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