PACES 1 Flashcards
(233 cards)
1
Q
Common causes of splenomegaly and investigations
A
Haem- CML, myelofibrosis, hereditary spherocytosis, haemolytic anaemia, ITP
Infective- malaria, visceral leishmaniasis, EBV, IE
Infiltrative- amyloidosis, Felty’s, SLE
Other- portal HTN, splenic vein thrombus, HIV
Investigations- FBC, blood film, thin/thick films, US, CT, LN biopsy
2
Q
Hereditary spherocytosis- signs, inheritance, investigation, management
A
Signs- Splenomegaly, anaemia, jaundice, cholecystectomy.
Autosomal dominant
Ix- FBC, retics, EMA/osmotic fragility test, direct coombs test (to rule out AIHA)
Mx- folic acid, splenectomy, transfusions
3
Q
Pulmonary fibrosis- signs, Ix, Mx, spirometry pattern
A
Signs- Clubbing, LTOT, cyanosis, fine creps, CT disease signs
Ix- CXR, HRCT, bloods (ANA, ENA, ANCA), spirometry, obs/ABG
Mx- pirfenidone, nintendanib, steroids, transplant, supportive
Spirometry- restrictive and reduced gas transfer
4
Q
Causes of upper zone lung fibrosis
A
Coal workers pneumoconiosis, histiocytosis, ank spond, ABPA, radiation, TB, silicosis, sarcoidosis
5
Q
Causes of lower zone lung fibrosis
A
RA, asbestosis, CT disease, IPF, other (drugs- MTX, nitro, amio)
6
Q
ADPKD- types, signs, complications, management
A
Type 1 and 2- type 1 more rapidly progressive to ESRF
Signs- uni/bilateral ballotable kidneys, signs of RRT/lines, nephrectomy
Complications- HTN, berry aneurysms, MVP, cystic liver
Management- RRT, transplant, nephrectomy, control HTN (ACEi/ARB), genetic counselling, 5 yearly screening for berry aneurysm, 3L fluid per day, screen family
7
Q
ABPA- what is it and who gets it? Ix? Mx?
A
Asthmatics, immunocompromised and CF patients- colonise aspergillus fungus in airways and unable to clear it they develop an immune response/hypersensitivity reaction
Ix- aspergillus RAST, IgE, eosinophils, CXR, HRCT
Mx- steroids, itraconazole
8
Q
EAA/hypersensitivity pneumonitis- pathology, causes, Mx
A
Exposure to allergen causes hypersensitivity reaction- fever, cough, sob, wheeze
Causes- mushroom worker, bird fancier, malt worker, farmers lung
Mx- steroids and avoid trigger
9
Q
Pneumonectomy- scar, signs, indications
A
Scar- lateral thoracotomy, drain scars
Signs- hemithorax fills with fluid, dull to percuss, no air entry, trachea deviated towards side of surgery, reduced chest expansion
Indications- cancer, TB, PTX, COPD, trauma, aspergilloma, abscess, CF
10
Q
Lobectomy- scar, signs indications
A
Scar- lateral thoracotomy, drain scars
Signs- may be normal. Hyperexpansion of remaining lobes, reduced air entry over lobectomy area
Indications- cancer, TB, PTX, COPD, trauma, aspergilloma
11
Q
FEV1 required for lobectomy and pneumonectomy
A
Pneumo >2L
Lobectomy >1.5L
12
Q
Stigmata of chronic liver disease
A
Dupuytrens contracture, palmar erythema, clubbing, gynaecomastia, jaundice, spider naevi, caput medusae, ascites, hepatomegaly
13
Q
Tuberous sclerosis- inheritance, complications
A
Autosomal dominant
Angiomyolipoma in kidneys, epilepsy, ash leaf/shagreen patches, hamartomas, cystic lung disease
14
Q
Causes of CLD
A
ETOH, NAFLD, viral hepatitis, AI hepatitis, PBC, PSC, A1AT deficiency, Wilson’s, HCC, liver mets, haemochromotosis, right heart failure
15
Q
What monitoring should be offered in CLD
A
6 monthly US and AFP for HCC, yearly OGD for varices
16
Q
Causes of hepatomegaly
A
Malignancy- HCC, mets
CLD- ETOH, NAFLD, PBC, haemochromotosis
Infective- acute/chronic Hep, abscess, cyst
Infiltrative- amyloid, gauchers, sarcoid
Vascular- budd-chiari
Congestive- CCF, constrictive pericarditis
17
Q
Budd-Chiari syndrome
A
Hepatic vein occlusion of unknown cause
18
Q
Wilson’s disease - inheritance, pathology, Ix, complications, Mx
A
Autosomal recessive
Copper accumulation in liver, heart, brain, eyes joints
Ix- high serum cooper, low caeruloplasmin, genetic testing
Parkinsonism, cirrhosis, psychosis, kayser-fleischer rings
Penicillamine, transplant, low copper diet
19
Q
Causes of hepatosplenomegaly
A
portal HTN
Haem- myelofibrosis, spherocytosis, AIHA, thalassaemia
Infection- malaria, visceral leishmaniasis, EBV, CMV
Infiltrative- sarcoid, amyloid
20
Q
Thalassaemia- signs, Ix, Mx, complications
A
Signs- frontal bossing, hepato/splenomegaly, pallor, jaundice, cholecystectomy scar
Ix- haemoglobin electrophoresis
Mx- transfusion, folic acid, splenectomy, iron chelation
Complications- iron overload
21
Q
What characterises bronchiectasis?
A
Abnormal dilation of bronchial walls due to loss of elasticity and muscular tone. Chronic inflammation,
retention of secretions, oedema, scarring and ulceration of bronchial wall. Recurrent infections cause further dilation resulting in a vicious cycle of impaired clearance, infection, and bronchial damage.
22
Q
Causes of bronchiectasis
A
Childhood infections: pertussis, TB
Congenital: CF, kartageners
Hypo-immune: CVID, hypogammaglobulinaemia
Hyper-immune: ABPA
Rheum: SLE, IBD, RA, Sjogrens
Idiopathic
23
Q
Where would you see VATS scars and give some indications
A
2-3 2cm port side scars on thorax
Indications: biopsy, removal of mass, pleurodesis
24
Q
Signs of pulmonary HTN
A
RV heave, palpable P2, loud P2, TR (PSM), displaced apex beat
25
Signs of thoracoplasty
Posterior thoracotomy scar, tracheal deviation towards affected side, flattening of chest wall, reduced expansion, absent ribs, dull percussion, reduced breath sounds
26
Types of pleural effusion and causes
Transudate: CCF, nephrotic syndrome hypothyroid, Meig's
Exudate: empyema, parapneumonic, TB, malignancy, SLE, RA, sarcoid, PE
Chylothorax
Haemothorax
27
HIV- common infections/complications
Candida, herpes zoster, PCP, TB, CMV, lymphoma, Kaposi's sarcoma
28
Types of jaundice and causes
1. Pre-hepatic: AIHA, sphero/elliptocytosis, Gilbert's, transfusion incompatibility, SCD, thalassaemia
2. Hepatic: paracetamol, ETOH, viral hepatitis, EBV, CMV, HCC, NASH, haemochromotosis, Wilson's
3. Post-hepatic: gallstones, biliary tract malignancy, PBC, PSC, pancreatitis
29
What are the clinical and echo markers of severe AS?
Clinical: lengthening of murmur, evidence of LV failure, quiet/absent S2, narrow pulse pressure
S4, thrill
Echo: area <1cm2, gradient >40mmHg
30
Where is a normal apex beat located?
5th ICS MCL
31
Causes of AR
Aortic dissection, IE, bicuspid AV, Marfans, syphilis, ank spond, SLE, RA
32
Indications for surgery in AR
Angina, CCF, EF <50%, aortic root diameter >50mm
33
Signs of an ASD
Parasternal heave and palpable thrill, fixed split S2, loud P2, ESM
34
Signs of Turner syndrome
Short stature, shield like chest, wide spaced nipples, webbed neck, high arched palate, coarctation of the aorta
35
Signs of coarctation of the aorta
Radio-radial/radio-femoral delay, thoracotomy scar, ESM over LSE radiating to back
36
Signs of HCM
AF, heaving apex beat, ESM at LSE (with no radiation and louder on valsalva), PSM of MR, signs of CCF
37
Causes of MR
Rheumatic heart disease, IE, IHD, functional MR due to LV dilatation, Marfans, SLE, Ehler's danlos, chordae rupture post MI, trauma
38
Signs of mitral stenosis
AF, malar flush, elevated JVP, RV heave/palpable S2, opening snap, mid-diastolic rumbling murmur, signs of anticoagulation
39
Signs and associations of MVP
Displaced apex beat, AF, mid-systolic click, with late crescendo-decrescendo murmur
Causes: idiopathic, CT disease (marfans, EDS, SLE), ADPKD, myotonic dystrophy
40
Causes of pHTN
1. Idiopathic/PAH
2. Secondary to left heart disease
3. Secondary to hypoxic lung disease
4. CTEPH
5. Other
41
Signs of TR
Distended JVP with giant V waves, RV heave, palpable P2, PSM at LSE, pulsatile hepatomegaly
42
Signs of PDA
wide pulse pressure, eisenmenger's, continuous machinery murmur in left subclavicular region, palpable thrill, S2 may be obscured by murmur
43
Features of ToF
VSD, PS, over-riding aorta, RVH
44
Signs and causes of VSD
Downs syndrome, parasternal heave and thrill, PSM at LSE (very loud all over precordium)
Causes: congenital, Down's, ToF, post MI, post surgery
45
CF- inheritance, signs, complications
Autosomal recessive
Short stature, clubbing, lines, bronchiectasis, diabetes
Diabetes, infertility, bronchiectasis, DIOS
46
4 causes of nephrotic syndrome
Minimal change disease, membranous glomerulonephritis, focal segmental glomerulosclerosis, mesangiocapillary glomerulonephritis
47
PD - signs
Asymmetric resting tremor, bradykinesia, show/shuffling/festinant gait, hypomimia, micrographia, autonomic signs, rigidity dysphonia
48
Types of PD
Idiopathic
PSP - vertical gaze palsy
MAS- autonomic and cerebellar symptoms
CBD - alien limb
Vascular - gait predominant
Drug induced
DLB- hallucinations and cognitive impairment
49
Mx of PD
Hold off if symptoms mild due to tolerance
1st levodopa and dopamine dexcarboxylase inhibitor (co-beneldopa, madopar)
COMT inhibitor
MAO-B inhibitor
Dopamine agonist
Amantadine
DBS
50
Indications for LTOT
pO2 <7.3 or pO2 <8 with secondary polycythaemia or right heart failure
51
Signs of COPD
Cyanosis, Co2 retention, LTOT, barrel shaped chest, tar staining, prolonged expiratory phase, cor pulmonale
52
Autoantibodies in CLD
PBC- anti mitochondrial
PSC - anti smooth muscle
AI hepatitis - anti LKM1, anti SM, ANA
53
NMO- signs, antibodies, how to differentiate
Transverse myelitis and optic neuritis (demyelination)
Symptoms more severe than MS but MRI changes less, no OCB
Anti aquaporin 4 ab's
54
Signs of lateral medullary syndrome and vessel affected
Posterior inferior cerebellar artery
Ipsilateral horners, contralateral hemisensory loss (pain and temp), nystagmus, dysphagia, vertigo
55
Pyramidal weakness pattern
UL's flexed
LL's extended
UNILATERAL
56
Causes of RAPD
Optic nerve pathology
CRAO/CRVO, glaucoma, MS, optic neuropathy, tumour, retinal detachment
GCA until proven otherwise in 50+
57
Causes of cerebellar dysfunction
Paraneoplastic
Alcohol
Stroke
Tumour
MS
58
What is INO
Failure of adduction of affected eye with nystagmus on abduction of contralateral eye
59
TACS vs PACS vs lacunar stroke
TACS: contralateral homonomous hemianopia, contralateral hemiplegia, high cortical dysfunction
PACS: 2/3 above
Lacunar: pure hemimotor/sensory loss
60
CI's to thrombolysis
ICH, SoL, seizure at onset, anticoagulation, uncontrolled HTN, LP within 1/52, surgery within 3/12, UGIB within 3/52, stroke within 3/12
61
Features of dermatomyositis and polymyositis
Dermatomyositis: Gottrons papules, heliotrope rash, elevated CK, legs affected more than arms
Polymyositis: progressive, symmetric, proximal weakness
62
Charcot-Marie-Tooth- signs, inheritance, Ix, Mx
5 different types of varying inheritance
Aka hereditary sensory motor neuropathy
Reduced sensation, wasting, weakness.
Pes cavus, inverted champagne bottle legs, footdrop, hammertoe
Ix: NCS
Mx: AFO, PT/OT
63
Causes of proximal myopathy
ETOH, drugs (statins, steroids), dermato/polymyositis, muscular dystrophy, MG/LEMS, Cushings, hypothroid, acromegaly
64
Causes of ptosis
3rd nerve palsy
Myotonic dystrophy
MG
Horner's
Congenital
Age
SoL
65
Triad and ab's in Miller-Fisher
Anti gq1b abs
Triad- areflexia, ataxia, ophthalmoplegia
66
Causes of nystagmus
Cerebellar disease, BPPV, posterior stroke, Wernicke's, MS, stroke, tumour, trauma, drugs
67
Top reasons for liver transplantation
Cirrhosis, acute hepatitis (paracetamol, infectious), HCC
68
Top reasons for renal transplantation
HTN, Diabetes, ADPKD, chronic glomerulonephritis
69
Haemochromotosis- inheritance, signs, investigations, management
HFE gene, autosomal recessive
Diabetes, bronzed, slate-grey, venesection scars, hepatomegaly, signs of CLD, arthralgia, cardiomyopathy
Ferritin >200 females and >300 males, elevated t sats
Regular venesection, desferrioxamine, transplant, family screening, ETOH avoidance
70
UMN pattern, unilateral weakness
Tumour, stroke, hemisection, brain abscess
71
UMN pattern, bilateral, pure motor
Hereditary spastic paraparesis
72
UMN pattern, bilateral, mixed motor and sensory
MS, SCD, syringomyelia, transverse myelitis, Freidrich's ataxia, cervical spondylosis
73
LMN pattern, pure motor, bilateral, proximal
Statins, steroids, hypothyroid, MG, LEMS, dermato/polymyositis, muscular dystrophy, MND, spinomuscular atrophy
74
LMN pattern, pure motor, bilateral, distal
Myotonic dystrophy, MND, polio
75
LMN pattern, pure motor, bilateral, asymmetric
Mononeuritis multiplex
76
LMN pattern, mixed motor and sensory, bilateral, proximal
CIDP
77
LMN pattern, mixed motor and sensory, bilateral, distal
Charcot-Marie-Tooth, diabetic peripheral neuropathy, alcohol related peripheral neuropathy, GBS, CIDP, paraneoplastic, cauda equina
78
LMN pattern, mixed motor and sensory, asymmetric
Radiculopathy, plexopathy, vasculitis, compression neuropathy, chemo
79
UMN signs
UMN: spasticity, hyperreflexia, clonus, positive Babinski. No fasciculations, mild wasting
80
LMN signs
LMN: flaccid, hyporeflexic / arreflexic, absent Babinski. Severe wasting with fasciculations
81
Dysphonia vs dysphasia vs dysarthia
Dysphasia - language disorder, inability to understand or generate speech
Dysarthia - speech disorder, articulation and pronunciation
Dysphonia - inability to properly formulate sound from the larynx
82
Causes of brainstem pathology
Vascular (ischaemic / haemorrhagic stroke)
Inflammatory (multiple sclerosis, acute disseminated encephalomyelitis)
Brainstem encephalitis (Bickerstaff’s encephalitis)
Central pontine myelinolysis
Trauma
Neoplastic (brainstem gliomas / ependymomas)
Arnold-Chiari malformation
Syringobulbia
83
Signs and causes of CNIII palsy
Ptosis, dilated pupil, down and out eye
Causes: posterior communicating artery aneurysm, DM, GCA, SoL, MS, cavernous sinus thrombosis, surgery, SLE, PAN
84
Signs and causes of CNVI palsy
Esotropia (permanent adduction of affected eye), paralysis of lateral gaze with diplopia
Causes: ischaemia, trauma, MS, SoL, stroke, sarcoid, GCA, raised ICP (false lateralising sign)
85
Signs and causes of CNVII palsy
Weakness of muscles of facial expression (forehead sparing if UMN lesion), hyperacusis
Causes: stroke, SOL, MS, lyme disease, sarcoid, syphilis, Bell's palsy, Ramsay-Hunt (HSV- ear vesicles)
86
Signs and causes of cavernous sinus syndrome
Signs: painful ophthalmoplegia, orbital congestion, orbital chemosis, periorbital oedema, proptosis, ipsilateral Horner’s syndrome, ipsilateral loss of facial sensation
Causes:
Vascular: carotid artery aneurysm, carotid-cavernous fistulas, cavernous sinus thrombosis
Infections: sinuses
Tumours: nasopharyngeal carcinoma, craniopharyngioma, pituitary adenoma, mets, Inflammatory: sarcoidosis, GPA
87
Bulbar palsy- what is it, signs, causes
Disorder of lower cranial nerves (7-12)
Signs: nasal speech, slurred words, fasciculating and weak tongue, absent jaw jerk, reduced gag reflex, drooling, dysphagia
Causes: MND, myotonic dystrophy, myasthenia gravis, MSA, syringobulbia, poliomyelitis, leptomeningeal disease, radiation damage, nasopharyngeal carcinoma
88
Signs and causes of Horner's syndrome
Signs: partial ptosis (overcome by upgaze and not fatigable), miosis, anhidrosis
Causes: stroke, syringomyelia, pancoast tumour, thyroid mass, cavernous sinus thrombus, ICA dissection,
89
Pathology, Signs of Freidrichs ataxia + inheritance
Damage to cerebellum, spinal cord and peripheral nerves. Causes mixed UMN/LMN signs
Autosomal recessive
Signs:
Progressive cerebellar ataxia
Hyporeflexia / arreflexia
Distal weakness, initially involving the lower limbs
Dorsal column loss, initially distal
Upgoing plantars
Dysphagia
Reduced visual acuity
Sensorineural hearing loss
Kyphoscoliosis
Pes cavus
Urinary incontinence
Cardiomyopathy
Diabetes mellitus
90
Signs of spinocerebellar ataxia + inheritance
Autosomal dominant
Signs:
Usually presents in third or fourth decade
Progressive cerebellar ataxia
Dysarthria, bulbar palsy
Increased tone
Hyperreflexia
Extensor plantar responses
May have peripheral neuropathy causing fasciculations and wasting
91
3 types of ataxia
1. Cerebellar
2. Sensory - loss of proprioception, romberg's +ve, stomping gait
3. Vestibular - vertigo, N&V
92
Describe sensory ataxia, signs and give causes
Loss of proprioception, near-normal coordination when the movement is visually observed by the patient, but marked worsening of coordination when the eyes are shut, pseudoathetosis, stomping gait, romberg's positive
Causes
Peripheral neuropathy- alcohol, diabetes, nutritional deficiency
DCML disruption- tabes dorsalis, SCD, posterior column demyelination
93
What is pseudoathetosis and what is the cause
Sensory ataxia
Random finger movements seen on outstretched hands with eyes closed
94
Signs and causes of vestibular ataxia
Vertigo + nystagmus
BPPV, vestibular neuritis
95
Causes of cerebellar ataxia
Paraneoplastic
Alcohol
Tumour
Stroke
Inherited- spinocerebellar, freidrichs
B12 deficiency
MS
Sarcoid
MSA
Lyme disease
Wilsons disease
Ataxic telangiectasia
96
Indications for lung transplant
CF
Bronchiectasis
Pulmonary fibrosis
COPD
Pulmonary vascular disease
97
Signs and causes of Brown Sequard
Signs: ipsilateral loss of vibration and proprioception, ipsilateral hypertonia, hyper-reflexia, pyramidal weakness, contralateral loss of pain and temperature
Causes: demyelination, trauma, tumour
98
Syringomyelia - signs
Cape like distribution of sensory loss, wasting of small muscles of hand
99
CES- signs, causes
Signs: sensory level, wasting, weakness, areflexia, bladder/bowel dysfunction
Causes: prolapsed disc, tumour, bony mets, epidural abscess
100
Causes of proximal weakness
Alcohol
Statins
Cushings, acromegaly, hypothyroidism
DMD, Becker's, FSHD
Dermato/polymyositis
MG/LEMS
101
What is S3? Give some causes
Additional heart sound heart just after S2. Heard in early-mid diastole, when the ventricles are passively filling. The sound is of blood striking a compliant left ventricles
Causes: LV dysfunction, normal in athletes/kids, MR, VSD (rapid ventricular filling)
102
Causes of wasting of hand muscles
Cervical myelopathy
Anterior horn cell disease
Motor neurone disease
C8/T1 radiculopathy
Pancoast’s syndrome
Lower brachial plexopathy
Cervical rib
Mononeuropathy
Charcot-Marie-Tooth disease
Myotonic dystrophy
Inclusion body myositis
Distal spinal muscular atrophy
Disuse atrophy
103
Give some extra-articular features of RA
Renal- glomerulonephritis
Eye- scleritis, episcleritis
Lung- fibrosis, effusions
Heart- pericarditis
Splenomegaly
Rheumatoid nodules
Anaemia
Carpal tunnel
104
What criteria are used to diagnose MS?
MacDonald's criteria- lesions disseminated in time and space
105
Signs/symptoms of MS
Eyes: optic neuritis, RAPD, INO, nystagmus, red desaturation
Cerebellar signs
Limbs: hypertonia, hyperreflexia, clonus, upgoing plantars, pyramidal weakness
Autonomic dysfunction- catheter
106
Suggest some investigations to be done in MS
MRI- peri-ventricular white matter changes seen on FLAIR sequence, gadolinium for determining acuity
LP- paired with serum for OCB
VEP's
Anti aquaporin4 ab's to r/o NMO
107
Suggest 3 patterns of MND
1. Limb - asymmetric distal weakness, wasting of small muscles of hand, difficulty in motor tasks
2. Bulbar - tongue fasciculations, change in speech, drooling, dysphagia, emotional lability
3. Respiratory - SoB, orthopnoea, OSA
108
What is Hoffmans sign and what is it indicative of
Flick the end of the middle finger- positive/pathological is the opposition of index finger and thumb
Indicative of cervical cord compression/myelopathy
109
Describe post-polio syndrome
Asymmetric flaccid paraparesis of a single limb
Reflexes usually absent, no UMN signs
Atrophy, fatigue, pain
110
Pathophysiology and signs of MG
Ab's directed against nicotinic acetylcholine receptors at the NMJ
Ocular/ generalised
Fatigable weakness, ptosis, diplopia, dysphagia, dysarthria, proximal weakness, complex ophthalmoplegia, bulbar weakness
111
Specific examination tests and Ix in MG
Look for thymectomy scar
Look upward for 20 seconds
Abduct both shoulders then repeatedly 'flap' one and test power again- should be weaker once fatigued
Ice pack test, tensilon test
nAChR ab's, antiMUSK, VGCC's (LEMS)
Electrophysiology
CT thorax
112
What could trigger a myasthenic crisis and how would you manage it
Infection, stress, non-compliance with treatment, dugs (b blockers, CCB's. lidocaine, abx)
IVIg, PEX, intubation to protect airway
113
Management of MG
Pyridostigmine (acetylcholinesterase inhibitor)
Steroids (may need to admit to monitor if giving high doses)
Immunosuppression (azathioprine, mycophenolate)
Thymectomy
114
Inheritance, associated features and signs of myotonic dystrophy
Autosomal dominant
Cataracts, diabetes, cardiomyopathy/conduction disease, MVP, low IQ
Myotonic facies, balding, ptosis, cataracts, dysarthria, distal weakness, grip myotonia,
115
How to examine a tremor
Observe arms resting, symmetry, speed, nature of tremor
Synkinesis- ask to open and close fist on one hand and see if resting tremor worsens
Outstretch arms and check past pointing
116
Triad of NPH
Incontinence
Gait disturbance
Cognitive impairment
117
What is Light's criteria?
Exudate = Pleural protein: serum protein > 0.5
Pleural LDH : serum LDH >0.6
118
Give some causes of clubbing
Resp: cancer, bronchiectasis, fibrosis, mesothelioma, TB
Cardio: IE, cyanotic congenital heart disease
GI: IBD, CLD, coeliac disease
119
Differential diagnoses of neck lump
1. Congenital e.g. branchial/thyroglossal cyst
2. Lymphadenopathy - reactive, neoplastic, inflammatory
3. Thyroid mass e.g. goitre, carcinoma, nodule
4. Lipoma
5. Salivary gland neoplasm
120
How to examine a neck lump
Hand- tremor, sweaty
Pulse ?AF
Face- eyes, pallor, eye movements
Neck- palpate nodes, palpate mass, ask to stick tongue out and swallow
Extra: heart sounds, splenomegaly
121
HHT - inheritance, presentations, complications
Autosomal dominant vascular disorder
Characterised by mucocutaneous bleeding and AVM's
Epistaxis, GI bleeding, SAH, haemoptysis
Mucocutaneous telangiectasia
122
Marfans syndrome- signs/complications
Scoliosis
Pectus deformity
Arachnodactyly
Micrognathia
High arched palate
Hypermobility
Lens dislocation
Myopia
Aortic aneurysm/dissection
MVP, MR/AR
Pneumothorax
123
Marfans- inheritance and genetics
Autosomal dominant
Defect in Fibrillin-1 gene
124
Management of Marfans
Lifelong b-blockers to protect aorta
Regular echo surveillance
Genetic counselling
Regular ophthalmology input
Aortic surgery if diameter >50mm or >45mm with FHx aortic dissection
125
Clinical features of non-proliferative diabetic retinopathy
No neovascularization
Microaneurysms
Soft exudates (cotton wool spots)
Hard exudates – lipid deposition
Haemorrhages (dot or blot)
Venous beading
126
Clinical features of proliferative diabetic retinopathy
Neovascularisation from disc/retinal vessels
127
What are the 4 stages of diabetic retinopathy, give complications
Mild, mod, severe non-proliferative
Proliferative
Vitreous haemorrhage
Retinal detachment
Macula oedema
128
Risk factors for diabetic retinopathy
Longer duration of diabetes, hyperglycaemia, pregnancy, hypertension and nephropathy.
129
CRVO- presentation, risk factors, fundoscopy appearance, management
Sudden onset, painless visual loss
RF's: age, diabetes, HTN, smoking, hypercoaguable, obesity, glaucoma
Signs: RAPD, diffuse retinal haemorrhage, dilated tortuous veins, macula oedema, cotton wool spots
Mx: refer ophtal, VEGF, laser
130
CRAO- presentation, risk factors, fundoscopy appearance, management
Sudden onset painless visual loss/disturbance
RF's; hypercoaguability, AF, carotid artery stenosis, GCA, PAN, SLE, polycythaemia, Waldenstroms
Signs: RAPD, pale oedematous retina with cherry red spot at macula
Mx: treat cause, ocular massage, intra-aterial thrombolytics
131
Retinitis pigmentosa associations
Primary RP, mitochondrial disorders (Kearns-sayre), Freidrich's ataxia
132
RP- presentation, fundoscopy signs, other associated signs
Loss of peripheral vision/tunnel vision 'bumping into things'
Peripheral bone spicule pigmentation with relative macula sparing
Waxy pallor of the optic disc
Attenuation of retinal blood vessels
PPM, proximal myopathy, ataxia
133
What are the types of scleroderma
Limited scleroderma
Systemic sclerosis (limited and diffuse cutaneous)
134
What is the difference between limited and diffuse systemic sclerosis and which ab's are found in each?
Limited cutaneous = skin changes distal to elbows and knees, and face. Aka CREST syndrome. Anti-centromere ab's
Diffuse cutaneous = skin changes proximal to elbow/knees. Renal, GI, lung and cardiac involvement. Anti-SCl-70 ab's
135
Risk factors for gout
Male
High alcohol/ red meat diet
Diuretics
Ciclosporin
CKD
HTN
TLS
Myeloproliferative disorders
136
Gout: investigations and management
Ix: joint aspiration (negatively birefringent crystals)
Acutely: NSAID's, colchicine, steroids
Chronic: allopurinol/febuxostat (if 2 or more attacks per year)
137
Features of ankylosing spondylitis
Back pain- worse in morning, better with exercise, better with NSAID's
Sacroiliitis
138
Features of ankylosing spondylitis
Back pain- worse in morning, better with exercise, better with NSAID's
Sacroiliitis
Achilles enthesitis
Aortic regurgitation
Apical pulmonary fibrosis
Atlanto-axial subluxation
Anterior uveitis
139
Ix/Mx of ankylosing spondylitis
Schober test- measure 10cm up 5cm down from dimples of venus, area should increase by >5cm on felxion
HLA-B27
X-rays
Echo
Not DMARD's
Long term NSAID's
Maybe rituximab
140
Triggers/links to psoriasis
Trauma- Koebner's phenomenon
Drugs- b-blocker, NSAID's, lithium, antimalarials
HIV
141
Management of psoriasis
Emollients
Topical steroids
Topical vitamin D
Topical tacrolimus
Phototherapy
142
Features and management of psoriatic arthritis
Features: psoriatic skin changes, OA/RA/arthritis mutilans, enthesitis, dactylitis, spondyloarthritis
Seronegative
Mx: MTX, sulfasalzine, leflunomide, ciclosporin
143
Complications of RA
Anaemia of chronic disease
Cushing's from steroids
Accelerated cardiovascular disease
Felty's syndrome
Osteoporosis
144
Management of RA
Steroids – exogenous Cushing’s, osteoporosis, cataracts, weight gain
Methotrexate – pulmonary fibrosis
Sulphasalazine – rash
Hydroxychloroquine – retinitis
Biologics – opportunistic infections
145
Triad in MEN1
Pituitary adenoma
Parathyroid adenoma
Pancreatic tumour (insulinoma, Zollinger-Ellison, VIPoma)
146
Symptoms of pituitary adenoma
Visual field defects- bitemporal hemianopia
Headaches
Diplopia
Hormone deficiencies (compression of non-functioning adenomas)
Decreased libido / fertility (hypogonadotropic hypogonadism)
Cold intolerance, lethargy (hypothyroidism)
Lethargy, postural dizziness (hypoadrenalism)
Hormone excess (functioning pituitary adenoma)
Acromegaly
Cushing’s disease
Galactorrhoea, amenorrhoea, erectile dysfunction (prolactinoma)
147
What hormones are released by the pituitary gland?
Anterior: TSH, LH/FSH, GH, prolactin, ACTH
Posterior: oxytocin, ADH
148
Signs/symptoms of Cushing's
Central adiposity, neck fat pad
Striae
Moon face
HTN
Hyperglycaemia
Cataracts
Proximal myopathy
Acne
Hursutism
149
Causes of Cushing's
Iatrogenic glucocorticoids
ACTH secreting pituitary adenoma
Adrenal tumour
SCLC/carcinoid tumour
150
Investigations for Cushing's
24 hour urine cortisol
Overnight dexamethasone suppression test
MRI pituitary
CT adrenals
151
Causes of hypothyroidism
Iodine deficiency
AI disease (Hasimoto's-- anti TPO ab's)
Thyroidectomy, radio-iodine, radiotherapy
Amiodarine, lithium
Secondary hypothyroidism e.g. pituitary tumour
152
Causes of hyperthyroidism
Excess levothyroxine
Grave's disease (TSH receptor ab's)
De Quervains thyroiditis
Post-partum thyroiditis
Toxic adenoma
Multinodular goitre
Lithium
Amiodarone
153
Sx/presentation of acromegaly
Headaches
Visual disturbance
Sweating
Carpal tunnel
Diabetes
HTN
Change in appearance
OSA
Macroglossia, teeth separation
Colonic polyps
154
Ix for acromegaly
IGF1
OGTT (failure to suppress GH is +ve)
MRI head
Complications: HbA1c, BP, echo, colonoscopy, TSH/ACTH/LH/FSH/prolactin, sleep study
155
Management of acromegaly
Trans-sphenoidal resection of adenoma
Octreotide/lanreotide (somatostatin analogue)
Pegvosimant (GH receptor antagonist)
156
How do you manage a TIA?
Historically ABCD2 score used
If symptoms within last week, refer for assessment within 24 hours. If symptoms > 1 week ago refer for Ix within a week.
CT head
Aspirin 300mg then 75mg OD
Statin
Not drive for 1 month
Lifestyle mods inc stopping smoking
TIA clinic- carotid dopplers, MRI
157
DVLA advice for group 1 licence for; TIA, stroke, MI, seizure
1. TIA: not drive for 4 weeks, don't ned to tell DVLA
2. Stroke: not drive for 4 weeks, do not need to tell DVLA unless significant remining disability may need reassessment
3. If PCI then 1 week ban, if no PCI then 4 weeks. Don't need to tell DVLA
4. Stop driving for 6 months awaiting further Ix, then after further assessment may be able to restart after 6 months/1 year seizure free. Need to tell DVLA
158
DVLA advice for group 2 licence for; TIA, stroke, MI, seizure
1. TIA/stroke: stop driving 1 year
2. MI: 6 weeks then ETT
3. Seizure: 5 years
159
Main points for DVLA advice in diabetes Group 1 vs 2
Group 1: no restrictions unless disabling hypos or insulin >3 months, then need to inform DVLA
Group 2: no restrictions if diet alone, if on any meds/insulin will need to inform DVLA and satisfy further requirements e.g. hypo awareness
160
When do you refer for RRT?
Refer to renal at CKD4-5 (eGFR <30), or if rapidly progressive CKD3. Aim to refer a year before RRT needed
161
Risks of splenectomy and how to mitiagte these
Risks: susceptible to infection (particularly encapsulated eg pneumococcus, haemophilus), thrombocytosis post-op.
Mitigation: aim to give all vaccinations >2 weeks pre-surgery, Pen V, education, medic alert bracelet
162
Causes of TR
Ebstein's anomaly, pHTN, PS, MS, RV dilatation, carcinoid, IE, rheumatic heart disease
163
How do you develop Eisenmenger's
Chronic left to right shunt results in pulmonary arterial hypertension. Pressure in the right heart eventually equalises then exceeds left heart and shunt reverses
164
Inheritanceof HCM
Autosomal dominant, or de novo
165
Considerations in genetic testing
1. Give cooling off period after consent to change mind
2. Warn about negative effects e.g. implication for insurance, implication on other family members
3. May find things we can't do anything about
4. May find things of uncertain significance
166
How often should diabetics be screened for retinopathy?
Yearly, more frequently in pregnancy
167
Stages/features of hypertensive retinopathy (CACO)
Stage 1 - copper and silver wiring, meaning an increased light reflex of the vessels
Stage 2 - AV nipping
Stage 3 - Cotton wool spots and flame haemorrhages
Stage 4 - optic disc swelling
Stage 3 or 4 should be considered a medical emergency and the patient urgently assessed for other end organ damage (LVH, proteinuria, encephalopathy), a possible underlying secondary cause (via the physical exam and initial investigations) and initiated on treatment
168
Questions to ask in a history of change in vision
Chronicity
Uni/bilateral
All/part of vision
Ability to see colour
Painful/painless
General health- any muscular symptoms, headaches
History of eye problems
PMH/DH/FH
169
Causes of optic disc swelling
Raised ICP- IIH, SOL, intracranial infection, venous sinus thrombosis
Optic neuritis
Ischaemic optic neuropathy- diabetes, HTN, GCA
Hypertensive retinopathy
170
Management of COPD
Non-pharm: pulmonary rehab, smoking cessation, vaccinations, dietician
Pharm: inhalers (SABA, SAMA, LABA, LAMA, ICS), nebulisers, oral steroids, oral theophylline, LTOT, carbocisteine
Also rescue pack
Surgery: bullectomy, volume reduction, transplant
171
What are some complications of diabetes
Retinopathy
Nephropathy
Neuropathy
Autonomic dysfunction e.g. gastric paresis
CVD
Neuropathic pain
Erectile dysfunction
172
HbA1c levels for diagnosis/targets
<42 normal
42-47 pre-diabetes
48+ - diabetes
If known diabetes, target <48
173
Alternatives to HbA1c and reasons for using
Total glycated haemoglobin, fructosamine estimation
Haemoglobinopathies, post-transfusion
174
Gestational diabetes- screening, management, post-partum check
If risk factors then OGTT at 24-28 weeks
If prev GD then OGTT ASAP
Initially trial diet/lifestyle change for 2 weeks with home BM monitoring. If not improved commence metforming then insulin
If fasting glucose >7 commence insulin immediately
Post-partum 6 week fasting glucose
175
Causes of secondary hypertension
Renal: PKD, glomerulonephritis
Endocrine: hyperthyroid, Cushing's, Conn's, acromegaly, phaeochromocytoma
CVS: co-arctation of the aorta, RAS
Iatrogenic: steroids, OCP, mirabegron
OSA
176
Risk factors for osteoporosis
Early menopause
Steroids/Cushing's
RA
Hyperthyroidism
Female
FHx
Smoking
ETOH
177
Causes of foot drop
Congenital: CMT
Traumatic: L4/5, sciatic nerve, common peroneal nerve injuries, disc prolapse, pressure from cast
Diabetes, ETOH,
Inflammatory: PAN, RA, churg strauss
Infective: HIV, Lymes, leprosy
Muscular: MG, MND
178
Pathophysiology and causes of Horner's syndrome
Disruption in sympathetic nerve supply to the eye- could be 1st order (brain to c spine), 2nd order (spinal cord to neck) or 3rd order (neck into face)
Causes:
Congenital
FON: stroke, SOL, wallenbergs, syringomyelia, trauma, MS
SON: pancoast tumour, trauma
TON: cavernous sinus thrombus, carotid artery dissection
179
Causes of a spastic paraparesis
Hereditary spastic paraparesis
CP
MND
MS
Freidrich's ataxia
180
Causes of flaccid paralysis
Trauma
Spinal abscess/epidural haematoma
Poliomyelitis
GBS
MG
Porphyria
Hypokalaemia
Cord compression
Spinal stroke
Acute transverse myelitis
Lyme disease
181
Inheritance and complications of Huntington's disease
Autosomal dominant
Chorea, athetosis, hemiballismus, cognitive impairment, psychiatric complications, rigidity, aspiration pneumonia, seizures, dysarthria, ataxia
182
DDx of chorea
Inherited: Huntington’s disease, Wilson’s disease.
Vascular: Stroke, polycythaemia rubra vera.
Immune mediated: Sydenham’s chorea, SLE, anti-phospholipid syndrome.
Hormonal: Pregnancy (chorea gravidarum), OCP use, hyperthyroidism.
Toxic: Anti-psychotics, dopaminergics in Parkinsonian patients, anti-epileptics.
Infectious: HIV, Lymes.
Paraneoplastic.
183
Management of MND
Riluzole
PEG feeding
NIV
Palliative care input
PT/OT
184
FSHD - inheritance, signs/features
Autosomal dominant
Bilateral ptosis, wasting of facial muscles, hearing aids, PPM, foot drop, winged scapula, proximal weakness, hearing aids
185
Causes of mixed UMN/LMN symptoms
MND
Freidrich's ataxia
Syringomyelia
Dual pathology
186
CT findings in ILD
Groundglass changes
Traction bronchiectasis
Honeycombing
Reticular markings
187
CT findings in bronchiectasis
Dilated airways
Signet ring sign
Bronchial wall thickening
Air trapping
Tram track sign
188
Give some symptoms of HIV seroconversion
Fever, fatigue, myalgia/arthralgia/ lymphadenopathy, malaise, rash, headache
189
Give some examples of AIDS defining illness/CD4 count threshold
CD4 count <200
PCP, TB
Candida
Kaposi's sarcoma
Invasive cervical cancer
CMV
Cryptococcal meningitis
PML
Cerebral toxoplasmosis
190
Indications for antiretroviral therapy in HIV
CD4 count <500
In setting of opportunistic infection
191
What are the 4 ethical principles
1. Beneficence - do good
2. Non-maleficence - do no harm
3. Autonomy- individuals with capacity have the right to make their own decision
4. Justice - act in BI of wider community e.g. fair allocation of resource
192
What principles are considered when making a deicison for osmeone who lacks capcacity
Act in their best interests
Choose the least restrictive option
Consider their prior/family wishes
Take all steps to ensure lack capacity
193
What is an ADRT?
A legal document instructing doctors that a patient does not want artificial life support should he become terminally ill
Can also say other treatments you would not want e.g. artificial feeding, NIV etc
194
Section 135/136
Police use section 135 to remove someone from a public place and take them to a place of safety, here the section 136 is used to detain them awaiting further MHA assessment
195
Section 5(4) vs section 5(2)
5(4) allows nurses to detain you for 6 hours in hospital, but NOT in the ED
5(2) allows a doctor to detain you in hospital for 72 hours for MHA assessment
196
Features of section 2
Detained for 28 days for further assessment of mental health disorder
197
Features of section 3
Detained for up to 6 months for treatment of a mental health disorder
198
Give some examples of notifiable diseases
TB, mumps, measles. rubella, meningitis, campylobacter, p;loio, brucellosis, cholera, SARS, scarlet fever, whooping cough, HUS, enteric fever
199
What must you ensure when you break confidentiality
You are doing so with legal backing
You tell the patient
You reveal the minimum information needed
200
When can you break confidentiality
When required by law or justified in the public interest
- Ordered by a judge
- Notification of infectious disease
- Risk to national security
- Can tell patients at risk of serious communicable disease if original patient will not and cannot be persuaded to do so e.g. HIV
201
Sx/signs of GCA
Headache, throbbing
Jaw claudication
Scalp tenderness
Proximal weakness of PMR
Systemic fever, myalgia
RAPD, pale optic disc
Absent temporal artery pulsation
202
Ix/Mx of GCA
Ix: FBC, U&E, LFT, CRP, ESR, PV
Mx: steroids immediately- 40mg if uncomplicated, 60mg if complicated. Refer ophthalmology- IV methyl pred if visual loss. Temporal artery biopsy
203
Causes of central vision loss (CCHOM)
Macular disease- diabetic maculopathy, ARMD
CRVO
Hypertensive retinopathy
Cataract
Optic neuritis
204
Causes of peripheral visual loss (CCRISG)
RP
Glaucoma
CRAO
Ischaemic optic neuropathy
Stroke
Chiasmal lesion
205
Causes of acute loss of vision
Stroke
Retinal vascular occlusion
GCA
206
Causes of gradual loss of vision
Nerve compression eg tumour
Inherited
Degenerative
Toxic
Nutritional
207
Causes of binocular diplopia
3/4/6 CN palsy
INO
MG
Thyroid eye disease
208
Antibody tests in thyroid disease
anti TPO ab's - Hashimoto's +/- Graves
Thyroid receptor ab's - Grave's
209
Signs in thyroid eye disease
Upper lid retraction
Proptosis
Corneal involvement
Sight loss
210
Screening test in UL exam
Observe at rest- tremor, ask to count from 10-1
Arms out- rebound
Pronator drift and pseudoathetosis
Winging of scapula
Myotonia
Past pointing
Arms behind head- proximal myopathy and scars
211
Spasticity vs rigidity
Spasticity is velocity dependent and pyramidal
Rigidity is not velocity dependent and extra pyramidal
212
Causes of issues at anterior horn cell
Polio, SMA, ALS
213
How to demonstrate visual/spatial neglect and how this helps localise lesion
Cortical problem
Give bilateral simultaneous stimulation eg flap both hands and they will only see one
214
Features of NF1
Cafe au lait patches >6
Neurofibromas
Scoliosis
Axillary freckling
Leisch nodules- melanin in Iris
Optic glioma
ADHD/ LD
Epilepsy
HTN
Phaeochromocytoma
215
Location of lesion in inferior and superior VF defect
PITS
parietal- inferior defect
Temporal- superior defect
216
How to examine eyes
Observe
Pupils and accommodation
Red reflex
Fundoscopy
Visual fields
Eye movements
217
Describe the surgical sieve
Vascular
Infective
Trauma
Autoimmune
Metabolic
Idiopathic
Neoplastic
Congenital
Degenerative
Endocrine
218
Process of diabetic foot exam
1. Observe- hair loss, ulcers, callous formation, Charcot joint, arches, amputation, scars, inspect footwear
2. Gait- stomping, broad stance, high steppage
3. Palliation- heat, pulses
4. Sensation- cotton wool, vibration, joint position sense
219
Inheritance and chromosome in NF1 and 2
Autosomal dominant
Chromosome 17
Chromosome 22
220
Features of NF2
Bilateral sensorineural deafness from bilateral acoustic neuroma
Skin changes uncommon
221
How to perform rheum/hand exam
Inspect hands: nail changes, scars, deforming polyarthropathy, rashes, bruising, raynauds, calcinosis, tophi, wasting
Inspect elbows
Inspect neck and scalp
Palpate- temperature, pulses, sensation, effusions, nodules
Movement/function- make a fist, oppose fingers and thumbs, prayer/reverse prayer sign, elbow extension
222
Effect of SUN on rheum conditions
Worse: lupus, dermatomyositis
Better: psoriasis
No change: sarcoid
223
Scalp involvement in rheum conditions
Scalp involved: psoriasis, lupus
Not involved: sarcoid, dermatomyositis
224
Which rheum conditions cause scarring
Sarcoid and discoid lupus
Psoriasis does not scar
225
Questions to ask in rheum history
When were you diagnosed- if <16 then JIA
Which joints affected
Ever had a rash
Current treatments- include infusions
Previous treatments trialled
226
What does the presence of nodules in RA mean
They are seropositive
227
Histological features of UC
Continuous inflamed mucosa
Crypt abscesses
Mucosal only
228
Extra-intestinal features of UC
Iritis, uveitis, episcleritis
Erythema nodosum
Pyoderma gangrenosum
Ank spond
Osteoporosis
PSC
Anaemia
VTE
229
Histological findings in Crohn’s
Transmural inflammation
Skip lesions
Fistulas
230
Escalation of treatment for UC
Steroids
5ASA
Azathioprine
Biologics eg inflixmab
Surgery
231
Define pulmonary hypertension
PAP >25mmHg at rest
232
MS- management and efficacy
Beta interferon
Glatiramer
Decrease relapses on RRMS but do not alter disease progression
Also fingolimod, natalizumab
233
Causes of SIADH
Drugs- ssri’s, carbamazepine, amitriptyline
Brain injury
Infections
Hypothyroid
Sclc
