Resp Cram Flashcards
What age can spirometry be attempted?
> 6 years
2 factors in correct technique when performing spirometry?
Flow-volume loop must be replicated 3x within 5%
Duration of expiratory manoeuvre must be >1sec
Most useful parameter on lung function test to measure disease progression in obstruction airways disease?
FEV1
Most sensitive parameter on lung function test for small airways disease?
FEF25-75%
Most sensitive parameter on lung function test for restrictive lung disease?
FVC
Utility of DLCO?
Disease of pulmonary interstitium or vasculitis
Utility of exhaled nitric oxide?
Monitoring asthma control - positive test suggest eosinophilic inflammation
Utility of nasal nitric oxide?
Screening tool for Primary Ciliary Dyskinesia/can be used for CF
3 most common causes of bronchiectasis?
CF
Post-infectious
Primary immunodeficiency
Recurrent episodes of >4/52 productive cough
Digital clubbing
Hx CF/immunodef
Recurrent aspiration
Bronchiectasis
Gold standard imaging for bronchiectasis and findings?
HRCT
Broncho-arterial ratio
Lower lobes more commonly affected
Complications of bronchiectasis?
Pneumothorax Empyema Poor growth Osteoporosis Cor Pulmonale
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
Chronic sinopulmonary infections
Persistent middle ear effusions
Laterality defects
Infertility
Primary ciliary dyskinesia
Most common defect on PCD?
Absence of dyniein arms (structures necessary for generation of cilia/sperm tail movement)
Kartagener Triad?
Situs inversus totalis
Chronic sinusitis
Bronchiectasis
Screening test and diagnostic test for PCD?
Screening - inhaled NO
Diagnostic - electron microscopy (shortening/absence of dynein arms)
CF class I mutation
No CFTR protein produced
G542X mutation
Caused by base substitution/frame shift -> premature stop codon
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
CF class III mutation
Defect in CFTR regulation = non-functional CFTR
G551D
Ivacaftor used to treat
CF class IV mutation
CFTR present but reduced chloride transport
R117H mutation
CF class V mutation
CFTR present but reduced levels
Key features of CF?
Failure to clear mucus secretions
Paucity of water in secretions
Elevated salt in sweat
Chronic infections limited to respiratory tract
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
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
