Disease Profiles 2 Flashcards

1
Q

Systemic Lupus Erythematous: Management - Mild-Moderate Disease presents how?

A

Skin disease with arthralgia

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2
Q

Systemic Lupus Erythematous: Management - Mild-Moderate Disease

A

Hydroxychloroquine
Short course of NSAIDs for symptom control
Steroids - IA for arthritis and topical for cutaneous manifestations

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3
Q

Systemic Lupus Erythematous: Management - Moderate-Severe Disease presents how?

A

Inflammatory arthritis with organ involvement

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4
Q

Systemic Lupus Erythematous: Management - Moderate-Severe Disease

A

Hydroxychloroquine
Microphenylate
Treat organ complications appropriately

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5
Q

Systemic Lupus Erythematous: Management - Moderate-Severe Disease acute flare up

A

Immunosuppressants - Methotrexate or Azathioprine
Oral steroids for short periods - tries to induce remission

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6
Q

Systemic Lupus Erythematous: Management - Severe Organ Disease e.g. Lupus Nephritis or CNS lupus

A

IV steroids and Cyclophosphamide

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7
Q

Systemic Lupus Erythematous: Management - What may be considered for unresponsive cases? (2)

A

IV immunoglobulin
Rituximab

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8
Q

Systemic Lupus Erythematous: Potential complication

A

Increased risk of PE - consider if X-Ray is normal

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9
Q

Systemic Lupus Erythematous: Subcutaneous Cutaneous Lupus presentation

A

Small erythematous lesions on the neck, shoulders and forearms that spares the face

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10
Q

Systemic Lupus Erythematous: Discoid Lupus Erythematous presentation

A

Non-cancerous chronic skin condition that has erythematous raised scaled plaques with active inflammation that is triggered by UV light exposure

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11
Q

Systemic Lupus Erythematous: Discoid Lupus Erythematous affects what areas? (3)

A

Face
Neck
Head

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12
Q

Systemic Lupus Erythematous: Urine Dipstick Test will show what?

A

Anti DNA antibodies - these are toxic to the kidney so must test if SLE is suspected

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13
Q

Systemic Lupus Erythematous: If the urine dipstick test is positive for AdsDNAA then what must be conducted?

A

Biopsy to confirm nephritis

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14
Q

Sjogren’s Syndrome

A

Autoimmune condition that affects the exocrine glands characterised by lymphocytic infiltrates

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15
Q

Sjogren’s Syndrome: Epidemiology of sexes

A

Higher prevalence in females

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16
Q

Sjogren’s Syndrome: Most common patient group

A

Middle aged women

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17
Q

Sjogren’s Syndrome: Can be secondary to what conditions? (2)

A

Rheumatoid Arthritis
SLE

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18
Q

Sjogren’s Syndrome: Pathophysiology

A

Immune system attacks the lacrimal and salivary glands

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19
Q

Sjogren’s Syndrome: 6 main symptoms

A

Dry eyes - feel gritty
Dry mouth - may lead to a fissured tongue
Dry throat
Vaginal dryness
Joint pains
Fatigue

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20
Q

Sjogren’s Syndrome: Signs - impact on the parathyroid gland

A

Parotid gland enlargement

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21
Q

Sjogren’s Syndrome: Oral signs

A

Increased dental caries

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22
Q

Sjogren’s Syndrome: Schirmers Test

A

Used to determine whether the eye produces enough tears to keep it moist - this will present with ocular dryness

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23
Q

Sjogren’s Syndrome: What would be present within the blood? (4)

A

Positive for Anti-Ro and Anti-La antibodies
Raised IgG
Raised plasma viscosity/ESR

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24
Q

Sjogren’s Syndrome: What is present on lip gland biopsy?

A

Lymphocytic infiltrates

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25
Sjogren's Syndrome: Management - Management for symptom control (3)
Tear replacement Salivary replacement Analgesia - for pain
26
Sjogren's Syndrome: Management - Main pharamaceutical used
Hydroxychloroquine - for arthralgia and fatigue
27
Sjogren's Syndrome: Complications (3)
Increased risk of lymphoma Peripheral neuropathy Interstitial lung disease
28
Systemic Sclerosis or Scleroderma
Systemic connective tissue autoimmune disease characterised by vasculopathy, autoimmunity and fibrosis
29
Systemic Sclerosis or Scleroderma: Two types
Diffuse Limited
30
Systemic Sclerosis or Scleroderma: Epidemiology of sexes
More common in women
31
Systemic Sclerosis or Scleroderma: Peak incidence
30-50 years old
32
Systemic Sclerosis or Scleroderma: Pathophysiology - Initiating Event
Etiologic agent combined with genetic predisposition
33
Systemic Sclerosis or Scleroderma: Pathophysiology - The initiating event induces activation of what? What does this activation cause?
Endothelial cells - causes leukocyte attraction and adhesion with tissue hypoxia
34
Systemic Sclerosis or Scleroderma: Pathophysiology - Myofibroblast formation occurs due to what?
Fibroblast, Fibrocyte and Endothelial cell activation
35
Systemic Sclerosis or Scleroderma: Diffuse SSc - Skin involvement occurs where?
Extremities - Above or below the elbows and knees + Face + Trunk
36
Systemic Sclerosis or Scleroderma: Diffuse SSc - Raynauds Presentation occurs when?
Coinciding with or following the onset of symptoms
37
Systemic Sclerosis or Scleroderma: Diffuse SSc - When does significant organ involvement occur?
Early
38
Systemic Sclerosis or Scleroderma: Diffuse SSc - What antibodies are associated with this? (2)
Anti-SCL-50 antibody Anti-RNA polymerase III
39
Systemic Sclerosis or Scleroderma: Limited SSc - Skin involvement occurs where?
Confined to the face, hands, forearms and feet
40
Systemic Sclerosis or Scleroderma: Limited SSc - When does Raynaud presentation occur?
Precedes the onset of other symptoms
41
Systemic Sclerosis or Scleroderma: Limited SSc - When does significant organ involvement occur?
Occurs later
42
Systemic Sclerosis or Scleroderma: Limited SSc - What antibody is associated?
Anti-centromere antibody
43
Systemic Sclerosis or Scleroderma: Clinical Presentation - Impacts on the face (4)
Small mouth with puckering Beaked nose Thickened and tight skin - lack of wrinkles Telangiectasia
44
Systemic Sclerosis or Scleroderma: Clinical Presentation - What is the triphasic phenomenon of Raynauds Disease? (3)
Blanching Acrocyanosis - purple or blue Reactive hyperaemia - redness
45
Systemic Sclerosis or Scleroderma: Clinical Presentation - Impact on the skin
Thickening
46
Systemic Sclerosis or Scleroderma: Clinical Presentation - Impact on the GIT (7)
Dysphagia - due to disruption of the vasculature around the oesophagus GORD Gastric Antravascular Ectasia (GAVE) Malabsorption Fluctuating bowel habit - constipation and diarrhoea Faecal incontinence
47
Systemic Sclerosis or Scleroderma: Clinical Presentation - Cardiopulmonary Impacts (3)
Interstitial lung disease Pulmonary arterial hypertension Myocardial Disease
48
Systemic Sclerosis or Scleroderma: Clinical Presentation - Renal impacts
Scleroderma renal crisis Non-specific progressive renal dysfunction
49
Systemic Sclerosis or Scleroderma: Clinical Presentation - Presentation of Scleroderma renal crisis
Uncontrolled hypertension with proteinuria and worsening renal function
50
Systemic Sclerosis or Scleroderma: Clinical Presentation - Scleroderma Renal Crisis associated with what? (2)
Associated with Anti-RNA Polymerase III Antibody High steroid dose
51
Systemic Sclerosis or Scleroderma: Clinical Presentation - Impact on the hands
Sclerodactyly - hardening of the skin of the hands to cause a claw like shape
52
Systemic Sclerosis or Scleroderma: Clinical Presentation of Diffuse SSc (5)
Joint contractures Skeletal myopathy Interstitial lung disease Myocardial involvement Renal crisis
53
Systemic Sclerosis or Scleroderma: Clinical Presentation of Limited SSc (40
Raynauds Disease - digital ischaemia Oesophageal disease Pulmonary hypertension Malabsorption
54
Systemic Sclerosis or Scleroderma: Red Flags of Clinical Presentation (5)
Onset of Raynaud's in mid-adulthood - with SOB and Telangiectasia Digital ulcers Ischaemia Skin tightness Loss of dexterity in the hands
55
Systemic Sclerosis or Scleroderma: What antibody is present in both types?
Anti-RNA Polymerase
56
Systemic Sclerosis or Scleroderma: What antibody is present in limited SSc?
Anti-centromere
57
Systemic Sclerosis or Scleroderma: What antibody is present in diffuse SSc?
Anti-topoisomerase (Anti-Scl-70)
58
Systemic Sclerosis or Scleroderma: Management steps of Raynaud's Disease (4)
1. Calcium Channel Blockers - Nifedipine first 2. PDE-5 Inhibitor - Sildenafil 3. Prostacyclin Infusion - Iloprost 4. Endothelin Receptor Antagonist - Bosentan
59
Systemic Sclerosis or Scleroderma: Pulmonary Hypertension Management - If not treated what will this lead to?
Right Heart Failure
60
Systemic Sclerosis or Scleroderma: Pulmonary Hypertension Management (4)
PDE-5 Inhibitor Endothelin Receptor Antagonists Eproprostenol infusions Oxygen
61
Systemic Sclerosis or Scleroderma: Pulmonary Hypertension Management - Examples of PDE-5 Inhibitors (30
Sildenafil Tadalafil Vardenafil
62
Systemic Sclerosis or Scleroderma: Pulmonary Hypertension Management - Examples of Endothelin Receptor Antagonists (3)
Bosentan Ambrisentan Macitentan
63
Systemic Sclerosis or Scleroderma: Stages of Pulmonary Fibrosis Management (4)
1. Mycophenolate mofetil - anti-inflammatory 2. Rituximab 3. Nintedanib - this is an anti-fibrotic 4. Cyclophosphamide - for aggressive disease
64
Systemic Sclerosis or Scleroderma: Renal Crisis Management (2)
ACE Inhibitors Dialysis for serious cases
65
Systemic Sclerosis or Scleroderma: Skin Fibrosis Management (2)
Methotrexate Mycophenolate
66
Spondyloarthrides: Epidemiology - Geographically more common when?
Northern hemisphere Scandinavian countries
67
Spondyloarthrides: Reactive Arthritis
Infection-induced systemic illness characterised primarily by an inflammatory synovitis from which viable microorganisms cannot be cultured
68
Spondyloarthrides: Reactive Arthritis - Most common preceding infections (2)
Urogenital Enterogenic
69
Spondyloarthrides: Reactive Arthritis - What genetic predisposition is present?
HLA-B27
70
Spondyloarthrides: Reactive Arthritis - Most common age group
20-40 years old
71
Spondyloarthrides: Reactive Arthritis - Causative organisms secondary to urogenital infection (2)
Chlamydia Neisseria
72
Spondyloarthrides: Reactive Arthritis - Causative organisms secondary to Enterogenic infection (3)
Salmonella Shigella Yersinia
73
Spondyloarthrides: Reactive Arthritis - Clinical Presentation - 1-4 weeks after infection - General symptoms (3)
Fever Fatigue Malaise
74
Spondyloarthrides: Reactive Arthritis - Clinical Presentation - 1-4 weeks after infection - Musculoskeletal presentations (2)
Asymmetrical monoarthritis or oligoarthritis Enthesitis
75
Spondyloarthrides: Reactive Arthritis - Clinical Presentation - 1-4 weeks after infection - Presentation of mucocutaneous lesions (4)
Keratodema Blenorrhagica Cicinate balanitis Painless oral ulcers Hyperkeratotic nails
76
Spondyloarthrides: Reactive Arthritis - Clinical Presentation - 1-4 weeks after infection - Ocular presentation (2)
Iritis Conjunctivitis
77
Spondyloarthrides: Reactive Arthritis - Clinical Presentation - 1-4 weeks after infection - Visceral manifestations (2)
Mild renal disease Carditis
78
Spondyloarthrides: Reactive Arthritis - Clinical Presentation of Reiters Syndrome (3)
Urethritis Conjunctivitis/Uveitis/Iritis Arthritis
79
Spondyloarthrides: Reactive Arthritis - Diagnosis - Blood results
Increased inflammatory markers
80
Spondyloarthrides: Reactive Arthritis - Diagnosis - What cultures are taken? (3)
Blood Urine Stool
81
Spondyloarthrides: Reactive Arthritis - Diagnosis - How is infection ruled out?
Joint fluid analysis
82
Spondyloarthrides: Reactive Arthritis - Management for most cases
90% of cases resolve spontaneously
83
Spondyloarthrides: Reactive Arthritis - Management for chronic progressive erosive disease
Immunosuppression
84
Spondyloarthrides: Enteropathic Arthritis
Inflammatory arthritis involving the peripheral joints and sometimes the spine in association with those with inflammatory bowel disease
85
Spondyloarthrides: Enteropathic Arthritis - Aetiology
Worsening of symptoms during flare ups of IBD
86
Spondyloarthrides: Enteropathic Arthritis - Pathophysiology
Organisms with high lipopolysaccharide in the cell wall trigger an immune reaction
87
Spondyloarthrides: Enteropathic Arthritis - Musculoskeletal presentation (2)
Arthritis in several joints Enthesitis
88
Spondyloarthrides: Enteropathic Arthritis - Most common joints affected (4)
Knees Ankles Elbows Wrists
89
Spondyloarthrides: Enteropathic Arthritis - Gastrointestinal symptoms (3)
Loose watery stools - with mucous and blood Weight loss Apthous oral ulcers
90
Spondyloarthrides: Enteropathic Arthritis - Impact on the eyes
Uveitis
91
Spondyloarthrides: Enteropathic Arthritis - Diagnosis - Blood results
Raises inflammatory markers - CRP and Plasma viscosity
92
Spondyloarthrides: Enteropathic Arthritis - Diagnosis - What is show on X-ray or MRI?
Sacroilitis
93
Spondyloarthrides: Enteropathic Arthritis - Diagnosis - What is shown on Ultrasound? (2)
Synovitis Tenosynovitis
94
Spondyloarthrides: Clinical Presentation - Two types of back pain
Mechanical Inflammatory
95
Spondyloarthrides: Characteristics of mechanical back pain
Worsened by activity Worse at the end of the day that recovers with rest
96
Spondyloarthrides: Characteristics of inflammatory back pain
Worsened by rest - early morning stiffness >30 minutes Improved with activity
97
Muscular Disease: 3 Types
Inflammatory Myopathies Polymyalgia Rheumatica Fibromyalgia
98
Muscular Disease: Inflammatory Myopathies is characterised by what?
Weakness
99
Muscular Disease: 3 Types of Inflammatory Myopathies
Polymyositis Dermatomyositis Immune Mediated Necrotising Myopathy (IMNM)
100
Muscular Disease: Polymyalgia Rheumatica characterised by what?
Pain and Stiffness
101
Muscular Disease: Fibromyalgia characterised by what?
Pain and Fatigue
102
Myopathy
Disease of the muscle in which muscle fibres do not function properly resulting in muscular weakness
103
Polymyositis: Epidemiology of the sexes
More common in females - twice as common
104
Polymyositis: Peak incidence
40-50 years
105
Polymyositis: Increased incidence in what condition?
Malignancy
106
Polymyositis: General presentation
Symmetrical muscle weakness in the proximal muscles
107
Polymyositis: Onset of inflammatory myopathies
Insidious onset that worsens with time
108
Polymyositis: Clinical Presentation - Lungs (2)
Inflammatory lung disease Respiratory muscle weakness
109
Polymyositis: Clinical Presentation -Oesophageal symptom
Dysphagia
110
Polymyositis: Clinical Presentation -Cardiac symptom
Myocarditis
111
Polymyositis: Clinical Presentation -General Systemic Symptoms (4)
Fever Weight loss Raynauds Disease Inflammatory arthritis
112
Polymyositis: Clinical Presentation - Presentation in the hands
Dryness
113
Polymyositis: Dermatomyositis
Inflammatory disorder of the skin and underlying tissue
114
Polymyositis: Dermatomyositis - Gottron's Papules
Pink or purple eruptions on the face, scalp, neck, shoulders and knuckles followed by swelling
115
Polymyositis: Dermatomyositis - Heliotrope Rash
Red or purple rash of the eyelids
116
Polymyositis: Dermatomyositis - 3 signs
Gottron's Papules Heliotrope Rash Shawl Sign
117
Polymyositis: Dermatomyositis - Shawl Sign
Widespread flat red area on the upper back, shoulders and back of the neck
118
Polymyositis: Clinical Examination - Two types of clinical examinations
Confrontational Testing Isotonic Testing
119
Polymyositis: Clinical Examination -Confrontational testing
Direct testing of power by pushing and pulling of muscles
120
Polymyositis: Clinical Examination -Isotonic testing
30 second sit to stand test that is repeated
121
Polymyositis: Main diagnostic test
Muscle Biopsy
122
Polymyositis: What does a muscle biopsy show?
Perivascular inflammation with muscular necrosis
123
Polymyositis: What is shown on electromyopathy? (3)
Increased fibrillations Abnormal motor potentials Complex repetitive discharges
124
Polymyositis: MRI may show what 4 characteristics?
Muscle inflammation Oedema Fibrosis Calcification
125
Polymyositis: Management options (3)
Corticosteroids Immunosuppression Biologics - IV Ig or Rituximab
126
Polymyositis: Complication
Increased risk of malignancy - Ovarian, Breast, Stomach, Lung, Bladder or Colon Cancer
127
Polymyositis: Risk of malignancy is greatest in what sex?
Men
128
Polymyositis
Idiopathic inflammatory myopathy causing symmetrical proximal muscle weakness
129
Polymyositis: Pathophysiology
CD8+ T cell mediated process against non-necrotic muscle fibres in response to nuclear and cytoplasmic autoantigens
130
Polymyositis: Those with interstitial lung disease may test positive for what antibody?
Anti-Jo-1 antibody
131
Polymyositis: Non-specific auto-antibodies
ANA Anti-RNP
132
Polymyositis: Myositis specific auto-antibodies
Anti-Jo-1 Anti-SRP
133
Polymyalgia Rheumatica
Inflammatory condition of unknown aetiology that affects elderly individuals
134
Polymyalgia Rheumatica: Epidemiology of age
Patients over 50 years
135
Polymyalgia Rheumatica: Geographical epidemiology
Higher incidence in northern regions
136
Polymyalgia Rheumatica: Associated with what condition?
Giant Cell Arteritis
137
Polymyalgia Rheumatica: Systemic Symptoms (4)
Fatigue Anorexia Weight loss Fever
138
Polymyalgia Rheumatica: Main symptom
Proximal myalgia of the hip and shoulder girdles
139
Polymyalgia Rheumatica: Onset of shoulder or hip pain
Morning stiffness - lasts for at least 45 minutes
140
Polymyalgia Rheumatica: Is pain improved or worsened with movement?
Worsened
141
Polymyalgia Rheumatica: Is muscle strength impacted?
No
142
Polymyalgia Rheumatica: Impact on the nerves
Carpal tunnel syndrome
143
Polymyalgia Rheumatica: Diagnosis relies on what?
Clinical diagnosis
144
Polymyalgia Rheumatica: Blood results
Increased inflammatory markers - increased ESR, Plasma viscosity and CRP
145
Polymyalgia Rheumatica: Temporal or Giant Cell Arteritis
Granulomatous arteritis of large vessels e.g. in the temporal arteries
146
Polymyalgia Rheumatica: Temporal or Giant Cell Arteritis - Symptoms (4)
Headache Scalp tenderness Jaw claudication Amaurosis fugax - visual loss Tender enlarged non-pulsatile temporal arteries
147
Polymyalgia Rheumatica: Main principle
There is a rapid response to low dose steroids - the dose should then be gradually reduced over 18-24 months
148
Polymyalgia Rheumatica: Management - Polymyalgia Rheumatitis
Start prednisolone at 15mg daily
149
Polymyalgia Rheumatica: Management - Giant Cell Arteritis
Start prednisolone at 40-60mg daily
150
Fibromyalgia
Neurosensory disorder characterised by chronic musculoskeletal pain
151
Fibromyalgia: Epidemiology of sexes
Increased prevalence in women
152
Fibromyalgia: What lifestyle factors may initiate this? (2)
Emotional or physical trauma
153
Fibromyalgia: May occur secondary to what? (2)
Rheumatoid Arthritis SLE
154
Fibromyalgia: Has what impacts on the nervous pathways? (3)
Increased cutaneous nociception Increased sympathetic outflow Increased muscle nociception
155
Fibromyalgia: Patients tend to have a reduction in what?
Threshold to sensations - pain/heat/noise/strong odours
156
Fibromyalgia: Length of time
>3 months of widespread pain
157
Fibromyalgia: Where is pain felt?
Symmetrically above and below the waist and at the axial spine
158
Fibromyalgia: Central symptoms (5)
Chronic headaches Dizziness Sleep disorders Cognitive impairment Anxiety/Depression
159
Fibromyalgia: Msucular symptoms (3)
Myofascial pain Fatigue Twitches
160
Fibromyalgia: Systemic symptoms (3)
Pain Weight gain Cold symptoms
161
Fibromyalgia: Symptom of the urinary tract
Problems urinating
162
Fibromyalgia: Symptom. ofthe reproductive tract
Dysmenorrhoea
163
Fibromyalgia: Diagnosis criteria (4)
Patient experiences widespread pain with unrefreshed sleep, cognitive symptoms and fatigue that have been present at the same level for >3 months
164
Fibromyalgia: Management - Examples of analgesia (2)
Gabapentin Pregabalin
165
Fibromyalgia: Management - Options (3)
Graded exercise programme Cognitive behavioural therapy Anti-depressants
166
Vasculitis
Inflammation of blood vessels
167
Vasculitis: Primary vasculitis
The result of an inflammatory response that targets the vessel wall with no known cause
168
Vasculitis: Secondary vasculitis
Vasculitis that occurs as a result of infection, drugs, toxin, cancer or as part of another inflammatory disorder
169
Vasculitis: Can lead to what 3 pathological states?
Inflammation - can block the blood vessel Ischaemia Necrosis of tissue
170
Vasculitis: Classifications - Large vessel vasculitis (2)
Takayasu arteritis Giant cell arteritis
171
Vasculitis: Classifications - Medium vessel vasculitis (2)
Polyarteritis nodosa Kawasaki disease
172
Vasculitis: Classifications - ANCA-associated small vessel vasculitis (3)
Microscopic polyangiitis Granulomatosis with polyangitis (Wegner Syndrome) Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)
173
Vasculitis: Classifications - Immune complex mediated small vessel vasculitis (3)
Cryoglobulinaemic vasculitis IgA Vasculitis (Henoch-Schonlein Syndrome) Hypocomplementeric Urticarial Vasculitis
174
Vasculitis: Systemic symptoms (4)
Fever Malaise Weight Loss Fatigue
175
Large Vessel Vasculitis
Primary vasculitis that causes chronic granulomatous inflammation - mainly of the aorta and its major branches
176
Large Vessel Vasculitis: Two main types
Temporal giant cell arteritis Takayasu Arteritis
177
Large Vessel Vasculitis: Takayasu Arteritis - Main age of incidence
<40 years old
178
Large Vessel Vasculitis: Takayasu Arteritis - Epidemiology of sexes
More common in females
179
Large Vessel Vasculitis: Takayasu Arteritis - More prevalent in what population?
Asian
180
Large Vessel Vasculitis: Takayasu Arteritis - Early features (6)
Low grade fever Malaise Night sweats Weight loss Arthralgia Fatigue
181
Large Vessel Vasculitis: Takayasu Arteritis - What is the main feature of this?
Claudication in the upper and lower limbs
182
Large Vessel Vasculitis: Takayasu Arteritis - If untreated what can occur? (2)
Vascular stenosis Aneurysms
183
Large Vessel Vasculitis: Takayasu Arteritis - Bruit is most common where?
In the carotid arteries
184
Large Vessel Vasculitis: Takayasu Arteritis - Blood results
Increased inflammatory markers
185
Large Vessel Vasculitis: Takayasu Arteritis - What imaging is used?
MR Angiogram - detects thickened vessel walls and stenosis
186
Large Vessel Vasculitis: Giant Cell Arteritis - Most common age
50-60 years old
187
Large Vessel Vasculitis: Giant Cell Arteritis - Strong association with what?
Polymyalgia rheumatica
188
Large Vessel Vasculitis: Giant Cell Arteritis - Central symptom
Unilateral acute temporal headache with focal tenderness on direct palpation
189
Large Vessel Vasculitis: Giant Cell Arteritis - Symptoms in the head (3)
Jaw claudication - due to ischaemia of the maxillary artery Visual disturbances Visual loss - amaurosis fugax
190
Large Vessel Vasculitis: Giant Cell Arteritis - When should this diagnosis be considered?
Differential diagnosis of a new-onset headache in 50 year old patients or older with an elevated ESR, CRP or plasma viscosity
191
Large Vessel Vasculitis: Giant Cell Arteritis - First line test
Temporal Artery US
192
Large Vessel Vasculitis: Giant Cell Arteritis - Gold standard test
Temporal artery biopsy
193
Large Vessel Vasculitis: Giant Cell Arteritis - Why may a temporal artery biopsy be negative?
Due to skip lesions
194
Large Vessel Vasculitis: Giant Cell Arteritis - Presentation on biopsy (3)
Transmural inflammation of all 3 layers Patchy infiltration by lymphocytes, macrophages and multinucleated giant cells Vessel wall thickening causing distal ischaemia
195
Large Vessel Vasculitis: Management - First step
Prednisolone 40-60 mg
196
Large Vessel Vasculitis: Management - Long term for Takayasu Arteritis
Steroid sparing agents - Leflunamide or Methotrexate
197
Large Vessel Vasculitis: Management - Long term for Giant Cell Arteritis
Gradual reduction in steroid dose over 18-24 months
198
Large Vessel Vasculitis: Management - For resistant or relapsing GCA
Toclizumab
199
ANCA-associated small vessel vasculitis: Aetiology - Causes (3)
S. aureus E. coli Klebsiella pneumoniae
200
ANCA-associated small vessel vasculitis: Aetiology - Environmental factors
Silica
201
ANCA-associated small vessel vasculitis: Aetiology - Drugs (4)
Propylthiouracil Hydralazine D-penicillamine Minocycline
202
ANCA-associated small vessel vasculitis: 3 types
EGPA - Eosinophilic Granulomatosis with Polyangiitis GPA - Granulomatosis with Polyangiitis MPA - Microscopic Polyangiitis
203
ANCA-associated small vessel vasculitis: EGPA triad
Late onset asthma Eosinophilia Granuloma
204
ANCA-associated small vessel vasculitis: Granulomatosis with Polyangiitis
Granulomatous inflammation affecting the small and medium sized vessels in the upper and lower respiratory tract, eyes or kidneys
205
ANCA-associated small vessel vasculitis: Granulomatosis with Polyangiitis - More common in what countries?
Northern Europe
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ANCA-associated small vessel vasculitis: Granulomatosis with Polyangiitis - Epidemiology of sexes
More common in males
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ANCA-associated small vessel vasculitis: Granulomatosis with Polyangiitis - Typical age on diagnosis
35-55 years
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ANCA-associated small vessel vasculitis: Granulomatosis with Polyangiitis - ENT features (6)
Sinusitis Saddle nose -due to cartilage damage from ischaemia Nose bleeds Oral ulcers Deafness Sub-glottic inflammation
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ANCA-associated small vessel vasculitis: Granulomatosis with Polyangiitis - Respiratory Symptoms (4)
Haemoptysis Pulmonary infiltrates Diffuse alveolar haemorrhage Cavitating nodules on CXR
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ANCA-associated small vessel vasculitis: Granulomatosis with Polyangiitis - Ocular features (6)
Conjunctivitis Episcleritis Uveitis Optic nerve vasculitis Retinal artery occlusion Proptosis - bulging of one or both eyes
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ANCA-associated small vessel vasculitis: Granulomatosis with Polyangiitis - Cutaneous features (2)
Palpable purpura Cutaneous ulcers
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ANCA-associated small vessel vasculitis: Granulomatosis with Polyangiitis - Renal feature
Necrotising glomerulonephritis
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ANCA-associated small vessel vasculitis: Granulomatosis with Polyangiitis - Nervous features (3)
Mononeuritis multiplex Sensorimotor polyneuropathy Cranial nerve plexus
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ANCA-associated small vessel vasculitis: Blood results (3)
C3 and C4 initially increased - they then fall due to destruction in active disease ESR/PV/CRP increased Anaemia is common
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ANCA-associated small vessel vasculitis: Eosinophilic Granulomatosis with Polyangiitis - Affects what blood vessels?
Small and medium sized vessels
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ANCA-associated small vessel vasculitis: Eosinophilic Granulomatosis with Polyangiitis - Affects what systems of the body?
Respiratory Tract Skin
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ANCA-associated small vessel vasculitis: Microscopic Polyangiitis
Necrotising vasculitis of the small vessels with few immune deposits
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ANCA-associated small vessel vasculitis: Microscopic Polyangiitis - Typical location (3)
Pulmonary system Renal system Skin
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ANCA-associated small vessel vasculitis: Microscopic Polyangiitis - What is a common clinical presentation?
Necrotising glomerulonephritis
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ANCA-associated small vessel vasculitis: Diagnosis - Autoantibodies - ANCAs
Anti-neutrophil cytoplasmic antibodies
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ANCA-associated small vessel vasculitis: Diagnosis - Autoantibodies - What do ANCAs do?
Auto-antibodies against antigens in the cytoplasm of neutrophil granulocytes
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ANCA-associated small vessel vasculitis: Diagnosis - Autoantibodies - What ANCA is present in GPA?
cANCA
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ANCA-associated small vessel vasculitis: Diagnosis - Autoantibodies - What ANCA is present in EGPA?
pANCA
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ANCA-associated small vessel vasculitis: Diagnosis - Autoantibodies - What ANCA is present in MPA?
pANCA
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ANCA-associated small vessel vasculitis: Diagnosis - Autoantibodies - PR3 present most commonly in what type?
GPA
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ANCA-associated small vessel vasculitis: Diagnosis - Autoantibodies - MPO most common in what types?
EGPA MPA
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ANCA-associated small vessel vasculitis: Management - Localised disease definition
Upper and/or lower respiratory tract disease without any other systemic involvement or constitutional symptoms
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ANCA-associated small vessel vasculitis: Management - Early systemic definition
Any systemic involvement without any organ or life threatening circumstances
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ANCA-associated small vessel vasculitis: Management - Generalised definition
Renal (Creatinine <500) or other organ threat
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ANCA-associated small vessel vasculitis: Management - Systemic definition
Renal (Creatinine >500) or other vital organ failiure
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ANCA-associated small vessel vasculitis: Management - Refractory disease definition
Progressive disease that is unresponsive to steroids and cyclophosphamide
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ANCA-associated small vessel vasculitis: Management - Localised or Early systemic disease
Methotrexate + Steroids
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ANCA-associated small vessel vasculitis: Management - Second line amanagement for localised or early systemic disease
Azathioprine + steroids
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ANCA-associated small vessel vasculitis: Management - First line management for generalised disease
Cyclophosphoamide + Steroids followed by azathioprine for maintenance
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ANCA-associated small vessel vasculitis: Management - Second line management for generalised disease
Rituximab + Steroids
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ANCA-associated small vessel vasculitis: Management - First line management for systemic disease
Cyclophosphamide + Steroids followed by azathioprine for maintenance
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ANCA-associated small vessel vasculitis: Management - Second line management for Systemic disease
Rituximab + Steroids
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ANCA-associated small vessel vasculitis: Management - Management for systemic disease with creatinine > 500
Plasma exchange
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ANCA-associated small vessel vasculitis: Management - Refractory Disease
IV immunoglobulins Rituximab
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Non-ANCA-associated small vessel vasculitis: Two types
Henoch-Schlein Purpura Cryoglobulinaemia
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Non-ANCA-associated small vessel vasculitis: Henoch-Schlein Purpura
Acute immunoglobulin A mediated disorder with generalised vasculitis involving the small vessels of the skin, GIT, Kidneys, Joints and CNS
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Non-ANCA-associated small vessel vasculitis: Cryoglobulinaemia
Increased serum cryoglobulin with no dominant IgA deposits
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Non-ANCA-associated small vessel vasculitis: Henoch-Schlein Purpura - Peak age of incidence
2-11 years
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Non-ANCA-associated small vessel vasculitis: Henoch-Schlein Purpura - Most patients have what prior to disease? (3)
Preceding illness 1-3 weeks before - URTI, Pharyngeal infection or GI infection
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Non-ANCA-associated small vessel vasculitis: Henoch-Schlein Purpura - Most common associated organism
Group A streptococcus
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Non-ANCA-associated small vessel vasculitis: Henoch-Schlein Purpura - GI presentation (2)
Colicky abdominal pain Blood diarrhoea
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Non-ANCA-associated small vessel vasculitis: Henoch-Schlein Purpura - Skin manifestation
Purpuric rash over the gluteal region and lower limbs
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Non-ANCA-associated small vessel vasculitis: Henoch-Schlein Purpura - In 50% of cases what system is involved?
Renal
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Non-ANCA-associated small vessel vasculitis: Henoch-Schlein Purpura - Management
Self-limiting - resolves in 8 weeks
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Non-ANCA-associated small vessel vasculitis: Henoch-Schlein Purpura - What must be screened?
Urinalysis to screen for renal involvement
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Paediatric Orthopaedic Disorders
A persisting qualitative motor disorder appearing before the age of three years due to non-progressive damage to the encephalon occuring before growth of the CNS is complete
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Paediatric Orthopaedic Disorders: Pathophysiology of Muscle weakness
1. Pathology leads to loss of connections to the lower motor neurones 2. Negative features of upper motor neurone syndrome
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Paediatric Orthopaedic Disorders: Symptoms. ofmuscle weakness pathologies (4)
Weakness Fatifuability Poor balance Sensory deficits
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Paediatric Orthopaedic Disorders: Mechanical mechanism of Muscle weakness
1. Induces muscle shortening 2. Causes bony torsion and joint instability 3. Degenerative arthritis
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Paediatric Orthopaedic Disorders: Pathophysiology of muscle spasticity
1. CNS pathology leads to loss of inhibition of lower motor neurones 2. Positive features of upper motor syndrome
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Paediatric Orthopaedic Disorders: Symptoms of muscle spasticity (4)
Spasticity Hyperreflexia Clonus - involuntary muscle contractions Co-contraction
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Paediatric Orthopaedic Disorders: Neural mechanism of muscle spasticity
1. Induces muscle shortening 2. Induces bony torsion and joint instability 3. Degenerative arthritis
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Ambulation
The ability to walk without the need for any kind of assistance
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Congenital Talipes Equinovarus
Developmental disorder of the lower limb defined as fixation of the foot in adduction, suppination. andvarus position
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Genu Varum
Bow legged structure - the legs curve outward at the knees whilst the feet and ankles touch
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Genu Varum: When is this normal?
Those under the age of 2
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Genu Varum: May be considered abnormal if what is present? (4)
Unilateral Severe Short stature Painful
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Genu Varum: 5 associated pathophysiologies
Skeletal dysplasia Rickets Enchondroma Blounts Disease Trauma - may cause physeal injury
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Genu Varum: Blounts Disease
Growth arrest of the medial tibial physis of unknown aetiology
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Genu Valgum
Legs touch at the knees whilst their feet and ankles are spread apart
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Genu Valgum: When is this normal?
Peak age at 3.5 years
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Genu Valgum: 3 causative pathologies
Tumours - Enchondroma or Osteochondroma Rickets Neurofibromatosis
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Genu Valgum: Refer if (4)
Asymmetric Painful Severe - greater than 2 Standard deviations over normal >8cm intermalleolar distance at age 11
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In-toeing
Refers to a child who, when walking and standing, will have feet that point towards the midline
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In-toeing: May be releated to what 3 pathophysiologies?
Femoral neck anteversion Internal tibial torsion Metatarsus adductus
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In-toeing: Often exaggerated by what?
Running
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In-toeing: Femoral Neck Anteversion - Normal physiology of the femur
Normally points slightly anteriorly to increase the internal rotation of the hip
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In-toeing: Femoral Neck Anteversion - Normal angle of femur at birth
30-40 degrees
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In-toeing: Femoral Neck Anteversion - Normal angle of femur at maturity
10-15 degrees
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In-toeing: Internal Tibial Torsion is usually seen when?
1-3 years old - resolved by 6 years
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In-toeing: Metatarsus Adductus - management if not passively corrected
Serial casting between 6 to 12 months
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Curly Toes
Minor overlapping of the toes
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Curly Toes: Most common toes affected?
3rd or 4th toes
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DDH
Developmental Dysplasia
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Developmental Dysplasia
Dislocation or subluxation of the femoral head during the perinatal period that affects subsequent development of the hip joint
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Developmental Dysplasia: Epidemiology of sexes
More common in women
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Developmental Dysplasia: What hip. ismost commonly affected?
Left hip
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Developmental Dysplasia: High in what race?
Native americans
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Developmental Dysplasia: Low in what race?
African
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Developmental Dysplasia: What hormone increase the risk of this?
Relaxin - only acts on females so increased risk in women
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Developmental Dysplasia: Risk Factors (4)
Breech birth Oligohydramnios - larger children First born >4kg Moulded baby - e.g. twins
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Developmental Dysplasia: What screening is conducted for this?
Neonatal baby checks Selective US Screening - conducted on high risk births 6-8 week GP check
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Developmental Dysplasia: What patients are screened by US in Scotland?
Breech births 1st Degree family history Moulded babies
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Developmental Dysplasia: Baby Hip Examination - what is inspected?
Asymmetry Loss of knee height Crease of asymmetry Less abduction in flexion
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Developmental Dysplasia: Barlows Test - what is a positive test?
Adduct the hip whilst applying light pressure on the knee with your thumb to direct the force posteriorly If the hip is unstable - palpable subluxation or dislocation
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Developmental Dysplasia: Ortolani's Test - what is a positive test?
Flex the hips and knees of a supine patient to 90 degrees - with your index fingers place anterior pressure on the greater trochanter and gently abduct the infants legs If there is a distinctive clunk heard. andfelt as the femoral head relocates anteriorly into the acetabulum - this is a positive test
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Developmental Dysplasia: Gold standard investigation
Ultrasound
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Developmental Dysplasia: When is US most helpful and why?
Under 3 months of age as the ossification nucleus begins to develop after 3 months
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Developmental Dysplasia: When are X-Rays useful and why?
Most useful after 4-6 months of age - as before this the femoral head epiphysis is unossified
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Developmental Dysplasia: Management for early DDH
Pavlik Harness 23-24 hours per day for up to 12 weeks until the Ultrasound is normal
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Developmental Dysplasia: Pavlik Harness description
Harness holding the hips in an abducted and flexed position
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Developmental Dysplasia: Management of Late DDH
Surgery -Closed Reduction spica or Open reduction spica
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Developmental Dysplasia: Option for children with persistent dislocation over 18 months old
Open reduction with an osteotomy
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SUFE
Slipped Upper Femoral Epiphysis
300
Slipped Upper Femoral Epiphysis
Condition mainly affecting overweight pre-pubertal adolescent boys where the femoral head epiphysis slips inferiorly in relation to the femoral neck
301
Slipped Upper Femoral Epiphysis: Peak incidence age
8-18 years old
302
Slipped Upper Femoral Epiphysis: Risk Factors (4)
Pubertal growth Endocrine or Metabolic dysfunction - e.g. Pituitary or Thyroid dysfunction Renal osteodystrophy Increased weight
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Slipped Upper Femoral Epiphysis: Pathophysiology Mechanism
The growth plate physis is not strong enough to support the body weight and the femoral epiphysis slips due to the strain
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Slipped Upper Femoral Epiphysis: What ay prevent onset?
Growth spurt
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Slipped Upper Femoral Epiphysis: Main symptom
Hip, groin, thigh or knee pain with a limp
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Slipped Upper Femoral Epiphysis: How can some patients present with just pain in the knee?
Due to the obturator nerve supplying the hip and knee joint
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Slipped Upper Femoral Epiphysis: 2 clinical signs
Antalgic gait Loss of internal rotation of the hip
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Slipped Upper Femoral Epiphysis: Impact on the lower limb (3)
Short Loss of internal rotation Loss of deep flexion
309
Slipped Upper Femoral Epiphysis: What diagnostic test is conducted?
X-Ray in lateral view to ensure mild degrees of slip are detected
310
Slipped Upper Femoral Epiphysis: Management
Urgent surgery to pin the femoral head
311
Slipped Upper Femoral Epiphysis: Risk of managing severe acute slip with gentle manipulation
Avascular necrosis
312
Slipped Upper Femoral Epiphysis: Chronic severe slios may require what management?
Osteotomy