Respiratory Flashcards
Types of Pneumonia (+organisms)
Community-Acquired (CAP)
Hospital-Acquired (Nosocomial)
Aspiration Pneumonia
Immunocompromised
1.) Community-Acquired Pneumonia
- S.pneumoniae (90%), H. influenzae (most common in COPD), Moraxella catarrhalis, Klebsiella pneumoniae, S.aureus (most common after the flu)
- rapid onset w/ fatal outcome in a short period of time
- atypical (can last several weeks): Mycoplasma pneumoniae, Chlamydia pneumoniae, Legionella pneumophilia
2.) Nosocomial Pneumonia - LRTI in hospitalised patients >48hrs after admission w/o incubated before
- organisms: g-ves (E. coli, Klebsiella), S. aureus
- Klebsiella is a most common cause of nosocomial pneumonia, often affecting diabetics and alcoholics
- pseudomonas is common especially in patients with bronchiectasis or cystic fibrosis, or patients with in ITU with a ventilator
3.) Aspiration Pneumonia - aspiration of food, drink, saliva or vomit leading to an infection. Occurs due to:
- altered consciousness: anaesthesia, alcohol excess, drug OD
- swallowing problems: neuromuscular problems or oesophageal disease
- causative organisms: Klebsiella, oral flora and anaerobes
4.) Pneumonia in the Immunocompromised
- pneumocystis jiroveci, aspergillus, cytomegalovirus
Diagnosis of Pneumonia
Clinical Features
CURB-65
Investigations
1.) Clinical Features
- fever, SOB, productive cough (green sputum)
- pleuritic chest pain
- dull on percussion, ↑vocal resonance
- atypical: flu like sx, dry cough, deranged LFTs
- Legionella: hyponatraemia +/- lymphopenia or deranged LFTs
- Mycoplasma: erythema multiforme, haemolytic anaemia/ITP, encephalitis/GBS, myo/pericarditis
2.) CURB-65 - assessment to aid management
- Confusion (new), Urea (>7 mmol/L), RR (>30), BP (systolic <90 or diastolic <60), Age >65
- 0 or 1 managed as outpatient, 2 inpatient, 3+ consider ICU admission
3.) Investigations
- bloods: ↑WCC, ↑CRP, U+Es, sputum culture, ABG,
- blood cultures
- chest X-ray: consolidation, cavitating opacities in upper lobe (Klebsiella)
- atypical pneumonia screen: serology for mycoplasma, urinary antigen test for Legionella
Management of Pneumonia
General Measures
Antibiotics for Community-Acquired Pneumonia
Antibiotics for Atypical Organisms
Antibiotics for Noscocmial Pneumonia
Follow Up
1.) General Management
- A-E assessment, analgesia, stay hydrated
- IV fluids and oxygen in more severe illness
2.) Antibiotics for Community-Acquired Pneumonia
- mild (0/1): PO amoxicillin 500mg TDS for 5 days, OR PO doxycycline 200mg TDS for 5 days (pen allergy)
- moderate (2): PO amoxicillin 1g TDS + PO doxycycline 200mg TDS for 5 days, doxycycline only if pen allergic
- severe (3+): IV co-amoxiclav 1.2g TDS + PO doxycycline 200mg TDS for 5 days OR IV meropenem 1g TDS + PO doxycycline 200mg TDS for 5 days
3.) Antibiotics for Atypical Organisms - don’t have cell wall so you target protein synthesis
- macrolides (erythromycin or clarithromycin) or tetracycline (doxycycline)
4.) Antibiotics for Noscocmial Pneumonia - g-ve organisms are most common so IV Co-Amoxiclav given
5.) Follow Up
- HIV test, flu and pneumococcal vaccine
- immunoglobulins: pneumococcal, H influenzae IgG
- follow up in 6 weeks with a repeat CXR
NICE guidelines for Asthma Management
Assessment and Signs of Uncontrolled Asthma
Newly Diagnosed Asthma
Regular Preventer
Initial Add-On
MART Therapy
Additional Control
1.) Assessment - symptoms, lung function, optimise inhaler technique and adherence, eliminate triggers
2.) Scaling the Algorithm - uncontrolled asthma:
- using SABA or experiencing symptoms > 2x a week
- waking up at night due to asthma at least once a week
- others signs of poorly controlled asthma: asthma interfering with usual activities, reducing PEFR (home monitoring)
1.) Newly Diagnosed Asthma - SABA PRN
- INH salbutamol PRN
2.) Regular Preventer - low dose ICS BD + SABA prn
- if SABA alone is ineffective or new asthma with symptoms >3x a week or waking at night w/ asthma
- ICS is usually beclomethasone
3.) Initial Add-On - low dose ICS + SABA + LTRA/LABA
- LTRA instead of a LABA simply because its cheaper
- if LTRA is ineffective, switch to more expensive LABA
4.) MART Therapy - combination inhaler containing an ICS + LABA (formetrol) e.g. fostair inhaler
- SABA + MART +/- LTRA
5.) Additional Control - increasing dose of ICS
- increasing ICS to a medium dose
Severities of Asthma Exacerbation
Mild
Moderate
Severe
Life-Threatening (+ Near-Fatal)
1.) Mild - peak expiratory flow rate (PEFR) >75%
2.) Moderate - PEFR 50-75%
3.) Severe - any one of the following:
- PEFR 33-50% of best or predicted
- unable to speak in full sentences
- RR >25, HR >110
4.) Life-Threatening - any one of the following:
- PEFR < 33% of best or predicted
- sats <92% or ABG pO2 < 8kPa
- pCO2 normal
- cyanosis, hypoventilation (↓RR), silent chest
- hypotension or arrhythmias, exhaustion, confusion
- Near-Fatal: raised pCO2 (CO2 retention)
Management of Asthma Exacerbation
Initial Steps
Severe
Life Threatening/Near Fatal
Criteria for Safe Discharge
1.) Initial Steps - also for mild or moderate
- NEB salbutamol 5mg (can repeat after 15 mins)
- PO prednisolone 40mg or IV hydrocortisone
2.) Severe
- add NEB ipratropium bromide 500mcg
- consider back to back salbutamol (6 min intervals, up to 5 times)
3.) Life Threatening/Near Fatal
- IV aminophylline, consider IV salbutamol
- urgent ITU or anaesthetic referral
- urgent portable CXR
4.) Criteria for Safe Discharge
- >5 days of PO prednisolone
- PEFR >75%, provide PEFR meter and action plan
- stop regular nebulisers for 24hrs before discharge
- inpatient asthma nurse review to reassess inhaler technique and adherence
- GP follow up within 2 days, respiratory clinic follow up within 4 week
COPD
Aetiology
Risk Factors/Causes
Clinical Features
Investigations
1.) Aetiology - airflow limitation
- chronic bronchitis: inflammation/fibrosis of small airways –> ↑airway resistance
- emphysema: alveolar breakdown (parenchymal destruction) –> ↓elastic recoil, can cause lung collapse
2.) Risk Factors/Causes
- smoking (90% in the UK)
- inherited alpha-1 anti-trypsin deficiency
- air pollution, illicit drug use, biomass exposure
3.) Clinical Features
- worsening SOB, recurrent cough and wheeze
- chronic sputum production, recurrent LRTIs
- purse lip breathing, accessory muscles, cyanosis
- barrel-shaped chest, expiratory wheeze (auscultation)
- heart failure signs (due to pulmonary hypertension)
4.) Investigations
- spirometry: irreversible obstructive patterns, FEV1:FVC < 70%
- severity: mild (FEV1 >80% + sx), mod (FEV1 50-80%), severe (30-50%). v severe (<30%)
- CXR: hyperinflation, exclude differentials (e.g. cancer)
- HR-CT: assess emphysema, potential bronchiectasis
- alpha-1 antitrypsin blood test (younger patients)
Management of COPD
Non-Pharmacological
Bronchodilators for Asthmatic Features
Bronchodilators for Non-Asthmatic Features
Others
COPD Exacerbation
Long Term Oxygen Therapy
1.) Non-Pharmacological
- smoking cessation (improves mortality)
- pulmonary rehab: 6-12wk exercise program
2.) Bronchodilators for Asthmatic Features - eosinophilia, atopy
- 1° salbutamol (SABA) or ipratropium (SAMA) first line
- 2° ICS + LABA (salmeterol/formeterol) + [SABA/SAMA]
- 3° LAMA (tiotropium) + [ICS + LABA + SABA/SAMA]
3.) Bronchodilators for Non-Asthmatic Features
- 1° salbutamol (SABA) or ipratropium (SAMA) first line
- 2° combined LABA + LAMA + [SABA/SAMA]
4.) Others
- mucolytics, steroids, non-invasive ventilation
- lung volume reduction surgery (improves mortality)
4.) COPD Exacerbation - infective (H.influenzae most common) or non-infective
- non-infective: environmental, allergies, HF, anaemia, PE medications
- sputum sample, FBC, CRP, CXR, Abx if fever
- oxygen: 28% venturi on 4L, aim for 88-92% via ABG
- NEB salbutamol and ipratropium
- PO prednisolone 30mg OD for 7 days
- BiPAP (NIV) if T2RF (pCO2 >6) AND acidotic (pH <7.35)
- consider IV aminophylline, ITU referral if pH <7.25
5.) Long Term Oxygen Therapy
- indications: any one of cyanosis, polycythaemia, raised JVP, FEV1 < 30%, oxygen saturations <92% or peripheral oedema
- continuous oxygen therapy for >16hrs a day
- pO2 consistently below 7.3kPa or 8kPa w/ pulmonary hypertension, secondary polycythaemia or peripheral oedema
- must be no longer smoking due to risk of fire and explosions
- must NOT be hypercapnic (pCO2 <6)
Pleural Effusion
Clinical Features
Transudative
Exudative
Investigations
Management
1.) Clinical Features - gradual onset
- SOB, pleuritic chest pain
- ↓chest expansion, ↓breath sounds, ↓vocal resonance
- stony dull percussion
2.) Transudative - ↑formation of pleural fluid
- often bilateral due to more systemic causes
- HF (↑capillary HP)
- hypoalbuminemia : nephrotic syndrome, liver failure, peritoneal dialysis
- liver cirrhosis (pulmonary hypertension)
3.) Exudative - inflammation –> ↑capillary permeability
–> protein leakage
- causes: infection (pneumonia, TB), cancer, PE
4.) Investigations
- CXR: blunting of costo/cardiophrenic angles, meniscus sign, mediastinal shift (if large)
- US-guided pleural aspiration: fluid sent for testing of
pH, protein (>30g/dL = exudative), LDH, cytology, MC+S
- staging CT w/contrast: underlying pathology
- bloods: routine inc clotting, bone profile, LDH
- ECG, ECHO (?HF)
5.) Light’s Criteria for Exudative Effusion - used for borderline cases where protein is in between 25-35
- protein pleural:serum ratio >0.5
- LDH pleural:serum ratio >0.6
- pleural LDH > 2/3 of serum LDH (upper normal limit)
5.) Management - treat underlying cause
- urgent chest drain only if suspected pleural infection: pleural fluid is turbid/cloudy or pleural fluid pH <7.2
- indwelling pleural catheter for recurrent effusions
- chest drain relative contraindications: INR > 1.3, platelet count < 75, pulmonary bullae, pleural adhesions
- pleurodesis to remove pleural space
Tension Pneumothorax
Definition
Aetiology
Clinical Features
Chest X-Ray
1.) Definition - any pneumothorax causing mediastinal shift and cardiovascular collapse (mechanical shock)
2.) Aetiology
- primary spontaneous pneumothorax
- underlying lung disease: asthma, COPD, pneumonia, TB, lung cancer, bronchiectasis, CF
- trauma: fractured rib or chest trauma
- iatrogenic: e.g. ventilation, insertion of central lines, pacemakers
3.) Clinical Features
- pleuritic chest pain, SOB, fatigue, severe distress
- ↑RR, ↑HR, displaced apex beat (mediastinal shift)
- tracheal deviation, hyper-resonance on percussion
- reduced/absent breath sounds, ↓chest movement, ↓vocal resonance
- haemodynamic (obstructive) shock: ↑pressure in the chest wall due to ↑air causes reduced venous return (compresses great veins)
4.) Chest X-Ray
- mediastinal shift and displaced apex
- hyperlucent (↑air) and absent lung markings
- visible edge of collapsed lung
Management of Pneumothorax
Definitions
Management of Primary Pneumothorax
Management of Secondary Pneumothorax
Management of Tension Pneumothorax
Discharge Advice
1.) Definitions
- primary spontaneous pneumothorax: often due to a small sub-pleural bleb or bulla that bursts allowing air into the air cavity, common in young males with tall/thin frames that also smoke
- secondary pneumothorax: age >50 w/ significant smoking history OR evidence of underlying lung disease on examination or CXR
- tension pneumothorax: any pneumothorax causing mediastinal shift and cardiovascular collapse (mechanical shock)
2.) Management of Primary Pneumothorax
- needle aspiration (w/ 16-18G cannula): if rim of air >2cm OR SOB
- chest drain and admission: failed needle aspiration
- discharge + OP review in 2-4 weeks: rim of air <2cm w/o SOB, also after successful needle aspiration (<2cm and improved breathing)
3.) Management of Secondary Pneumothorax - all require admission or 24hr observation regardless of treatment
- needle aspiration: rim of air 1-2cm w/o SOB
- chest drain and admission: rim of air >2cm OR SOB, failed aspiration
- 24hr observation (w/ high flow O2) for all patients: asymptomatic + rim of air < 1cm and also after successful needle aspiration
4.) Management of Tension Pneumothorax
- emergency needle decompression of the chest: insert cannula into the 2nd ICS in mid-clavicular line
- chest drain (5th ICS, mid-axillary line): replaces cannula when patient is stable. drain is removed once lungs are fully expanded
5.) Discharge Advice
- smoking: advised to avoid smoking to reduce risk of further episodes
- fitness to fly: contraindicated until 1 week post OP CXR in review
- scuba diving: permanently avoided unless the patient has undergone bilateral surgical pleurectomy and has normal lung function and chest CT scan post-op
Pulmonary Embolism
Risk Factors
Clinical Features
Investigations
Management
Thrombolysis
1.) Risk Factors
- surgery: abdo/pelvic, hip/knee replacement, post op in ITU
- obstetric: late pregnancy, C-section
- malignancy, varicose veins, ↓mobility, previous DVT/PE
2.) Clinical Features - sudden onset
- pleuritic chest pain, SOB, haemoptysis
- low cardiac output –> shock (massive PE)
- suspect PE in patients with low O2 sats refractory to high flow oxygen
3.) Investigations
- CTPA (gold-standard) or echo (RV enlargement/strain)
- raised D-dimers
- ECG: sinus tachycardia, right axis deviation, T wave inversion in leads V1-V4 +/- II/III/aVF, RV strain (S1Q3T3, deep S in I, Q in III, T-inversion in III)
- VQ scan in renal impairment (can’t do CTPA)
4.) Management - A-E assessment inc:
- O2 and IV fluids if hypoxic or hypotensive
- thrombolysis if massive PE (haemodynamic instability)
- anticoagulation e.g. apixaban
5.) Thrombolysis - IV alteplase
- contraindications: haemorrhagic stroke, ischaemic stroke (< 6mths), CNS neoplasia, recent trauma/surgery, GI bleed < 1 month, bleeding disorder, aortic dissection
- complications: bleeding, hypotension, stroke, intracranial haemorrhage, reperfusion arrhythmias, allergic reaction, thrombus embolisation
Massive Haemoptysis
Clinical Features
Management
1.) Clinical Features
- >240ml in 24hrs OR >100ml/day over consecutive days
2.) Management
- A-E assessment, lie patient on side of suspected lesion (if known)
- PO/IV Tranexamic Acid for 5 days
- stop any NSAIDs, aspirin, or anticoagulants (consider vitK)
- abx if suspected respiratory tract infection
- CT aortogram: IR may undertake bronchial artery embolization
Anaphylaxis
Pathophysiology
Clinical Features
Emergency Management
Management After Stabilisation
1.) Pathophysiology - type 1 (IgE mediated/Allergy) hypersensitivity reaction
- IgE –> antigen –> mast cell activation –> widespread histamine release
2.) Clinical Features
- bronchospasm: wheeze and chest tightness
- angio-oedema, urticaria, pruritus
- hoarseness, stridor, bronchial obstruction
3.) Emergency Management
- remove trigger, maintain airway, 100% O2
- IM adrenaline 0.5mg (can repeat every 5 mins) [0.3mg if 6-12yrs, 0.15mg if 6mths-6yrs]
- refractory anaphylaxis (no improvement with 2 doses of IM adrenaline): consider IV adrenaline w/ expert help
- NEB salbutamol for bronchospasm, NEB adrenaline for laryngeal oedema
4.) Management After Stabilisation
- non-sedating oral antihistamines may be given following initial stabilisation especially in patients with persisting skin symptoms
- can measure serum tryptase levels to confirm anaphylaxis as they remain elevated up to 12hrs after an acute episode of anaphylaxis
- referral to a specialist allergy clinic (all patients with a new diagnosis)
- patients given an Epi-pen in interim before ^
Bronchiectasis
Pathophysiology
Clinical Features
Investigations
Management
1.) Pathophysiology - chronic inflammation causing irreversible dilation of one or more bronchi
- deformed bronchi have poor mucus clearance so increased susceptibility to bacterial infections
- organisms: H. influenzae, S. pneumoniae P. aeruginosa, Klebsiella spp. S.aureus (w/ CF)
Moraxella catarrhalis, fungi, mycobacteria,
2.) Clinical Features
- chronic cough and daily sputum production
- SOB on exertion, chest pain, haemoptysis,
- fever, fatigue, weight loss, recurrent LRTIs,
- examination: fine inspiratory crackles (rales), wheeze (associated with CF), finger clubbing
- exacerbation: deterioration in 3+ key sx for >48hrs: cough, SOB, fatigue, haemoptysis, sputum purulence
3.) Investigations - for the diagnosis and cause
- CXR: can be normal or may show bronchial wall thickening or airway dilatation
- HR-CT (gold): signet ring sign (dilated bronchus is larger than the accompanying pulmonary artery)
- bronchoscopy: for those with focal bronchiectasis on HR-CT or evidence of possible airway abnormality
- underlying cause: sputum culture, Cl sweat test, FBC, HIV test, immunoglobulin panel, abs for vaccinations
- pulmonary function tests: FEV1:FVC < 70%, elevated RV:TLC (air trapping), ↓diffusing capacity for CO
4.) Management - treat the underlying cause
- physiotherapy: mucus/airway clearance/postural drainage
- Abx for acute exacerbations ?prophylactic Abx
- vaccines e.g. flu, bronchodilators (if wheezing)
- pulmonary rehab: if MRC dyspnea score 3+
- complications: recurrent infection, lung abscess, pneumothorax, life-threatening haemoptysis, poor growth and development
Causes of Bronchiectasis
Post Infective
Immune Deficiency
Mucociliary Clearance Defects
Obstruction
Toxic Insult
Others
1.) Post Infective - bronchial damage causes bronchiectasis after a severe LRTI:
- S.pneumoniae, S.aureus, adenovirus, influenza virus, measles, Bordetella pertussis (whooping cough), TB
2.) Immune Deficiency - increased infection risk
- hypogammaglobulinaemia, common variable immune deficiency (CVID) or IgA/IgG deficiency
- secondary immune deficiency (HIV, malignancy)
- ataxia telangiectasia
3.) Mucociliary Clearance Defects
- cystic fibrosis (most common cause of bronchiectasis)
- primary ciliary dyskinesia, Young’s syndrome, Kartagener syndrome
4.) Obstruction
- foreign bodies, tumours, extrinsic lymph nodes
5.) Toxic Insult
- gastric aspiration, inhalation of toxic chemicals/gases
6.) Others - alpha-1 antitrypsin deficiency, allergic bronchopulmonary aspergillosis,
- IBD, RA (associated with bronchiectasis)
Aetiology and Pathophysiology of Cystic Fibrosis
Aetiology
Respiratory Tract
Pancreas
Gastrointestinal Tract
Reproductive Tract
1.) Aetiology - multisystem, autosomal recessive disorder affecting mucus glands all over the body
- caused by a genetic mutation/defect in the CFTR gene on chromosome 7 affecting chloride channels
2.) Respiratory Tract - defective CFTR disrupts Cl- movement and reduces Na/water reabsorption
- this impairs mucus clearance as secretions are less watery so there is reduced airway surface liquid
- this provides a niche for bacterial growth making CF patients increasingly susceptible to airway infections
3.) Pancreas - defective CFTR leads to occlusion of the pancreatic duct in-utero, causing permanent damage
- this causes pancreatic insufficiency in CF patients
- over time, the endocrine pancreas is also affected which can lead to CF-related diabetes mellitus
4.) GI Tract - defective CFTR causes the SI to secrete viscous mucus –> bowel obstruction in-utero
- this causes meconium ileus in the infant
- CF can also cause cholestasis in the biliary tree which can result in neonatal jaundice
- in later life, it can cause distal intestinal obstruction syndrome (DIOS) and CF-related liver disease
5.) Reproductive Tract - defective CFTR leads to congenital absence of the vas deferens causing:
- male infertility (98% of men): healthy sperm but can’t get from the testes to the ejaculate
- reduced fertility in women due to thicken cervical mucus but they can still get pregnant
Clinical Features and Diagnosis of Cystic Fibrosis
Presentation of Cystic Fibrosis
Other Symptoms and Signs of Cystic Fibrosis
Chloride Sweat Test
Other Investigations
1.) Presentation of Cystic Fibrosis - depends on the age
- neonates: meconium ileus (no passing of meconium w/in 24hrs, abdo distension, and bilous vomiting), positive in the newborn heel prick test, failure to thrive, prolonged neonatal jaundice
- infancy: failure to thrive, recurrent chest infections, steatorrhoea (pancreatic insufficiency)
- childhood: rectal prolapse, nasal polyps, sinusitis
- adolescence: DM (pancreatic insufficiency), chronic lung disease, DIOS, gallstones, liver cirrhosis
2.) Other Symptoms and Signs of Cystic Fibrosis
- chronic cough, thick sputum production, recurrent RTIs
- abdominal pain and bloating, steatorrhea
- concentrated salt in sweat, failure to thrive
- signs: nasal polyps, finger clubbing, crackles and wheeze on auscultation, abdo distension
3.) Chloride Sweat Test - diagnostic w/ fitting clinical hx, may be supported w/ 2 identified mutations
- measures the electrolyte concentration in a sweat sample (collected by pilocarpine iontophoresis)
- Cl >60mM is suggestive of CF, 40-60mM is borderline (needs repeating)
- test can fail if there’s an insufficient sample (small baby)
- false +ve: malnutrition, adrenal insufficiency, glycogen storage diseases, nephrogenic DI, hypothyroidism, hyperparathyroidism, G6PD, ectodermal dysplasia
- false -ve: skin oedema due to hypoalbuminaemia secondary to pancreatic exocrine insufficiency
4.) Other Investigations
- annually: CXR or CT, OGTT (teenagers), LFTs, clotting, bone profile
- cough/sputum sample at every clinical encounter
- lung function: spirometry/lung clearance index
- faecal elastase, HR-CT, genetic analysis
Management of Cystic Fibrosis
Nourishment and Exercise
Managing/Preventing Airway Infections
Use of Antibiotics
Other
1.) Nourishment and Exercise
- exercise improves resp function and reserve, and helps clear sputum
- high-calorie diet is required due to malabsorption, ↑respiratory effort, coughing, infections, physiotherapy
- pancreatic insufficiency: enzyme supplementation (Creon) w/ fatty meals, vitamin A/D/E/K supplements
- poor weight gain: build up milkshakes to supplement meals, supplemental enteral feeding (via gastrostomy) in extreme cases of malnutrition
2.) Managing/Preventing Airway Infections
- active segregation of CF patients reduces cross-infection esp those w/ Pseudomonas and TB
- vaccinations: e.g. pneumococcal, influenza, varicella
- chest physio (daily) to clear mucus and ↓ infection and colonisation risk
- continual sputum samples (or cough swabs) to identify colonising organisms: common ones include S.aureus, H.influenzae, P.aeruginosa
- treat infections w/ at least 2wks of high dose Abx
- Pseudomonas infection has ↑morbidity/mortality as it is harder to eradicate due to antibiotic resistance
3.) Use of Antibiotics
- high doses for at least 2wks if oral
- IV abx if not responsive to oral antibiotics, or as part of some pseudomonas eradication regimens
- need repeat culture to ensure the infection is cleared
- prophylactic flucloxacillin used in infants (until 3yrs) to protect against bacterial infections (esp S.aureus)
- chronic Pseudomonas infections treated w/ long-term inhaled abx (tobramycin) to suppress its growth
- regular azithromycin reduces exacerbations and improves lung function in all CF patients
4.) Other
- annual review: clinical sx, abx courses, sputum samples, bloods (FBC, U+Es, LFTs, clotting, HbA1c, vit A/D/E levels), lung function tests, CXR, OGTT (>12s)
Complications of Cystic Fibrosis
Respiratory Infections
Low Body Weight
Distal Intestinal Obstruction Syndrome
CF Related Diabetes
Others
1.) Respiratory Tract
- bronchiectasis, haemoptysis, pneumothorax
- pulmonary hypertension and right heart strain
- respiratory failure (occurs eventually in CF patients)
- nasal polyps which may be associated with sinusitis
- allergic bronchopulmonary aspergillosis (ABPA): an immune response to Aspergillus spp, initially treated w/ oral corticosteroids (prednisolone) and itraconazole
2.) Gastrointestinal Tract
- cholestasis, gallstones, liver cirrhosis, liver disease
- rectal prolapse: may be due to frequent passage of bulky stools, initially tx w/ laxative and pancreatic enzyme replacement to minimise straining
- distal intestinal obstruction syndrome (DIOS): distal ileum obstruction due to a slower intestinal tract, sx: colicky abdo pain, palpable mass in the RLQ
3.) Endocrine
- CF-related diabetes: associated with a rapid decline in lung function and disease progression, sx inc weight loss, anorexia, ↓lung function, DKA is rarer as there is not a lack of insulin
- delayed puberty: avg delay of 2 years, can lead to ↓ bone mineral density, predisposing children to fractures in adolescence and adulthood
4.) Other
- arthritis, reduced bone mineral density
- Sub or infertility in later adolescence/adulthood
Interstitial Lung Disease
Causes of Interstitial Lung Disease
Clinical Features
Investigations
Management
1.) Causes of Interstitial Lung Disease
- conditions: RA, SLE, CT disorders
- drug-induced: asbestos, methotrexate, amiodarone, nitrofurantoin, cyclophosphamide, bleomycin
2.) Clinical Features
- progressive SOB on exertion, dry cough
- bibasal fine inspiratory crepitation
- clubbing
3.) Investigations
- HRCT: the gold standard for diagnosis
- CXR: bilateral diffuse shadowing
- spirometry: restrictive (FEV1:FVC >70%), and impaired gas exchange (reduced TLCO)
- specific blood tests: ANA, rheumatoid factor, ANCA, anti-GBM, ACE, HIV
4.) Management
- remove occupational exposure, avoid drug-associated
- pulmonary rehab, stop smoking
- N-acetylcysteine, immunosuppressants, pirfenidone
- supplementary oxygen, lung transplant
Different Types of Interstitial Lung Diseases
Usual Interstitial Pneumonia
Extrinsic Allergic Alveolitis
Sarcoidosis
1.) Usual Interstitial Pneumonia - most common type, usually idiopathic
- clubbing, ↓chest expansion, fine inspiratory crepitations,
- may have features of pulmonary hypertension
- non-specific interstitial pneumonia (NSIP) also exists
2.) Extrinsic Allergic Alveolitis - aka hypersensitivity pneumonitis
- acute: 4-8hrs, reversible, spontaneously settles in 1-3 days, can recur
- chronic: chronic exposure, less reversible
3.) Sarcoidosis - multisystem inflammatory condition of unknown cause
- non-caseating granulomas, immunologic response
- can affect nearly all organs e.g. cardiac, brain, kidneys
- treatment is steroids
Smoking Cessation
Medical Therapy
Nicotine Replacement Therapy
Varenicline
Bupropion
1.) Medical Therapy
- should offer nicotine replacement therapy, varenicline or bupropion
- prescribed as part of a commitment to quit at a target stop date
- prescribed to last only until 2 weeks after the target stop date
- target stop date is normally after 2 weeks of NRT therapy, and 3-4 weeks for varenicline and bupropion (allows for different MOAs)
- do not offer NRT, varenicline or bupropion in any combination
- if unsuccessful using NRT, varenicline or bupropion, do not offer a repeat prescription w/in 6 months unless special circumstances
- first-line intervention in pregnancy is CBT, motivational interviewing or structured self-help and support from NHS Stop Smoking Services
- pregnant women should remove the patches before going to bed
2.) Nicotine Replacement Therapy - many different forms including:
- nicotine patches, other forms inc gum, inhalator, lozenge or nasal spray
- combination can be offered to people who show a high level of dependence on nicotine or who have found single forms of NRT
- side effects: nausea & vomiting, headaches and flu-like symptoms
3.) Varenicline - nicotinic receptor partial agonist
- started 1 week before the patient’s target stop date
- recommended course is 12 weeks (only continue if not smoking)
- side effects: nausea (common), headache, insomnia, abnormal dreams
- caution usage in hx of depression or self harm
- contraindicated in pregnancy and breast feeding
4.) Bupropion - nicotinic antagonist AND noradrenaline and dopamine reuptake inhibitor
- started 1 to 2 weeks before the patients target stop date
- side effects: small risk of seizures (1:1000)
- contraindications: epilepsy, pregnancy and breastfeeding e
- eating disorder is a relative contraindication
