Respiratory Flashcards

1
Q

Types of Pneumonia (+organisms)

Community-Acquired (CAP)
Hospital-Acquired (Nosocomial)
Aspiration Pneumonia
Immunocompromised

A

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

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2
Q

Diagnosis of Pneumonia

Clinical Features
CURB-65
Investigations

A

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

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3
Q

Management of Pneumonia

General Measures
Antibiotics for Community-Acquired Pneumonia
Antibiotics for Atypical Organisms
Antibiotics for Noscocmial Pneumonia
Follow Up

A

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

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4
Q

NICE guidelines for Asthma Management

Assessment and Signs of Uncontrolled Asthma

Newly Diagnosed Asthma
Regular Preventer
Initial Add-On
MART Therapy
Additional Control

A

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

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5
Q

Severities of Asthma Exacerbation

Mild
Moderate
Severe
Life-Threatening (+ Near-Fatal)

A

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)

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6
Q

Management of Asthma Exacerbation

Initial Steps
Severe
Life Threatening/Near Fatal
Criteria for Safe Discharge

A

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

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7
Q

COPD

Aetiology
Risk Factors/Causes
Clinical Features
Investigations

A

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)

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8
Q

Management of COPD

Non-Pharmacological
Bronchodilators for Asthmatic Features
Bronchodilators for Non-Asthmatic Features
Others
COPD Exacerbation
Long Term Oxygen Therapy

A

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: using venturi mask, 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)

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9
Q

Pleural Effusion

Clinical Features
Transudative
Exudative
Investigations
Management

A

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
- US-guided pleural aspiration: protein >30g/dL = exudative, Light’s criteria (pleural:serum protein/LDH >0.5/0.6 suggests exudative)
- pleural fluid for cytology, MC+S, AAFB
- CXR: blunting of costo/cardiophrenic angles, meniscus sign, mediastinal shift (if large)
- staging CT w/contrast: underlying pathology
- bloods: routine inc clotting, bone profile, LDH
- ECG, ECHO (?HF)

5.) Management - treat underlying cause
- urgent chest drain only if underlying empyema (visible pus 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

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10
Q

Tension Pneumothorax

Definition
Aetiology
Clinical Features
Chest X-Ray

A

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

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11
Q

Management of Pneumothorax

Definitions
Management of Primary Pneumothorax
Management of Secondary Pneumothorax
Management of Tension Pneumothorax
Discharge Advice

A

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

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12
Q

Pulmonary Embolism

Risk Factors
Clinical Features
Investigations
Management
Thrombolysis

A

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

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13
Q

Massive Haemoptysis

Clinical Features
Management

A

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

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14
Q

Anaphylaxis

Pathophysiology
Clinical Features
Emergency Management
Management After Stabilisation

A

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 ^

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15
Q

Bronchiectasis

Pathophysiology
Clinical Features
Investigations
Management

A

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, P. aeruginosa, Moraxella catarrhalis, fungi, mycobacteria, S.aureus (w/ CF)

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
- 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

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16
Q

Causes of Bronchiectasis

Post Infective
Immune Deficiency
Mucociliary Clearance Defects
Obstruction
Toxic Insult
Others

A

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)

17
Q

Aetiology and Pathophysiology of Cystic Fibrosis

Aetiology
Respiratory Tract
Pancreas
Gastrointestinal Tract
Reproductive Tract

A

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

18
Q

Clinical Features and Diagnosis of Cystic Fibrosis

Presentation of Cystic Fibrosis
Other Symptoms and Signs of Cystic Fibrosis
Chloride Sweat Test
Other Investigations

A

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

19
Q

Management of Cystic Fibrosis

Nourishment and Exercise
Managing/Preventing Airway Infections
Use of Antibiotics
Other

A

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)

20
Q

Complications of Cystic Fibrosis

Respiratory Infections
Low Body Weight
Distal Intestinal Obstruction Syndrome
CF Related Diabetes
Others

A

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

21
Q

Interstitial Lung Disease

Causes of Interstitial Lung Disease
Clinical Features
Investigations
Management

A

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

22
Q

Different Types of Interstitial Lung Diseases

Usual Interstitial Pneumonia
Extrinsic Allergic Alveolitis
Sarcoidosis

A

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

23
Q

Smoking Cessation

Medical Therapy
Nicotine Replacement Therapy
Varenicline
Bupropion

A

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