Paediatric Respiratory Flashcards

1
Q

What is acute epiglottitis?

A

rare

serious infection caused by haemophilus influenzae type B.

treat quick or airway obstruction.

used to be childhood in uk but now more common in adults because of immunisation programme.

decreased since HiB vaccine

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2
Q

features of acute epiglottitis?

A

rapid onset
high temp, generally unwell
stridor
drooling of saliva
“tripod” position : pt finds it easier breathing if learning forward and extending neck in seated position

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3
Q

how would you diagnose acute epiglottitis?

A

direct visualisation by senior airway staff.

x-ray poss? esp if concerned about foreign body:
- lateral vein in acute epiglottitis will show swelling of epiglottis - the thumb sign

a posterior-anterior view in croup will show subglottic narrowing - steeple sign

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4
Q

how would you manage acute epiglottitis?

A

oxygen
iv abx

senior involvement - emergency airway support - anesthetics, ent
ENDOTRACHEAL INTUBATION might needed to protect airway.

if suspected dont examine throat due to risk of acute airway obstruction.
only done by senior staff that can intubate if needed.

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5
Q

what is the epiglottis?

A

The epiglottis is a flap of cartilage located in the throat that prevents food and liquid from entering the lungs while swallowing

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6
Q

What is croup?

A

URTI in infants+toddlers.

characteristed by stridor caused by combo of laryngeal oedema and secretions.

PARAINFLUENZA VIRUSES account for most cases.

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7
Q

epidemiology of croup

A

peak incidence at 6mnths-3yrs

autumn more common

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8
Q

features of croup

A

cough - barking seal like. worse at night

stridor - dont examine throat due to risk of airway obstruction

fever
coryzal sx
increased work of breathing eg retraction

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9
Q

when would you consider admitting a child with croup?

A

moderate or severe croup
under 3 months
known upper airway abnormalities like downs or laryngomalacia

uncertainty of diagnosis - differentials are: acute epiglottitis, bacterial tracheitis, peritonsillar abscess and foreign body inhalation)

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10
Q

how would you investigate croup?

A

clinically mostly

if cxr:
posterior anterior view would show subglottic narrowing - steeple sign

in acute epiglottitis an anterior view would show the thumb sign - swelling of epiglottitis

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11
Q

how would you manage croup?

A

single dose of oral dexamethasone - 0.15mg/kg to all kids regardless of severity

prednisolone alternative if dex not available

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12
Q

grading the severity of croup

mild
moderate
severe

A

mild:
ocassional barking cough
no audible stridor at rest
no/mild suprasternal and/or intercostal recession
happy child - eat drink play

moderate:
frequent barking cough
easily audible stridor at rest (indication for admission)
-suprasternal and sternal wall retraction at rest
-no/little distress or agitation
- child can be placated and is interested in surroundings

severe:
frequent barking cough
prominent inspiratory/poss expiratory strioder @ rest

marked sternal wall retrctions
tachycardia with more seveere obstructive sx and hypoxeia

signficant distress and agitation, or lethargy or restlessness (sign of hypoxaemia)

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13
Q

how can wheezes be divided?

A

episodic viral: only when pt has URTI and is sx free inbetween episodes

multiple trigger wheeze: aswell as URTI other factors trigger it like exercise, allergens , cigarette smoke

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14
Q

is episodic viral wheeze associated with an increase risk of asthma in later life?

A

NO !!!

but a proportion that have multiple trigger wheeze will develop asthma

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15
Q

how would you managed episodic viral wheeze?

A

encourage parents to stop smoking

symptomatic tx
1st like: salbutamol - short acting beta 2 agonist . or anticholinergic via a spacer.

next step:
intermittent leukotreine receptor antagonist (montelukast) , intermittent inhaled corticosteroids (beclamethasone), or boht

little role for oral pred in kids that dont need hospitalising.

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16
Q

how would you treat multiple trigger wheeze?

A

tell parents stop smoking

trial either inhaled corticosteroids (beclamethasone) or leukotreine receptor antagonist - montelukast

for 4-8 weeks.

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17
Q

What is pneumonia?

A

any inflammatory condition affecting alveoli of the lungs, usually secondary to bacterial infection but can be viral/fungal too.

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18
Q

causes of pneumonia

A

bacterial - MC
viral
Fungal - eg pneumocystic jiroveci

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19
Q

what is the most common cause of pneumonia?

tell me a little about it

A

streptococcus pneumonia - pneumococcus - bacteria

80% pts

high fever
rapid onset
herpes labialis

vaccine for pneumococcus available

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20
Q

tell me all bacterial causes of pneumonia

A

streptococcus pneumoniae

haemophilus influenzae - copd pts

staphylococcus aureus - pt following influenze infection

mycoplasma pneumoniae - atypical. dry cough and atypical chest signs/xray : autoimmune haemolytic anemia, erythema multiforme could be seen

legionella pneumophilia - atypical pneumonia.
hyponatremia and lymphopenia common.
classic secondary to infected AC unit.

klebsiella pneumonia - alcholics

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21
Q

tell me the fungal cause of pneumonia

A

pneumocystis jiroveci -

hiv pts

dry cough
exercise-induced desaturations

absence of chest signs

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22
Q

what is idiopathic interstitial pneumonia

A

non-infective cause of pneumonia

eg’s:

cryptogenic organising pneumonia - form of bronchiolitis : comp of : RA or amiodarone therapy.

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23
Q

SYMPTOMS OF PNEUMONIA

A

COUGH
SPUTUM (purulent - rust coloured/bloodstained)
DYSPNOEA
CHEST PAIN : POSS PLEURITIC
FEVER
malaise

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24
Q

SIGNS OF PNEUMONIA

A

SIGNS OF SYSTEMIC INFLAMMATORY RESPONSE: fever tachycardia

reduced oxygen saturations - below 95% or below 88% in COPD pts
tachypnoea

auscultation:
reduced breath sounds
bronchial breathing
crepitations/crackles

dullness on percussion (fluid)

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25
Q

community vs hospital acquired pneumonia

difference

why is that even important?

A

most within community - CAP

within hospital 48hrs or more after admission: HAP.

causative organisms diff and therefore 1st line abx is different.

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26
Q

investigating pneumonia

A

cxr : consolidation (opacity on xray film in area of infection) - possibly effusion too

bloods:
fbc - neutrophilia in bacterial infection
u+e : check for dehydration - U for urea in curb 65
other changes in atypical pneumonia

CRP: raised in response to infection
blood cultures
sputum sample: causative organism after culture

ABG: if ox sats are low or if pt has pre-existing resp disease : COPD

check legionella antibodies - in intermediate/high risk pts

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27
Q

what is the scoring system to risk stratify pneumonia patients? (primary care)

A

CRB 65
IN PRIMARY CARE ITS CRB-65 because u cant get serum urea result

C - confusion - AMTS (abbreviated mental test score) - less than or equal to 8/10
U
R - RR 30 or more /min
B- bp - systolic less than or equal to 90 and/or diastolic less than or equal to 60 mmHg
65 - AGE EQUAL TO OR MORE THAN 65

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28
Q

interpreting crb 65 score (primary care)

A

home based care if crb65 is 0 - oral abx - amoxicillin 1st line
1-2: intermediate risk - 1-10% mortality
3/4 : high risk - 10% + mortaility

hospital assessment for all other pts - particularly if score 2 or more

if crp is less than 20 mg/L : no abx
if 20-100 - delayed abx prescription
if over 100 - abx

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29
Q

CURB 65 - hospital

A

c - confusion - amts - <=8/10
u - urea>7 mmol/L
r - rr >=30/min
b - bp <= 90/60
65 - age>=65

home based care if : 0/1 - low risk - <3% mortality
hospital-based care - 2+ - intermediate risk - 3-15% mortality
intensive care assessment if 3+ - high risk - 15%+ mortality risk

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30
Q

risk factors of pneumonia

A

under 5 or over 65
smoking
ivdu
recent viral RTI
chronic resp disease: CF, COPD
immunosuppression: cytotoxic drug therapy , HIV
pt risk of aspiration : parkinsons , oesophageal obstruction

non resp co-mordity: DM, CV disease

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31
Q

pathophysiology of pneumonia

A

pathogen enters lower resp tract.
starts inflammatory cascade.
neutrophils migrated to infected alveoli.
release cytokines - active immune response. induce fever.

accumulation of lfuid and pus in alveoli.
impairs gas exchange
= leads to hypoxic state = characteristic of pneuomonia.

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32
Q

management of pneumonia

A

low severity CAP:
- amoxicillin - 1st line
if allergy: macrolide (erythromcycin) /tetracycline
5 day course of abx.

moderate/high severity CAP:
- 7-10 day course
- beta-lactamase penicillin : coamoxiclav, ceftriaxone/piperacillin with tazobactam and a macrolide.

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33
Q

what is the discharge criteria for pneumonia pt ?

A

you cant if in the last 24 hrs they have 2 or more of:

  • temp higher than 37.5
  • rr 24 breaths per min or more
    HR over 100 bpm
    systolic bp: 90 or less
    ox sats 90 % O/A
    abnormal mental status
    inability to eat without assistane
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34
Q

what do doctors tell pts about symptoms clearing up?

A

1 wk - fever resolved
4wk - chest pain/sputum reduced
6wk - cough and breathless reduced
3mths - most sx resolves - fatigue poss
6mth- normal

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35
Q

when should you repeat CXR in pneumonia pts?

why?

A

cxr at 6 weeks after clinical resolution.

make sure consolidation resolved.
no underlying secondary abnormality - eg lung tumour.

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36
Q

what is the most likely causative agent in bacterial pneumonia in children?

A

s.pneumoniae

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37
Q

treatment of pneumonia in kids

A

amox - 1st line

macrolides (erythromcyin) added if no response to above.

macrolides : if mycoplasma or chlamydia suspected

in pneumonia with influenza: co-amoxiclav

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38
Q

What is Mycoplasma Pneumoniae?

A

cause of atypical pneumonia affecting younger pts.

epidemics of it usually occur every 4 yrs.

associated with erythema multiforme + cold autoimmune haemolytic anaemia.

may not respond to penicillin’s or cephalosporins because it lacks peptidoglycan cell wall.

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39
Q

features of mycoplasma pneumoniae

A

disease typically prolonged and gradual onset

flu-like sx classically precede dry cough

bilateral consolidation on x-ray

complications might occur as below

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40
Q

investigations of mycoplasma pneumoniae

A

diagnosis generally by mycoplasma serology

positive cold agglutination test = peripheral blood smear may show rbc agglutination

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41
Q

Management of mycoplasma pneumoniae

A

doxycycline
or

macrolide (erythromcyin/clarithromycin)

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42
Q

difference between legionella pneumonia and mycoplasma pneumonia

A

legionella : lymphopenia
mycoplasma: haemolytic anaemia/ITP

legionella: hyponatremia
mycoplasma: erythema multiforme. encephalitis/GBS. peri/myocarditis.

legionella: diagnosis - urinary antigen
mycoplasma pneumonia: diagnosis - serology

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43
Q

similarities of legionella and mycoplasma pneumoniae

A

atypical pneumonia
flu-like sx
dry cough
deranged lfts

treat with macrolide - erythromycin

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44
Q

what is aspiration pneumonia?

A

pneumonia caused by foreign materials gaining entry into bronchial tree - usually oral/gastric contents : food and saliva.

depending on acidity of aspirate, a chemical pneumonitis can happen - and bacterial pathogens adding to the inflammation.

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45
Q

how can aspiration pneumonia happen?

A

incompetent swallowing mechanism like in stroke,MS, intoxication.
or iatrogen causes: intubation.

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46
Q

risk factors of aspiration pneumonia

A

poor dental hygiene
swallowing difficulties
prolonged hospitalisation or surgical procedures
impaired consciousness
impaired mucociliary clearance

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47
Q

most common sites affected for aspiration pneumonia

and why?

A

right middle and lower lung lobes

due to larger calibre and more vertical orientation of the right main bronchus.

48
Q

bacteria implicated in aspiration pneumonia - aerobic

A

strept pneumonia
staph aureus
haem influenza
pseduomonas aeruginosa
klebsiella: seen in aspiration lobar pneumonia in alcholics

49
Q

bacteria implicated in aspiration pneumonia - anaerobic

A

bacteroides
prevotella
fusobacterium
peptostreptococcus

50
Q

what is pneumocystis jiroveci

A

also called carinnii pneumonia (PCP)

unicellular eukaryote - fungus

MC opportunitistic infection in AIDS

51
Q

features of pneumocystis jiroveci pneumonia : hiv

A

dyspnoea
dry cough
fever
very few chest signs

52
Q

name a common comp of pneumocystic jiroveci pneumonia

A

pneumothorax

53
Q

name 3 extrapulmonary manifestations of pneumocystis jiroveci pneumonia

A

RARE 1-2%

hepatosplenomegaly
lymphadenopathy
choroid lesions

54
Q

investigations of pneumocystis jiroveci pneumonia

A

CXR - bilateral interstitial pulmonary infiltrates. can present with lobar consolidation. could even be normal.

Exercise-induced desaturation : PCP causes this

sputum: fails to show PCP - so you do BAL - bronchoalveolar lavage - shows PCP - silver stain shows characteristic cysts!!

55
Q

management of pneumocystis jiroveci pneumonia

A

co-trimoxazole

IV pentamidine in severe case

aerosolized pentamidine alternative tx for PCP but less effective and risk of pneumothorax too.

steroids: if hypoxic (po2<9.3) then steroids reduce rep failure risk by 50% and death by 1/3

56
Q

at what cd4 count should patient recieve prophylaxis for pneumocystis jiroveci pneumonia

A

less than 200/mm3

57
Q

complications of mycoplasma pneumoniae

A

pericarditis/myocarditis

renal: acute glomerulonephritis

gi: hepatitis, pancreatitis

cold agglutins (IgM): poss haemolytic anaemia, thrombocytopenia

erythema multiforme: erythema nodosum

meningoencephalitis, GBS, immune mediated neuro diseases

bullous myringitis: painful vesicles on tympanic membrane.

58
Q

What is asthma?

A

chronic inflammatory disorder of airways secondary to t1 hypersensitivity.

variable sx
reversible bronchospasm - resulting in airway obstruction.

present at any age. - typical childhood.

59
Q

risk factors of asthma

A

personal/fhx of atopy

antenatal factors: maternal smoking, viral infection during pregnancy (RSV)

low birth weight
not being breastfed
maternal smoking around child
air pollution
exposure to high conc of allergens (house dust mite)

hygiene hypothesis: reduced exposure to infectious agents in kids prevents normal development of immune system = th2 predominant response

60
Q

patients with asthma suffer from which other IgE mediated atopic conditions

A

ATOPY

atopic dermatitis (eczema)

allergic rhinitis (hay fever)

61
Q

fun fact about nasal polyps and asthma patients

A

pt most sensitive to asthma also suffer from nasal polyps

62
Q

what drug are asthma pts sensitive to

A

aspirin

63
Q

How would you figure our occupational asthma?

common occupational allergens

A

may present with concern of chemicals at work. (worsening their asthma)

observe reduced peak flows during working week with normal readings when not at work.

isocyanates MC - spray paint, foam moulding using adhesives
platinum salts, epoxy resins, proteolytic enzymes, soldering flux resin
flour, glutaraldehyde

SERIAL MEASUREMENTS OF PEAK EXPIRATORY FLOW - AT WORK AND AWAY.

64
Q

symptoms of asthma

A

cough: often worse at night
dyspnoea
wheeze, chest tightness

65
Q

signs of asthma

A

expiratory wheeze on auscultation

reduced peak expiratory flow rate (PEFR)

66
Q

explain spirometry

tell me the key metrics

A

spirometry - measure amount (volume) and speed (flow) of air during exhalation and inhalation.

can help categorising resp disorder as obstructive (obstruction to airflow like bronchoconstriction in asthma) or restrictive where there is restriction to lungs like lung fibrosis)

fev1: forced expiratory volume - volume exhaled at the end of the 1st second of forced expiration

fvc: forced vital capacity - volume exhaled after maximal expiration following full inspiration

67
Q

what is the investigations in asthma?

A

spirometry :
fev1 - significantly reduced
fvc: normal
fev1% (fev1/fvc) <70%

cxr: particularly in older pts with smoking hx

Fractional exhaled nitric oxide (FeNO): 40 ppb in adults or 35ppb (upto 16) considered positive

68
Q

tell me a little bit about FeNO

A

fractional exhaled nitric oxide

nitric oxide produced by 3 types of nitric oxide synthases (NOS)

one of the types is iNOS - inducible - rises in inflammatory cells - particularly eosinophils

levels of NO therefore typically correlate with inflammation levels

69
Q

features of acute asthma

A

worsening dyspnoea , wheeze and cough - not responding to salbutamol

might be trigged by RTI

70
Q

how to split acute severe asthma into moderate, severe or life threatening

A

PEFR

moderate: PEFR 50-75% - BEST OR PREDICTED - speech normal - RR<25/MIN - Pulse <110 bpm

severe: PEFR : 33-50% or predicted. cant complete sentences. RR>25/min. Pulse >110 bpm

life- threatening : PEFR <33% AT BEST OR PREDICTED. ox sats <92%. - silent chest, cyanosis or feeble resp effort. bradycardia, dysrhythmia or hypotension. exhaustion, confusion or coma

71
Q

in an acute asthma attack what would indicate exhausation and therfore lifethreatning?

A

normal pCO2

72
Q

at what age should a patient with suspected asthma have objective tests?

A

5 or older.

73
Q

diagnostic testing for asthma in 5-16 yr olds

A

spirometry with a bronchodilator reversibility test (BDR)

FeNO test request if normal spirometry or obstructive spirometry with negative BDR

74
Q

how would you make a diagnosis for a pt under 5 for asthma?

A

clinical judgement

75
Q

patients 17 or over how would you diagnosis with asthma?

A

ask pt if sx better on days from work/.during holidays. - if so refer poss occupational asthma.

spirometry with BDR

FeNO test

76
Q

results quick summary for asthma

A

FENO : adults - 40ppb children 35ppb - that or more

Spirometry : fev1/fvc less than 70% - obstructive

BDR : adults : improvement in FEV1 of 12% or more and increase in volume of 200ml or more - positive
children : improvement in fev1 of 12% or more

77
Q

how would you identify “near fatal asthma”

A

raised pCO2 and/or requiring mechanical ventilation with raised inflation pressures.

78
Q

when would you recommend cxr in acute severe asthma?

A

if lifethreatening

suspected pneumothorax

failure to respond to tx.

79
Q

when would you do an ABG for acute severe asthma

A

in pts with ox sats <92%

80
Q

how would you manage acute severe asthma?

A

admission : lifethreatening, not responding to tx, pregnant,

ox: if hypoxaemic supplemental ox, if acutely unwell 15L of supplemental via non-rebreathe mask - then titre down to flow rate when maintaining 94-98% sats

bronchodilation with SABA : salbutamol, terbutaline. give by standard pressuring metered dose inhaler (pMDI) if not life threatening or near fatal. or oxygen driven nebuliser
if life threatening , NEVBULISED SAMA

corticosteroid: 40-50mg of pred orally daily, continue for 5 days until pt recovers from attack. - continue normal meds too. included inhaled corticosteroids.

ipratroopium bromide : if severe/life threatening asthma - or if pts havent responded to beta agonist/corticoteroid tx, nebulised ipratoprium bromide, a SAMA.

IV MAGNESIUM SULPHATE: can give for severe/life threatening asthma

consultation required to give iv aminophylline

81
Q

what happens if patient is admitted into hospital and fails to respond to tx and are becoming acidotic?

A

ITU/HDU

intubation and ventilation

extracorporeal membrane oxygenation (ECMO)

82
Q

what is the criteria for discharge for acute severe asthma attack?

A

stable on discharge meds - no nebulisers or oxygen for 12-24 hrs

inhaler technique checked and recordd

PEF >75% of best or predicted.

83
Q

general management of asthma - adults

A

stepwise

1- new asthma diagnosis - SABA
2- not controlled prior or new diagnosis with symptoms 3 or more/week or night-time waking : SABA + low dose ICS
3 - SABA + low dose ICS + LTRA
4- saba + low dose ics+ LABA (continue ltra is respond well)
5 - SABA +/- ltra - SWITCH ICS/LABA FOR A maintenance and reliver therapy(MART) that includes a low dose ICS
6- SABA +/- LTRA + medium dose ICS MART
or consider changing back to fixed dose of moderate dose ics and a seperate LABA
7 - SABA +/LTRA + ONE OF :

INCREASE ICS TO HIGH DOSE
TRIAL ADDITIONAL DRUG - LAMA OR THEOPHYLINE

84
Q

What is a mart?

A

mainentance and reliever therapy

ics+ laba in single inhaler - used for both daily maintenance and relief of sx as required.

only available for ics+ laba in which laba has a fast acting component eg formoterol

85
Q

dosing of inhaled corticosteroid doses

A

low dose: <=400 micrograms budesonide or equivalent = low dose

400 micrograms-800 budesonide or equivalent = moderate

over 800 - high dose

86
Q

assessing acute attack of asthma in children

moderate and severe

A

moderate: ox over 92 and no clinical features of asthma

spo2 under 92%

PEF 33-50% best or predicted

too breathless to talk or feed

hr : over 125 - if over 5. or over 140 if 1-5 yrs

rr :
over 30 - if over 5 or over 40 breaths per min if 1-5 yrs

use of accessory neck muscles

87
Q

assessing acute asthma attack in children
life threatening

A

spo2 less than 92%

PEF <33% best or predicted

silent chest

poor resp effort

agitation

altered conciousness

cyanosis

88
Q

asthma management for children 5-16

A

same stepwise as adults.
only difference is when you give ics : it is a paediatric low,moderate, high dose

also in step 4. stop ltra if it hasnt helped.

89
Q

management of asthma in children under 5

A

stepwise

1 - SABA - newly diagnosed
2- SABA + 8wk trial of paediatric moderate dose of ICS
3. SABA + Paeds low ics + ltra
4 - stop ltra and refer to paeds asthma specialist.

90
Q

tell me a little bit about stepping down tx in asthma pts

A

every 3 months.

not a strict move from step 3 to 2 - take into account duration of tx, side effects and pt preference.

when reducing dose of ics - 25-50% at a time.

91
Q

in step 2 of stepwise management of asthma in children under 5, what to do if sx didnt resolve with the trial of ics

A

alternative diagnoses is likely

92
Q

what would you do if in step 2 of stepwise management of asthma in children under 5, the sx resolved then reoccured within 4 weeks of stopping it?

longer than 4 weeks after stopping?

A

restart ics at paeds low dose as 1st line maintenance therapy

if they start after 4 weeks then repeat 8 wk trial of ics

93
Q

paediatric ics dosing - asthma

A

<=200 micrograms budesonide or equivalent = paeds low dose

200-400 - moderate
over 400 - high dose

94
Q

when stratifying a child for moderate,severe, and life threatening attack what is the diff?

A

severe for under 5 is hr over 140. for over 5 its over 125.
for under 5 is over 40/min for over 5 its over 30/min

and ofcourse you cant do PEF in under 5
so
over 5
pef>50% - mod
pef 33-50 - severe
pef <33% - lie threatening

95
Q

for a kid with mild-moderate acute asthma what to do you do?

A

bronchodilator therapy :
give beta 2 agonist via a spacer : - for kid under 3 use close-fitting mask

1 puff every 3-60 seconds upto max of 10 puffs
if sx not controlled repeat beta 2 agonist and refer to hosp

steroids:
give to all kids with exacerbation
tx for 3-5 days

dosing of pred:
2-5 : 20mg OD / 1-2mg/kg OD - max 40mg
over 5 : 30-40 mg od / 1-2mg/kg OD - max 40mg

96
Q

what is bronchiolitis?

A

acute bronchiolar inflammatory.

respiratory syncytial virus - RSV - pathogen in 75-80% cases.

other cases: mycoplasma, adenoviruses

could be secondary to bacterial infection.

more serious if bronchopulmonary dysplasia (eg premature), congenital heart disease or CF

97
Q

epidemiology of bronchiolitis

A

MC of serious lrti in under 1 - 90% are 1-9 mths - peak incidence 3-6 mths.

material IgG gives protection to newborns against RSV.

higher incidence in winter

98
Q

features of bronchiolitis

A

coryzal sx (including mild fever) precede:

  • dry cough
  • increasing breathlessness
  • wheezing, fine inspiratory crackles (not always)
  • feeding difficulties with increasing dyspnoea = often reasons for hosp admission
99
Q

when should you do immediate referral by ambulance for bronchiolitis pt?

A

if they have any of :

apnoea - observed/reported
child looks unwell to professional
central cyanosis
persistent ox sats less tha 92 on air
severe resp distress: grunting, marked chest recession, RR over 70/min

100
Q

when should you consider referral to hospital for bronchiolitis pt?

A

RR over 60/min
clinical dehydration
difficulty breastfeeding/inadequate oral fluid intake (50-75% of usual volume)

101
Q

what investiagation would you do for bronchiolitis?

A

immunofluorescence of nasopharyngeal secretions might show RSV

102
Q

how would you manage bronchiolitis?

A

mostly supportive - no abx

humidified ox via head box - if ox sats persistently under 92

nasogastric feeding - if kid cant take enough fluid/feed by mouth

suction - if excessive upper airway secretions

103
Q

what is cystic fibrosis?

A

autosomal recessive.

104
Q

organisms which may colonise CF patients

A

-Staphylococcus aureus
-pseudomonas aeruginosa
- burkholderia cepacia - previously known as - pseudomonas cepacia

  • aspergillus
105
Q

80% of CF cases are due to what genetics?

A

delta F508 on long arm of chromosome 7.

106
Q

CF affects how many births?

carrier rate?

A

1 per 2500 births

carrier rate : 1 in 25

107
Q

Presenting features of cystic fibrosis

A

neonatal period (20% approx.) - meconium ileus, less commonly prolonged jaundice

recurrent chest infections (40%)

malabsorption (30%) : steatorrhea (due to fat malabsorption) , failure to thrive

other features (10%) - liver disease

108
Q

other non presenting features of cystic fibrosis

A

short stature
DM
delayed puberty
rectal prolapse (due to bulky stools)
nasal polyps
male infertility (absent vas deferens leading to infertility) : female subfertility (delay in conceiving naturally)

109
Q

when is cf picked up?

A

newborn screening programmes or early childhood.

5% pts - after 18

110
Q

cystic fibrosis : how to diagnose?

A

sweat test:
- patient with CF have abnormally high sweat chloride
- normal value <40 mEq/l, CF : >60 mEq/l

111
Q

causes of false positive sweat test - cystic fibrosis

A

malnutrition
adrenal insufficiency
G6PD
ectodermal dysplasia
hypothyroidism, hypoparathyroidism
nephrogenic diabetes insipidus
glycogen storage diseases

112
Q

causes of false negative sweat test - cystic fibrosis

A

skin oedema due to :
hypalbuminaemia/hypoproteinaemia secondary to pancreatic exocrine insufficiency

113
Q

chronic management of cystic fibrosis

A

twice daily chest physio and postural drainage - parents taught how to do. - deep breathing poss?

high calorie high fat intake.

minimise contact with each other prevent cross infection with BURKHOLDERIA CEPACIA complex and PSEDUOMONAS AERUGINOSA.

Vit supplementation
pancreatic enzyme supplements - take with meals

lung transplantation - but if you have chronic infection with burkholderia cepacia it is a cf-specific CI to lung transplant.

114
Q

what drug is used to treat cf patients homozygous for delta F508 mutation ?

A

lumacaftor/ ivacaftor (orkambi)

treats CF patients homozygous for delta F508 mutation

115
Q

how does lumacaftor work?

A

increases number of CFTR proteins that are transported to the cell surface.

116
Q

how does ivacaftor work?

A

potentiator of CFTR that is already at cell surface.

increases probability that defective channel will be open and allow chloride ions to pass through channel pore.