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PACES > Station 5 > Flashcards

Station 5 Flashcards

1
Q

Tuberous sclerosis features

A

AD inheritance (variable penetrance)

Epilepsy
Cognitive deficits
Tuberous CNS masses
Periungal fibromas
Adenoma sebaceum
Retinal phakomas
Shagreen patches (roughened)
Ash leaf marks
Respiratory
- cystic lung disease
- fibrosis
- recurrent pneumathoraces
Abdominal
- polycystic kidneys

Increased risk of renal / CNS malignancy

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2
Q

Neurofibromatosis features

A

AD inheritance

>5 cafe au lait spots
axillary freckling 
cutaneous neurofibromas
epilepsy
increased risk of intracerebral tumours
lisch nodules (iris)
CVS: phaeo, coarctation, high blood presure
MSK: scolios, lordosis / kyphosis, fractures / dislocations
Pulmonary fibrosis

NF2 specific

  • less cutaneous presentation
  • bilateral acoustic neuromas
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3
Q

Rheumatoid arthritis features

A

Hands

  • Z thumb
  • boutonniere deformity
  • swan neck deformity
  • MCP swelling, subluxation with ulnar deviation
  • disuse atrophy
  • carpal tunnel syndrome

Elbows
- rheumatoid nodules

Eyes

  • keratoconjunctivitis sicca (sjogren’s highly associated)
  • scleritis / iritis / episcleritis

Respiratory

  • pleurisy / pleural effusions
  • ILD

CVS

  • increased risk IHD
  • pericardial effusion / pericarditis

Vasculitis
- rash / peripheral neuropathy

Felty’s syndrome: leukopenia, splenomegaly, RA

Atlanto-axial subluxation

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4
Q

XRAY rheumatoid arthritis

A

Soft tissue swelling
Loss of joint space
Periarticular osteopenia & erosions
Deformity

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5
Q

Xray in OA

A

Loss of joint space
Subchondral sclerosis & cysts
Osteophytes

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6
Q

Rheumatoid arthritis management

A

General

  • PT/OT
  • MDT for extra-articular features
1) NSAIDs 
\+ start DMARD
---> MTX
2) steroids for flares 
3) biologics - anti TNF e.g. infliximab
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7
Q

SLE features

A

General: fatigue, fever

Hands / MSK

  • arthralgia, myalgia
  • Jaccoud’s arthropathy - non-deforming, resolves with making fist
  • avascular necrosis of the hip

Mucocutaneous

  • mouth ulcers
  • photosensitive rashes
  • malar rash
  • discoid lupus with scarring alopecia
  • vasculitic rash
  • livedo reticularis

Cardiorespiratory

  • pleurisy
  • ILD
  • pericarditis

Renal

  • hypertension
  • nephrotic syndrome
  • can progress to ESRF

Neuropsych

Haematological

  • autoimmune haemolysis
  • APLS
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8
Q

SLE management

A 
Acutely with usual low dose maintenance
- prednisolone
Hydroxychloroquine 
Immunosuppressants
- MMF - particularly if renal disease
- MTX
Acute renal failure -> IV cyclophosphamide 
Rituximab
Antiphospholipid -> warfarin
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9
Q

Hypertensive retinopathy

A

1) Silver wiring
2) AV nipping
3) Flame haemorrhages, cotton wool spots, exudates
4) Papilloedema

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10
Q

Diabetic retinopathy

A

Pre-proliferative

  • microaneurysms
  • dot and blot haemorrhages
  • cotton wool spots

Proliferative

  • neovascularisation of disc / macula
  • vitreous haemorrhage
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11
Q

Features of malaria

A

Non specific symptoms
- fevers, chills, malaise, headache, myalgia, diarrhoea

Severe

  • sepsis
  • renal failure
  • liver failure
  • coagulopathy / DIC
  • cerebral involvement
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12
Q

Dengue

A

Initial
- high fever, maculopapular rash, headache, GI upset

Haemorrhagic?

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13
Q

Typhoid

A

Fever, rigors, relative bradycardia -> GI upset, rose spots -> GI perforation

Ix: stool mcs

Mx: depends on local resistance, but ceftriaxone

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14
Q

Causes of traveller’s diarrhoea

A 
E Coli - bloody
Shigella - bloody
Salmonella - bloody
Campylobacter - bloody
Malaria
Typhoid
Dengue
Entomoeba histolytica - bloody
Giardiasis - parasite
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15
Q

HLA-B27 associations

A

Psoriasis
Ankylosing spondylitis
IBD
Reactive arthritis

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16
Q

Associations with SAH

A

ADPKD
Marfan’s
Ehlers Danlos

17
Q

Carbimazole counselling

A
  • marrow suppression -> must attend if feel feverish / unwell for blood count
  • explore family plans!
  • –> start after thyroid disease controlled
  • –> if planning for family soon, propylthiouracil
18
Q

Radioiodine cautions

A
  • makes Grave’s eye disease worse
  • cannot be used in pregnancy / breastfeeding
  • avoid if iodine allergy
  • avoid close contact with others 12 days, pregnant & children 30 days
19
Q

Thyroid eye disease grading

A

0-6 scale

No symptoms -> soft tissue swelling -> proptosis -> extraocular involvement -> corneal involvement -> sight loss

20
Q

Long-term risks hyperthyroidism

A

Heart failure, arrhythmias, hypertension, osteoporosis

21
Q

Complications of OSA

A

Pulmonary hypertension -> breathlessness, peripheral oedema with progressive right heart failure

22
Q

Pyrexia unknown origin causes

A 
Malignancy e.g. haematological (B symptoms)
Infection
- HIV
- TB 
- EBV
- subacute infective endocarditis 
- malaria
Rheumatological conditions!
23
Q

Arterial insufficiency signs

A 
Punched out ulcers
Loss of hair
Shiny skin
Loss of pulses 
Cool peripheries
24
Q

Causes of peripheral visual field loss

A 
Pituitary pathology
Diabetic retinopathy
Retinitis pigmentosa
Glaucoma
Retinal artery occlusion
Ischaemic optic neuropathy
25
Q

Retinitis pigmentosa features and associated syndromes

A

sporadic, AD, AR, X-linked
Progressive visual loss from childhood, peripheral, night vision, colour vision first
Bone spicule appearance

Associated syndromes

  • refsum’s disease (phytanic acid metabolism)
  • –> deafness
  • –> cerebellar ataxia
  • –> peripheral neuropathy
  • –> icthyosis
  • –> pes cavus
  • usher syndrome - + congenital SN hearing loss (commonest cause deaf/blindness UK)
26
Q

Dermatomyositis signs

A 
Mechanic hands
Gottron papules knuckles
Heliotrope rash
V shape sign chest 
Hyperpigmentation
ILD? 
Proximal myopathy
Myositis
27
Q

Anti synthetase syndrome

A

Anti jo / SRP (synthetase abs) + ILD + polymyositis or dermatomyositis

28
Q

Polyglandular autoimmune syndromes

A

Type 1 AIRE AR mutation: chronic mucocutaneous candidiasis, Addison’s, hypoparathyroidism, other autoimmune disease

Type 2: addison’s, type 1 diabetes, hypo/hyperthyroidism

29
Q

MEN

A

MEN1 - AD

  • pituitary adenoma
  • pancreatic tumour
  • parathyroid hyperplasia

MEN2a - AD

  • medullary thyroid
  • phaeochromocytoma
  • parathyroid hyperplasia

MEN2b - AD

  • medullary thyroid
  • phaeo
  • marfanoid
  • neurofibromas
30
Q

Methotrexate counselling

A
  • immunosuppressant -> seek medical advice if feel unwell
  • common SEs: nausea, diarrhoea, mouth ulcers, hair loss -> all improved by taking folic acid
  • baseline bloods, CXR, LFTs, renal function
  • avoid etoh
  • avoid pregnancy
31
Q

Rheumatoid arthritis diagnostic criteria

A

American college of Rheumatology criteria

  • number of joints
  • size of joints
  • duration of symptoms
  • positive serology
  • elevated inflammatory markers
32
Q

Causes of recurrent VTE

A

RFs VTE

  • immobilisation
  • long haul travel
  • surgery
  • malignancy
  • drugs: OCP, HRT

Thrombophilias

  • APLS
  • Factor V leiden
  • Protein C / S deficiency
  • Antithrombin deficiency
  • Active malignancy
  • Nephrotic syndrome

Other conditions

  • nephrotic syndrome
  • IBD
33
Q

Well’s score DVT & VTE

A

PE
>4 - PE likely -> CTPA
4 or less -> D dimer first

DVT
3 or higher - DVT likely -> duplex
<3 -> D-dimer first

  1. 5x kg weight enoxaparin
    - > NOACs e.g. rivaroxaban

3 months if known trigger
6 months if none
Consider longer if cancer / other active disease ongoing

34
Q

MTX toxicity mx

A

Stop methotrexate and statin
Repeat LFTs in 2 weeks
Monitor +/- consider restarting (altered dose) or switch

35
Q

PE severity

PESI score in PE

A

Massive - hypotensive <90mmHg, bradycardia, cardiac arrest

Submassive - RV strain/dysfunction, raised troponin

PESI 0 - can be managed as OP

Otherwise manage as IP

36
Q

Systemic sclerosis management

A 
Telangiectasia: camouflage creams
Scleroderma: immunosuppression
HTN: ACEi
Raynaud's: hand warmers, calcium channel blockers 
Reflux: PPI / antacids
Oesophageal dysmotility: prokinetics 
ILD: immunosuppression, transplant
PHT: sildenafil / bosentan
Inflammatory arthritis: low dose steroid taper (beware scleroderma renal crisis)
37
Q

Risk factors / associations IIH

A 
Female
Obesity
Cushing's
Hypothyroidism
PCOS
Systemic disease: CKD
Drugs: GH, tetracyclines, (OCP)
38
Q

Migraine

A

Aura
Unilateral
Dark room, photophobia, phonophobia

RFs: etoh, caffeine, poor sleep, stress, diet, menstrual cycle

Mx

  • Acute
  • –> simple analgesia
  • –> triptans
  • –> metoclopramide
  • Chronic
  • –> propranalol
  • –> topiramate
  • –> lifestyle
39
Q

Cluster headache management

A 
Acute
- oxygen
- SC / nasal triptan 
Chronic
- verapamil

PACES (6 decks)

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