Clinical Consultation Flashcards

1
Q

Causes of breathlessness in ankylosing spondylitis

A

Anaemia
Apical pulmonary fibrosis
Aortic regurgitation
Mechanical restriction

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2
Q

Treatment in ankylosing spondylitis

A

Patient education
PT/OT
Hydrotherapy
Smoking cessation
NSAIDS - naproxen/meloxicam
Anti-TNF agents eg adalimumab
Anti-Il-17 - secukinumab
Jak inhibitors - upadacitinib

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3
Q

Pretreatment considerations with Anti-TNF treatment

A

Immunisations
Screen for latent/active Tb

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4
Q

Ix in ankylosing spondylitis

A

FBC - Anaemia
CRP and ESR
U&Es and LFTS
HLA-B27
CXR if chest symptoms
XR of spine and pelvis - syndesmophytes and sacroilitis, fusion of spine
MRI spine can consider
AS symptom index - out of ten- >4 - active disease

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5
Q

Management of acute IBD

A

FBC
CRP
U&Es and LFTs
Stool culture
Faecal calprotectin
AXR
Analgesia
IV hydrocortisone
IVF
pLMWH

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6
Q

Complications of IBD

A

Dilatation of bowel loops
Fistulating disease
Perforation
Abscess formation
Refractory to medical management

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7
Q

Tx following acute flare of IBD

A

Tapering course of steroids
Escalation of DMARDs eg anti-TNF agent
IBD nurse
Dietician
Flexi Sig

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8
Q

Differences between Crohns and UC on sigmoidoscopy

A

Crohns- aphthous ulcers, skip lesions, cobblestone appearances, transmural inflammation, normal rectum

UC - superficial inflammation restricted to mucosa/submucosa, crypt abscesses

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9
Q

Malignancy risk in IBD

A

Increased risk of colon cancer, have a colonoscopy 10 years post diagnosis, and further scopes as per risk category

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10
Q

Steroid sparing agents in IBD

A

Methotrexate
Azathioprine
Ciclosporin
Anti-TNF for refractory Crohns
Rectal steroids

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11
Q

Lhermitte’s Phenomenom

A

Electric shock sensation on flexion of the neck often going down the back

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12
Q

Hoffmans sign

A

Hold the PIPs of the middle finger, flick the tip of the middle finger - positive if the index or thumb extend signifying an UMN lesion in keeping with cervical myelopathy

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13
Q

Causes of myelopathy

A

Trauma
Neoplastic
MS
Vascular
Hereditary spastic neuropathy

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14
Q

Ix of myelopathy

A

FBC
ESR
Autoimmune screen
U&Es and LFTs
B12
Copper studies
Urgent MRI

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15
Q

Management of degenerative cervical myelopathy

A

Referral to neurosurgery
Analgesia
PT with neurorehab
OT

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16
Q

Findings in spastic paraparesis

A

Muscle weakness and spasticity
Urinary retention

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17
Q

Pyramidal signs

A

Hyperreflexia
Weakness especially in extensors
Spasticity
Babinski positive

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18
Q

Management of suspected meningitis

A

A-E approach
FBC - WCC
CRP
U&Es and LFTS
Coag
Broad spectrum abs
Dexamethasone
IVF
CT head - if signs of raised ICP, papilloedema, seizures or focal neurology
LP - CSF protein, glucose (and paired serum) viral PCR, cultures

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19
Q

Differences between LP results in viral vs bacterial

A

Turbid appearance in bacterial, can be clear in viral
High protein in bacterial, may be normal in viral
Low Glucose in bacterial, may be normal in viral
Positive gram stain in bacterial
Neutrophil predominant high WCC in bacterial, lymphocyte predominant in viral

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20
Q

Complications of bacterial meningitis

A

Death
Deafness
Blindness
Cognitive issues
Amputation as a result of sepsis

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21
Q

Treatment of migraine

A

Simple analgesia - paracetamol and NSAIDS
Triptan
Antiemetic

Prophylaxis can be given dependent on p

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22
Q

Investigations in acromegaly

A

ECG
FBC
U&Es and LFTs
Random IGF1
OGTT with serial GH measurements
MRI brainn with pituitary views

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23
Q

Complications of acromegaly

A

Bilateral carpal tunnel syndrome
Cardiomyopathy
IHD
HTN
T2DM
Visual impairment

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24
Q

Treatments in acromegaly

A

Trans-sphenoidal resection
Dopamine receptor agonists - bromocriptine and cabergoline
Somatostatin analogues - octreotide
Radiotherapy

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25
Q

Complications of pituitary resection in acromegaly

A

Pan hypopituitarism

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26
Q

Epworth score

A

Score >11 signifies presence of OSA

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27
Q

Treatment of OSA

A

If confirmed on polysomnography can trial CPAP overnight

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28
Q

Scar present in renal transplant

A

Rutherford-Morrison

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29
Q

Secondary issues in Osteogenesis Imperfecta

A

Bicuspid aortic valve
Aortic degneration
Poor hearing
Small stature
Barrel chested
Scoliosis
Translucent teeth
Bowing of long bones
Joint hypermobility
Skin hyper laxity
Retinal detachment and haemorrhages

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30
Q

Genetic basis ofbsteogenesis imperfecta

A

Usually AD
Can be de novo

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31
Q

Side effects of bisphosphonates

A

Oesophagitis/gastritis
Osteonecrosis of the jaw

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32
Q

Findings in Ehlers-Danlos

A

Fragile skin - ecchymoses and fish mouth scars
Hyperextensible skin
Joint hyper mobility

Mitral valve prolapse
Aortic dilatation - risk of dissection/rupture

Aneurysm rupture and dissection
Bowel perforation and bleeding

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33
Q

Causes of combined venous and arterial clots

A

APLS
MPN e.g PV, myelofibrosis and essential thrombocytosis

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34
Q

Complications of anti phospholipid syndrome

A

TIA/Stroke
DVT
PE
Livido reticularis
Thrombocytopenia
Thrombophlebitis
Foetal loss

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35
Q

Causes of CVA in younger patients

A

APLS
Extra cranial haemorrhage
Vasculitis
Cardiac Myxoma
Subacute infective endocarditis
AF
Sickle cell disease
Premature atheroma
Substance abuse - cocaine and methamphetamine
Mitochondrial problems eg MELAS

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36
Q

Hypertensive disorders of pregnancy

A

Chronic hypertension:
- Presents prior to pregnancy or at booking <20 weeks

Gestational hypertension:
- New hypertension >20 week without proteinuria
- Relabeled as chronic/primary hypertension if >12 weeks post partum

Pre eclampsia:
- New hypertension >20 weeks of pregnancy with one or both of proteinuria (PCR >30mg/mmol), OR renal/liver involvement, haematological complications, uteroplacental dysfunction

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37
Q

anti-hypertensive agents in pregnancy

A

Labetolol
Nifedipine - 1st choice in afro-Caribbean women
Methylodopa

Can also give aspirin 150mg ON to decrease risk of pre eclampsia

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38
Q

Target BP in pregnancy

A

Aim for BP <135/85

Initiate treatment in women with BP >140/90

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39
Q

Pre eclampsia features

A

Maternal AKI
Liver dysfunction - especially increase in ALT
Neurological features - headache/confusion/flashing lights
Haemolysis or thrombocytopenia
Fetal growth restriction

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40
Q

Ix in pre-eclampsia

A

Urine dip
PCR or ACR
Placental growth factors
Growth scans

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41
Q

Long term risks of hypertension in pregnancy

A

20% risk of hypertension
20% risk of pre-eclampsia
Long term CV risks

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42
Q

Causes of breathlessness in pregnancy

A

Asthma
LRTI
PE
Pneumothorax
Peripartum cardiomyopathy
DKA

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43
Q

Management of peripartum cardiomyopathy

A

Oxygen
Diurese with furosemide
Nitrates
ACEi - only in postpartum
Consider beta-blockers after liaising with obstetric cardiologists
Liaise with obstetric team for delivery

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44
Q

Risk factors for PE in pregnancy

A

No validated scoring systems

> 35 years
BMI >30
Multiple pregnancy
Para 3+
Hyperemesis
Post partum after prolonger labour, operative delivery or PPH >1L

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45
Q

Length of anticoagulation in pregnancy

A

Continue for full duration of pregnancy, for at least 3 months in total and for at least 6 weeks post partum

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46
Q

Contraindications for thrombolysis in stroke

A
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47
Q

Complications post stroke

A

VTE - IPC
Further stroke
Haemorrhagic transformation
Malignant middle cerebral artery syndrome
Seizures
Aspiration pneumonia
Spasticity
Falls
urinary and faecal incontinence

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48
Q

Secondary prevention in stroke management

A

Antiplatelets if no AF - clopidogrel long term
Anticoagulation if AF found
Statin
Weight loss
Stop smoking
Optimisation of blood pressure and diabetes
Endarcterectomy if >70% stenosis in carotid artery on ipsilateral side to stroke

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49
Q

What territory of infarct corresponds to homonymous hemianopia without macular sparing

A

MCA - in posterior stroke macula will be spared due to occipital pole receiving blood supply from the MCA

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50
Q

Differential for bilateral visual loss

A

Pituitary tumour
Glaucoma
Diabetic retinopathy and maculopathy
Bilateral cataracts
Idiopathic intracranial hypertension
Leber’s hereditary optic neuropathy
Retinitis pigmentosa
Vitamin A deficiency

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51
Q

What is retinitis pigmentosa

A

Progressive inherited retinal degeneration chcarcterised by by loss of photo receptors

No treatment, though vitamin A may slow progression

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52
Q

What conditions are associated with retinitis pigmentosa?

A

Refsum’s syndrome - muscle weakness and ataxia
Usher’s syndrome - sensorineural deafness
Kearn-Sayre’s syndrome
Abetalipoproeinaemia

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53
Q

Mx of reactive arthritis

A

Analgesia
Treating underlying cause

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54
Q

Causes of reactie arthritis

A

STI - chlamydia
Diarrhoeal illnesses

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55
Q

Genetic links with reactive arthritis

A

HLA B27

Associated with seronegative spondyloarthropathies - enteric arthritis, psoriatic arthritis, ankylosing spondylitis

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56
Q

Causes of hypothyroism

A

Hashimoto’s thyroiditis - lymphocytic destruction of thryroid follicles

De quervains thyroiditis

Iatrogenic hypothyroidism - post thyroidectomy or radio-iodine, amiodarone, lithium, carbimazole, propyl thiouracil

Post partum thyroiditis

Iodine deficiency

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57
Q

Symptoms in hypothyroidism

A

Tired and low energy levels
Constipation
Cold intoleranceMental slowing
Weight gain
Hair loss
Decreased libido
Oedema

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58
Q

Ix in hypothyroidism

A

TFTs - T3, T4 and TSH
TPO and anti-thyroglobulin - positive in Hashimoto’s
Lipid profile
FBC - microcytic anaemia
U&Es - Na

USS thyroid - goitre/FNA

CXR - retrosternal extension of goitre

Cortisol - to screen for Cushing’s

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59
Q

Associated conditions with hypothyroidism

A

T1DM
Vitiligo
Addison’s disease

Hypercholesterolaemia

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60
Q

Clinical findings in hypothyoidism

A

Bradycardi
Cool peripheries
Peaches and cream complexion - anaemia and carotenaemia
Peri-orbital oedema
Loss of eyebrows
Xanthelasma
Thinning hair
Goitre or thyroidectomy scar
Slow relaxing ankle jerk

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61
Q

Complications of hypothyroidism

A

Pericardial effusion
CCF
Carpel tunnel syndrome
Proximal myopathy
Ataxia

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62
Q

Conditions to be aware of when starting thyroid replacement in hypothyroidism

A

May unmask Addisons - precipitate crisis
May precipitate angina

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63
Q

Management of thyroid crisis

A

IV beta blockers - e.g propranolol unless contraindicated e.g. severe asthma (then use CCB - diltiazem)

Lugol’s iodine

Carbimazole/propylthiouracil

Hydrocortisone

Patients who fail medical therapy should be treated with plasma exchange or thyroidectomy

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64
Q

Causes of thyroid storm

A

Illness/infection
Recent iodine contrast
Trauma
Withdrawal or non-compliance of thyroid treatment
Myocardial infarction or stroke
DKA
Overdose of levothyroxine

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65
Q

Management of myxoedema coma

A

May require ITU
Ventilation if needed - rest failure from airway obstruction or macroglossia

Hydrocortisone
IV/PO thyroid
IV fluids

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66
Q

Differentials for myasthenia gravis

A

Guillain Barre
Miller Fischer syndrome
Lambert Eaton syndrome

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67
Q

Ix in myasthenia gravis

A

Anti-acetylcholinesterase (positive in 20%) and Anti-MuSK (positive in 15%)

TFTs - graves present in 5%

CXR
CT chest - ?thymoma (present in 10%)

MRI head if bulbar involvement present

CT/MRI orbits if thyroid opthalmoplegia suspected

Nerve conduction studies -decrement of compound muscle action amplitude with repetitive stimulation

Tension test - less commonly less

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68
Q

Mx in myasthenia gravis

A

Pyridostigmine
Steroids
Steroid sparing treatments - azathioprine (check TPMT levels prior) or MMF
IVIG or plasma exchange

Note have steroid dip - symptoms may worsen when starting

Thymectomy in some groups

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69
Q

Associations with myasthenia gravis

A

Other autoimmune disease - diabetes mellitus, RA, thyrotoxicosis, SLE, thymomas

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70
Q

Lambert Eaton Myasthenia Syndroma

A

Paraneoplastic - VGCC abs present, association with SCLC

EMG - increment on repetitive stimulation
Diminished reflexes that become brisker after exercise

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71
Q

Associated conditions with spina bifida

A

Neuropathic bladder
Constipation
Leg spasticity
Hydrocephalus - sometimes requiring VP shunts
Arnold-Chiari malformations
Recurrent meningitis
Syrinxes

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72
Q

Risk factors developing spina bifida

A

Deficiency of folate during 1st trimester
Family history of NTDs
Anti-epileptics - sodium valproate
Methotrexate
Poorly controlled diabetes

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73
Q
A
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74
Q

Why does the murmur of a VSD sound louder on expiration

A

Expiration increases venous return and therefore increases the amount of blood going through the VSD

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75
Q

Management of VSD

A

If evidence of left ventricular failure or reversal of shunt (right to left) - will need to consider closure of VSD either through patch or VSD closure device

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76
Q

Management of coarctation of aorta

A

Endovascular stenting - balloon angioplasty and stent placement
Surgical open repair - resection and end-end anastomosis

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77
Q

Signs in infective endocarditis

A

Oslers nodes
Janeway lesions
Splinter haemorrhages
Roth spots in eyes
Septic emboli distally
Poor dentition
Intravenous drug use
Haematuria on urine dipstick

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78
Q

Associations with coarctation of the aorta

A

PDA
VSD

Berry aneurysms
Turner syndrome

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79
Q

Presentation of coarctation of Aorta

A

Heart failure in Children

Hypertension
Heart failure in adults

Leg claudication
Headaches
Epistaxis

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80
Q

Clinical signs in coarctation of aorta

A

Radio-radial delay
Weak femoral pulses
Radio-femoral delay

Systolic ejection click (bicuspid vale)
Pansystolic murmur - VSD
Adjacent bruits - arterial collaterals e.g scapula, anterior axillary area,

Mid systolic murmur in infraclavicular area or posteriorly over left scapula

ECG - LV hypertrophy
CXR - posterior rib notching, figure 3 sign

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81
Q

Complications of coarctation of aorta

A

Stroke
Heart failure
Aortic rupture

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82
Q

Side effects of methotrexate

A

Anaemia
Bone marrow suppression
Pulmonary fibrosis
Liver dysfunction

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83
Q

Extra-articular side effects of Rheumatoid arthritis

A

Eyes/face:
Sicca symptoms - dry eyes, throat
Scleritis (pain and redness) or episcleritis (redness)
- scleromalacia (blue grey discolouration of eye)
Kerotconjunctivits sicca
Steroid induced cataracts
Fatigue

Lungs:
Pulmonary fibrosis
Fibrosing alveoli’s
Obliterative bronchiolitis
Lung nodules
Pleural effusion

Heart:
Pericarditis (constrictive)
Pericardial effusion

Renal:
Nephrotic syndrome - membranous glomerulonephritis
Renal amyloidosis

Neuro:
Carpal tunnel syndrome
Peripheral neuropathies
Felty’s Syndrome - splenomegaly and neutropenia

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84
Q

Causes of anaemia in rheumatoid arthritis

A

Anaemia of chronic disease
Secondary to renal disease
Iron deficiency anaemia - due to gastric ulceration with medications (NSAIDs)
B12 deficiency - pernicious anaemia
Bone marrow suppression with DMARDs
Autoimmune haemolytic anaemia
Felty’s syndrome (splenomegaly)

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85
Q

Radiological features in RA

A

Soft tissue swelling
Loss of joint space with subluxation
Periarticular osteopenia
Joint erosions

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86
Q

Genetic predisposition in RA

A

HLA DR4

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87
Q

Hand findings in RA

A

Volar subluxation and ulnar deviation at the MCPJs
Swan neck - Hyperextension of of PIPJ and flexion of the DIPJ
Boutonieres deformities - Flexion of the PIPJ and hyperextension of the PIPJ
Z shaped deformity of thumbs
Swelling and subluxation of the ulnar styloid
Carpal tunnel scar
Wasting of the dorsal interosseous muscles
Pain, swelling and restriction of joints in RA

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88
Q

How to measure disease activity in RA

A

DAS28
- Involves number of swollen and tender joints, ESR/CRP and objective assessment of the patients global health

DAS >5.1 - Active disease

DAS <2.8 - remission

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89
Q

Poor prognostic factors in RA

A

Seropositive disease - rheumatoid factor or anti-ccp
Articular erosions at onset
Severe disease activity at onset

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90
Q

Biologics used in RA

A

Anti-TNF alphas - adalimumab, etanercept, inflixamab

Anti-CD20 - rituximab

T cell co-receptor blocker - Abatercept

Anti IL-6 - Rocilizumab

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91
Q

DMARDs used in RA

A

Methotrexate
Leflunamide
Sulphasalazine
Hydroxychloroquire

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92
Q

Mode of inheritance in ataxia Telangiectasia

A

Autosomal dominant in ATM gene

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93
Q

Symptoms in ataxia telangiectasia

A

Ataxia

Dysarthria and dysphasia

Nystagmus

Dysdiadokinesia

Susceptibility to infections

Increased risk of cancer esp leukaemia and lymphoma

Telangiectasia

94
Q

Average onset of symptoms in ataxia telangiectasia

A

Before age of 5 years

95
Q

Mode of inheritance in Friedrich’s Ataxia

A

Autosomal recessive

96
Q

Gene implicated in Friedrich’s ataxia

A

Frataxin - anticipation

97
Q

Signs in Friedrich’s Ataxia

A

Wheelchair user
Pes cavus
Bilateral cerebellar signs
Combined UMN and LMN signs
- Leg wasting with absent reflexes and bilateral upping planters
Posterior column signs - loss of vibration and joint position senses

Kyphoscoliosis
optic atrophy - 30%
High arched palate
Sensorineural deafness
Murmur associated with HOCM
Diabetes testing - 10% develop diabetes

98
Q

Causes of clubbing

A

Familial

Cardiac disease:
- Infective endocarditis
- Congenital heart disease

Respiratory disease:
- Lung cancer
- ILD
- Bronchiectasis
- Cystic fibrosis
- Asbestosis
- EAA
- Lung abscess
- HHT with pulmonary AVMs

Coeliac disease

Cirrhosis of the liver

Ulcerative colitis

Hyperactive thyroid

99
Q

Genetics in HHT

A

Autosomal dominant

100
Q

Clinical symptoms in HHT

A

Vascular dysplasia causing telangiectasia and AVMs

Epistaxis

Telangiectasia and AVMs in the GI tract causing acute haemorrhage or chronic slow bleeding with resulting iron-deficiency anaemia.

Pulmonary AVMs occur may present as dyspnoea, cyanosis, bruits, high-output heart failure, clubbing and paradoxical cerebral emboli that may cause stroke and cerebral abscess. They can also lead to haemoptysis and haemothorax. Pulmonary hypertension can occur.

AVMs in the liver can cause high-output cardiac failure or cirrhosis.

Some patients have cerebral involvement resulting in headache, seizures or epilepsy, intracranial haemorrhage and stroke.

101
Q

Differentials in bihilar lymphadenopathy

A

Sarcoidosis
Thymoma
Lymphoma
TB
Pneumoconiosis e.g silicosis, berylliosis

102
Q

Treatment in sarcoidosis

A

NSAIDs for arthralgia

Referral to respiratory and ophthalmology

Steroids indicated if:
- Stage 2-4 on CXR e.g BHL + infiltrates, infiltrates or fibrosis
- Eye involvement
- Hypercalcaemia
- Neurological involvement
- Cardiac involvement

40mg prednisolone with GI and bone protection

Monitor with ESR and ACE

Steroid sparing agents = methotrexate, hydroxychloroquine, ciclosporin, cyclophosphamide, infliximab

Surgery- lung transplant, pacemaker

103
Q

Investigations in SLE

A

FBC, U&E, LFTs, Clotting
ESR, CRP
ANA, RF, anti-CCP, ANCA (+PR3/MPO), Immunoglobulins, complement (+/- cryoglobulins)
Antiphospholipid Abs (anticardiolipin, antiB2GPI), lupus anticoagulant screen

Anti-dsDNA, antiscl70, anticentromere Ab, AntiRo, AntiRNP, antism, Antijo1, AntiLa, Antism/RNP, antichromatin, antihistone

Miscellaneous: ACE (sarcoid), ferritin (stills),anti-GBM (goodpastures)

Nailfold capillaroscopy

Pregnancy test

Renal: BP, urine dipstick for blood and protein, urine PCR, renal USS, renal biopsy

Joints/Spine: Xrays/USS, aspiration, MRI

CVS: ECG, troponin, ECHO, BNP, cMRI

Resp: Sats, ABG, CXR, pulmonary function tests, HRCT, Right heart catheterisation, BAL, broncoscopy

104
Q

Antibodies found in SLE

A

ANA (95% sensitive, low specificity). Positive in 98% of SLE patients.

Antiribosomal P (specific, can be positive in ANA negative patients).

AntidsDNA (70% of SLE, 95% specific)

Antism (pathognomic for SLE) Positive in 10-30%

Antiphospholipid Abs positive in 30-50%

Anti Ro and La (neonatal lupus, heart block)

Anti-RNP (mixed connective tissue disease)

RF (positive in 40%)

Antihistone antibodies for drug induced lupus

105
Q

Diagnosis of SLE

A

ACR criteria - 4 out of 11 of SOAP BRAIN MD

Serositis - pericarditis/pleuritis
Oral mucosa
Arthritis
Photosensitivity

Blood
- lymphopenia
- Haemolytic anaemia
- Leucopenia
Renal
ANA
Immunological
Neurological - seizures and psychosis

Malar rash
Discoid rash

106
Q

Treatment in SLE

A

Conservative: Sunscreen, pregnancy advice

Medical: Manage CVS risk factors. ACEI for proteinuria.

Mild-moderate lupus (MSK and mucocutaneous features): low dose oral prednisolone and bone and GI protection, hydroxychloroquine, azathioprine, methotrexate. NSAIDs for joint pain (watch U+E)

Severe (major organ involvement): iv methylprednisolone, cyclophosphamide, mycophenolate mofetil, rituximab/belimumab

Surgical: renal transplant

107
Q

Causes of erythema nodosum

A

Streptococcus
OCP
Rickettsia
Eponymous - Behcets

Sarcoidosis
Hansen’s disease - leprosy
IBD - usually UC
Infections - TB (cutaneous)
Idiopathic
NHL
Sulfonamides

Pregnancy

108
Q

Diagnosis of HHT

A

Diagnosis is made using the Curacao Criteria. If 3 criteria are present the diagnosis is definite. If 2 criteria are present, the diagnosis is suspected or possible. If only 1 criterion is present the diagnosis is unlikely.

1 Epistaxis that is spontaneous and recurrent

2 Multiple mucocutaneous telangiectasia at characteristic sites such as the lips, nose, tongue and mucous membranes, fingers, conjunctiva

3 Visceral lesions- GI telangiectasia, Pulmonary, Hepatic, Cerebral AVMs

4 Family History in a first degree relative. Genetic testing can be performed to confirm the diagnosis if necessary.

109
Q

Causes of epistaxis

A

Trauma
Low platelets
Drugs - antiplatelets etc
Malignancy
Cocaine
Wegener’s granulomatosis (now called Granulomatosis with Polyangiitis)
HHT

110
Q

Management of HHT

A

Managing epistaxis
- Nasal humidification
- Ointments
- Saline spray
- Tamoxifen
- TXA
- Laser therapy or cauterisation

Blood transfusion if needed

Embolisation or ablation of GI AVMs

Treatment of iron deficiency

111
Q

Clinical Presentation in MEN1

A

Autosomal dominant MEN gene defects

Pituitary adenoma
Parathyroid hyperplasia
Pancreatic tumours and gastronomes

112
Q

Clinical Presentation in MEN2A

A

Autosomal dominant RET gene defects

Parathyroid hyperplasia
Medullary thyroid carcinoma
Phaechromocytoma

113
Q

Clinical Presentation in MEN2B

A

Autosomal dominant RET gene defects

Marfanoid body habitus
Mucosal neuromas
Medullary thyroid carcinoma
Phaechromocytoma

114
Q

What is Lofgren’s Disorder

A

Bihilar lymphadenopathy, erythema nodosum, fever, weight loss, arthralgia, uveitis

115
Q

Investigation in sarcoidosis

A

Obs and urine dip

Bloods- ESR, CRP, FBC, LFTs, ACE, Calcium, Immunoglobulins, U+E, vitamin D, TFTs

24 hour urine for calcium if raised serum calcium

Respiratory tests: sats and ambulatory oximetry, CXR, spirometry, BAL, bronchoscopy and biopsy (send biopsy for mycobacterial and fungal testing and histology), HRCT

Cardio tests: ECG, echo, 24 hour Holter, cardiac MRI

Abdo tests: urine dipstick, USS, CT

Eye tests: slit lamp examination, visual acuity, fundoscopic examination

Neuro tests: CT/MRI, LP

LN biopsy/skin biopsy/peripheral nerve biopsy

116
Q

Ocular manifestations of sarcoidosis

A

Uveitis
Granulomas which may causes visual impairment or retinal detachment
Conjunctivitis
Keratoconjunctivitis sick
Neurosarcoidosis - papilloedema, nystagmus, visual field defects

117
Q

Differential diagnoses in SLE

A

Drug-induced lupus
RA
Stills disease
Undifferentiated or mixed connective tissue disease
Primary sjogrens
Antiphospholipid syndrome
Fibromyalgia
Lymphoma
Infection
Pulmonary-renal syndrome (goodpastures, ANCA-associated vasculitis)
Systemic sclerosis
Dermatomyositis

118
Q

Blood tests showing lupus flare

A

Low lymphocytes
Low complement
Normal/low CRP
Raised ESR
Anaemia, thrombocytopenia
Raised antidsDNA

119
Q

Diagnosis of APLS

A

Suspect if:

Arterial thrombosis <50 years old
Unprovoked venous thrombosis < 50 years old
Recurrent thrombosis
Both arterial and venous events
Unusual sites eg. renal, liver, cerebral sinuses, mesenteric, vena cava, retinal
Obstetrical: fetal loss (miscarriage after 10 weeks/3 unexplained miscarriages <10 weeks), recurrent miscarriages, early/severe preeclampsia, unexplained intrauterine growth restriction

Lab criteria on 2 occasions 12 weeks apart: anticardiolipin Abs, Anti-B2GPI, lupus anticoagulant, raised APTT.

120
Q

Management of APLS

A

Avoid oestrogen contraceptives, prophylactic aspirin
Heparin for thrombosis then warfarin

121
Q

Causes of morbidity and mortality in SLE

A

Infections,
Atherosclerosis
Osteoporosis
Malignancy especially lymphoma, lung cancer, cervical.

122
Q

Causes of drug induced SLE

A

Hydralazine
procainamide
Isoniazid
Phenytoin
Interferon

Associated with anti-histone Abs

123
Q

Symptoms in thyrotoxicosis

A

Weight loss
Anxiety
Palpitations
Tachycardia
Diarrhoea
Heat intolerance
Tremor
Sweating
Irritable mood
Breathlessness
Weakness going up stairs
Eye symptoms - grittiness, loss of vision, loss of colour, eye pain, blurred or double vision

124
Q

Clinical course of Grave’s disease

A

50% chance of cure / 50% chance of relapse - can have further treatment with carbimazole or trial of radioactive iodine

125
Q

Issues with radioactive iodine with thyroid eye disease

A

May exacerbate active thyroid eye disease

126
Q

Treatment in Graves disease

A

Aim to treat medically - usually takes 1.5-2 years

If relapses can trial medical treatment again or radioactive iodine or surgery

Medical Management: beta-blocker for symptomatic relief (e.g. propranolol), carbimazole or propylthiouracil (during 1st trimester).

Indications for surgery: relapse after stopping antithyroid drugs, poor compliance with meds, intolerance of meds, cosmesis, compression, symptomatic and planning pregnancy, uncontrolled on meds, suspicious nodules

Complications of surgery: hypoparathyroidism, damage to recurrent laryngeal nerve, hypothyroidism, recurrence of hyperthyroidism

127
Q

Antibody found in Grave’s Disease

A

Abs to TSHr

128
Q

Worrying symptoms in thyroid eye disease

A

Optic neuropathy - visual acuity and colour vision

Exposure keratopathy

Double vision

129
Q

Treatment in thyroid eye disease

A

If mild - selenium, and topical lubricants

If by NOSPECS criteria >3 (ie more than or equal to proptosis consider IV methylpred weekly for 6 weeks

Cyclosporin may be given as a steroid sparing agent

Can try orbital decompression or radiotherapy if still no benefit

130
Q

Differential diagnosis for proptosis

A

Thyroid eye disease
Orbital tumour/met/granuloma
Caroticocavernous fistula
Orbital cellulitis
AVM
Cavernous sinus thrombosis

131
Q

Causes of thyrotoxicosis

A

Graves disease
Toxic multi nodular goitre
Solitary toxic nodule

Excess levothyroxine replacement

Ectopic:
- Pituitary adenoma
- Hypothalamic mass
- Ovarian teratoma, choriocarcinoma

Thyroiditis:
- Subacute (de Quervains)
- Post radiation
- Post partum
- Drug induced e.g amiodarone

132
Q

Extra renal manifestations of PCKD

A

Cysts in other areas particularly the liver leading to polycystic liver disease

Berry aneurysms

Mitral valve prolapse

133
Q

What is Charcot’s arthropathy

A

Painless deformity and destruction a joint with new bone formation following repeated minor trauma secondary to loss of sensation

134
Q

Causes of Charcot’s arthropathy

A

Tabes dorsalis - hip and knee
Diabetes - foot and ankle
Syringomelia - elbow and shoulder

135
Q

Treatment in Charcot’s arthropathy

A

Immobilisation
Referral to orthopaedics for surgical intervention
Bisphosphonates can help

136
Q

Causes of secondary hypertension

A

Renal:
- CKD secondary to glomerulonephritis
- ADPCKD
- Renovascular disease e.g renal artery dysplasia

Endocrine:
- Cushings
- Conns
- Bilateral adrenal hypertrophy
- Liddle’s (low potassium and metabolic alkolosis, acts on ENaC transporter, treated with amiloride)
- Acromegaly
- Phaechromocytoma
- Carcinoid

Aortic coarctation

Pre-eclampsia

137
Q

Symptoms to ask in hypertension history

A

Symptoms for hypertensive emergency:
- Headache
- Visual disturbance
- Chest pain

Previous blood pressure reading

PMHx: renal disease, CVD or PVD, smoking, diabetes, thyroid disease, cardiac disease in childhood

Associated symptoms:
- Abdominal/flank pain
- Polyuria
- Muscle cramps (Conn’s)
- Frothy urine?
- Change in hand size
- Flushing or dumping sx
- Anxiety or palpitations or sweating

Illicit drug use

?Pregnancy

138
Q

Blood pressure targets

A

HTN if in clinic BP >140/90 on two measurements - use ABPM to confirm diagnosis (135/85 on ABPM)

Graded by British hypertension society guidelines:
Stage 1. Clinic BP >140/90 or ABPM >135/85
Stage 2. Clinic BP >160/100 or ABPM >150/95
Stage 3. Clinic SBP >180 or DBP >120

Treat if stage 1 hypertension and evidence of end organ damage, IHD, diabetes, CKD or 10 year CVS risk >10%

Treat all stage 2 hypertension

Arrange same day admission if severe hypertension and grade 3 or 4 retinopathy (or other concerns e.g. new renal impairment)

139
Q

Findings in hypertensive retinopathy

A

Grade 1. Silver wiring
Grade 2. Plus arteriovenous nipping
Grade 3. Plus cotton wool spots and flame haemorrhages
Grade 4 Plus papilloedema

140
Q

Ix in hypertension

A

x2 BP readings, and alternate BP readings on arms

Urine dip with PCR
ECG - LVH

Bloods:
- FBC
- U&Es - renal impairment low bicarb (conns)
- LFTs
- Aldosterone and renin ratio

CXR
ECHO

USS kidney

Special tests:
- Plasma meatnephrines
- Dexamethasone suppression tests
- IGF1
- MRA renal arteries
- PET - adrenal tumours
- MIBG - for phaechromocytomas

141
Q

Treatment in Hypertension

A

Lifestyle advice firstling - increase exercise, salt restriction, lose weight, reduce alcohol, stop smoking

ACEi or ARB if <55yrs first line
CCB if afro-Caribbean or >55yrs

A+B second line
A+B+D (thiazide-like diuretic 3rd line e.g. indapamide)

Then consider spironolactone, beta blocker or alpha blocker

Aim <140/90, except
- >80yrs - aim <150/90
- If ACR >70 - aim <130/80

Consider cardiovascular risk and consider statin and aspirin

142
Q

Conditions seen in malignant hypertension

A

Aortic dissection
PRES - posterior reversible encephalopathy syndrome
Aortic aneurysm
MI
Stroke
Left ventricular failure

If above conditions present invasive blood pressure monitoring and IV anti-hypertensive agents e.g GTN, labetalol and alpha blocker if considering phaechromocytoma

143
Q

Conditions in MEN syndromes

A

MEN1 (MEN oncogene):
- Pituitary
- Parathyroid hyperplasia
- Pancreas

MEN2A (RET oncogene):
- Parathyroid hyperplasia
- Phaechromocytoma
- Medullary carcinoma

MEN2B (RET oncogene)
- Phaechromocytoma
- Medullary hyperplasia
- Mucosal neuromas
- Marfanoid habitus

144
Q

Grading of diabetic retinopathy

A
  1. Background retinopathy +/- maculopathy
    - Hard exudates
    - Flame and dot haemorrhages
    - Microaneurysms
    - Annual retinal screening
  2. Pre-proliferative retinopathy +/- maculopathy
    - Multiple cotton wool spots
    Multiple dot haemorrhages
    - Venous beading
    - Intraretinal microvascular abnormalities
    - Routine referral to ophthalmology
  3. Proliferative retinopathy +/- maculopathy
    - Neovascularisation of the disease
    - New vessels elsewhere
    - pan retinal photocoagulation from previous treatment
    - Urgent referral to ophthalmology

Diabetic maculopathy - macular oedema or hard exudates with one disc space of the fovea - routine referral to ophthalmology unless reduced vision (then urgent)

145
Q

Treatment of diabetic retinopathy

A

Tight glycemic control

Treat other factors - hypertension, hypercholesterolaemia, smoking cessation

VEGF inhibitors

Photocoagulation

146
Q

Complications of proliferative diabetic retinopathy

A

Vitreous haemorrhage
Traction retinal detachment
Neovascular glaucoma due to rubeosis irisis

147
Q

Symptoms in ankylosing spondylitis

A

Back and joint pain/stiffnes - >1hr in the morning
Enthesitis - tenderness at the tendon insertion points e.g. Achilles heel, planter fasciitis
Fever
Fatigue

148
Q

Special test in Ankylosing spondylitis

A

Schober’s test - mark a spot and then measure 5cm inferiorly and 10cm superiorly on the dorsal spine - if expands <5cm on forward flexion positive test

149
Q

Associated conditions with ankylosing spondylitis

A

Anterior uveitis
Aortic regurgitation
Apical fibrosis
AV block
Arthropathy e.g psoriatic and psoriasis

150
Q

Risk scores used in ACS syndrome

A

GRACE score - 6 month mortality

TIMI score - 14 day risk of mortality, new or recurrent MI, or severe recurrent ischaemia necessitating cardiac revasculairsation

151
Q

Causes of pericarditis

A

Infection:
- Viral
- HIV

Autoimmune:
- SLE
- Rheumatoid

Post MI - Dresslers

Uraemia

Cancer

152
Q

Clinical signs of cardiac tamponade

A

Beck’s Triad

Hypotension
Raised JVP
Muffled heart sounds

Tachycardia will also be an early sign

153
Q

Pathophysiology of Eisenmenger’s syndrome

A

Large often congenital heart defects e.g. VSD, PDA, ToF, AVSD

Permenant vascular changes, PAH and elevated pulmonary vascular resistance

Reversal of the shunt direction to RTL causing cyanosis

154
Q

Treatment of Eisenmenger’s

A

Poor prognosis - 50% mortality at one year

Medical:
- Pulmonary vasodilator therapy e.g. endothelia receptor antagonist, PDE5 inhibitor, epoprostenol
- Anticoagulation
- Symptomatic - iron supplementation, diuretic, anti-arrhythmic, oxygen (if hypoxaemia responsive)
- Supportive - contraception (pregnancy contraindicated), avoidance of extreme heat or dehydration

Surgical:
- Correction of shunt generally contraindicated
- Heart lung transplant

155
Q

Common types of headache

A

Migraine:
- Associated with aura, photo- and photophobia, last <72 hours, unliateral
- Triptans, aspirin and NSAIDs in acute
- Topiramate, propranolol, amitriptyline in chronic

Tension:
- Bilateral, throbbing

Cluster headache:
- Very intense, lasting 15 mins, supraorbital, associated with lacrimation and rhinorrhea
- Verapamil for prophylaxis, oxygen and triptans in acute

Medication overuse
- Episodic headache in patients using analgesia >10 days/month for >3 months

Trigeminal neuralgia:
- Stabbing pain in trigeminal nerve distribution

156
Q

Screening tool for Osteoporosis

A

FRAX - calculates the ten year probability of fracture

157
Q

Ix in osteoporosis

A

Bloods:
- FBC
- U&Es, LFTs
- TFTs
- CRP and ESR
- Calcium and phosphate (Pagets)
- Vitamin D
- Immunoglobulins and protein electrophoresis

X rays of affected area

DEXA scan - if for primary prevention refer if >10% risk of fracture in ten years in scoring tools

158
Q

Treatment in osteoporosis

A

IF T score <-2.5:
- Oral bisphophonates
- If not tolerated can suggest zolendronic acid, raloxifene and denosumab

159
Q

Treatment severity score in psoriasis

A

Psoriasis area and severity Index (PASI)

160
Q

Management in psoriasis

A

Topical treatment first line:
- topical corticosteroids OD - can be increased to BD if satisfactory control not gained
- Vitamin D analogue OD - may be increased to BD if not satisfactory control
- Coal tar preparation, shampoos can be used in scalp psoriasis
- Dithranol

Phototherapy
- UVB
- UVA with psoralen (PO or topical)for palmoplantar pustulosis or plaque psoriasis - increased risk of cancer (especially SCC)
- Avoid PUVA in those at risk of SCCs in future ie younger patients, light skin types (Fitzpatrick 1 and 2), likely to require long term cyclosporin, or previous skin cancer

Systemic treatments
- Offered if psoriasis cannot be controlled with topical therapy, it has significant impact on psychological or social well-being and one of:
- - Psoriasis is extensive >10% of BSA or PASI score >10
- - Associated with significant functional impairment or high levels of distress
- - Phototherapy cannot be used or associated with rapid relapse

  • Can use methotrexate, ciclosporin or acreitin (vitamin A analogue), apremilast (PDE4 inhibitor)
    Can then use biologics - TNFa, IL-17 and IL-23
161
Q

Risk factors for fragility fractures

A

Older age
Female sex
Low BMI
Smoking
Alcohol use
Early menopause
History of falls
Previous fragility fracture
Use of corticosteroids
Secondary causes of osteoporosis

162
Q

Secondary causes of osteoporosis

A

Malabsoprtion:
- Coeliac disease
- Chronic pancreatitis (malabsorption)

Endocrine:
- Hyperthyroidism
- Hyperparathyroidism
- Diabetes
- Cushings
- Hypogonadism

Use of steroids:
- COPD
- Rheumatoid arthitis

Loss of nutrients:
- CKD
- Chronic liver failure

Medications:
- Steroids
- GNRH inhibitors

163
Q

Fragility fracture treatment

A

MDT involvement

Orthopaedic team for surgical management if required
Analgesia
PT
OT
Dietician
Patient education - smoking, weight, alcohol

May require DEXA scan - NCE guidelines state if >75 and fragility fracture to treat accordingly

Calcium and vitamin D supplements
Bisphosphonates
Denosumab
Raloxifene
Teriparatide
HRT in younger women with premature menopause

164
Q

Life threatening signs of asthma

A

A - Arrhythmia/altered conciseness level
C - cyanosis or normal PaCO2 4.6 - 6kPA
H - Hypoxia <8kPa, hypotension
E - exhaustion
S - silent chest
T - Threatening PEFR - <33%
92 - Sats <92%

165
Q

Criteria for safe discharge post asthma exacerbation

A

PEFR >75% of predicted
Check inhaler technique and able to record PEFR
Written asthma management plan
Follow up arranged with Patients GP/asthma nurse and with hospital

166
Q

Medical management of asthma

A
  1. SABA PRN - salbutamol
  2. SABA plus ICS
  3. SABA plus ICS plus LRTA (montelukast)
  4. SABA plus moderate dose ICS plus LABA plus LRTA (if helpful)
  5. Change ICS to high dose, can add theophylline
  6. Consider biologics e.g omalizumab
  7. Addition of steroids

If symptoms not controlled on moderate dose - check FeNO level and eosinophils - if raised refer to secondary care team

If symptoms not controlled on high dose ICS refer to secondary care team

168
Q

Treatment in CTEPH (Group 4 PH)

A

Riociguat - stimulator of soluble guanlyte cyclase

Continued oral anticoagulation

Interventions:
- Balloon pulmonary angioplasty
- Pulmonary endarterectomy

169
Q

Differentials in persistent fever

A

Infection:
- Infective endocarditis
- Malaria
- Abscess
- EBV, CMV, HIV

Inflammatory disease:
- Vasculitis
- SLE
- RA

Malignancy

Drug-induced
- Malignancy hyperpyrexia syndrome

Genetic:
- Familial Mediterranen fever

Factitious

170
Q

Ix in persistent fever

A

Urine Dip

ECG - PR interval

Bloods:
- FBC
- U&Es and LFTs
- CRP and ESR
- ANCA and autoimmune screen
- Rh factor
- HIV, EBV and CMV
- Immunoglobulins and SPEP
- Malaria thick and thin films
- EBV monospot
- CK - malignant hyperthermia

Radiology:
- CXR
- CTTAP
- PET CT
- White cell scintigraphy

171
Q

Diagnosis of coeliac disease

A

Anti-TTG and total IgA
Duodenal biopsy

172
Q

Ix in anaemia

A

Bloods:
- FBC
- Iron studies and ferritin
- B12 and folate
- Hb electrophoresis - thalassaemia
- Anti-TTG and total IgA

Faecal occult blood

Endoscopy

CT abdomen

173
Q

Complications of sickle cell disease

A

Acute:
- Vaso-occlusive crisis
- Acute chest syndrome
- Stroke
- PE
- Infection
- Gallstones
- Anaemia
- Aplastic crisis (parvovirus 19)
- Osteomyelitis
- AKI

Chronic complications:
- Pain
- Anaemia
- Pulmonary hypertension
- Chronic sickle lung
- Sickle retinopathy
- Leg ulcers
- Priapism
- CKD

174
Q

Treatment of sickle cell disease

A

Treatment in acute crises:
- Oxygen +/- CPAP
- IV fluids
- Analgesia
- Antibiotics if evidence of infection
- Blood transfusion/exchange transfusion

Following acute crisis:
- Drink plenty of fluids
- Warm clothes - avoid sudden temp changes
- NSAIDs and paracetamol
- Hydroxycarbamide or exchange transfusions if frequent crises
- Long term folic acid
- Penicillin prophylaxis and vaccinations in patients with functional hyposplenism
- Patient education

175
Q

Causes of arterial and venous thrombosis

A

APLS
Myeloproliferative disorders - ET, polycythaemia, myelofibrosis
Paraxysmal nocturnal haemoglobinuria

PFO/VSDs - venous clot into arterial system

176
Q

Causes of polycythaemia

A

Primary polycythaemia:
- Polycythaemia rubra vera

Secondary polycythaemia:
- Respiratory disease e.g. COPD, OSA
- Cyanotic heart disease e.g. congenital heart disease
- Testosterone replacement/injection
- EPO secreting tumours - renal cell carcinoma, medullary haemangiomas, parathyroid tumours
- PCKD - secretion of EPO

Apparent polycythaemia:
- Dehydration
- Diuretics
- Smokers or alcoholics

177
Q

Ix in polycythaemia

A

FBC - raised HCT
U&Es and LFTs
JAK2
EPO

Epworth score
Polysomnography
ABG

CXR
USS abdomen - spleen
CT abdo/pelvis

Red cell mass scintigraphy

Bone marrow biopsy

178
Q

Complications of polycythaemia rubra vera

A

Thrombosis
AML

Treated with aspirin
Lifelong surveillance for AML

179
Q

Investigations in nephrotic syndrome

A

Urine dip
UPCR

Bloods:
- FBC
- U&Es and LFTs
- Coagulation
- Lipids
- BBV
- ANCA
- Rh factor and ANA
- Complement
- Hba1c
- Anti-phospholipase A2 receptor abs (PLA2R)
- Immunoglobulin and SPEP

CXR
renal tract US
CTPA or doppler if concur for clots

Renal biopsy

180
Q

Causes of glomerulonephritis

A

Primary:
- Membranous nephropathy
- FSGS
- Minimal change
- IgA Nephropathy

Secondary causes:
- Amyloidosis
- SLE
- RA
- HIV
- Hep B and C
- Drugs: penicillamine, NSAIDs, heroin and gold
- Diabetes
- Malignancy - solid organ tumours, leukaemia, lymphoma, myeloma

181
Q

Complications of PSC

A

Progression of PSC to cirrhosis
Development of new biliary stricture
Cholelithiasis - 1/3 of PSC patients develop gallstones
Cholangiocarcinoma
HCC
Gallbladder cancer
Bowel cancer with liver mets

182
Q

Treatments in PSC

A

Mostly supportive

UCDA
Antihistamines
Opiates

Bile duct angioplasty/stent

Liver transplant

183
Q

Treatment in Crohns disease

A

Mild:
- Oral mesalazine
- Oral steroids

Moderate:
- Iv steroids
- IV infliximab

Maintenance:
- Azathioprine
- Methotraxate
- TNFa agents

184
Q

Treatment in UC

A

Mild:
- PR mesalazine
- Oral/topical steroids e.g. budesonide

Moderate:
- IV steroids
- IV ciclosporin

Maintenance:
- Azathioprine
- 5-ASA

185
Q

Complications of IBD

A

Crohn’s:
- Anaemia
- Fistula
- Abscess formation
- Malnutrition
- Intestinal obstruction

UC:
- Anaemia
- Colon cancer
- Toxic dilatation
- Perforation

186
Q

Extra-intestinal manifestations of IBD

A

Eyes:
- Anterior uveitis
- Episcleritis
- Iritis

Mouth:
- Aphthous ulcers

Joints:
- Large joint arthritis
- Seronegative arthritis

GI:
- PSC
- Systemic amyloidosis

Skin:
- Erythema nodosum
- Pyoderma gangrenous
- Clubbing

187
Q

Associated disease with venous leg ulcers

A

Varicose veins
Chronic venous insufficiency
CCF
DVT

188
Q

Investigations in venous ulceration

A

Doppler ultrasound

ABPI:
- 0.8 - 1.2 is normal
- >1.3 - calcified
- <08 implies arterial insufficiency

Arteriography

189
Q

Forms of joint disease in psoriatic arthritis

A

DIPJ involvement
Large joint mono/oligo arthritis
Seronegative
Sacroilitis
Arthritis mutilans

190
Q

Exacerbating factors in psoriasis

A

Trauma
Smoking
Stress
Alcohol
Beta blockers

191
Q

Symptoms in Henoch-Scholein Purpura

A

Purpuric rash - Usually on extensor surfaces

Abdominal pain

Arthralgia

192
Q

Precipitants of HSP

A

Infections:
- Streptococci, HSV, parvovirus B19

Drugs:
- Antibiotics

193
Q

Complications of HSP

A

Renal involvements - IgA nephropathy - haematuria with proteinuria

Hypertension

194
Q

What is HSP?

A

Small vessel vasculitis - IgA and C3 deposition

Normal or raised platelet count

Most spontaneous recover although steroids can help recovery and treat arthralgia

195
Q

Concerning features in skin lesions

A

Asymmetrical
Border irregularity
Colour - black - often irregular pigmentation
Diameter >6mm
Enlarging

196
Q

Treatment in malignant melanoma

A

Excision

Staged on Below thickness +/- sampling of draining lymph nodes

Surveillance for low risk node negative disease

High risk or node positive disease - immunotherapy

197
Q

Treatment in SLE

A

Mild disease (cutaneous or joint involvement only):
- Topical steroids
- HCQ

Moderate disease (plus organ involvement):
- Prednisolone
- Azathioprine

Severe disease (+ severe inflammatory involvement of vital organs):
- Methylpred
- MMF
- Cyclophosphamide
- Azathioprine

With APS consider aspirin

198
Q

Examination findings in systemic sclerosis

A

Hands:
- Raynauds
- sclerodactyly - prayer sign
- calcinosis - may ulcerate
- assess function

Face
- tight skin
- beaked nose
- microstomia
- telangiectasia
- alopecia
- peri oral furrowing

Skin:
- Morphoea - patches of sclerotic wkin
- en coup de sabre

Interstitial fibrosis

Cardiac
- pulmonary hypertension - RV heave, loud P2 and TR
- heart failure
- pericardial rub

Urine dip - proteinuria

199
Q

Symptoms in limited systemic sclerosis

A

Distribution limited below elbows, below knees and face

Slow progression

CREST syndrome:
- Calcinosis
- Raynauds
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasia

201
Q

Symptoms in diffuse systemic sclerosis

A

Widespread cutaneous and early visceral involvement

Rapid progression

202
Q

Antibodies in systemic sclerosis

A

ANA positive in 95%

Limited disease - anti Centromere

Diffuse disease - anti Scl 70 (anti-topoisomerase I)

Anti-RNA polymerase III - increased cancer risk in diffuse disease, increased risk of dermatological complications, and increased risk of renal crisis

203
Q

Investigations in systemic sclerosis

A

Obs - HTN

Urine dip - proteinuria
ECG - conduction blocks, r axis deviation

Bloods:
- FBC
- U&ES
- LFTS
- Antibodies

Joint X rays
CXR

HRCT
PFTs

ECHO
Cardiac MRI
Right heart catheterisation

OGD
Esophageal manometry
Hydrogen breath tests for basterial overgrowth

204
Q

Treatment in systemic sclerosis

A

MDT - OT, PT, SLT, dietician, palliative care, resp, GI, and cardio team

Derm:
- camouflage creams
- CCBs if raynauds
- PO PDE-5 inhibitors e.g. sildenafil
- gloves, hand warmers
- prostacyclin infusion if ulceration

Renal:
- ACEi

Gastro:
- PPI for gastric reflux
- If signs of bacterial overgrowth syndrome - rifaxamin

Immunomodulators:
- MMF
- Biologics - Rituximab (anti CD 20) and tociluzamab (IL-6) - trial evidence
- Evidence that anti-fibrotic agents such as nintedanib are beneficial
- IV cyclophosphamide

Avoid steroids to prevent precipitation of renal crisis
- If renal crisis use ACEi

205
Q

Cardiac complications of Marfans

A

Mitral valve prolapse
Bicuspid aortic valve
Coarctation of the aorta

206
Q

Management in Marfans

A

Surveillance:
- Monitoring of aortic root size with annual trans thoracic ECHO

Treatment:
- ACEi and Beta blockers to slow aortic root dilatation
- Pre-emptive aortic root surgery if diameter >5cm or increasing rapidly (>1cm/year)

Screen family members and genetic counselling of affected first degree relatives

207
Q

Associated conditions with Paget’s disease

A

Entrapment neuropathy:
- Carpel tunnel
- visual problems - optic atrophy
- Cauda equina
- Deafness

Fragility fractures
Osteoarthritis
Arthralgia

Kidney stones

CCF - high output state

Osteogenic sarcoma

208
Q

Causes of angioid streaks

A

Paget’s disease
Ehlers-Danlos
Pseudoxanthoma elasticum

209
Q

Ix in Paget’s disease

A

Elevated ALP, normal calcium and phosphate

Radiology:
- Moth eaten bones
- Increased uptake on bone scans

210
Q

Treatment of Pagets

A

Exercise, PT, OT

Symptomatic:
- Analgesia
- Hearing aid
- Carpel tunnel release
- Joint replacement

Medical:
- Vitamin D and bisphosphonates

211
Q

Risk factors for gout

A

Alcohol
Red meat
Obesity
Drugs - diuretics or CNIs
CKD
Lymphoproliferative disorders

212
Q

Treatment in gout

A

Acute:
- Increase fluid intake
- High dose NSAIDS
- Colchicine
- Prednisolone

Chronic:
- Titrate allopurinol upwards aiming urate <300
- Febuxostat (xanthine oxidase inhibitor)

213
Q

Findings of OA on XR

A

LOSS

Loss of joint space
Osteophytes
Subchonral cysts
Sclerosis

214
Q

Causes of hypercalcaemia

A

Primary Hyperparathyroidism

Malignancy:
- Lytic lesions
- PTHrp release - usually lung SCC
- Vitamin D release - lymphomas

Sarcoid
Addisons
Hyperthryoidism

Medications:
- Vitamin D
- Thiazide diuretics
- Lithium

Prolonged immobility

215
Q

Causes of amenorrhoea

A

Primary causes:
- Genetic - e.g. Turners

Secondary:
- Pregnancy
- Postpartum and breastfeeding
- Pills e.g, OCP, risperidone, antidepressant e.g SSRIs, danazol (used in endometriosis), chemotherapy
- Premature menopause
- Polycystic ovarian syndrome
- Pituitary tumour - e.g. prolactinomas

216
Q

Cardiovascular issues in Turner’s syndrome

A

ASD
Coarctation of aorta
Bicuspid aortic valve
Hypertension

217
Q

Complications of Acromegaly

A

Acanthosis nigricans
BP high
Carpel tunnel - bilaterally
Diabetes mellitus
Enlarged organs
Field defect - bitemporal hemianopia
Goitre, GI malignancy
Heart failure, hirsutism, hypopituiatrism
IGF-1 high
Joint arthropthy
Kyphosis
Lactation - galactorrhoea
Myopathy

218
Q

Ix in acromegaly

A

Diagnosis:
- IGF-1
- OGTT with serial GH measurement
- MRi pituiatry fossa

Also assess pituitary function:
- TFTs, LH and FSH, prolactin, ACTH, testosterone

Complications:
- CXR - cardiomegaly
- ECG - ischaemia
- Glucose and Hba1c
- Visual field testing
- Polysomnography - OSA due to macroglossia

219
Q

Management in Acromegaly

A

Surgery: Trans-sphenoidal resection

Medical:
- Somatostatin analogues - ocreotide
- Dopamine agonists - cabergoline
- Pegvisomant - GH receptor antagonists

Radiotherapy

Follow ups:
- Blood monitoring - GH and prolactin
- MRI head
- ECG
- Visual fields

220
Q

Causes of Cushing’s disease

A

ACTH dependent:
- Cushing’s disease - pituitary adenoma
- Ectopic secretion of ACTH e.g. SCLC

ACTH independent:
- Adrenal adenoma or carcinoma
- Exogenous steroids

221
Q

Ix of Cushigns syndrome:

A

Confirm high cortisol:
- 24 hour urine collection
- Low dose dexamethasone suppression test

Suppressed cortisol - pseudo-bushings - alcohol/depression/obesity

Identify causes:
- ACTH level - if high ACTH dependent
- - High dose dexamethasone test - suppressed cortisol if pituitary cause, non-suppressed if ectopic or ACTH independent

  • MRI pituitary
  • CT abdo
  • Bilateral inferior petrosal sinus vein sampling - to confirm pituitary
222
Q

Treatment in Cushing’s

A

Surgical:
- Pituitary surgery - tran-sphenoidal
- Adrenalectomy

Pituitary irradiation

Medical
- Metyrapone

223
Q

Causes of proximal myopathy

A

Inherited:
- Myotonic dystrophy
- Muscular dystrophy

Endocrine:
- Cushings
- Hyperparathyroidism
- Hyperthryoidism

Inflammatory:
- RA
- Polymyositis

Osteomalacia

Malignancy:
- Paraneoplastic
- Lambert-Eaton Syndrome

Drugs:
- Alcohol
- Steroids

224
Q

Causes of Addison’s disease

A

Autoimmune
TB
Adrenal mets
Amyloidosis
Bilateral adrenalectomy
Waterhouse- Friedrichsen syndrome - meningococcal septicaemia and adrenal infarction

225
Q

Investigations in Addisons

A

Bloods:
- Low Na, high K
- Urea high - dehydration
- Adrenal autoantibodies
- TFTs

Morning cortisol
Short Synacthen test
ACTH

CT abdo - adrenal imaging

Pituitary imaging - MRi

Renin/aldosterone level - concurrent mineralocorticoid deficiency

226
Q

Causes of erythema nodosum

A

Idiopathic

Inflammatory disorders:
- IBD
- Sarcoidosis
- SLE
- Sjogrens

Infections:
- Streptococci
- URTI
- Mycoplasma
- TB
- Leprosy

Medications:
- OCP
- Penicillins
- Sulfonamides

Pregnancy

Malignancy:
- NHL
- AML

227
Q

Treatment in dementia

A

MDT approach:
- OT - cognitive rehabilitation
- SW
- Dietician
- Support groups

Medical:
- Antidepressants
- AChEi - donepezil, galantamine or rivastigmine in mild/moderate disease
- Memantine if intolerant of AChEi or in severe disease
- Antipsychotics for BPSD - if not amenable to conservative measures and if patients at risk of harm to themselves or others

228
Q

Driving restrictions for medical conditions:
- Stroke
- ACS
- CABG
- ICD
- Epilepsy
- Diabetes
- Syncope
- Brian tumours inc mets

A

Stroke/TIA:
- 1 month ban if recovers to good functioning, no need to tell DVLA unless residual loss of function

ACS:
- No driving for one week if successful PCI and no other interventions planned
- 4 weeks if no coronary intervention
- No driving if Group 2 and must notify DVLA

CABG:
- No driving for 4 weeks

ICD:
- No driving for 1 month and must inform DVLA
- No driving for Group 2

Epilepsy/seizure:
- First seizure - 6 months if seizure free
- Seizure while awake - 1 year
- If seizure due to medication change - 6 months
- For bus/lorry drivers - 5 years if single seizure, 10 years if epilepsy and on treatment

Diabetes:
- Inform DVLA if on insulin or having disabling hypoglycaemia
- If lorry driver inform if on any treatment
- Not to drive if having more than one episode of severe hypoglycaemia and inform DVLA
- Check BMs prior to starting journey and within two hors of starting journey

Syncope:
- Vasovagal -may drive
- Unexplained - may not drive for 6 months

Brain tumours
- Should not drive and should notify the DVLA

229
Q

Causes of Raynauds

A

Primary Raynauds

Secondary causes:
- Rheumatological diseases
– Systemic sclerosis
– RA
– SLE
– Polymyositis

Vascular disease:
– Berger’s - IgA vasculitis

Medications
– Beta blockers
– ADHD medications - methylphenidate

Hypothyroidism

Occlusive vascular disease e.g. thoracic outlet obstruction

Peripheral nueropathies:
- Injury to nerves especially repetitive motions e.g. using jackhammer
- Carpal tunnel (unilateral)
- Chemotherapy

230
Q

Differentiating primary vs secondary causes of Raynauds

A

Primary:
- Younger age of onset - teens, young 20s
- Bilateral
- No associated positive immunology
- Can have family history
- Should have complications e.g. digital ulceration

Secondary:
- Late onset - usually >30yrs
- May be unilateral or bilateral
- May have positive immunology
- Can have pitting ulcers/scarring

231
Q

Clinical signs in neurofibromatosis

A

Cutaneous neurofibromas
Cafe au lait patches
Lisch nodules
Axillary freckling

Neuropathy with palpable nerves

Bibasal crackling - pulmonary fibrosis

Raised blood pressure - associated with phaechromocytomas and renal artery stenosis

Reduced visual acuity

232
Q

Complications of neurofibromatosis

A

Phaechromocytoma

Renal artery stenosis

Intellectual disability
Epilepsy
Scoliosis