Pathology of Soft Tissues

Question 1

Duchenne inheritance

Answer 1

X-linked recessive

Question 2

Becker inheritence

Answer 2

X-linked recessive

Question 3

Limb girdle inheritance

Answer 3

Autosomal recessive

Question 4

Fascioscapulohumeral inheritance

Answer 4

Dominant

Question 5

Scapulohumeral inheritance

Answer 5

Autosommal recessive

Question 6

Oculopharyngeal inheritance

Answer 6

Dominant

Question 7

Myotonic dystrophy inheritance

Answer 7

Dominant

Question 8

Mutations in dystrophin gene on long arm chromosome X

Alterations in anchorage of actin cytoskeleton to basement membrane

Fibres liable to tearing

Uncontrolled Ca2+ entry into cells

Answer 8

DMD

Question 9

Muscle weakness
Myotonia
Non-muscle features (frontal balding, cardiomyopathy, low intelligence)

Answer 9

Myotonic Dystrophy

Question 10

Atrophy of type 1 fibres

Central nuclei

Ring fibres

Fibre necrosis

Fibrofatty replacement

Answer 10

Myotonic dystrophy

Question 11

Endomysial lymphocytic infiltrate, invasion of muscle by CD8 + T lymphocytes

Answer 11

Polymyositis

Question 12

T-cells in polymyositis?

Answer 12

CD8+

Question 13

Upper body erythema, swelling of eyelids with purple discolouration

Answer 13

Polymyositis

Question 14

Weakness
Proptosis
Fatigue
Dysphagia

Answer 14

Myasthenia Gravis

Question 15

Classical triad of rhabdomyolysis?

Answer 15

Myalgias
Generalised weakness
Darkened urine

Question 16

Myoglobinuria
Hyperkalaemia
Necrosis & shock

Answer 16

Rhabdomyolysis
Outcome:
Acute Renal Failure (breakdown products of skeletal muscle and myoglobin are harmful to the kidneys)

Hypovolaemia & hyperkalaemia

Metabolic acidosis

Disseminated intravascular coagulation

Question 17

Outcome of rhabdomyolysis?

Answer 17

Acute renal failure
Hypovolaemia
Hyperkalemia 
Metabolic acidosis
Disseminated intravascular coagulation

Question 18

Drugs which can cause SLE?

Answer 18

Hydralazine

Pracainamide

Question 19

Visceral lesions of SLE which type of hypersensitivity?

Answer 19

Type 3

Question 20

Haematological effects of SLE mediated by which type of sensitivity?

Answer 20

Type II

Question 21

Clinical features of PAN

Answer 21

Clinical features
Non-specific +/- organ specific features such as hypertension, haematuria, abdominal pain, melaena, diarrhoea, mononeuritis multiplex, rash, cough, dyspnoea

Question 22

Clinical features
Non-specific +/- organ specific features such as hypertension, haematuria, abdominal pain, melaena, diarrhoea, mononeuritis multiplex, rash, cough, dyspnoea

Answer 22

PAN, lol

Question 23

Biopsy; fibrinoid necrosis of vessels

Serum contains pANCA (perinuclear antineutrophil cytoplasmic autoantibody)

Answer 23

PAN

Question 24

Biopsy findings of PAN

Answer 24

Fibrinoid necrosis

pANCA

Question 25

Causes of death because of scleroderma

Answer 25

Renal failure secondary to malignant hypertension Severe respiratory compromise Cor pulmonale Cardiac failure or arrhythmias secondary to myocardial fibrosis

Question 26

Scleroderma and fibrosis of organs

Answer 26

Excessive fibrosis of organs and tissues (excessive collagen production) Skin – tight, tethered,  joint movement GI tract – fibrous replacement of muscularis Heart – pericarditis and myocardial fibrosis Lungs – interstitial fibrosis Kidneys – affects arteries, leads to hypertension Musculoskeletal system – polyarteritis and myositis

Question 27

Crest syndrome

Answer 27

``` Calcinosis Raynaud’s Oesophageal dysfunction Sclerodactyly Telangectasia ```