develop

  • Check LFTs
  • Usually seen hepatosplenomegaly
  • USS
  • Liver transplant in liver failure if good lung function

Osteoporosis - 33% adults

  • Take calcium, vit D, bisphosphonates as required
  • Regular DEXA scans
97
Q

What follow up is required for CF pts?

A

Recommended to be seen twice yearly by MDT working in specialist centre

Investigations performed at regular intervals:

1) Pulmonary function tests
2) DEXA scan
3) Blood glucose
4) Respiratory cultures
5) Liver USS
6) Vit D levels

98
Q

What vaccines are offered in CF populations?

A

1) Annual influenza
2) Pneumococcal

+ varicella antibodies checked for and varicella vaccine offered to children who need it

99
Q

What is the estimated survival for a child born with CF?

A

40-50yrs

Year 4 PAEDS (23 decks)

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