MSK and rheumatology + second half of liver Flashcards
(284 cards)
1
Q
What is the function of articular cartilage?
A
- reduce friction
-shock absorption
2
Q
What is the function of synovial membrane?
A
- highly vascularised
-secretes and absorbs synovial fluid
3
Q
What is the function of synovial fluid?
A
- lubrication
-shock absorption - nutrient distribution ; hyaline cartilage is avascular and relies on diffusion from SF
4
Q
What are the inflammatory markers?
A
- ESR
-CRP
-Auto-antibodies
-RF
-ANA
5
Q
What is ESR?
A
Erythrocyte sedimentation rate
-rises with inflammation/infection
-rises and falls slowly
6
Q
What is CRP?
A
C-reactive protein
-acute phase protein
-released in inflammation/ infection
-produced by liver in response to IL-6 (pro- inflammatory cytokine)
-rises and falls rapidly
7
Q
What are auto-antibodies?
A
Immunoglobulins that bind to self antigens
8
Q
What is rheumatoid factor?
A
An auto-antibody found in people with rheumatoid arthritis
9
Q
What is anti - ccp?
A
Anti-citrullinated protein antibodies are autoantibodies that are directed against peptides and proteins that are citrullinated. They are present in the majority of patients with rheumatoid arthritis.
10
Q
What is ANA?
A
Anti-nuclear antibody - binds to antigens within cell nuclear
11
Q
What is osteoarthritis?
A
-most common type of arthritis
-Non inflammatory degenerative joint disease
where the cartilage within a joint begins to break down and the underlying bone begins to change.
12
Q
What are the risk factors of osteoarthritis?
A
- 50+
- affects women 50+ more
-obesity
-occupation/sports
-genetic (COL2A1 - genetic predisposition)
13
Q
What is the pathology of osteoarthritis?
A
-Imbalance between cartilage breakdown and repair
-Increase in chondrocytes metalloproteinase secretion
-Degrades T2 collagen and causes cysts
-Bone attempts to overcome this with type 1 collagen -abnormal bony growth (osteophytes) +remodelling
14
Q
What is the presentation of osteoarthritis?
A
-Transient (little <30min) morning pain –> worse as day goes on
-Bouchard + Hebderen nodes on fingers
-assymetrical, hard non inflamed joint
-typically most stressed joints in body - hip/knee/base of toe/thumb
-no extra articular Sx
15
Q
Where are the hebderen nodes?
A
distal interphalangeal joint
16
Q
Where are the Bouchard nodes?
A
Proximal interphalangeal joint
17
Q
What is the diagnosis for osteoarthritis?
A
XR - LOSS
-loss of joint space
-osteophytes
-subchondral sclerosis
-sunchondral cysts
- normal bloods
18
Q
What is the treatment of osteoarthritis?
A
- lifestyle change - weight bearing , physio
-NSAID pain relief
-Last resort -consider surgery (arthroplasty - for knee+hip replacement)
19
Q
What is rheumatoid arthritis ?
A
Autoimmune inflammatory polyarthritis
- symmetrical
20
Q
What are the risk factors of rheumatoid arthritis?
A
- women 30-50
(3x more likely than M premenopausally ) - smoking
-HLADR4/HLADRB1 - genetic link - after menopause M=F
21
Q
What is the pathology of RA?
A
- arginine -> citrulline mutation in T2 collagen; anti-ccp formation
-IFN-a cause further pro inflammatory recruitment to synovial - synovial lining expands and tumour like mass (pannus) grows past joint margins
-pannus destroys subchondral bone + articular cartilage
22
Q
What is the presentation of RA?
A
- often worse in the morning >30min, eases as day goes on
-Hand; swan neck, z thumb, ulnar finger deviation
-bakers cyst - popliteal synovial sac bulge - symmetrical hot inflamed joints
-mc in wrist/hand and feet
-extra articular - lungs (PE), heart (increased IHD), eyes(dry eyes), spinal cord compression, CKD, rheumatoid skin nodules
23
Q
What is the diagnosis of RA?
A
Bloods = Increased ECR +CRP
normocytic normochromic anaemia - mc from chronic disease, also can cause - microcytic (NSAID use->PUD->FE def anaemia) and macrocytic from methotrexate use -inhibits folate
Serology = positive anti-ccp, positive RF
XR-LESS
-loss of joint space
-erosions
-soft tissue swelling
-soft bones
24
Q
What is the treatment for rheumatoid arthritis?
A
DMARD- Methotrexate - GS treatment
NSAID - analgesia
-Intra articular steroid injection if very painful
-Biologics (good but expensive)
1st line- TNF -d-inhibitor - infliximab
2nd line- B cell inhibitor- rituximab
25
What is a DMARD?
Disease modifying anti rheumatic drugs
26
When is methotrexate contraindicated ?
- Pregnant as folate inhibitor therefore DNA synthesis affected
27
What are crystalline arthropathies?
a group of joint disorders caused by deposits of crystals in joints and the soft tissues around them.
28
What are the two main types of crystals that account for the majority of crystal induced arthritis?
- sodium urate crystals
-calcium pyrophosphate crystals
29
What are sodium urate crystals?
- needle shaped (long and thin) crate crystals
-negatively birefringent under polarised light
30
What are calcium pyrophosphate crystals?
- small rhomboid brick shaped pyrophosphate crystals
-positively birefringent under polarised light
31
What is Gout?
Inflammatory arthritis associated with high levels of uric acid and intra -articular monosodium rate crystals deposited in joints
32
What is the epidemiology of gout?
- middle aged overweight men
33
What are the risk factors of gout?
- purine rich foods (meat, seafood, beer)
-CKD + diuretics
34
What is the pathology of gout?
purines break down into uric acid by xanthate oxidase - uric acid either excreted by kidneys or --> monosodium urate by binding with sodium ions
- increase in uric acid/CKD = impaired excretion therefore increase in monosodium rate
35
What is the presentation of gout?
Monoarticular; usually big toe (metatarsophalangeal joint) - sudden onset, severe swollen red toe, can't put weight on it
DDX= septic arthritis
36
\What is the diagnosis of gout?
Joint aspirate + polarised light microscopy; negatively birefringent needle shaped crystals -GS
37
What is the treatment of Gout?
Diet = low purines and high dairy
NSAIDs, then consider colchicine, then steroid injection - acute gout
Prevention = allopurinol (xanthine oxidase inhibitor to decrease uric acid production)
38
What is pseudo gout?
Calcium pyrophosphate crystals deposit along joint capsule
39
What is the epidemiology of pseudo gout?
elderly females / 70+
40
What are the risk factors for pseudo gout?
diabetes, metabolic disease , osteoarthritis
41
What is the presentation of pseudo gout?
often poly-articular with knee commonly involved - swollen hot red joint
42
What is the diagnosis of pseudo gout?
joint aspiration and polarised light microscopy --> positively birefringent, rhomboid shaped crystals
43
What is the treatment for pseudo gout?
only acute management:
-NSAIDs then colchicine, then steroid injection
- no prevention drug
44
What is osteoporosis?
A systemic skeletal disease characterised by low bone density by 2.5+ standard deviations below young adult mean volume (T<2.5)
45
What is the epidemiology of osteoporosis?
50+ postmenopausal caucasian women
46
What are the risk factors of osteoporosis ?
SHATTERED
S-teroids
h-yperthyroid/ parathyroid
a-lcohol+ smoking
t-hin
t-estosterone low
e-arly menopause - low oestrogen
r-enal/liver failure
e-rosive+ inflammatory disease
d-MT1 or malabsorption
47
What is the presentation of osteoporosis?
only fractures -
-proximal femur
-colle's -fractured wrist
-compression vertebral crush
48
What is the diagnosis of osteoporosis?
DEXA scan - GS
- dual energy XR absorptiometry
- yields T-score - compares patient BMD to reference
-FRAX score - fracture risk assessment tool - assess fracture risk in osteoporotic patients
49
What is T score?
Young adult bone density :
0
50
What is osteopenia?
- precursor to osteoporosis
- defined as bone density 1-2.5 standard deviations below normal young adult mean value
51
What is the treatment for osteoporosis?
1- Bisphosphonates (alendronate inhibit RANK-L signal therefore osteoclastic inhibitors)
2- mAB Denosumab (inhibits RANKL)
HRT - oestrogen /testosterone
Oestrogen receptor modulated - raloxifene
Recombinant PTH
52
What is systemic lupus erythematous?
Hypersensitivity T3 reaction (Antigen-antibody complex deposition)
-Autoimmune systemic inflammation
53
What is the epidemiology of SLE?
- females
-african carribean
-20-40
-premenopause
54
What are the risk factors for SLE?
- female - 12x more than men
-HLAB8/DR2/DR3
-Drugs -isoniazid
55
What is the pathology of SLE?
impaired apoptotic debris presented to TH2--> B cell activation --> antigen-antibody complexes
56
What is the presentation of SLE?
- butterfly rash+photosensitivity
-glomerulonephritis(nephritic syndrome)
--seizures
-mouth ulcers
-joint pain
-raynaud's
-serositis
57
What is the diagnosis of SLE?
Bloods - anaemia, high ESR + normal CRP
Urine dipstick - hameaturia + proteinuria
Serology : ANA Ab's - 99% cases
Anti ds DNA Ab's - used to monitor progression
-Low c3 + c4
58
What is the treatment for SLE?
Lifestyle (low sunlight, stop triggering drugs)
-corticosteroids
-hydroxychloroquine
-NSAIDs
59
What is antiphospholipid syndrome?
Associated with antiphospholipid antibodies where blood becomes prone to clotting. Can be primary, idiopathic or secondary to other disease, like SLE.
Characterised by thrombosis, recurrent miscarriages and apl Ab's
60
Who does Antiphospholipid syndrome effect more?
females
61
What is the presentation of antiphospholipid syndrome?
CLOTS:
Coagulopathy
lived reticular - purple discolouration of skin
obstetric issues - miscarriage
thrombocytopenia
-high risk of stroke, MI, DVT
62
What is the diagnosis of aPL syndrome?
- lupus anticoagulant
-anticardiolipin antibodies
-anti B2 glycoprotein-1 antibodies
63
What is the treatment of aPL syndrome?
1st line - warfarin long term if have had a thrombosis
- if pregnant give aspirin +heparin
prophylaxis = not had thrombosis = aspirin
64
What is sjorgen syndrome?
Autoimmune destruction of exocrine glands, especially the lacrimal and salivary glands
65
What is the epidemiology of sjorgen's?
-females 40-50
-FHx
-HLAB8/DR3
-can be primary or secondary to other autoimmune diseases - SLE
66
What is the presentation of sjorgen's?
Dry eyes
-Xerstomia - mouth/ vaginal dryness
67
What is the diagnosis of sjorgen's?
serology = Anti-Ro / Anti LA Ab's - ANA often positive
Schirmer test- induce tears + place filter paper under eyes. Tears travel <10mm (should be 20mm+)
68
What is the treatment of sjorgen's?
Artificial tears, saliva + lubricant for sexual activity
- sometimes hydroxychloroquine given
69
What is a complication of sjorgen's?
increase risk of lymphomas
70
What is scleroderma?
is a group of rare autoimmune diseases that involve the hardening and tightening of the skin. It may also cause problems in the blood vessels, internal organs and digestive tract.
71
What are the subtypes of scleroderma?
Limited - crest
Diffuse
72
What is limited scleroderma?
most common type of scleroderma, limited cutaneous scleroderma
73
What is diffuse scleroderma?
subtype of scleroderma where excess collagen production causes skin thickening over large areas of the body.
-can be significant associated organ damage
74
What is the presentation of CREST?
C- calcinosis (Ca deposits in SC tissue) -> renal failure
R- raynaud's - digit ischemia due to sudden vasospasm in response to cold
E- oesophageal dysfunction/strictures -> GI Sx
S-sclerodactyly - thickening and tightening of skin on fingers/ toes - > movement restriction
T- telangiectasia (spider veins) --> risk of pulmonary hypertension
75
What is the diagnosis of crest scleroderma?
Anti centromere antibodies
ANA often positive
76
What is the treatment of scleroderma?
No cure
Treat Sx
77
What is polymyositis/dermatomyositis?
Inflammation + necrosis of skeletal muscle
- when skin is involved = dermatomyositis
78
What is the epidemiology of polymyositis?
- female
-HLAB8/DR£ genetic link
79
What is the presentation of polymyositis?
- symmetrical wasting of muscles of shoulders and pelvic girdle
- hard to stand from sitting, to squat, to put hands on top of head
-Dermatomyositis - skin change (gottron's papules -scales on knuckles, heliotrope - purple eyelid)
80
What is the diagnosis of polymyositis?
muscle fibre biopsy necrosis - diagnostic
Lactate dehydrogenase +creatinine Kinase raised
Anti Jo1 +
Anti Mi2 Ab's - but only in dermatomyositis
81
What is the treatment for polymyositis?
Bed rest
Prednisolone
82
What is fibromyalgia?
Chronic widespread pain (ask) for 3+ months with all other. causes ruled out
83
What is the epidemiology/RF of fibromyalgia?
females
depression
stress
poor
60+
84
What is the presentation of fibromyalgia?
stressed depressed female, 60+, fatigue, sleep disturbance, morning stiffness, pain
85
What pain pathway is affected in fibromyalgia?
non nociceptive pathway is affected - neuropathic pain + CNS processing of pain
86
What is the diagnosis of fibromyalgia?
- NO serological markers (ANA,aPL, anti-ccp/RF)
-NO high ESR/CRP
-PAIN in 11/18 palpation sites therefore clinical diagnosis
87
What is the treatment of fibromyalgia?
-educate patient + physiotherapy
-antidepressants for severe neuropathies - amitriptyline + CBT
88
What is Ddx of fibromyalgia?
polymyalgia rheumatica
89
What is Polymyalgia rheumatica (PMR)?
Large cell vasculitis presenting as chronic pain syndromes (affects muscles and joints)
- females , always 50+
-association with GCA
90
What is the diagnosis for PMR?
-High ESR + CRP -diagnostic
-temporal artery biopsy may show GCA, and may have anaemia of chronic disease
91
What is the treatment of PMR?
oral prednisolone
92
What is vasculitis?
Inflammatory disorder of the blood vessel walls, which can affect any organ by causing destruction or stenosis of a vessel
93
What are the types of vasculitis?
Large, medium and small vessel
94
What is the general treatment for vasculitis?
Corticosteroids- when given long term consider GI and bone protection -PPI, bisphosphonates
95
What are the types of large vessel vasculitis?
-GCA
-Takayatsu
96
What is takayatsu?
Asian/ Japanese women mainly affects aortic arch
-otherwise same as GCA
97
What is GCA?
Giant cell arteritis - inflammatory granulomatous arteries of large cerebral arteries, and affects the aorta and its main branches
98
What is the presentation of GCA?
-50+
-caucasian female
-unilateral temple headache, jaw claudication, +/- vision loss
-Temporal scalp tenderness
99
What is the pathology of GCA?
Affects branches of external carotid - scalp tenderness = temporal branch
Vison =opthalmic branch
Jaw= facial branch
100
What is the diagnosis of GCA?
-High ESR +/- CRP -1st line
-Temporal artery biopsy diagnostic = granulomatous inflammation of media + intima - Kathy skip lesions therefore take big chunk
101
What is the treatment of GCA?
Corticosteroids - prednisolone
102
What is the complication with GCA?
sudden painless vision loss in one eye
- temporary = amaurosis fugax
-may be permanent if not dealt with
TREAT- High dose IV methylprednisolone
103
What are the types of medium vessel vasculitis?
-Polyarteritis nodosa
-Buerger's disease
-Kawaski disease
104
What is polyarteritis nodosa?
Necrotosing vasculitis that causes aneurysms + thrombosis in medium sized vessels, leading to infarction in affected organs.
-males, associated with Hep B
105
What is the presentation of polyarteritis nodosa?
Severe systemic Sx:
-mononeuritis multiplex (ischemia of vasa nervorum)
-GI bleeds (mesenteric artery)
-CKD/AKI (renal arter)
-skin sc nodules + haemorrhage
-abdo pain+ anorexia
-hypertension
106
What is the diagnosis of polyarteritis nodosa?
CT angiogram - micro aneurysms - 'Beads on a string"
Biopsy (Kidney) - necrotising vasculitis due to htn
107
What is the treatment of polyarteritis nodosa?
Corticosteroids
Control HTN - ACE-i
Hep B treatment after corticosteroids -antiviral agent +plasma exchange
108
What is Buerger's disease?
-Male smokers 20-40
-peripheral skin necrosis
-Thromboangiitis obliterans (Buerger disease) is caused by small and medium blood vessels that become inflamed and swollen
109
What is Kawasaki disease?
-Children
-causes coronary artery aneurysms
110
What are the types of small vessel vasculitis?
- Eosinphillic granulomatosis with polyangitis (churg strauss disease)
-Granulomatosis with polyangitis (Wegener's disease)
-Henloch schonlein purpura
111
What is Eosinphillic granulomatosis with polyangitis (EGPA)?
- small and medium vasculitis
-associated with lung and skin problems
-severe asthma
-pANCA positive
-Corticosteroids
112
What is pANCA?
perinuclear anti-neutrophil cytoplasmic antibodies
113
What is granulomatosis with polyangitis (GPA)?
- small vessel vasculitis
-affcets respiratory tract +Kidney
-saddle shaped nose/epistaxis/cough
-cause of glomerulonephritis
-cANCA positive
-corticosteroids
114
What is cANCA?
cytoplasmic anti–neutrophil cytoplasmic antibodies
115
What is Henloch Schonlein Purpura?
-IgA vasculitis , IgA deposits in small blood vessels in GBM on kidney
-DDx for IgA nephropathy but HSP has purpuric rash on lower limbs + affects other organs - joints/abdo /renal
-rest+analgesia
116
What are spondylarthropathies?
Assymetrical seronegative (RF negative) arthritis, associated with HLAB27
117
What is HLAB27?
Human leukocyte antigens B27
Class 1 surface antigen, encoded by major histocompatibility complex
118
What are the types of spondylarthropathies?
-ankylosing spondylitis
-psoriatic arthritis
-reactive arthritis
-IBD associated arthritis
119
What are the general features of spondylarthropathies?
SPINEACHE
-sausage fingers (Dactylitis)
-psoriasis
-inflammatory back pain
-NSAIDs good response
-Enthesitis - inflamed heel tendon
-Arthritis
-chrons or collitis
-HLAB27
-Eyes-uveitis
120
What is ankylosing spondylitis?
Abnormal stiffening of joints (sacroiliac + vertebral) due to new bony formation
121
What is the epidemiology go ankylosing pondylitis?
- more common in young males
-HLAB27 positive
122
What is the presentation of ankylosing spondylitis?
young male with progressively worsening back stiffness; worst in morning and at night, better with exercise
-anterior uveitis
-enthesitis
-dactylitis
- can be kyphosis - abnormal rounding of upper back
-Schober test - shows low lumbar flexion
123
What is the pathology of ankylosing spondylitis?
syndesmophytes (vertical abnormal bony growths) replace spinal bone damage by inflammation+ make spine less mobile
- causes inflamed tendons eyes, fingers
124
What is the diagnosis for ankylosing spondylitis?
XR =show bamboo spine + sacroilitis + squared vertebral bodies +syndesmophytes
MRI- can show sacroilitpis before XR therefore better screening tool
-ESR/CRP raised
HLAB27 positive
125
What is sacroilitis?
an inflammation of the sacroiliac joint (SI), usually resulting in pain
126
What are syndesmophytes?
bony outgrowths from the spinal ligaments as they attach to adjacent vertebral bodies
127
What is the treatment for ankylosing spondylitis?
symptoms = exercise +NSAID
DMARD - TNF alpha blockers - influximab
128
What is psoriatic arthritis?
Autoimmune arthritis characterised by red scaly patches - associated with psoriasis
129
What is the epidemiology of psoriatic arthritis?
-10-40% with psoriasis develop within 10years
130
What is the presentation of moderate psoriatic arthritis?
Inflamed DIPJ +
-nail dystrophy(onycholysis)
-Dactylitis
-Enthesitis
-psoriatic rash on skin - hidden sites (behind ears, scalp , under nails , penile
131
What is the presentation of severe psoriatic arthritis?
Arthritis mutilans (5%0 = pencil in cup deformity- osteolysis of bone = progressive shortening ; fingers telescope in on themselves
132
What is the treatment for psoriatic arthritis?
Symptoms - NSAIDs (steroid injection if severe)
DMARD- methotrexate if fails anti TNF- influximab
If fails - IL 12+23 inhibits -ustekinumab
133
What is reactive arthritis?
Sterile inflammation of synovial membranes + tendons, reacting to distant infection(usually GI,STI)
134
What is the aetiology of reactive arthritis?
Gastrointestinal= C.jejuni, salmonella, shigella
STI= C.trachomatis -mc
N.gonorrhoea
135
What is the presentation of reactive arthritis?
Reiter's triad:
- can't see, pee, climb a tree
- uveitis, urethritis , arthritis, enthesitis
-keratoderma blenorrhagica
136
What is the main Ddx to reactive arthritis?
septic arthritis - hot red swollen joints +Hx of infection
137
What is the diagnosis for reactive arthritis?
Joint aspirate MC+S = shows no organism
Plane polarised light microscopy = negative for crystal arthropathy
-ESR/CRP raised, HLAB27 positive
-sexual health review +stool culture
138
What is the treatment fo reactive arthritis?
Symptoms = NSAIDs + steroid injection
- mostly single attack - if happens 6+ months of recurrence it is chronic - methotrexate
139
What is a type of infective arthritis?
septic arthritis
140
What is septic arthritis?
Direct bacterial infection of joint (either direct access or haematogenous spread)
141
What is a septic arthritis medical emergency?
- acutely inflamed joint with fever, typically knee
- extremely painful
- can destroy knee <25hr
142
What organisms cause infective arthritis ?
S.aureus-mc
H.influenza (children)
N.gonorrhoea
E.coli/pseudomonas (IVDU)
143
What are the RF of septic arthritis ?
- IVDU
-Immunosupression
-recent surgery
-trauma
-prosthetic joints
-inflammatory joint disease
144
What is the diagnosis of septic arthritis?
urgent joint aspirate with MC+s and polarised light microscopy
septic = ID causative organism
-Raised ESR/CRP, consider blood culture + sexual health review
145
Want is the treatment for septic arthritis?
Joint aspirate (drainage) then empirical Abx
- if on methotrexate, ant-TNF stop these
-if on steroids - double prednisolone dose(increase stress response)
-NSAIDs for analgesia
146
What Abx would be given for Gram negatives ; E.coli, P.aeruginosa ?
Flucoxacillin
147
What Abx would you give S.aureus?
Vancomycin
148
What Abx is used to treat N. gonorrhoea?
IM ceftriaxone + azithromycin
149
What is osteomyelitis?
Acutely inflamed infected bone marrow, haematogenous or local spread
150
What spread is most common in children for osteomyelitis?
haemogenous spread
151
What spread is the most common in adults for osteomyelitis?
local
152
What organisms cause osteomyelitis ?
S.aureus (90%)
Salmonella in sickle cell patients
153
What are the RF of osteomyelitis?
- IVDU
-immunosuppression
-PVD
-DM
-sickle cell anemia
-inflam.arthrtiid
-trauma
154
What is the pathology of osteomyelitis?
Direct inoculation/ local spread/ haematogenous spread--> acute bone change to inflammation and bone oedema
Chronic bone change to Sequestra (necrotic bone embedded in pus)
Involucrum (thick sclerotic bone packed around sequestra to compensate for support)
155
What is the presentation of osteomyelitis?
Acutely -dull bony pain +hot swollen/ worse with movement
Chronic - deep ulcers
156
What is a Ddx of osteomyelitis?
Charcot joint
157
What is Charcot joint?
Damage to sensory nerves due to diabetic neuropathy
- causes progressive degeneration of weight bearing joint + bony destruction.
-often affects foot, presents with diabetic feet
158
What is the diagnosis of osteomyelitis?
BM biopsy + culture +blood+MC/s = ID causative agent
XR - osteopenia then MRI- bone marrow oedema
Raises ESR/CRP
159
What is the treatment of osteomyelitis?
Immobilise + Abx
MRSA+S.aureus = vancomycin or teicoplannin
Fusidic acid = treats S.aureus
Flucoxacillin = Salmonella
160
Why would you use Teicoplannin rather than Vancomycin to treat S.aureus?
longer lasting than vancomycin but more side effects - AF upset/ pruritus
161
What would a positive BM biopsy of caseating granulomas suggest?
Tuberculosis osteomyelitis
162
What are the primary bone tumours?
- osteosarcoma
-Ewing sarcoma
-Fibrosarcoma
-Chondrosarcoma
- rarely seen, mostly in children
163
What are the secondary causes of bone tumours?
Breast
Lung --------Both osteolytic
Prostate
Thyroid
RCC -------> all osteosclerotic
Myeloma- can cause bone pain- old crab
164
What is osteosarcoma?
-Most common primary bone malignancy
-associated with Pagets
-Px 15-19
-Mets to lung
-XR - 'sunburst' appearing bone
165
What is Ewing sarcoma?
-From mesenchymal steam cells
-15 years
-rare
166
What is chondrosarcoma?
Cartilage cancer
167
What are the general symptoms of bone tumours?
-Local (depend on tumour) - severe pain worst at night
-low range of motion of long bone, vertebrae
-Systemic = weight loss, fever, fatigue, malaise
168
What is the diagnosis of bone tumours?
Skeletal isotope scan - shows change before XR
XR- osteolysis (osteosclerosis -> prostatic mets)
-High ALP,ESR/CRP
-hypercalcemia of malignancy
169
What is the general treatment for bone tumours?
Chemo/ radiotherapy
Bisphosphonates
170
What is osteomalacia?
Defective bone mineralisation
Before epiphyseal fusion = rickets
After- osteomalacia
171
What is the pathology of osteomalacia ?
Due to VIT D deficiency therefore low calcium and phosphate (hydroxyapatite mineral of bone)
172
What are the causes of osteomalacia?
-HyperPTH (increase Ca release from bone therefore less available for formation)
-Vit D deficiency - malabsorption / reduced intake/ poor sunlight
-CKD/Renal failure - low active vit D production
-Liver failure = low reaction in vit D pathway
-Anticonvulsant drugs - increase CYP450 metabolism of Vit D
173
What is the Vitamin D pathway?
7-Dehdrocholesterol -> Cholecalciferol (inactive vit D) --> 25-hydroxyvitD -->!,25-DihydroxyvitD (activated D3) -bone, GIT,Renal DCT
174
What is the presentation of osteomalacia?
osteomalacia- fractures, proximal weakness + difficulty weight bearing
175
What is the presentation of rickets?
-skeletal deformities
-Knocked knees + bowed legs
-Wide epiphysis
176
What is the diagnosis of osteomalacia?
BM biopsy = incomplete mineralisation (diagnostic)
- bloods =hypocalcemia, raised PTH, low serum calcium, low 25dihydroxyvitD
-XR = looser's zones (defective mineralisation )
177
What is the treatment for osteomalacia?
Vitamin D replacement (calcitriol)
-Increase dietary intake - D3 tablets ,eggs
178
What is Paget's disease?
Focal disorder of bone remodelling (areas of patchy bone due to improper osteoblast/ class function therefore areas of sclerosis + lysis
179
What is the epidemiology of Paget disease?
Idiopathic
Females - 40+
180
What is the presentation of Paget's disease?
-Bone pain
-Bone change/ skull change
-Neurological Sx = Nerve compression of CN8 (Deafness)
-Hydrocephalus = Sylvian aqueduct blockage
181
What is the diagnosis of Paget's disease?
XR - osteoporosis circumscripta
-cotton wool skull - Skull change areas of lysis and sclerosis
Urinary hydroxyproline = protein constituent of bone collagen , good marker of disease progression
-high ALP
182
What is the treatment for Paget's disease?
Bisphosphonates
NSAIDs - for pain relief
183
What are two connective tissue disorders?
-MARFAN's
-Ehlers Danlos
184
What is Marfan's disease?
Autosomal dominnat FBN1 mutation
Low connective tissue tensile strength due to FBN1 mutation which is involved in making fibrillin
185
What is fibrillin?
An important component of connective tissue
186
What is the presentation of marfan's syndrome?
Marfan's body habitus - tall and thin, long fingers , pectus excavatum/carinatum,Arachnodactyly
187
What are the aortic complications of Marfan's?
- aortic regurgitation murmur
-AA,Aortic dissection
188
What is the diagnosis of Marfan's?
Clinical presentation
FBN-1 mutation
189
What is Ehlers Danlos?
Autosomal dominant mutation affecting collagen proteins
13 subtypes
190
What is the presentation of Ehlers Danlos?
Joint hyper mobility + CV complications - mitral regurgitation and AAA,AD
191
What is the diagnosis of Ehlers Danlos?
Clinical presentation
Collagen mutation
192
What are complications of connective tissue disorders?
Weak valves and weak vessels
- need to monitor regurgitation murmurs
-AA ruptures/ Aortic dissection
193
What is mechanical backpain?
Common patient complaint in community
- Common, often self limiting
-may be normal especially people 20-55, trauma/ work related
-signs for serous pathology= elderly, neuropathic pain
194
What is lumbar spondylosis?
Degenration of IV disc, loses its compliance + thins over time
- older patients
-initially aSx, progressively worsens
-L4/5 or L5/S1- mc
-XR if serious pathology affected
-Analgesia +physiotherpay
195
What is the mechanism of methotrexate?
It is a DMARD = they completely inhibit dihydrofolate reductase therefore inhibits folate synthesis
196
What are the side effects/ risk of methotrexate?
At risk of folate deficiency so they need to the 5mg once weekly folate supplements but not on the same day as methotrexate taken
197
What is monitored when a patient is on methotrexate?
FBC,LFT,U+E
- before treatment
every 1-2 weeks until therapy stabilised
-every 2-3 months after
198
What is acute pancreatitis ?
Acute inflammation of pancreatic gland
- reversible
199
What are the causes of pancreatitis?
I get smashed
Idiopathic
gall stones
ethanol
trauma
steroids
mumps
autoimmune
spider bite
hypercalcemia
ERCP
Drugs
200
What is the most common cause of acute pancreatitis?
Gall stones
201
What is the pathology of acute pancreatitis?
-Gall stones obstruct pancreatic secretions therefore accumulated digestive enzymes in pancreas
- Host defences (A1AT, pancreatic secretory transport inhibition) soon overwhelmed - autodigetsions --> inflammation and enzymes leak in blood
-increase intracellular calcium causing activation of trypsinogen which digests pancreas
202
What is the presentation of acute pancreatitis?
Sudden severe epigastric pain radiating to back
+ jaundice, pyrexic, stearrhoea, grey turner signs (flank bleeding) and Cullen sign (periumbilical bleeding)
203
What is the diagnosis of acute pancreatitis?
Bloods: high serum anylase/ lipase - GS - lipase more specific
Erect CXR to exclude gastroduodenal perforation
AUSS - diagnostic for gall stones - CT/MRI extent of damage
204
For a diagnosis of acute pancreatitis to be made what characteristics should the patient have?
- characteristic signs/symtpoms
-raised amylase/lipase
-Radiological evidence
205
What are the pancreas scoring symptoms?
APACHE2 = assess severity within 24hr
Glasgow + ransen's score- prediction of severe attack, only after 48hr
206
What is the treatment for acute pancreatitis?
Acute emergency Px= assess severity with scoring is key
- NG tube if feeding
-IV fluid
-Analgesia -IV fluid
-catheterise
-Abx prophylaxis
207
What are the complications of acute pancreatitis?
SIRS - systemic inflammatory response syndrome
2< of:
- tachycardia
-tachypnoea
-pyrexia
-raised WCC
208
What is chronic pancreatitis?
3+ months of pancreatic deterioration ; irreversible pancreatic inflammation and fibrosis
209
What is the most common cause of chronic pancreatitis?
most common = alcohol
- CKD/CF/Trauma/ recurrent acute pancreatitis
210
What is the presentation of chronic pancreatitis?
Epigastric pain radiating to the back
-exacerbated by alcohol
-exocrine (steatorrhoea) and endocrine (T2DM) dysfunction
211
What is the diagnosis of chronic pancreatitis?
Bloods: lipase and amylase unlikely to be raised in severe cases as there's none left to leak out
-fecal elastase = high - indicator of exocrine function
Abdo USS + CCT= detects pancreas calcification and dilated pancreatic duct -diagnostic
212
What is the treatment for chronic pancreatitis ?
-alcohol cessation
-NSAIDs for Sx pain
-Pancreatic supplements - insulin/ enzymes
213
What is a Ddx for chronic pancreatitis?
Pancreatic cancer
214
What is a ddx fro acute pancreatitis ?
AAA
215
What is autoimmune pancreatitis?
similar presentation to chronic - as a result os autoimmune process
- high in Japan
-High serum IgG4
-oral prednisolone
216
What is the pathology of chronic pancreatitis?
- obstruction of bicarbonate secretion in the pancreatic lumen causes early activation of trypsinogen and auto digestion --> replaced by fibrosis
217
What is portal hypertension?
A complication of cirrhosis; pathologically it is an increase in pressure in portal vein
218
What are the pre-hepatic causes of portal hypertension?
Portal vein thrombosis
219
What is the intrahepatic cause of portal HTN?
Cirrhosis - mc in UK
Schistomiasis - common worldwide
220
What are the post hepatic causes of portal HTN?
Budd Chiari - hepatic vein obstruction
RHS herat failure
Constrictive pericarditis
221
What is the pathology of portal hypertension?
- normal pressure 5-8mmHg in portal vein
-Cirrhosis = increased resistance to flow therefore splanchnic dilation and compensatory increased cardiac output - results in fluid overload in portal vein (pressure 10+=bad 12+=severe)
Results in collateral blood shunting to gastroesophogeal veins - becoming oesophageal varices
222
What is the presentation of portal HTN?
- mostly aSx
- present when oesophageal varices rupture
223
What are oesophageal varices?
dilated esophageal veins connecting the portal and systemic circulations
If varices haemorrhage = Px with haematemesis
-from long Hx of alcoholism/ cirrhosis
224
What is the diagnosis of oesophageal varices?
OGD - oesophagogastroduodenoscopy
Upper GI
225
What is the acute treatment for oesophageal varices?
Acutely : resus until haemodynamically stable - consider blood transfusion
226
What is the treatment for oesophageal varices to stop the bleed?
1. IV terupressin
2. Variceal banding
3. TIPPS (transjugular intraheptic portosytsgemic shunt - decrease portal pressure by diverting blood to other large veins)
227
What is the treatment of oesophageal varices to prevent the bleed?
Bb - non selective - propanolol + nitrates
Repeat vatical banding
- liver transplant
228
What is a Ddx of oesophageal varices?
mallory weiss tear (tear of oesophageal mucosa) - increased abdo pressure- retching
229
What are ascites?
Accumulated free fluid in abdo cavity, a complication of cirrhosis
230
What are the causes of ascites?
local inflammation - peritonitis, TB , abdo cancer
Low protein - nephrotic syndrome , liver failure - hypoalbuminemia
Flow stasis - cirrhosis, Budd chiari, constrictive pericarditis
231
What is the presentation of ascites?
Abdo distention
Shifting dullness- tap on central abdo= resonant , tap on flank = fluid
Patient will lay on side and tap on flank = resonant as fluid has shifted
May have jaundice + pruritus
232
What is the diagnosis of ascites?
Shifting dullness on exam
Ascitic tap -
1. cytology (WCC counts ) +MC+S
2. Protein measurement
233
What is transudate?
<30g/L protein - Serum albumin -ascitic gradient <11g/L
- clear fluid
-fluid due to high hydrostatic pressure = portal hypertension, Budd Chiari, constrictive pericarditis, Nephrotic syndromes
234
What is exudate?
>30g/L protein - serum albumin - ascites gradient >11g/L
- cloudy fluid
-fluid due to inflammatory mediated exudation or low oncotic pressure = malignancy, peritonitis, pancreatitis
235
What is the treatment of ascites?
- treat underlying cause
-Diuretic (increase Na+ excretion therefore more fluid pushed out --> spironolactone
-paracentesis
-liver transplant
236
What is peritonitis?
Inflamed peritoneal cavity
237
What are the sensations in the abdomen?
T5-9 = epigastric = greater splanchnic , foregut
T10-11 = umbilical = lesser splanchnic =midgut
T12 = hypogastric = least splanchnic = hind gut
238
What are primary types of peritonitis?
- ascites
-spontaneous bacterial peritonitis -mc
239
What are the secondary types of peritonitis ?
underlying cause = bile/ chemical cause or malignancy
240
What are the bacterial causes of peritonitis ?
Gram neg = E.coli + Klebsiella - colliform rods
Gram pos = Staph Aureus (cocci)
241
What are the chemical causes of peritonitis?
-bile
-old clotted blood
-ruptured ectopic pregnancy
242
What is the presentation of peritonitis?
- sudden onset severe abdomen pain , then collapse + septic shock + fever
Rigidity helps pain - guarding, holding on to abdomen to stop it moving
-poorly localised - poorer sensation of pain
-Well localised - more inflamed - better localised sensation
+/- ascites
243
What is the diagnosis of peritonitis?
Ascitic tap shows neutrophilia, cultures MC+s shows causative agent
Raised ESR +CRP
Exclude pregnancy + bowel obstruction
Erect CXR shows air under diaphragm
244
What is the treatment for peritonitis?
ABCDE
Treat underlying cause - IV fluid + IV Abx
Surgery - peritoneal lavage
245
What are the complications of peritonitis?
Septicema if not TX early
Subphrenic, pelvic abscesses, paralytic ulcers
246
What are the metabolic diseases?
Haemochromatosis
Wilson's disease
A1AT deficiency
247
What is haemoochromatosis ?
Auto recessive mutation of HFE gene on chromosome 6 result in excess body Fe/
248
What are the risk factors of hameochromatosis?
Males - 50
FHx
- less likely in women as they lose iron via mantra cycle
249
What is the pathology of haemochromatosis?
Excess Fe uptake by transferrin-1 and low hepcidin synthesis (protein that regulates Fe homeostasis)
Fe accumulation (normally total body Fe 3-4g, here 20-30g) +fibrosis of organs ; Liver (+pancreas, kidney , heart , akin)
250
What is the presentation of haemochromatosis?
Fatigue, joint pain,Hypogonadism, slate grey skin, liver cirrhosis, osteoporosis, heart failure
Gross Fe overload triad:
-Bronze statue skin
-Hepatomegaly
-T2DM
251
What is the diagnosis of haemochromatosis?
Fe studies; Raised serum Fe/ ferritin/ transferrin saturation
Low Total iron binding capacity - don't want to bind more iron
Genetic test - HFE mutation
Liver biopsy - assess degree of liver damage with Prussian blue stain
252
What is the treatment for haemochromatosis?
1st line - Venesection - regular removing blood
If CI= desfernoxamine (chelation therapy)
Lifestyle changes - low iron diet, avoid fruits
253
What is Wilson's disease?
Auto recessive mutation of ATP7B gene on chromosome 13; Excess body Cu
254
What are the risk factors of Wilson disease?
FHx - young patient around 20
255
What is the pathology of Wilson's disease?
Impaired Cu biliary excretion + normal transport bound to ceruloplasmin ; increased copper accumulated in liver and CNS(basal ganglia)
256
What is the presentation of Wilson's disease?
hepatic = liver disease / jaundice
Neurological = Parkinsonism , memory issues
Opthalmogical = Kayser Fleischer Rings (Cu deposits in cornea) - PATHOGNAMONIC
257
What is the diagnosis of Wilson's disease?
Cu tests; low serum copper + low ceruloplasmin - more Cu deposited in tissue rather than in bloodstream mainly due to the fact there's low ceruloplasmin
Liver biopsy - high copper / hepatitis - GS
MRI brain - cerebellar + BG degeneration
258
What is the treatment of Wilson's?
1st line - Penicillamine ( Cu chelation, lifelong)
-Change diet , avoid high copper food
Liver transplant -last resort
259
What is A1AT deficiency?
Autosomal recessive mutation of protease inhibitor gene (serpina-1 gene) on chromosome 14 causing a deficiency of alpha 1 antitrypsin enzyme
260
What is the pathology of A1AT deficiency?
A1AT normally inhibits neutrophil elastase which degrades elastic tissue
Deficient A1AT= causes an increase in NE:
-Lungs - degrades elastic tissue causing alveolar duct collapse, air trapping, and characteristic panacinar emphysema
-Liver - unprotected - soon becomes fibrotic ; Cirrhosis +HCC risk (A1AT made by liver - liver fibrosis will make synthetic function worse)
261
What is the presentation of A1AT deficiency disease?
-young, middle aged male, no smoking Hx
-dyspnoea, chronic cough, sputum production, barrel chest
-Jaundice
262
What is the diagnosis of A1AT deficiency?
Serum A1AT low <20mmol/L
Barrel chest on exam , CXR shows hyperinflate lungs
CT- panacinar emphysema
LFT- obstruction FEV1;FVC <0.7
Genetic - PI mutation
263
What is the treatment of A1AT deficiency?
- no cure
-stop smoking
-manage emphysema - inhalers
- consider hepatic decompensation patients for transplant
264
What is a paracetamol overdose?
Paracetamol ingestion >75mg/Kg
- 50% all fulminant liver failure in UK
265
What is the presentation of paracetamol overdose?
acute severe RUQ pain, Severe n+v
266
How is paracetamol normally metabolised?
paracetamol (90%) metabolised in the liver by Phase 2 conjugation/glucuronidtaion then excreted by urine
Phase 1 (10% of paracetamol) metabolised via CYP450 pathway and forms NAPQI which is toxic , conjugated with glutathione to become non toxic and can be excreted
267
What happens to paracetamol metabolism if there is an overdose?
Phase 2 pathway is saturated, so phase 1 pathway is used, glutathione is depleted therefore a build up of NAPQI in liver
268
What is the treatment of paracetamol overdose?
Activated charcoal (within 1hr paracetamol ingestion) + N-acetyl- cysteine
269
What is a hernia?
protrusion of an organ through defect in its containing cavity
270
What are the types of hernia?
Reducible - manually push back into place
Irreducible - obstructed (intestinal obstruction)
-strangulated (intestinal ischemia)
-Incarcerated
271
What is a hiatal hernia?
stomach hernia through diaphragm aperture
272
What is a rolling hiatal hernia?
20%
LES stays in abdo, part of funds rolls into thorax
273
What is a sliding hiatal hernia?
80%
LES slides into abdo
274
What are the Sx of hiatal hernia?
Gord
Dysphagia
- obese women , 50+
275
What is the diagnosis of hiatal hernia?
CXR
Barium swallow - diagnostic
OGD
276
What is an Inguinal hernia?
Protrusion of abdo contents through inguinal canal
277
What is direct inguinal hernia?
20%
In hesselbach's triangle , medial to inferior epigastric artery
278
What is indirect inguinal hernia?
90%
Lateral to inferior epigastric artery
279
What are the risk factors of inguinal hernia?
male
Hx of heavy lifting / abdo pressure
280
What is the presentation of inguinal hernia?
Painful swelling groin , points along groin margin
281
What is the diagnosis of inguinal hernia?
usually clinical
AUSS/CT/MTI if unsure
282
What is a femoral hernia?
bowel through femoral canal
283
What are the RF and complications of femoral hernia?
Female - middle age
- likely to strangulate due to rigid femoral canal borders
284
What is the presentation of femoral after?
Swelling in upper thigh pointing down
