MRCP OFFICIAL PAPER

Question 1

Mx for secondary hyperparathyroidism?

Answer 1

The patient has a raised serum creatinine with hypocalcaemia and a raised serum phosphate. This pattern is consistent with CKD with impaired renal phosphate secretion and deficient activation of vitamin D (leading to impaired gut absorption of calcium and hypocalcaemia).

The raised plasma parathyroid hormone is due to secondary hyperparathyroidism in an attempt to restore the serum calcium concentration to normal. The leg pain is arising from renal bone disease.

The correct answer is Alfacalcidol. Correction of the vitamin D deficiency is the key to restoring normal calcium homeostasis, and must be with alfacalcidol which replaces the deficient activation step in CKD. Ergocalciferol cannot be activated in CKD.

Basic problems in chronic kidney disease (CKD):
1-alpha hydroxylation normally occurs in the kidneys → CKD leads to low vitamin D
the kidneys normally excrete phosphate → CKD leads to high phosphate

This, in turn, causes other problems:
the high phosphate level ‘drags’ calcium from the bones, resulting in osteomalacia
low calcium: due to lack of vitamin D, high phosphate
secondary hyperparathyroidism: due to low calcium, high phosphate and low vitamin D.

Question 2

Enterobiasis features?

Answer 2

Due to organism Enterobius vermicularis.
- Common cause of pruritus ani
- Diagnosis usually made by placing scotch tape at the anus, this will trap eggs that can then be viewed microscopically.
- Treatment is with Mebendazole.

Question 2

Stool culture results cysts identified on modified acid-fast
stain?

Answer 2

Cryptosporidium parvum.

The protozoan Cryptosporidium parvum is a common cause of infectious diarrhoea. In healthy persons, it usually causes a self-limiting illness. However, in the immunocompromised it can cause severe, prolonged diarrhoea and treatment is very difficult. Cryptosporidium parvum is diagnosed by performing an acid-fast stain on faeces and this demonstrates the characteristic cysts of this microorganism.

Treatment is with metronidazole.

Question 3

Giardiasis features?

Answer 3

Giardiasis is caused by the flagellate protozoan Giardia lamblia. It is spread by the faeco-oral route.

Risk factors:
- foreign travel
- swimming/drinking water from a river or lake
- male-male sexual contact

Features:
- non-bloody diarrhoea
- steatorrhoea
- bloating, abdominal pain
- flatulence
- weight loss
- malabsorption and lactose intolerance can occur

Ix stool microscopy for trophozoite and cysts: sensitivity of around 65%

Mx Metronidazole.

Question 4

Amoebiasis features?

Answer 4

Amoebiasis is caused by Entamoeba histolytica (an amoeboid protozoan) and spread by the faecal-oral route. Amoebiasis also causes liver and colonic abscesses.

Amoebic dysentery:
- Profuse, bloody diarrhoea

Stool microscopy may show trophozoites if examined within 15 minutes or kept warm (known as a ‘hot stool’).

Treatment:
- Oral metronidazole
- A ‘luminal agent’ (to eliminate intraluminal cysts) is recommended usually as well e.g. diloxanide furoate

Question 5

Rapid development of renal failure in pt with fatigue, ankle swelling and arthralgia?

Answer 5

Crescenteric Glomerulonephritis.

This elderly woman’s serum creatinine has risen from normal to 252 µmol/L in less than 6 months. This feature, along with tiredness, arthralgia, a urinalysis that shows moderate amounts of protein and blood, and a mild anaemia makes it likely that she has an ANCA-associated vasculitis. A crescentic glomerulonephritis is the typical renal histological finding in these circumstances.

RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS is a term used to describe a rapid loss of renal function associated with the formation of epithelial crescents in the majority of glomeruli.

causes include Goodpasture’s, ANCA positive vasculitis

Question 6

Features of IgA nephropathy?

Answer 6

IgA nephropathy (AKA Berger’s disease) is the commonest cause of glomerulonephritis worldwide.
It classically presents as macroscopic haematuria in young people following an upper respiratory tract infection.

Thought to be caused by mesangial deposition of IgA immune complexes.
there is considerable pathological overlap with HSP.

Histology: Mesangial hypercellularity, positive immunofluorescence for IgA & C3

Question 7

IgA nephropathy vs post-streptococcal glomerulonephritis?

Answer 7

1. Post-streptococcal glomerulonephritis is associated with low complement levels.
2. Main symptom in post-streptococcal glomerulonephritis is proteinuria (although haematuria can occur).
3. There is typically an interval between URTI and the onset of renal problems in post-streptococcal glomerulonephritis.
   IgA nephropathy develops 1 -2 days after URTI, young male, macroscopic haematuria.

Post streptococcal GN develops 1 - 2 weeks after URTI. Associated with PROTEINURIA and low complement.

Question 8

Membranous glomerulonephritis?

Answer 8

Membranous glomerulonephritis is the commonest type of glomerulonephritis in adults and is the third most common cause of ESRF. It usually presents with nephrotic syndrome or proteinuria.

Renal biopsy demonstrates:
- Electron microscopy: the basement membrane is thickened with subepithelial electron dense deposits. This creates a ‘spike and dome’ appearance.

Mx:
1. All patients should receive an ACE inhibitor or an angiotensin II receptor blocker (ARB):
these have been shown to reduce proteinuria and improve prognosis.

2. Immunosuppression
   as many patients spontaneously improve only patient with severe or progressive disease require immunosuppression
   - corticosteroids alone have not been shown to be effective. A combination of corticosteroid + another agent such as cyclophosphamide is often used.

consider anticoagulation for high-risk patients

Question 9

FSGS features?

Answer 9

Focal segmental glomerulosclerosis (FSGS) is a cause of NEPHROTIC syndrome and chronic kidney disease. It generally presents in young adults.

Focal segmental glomerulosclerosis is noted for having a high recurrence rate in renal transplants.

Renal biopsy:
Focal and segmental sclerosis and hyalinosis on light microscopy
effacement of foot processes on electron microscopy.

Mx: Steroids +/- immunosuppressants

Question 10

Features of antiphospholipid syndrome?

Answer 10

1. Venous/arterial thrombosis
2. Recurrent miscarriages
3. Livedo reticularis
   other features: pre-eclampsia, pulmonary hypertension

• Anticardiolipin antibodies
• Anti-beta2 glycoprotein I (anti-beta2GPI) antibodies
• Lupus anticoagulant
• Thrombocytopenia
• Prolonged APTT

Mx:
- Primary thromboprophylaxis: Low-dose aspirin
- Secondary thromboprophylaxis:
initial venous thromboembolic events: lifelong warfarin with a target INR of 2-3

Question 11

19-year-old woman presented 2 weeks after having had a sore throat. She had multiple, 5-mm diameter, scaly, erythematous papules over her trunk and limbs. Dx?

Answer 11

The description of the rash, in particular the size of the lesions, scaling and widespread distribution with relative sparing of the face, is typical of guttate psoriasis.

It may be precipitated by a streptococcal infection 2-4 weeks prior to the lesions appearing.

tear drop scaly papules on the trunk and limbs.

Question 12

Guttate psoriasis vs Pityriasis Rosea??

Answer 12

GUTTATE:
- Classically preceded by a streptococcal sore throat 2-4 weeks
- Appearance ‘Tear drop’, scaly papules on the trunk and limbs

PITYRIASIS ROSEA:
- Many patients report recent respiratory tract infections but this is not common in questions.

• Herald patch followed 1-2 weeks later by multiple erythematous, slightly raised oval lesions with a fine scale confined to the outer aspects of the lesions.

May follow a characteristic distribution with the longitudinal diameters of the oval lesions running parallel to the line of Langer. This may produce a ‘fir-tree’ appearance

Question 13

Thumbprinting on AXR?

Answer 13

Thumbprinting is a radiographic sign of large bowel wall thickening, usually caused by oedema, related to an infective or inflammatory process (colitis). The normal haustra become thickened at regular intervals appearing like thumbprints projecting into the aerated lumen.

?Usually colitis

Question 14

Episodic joint pain in both knees. Uric acid is normal.
Evidence of liver impairment and diabetes.

Answer 14

The most common reason for this would be crystal arthritis, either gout or pseudogout. The serum uric acid is normal, and it can be normal in both of the conditions. The history of alcohol intake is perhaps tipping us towards gout as being a more likely possibility. However, when we get further information that this man also has metacarpophalangeal involvement with hepatosplenomegaly and type 2 diabetes mellitus, it does point us in the direction of this being more systemic problem.

Going through the options, the only plausible one is haemochromatosis, which can produce an episodic arthritis of this type.
haemochromatosis leading to chondrocalcinosis and pseudogout.

Question 15

Mx for hypertriglyceridaemia?

Answer 15

Fibrates are generally used first-line.
Ciprofibrate!

• Ezetimibe: Ezetimibe inhibits the intestinal absorption of cholesterol.
  If used alone, it has a modest effect on lowering LDL-cholesterol.
• Nicotinic acid: It lowers both cholesterol and triglyceride concentrations by inhibiting synthesis; it also increases HDL-cholesterol.

Statin - Mainly for high cholesterol?

Question 16

What is Psuedomyxoma peritonei?

Answer 16

Pseudomyxoma peritonei is a rare mucinous tumour most commonly arising from the appendix. The disease is characterised by the accumulation of large amounts of mucinous material in the abdominal cavity.

CT findings would show multiple low attenuating lesions scalloping the liver margin.

Question 17

Features of Cholesterol embolisation?

Answer 17

1. Eosinophilia
2. Purpura
3. Renal failure
4. Livedo reticularis

The patient has low levels of anticardiolipin antibodies, but these are present in up to 10% of the general population and are not diagnostic of the antiphospholipid antibody syndrome.
In addition, antiphospholipid antibody syndrome does not usually cause skin ulceration.

Calciphylaxis usually starts as tender violaceous subcutaneous nodules which progress to necrotic ulceration.
Calciphylaxis is a rare complication of ESRF. It results in deposition of calcium within arterioles causing microvascular occlusion and necrosis of the supplied tissue. It most commonly affects the skin and presents with painful necrotic skin lesions. Warfarin is widely reported as causing/exacerbating calciphylaxis in high risk patients,

Coumarin necrosis typically occurs within 2 weeks of starting warfarin.
(warfarin induced skin necrosis)

Thromboembolism from arteriovenous fistula would not cause skin changes in the trunk and legs.

Question 18

Mx for Pyoderma gangrenosum?

Answer 18

The image shows well-demarcated, full-thickness skin ulceration around the stoma. There is a little slough peripherally and no overt features of bacterial infection. The ulcer edges have a punched-out appearance and the wound edges reveal violaceous erythema.

These are features of pyoderma gangrenosum, which is a recognised complication of Crohn’s disease. Oral prednisolone is first line.

Question 19

Anti-thyroid peroxidase antibodies?

Answer 19

Usually seen in Hashimoto’s thyroiditis.
sometimes raised in Graves’ disease

Question 20

24 hour ECG findings.

Answer 20

The short burst of atrial fibrillation is a common finding in the elderly and is not the same as PAF (which would require at least 30 seconds) and so is not an indication for oral anticoagulation. A permanent pacemaker would be only indicated for a pause of greater than 3 seconds in someone who is awake with or without symptoms or for a pause of less than 3 seconds in someone with definite symptoms.

Question 21

Features of McArdle syndrome?

Answer 21

Glycogen storage disease, this causes decreased muscle glycogenolysis.

1. Muscle pain and stiffness following exercise
2. Muscle cramps
3. Myoglobinuria
4. Low lactate levels during exercise

Question 22

Pt presenting with fasciculations all over with normal power and sensation?

Answer 22

Benign fasciculations.

In motor neurone disease, fasciculations are associated with weakness and the clinical disorder presents in ONE LIMB, not all over. There may be fasciculation and weakness in one limb and some fasciculations elsewhere, but not as widespread as this.

McArdle syndrome causes cramps on initial exertion, but no fasciculations; myotonic dystrophy has delayed relaxation following sustained exertion but no fasciculations and polymyositis has neither cramps nor fasciculations.

Question 23

Aspiration pneumonia commonly affects which lobe?

Answer 23

Right lower-zone opacification.
The right lower lung lobe is the most common site of infiltrate formation due to the larger caliber and more vertical orientation of the right mainstem bronchus.

Question 24

What is organising pneumonia?

Answer 24

Cryptogenic organising pneumonia (COP) is a non-infectious pneumonic process occurring in the context of pre-existing inflammatory or autoimmune conditions such as rheumatoid arthritis and it can mimic bacterial pneumonia in its X-ray appearance. Crackles are usually present and inflammatory markers such as the ESR are raised. It responds to corticosteroids rather than antibiotics.

This woman has bilateral mid- and lower-zone findings on examination and on the chest X-ray. Tuberculosis usually presents with unilateral or bilateral upper-zone cavitation/consolidation.

Rheumatoid lung disease tends to give bilateral basal opacification on a chest X-ray, often reticulonodular in character (honeycomb pattern on high-resolution CT scan of chest).

Question 25

Keratoderma blenorrhagica is seen in which condition?

Answer 25

Reactive arthritis.

Gonococcal arthritis is unlikely as it is usually a migratory polyarthritis.

Syphilis is not usually associated with arthritis, and involvement of the palms and soles is a feature of secondary syphilis.

Question 26

Features of subacute combined degeneration of the spinal cord?

Answer 26

Subacute combined degeneration of the spinal cord is due to vitamin B12 deficiency resulting in impairment of the dorsal columns, lateral corticospinal tracts and spinocerebellar tracts.

1. Dorsal column involvement:
   - Distal tingling/burning/sensory loss is symmetrical and tends to affect the legs more than the arms
   - Impaired proprioception and vibration sense
2. Lateral corticospinal tract involvement
   - muscle weakness, hyperreflexia, and spasticity
   - upper motor neuron signs typically develop in the legs first
   - brisk knee reflexes
   - absent ankle jerks
   - extensor plantars
3. Spinocerebellar tract involvement
   - sensory ataxia → gait abnormalities
   - positive Romberg’s sign

Question 27

Diabetic Amyotrophy?

Answer 27

Diabetic amyotrophy is also known as proximal diabetic neuropathy.

Typical features include:
1. Pain is usually in the first symptom, often in the hips or buttocks

2. This is followed by weakness, for example difficulty getting out of a chair

Question 28

Features of syringomyelia?

Answer 28

Syringomyelia (‘syrinx’ for short) describes a collection of cerebrospinal fluid within the spinal cord.

Causes:
- Chiari malformation (portion of the cerebellum and/or brainstem pushing out through a defect in the back of the head or neck).
- Trauma
- Tumours
- Idiopathic

Features:
1. A ‘cape-like’ (neck, shoulders and arms):
- Loss of sensation to temperature but the preservation of light touch, proprioception and vibration.
- Classic examples are of patients who accidentally burn their hands without realising.
- This is due to the crossing spinothalamic tracts in the anterior commissure of the spinal cord being the first tracts to be affected.

2. Spastic weakness (predominantly of the lower limbs)
3. Neuropathic pain
4. Upgoing plantars
5. Autonomic features:
   - Horner’s syndrome due to compression of the sympathetic chain, but this is rare
   - bowel and bladder dysfunction
   - scoliosis will occur over a matter of years if the syrinx is not treated

Question 29

Thyroid mass with previous phaechromocytoma. What Ix to perform next?

Answer 29

Phaeochromocytomas are rare tumours and are even more rarely associated with genetic conditions such as von Hippel–Lindau disease and multiple endocrine neoplasia type 2 (MEN2). The presence of a thyroid mass raises the possibility of a calcitonin-producing medullary cell tumour (MCT). Thus, the correct answer is to measure plasma calcitonin.

Question 30

Acalculous cholecystitis?

Answer 30

Acalculous cholecystitis (inflammation of the gallbladder in the absence of gallstones) typically occurs in the very ill patient on the intensive care unit, or after extensive burns. The thickened gallbladder wall and pericholecystic fluid indicate inflammation in the gallbladder wall.

Question 31

Features of microscopic polyangiitis?

Answer 31

This patient has microscopic polyangiitis which is one of the ANCA-associated vasculitides. The presence of haematuria, fever and raised inflammatory markers are frequent findings in this condition. It is most common in the elderly.

Microscopic polyangiitis is a small-vessel ANCA vasculitis.

Features:
- Renal impairment: raised creatinine, - Haematuria, proteinuria
- Fever
- Other systemic symptoms: lethargy, myalgia, weight loss
- Rash: palpable purpura
- Cough, dyspnoea, haemoptysis
mononeuritis multiplex

Investigations
- pANCA (against MPO) - positive in 50-75%
- cANCA (against PR3) - positive in 40%

Question 32

Side effects of ACE-inhibitors?

Answer 32

1. Cough:
   occurs in around 15% of patients and may occur up to a year after starting treatment. Thought to be due to increased bradykinin levels.
2. Angioedema: may occur up to a year after starting treatment
3. Hyperkalaemia

Intermittent swelling of face and tongue suggests a diagnosis of angioedema without urticaria. Angioedema can be inherited or acquired, allergic or drug-induced. Hereditary angioedema usually occurs for the first time in childhood or adolescence. Drug-induced angioedema without urticaria is most frequently associated with ACE inhibitors and, less frequently, angiotensin II receptor blockers. This represents a pharmacological effect on bradykinin metabolism rather than allergy.

Question 33

Adverse effect of Cyclophosphamide?

Answer 33

1. Haemorrhagic cystitis: incidence reduced by the use of hydration and mesna
2. Myelosuppression
3. Transitional cell carcinoma

Question 34

Cryoglobulinaemia?

Answer 34

Cryoglobulinemia is a medical condition in which the blood contains large amounts of pathological cold sensitive antibodies called cryoglobulins – proteins (mostly immunoglobulins themselves) that become insoluble at reduced temperatures.

• Raynaud’s only seen in type I
• vascular purpura
• distal ulceration
• arthralgia
• renal involvement
• diffuse glomerulonephritis

low complement (esp. C4)
high ESR

Question 35

Adherent grey membrane over soft palate and tonsils?

Answer 35

Diphtheria.

Vincent angina can also cause a pharyngeal membrane but there is usually also severe local tissue destruction.

Question 36

Lichen planus appearance?

Answer 36

Lichen planus causes purple, flat-top papules as shown in the image.

Dermatitis herpetiformis classically affects extensor aspects of limbs and lesions are vesicles or excoriations rather than papules.

Tinea corporis usually has an annular configuration and, as with atopic eczema and guttate psoriasis usually produces scale.

Question 37

Myotonic dystrophy?

Answer 37

General features
- Myotonic facies (long, ‘haggard’ appearance)
- Frontal balding
- Bilateral ptosis
- Cataracts
- Dysarthria

Other features:
- Myotonia (tonic spasm of muscle)
- Weakness of arms and legs (distal initially)
- Mild mental impairment
- Diabetes mellitus
- Testicular atrophy
- Cardiac involvement: heart block, cardiomyopathy
- Dysphagia

Bilateral ptosis, facial weakness, distal weakness!!!!

Question 38

Features of Myasthenia Gravis?

Answer 38

Myasthenia gravis is an autoimmune disorder resulting in insufficient functioning acetylcholine receptors.

The key feature is muscle fatigability!! - Muscles become progressively weaker during periods of activity and slowly improve after periods of rest!

• Extraocular muscle weakness
• Diplopia
• Proximal muscle weakness: face, neck, limb girdle
• Ptosis
• Dysphagia

Associations:
1. Thymomas in 15%
2. Autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, rheumatoid, SLE

Ix:
- Single fibre electromyography: high sensitivity (92-100%)
- CT thorax to exclude thymoma
- CK normal
- Antibodies to acetylcholine receptors

Mx: Long-acting acetylcholinesterase inhibitors, Pyridostigmine is first-line

Myasthenic crisis:
- Plasmapheresis
- Intravenous immunoglobulins

Question 39

Features of MND?

Answer 39

Present with both UMN and LMN signs.

1. Asymmetric limb weakness is the most common presentation of ALS.
2. The mixture of lower motor neuron and upper motor neuron signs.
3. Wasting of the small hand muscles/tibialis anterior is common.
4. Fasciculations
5. Absence of sensory signs/symptoms!!

• Doesn’t affect external ocular muscles
• No cerebellar signs

Mx: Riluzole, NIV, PEG

Question 40

Miller Fisher syndrome?

Answer 40

Variant of Guillain-Barre syndrome
associated with ophthalmoplegia, areflexia and ataxia.

• The eye muscles are typically affected first
• usually presents as a descending paralysis rather than ascending as seen in other forms of Guillain-Barre syndrome
• anti-GQ1b antibodies are present in 90% of cases

Question 41

Features of GBS?

Answer 41

Guillain-Barre syndrome describes an immune-mediated demyelination of the PERIPHERAL NERVOUS SYSTEM often triggered by an infection (classically Campylobacter jejuni).

• Progressive, symmetrical weakness of all the limbs.
• Weakness is classically ascending i.e. the legs are affected first.
• Reflexes are reduced or absent.
• Sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs.
• Respiratory muscle weakness
• Cranial nerve involvement: Diplopia,
  bilateral facial nerve palsy,
  oropharyngeal weakness is common
• Urinary retention, diarrhoea

LP: Rise in protein with a normal WCC.

Nerve conduction studies:
Decreased motor nerve conduction velocity (due to demyelination)

Mx: IVIG

FVC regularly to monitor respiratory function

Question 42

Features of idiopathic intracranial hypertension?

Answer 42

Idiopathic intracranial hypertension is a condition classically seen in young, overweight females.

Risk factors:
Obesity
Female sex
Pregnancy
Drugs:
- COCP
- steroids
- tetracyclines
- retinoids
- lithium

• Headache
• Blurred vision
• Papilloedema (usually present)
• Enlarged blind spot
• Sixth nerve palsy may be present

Mx:
1. Carbonic anhydrase inhibitors e.g. Acetazolamide
2. Topiramate is also used, and has the added benefit of causing weight loss in most patients
3. Repeated lumbar puncture may be used as a temporary measure but is not suitable for longer-term management

Question 43

Features of cerebral venous thrombosis?

Answer 43

Cerebral venous thrombosis (CVT) is a neurological emergency. It is a rare cause of cerebral infarction resulting from thromboembolic occlusion of cerebral veins and sinuses.

CVT typically affects younger individuals when compared to arterial strokes (median age of CVT onset is 37 years) . It is 3 times more common in females, likely as a result of increased risk of thromboembolisms in pregnancy and with oral contraceptive use.

Question 44

Malignant hyperthermia features?

Answer 44

Condition often seen following administration of anaesthetic agents
characterised by hyperpyrexia and muscle rigidity.

• Halothane
• Suxamethonium

Question 45

Serotonin syndrome vs Neuroleptic Malignant Syndrome?

Answer 45

There is no drug history to tease out the answer, but serotonin syndrome is more likely to present with shivering, hyper-reflexia and clonus (NMS classically has lead-pipe rigidity).

Question 46

How to calculate Anion Gap and normal value?

Answer 46

The anion gap is calculated by:

(sodium + potassium) - (bicarbonate + chloride)

A normal anion gap is 8-14 mmol/L.

Question 47

Causes of a normal anion gap or hyperchloraemic metabolic acidosis?

Answer 47

• Gastrointestinal bicarbonate loss: diarrhoea, ureterosigmoidostomy, fistula
• Renal tubular acidosis
• Drugs: e.g. acetazolamide
• Ammonium chloride injection
• Addison’s disease

Question 48

Causes of a raised anion gap metabolic acidosis?

Answer 48

• Lactate: shock, hypoxia
• Ketones: diabetic ketoacidosis, alcohol
• Urate: renal failure
• Acid poisoning: Salicylates, Methanol
  5-oxoproline: chronic paracetamol use

MUDPILES.

Question 49

Antihypertensive in pregnancy?

Answer 49

The patient has gestational hypertension and NICE guidelines (Hypertension in Pregnancy 2010) recommend labetalol to achieve a target BP of systolic <150 mmHg and diastolic BP between 80 and 100 mmHg. The NICE guideline suggests methyldopa and nifedipine as alternatives to labetalol.

ACE inhibitors, angiotensin receptor blocking drugs (ARBs) and thiazide diuretics can all be associated with an increased risk of fetal abnormality.

Question 50

Granulomatosis with polyangiitis?

Answer 50

1. Upper respiratory tract: epistaxis, sinusitis, nasal crusting
2. Lower respiratory tract: dyspnoea, haemoptysis
3. Rapidly progressive glomerulonephritis (‘pauci-immune’, 80% of patients)
4. Saddle-shape nose deformity
5. Also: vasculitic rash, eye involvement (e.g. proptosis), cranial nerve lesions

cANCA positive in > 90%, pANCA positive in 25%

CT scan: Cavitating Lesions!!

Question 51

Features of Churgg Straus, Eosinophilic granulomatosis with polyangiitis?

Answer 51

1. Asthma
2. Blood eosinophilia (e.g. > 10%)
3. Paranasal sinusitis
4. Mononeuritis multiplex
5. pANCA positive in 60%

Question 52

Features of multiple myeloma?

Answer 52

CRABBI

Calcium:
- Hypercalcaemia due primarily to increased osteoclastic bone resorption. Leads to constipation, nausea, anorexia and confusion.

Renal:
- Monoclonal production of immunoglobulins results in light chain deposition within the renal tubules which causes renal damage.

Anaemia:
- Bone marrow crowding suppresses erythropoiesis leading to anaemia
this causes fatigue and pallor.

Bleeding:
- Bone marrow crowding also results in thrombocytopenia which puts patients at increased risk of bleeding and bruising.

Bones:
- Bone marrow infiltration by plasma cells and cytokine-mediated osteoclast overactivity creates lytic bone lesions. This may present as pain (especially in the back) and increases the risk of pathological fractures.

Infection
- A reduction in the production of normal immunoglobulins results in increased susceptibility to infection.

Question 53

Prokinetic anti-emetic?

Answer 53

A succussion splash suggests delayed gastric emptying and the endoscopy findings suggest that this is due to gastroparesis rather than gastric outlet obstruction.

Of the three antiemetics offered, only metoclopramide has prokinetic effects, and so this would be the first choice. Although corticosteroids may help all these symptoms, the risk of cumulative adverse effects would mean that this would not be the first choice of treatment without trying antiemetics first.

Question 54

Cushing causes?

Answer 54

1. ACTH dependent causes:
   - Cushing’s disease (80%): pituitary tumour secreting ACTH producing adrenal hyperplasia.
   - Ectopic ACTH production (5-10%): e.g. small cell lung cancer is the most common causes.
2. ACTH independent causes:
   - Iatrogenic: steroids
   - Adrenal adenoma (5-10%)
   - Adrenal carcinoma (rare)
3. Pseudo-Cushing
   - mimics Cushing’s
   - often due to alcohol excess or severe depression
   - causes false positive dexamethasone suppression test or 24 hr urinary free cortisol
   - insulin stress test may be used to differentiate

Question 55

Which glomerulonephritides responds best to steroids?

Answer 55

Minimal Change disease.

1. Nephrotic syndrome
2. Highly selective proteinuria
   only intermediate-sized proteins such as albumin and transferrin leak through the glomerulus.
3. Renal biopsy:
   normal glomeruli on light microscopy
   electron microscopy shows fusion of podocytes and effacement of foot processes

Oral corticosteroids: majority of cases (80%) are steroid-responsive
cyclophosphamide is the next step for steroid-resistant cases.

Question 56

Ix for Pemphigus Vulgaris?

Answer 56

The definitive diagnostic test for pemphigus vulgaris is direct immunofluorescence of skin.

Electron microscopy could be used in the diagnosis of epidermolysis bullosa, which would present at a younger age.

Question 57

Renal transplant patient presents with firm keratotic nodule on the back of his right hand. Diagnosis and management?

Answer 57

A short history of a rapidly growing keratotic nodule, on a sun-exposed site, in an immunosuppressed patient, points strongly to a diagnosis of squamous cell carcinoma (SCC). The optimum management of SCC is surgical excision and histological analysis to confirm diagnosis and ensure complete removal. Cryotherapy and laser therapy would neither guarantee removal, nor provide histological proof of clearance. Reduced immunosuppression and topical keratolytic therapy would be ineffective.

Question 58

Systemic treatment for Pityriasis verisolor not responsive to topical?

Answer 58

The asymptomatic, fine, SCALY, reddish-brown, macular, coalescent rash over the upper trunk is typical of pityriasis versicolor caused by the yeast Malassezia furfur.

The only therapy listed that is effective against yeast is Itraconazole.

Question 59

Mx for Prolactinoma?

Answer 59

This patient has a macroprolactinoma as evidenced by the very high prolactin levels. First-line treatment of these tumours is dopamine agonist therapy followed by surgery (usually transfrontal). Radiotherapy treatments are reserved for patients who are either intolerant or resistant to dopamine agonists. The tumours can start to shrink rapidly after initiation of treatment, with visual field improvements discernible within days and visible shrinkage evident on imaging within weeks. Normalisation of prolactin levels occur in up to 90% of all prolactinoma patients.

Remember that non-prolactin secreting pituitary tumours can cause elevation of prolactin levels by compressing the pituitary stalk and preventing delivery of dopamine, which usually inhibits prolactin production. Prolactin levels are generally between 600–3000 mU/L in this situation, not as high as in macroprolactinoma.

Question 60

Triad of normal pressure hydrocephalus?

Answer 60

1. Urinary incontinence
2. Dementia and bradyphrenia
3. Gait abnormality (may be similar to Parkinson’s disease)

Secondary to reduced CSF absorption at the arachnoid villi. Symptoms typically develop over a few months.

CT: Hydrocephalus with ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement.

Mx: Ventriculoperitoneal shunting

Question 61

Types of dressings?

Answer 61

A hydrocolloid dressing will maintain tissue integrity.

Alginate dressings are useful in exudative wounds.

Antibacterial and silver-impregnated dressings are indicated only when there are signs of local infection.

Hydrocolloid dressings are used for more advanced ulcers, where they are used to pack the wound.

Question 62

Switch to what blood thinner for HIT?

Answer 62

This patient has developed a deep vein thrombosis despite prophylactic heparin. His platelet count has also fallen by more than 50% of baseline level. These occurred 7 days after starting heparin. These clinical findings are typical of heparin-induced thrombocytopenia (HIT).

HIT is caused by the development of IgG antibodies directed against a complex of platelet factor 4 (PF4) and heparin. These antibodies activate platelets leading to thrombosis despite thrombocytopenia. It is more common with the use of unfractionated heparin than low-molecular-weight heparin. If the platelet count falls by 30% or more and/or the patient develops new thrombosis or redness at the heparin injection sites between days 4 and 14 of heparin administration, HIT should be considered.

The treatment of HIT is to stop any form of heparin and anticoagulate with argatroban or danaparoid. Direct oral anticoagulants are not licensed for use in HIT and should be avoided in chronic kidney disease stage 4. Warfarin should not be used until the platelet count has recovered to the normal range. When introduced, argatroban or danaparoid must be continued until the INR is therapeutic.

Question 63

Features of HIT?

Answer 63

• Immune mediated - antibodies form against complexes of platelet factor 4 (PF4) and heparin.
• Usually does not develop until after 5-10 days of treatment.
• Despite being associated with low platelets HIT is actually a prothrombotic condition.
• Features include a greater than 50% reduction in platelets, thrombosis and skin allergy.
• Address need for ongoing anticoagulation:
  Direct thrombin inhibitor e.g. Argatroban
  Danaparoid

Question 64

Treatment for CLL?

Answer 64

The most common cause of an isolated lymphocytosis in an adult is chronic lymphocytic leukaemia (CLL). The diagnosis is usually made incidentally from a full blood count checked for another reason. The diagnosis is confirmed by immunophenotyping of peripheral blood white cells. It is a smouldering disease and incurable. Treatment of asymptomatic early cases has not improved life expectancy. Many patients, therefore, do not require anti-leukaemia treatment but active monitoring only.

The indications for anti-leukaemia treatment in CLL are constitutional symptoms such as weight loss, fever or night sweats, bone marrow failure manifest by anaemia and thrombocytopenia and symptomatic enlargement of lymph nodes, spleen or liver. This patient is well and does not have any indication for treatment.

If treatment is indicated for this patient, anti-CD20 monoclonal antibody (e.g. obinutuzumab), chlorambucil or bendamustine is appropriate. Ibrutinib, which is an inhibitor of Bruton’s tyrosine kinase (BTK), is used only if there is TP53 mutation. Corticosteroids are used only for autoimmune cytopenias (e.g. autoimmune thrombocytopenic purpura) associated with the underlying leukaemia. Splenectomy is not an option for treatment of CLL.

Question 65

TTP?

Answer 65

This patient has anaemia and thrombocytopenia with fragmentation of red cells apparent in blood film. This is suggestive of a microangiopathic haemolytic anaemia. Given the history of fever and headaches, no significant renal impairment and normal coagulation screen, the diagnosis is most likely to be thrombotic thrombocytopenic purpura (TTP). Raised serum lactate dehydrogenase is a feature of TTP and can be used to guide response to treatment. TTP typically presents with neurological symptoms and signs such as headaches, seizures, altered consciousness or focal neurological deficits.

Haemolytic uraemic syndrome (HUS) and TTP may overlap. However, a classic case of HUS would present with severe acute kidney injury typically following a bout of bloody diarrhoea caused by Escherichia coli O157.

Immune thrombocytopenic purpura is unlikely here as it does not cause fever or headaches, and is not associated with red cell fragmentation.

Disseminated intravascular coagulation (DIC) can be excluded here by normal coagulation screen.

Acute myeloid leukemia is the least likely cause of this patient’s thrombocytopenia as the patient’s neutrophil count is normal and blood film shows no abnormal white cells. DIC may be a feature of acute myeloid leukaemia but the patient does not have DIC.

Question 66

Infective endocarditis organism associated with bowel cancer?

Answer 66

Streptococcus gallolyticus is part of the normal large bowel flora. In patients with bowel pathology, such as a colonic cancer, this organism can cause transient bacteraemia and this can lead to endocarditis. Any patient with endocarditis caused by this organism should have large-bowel investigations (e.g. a CT scan of abdomen or colonoscopy).

Streptococcus agalactiae (sometimes described as group B streptococcus) is associated with the genital tract.

Streptococcus milleri causes liver and brain abscesses.

Streptococcus pyogenes causes pharyngitis and cellulitis.

Streptococcus viridans can cause endocarditis but is not usually associated with bowel pathology.

Question 67

Treatment for C.Diff?

Answer 67

If a patient with Clostridium difficile infection is failing to respond to oral metronidazole, the treatment should be changed to oral vancomycin.

Intravenous vancomycin is not effective for Clostridium difficile infection as it does not cross the bowel wall.

Intravenous metronidazole is used as an addition to oral vancomycin for patients with complicated or life-threatening disease where there is concern that the vancomycin may be delivered too slowly to the colon.

Oral lactobacillus is known as probiotic therapy. This can be used as an adjunctive therapy but is of uncertain benefit.

Question 68

Organism responsible for causing meningitis from ear infection?

Answer 68

This patient has meningitis complicating an ear infection.

This clinical situation is nearly always due to Streptococcus pneumoniae. The very high protein is consistent with Streptococcus pneumoniae infection or Mycobacterium tuberculosis infection.

Haemophilus influenzae and Neisseria meningitides also cause bacterial meningitis but there is usually no preceding history of sinusitis or ear infection.

Listeria spp. causes a lymphocytic meningitis in the elderly and immunocompromised.

Mycobacterium tuberculosis causes chronic lymphocytic meningitis.

Question 69

Tx for Herpes Simplex Encephalitis?

Answer 69

MRI scan shows evidence of increased signal in the left temporal lobe, confined predominantly to the grey matter, which is very suggestive for herpes simplex encephalitis.

CSF examination shows an increased white cell count, predominantly of lymphocytes. Herpes simplex meningo-encephalitis is the most likely diagnosis.

The treatment is aciclovir.

Question 70

Features of herpes simplex encephalitis?

Answer 70

The virus characteristically affects the temporal lobes - questions may give the result of imaging or describe temporal lobe signs e.g. Aphasia.

1. Fever, headache, psychiatric symptoms, seizures, vomiting
2. Focal features e.g. aphasia
3. Peripheral lesions (e.g. cold sores) have no relation to the presence of HSV encephalitis

HSV-1 is responsible for 95% of cases in adults.
- Typically affects temporal and inferior frontal lobes

Ix:
CSF: lymphocytosis, elevated protein

CT: medial temporal and inferior frontal changes (e.g. petechial haemorrhages) - normal in one-third of patients.

Question 71

weakness of his left triceps and extension of left metacarpophalangeal joints. There was a reduced left triceps tendon reflex. He had sensory impairment, mainly in the third digit of his left hand, but also, to a lesser extent, in his index and ring fingers.

Answer 71

ANS: C7 Nerve root Compression

• Axillary nerve compression gives deltoid weakness and a patch of sensory disturbance over the upper outer arm (not hand).
• Medial trunk compression gives medial forearm pain and ulnar sensory disturbance (not more lateral hand and no triceps weakness).
• Radial nerve weakness gives wrist drop

Question 72

Patient present with diplopia and respiratory involvement. Anti acetylcholine receptor antibodies are negative.

Answer 72

This patient has presented with ptosis, diplopia, bulbar symptoms and respiratory involvement. In conjunction with a positive edrophonium chloride test, myasthenia gravis is the most likely diagnosis.

In botulism, patients commonly have dilated or fixed pupils.

Miller Fisher syndrome is characterised by the clinical triad of ophthalmoplegia, ataxia and areflexia.

In organophosphate poisoning, there is diaphoresis and diarrhoea, miosis, bradycardia, bronchospasm, bronchorrhoea, nausea and vomiting, excess lacrimation, hypersalivation, anxiety, emotional lability, restlessness, confusion, ataxia, tremors, seizures, and coma.

The creatine kinase level is usually in the thousands in polymyositis.

Question 73

Mx for Cluster headache?

Answer 73

The name relates to the pattern of the headaches - they typically occur in clusters lasting several weeks, with the clusters themselves typically once a year.

Cluster headaches are more common in men (3:1) and smokers. Alcohol may trigger an attack and there also appears to be a relation to nocturnal sleep.

• Intense sharp, stabbing pain around one eye.
• Pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours
• Patient is restless and agitated during an attack due to the severity
  clusters typically last 4-12 weeks
• Accompanied by redness, lacrimation, lid swelling
• Nasal stuffiness
• Miosis and ptosis in a minority

Investigations
- underlying brain lesions are sometimes found even if the clinical symptoms are typical for cluster headache
- MRI with gadolinium contrast is the investigation of choice

Management
Acute
1. 100% oxygen (80% response rate within 15 minutes)
2. Subcutaneous triptan (75% response rate within 15 minutes)

Prophylaxis:
- Verapamil is the drug of choice
- There is also some evidence to support a tapering dose of prednisolone

Question 74

Mx for systemic sclerosis renal crisis?

Answer 74

This woman is having a systemic sclerosis renal crisis, which is characterised by very marked overactivation of the renin angiotensin aldosterone system. Therefore, an ACE inhibitor is the drug of choice, of which ramipril is the only option from the choices given.

Question 75

Triad of HUS?

Answer 75

1. Acute kidney injury
2. Microangiopathic haemolytic anaemia
3. Thrombocytopenia

Fragmented blood film: schistocytes and helmet cells

Question 76

Refeeding syndrome blood abnormalities?

Answer 76

1. Hypophosphataemia:
   This is the hallmark symptom of refeeding syndrome.
   may result in significant muscle weakness, including myocardial muscle (→ cardiac failure) and the diaphragm (→ respiratory failure)
2. Hypokalaemia
3. Hypomagnesaemia: may predispose to torsades de pointes
4. Abnormal fluid balance

Question 77

Features of Acute interstitial nephritis?

Answer 77

Acute interstitial nephritis accounts for 25% of drug-induced acute kidney injury.

Causes
Drugs: the most common cause, particularly antibiotics
- penicillin
- rifampicin
- NSAIDs
- allopurinol
- furosemide
- systemic disease: SLE, sarcoidosis, and Sjögren’s syndrome
- infection: Hanta virus , staphylococci

Fever, rash, arthralgia
Eosinophilia
Mild renal impairment
Hypertension

Investigations:
- sterile pyuria
- white cell casts

Question 78

Urine electrophoresis shows free lambda light chains with normal serum protein electrophoresis?

Answer 78

He has free lambda light chains in his urine and this in conjunction with his presentation, makes AL amyloidosis the most likely diagnosis. Some patients with AL amyloid will have an underlying diagnosis of multiple myeloma but the fact that this patient does not have hypercalcaemia nor a serum paraprotein means that AL amyloidosis is a more likely diagnosis than multiple myeloma.

Question 79

Long term pharmacological management for patient presenting with raised urinary calcium and renal stone?

Answer 79

Bendroflumethiazide.
This man has developed renal calculi in the context of hypercalcuria. Bendroflumethiazide reduces urinary calcium excretion

• Allopurinol for Urate stones.
• Penicillamine is for the treatment of cystinuria).
• Furosemide increases urinary calcium excretion.
• A long-term low-calcium diet would have a detrimental effect on his bone health.

Question 80

Prevention of renal stones?

Answer 80

1. Calcium stones may be due to hypercalciuria, which is found in up to 5-10% of the general population.
   - High fluid intake
   - Add lemon juice to drinking water
   - Avoid carbonated drinks
   - Limit salt intake
   - Potassium citrate may be beneficial
   - Thiazides diuretics (increase distal tubular calcium resorption)
2. Oxalate stones
   - Cholestyramine reduces urinary oxalate secretion.
   - Pyridoxine reduces urinary oxalate secretion.
3. Uric acid stones
   - Allopurinol
   - Urinary alkalinization e.g. oral bicarbonate

Question 81

Features of Allergic bronchopulmonary aspergillosis?

Answer 81

Allergic bronchopulmonary aspergillosis results from an allergy to Aspergillus spores. In the exam questions often give a history of bronchiectasis and eosinophilia.

• Eosinophilia
• Flitting CXR changes
• Positive radioallergosorbent (RAST) test to Aspergillus
• Positive IgG precipitins (not as positive as in aspergilloma)
• Raised IgE

Mx: Oral glucorticoids

Only allergic bronchopulmonary aspergillosis and eosinophilic granulomatosis with polyangiitis could explain the asthmatic features (wheeze) in the context of an eosinophilia, raised IgE and pulmonary infiltrates on the chest X-ray.

Question 82

Ix for Sarcoidosis?

Answer 82

Sarcoidosis is a systemic granulomatous disease of unknown aetiology, characterized by the formation of non-caseating granulomas, which can affect any organ. However, the lungs and intrathoracic lymph nodes are the most commonly involved sites.

Sarcoidosis is around 3 times more common in people of Black African ethnicity.

The most common symptoms include cough, dyspnea, chest pain, and constitutional symptoms such as fever, fatigue, and weight loss. The involvement of other organs can lead to various manifestations, including uveitis, peripheral neuropathy, cardiac arrhythmias, and hepatic dysfunction. Skin manifestations, such as erythema nodosum and lupus pernio, are also common.

ACE levels have a sensitivity of 60% and specificity of 70% and are therefore not reliable in the diagnosis of sarcoidosis although they may have a role in monitoring disease activity.

Spirometry: may show a restrictive defect.

Transbronchial needle aspiration!!
Tissue biopsy: non-caseating granulomas.*

• Gallium-67 scan - not used routinely
• Kveim test (where part of the spleen from a patient with known sarcoidosis is injected under the skin) is no longer performed due to concerns about cross-infection.

Question 83

What is Lofgren’s syndrome?

Answer 83

Lofgren’s syndrome is an acute form of the disease characterised by bilateral hilar lymphadenopathy (BHL), Erythema nodosum, Fever and Polyarthralgia.
It usually carries an excellent prognosis.

Question 84

Antibody in polymyositis patient?

Answer 84

Anti-Jo-1. Anti-Jo-1 antibody is a myositis-specific antibody.

Anticentromere antibody is specific for limited cutaneous systemic sclerosis.

Anti-double-stranded DNA antibody is specific for systemic lupus erythematosus.

Anti-topoisomerase antibodies (Scl-70) are found in some patients with systemic sclerosis.

Anti-myeloperoxidase is found in patients with ANCA-associated vasculitis.

Question 85

Antihypertensive for pregnant patient or women planning for pregnancy?

Answer 85

1. Labetalol is first-line therapy
2. Alternatives include Methyldopa and Nifedipine.

ACE inhibitors and angiotensin receptor blockers can cause congenital abnormalities.

Atenolol has been linked to intrauterine growth restriction.

Question 86

Female patient on warfarin for recurrent DVTs who is pregnant. What to do about anticoagulation?

Answer 86

Substitute to LMWH until term!

Warfarin is teratogenic and crosses the placenta with risk of congenital malformations, and placental, fetal, or neonatal haemorrhage, especially during the last few weeks of pregnancy and at delivery. Therefore, warfarin should be avoided in pregnancy, especially in the first and third trimesters (BNF).

Fondaparinux is not advised to be used in pregnancy.

Low-molecular-weight heparin is preferred to unfractioned heparin as it less likely to cause osteoporosis and heparin-induced thrombocytopenia.