Paediatricts- respiratory Flashcards

1
Q

Conditions which are tested on the Guthrie test (5 days)

A
  • Sickle cell disease
  • Cystic fibrosis
  • Congenital hypothyroidism
  • Phenylketouria
  • Medium chain acyl-CoA dehydrogenase deficience (MCADD)
  • Maple syrup urine disease
  • Isovaleric acidaemia (IVA)
  • Glutaric aciduria type 1(GA1)
    *homocystinuria (pyridoxine unresponsive) (HCU)
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2
Q

Cystic fibrosis- Pathophysiology

A
  • It is due to a defect in the cystic fibrosis transmembrane conductance regulator gene (CFTR), which codes a cAMP-regulated chloride channel.
  • Causes excess sodium and water re-absorption resulting in dehydration and impaired clearance of respiratory secretions.
  • The airway becomes obstructed with respiratory secretions where microbes grow causing infection, inflammation and eventual tissue destruction.
  • This leads to bronchiectasis and eventual respiratory failure.
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3
Q

Cystic fibrosis- genetics

A
  • Cystic fibrosis (CF) is an autosomal recessive disorder causing increased viscosity of secretions (e.g. lungs and pancreas).
  • In the UK 80% of CF cases are due to delta F508 on the long arm of chromosome 7. Cystic fibrosis affects 1 per 2500 births, and the carrier rate is c. 1 in 25
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4
Q

Organisms which may colonise CF patients

A
  • Staphylococcus aureus
  • Pseudomonas aeruginosa
  • Burkholderia cepacian i.e. Pseudomonas cepacia
  • Aspergillus
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5
Q

CF diagnosis

A

Sweat test
* Patients with CF have abnormally high sweat chloride
* Normal value <40 mEq/l, CF is indicated by >60 mEq/l
* Causes of false positive: malnutrition, adrenal insufficiency, glycogen storage disease, nephrogenic diabetes insipidus, hypothyroidism, hypoparathyroidism, G6PD, ectodermal dysplasia
* The most common reason for false negative tests is skin oedema, often due to hypoalbuminaemia/hypoproteinaemia secondary to pancreatic exocrine insufficiency

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6
Q

CF- Presenting features

A
  • neonatal period (around 20%): meconium ileus, less commonly prolonged jaundice
  • recurrent chest infections (40%)
    *malabsorption (30%): steatorrhoea, failure to thrive. Due to pancreatic insufficiency, can be confirmed with a stool sample to perform a faecal elastase test. Low level suggests insufficiency (<200 ug E1/g faeces)
  • other features (10%): liver disease
  • Normally diagnosed in the newborn screening programmes
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7
Q

Other features of cystic fibrosis

A
  • short stature
  • diabetes mellitus
  • delayed puberty
  • rectal prolapse (due to bulky stools)
  • nasal polyps
  • male infertility, female subfertility
  • Life expectancy: 45
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8
Q

Management of cystic fibrosis

A
  • regular (at least twice daily) chest physiotherapy and postural drainage. Parents are usually taught to do this. Deep breathing exercises are also useful
  • high calorie diet, including high fat intake*
  • patients with CF should try to minimise contact with each other to prevent cross infection with Burkholderia cepacia complex and Pseudomonas aeruginosa
  • vitamin supplementation
  • pancreatic enzyme supplements taken with meals
  • lung transplantion
  • chronic infection with Burkholderia cepacia is an important CF-specific contraindication to lung transplantation
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9
Q

Medication for cystic fibrosis

A
  • Sodium chloride: aids growth until on a fully weaned diet. Older also require additional salt if they are in a hot climate for a period of time
  • Flucloxacillin: used as prophylaxis against staph aureus in the lungs
  • Creon: contains pancreatic enzymes to aid absorption of fat for patients with pancreatic insufficiency
  • Abidec: a multivitamin to reduce issues related to malabsorption
  • Vitamin A, D & E: fat soluble vitamins for pancreatic insufficient patients only
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10
Q

Lumacaftor/Ivacaftor (orkambi)

A
  • is used to treat cystic fibrosis patients who are homozygous for the delta F508 mutation
  • lumacaftor increases the number of CFTR proteins that are transported to the cell surface
  • ivacaftor is a potentiator of CFTR that is already at the cell surface, increasing the probability that the defective channel will be open and allow chloride ions to pass through the channel pore
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11
Q

Cystic fibrosis infection-

A
  • Common organisms: Staph aureus, Haemophillus influenzae, Moraxella Catarrhalis
  • Treatment: 2 weeks of oral antibiotics (amoxicillin/co-amoxiclav). If don’t respond to antibiotics or have signs of LRTI use two weeks of IV Cefuroxime
  • Organisms which can have a long term impact on lung function: Pseudomonas Aeroginosa, Mycobacterium Abscessus, Burkholderia Cepacia
  • Mycobacterium Abscessus & Burkholderia Cepacia are very significant and always require IV antibiotics
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12
Q

Pseudomonas Aeuroginosa treatment

A
  • If child is well, the infection can be treated with 2 weeks of oral ciprofloxacin & 3 months of inhaled tobramycin/colomycin.
  • If the child is unwell or displays signs of a Lower Respiratory Tract Infection (LRTI) then he or she requires admission for 2 weeks of IV ceftazidime and IV tobramycin & 3 months of inhaled colomycin. They would also receive intensive physiotherapy & dietician input.
  • After 3 separate growths of Pseudomonas a child is considered to be colonised. When a child isolates Pseudomonas they must be seen in a Pseudomonas positive clinic to prevent cross contamination.
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13
Q

Advanced CF

A
  • Barrel shaped chest with crepitations
  • Clubbing
  • Reduced FEV1
  • Hyperinflation and bronchiectasis- the airways become abnormally widened and scarred with a build up of thick mucus which is prone to infection
  • May have a portocath for IV administration of antibiotics
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14
Q

Allergic rhinitis- classification

A
  • Almost 80% children with Asthma will have Allergic Rhinitis
  • Can be classified as intermittent (< 4 days/week or <4 weeks per year) or Persistent ( > 4 days/week and >4 weeks per year)
  • Can also be classified as mild (normal sleep and daily activity) or moderate to severe (disrupts sleep and normal activities)
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15
Q

Allergic rhinitis- symptoms

A
  • Presents usually with nasal symptoms (rhinorrhea, itching, sneezing, blocked nose), Eye symptoms (conjunctivitis, runny itchy eyes)
  • Can present with mouth breathing or chronic otitis media with effusion
  • Signs may include the “allergic salute” (from constant nose rubbing), Dennie- Morgan lines ( infraorbital line or fold from chronic rubbing)
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16
Q

Symptoms suggestive of allergic conjunctivitis

A

1 or more of the following symptoms for >1h on most days:
* Symptoms associated with rhinitis
* Bilateral eye symptoms
* Eye itching
* Red eye
* No photophobia

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17
Q

Symptoms suggestive of allergic rhinitis

A

2 or more of the following symptoms for >1 hour most days
* Watery rhinorrhea
* Sneezing, especially paroxysmal
* Nasal obstruction
* Nasal pruritis
* +/- conjunctivitis

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18
Q

Treatment of allergic rhinitis

A
  • Allergen avoidance if known
  • Antihistamines for mild
  • Nasal steroids for moderate/severe
  • Montelukast (LTRA) can be added to steroids
  • Immunotherapy is in sublingual and subcutaneous forms in severe cases
  • Important to treat as it has a significant impact on quality of life
  • May help to control asthma better if co-exists
  • Allergic conjunctivitis: eye drops (sodium cromoglycate or olopatadine)
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19
Q

Pneumonia causes

A

Infection affecting the distal airways especially alveoli

  • Viruses ( RSV, Parainfluenza, Influenza, adenovirus)
  • Bacteria (Strep Pneumoniae, Group A Strep, Haemophilus Influenzae, Moraxella Catarrhalis)
  • Atypical organisms ( eg Mycoplasma)

S.pneumoniae is the most likely causative agent of bacterial pneumonia in children then Mycoplasma Pneumoniae and Chlamydia Pneumoniae.

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20
Q

Treatment for pneumonia

A
  • Amoxicillin is first-line for all children with pneumonia
  • Macrolides (Clarithromycin) may be added if there is no response to first line therapy
  • Macrolides should be used if mycoplasma or chlamydia is suspected
  • In pneumonia associated with influenza, co-amoxiclav is recommended
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21
Q

Pneumonia- symptoms and investigations

A
  • Clinical features- cough with or without wheeze, fever, increased work of breathing, chest or abdominal pain, crackles, bronchial breathing
  • Remember to count respiratory rate as tachypnoea may be the only sign and is easily missed
  • Most children do not require any investigation
  • Blood tests chest X ray, IV antibiotics needed only if likely to require intensive care or if complications like pleural effusion or empyema suspected or underlying chest problems e.g. CF, Immunodeficiency
22
Q

Pneumonia- advice at home

A
  • Use either paracetamol or ibuprofen with fever, make sure the child is drinking adequately and passing urine
  • Don’t give both simultaneously
  • Don’t reduce fever by under dressing the child or using tepid sponging
  • Continue breastfeeding
23
Q

Pneumonia- seek medical advice when

A
  • Breathing rate increases or there are any episodes of apnoea, signs of cyanosis, or increased effort of breathing (such as grunting, nasal flaring, or marked chest recession).
  • The child’s fluid intake is reduced to 50–75% of normal, or there are signs of dehydration such as dry mouth or no wet nappy for 12 hours.
  • The child becomes less responsive or difficult to rouse.
  • There is persistent worsening of fever, or if fever does not settle within 48 hours of initiation of antibiotic treatment.
24
Q

Bronchiolitis: key features

A
  • Common in babies and in young children below 2 years of age
  • Usually caused by viruses (mainly RSV), other causes: mycoplasma, adenoviruses
  • May be secondary to a bacterial infection
    *More serious if bronchopulmonary dysplasia i.e. premature, congenital heart disease or cystic fibrosis
  • Usually presents with a characteristic cough or difficulty feeding
  • Most children do not need any investigation
  • Treatment is supportive (oxygen and Nasogastric feeds as needed)
    *Palivizumab (RSV vaccine ) is offered to high risk babies ( eg congenital heart disease, babies on home oxygen)
25
Q

Allergic rhinitis- medical management examples

A

Initial/mild to moderate
- intranasal antihistamine (azelastine)
- OR 2nd gen non-sedating oral antihistamine (loratadine, cetirizine)
- OR intranasal chromone (sodium cromoglicate)

Moderate to severe/persistent/2nd line
- intranasal corticosteroid (mometasone, fluticasone)
- Consider eye drops e.g. cromoglicate, olopatadine

26
Q

Bronchiolitis= babies at high risk of developing severe disease and requiring admission to hospital

A
  • Premature babies (< 32 weeks)
  • Chronic lung disease (home oxygen)
  • Congenital heart disease
  • Young babies (less than 12 weeks old)
  • Neuromuscular disease
  • Consider admission if there are social concerns for example parents with learning difficulties, young parents with poor support, remote location and difficulty accessing care
27
Q

Symptoms of Bronchiolitits

A

Coryzal features (including mild fever) precede:
* Dry cough
* Increasing breathlessness
* Wheezing, fine inspiratory crackles
* Feeding difficulties associated with increasing dyspnoea and often the reason for hospital admissions
* Increased work of breathing: tachypnoea, nasal flaring, head bobbing, tracheal tug, intercostal/sternal recessions and abdominal breathing

28
Q

Bronchiolitis: NICE recommends immediate referral (usually by 999 ambulance) if they have any of the following

A
  • apnoea (observed or reported)
  • child looks seriously unwell to a healthcare professional
  • severe respiratory distress, for example grunting, marked chest recession, or a respiratory rate of over 70 breaths/minute
  • central cyanosis
  • persistent oxygen saturation of less than 92% when breathing air.
29
Q

Bronchiolitis: NICE recommend that clinicians consider referring to hospital if any of the following apply

A
  • a respiratory rate of over 60 breaths/minute
  • difficulty with breastfeeding or inadequate oral fluid intake (50-75% of usual volume ‘taking account of risk factors and using clinical judgement’)
  • clinical dehydration.
30
Q

Bronchiolitis: investigations and management

A
  • Investigations: immunofluorescence of nasopharyngeal secretions may show RSV
  • Management is largely supportive
  • Humidified oxygen is given via a head box and is typically recommended if the oxygen saturations are persistently < 92%
  • Nasogastric feeding may be needed if children cannot take enough fluid/feed by mouth
  • Suction is sometimes used for excessive upper airway secretions
  • Severe cases: CPAP, endotracheal intubation and ventilation
31
Q

Bronchiolitis: discharge criteria

A
  • Oxygen saturation maintained at >92% for 4 hours including a period of sleep
  • Adequate oral intake (75% of normal)
  • If babies are seen at the beginning of their illness inform the parents that they need to be carefully observed as they often get worse (day 3) before they get better
32
Q

Bronchiolitis: High risk babies

A
  • Babies who have chronic lung disease of prematurity
  • Babies on home oxygen
  • Presence of Immunodeficiency
  • Presence of Congenital heart disease
  • These children are given the palivizumab injections in winter
33
Q

Asthma: key features

A
  • Clinical diagnosis
  • Usually difficult to do spirometry in children <7 years old
  • Normal spirometry does not exclude asthma but an obstructive picture ( FEV1/FVC ratio <80%, At least 12% increase to FEV1 post salbutamol ) or a high exhaled Nitric oxide measurement ( >20 ppb) support the diagosis
  • Other atopic conditions ( Eczema, Allergic Rhinitis, Food allergy) and a positive family history may be present
    *children with asthma and nut allergy are at risk of anaphylaxis
34
Q

Asthma: symptoms

A
  • Almost all children with asthma have intermittent cough, wheeze and/or exercise induced symptoms but only a quarter of children with these symptoms have asthma
  • Two third of children with a cluster of symptoms have asthma
  • Wheeze : continuous high pitched musical sound coming from the chest
  • Wheeze heard by a healthcare professional on auscultation increases the probability of asthma
35
Q

Acute asthma

A
  • Most children will improve with bronchodilators ( salbutamol +/- ipratropium bromide and oral steroids)
  • Nebulised magnesium sulphate is sometimes added to nebulised bronchodilators in severe asthma attacks
  • Intravenous treatment with either salbutamol, aminophylline or magnesium sulphate may be needed in severe or life threatening asthma attacks
  • Inhaled steroids, Leukotriene receptor antagonists ( Montelukast), long acting beta 2 agonists are used in a stepwise manner in chronic asthma
36
Q

Moderate acute asthma attack

A
  • Able to talk in sentences
  • SpO2 >92%
  • PEF >50% best or predicted
  • In children >5: heart rate <125/min, respiratory rate <30/min
  • In children 1-5: heart rate <140/min, respiratory rate <40/min
37
Q

Asthma: severe attack

A
  • SpO2 <92%
  • PEF 33-50% best or predicted
    *Too breathless to talk or feed
  • Heart rate: >125 (>5 years), >140 (1-5 years)
  • Respiratory rate: >30 breaths/min (>5 years), >40 (1-5 years)
  • Use of accessory neck muscles
38
Q

Asthma: Life threatening attack

A
  • SpO2 <92%
  • PEF <33% best or predicted
  • Silent chest
  • Poor respiratory effort
  • Agitation
  • Altered consciousness
  • Cyanosis
39
Q

Treatment for children with mild to moderate acute asthma

A
  • Bronchodilator therapy: give a beta- 2 agonist via a spacer (for a child <3 years use a close fitting mask). Give 1 puff every 30-60 seconds up to a maximum of 10 puffs. If symptoms are not controlled repeat beta-2 agonist and refer to hospital
  • Steroid therapy: should be given to all children with an asthma exacerbation, treatment should be given for 3-5 days
  • Will need regular nebulisers (salbutamol) until able to maintain oxygen saturation above 94% in air and clinically the work of breathing improves at which point can be switched to an inhaler via a space
40
Q

Treatment for severe exacerbation of asthma

A
  • Oxygen via facemask
  • Nebulised salbutamol; 5mg
  • Consider Prednisolone 30-40mg or IV Hydrocortisone 4mg/kg
  • If poor response add nebulised Ipratropium Bromide 250mg
  • Will need regular nebulisers (salbutamol) until able to maintain oxygen saturation above 94% in air and clinically the work of breathing improves at which point can be switched to an inhaler via a space
41
Q

Treatment for life threatening asthma

A
  • Oxygen
  • Nebulised salbutamol 5mg plus Ipratropium Bromide 250micrograms
  • Consider IV Hydrocortisone 4mg/kr or 100mg
  • Discuss with consultant paediatrician
  • Will need regular nebulisers (salbutamol) until able to maintain oxygen saturation above 94% in air and clinically the work of breathing improves at which point can be switched to an inhaler via a space
42
Q

Usual prednisolone dose

A
  • Age 2-5 years: 20mg OD or 1-2mg/kg od (max 40mg)
  • > 5 years: 30-40mg od or 1-2 mg/kg od (max 40mg)
43
Q

Asthma: steps of treatment aged 5-16

A
  1. Newly diagnosed asthma: SABA
  2. Not controlled by step 1 or Newly diagnosed asthma with symptoms >=3/week or night time waking= SABA + paediatric low dose ICS
  3. SABA + paediatric low dose ICS + LTRA
  4. SABA + paediatric low dose ICS + LABA. Stop LTRA
  5. SABA + switch ICS/LABA for a maintenance and reliever therapy (MART), that includes a paediatric low-dose ICS
  6. SABA + paediatric moderate dose ICS MART
44
Q

Asthma: steps of treatment for children under 5

A
  1. Newly diagnosed: SABA
  2. Not controlled on previous step OR newly diagnosed asthma with symptoms >=3/ week or night time waking= SABA + an 8 week trial of paediatric MODERATE dose ICS
  3. SABA + paediatric low dose ICS + LTRA
  4. Stop the LTRA and refer to an paediatric asthma specialist
45
Q

Asthma: after 8 weeks stop the ICS and monitor the childs symptoms

A
  • if symptoms did not resolve during the trial period, review whether an alternative diagnosis is likely
  • if symptoms resolved then reoccurred within 4 weeks of stopping ICS treatment, restart the ICS at a paediatric low dose as first-line maintenance therapy
  • if symptoms resolved but reoccurred beyond 4 weeks after stopping ICS treatment, repeat the 8‑week trial of a paediatric moderate dose of ICS
46
Q

Asthma: Maintenance and reliever therapy (MART)

A
  • a form of combined ICS and LABA treatment in which a single inhaler, containing both ICS and a fast-acting LABA, is used for both daily maintenance therapy and the relief of symptoms as required
  • MART is only available for ICS and LABA combinations in which the LABA has a fast-acting component (for example, formoterol)
47
Q

Acute asthma: reducing salbutamol puffs when going home

A
  • Step 1: take 10 puffs of salbutamol, using the spacer every 4 hours. Do this for 36 hours
  • Step 2: take 6 puffs of salbutamol, using a spacer as above every 4 hours for 36 hours
  • Step 3: take 2 to 4 puffs of salbutamol as above, every 4 hours for 36 hours
48
Q

Acute asthma: stridor

A
  • Upper airway obstruction
  • Most commonly the result of laryngeal and tracheal infection
  • Differential diagnosis: Croup, Foreign body, Congenital abnormality, Epiglottis, Bacterial tracheitis, Anaphylaxis
49
Q

Croup- key features

A
  • 95% of laryngotracheal infections
  • Viral in origin
  • Parainfluenza, rhinovirus, RSV, influenza
  • Symptoms: Hoarseness, ‘barking cough’, stridor, worse at night, fever, coryzal symptoms
  • Peak incidence 6 months- 3 years, more common in autumn
50
Q

Croup- management

A
  • ABCDE
  • Oral Dexamethasone/prednisolone or if severe, nebulised adrenaline
  • If the child is too unwell to receive medication, use inhaled budesonide (2 mg nebulised as a single dose) or intramuscular dexamethasome (0.6 mg/kg as a single dose)