Medicine Flashcards
1
Q
Pellagra is caused by deficiency of…
A
Niacin/ vitamin B3
2
Q
Features of pellagra
A
Dermatitis, diarrhoea, and mental disturbance
Niacin/ vitamin B3 deficiency
3
Q
Commonest cause of nephrotic syndrome in children
A
Minimal change disease
4
Q
Minimal change disease biopsy findings
A
Abnormal fused podocytes on EM
Normal light microscopy
5
Q
Signs/symptoms of minimal change disease
A
Associated with URTI
6
Q
Components of the blatchford score
A
Blood urea
Hb
Systolic BP
Others: Malaena Syncope Pulse Hepatic disease Cardiac failure
0 identifies low-risk patients who might be suitable for outpatient management
7
Q
Scores used in assessment of GI bleeding
A
Rockall score
Glasgow-Blatchford score
8
Q
Components of full Rockall score
A
Age
Shock
Comorbidities: e.g. CHF, IHD, major morbidity, renal failure
Diagnosis: e.g. mallory weiss or malignancy
Evidence of bleeding: e.g. spurting or visible vessel
9
Q
3 key features of nephrotic syndrome
A
Proteinuria: >3g/24h
Hypoalbuminaemia: <35g/L
Oedema: periorbital, ascites, genital, peripheral
Also hyperlipidaemia
10
Q
Key features of post-streptococcal acute glomerulonephritis
A
Young child
Smoky urine
Develops 1-2 weeks after sore throat/skin infection
Increased ASOT
Reduced C3
IgG and C3 deposition on biopsy
Most resolve, minority develop rapidly progressing glomerulonephritis
11
Q
3 types of rapid progressing glomerulonephritis
A
Type 1: Anti-GBM/ good pastures (only 5%)
Type 2: Immune complex deposition
Type 3: Pauci immune (50%) e.g. Wegener’s
12
Q
Examples of type 3 rapid progressing glomerulonephritis
A
Wegener’s (cANCA)
Microscopic polyangiitis (pANCA)
Churg-Strauss (pANCA)
13
Q
Treatment for Torsades de Pointes
A
IV MgSO4
14
Q
TSH and T4 levels in primary hypothyroidism
A
TSH High
T4 low
(if both low it is secondary)
15
Q
What is Corrigan’s sign?
A
Carotid pulsation seen in patients with aortic regurgitation
16
Q
Factors that increase BNP levels
A
Left ventricular hypertrophy
Ischaemia Tachycardia Right ventricular overload Hypoxaemia (including pulmonary embolism) GFR < 60 ml/min Sepsis COPD Diabetes Age > 70 Liver cirrhosis
17
Q
Factors that reduce BNP levels
A
Obesity Diuretics ACE inhibitors Beta-blockers Angiotensin 2 receptor blockers Aldosterone antagonists
18
Q
Cardiac features of Marfan’s syndrome
A
Dilation of the aortic sinuses (seen in 90%) which may lead to aortic aneurysm
Aortic dissection (leading cause of death)
Aortic regurgitation
Mitral valve prolapse (75%)
19
Q
Normal ECG variants in athletes
A
sinus bradycardia
junctional rhythm
first degree heart block
Wenckebach phenomenon
20
Q
Features of SIRS
A
at least 2 of the following:
body temperature less than 36°C or greater than 38.3°C
heart rate greater than 90/min
respiratory rate greater than 20 breaths per minute
blood glucose > 7.7mmol/L in the absence of known diabetes
white cell count less than 4 or greater than 12
21
Q
Key features of HHS
A
- ) Severe hyperglycaemia
- ) Dehydration and renal failure
- ) Mild/absent ketonuria
Confirmation of HHS:
Dehydration
Osmolality >320mosmol/kg
Hyperglycaemia >30 mmol/L with pH >7.3, bicarbonate >15mmolL and no significant ketonenaemia <3mmol/L
22
Q
Precipitants of HHS
A
New diagnosis of type 2 diabetes Infection High dose steroids Myocardial infarction Vomiting Stroke Thromboembolism Poor treatment compliance
23
Q
Presentation of PBC (key features)
A
Usually Middle aged females (>50)
Jaundice occurs late (survival <2 yrs after jaundice)
Pruritus
Pigmentation of face
Bones: osteomalacia, osteoporosis (reduced vit D)
Big organs: Hepatosplenomegaly
Cirrhosis and coagulopathy (reduced Vit K)
Cholesterol raised: xanthelasma, xanthomata
Steatorrhhoea
24
Q
Treatment of PBC
A
Symptoms
Pruritus: colestyramine, naltrexone
Diarrhoea: codeine
Osteoporosis: Bisphosphonates
Specific
ADEK vitamins
Ursodeoxycholic acid (improves LFTs but no effect on mortality or transplant need).
Transplant
25
Treatment of PSC
Symptoms
Pruritus: colestyramine, naltrexone
Diarrhoea: codeine
Specific
ADEK vitamins
Ursodeoxycholic acid (improves cholestasis only)
Abx for cholangitis
Endoscopic stenting for dominant strictures
Transplant
26
Key differences in pathology of PSC and PBC
PSC involves intra- and extra-hepatic ducts, PBC only involves intrahepatic ducts
PSC: Bile duct destroyed by chronic granulomatous inflammation leading to cirrhosis
PBC: chronic biliary obstruction leads to secondary biliary cirrhosis
Biopsy:
PBC: non-caseating granulomas
PSC: fibrous, obliterative cholangitis
Antibodies:
PBC: Anti-mitochondrial antibodies
PSC: p-ANCA
27
Presentation of PSC. Key features
Jaundice
Pruritus
Fatigue
Abdo pain
28
Charcot's triad refers to presenting features of...
| Features are...
Ascending cholangitis
RUQ pain
Fever
Jaundice
29
Dyspepsia
| When to make an urgent referral for an endoscopy
All patients who have dysphagia
All patients who have an upper abdominal mass consistent with stomach cancer
Patients aged >= 55 years who've got weight loss, AND any of the following:
upper abdominal pain
reflux
dyspepsia
30
Dyspepsia
| When to make a non-urgent referral for endoscopy
Patients with haematemesis
Patients aged >= 55 years who've got:
treatment-resistant dyspepsia or
upper abdominal pain with low haemoglobin levels or
raised platelet count with any of the following: nausea, vomiting, weight loss, reflux, dyspepsia, upper abdominal pain
nausea or vomiting with any of the following: weight loss, reflux, dyspepsia, upper abdominal pain
31
Typical presentation of Giardiasis
Prolonged, non-bloody diarrhoea
32
MDRD formula for eGFR uses which variables?
serum creatinine
age
gender
ethnicity
33
Key test to differentiate pre-renal uraemia and ATN
Urine sodium
< 20 mmol/L in pre-renal uraemia (as tubules able to reabsorb sodium leading to low urine sodium
> 30 mmol/L in ATN (a necrosed tubule cant reabsorb sodium)
34
ECG Changes in pericarditis
Widespread concave ST elevation and PR depression throughout most of the limb leads (I, II, III, aVL, aVF) and precordial leads (V2-6).
Reciprocal ST depression and PR elevation in lead aVR (± V1).
Sinus tachycardia is also common in acute pericarditis due to pain and/or pericardial effusion.
35
What is a cannon 'A' wave caused by?
It is caused by the ventricles contracting against a closed atrio-ventricular valve and sending a pressure wave up the jugular vein. This is seen in complete heart block and sometimes pulmonary embolism.
36
What is Kussmaul's sign?
JVP increasing with inspiration (it should fall). It is a feature of constrictive pericarditis. It is caused by impaired filling of the right ventricle due to a poorly compliant myocardium or pericardium.
37
Hep B post exposure prophylaxis
HBsAg positive source: if the person exposed is a known responder to HBV vaccine then a booster dose should be given. If they are in the process of being vaccinated or are a non-responder they need to have hepatitis B immune globulin (HBIG) and the vaccine (accelerated course)
unknown source: for known responders the green book advises considering a booster dose of HBV vaccine. For known non-responders HBIG + vaccine should be given whilst those in the process of being vaccinated should have an accelerated course of HBV vaccine
Normal HBV vaccine is 3 doses. 2nd after 1 month, 3rd after an additional 5 months.
38
List King's College Hospital criteria for liver transplantation (paracetamol liver failure)
Arterial pH < 7.3, 24 hours after ingestion
or all of the following:
prothrombin time > 100 seconds
creatinine > 300 µmol/l
grade III or IV encephalopathy
39
Treatment of amoebiasis
Metronidazole
40
Advice to be given regarding safe consuption of alcohol
Men and women should drink no more than 14 units of alcohol per week
'if you do drink as much as 14 units per week, it is best to spread this evenly over 3 days or more'
Pregnant women should not drink.
41
First line treatment of hereditary haemochromatosis
Venesection
Desferrioxamine is second line
42
Causes of chronic pancreatitis
Alcohol
Genetic: CF, Hereditary pancreatitis
Immune: Lymphoplasmocytic sclerosing pancreatitis (raised IgG4)
Raised Triglycerides
Structural: obstruction by tumour, pancreas divisum
43
One unit of alcohol is equivalent to...
One unit of alcohol is equal to 10 ml of alcohol. The 'strength' of an alcoholic drink is determined by the 'alcohol by volume' (ABV).
44
List some gluten containing foods
wheat: bread, pasta, pastry
barley*: beer
rye
oats
Some notable foods which are gluten-free include:
rice
potatoes
corn (maize)
45
Ovarian cancer tumour marker
CA125
46
What are lacunar infarcts?
Small infarcts around the basal ganglia, internal capsule, thalamus and pons
May result in pure motor, pure sensory, mixed motor and sensory signs or ataxia.
47
Features of a total anterior circulation stroke
Involves middle and anterior cerebral arteries
All 3 of following should be present:
1. unilateral hemiparesis and/or hemisensory loss of the face, arm & leg
2. homonymous hemianopia
3. higher cognitive dysfunction e.g. dysphasia
48
Features of partial anterior circulation infarcts
Involves smaller arteries of anterior circulation e.g. upper or lower division of middle cerebral artery
Two of the following are present
1. unilateral hemiparesis and/or hemisensory loss of the face, arm & leg
2. homonymous hemianopia
3. higher cognitive dysfunction e.g. dysphasia
49
Features of lacunar infarcts
Involves perforating arteries around the internal capsule, thalamus and basal ganglia
presents with 1 of the following:
1. unilateral weakness (and/or sensory deficit) of face and arm, arm and leg or all three.
2. pure sensory stroke.
3. ataxic hemiparesi
50
Features of posterior circulation infarcts
```
Involves vertebrobasilar arteries
presents with 1 of the following:
1. cerebellar or brainstem syndromes
2. loss of consciousness
3. isolated homonymous hemianopia
```
51
Features of normal pressure hydrocephalus
Caused by reduced CSF absorption at the arachnoid villi.
Triad of dementia and bradyphrenia, urinary incontinence, and gait abnormality (similar to PD)
52
Window for thrombolysis in stroke
Thrombolysis should only be given if it is administered within 4.5 hours of onset of stroke symptoms (unless as part of a clinical trial)
53
What constitutes a high protein level in CSF
>1g
54
What constitutes a low glucose level in CSF
<1/2 the plasma glucose
55
Key features of multiple system atrophy
parkinsonism (rigidity>tremor)
autonomic disturbance (atonic bladder, postural hypotension)
cerebellar signs
56
Antiepileptic drug safest in pregnany
Lamotrigine
57
First line antiepileptic in generalized epilepsy
Sodium valproate
58
Second line antiepileptic in generalized epilepsy
Lamotrigine (also safest in pregnancy)
59
Migraine prophylaxis in asthmatics
Topiramate
60
First line antiepileptic for partial seizures
Carbamazepine
61
4 Types of Motor Neuron Disease (and key features)
Amyotrophic lateral sclerosis (50% of patients)
typically LMN signs in arms and UMN signs in legs
in familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutase
Primary lateral sclerosis
UMN signs only
Progressive muscular atrophy
LMN signs only
affects distal muscles before proximal
carries best prognosis
Progressive bulbar palsy
palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
carries worst prognosis
62
Key features of Wilson's disease
Liver: hepatitis, cirrhosis
Neurological: basal ganglia degeneration, speech and behavioural problems are often the first manifestations. Also: asterixis, chorea, dementia
Kayser-Fleischer rings
Renal tubular acidosis (esp. Fanconi syndrome)
Haemolysis
Blue nails
63
Interpretation of ABCD score post TIA
People who have had a suspected TIA who are at a higher risk of stroke (that is, with an ABCD2 score of 4 or above) should have:
aspirin (300 mg daily) started immediately
specialist assessment and investigation within 24 hours of onset of symptoms
measures for secondary prevention introduced as soon as the diagnosis is confirmed, including discussion of individual risk factors
If the ABCD2 risk score is 3 or below:
specialist assessment within 1 week of symptom onset, including decision on brain imaging
if vascular territory or pathology is uncertain, refer for brain imaging
64
Biologicals used in MS
Natalizumab (anti-VLA4)
| Alemtuzumab (Anti CD52)
65
Treatment of venous sinus thrombosis
Heparin or LMWH 1st line
If poor prognosis or lack of response endovascular treatment is another option
Lack of evidence for antiplatelets
66
Weakness in myasthenia gravis is worsened by...
Pregnancy
Infection
Emotion
Drugs e.g. b-blockers, gent, tetracyclines, opiates)
67
Features of cluster headache
pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours
clusters typically last 4-12 weeks
intense pain around one eye (recurrent attacks 'always' affect same side)
patient is restless during an attack
accompanied by redness, lacrimation, lid swelling
nasal stuffiness
miosis and ptosis in a minority
68
Trochlear (CNIV) nerve supplies
Superior oblique muscle.
| Controls downward gaze
69
Features of Subacute combined degeneration of the spinal cord
Mention clinical features and tracts affected
Clinical features:
1. Bilateral spastic paresis
2. Bilateral loss of proprioception and vibration sensation
3. Bilateral limb ataxia
Affects the:
1. Lateral corticospinal tracts
2. Dorsal columns
3. Spinocerebellar tracts
70
Cranial nerve responsible for:
a) gag reflex
b) Corneal reflex
a) CNIX
| b) CNV
71
Subacute degeneration of the spinal cord is caused by...
vitamin B12 & E deficiency
72
Reflexes absent in bulbar palsy
Jaw jerk
| Gag reflex
73
Plaques involved in Alzheimer's disease
Cortical plaques due to deposition of type A-Beta-amyloid protein and intraneuronal neurofibrillary tangles caused by abnormal aggregation of the tau protein
74
HIV complication leading to widespread demyelination
Progessive multifocal leukoencephalopathy
75
Features of Progressive multifocal leukoencephalopathy (PML)
widespread demyelination
due to infection of oligodendrocytes by JC virus (a polyoma DNA virus)
symptoms, subacute onset : behavioural changes, speech, motor, visual impairment
CT: single or multiple lesions, no mass effect, don't usually enhance. MRI is better - high-signal demyelinating white matter lesions are seen
76
What is Uhthoff's phenomenon
Seen in MS.
| Worsening of vision with rise in body temperature.
77
Key features of neurofibromatosis type 2
Bilateral acoustic neuromas
| Multiple intracranial schwannomas, mengiomas and ependymomas
78
Key features of neurofibromatosis type 1
```
Café-au-lait spots (>= 6, 15 mm in diameter)
Axillary/groin freckles
Peripheral neurofibromas
Iris hamatomas (Lisch nodules) in > 90%
Scoliosis
Pheochromocytomas
```
79
Features of LGL (Lown-Ganong-Levine syndrome)
very short PR interval
normal P waves and QRS complexes
absent delta waves
80
Signs/symptoms of lateral medullary syndrome
DANVAH
```
Dysphagia
Ataxia
Nystagmus
Vertigo
Anaesthasia: ipsilateral, absent corneal reflex, contralateral pain loss, Horners syndrome (ipsilateral)
Horner's syndrome
```
81
Features of Millard-Gubler syndrome
Pontine infarct
6th and 7th CN nuclei and corticospinal tracts
LMN facial palsy and loss of corneal reflex
Contralateral hemiplegia
82
Causes of locked in syndrome
```
Pontine stroke (ventral pontine infarct)
Central pontine myelinolysis
```
83
5 As of Complex partial seizures
Aura
Automatisms
Awareness lost: motor arrest, motionless stare
Autonomic: change in skin colour, temp, palps
Amnesia
84
Presentation of encephalitis
```
Infectious prodrome: fever, rash, LNs, cold sores, congunctivitis, mengingeal signs.
Bizarre behaviour or personality change
Confusion
Fever
Focal neurological signs
Seizures
Reduced GCS can lead to coma
Hx of travel or animal bite
```
85
Key investigation and finding for cerbral absecess
CT/MRI showing ring-enhancing lesion
86
Organisms causing cerebral absecess
Frontal sinus/teeth: Strep milleri, orpharyngeal anaerobes
| Ear: Bacteroides, other anaerobes
87
Signs of cerebral abscesses
```
Seizures
Fever
Localising signs
Signs of raised ICP
Signs of infection elsewhere
```
88
Features of absence seizures
ABrupt
Short (<10s)
Eyes (glazed, blank stare)
Normal: intelligence, examination, brain scan
Clonus or automatisms may occur
EEG: 3Hz spike and wave
Stimulated by hyperventilation and photics (light)
89
Seizure involving sensory disturbance suggests localisation in which lobe?
Parietal
90
Features of seizures localised in temporal lobe
```
Automatisms
Delusional behaviour
Emotional disturbance Deja/Jamais vu
Abdominal (rising n/v)
Tastes
Smells
```
91
Reflex to test C5
Muscle to test for C5
Area to test C5 sensation
Supinator jerk
Supraspinatus
Patch area
92
Reflex to test C6
Muscle to test C6
Area to test C6 sensation
Biceps jerk
Biceps and barchioradialis
Thumb
93
Reflex to test C7
Muscle to test C7
Area to test C7 sensation
Triceps jerk
Triceps
Middle finger
94
Muscles to test C8
| Area to test C8 sensation
(No reflex for C8)
Finger flexors and intrinsic hand muscles
Little finger
95
What is Lhermitte's sign
Neck flexion causes tingling down spine. Seen in cervical spondylosis.
96
Features of NF type 1
Cafe au lait
Axillary freckling
Fibromas: subcutneous and plexus
Eye: lisch nodules, brown translucent iris hamartomas
Neoplasia: meningioma, ependyoma, astrocytoma, phaeochromocytoma, chronic or acute myeloid leukaemia
Orthopaedic: kyphoscoliosis, sphenoid dysplasia
IQ reduced and epilepsy
Renal: RAS leading to high BP
97
Features of corticobasilar degeneration
EPS+ 4As
Aphasia, dysArthria, Apraxia
Akinetic rigidity in one limb
Atereognosis (don't recognise something by feeling)
Alien limb phenomenon
98
Features of multiple systems atrophy
EPS+
Autonomic dysfunction (post hypotention, bladder dysfunction)
Cerebellar and pyramidal signs (plantar extension and hyperreflexia)
Rigidity>tremor
99
Features of progressive supranuclear palsy
EPS+
Postural instability causing falls
Speech disturbance (and dementia)
Palsy of vertical gaze
100
Features of lewy body dementia
EPS+
Fluctuating cognition
Visual hallucinations
101
Causes of Parkinsonism. List the categories and give examples
```
Degenerative e.g. PD or corticobasilar degeneration
Infection: CJD, syphilis, HIV
Vascular: multiple infarcts in the SN
Drugs: Antipsychotics, metoclopramide
Trauma: CTE (dementia pugilistica)
Genetic: Wilson's disease
```
102
What is cataplexy?
Cataplexy describes the sudden and transient loss of muscular tone caused by strong emotion (e.g. laughter, being frightened). Around two-thirds of patients with narcolepsy have cataplexy.
Features range from buckling knees to collapse.
103
Key side effects of methotrexate
Hepatotoxic
Pulmonary fibrosis
Myelosuppression
104
Key side effects of sulfasalazine
Myelosupression
Hepatotoxic
SJS
Reduced sperm count
105
Key side effect of hydroxychloroquine
Myelosupression
Retinopathy
Seizures
106
Key side effect of penicillamine
Drug induced lupus
Taste change
Myelosupression
107
Biologicals used in RA
Infliximab (chimeric anti-TNF)
Etanercept (TNF receptor)
Adalimumab (human anti-TNF)
Ritiximab (anti-CD20) only used if not responding to anti-TNF
108
Score used to monitor RA
DAS 28
109
Features of RA in hands
```
Swan neck
Boutonnière
Z-thumb
Ulnar deviation of the fingers
Dorsal subluxation of ulnar styloid
```
110
Features of RA
```
Arthritis: symmetrical polyarthritis MCPs and PIPs of hands and feet. Pain swelling and deformity
Nodules
Tenosynovitis
I
C
C
P
O
R
F
```
111
Cranial nerves affected by bulbar palsy
9-12
112
Signs of bulbar palsy
LMN lesions of tongue, talking and swallowing:
Flaccid fasciculating tongue
Speech: quiet or nasal
Normal/abscent jaw jerk
Loss of gag reflex
113
Features of pseudobulbar palsy
Bilateral lesions above the mid-pons leading to UMN lesions of swallowing and talking (not CN nuclei except lower part of 7 have bilateral cortical representation)
```
Signs:
Spastic tongue
Slow tongue movements with slow and deliberate speech
Brisk jaw jerk
Emotional incontinence
```
114
Causes of pseudobulbar palsy
MS
MND
Stroke
115
MND Classification
ALS: 50% of cases. Loss of motor neurons in cortex and anterior horn. Leads to UMN signs (legs), LMN signs (arms)
Progressive bulbar palsy: 10% of cases. Only affects CN9-12 and causes LMN signs
Progressive muscular atrophy: Anterior horn cell lesion leading to LMN signs only. Distal to proximal. Better prognosis than ALS.
Primary lateral sclerosis: loss of Betz cells (giant pyramidal cells in primary motor cortex). UMN signs mainly.
116
Drug used in MND
Riluzole.
| Prolongs life by 3 months
117
Differences between myasthenia gravis and LEMS
In LEMS:
Ab against Voltage gated calcium channels and:
Leg weakness early
Extra: autonomic and areflexia
Movement improves symptoms temporarily
Small response to edrophomium (tensilon)
118
```
List nerve roots that form:
Median nerve
Ulnar nerve
Radial nerve
Phrenic nerve
Sciatic
Common peroneal
Tibial nerve
```
```
Median: C6-T1
Ulnar nerve: C7-T1
Radial Nerve: C5-T1
Phrenic nerve: C3-C5
Sciatic nerve: L4-S3
Common peroneal: L4-S1
Tibial: L4-S3
```
119
Signs of sciatic nerve damage
Motor: weakness and wasting of hamstrings and all muscles below knee
Sensory loss below knee laterally and foot
120
Signs of damage to common peroneal nerve
Sensory loss below knee laterally
Foot drop, can't walk on heels (or do impetus turn!)
Weak ankle dorsiflexion and eversion but inversion intact.
121
Common location and cause of damage to common peroneal nerve
Fibular head.
Cross legged sitting
Trauma
122
Common cause of sciatic nerve damage
Pelvic tumour
| Pelvic or femoral fracture
123
Signs of damage to tibial nerve
Can't plantarflex (so can't go en point, messy jive kicks)
Can't flex toes or invert foot.
Loss of sensation sole of foot (stomping gait)
124
Nerves affected in Erb's palsy
C5-6
125
Nerves affected in Klumpke's palsy
C8-T1
126
Muscles supplied by median nerve
lateral two lumbricals
opponens pollicis
abductor pollicis brevis
flexor pollicis brevis
127
DIfference between a medical and surgical 3rd nerve palsy
“Medical”: pupil sparing (and painless)
“Surgical”: pupil fixed and dilated
Parasympathetic fibres are situated on the periphery of the 3rd nerve trunk and so are the first to be affected by compression resulting in a fixed and dilated pupil.
The classic cause of a “surgical” 3rd nerve palsy is a posterior communicating artery aneurysm. The vaso vasorum which supplies the 3rd nerve starts from the centre and supplies out radially.
In “medical” 3rd nerve palsies the centre of the 3rd nerve is affected first leaving the parasympathetic fibres and therefore pupillary constriction intact until the end.
128
Features of Webers syndrome
Webers syndrome: ipsilateral third nerve palsy with contralateral hemiplegia --> signifies a midbrain stroke.
129
Sites of radial nerve damage
Axilla
Humerus
Wrist
130
Motor signs of radial nerve damage:
Low
High
V. high
Low: finger drop
High: Wrist drop
V. High: Triceps paralysis and wrist drop
131
Location of sensory loss in radial nerve injury
Anatomical snuff box
132
Condition causing hypercoagulable state with paradoxical prolonged APTT
Antiphospholipid syndrome, SLE
133
Cardinal signs of syringomyelia
Dissociated sensory loss: absent pain and temp, but preserved proprioception, soft touch and vibration.
Wasting and weakness of hands with/wo claw hand
Loss of reflexes in upper limb
Charcot joints: shoulder and elbow
134
Features of syringomyelia
Dissociated sensory loss: absent pain and temp, but preserved proprioception, soft touch and vibration.
Wasting and weakness of hands with/wo claw hand
Loss of reflexes in upper limb
Charcot joints: shoulder and elbow
UMN weakness in lower limbs and +ve babinski
Horner's
135
Causes of synringomyelia
Blocked CSF circulation with reduced flow from posterior fossa due to:
Arnold Chiari malformation
Masses
Spina bifida
Cord trauma, myelitis, cord tumours, AVM
136
ABCD2 scoring
```
A Age >= 60 years 1
B Blood pressure >= 140/90 mmHg 1
C Clinical features
- Unilateral weakness 2
- Speech disturbance, no weakness 1
D Duration of symptoms
- > 60 minutes 2
- 10-59 minutes 1
Patient has diabetes 1
```
137
Steps to take if ABCD2 score is 4 or more (and symptoms have resolved)
aspirin (300 mg daily) started immediately
specialist assessment and investigation within 24 hours of onset of symptoms
measures for secondary prevention introduced as soon as the diagnosis is confirmed, including discussion of individual risk factors
138
Chromosome affected in NF type 2
22
139
```
Movement to test:
L2
L3
L4
L5
S1
```
L2: Hip flexion (and adduction)
L3: Knee extension (and adduction)
L4: Foot inversion and dorsiflexion (and knee extension)
L5: Great toe dorsiflexion (also foot inversion and dorsilfexion, knee flexion, hip extension and adduction)
S1: Foot eversion (and foot and toe plantarflexion, knee flexion)
140
Knee jerk tests which nerve root?
L3 and L4
141
Ankle jerk tests which nerve root?
S1
142
Back pain red flegs
```
Age under 20 or over 55
Neurological disturbance
Sphincter disturbance
Bilateral or alternating leg pain
Current or recent infection
Fever, wt loss, night sweats
Hx of malignancy
Thoracic back pain
Morning stiffness
Acute onset in elderly people
Constant or progressive pain
Nocturnal pain
```
143
Risk factors for OA
Age
Obesity
Joint abnormality
144
Key signs of OA
Bouchards and Heberdens nodes
Thumb CMC joint squaring
Fixed flexion deformity
145
Radiological signs of OA
Loss of joint space
Subchondral cysts
Subchondral sclerosis
Osteophytes
146
Radiological signs of RA
```
Loss of joint space
Soft tissue swelling
Peri-articular osteopenia
Deformity
Subluxation
```
147
Common organisms causing septic arthritis
Staph aureus (commonest)
Gonococcus (commonest in young sexually active)
Streps
Gm -ve bacilli
148
Signs and symptoms of septic arthritis
Acute inflamed tender swollen joint
Systemically unwell
Reduced range of movement
149
Features of RA
Mnemonic: ANTI CCP Or RF
Arthritis, symmetrical polyarthritis, MCPs and PIPs of hands and feet. Pain swelling and deformity.
Nodules: elbows, feet, fingers, heel. Firm, non-tender, mobile or fixed
Tenosynovitis: de quervains and atlanto-axial subluxation
Immune: AIHA, vasculitis, Amyloid, Lymphadenopathy
Cardiac: peridarditis and pericardial effusion
Carpal tunnel syndrome
Pulmonary: fibrosing alveolitis, pleural effusions
Opthalmic: episcleritis, sjogrens
Raynaud's
Felty's: RA+splenomegaly+neutropenia
150
Features required for RA diagnosis
```
4 out of 7:
Morning stiffness of >1h for at least 6 weeks
Arthritis in at least 3 joints
Arthritis of hand joints
Symmetrical
Rheumatoid nodules
+RF
Radiographic changes
```
151
What are tophi?
Urate deposits in pinna and tendons. Seen in gout.
152
Causes of gout
Drugs e.g. NSAIDS, diuretics (thiazides)
Reduced urate excretion: genetic, renal impairment
Increased cell turnover: lymphoma, leukaemia, psoriasis, haemolysis, tumour lysis syndrome
Alcohol excess
Purine rich foods: red meat and seafood, port
153
Gout: findings on polarised light microscopy
Negatively birefringent needle-shaped crystals
154
Urate levels in gout
Increased or normal
155
X-ray findings in gout
Changes are late.
Punchout out erosions (erosive arthritis!!) in juxta articular bone.
Reduced joint space late in disease
156
Precipitants of gout
Prolonged fast, thiazides, cytotoxics, surgery, infection
157
Differentials in cases of gout
Septic arthritis
Pseudogout
Haemarthrosis
158
Pseudogout: findings in polarised light microscopy
Positively birefringent rhomboid-shaped crystals
159
Crystals in pseudogout are formed of...
Calcium pyrophosphate
160
Radiological findings in pseudogout
Possible soft tissue calcium deposition (chondrocalcinosis)
161
Treatment of psuedogout
Analgesia
NSAIDs
PO, IM or intra-articular steroids
162
Presentation of acute pseudogout
```
Acute monoarthropathy (usually knee, wrist or hip)
Usually spontaneous and self limiting
```
163
Presentation of chronic pseudogout
Destructive changes like OA
| Can present as polyarthritis (pseudo-rheumatoid)
164
Risk factors for pseudogout
```
Age (older)
OA
DM
HYPOthyroidism
HYPERparathyroidism
Wilson's disease
Hereditary haemochromatosis
```
165
Presentation of ankylosing spondylitis
Presents in late teens/early 20s. Men earlier than women.
Gradual onset back pain. Pain radiates from SI joints to hips and buttocks. Pain is worse at night with early morning stiffness and is relieved by exercise.
Progressive loss of all spinal movements.
Possible thoracic kyphosis and neck hyperextension (question mark posture)
Enthesitis (is inflammation of the entheses, the sites where tendons or ligaments insert into the bone) of Achilles tendonitis and plantar fasciitis.
Chostochondritis
166
Examples of seronegative spondylarthropathies
Ankylosing spondylitis
Psoriatic arthritis
Enteropathic arthritis
167
What is Schober's test?
Schober's test assesses the amount of lumbar flexion.
In this test a mark is made at the level of the posterior iliac spine on the vertebral column
(approximately at the level of L5.)
Place one finger 5cm below this mark and another finger at about 10cm above this mark. The patient is then instructed to touch his toes. If the increase in distance between the two fingers on the patients spine is less than 5cm then this is indicative of a limitation of lumbar flexion.
168
Incompetence of the... valve can be seen in ankylosing spondylitis
Aortic valve
169
Key radiological change in anylosising spondylitis
Bamboo spine: calcification of ligaments, periosteal bone formation
170
Extra-articular manifestations of ankylosing spondylitis
Osetoporosis
Acute iritis/anterior uveitis
Aortic valve incompetence
Apical pulmonary fibrosis
171
Allele found in ankylosing spondylitis
HLA-B27
172
Management of ankylosing spondylitis
Conservative: exercise and physio
Medical: NSAIDs, Anti-TNF if severe, Local steroid injections, Bisphosphonates
Surgical: Hip replacement to reduce pain and increase mobility
173
Features of psoriatic arthritis
Asymmetrical oligoarthritis (2-4 joints) is commonest
Distal arthritis of the DIP joints
Symmetrical polyarthritis (like RA but no DIP sparing)
Spinal (like ank spon)
May predate the skin disease
Psoriatic plaques
Nail pitting, subungual hyperkeratosis, onchyolysis (painless separation of the nail from the nail bed.)
Enthesitis: achilles tendonitis, plantar fasciitis
Dactlylitis
174
Nail changes in psoriasis
Nail pitting, subungual hyperkeratosis, onchyolysis (painless separation of the nail from the nail bed.)
175
Radiological changes in psoriatic arthritis
Erosions: pencil in cup deformity
176
Features of reactive arthritis
Sterile
Appears 1-4 weeks post urethritis or dysentery
Organisms:
Urethra: chlamydia
Dysentery: Campylobacter, salmonella, shigella, yersina
Asymmetrical lower limb oligoarthritis esp knee
Iritis/conjunctivitis
Keratoderma blenorrhagica: plaques on soles/palms
Circinate balanitis: painless ulceration
Enthesitis
Mouth ulcers
177
Features of Behcet's disease
```
Systemic vasculitis (unknown cause)
Recurrent oral and genital ulceration
Anterior and posterior uveitis
Erythema nodosum
Vasculitis
Non-erosive large joint oligoarthropathy
CN palsies
Diarrhoea and colitis
```
178
Behcet's disease is seen in which ethnic groups
Turks
Mediterraneans
Japanese
179
Investigation for Behcets disease
Skin prick leads to papule formation
180
Features of sjogren's syndrome
Reduced tear production and salivation
Bilateral parotid swelling
Vaginal dryness
Systemic features e.g. polyarthritis, Raynaud's and bibasal pulmonary fibrosis.
181
Sjogren's is associated with which autoimmune conditions
Secondary to: RA, SLE, Systemic sclerosis
| Associated with: AIHA, PBC, thyroid disease
182
Antibodies found in Sjogrens
ANA: Anti Ro, La and RF
183
Ischaemia in Raynaud's is triggered by...
Cold
| Emotion
184
Treatment for Raynaud's
Wear gloves
CCB e.g. nifedipine
ACEi
IV prostacyclin
185
Causes of secondary Raynaud's
Systemic sclerorosis, limited sclerosis, RA, sjogren's
Thrombocytosis, PV
B-blockers
186
Presentation of Raynaud's
Triphasic colour change: White, blue, crimson
| Possible digitial ulceration and gangrene
187
Features of limited cutaneous systemic sclerosis
```
Calcinosis
Raynaud's
oEsophageal dysmotility
Sclerodactyly
Telangiectasia
```
The skin involvement is limited to the hands, feet and face. CAN get Pulmonary HTN (15%)
Slow onset (compared to diffuse systemic sclerosis)
188
Features of diffuse systemic sclerosis
CREST with more diffuse skin involvement.
Rapid skin thickening and Raynaud's
Organ fibrosis leading to:
GI: reflux, aspiration, dysphagia, anal incontinence
Lung: fibrosis and pulmonary HTN
Cardiac: Arrhythmias and conduction defects
Renal: Acute HTN crisis (commonest cause of death)
189
Antibodies in:
CREST syndrome
Diffuse systemic sclerosis
CREST: anti-centromere
Diffuse: Anti-Scl70/topoisomerase, RNA polymerase 1,2,3
190
Management of systemic sclerosis (limited and diffuse)
Conservative:
Exercise and skin lubricants to reduce contractures
Hand warmers: Raynaud's
Medical:
Immunosuppression: cyclosporine, cyclophosphamide
Raynaud's: CCBs, ACEi, IV Prostacyclin
Renal: Intensive BP control: ACE is 1st line.
Oesphageal: PPI, prokinetics (metoclopramide)
PHT: Sildenafil, bosentan
Telangiectasia: Make-up. Light therapy.
191
Features of polymyositis
```
Striated muscle inflammation.
Muscle weakness: Progressive. Symmetrical. Proximal.
Wasting: pelvic girdle, shoulder.
Dysphagia, dysphonia,
Respiratory weakness
Myalgia and arthralgia
Commoner in females
Often paraneoplastic (lung, pancreas, breast, bowel)
```
192
Skin features of dermatomyositis
Heliotrope rash
Macular rash (shawl sign +ve over back and shoulders)
Nailfold erythema
Gottron's papules: knuckles, elbows, knees
Mechanics hands: painful, rough skin, cracking of finger tips
Subcutaneous calcifications
Also get retinopathy: haemorrhages and cotton wool spots
193
Extramuscular features of polymyositis and dermatomyositis
```
Fever
Arthritis
Bibasal pulmonary fibrosis
Raynaud's
Myocarditis and arrhythmias
```
194
Antibodies in polymyositis and dermatomyositis
Anti-Jo1
195
Investigations in polymyositis
Muscle enzymes: raised CK, ALT, AST, LDH
Ab: anti Jo1
EMG
Muscle biopsy: inflammaed striated muscle
Screen for malignancy: CXR, mammogram, tumour markers, pelvic US, Abdo US, CT
196
Treatmement of polymyositis and dermatomyositis
Steroids
Cytotoxics: AZT, MTX
Don't forget to screen for malignancy!
197
Features of SLE
Relapsing remitting. Constitutional symptoms.
A RASH POINts MD
Arthritis: non-erosive involving peripheral joints.
Renal: proteinuria and HTN
ANA positive in 95%
Serositis: pleuritis, pericarditis.
Haematological: AIHA, low WCC, low plt
Photosensitivity
Oral ulcers
Immune phenomenon: Anti-dsDNA, sm, phospholipid
Neurological: seizures, psychosis
Malar rash (nasolabial fold sparing)
Discoid rash (mainly face and chest, initally erythema then hyperkeratotic papules )
198
SLE antibodies
ANA: anti-dsDNA, Ro, La, Sm, RNP
199
Methods of monitoring SLE disease activity
C3, C4 (low in active)
ESR high if active
Anti-dsDNA titres
Others
Urine: dip stick for protein, PCR
FBC, U+E, CRP (usually normal)
200
Drugs that induce lupus
Procainamide
Phenytoin
Hydralazine
Isoniazid
201
Features of drug induced lupus
Anti-histone Ab (100%)
Mostly skin and lung signs
Remits if drug stopped
Phenytoin, procainamide, isoniazide, hydralazine.
202
Antibodies in antiphospholipid syndrome
Anti-cardiolipin
| Lupus anticoagulant
203
SLE management
Severe flares (pericarditis, CNS disease, AIHA, nephritis): IV cyclophosphamide, High dose prednisolone.
Cutaneous: topical steroids to treat, sun cream for prevention
Maintenance for joints and skin: NSAIDs, hydroxychloroquine, low dose steroids (option)
Lupus nephritis: ACEi for proteinuria. Immunosupression if aggressive GN
204
False positive anti-cardiolipin Abs seen in...
Syphilis
205
List 4 ANCA negative vasculitides
Goodpasture's
Henloch Schonlein
Cryoglobulinaemia
Cutaneous Leukocytoclastic vasculitis
206
Features of GCA
```
Associated with polymyalgia rheumatica in 50%
Fever, malaise, fatigue
Headache
Jaw claudication
Amourosis fugax
Prominant temporal arteries (with or without plusation)
Raised ESR and CRP
Raised Plt
```
207
Investigations in suspected GCA
```
High ESR and CRP
High ALP
Low Hb (Normo normo)
High Plt
Temporal artery biopsy within 3 days (skip lesions can occur)
DO NOT DELAY TREATMENT
```
208
Treatment of GCA
High dose steroids (e.g. pred 40-60mg oral) and taper slowly.
PPI and alendronate cover
209
GCA is rare under the age of...
55
210
Polymyalgia rheumatic increases the risk of...
GCA.
| 15% develop it.
211
Features of polymyalgia rheumatica
```
Sudden/subacute onset
Fever, fatigue, weight loss
Severe pain and stiffness in the shoulders, neck and hips
Symmetrical
Worse in morning
No weakness
High ESR, CRP, ALP, plasma viscosity
Can develop mild polyarthritis, tenosynovitis and carpal tunnel syndrome
15% develop GCA
```
212
Investigations in suspected polymyalgia rheumatica
High CRP, ESR, ALP, plasma viscosity
| Normal CK
213
Treatment of polymyalgia rheumatica
15mg/day oral prednisolone and then taper according to ESR and symptoms
PPI and alendronate cover
214
Features of granulomatosis with polyangiitis
URT: chronic sinusitis, epistaxis, saddle-nose
LRT: Cough, haemoptysis, pleuritis
Renal: RPGN, haematuria, proteinuria
Other: palpable purpura, conjunctivitis, keratitis, uveitis
cANCA
CXR: Bilateral nodular and cavity infiltrates
Granulomatous necrotizing small vessel vasculitis
215
Radiological findings in granulomatosis with polyangiitis
CXR: Bilateral nodular and cavity infiltrates
216
Treatment of granulomatosis with polyangiitis
Immunosuppression:
Cyclophsphamide, Rituximab, MTX
Azathioprine, Rituximab or MTX for maintenance.
217
Features of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
```
Late onset asthma
Eosinophilia
Granulomatous small vessel vasculitis leading to:
RPGN
Palpable purpura
GIT bleeding
```
pANCA
218
Treatment of Features of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
Prednisolone
Cyclophosphamide is severe multi-organ
Azathioprine or MTX for maintenance
219
Key features of microscopic polyangiitis
RPGN
Haemoptysis
Palpable purpura
pANCA
220
What is sarcoidosis?
Sarcoidosis is a multi-system disease involving abnormal collections of inflammatory cells known as granulomas. Sarcoidosis can cause facial palsies, parotid enlargement, hypercalcaemia and ocular problems, as seen in this case.
221
Features of Marfan's syndrome
tall stature with arm span to height ratio > 1.05
high-arched palate
arachnodactyly
pectus excavatum
pes planus
scoliosis of > 20 degrees
heart: dilation of the aortic sinuses (seen in 90%) which may lead to aortic aneurysm, aortic dissection, aortic regurgitation, mitral valve prolapse (75%),
lungs: repeated pneumothoraces
eyes: upwards lens dislocation (superotemporal ectopia lentis), blue sclera, myopia
dural ectasia (ballooning of the dural sac at the lumbosacral level)
222
Genetics of marfans (mode of inheritance and affected gene)
Marfan's syndrome is an autosomal dominant connective tissue disorder. It is caused by a defect in the fibrillin-1 gene on chromosome 15
223
Anti Ro is associated with
Foetal heart block
SLE
Sjogrens
224
Anti La is associated with
SLE, Sjogren's
225
Anti-Sm antibodies are associated with...
SLE
226
Anti-RNP antibodies are associated with...
SLE
| MCTD (mixed connective tissue disease)
227
Anti-Jo1 antibodies are associated with...
Polymyositis and dermatomyositis
228
Anti-centromere antibodies are associated with...
Limited cutaneous systemic sclerosis
229
Anti-Scl70 antibodies are associated with...
Diffuse systemic sclerosis
230
Anti RNA polymerase (1,2,3) antibodies are associated with...
Diffuse systemic sclerosis
231
Features of sarcoidosis
Acute: erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, polyarthralgia
Insidious: dyspnoea, non-productive cough, malaise, weight loss
Skin: lupus pernio
Hypercalcaemia: macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)
232
Signs of iron deficiency anaemia
Koilonychia
Angular stomatitis/cheilosis
Post-cricoid web (Plummer-Vinson)
233
Causes of sideroblastic anaemia
Congenital
Acquired:
Myelodysplastic/myeloproliferative disease
Drugs: chemo, anti-TB, lead
234
Site of B12 absorption
Terminal ileum
235
Sources of folate
Green veg, nuts, liver, poultry, pork, shellfish
236
Sources of B12
Meat, fish, dairy
237
B12/folate deficiency blood film
Macrocytic anaemia
Hypersegmented PMN
Target cells if ETOH/liver
238
Causes of folate deficiency
Intake: poor diet
Demand: pregnancy, malignancy, haemolysis
Malabsoprtion: coeliac, crohns
Drugs: alcohol, phenytoin, MTX
239
Location of folate absorption
Proximal jejunum
240
Causes of B12 deficiency
```
Reduced intake (vegan)
Reduced intrinsic factor: pernicious anaemia, post-gastrectomy
Terminal ileum: Crohn's, ileal resection, bacterial overgrowth
```
241
Features of subacute degeneration of the spinal cord
Pernicious anaemia
Symmetrical dorsal column and corticospinal tract loss.
Distal sensory loss esp proprioception and vibration
Ataxia with wide gait and +ve Rombergs
Mixed upper and lower motor neuron signs:
Spastic paraparesis
Brisk knee jerk, upgoing plantars
Absent ankle jerk
242
Pernicious anaemia increases the risk of...
Gastric adenocarcinoma
243
Features of warm AIHA
IgG mediated bind at 37 degrees
Extravascular haemolysis and spherocytes
DAT+ve
Caused by: SLE, RA, idiopathic
244
Treatment of warm AIHA
Immunosupression
| Splenectomy
245
Treatment of cold AIHA
Avoid cold
| Rituximab
246
Features of cold AIHA
```
IgM mediated bind at <4 degrees
Intravascular haemolysis
May cause agglutination
DAT+ve for complement alone
Causes: mycoplasma, idiopathic
```
247
Organism that causes haemolytic uraemic syndrome
E.coli O157:H7
248
Features of HUS
```
MAHA
Thrombocytopenia
Renal failure
Bloody diarrhoea and abdo pain
Usually resolves spontaneously
```
249
Blood film in HUS
Shistocytes
| Low plt
250
Features of thrombotic thrombocytopenic purpura
```
Adult females with the pentad:
Fever
CNS signs: confusion, seizures
MAHA
Thrombocytopenia
Renal failure
```
Can be genetic or acquired
251
Treatment of TTP
Immunosupression
Plasmaphoresis
Splenectomy
252
Complications of hereditary spherocytosis
```
Aplastic crisis (parvovirus B19)
Megaloblastic crisis
```
253
Treatment of hereditary spherocytosis
Folate and splenectomy (after childhood)
254
Features of hereditary spherocytosis
```
Anaemia and reticulocytosis
Splenomegaly
Pigment gallstones
Jaundice
DAT -ve
(MCV of little value due to high MCV of reticulocytes and spherocytes with low MCV. Results in normal or slightly low MCV)
```
255
Investigations in hereditary spherocytosis
```
Osmotic fragility (high)
DAT: -ve
Spherocytes
Low Hb
High reticulocytes (depends on severity)
```
256
Triggers for haemolysis in G6PD deficiency
Fava beans (broad beans)
Mothballs (napthalene)
Infection
Drugs: antimalarials, henna, dapsone, sulphonamides
257
Mode of inheritance:
G6PD deficiency
Hereditary spherocytosis
G6PD deficiency: x-linked recessive
| Spherocytosis: autosomal dominant
258
G6PD deficiency affects which metabolic pathway
Pentose phosphate shunt
259
G6PD deficiency blood film features
Irregularly contracted cells
Heinz bodies
Bite cells
260
Investigations in suspected G6PD deficiency
Film:
Irregularly contracted cells
Heinz bodies
Bite cells
G6PD assay after 8 weeks as reticulocytes have high G6PD
261
Mutation in sickle cell anaemia
Point mutation (glu to val) in the B globin chain
262
Hb levels in sickle cell anaemia
60-90
263
Features if HbS red cells
```
Sickled
Dehydrated
Rigid
Adherant (to vessel wall)
Reduced life span (haemolysis)
```
264
Investigations in suspected HbSS
```
Hb 60-90
Sickle cells
Target cells
Hb electrophoresis (neonatal screening)
Reticulocytes
Raised bilirubin
```
265
Triggers for sickle cell crisis
Dehydration
Infection
Cold
Hypoxia
266
Complications of HbSS
Sequestration crisis
Splenic infarction: leads to atrophy and hyposplenism
Infection e.g. osteomyelitis
Aplastic crisis: parvovirus B19 infection
Gallstones
267
Clinical features of sickle cell anaemia
Mnemonic: SICKLED
Splenonomegaly: possible sequestration crisis
Infarction: stroke, spleen, AVN, leg ulcers
Crises: pulmonar, mesenteric, pain
Kidney disease
Liver, lung disease
Erection: priapism
Dactylitis
268
Management of sickle cell anaemia (chronic)
Pen V 250mg BD
Folate 5mg OD
Hydroxycarbamide if frequent crises
269
vWF stabilises which coagulation factor?
F8
270
Staging system for Hodgkin's and Non-Hodgkin'a Lymphoma
Ann Arbor system:
1: single LN region
2: >= nodal areas on same side of the diaphragm
3: Nodes on both sides of the diaphragm
4: Spread beyond nodes e.g. liver and BM
add B if constitutional symptoms
271
Features of Hodgkin's lymphoma
Painless lymphadenopthy. Asymetric. Usually cervical.
Possible Alcohol induced LN pain.
Mediastinal LN may cause mass effect due to bronchial or SVC obstruction
Spreads contigiously to other LNs
Fever, night sweats, weight loss
Reed-Sternberg cells
272
Treatment of Hodgkin's Lymphoma
ABVD regimen
A – Doxorubicin (Adriamycin ®)
B – Bleomycin
V – Vinblastine (Velbe ®)
D – Dacarbazine (DTIC).
273
Features of Non-Hodgkin's lymphoma
Painless symmetric lymphadenopathy
Multiple sites
Spreads discontinuously
Fever, night sweats, weight loss
Pancytopenia and hyperviscosity
```
Extranodal features:
Skin (esp is T cell)
CNS
Oropharynx and GI tract
Splenomegaly
```
274
Classification of non-Hodgkin's Lymphoma
```
Low Grade B Cell
Follicular
Small cell lymphocytic
Marginal zone (inc MALTomas)
Lymphoplasmacytoid (e.g. Waldenstrom's)
```
High Grade B Cell
Diffuse large B cell (commonest type of NHL)
Burkitt's
T Cell:
Adult T cell lymphoma: Caribbeans and Japanese- HTLV-1
Enteropathy associated T cell lymphoma: Chronic coeliac
Cutaneous T cell lymphoma
Anaplastic large cell
275
Treatment of polycythaemia vera
Aspirin 75mg OD
Venesection if young
Hydroxycarbamide if older/high risk
276
Complications of polycythaemia vera
Thrombosis and haemorrhage
30% progress to myelofibrosis
5% progress to AML
277
Features of essential thrombocythaemia
Thrombosis: arterial and venous
Bleeding e.g. mucous membranes (abnormal plt function)
Erythromelagia: sudden, severe burning in hands and feet with redness of the skin
Plt>600 (often >1000)
Increased megakaryocytes in BM
50% JAK2+ve
278
Features of primary myelofibrosis
Clonal proliferation of megakaryocytes leading to increased PDGF expression and myelofibrosis.
Extramedullary haematopoesis e.g. liver, spleen
Elderly
Massive HSM
Fever, night sweats, weight loss
BM failure: Anaemia, infections, bleeding
279
Treatment of essential thrombocythaemia
Hydroxycarbamide if Plt>1000
| Anagralide may be used
280
Primary myelofibrosis investigations
FBC: Cytopenias
Film: leukoerythroblastic with teardrop poikilocytes
BM: dry tap, need trephine biopsy
50% JAK2+ve
281
Treatment of primary myelofibrosis
Supportive: blood products
Splenectomy
Allogeneic BMT may be curative in younger patients
Ruxolitinib (anti-JAK2) for improvement of severe constitutional symptoms or severely symptomatic splenomegaly.
282
Gene mutation in acute promyelocytic leukaemia
t(15;17)
283
Features of chronic lymphocytic leukaemia
```
Clone of mature B cells
Often asymptomatic
Symmetrical painless lymphadenopathy
HSM
Anaemia
Fever, weight loss, night sweats
Lymphocytosis
Smear cells
Reduced Ig
+ve DAT
Can remain stable for years death commonly due to infection.
```
284
Evan's syndrome is...
AIHA + ITP
285
Chemotherapy for CLL
Cyclophosphamide
Fludarabine
Rituximab
286
Complications of CLL
Evan's syndrome
Infection
Marrow failure/infiltration
Transformation to large B cell lymphoma
287
Features of CML
```
Fever, lethargy, weight loss, night sweats
Massive HSM causing abdo discomfort
Bruising/bleeding (platelet dysfunction)
Gout
Hyperviscosity
High WCC (myeloid)
Anaemia and low plt
High urate
Ph chromosome positive
```
288
CML investigations
```
Philadelphia chromosome +ve. t(9;22) formation of BCR-ABL fusion gene. Positive in 80% of CML
WCC: raised. Myeloid cells.
Anaemia and low plt
High urate
Blasts dependent on phase of disease.
```
289
Treatment of CML
Imatinib (tyrosine kinase inhibitor)
| BMT is blast crisis or TK-refractory
290
3 Phases of CML
Chronic phase: <5% blasts
Accelerated phase: 10-19% blasts
Blast phase: usually AML >20% blasts
291
Key features of multiple myeloma
CRAB
Calcium high
Renal insufficiency
Anaemia <100g/L (BM infiltration. Also low plt and low neutrophils)
Bone lesions: bone pain and backache, vertebral collapse, pathological fractures
Clonal plasma B cells
Urinary monoclonal light chains (Bence Jones Protein)
Recurrent bacterial infections
292
Complications of multiple myeloma
```
Hypercalcaemia
Neuro: compression, raised Ca, amyloid
AKI
Stroke due to hyperviscosity
AL-amyloid
```
293
Investigations in suspected multiple myeloma
```
FBC: normocytic, normochromic anaemia
Film: rouleaux, plasma cells, cytopenias
Raised ESR, raised Cr and urea,
High calcium but normal ALP
Urine: bence jones proteins
```
X-ray: punched out lytic lesions, pepper pot skull, vertebral collapse, fractures
294
For diagnosis of multiple myeloma BM plasma cells must be at least...
10%
295
Treatment of multiple myeloma
Specific: chemo e.g. lenalidomide and low dose dex followed by BMT. Chemo only if unfit.
Supportive:
Bone pain: analgesia (avoid NSAIDs) and bisphosphonates
Anaemia: transfusions and EPO
Renal impairment: adequate hydration and dialysis
Infections: broad spectrum abx and IVIG if recurrent
Complications:
High Ca: aggressive hydration, furosemide, bisphosphonates
Cord compression: MRI, Dex, local radiotherapy
Hyperviscosity: plasmapheresis (remove light chains)
AKI: rehydration and dialysis
296
Features of smouldering myeloma
Asymptomatic
Monoclonal protein
BM plasma cells of at least 10%
No CRAB
297
MGUS (Monoclonal gammopathy of undetermined significance)
Serum monoclonal protein under 30g/L
Clonal BM plasma cells under 10%
No CRAB
298
Features of Waldenstroms Macroglobulinaemia
Lymphoplasmacytoid lymphoma producing monoclonal IgM band
Hyperviscosity producing CNS and ocular symptoms
Lymphadenopathy and splenomegaly
299
Ig type in Waldenstroms Macroglobulinaemia
IgM
300
Investigations in suspected Waldenstroms macroglobulinaemia
Monoclonal IgM paraprotein in serum
| High ESR
301
Definition of amyloidosis
Group of disorders
| Extracellular deposits of protein in an abnormal fibrillar form that is resistant to degradation
302
Signs of hyperviscosity syndrome
CNS: headache, confusion, seizures, falls
Visual: retinopathy producing visual disturbance
Bleeding: mucus membranes, GI, GU
Thrombosis
303
Causes of AL amyloidosis
Primary occult plasma cell proliferation (primary AL amyloid)
Secondary: Multiple myeloma, Waldenstroms, MGUS, lymphoma
304
Causes of AA amyloidosis
Chronic inflammation:
RA
IBD
Chronic infection e.g. TB, bronchiecyasis
305
Amyloid in AA amyloidosis is derived from...
SAA (serum amyloid A). An acute phase protein.
306
Investigations in suspected amyloidosis
Biopsy affected tissue
| Apple green birefringence with Congo red stain under polarised light
307
Features of AL amyloidosis
Renal: proteinuria and nephrotic syndrome
Heart: restrictive cardiomyopathy, arrhythmias (sparkling appearance on echo)
Nerves: peripheral and autonomic neuropathy. Carpal tunnel
GIT: macroglossia, malabsorption, perforation, haemorrhage, hepatomegaly, obstruction
Vascular: periorbital purpura (characteristic)
308
Features of AA amyloidosis
Renal:
Proteinuria and nephrotic syndrome
Hepatosplenomegaly
309
Treatment of AA amyloidosis
May improve with underlying condition
310
Treatment of AL amyloidosis
May respond to therapy for multiple myeloma
311
Features of familial amyloidosis
Autosomal dominant
Mutations in transthyretin
Sensory or autonomic neuropathy
Liver transplant may be curative
312
Treatment of familial amyloidosis
Liver transplant may be curative
313
Causes of massive splenomegaly (above 20cm)
```
CML
Myelofibrosis
Malaria
Leishmaniasis
Gaucher's
```
314
Blood film in hyposplenism
Howell Jolly bodies
Target cells
Pappenheimer bodies
315
Hyposplenisms increases susceptibility to which organisms
Encapsulated: Haemophilus, pneumococcus, meningococcus
316
Immunisations post splenectomy
Pneumovax (repeat every 5 years)
Hib if not done in childhood
Men C if not done in childhood
Yearly flu
317
Causes of hyposplenism
Splenectomy
Sickle Cell Disease
Coeliac disease
IBD
318
Causes of splenomegaly
Haematological:
Haemolysis: Hereditary spherocytosis, SCD
Myeloproliferative disease: MF, CML, PV
Leukaemia, lymphoma
Infective:
EBV, CMV, hepatitis, HIV
TB, infective endocarditis
malaria, leishmaniasis, hydatid disease
Portal HTN: budd chiari, cirrhosis
Connective tissue disease: Sjogren's RA, SLE
Other: Sarcoid, amyloidosis, Gaucher's, primary antibody deficiency
319
PEFR % of predicted in asthma exacerbations:
Moderate
Severe
Very severe
PEF >50–75% best or predicted
PEF 33–50% best or predicted
PEF <33% best or predicted
320
Causes of upper zone lung fibrosis
A PENT
```
Aspergillosis
Pneumoconiosis
Extrinsic allergic alveolitis
Negative sero-arthropathy
TB
```
321
Causes of lower zone lung fibrosis
STAIR
```
Sarcoidosis (mid zone)
Toxins
Asbestosis
Idiopathic pulmonary fibrosis
Rheum: SLE, RA, Scleroderma, Sjogren's, PM/DM
```
322
Drugs causing lung fibrosis
BANS ME
```
Bleomycin/busulfan
Amiodarone
Nitrofurantoin
Sulfasalazine
MEthotrexate
```
323
Patients with a CURB-65 score of... should be managed in hospital
2 or more
324
Most common organism causing infective exacerbation of COPD
H. influenzae
325
Features of PCP infection
```
Caused by pneumocystis jiroveci
Dry cough
Exertional dyspnoea
Fever
HIV complication
CXR shoes bilateral perihilar interstitial shadowing
Diagnose by visualizing in sputum
```
326
Features of CMV retinitis
Reduced acuity
Eye pain
photophobia
Pizza sign on fundoscopy
327
Kaposi sarcoma caused by infection with...
HHV8
328
Treatment of progressive multifocal leukoencephalopathy (in HIV)
HAART
| Lack of evidence for other treatments
329
Complications of chickenpox
Pneumonitis, haemorrhage, encephalitis
| Teratogenic
330
Complications of EBV
```
Splenic rupture
CN lesion
Ataxia
GBS
Pancytopenia with megaloblastic marrow
Meningioencephalitis
Chronic fatigue
Burkitt's lymphoma (jaw or abdo mass)
```
331
Features of EBV infection
```
Fever
Malaise
Sore throat
Cervical lymph nodes +++
Splenomegaly
Hepatitis causing hepatomegaly and jaundice
```
332
Investigations in suspeced EBV
Blood smear shows lymphocytosis
| Positive monospot test (previously Paul Bunnel test) a test for Ab produced in response to EBV infection.
333
EBV infects which cell type?
B cells
334
Owl's eye inclusions are seen in...
CMV
335
...% of people infected with hepatitis C develop chronic infection
85%
336
Indications for Hepatitis C treatment
Chronic hepatitis (detectable HCV viral level over a six-month period)
Fibrosis
Raised ALT
337
Hepatitic C treatment
If indicated e.g. chronic infection
Interferon
Ribavarin
However choice is complex and depends on genotyping. Many new options are available.
338
Infective causes of bloody diarrhoea
```
EHEC E.coli
Campylobacter
Shigella
C. Diff (but usually watery)
Entamoeba histolytica
```
339
Commonest cause of travellers' diarrhoea
E. coli (ETEC)
340
Features of giardiasis
```
Bloating
Explosive diarrhoea
Offensive gas
1-4 incubation period
Malabsorption leading to steatorrhoea and weight loss
```
341
Features of amoebic dysentery
```
Dysentery
Wind
Tenesmus
Bloody stools
1-4 week incubation period
Weight loss if chronic
Can lead to liver abscess
```
342
Treatment of amoebic dysentery
Metronidazole (800mg TDS) 5 days or 10 days if liver abscess
Tinidazole
343
Features of Lyme disease
Early localised: erythema migrans (target lesion)
Early disseminated:
Malaise, LN, migratory arthritis, hepatitis
Late persistent: arthritis, focal neuro (Bell's Palsy), heart block, myocarditis
Lymphocytoma: red/blue ear lobe.
344
List the Centor criteria
```
The Centor criteria* are as follows:
Presence of tonsillar exudate
Tender anterior cervical lymphadenopathy or lymphadenitis
History of fever
Absence of cough
```
345
Formula for corrected QT interval
Framingham formula: QT + 0.154 (1 – RR)
| Bazett’s formula: QTC = QT / √ RR
346
On an ECG 1 small square=
0.04ms/0.1mV
347
Features of polycystic kidney disease
MISSHAPES
```
Mass: abdo mass and flank pain
Infected cyst
Stones
SBP raised
Haematuria or haemorrhage into cyst
Aneurisms: berry leading to SAH
Polyuria
Extra-renal cysts e.g. liver
Systolic murmur: mitral valve prolapse
```
348
What is Kussmaul's sign
The JVP increasing with inspiration is known as Kussmaul's sign and can be a feature of constrictive pericarditis. It is caused by impaired filling of the right ventricle due to a poorly compliant myocardium or pericardium. The JVP should fall with inspiration due to reducing pressure in the thoracic cavity.
349
Key differences between cardiac tamponade and constrictive pericarditis
Cardiac tamponade Constrictive pericarditis
JVP Absent Y descent X + Y present
Pulsus paradoxus Present Absent
Kussmaul's sign* Rare Present
Characteristic features Pericardial calcification on CXR
350
ECG changes tricyclic overdose
ECG changes include:
sinus tachycardia
widening of QRS
prolongation of QT interval
