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MRCP PACES B Flashcards

Recognize and interpret high-yield MRCP PACES cardiovascular, respiratory, and systemic examination findings, including murmurs, congenital conditions, and multisystem disease associations, to guide diagnosis and management. (251 cards)

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1
Q

Why should metallic valves be avoided in women of child-bearing age?

A

High risk of thromboembolic complications due to pregnancy’s procoagulant state.

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2
Q

What are some initial medical options for acute mitral regurgitation?

A
  • Nitrates
  • Diuretics
  • Sodium nitroprusside
  • Inotropes
  • Intra-aortic balloon pump
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3
Q

How does standing and squatting affect murmurs?

A
  • STANDING: Increases mitral valve prolapse and HOCM murmurs
  • SQUATTING: Increases most other murmurs (e.g. VSD, AS, AR, MR)
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4
Q

What can cause continuous murmurs?

A
  • PDA
  • AV fistula
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5
Q

What causes Marfan syndrome?

A

Autosomal dominant mutation in fibrillin-1 gene resulting in loss of elasticity of connective tissues.

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6
Q

What are some clinical features of Marfan syndrome?

A
  • Arachnodactyly
  • High arched palate
  • Tall with long limbs
  • Chest wall deformities (e.g. pectus excavatum)
  • Scoliosis
  • Pes planus
  • Aortic regurgitation
  • Aortic dissection
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7
Q

Why might someone with Marfan syndrome have a large scar from their abdomen to their back?

A

Aortic repair due to dissection.

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8
Q

Which investigations should be requested in suspected Marfan syndrome?

A
  • Echo (aortic regurgitation)
  • CT Aorta (check for dilation + dissection)
  • CXR (pneumothorax, dissection)
  • Fibrillin-1 blood test
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9
Q

What are the cardiac manifestations of Marfan syndrome?

A
  • Aortic dilatation
  • Aortic regurgitation
  • Aortic dissection
  • Mitral valve prolapse
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10
Q

What are the indications for aortic root replacement in people with Marfan syndrome?

A
  • Dilation of > 50 mm at the aortic root
  • Dilation of > 45 mm if family history of aortic dissection
  • Expansion of aortic root of > 3 mm per year
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11
Q

What are the causes of systolic murmurs?

A
  • Aortic and pulmonary stenosis
  • Aortic sclerosis
  • HOCM
  • ASD
  • VSD
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12
Q

What are the symptoms of significant pulmonary stenosis?

A
  • Breathlessness on exertion
  • Peripheral oedema
  • Presyncope and syncope
  • Fatigue
  • Chest pain
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13
Q

What clinical features may be seen on examination of a patient with significant pulmonary stenosis?

A
  • Raised JVP
  • RV heave
  • Pansystolic murmur (functional TR)
  • Peripheral oedema
  • Widely split second heart sound with loud P2
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14
Q

What are some echocardiographic features of severe pulmonary stenosis?

A
  • Gradient > 64 mm Hg
  • Velocity > 4 m/s
  • Valve area < 1 cm²
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15
Q

What causes Noonan syndrome?

A

Autosomal dominant condition caused by a few different mutations.

PTPN11 and SOS1 are most common.

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16
Q

What are the clinical features of Noonan syndrome?

A

FACIAL

  • Triangular-shaped face
  • Hypertelorism
  • Short, webbed neck
  • Low set ears

OCULAR

  • Strabismus
  • Ptosis
  • Refractive errors

OTHER

  • Pectus excavatum
  • Cubitus valgus
  • Mild learning disability
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17
Q

What are the cardiac complications of Noonan syndrome?

A
  • Pulmonary stenosis (most common)
  • HOCM
  • ASD
  • VSD
  • Tetralogy of Fallot
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18
Q

How is Noonan syndrome managed?

A
  • NON-MEDICAL: monitor growth and puberty, educational support, feeding support
  • MEDICAL: medical treatment of heart failure, valve replacement
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19
Q

What are the features of tetralogy of Fallot?

A
  • Pulmonary stenosis
  • RV hypertrophy
  • VSD
  • Overriding aorta
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20
Q

What are some congenital causes of pulmonary stenosis?

A
  • Noonan syndrome
  • Alagille syndrome
  • Tetralogy of Fallot
  • Congenital rubella syndrome
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21
Q

What are some acquired causes of pulmonary stenosis?

A
  • Carcinoid syndrome
  • Rheumatic heart disease
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22
Q

How is pulmonary stenosis categorized based on echo findings?

A

Categorized by valve gradient:

  • < 36 mm Hg: mild
  • 36-64 mm Hg: moderate
  • > 64 mm Hg: severe
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23
Q

What are some causes of mitral valve prolapse?

A
  • Marfan syndrome
  • Ehlers-Danlos syndrome
  • Polycystic Kidney Disease
  • Osteogenesis Imperfecta
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24
Q

What vascular complications are associated with Ehlers-Danlos syndrome?

A
  • Aortic dissection
  • Aortic aneurysm
  • Spontaneous coronary artery dissection
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25
How can you test for **hypermobility**?
* Bend fingers back * Make thumb touch wrist
26
What are the main complications of mitral valve prolapse?
* Infective endocarditis * Thromboembolic events (e.g. stroke) * Sudden death * Heart failure
27
What **murmur** is heard in mitral valve prolapse?
Dynamic systolic click and mid-late systolic murmur
28
Which patients with mitral valve prolapse are considered higher risk?
* Moderate-severe mitral regurgitation * Reduced LV function * Increased end systolic diameter * AF * Left atrial enlargement * Age > 50 years * Valve thickening > 5 mm * Flail leaflet
29
What is a **mitral clip**?
Percutaneous repair option for mitral valve prolapse in people with suitable anatomy and no surgical option.
30
What does the ductus arteriosus connect?
Proximal pulmonary trunk and descending aorta (just distal to left subclavian artery).
31
Which cases of PDA are considered for **closure**?
* LV volume overload * RV pressure overload ## Footnote This is usually device closure which is done percutaneously.
32
What murmur is associated with PDA?
* Continuous machine-like murmur (higher flow during systole) * Heard best in the second intercostal space just left of sternum * Radiates to back near scapulae
33
Which investigations should be requested in suspected PDA?
* Echo (check for LV volume overload, estimate PA pressure) * Cardiac MRI * Cardiac CT ## Footnote If PA pressure high on echo --> invasive testing (cardiac catheterisation).
34
What are the **benefits** of invasive cardiac catheterisation?
* Measurement of right sided pressures * Calculate pulmonary and systemic vascular resistance
35
What are the clinical features of a PDA?
* Left subclavian thrill * Continuous machinery murmur * Wide pulse pressure and bounding pulse
36
How is PDA managed?
* Babies: Indomethacin * Open surgical or endovascular closure ## Footnote Small PDAs without any adverse features do not require closure.
37
How can pulmonary stenosis be distinguished from patent ductus arteriosus on examination?
* PDA is louder on expiration * PDA radiates to the left scapula (whereas PS tends to radiate to centre of back)
38
How does inspiration make right sided murmurs louder?
* Decreases intrathoracic pressure * Increases venous return to the right side of the heart * Increases blood flow across right sided valves resulting in more turbulent flow and louder murmurs
39
What are the **cardiac** causes of clubbing?
* Congenital cyanotic heart disease * Infective endocarditis * Atrial myxoma
40
What are the **gastrointestinal** causes of clubbing?
* Cirrhosis * Coeliac disease * Crohn's disease
41
What is **Eisenmenger syndrome**?
Reversal of a left-to-right shunt in people with a long-standing cardiac defect such as an ASD, VSD or PDA.
42
What are the clinical signs of Eisenmenger syndrome?
* Dyspnoea * Cyanosis * Clubbing * Pulmonary hypertension signs (elevated JVP, functional TR) * Parasternal heave (pressure overloaded RV)
43
Which congenital syndromes cause defects that may result in **Eisenmenger syndrome**?
* Down syndrome (VSD and AVSD) * Edwards syndrome (VSD and ASD) * DiGeorge syndrome (interrupted aortic arch, TOF, VSD)
44
Why would the intensity of a VSD murmur decrease in Eisenmenger syndrome?
The reversal of flow means that the murmur is quieter.
45
What consideration needs to be taken for anyone with a VSD who is undergoing surgery on infected tissue?
Endocarditis prophylaxis
46
Which medications may be used in the management of **pulmonary hypertension**?
* Endothelin antagonists (e.g. bosentan) * Phosphodiesterase 5 inhibitors (e.g. sildenafil) * Prostanoid infusions
47
What are the indications for closure in patients with VSDs who have not yet developed Eisenmenger syndrome?
* Pulmonary to systemic blood flow ratio > 2 (significant shunting) * LV dysfunction * History of endocarditis * Acute septal rupture following MI
48
What are the clinical features of a **VSD**?
* Systolic murmur * Parasternal heave
49
How is a VSD managed?
Spontaneous closure possible in small muscular defects * **MEDICAL**: Diuretics, ACE inhibitors * **SURGICAL**: Percutaneous closure
50
What are the main complications of **Eisenmenger syndrome**?
* RV failure * Massive haemoptysis * Cerebral embolism * Infective endocarditis
51
What is 'functional' or 'secondary' mitral regurgitation?
When dilation of the left atrium or left ventricle (e.g. due to volume overload) prevents the mitral valve leaflets from properly opposing.
52
What complications may arise following surgery for **tetralogy of Fallot**?
* Pulmonary regurgitation (due to intervention to dilate RV outflow obstruction) * Endocarditis * Paradoxical embolism * Arrhythmia
53
How is tetralogy of Fallot surgically managed?
* Close VSD with Dacron patch * Resection of obstructing muscle tissue in right ventricle and enlarging outflow pathway with patch
54
What is a **Blalock-Taussig shunt**?
Synthetic shunt between brachiocephalic trunk and pulmonary artery to enhance pulmonary blood flow whilst awaiting further intervention. ## Footnote This is why some TOF patients may have large posterior thoracotomy scars. Causes pulse differential.
55
What are the causes of **cyanotic heart disease**?
* TOF * TGA * Tricuspid atresia * Pulmonary atresia * Ebstein's anomaly * Eisenmenger syndrome
56
What are the causes of **acyanotic heart disease**?
* ASD * VSD * Coarctation of the aorta * PDA * Aortic stenosis
57
What are possible causes of a **posterolateral thoracotomy scar**?
* Previous lobectomy or pneumonectomy * Wedge resection * Bullectomy * Surgery on aorta (e.g. coarctation) * Blalock-Taussig shunt
58
What are the echocardiogram features of **restrictive cardiomyopathy**?
* Good LV systolic function * Biventricular diastolic dysfunction * Biatrial dilatation
59
What are some causes of **restrictive cardiomyopathy**?
**PRIMARY** * Loeffler's endocarditis (eosinophilic infiltration) * Endomyocardial fibrosis **SECONDARY** * Amyloidosis (most common) * Sarcoidosis * Iron overload * Scleroderma * Radiotherapy
60
Which investigations are useful in suspected restrictive cardiomyopathy?
* **BLOODS**: liver function (congestion), cardiac enzymes * ECG: AF * Echo * Cardiac catheterisation (elevated right heart pressures) * Cardiac MRI (helps distinguish from constrictive pericarditis) * Biopsy
61
How is restrictive cardiomyopathy managed?
* Heart failure management (diuretics, ACE inhibitors) * AF management (beta-blockers, anticoagulation) * PPMs and ICDs * Heart transplant
62
How can you distinguish between **constrictive pericarditis** and **restrictive cardiomyopathy** through investigations?
* **CXR**: pericardial calcification * **Echo**: hyperechoic and thickened pericardium * Cardiac MRI ## Footnote NOTE: restrictive pericarditis can be treated surgically with pericardial stripping
63
What are some causes of **constrictive pericarditis**?
* Viral or bacterial pericarditis (e.g. Coxsackie B, TB) * Post-surgical (e.g. CABG) * Radiation
64
How do the bell and diaphragm of the stethoscope differ?
* **Bell** for LOW frequency sounds * **Diaphragm** for HIGH frequency sounds
65
How is secondary mitral regurgitation managed?
Heart failure management and medication optimisation.
66
Which nerve root is responsible for **shoulder abduction**?
C5
67
Which nerve root is responsible for **elbow flexion** and **wrist extension**?
C6
68
Which nerve root is responsible for **elbow extension**?
C7
69
Which nerve root is responsible for **thumb extension** and **ulnar deviation**?
C8
70
Which nerve root is responsible for **finger abduction**?
T1
71
Which nerve root is responsible for **hip flexion**?
L2
72
Which nerve root is responsible for **knee extension**?
L3
73
Which nerve root is responsible for **ankle dorsiflexion**?
L4
74
Which nerve root is responsible for **big toe extension**?
L5
75
Which nerve root is responsible for **ankle plantarflexion**?
S1
76
Which nerve root is responsible for **knee flexion**?
S2
77
What is the **Gallavardin phenomenon**?
Radiation of an aortic stenosis murmur to the apex thus mimicking mitral regurgitation.
78
What can you do to make a diastolic murmur louder?
Lean the patient forward.
79
What can you do to make a murmur from HOCM louder?
Valsalva manoeuvre
80
What are the main causes of **mitral stenosis**?
* Rheumatic heart disease * Calcification * Fabry's disease * Rheumatoid arthritis * SLE * Carcinoid syndrome * Whipple's disease
81
What is **Fabry's disease**?
**X-linked** disorder caused by a mutation in the alpha-galactosidase A gene. ## Footnote Characterised by neuropathic pain, angiokeratomas, gastrointestinal issues, heart valve defects, LVH, and stroke.
82
Which standard panel of investigations should be requested for patients with valvular disease?
* ECG * CXR * Echo * Exercise testing ## Footnote Urine dipstick and 3 x blood cultures if suspecting endocarditis.
83
What are the criteria for diagnosing **infective endocarditis**?
**Duke's criteria**: Diagnostic if either: * 2 Major * 1 Major + 3 Minor * 5 Minor
84
Which organisms most commonly cause infective endocarditis?
* Streptococci (viridans and gallolyticus) * Staphylococcus aureus * HACEK (Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, and Kingella)
85
What are the major Duke's criteria?
* Positive blood cultures for typical organisms * Evidence of endomyocardial involvement (e.g. new valve regurgitation, echocardiogram findings)
86
What is an Austin Flint murmur?
Rumbling diastolic murmur heard at the apex in patients with severe aortic regurgitation. ## Footnote Occurs when the regurgitant jet coming from the aortic valve during diastole strikes the mitral valve leaflet causing premature closing (commonly mistaken for mitral stenosis).
87
What are the causes of **aortic regurgitation**?
* Rheumatic heart disease * Connective tissue disease (e.g. Marfan's, Ehlers-Danlos) * Ankylosing Spondylitis * SLE * Aortic dissection * Infective endocarditis
88
What is **balloon valvuloplasty**?
Temporising procedure that involves dilating a stenosed valve to improve its function whilst awaiting more definitive management
89
Which type of murmur is concerning in someone with a valve replacement?
Regurgitant murmurs | (Suggests that the valve may not be fully competent) ## Footnote It is quite common to get flow murmurs.
90
Why is PR prolongation of significance in people with a valve replacement?
May be suggestive of an aortic valve abscess.
91
What are the main complications of valve replacement?
* Infection (endocarditis) * Stroke * Bleeding (from anticoagulation) * Haemolysis (from stenosed valve)
92
What are the main indications for a permanent pacemaker?
* Bradyarrhythmias (3rd degree, Mobitz type 2) * Sick sinus syndrome with symptoms
93
What are the indications for an **ICD**?
**PRIMARY** (inherited risk of sudden cardiac death) * Long QT syndrome * Brugada * HOCM * MI > 4 weeks ago and meeting certain EF/VT criteria **SECONDARY** * After VT or VF arrest * Spontaneous sustained VT causing syncope/haemodynamic compromise * Sustained VT with EF < 35%
94
Who would benefit from CRT?
Heart failure with LVEF < 35% and ventricular conduction impairment ## Footnote e.g. broad QRS, LBBB
95
What can help distinguish arthralgia in hereditary haemochromatosis from osteoarthritis?
Chondrocalcinosis | (and pseudogout)
96
Which blood test results should prompt further investigations for hereditary haemochromatosis?
Raised ferritin and transferrin saturation > 45%
97
What are the main clinical features of hereditary spherocytosis?
* Jaundice * Anaemia * Splenomegaly
98
How is hereditary spherocytosis treated?
* Blood transfusions * Folic Acid * Splenectomy
99
How should suspected **coeliac disease** be investigated?
* Bloods (check for anaemia, check anti-tTG whilst on gluten-containing diet) * Endoscopy (subtotal villous atrophy with crypt hyperplasia)
100
Where is a transplanted pancreas attached in an SPK?
To the small bowel. ## Footnote Used to be connected to the bladder (urinary lipase can be monitored for rejection).
101
Which patients undergo an **SPK**?
Poorly controlled T1DM with renal failure | (sometimes done for T2DM) ## Footnote SPK has a better 10-year survival than isolated kidney transplant in patients with diabetic nephropathy.
102
What does the **scar** in an SPK look like?
Two scars in each iliac fossa or midline laparotomy
103
How do you assess for **encephalopathy** in liver disease?
* Asterixis * Constructional dyspraxia (can't draw a 5-pointed star)
104
Which prognostic scores are used in **cirrhosis**?
* Child-Pugh (ascites, encephalopathy, INR, bilirubin and albumin) * UK MELD (INR, creatinine, bilirubin, sodium)
105
What are the main causes of **cirrhosis**?
* Alcohol * Viral hepatitis * Non-alcoholic liver disease * Medications (e.g. methotrexate) * PBC * PSC * Wilson's disease * A1AT deficiency
106
What is a clinical clue that alcohol is the cause of cirrhosis?
Parotid swelling
107
Which gene is involved in the hereditary form of pancreatitis?
* PRSS1 (autosomal dominant) * SPINK1 ## Footnote Cystic fibrosis is another inherited cause of pancreatitis.
108
What are the main causes of pancreatitis?
* Alcohol * Gallstones * Drugs (e.g. azathioprine) * ERCP * Autoimmune * Mumps * Hypercalcaemia * Hyperlipidaemia
109
What are the complications of pancreatitis?
* **ACUTE**: SIRS, respiratory failure * **CHRONIC**: portal vein thrombosis, chronic pancreatitis, pseudocyst formation
110
How are **pseudocysts** drained?
Usually with an endoscopic approach using ultrasound guided drainage . ## Footnote Can be stented using an Axios stent.
111
Why does **creon** need to be given with a **PPI**?
To prevent the acidity from breaking down creon in the stomach.
112
What are some of the complications of **chronic pancreatitis**?
* Pain * Type 3c diabetes * Strictures * Compressive biliary obstruction
113
How is chronic pancreatitis managed?
* Creon * Stop drinking alcohol * Stent insertion for biliary obstruction or pancreatic duct strictures * Pain management
114
Why are ileostomies spouted rather than flush to the skin?
To prevent the contents from irritating the skin.
115
What are some indications for **emergency surgery** in patients with inflammatory bowel disease?
* Toxic megacolon * Haemorrhage * Perforation ## Footnote Non-emergency: fistula, poor symptom control despite maximal medical therapy
116
What is the purpose of leaving a **mucus fistula** when operating on a patient with inflammatory bowel disease?
It allows the distal bowel to discharge gas and secretions. ## Footnote It may be done to preserve the distal bowel/rectum in case anastomotic surgery is performed in the future (e.g. ileal pouch anal anastomosis).
117
What are some extra-GI manifestations of inflammatory bowel disease?
* Erythema nodosum * Arthritis * Liver dysfunction * Aphthous ulcers
118
What are the main pharmacological agents used in the management of **ulcerative colitis**?
* 5-ASAs * Steroids (acute phase) * Steroid-sparing agents (e.g. azathioprine, methotrexate) * Biologics (e.g. infliximab)
119
What are some of the side-effects of **ciclosporin** use?
* Gum hypertrophy * Hypertension * Increased risk of cancer (esp. post-transplant lymphoproliferative disorders)
120
What are some clinical features of **portal hypertension**?
* Caput medusae * Splenomegaly (this may not change post transplant) * Rectal varices
121
What are the indications for **liver transplant**?
* Acute and chronic liver failure * Hepatocellular carcinoma ## Footnote Causes include drug-induced liver injury (e.g. paracetamol), alcoholic liver disease, autoimmune hepatitis, NASH, viral hepatitis.
122
What are the criteria that need to be fulfilled for a patient to be listed for an **elective liver transplant**?
* UKELD of 49 or more * Portopulmonary hypertension with significant response to prostacycline analogues, sildenafil or bosentan * Severe acute alcoholic hepatitis if expected survival without transplant is < that with transplant * Post-transplant 5-year survival of > 50% * HCC number and diameter of tumours and rate of progression
123
What are clinical features that distinguish **pneumonectomy** from **lobectomy**?
* **Tracheal deviation**: definite in pneumonectomy, possible in lobectomy * **Absent breath sounds**: present in pneumonectomy, may be normal/reduced in lobectomy * **Dull percussion note**: noted in pneumonectomy
124
Why does a lateral thoracotomy scar from a **lobectomy** suggest **non-small cell lung cancer** more than **small cell lung cancer**?
* **Non-small cell lung cancer** is more common. * **Small cell lung cancer** is rapidly progressive and often not suitable for surgery.
125
Which operations may have been performed in a patient who has had a posterior thoracotomy?
* Lobectomy * Pneumonectomy * Bullectomy * Apical pleurectomy (for recurrent pneumothoraces) * TB (only in old patients) * Trauma * Lung abscess
126
What are some indications for a **VATS** procedure?
* Lobectomy (NOT pneumonectomy) * Pleurectomy (may be done for recurrent pneumothoraces) * Pleurodesis * Bullectomy
127
When might a patient be considered for surgical management of a pneumothorax?
* Recurrent pneumothoraces * Persistent air leak
128
How does **VATS** compare to an **open thoracotomy**?
**VATS** is less invasive, has lower pain risk, and better recovery, but has a higher recurrence risk.
129
How is a primary spontaneous pneumothorax managed?
* If size > 2 cm and/or breathless --> aspirate with 16-18 G cannula (< 2.5 L) --> Chest Drain * If size < 2 cm and not breathless --> consider discharge and review in 2-4 weeks
130
How is a secondary spontaneous pneumothorax managed?
131
What are the sites of needle aspiration and chest drain insertion?
* Needle aspiration --> 2nd ICS MCL or safe triangle * Chest drain --> safe triangle
132
What are some complications of **pneumothoraces**?
* Surgical emphysema * Tension pneumothorax (resulting in hypotension and cardiac arrest)
133
What are some causes of **wheeze**?
* Asthma * COPD * Bronchiectasis * Bronchiolitis Obliterans (secondary to viral infections, pollutants, graft-versus-host disease)
134
Which investigations are useful in the diagnosis of **asthma**?
* Bloods (FBC, CRP to look at inflammatory markers and eosinophils) * Peak Flow Diary (normal variability: 20%) * CXR * Spirometry
135
What **spirometry** results would you expect to see in asthma?
* FEV1/FVC Ratio < 0.7 * Bronchodilator reversibility: 200 mL or 15% improvement in FEV1
136
Outline the **management guidelines** for asthma.
1. SABA (salbutamol) 2. ICS (beclomethasone or budesonide) 3. ICS + LABA (e.g. formoterol) OR MART 4. Increase ICS or add Montelukast 5. Add LAMA or Theophylline 6. Specialist (oral steroids, omalizumab, ciclosporin) ## Footnote Intervention: bronchial thermoplasty
137
What are some causes of **interstitial lung disease**?
* Idiopathic **pulmonary fibrosis** * **Connective tissue diseases**: rheumatoid arthritis, scleroderma, SLE * **Drugs**: amiodarone, methotrexate * **Other**: sarcoidosis, asbestosis, silicosis, extrinsic allergic alveolitis
138
Which investigations should be requested in patients with suspected **interstitial lung disease**?
* SaO2 * ABG * Autoantibodies (ANA, RF, anti-CCP, ACE) * CXR * HRCT * Spirometry (restrictive + reduced TF) * Bronchoscopy and biopsy
139
How is **interstitial lung disease** managed?
* Steroids (if deemed steroid responsive as in sarcoidosis) * MDT approach (OT/PT/respiratory nurses) * Pirfenidone or Nintedanib * Lung transplantation
140
What are the main findings on HRCT in a patient with interstitial lung disease?
* Ground glass opacities (suggestive of inflammation that may be steroid responsive) * Honeycombing
141
Which cause of interstitial lung disease is most commonly associated with **clubbing**?
Idiopathic pulmonary fibrosis
142
What is a restrictive pattern on spirometry?
FEV1 < 80% FVC < 80% FEV1/FVC > 0.7
143
How is non-specific interstitial pneumonia managed?
* Steroids * Steroid-sparing agents (e.g. azathioprine, mycophenolate) ## Footnote NOTE: Prognosis for NSIP is much better than for IPF.
144
What is the **life expectancy** in idiopathic pulmonary fibrosis?
2-5 years
145
What are the main causes of **upper lobe predominant pulmonary fibrosis**?
* TB * Silicosis * Extrinsic allergic alveolitis * Ankylosing spondylitis * Cystic fibrosis
146
What are the main causes of **lower lobe predominant pulmonary fibrosis**?
* Idiopathic pulmonary fibrosis * Asbestosis * Rheumatoid arthritis * Scleroderma
147
Why might someone with cystic fibrosis have a **portacath**?
For long-term intravenous antibiotics
148
Why might someone with cystic fibrosis have a **PEG tube**?
To supplement their nutritional intake (if struggling to maintain weight with oral feeding alone in the context of pancreatic insufficiency and being in a catabolic state).
149
Which gene mutation causes **cystic fibrosis**?
CFTR gene on chromosome 7 | (autosomal recessive) ## Footnote Most common mutation is Delta F508 mutation.
150
What is a **button gastrostomy**?
Shortened gastrostomy tube that lies flush to the chest wall and is more discrete.
151
What are the main clinical manifestations of **cystic fibrosis**?
* Bronchiectasis * Recurrent infections * Pancreatic exocrine and endocrine insufficiency * Cystic fibrosis liver disease * Osteopaenia * Male infertility * Nasal polyps * Constipation * Gallstones
152
How is **cystic fibrosis** screened for in the UK?
All infants have a heel prick test which measures immune reactive trypsinogen. Raised levels will prompt a panel of gene tests for the most common genetic causes. Later, can do sweat test (raised chloride in CF).
153
Which organisms chronically colonise patients with **cystic fibrosis**?
* Pseudomonas aeruginosa * Burkholderia cepacia (can't have lung transplant) * Non-tuberculous mycobacteria (can't have lung transplant) * Staphylococcus aureus (in childhood)
154
How are the chest manifestations of cystic fibrosis managed?
* Regular chest physiotherapy * Exercise * Nebulisers (including mucolytics) * Prophylactic antibiotics ## Footnote New development: ivacaftor/lumacaftor (Orkambi)
155
What is the life expectancy for people with **cystic fibrosis** in the UK?
40 years
156
What is the incidence of cystic fibrosis in the UK?
1 in 2500 live births
157
What are some differential diagnoses for cystic fibrosis?
* Primary immunodeficiency * Primary ciliary dyskinesia * Chronic aspiration
158
Which scar would you expect in someone with a **bilateral lung transplant**?
* Clamshell * Midline Sternotomy ## Footnote NOTE: They may also have intercostal drain scars.
159
What are the criteria for **lung transplantation**?
Chronic end stage lung disease with: * > 50% risk of death from end stage lung disease within 2 years if transplant not performed * > 80% likelihood of surviving at least 90 days post transplant * > 80% likelihood of surviving 5 years post-transplant provided the graft functions
160
What are the most common **indications** for lung transplant?
* Interstitial lung disease * Cystic fibrosis * COPD * Pulmonary vascular diseases ## Footnote NOTE: bilateral lung transplant has better survival than single lung transplant. Bilateral is better in CF to prevent spillage of chronic infection from bad lung into good lung.
161
What are some **complications** of lung transplantation?
* Acute rejection * Chronic rejection (bronchiolitis obliterans syndrome) * Immunosuppression complications (opportunistic infections, malignancy (PTLD, skin malignancies), cushingoid, tremor in tacrolimus) * Bronchial stenosis
162
What are the main differences in indications for single vs double lung transplantation?
**SINGLE** * COPD * Interstitial lung disease **DOUBLE** * Suppurative lung disease (cystic fibrosis, generalised bronchiectasis) ## Footnote NOTE: but given better survival of double lung transplant, it may be performed in any of the above cases.
163
What are the **absolute contraindications** for lung transplantation?
* Recent malignancy * Significant untreatable other organ dysfunction (heart/liver/kidney/brain) * Chronic infections (Mycobacterium abscessus, Burkholderia cepacia) * Significant chest wall or spinal deformity * Non-adherence to treatment * Significant psychiatric or psychological conditions * Substance abuse * Sepsis * Acute MI * BMI > 35
164
When should patients with **cystic fibrosis** be referred for lung transplantation?
* FEV1 < 30% * Significant pulmonary artery hypertension * High exacerbation frequency * Recurrent pneumothoraces * Life-threatening haemoptysis (despite bronchial artery embolisation) * Need for NIV
165
Which scoring system is used to categorise the severity of COPD?
BODE Index ## Footnote NOTE: Score of >7 should be considered for lung transplantation.
166
Which combination of immunosuppressants tend to be used in lung transplantation?
* Prednisolone * Calcineurin inhibitor (e.g. tacrolimus) * Nucleotide blocker (e.g. azathioprine, MMF) ## Footnote NOTE: Also need prophylactic medications to prevent opportunistic infections.
167
What are the main features of **yellow nail syndrome**?
* Slow-growing and thickened finger nails * Bronchiectasis or pleural effusions * Lymphoedema
168
What are some causes of **bronchiectasis**?
* Idiopathic * Tuberculosis * Past severe pneumonia (e.g. Pertussis) * Cystic fibrosis * Primary ciliary dyskinesia * Immunoglobulin deficiencies * Rheumatoid arthritis * Inflammatory bowel disease * Yellow Nail Syndrome * ABPA
169
Which investigations should be requested in a patient with bronchiectasis?
* Sputum MCS + Fungal + Mycobacterial * Spirometry * CXR * HRCT * HIV test * Immunoglobulin levels * Aspergillus precipitins * Autoimmune screen (if CTD suspected) ## Footnote If under 40, check for cystic fibrosis.
170
How is **primary ciliary dyskinesia** diagnosed?
Nasal biopsy or nasal potential difference
171
How is **bronchiectasis** managed?
* Daily physiotherapy (postural drainage and ACBT) * Relevant vaccinations (e.g. pneumococcal and influenza) * Mucolytics (e.g. carbocisteine) * Nebulised hypertonic saline * Prophylactic azithromycin * Surgery (for localised bronchiectasis)
172
What are some extra-pulmonary clinical features of lung cancer?
Radiotherapy tattoos Clubbing Hypertrophic pulmonary osteoarthropathy Wasting of small hand muscles Hoarse voice (recurrent laryngeal nerve palsy) Cachexia Horner's syndrome SVCO (dilated chest wall veins and facial swelling)
173
Which tests should be requested in suspected **lung cancer**?
* FBC (evidence of infection) * U&E (hyponatraemia in SIADH) * Calcium (raised in PTHrP or mets) * LFT (liver mets) * Clotting (for biopsy) * Biopsy (bronchoscopy, percutaneous or mediastinoscopy) * Aspirate pleural effusion * Lymph node biopsy * Spirometry (fitness for surgery) * Staging CT
174
What are the main types of **lung cancer**?
* Small Cell (20%) * Non-Small Cell * Squamous cell lung cancer * Adenocarcinoma (MOST COMMON) * Large cell lung cancer
175
What are the **main differences** between adenocarcinoma and squamous cell carcinoma of the lung?
* **Adenocarcinoma** is more common in patients who have never smoked and tend to be peripheral * **Squamous cell** tends to be more central and associated with smoking. May produce PTHrP. More likely to cavitate.
176
What are the main **treatment** approaches for lung cancer?
* Surgery (mainly for localised non-small cell lung cancer) * Chemo- and radiotherapy * Targeted therapies (e.g. EGFR and PDL1 inhibitors like gefitinib and pembrolizumab) * Palliative management
177
Which **paraneoplastic syndromes** are associated with lung cancer?
* Lambert-Eaton myasthenic syndrome * SIADH * Hypercalcaemia (PTHrP) * Cushing syndrome (ectopic ACTH)
178
List some causes of **transudative pleural effusions**.
* Left ventricular failure * Renal failure * Hypoalbuminaemia (nephrotic syndrome, cirrhosis) * Myxoedema * Peritoneal dialysis
179
List some causes of **exudative pleural effusions**.
* Bacterial pneumonia * Tuberculosis * Malignancy * CTDs (e.g. rheumatoid arthritis) * Vasculitis
180
Which tests should a pleural tap be sent for?
* pH * Protein * LDH * Cytology * Gram stain * AFB stain * Culture ## Footnote Other: glucose (low in infection, malignancy and rheumatoid arthritis), amylase (raised in pancreatitis), triglycerides (chylothorax)
181
What features would allow a pleural aspirate to be labelled as an **exudate**?
* Protein > 35 g/L * pH < 7.1 * If 25-35 g/L then Light's criteria: * Fluid: serum protein ratio > 0.5 * Fluid: serum LDH ratio > 0.6 * Fluid LDH > 2/3 upper limit of normal serum LDH
182
What are the indications for inserting a **chest drain** after a pleural aspirate?
* pH < 7.2 (empyema) * Positive Gram stain or positive culture * Frank pus
183
Which investigations should be considered if the results of a pleural aspirate in a patient with an effusion is not conclusive?
* CT Thorax * Pleural biopsy * Medical thoracoscopy (visualisation of pleura and biopsy)
184
What FEV1 cut-offs are used to determine suitability for a lobectomy or pneumonectomy?
* Pneumonectomy > 2 L * Lobectomy > 1.5 L ## Footnote NOTE: Other investigations include walk test, formal cardiopulmonary exercise test and echocardiogram.
185
What is the main indication for a **pneumonectomy**?
Large, central non-small cell lung cancer or tumours affecting both lobes. ## Footnote NOTE: Another indication is bronchiectasis.
186
What is a **Chamberlain procedure**?
Parasternal mediastinotomy in someone who also has a pneumonectomy. This allows access to mediastinal lymph nodes.
187
What are some differentials for **bibasal crepitations**?
* Interstitial lung disease * Heart failure * Bilateral pneumonia * Bronchiectasis
188
What can cause **breathlessness** with normal lung sounds?
* PE * Anaemia * Obesity hypoventilation * Anxiety * Pulmonary hypertension
189
Which investigations should be requested in suspected **obstructive sleep apnoea**?
* Polysomnography * FBC (polycythaemia) * Morning ABG * CXR, ECG and Echo (pulmonary hypertension)
190
How is **OSA** managed?
* Weight loss * Smoking cessation * Nocturnal CPAP * Oral appliances * Drugs (e.g. modafinil)
191
What are some **complications** of OSA?
* Hypertension * MI * Stroke * Pulmonary hypertension
192
What is **Felty's syndrome**?
A triad of rheumatoid arthritis, splenomegaly and neutropaenia.
193
Why is platelet count low in **alcohol excess**?
* Alcohol has direct toxic effects on the bone marrow * Splenomegaly can lead to sequestration
194
List some features associated with **hepatosplenomegaly** and the disease that they correlate with.
* **Anaemia**: myeloproliferative disorders * **Lymphadenopathy**: lymphoma, TB, sarcoidosis * **Parkinsonism**: Wilson's disease * **Xanthoma/Xanthelasma**: PBC * **Arthropathy**: Haemochromatosis * Yellow-brown **skin pigmentation**, eye movement disorders, myoclonus: Gaucher's disease
195
What causes **Wilson's disease**?
* Mutation in the ATP7B gene (involved in copper secretion) * Autosomal recessive
196
Which antibodies are associated with **autoimmune hepatitis**?
* ANA * ASMA * Anti-LKM1
197
What criteria is used to determine the need for liver transplantation in **paracetamol overdose**?
* King's college criteria * Based on INR, creatinine, pH and encephalopathy
198
What are key differences between **Crohn's disease** and **ulcerative colitis**?
* **Crohn's disease** affects any part of the gastrointestinal tract and can have skip lesions. * Causes transmural **inflammation**. * More associated with **fistulae development**. * Histology shows granulomas, while **ulcerative colitis** shows crypt abscesses.
199
What are some causes of **renal enlargement**?
* ADPKD * Hydronephrosis * Renal tumours * Amyloidosis
200
Which **opportunistic infections** can occur in patients being immunosuppressed for a renal transplant?
* CMV * BK Virus * JC Virus (PML) * PCP
201
What are the main complications of **hereditary haemorrhagic telangiectasia**?
* Recurrent epistaxis * Gastrointestinal haemorrhage * Anaemia * Intracranial bleeds
202
What are the possible clinical features of **Peutz-Jeghers syndrome**?
* Mucocutaneous pigmentation * Anaemia * Abdominal scars from bowel resection ## Footnote PJS is autosomal dominant.
203
List some secondary causes of **Raynaud's phenomenon**.
* SLE * Systemic sclerosis * Rheumatoid arthritis * Dermatomyositis * Mixed connective tissue disease * Beta blockers * Atherosclerosis
204
How is **Raynaud's phenomenon** managed?
* Keep body warm * Nifedipine
205
Which features are suggestive of a **secondary cause** of Raynaud's phenomenon?
* Digital ulcers, gangrene or severe ischaemia * Onset > 30 years * Episodes are intense, painful or asymmetrical * Clinical features of connective tissue disorders (e.g. sclerodactyly) * Abnormal nail fold capillaries
206
What are the different groups of **driving licences**?
* Group 1: Car * Group 2: Bus or Lorry
207
What driving rules should be given to people after having an **MI**?
**For Car or Motorbike** * 1 week if successful angioplasty * 4 weeks if unsuccessful angioplasty * 4 weeks if MI but no angioplasty * NO need to inform DVLA **For Bus or Lorry** * Tell DVLA and don't drive for 6 weeks * If passing exercise tolerance test at this point, can be cleared to drive again
208
What rules about driving are applied to people who have **seizures**?
**Group 1 - stop driving for 1 year UNLESS:** * First fit (no driving for 6 months) * All seizures in the last 3 years are nocturnal * Fit was provoked **Group 2 - stop driving for 10 years** * Can only restart if seizure-free for 10 years and not on antiepileptics during this time
209
What rules apply to patients with **diabetes** who want to drive?
**Group 1** * Generally fine provided that you don't have severe hypos, frequent hypos, poor vision or hypoglycaemia unawareness * Inform DVLA if on insulin **Group 2** * Inform DVLA * Can drive if 3 month history of satisfactory glucometer readings
210
How long can you stop driving for after a **stroke/TIA**?
* **Group 1**: 1 Month (No DVLA) * **Group 2**: 1 Year (Tell DVLA) ## Footnote Ongoing ability to drive is dependent on the effect of the CVA.
211
Outline the criteria for **brainstem death**.
* All brainstem reflexes are absent (e.g. fixed and non-responsive pupils, absent corneal reflex, no response to supraorbital pressure) * Done by 2 medical practitioners registered for at least 5 years (at least one of them being a consultant) * 2 sets of tests to be performed
212
What are the four stages of **diabetic retinopathy**?
* **Stage 1**: Background diabetic retinopathy (microaneurysms, hard exudates) * **Stage 2**: Pre-proliferative (multiple microaneurysms, soft exudates) * **Stage 3**: Proliferative (new vessel formation) * **Stage 4**: Maculopathy (hard exudates in macula) ## Footnote Also think of photocoagulation burns.
213
How is **type 2 diabetes mellitus** managed?
* **STEP 1**: Metformin * **STEP 2**: Metformin + Sulfonylurea OR DPP4 Inhibitor OR Pioglitazone OR SGLT2 Inhibitor * **STEP 3**: Triple Therapy * **STEP 4**: Metformin + Sulfonylurea + GLP1 Agonist (BMI > 35) ## Footnote Insulin therapy can be considered at any stage.
214
What is usually assessed at a routine diabetes review?
* HbA1c * Feet (ulcers, sensation) * Eyes (fundoscopy) * Blood Pressure * Cholesterol * Renal Function
215
Which conditions cause **loss of peripheral vision**?
* Retinitis pigmentosa * Glaucoma * Previous CVA (causing hemianopia)
216
What are the differences in the causes of monocular vs binocular diplopia?
* **Monocular**: Cataract or corneal pathology * **Binocular**: Imbalance in extra-ocular muscles (e.g. nerve palsy, thyroid eye disease, myasthenia gravis, ocular myopathy) ## Footnote NOTE: Myasthenia and ocular myopathies can cause variable diplopia.
217
What is the **cover test**?
In a patient with strabismus, cover each eye in turn and ask the patient to focus on you. The 'bad' eye is the one that will have to adjust to focus on you when the 'good' eye is covered.
218
Why is **pupil-involving third nerve palsy** a cause for concern?
It is suggestive of a mass lesion (e.g. aneurysm) compressing the optic nerve including its parasympathetic fibres which lie on the outside of the nerve.
219
Which structures pass through the **cavernous sinus**?
**THROUGH** * CN VI * Carotid plexus (post-ganglionic sympathetic fibres) * Internal carotid artery **THROUGH WALL** * CN III * CN IV * CN V (V1 and V2)
220
What is **Holmes-Adie syndrome**?
* At least 1 abnormally dilated pupil with no light response * Loss of deep tendon reflexes * Abnormalities of sweating
221
What is **optic atrophy**?
Pale optic disc due to death of retinal ganglion cell axons of the optic nerve.
222
What are the most common causes of **optic atrophy**?
* Optic neuritis (MS, NMO) * Glaucoma * Ischaemic optic neuropathy * Drugs (e.g. ethambutol) * Vitamin B12 deficiency
223
What are some causes of **Horner's syndrome**?
* CNS lesion (stroke, tumour) * Idiopathic * Pancoast lung tumour * Carotid artery dissection/aneurysm * Iatrogenic (e.g. post-surgical)
224
What are the main clinical features of **retinitis pigmentosa**?
* Night blindness * Loss of peripheral vision ## Footnote NOTE: Variable inheritance and penetrance (can be AD, AR or X-linked). No cure.
225
What should you examine in a patient with **acromegaly**?
* Hands (large and doughy) * Teeth (spaced) * Visual fields * Heart (LVH)
226
Which investigations should be requested for a patient with suspected **acromegaly**?
* IGF1 (raised) * Glucose tolerance test * HbA1c * MRI Brain
227
How is **acromegaly** managed?
* **FIRST LINE**: Trans-sphenoidal Hypophysectomy * **Adjuncts**: Radiotherapy * **Medical**: Dopamine agonists (e.g. bromocriptine), somatostatin analogues (e.g. octreotide) ## Footnote NOTE: Post-operatively, patients may require hormone replacement (e.g. thyroxine, hydrocortisone, sex steroids).
228
Aside from the neck, what else should be examined in a patient with suspected **Graves' disease**?
* Pulse (AF) * Eye movements (lid lag, exophthalmos) * Pretibial myxoedema
229
How is Graves' disease managed?
* Radioiodine * Surgery * Medical (thionamides such as carbimazole and propylthiouracil)
230
What guidance is offered to people receiving radioiodine treatment for Graves' disease?
Strict rules in 1st week regarding close physical contact with others (esp. pregnant women and children). ## Footnote Do not get pregnant for 6 months after treatment.
231
What are some of the complications of treatment of Graves' disease?
* **Medical** --> agranulocytosis, rash * **Surgical** --> recurrent laryngeal nerve palsy, hypoparathyroidism, thyroxine replacement * **Radioiodine** --> follow radioiodine rules
232
Which investigations should be requested in a patient with suspected **rheumatoid arthritis**?
* ESR/CRP * Rheumatoid factor * Anti-CCP antibodies * X-Rays
233
How is rheumatoid arthritis managed?
* **Analgesia**: paracetamol, NSAIDs * **DMARDs**: methotrexate, sulfasalazine, hydroxychloroquine * **Biologics**: infliximab, etanercept (usually after 2 DMARDs have been unsuccessful) ## Footnote Exercise and physio.
234
What are the main side-effects of **methotrexate**?
* Immunosuppression * Hepatotoxicity * Pneumonitis * Pulmonary fibrosis * Teratogenic
235
What are the five types of **psoriatic arthritis**?
* Symmetrical polyarthritis * Asymmetrical oligoarthritis * Distal interphalangeal joint involvement * Arthritis mutilans * Spondyloarthropathy
236
What are some specific features of **psoriasis**?
* Plaques (extensor surfaces, flexural or guttate) * Nail changes (onycholysis, hyperkeratosis) * Koebner phenomenon
237
How is psoriasis managed?
* **TOPICAL**: coal tar, calcipotrol, dithranol * **SYSTEMIC**: methotrexate, ciclosporin, anti-TNF, PUVA ## Footnote NOTE: for psoriatic arthritis, the mainstay of treatment is methotrexate and anti-TNFs
238
What's **Jaccoud's arthropathy**?
Chronic non-erosive reversible joint disorder most commonly associated with SLE.
239
Which investigations should be requested in a patient with suspected **SLE**?
* Urinalysis * U&E * ANA * dsDNA * ENAs (e.g. anti-Smith, anti-Ro) * ESR * CRP * Complement
240
What are the main **treatment options** for SLE?
* **Mild**: Hydroxychloroquine * **Moderate**: Azathioprine, MMF, Prednisolone * **Severe**: Cyclophosphamide, Rituximab
241
What are the main differences between limited and diffuse systemic sclerosis?
* **LIMITED**: Anti-centromere. Limited to below elbows and below knees. * **DIFFUSE**: Anti-Scl70 + Anti-RNA polymerase II antibody * Affects the entire body.
242
List some clinical features of **systemic sclerosis**.
* Sclerodactyly * Digital ulcers * Calcinosis * Microstomia * Interstitial lung disease (basal) * Dysphagia * Loud P2
243
Which investigations should be requested in suspected **scleroderma**?
* **BLOODS**: ANA, anti-centromere, anti-Scl70 * **RESPIRATORY**: CXR, HRCT, lung function * **CARDIAC**: ECG, echo * **GI**: barium swallow
244
How is **systemic sclerosis** managed?
* **RENAL CRISIS**: aggressive BP control (ACEi) * **RAYNAUD'S**: cold avoidance, CCB, prostaglandin analogues * **SKIN THICKENING**: cyclophosphamide, methotrexate * **OESOPHAGEAL**: prokinetics * **ILD**: steroids, pulmonary rehab, lung transplant
245
How is **gout** managed?
* **Acute**: NSAIDs, colchicine * **Chronic**: allopurinol, rasburicase
246
Which investigations should be requested in suspected **ankylosing spondylitis**?
* CRP, ESR and HLA B27 * XR Sacroiliac Joints and Spine * CXR/HRCT * ECG and Echocardiogram
247
How is ankylosing spondylitis managed?
* Physiotherapy * NSAIDs * Biologics ## Footnote NOTE: Bath Ankylosing Spondylitis Disease Activity Index is used to guide treatment. >4 means active disease.
248
What are some of the features of **Paget's disease**?
* Bone pain * High output cardiac failure * Deafness
249
How is Paget's disease managed?
* Analgesia * Bisphosphonates
250
What is **neuromyelitis optica**?
Spectrum of autoimmune diseases characterised by acute inflammation of the optic nerve and the spinal cord (transverse myelitis). Has a relapsing-remitting course that mimics MS. ## Footnote Associated with aquaporin 4 antibodies.
251
What **Maddrey's Score** is suggestive of severe alcoholic hepatitis?
**32 or more**: associated with high mortality and may benefit from steroids
MRCP
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