Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

PACES > Station 2/5 > Flashcards

Station 2/5 Flashcards

(164 cards)

Start Studying
1
Q

What HLA is associated with RA?

A

HLA DR1/DR4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Which joints are typically affected in RA?

A

Any small or large joint that has synovial fluid. This includes C1/C2, MCPs, PIPs (not typically DIPs), and larger joints such as wrist, elbow, shoulder, knee.

It is a symetrical, inflammatory deforming arthropathy with extra-articular manifestations.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the radiological features of RA?

A

Subluxation
Synovitis
Soft tissue swelling
Joint space erosion
Ulnar deviation
Peri-articular osteopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the extra-articular manifestations of RA?

A

Anaemia

Felty’s

Lung fibrosis, fibrosing alveolitis, pleural effusions, lung nodules

Ophthalmic - sicca (most common), iritis, chloroquine retinopathy

IHD (same risk as T2DM), Pericarditis, conduction defects

Skin - rheumatoid nodules (subcutaneous)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What treatments do you know of for RA?

A
  1. Bridging steroids for flares
    Early DMARD’s
  2. MTX +/- second agent, such as leflunomide, sulfasalazine
  3. Anti-TNF (Infliximab)
  4. Anti-CD20 (Rituximab)
  5. T-cell co-receptor blocker (Abatacept)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the complications of MTX?

A
  1. Marrow suppression
  2. Accidental overdose
  3. Hepatotoxicity
  4. GI side effects including ulcerative stomatitis

Contraindicated
1. Pregnancy
2. Breast-feeding
3. Active infection
4. Pleural effusion
5. Ascites

It is contraindicated in pregnancy and contraception is recommended for 6 months after discontinuation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

How do you quantify a RA flare?

A

DAS28
1. Number of swollen/tender joints
2. Global pain score
3. ESR/CRP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the diagnostic criteria for RA?

A

ACR/EULAR
1. No. LARGE joints
2. No. SMALL joints
3. ESR/CRP
4. Anti-RF/CCP
5. >6/52

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the characteristic hand signs of RA?

A
  1. Wrist subluxation
  2. Thickened synovium
  3. Ulnar deviation
  4. Boutoiniers (PIP flex.d)
  5. Swan neck (PIP ex.d)
  6. Z thumb (MP hyper-ext)
  7. Rheumatoid nodules (elbows)
  8. Arthroplasty scars
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Talk me through examination of hands?

A
  1. Look for scars and deformimties
  2. Feel for warmth
  3. Assess muscle bulk
  4. Feel pulses
  5. Examine each joint in turn, counting tender/swollen joints
  6. Mobility: fist+release, prayer sign, upside down prayer sign
  7. Strength: OK, finger abduction, pincer grip
  8. Look at elbows
  9. Look at eyes (scleritis, episcleritis, scleromalacia)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the pattern of distribution of psoriatic arthritis, and how is it different from RA?

A

PsA usually affects smaller, distal joints and leads to dactylitis and juxta-articular new bone formation.
It also leads to enthesitis, nail changes, and skin psoriasis.

HLAB27 is associated with axial spondyloarthropathy
HLADR4 is associated with a RA type phenotype

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

How do you diagnose Psoriatic arthropathy?

A

CASPAR criteria can be used for early disease

  1. Psoriasis, or FHx
  2. Psoriatic nail changes
  3. Dactylitis
  4. Juxta-articular new bone formation
  5. Absent RF CCP
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the pathophysiology of skin psoriasis?

A
  1. Non-contagious autoimmune condition that leads to excessive inflammation and damage to keratinocytes; thought to be mediated by T HELPER cells
  2. Dilated blood vessels = infiltration of immune cells and secretion of inflammatory cytokines eg TGFb IL12, IL17, IL23.
  3. Destruction of keratinocytes and hyperproliferation leading to thickened stratum corneum and keratinisation
  4. Skin bruises easily and reveals underlying dilated blood vessels
  5. Contrast with eczema which is an IgE mediated Type 1 hypersensitivity reaction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the radiographic features of Psoriatic arthritis?

A
  1. Juxta-articular new bone formation
  2. Arthritis mutilans (pencil in cup)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How do you treat PsA, and what are the key differences to treamtent of RA?

A

Early disease is managed with NSAIDs; DMARD’s commenced later.
DMARDs like MTX and leflunomide are effective for skin and joints. Hydroxychloroquine avoided due to skin flares. Steroids avoided due to flares of skin upon withdrawal
Local cortisone effective for enthesitis
Biologics for severe disease including
1. Anti-TNF (infliximab)
2. Anti-IL12/23 (ustekizumab)
3. PDE4 inhibitors (apremilast)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What nail changes do you see in PsA?

A

Transverse nail ridging
Pitting
Onycholysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is Koebner’s phenomenon?

A

Plaques over sites of trauma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What % of patients with Psoriasis develop PsA and vice verca?

A

10% of patients with psoriasis will develop arthritis
Some develop arthropathy before psoriatic skin changes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What important extra-articular manifestations of Psoriatic arthritis are you aware of?

A

With all spondyloarthropathies:
-aortitis
-aortic regurgitation
-iritis
-apical lung fibrosis
-colitis
-enthesitis (specific for spondyloarthropathies)

DDx from other spondyloarthropathies by more asymetry, and more peripheral joint involvement.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What patterns of joint involvement do you see in psoriasis?

A
  1. Asymetrical oligoarthritis (25%)
  2. Symmetrical polyarthritis rheumatoid pattern - HLADR4
  3. DIP arthritis (10%)
  4. Arthritis mutilans (aggressive and rare)
  5. Spondylitis +/- sacroilitis (HLAB27) in 40%!!!!!
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are the key differences between PsA and RA?

A
  1. Equal sex distribution (RA more common in women)
  2. Asymmetric
  3. Distal (with some exceptions)
  4. Enthesitis
  5. Spondylitis in 40%!
  6. Early bone deformity
  7. Majority RF / CCP negative
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Steps of a knee exam

A
  1. Gait
  2. Erythema
  3. Patellar tap + sweep
  4. Feel joint lines
  5. Feel collaterals
  6. Feel for Baker’s cyst
  7. Active flexion
  8. Feel for crepitus
  9. Straight leg raise
  10. Knee hyperextension
  11. Sagging of knee when flexed 90 (PCL)
  12. Anterior drawer test (ACL)
  13. Test collaterals
  14. Alternative meniscal test (weight bear + twist)
  15. Patellar apprehension test
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Steps of a hip exam

A
  1. Gait
  2. Test for pelvic tilt
  3. Palpate - GT, ASIS
  4. True vs. apparent leg length
  5. Assess for fixed flexion deformity
  6. Active abduction + adduction
  7. Passive abduction + adduction
  8. External hip rotation
  9. Internal hip rotation
  10. FABER
  11. FADIR
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What do you know about mitral stenosis?

A
  1. AF
  2. PHTN
  3. Malar flush
  4. TAPPING apex beat (palpable first HS)
  5. Murmur = opening click in diastole: the earlier the click, the higher the pressure in the LA. This is followed by low pitched descrescendo-crescendo diastolic murmur heard best in expiration in left lateral with bell.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What are the causes of mitral stenosis?
1. Rheumatic fever 2. Congenital 3. SLE 4. Carcinoid syndrome 5. Fabry's disease
26
Which other condition can MS mimic?
LVSD. If the mitral valve area is <1, this can mimic LV failure when the LV is functionally normal
27
What are the complications of Mitral Stenosis?
LA dilation Pul HTN + pulmonary oedema Malar flush
28
What are the ECG / CXR findings in MS?
AF LAH (bifid P in II) LA dilation (biphasic or inverted P's in V1-V2) Double RHB Splaying of carina Orterners syndrome
29
Signs of severe pulmonary stenosis?
1. Ejection click and systolic murmur heard loudest in pulmonary area 2. Large v waves (TR) 3. Widely split S2 4. S4
30
Causes of PS?
Valvular Congenital Rheumatic Noonan's Down's Syndrome Supravalvular Williams Rubella Subvalvular Fallot's
31
What is wide splitting of S2?
During inspiration = more blood returns to chest = more volume in right heart = delayed emptying of RV = delayed Pulmonic valve closure. When there is a problem with the right side of the heart this takes longer: PS RBBB ASD VSD
32
What is reverse splitting of S2 and what causes it?
LV stress = delayed LV emptying S1 then P2 then A2. During inspiration, the split disappears as P2 and A2 come closer together. Causes LBBB HOCM Sev AS WPW B (right accessory pathway)
33
What is the pathophysiology of HOCM?
Hypertrophic OBSTUCTIVE cardiomyopathy is evidence of an LVOT gradient
34
Clinical signs of HOCM?
1. Prominent a due to septal bulging 2. Double carotid impulse 3. Reverse split S2 4. MR (anterior motion of valve) 5. Louder during Valsalva (reduced pre-load = reduced Starling = obstructed outflow)
35
Genetics of HOCM?
Autosomal Dominant Several genes, all encoding sarcomeric proteins Associated with Friedrich's ataxia, Fabry's, WPW
36
What are the ECG findings of HOCM?
1. LVH criteria (S1+R6 >35mm) 2. Deep antero-lateral TWI 3. Deep infero-lateral Q waves
37
What must you avoid in HOCM?
Drugs that reduce pre-load as this accentuates the outflow gradient. EG nitrates, inotropes
38
Echo findings of HOCM?
MR - MR SAM - systolic anterior motion of mitral valve ASH - assymetric septal hypertrophy
39
Management of HOCM?
Amiodarone if AF Beta-blockers Cardioverter defibrillator Dual chamber pacemaker Endocarditis prophylaxis
40
What is the inheritence of Marfan Syndrome?
Autosomal Dominant mutation in fibrillin gene that codes for collagen
41
What do you know about the genetics of Ehler Danlos syndrome (EDS)?
AD, AR, denovo mutations in gene encoding for collagen III Principally affects joints, blood vessels, heart, eyes
42
What are the complications of EDS?
1. Joints = deformities, pain, impaired function 2. Skin = easy bruising and fragility 3. Heart = MVP, MR, AR, Aortic dissection, cardiac conduction abnormalities 4. Vascular = haemorrhage 5. GI = bleeding, diverticulae, diaphragmatic herniation, colonic rupture 6. Eyes = glaucoma, retinal detachment
43
What do you know about Osteogenesis imperfecta?
AD mutation usually in COL1A1/2 that codes for collagen I This leads to B: weak bones and atraumatic fractures I: Eyes - blue sclera (loss of collagen = choroidal veins visible) T: Teeth - dentinogenesis imperfecta E: Ear: otosclerosis (conductive hearing loss) Cardiac: Valvular disease - MVP, bicuspid aortic valve, AR/aortic root dilation Rx: 1. Vit D/calcium/bisphosphonates 2. OT/PT 3. Hearing aids 4. Valve replacement/repair if needed
44
DDX blue sclera
1. EDS 2. Marfan 3. OI 4. Pseudoxanthoma elasticum 5. Diamond Blackfan (Pure red cell aplasia)
45
What do you know about Pseudoxanthoma elasticum?
AD/AR ABCC6 Abnormal MINERALISATION of ELASTIN Mid-dermal deposition of calcium that triggers a metabolic response Dermis: Pseudoxanthoma plaques and plucked chicken) in axilla/groin/neck Eyes: Angoid streaks (85%) Heart: AR, MR, MVP Vessels: Early plaques, CAD, PVD, HTN, Renovascular disease GI: haemorrhage due to friable vessels
46
What is Systemic sclerosis?
Damage of endothelial vessels and the presence of certain environmental triggers leads to abnormal repair and fibroblast recruitment with ensuing damage to tissue. Triggers include CMV, PB19, silica, malignancies, and even some drugs like bleomycin. This leads to thickening and ischaemia of tissue.
47
What patterns of systemic sclerosis do you know of, and how do they differ?
Limited cutaneous scleroderma affects the fingers and arms predominantly. Raynaud's is the PC in 70%. Patients also develop calcinosis of the digits, Raynaud's, Esophogeal dysmotility, Sclerodactyly, and Telangiectasia. CREST syndrome is associated with later internal organ (particularly lung) involvement, and carries a better prognosis. ANA (anti-centromere) Diffuse scleroderma is associated with early internal organ involvement, and the mortality comes from lung fibrosis. The skin of the arm, face, neck, trunk and legs are involved. ANA (anti-Scl70)
48
What are the consequences of Systemic Sclerosis?
1. GI: Oesophogeal dysfunction, dysphagia, malaborption, SIBO, PBC 2. Skin: Raynaud's, gangrene 3. MSK: arthritis, osteopenia (malabrption) 4. Renal: HTN, scleroderma renal crisis 5. Resp: ILD, pleural effusions 6. CVD: Restrictive cardiomyopathy, pericarditis, PHTN, conduction defects
49
How do you treat systemic sclerosis?
MDT - resp, cardio, renal, GI Patient education, counselling, psychology (lots of uncertainty) Symptom Control PPI, rifaximin (SIBO) Vasodilators Dihydropiridine CCB like nifedipine, amlodipine - For Raynaud's Sildenfanil for PHTN IV prostanoids for severe Rayanud's/PHTN Targeted Immunomodulatory - MMF, Rituximab, Tocilizumab (IL-6). Stem cell transplant in high risk patients (this comes with a 10% mortality so need risk vs. benefit discussion) Anti-fibrotic - Nintedanib ****AVOID high dose steroids as this can precipitate renal scleroderma crisis****
50
What is the usefulness of nailfold capilloroscopy in Systemic sclerosis?
The nailfold is one of the few places you can visualise capilaries. There is significant capillary dropout due to microangiopathy, with regeneration of poorly constructed vessels in systemic sclerosis/Raynauds
51
What are the diagnostic criteria for lupus?
ANA >1:80 and signs/symptoms/labs suggestive of lupus: Labs: Anti-dsDNA, Anti-smith, Anti-cardiopilin, lupus anti-coagulant, low C3/C4 1. Constitutional 2. Neurological 3. Mucocutaneous 4. Photosensitivity 5. MSK 6. Serosal 7. Renal 8. Haematological
52
What are the classic skin features of lupus?
Discoid lupus (sun-exposed) Malar rash on face sparing naso-labial folds Painless mucosal ulcers Tender small joints
53
What labs are needed to diagnose SLE?
ANA Anti-dsDNA Anti-Smith AntiU1 RNP Anti-Ro (strongly implicated in lupus of pregnancy) Anti-La Low complement
54
What pregnancy complications of lupus do you know?
Auto-antibodies can cross the placenta and lead to congenital heart block (Anti-Ro)
55
What are the renal manifestations of lupus?
6 different types of lupus nephritis, most common and most severe is diffuse proliferative lupus nephritis
56
How do you treat lupus?
Mainstay is hydroxychloroquine +/- other DMARDs like AZA, MMF, calcineurin inhibtors (ciclosporin/tac) Steroids for flares ACEi if proteinuria
57
What are the systemic features of lupus?
Anaemia Arthritis Raynauds Dermatomyositis Discoid rash Malar rash Mouth ulcers ILD, pleural effusions, pleurisy pericarditis, Libman-Sacks endocarditis, PHTN CNS involvement Renal HTN, nephritis Haematological - lymphadenopathy, splenomegaly Eyes - Sjogren syndrome
58
What drug causes of lupus do you know?
Positive anti-histone antibodies, negative anti-dsDNA. Resolution of effects upon cessation. F:M 1:1 (cc SLE F:M 9:1) OCP Hydralazaine Procainamide (most common) Carbamazapine Phenytoin Chlorpromazine Tetracyclines
59
What are the cardinal features of ankylosing spondylitis?
1. Stiffening of vertebral bodies with ensuing inflammation. Also involves eyes and blood vesels. Can involve C1/C2 leading to atlando-axial subluxation 2. Seronegative arthropathy 3. Strongly associated with HLA-B27 MHC class I 4. Primarily affects the intervertebral and facet joints of the spine, and the sacro-iliac joint. 5. Inflammation draws in fibroblasts that deposit fibrin which limits movement. Eventually osteoblasts lay down new bone which leads to immobility.
60
What are the non-spine features of ank spond?
1. Anterior uveitis 2. Aortic regurgitation 3. AV conduction defects 4. Enthesitis 5. Apical fibrosis 6. Ank spond is a rare cause of amyloidosis
61
Treatment of Ank Spond
1. NSAIDs 2. Excercise 3. DMARD's in severe cases 4. Biologics such as anti-TNF (adalimumab, etanercept) and anti-IL17 5. Rarely, surgery (spinal surgery is usually avoided)
62
What is osteomalacia?
Faulty bone mineralisation caused by low Vit D, low Calciun, low phosphate. In children this leads to rickets, in adults it leads to softening of the bones due to poor mineralisation
63
How do PTH and Vit D regulate calcium?
Vit D is activated in the liver (25OH) then kidneys (1OH) 1,25VitD is responsible for re-absorption of calcium in the kidneys, and increased calcium/phosphate reabsorption in the gut. PTH stimulates bone resorption to release calcium into the blood. It also stimulates phosphate renal excretion and calcium gut absoroption.
64
What are the signs of ostemalacia?
Bone fragility Prox muscle werakness Muscle spasms (low ca) X ray - looser zones Labs Low Vit D, Low calcium, raised ALP (trying to stimulate bone actiivty). Low calcium stimulates PTH which stimulates phosphate excretion. Low Vit D, Low Ca, Low Pho Raised ALP and raised PTH
65
What is the pathophysiology of Paget's disease?
1. Excessive bone remodelling This leads to skeletal deformities nad fractures. Can involve one bone, or the entire skeleton Might have an infective/genetic component, and most frequently seen in men of northern latitudes Three phases 1) Lytic phase 2) Blast phase 3) Sclerotic phase - disorganised and fragile bone Leads to -Kyphosis -Pelvic assymetry -Bow legs -Arthritis -Optic, Vestibulocochlear nerve entrapment -High output cardiac failure Diagnosis 1. Skeletal signs 2. Raised ALP, rest bloods normal 3. Bone biopsy Rx: 1. Symptom control 2. Bisphosphonates 3. Surgery
66
What is osteoporosis and how is it different from osteomalacia?
Balance tips towards bone breakdown Leads to reduced number of osteoid units, however the cells are functionally normal and there is preserved mineralisation (in contrast to osteomalacia where there is reduced mineralisation)
67
What are risk factors for osteoporosis?
Post-meonopauseal Low calcium Alcohol Smoking Steroids Thyroxine Senile osteoporosis Reduced gravity/weight bearing excercises
68
What is the FRAX score?
10-year fracture risk score. If FRAX comes out intermediate, a DEXA is recommended. Components of FRAX score include Age, sex, ethnicity, prev fracture history, RA, DM, alcohol, secondary osteoporosis, and family history.
69
What is the T / Z score?
Compares the density of the long bones to matched controls.
70
What is osteopetrosis?
Inherited dysfunction of osteoclasts that leads to reduced burn turnover and disproportionate osteoblast activity. Can be AR or AD. -Fractures due to poorly flexible bones -Extramedullary haematopoeisis -Nerve compression
71
How is Ank Spond disease activity assessed?
Bath index of disease severity 10 point score >4 = active disease
72
How do you assess disease severeity of acute flares in UC?
Truelowe and Witts Mild: <4 stools a day Mod: 4-6 stools a day, no sys upset, CRP <30 Sev: >6 and any signs of systemic upset
73
What are the indications for surgery in a flare of IBD?
1. Refractory to medical therapy 2. Any evidence of dilatation on imaging 3. Any evidence of fistulating disease
74
What are the endoscopic differences between Crohns and Ulcerative colitis?
UC is superficial Crohns is full thickness (trans-mural) UC affects the large bowel exclusively (with backwash ileitis) Crohns can affect the entire GI tract Crohns will have skip lesions (normal tissue) UC does not have skip lesions Crohns can affect the terminal ileum exclusively UC always starts in the rectum and progresses proximally Crohns - aphthous ulcers, cobblestone appearance UC - crypt abscesses
75
Are there concerns for malignancy in IBD?
Yes, if there is colitis there is concern for CRC, particularly in UC After 10 years - index surveillance colonoscopy to detect early pre-malignant changes; further colonoscopy depending on risk level at that stage.
76
What is the pathophysiology of psoriasis?
Abnormal immunological response in the epidermal layers that leads to excessive inflammation and damage to the skin. This is mediated by dendritic cells acting as antigen presentation cells and T cells that release inflammatory cytokines including TNF, IL17, IL23
77
How do you assess disease severity in psoriasis?
Psoriasis area and severity index (PASI), and body surface area involved
78
What environmental triggers for psoriasis do you know?
Trauma Sunburn Stress Smoking Alcohol Beta haemolytic strep Drugs (Lithium, ACEi's, beta-blockers, NSAID's, terbinafine, anti-malarials)
79
What are the primary treatments for psoriasis?
Moistuirsers and emollients Potent top steroid + top Vit D (calciptriol) - 4 week courses If no improvement, can use top Vit D BD Coal tar (inhibits DNA synthesis) Dithranol (inhibits DNA synthesis) Phototherapy (UVB, or UVA + psoralen) Systemic treatments including MRX, ciclosporin, biologics (ustekinumab, adalimumab, infiliximab)
80
What variants of psoriasis do you know?
Plaque Pustular Guttate Palmoplantar Erythrodermic Inverse psoriasis (flexor surfaces)
81
What is Auspitz sign?
Stripping of skin in psoriasis reveals underlying hypertrophied dermal capillaries
82
What is Koebner phenomenon?
Plaque forms over sites of trauma
83
What genes are implicated in NF?
NF1 on chromosome 17 NF2 on chromosome 22 Both these genes are TSG's. Their absence leads to the fibrous tumours arising from the nerves - fibromas
84
What are the cutaneous manifestations of NF1?
Neurofibromas - cutaneous bumps under skin CAL's (>6) Axillary/groin freckling Lisch iris nodules
85
What are the manifestations of NF2?
Schwannomas (acoustic neuromas) Meningiomas Cataracts Retinal hamartomas Less frequent skin manifestations
86
Other than skin and nerve involvement, what other organs can be affected in NF?
Lungs - apical fibrosis Heart - restrictive cardiomyopathy Spine - scloliosis Adrenal - phaeo Kidneys - Renal artery stenosis
87
What are the responsible genes in Tuberous Sclerosis?
TSC1 and TSC2. Both are inherited in an autosomal dominant manner, and their absence leads to uncontrolled mTOR activity. This leads to benign tumours throughout the body (made up of various cell types) and an increased lifetime risk of cancer
88
Which organs are most often affected in Tuberous Sclerosis?
Brain - gliomas, epindymomas, astrocytomas Skin - angiofibromas (face), subungal fibromas, ash leaf spots, Shagreen patches Kidneys - angiomyolipolas (can bleed) Lungs - lymphangioleiomyomatosis (cysts) Eyes - reintal hamartomas.
89
What investigations need to be sent from an LP for suspected meningitis?
1. Cell count and differential 2. Protein, glucose, lactate 3. Gram stain and culture 4. Viral studies Bacterial meningitis Raised WCC, pred Neutrophils High protein Low glucose Positive gram strain Viral meningitis Raised WCC, pred lymphocytes Normal/High protein Normal glucose Viral PCR+
90
Comps of bacterial meningitis
1. Severe morbidity 2. Deafness 3. Blindness 4. Cognitive impairment 5. Vascular - amputations due to sepsis
91
What are the manifestations of hereditary haemorrhagic telangiectasia?
Skin - telangiectasia Mucus membranes - epistaxis GI - bleeding Lungs - AV malformations CNS - SAH, migraines, spinal AV = paraparesis Liver - can lead to liver failure due to AV malformations
92
What is your differential for telangiectasia?
Systemic Sclerosis SLE HHT Mitral stenosis Polycythaemia Carcinoid syndrome
93
What organs can be affected in acromegaly?
Soft tissue - hands, feet, face Face: macroglossia, prominent supraorbital ridge, prominent jaw Cardiac - cardiomyopathy, IHD, HTN Endocrine - T2DM Visual impairment - bitemporal hemianopia
94
Rx for Acro?
Surgery RT Octreotide (somatostatin analogues) Pegvisimont (GH receptor antagonist)
95
OSA symptom scoring criteria
STOP-BANG questioniarre Epworth (>11)
96
OSA Diagnostic test?
Overnight polysomnography for apnoea hypopnea index
97
What is the reason OSA leads to daytime somnolensce?
Overnight arousal and frequent waking leads to poor quality of sleep and fatigue
98
What is the pathophysiology of dermatomyositis?
1. Typically involves skin and muscle tissue 2. Abnormal immune response and activation of the complement system that leads to recruitment of the membrane attack complex (MAC). 3. Due to molecular mimicry, the MAC recognises self-antigens as foreign and leads to cell lysis 4. Soluble antigens released from cell lysis that are in the blood deposit in carious blood vessels and this eventually leads to tissue hypoxia due to activation of the MAC complex
99
What are triggers for dermatomyositis?
Genetic: HLADR3/DR5 Environment: Cocksackie virus, cancers
100
Which cancers are associated with dermatomyositis?
An underlying malignancy is found in 20-25% of cases of dermatomyositis. These are typically ovarian, lung, breast
101
What are the clinical manifestations of dermatomyositis?
Skin: Gottrens papules Heliotrope rash Malar rash Shawl sign Hypertrophy of cuticles Non-scarring alopecia. Palmar hyperkeratosis (mechanics hands). Photosensitive skin changes. Muscles: Prox weakness Atrophy Dysphagia Resp muscle weakness Lungs: Fibrosing alveolitis Organising pneumonia
102
What investigations would you perform for suspected dermatomyositis?
1. FBC UE 2. CK 3. ANA, Anti Jo1, Anti SRP, Anti M2 4. Muscle bx 5. Directed screening for underlying malignancy
103
DDx for Raynaud's
1. RA 2. SS 3. Lupus 4. Dermatomyositis 5. Polymyositis 6. Myeloproliferative disorders 7. Hep B/C 8. Thoracic outlet syndrome 9. Atherosclerosis 10. Carpel tunnel 11. Hypothyroidism 12. Drugs (beta blockers, cyclosporin)
104
What are the differences between Raynaud's disease (primary) and Raynaud's phenomenon (secondary)?
Primary - <40y No associated systemic features NO nailfold capillaries Never necrotic Normal ESR Normal ANA
105
What drugs can cause Raynaud's?
Betablockers OCP Ciclosporin Chemo
106
Treatment for Raynaud's
CCB's Prostacyclins Nitrates
107
Categorise lymphomas
A silly system, but commonly classified as Hodgkin's (Reed Sternberg B-cells) and non-Hodgkins. Non-Hodgkins includes: High-grade Lymphoblastic (behaves like ALL) B-cell (Burkitt's, DLBCL, Mantle cell, Follicular)
108
How do you stage Hodgkin's lymphoma?
Ann-Arbour staging 1. Single node 2. More than one node, same side of diaphragm 3. Above and below diaphragm 4. Extra-nodal B symptoms at ant stage
109
What is the treatment for Hodgkin's lypmphoma?
ABVD Adriamycin Bleomycin Vincristine Doxarubicin
110
What is the main treatment for hight grade NHL?
R-CHOP Ritux Cyclophosphamide Hydroxydaunorubicin Oncovincristine Pred and then maintenance Rituximab
111
What are the three cardinal features of anti-phospholipid syndrome?
1. Thrombocytopenia (antibodies against platelets) 2. Recurrent miscarriages 3. Arterial (stroke) and/or venous clots (DVTs) 4. Livedoreticularis
112
Can APS co-exist with other conditions?
APS can either be primary, or associated with other conditions such as SLE, other autoimmune and lymphoproliferative disorders
113
What specific tests will you perform in suspected APS?
Autoimmune screen anti-cardiolipin Anti-beta2glycoprotein 1 anti-phospholipid Lupus anticoagulant
114
How do you treat APS?
Primary thromboprophylaxis - low dose aspirin Secondary thromboprophylaxis - warfarin (INR 2-3, increased to 3-4 if clot on warfarin)
115
What causes for strokes in young patients do you know off?
1. Extra-cranial - neck hyperextension 2. Vasculitis 3. Cardiac - embolic (mxyoma, endocarditis, valvular pathology, AF) 4. Substance misuse - cocaine, methamphetamine 5. APS 6. Familial hyperlipidaemia 7. Sickle cell disease 8. Rare metabolic/mitochondrial causes such as MELAS
116
Causes for erythema nodosum?
STREP THROAT! TB Sarcoid (Lofgren's) Myeloproliferative disorders IBD Behcet's Drugs (OCP, penicillin, sulphonylureas)
117
Describe erythema multiforme
Immune mediated mucocutaneous condition characterised by target lesions +/- mucosal involvement. Start as distal papules that spread proximally, later forming target lesions (concentric rings of colour variations)
118
What causes erythema multiforme?
HSV CMV, EBV, influenza, COVID-19 Medications - OCP, penicllin, carbamazapine Vaccinations SLE Sarcoidosis Malignancy Mycoplasma pneumonia - now considered a distinct entity
119
Name some systemic causes for livedo reticularis
APS PRV SLE PAN GPA/EGPA Walendenstrom's Cholesterol embolism
120
What conditions are commonly associated with vitiligo?
Apolecia areata Hashimotos Graves Addisons Hypoparathyroidism DM IPF PBC Pernicious anaemia Atrophic gastritis
121
What do you know about bullous pemphigoid?
Bullous = Basement Autoimmune type 2 hypersensitivity reaction against hemidesmosomes that anchor basal cells to basal membrane IgG targets BPAG1+2. This leads to mast cell degranulation and inflammation Genetic component and environmental triggers (furosemide, penicillamine, NSAIDs, abx) Nikolsky sign negative (no intraepidermal splitting) Rx topical and sytemic steroids
122
What do you know about Pemphigous Vulgaris?
Autoimmune type 2 hypersensitivity reaction against intra-epidermal desmosomes that hold keratinocytes together in the stratum spinosum layer Can either affect mucosal cells only (Desmoglein 3) or skin+mucosal cells (Desmoglein 1+3) Histologically there is tombstoning (intra-epidermal disruption of keratinocytes) Nikolsy sign positive (lateral pressure causes a split between upper and lower layers of epidermis) Rx: topical and oral steroids, rituximab, oral rinses with lidocaine
123
What do you know about Peutz Jegher's syndrome?
Autosomal dominant mutation in the TSG STK11 that is expressed throughout the GI tract and also various other tissue including skin, breast, lung, ovaries, testicles Leads to the development of hamartomas in the GI tract that can become malignant. These can lead to bleeding and obstruction. Skin manifestations include flat mucocutaneous melanocytic macules on the mucosal surfaces and also palms and soles Risk of GI, breast, lung, ovarian, testicular cancer
124
What is necrobiosis lipoidica?
Typically affects young female diabetic patients It is also associated with hypertension, thyroid disease and obesity It is a disorder of BLOOD VESSELS Starts as a red papule, becomes a plaque, and then slowly enlarges to a yellow patch with a red rim Can ulcerate and turn into a squamous cell cancer
125
What is pyoderma granulosum?
Extremely painful Rapidly enlarging tender full-thickness ulcer with blue borders Non-infectious neutrophilic dermatosis More common in over 50's Frequently associated with autoimmune conditions such as IBD, RA, GPA Rx: Urgent immunosupression
126
What do you know about colorectal cancer genetics?
1. Lynch Syndrome (AD, MSH2, MLH1, MSH6, PMS2) 2. FAP - Chr5, autosomal dominant - large bowel polyposis syndrome 3. Gardener's - variant of FAP (5q21) - CRC, skull osteomas, epidermoid cysts, thyroid cancer
127
Talk me through the Wells DVT score
Paralysis Cancer Immobilised Prev DVT Is an alternative diagnosis as likely? Calf swelling, leg swelling, collaterals, tenderness along deep venous system, pitting oedema 1 = D-Dimer >=2 Doppler
128
Talk me through the Wells PE score
DVT Cancer Immobilised Prev DVT Is PE as equally likely as other diagnosis HR Haemoptysis 1 = D-Dimer >2 = CTPA/VQ
129
What are the components of a thrombophilia screen?
1. Factor V Leiden 2. Prothrombin mutation 3. Protein C 4. Protein S 5. Anti-thrombin III (rare but aggressive) Drug history (OCP)
130
Are there any scoring criteria you can use to risk stratify patients for inpatient/outpatient Rx for PE?
PESI Score predicts 30-day mortality and takes into consideration history, and clinical findings
131
What are contraindications for thrombolysis of PE?
Intracranial disease/bleed Ischaemic stroke (<3mo) Brain/spine surgery (<3mo) Internal bleeding Coag disorder Recent head trauma
132
VQ vs. CTPA risks to pregnant pt?
Both have radiation - CTPA radiation comes from the X-rays, VQ radiation comes from the dye. CTPA = higher dose of X-ray radiation to breast tissue VQ = higher dose of dye-ionising radiation to fetus, but still very small amounts
133
Do you know the inheritence of Retinitis Pigmentosa?
AD, AR, X-linked recessive, and 30% are de novo.
134
Do you know of any syndromes involving retinitis pigmentosa?
Kearn Sayre: Mitochondrial. Ophthalmoplegia+cerebellar ataxia. Usher: AR. Sensosineuronal deafness. Refsum: Ataxia+muscle wasting. Alport: X linked recessive. Type IV collagen. Haematuria+GS+SN deafness.
135
What are the clinical features of diabetic retinopathy?
Non-Proliferative Microaneurysms Blot haemorrhages Hard exudates Soft exudates Venous beading Mild - microaneurysm Mod - microaneurysm and other signs Sev - involves all 4 quadrants Proliferative - neovasc
136
What is the treatment of proliferative diabetic retinopathy?
If disease involves fovea = anti-VEGF (benralizumab) If disease does not involve fovea = laser photocoagulation
137
What are the clinical features of hypertensive retinopathy?
1: Silver wiring 2: AV nipping 3: flame/blot haemorrhages and hard exudates 4. Papilloedema
138
What is the pathophysiology of retinal vein occlusion?
Arterial compression - usually retinal vein BRANCHES Thrombus - usually the central retinal vein
139
What are the clinical signs and symptoms of a retinal vein occlusion?
1. Acuity 2. RAPD 3. SWOLLEN disc (ddx optic) 4. Retinal haemorrhages (stormy sunset) 5. Might develop neovasc an acute angle closure glaucoma
140
What are the causes of a retinal arterial occlusion?
The retinal artery is a terminal branch of the ophthalmic artery. Its occlusion can produce retinal infarction. Can be: 1. Embolic 2. Rapid raised IOP 3. GCA 4. Profound hypotension 5. Vasospasm 6. Hypercoagulable states
141
What are the signs and symptoms of retinal artery occlusion?
1. Sudden painless visual loss 2. Pale retina 3. Cherry red spot on fovea (choroidal arteries unaffected) 4. RAPD
142
What is the treatment for central retinal artery occlusion?
1. Occular massage 2. IV acetazolamide/anterior segment paracentesis (reduce IOP) 3. Treat underlying factor
143
What are the risk factors for macular degeneration?
Age FHx Smoking Poor nutrition Myopia
144
What are the clinical signs of macular degnereation?
Dry: Drusen + atrophy Wet: Neovascularisation in choroid
145
What are the symptoms of macular degeneration?
Central visual loss Distortion
146
What is the treatment for macular degeneration?
Anti-VEGF (ranibizumab)
147
What are the clinical findings of anterior uveitis?
1. Red 2. Painful 3. Asymetric pupil
148
What conditions is uveitis associated with?
1. Ank Spond 2. Behcet's 3. MS 4. Infections: HSV, TB, HIV
149
What are the risk factors for acute angle closure glaucoma?
Asian, short axial eyeball, Age, pupillary dilation
150
What are the symptoms of acute angle closure glaucoma?
Extreme pain Blurred vision Haloes Red eye Mid-dilated or fixed dilated pupil Corneal oedema Raised IOP
151
What is the treatment for acute angle closure glaucoma?
Topical betablockers/pilocarpine IV acetazolamide Peripheral laser iridotomy (hole in iris)
152
What is keratitis?
Inflammation of the cornea Site threatening emergency Caused by infection (bacterial, fungal, viral) or inflammation (blepharitis induced irritation) Ix - slit lamp and fluorescein = corneal ulcer
153
Scleritis vs. episcleritis?
1. Both associated with inflammatory conditions such as IBD and RA 2. Scleritis is painful, episcleritis is not 3. Both look pretty similar 4. Scleritis is associated with photophobia and gradual decrease in visual acuity 5. In episcleritis, injected vessels are mobile with gentle pressure and will blanch with phenylephrine drops 6. Scleritis needs oral NSAID's /steroids, most cases of episcleritis can be managed conservatively
154
What do you know about deQuevarian's thyroiditis?
Typically post a viral illness Phase 1: Lymphocytic infiltration: Hyperthyroidism, raised ESR Phase 2: euthyroid Phase 3: Hypothyroidism for weeks-months Phase 4: Back to normal Ix: Thyroid scintigraphy: globally reduced uptake of I131 Rx: Self-limiting Pain control - NSAIDs Steroids if hypothyroid
155
What classes of diabetic drugs do you know for patients with T2DM?
1. Biguanide: increase liver and muscle insulin sensitivity (metformin) 2. SGL-2 inhibitor: Inhibits re-absorption (dapaglifozin). 3. DPP4 inhibitors: reduce breakdown of incretins (sitagliptin, linagliptin). Caution if hx pancreatitis/pancreatic cancer. Can use linagliptin in renal impairment 4. Sulphonylureas: Increase insulin secretion (glizlazide) - risk of hypo's 5. Pioglitazone: PPAR-gamma activator = more glucose transports - risk of fluid retention, osteoporotic fractures, and bladder cancer
156
What are the complications of spina bifida?
1. Urinary stasis + recurrent infections 2. Lower limb spasticity 3. Hydrocephalus 4. Arnold-Chiari malformations 5. Recurrent meningitis 6. Syrinx
157
Which malignancies are associated with acanthosis nigricans?
Gastric (most strongly)
158
What conditions are associated with acanthosis nigricans?
1. T2DM 2. Obesity 3. PCOS 4. Acromegaly 5. Cushing's
159
What are the features of Williams syndrome?
1. Spotaneous microdeletion 2. Mild intellectual impairment 3. Characteristic facies with broad forehead 4. Supravalvular aortic stenosis
160
Steroids in ascending order of potency
1. Hydrocortisone (Mild) 2. Betamethasone 0.025% 3. Fluticasone/Betamethasone 0.1% 4. Clobetasol
161
What is the pathophysiology of eczema?
Allergen enters the dermis and triggers a Type I hypersensitivity reaction. On repeat exposure there is IgE mediated mast cell degranulation. Self-perpetuating cycle of skin breakdown, skin dryness, and itching.
162
What are the treatments for eczema?
1. Identify triggers 2. Moisturise skin 3. Steroids 4. Topical tacrolimus 5. Immunosuppresion 6. UV radiation
163
Causes of galacotthoea?
Physiological (pregnancy) Prolactinoma Exercise, stress Acromegaly (1/3 of patients) PCOS Hypothyroidism (Raised TRH stimulates prolactin release) Drugs (metoclopromide, domperidone, haloperidol, rarely SSRI's)
164
Sickle cell
AR HBB gene (beta globin) Remember HbA is composed of a2b2 Low haptoglobin (intravasc haemolysis) Scleral icterus, jaundice, bilirubin gallstones Bone marrow hyperplasia - enlarged skull Hepatomegaly (extramedullary haematopoiesis) Autosplenectomy (R/O Strep, haemophilius, Neisseria, Salmonela) Chest syndrome Renal necrosis Priapism Rx: Prophylaxis, treat infections early, pain relief, hydroxyurea (increases gamma globin = ^ HbF)

PACES (6 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions