Paeds: Resp Flashcards

1
Q

Cystic Fibrosis

what is it

A

an autosomal recessive genetic condition affecting mucus glands

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2
Q

Cystic Fibrosis

what is it caused by

A

a genetic mutation of the cystic fibrosis transmembrane conductance regulatory gene

on Ch7

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3
Q

Cystic Fibrosis

what is the most common variant of the cystic fibrosis transmembrane conductance regulatory gene

A

the delta-F508 mutation: codes for cellular channels, particularly a type of chloride channel

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4
Q

Cystic Fibrosis

what causes a lack of lipase in the digestive tract

A

thick pancreatic and biliary secretions that cause a blockage of the ducts

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5
Q

Cystic Fibrosis

what causes the susceptibility to airway infections and bacterial colonisation

A

low volume thick airway secretions that reduce airway clearance

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6
Q

Cystic Fibrosis

what causes male infertility

A

congenital bilateral absence of the vas deferens

the sperm have no way of getting from the testes to the ejaculate

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7
Q

Cystic Fibrosis

what is the mode of inheritence

A

autosomal recessive

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8
Q

Cystic Fibrosis

both parents are healthy, one sibling has cystic fibrosis and a second child does not have the disease, what is the likelihood of the second child being a carrier?

A

2 in 3

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9
Q

Cystic Fibrosis

when is it screened

A

at birth with the newborn bloodspot test

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10
Q

Cystic Fibrosis

what is often the first sign

A

meconium ileus (not passing meconium within 24h)

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11
Q

Cystic Fibrosis

sx

A
  • chronic cough
  • thick sputum production
  • recurrent resp tract infections
  • steatorrhoea
  • abdo pain and bloating
  • salty kisses
  • failure to thrive
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12
Q

Cystic Fibrosis

why does steatorrhoea occur

A

due to the lack of fat digesting lipase enzymes

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13
Q

Cystic Fibrosis

signs

A
  • low weight or height
  • nasal polyps
  • finger clubbing
  • crackle + wheezes on auscultation
  • abdo distention
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14
Q

what are causes of clubbing in children

A
  • hereditary clubbing
  • cyanotic heart disease
  • infective endocarditis
  • cystic fibrosis
  • TB
  • IBD
  • liver cirrhosis
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15
Q

Cystic Fibrosis

what are the 3 key methods for establishing a diagnosis

A
  • newborn blood spot testing
  • sweat test
  • genetic testing for CFTR gene during pregnancy
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16
Q

Cystic Fibrosis

what is the gold standard for diagnosis

A

sweat test

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17
Q

Cystic Fibrosis

what happens during a sweat test

A
  • pilocarpine applied to skin patch
  • electrodes places on either side and current is passed between electrodes
  • this causes skin to sweat
  • sweat absorbed by filter paper
  • sent to lab for Chloride conc
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18
Q

Cystic Fibrosis

what is the diagnostic concentration in the sweat test

A

> 60mmol/l

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19
Q

Cystic Fibrosis

why does bacteria colonate in these pts easily

A

they struggle to clear secretions in the airway

perfect environment with moisture and oxygen to colonise

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20
Q

Cystic Fibrosis

what are key microbial colonisers

A

Staph aureus

pseudomonas

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21
Q

Cystic Fibrosis

how do pts prevent staph aureus infection

A

take long term prophylactic flucloxacillin

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22
Q

Cystic Fibrosis

why should CF patients stay away from each other

A

risk of spreading pseudomonas

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23
Q

Cystic Fibrosis

why are pseudomonas difficult

A

resistant to multiple antibiotics

significant increase in morbidity and mortality

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24
Q

Cystic Fibrosis

trx for Pseudomonas colonisation

A

long term nebulised abx: tobramycin

PO ciprofloaxin alternatively

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25
Q

Cystic Fibrosis

mnx

A
  • chest physio
  • exercise
  • high calorie diet
  • CREON tablets
  • prophylactic flucloxacillin
  • treat chest infections
  • salbutamol
  • neb DNase (dornase alfa)
  • neb hypertonic saline
  • vaccinations: pneumococcal, influenza, varicella
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26
Q

Cystic Fibrosis

what is Nebulised DNase (dornase alfa)

A

an enzyme that can break down DNA material in respiratory secretions, making secretions less viscous and easier to clear

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27
Q

Cystic Fibrosis

why do they need follow up every 6m

A
  • sputum culture for pseudomonas

- screening for diabetes, osteoporosis, vitamin D deficiency and liver failure

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28
Q

Bronchiolitis

what is it

A

inflammation and infection in the bronchioles, the small airways of the lungs

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29
Q

Bronchiolitis

what is it caused by

A

Respiratory syncytial virus (RSV)

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30
Q

Bronchiolitis

what age is it most common in

A

<6m

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31
Q

Bronchiolitis

what is heard on auscultation

A

wheeze and crackles

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32
Q

Bronchiolitis

why does it have a significant effect on infants and not adults

A

the airways of infants are very small to begin with

so even the smallest amount of inflammation and mucus in the airway has a significant effect on the infant’s abaility to circulate air to the alveoli and back out

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33
Q

Bronchiolitis

presentation

A
  • Coryzal sx
  • resp distress signs
  • dyspnoea
  • tachypnoea
  • poor feeding
  • mild fever
  • apnoeas
  • wheeze and crackles on auscultation
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34
Q

Bronchiolitis

what are coryzal sx

A
  • snotty nose
  • sneezing
  • mucus in throat
  • watery eyes
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35
Q

Bronchiolitis

signs of respiratory distress (9)

A
  1. raised RR
  2. use of accessory muscles
  3. intercostal recessions
  4. subcostal recessions
  5. nasal flaring
  6. head bobbing
  7. tracheal tugging
  8. cyanosis
  9. abnormal airway noises
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36
Q

Bronchiolitis

what is wheezing

A

whistling sound caused by narrowed airways, typically heard during expiration

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37
Q

what is grunting

A

caused by exhaling with the glottis partially closed to increase positive end-expiratory pressure

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38
Q

what is stridor

A

a high pitched inspiratory noise caused by obstruction of the upper airway, for example, in croup

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39
Q

Bronchiolitis

what is the typical RSV course

A
  • starts with URTI: coryzal sx
  • half get better, half develop chest sx over the next 1-2d
  • sx last 7-10d
  • fully recover in 2-3w
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40
Q

Bronchiolitis

what day are sx generally worst on

A

day 3-4

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41
Q

Bronchiolitis

what are infants more likely to have during childhood if they have had bronchiolitis as an infant

A

viral induced wheeze

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42
Q

Bronchiolitis

reasons for admission

A
  • <3m
  • preexisting condition: prem, Down’s, CF
  • 50-75% less of their normal intake of milk
  • clinical deyhdration
  • RR>70
  • O2 sats <92%
  • mod-sev resp distress
  • apnoeas
  • parents not confident
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43
Q

Bronchiolitis

mnx

A

supportive:

  • adequate intake,
  • saline nasal drops and suctioning
  • O2 if <92%
  • ventilatory support if required
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44
Q

Bronchiolitis

mnx: why is it important to avoid overfeeding

A

a full stomach will restrict breathing

start with small frequent feeds and gradually increase as tolerated

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45
Q

Bronchiolitis

what are the steps in order of severity for ventilatory support

A
  1. high flow humidified O2
  2. CPAP
  3. intubation + ventilation
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46
Q

Bronchiolitis

what is high flow humidified O2

A

via tight nasal cannula

delivers air + O2 continuously with some added pressure

it adds positive end-expiratory pressure to maintain the airway at the end of expiration

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47
Q

Bronchiolitis

what is CPAP

A

sealed nasal cannular performs similarly to high flow (Airvo ot Optiflow)

but can deliver much higher and more controlled pressures

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48
Q

Bronchiolitis

what does intubation and ventilation involve

A

inserting an endotracheal tube into the trachea to fully control ventilation

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49
Q

Bronchiolitis

what inx is useful in severe resp distress and in monitoring children who are having ventilatory support

A

capillary blood gas

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50
Q

Bronchiolitis

what are the most helpful signs of poor ventilation

A
  • rising pCo2

- falling pH

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51
Q

Bronchiolitis

what does rising pCO2 show

A

the airways have collapsed and can’t clear waste CO2

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52
Q

Bronchiolitis

what does a falling pH show

A

CO2 is building up and not able to buffer the acidosis this creates

this is resp acidosis

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53
Q

Bronchiolitis

which babies are high risk

A

ex-premature

congenital heart disease

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54
Q

Bronchiolitis

what is given to high risk babies

A

Palivizumab

55
Q

Bronchiolitis

what is Palivizumab

A

a monoclonal antibody that targets RSV

monthly injection is given as prevention against RSV

56
Q

Bronchiolitis

why is Palivizumab not a true vaccine

A

it does not stimualte the infant’s immune response

it provided passive protection

57
Q

Viral-Induced Wheeze

what is it

A

an acute wheezy illness caused by a viral inection

58
Q

Viral-Induced Wheeze

pathophysiology

A

virus

inflammation and oedema, swelling the walls of the airways

smooth muscle constricts

wheeze due to air flowing through these narrow airways

59
Q

Viral-Induced Wheeze

what is Poiseuille’s law

A

flow rate is proportional to the radius of the tube to the power of 4

slight narrowing of an infant’s airway leads to a proportionally larger restriction in airflow.

60
Q

Viral-Induced Wheeze

what are they more at risk of developing in later life

A

asthma

61
Q

Viral-Induced Wheeze

what is the difference between asthma and viral induced wheeze

A

viral induced wheeze:

  • presenting before 3y
  • no atopic hx
  • only occurs during viral infections

asthma:

  • other triggers: cold, dust, emotions
  • reversible airflow obstruction
62
Q

Viral-Induced Wheeze

presentation

A
  • initially viral illness
  • SOB
  • signs of resp distress
  • expiratory wheeze throughout the chest
63
Q

what could a focal wheeze show

A

focal airway obstruction such as an inhaled foreign body or tumour

64
Q

Viral-Induced Wheeze

mnx

A

same as acute asthma in children

65
Q

what is the most common side effect of salbutamol

A

tachycardia

66
Q

Acute asthma

signs of moderate severity

A
  • Peak flow > 50 % predicted

- Normal speech

67
Q

Acute asthma

signs of severe severity

A
  • Peak flow < 50% predicted
  • Saturations < 92%
  • Unable to complete sentences in one breath
  • Signs of respiratory distress
  • RR:
    > 40 in 1-5 years
    > 30 in > 5 years
  • HR
    > 140 in 1-5 years
    > 125 in > 5 years
68
Q

Acute asthma

signs of life threatening severity

A
  • Peak flow < 33% predicted
  • Saturations < 92%
  • Exhaustion and poor respiratory effort
  • Hypotension
  • Silent chest
  • cyanosis
  • altered consciousness/confusion
69
Q

Acute asthma

stepwise approach in mnx

A
  1. salbutamol inhaler via spacer (10 puffs/2hr)
  2. nebulisers: salbutamol/ipratropium bromide
  3. PO prednisolone (1mg/kg OD for 3d)
  4. IV hydrocortisone
  5. IV magnesium sulphate
  6. IV salbutamol
  7. IV aminophylline
70
Q

Acute asthma

why Consider monitoring the serum potassium when on high doses of salbutamol

A

salbutamol causes potassium to be absorbed from the blood into the cells.

71
Q

Croup

what age does it affect

A

6m - 2y

72
Q

Croup

what is it

A

URTI causing oedema in the larynx

73
Q

Croup

classic cause

A

parainfluenza virus

also:
influenza
adenovirus

74
Q

Croup

croup caused by diphtheria leads to what

A

epiglottitis

75
Q

Croup

presentation

A
  • ‘barking’ cough occurring in clusters
  • low grade fever
  • increased work of breathing
  • hoarse voice
  • stridor
76
Q

Croup

conservative mnx

A

most cases can be managed at home:

  • fluids + rest
  • sit child up + comfort them
  • hand wash
  • stay off school
77
Q

Croup

medical mnx

A

DEXAMETHASONE
single dose 150mcg/kg
repeated after 12h if required

78
Q

Croup

medical mnx where dexamethasone trx is not available (in GP)

A

prednisolone

79
Q

Croup

Stepwise options in severe croup

A
  • PO dexamethasone
  • Oxygen
  • neb budesonide
  • neb adrenaline
  • intubation + ventilation
80
Q

Whooping Cough

cause

A

Bordetella pertussis (a gram negative bacteria)

81
Q

Whooping Cough

presentation

A
  • starts with mild coryzal sx, low grade fever, dry cough
  • 1w later, paroxysmal cough + coughing fits until out of breath
  • loud inspiratory whoop when the coughing ends
  • apnoea
82
Q

Whooping Cough

patients can cough so hard they ___

A

faint, vomit or even develop a pneumothorax

83
Q

Whooping Cough

inx to confirm dx

A

nasopharyngeal or nasal swab with PCR testing or bacterial culture

within 2-3w of onset of sx

84
Q

Whooping Cough

inx if cough has been present >2w and aged 5-16y

A

anti-pertussis toxin immunoglobulin G

in the oral fluid

85
Q

Whooping Cough

inx if cough has been present >2w and aged over 17y

A

anti-pertussis toxin immunoglobulin G

in the blood

86
Q

Whooping Cough

is it notifiable

A

yes

87
Q

Whooping Cough

mnx

A

supportive

88
Q

Whooping Cough

mnx for vulnerable or acutely unwell patients, those <6m and patients with apnoeas, cyanosis or patients with severe coughing fits

A
  • may need to admit
  • ## azithromycin /erythromycin/clarithromycin within first 21d
89
Q

Whooping Cough

which close contacts are given prophylactic abx

A

close contacts in a vulnerable group e.g. pregnant, unvaccinated infants, healthcare workers with close contact with children or pregnant women

90
Q

Whooping Cough

prognosis

A

‘100 day cough’

sx typically resolve within 8w

91
Q

Whooping Cough

key complication

A

bronchiectasis

92
Q

Ddx for stridor

A
  • croup
  • epiglottitis
  • foreign body
93
Q

Epiglottitis

cause

A

haemophilus influenza type B

94
Q

unvaccinated child presenting with a fever, sore throat, difficulty swallowing that is sitting forward and drooling

what is it

A

epiglottitis - a emergency

95
Q

Epiglottitis

presentation

A
  • sore throat
  • stridor
  • drooling
  • tripod position (sat forward with hand on each knee)
  • high fever
  • difficulty or painful swallowing
  • muffled voice
  • scared + quiet child
  • septic + unwell appearance
96
Q

Epiglottitis

should you examine the throat

A

no

97
Q

Epiglottitis

inx

A

lateral xray of the neck

98
Q

Epiglottitis

what will the lateral xray of the neck show

A

thumb sign” or “thumbprint sign”.

soft tissue shadow that looks like a thumb pressed into the trachea

caused by the oedematous + swollen epiglottis

99
Q

Epiglottitis

immediate mnx

A
  • do not distress child
  • alert most senior paediatrician + anaesthetist
  • ensure airway is secure:
    may need intubation or tracheostomy
100
Q

Epiglottitis

additional trx once airway is secure

A
  • IV ceftriaxone

- dexamethasone

101
Q

Epiglottitis

common complication

A

epiglottic abscess

collection of pus around the epiglottis.

102
Q

Epiglottitis

trx of an epiglottic abscess

A

similar to epiglottitis

103
Q

Chronic Asthma

Children are usually not diagnosed with asthma until they are at least ___

A

2-3y

104
Q

Chronic Asthma

inx where there is intermediate probability of asthma or diagnostic doubt

A
  • spirometry w/ reversibility testing (>5y)
  • direct bronchial challenge test w/ histamine or methacholine
  • fractional exhaled NO (FeNO)
  • peak flow diary
105
Q

Chronic Asthma

medical therapy <5y

A

1) SABA
2) + low does ICS or LTRA (montelukast)
3) + other option from step 2
4) refer

106
Q

Chronic Asthma

medical therapy aged 5-12y

A

1) SABA
2) + low does ICS
3) + LABA (salmeterol) only if good response
4) switch to medium dose ICS + consider adding PO LTRA, PO theophylline
5) switch to high dose ICS
6) refer. may need PO steroids

107
Q

Chronic Asthma

medical therapy aged >12y (same as adults)

A

1) SABA
2) + low dose ICS
3) + LABA (salmeterol)
4) switch to medium dose ICS + consider trial of PO LTRA, theophylline or inh LAMA (tiotropium)
5) switch to high dose ICS + combine trx from step 4 + PO SABA. Refer
6) + PO steroids

108
Q

Chronic Asthma

mum worried that ICS slows growth. What do you say?

A
  • can cause a small reduction in final adult height of up to 1cm when using long term (>12m)
  • ICS is used to prevent poorly controlled asthma and asthma attacks that could lead to higher doses of PO steroids
  • poorly controlled asthma can lead to more significant impact on growth + development
  • regular asthma reviews to ensure they are growing well and on the minimal dose required to effectively control symptoms.
109
Q

Pneumonia

characteristic chest signs of pneumonia

A
  • bronchial breath sounds
  • focal coarse crackles
  • dullness to percussion
110
Q

Laryngomalacia

what is it

A

the larynx above the vocal cords (the supraglottic larynx) is structured in a way that allows it to cause partial airway obstruction.

‘floppy airway’

111
Q

Laryngomalacia

what is stridor

A

a harsh whistling sound caused by air being forced through an obstruction of the upper airway

112
Q

Laryngomalacia

what is the structural change

A

the aryepiglottic folds are shortened, which pulls on the epiglottis and changes it shape to a characteristic “omega” shape.

The tissue surrounding the supraglottic larynx is softer and has less tone in laryngomalacia, meaning it can flop across the airway

113
Q

Laryngomalacia

at what age does it peak

A

6m

114
Q

Laryngomalacia

presentation

A
  • inspiratory stridor
  • more prominent when feeding, upset, lying on back or URTI
  • difficulties feeding
  • not usually associated with resp distress
115
Q

Laryngomalacia

mnx

A

no interventions are required and the child is left to grow out of the condition.

Rarely tracheostomy may be necessary

116
Q

Chronic Lung Disease of Prematurity

what is it aka

A

bronchopulmonary dysplasia

117
Q

Chronic Lung Disease of Prematurity

features

A
  • resp distress syndrome
  • low O2
  • increased work of breathin g
  • poor feeding + weight gain
  • crackles + wheezes on chest auscultation
  • increased susceptibility to infection
118
Q

Chronic Lung Disease of Prematurity

prevention before birth

A
  • corticosteroid to mother that show signs of premature labour at <36w gestation
119
Q

Chronic Lung Disease of Prematurity

once the neonate is born, the risk can be reduced by?

A
  • CPAP rather than intubation + ventilation when possible
  • caffeine to stimulate resp effort
  • not over- oxygenating
120
Q

Chronic Lung Disease of Prematurity

how is dx made

A
  • CXR changes
  • require O2 after 36w gestational age
  • sleep study to assess their oxygen saturations
121
Q

Chronic Lung Disease of Prematurity

mnx

A
  • may be discharged from the neonatal unit on a low dose of O2 to continue at home
  • followed up to wean the oxygen level over the first year of life.
122
Q

Chronic Lung Disease of Prematurity

what do babies with CLDP every month

A

palivizumab injections to protect against RSV to reduce risk of bronchiolitis

123
Q

Primary Ciliary Dyskinesia

aka

A

Kartagner’s syndrome

124
Q

Primary Ciliary Dyskinesia

what is it

A

an autosomal recessive condition affecting the cilia of various cells in the body

125
Q

Primary Ciliary Dyskinesia

whom is it more common in

A

consanguinity

126
Q

Primary Ciliary Dyskinesia

pathophysiology

A
  • dysfunction of motile cilia
  • build up mucus in lungs
  • good site for infection
  • similar to CF with frequent chest infections
127
Q

Primary Ciliary Dyskinesia

where is there reduced fertility

A

It also affects the cilia in the fallopian tubes of women and the tails (flagella) of the sperm in men

128
Q

Primary Ciliary Dyskinesia

what is Kartagner’s triad

A

3 key features of PCD:

  1. paranasal sinusitis
  2. bronchiectasis
  3. situs inversus
129
Q

Primary Ciliary Dyskinesia

what is situs inversus

A

where all the internal (visceral) organs are mirrored inside the body

130
Q

Primary Ciliary Dyskinesia

what is dextrocardia

A

when only the heart is reversed

131
Q

Primary Ciliary Dyskinesia

which condition is it strongly associated with

A

situs inversus

132
Q

Primary Ciliary Dyskinesia

diagnostic inx

A
  • nasal brushing or bronchoscopy to obtain sample of ciliated epithelium of the upper airway
133
Q

Primary Ciliary Dyskinesia

mnx

A

similar to CF + bronchiectasis:

  • daily physio
  • high calorie diet
  • abx