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Flashcards in Pathology Deck (251):
1

What surrounds the entire skeletal muscle?

epimysium

2

What surrounds the bundles of skeletal muscle fibres?

perimysium

3

What surrounds the individual muscle fibres?

endomysium

4

What are the 3 main indications for muscle biopsy?

-evidence of muscle disease (eg weakness, muscle symptoms, elevated CK)
-presence of neuropathy
-presence of vascular disorder (vasculitis)

5

what is fasciculation?

uncontrolled twitching of muscle

6

When CK level is higher than normal, in the 'low' classification, what type of disorder does this indicate?

neurogenic disorder

7

when CK level is higher than normal, in the 'intermediate' classification, what type of disorder does this indicate?

inflammatory myopathy

8

when CK level is higher than normal, in the 'high' classification, what type of disorder does this mean?

muscular dystrophy

9

what are the 5 main groups of myopathies?

-muscular dystrophies
-inflammatory myopathies
-congenital myopathies
-secondary myopathies
-metabolic myopathies

10

Why are ring fibres sometimes formed during muscle dystrophy?

condensation of the cytoplasm

11

Why can there be enlarged nuclei and increased number of nuclei during muscle dystrophy?

due to attempted regeneration co-existing with the atrophy

12

What type of inheritance is Duchenne's Muscular Dystrophy (DMD)?

x-linked

13

What muscles does Duchenne's Muscular Dystrophy (DMD) mainly affect?

muscles of the pelvic girdle

14

What type of inheritance is Becker's Muscular Dystrophy (BMD)?

x-linked

15

What muscles does Becker's Muscular Dystrophy (BMD) mainly affect?

muscles of the pelvic girdle

16

When is the onset of Duchenne's Muscular Dystrophy (DMD)?

2 - 4 years old

17

What is the life expectancy of someone with Duchenne's Muscular Dystrophy (DMD)?

20

18

What happens to the calves of patients with Duchenne's Muscular Dystrophy (DMD)?

pseudohypertrophy

19

What mutation causes Duchenne's Muscular Dystrophy (DMD)?

Mutations in dystrophin gene on long arm of chromosome X

20

What causes the different severities of Duchenne's Muscular Dystrophy (DMD)?

mutations resulting in frame shift- severe
mutations resulting in altered binding- moderate to severe
mutations in middle rod- mild (Beckers)

21

What does the mutation in the dystrophin gene in Duchenne's Muscular Dystrophy cause? (in terms of basement membrane and actin and Ca++ entry)

changes anchorage of actin cytoskeleton to basement membrane

uncontrolled Ca++ entry into cells

22

What does the change in anchorage of actin cytoskeleton to basement membrane in Duchenne's Muscular Dystrophy do to the muscle fibres?

makes them more liable to tear

23

What gene is mutated in Becker's Muscular Dystrophy (BMD)?

dystrophin
(variant of DMD)

24

compare disease progress of Beckers to Duchennes?

Beckers progression is much slower

25

What is myotonia?

inability to relax voluntary muscles after vigorous effort

26

What are inflammatory myopathies?

primary inflammation of muscle

27

What is polymyositis?

a chronic inflammatory myopathy
autoimmune

28

compare inflammatory myopathies to muscular dystrophies in terms of pain?

no pain in dystrophies, pain is present in inflammatory myopahties

29

What autoimmune proteins are found in polymyositis?

antibodies to muscle antigens

30

what type of lymphocytes are mainly involved in polymyositis?

cytotoxic lympocytes

31

what is dermatomyositis?

polymyositis with skin changes
a chronic inflammatory myopathy
autoimmune

32

What type of skin changes are present in dermatomyositis?

upper body erythema
swelling of eyelids with purple discolouration

33

what is the aetiology of polymyositis?

unknown

34

what is the aetiology of dermatomyositis?

unknown
but 10% associated with underlying malignancy

35

what type of lymphocytes are mainly involved in dermatomyositis?

helper T cells

36

compare the histology of polymyositis and dermatomyositis?

no histological differences, need to use other tests eg fluorescent staining

37

What are neurogenic disorders of muscle?

stereotyped changes after nerve damage with subsequent re-innervation
(pathology in motor neuron not muscle)

38

What is motor neurone disease?

progressive degeneration of anterior horn cells causing denervation atrophy, fasciculation and weakness

39

What type of inheritance is spinal muscular atrophy?

autosomal recessive

40

compare motor neurone disease to spinal muscular atrophy?

same pathology but different aetiology
MND is sporadic
spinal muscular atrophy is inherited

41

What is myasthenia gravis?

an autoimmune disease causing weakness, proptosis, fatigue and dysphagia

42

What is proptosis?

bulging of the eye

43

who commonly gets myasthenia gravis?

women between 20 and 40

44

what are the main causes of myasthenia gravis?

thymoma or thymic hyperplasia

45

What antibodies are produces in myasthenia gravis?

antibodies to ACh recepto in post-synaptic cleft

46

What is rhabdomyolysis?

breakdown of skeletal muscle

47

what are the 4 major side effects of rhabdomyolysis?

-myoglobinuria (which causes acute renal failure)
-hyperkalaemia
-necrosis
-shock

48

what are the main causes of rhabdomyolysis?

massive trauma
pressure induced
mushroom poisoning
drug side effects

49

What is the main management of connective tissue disorders?

anti-inflammatory/immunosuppression therapy

50

what antibodies are produces in SLE?

antinuclear antibodies (ANAs), anti-ds DNA, anti- Ro, anti-RNP

51

what skin effects of SLE can be seen?

butterly rash
discoid lupus erythematosus

52

what are the effects of SLE on the joints?

arthralgia

53

what are the effects of SLE on the kidneys?

glomerulonephritis

54

what are the effects of SLE on the central nervous system?

psychiatric symptoms
focal neurological symptoms

55

what are the effects of SLE on the cardiovascular system?

pericarditis
myocarditis
necrotising vasculitis

56

what are the effects of SLE on the immune system?

lymphadenopathy
splenomegaly

57

what are the effects of SLE on the lungs?

pleuritis
pleural effusions

58

what are the effects of SLE on haematological system?

anaemia
leukopenia
thrombophilia

59

what hypersensitivity reaction is SLE?

type 3 (visceral lesions)- deposition of IgG
type 2 (haematological effects)- lysis of RBCs

60

What is polyarteritis nodosa?

inflammation and fibrinoid necrosis of small/medium arteries

61

what is the aetiology of polyarteritis nodosa?

unknown
small association with Hep B carriage

62

where is the pain and stiffness most present in polymyalgia rheumatica?

shoulder and pelvid girdle
(no muscle weakness)

63

what is the treatment for polymyalgia rheumatica?

steroids
prednisolone 15mg for 18 months

64

who are the people most likely to get polymyalgia rheumatica?

elderly women

65

when is pain and stiffness usually at its worst in polymyalgia rheumatica?

morning

66

what is termporal arteritis?

giant cell arteritis of the temporal artery (vasculitis)

67

what is scleroderma?

excessive fibrosis of organs and tissues due to excessive collagen production

68

scleroderma is associated with CREST syndrome, what is this?

calcinosis
raynauds
oesophageal dysfunction
sclerodactyly
telangectasia

69

what is sclerodactyly?

thickening of skin on fingers and toes

70

what is telangectasia?

dilation of small capillaries so they appear like red or purple clusters on teh skin

71

what are the 4 main causes of death for patients with scleroderma?

-renal failure (secondary to malignant hypertension)
-severe respiratory compromise
-cor pulmonale
-cardiac failure/arrhymias (secondary to myocardial fibrosis)

72

What is the name of a cartilage capped bony projection arising on the external surface of the bone which contains a marrow cavity continuous with that of the underlying bone?

osteochondroma
(exostosis)

73

who tends to develop osteochondroma?

males and females under 20

74

where do osteochondromas tend to develop?

epiphyses of long bones

75

what is the presumed cell of origin of osteochondromas?

chondrocytes

76

are osteochondromas benign or malignant?

benign

77

why can osteochondromas be surgically removed even if they are benign?

can sometimes cause pain or irritation

or if it's growing in size (may become malignant)

78

what is a chondroma?

benign hyaline cartilage tumour

79

where do chondromas arise?

medullary cavity of bones of hands and feet
usually at metaphysis

80

who tends to get chondromas?

young adults
men > women

81

What is Mafucci's syndrome?

multiple enchondromas associated with multiple haemangiomas
(premalignant)

82

What is Ollier's Disease?

a rare developmental disorder involving multiple enchodromas of one extremity (usually unilateral)
(premalignant)

83

what type of inheritance is Ollier's disease

not hereditary or familial

84

compare maffucci's syndome to ollier's disease in terms of malignant transformation risk?

Maffucci's syndrome has a greater malignant transformation risk than Ollier's disease

85

What is the name of a benign osteoblastic tumour composed of a central core of vascular osteoid tissue (immature) and peripheral zone of sclerotic bone?

osteoid osteoma

86

who tends to get osteoid osteomas?

children and young adults
M > F

87

where are osteoid osteomas found?

poximal femur,
diaphysis of long bones,
vertebrae

88

what is the distinct clinical picture of a person with an osteoid osteoma?

dull pain which is worse at night
pain relieved by aspirin or NSAIDs

89

what is the name of a benign cartilage tumour arising in bone?

chondroblastoma

90

where are chondroblastomas found?

epiphysis of long bones

91

who tends to get chondroblastomas?

20 -30 year olds

92

what is the treatment for a chondroblastoma?

biopsy and curettage
plus adjuvant liquid nitrogen

93

who tends to get giant cell tumours?

25-40 year olds
F > M

94

where is the site of giant cell tumours?

metaphsis and epiphysis long bones, often around the knee and distal radius
can occur in pelvis and spine
can involve subchondral bone and joint

95

what is the presumed cell of origin of a giant cell tumour?

osteoclast

96

is a giant cell tumour benign or malignant?

benign
but locally aggressve

97

what is the treatment for a giant cell tumour?

intralesional excision
very aggressive lesions may need joint replacement

98

what happens to giant cell tumours when they are radiated?

undergo malignant transformation

99

what is the name of a benign, self-limited tumour that produces osteoid and bone?

osteoblastoma

100

are osteoblastomas benign or malignant?

benign
but locally aggressive

101

where are osteoblastomas found?

metaphysis or diaphysis of long bones

102

what is the treatment for an osteoblastoma?

surgical resection by:
curettage
intralesional excition
en bloc resection

103

what is the name of a very rare tumour arising from notochord remnants?

chordoma

104

where are chordomas usually found?

midline
often in sacral region or base of skull

105

who tends to get chordomas?

40+
F > M

106

are chordomas benign or malignant?

benign
but locally aggressive

107

what is the treatment for chordomas?

generally difficult to resect
radiation may be helpful
chemotherapy for late stage disease

108

what is the name of a malignant tumour of osteoblasts which form osteoid?

osteosarcoma

109

who tends to get osteosarcomas?

young adults
older patients with predisposing conditions (eg Pagets)
M > F

110

where are osteosarcomas usually found?

bones around the knee,ie particularly distal femur, proximal tibia
also proximal femur, proximal humerous and pelvis

111

what needs to be produced by an osteosarcoma for diagnosis?

osteoid producion is essential for diagnosis

112

what are the 3 histological variants of conventional osteosarcoma?

osteoblastic
chondroblastic
fibroblastic

113

what is the treatment of an osteosarcoma?

biopsy, CT, bone scan
pre-op chemo
surgical resection
post-op chemo

114

what tumour is composed of malignant chondrocytes?

chondrosarcoma

115

which is the most common primary malignant tumour of bone?

osteosarcoma

116

what is the treatment of a chondrosarcoma?

wide surgical excision

117

what are the 3 main types of malignant bone cancer?

osteosarcoma
chondrosarcoma
Ewing's Sarcoma

118

who tends to get Ewing's Sarcoma?

10 - 20 year olds
M > F

119

what is Ewing's Sarcoma?

a primary malignant tumour of the primitive cells in the bone marrw

120

where does Ewing's Sarcoma occur?

metpahysis and diaphysis of:
1. femur
2. tibia
3. humerous

121

what is the treatment of Ewing's Sarcoma?

surgery
radiation therapy
chemotherapy
post-op adjuvant chemo

122

what is the name of the tumour caused by malignant proliferation of plasma cells in bone marrow?

multiple myeloma

123

who tends to get multiple myeloma?

elderly

124

what is the usual result of multiple myeloma?

bone destruction of axial skeleton

125

what is more common: primary bone tumours or secondary bone tumours?

secondary mets

126

what is the most common benign bone tumour?

osteochondroma

127

what type of inheritance is the hereditary disorder which causes multiple osteochondromas?

autosomal dominant

128

what are enchondromas caused by?

failure of normal endochondral ossification at the growth plate

129

why can enchondromas lead to pathological fractures?

because even though they are usually asymptomatic, they weaken the bone

130

what is the treatment for an enchondroma which has caused a fracture or is threatening to cause a fracture?

curettage (once one has healed)
+ filled with bone graft to strengthen bone

131

what is a simple bone cyst?

a single cavity, fluid filled, benign cyst in a bone

132

where are simple bone cysts generally found?

metaphysis of long bones particularly proximal femur and humerous
can be found in talus or calcaneous

133

why can simple bone cysts lead to pathological fractures?

because they weaken the bone

134

what is the treatment for a simple bone cyst?

curettage
+ filled with bone graft to strengthen

135

what is the probable cause of a simple bone cyst?

developmental defect

136

what is an aneurysmal bone cyst?

multiple fluid-filled chambers within a bone which contain blood or serum

137

what is the probable cause of an aneurysmal bone cyst?

small arteriovenous malformation

138

where do aneurysmal bone cysts occur?

metaphyses of long bones, flat bones and vertebral bodies

139

why do aneurysmal bone cysts cause pain?

because they are locally aggressive so cause cortical expansion and destruction

140

what is the treatment of an aneurysmal bone cyst?

curettage
+ filled with bone graft to strengthen

141

why are giant cell tumours of the bone painful?

destruction of cortex

142

despite being classified as benign, what typically malignant feature do they have?

can metastasise

usually causing benign pulmonary giant cell tumours

143

what is the characteristic sign of a giant cell tumour of the bone on an x-ray?

soap bubble appearance

144

what is the name of the genetic condition which results in lesions of fibrous tissue within bone and immature bone?

fibrous dysplasia

145

when does fibrous dysplasia tend to occur?

adolescence

146

compare monostatic fibrous dysplasia to polystatic fibrous dysplasia?

monostatic fibrous dysplasia- affects only one bone
polystatic fibrous dysplasia- affects more than one bone

147

what deformity is typical of fibrous dysplasia if there is extensive involvement of the proximal femur?

shepherd's crook deformity

148

what can be used to reduce pain in fibrous dysplasia?

biphosphates

149

what should be done to treat pathological fractures caused by fibrous dysplasia?

internal fixation (for stability) and cortical bone graphs (for strength)

150

how do you treat osteoid osteomas?

many resolve spontaneously
others require CT guided radio-frequency alation or en bloc resection

151

what are the red flag symptoms for bone cancer?

constant pain
worse at night
systemic symptoms (eg weight loss, loss of appetite, fatigue)

152

are osteosarcomas radiosensitive?

no

153

where are chondrosarcomas likely to be found?

pelvis
proximal femur

154

compare osteosarcoma and chondrosarcoma in terms of mets rate??

osteosarcoma metastasizes faster than chondrosarcoma

155

are osteosarcomas responsive to adjuvant chemotherapy?

yes

156

are chondrosarcomas radiosensitive?

no

157

are chondrosarcomas responsive to adjuvant chemotherapy?

no

158

what are fibrosarcomas and malignant fibrous histiocytma?

fibrous malignant primary bone tumours which tend to occur in abnormal bone (eg bone infarcts, fibrous dysplasia and pagets disease)

159

which malignant bone cancer has the poorest prognosis?

Ewing's Sarcoma

160

are Ewing's Sarcomas radiosensitive?

yes

161

are Ewing's Sarcomas responsive to chemotherapy adjuvants?

yes

162

what is neo-adjuvant chemotherapy?

chemotherapy given before surgery

163

What are the 5 most common primary malignant tumours which commonly metastasize to bone?

1. breast carcinoma
2. prostate carcinoma
3. lung carcinoma
4. renal cell carcinoma
5. thyroid adenocarcinoma

164

what is the most common benign soft tissue tumour?

lipoma

165

what is a lipoma?

a neoplastic proliferation of fat

166

where do lipomas occur?

usually in the subcutaneous tissue,
can occur in the muscle

167

what is an angiosarcoma?

malignant tumour that arises from blood vessels

168

what is a liposarcoma?

malignant tumour that arises from fat

169

what is a rhabdomyosarcoma?

a malignant tumour that arises from skeletal muscle

170

what is a synovial sarcoma?

a malignant tumout that arises from the synovial lining of joints or tendons

171

what is a ganglion cyst?

a synovial-fluid-filled swelling that occurs around a synovial joint or synovial tendon sheath
(outpouching of synovium lining)

172

why do ganglion cysts form?

as a result of herniation or out-pouching of a weak portion of joint capsule of tendon sheath

173

what is a juvenile Baker's cyst?

a ganglion cyst caused by a developmental weakness

174

what is an adults Baker's cyst?

a ganglion cyst caused by a build up of pressure within the joint capsule

175

what is a bursa?

a small fluid filled sac lined by synovium around a joint which prevents friction between tendons, bones, muscles and skin

176

what can cause bursitis?

repeated pressure/trauma
bacterial infection
gout

177

how do you treat an abscess?

incision and drainage
will not resolve with antibiotics alone

178

what similar end result to osteochondritis and avascular necrosis share?

an area of bone undergoes localised necrosis

179

what is avascular necrosis?

ischaemic necrosis of bone
(predominantly in adults)

180

why can alcoholism, steroid abuse and primary hyperlipidaemia lead to avascular necrosis?

alcoholism and steroid abuse alter fat metabolism and can result in mobilisation of fat into the circulation which can cause ischaemia
primary hyperlipidaemia causes fat to be in circulation as well

181

what is the treatment of early avascular necrosis? (ie before articular surface has collapse)

drilling under fluoroscopy to decompress bone

(prevents further necrosis and helps healing)

182

what is the treatmend of late stage avascular necrosis? (ie articular surface has collapsed)

joint replacement

183

what does the modified beighton score clinically assess?

if the patient has joint hypermobility syndrome

184

what do patients with joint hypermobility syndrome usually present with? (if no underlying genetic syndrome)

arthralgia
premature osteoarthritis

185

what are the 5 signs of inflammation?

rubor (red)
calor (hot)
dolor (pain)
tumor (swelling)
functio laesa (loss of function)

186

what is sequestrium?

dead and dying bone separated from normal bone
(a complication of osteomyelitis)

187

what is involucrum?

new bone forming on the outside, just deep to the periosteum
(a complication of osteomyslitis)

188

why is pus within a joint an emergency?

pus destroys articular cartilage

189

why is needle aspiration of a ganglion cyst not recommended?

recurrence is common

190

what is usually the first clinical sign of hp patholgoy?

loss of internal rotation

191

what do patients with avascular necrosis of the femoral head present with?

groin pain

192

what are the late signs of avascular necrosis of the femoral head on radiography?

sclerotic changes
hanging rope sign
irregularity of articular surface

193

why does a lytic zone form at the neck of a femoral head damaged by avascular necrosis?

lytic zone formed by granulation tissue (attempted repair)

194

what sign on x-ray suggest avascular necrosis and is due to the lytic zone?

hanging rope sign

195

what is morton's neuroma?

irritation of the plantar interdigital nerves (due to repeated trauma) causing neuromas to form

196

what are neuromas?

swollen and inflamed nerves

197

what do patients with morton's neuroma present with?

burning pain and tingling radiating into affected toes

198

who is more likely to get morton's neuroma- M or F?

females

199

which nerves are most commonly affected by morton's neuroma?

3rd interspace nerve is the most common followed by the 2nd interspace nerve

200

what test assesses for morton's neuroma?

mulders test

201

what is mudlers test?

squeezing the forefoot with your hand
in a positive test symptoms may be reproduced or there will be a characteristic click

202

what is used for diagnosis of morton's neuroma?

Ultrasound

203

what is the conservative management of morton's neuroma?

metatarsal pad or insole
steroid and local anaethetic injections

204

what is myositis ossificans?

ossification within a muscle after injury (can be after surgery)

205

what is pes planus?

loss of the medial longitudinal arch of the foot- flat foot

206

what is the ligament in the ankle which is most commonly sprained?

ATFL
(anterior talofibular ligament)

207

what test assesses whether flat footedness is flexible or stiff?

jacks test- push big toe up

208

what tendon dysfunction is the most common cause of acquired flatfoot deformity?

tendon of posterior tibialis

209

what is the primary dynamic stabiliser of the medial longitudinal arch?

tibialis posterior tendon

210

who tends to get tibialis posterior dysfunction?

obese middle aged females

211

what pain and swelling is very typical to tibialis posterior dysfunction?

pain/swelling posterior to medial malleolus

212

what can happen to the big toes in tibialis posterior dysfunction?

can move laterally (hallux valgus)

213

what type of surfaces do patients with tibialis posteiror dysfunction not life?

uneven surfaces

214

what can happen to the heel of the foot in tibialis posterior dysfunction?

heel goes into valgus

215

what can happen to the heel of the foot of a patient with pes cavus?

moves into varus

216

what are the 4 main causes of plantar fasciitis?

-physical overload (excessive exercise or weight)
-seronegative arthropathy
-diabetes
-abnormal foot shape

217

what is the main treatment of plantar fasciitis?

conservative:
NSAIDs
night splint + taping
physio
steroid injections

218

how long does plantar fasciitis take to self-limit?

18-24 months

219

how does the incidence of hallux valgus change with age?

increases with age

220

is hallux valgus mainly unilateral or bilateral?

bilateral

221

what are the 5 main problems of hallux valgus?

transfer metatarsalgia
lesser toe impingement
pain
cosmesis of deformity
shoe difficulties

222

why does hallux valgus cause transfer metatarsalgia?

loss of function of big toe
lesser toes are used for power
these toes eventually become sore

223

what is hallux rigisus?

OA of 1st MTP joint

224

what is the commonest area for morton's neuroma and why?

between 3rd and 4th digit (because these are very mobile so are constantly rubbing against each other)

225

what is the first line treatment for morton's neuroma?

steroid injections

226

what type of condition is curly toes?

paediatric condition

227

if a swelling is 'fluctuant', what type of material is within it?

semi-solid material

228

what is an abscess?

a discreet collectin og pus

229

what is the management of bursitis? (which hasn't become secondarily affected)

NSAIDs
analgesia

230

what is the management of bursitis that has become secondarily affected?

incision and drainage
antibiotics

231

how many bursae are around the knee?

4 bursae

232

what actually is the bunion on the medial side of the foot formed by hallux valgus?

an inflamed bursa

233

why do bouchards and heberdens nodes occur?

chronic trauma

234

what arthritis are bouchards nodes present in?

OA or RA

235

what arthritis are heberdens nodes present in?

OA

236

what is myositis ossificans?

ossificaiton (calcification) of a muscle haematoma

(outside the bone, in a muscle)

237

what usually happens before myositis ossificans?

large trauma (haematoma occurs)

238

when can you intervene with myositis ossificans?

only if symptoms demand
and only once the ossification has matured (otherwise risk of recurrence)

239

what joints are most commonly affected by OA?

DIP joints

240

what are mucous cysts?

outpouching of synovial fluid from DIP joints

241

what condition are mucous cysts associated with?

osteoarthrtis

242

what is DeQuervain's tendonitis?

tendonitis of the tendons which control the thumb

243

when is ficklesteins test positive?

in Dequervain's tendonitis

244

in dupuytren's contractures what do the fibroblasts change in to?

myofibroblasts

245

what simple test is a way to determine if the dupuytren's contractures are severe enough to require surgery?

table top test

246

what is paronychia?

infection within the nail fold (essentially an abscess)

247

wat is tendinopathy?

disease of a tendon

248

what is tendonitis?

acute tendon injury accompanied by inflammation

249

what is tendonosis?

chronic tendon injury with damage to tendon at cellular level

250

what is tenosynovitis?

inflammation of the tendon sheath

251

what is enthesopathy?

inflammation of the tendon origin or the insertion