Resp Cram Flashcards by Olivia Wells

What age can spirometry be attempted?

> 6 years

How well did you know this?

Not at all

Perfectly

2 factors in correct technique when performing spirometry?

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

Duration of expiratory manoeuvre must be >1sec

How well did you know this?

Not at all

Perfectly

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

FEV1

How well did you know this?

Not at all

Perfectly

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

FEF25-75%

How well did you know this?

Not at all

Perfectly

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

FVC

How well did you know this?

Not at all

Perfectly

Utility of DLCO?

Disease of pulmonary interstitium or vasculitis

How well did you know this?

Not at all

Perfectly

Utility of exhaled nitric oxide?

Monitoring asthma control - positive test suggest eosinophilic inflammation

How well did you know this?

Not at all

Perfectly

Utility of nasal nitric oxide?

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

How well did you know this?

Not at all

Perfectly

3 most common causes of bronchiectasis?

CF
Post-infectious
Primary immunodeficiency

How well did you know this?

Not at all

Perfectly

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

Bronchiectasis

How well did you know this?

Not at all

Perfectly

Gold standard imaging for bronchiectasis and findings?

HRCT
Broncho-arterial ratio
Lower lobes more commonly affected

How well did you know this?

Not at all

Perfectly

Complications of bronchiectasis?

Pneumothorax
Empyema
Poor growth
Osteoporosis
Cor Pulmonale

How well did you know this?

Not at all

Perfectly

Management of bronchiectasis?

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

How well did you know this?

Not at all

Perfectly

Chronic sinopulmonary infections
Persistent middle ear effusions
Laterality defects
Infertility

Primary ciliary dyskinesia

How well did you know this?

Not at all

Perfectly

Most common defect on PCD?

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

How well did you know this?

Not at all

Perfectly

Kartagener Triad?

Situs inversus totalis
Chronic sinusitis
Bronchiectasis

How well did you know this?

Not at all

Perfectly

Screening test and diagnostic test for PCD?

Screening - inhaled NO

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

How well did you know this?

Not at all

Perfectly

CF class I mutation

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

How well did you know this?

Not at all

Perfectly

CF class II mutation

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

How well did you know this?

Not at all

Perfectly

CF class III mutation

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

How well did you know this?

Not at all

Perfectly

CF class IV mutation

CFTR present but reduced chloride transport

R117H mutation

How well did you know this?

Not at all

Perfectly

CF class V mutation

CFTR present but reduced levels

How well did you know this?

Not at all

Perfectly

Key features of CF?

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

How well did you know this?

Not at all

Perfectly

Newborn screening pathway for CF?

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

How well did you know this?

Not at all

Perfectly

Gold standard test for CF?

Sweat test | >60mEq/L chloride

Respiratory manifestation of CF?

``` Progressive lung disease Nasal polyps Allergic bronchopulmonary aspergillosis Pneumothorax Cor Pulmonale ```

GI/hepatic manifestations of CF?

``` Mec ileus/Mec plug Distal intestinal obstruction Rectal prolapse Cirrhosis Cholelithiasis ```

Pancreatic manifestations of CF?

Exocrine pancreatic def Endocrine pancreatic def Pancreatitis

Genitourinary manifestations of CF?

Delayed sexual development Infertility Inguinal hernia/hydrocele/undesc testes

``` CF pt with Wheeze Rust coloured sputum High IgE/eosinophils Hyperinflated XR ```

Allergic Bronchopulmonary Aspergillosis

Factors associated with slower rate of respiratory decline in CF?

Male | Exocrine pancreatic deficiency

Most common non-respiratory complications of CF?

CF Related Diabetes Mellitus | Impaired insulin secretion AND insulin resistance

Mutation specific therapy for children w deltaF508 mutation?

Lumacaftor-ivacaftor if <11yrs | Tezacaftor-ivacaftor if >12yrs

When single agent ivacaftor is used?

Gating/residual function mutations

Most common bacterial infections in CF?

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

Risk factors for severe disease in asthma?

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

Signs of salbutamol toxicity in asthma?

Tachycardia Tachypnoea Metabolic acidosis (high lactate)

How many puffs in Ventolin canister?

200

When is montelukast preferred to ICS?

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

Cause of increased mortality with use of LABA?

Phosphorylation and internalisation of Beta2 receptor

Asthma treatment steps?

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

SEs of montelukast?

Neuropsychiatric - nightmares, hallucinations, mood/behaviour change

SEs of ICS?

Dysphonia, oral candidiasis | Low bone density, skin thinning, bruising, poor growth

Conditions assoc w choanal atresia?

CHARGE Treacher-Collins Kallman VATER

Stridor in laryngomalacia caused by?

Decreased laryngeal tone results in supraglottic collapse during inspiration

Most common causes of stridor?

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

Difference in presentation between tracheomalacia and bronchomalacia?

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

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

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

Most common location for bronchogenic cyst?

Right side, middle mediastinum

Iron def anaemia Haemoptysis Alveolar infiltrate on CXR

Pulmonary haemosiderosis

Genetic defect in congenital central hypoventilation?

PHOX2B (paired-like homeobox)

Peak prevalence of OSA in children?

2-6 years due to adenotonsillar hypertrophy | Site of UA closure in children at levels of T&As

Oximetry criteria for OSA?

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

Treatment of OSA?

Adenotonsillectomy CPAP Management of allergic rhinitis / obesity

Complications of OSA?

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

Daytime sleepiness Cataplexy Hypnogogic hallucinations Sleep paralysis

Narcolepsy

Difference between restless legs and periodic limb movement disorder?

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

How long to cease salbutamol prior to lung function challenge?

8hrs pre

Pathogen most associated with bronchiolitis obliteratans?

Adenovirus

Recurrent croup like episodes | Subglottic narrowing

Spasmodic croup

Cause of increased DLCO?

Pulmonary haemorrhage

2 prognostic factors for CF deterioration?

FEV1 | Rate of respiratory infections

Treatment choice for pseudomonas in CF?

If well - PO cipro and inhaled tobramycin | If unwell - IV ceftaz/mero and tobramycin

CF pt w Pseudamonas resistant to colistin?

Not pseudomonas - B Capecia instead

Most common chronic infection in bronchiectasis?

Haemophilus

RFs for bronchiectasis?

``` ATSI Lowe SES Low/not breastfed Prematurity Smoke exposure Lower maternal education ```

Glottic webs

22q11

Severe subcostal/intercostal recessions Dyspnoea Expiratory stridor

Tracheal stenosis

Scimitar Syndrome pathophysiology?

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

Pathogens causing epiglottitis?

HiB Step pneumo GAS Staph Aureus

Pathogen causing bacterial tracheitis?

Staph Aureus

Secondary pneumonia after influenza cause?

Staph Aureus

Allergic bronchipulmonavry aspergillosis treatment?

Oral steroids | -Azole