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MRCP PACES Flashcards

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1
Q

What are you looking for from the end of the bed when performing an abdominal examination?

A

Jaundice
Tense ascites
Caput medusae
Tattoos
Nutritional status
Scars (surgery, peritoneal dialysis, central venous line, tunnelled line)
Spider naevi (5+)
Medications around bedside

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2
Q

What signs might you see in the hands when performing an abdominal examination?

A

Thin skin (steroid use)
Bruising (steroid use, coagulopathy)
Dupuytren’s contracture
Palmar erythema
Leukonychia (hypoalbuminaemia)
Koilonychia (severe IDA)

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3
Q

What might a fine tremor be suggestive of during an abdominal examination?

A

Alcohol withdrawal
Tacrolimus toxicity (if evidence of liver transplant such as Mercedez-Benz scar)

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4
Q

What signs might you see in the face during an abdominal examination?

A

Parotid swelling
Jaundice
Angular cheillits/stomatitis
Conjunctival pallor

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5
Q

What signs might you see in the mouth during an abdominal examination?

A

Ulceration (associated Crohn’s disease)
Dentition
Glossitis (iron and B12 deficiency)
Candidiasis

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6
Q

What is enlargement of Virchow’s node suggestive of?

A

Gastric malignancy (also called Trosier’s sign)

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7
Q

If you see that patient has a fistula on abdominal examination, what should you check for?

A

Is there a thrill? (i.e. is it functional)
Has it recently been needled? (i.e. they are currently on haemodialysis)
Check whether there are any renal transplant scars

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8
Q

What are some GI causes of pedal oedema?

A

Hypoalbuminaemia (liver impairment or nephrotic syndrome)

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9
Q

List some inherited cystic kidney conditions.

A

PKD
Von-Hippel Lindau syndrome
Tuberous sclerosis

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10
Q

What system is used to classify renal cysts in polycystic kidney disease based on contrast-enhanced CT findings?

A

Bosniak System
Bosniak 1: Simple Cyst
Bosniak 4: Malignant

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11
Q

What is the prevalence of ADPKD?

A

1 in 1000
Accounts for 10% of renal replacement patients in the UKaht

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12
Q

How common are simple renal cysts?

A

2% of those aged 50 years
20% of elderly people

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13
Q

What might you see on general inspection in a respiratory examination?

A

Scars
Chest wall asymmetry
Cyanosis
Increased work of breathing
Audible wheeze
Inhalers
Portable oxygen

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14
Q

What are you looking for in the hands of a patient whilst performing a respiratory examination?

A

Tar staining
Peripheral cyanosis
Rheumatoid arthritis (ILD)
Thickening of skin (scleroderma)

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15
Q

What are the respiratory causes of clubbing?

A

Suppurative lung disease (cystic fibrosis and bronchiectasis)
Lung cancer
Interstitial lung disease (IPF)

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16
Q

What might a fine tremor be suggestive of in a respiratory examination?

A

Salbutamol overuse
Tacrolimus toxicity (lung transplant)
CO2 retentionW

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17
Q

Why is an elevated JVP significant in a respiratory examination?

A

May be because of cor pulmonale

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18
Q

What is the hepatojugular reflex suggestive of?

A

Right sided heart failure

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19
Q

What might abnormal chest expansion be suggestive of?

A

Symmetrically Decreased: Stiff lungs (interstitial lung disease)
Hyperinflated Lungs: COPD, severe asthma

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20
Q

What do you look for on inspection in a cardiovascular examination?

A

Oxygen tank
Metallic clicks
Vein harvesting scars
Midline sternotomy scar
Lateral thoracotomy scars
Previous chest drain insertion scars

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21
Q

What might you see in the hands on a cardiovascular examination?

A

Warmth
Tendon xanthomata
Peripheral cyanosis
Clubbing
Ecchymosis (warfarin)
Palmar erythema
Janeway lesions
Osler’s nodes (painful)

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22
Q

What are you looking for in the face when performing a cardiovascular examination?

A

Malar flush (mitral stenosis)
Xanthelasma
Corneal arcus
Conjunctival pallor or haemorrhage

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23
Q

What signs are you looking for in the mouth during a cardiovascular examination?

A

Central cyanosis
High arched palate
Problems with dentition

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24
Q

What might cause a regularly irregular pulse?

A

Bi- and trigeminy

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25
What might a collapsing pulse be suggestive of?
Aortic regurgitation Patent ductus arteriosus Hyperdynamic circulation
26
How can the position of the apex beat be interpreted?
Displaced or thrusting: Mitral regurgitation Undisplaced or heaving: Aortic stenosis, LVH Tapping: Mitral stenosis
27
What are you looking for upon inspection of the lower limbs in a neurological examination?
Wasting Fasciculations
28
What is the difference of rigidity vs spasticity?
There is increased tone regardless of speed in rigidity, whereas the degree of increased tone increases with speed in spasticity
29
What are the four different types of eye movements?
Fast Saccades: Moving rapidly between two fixed points that are a distant apart Smooth Pursuit: Following a slowly moving object with smooth movements Vergence: Focusing on an object close to the face, Vestibulo-ocular: Being able to adjust the eyes to maintain focus on a fixed object in the context of involuntary head movement
30
What is the accommodation response?
Pupils constrict and eyes converge when suddenly focusing on a nearby object
31
What is athetosis suggestive of?
Damage to the basal ganglia
32
What is pseudoathetosis?
Writhing movements caused by a proprioceptive defect rather than due to damage to the basal ganglia
33
What should a cranial nerve palsy in the context of polycystic kidney disease make you suspicious of?
Intracranial aneurysm
34
What are the two mutations that can cause ADPKD?
PKD1 on Chromosome 16 (85%) PKD2 on Chromosome 4
35
What are some differences between PKD1 and PKD2?
PKD1 is more severe and tend to lead to end stage kidney disease at a younger age. It is also MORE common.
36
How does polycystic kidney disease present?
Abdominal pain Hypertension Blood test abnormalities Urinalysis abnormalities Haematuria UTI Intracranial aneurysms Genetic testing for family members
37
What are the main presenting features of von hippel lindau syndrome?
Headache Ataxia Dizziness Weakness Loss of vision Hypertension
38
What are the main associations of Von Hippel Lindau syndrome?
Renal cell carcinoma Phaeochromocytoma Spinocerebellar haemangioblastomas Retinal angiomas
39
What are the main features of tuberous sclerosis?
Epilepsy Learning disability Autism Hamartomas Renal cysts Angiomyolipomas (renal) Shagreen patches Ash-leaf spots Adenoma sebaceum
40
What are the main features of Alport syndrome?
Deafness (bilateral and sensorineural) Persistent microscopic haematuria Proteinuria and CKD Ocular abnormalities (e.g. lenticonus) Leiomyomas
41
How is ADPKD diagnosed?
Imaging (Ultrasound, CT, MRI) Genetic testing Ravine/Pei diagnostic criteria
42
How is ADPKD managed?
CONSERVATIVE - Low salt diet MEDICAL - Antihypertensives (RAAS blockade) - Tolvaptan limits cyst development SURGICAL - Remote problematic cysts and reduce mass effects - Renal replacement therapy
43
What proportion of patients with ADPKD have extra-renal manifestations?
70% Liver Cysts 10% Pancreatic Cysts 5% Berry Aneurysms Mitral valve prolapse
44
How does PBC tend to present?
Fatigue Pruritus Obstructive jaundice
45
What are some clinical features of PBC?
Hepatosplenomegaly Clubbing Xanthelasma Excoriation marks (from pruritus) Jaundice
46
What are the main causes of chronic liver disease?
Alcoholic liver disease Non-alcoholic fatty liver disease Viral hepatitis Autoimmune hepatitis Drug-induced liver disease Infiltrative disease (e.g. Wilson's disease, haemochromatosis)
47
Which antibody is associated with PBC?
Antimitochondrial (M2) antibody Also associated with raised ALP and IgM
48
How is PBC managed?
MEDICAL - Ursodeoxycholic Acid or Obeticholic Acid - Cholestyramine for pruritus SURGICAL - Liver transplant (for intractable pruritus and end stage disease) - Transplant has a good prognosis and low rate of recurrence
49
What are the main causes of chronic kidney disease?
Diabetes Hypertension Glomerulonephritis Polycystic Kidney Disease Chronic Pyelonephritis Reflux Nephropathy
50
What are the advantages of renal transplant over dialysis?
Better quality of life Increased survival (though generally fitter patients are chosen for transplant) More cost-effective in the long-term
51
When should a patient be worked up for renal transplant?
Ideally as they progress towards end stage renal failure but before starting dialysis
52
Which diseases are particularly at risk of recurrence in renal transplantation?
Focal segmental glomerulosclerosis Amyloidosis IgA Nephropathy Haemolytic uraemic syndrome
53
What are some contraindications for renal transplantation?
Current malignancy Severe poorly controlled comorbidity (e.g. COPD, heart failure) Active infection (e.g. viral hepatitis) Active substance misuse Uncontrolled psychiatric disease History of medication non-compliance
54
What is hyperacute rejection in the context of a renal transplant?
Due to presence of antibodies against donor kidney. Kidney swells and becomes dusky within minutes of revascularisation Transplant nephrectomy required Very rare
55
What is chronic transplant rejection?
Gradual decrease in kidney function that becomes apparent 3 months after transplant
56
What specific work up needs to be done ahead of renal transplantation?
ABO and HLA typing Virology (hep B, hep C and CMV) Urinalysis and culture Cardiovascular assessment
57
What are some of the effects of steroid use for immunosuppression?
Thin skin Easy bruising Cushingoid appearance Diabetes mellitus
58
What are some physical features of immunosuppressive therapy
Ciclosporin: Gingival hyperplasia Tacrolimus: Tremor Steroids: Cushingoid, easy bruising
59
What are some complications of immunosuppression?
New-onset diabetes after transplantation Cancer (especially squamous cell carcinoma and non-Hodgkin lymphoma) Opportunistic infections (BK, CMV)
60
What are some prognostic factors for renal transplants?
Primary diagnosis Previous episodes of rejection Total ischaemic time Donor factors (e.g. age)
61
Define end stage renal failure.
eGFR < 15 mL/min
62
What are the features of decompensation of chronic liver disease?
Jaundice Asterixis Ascites
63
What are some causes of hepatomegaly?
Cirrhosis Congestive cardiac failure Carcinoma Viral hepatitis Immune (PBC, PSC, autoimmune hepatitis) Infiltrative (haemochromatosis, amyloidosis, Wilson's disease)
64
What are the main investigations you would request in a patient with hepatomegaly?
FBC U&E LFT Clotting Glucose Ferritin Viral hepatitis screen Caeruloplasmin Autoantibodies ULTRASOUND ASCITIC TAP FIBROSCAN (extent of liver fibrosis)
65
What are the best markers of liver synthetic function?
Albumin Clotting (PT)
66
How can AST: ALT ratio be interepreted?
> 2 is suggestive of alcoholic liver disease Very high in ischaemic hepatitis
67
What are the different parameters that can be analysed on an ascitic tap and why would you request them?
Cell count (SBP) Gram stain Amylase/lipase (in pancreatitis) Cytology (cancer) SAAG (11 g/L) Glucose (low in infection)
68
What should all patients with cirrhosis have done?
Endoscopy to check for varices
69
Which tests are useful in the diagnosis of chronic pancreatitis?
Faecal elastase Albumin Vitamin D Magnesium
70
How is chronic pancreatitis treated?
Creon Pain management PPI
71
What are some causes of increased SAAG?
Cirrhosis Alcoholic hepatitis Budd-Chiari syndrome Heart failure
72
What are some causes of decreased SAAG?
Nephrotic syndrome Pancreatitis Malignancy Peritonitis
73
How is ascites managed?
Treat cause Drain Diuretics (often spironolactone then furosemide) Prophylactic antibiotics
74
What are some differentials for abdominal distension?
Fluid (ascites) Flatus Foetus (pregnancy) Obesity Tumour
75
What are some reasons for a nephrectomy in patients with ADPKD?
Debulking to provide room for transplant Renal cell carcinoma Recurrent urinary tract infections Chronic pain Excessive bleeding
76
How does autosomal recessive polycystic kidney disease differ from ADPKD?
It causes end stage renal impairment in childhood (much less common)
77
Why is peritoneal dialysis avoided in patients with ADPKD?
Mechanical difficulty performing peritoneal dialysis if abdomen contains bulky kidneys Increased risk of cyst infection
78
What would you request to complete the examination after performing an abdominal examination?
Observations Examine external hernial orifices and external genitalia Perform a DRE
79
What are the two forms of peritoneal dialysis?
Continuous ambulatory peritoneal dialysis Automated peritoneal dialysisW
80
When is dialysis recommended by NICE?
eGFR < 5-7 mL/min if asymptomatic Impacted by symptoms of uraemia on a daily basis
81
What are some symptoms that patients on dialysis may experience?
Breathlessness Fatigue Pruritus Loss of appetite Abdominal cramps Depression
82
What are the acute indication for dialysis?
Acidosis Pulmonary oedema Hyperkalaemia Uraemic complications
83
What are the most common immunosuppression regimens used in renal transplants?
Calcineurin inhibitor (e.g. tacrolimus) Antimetabolite (e.g. MMF) Steroid
84
After how long does continuous ambulatory peritoneal dialysis stop working?
3-6 years
85
How long does a fistula take to mature?
4-6 weeks N.B. you want to achieve a flow rate of 250-400 mL/min into the dialyser
86
What are some complications of peritoneal dialysis?
Bacterial peritonitis Ultrafiltration failure Encapsulating peritoneal sclerosis Hernia Fluid leak
87
What are the main complications of haemodialysis?
Hypotension Infection (e.g. line-related) Venous stenosis Air embolus Cardiovascular disease Dialysis-related amyloidosis Pseudogout
88
What are the main features of graft rejection after a renal transplant?
Fluid retention High blood pressure Rapidly rising creatinine
89
How does CMV infection manifest in transplant patients?
Myocarditis Encephalitis Retinitis Renal dysfunction Pneumonitis NOTE: treated with ganciclovir
90
What are some factors that favour the use of haemodialysis rather than peritoneal dialysis?
Previous peritonitis Severe malnutrition Catabolic states (e.g. intercurrent illness) Chronic severe respiratory disease (may compromise respiratory function)
91
What are the causes of splenomegaly?
INFILTRATION - Myelo- and lymphoproliferative disorders - Lymphoma - Amyloidosis -Sarcoidosis - Gaucher's disease - Lipid storage disease INCREASED FUNCTION - Spherocytosis - Thalassemia - Early sickle cell ABNORMAL FLOW - Cirrhosis - Hepatic or portal vein obstruction IMMUNE HYPERPLASIA - Tropical: chronic malaria, visceral leishmaniasis, brucellosis - Glandular fever - Infectious hepatitis DISORDERED IMMUNOREGULATION - Felty's syndrome - SLE
92
What other features should you look for on examination in a patient with splenomegaly?
Hepatomegaly and ascites: cirrhosis Jaundice: haemolytic anaemia, liver impairment Anaemia Lymphadenopathy: lymphoma, infection (e.g. EBV)
93
What are the causes of massive splenomegly?
Chronic myeloid leukaemia Myelofibrosis Gaucher's disease Chronic malaria Kala azar
94
What investigations should you request in a patient with splenomegaly?
FBC and film If suspicion of haematological malignancy: CT CAP, bone marrow aspirate/trephine, lymph node biopsy If suspicion of malaria: thick and thin films
95
What is hereditary spherocytosis and how is it diagnosed?
Autosomal dominant defect in the cell wall of red cells Diagnosis: EMA binding test (or osmotic fragility)
96
What would you see on the blood film of a patient with hereditary spherocytosis?
Small round red cells that have lost their central pallor Increased reticulocytes
97
Which causes of splenomegaly are more likely in YOUNG people?
- Spherocytosis - Thalassemia - Early sickle cell - Tropical: chronic malaria, visceral leishmaniasis, brucellosis - Glandular fever - Lymphoma
98
Why is it important to look inside the top lip of a patient when doing an abdominal examination?
To check for gingival hyperplasia (ciclosporin)
99
Which scars are associated with liver transplant?
Mercedez-Benz (bilateral rooftop with sternal extension) Makuuchi (inverted J)
100
What signs would suggest that a liver transplant is not working properly?
Ascites Asterixis Jaundice
101
Which features of chronic liver disease are likely to persist post-transplantation?
Gynaecomastia Dupuytren's contracture Splenomegaly (may change)
102
Is the size of a transplanted liver significant?
Difficult to interpret because it may be suggestive of a size mismatch between the donor and the recipient
103
What are some physical signs of post-transplant immunosuppression?
Scars from excision of SCCs Tremor (tacrolimus) Gingival hypertrophy (ciclosporin) Steroid (Cushingoid, diabetes mellitus cap glucose testing and insulin injections, easy bruising)
104
What clinical features may be suggestive of an underlying diagnosis of haemochromatosis?
Venesection scars Tanned appearance Hepatosplenomegaly
105
What are the main indications for liver transplantations in the UK?
Cirrhosis Hepatocellular carcinoma Acute fulminant liver failure (e.g. paracetamol overdose)
106
Define acute liver failure.
Multisystem disorder characterised by severe impairment of liver function with encephalopathy within 8 weeks of the onset of symptoms and no recognised underlying chronic liver disease.
107
Which variant syndromes are considered for liver transplantation?
Diuretic-resistant ascites Chronic hepatic encephalopathy Intractable pruritus Hepatopulmonary syndrome Polycystic liver disease Recurrent cholangitis
108
What are some contraindications for liver transplantation?
IV drug use Ongoing alcohol excess Significant medical or psychiatric comorbidities This is all discussed by a specialist MDT
109
Which scoring criteria is used to assess patient's need for liver transplantation?
UKELD (based on INR, creatinine, bilirubin and sodium) A score of 49 or more is considered for elective liver transplantation
110
Which scoring system is used to triage acute alcoholic hepatitis?
Maddrey's Score Glasgow Alcoholic Hepatitis Score
111
What are the main causes of ascites?
VASCULAR Portal hypertension in liver disease Budd-Chiari syndrome Congestive cardiac failure Constrictive pericarditis LOW ALBUMIN Cirrhosis Nephrotic syndrome Protein-losing enteropathy PERITONEAL Meig syndrome TB Malignancy (e.g. ovarian) MISC Pancreatitis Hyperthyroidism (advanced)
112
Which investigations should you request in a patient with ascites?
BLOODS: FBC, Clotting, LFT, viral serology, autoantibody screen, ferritin, caeruloplasmin, AFP IMAGING: Ultrasound, CXR (congestive cardiac failure) OTHER: Ascitic Tap
113
What are some indications for a transjugular liver biopsy?
Deranged coagulation Massive ascites
114
What causes hereditary haemochromatosis?
Autosomal recessive condition with variable penetrance Caused by mutation in the HFE gene (increases intestinal iron absorption)
115
How does hereditary haemochromatosis present?
Screening relatives Lethargy Arthralgia Skin pigmentation Hepatomegaly Chronic liver disease Diabetes mellitus Erectile dysfunction
116
How do men and women differ in the presentation of haemochromatosis?
Women tend to present later in life due to the protective effect of menstruationW
117
Which investigations are most important in the diagnosis of hereditary haemochromatosis?
Transferrin saturation (raised) Ferritin (raised) HFE genotyping Liver biopsy (not essential but can help assess stage of disease) Urinalysis and HbA1c for diabetes mellitus ECG for AF AFP for HCC Liver ultrasound for cirrhosis
118
How is hereditary haemochromatosis managed?
Venesection (3-4 times per year) Alternative: iron chelation Avoid food containing iron or vitamin C Avoid alcohol Transplant (if cirrhotic)
119
How should patients with hereditary haemochromatosis be monitored?
Regular FBC, transferrin saturation and ferritin monitoring Monitor HbA1c If cirrhotic, regular ultrasound and AFP monitoring
120
What is the prognosis in hereditary haemochromatosis?
If non-diabetic and non-cirrhotic, venesection returns life expectancy to normal
121
What are the main complications of hereditary haemochromatosis?
Cirrhosis (and HCC) Diabetes mellitus Cardiomyopathy
122
What are the main aspects of managing hepatic encephalopathy?
Lactulose (aiming for at least 2 bowel movements per day) Rifxamin NOTE: and identify and treat any precipitants (e.g. bleed)
123
How should an upper GI bleed be managed?
A to E assessment and appropriate resuscitation with IV fluids and/or blood products Prophylactic antibiotics (e.g. ceftriaxone) Terlipressin Calculate Glasgow-Blatchford score and discuss with on call endoscopist EMERGENCY: Sengstaken-Blakemore tube
124
How does spherocytosis present?
Anaemia Jaundice (including neonatal) Splenomegaly Screening of first degree relatives
125
What are some of the complication of hereditary spherocytosis?
Aplastic crisis Severe anaemia Gallstones
126
What mutation causes hereditary spherocytosis?
5 possible mutations with the most common (ANK1) being on chromosome 8
127
What is the outcome of splenectomy in moderate-to-severe cases of hereditary spherocytosis?
Virtually eliminates haemolysis
128
How can intercurrent infections affects people with hereditary spherocytosis?
Increase rate of haemolysis e.g. EBV can increase spleen size and worsen haemolysis
129
What is the main complication of hereditary spherocytosis?
Pigment gallstones
130
What are the relevant examinations to perform in a patient with suspected ankylosing spondylitis?
Listen for aortic regurgitation Listen for pulmonary fibrosis Assess chest expansion Assess Achilles' tendons Assess the axial spine
131
What are the motor functions of the ulnar nerve?
ANTERIOR FOREARM - Flexor carpi ulnaris (flex and adduct hand) - Flexor digitorum profundus medial half (flexed ring and little finger at DIP) HAND - Most intrinsic hand muscles (hypothenar muscles, medial lumbricals, adductor policis, palmar and dorsal interossei)
132
What is Froment's sign?
Test for ULNAR nerve palsy Flexion of thumb at interphalangeal joint when pulling paper away
133
What are the sensory components of the ulnar nerve?
Medial half of palm and dorsum of hand Medial 1 and a half fingers NOTE: there are 3 branches - palmar, superficial and dorsal
134
What are the main motor features of ulnar nerve damage at the elbow?
Flexion of wrist accompanied by abduction Weakness of finger abduction and adduction (due to interossei) Weakness of 4th and 5th digits (due to medial 2 lumbricals and hypothenar muscles) Weakness of thumb adduction Positive Froment sign
135
What are the main sensory features of ulnar nerve damage?
Loss of sensation of medial half of palm and dorsum of hand Loss of sensation over medial 1 and a half digits
136
What are the main motor features of ulnar nerve damage at the wrist?
Weakness of finger abduction and adduction Weakness of 4th and 5th digits (due to medial lumbricals and hypothenar muscles) Weakness of thumb adduction Froment's sign positive Wasting of hypothenar eminence
137
How does the sensory deficit in ulnar nerve palsy at the wrist level differ from the elbow?
Dorsal branch is usually unaffected in ulnar nerve palsy at the wristw
138
What are the main differences between ulnar nerve damage at the wrist and at the elbow?
Normal sensation over dorsal area More severe clawing (due to preserved innervation of flexor digitorum profundus) Normal wrist flexion without abduction
139
What is the ulnar paradox?
A lesion at the elbow produces a milder deformity than a lesion at the wrist This is because a lesion at the elbow also paralyses flexor digitorum profundus (this causes weakness of finger flexion to balance the weakness of finger extension caused by paralysis of the lumbricals)
140
What are the causes of ulnar nerve injury at the wrist and elbow?
Wrist: Lacerations to the anterior wrist Elbow: Trauma (e.g. supracondylar fracture), surgery
141
Which muscles are innervated by C8/T1 via the MEDIAN nerve?
Lateral 2 lumbricals Opponens policis Abductor policis brevis Flexor policis brevis
142
Which investigation could you request in a patient with a suspected ulnar nerve palsy?
EMG MRI of the neck should be considered if concerns about cervical radiculopathy
143
How is neuropathic pain managed?
Tricyclic antidepressant (e.g. amitriptyline) Then move on to pregabalin and gabapentin Alternative: SNRI (duloxetine) Non-Tablet: Capsaicin cream
144
What are the main causes of peripheral neuropathy?
Diabetes mellitus METABOLIC: uraemia, hyperthyroidism, vitamin B1/B6/B12 deficiencies TOXIC: chemotherapy (e.g. vincristin, cisplatin), antibiotics (e.g. isoniazid), alcohol excess INFLAMMATORY: CIDP, sarcoidosis, ANCA-positive vasculitis, rheumatoid arthritis MALIGNANT: paraneoplastic syndromes (e.g. lung cancer, myeloma)
145
Which tests should be done in a patient with peripheral neuropathy?
BEDSIDE: urinalysis, BM, fundoscopy (diabetic retinopathy) BLOODS: HbA1c, U&E, FBC (macrocytosis), B12, LFT (alcohol), TFT, ESR (chronic inflammation), serum electrophoresis Other: Nerve conduction studies
146
Why are nerve conduction studies useful in peripheral neuropathy?
It can distinguish between demyelinating or axonal It can tell whether the weakness is length-dependnent NOTE: demyelinating length-dependent neuropathies are much more likely to be immune-mediated (e.g. CIDP)
147
What are some aspects of managing peripheral neuropathy?
Treat cause (e.g. tight glycaemic control) Podiatry for foot care Physiotherapy (if gait issues)
148
Which investigations should be requested in suspected Charcot-Marie-Tooth disease?
Nerve conduction studies (severe and uniformly reduced responses) Genetic testing (autosomal dominant) NOTE: Nerve conduction studies will also tell you whether it is axonal or demyelinating in nature which helps identify the type of CMT
149
How is Charcot-Marie-Tooth disease managed?
No disease-modifying treatments Genetic counselling for family members OT/PT Orthotics
150
How is a homonymous hemianopia caused by a occipital stroke different from that caused by an MCA stroke?
Occipital stroke has macular sparing
151
Which investigations should be requested to identify potential causes in a patient with a stroke?
Echocardiogram Carotid Doppler Ambulatory ECG Monitoring
152
List some causes of a spastic paraparesis.
Spinal trauma Disc prolapse Multiple sclerosis Motor neurone disease Cerebral palsy Spinal infarct
153
What are the main investigations to request in suspected multiple sclerosis?
MRI (Brain and Spine) CSF (oligoclonal bands)
154
How is multiple sclerosis managed?
Acute Relapse: High dose IV steroids (methylprednisolone) Disease-Modifying Treatments (beta interferons, glatiramer acetate, natalizumab) Manage complications (baclofen for spasticity, psychological support, PT/OT, neuropathic pain medications, laxatives for constipation)
155
What are the different types of multiple sclerosis?
Relapsing-remitting (90%) Secondary progressive Primary progressive NOTE: 25% of RRMS becomes SPMS within 6 years
156
What are some features that you would see in cerebellar ataxia that you would not see in sensory ataxia?
Nystagmus Dysarthria
157
What are the main features of a sensory ataxia?
Impaired joint position and vibration sense Pseudoathetosis Finger nose test is ok with eyes open but struggle with eyes closed
158
What are the main causes of sensory ataxia?
CENTRAL: Dorsal column damage PERIPHERAL: diabetes mellitus, alcohol excess, uraemia, vitamin B1/6/12 deficiency
159
What is the most common cause of both central and peripheral sensory ataxia?
B12 deficiency
160
Which other neurodegenerative conditions is often comorbid in the context of ALS?
Frontotemporal dementia
161
What are the four types of motor neuron disease?
Amyotrophic lateral sclerosis Progressive bulbar palsy Progressive muscular atrophy Primary lateral sclerosis
162
What are some differentials for motor neuron disease?
Myopathy Stroke Guillain-Barre syndrome Myasthenia gravis Spinal cord tumours
163
Which investigations are useful in suspected motor neuron disease?
EMG (look for fasciculations and fibrillations) Nerve conduction studies
164
How is motor neuron disease managed?
Riluzole (small prognostic benefit) OT/PT SALT Dietician (nutritional plans) Addressing ventilatory issues (e.g. NIV)
165
What is Kennedy disease?
X-linked spinobulbar muscular atrophy CAG repeat associated lower motor neuron disease that presents with progressive weakness and wasting of limb and bulbar muscles Associated with gynaecomastia and subfertility
166
What is the prognosis for MND?
Varies depending on type 15-20% 5-year survival
167
What is myelopathy?
Injury to the spinal cord causing neurological symptoms
168
How is a myelopathy investigated?
Urgent MRI (if acute) May need urgent neurosurgical discussion
169
What are some causes of myelopathy?
ACUTE Disc prolapse Infarction Epidural abscess SUBACUTE Infection (e.g. mycoplasma induced transverse myelitis) CHRONIC B12 deficiency Inflammatory (e.g. MS) Spinal cord tumour Hereditary spastic paraparesis
170
What are the main clinical features of Kennedy's disease?
Lower motor neuron only Progressive weakness and wasting of limb and bulbar muscles Slow rate of progression PERIORAL FASCICULATIONS Gynaecomastia Subfertility
171
What are a head tremor and truncal ataxia associated with?
Cerebellar ataxia
172
What are the main causes of cerebellar ataxia?
ACUTE: haemorrhage, stroke, infection (varicella), autoimmune CHRONIC: alcohol excess, nutritional deficiency (vitamin E)
173
What are some classical features of polio?
Limb wasting and weakness Limb shortening Areflexia Pes cavus
174
Which conditions are associated with pes cavus?
Charcot-Marie-Tooth disease Polio Friedreich's Ataxia
175
What are the main features of post-polio syndrome?
Progressive weakness in previously affected areas Pain Cramping Fatigue
176
What is ataxia?
Group of conditions that are characterised by disorders of coordination, balance and speech.
177
What simple test can you use to distinguish sensory ataxia from cerebellar ataxia?
Sensory ataxia gets much worse with the eyes closed (e.g. not being able to do finger to nose with eyes closed)
178
Which unusual neurological features should raise suspicion of an immune-mediated polyneuropathy?
Non-length dependent (e.g. primarily proximal weakness) Marked asymmetry in neurological features Prominent sensory ataxia
179
What are the most common acute and chronic immune-mediated neuropathy?
ACUTE: Guillain-Barre syndrome CHRONIC: Chronic Inflammatory Demyelinating Polyneuropathy
180
How is Guillain-Barre syndrome managed?
Monitor FVC as evidence of respiratory compromise would warrant ABG and discussion with ICU (if FVC < 20 mL/kg) IVIG and plasma exchange
181
What is the prognosis of Guillain-Barre syndrome?
Most people make a full recovery though this could take months 5% mortality rate
182
Which investigations should be requested in suspected Guillain-Barre syndrome?
CSF Analysis (high protein) Nerve conduction studies (evidence of demyelination) FVC
183
What are the main clinical features of Guillain-Barre syndrome?
Rapidly evolving ascending pattern of weakness Variable sensory loss Flaccid muscle tone Hyporeflexia Post-infection (most commonly Campylobacter jejuni)
184
List some causes of transverse myelitis.
INFLAMMATION: MS, neuromyelitis optica, sarcoidosis, lupus INFECTION: VZV, Mycoplasma, TB, HTLV1
185
What are the main clinical features of a spastic paraparesis?
Hypertonia Brisk reflexes Upgoing plantars Weakness Reduced sensation
186
How can you test bradykinesia?
Finger snapping and rapid rotation of the wrists
187
In someone with Parkinsonian features, which other examination steps would you take to assess whether Parkinson's plus syndromes are a possibility?
Eye Movement (PSP) Nystagmus (cerebellar signs are associated with MSA) LSBP (autonomic dysfunction in MSA)
188
What are some early non-motor features of Parkinson's disease?
Anosmia REM sleep disorders Constipation
189
What are some other non-motor features of Parkinson's disease?
Depression Insomnia Pain Autonomic disturbance
190
What are the cardinal features of Parkinson's disease?
Tremor Rigidity Bradykinesia Gait instability
191
List some other causes of Parkinsonism other than Idiopathic Parkinson's Disease.
Progressive supranuclear palsy Multiple system atrophy Dementia with Lewy bodies Vascular Parkinsonism Drug-induced Parkinsonism
192
How is idiopathic Parkinson's disease diagnosed?
Largely a clinical diagnosis MRI and SPECT imaging may be considered
193
How is Parkinson's disease managed?
PHARMACOLOGICAL: Dopaminergic therapy (Levodopa, Dopamine Agonists), MAO-B inhibitors (selegiline), COMT inhibitors (entacapone), amantadine SURGICAL: Deep brain stimulation NON-MEDICAL: PT/OT, SALT
194
How do you assess speech?
1. Comprehension (1-, 2- and 3-step commands) 2. Repetition (baby hippopotamus) 3. Naming (e.g. pen) 4. Spontaneous speech (normal articulation, phonological errors, word retrieval difficulty, hesitancy, anomia)
195
Which hemisphere is dominant in language?
Left
196
What are the main features of posterior circulation strokes?
Cranial nerve involvement Disorder of conjugate eye movements Cerebellar signs Homonymous hemianopia Cortical blindness Unilateral or bilateral motor or sensory deficit
197
What are the main features of total anterior circulation strokes?
Hemiplegia (contralateral) Homonymous hemianopia (contralateral) Aphasia or visuospatial disturbance Hemisensory deficit (contralateral)
198
What are the features of a partial anterior circulation stroke?
Two of the following need to be present for a diagnosis of a PACS: Unilateral weakness (and/or sensory deficit) of the face, arm and leg. Homonymous hemianopia. Higher cerebral dysfunction (dysphasia, visuospatial disorder)
199
Which features on neurological examination are associated with raised intracranial pressure?
6th cranial nerve palsy Papilloedema Visual field defect
200
What features of a history would make you think of an immune-mediated cause of a polyneuropathy?
Waxing and waning Abrupt onset Starting in the hands, trunk or face (non-length dependent) Prominent sensory ataxia (worse in the dark/eyes closed) Features of associated conditions (inflammatory arthropathy, sicca syndrome)
201
Which additional things should you do when examining a patient with a suspected movement disorder?
Assess for tremor in different positions (e.g. hands in front of face) Finger snapping (bradykinesia) Eye movement testing
202
What are the main causes of chorea?
ACUTE: Stroke (asymmetric), hypoglycaemic CHRONIC: Sydenham's chorea (rheumatic fever), SLE, Huntington's disease
203
What is Huntington's disease caused by?
Autosomal dominant trinucleotide repeat disorder (CAG) on chromosome 4
204
How is Huntington's disease investigated?
Largely a clinical diagnosis (presence of a family history helps) Genetic testing (blood) MRI and CT can show loss of striatal volume
205
How is Huntington's disease managed?
MDT approach Genetic counselling OT/PT Clinical trials
206
If you think that reflexes may be brisk, what can you do to check whether it is pathologically brisk?
Finger jerks Hoffman sign
207
What is a positive Hoffmann sign suggestive of?
Corticospinal tract dysfunction in the cervical segments of spinal cord
208
What pattern of weakness is seen in myopathies and in neuromuscular junction disorders?
Proximal weakness
209
Which additional features of an examination should be performed if myasthenia gravis is suspected?
Fatiguable ptosis Oculoparesis
210
Which investigations should be requested in suspected myasthenia gravis?
BEDSIDE: Ice test BLOODS: ACh receptor antibodies, MuSK antibodies, TFT OTHER: single-fibre EMG, edrophonium test, CT/MRI to check for thymoma
211
How is myasthenia gravis treated?
Cholinesterase inhibitor (e.g. pyridostigmine) Steroids (immunosuppression) Steroid-sparing agents (e.g. azathioprine) MYASTHENIC CRISIS: IVIG and Plasmapheresis (and ventilatory support) SURGICAL: Thymectomy
212
What causes Friedreich's ataxia?
Autosomal recessive condition caused by GAA repeat expansion of the frataxin gene
213
What are the main features of Friedreich's ataxia?
Progressive ataxia Absence of deep tendon reflexes Spasticity Peripheral sensory neuropathy Dysarthria
214
What are some non-neurological manifestations of Friedreich's ataxia?
HEART: cardiomyopathy, arrhythmias, heart failure MSK: kyphoscoliosis PANCREAS: diabetes mellitus EYES: optic atrophy
215
Which investigations should be requested in suspected Friedreich's ataxia?
Genetic studies Nerve conduction studies ECG and echo Vitamin B12 and vitamin E levels MRI brain and spinal cord
216
How is Friedreich's ataxia managed?
MDT approach OT/PT SALT Orthotics Cardiology (for cardiomyopathy) Genetic counselling Diabetes management
217
What is seronegative myasthenia gravis?
Clinical and neurophysiological evidence of myasthenia gravis but without antibodies
218
At what time within the onset of myasthenia gravis affecting the eyes would you expect the disease to become generalised?
2 years If no generalisation at this point, they can be diagnosed with ocular myasthenia
219
What is the 'steroid dip' seen in patients with myasthenia gravis?
Weakness gets worse when treatment is started NOTE: if severe weakness, they should be admitted with steroids are started
220
How do you test for fatiguable ptosis?
Sustained upgaze
221
What is a midline sternotomy scar suggestive of?
CABG (check for vein harvesting) Valve replacement Heart transplant
222
What are the common indications for aortic valve replacement?
Severe symptomatic aortic stenosis or regurgitation Infective endocarditis
223
What are the advantages and disadvantages of a mechanical heart valve?
ADVANTAGE Longer-lasting Durable (15-20 years) DISADVANTAGE Requires lifelong anticoagulation NOTE: bioprosthetic valves only last around 10 years
224
What are the main causes of aortic stenosis?
Senile calcification Bicuspid aortic valve Rheumatic heart disease Lupus Fabry's disease
225
What are some signs of severity in aortic stenosis?
Quiet second heart sound Long duration to the murmur Low volume pulse Forceful apex beat
226
What are the causes of an ejection systolic murmur?
Aortic stenosis Aortic sclerosis HOCM Pulmonary stenosis
227
Which investigations should be requested in suspected aortic stenosis?
ECG (LVH by voltage criteria) Echocardiogram CXR (heart failure)
228
What are the management options for severe aortic stenosis?
Metallic or Tissue Aortic Valve Replacement TAVI
229
Which medications should be avoided in severe aortic stenosis?
ACE inhibitors Nitrates NOTE: in severe aortic stenosis, the ventricle is unable to augment its stroke volume in the context of a reduction in preload. Afterload is fixed they are preload dependent.
230
What are some echocardiographic features of severe aortic stenosis?
Peak velocity of > 4 m/s Mean gradient > 40 mm Hg Valve area < 1 cm^2
231
What are the indications for mitral valve replacement?
Mitral stenosis Mitral regurgitation Infective endocarditis
232
In a young person with a midline sternotomy scar, what should you consider?
Mitral valve REPAIR (rather than replacement) This is preferred to mitral valve replacement
233
What are the different types of mitral valves?
Ball and cage Single tilting disc Bileaflet
234
Which bacterium causes prosthetic valve infection within 6 months of implantation?
Staphylococcus epidermidis
235
What is a fixed split S2?
It means that there is a discrepancy between the aortic and pulmonary valve closing leading to a double heart sound Physiological splitting refers to splitting that only occurs during inspiration. Whereas fixed splitting is present during inspiration and expiration.
236
What are some causes of a fixed split S2?
Pulmonary hypertension Right heart failure Atrial septal defect
237
What are some features of pulmonary hypertension noted on examination?
Raised JVP Right ventricular heave Loud P2 (fixed split second heart sound) Peripheral fluid overload
238
What are the main causes of a pansystolic murmur?
Mitral regurgitation Tricuspid regurgitation VSD
239
What clinical features would be suggestive of SEVERE mitral regurgitation?
Features of pulmonary hypertension (raised JVP, RV heave, loud P2) Displaced apex/heave Breathlessness and fluid overload
240
What is the JVP a reflection of?
Pressures within the right atrium
241
What are the indications for mitral valve replacement?
Symptomatic mitral regurgitation Pulmonary hypertension Fluid overload Asymptomatic with declining ejection fraction (< 50%) or LV dilatation (> 50 mm) Acute mitral regurgitation following MI Can also be considered in some people with severe MR but without the above where surgical risk is low and durable repair is likely
242
What are the potential causes of mitral regurgitation?
Age-related mitral regurgitation Mitral valve prolapse (including connective tissue disorders) Papillary muscle rupture Rheumatic fever Infective endocarditis Cardiomyopathy (ventricular dilation)
243
Why is a urine disptick useful in the context of a new heart murmur?
Microscopic haematuria and proteinuria are associated with infective endocarditis
244
Which ECG features might you see in mitral valve disease?
P mitrale AF
245
What causes an S3 heart sound?
Rapid ventricular filling of a compliant left ventricle S3 = KENTUCKY
246
What causes an S4 heart sound?
Atria contracting against stiff ventricles S4 = TENNESSEE
247
What are the main differences between an S3 and split S2?
S2 split is higher pitch S2 is heart over pulmonary area, whereas S3 is heart at apex
248
Who should be recommended a bioprosthetic valve?
65 years + for mitral valves 70+ years for aortic valves Patients with high risk of haemorrhage (so not for anticoagulation) Patients who are not compliant with medications Young women of childbearing age
249
Why should metallic valves be avoided in women of child-bearing age?
High risk of thromboembolic complications given relative procoagulant state of pregnancy
250
What are some initial medical options for acute mitral regurgitation?
Nitrates Diuretics Sodium nitroprusside Inotropes Intra-aortic balloon pump
251
How does standing and squatting affect murmurs?
STANDING: Increases mitral valve prolapse and HOCM murmurs SQUATTING: Increases most other murmurs (e.g. VSD, AS, AR, MR)
252
What can cause continuous murmurs?
PDA ASD AV fistula
253
What causes Marfan syndrome?
Autosomal dominant mutation in fibrillin-1 gene resulting in loss of elasticity of connective tissues
254
What are some clinical features of Marfan syndrome?
Arachnodactyly High arched palate Tall with long limbs Chest wall deformities (e.g. pectus excavatum) Scoliosis Pes planus Aortic regurgitation Aortic dissection
255
Why might someone with Marfan's syndrome have a large scar extending from their abdomen to their back?
Aortic repair (e.g. in dissection)
256
Which investigations should be requested in suspected Marfan syndrome?
Echo (aortic regurgitation) CT Aorta (check for dilation + dissection) CXR (pneumothorax, dissection) Fibrillin-1 blood test
257
What are the cardiac manifestations of Marfan syndrome?
Aortic dilatation Aortic regurgitation Aortic dissection Mitral valve prolapse
258
What are the indications for aortic root replacement in people with Marfan syndrome?
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
259
What are the causes of systolic murmurs?
Aortic and pulmonary stenosis Aortic sclerosis HOCM ASD VSD
260
What are the symptoms of significant pulmonary stenosis?
Breathlessness on exertion Peripheral oedema Presyncope and syncope Fatigue Chest pain
261
What clinical features may be seen on examination of a patient with significant pulmonary stenosis?
Raised JVP RV heave Pansystolic murmur (functional TR) Peripheral oedema Widely split second heart sound with loud P2
262
What are some echocardiographic features of severe pulmonary stenosis?
Gradient > 64 mm Hg Velocity > 4 m/s Valve area < 1 cm^2
263
What causes Noonan syndrome?
Autosomal dominant condition caused by a few different mutations (PTPN11 and SOS1 are most common)
264
What are the clinical features of Noonan syndrome?
FACIAL Triangular-shaped face Hypertelorism Short, webbed neck Low set ears OCULAR Strabismus Ptosis Refractive errors OTHER Pectus excavatum Cubitus valgus Mild learning disability
265
What are the cardiac complications of Noonan syndrome?
Pulmonary stenosis (most common) HOCM ASD VSD Tetralogy of Fallot
266
How is Noonan syndrome managed?
NON-MEDICAL: monitor growth and puberty, educational support, feeding support MEDICAL: medical treatment of heart failure, valve replacement
267
What are the features of tetralogy of Fallot?
Pulmonary stenosis RV hypertrophy VSD Overriding aorta
268
What are some congenital causes of pulmonary stenosis?
Noonan syndrome Alagille syndrome Tetralogy of Fallot Congenital rubella syndrome
269
What are some acquired causes of pulmonary stenosis?
Carcinoid syndrome Rheumatic heart disease
270
How is pulmonary stenosis categorised based on echo findings?
Based on valve gradient < 36 mm Hg: mild 36-64 mm Hg: moderate > 64 mm Hg: severe
271
What are some causes of mitral valve prolapse?
Marfan syndrome Ehlers-Danlos syndrome Polycystic Kidney Disease Osteogenesis Imperfecta
272
What vascular complications are associated with Ehlers-Danlos syndrome?
Aortic dissection Aortic aneurysm Spontaneous coronary artery dissection
273
How can you test for hypermobility?
Bend fingers back Make thumb touch wrist
274
What are the main complications of mitral valve prolapse?
Infective endocarditis Thromboembolic events (e.g. stroke) Sudden death Heart failure
275
What murmur is heard in mitral valve prolapse?
Dynamic systolic click and mid-late systolic murmur
276
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 Ho Valve thickening > 5 mm Flail leaflet
277
What is a mitral clip?
Percutaneous repair option for mitral valve prolapse in people with suitable anatomy and no surgical option
278
What does the ductus arteriosus connect?
Proximal pulmonary trunk and descending aorta (just distal to left subclavian artery)
279
Which cases of PDA are considered for closure?
LV volume overload RV pressure overload This is usually device closure which is done percutaneously
280
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
281
Which investigations should be requested in suspected PDA?
Echo (check for LV volume overload, estimate PA pressure) Cardiac MRI Cardiac CT If PA pressure high on echo --> invasive testing (cardiac catheterisation)
282
What are the benefits of invasive cardiac catheterisation?
Measurement of right sided pressures Calculate pulmonary and systemic vascular resistance
283
What are the clinical features of a PDA?
Left subcalvian thrill Continuous machinery murmur Wide pule pressure and bounding pulse
284
How is PDA managed?
Babies: Indomethacin Open surgical or endovascular closure Small PDAs without any adverse features do not require closure
285
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)
286
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
287
What are the cardiac causes of clubbing?
Congenital cyanotic heart disease Infective endocarditis Atrial myxoma
288
What are the gastrointestinal causes of clubbing?
Cirrhosis Coeliac disease Crohn's disease
289
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.
290
What are the clinical signs of Eisenmenger syndrome?
Dyspnoea Cyanosis Clubbing Pulmonary hypertension signs (elevated JVP, functional TR) Parasternal heave (pressure overloaded RV)
291
Why would the intensity of a VSD murmur decrease in Eisenmenger syndrome?
The reversal of flow means that the murmur is quieter
292
What consideration needs to be taken for anyone with a VSD who is undergoing surgery on infected tissue?
Endocarditis prophylaxis
293
Which medications may be used in the management of pulmonary hypertension?
Endothelin antagonists (e.g. bosentan) Phosphodiesterase 5 inhibitors (e.g. sildenafil) Prostanoid infusions
294
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
295
What are the clinical features of a VSD?
Systolic murmur Parasternal heave
296
How is a VSD managed?
Spontaneous closure possible in small muscular defects MEDICAL: Diuretics, ACE inhibitors SURGICAL: Percutaneous closure
297
What are the main complications of Eisenmenger syndrome?
RV failure Massive haemoptysis Cerebral embolism Infective endocarditis
298
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
299
What complications may arise following surgery for tetralogy of Fallot?
Pulmonary regurgitation (due to intervention to dilate RV outflow obstruction) Endocarditis Paradoxical embolism Arrhythmia
300
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
301
What is a Blalock-Taussig shunt?
Synthetic shunt between brachiocephalic trunk and pulmonary artery toWa enhance pulmonary blood flow whilst awaiting further intervention This is why some TOF patients may have large posterior thoracotomy scars. Causes pulse differential.
302
What are the causes of cyanotic heart disease?
TOF TGA Tricuspid atresia Pulmonary atresia Ebstein's anomaly Eisenmenger syndrome
303
What are the causes of acyanotic heart disease?
ASD VSD Coarctation of the aorta PDA Aortic stenosis
304
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
305
What are the echocardiogram features of restrictive cardiomyopathy?
Good LV systolic function Biventricular diastolic dysfunction Biatrial dilatation
306
What are some causes of restrictive cardiomyopathy?
PRIMARY Loeffler's endocarditis (eosinophilic infiltration) Endomyocardial fibrosis SECONDARY Amyloidosis (most common) Sarcoidosis Iron overload Scleroderma Radiotherapy
307
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
308
How is restrictive cardiomyopathy managed?
Heart failure management (diuretics, ACE inhibitors) AF management (beta-blockers, anticoagulation PPMs and ICDs Heart transplant
309
How can you distinguish between constrictive pericarditis and restrictive cardiomyopathy through investigations?
CXR: pericardial calcification Echo: hyperechoic and thickened pericardium Cardiac MRI NOTE: restrictive pericarditis can be treated surgically with pericardial stripping
310
What are some causes of constrictive pericarditis?
Viral or bacterial pericarditis (e.g. Coxsackie B, TB) Post-surgical (e.g. CABG) Radiation
311
How do the bell and diaphragm of the stethoscope differ?
Bell for LOW frequency sounds Diaphragm for HIGH frequency sounds
312
How is secondary mitral regurgitation managed?
Heart failure management and medication optimisation
313
Which nerve root is responsible for shoulder abduction?
C5
314
Which nerve root is responsible for elbow flexion and wrist extension?
C6
315
Which nerve root is responsible for elbow extension?
C7
316
Which nerve root is responsible for thumb extension and ulnar deviation?
C8
317
Which nerve root is responsible for finger abduction?
T1
318
Which nerve root is responsible for hip flexion?
L2
319
Which nerve root is responsible for knee extension?
L3
320
Which nerve root is responsible for ankle dorsiflexion?
L4
321
Which nerve root is responsible for big toe extension?
L5
322
Which nerve root is responsible for ankle plantarflexion?
S1
323
Which nerve root is responsible for knee flexion?
L5/S1
324
What is the Gallavardin phenomenon?
Radiation of an aortic stenosis murmur to the apex thus mimicking mitral regurgitation
325
What can you do to make a diastolic murmur louder?
Lean the patient forward
326
What can you do to make a murmur from HOCM louder?
Valsalva manoeuvre
327
What are the main causes of mitral stenosis?
Rheumatic heart disease Calcification Fabry's disease Rheumatoid arthritis SLE Carcinoid syndrome Whipple's disease
328
What is Fabry's disease?
X-linked disorder caused by a mutation in the alpha-galactosidase A gene Characterised by neuropathic pain, angiokeratomas, gastrointestinal issues Heart valve defects and LVH Stroke
329
Which standard panel of investigations should be requested for patients with valvular disease?
ECG CXR Echo Exercise testing (Urine dipstick and 3 x blood cultures if suspecting endocarditis)
330
What criteria area applied in the diagnosis of infective endocarditis?
Duke's criteria Diagnostic if either: 2 Major 1 Major + 3 Minor 5 Minor
331
Which organisms most commonly cause infective endocarditis?
Streptococci (viridans and gallolyticus) Staphylococcus aureus HACEK (Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, and Kingella)
332
What are the major Duke's criteria?
Positive blood cultures for typical organisms Evidence of endomyocardial involvement (e.g. new valve regurgitation, echocardiogram findings)
333
What is an Austin Flint murmur?
Rumbling diastolic murmur heard at the apex in patients with severe aortic regurgitation 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)
334
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
335
What is balloon valvuloplasty?
Temporising procedure that involves dilating a stenosed valve to improve its function whilst awaiting more definitive management
336
Which type of murmur is concerning in someone with a valve replacement?
Regurgitant murmurs (suggests that the valve may not be fully competent) It is quite common to get flow murmurs
337
Why is PR prolongation of significance in people with a valve replacement?
May be suggestive of an aortic valve abscess
338
What are the main complications of valve replacement?
Infection (endocarditis) Stroke Bleeding (from anticoagulation) Haemolysis (from stenosed valve)
339
What are the main indications for a permanent pacemaker?
Bradyarrhythmias (3rd degree, Mobitz type 2) Sick sinus syndrome with symptoms
340
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%
341
Who would benefit from CRT?
Heart failure with LVEF < 35% and ventricular conduction impairment (e.g. broad QRS, LBBB)
342
What can help distinguish arthralgia in hereditary haemochromatosis from osteoarthritis?
Chondrocalcinosis (and pseudogout)
343
Which blood test results should prompt further investigations for hereditary haemochromatosis?
Raised ferritin and transferrin saturation > 45%
344
What are the main clinical features of hereditary spherocytosis?
Jaundice Anaemia Splenomegaly
345
How is hereditary spherocytosis treated?
Blood transfusions Folic Acid Splenectomy
346
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)
347
Where is a transplanted pancreas attached in an SPK?
To the small bowel Used to be connected to the bladder (urinary lipase can be monitored for rejection)
348
Which patients undergo an SPK?
Poorly controlled T1DM with renal failure (sometimes done for T2DM) SPK has a better 10-year survival than isolated kidney transplant in patients with diabetic nephropathy
349
What does the scar in an SPK look like?
Two scars in each iliac fossa or midline laparotomy
350
How do you assess for encephalopathy in liver disease?
Asterixis Constructional dyspraxia (can't draw a 5-pointed star)
351
Which prognostic scores are used in cirrhosis?
Child-Pugh (ascites, encephalopathy, INR, bilirubin and albumin) UK MELD (INR, creatinine, bilirubin, sodium)
352
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
353
What is a clinical clue that alcohol is the cause of cirrhosis?
Parotid swelling
354
Which gene is involved in the hereditary form of pancreatitis?
PRSS1 (autosomal dominant) SPINK1 Cystic fibrosis is another inherited cause of pancreatitis
355
What are the main causes of pancreatitis?
Alcohol Gallstones Drugs (e.g. azathioprine) ERCP Autoimmune Mumps Hypercalcaemia Hyperlipidaemia
356
What are the complications of pancreatitis?
ACUTE: SIRS, respiratory failure CHRONIC: portal vein thrombosis, chronic pancreatitis, pseudocyst formation
357
How are pseudocysts drainaed?
Usually with an endoscopic approach using ultrasound guided drainage Can be stented using an Axios stent
358
Why does creon need to be given with a PPI?
Prevent the acidity from breaking down creon in the stomach
359
What are some of the complications of chronic pancreatitis?
Pain Type 3c diabetes Strictures Compressive biliary obstruction
360
How is chronic pancreatitis managed?
Creon Stop drinking alcohol Stent insertion for biliary obstruction or pancreatic duct strictures Pain management
361
Why are ileostomys spouted rather than flush to the skin?
To prevent the contents from irritating the skin
362
What are some indications for emergency surgery in patients with inflammatory bowel disease?
Toxic megacolon Haemorrhage Perforation Non-emergency: fistula, poor symptom control despite maximal medical therapy
363
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 It may be done to preserve the distal bowel/rectum in case anastomotic surgery is performed in the future (e.g. ilealpouch anal anastomosis)
364
What are some extra-GI manifestations of inflammatory bowel disease?
Erythema nodosum Arthritis Liver dysfunction Aphthous ulcers
365
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)
366
What are some of the side-effects of ciclosporin use?
Gum hypertrophy Hypertension Increased risk of cancer (esp. post-transplant lymphoproliferative disorders)
367
What are some clinical features of portal hypertension?
Caput medusae Splenomegaly (this may not change post transplant) Rectal varices
368
What are the indications for liver transplant?
Acute and chronic liver failure Hepatocellular carcinoma Causes include drug-induced liver injury (e.g. paracetamol), alcoholic liver disease, autoimmune hepatitis, NASH, viral hepatitis
369
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
370
What are some clinical features that can help distinguish pneumonectomy from lobectomy?
Tracheal deviation (definite in pneumonectomy, possibility in lobectomy) Absent breath sounds in pneumonectomy (may be normal or reduced in lobectomy) Dull percussion note in pneumonectomy
371
Why is a lateral thoracotomy scar from a lobectomy more suggestive of non-small cell lung cancer than small cell lung cancer?
Non-small cell lung cancer is more common Small cell lung cancer is rapidly progressive and is often not amenable to surgical management
372
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
373
What are some indications for a VATS procedure?
Lobectomy (NOT pneumonectomy) Pleurectomy (may be done for recurrent pneumothoraces) Pleurodesis Bullectomy
374
When might a patient be considered for surgical management of a pneumothorax?
Recurrent pneumothoraces Persistent air leak
375
How does VATS compare to an open thoracotomy?
VATS is less invasive Lower risk of pain and better recovery Risk of recurrence is greater for VATS
376
How is a primary spontaneous pneumothorax managed?
Asymptomatic OR <2cm - regular outpatient review Symptomatic + >2cm - needle aspiration > chest drain
377
How is a secondary spontaneous pneumothorax managed?
Asymptomatic or <2cm - admit for observation Symptomatic - chest drain > after removal for OP f/u in 2-4 weeks
378
What are the sites of needle aspiration and chest drain insertion?
Needle aspiration --> 2nd ICS MCL or safe triangle Chest drain --> safe triangle
379
What are some complications of pneumothoraces?
Surgical emphysema Tension pneumothorax (resulting in hypotension and cardiac arrest)
380
What are some causes of wheeze?
Asthma COPD Bronchiectasis Bronchiolitis Obliterans (secondary to viral infections, pollutants, graft-versus-host disease)
381
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
382
What spirometry results would you expect to see in asthma?
FEV1/FVC Ratio < 0.7 Bronchodilator reversibility: 200 mL or 15% improvement in FEV1
383
Outline the management guidelines for asthma.
Recent 2024 change 1: As needed low-dose ICS/LABA 2: Low-dose MART 3: Moderate-dose MART 4: Add LTRA OR LAMA 5: Add in/switch to alternative
384
What are some causes of interstitial lung disease?
Idiopathic pulmonary fibrosis Sarcoid CTD: rheumatoid arthritis, scleroderma, SLE, ankSpond Iatrogenic: amiodarone, nitro, radiation Exposure: Silicosis, asbestosis, farmers lung (hypersensitviity pneumonitis)
385
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
386
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
387
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
388
Which cause of interstitial lung disease is most commonly associated with clubbing?
Idiopathic pulmonary fibrosis
389
What is a restrictive pattern on spirometry?
FEV1 < 80% FVC < 80% FEV1/FVC > 0.7
390
How is non-specific interstitial pneumonia managed?
Steroids Steroid-sparing agents (e.g. azathioprine, mycophenolate) NOTE: prognosis for NSIP is much better than for IPF
391
What is the life expectancy in idiopathic pulmonary fibrosis?
2-5 years
392
What are the main causes of upper lobe predominant pulmonary fibrosis?
TB Silicosis Extrinsic allergic alveolitis Ankylosing spondylitis Cystic fibrosis
393
What are the main causes of lower lobe predominant pulmonary fibrosis?
Idiopathic pulmonary fibrosis Asbestosis Rheumatoid arthritis Scleroderma
394
Why might someone with cystic fibrosis have a portacath?
For long-term intravenous antibiotics
395
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)
396
Which gene mutation causes cystic fibrosis?
CFTR gene on chromosome 7 (autosomal recessive) Most common mutation is Delta F508 mutation
397
What is a button gastrostomy?
Shortened gastrostomy tube that lies flush to the chest wall and is more discrete
398
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
399
How is cystic fibrosis screened for the 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)
400
Which organisms chronically colonise patients with cystic fibrosis?
Pseudomonas aeruginosa Burkholderia cepacia (can't have lung tx) Non-tuberculous mycobacteria (can't have lung tx) Staphylococcus aureus (in childhood)
401
How are the chest manifestations of cystic fibrosis managed?
Regular chest physiotherapy Exercise Nebulisers (including mucolytics) Prophylactic antibiotics New development: ivacaftor/lumacaftor (Orkambi)
402
What is the life expectancy for people with cystic fibrosis in the UK?
40 years
403
What is the incidence of cystic fibrosis in the UK?
1 in 2500 live births
404
What are some differential diagnoses for cystic fibrosis?
Primary immunodeficiency Primary ciliary dyskinesia Chronic aspiration
405
Which scar would you expect in someone with a bilateral lung transplant?
Clamshell Midline Sternotomy NOTE: they may also have intercostal drain scars
406
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
407
What are the most common indications for lung transplant?
Interstitial lung disease Cystic fibrosis COPD Pulmonary vascular diseases 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.
408
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
409
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) NOTE: but given better survival of double lung transplant, it may be performed in any of the above cases
410
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
411
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
412
Which scoring system is used to categorise the severity of COPD?
BODE Index NOTE: score of >7 should be considered for lung transplantation
413
Which combination of immunosuppressants tend to be used in lung transplantation?
Prednisolone Calcineurin inhibitor (e.g. tacrolimus) Nucleotide blocker (e.g. azathioprine, MMF) NOTE: also need prophylactic medications to prevent opportunistic infections
414
What are the main features of yellow nail syndrome?
Slow-growing and thickened finger nails Bronchiectasis or pleural effusions Lymphoedema
415
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
416
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) If under 40, check for cystic fibrosis
417
How is primary ciliary dyskinesia diagnosed?
Nasal biopsy or nasal potential difference
418
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)
419
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)
420
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
421
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
422
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.
423
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
424
Which paraneoplastic syndromes are associated with lung cancer?
Lambert-Eaton myasthenic syndrome SIADH Hypercalcaemia (PTHrP) Cushing syndrome (ectopic ACTH)
425
List some causes of transudative pleural effusions.
Left ventricular failure Renal failure Hypoalbuminaemia (nephrotic syndrome, cirrhosis) Myxoedema Peritoneal dialysis
426
List some causes of exudative pleural effusions.
Bacterial pneumonia Tuberculosis Malignancy CTDs (e.g. rheumatoid arthritis) Vasculitis
427
Which tests should a pleural tap be sent for?
pH Protein LDH Cytology Gram stain AFB stain Culture Other: glucose (low in infection, malignancy and rheumatoid arthritis), amylase (raised in pancreatitis), triglycerides (chylothorax)
428
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
429
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
430
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)
431
What FEV1 cut-offs are used to determine suitability for a lobectomy or pneumonectomy?
Pneumonectomy > 2 L Lobectomy > 1.5 L NOTE: other investigations include walk test, formal cardiopulmonary exercise test and echocardiogram
432
What is the main indication for a pneumonectomy?
Large, central non-small cell lung cancer or tumours affecting both lobes NOTE: another indication is bronchiectasis
433
What is a Chamberlain procedure?
Parasternal mediastinotomy in someone who also has a pneumonectomy This allows access to access mediastinal lymph nodes
434
What are some differentials for bibasal crepitations?
Interstitial lung disease Heart failure Bilateral pneumonia Bronchiectasis
435
What can cause breathlessness with normal lung sounds?
PE Anaemia Obesity hypoventilation Anxiety Pulmonary hypertension
436
Which investigations should be requested in suspected obstructive sleep apnoea?
Polysomnography FBC (polycythaemia) Morning ABG CXR, ECG and Echo (pulmonary hypertension)
437
How is OSA managed?
Weight loss Smoking cessation Nocturnal CPAP Oral appliances Drugs (e.g. modafinil)
438
What are some complications of OSA?
Hypertension MI Stroke Pulmonary hypertension
439
What is Felty's syndrome?
A triad of rheumatoid arthritis, splenomegaly and neutropaenia
440
Why is platelet count low in alcohol excess?
Alcohol has direct toxic effects on the bone marrow Splenomegaly can lead to sequestration
441
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
442
What causes Wilson's disease?
Mutation in the ATP7B gene (involved in copper secretion) Autosomal recessive
443
Which antibodies are associated with autoimmune hepatitis?
ANA ASMA Anti-LKM1
444
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
445
What are some key differences between Crohn's disease and ulcerative colitis?
Crohn's affects any point from mouth to anus and can have skip lesions Crohn's causes transmural inflammation Crohn's is more associated with the development of fistulae Crohn's histology is characterised by granulomas whereas ulcerative colitis is characterised by crypt abscesses
446
What are some causes of renal enlargement?
ADPKD Hydronephrosis Renal tumours Amyloidosis
447
Which opportunistic infections can occur in patients being immunosuppressed for a renal transplant?
CMV BK Virus JC Virus (PML) PCP
448
What are the main complications of hereditary haemorrhagic telangiectasia?
Recurrent epistaxis Gastrointestinal haemorrhage Anaemia Intracranial bleeds
449
What are the possible clinical features of Peutz-Jeghers syndrome?
Mucocutaneous pigmentation Anaemia Abdominal scars from bowel resection PJS is autosomal dominant
450
List some secondary causes of Raynaud's phenomenon.
SLE Systemic sclerosis Rheumatoid arthritis Dermatomyositis Mixed connective tissue disease Beta blockers Atherosclerosis
451
How is Raynaud's phenomenon managed?
Keep body warm Nifedipine
452
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
453
What are the different groups of driving licences?
Group 1: Car Group 2: Bus or Lorry
454
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
455
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
456
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
457
How long can you stop driving for after a stroke/TIA?
Group 1: 1 Month (No DVLA) Group 2: 1 Year (Tell DVLA) Ongoing ability to drive is dependent on the effect of the CVA
458
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
459
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) Also think of photocoagulation burns
460
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) Insulin therapy can be considered at any stage
461
What is usually assessed at a routine diabetes review?
HbA1c Feet (ulcers, sensation) Eyes (fundoscopy) Blood Pressure Cholesterol Renal Function
462
Which conditions cause loss of peripheral vision?
Retinitis pigmentosa Glaucoma Previous CVA (causing hemianopia)
463
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) NOTE: myasthenia and ocular myopathies can cause variable diplopia
464
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
465
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
466
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)
467
What is Holmes-Adie syndrome?
At least 1 abnormally dilated pupil with no light response Loss of deep tendon reflexes Abnormalities of sweating
468
What is optic atrophy?
Pale optic disc due to death of retinal ganglion cell axons of the optic nerve
469
What are the most common causes of optic atrophy?
Optic neuritis (MS, NMO) Glaucoma Ischaemic optic neuropathy Drugs (e.g. ethambutol) Vitamin B12 deficiency
470
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)
471
What are the main clinical features of retinitis pigmentosa?
Night blindness Loss of peripheral vision NOTE: variable inheritance and penetrance (can be AD, AR or X-linked). No cure.
472
What should you examine in a patient with acromegaly?
Hands (large and doughy) Teeth (spaced) Visual fields Heart (LVH)
473
Which investigations should be requested for a patient with suspected acromegaly?
IGF1 (raised) Glucose tolerance test HbA1c MRI Brain
474
How is acromegaly managed?
FIRST LINE: Trans-sphenoidal Hypophysectomy Adjuncts: Radiotherapy Medical: Dopamine agonists (e.g. bromocriptine), somatostatin analogues (e.g. octreotide) NOTE: post-operatively, patients may require hormone replacement (e.g. thyroxine, hydrocortisone, sex steroids)
475
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
476
How is Graves' disease managed?
Radioiodine Surgery Medical (thionamides such as carbimazole and propylthiouracil)
477
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) Do not get pregnant for 6 months after treatment
478
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
479
Which investigations should be requested in a patient with suspected rheumatoid arthritis?
ESR/CRP Rheumatoid factor Anti-CCP antibodies X-Rays
480
How is rheumatoid arthritis managed?
Analgesia: paracetamol, NSAIDs DMARDs: methotrexate, sulfasalazine, hydroxychloroquine Biologics: infliximab, etanercept (usually after 2 DMARDs have been unsuccessful) Exercise and physio
481
What are the main side-effects of methotrexate?
Immunosuppression Hepatotoxicity Pneumonitis Pulmonary fibrosis Teratogenic
482
What are the five types of psoriatic arthritis?
Symmetrical polyarthritis Asymmetrical oligoarthritis Distal interphalangeal joint involvement Arthritis mutilans Spondyloarthropathy
483
What are some specific features of psoriasis?
Plaques (extensor surfaces, flexoral or guttate) Nail changes (onycholysis, hyperkeratosis) Koebner phenomenon
484
How is psoriasis managed?
TOPICAL: coal tar, calcipotrol, dithranol SYSTEMIC: methotrexate, ciclosporin, anti-TNF, PUVA NOTE: for psoriatic arthritis, the mainstay of treatment is methotrexate and anti-TNFs
485
What's Jaccoud's arthopathy?
Chronic non-erosive reversible joint disorder most commonly associated with SLE
486
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
487
What are the main treatment options for SLE?
Mild: Hydroxychloroquine Moderate: Azathioprine, MMF, Prednisolone Severe: Cyclophosphamide, Rituximab
488
What are the main differences between limited and diffuse systemic sclerosis?
LIMITED: Anti-centromere. Limited to below elbows and below knees. DIFFUSE: Anti-Scl70. Entire body.
489
List some clinical features of systemic sclerosis.
Sclerodactyly Digital ulcers Calcinosis Microstomia Interstitial lung disease (basal) Dysphagia Loud P2
490
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
491
How is systemic sclerosis managed?
Vascular (Raynaud's/PAH): CCB, PDEi, prostanoids Fibrosis (lung/skin) - immune suppression (MMF, cyclophosphamide), antifibrotics Renal: ACEi GI: PPI, prokinetic
492
How is gout managed?
Acute: NSAIDs, colchicine Chronic: allopurinol, rasburicase
493
Which investigations should be requested in suspected ankylosing spondylitis?
CRP, ESR and HLA B27 XR Sacroiliac Joints and Spine CXR/HRCT ECG and Echocardiogram
494
How is ankylosing spondylitis managed?
Physiotherapy NSAIDs Biologics NOTE: Bath Ankylosing Spondylitis Disease Activity Index is used to guide treatment. >4 means active disease.
495
What are some of the features of Paget's disease?
Bone pain High output cardiac failure Deafness
496
How is Paget's disease managed?
Analgesia Bisphosphonates
497
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. Associated with aquaporin 4 antibodies
498
What are some of the clinical manifestations of neuromyelitis optica?
Spinal Cord Dysfunction: muscle weakness, reduces sensation, loss of bladder or bowel function Optic Nerve: Reduced visual acuity and loss of colour vision
499
What are some key differences between neuromyelitis optica and multiple sclerosis?
NMO presents between 40-50 years whereas MS presents between 20-30 years NMO tends to have more severe attacks
500
How is neuromyelitis optica managed?
Acute: Potent Steroids Prophylaxis: Monoclonal antibodies, azathioprine, methotrexate
501
What are some complications associated with renal transplant?
Failure/Rejection Opportunistic Infections (CMV, BK, PJP) Malignancy (SCC, lymphoma) Medication Side-Effects (e.g. hypertension, tremor, Cushingoid)
502
What causes Alport syndrome?
Inherited condition characterised by a defect in collagen IV
503
Define TIA.
Acute focal neurological symptoms that resolve completely within 24 hours of onset
504
How should a TIA be managed?
Immediately: 300 mg Aspirin Assess urgently within 24 hours If presenting after 1 week, urgent referral to stroke specialist within 1 week Long-Term once confirmed: 300 mg Aspirin AND Clopidogrel STAT followed by 75 mg clopidogrel OD for life and 75 mg aspirin OD for 21 days Start statin therapy
505
Who should be offered primary prevention statin?
Aged 25-84 years with QRISK3 > 10% Type 1 Diabetes Mellitus (esp over 40 years or had DM for > 10 years) Aged > 85 years
506
How should patients be followed up after starting statin therapy?
Repeat lipid profile 3 months after starting therapy (aim for 40% decrease) - If not achieved, consider ezetimibe - Review LFT Aim < 5 mmol/L total cholesterol , < 3.4 LDL, > 1 HDL
507
What counts as a unit of alcohol?
10 mL or 8 g of pure alcohol
508
Outline the management of hypertension.
STEP 1 (< 55 years, T2DM of any age): ACE inhibitor or ARB STEP 1 (> 55 years, Black): CCB STEP 2: Add the other STEP 1 or a thiazide-like diuretic (e.g. ACEi + CCB) STEP 3: ACEi/ARB + CCB + Thiazide-like STEP 4: If K < 4.5 --> Spironolactone, if > 4.5 --> alpha- or beta-blocker
509
What is the typical pattern of joint involvement in rheumatoid arthritis?
Symmetrical polyarthritis affecting small joints of hands and EXCLUDING the DIP
510
What are the main clinical features of rheumatoid arthritis?
Symmetrical polyarthritis affecting small joints of hands Ulnar deviation of MCP joints Boutonniere's and swan neck deformity Z thumb deformity Rheumatoid nodules
511
Which scars might you see in a patient with rheumatoid arthritis?
Carpal tunnel decompression Swanson's arthroplasty Wrist arthrodesis Ulnar styloidectomy Wrist synovectomy
512
What are some extra-articular features of rheumatoid arthritis?
BLOOD: anaemia, splenomegaly SKIN: nodules, pyoderma gangrenosum EYE: scleritis CVD: valvular disease RESP: pulmonary fibrosis (NSIP) RENAL: amyloid NEURO: peripheral neuropathy, mononeuritis multiplex
513
How is methotrexate toxicity treated?
Folinic acid
514
What are some X-ray features of rheumatoid arthritis?
Juxta-articular osteopaenia Erosions Soft tissue swelling
515
Which HLA types are associated with psoriatic arthritis?
HLA B27 (AS-type) HLA DR4 (RhA-type)
516
What are the main clinical manifestations of SLE?
Serositis (pleurisy) Oral ulcers Arthritis Photosensitivity, alopecia Blood abnormalities (cytopaenia) Renal (dysfunction, proteinuria) ANA Immunological (dsDNA, ANA) Neurological (seizures, psychosis) Rash (discoid, malar)
517
Which antibodies are associated with Sjogren's disease?
Anti-Ro and Anti-La
518
Which conditions predispose to the development of gout?
CKD Hypothyroidism Obesity Myelo- and lymphoproliferative disorders Psoriasis Hyperparathyroidism Drugs (thiazides, levodopa, aspirin)
519
Which drugs might the medications used to treat gout interact with?
Allopurinol and azathioprine --> bone marrow suppression Allopurinol and warfarin --> increased INR Colchicine and statins --> increased myopathy
520
What is red desaturation suggestive of?
Optic nerve disease
521
List some causes of sudden loss of vision.
Vitreous haemorrhage Retinal artery or vein occlusion Stroke Trauma Retinal detachment
522
What is an RAPD suggestive of?
Optic nerve disease Widespread retinal damage
523
Which investigations are useful in suspected optic neuritis?
BLOODS: ESR, ANA, B12, HbA1c Formal perimetry Visual evoked potentials MRI brain and orbits Temporal artery biopsy (GCA)
524
What are some causes of a sore prominent eye with visual disturbance?
Medical causes of tear film disturbance Sjogren syndrome Thyroid eye disease Sarcoidosis
525
How is thyroid eye disease managed?
Artificial tears Systemic immunosuppression with steroids (for severe disease/acute) Stop smoking Optimise thyroid function
526
List some causes of bitemporal hemianopia.
Pituitary adenoma Craniopharyngioma Meningioma
527
What causes spastic paraparesis with a sensory level?
Cord compression (e.g. disc herniation) Cord infarction Transverse myelitis (e.g. MS, mycoplasma)
528
What causes spastic paraparesis with dorsal column dysfunction (joint position and vibration sense loss)?
Demyelination (Multiple sclerosis) Friedreich’s ataxia Subacute combined degeneration of the cord
529
What causes spastic paraparesis with spinothalamic dysfunction (pain and temperature)?
Syringomyelia Anterior spinal artery infarction
530
What causes spastic paraparesis with cerebellar signs?
Demyelination (Multiple sclerosis) Friedreich’s ataxia Spinocerebellar ataxia Arnold Chiari Malformation Syringomyelia
531
What causes spastic paraparesis with small hand muscle wasting?
Cervical myelopathy (C5-T1) Anterior horn cell disease (motor neuron disease) Syringomyelia
532
What causes spastic paraparesis and UMN signs in the upper limbs?
Cervical myelopathy (above C5) Bilateral strokes
533
List some causes of flaccid paraparesis.
ANTERIOR HORN CELL: MND Cauda equina syndrome MOTOR NEURONE: GBS, CIDP, Charcot-Marie-Tooth disease NMJ: myasthenia gravis, LEMS MUSCLE: myositis, thyroid
534
How does Charcot-Marie-Tooth disease manifest?
Distal wasting of legs (inverted champagne bottle) Weakness and mild sensory loss Sensory ataxia (positive Romberg) Areflexia Bilateral foot drop High stepping gait Pes cavus
535
What is Miller-Fisher syndrome?
Proximal variant of GBS characterised by ataxia, ophthalmoplegia and areflexia. Associated with anti-GQ1b antibodies
536
Where should you look for a scar in a patient with foot drop?
Around the fibular head
537
What are the possible causes of foot drop?
Common Peroneal Nerve Palsy: trauma, compression by cast Sciatic Nerve Palsy: trauma Lumbosacral Plexopathy: tumour L5 nerve root: disc prolapse Motor neuron disease
538
How can you distinguish common peroneal nerve palsy from L5 radiculopathy?
Common peroneal nerve: inversion intact, lost sensation between 1st/2nd toes only L5: lost inversion, eversion and dorsiflexion, lost sensation on sole of foot and anterolateral shin
539
What are the main clinical features of myotonic dystrophy?
Myopathic facies Frontal balding Ptosis Myotonia Wasting and weakness of face/neck/limb muscles (distal > proximal) Reduced reflexes NORMAL sensation OTHER: gynaecomastia, pacemaker, diabetes mellitus
540
List some differentials for ptosis.
Horner syndrome 3rd nerve palsy Myotonic dystrophy (bilateral) Myasthenia gravis (bilateral)
541
What is myotonic dystrophy?
Most common adult muscular dystrophy caused by expansion of CTG repeat on chromosome 19 Autosomal DOMINANT inheritance of DMPK gene Causes abnormally sustained muscle contraction after voluntary contraction ceases Starts in adulthood
542
Which investigations should be considered in suspected myotonic dystrophy?
EMG Muscle biopsy CK Genetic testing (DMPK and CNBP genes) For associated features: HbA1c, ECG, CXR (cardiomegaly), slit lamp (cataracts)
543
Outline the management of myotonic dystrophy.
MDT: ophthalmologist, cardiologist, endocrinologist, SALT, physio MEDICAL: phenytoin/quinine/procainamide for myotonia COMPLICATIONS: pacemaker, diabetes management Screen relatives
544
What are some causes of palmar erythema?
Cirrhosis Hyperthyroidism Pregnancy Polycythaemia
545
What are some causes of gynaecomastia?
Physiological (puberty) Cirrhosis Drugs (e.g. spironolactone)
546
What are some indications for splenectomy?
Rupture Hereditary spherocytosis
547
What additional measures must be taken to protect patients after a splenectomy?
Vaccination (pneumococcal, meningococcal, Haemophilus influenaze) Prophylactic penicillin MedicAlert bracelet
548
List some causes of gum hypertrophy.
Drugs (e.g. ciclosporin) Acute myelomonocytic leukaemia Pregnancy
549
What are some historical surgical techniques used for TB?
Plombage Phrenic nerve crush Thoracoplasty (removal of ribs to collapse lung)
550
What surgical measures may be considered in COPD?
Bullectomy Endobronchial valve Lung reduction surgery Single lung transplant
551
What are some complications of an ASD?
Paradoxical embolus Atrial arrhythmias Pulmonary hypertension
552
How is HOCM managed?
ICD Beta-blockers Septal myomectomy Genetic counselling
553
What are some signs associated with dominant parietal lobe dysfunction?
Dysphasia Gerstmann syndrome: dysgraphia, dyslexia, dyscalculia, L-R disorientation
554
What are some features of non-dominant parietal lobe dysfunction?
Spatial neglect Constructional apraxia
555
What causes lateral medullary syndrome?
Posterior inferior cerebellar artery occlusion NOTE: it is the most common brainstem vascular lesion
556
What are the main features of lateral medullary syndrome?
IPSILATERAL: loss of facial sensation, cerebellar signs, Horner syndrome CONTRALATERAL: loss of pain and temperature sensation of body
557
What are the main clinical features of syryngomyelia?
LMN: weakness and wasting of small muscles of hand, reduced reflexes SPINOTHALAMIC: loss of pain and temperature sensation at level of lesion (e.g. arms), preserved joint position and vibration (dorsal columns) Charcot joints Scars from painless burns UMN: may be UMN signs below level of lesion
558
What are some causes of wasting of the small muscles of the hand?
ANTERIOR HORN CELL: MND, syringomyelia, DCM BRACHIAL PLEXUS: cervical rib, pancoast tumour PERIPHERAL: neuropathy, ulnar nerve lesion MUSCLE: disuse atrophy (e.g. RhA)
559
What are some causes of a predominantly SENSORY peripheral neuropathy?
Diabetes mellitus Alcohol Drugs (e.g. isoniazid) B12 deficiency
560
What are some causes of a predominantly MOTOR peripheral neuropathy?
Guillain-Barre syndrome Lead toxicity Charcot-Marie-Tooth disease
561
What is mononeuritis multiplex?
Painful, asymmetrical, asynchronous sensory and motor peripehral neuropathy involving damage to at least 2 separate nerve areas
562
What are some causes of mononeuritis multiplex?
Diabetes mellitus CTD (SLE, rheumatoid arthritis) Vasculitis (PAN) Infection (e.g. HIV) Malignancy
563
What are the main features of a cerebellopontine angle tumour?
CN 8: hearing loss, tinnitus, vertigo, gait dysfunction Can affect CN 5, 6 and 7 Also has cerebellar features
564
What causes cerebellopontine angle syndrome?
Acoustic neuroma Meningioma Cerebellar astrocytoma
565
How is Bell's palsy treated?
Prednisolone 50 mg for 10 days (if presenting within 72 hours) Eye protection 80% make a full recovery
566
What is the significance of forehead sparing in facial nerve palsy?
The facial nerve nucleus is located in the pons and has separate branches going to the upper and lower face The branch going to the upper face receives inputs from both cerebral hemispheres, so a UMN lesion would spare forehead function An LMN lesion would affect all motor output to both the upper and lower face meaning that the forehead is NOT spared
567
What are some differentials for facial nerve palsy?
Bell's palsy Mononeuropathy due to diabetes/Lyme disease Tumour/trauma MS Stroke
568
List some causes of bilateral facial nerve palsy.
Guillain-Barre syndrome Myasthenia gravis Sarcoidosis Lyme disease
569
What are some causes of bilateral ptosis?
Congenital Senile Myasthenia gravis Myotonic dystrophy Mitochondrial cytopathies (e.g. Kearns-Sayre) Bilateral Horner syndrome
570
How is tuberous sclerosis inherited?
Autosomal dominant with variable penetrance
571
What clinical features are associated with neurofibromatosis?
Cutaneous neurofibromas Cafe au lait patches Axillary freckling Lisch nodules Phaeochromocytoma Neuropathy Lung Fibrosis Scoliosis Learning difficulty
572
What are the different types of neurofibromatosis?
Autosomal dominant NF1 (Chr 17): peripheral form NF2 (Chr 22): central form characterised by bilateral acoustic neuromas
573
What scoring criteria are used in the assessment of obstructive sleep apnoea?
Epworth sleep score (out of 24 and a score of 11 is suggestive of OSA)
574
What heart abnormality is associated with osteogenesis imperfecta?
Bicuspid aortic valve Other associations include short height, joint hypermobility and hearing loss
575
How is osteogenesis imperfecta inherited?
Autosomal dominant But can be de novo mutations
576
What are some causes of stroke in the young?
Paradoxical embolus (PFO) Antiphospholipid syndrome Vertebral or carotid artery dissection MELAS/CADASIL
577
How is antiphospholipid syndrome managed?
History of blood clots: Warfarin No history of blood clots: Aspirin or Clopidogrel
578
How is an acute stroke managed?
CT head to rule out bleed Aspirin 300 mg OD for 2 weeks, then clopidogrel 75 mg OD < 4.5 hours for thrombolysis < 6-24 hours for thrombectomy and thrombolysis
579
How is retinitis pigmentosa inherited?
Can be autosomal dominant, recessive or X-linked
580
How is retinitis pigmentosa managed?
There is no cure Largely supportive with visual aids
581
What are some causes of bilateral visual loss?
Retinitis pigmentosa Glaucoma Cataracts Vitamin A deficiency Diabetes mellitus
582
Which conditions are associated with HLA-B27?
Reactive arthritis Psoriatic arthritis Ankylosing spondylitis Enteric arthritis
583
What is Hashimoto's thyroiditis?
Autoimmune condition characterised by lymphocytic destruction of thyroid follicles (most common cause of hypothyroidism in the UK)
584
When should TSH levels be checked after adjusting the dose in hypothyroidism?
8-12 weeks
585
What are some differentials for myasthenia gravis?
Thyroid ophthalmopathy Myotonic dystrophy Motor neuron disease Lambert-Eaton Myasthenic Syndrome Miller Fisher variant of GBS
586
What is a 'complicated' urinary tract infection?
UTI in someone with a structurally abnormal urinary tract Includes any UTI in a man
587
How can spina bifida manifest?
Bladder and bowel dysfunction Paraparesis Sensory impairment Hydrocephalus Syrinx formation
588
What increases the risk of developing spina bifida?
Folic acid deficiency Folate antagonists (e.g. methotrexate) Antiepileptic medications (e.g. valproate)
589
Which other cardiac abnormalities are associated with coarctation of the aorta?
Bicuspid aortic valve VSD PDA
590
What is scleromalacia perforans?
Rare form of scleritis that results in blackish/bluish discoloration of the sclera Occurs in patients with a long history of severe rheumatoid arthritis
591
Which scoring system is used to assess the severity of symptoms in rheumatoid arthritis?
Disease Activity Score 28 Based on number of tender and swollen joints and the ESR > 5.1 is active disease
592
Which medications can worsen myasthenia gravis?
Antibiotics (fluoroquinolones, macrolides and aminoglycosides) Magnesium Statins Beta-Blockers
593
What are the main clinical features of pseudoxanthoma elasticum?
Yellow bumps/papules on skin Elastic skin Vision problems (retinal haemorrhages, angioid streaks) Premature calcification of blood vessels
594
What are some of the clinical features of Ehlers-Danlos syndrome?
Hypermobility Stretchy skin Easy bruising Poor wound healing Chronic pain

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