Resp Cram Flashcards

1
Q

What age can spirometry be attempted?

A

> 6 years

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2
Q

2 factors in correct technique when performing spirometry?

A

Flow-volume loop must be replicated 3x within 5%

Duration of expiratory manoeuvre must be >1sec

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3
Q

Most useful parameter on lung function test to measure disease progression in obstruction airways disease?

A

FEV1

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4
Q

Most sensitive parameter on lung function test for small airways disease?

A

FEF25-75%

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5
Q

Most sensitive parameter on lung function test for restrictive lung disease?

A

FVC

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6
Q

Utility of DLCO?

A

Disease of pulmonary interstitium or vasculitis

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7
Q

Utility of exhaled nitric oxide?

A

Monitoring asthma control - positive test suggest eosinophilic inflammation

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8
Q

Utility of nasal nitric oxide?

A

Screening tool for Primary Ciliary Dyskinesia/can be used for CF

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9
Q

3 most common causes of bronchiectasis?

A

CF
Post-infectious
Primary immunodeficiency

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10
Q

Recurrent episodes of >4/52 productive cough
Digital clubbing
Hx CF/immunodef
Recurrent aspiration

A

Bronchiectasis

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11
Q

Gold standard imaging for bronchiectasis and findings?

A

HRCT
Broncho-arterial ratio
Lower lobes more commonly affected

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12
Q

Complications of bronchiectasis?

A
Pneumothorax
Empyema
Poor growth
Osteoporosis
Cor Pulmonale
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13
Q

Management of bronchiectasis?

A

Decrease airway obstruction (chest physio, postural drainage, bronchodilators, azithromycin)
Infection control (imms, antibiotics)
Reduce tobacco smoke exposure
May require lobectomy or lung transplant

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14
Q

Chronic sinopulmonary infections
Persistent middle ear effusions
Laterality defects
Infertility

A

Primary ciliary dyskinesia

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15
Q

Most common defect on PCD?

A

Absence of dyniein arms (structures necessary for generation of cilia/sperm tail movement)

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16
Q

Kartagener Triad?

A

Situs inversus totalis
Chronic sinusitis
Bronchiectasis

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17
Q

Screening test and diagnostic test for PCD?

A

Screening - inhaled NO

Diagnostic - electron microscopy (shortening/absence of dynein arms)

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18
Q

CF class I mutation

A

No CFTR protein produced
G542X mutation
Caused by base substitution/frame shift -> premature stop codon

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19
Q

CF class II mutation

A

Defect in CFTR protein processing = very low/no protein reaching apical membrane
Most common form
Caused by Delta F 508 deletion

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20
Q

CF class III mutation

A

Defect in CFTR regulation = non-functional CFTR
G551D
Ivacaftor used to treat

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21
Q

CF class IV mutation

A

CFTR present but reduced chloride transport

R117H mutation

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22
Q

CF class V mutation

A

CFTR present but reduced levels

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23
Q

Key features of CF?

A

Failure to clear mucus secretions
Paucity of water in secretions
Elevated salt in sweat
Chronic infections limited to respiratory tract

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24
Q

Newborn screening pathway for CF?

A

Serum trypsinogen on NST >99th centile -> gene testing
If 2 CF genes = CF clinic
If 1 CF gene = sweat test

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25
Q

Gold standard test for CF?

A

Sweat test

>60mEq/L chloride

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26
Q

Respiratory manifestation of CF?

A
Progressive lung disease
Nasal polyps
Allergic bronchopulmonary aspergillosis
Pneumothorax
Cor Pulmonale
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27
Q

GI/hepatic manifestations of CF?

A
Mec ileus/Mec plug
Distal intestinal obstruction
Rectal prolapse
Cirrhosis
Cholelithiasis
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28
Q

Pancreatic manifestations of CF?

A

Exocrine pancreatic def
Endocrine pancreatic def
Pancreatitis

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29
Q

Genitourinary manifestations of CF?

A

Delayed sexual development
Infertility
Inguinal hernia/hydrocele/undesc testes

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30
Q
CF pt with
Wheeze
Rust coloured sputum
High IgE/eosinophils
Hyperinflated XR
A

Allergic Bronchopulmonary Aspergillosis

31
Q

Factors associated with slower rate of respiratory decline in CF?

A

Male

Exocrine pancreatic deficiency

32
Q

Most common non-respiratory complications of CF?

A

CF Related Diabetes Mellitus

Impaired insulin secretion AND insulin resistance

33
Q

Mutation specific therapy for children w deltaF508 mutation?

A

Lumacaftor-ivacaftor if <11yrs

Tezacaftor-ivacaftor if >12yrs

34
Q

When single agent ivacaftor is used?

A

Gating/residual function mutations

35
Q

Most common bacterial infections in CF?

A

S Aureus - MOST COMMON
Haemophilus - 2nd most common
Pseudamonas - risk factor for pulmonary decline/reduced survival
B cenocapecia - risk factor for pulmonary decline/reduced survival and contra-indication to transplant

36
Q

Risk factors for severe disease in asthma?

A

Previous ICU admission
Poor compliance w therapy
Poorly controlled - significant interval symptoms

37
Q

Signs of salbutamol toxicity in asthma?

A

Tachycardia
Tachypnoea
Metabolic acidosis (high lactate)

38
Q

How many puffs in Ventolin canister?

A

200

39
Q

When is montelukast preferred to ICS?

A

Child unable to use inhaled therapy
Significant allergic rhinitis
Parental concern RE inhaled steroids

40
Q

Cause of increased mortality with use of LABA?

A

Phosphorylation and internalisation of Beta2 receptor

41
Q

Asthma treatment steps?

A
1 = SABA PRN
2 = low dose ICS / montelukast + SABA PRN
3 = high dose ICS / low dose ICS and montelukast / ICS w LABA + SABA PRN
42
Q

SEs of montelukast?

A

Neuropsychiatric - nightmares, hallucinations, mood/behaviour change

43
Q

SEs of ICS?

A

Dysphonia, oral candidiasis

Low bone density, skin thinning, bruising, poor growth

44
Q

Conditions assoc w choanal atresia?

A

CHARGE
Treacher-Collins
Kallman
VATER

45
Q

Stridor in laryngomalacia caused by?

A

Decreased laryngeal tone results in supraglottic collapse during inspiration

46
Q

Most common causes of stridor?

A

1 - laryngomalacia
2 - subglottic stenosis
3 - vocal cord palsy

47
Q

Difference in presentation between tracheomalacia and bronchomalacia?

A

Tracheomalacia - croupy/barking cough with resp distress, wheeze/stridor
Bronchomalacia - monophonic wheeze and persistent congestion

48
Q

Difference between intra-lobar and extra-lobar pulmonary sequestration?

A

Intra-lobar (80%) -blood drains to pulmonary veins, generally lower lobe, does not have its own pleura
Extra-lobar (20%) blood drains to IVC, has its own pleura, usually left lung, more common boys

49
Q

Most common location for bronchogenic cyst?

A

Right side, middle mediastinum

50
Q

Iron def anaemia
Haemoptysis
Alveolar infiltrate on CXR

A

Pulmonary haemosiderosis

51
Q

Genetic defect in congenital central hypoventilation?

A

PHOX2B (paired-like homeobox)

52
Q

Peak prevalence of OSA in children?

A

2-6 years due to adenotonsillar hypertrophy

Site of UA closure in children at levels of T&As

53
Q

Oximetry criteria for OSA?

A

> 3 clusters of desalts with at least 3 <90%

54
Q

Treatment of OSA?

A

Adenotonsillectomy
CPAP
Management of allergic rhinitis / obesity

55
Q

Complications of OSA?

A

Neurocognitive - behaviour, hyperactivity, impulsivity
FTT - increased BMR, insufficient GH release
Systemic HTN & PulmHTN
Eneuresis
Parasomnias

56
Q

Daytime sleepiness
Cataplexy
Hypnogogic hallucinations
Sleep paralysis

A

Narcolepsy

57
Q

Difference between restless legs and periodic limb movement disorder?

A

Restless legs - incontrollable urge to move legs and relieved by movement. Assoc w iron def
PLMD - stereotyped movements 20-40sec during sleep during which child is unaware of moving

58
Q

How long to cease salbutamol prior to lung function challenge?

A

8hrs pre

59
Q

Pathogen most associated with bronchiolitis obliteratans?

A

Adenovirus

60
Q

Recurrent croup like episodes

Subglottic narrowing

A

Spasmodic croup

61
Q

Cause of increased DLCO?

A

Pulmonary haemorrhage

62
Q

2 prognostic factors for CF deterioration?

A

FEV1

Rate of respiratory infections

63
Q

Treatment choice for pseudomonas in CF?

A

If well - PO cipro and inhaled tobramycin

If unwell - IV ceftaz/mero and tobramycin

64
Q

CF pt w Pseudamonas resistant to colistin?

A

Not pseudomonas - B Capecia instead

65
Q

Most common chronic infection in bronchiectasis?

A

Haemophilus

66
Q

RFs for bronchiectasis?

A
ATSI
Lowe SES
Low/not breastfed
Prematurity
Smoke exposure
Lower maternal education
67
Q

Glottic webs

A

22q11

68
Q

Severe subcostal/intercostal recessions
Dyspnoea
Expiratory stridor

A

Tracheal stenosis

69
Q

Scimitar Syndrome pathophysiology?

A

Pulm venous blood from R lung returns to the IVC instead of put arteries
Results in L to R shunt

70
Q

Pathogens causing epiglottitis?

A

HiB
Step pneumo
GAS
Staph Aureus

71
Q

Pathogen causing bacterial tracheitis?

A

Staph Aureus

72
Q

Secondary pneumonia after influenza cause?

A

Staph Aureus

73
Q

Allergic bronchipulmonavry aspergillosis treatment?

A

Oral steroids

-Azole