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K1s Path > Histopathology > Flashcards

Histopathology Flashcards

1
Q

Definition of Atherosclerosis

A

an arteriosclerosis characterized by atheromatous deposits in and fibrosis of the inner layer of the arteries: intimal lesions

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2
Q

Major risk factors for atherosclerosis

A 
Age 
Gender
Genetics
Hyperlipidaemia
Hypertension
Smoking
Diabetes Mellitus
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3
Q

What are the 3 stages of atheromatous plaque formation?

A

1 - Raised lesion
2 - Soft lipid core
3 - White fibrous cap

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4
Q

How does gender affect risk of artherosclerosis?

A

Premenopausal women protected (HRT no protection)

Postmenopausal risk increases (older ages greater than men)

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5
Q

Other (not major) risk factors for atherosclerosis

A 
Inflammation
Hyperhomocyteinaemia
Metabolic syndrome
Lipoprotein (a)
Haemostasis (procoagulation)
Lack of exercise
Stress
Obesity
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6
Q

What are the pathological steps of the response to injury hypothesis?

A
  • Endothelial injury
  • Lipoprotien accumulation (LDL)
  • Monocyte adhesion to endothelium
  • Monocyte migration into intima -> macrophages & foam cells
  • Platelet adhesion
  • Factor release
  • Smooth muscle cell recruitment
  • Lipid accumulation -> extra & intracellular, macrophages & smooth muscle cells
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7
Q

What is the earliest for of atheroslerotic lesion

A 
Fatty streak
Lipid filled foamy macrophages
No flow disturbance
In virtually all children >10yrs
Relationship to plaques uncertain
Same sites as plaques
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8
Q

What are the 3 principle components of an atherosclerotic plaque?

A

Cells - including smooth muscle cells, macrophages and leukocytes
Extra Cellular Matrix - including collagen
Intracellular and extracellular lipid

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9
Q

When cardiac demand becomes greater than arterial supply, this is know as?

A

Critical stenosis
Occurs at ~70% occlusion (or diameter <1mm)
Causes “stable” angina

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10
Q

In what kind of plaque does acute change prodominantly occur and what kind of change can happen?

A

Majority of plaques that show acute change show only mild to moderate luminal stenosis prior to acute change.

1) Rupture – exposes prothrombogenic plaque contents
2) Erosion - exposes prothrombogenic subendothelial basement membrane
3) Haemorrhage into plaque – increase size

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11
Q

What are the characteristics of a vulnerable plaque?

A

Lots foam cells or extracellular lipid
Thin fibrous cap
Few smooth muscle cells
Clusters inflammatory cells

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12
Q

How does IHD present?

A

Angina pectoris
Myocardial infarction
Chronic IHD with heart failure
Sudden cardiac death.

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13
Q

What is the pathogenesis of IHD

A

insufficient coronary perfusion relative to myocardial demand due to chronic progressive atherosclerotic narrowing of epicardial coronary arteries and variable degrees of superimposed plaque change, thrombosis and vasospasm

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14
Q

What is Prinzmetal angina?

A

Chest pain caused by coronary vasospasm

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15
Q

What is the histological evolution of and MI?

A

< 6 hours - normal histology (CK-MB also normal)
6-24 hrs - loss of nuclei, homogenous cytoplasm, nerotic cell death
1-4 days - infiltration of polymorphs then macrophages (clear up debris)
5-10 days - removal of debris
1-2 weeks - granulation tissue, new blood vessels, myofibroblasts, collagen synthesis
Weeks-months - strengthening, decellularising scar

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16
Q

What % of MI’s are asymptomatic and who gets them?

A

10-15%

common in elderly and diabetics

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17
Q

What factors predict a worse prognosis from MI?

A

Older age
Female
DM
Previous MI

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18
Q

What are the main complications of an MI?

A
  • Contractile dysfunction: cardiogenic shock
  • Arrhythmias
  • Myocardial rupture
  • Pericarditis: Dressler syndrome
  • RV infarction
  • Infarct extension - new necrosis adjacent to old
  • Infarct expansion - ventricular aneurysm
  • Mural thrombus - embolisation
  • Papillary muscle rupture - valve disfunction
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19
Q

How long after a MI does myocardial rupture usually occur?

A

Mean 4-5 days, range 1-10 days

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20
Q

What is sudden cardiac death?

A

Unexpected death from cardiac causes in individuals without symptomatic heart disease or early (1hr) after onset of symptoms

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21
Q

What causes sudden cardiac death?

A

Acute myocardial ischaemia triggers lethal arrhythmia on a background of asympotmatic IHD. MI usually causes electrical instability at sites distant from conduction system often near scars from old MIs
10% have non-atherosclerotic causes (e.g. long QT)

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22
Q

What causes nutmeg liver?

A

Right-sided heartfailure due to increased pressure

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23
Q

What are the causes of cardiac failure?

A 
Ischaemic heart disease
Valve disease
Hypertension
Myocarditis
Cardiomyopathy
Left sided heart failure causes Right failure
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24
Q

Complications of cardiac failure

A

Sudden Death
Arrhythmias
Systemic emboli
Pulmonary oedema with superimposed infection

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25
Q

What are the histological findings of cardiac failure?

A

Macroscopically - dilated ventricles with scarring and thinning of walls
Microscopically - fibrosis and replacement of ventricular myocardium

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26
Q

What are the different patterns of cardiomyopathy?

A 
Too thin (dilated)
Too thick (hypertrophic)
Too stiff (restrictive)
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27
Q

What are the causes of dilated cardiomyopathy?

A 
Caused by a progressive loss of mycocytes.
Idiopathic
Infective - myocarditis
Toxic - alcohol, chemotherapy
Hormonal - thyroid, DM, peripartum
Genetic - haemochromatosis
Immunological - myocarditis, sarcoidosis
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28
Q

What are the causes of hypertrophic cardiomyopathy (HOCM)?

A

50% familial (AD) beta-myosin heavy chain mutation.
Leading cause of sudden cardiac death in young athletes.
Left ventricular hypertrophy without dilation narrows left ventricular outflow tract.

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29
Q

What are the causes of restrictive cardiomyopathy?

A

Impaired ventricular compliance.
Idiopathic
Secondary to amyloidosis, sarcoidosis, radiation-induced fibrosis.

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30
Q

In what order does chronic rheumatic valvular disease affect the different heart valves?

A

Mitral > Aortic > Tricuspid > Pulmonic

Predominately left-sided, 48% mitral alone.

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31
Q

What is the commonest causes of aortic stenosis?

A

Calcific aortic stenosis, calcium deposits in valve impairs opening causing outflow tract obstruction.

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32
Q

What are the causes of aortic regurgitation?

A 
Rigidity - rheumatic, degenerative 
Destruction - microbial endocarditis 
Disease of aortic valve ring causing dilation; 
- Marfan's Syndrome 
- Dissecting aneurysm
- Syphilitic aortitis 
- Ankylosing spondylitis
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33
Q

Examples of drugs which can induce SLE

A

Hydralazine and procainamide

Drug-induced SLE rarely affects kidneys and is associated with anti-histone antibodies

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34
Q

What characteristic histology is seen in the blood with SLE?

A

LE cells - a neutrophil or macrophage that has phagocytized the denatured nuclear material of another cell - LE body

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35
Q

What are the diagnostic criteria for SLE?

A 
4 out of 11 of;
Serositis (pericarditis, pleuritis)
Oral ulcers
Arthritis
Photosensitivity
Blood disorders (AIHA, ITP)
Renal involvement
ANA +ve
Immune phenomena (dsDNA, anti-sm etc)
Neuro symptoms
Malar rash
Discoid rash
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36
Q

Which antibodies are associated with Sjögren’s syndrome?

A

Anti-Ro

Anti-La

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37
Q

How can SLE affect the heart valves?

A

Libman–Sacks endocarditis is a form of nonbacterial (sterile) endocarditis that is seen in SLE. It is one of the most common cardiac manifestations of lupus (most common = pericarditis).

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38
Q

Which autoantibodys are associated with systemic scleroderma?

A

Limited (CREST) = anti-centromere

Diffuse = Anti Scl-70 (DNA topoisomerase)

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39
Q

What does CREST stand for?

A 
Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly
Telagiectasia
Also associated with Pulmonary hypertension
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40
Q

What is a form of scleroderma localised to the skin also know as?

A

Morphoea

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41
Q

What are the characteristics of systemic scleroderma?

A
  • Fibrosis and accumulation of collagen in skin and organs
  • Vascular alterations: ‘onion skin’ intimal thickening of small arteries
  • Autoantibodies
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42
Q

What is the skin involvement in limited systemic scleroderma?

A

Face and distal to elbows and knees.

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43
Q

What are polymyositis and dermatomyositis associated with?

A

25-50% have underlying malignancy, male predominance. Bronchus, breast and GI malignancy most commonly.

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44
Q

What are the signs and symptoms of polymyositis and dermatomyositis?

A
  • Proximal muscle weakness, raised CK & abnormal EMG

- DM also has heliotrope rash and Gottron’s papules

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45
Q

Which autoantibody is associated with dermatomyositis?

A

Anti-Jo-1 (tRNA synthetase)

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46
Q

What does a young child with a week long fever, eythematous palms and soles, conjunctivitis and a swollen tongue have?

A

Kawasaki’s disease

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47
Q

What are the clinical features of Kawasaki’s disease?

A 
Affects children < 5 yrs
High fever > 5 days
plus 4 of;
- non-purulent conjunctivitis
- red mucous membranes (strawberry tongue)
- cervical lymphadenopathy
- rash (polymorphous)
- red, oedematous palms and soles or later desquamation
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48
Q

What possible complications are there with Kawasaki’s disease?

A

Coronary artery aneurysms, MI and sudden death.

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49
Q

What is the treatment for Kawasaki’s disease?

A

IVIG within first 10 days reduces risk of coronary artery aneurysms.
Aspirin reduces thrombosis risk.
Self-limiting

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50
Q

Which infection is associated with polyarteritis nodosa?

A

30-40% have underlying Hepatitis B infection

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51
Q

Which organs are commonly affected in polyarteritis nodosa?

A 
Any organ but most common are:
kidneys (80%)
heart (70%)
liver (65%)
GIT (50%)
Spares lungs
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52
Q

What are the clinical features of polyarteritis nodosa?

A 
PUO, weight loss 
Muscle aches
Neuropathy 
Haematuria, renal failure
Hypertension 
Abdominal pain, melaena
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53
Q

What might be the diagnosis in a patient with features of polyarteritis nodosa + lung involvement and an eosinophilia?

A

Churg-Strauss syndrome

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54
Q

What are the histological features of polyarteritis nodosa?

A

Rosary sign (beading)
Nodular appearance to medium sized vessels on angiography - microaneurysms
Microscopically necrotising arteritis with inflammation

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55
Q

What is the commonest form of arteritis?

A

Temporal arteritis

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56
Q

What type of hypersensitivity reaction is temporal arteritis?

A

Type IV reaction to vessel wall components

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57
Q

What is amyloid?

A

Deposition of an abnormal proteinaceous substance in non branching fibrils.
Beta-pleated sheet structure.
Resistant to enzymatic degradation.
Variety of different proteins can be involved.

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58
Q

What is the most common form of amyloidosis?

A

AL (light-chain) amyloidosis, associated with multiple myeloma. Bence-Jones protein in blood and urine.

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59
Q

What is AA amyloidosis?

A

Serum Amyloid A (SAA) acute phase protein deposited as amyloid, often secondary to chronic infections / inflammation.

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60
Q

What type of amyloid is deposited in Alzheimer’s disease?

A

Amyloid-beta (Aβ)

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61
Q

What is always present in amyloid?

A

Serum amyloid P component (SAP), ~10%

Pentagonal constituent of amyloid deposits

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62
Q

How can amyloid be visualised in a tissue sample?

A

Stains with Congo red dye.

Shows apple green birefringence under polarised light.

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63
Q

What are the common clinical features of amyloidosis?

A 
Kidney: nephrotic syndrome
Heart: conduction defects, restrictive cardiomyopathy, heart failure
Hepatosplenomegaly
Macroglossia
Neuropathies: e.g. carpal tunnel
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64
Q

Which type of amyloidosis is associated with haemodialysis?

A

Beta-2-microglobulin amyloid deposition

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65
Q

What is the most common form of familial amyloidosis?

A

Familial Mediterranean Fever (autoinflammatory disease) - AA amyloid deposition

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66
Q

What is sarcoidosis?

A

Multisystem disorder based on a cell mediated immune response and characterised by non-caseating granulomas in many tissues

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67
Q

Which organ systems are characteristically involved in sarcoidosis?

A

Skin, lymphoid system and lungs (most common)

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68
Q

How can sarcoidosis present in the skin?

A

Erythema nodosum
Lupus pernio
Skin nodules

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69
Q

How can sarcoidosis affect the eyes?

A

Uveitis
Uveoparotitis - bilateral uveitis, parotid enlargement +/- facial nerve palsy.
Retinal inflammation

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70
Q

What systemic effects are there with sarcoidosis?

A

Hypergammaglobulinaemia
Raised serum ACE
Hypercalcaemia due to increased activation of Vit D by granulomas

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71
Q

What histological findings are there when looking at a sacroid granuloma?

A

Non-caeseating granuloma with Schaumann and asteroid bodies

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72
Q

Definition of neurodegenerative disease

A

Progressive, irreversible conditions that lead to neuronal loss – often caused by intra or extracellular accumulation of a misfolded protein; usually sporadic (less than 10% genetic); lead to dementia.

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73
Q

Definition of dementia

A

A serious loss of global cognitive ability in a previously unimpaired person, beyond what might be expected from normal ageing. It may be static, the result of a unique global brain injury, or progressive due to neurodegeneration

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74
Q

What are the pathological correlates of neurodegeneration?

A 
Macroscopically: brain atrophy (diffuse or selective)
Microscopically: often misfolded protein
- neuronal loss
- damage of synapses (early)
- exocytotoxicy (excess of NTs)
- microglial activation
- reactive astrocytosis
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75
Q

What is a Lewy body?

A

Abnormal aggregates of protein (mostly alpha-synuclein) that develop inside nerve cells in Parkinson’s disease (PD), Lewy body dementia and some other disorders.

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76
Q

How does multiple sclerosis usually present?

A

In patients 20-40 yrs old with focal symptoms, optic neuritis and poor coordination.

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77
Q

How is Multiple sclerosis classified?

A 
Primary progressive (10% - get continually worse)
Relapsing remitting (better between episodes but progresses over years)
Rare variants (neuromyelitis optica (Devic disease), Marburg disease, Balo disease)
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78
Q

What can cause demyelinating lesions?

A
  • Viral infections (PML, HIV)
  • Genetic (Leukodystrophies)
  • Autoimmune (multiple sclerosis, acute haemorrhagic encephalomyelitis, acute disseminated encephalomyelitis)
  • Nutritional/metabolic (central pontine myelinolysis, B12 deficiency)
  • Toxic (radiotherapy, chemical agents)
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79
Q

How does multiple sclerosis appear histologically?

A

MS plaques showing sharp margins of myelin loss with central veins.
Inactive plaques = glial scar

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80
Q

Which pathological proteins are associated with Alzheimer’s disease?

A

Tau and Beta-amyloid

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81
Q

What is bone made of?

A

INORGANIC - 65%
- calcium hydroxyapatite (10Ca 6PO4 OH2)
ORGANIC - 35%
- bone cells and protein matrix

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82
Q

What two main types of bone are there and give examples?

A

Cortical: long bones e.g. femur 80-90% calcified
Cancellous: vertebrae & pelvis 15-25% calcified

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83
Q

Which type of bone cell is multinucleate?

A

Osteoclast

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84
Q

What does RANK stand for?

A

Receptor Activator for nuclear factor kB

Important for osteoclastogenesis

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85
Q

Which cell type expresses RANK ligand and RANK?

A

RANK ligand: stromal cell / osteoblast

RANK: osteoclast precursor and osteoclast

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86
Q

Definition of metabolic bone disease

A

Disordered bone turnover due to imbalance of various chemicals in the body (vitamins, hormones, minerals etc)
Overall effect is reduced bone mass (osteopaenia) often resulting in fractures with little or no trauma

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87
Q

What are the three main categories of metabolic bone disease?

A
  1. Non-endocrine (e.g. age related osteoporosis)
  2. Related to endocrine abnormality (Vit D; Parathyroid hormone)
  3. Disuse osteopaenia
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88
Q

What is the usual site for a bone biopsy?

A

Iliac crest

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89
Q

What are the causes of osteoporosis?

A

1º - age, post-menopause
2º - drugs, systemic disease
90% cases due to insufficient Ca intake and post-menopausal oestrogen deficiency

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90
Q

What DEXA scan scores define osteoporosis?

A

T score >2.5 SD below normal peak bone mass = osteoporosis

T score between 1 & 2.5 SD below normal peak bone mass = osteopaenia

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91
Q

What is the effect of increased PTH secretion on the kidenys and bone?

A

Kidney: stimulates Ca reabsorption & VitD activation
Bone: Stimulates osteoclasts hence resorption

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92
Q

What are the different types of osteomalacia?

A
  1. Deficiency of vitamin D
  2. Deficiency of PO4
    Osteomalacia = Defective bone mineralisation
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93
Q

What type of fractures can be seen in osteomalacia?

A

Horizontal fractures / pseudofractures, also called Looser’s zones.

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94
Q

Symptoms of hyperparathyroidsm/hypercalaemia

A

Stones (Ca oxalate renal stones)
Bones (osteitis fibrosa cystica, bone resorption)
Abdominal groans (acute pancreatitis)
Psychic moans (psychosis & depression)

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95
Q

What underlying changes occurs in renal osteodystrophy?

A 
PO4 retention – hyperphosphataemia
Hypocalcaemia as a result of decreased vit D
2o hyperparathyroidism
Metabolic acidosis
Aluminium deposition
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96
Q

What are the different phases of Paget’s disease?

A
  1. Osteolytic
  2. Osteolytic-osteosclerotic
  3. Quiescent osteosclerotic
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97
Q

What is the clinical presentation of Paget’s disease?

A

Pain, microfractures, nerve compression (incl. Spinal N and cord)
Skull changes may put medulla at risk
+/- haemodynamic changes, cardiac failure
Development of sarcoma in area of involvement 1%

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98
Q

What type of patients usually get Paget’s disease?

A

Onset > 40y
M=F
Rare in Asians and Africans

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99
Q

What is Hirschsprung’s disease associated with?

A

Down’s syndrome and RET proto-oncogene Cr10

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100
Q

What would a biopsy of affected bowel in Hirschsprung’s disease show?

A

Hypertrophied nerve fibres but no ganglia

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101
Q

Which segments of bowel are prone to volvulus in the young and elderly?

A

Infants - small bowel

Elderly - sigmoid colon

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102
Q

What is pseudomembranous colitis?

A

Acute, antibiotic associated colitis with pseudomembrane formation, caused by protein exotoxins of C. difficile

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103
Q

Non-caseating granulomas in the bowel are found with which disease?

A

Crohn’s disease

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104
Q

What are the histological features of Crohn’s disease?

A 
Whole GI tract can be affected
Skip lesions
Transmural inflammation
Non-caseating granulomas
Sinus/fistula formation
Cobblestone mucosa
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105
Q

What are the extra-intestinal manifestations of Crohn’s disease?

A

Arthritis
Uveitis
Stomatitis/cheilitis
Skin lesions: pyoderma gangrenosum, erythema multiforme, erythema nodosum

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106
Q

What major complications are there with ulcerative colitis?

A

Severe haemorrhage
Toxic megacolon
Adenocarcinoma 20-30 x increased risk

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107
Q

What are the extra-intestinal manifestations of ulcerative colitis?

A 
Arthritis
Myositis
Uveitis/iritis
Erthema nodosum, pyoderma gangrenosum
Primary sclerosing cholangitis
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108
Q

What different types of colorectal polyps are there?

A

Non-neoplastic: Hyperplastic, inflammatory (pseudo-polyps), hamartomatous (juvenile, Peutz Jeghers)
Neoplastic: Tubular adenoma, Tubulovillous adenoma, Villous adenoma

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109
Q

What are the risk factors for cancer with regard to bowel adenomatous polyps?

A
  • Size
  • Proportion of villous component
  • Degree of dysplastic change within polyp (low or high)
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110
Q

What is familial adenomatous polyposis coli?

A

AD mutation in APC tumour suppressor leading to 1000’s of adenomatous polyps by 25, ~100% develop cancer within 10-15 years

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111
Q

Which gene causes FAP and where is it located?

A

APC, chromosome 5q21

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112
Q

What is Gardner’s syndrome?

A

Same clinical, pathological and etiological features and Ca risk as FAP but with extra-intestinal manifestations.

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113
Q

What extra-intestinal manifestations are there with Gardner’s syndrome?

A
  • Multiple osteomas of skull and mandible
  • Epidermoid cysts
  • Desmoid tumours
  • Dental caries, unerrupted supernumery teeth
  • Post-surgical mesenteric fibromatoses
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114
Q

What is HNPCC?

A

Hereditary non-polyposis colorectal cancer.
AD mutation of mismatch repair genes leading to DNA replication errors.
3-5% of all colorectal cancers

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115
Q

Apart from bowel, what other cancers are associated with HNPCC?

A

Endometrium, prostate, breast, stomach.

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116
Q

What is the most common form of colorectal cancer?

A

98% adenocarcinoma

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117
Q

What are the symptoms of colorectal cancer?

A

Bleeding, change in bowel habit, anaemia, weight loss, pain, fistula.

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118
Q

What is the staging system for colorectal cancer?

A 
Dukes' staging
A - confined to wall of bowel
B - through wall (muscular layer) of bowel
C - lymph node metastases
D - distant metastases
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119
Q

What are the 4 key features of liver cirrhosis?

A
  1. whole liver involved
  2. fibrosis
  3. nodules of regenerating hepatocytes
  4. distortion of liver vascular architecture (shunting of blood
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120
Q

What are the causes of and histological changes in acute hepatitis?

A

Mainly caused by drugs and viruses.
Get ‘spotty necrosis’
Acute defined as lasting < 6 months

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121
Q

What are the main causes of chronic hepatitis?

A

Viruses
Drugs
Auto-immune

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122
Q

What do grade and stage relate to r.e. chronic hepatitis?

A

Severity of inflammation = grade

Severity of fibrosis = stage (most important)

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123
Q

What different classifications of liver inflammation are there?

A

Portal inflammation
Interface hepatitis (piecemeal necrosis)
Lobular inflammation

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124
Q

What are Mallory-Denk bodies?

A

An inclusion found in the cytoplasm of liver cells most common in alcoholic hepatitis and alcoholic cirrhosis

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125
Q

Is alcohol toxic to the liver?

A

Not directly, it is converted to acetaldehyde which is toxic, affects zone 3 hepatocytes most as they are most metabolically active

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126
Q

Is alcoholic liver cirrhosis macro- or micronodular?

A

Micronodular

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127
Q

What is the histological picture in primary biliary cirrhosis?

A

Bile duct loss associated with chronic inflammation (with granulomas)

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128
Q

What auto-antibody is specific for primary biliary cirrhosis?

A

Anti-mitochondrial antibodies

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129
Q

What is the histological picture in primary sclerosing cholangitis?

A

Periductal bile duct fibrosis (onion-skinning) and scarring causing cholestasis

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130
Q

Which disease increases the risk of cholangiocarcinoma?

A

Primary sclerosing cholangitis

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131
Q

What defect is there in haemochromatosis?

A

HFE gene mutation on chromosome 6 leads to unregulated gut iron absorption - ‘chocolate liver’

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132
Q

What is the difference between haemochromatosis and haemosiderosis?

A

Haemochromatosis - gut absorped iron deposited in hepatocytes leading to fibrosis
Haemosiderosis - RBC iron (repeat transfusions) stored in kuffer cells with no risk of fibrosis

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133
Q

What is the defect in Wilson’s disease?

A

AR gene mutation on chromosome 13 which prevents copper excretion into bile leading to accumulation in tissues (liver and CNS)

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134
Q

What auto-antibody is specific for autoimmune hepatitis?

A

Anti-smooth muscle actin antibodies

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135
Q

What is the defect in alpha-1-antitrypsin deficiency?

A

Not with the enzyme itself, but there is a failure to secrete the enzyme from hepatocytes leading to intra-cytoplasmic inclusions and hepatitis

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136
Q

What is the commonest liver cancer?

A

Metastatic liver adenocarcinoma

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137
Q

What different primary hepatic tumours are there?

A

From hepatocytes: hepatocellular carcinoma, hepatoblastoma
From bile duct cells: cholangiocarcinoma
From blood vessels: haemangiosarcoma

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138
Q

Does viral hepatitis cause macro- or micronodular cirrhosis?

A

Macronodular

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139
Q

What is inflammation / infection of the Fallopian tubes and ovaries called?

A

Fallopian tube: salpingitis

Ovary: oopheritis

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140
Q

Which organisms cause infections of the female genital tract but are not associated with any serious complications?

A

– Candida: Diabetes mellitus, oral contraceptives and pregnancy enhance development of infection
– Tichomonas vaginalis: protozoan
– Gardenerella: gram negative bacillus causes vaginitis

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141
Q

Which organisms are a major cause of infertility in females?

A

Chlamydia and Gonorrhoea

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142
Q

What complications can occur with mycoplasma infection of the female gential tract?

A

Spontaneous abortion and chorioamnionitis

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143
Q

What two main causes of PID are there?

A
  • lower genital tract infection spreading upwards (mucosal)

- secondary infection following abortion: starts in uterus (deeper tissues)

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144
Q

Which organisms cause PID secondary to abortion?

A

Staph, stept, coliform bacteria and clostridium perfringens

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145
Q

What complications can there be with PID?

A
  • Peritonitis
  • Intestinal obstruction due to adhesions
  • Bacteremia
  • Infertility
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146
Q

What complications can there be with salpingitis?

A
  • Plical fusion
  • Adhesions to ovary
  • Tubo-ovarian abscess
  • Peritonitis
  • Hydrosalpinx
  • Infertility
  • Ectopic pregnancy
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147
Q

What is the mean age of patients who get cervical cancer?

A

45-50 years

2nd most common cancer affecting women

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148
Q

What are the main risk factors for the cervical cancer?

A
  • Human Papilloma Virus - present in 95%
  • Many sexual partners
  • Sexually active early
  • Smoking
  • Immunosuppressive disorders
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149
Q

Which are the most common types of HPV that are associated with cancer and which cancers are they associated with?

A

16 and 18
Cervical cancer
Also, vulval, vaginal, penile, and anal cancer

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150
Q

Which are the most common types of low risk HPV and what do they causes?

A

6 and 11

Oral and genital warts

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151
Q

What main types of cervical carcinoma are there are what is the most common?

A

Squamous cell carcinoma

Adenocarcinoma (20% of all invasive cases)

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152
Q

What staging system is used for cervical cancer?

A

FIGO stage (International Federation of Gynecology and Obstetrics) also used for other gynecological cancers

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153
Q

How does HPV transform cells?

A

It expresses proteins E6 and E7 which bind to and inactivate P53 and Retinoblastoma gene (Rb), respectively.
This interferes with apoptosis and increases unscheduled cellular proliferation which contributes to oncogenesis.

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154
Q

What are the 2 distinct biological states of HPV infection?

A
  • Non productive or latent infection: infection can only be identified by molecular methods
  • Productive viral infection: has characteristic cytological and histological features
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155
Q

What are the screening intervals for the cervical screening program?

A 
25 = First invitation 
25-49 = 3 yearly 
50-64 = 5 yearly 
65+ = Only screen those who have not been screened since age 50 or have had recent abnormal tests
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156
Q

Which types of HPV does the cervical cancer vaccine protect against?

A

6, 11, 16, 18

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157
Q

What is the commonest uterine tumour?

A

Leiomyoma: smooth muscle tumour of myometrium aka fibroid. May be intramural, submucosal or subserosal. Malignant counterpart rare = Leiomyosarcoma

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158
Q

What causes endometrial hyperplasia?

A

A state of persistent oestrogen production e.g. PCOS, Granulosa cell tumours, etc.

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159
Q

What are the risk factors for endometrial carcinoma?

A

– Excessive oestrogen stimulation
– Nulliparity
– Obesity
– Diabetes mellitus

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160
Q

What are type I endometrial carcinomas?

A

Endometrioid, mucinous and secretory adenocarcinomas
Younger age
Oestrogen dependent
Often associated with atypical endometrial hyperplasia
Low grade tumours, superficially invasive

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161
Q

What are type II endometrial carcinomas?

A 
Serous and clear cell carcinomas 
Older, postmenopausal 
Less oestrogen dependent 
Arise in atrophic endometrium 
High grade, deeper invasion, higher stage
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162
Q

What are the different FIGO stages for endometrial carcinoma?

A

Stage 1 – confined to uterus
Stage 2 – spread to cervix
Stage 3 – spread to adnexae, vagina, local lymph nodes (pelvic or para-aortic)
Stage 4 – other pelvic organs distant spread inc any other distant lymph node groups

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163
Q

What is gestational trophoblastic disease?

A

A spectrum of tumours and tumour like conditions characterised by proliferation of of pregnancy associated trophoblastic tissue. Includes:
– Complete and partial mole
– Invasive mole
– Choriocarcinoma

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164
Q

What are the characteristics of complete and partial moles?

A

1 in 1000 pregnancies
Most present with spontaneous abortion
Excessive level of HCG hormone
Risk of malignant development only with complete moles (2.5%)

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165
Q

How do hydatidiform moles form?

A

Complete: An empty egg fertilised by 1 or 2 sperms (46XX or 46XY)
Partial: A normal egg fertilised by 2 sperms (XXX, XXY, XYY) or a normal egg fertilised by a sperm carrying unreduced paternal genome (XXY)

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166
Q

What are the characteristics of choriocarcinoma?

A
  • 1 in 20,000-30,000 pregnancies
  • Rapidly invasive, widely metastasising (lung, vagina, brain, liver, kidney)
  • Responds well to chemotherapy
  • 50% arise in moles
  • 25% arise in previous abortion
  • 22% arise in normal pregnancy
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167
Q

What is endometriosis?

A

Presence of endometrial glands and stroma outside the uterus which is functional and bleeds at time of menstruation -> pain, scarring and infertility.
Affects 10% of premenopausal women.

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168
Q

What is the origin of endometrial tissue in endometriosis?

A
  • Metaplasia of pelvic peritoneum

* Implantation of endometrium, retrograde menstruation

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169
Q

What is adenomyosis?

A

Ectopic endometrial tissue deep within the myometrium.

Causes dysmenorrhoea.

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170
Q

What risk factors are associated with ovarian cancer?

A
  • Most significant is genetic predisposition
  • Family history of ovarian and breast cancers
  • Infertility
  • Endometriosis
  • Hormone replacement therapy
  • Pelvic inflammation
  • Nulliparity, early menarche, late menopause
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171
Q

What is the commonest type of malignant ovarian tumour?

A

95% Epithelial tumours - 65% of all ovarian tumours

Serous most common subtype.

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172
Q

What is the incidence pattern of ovarian cancers?

A
  • Germ cell tumours have bimodal distribution; 15-21 and 65-69 (95% benign)
  • 50% of epithelial are 45-65
  • Sex cord tumours most common in post-menopausal women
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173
Q

What is the difference between type I and type II ovarian tumours?

A 
Type I (20%) - Low grade, relatively indolent, arise from well characterised precursors (BOT) and endometriosis
Type II - High grade, mostly serous type, aggressive, >75% have p53 mutations, no precursor lesions
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174
Q

What are the different sub-types of epithelial ovarian cancer?

A
  • Serous (most common)
  • Mucinous (gastrointestinal or endocervical type)
  • Endometrioid (associated with endometriosis)
  • Clear cell (strong association with endometriosis)
  • Transitional
  • Mixed types
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175
Q

What are the types of benign ovarian tumours?

A
  • Serous Cystadenomas
  • Cystadenofibromas
  • Mucinous cystadenomas
  • Brenner tumour
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176
Q

A patient newly diagnosed with an endometrioid ovarian tumour should also be investigated for..?

A

Endometrioid carcinoma in uterus as co-existance is common

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177
Q

What are the different subtypes of sex cord stromal tumour?

A

Fibromas: benign
Granulosa cell tumor: may produce estrogen
Thecoma: benign, may secrete oestrogen, or rarely androgens
Sertoli-Leydig cell tumor: may be androgenic

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178
Q

What is the difference between a mature and immature teratoma?

A

Mature: benign, all mature adult type tissues (e.g. teeth, hair) transformation rare
Immature: malignant, presence of embryonic elements, grows rapidly, penetrates the capsule, forms adhesions and spreads to lymph nodes, lung, liver etc.

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179
Q

Germ cell tumour subtypes?

A 
Teratoma
Dysgerminoma
Yolk sac tumour 
Embryonal carcinoma
Choriocarcinoma
180
Q

What are Krukenberg tumours?

A

A malignancy in the ovary (often bilateral) that metastasised from a primary site, commonly colorectal, gastric or breast.
May be composed of signet ring, mucin-rich cells.
May be first presentation of primary tumour.

181
Q

What familial syndromes are associated with an increased risk of ovarian cancer?

A

1 - familial breast-ovarian cancer syndrome
2 - site-specific ovarian cancer
3 - cancer family syndrome (Lynch type II)
All are autosomal dominant, 1+2 associated with BRCA1 mutations and account for 90% familial ovarian cancers

182
Q

What type of ovarian cancers are associated with HNPCC and BRCA mutations?

A
  • Serous tumours: BRCA - commonest
  • Mucinous tumours: HNPCC
  • Endometrioid carcinoma: HNPCC
    Ovarian cancer <30 yrs more likely to be due to HNPCC mutations (MSH2 and MLH1) than BRCA1/2
183
Q

What is a papillary hidradenoma?

A

A benign nodule of ectopic breast tissue usually found on the labia majora or the interlabial folds. May ulcerate and be confused with carcinoma of the vulva.

184
Q

What is the commonest vulval malignancy?

A

SCC

May be associated with or secondary to HPV or lichen sclerosus.

185
Q

What type of vaginal cancer was associated with an old treatment to prevent adverse pregnancy outcomes?

A

Clear cell adenocarcinoma in daughters if mother was treated during pregnancy with Diethyl stilbosterol (DES) for threatened abortion.

186
Q

BCR-ABL is associated with which disease?

A

CML

187
Q

What are myeloproliferative neoplasms (MPNs)?

A

A group of neoplastic/clonal disorders of haemopoeitic stem cells characterised by the overproduction of one or more of the mature myeloid cellular elements of the blood. There is often increased BM fibrosis and cases may progress to acute leukaemia.

188
Q

How do leukaemia, MPN and MDS differ?

A

Leukaemia: Proliferation without differentiation
MPN: Proliferation and full differentiation
MDS: Ineffective proliferation and differentiation

189
Q

What cell counts are increased in polycythaemia vera?

A

Predominantly increased RBCs

Also platelets and granulocytic cells and a true increase in plasma volume

190
Q

What symptoms do patients with polycythaemia vera present with?

A 
Symptoms of increased hyper viscosity:
- Headaches, light-headedness, stroke
- Visual disturbances
- Fatigue, dyspnoea
Increased histamine release:
- Aquagenic pruritus
- Peptic ulceration
191
Q

What signs do patients with polycythaemia vera present with?

A
  • Variable splenomeagaly
  • Plethora
  • Erythromelalgia: red painful extremeties
  • Thrombosis
  • Retinal vein engorgement
  • Gout due to increased red cell turnover and overproduction of uric acid
  • Absence of other causes of increased haematocrit
192
Q

What mutation is diagnostic for polycythaemia vera?

A

JAK2 V617F

193
Q

What is pseudopolycthaemia?

A

Also has a high haematocrit but reduced plasma volume and normal RBC mass

194
Q

What does a patient with a purely raised RBC count and exon 12 mutation have?

A

Erythrocytosis

No raised WBCs or PLTs

195
Q

What is the target haematocrit with treatment in polycythaemia vera? And what treatment is used?

A

<45%
Venesection and Cytoreductive therapy (e.g. hydroxyurea)
Aspirin used to decrease risk of thrombosis

196
Q

How is idiopathic erythrocytosis managed?

A

Venesection only

Less likely to transform to MF or AML

197
Q

What is essential thromocythaemia?

A

Chronic MPN mainly involving megakaryocytic lineage

with sustained thrombocytosis >600x10^9/L

198
Q

What symptoms do patients with essential thromocythaemia present with?

A
  • Incidental finding in half the patients
  • Thrombosis: arterial or venous
  • Bleeding: mucous membrane and cutaneous
  • Minor: headaches, dizziness visual disturbances
  • Splenomegaly usually modest
199
Q

What conditions are associated with mutations of JAK2?

A

Polycythaemia vera ~100%
Essential thromocythaemia ~50%
Myelofibrosis ~50%

200
Q

What treatment is available for essential thromocythaemia?

A
  • Aspirin: to prevent thrombosis
  • Anagrelide: specific inhibition of platelet formation, side effects include palpitations and flushing, increases MF
  • Hydroxycarbamide/urea: antimetabolite. Suppression of other cells as well. Possible mildly leukaemogenic
  • Alpha interferon: may have a place in the treatment of patients below 40 years of age or in pregnancy.
201
Q

How are patients with essential thromocythaemia risk stratified?

A
  • Low risk: 60, Thrombosis, ischaemic or haemorrhagic symptoms, Platelets >1000x10^9/L, Other thrombotic risk factors
202
Q

What is chronic idiopathic myelofibrosis?

A

A clonal myeloproliferative disease with proliferation mainly of megakaryocytes and granulocytic cells, associated with reactive bone marrow fibrosis and extramedullary haematopoieisis.
Can be primary or secondary to PV or ET

203
Q

What symptoms and signs do patients with essential thromocythaemia present with?

A
  • Incidental in 30%
  • Cytopenias: anaemia or thrombocytopenia
  • Thrombocytosis
  • Splenomegaly: may be massive e.g. Budd-Chiari syndrome
  • Hepatomegaly
  • Hypermetabolic state: weight loss, fatigue, dyspnoea, night sweats, hyperuricaemia
204
Q

What signs in a peripheral blood film are there with myelofibrosis?

A 
Leucoerythroblastic picture
Tear drop poikilocytes
Giant platelets
Circulating megakaryocytes
Basophilia
205
Q

What treatment is available for patients with myelofibrosis?

A
  • Transfusions: may become increasingly difficult because of splenomegaly, platelet transfusions often ineffective.
  • Splenectomy for symptomatic relief: often hazardous and followed by worsening of condition
  • Cytoreductive: hydroxycarbamide for thrombocytosis but may worsen anaemia
  • Thalidomide with or without prednisolone for select patients
  • Bone marrow transplant for young patients
206
Q

What type of biopsy are preferable for suspected bone tumours?

A

(Jamshidi) Needle biopsy guided by US or CT

Prevents local contamination

207
Q

Examples of tumour-like conditions which would be part of a differential for a suspected bone tumour

A 
Fibrous dysplasia
Metaphyseal fibrous cortical defect/non-ossifying fibroma
Reparative giant cell granuloma
Ossifying fibroims
Simple bone cyst
208
Q

What condition may have a ‘soap bubble’ osteolytic appearance on x-ray? What is seen on histology?

A

Fibrous dysplasia
Hip may also get shepherd’s crook deformity.
Histology shows thin, irregular (Chinese character-like) bony trabeculae

209
Q

What is McCune–Albright syndrome?

A
  • Endocrine disease, such as in precocious puberty
  • Polyostotic fibrous dysplasia
  • Unilateral Café-au-lait spots
210
Q

What mutation is associated with fibrous dysplasia?

A

GNAS on chromosome 20q13

211
Q

What is the commonest site of an enchondroma?

A

Hands and feet.

They are a cartilage cyst found in the bone marrow and may cause a fracture.

212
Q

What is the commonest site of an osteochondroma?

A

Knee and proximal ends of long bones.

They are a type of benign tumour that consists of cartilage and bone usually on a peduncle.

213
Q

What are the commonest sites affected by fibrous dysplasia?

A

Fibrous dysplasia can occur in any part of the skeleton but the bones of the skull, thigh, shin, ribs, upper arm and pelvis are most commonly affected.

214
Q

What is the commonest site of a Giant cell tumour of bone?

A

The epiphyseal/metaphyseal region of long bones, most commonly around the knee.
It is characterised by the presence of multinucleated giant cells (osteoclast-like cells).

215
Q

What are the commonest sites of an osteosarcoma?

A 
Osteosarcoma tends to affect regions around the;
knee 60%
hip 15%
shoulder 10%
jaw 8%
216
Q

What is Codman’s triangle?

A

Triangular area of new subperiosteal bone that is created when a lesion, often a tumour, raises the periosteum away from the bone. Can be seen with osteosarcoma.

217
Q

What is osteosarcoma and what does it look like histologically?

A

The most common type of primary malignant bone tumour that exhibits osteoblastic differentiation and produce malignant osteoid.
Malignant mesenchymal cells +/- bone and cartilage formation

218
Q

What are the commonest sites of a chondrosarcoma?

A

Pelvis, shoulder, proximal ends of long bones, base of skull.
Affects older patients >40 yrs

219
Q

What does an chondrosarcoma look like histologically?

A

Malignant chondrocytes =/- chondroid matrix.

May dedifferentiate to high grade sarcoma

220
Q

What are the commonest sites of Ewing’s sarcoma?

A

Pelvis, femur, humerus, ribs and clavicle.
Occurs in middle of bones - diaphysis
80% patients <20

221
Q

What is Ewing’s sarcoma and what does it look like histologically?

A

A malignant small, round, blue cell tumour.

Sheets of small round cells.

222
Q

What mutation is associated with Ewing’s sarcoma?

A

Specific chromosome translocation 11:22 (EWS/Fli1)

223
Q

What are the commonest sites of a soft-tissue sarcoma/tumour?

A

Can affect anywhere but majority occur in large muscles of limbs, chest wall, mediastinum, retroperitoneum.

224
Q

What risk factors are associated with soft-tissue sarcomas?

A 
Genetic - Retinoblastoma gene
Chemical carcinogens e.g. agent orange
Physical (asbestos, foreign body)
Viruses (HIV - kaposi's)
Immunodeficiency
225
Q

What different types of soft-tissue sarcomas are there?

A

Spindle cell tumours
Myxoid tumours
Pleomorphic tumours

226
Q

What cells are seen with a normal cervical smear?

A

Superficial and intermediate squamous cells

227
Q

How are samples collected in exfoliative cytology?

A

Cells are dislodged or spontaneously shed from a surface ie. Bronchial washings and brushings, serous cavity effusions etc. Not FNA’s

228
Q

What possible side effects could there be from FNA?

A 
Typically nothing
Bruising
Fainting
(Pneumothorax – site dependent!)
(Infarction of lesion)
229
Q

What are the acute and chronic dangers with cocaine use?

A

– Acute dangers : cardiac dysrythmias, acute heart failure, myocardial infarction
– Slowly developing damage to the myocardium, ventricular arrhythmias, sudden death
– Lethal syndrome of excited delirium, occurs in regular users within 24 hrs of last dose
– Effects prolonged if used with ethanol, get cocaethylene formed

230
Q

What are the major points of forensic toxicology concern for ecstasy?

A

aka. MDMA.
– Few deaths
– Large OD causes direct toxic effect on heart
– Can cause hyperthermia, leads to rhabdomyolysis, leads to muscle necrosis and renal failure

231
Q

What are the major points of forensic toxicology concern for Methadone?

A

– Tolerance
– After ingestion fatal amount takes 4-6 hours to die
– Additive effects other respiratory depressant drugs
– 5 mL can kill a child, 60 mL can kill healthy adult male
– Maintenance dose can vary from 5 to 200 mL

232
Q

Under Section 3 of the Coroner’s Act 1887, which deaths are reported to the coroner?

A

Violent
Unnatural or sudden
Cause of death is unknown

233
Q

What problems are there with interpreting forensic toxicology?

A
  • Tolerance
  • Site dependence
  • PM redistribution of drugs (NB PM blood concentration cannot be used to calculate the dose)
  • Individual variation in response
  • Stability of drugs
234
Q

Why is hair used for analysis of drugs?

A
  • Can only detect drugs <12 hrs in blood
  • Urine, typically 2-3 days
  • Hair: only specimen good for long term drug use
  • Drugs incorporated into hair from blood during the growth phase
  • Hair growth ~1cm/month – “tape-recording of drug use”
235
Q

What are the applications of hair drug analysis?

A
  • Child custody cases
  • Investigating spiked drinks defences
  • Drug naïve deaths
  • Monitoring drug use prior to return of driving license – Germany, Italy
  • Investigation of drug use in exhumed/putrefied bodies
  • Employment, pre-employment screening - USA
236
Q

What are the features of chronic pancreatitis?

A
  • progressive fibroinflammatory disease
  • diabetes, steatorrhoea & radiographic calcifications
  • permanent impairment of function
  • irreversible morphological changes
237
Q

What are the non-alcohol related causes of chronic pancreatitis?

A
  • hereditary
  • autoimmune
  • metabolic (hypercalcaemia, hyperlipidaemia)
  • idiopathic
  • anatomic abnormalities (e.g. paraduodenal pancreatitis)
  • obstructive (e.g. gallstones)
  • trauma
238
Q

What is the pathogenesis of alcohol related chronic pancreatitis?

A
  • chronic alcohol consumption increases protein concentration within pancreatic juice
  • intraductal precipitation of plug-forming secretions
  • subsequent calcification
  • intraductal calculi -> duct obstruction
  • secondary acinar atrophy & periductal fibrosis
239
Q

What are the microscopic features of alcohol related chronic pancreatitis?

A
  • preservation of normal lobular architecture
  • irregular loss of acinar and ductal tissue
  • ductal dilatation (ectasia)
  • chronic inflammation & fibrosis
  • fibrosis is irregular in distribution in periductal, intralobular & interlobular areas
  • hyperplasia & hypertrophy of nerve fibres
240
Q

What are the macroscopic features of alcohol related chronic pancreatitis?

A
  • focal, segmental or diffuse involvement
  • involved areas are indurated & fibrotic
  • distorted cystically dilated ducts
  • plugs of calcified protein (calculi)
  • pancreas may become rock hard & undergo atrophy
  • shrunken & irregular contour in advanced cases
  • pseudocysts of variable size (up to 10cm) lined by fibrous tissue, inflammation & granulation tissue (no epithelial lining)
241
Q

What microscopic feature is helpful in differentiating chronic pancreatitis from duct type adenoCa?

A

preservation of lobular architecture

242
Q

What are PanIN 1A and 1B?

A

Cellular changes that can be observed in chronic pancreatitis
PanIN 1A - mucous cell metaplasia
PanIN 1B - papillary epithelial hyperplasia

243
Q

What are the features of hereditary pancreatits?

A
  • usually begins in childhood or early adolescence
  • rare: 2% of chronic pancreatitis pts
  • recurrent pancreatitis
  • family history of pancreatic disease
  • characteristic PRSS1 mutation (cationic trypsinogen gene)
  • 50-fold increased risk of ductal adenoCa
244
Q

What is Lymphocytic Sclerosing Pancreatitis?

A

Autoimmune duct-centric pancreatitis with high serum IgG4, (obliterative) phlebitis of medium-sized veins and IgG4+ plasma cells around ducts.
Often associated with other systemic AI diseases

245
Q

What is groove pancreatitis?

A

AKA paraduodenal pancreatitis - within & surrounding duodenal wall near minor ampulla
‘groove’ - usually involves an area located between the CBD, pancreas & duodenum
Chronic inflammation of duodenum that extends to pancreatic parenchyma

246
Q

What is a pancreatic pseudocyst?

A
  • consequence of severe acute on chronic pancreatitis or secondary to trauma, surgical complication
  • thick walled fibrous capsule without epithelial lining
  • sequela of extensive liquefactive necrosis of peripancreatic fat tissue and/or intrapancreatic parenchyma due to activated pancreatic enzymes
  • can be anywhere in pancreas
  • cyst fluid analysis – high amylase levels >500U/L
247
Q

What different types of tumour are there of the pancreas?

A
  • Ductal adenocarcinoma
  • Pancreatic Intraepithelial neoplasia (PanIN)
  • Cystic Tumours: serous or mucinous cystic neoplasm
  • Intraductal Papillary Mucinous Neoplasm (IPMN)
  • Pancreatic Endocrine Neoplasm: well or poorly dif
248
Q

What are the risk factors for pancreatic ductal adenocarcinoma?

A
  • smoking & high dietary fat intake
  • acquired chronic pancreatitis & diabetes
  • hereditary chronic pancreatitis
  • heritable genetic syndromes - BRCA2, FAMMM, P-J syndrome, familial pancreatitis, HNPCC
249
Q

What is the T staging for pancreatic ductal adenocarcinoma?

A

Tis – Ca in situ (PanIN 3)
T1 – tumour limited to pancreas, 20mm
T3 – tumour extends directly into any of the following: duodenum, bile duct, peripancreatic tissues
T4 – tumour extends into stomach, spleen, colon, adjacent large vessels

250
Q

What type of pancreatic tumour is composed of large stromal mucin lakes within which are suspended relatively scanty strips and clusters of cells with individual cells having a signet ring configuration?

A

Colloid carcinoma (mucinous non cystic Carcinoma)

251
Q

What type of pancreatic tumour is poorly differentiated, lacks gland formation & has a pushing growth pattern at the periphery with large, syncytial tumour cells?

A

Medullary Carcinoma

252
Q

What type of pancreatic tumour contains neoplastic glands resembling conventional ductal adenoCa as well as large nests of cells with squamous differentiation?

A

Adenosquamous carcinoma

253
Q

What type of pancreatic tumour has enormous tumour cells growing in solid sheets & have markedly atypical nuclei?

A

Undifferentiated carcinoma

254
Q

What is Pancreatic Intraepithelial Neoplasia?

A
  • precursor of ductal adenoCa
  • series of increasingly proliferative changes within the epithelium of pancreatic ducts
  • graded as PanIN 1A, 1B, 2 & 3
255
Q

What type of pancreatic lesion is grossly well circumscribed & composed of small cysts, each measuring <1cm, separated by thin translucent septa?

A

Serous cystic neoplasm e.g. Microcystic serous cystadenoma

256
Q

What are the microscopic features of a serous cystic neoplasm?

A
  • cysts lined by flattened cuboidal epithelium
  • clear cytoplasm & well defined cytoplasmic borders
  • small round uniform nuclei with dense chromatin
  • accumulation of glycogen
257
Q

What type of pancreatic lesion is grossly well circumscribed, often involves the tail of pancreas & contains numerous large 1-5cm cysts?

A

Mucinous cystic neoplasm

258
Q

What are the microscopic features of a mucinous cystic neoplasm?

A
  • similar to ovarian mucinous neoplasm
  • tall columnar cells with abundant apical mucin
  • bland with uniform, basally oriented nuclei
  • minimal architectural complexity
  • subepithelial hypercellular spindle cell stroma (ovarian-like) – must be present for diagnosis
  • stromal cells express oestrogen, progesterone and inhibin
259
Q

What is an Intraductal Papillary Mucinous Neoplasm?

A
  • characterized by intraductal proliferation of mucinous cells usually arranged in a papillary pattern (intestinal, gastric foveolar, pancreatobiliary)
  • multilocular cystic mass or abundant papillary nodules
  • mucin extrusion through the ampulla diagnostic
  • radiographic finding of ectatic (dilated) ducts
  • 2 main types: Main and Branch duct type
  • Good prognosis, most managed by conservative resection
260
Q

What is the normal lining of the oesophagus?

A
  • Proximal 2/3 squamous epithelium
  • Distal 1/3 columnar epithelium
  • Join at squamo-columnar junction / Z-line
261
Q

What is the normal lining of the body of the stomach?

A
  • Foveolar epithelium (columnar, mucin secreting)

- Specialised glands (parietal and chief cells)

262
Q

What is the normal lining of the antrum of the stomach?

A
  • Foveolar epithelium (columnar, mucin secreting)

- Non-specialised glands

263
Q

What is the normal lining of the duodenum?

A
  • Glandular epithelium with goblet cells

- Villous architecture (villus:crypt ration >2:1)

264
Q

A tissue sample with Brunners glands is from where?

A

Proximal part of duodenum. They are seen in the lamina propria.

265
Q

What is the grading system for oesophagitis?

A

Los Angeles classification (A-D)

266
Q

What is the commonest cause of oesophagitis?

A

Gastro-oesophageal reflux disease (GORD) / reflux oesophagitis

267
Q

What are the complications of GORD?

A

Ulceration -> Haemorrhage, Perforation
Fibrosis -> Stricture
Barrett’s oesophagus

268
Q

What is Barrett’s oesophagus?

A

Metaplasia of squamous mucosa to columnar epithelium with goblet cells (intestinal type epithelium) due to chronic GORD.
Can lead to adenocarcinoma: metaplasia -> dysplasia -> Ca

269
Q

What are the risk factors for squamous cell oesophageal carcinoma?

A
  • alcohol and smoking
  • achalasia of cardia
  • Plummer-Vinson syndrome
  • nutritional deficiencies
  • nitrosamines
  • HPV
270
Q

How does squamous cell oesophageal carcinoma present?

A
  • 6x more common in afro-carribeans, M>F
  • 50% in middle 1/3
  • progressive dysphagia
  • odynophagia
  • anorexia
  • severe weight loss
  • grows and spreads rapidly to liver and local structures
271
Q

What are common causes of acute gastritis?

A
  • 0 NSAIDs: Aspirin, Ibuprofen etc
  • Corrosives (bleach)
  • Infection (acute H.pylori)
272
Q

What are common causes of chronic gastritis?

A
  • H.pylori, H.heilmanni
  • Autoimmune (pernicious anaemia)
  • Alcohol
  • Smoking
  • Other infection (CMV, HSV, Strongyloides)
  • Crohn’s Disease
273
Q

How are H.pylori often described in biopsies?

A

Seagull shapped

274
Q

What possible complications are there from a gastric ulcer?

A
  • Bleeding: iron def anaemia, shock
  • Perforation: peritonitis, abscess formation
  • Malignancy
275
Q

What is commonest type of gastric cancer?

A

90% carcinomas

Also MALTomas / Lymphoma

276
Q

What is the cause and treatment of gastric lymphoma?

A

Chronic inflammation due to H/pylori

Treat with PPIs + antibiotics to eradicate infection and hence chronic antigen stimualtion

277
Q

What auto-antibodies are present in pernicious anaemia?

A

Parietal cell antibodies 90%

Intrinsic factor antibodies 60%

278
Q

What complications can occur with pernicious anaemia?

A
  • Vit B12 deficiency
  • Stomach body atrophy
  • Malignancy
279
Q

What other pathogens can cause duodenitis, esp in immunosuppressed?

A 
CMV
Microsporidiosis
Cyryptosporidiosis
Giardia lamblia
Tropheryma whippelii (Whipples disease)
280
Q

How does pain experienced with duodenal and gastric ulcers differ?

A

Gastric: worse with food, better with antacids
Duodenal: better with food and milk, worse at night

281
Q

What does endoscopy and a duodenal biopsy show in coeliac disease?

A
  • Scalloping with smooth shiny mucosa

- Villous atrophy, crypt hyperplasia, intraepithelial lymphocytes

282
Q

How is partial villous atrophy graded?

A

Marsh Criteria 0-4

283
Q

Why might serology be inaccurate in coeliac disease?

A

Because (irish) patients are likely to have IgA deficiency also, need duodenal biopsies on and off gluten diet

284
Q

What malignancy are patients with poorly controlled coeliac disease at an increased risk of?

A

Duodenal T-cell MALToma/lymphoma

~10% progress if not treated adequately

285
Q

What is the commonest type of urinary calculi and why do patients get them?

A

75% = calcium oxalate
Most related to absorptive hypercalciuria, some to renal hypercalciuria
15% = Magnesium ammonium phosphate
5% = uric acid

286
Q

What are magnesium ammonium phosphate urinary calculi associated with?

A

Infections with urease-producing organisms e.g. Proteus which alkalinises the urine. Can form very large ‘staghorn calculi’

287
Q

What are the common points of renal stone impaction?

A

Pelvic-uriteric junction, pelvic brim and Vesico-uriteric junction

288
Q

What is a renal papillary adenoma?

A

Benign renal epithelial tumour with a papillary or tubular architecture and size of 5mm or less
Often found incidentally

289
Q

What is a renal oncocytoma?

A

Completely benign oncocytic renal epithelial neoplasm. Most cases sporadic and discovered incidentally.
Mahogany brown colour.

290
Q

What is a renal angiomyolipoma?

A

Benign mesenchymal tumour of the kidney composed to variable amount of fat, smooth muscle and thick-walled blood vessels
Most sporadic, some associated with tuberous sclerosis
May present with flank pain due to haemorrage

291
Q

What is the commonest renal malignancy and what risk factors are associated with it?

A

Renal cell carcinoma - malignant epithelial tumour
Risk factors include smoking, hypertension, obesity, environmental chemicals, long-term dialysis
Genetic syndromes e.g. von Hippel Lindau

292
Q

What different subtypes of renal cell carcinoma are there?

A

70% clear cell - golden yellow tumours with haemorrgaic areas
15% Papillary - friable brown tumour, >5mm papillary adenoma
5% Chromophobe - solid brown tumour

293
Q

Which renal tumour often shows loss of genetic material at chromosome 3p?

A

Clear cell renal cell carcinoma

294
Q

Which renal tumour often genetically shows trisomy of chromosomes 7 and 17?

A

Papillary renal cell carcinoma

295
Q

What grading system is used for renal cell carcinoma?

A

Fuhrman system 1-4

296
Q

What is the overall 5 year survival rate for renal cell carcinoma?

A

~60%

297
Q

What is Wilm’s tumour?

A

aka Nephroblastoma, a malignant childhood (2-5yr) renal neoplasm which presents as an abdominal mass.
Histologically see ‘small round blue cells’
Good prognosis

298
Q

What are transitional cell carcinomas?

A

aka urothelial carcinomas are a group of epithelial neoplasms arising in the urothelial tract, most commonly the bladder. Associated with smoking.

299
Q

How are urothelial carcinomas classified?

A
  • Non-invasive papillary urothelial carcinomas

- Invasive (infiltrating) urothelial carcinomas

300
Q

What are non-invasive papillary urothelial carcinomas and how are they treated?

A

Frond-like growths projecting from bladder mucosa, present with haematuria.
Resection at cystoscopy +/- intravesical chemotherapy depending on grade. Follow up.

301
Q

What are invasive (infiltrating) urothelial carcinomas and how are they treated?

A

Malignant / invasive urothelial tumour.
Tumours only invading lamin propria can be treated with resection and intravesical chemotherapy.
Tumours invading detrusor muscle or beyond required cystectomy +/- radiotherapy +/- systemic chemotherapy.

302
Q

What treatment is available for benign prostatic hyperplasia?

A

Medical
- alpha-blockers: doxazosin
- 5-alpha-reductase inhibitors: finasteride, dutaseride
Surgical
- transurethral resection of the prostate (TURP)

303
Q

What mutations are often found in prostatic carcinoma?

A 
GST-pi
PTEN
AMACR
p27
E-cadherin
304
Q

How is prostate cancer usually diagnosed?

A

From needle biopsy following a raised serum prostate specific antigen (PSA) level in an asymptomatic patient

305
Q

What prognostic indicator is used for prostate cancer?

A

Gleason score (6-10) - higher the score mean more aggressive behaviour

306
Q

What are the main treatment options for early stage prostate cancer?

A

Active surveillance
Radical prostatectomy
Radical radiotherapy

307
Q

What risk factors are there for testicular germ cell tumours?

A

Cryptorchidism increases risk 3-5 fold
Prenatal risk factors include low birth weight and small for gestational age
Most arise from precursor lesion - intratubular germ cell neoplasia (ITGCN)

308
Q

What different types of testicular germ cell tumour are there?

A

Seminoma - resembles germ cells
Embryonal carcinoma - resembles embryonic tissue
Yolk sac tumour - resembles yolk sac tissue
Immature teratoma - resembles foetal tissue
Mature teratoma - resembles adult tissues
Choriocarcinoma - resembles placental tissue
Often mixed

309
Q

What types of testicular non-germ cell tumours are there?

A

Testicular lymphoma - older men, high grade, poor survival
Leydig cell tumours - most benign
Sertoli cell tumours - most benign

310
Q

What are the stages of fracture repair?

A
  1. Organisation of haematoma at # site (pro-callus)
  2. Formation of fibrocartilaginous callus
  3. Mineralisation of fibrocartilaginous callus
  4. Remodelling of bone along weight-bearing lines
311
Q

What are the common sites of osteomyelitis?

A 
Adults:
- vertebrae
- jaw (2º to dental abscess)
- toe (2º to diabetic skin ulcer) (>3mm)
Children
- long bones (usually metaphysis)
312
Q

What are the common causative organisms of osteomyelitis?

A 
Adults:
- Staph Aureus (90%)
- E. Coli
- Klebsiella
- Salmonella (associated with sickle cell disease)
- Psuedomonas (IVDA)
Neonates:
- Haemophilus influenzae
- Group B Streptococcus
- Occasionally entrobacter
313
Q

When does osteomyelitis show x-ray changes and what are they?

A
  • usually appear 10 days or so post onset
  • Mottled rarefaction and lifting of periosteum
  • > 1week - involucrum: irregular sub-periosteal new bone formation
  • Later - irregular lytic destruction (takes 10-14days)
  • Some areas of necrotic cortex may become detached called sequestra (takes 3-6 weeks)
314
Q

How might TB osteomyelitis present?

A
  • Affects immunocompromised patients
  • Spinal disease (50% cases) may result in psoas abscess and severe skeletal deformity (Pott’s disease)
  • Systemic amyloidosis may result in protracted cases
315
Q

What type of cell is associated with TB osteomyelitis?

A

Langhans type Giant cell

316
Q

What problems can syphilitic osteomyelitis causes?

A 
Congenital skeletal lesions:
- Osteochondritis
- Osteoperiostitis
- Diaphyseal osteomyelitis
Acquired late skeletal lesions:
- Non-gummatous periostitis
- Gummatous inflammation of bone and joints
- Neuropathic joints (Tabes Dorsalis)
- Neuropathic shaft fractures
317
Q

What main sites are affected by osteoarthritis?

A 
Vertebrae
Hips
Knees
DIPJ hand
Carpometacarpal joints
Metatarsophalangeal joints
318
Q

How many patients with rheumatoid arthritis are rheumatoid factor positive?

A

80%

RF mostly IgM, forms immunocomplexes with IgG

319
Q

What are the histological findings in rheumatoid arthritis?

A

Proliferative synovitis with;
1. Thickening of synovial membranes ( villous)
2. Hyperplasia of surface synoviocytes
3. Intense inflammatory cell infiltrate
4. Fibrin deposition and necrosis
Pannus is the exuberant inflamed synovium on the articular surface

320
Q

What subsets of pseudogout are there?

A

a) Sporadic
b) Metabolic
c) Hereditary
d) Traumatic

321
Q

What are the clinical effects of a prolactinoma?

A
  • Amenorrhea, galactorrhea, loss of libido, infertility

- Usually diagnosed earlier in females of reproductive age

322
Q

What are the clinical effects of a growth hormone adenoma?

A
  • Prepubertal children - gigantism
  • Adults - acromegaly
  • Diabetes mellitus, muscle weakness, hypertension, congestive cardiac failure
323
Q

What are the causes of hypopituitarism?

A

Nonsecretory pituitary adenomas (commonest)
Ischaemic necrosis:
- Most commonly post-partum (Sheehan’s syndrome)
- DIC, sickle cell anaemia, elevated intracranial pressure, shock.
Ablation of pituitary by surgery or irradiation

324
Q

What are the clinical manifestation of anterior pituitary hypofunction?

A
  • Children: growth failure (pituitary dwarfism)
  • Gonadotrophin deficiency - amenorrhea and infertility in women. Decreased libido and impotence in men
  • TSH and ACTH deficiency - hypothyroidism and hypoadrenalism
  • Prolactin deficiency - failure of post-partum lactation
325
Q

What are the causes of a non-toxic goitre?

A

Common if there is impaired synthesis of thyroid hormone - most often due to iodine deficiency.
May be seen at puberty particularly in females.
May be due to ingestion of substances that interfere with thyroid hormone synthesis e.g. brassicas.
May be due to hereditary enzyme defects.

326
Q

What are the primary causes of thyrotoxicosis?

A

Grave’s disease
Hyperfunctioning multinodular goitre
Hyperfunctioning adenoma
Thyroiditis

327
Q

What are the non-primary causes of thyrotoxicosis?

A

Secondary: TSH secreting pituitary adenoma (rare)
Not associated with thyroid disease:
- Struma ovarii (ovarian teratoma with ectopic thyroid)
- Factitious thyrotoxicosis (exogenous thyroid intake)

328
Q

What are the causes of hypothyroidism?

A 
Primary:
- Postablative (after surgery or radioiodine therapy)
- Primary idiopathic
- Hashimoto’s thyroiditis
- Iodine deficiency
- Congenital biosynthetic defect
Secondary:
- Pituitary or hypothalamic failure (uncommon)
329
Q

What are the types of thyroid carcinoma?

A

Papillary (75-85%) associated with radiation exposure
Follicular (10-20%)
Medullary (5%)
Anaplastic (<5%)

330
Q

What are the diagnostic features of a papillary thyroid carcinoma?

A
  • Optically clear nuclei
  • Intranuclear inclusions
    May have papillary architecture
    May be psammoma bodies - accumulations of calcium
    Lymphatic spread
331
Q

How does follicular thyroid carcinoma spread?

A

Usually metastasise via bloodstream to lungs bone and liver

332
Q

What is medullary thyroid carcinoma and what is characteristic about it?

A

Neuroendocrine neoplasm derived from parafollicular C cells
80% sporadic - adults 5-6th decade
20% familial - MEN - younger patients
Characteristically has amyloid deposits of calcitonin

333
Q

What are the characteristics of anaplastic thyroid carcinoma?

A

Occur in elderly patients
Very aggressive
Metastases common
Most cases death within one year due to local invasion

334
Q

What are the causes of primary hyperparathyroidism?

A

80-90% - solitary adenoma
10-20% hyperplasia of all 4 glands
- Sporadic or component of MEN type 1
<1% carcinoma

335
Q

What are the causes of hypoparathyroidism and the clinical manifestations?

A
  • Surgical ablation (majority), Congenital absence, Autoimmune
  • Neuromuscular irritability: tingling, muscle spasms, tetany
  • Cardiac arrhythmias
  • Fits
  • Cataracts
336
Q

What are the endogenous causes of Cushing’s syndrome?

A
  • Cushing’s disease >50% ACTH-producing adenoma in pituitary: nodular coritcal adernal hyperplasia
  • Primary adrenal neoplasm ~30%
  • Bilateral hyperplasia
  • Ectopic ACTH by non-endocrine tumours e.g. small cell lung carcinoma
337
Q

What are the causes of primary adrenal insufficiency?

A

Acute:
- Haemorrhage
- Sepsis (Waterhouse-Friderichson syndrome)
- Sudden withdrawal of corticosteroid therapy
Chronic (Addison’s disease):
- Autoimmune (75-90%)
- TB
- HIV
- Metastatic tumour (lung and breast particularly)
- Rarely amyloid, fungal infections, haemochromatosis, sarcoidosis

338
Q

What is important to know about phaeochromocytomas?

A
  • 10% associated with familial syndrome inc. MEN 2A and 2B, von Hippel-Lindau disease and Sturge-Weber syndrome
  • 10% are bilateral
  • 10% are malignant
  • 10% of catecholamine-secreting tumours arise outside the adrenal (paragangliomas)
339
Q

What features of endocrines tumours might suggest multiple endocrine neoplasia?

A
  • Occur at a younger age than sporadic tumours
  • Arise in multiple endocrine organs
  • Often multifocal in one organ
  • Often preceded by hyperplasia
  • Usually more aggressive than sporadic tumours
340
Q

How is cytopathology used and coded in breast disease?

A 
- investigating nipple discharge and palpable lumps
C1 = inadequate
C2 = benign
C3 = atypia, probably benign
C4 = suspicious of malignancy
C5 = malignant
341
Q

How does duct ectasia normally present and what findings are there?

A
  • Inflammation and dilation of large breast ducts that presents with nipple discharge, sometimes breast pain, breast mass and nipple retraction
  • Discharge cytology shows macrophages and proteinaceous material
  • Histology shows dilated ducts with periductal inflammation and filled with secretions
  • Benign condition with no increased risk of malignancy
342
Q

How does acute mastitis normally present and what findings are there?

A
  • Painful red breast often seen in lactating women due to cracked skin and stasis of milk
  • Staphylococci the most common causative organism.
  • FNA cytology shows abundant neutrophils.
  • Histology shows acute inflammation +/- abscess formation
343
Q

How does fat necrosis normally present and what findings are there?

A
  • A firm breast lump associated with trauma, surgery, or radiotherapy
  • FNA cytology shows degenerate fat, foamy macrophages and giant cells
  • Histology shows degenerate adipocytes surrounded by foamy macrophages, giant cells, lymphocytes and plasma cells. Later changes include fibrosis and calcification.
344
Q

How does fibrocystic breast change normally present and what findings are there?

A
  • A spectrum of changes which reflect normal, albeit exaggerated, hormonal responses
  • Very common, >1/3 of premenopausal women
  • Presents with cyclical breast lumpiness and nodularity
  • Histological changes include cysts, apocrine metaplasia, adenosis, mild usual epithelial hyperplasia and stromal hyperplasia
  • No increased risk of malignancy
345
Q

How does a fibroadenoma normally present in the breast and what findings are there?

A
  • Common benign fibroepithelial tumour
  • Circumscribed mobile lump in women 20-30 yrs
  • FNA cytology shows branching sheets of epithelium, bare bipolar nuclei and stroma
  • Histology shows a multinodular mass composed of expanded intralobular stroma and compressed slit-like ducts
346
Q

What are phyllodes tumours?

A
  • An uncommon group of potentially aggressive fibroepithelial neoplasms of the breast
  • Present as enlarging masses in women aged over 50.
  • May arise within pre-existing fibroadenomas
  • Vast majority behave in a benign fashion but a small proportion can behave more aggressively
347
Q

How does an intraductal papilloma normally present in the breast and what findings are there?

A
  • Common benign papillary tumour arising with the duct system in women 40-60 yrs
  • Presents with nipple discharge or a mass
  • Discharge cytology = branching papillary groups of epithelium
  • Histology = papillary mass within a duct lined by epithelium and myoepithelium
348
Q

What is a radial scar?

A
  • Benign sclerosing lesion of the breast characterised by a central zone of scarring surrounded by a radiating zone of proliferating glandular tissue
  • Presents as a stellate mass on mammography, closely mimicking carcinoma
  • Histology = a central elastotic nidus surrounded by a proliferative corona
349
Q

What are proliferative breast diseases?

A

• A diverse group of intraductal epithelial proliferations associated with an increased risk, of greatly varied magnitude, for subsequent development of invasive breast carcinoma.
• Picked up on mammography or incidentally in breast tissue excised for other reasons.
• Includes:
- florid usual epithelial hyperplasia (1.5-2x risk)
- flat epithelial atypia (4x risk)
- in situ lobular neoplasia (7-12x risk)

350
Q

What is ductal carcinoma in situ (DCIS) and what findings are there?

A
  • A neoplastic intraductal epithelial proliferation associated with an inherent, but not inevitable, risk of progression to invasive breast carcinoma
  • Histology shows ducts filled with atypical epithelial cells
  • Graded into low, intermediate or high grade according to degree of nuclear atypia
  • 85% detected as microcalcification on mammography
351
Q

What is the lifetime risk of invasive breast carcinoma in women?

A
  • 1 in 8/9

- Incidence rates rise rapidly with increasing age: most cases occur in older women.

352
Q

What risk factors are there for breast cancer?

A
  • early menarche
  • late menopause
  • increased weight
  • high alcohol consumption
  • oral contraceptive use
  • positive family history
  • BRCA mutations: lifetime risk up to 85%
353
Q

What are the two genetic pathways that lead to invasive breast cancer?

A
  • “Low grade” breast carcinomas tend to arise from low grade DCIS or in situ lobular neoplasia and show 16q loss
  • “High grade” breast carcinomas arise from high grade DCIS and show complex karyotypes with many unbalanced chromosomal aberrations
354
Q

What different histological types of breast carcinoma are there?

A
  • ductal (80%)
  • lobular (15%)
  • tubular (5%)
  • mucinous (5%)
355
Q

What FNA cytology and histology findings are there for invasive breast carcinoma?

A
  • FNA cytology shows many poorly cohesive atypical epithelial cells
  • Histology shows infiltrating atypical epithelial cells
356
Q

How are invasive breast cancers graded?

A

1) tubule formation
2) nuclear pleomorphism
3) mitotic activity
Each scored 1-3, added together:
3-5 points = grade 1 (well differentiated)
6-7 points = grade 2 (moderately differentiated)
8-9 points = grade 3 (poorly differentiated)

357
Q

What receptors are tested for in breast cancer and what do they suggest?

A
  • oestrogen receptor (ER), progesterone receptor (PR) and Her2 status
  • Low grade types tend to be ER, PR positive and Her2 non-amplified
  • High grade types tend to be ER, PR negative and Her2 amplified
358
Q

What prognostic factors are there for breast cancer?

A
  • most important prognostic factor = status of axillary lymph nodes
  • tumour size
  • histological type
  • histological grade
359
Q

What is the aim of the NHS Breast Screening Programme and who is targeted?

A
  • to pick up DCIS or early invasive carcinomas

- women aged 47-73 are invited for screening every 3 years

360
Q

What % of women have an abnormal screening mammogram? How are they assessed?

A
  • ~5%
  • they are recalled to an assessment clinic for further investigation
  • may include more mammograms or an ultrasound plus core biopsy or FNA
361
Q

How are core biopsies taken as part of the breast screening program coded?

A 
B1 = normal breast tissue
B2 = benign abnormality
B3 = lesion of uncertain malignant potential
B4 = suspicious of malignancy
B5 = malignant (B5a = DCIS, B5b = invasive carcinoma)
362
Q

What are the histological findings of gynaecomastia?

A

Breast ducts show epithelial hyperplasia with typical finger-like projections extending into the duct lumen. The periductal stromal is often cellular and oedematous.

363
Q

Where do immune complexes deposit in the kidney?

A
  • mesangial
  • subendothelial
  • subepithelial
    Related to size and charge
    Complexes may be formed with endogenous (auto) antigens e.g. SLE, or exogenous antigens e.g. from infective organisms and deposit at different rates.
364
Q

What are the common congenital disorders of the kidneys?

A

Bilateral or unilateral agenesis
Ectopic kidney e.g. pelvic
Horseshoe kidney (1:500 to 1:1000)

365
Q

What is adult polycystic kidney disease?

A
  • AD (1:400-1:1000), 10% of ESRF patients.
  • Cysts arise from any portion of the nephron
  • Renal failure develops from 40-70 years.
  • PKD1 and PKD2 genes have been identified
  • Also get cysts in liver and beri-aneurysms in brain
366
Q

Which group of patients acquire renal cysts and what are they associated with?

A
  • Cysts commonly arise in the kidneys of patients with ESRF after a prolonged period on dialysis (acquired cystic disease).
  • Carcinoma may occur in these cysts (7% of patients at 10 years)
367
Q

What are the causes of acute kidney injury?

A 
Pre-renal:
- Failure of perfusion
Renal:
- Acute tubular injury
- Acute glomerulonephritis
- Thrombotic microangiopathy
Post-renal:
- Obstruction to urine flow
368
Q

What causes acute tubular injury?

A

aka acute tubular necrosis

  • Ischaemia or toxins e.g. myoglobin, drugs
  • NSAIDs and cox2 inhibitors predispose
369
Q

What morphological changes are there to tubular epithelial cells in acute tubular injury and why does renal function decrease?

A
  • They show a range of changes from loss of brush border to detachment from the basement membrane with formation of intraluminal granular casts.
  • Reduction in GFR is due to a combination of tubular obstruction, tubular leak and haemodymic changes. Tubules have excellent capacity to repair if the underlying insult is corrected
370
Q

What are glomular crescents and what are they associated with?

A

Glomerulonephritis sufficient to cause acute renal failure is almost always associated with glomerular crescents which form in response to holes in the GBM caused by macrophages and neutrophils.

371
Q

What are the causes of crescentic glomerulonephritis?

A
  • Immune complex disease
  • Anti-GBM disease
  • Pauci-immune (scanty antibody deposits)
    Crescentic glomerulonephritis leads rapidly to irreversible renal damage but is often treatable. Therefore diagnosis and treatment are urgent!
372
Q

What are the commonest causes of immune complex-associated crescentic glomerulonephritis?

A
  • SLE
  • IgA nephropathy
  • Post-infectious glomerulonephritis
    Immune complexes can be detected by immunohistochemistry and electron microscopy
373
Q

What antibodies are associated with pauci-immune crescentic glomerulonephritis?

A

Circulating anti-neutrophil cytoplasm antibodies (ANCA) cause neutrophil activation leading to glomerular necrosis. Typically patients also have vasculitis in other organs – e.g skin rash, lung haemorrhage

374
Q

What is thrombotic microangiopathy?

A
  • Damage to endothelium of glomeruli and vessels leading to thrombosis.
  • Associated with damage to RBCs causing haemolytic anaemia with red cell fragments on the blood film = haemolytic uraemic syndrome (HUS)
375
Q

What forms of thrombotic microangiopathy are there?

A
  1. Diarrhoea associated. Caused by bacterial (usually E.coli) infection of the gut that releases a toxin that targets renal endothelium
  2. Atypical (non-diarrhoeal) – often associated with abnormalities of proteins that control activation of the alternative pathway of complement – may be familial.
376
Q

What is the definition of nephrotic syndrome?

A

Breakdown of selectivity of the glomerular filtration barrier leading to massive protein leak

  • Proteinuria > 3.5g/day
  • Hypoalbuminaemia
  • Oedema
  • Hyperlipidaemia
377
Q

What are the causes of nephrotic syndrome?

A 
Systemic:
- Diabetes mellitus
- Amyloidosis
- SLE
Primary glomerular disease:
- Minimal change disease
- Focal and segmental glomerulosclerosis
- Membranous glomerulonephritis
378
Q

What histological changes are seen with diabetes associated nephrotic syndrome?

A

Diabetic glomerulosclerosis - glomeruli show capillary wall thickening and mesangial increase with nodule formation. Arterioles show hyaline deposition

379
Q

What are the characteristics of renal amyloidosis?

A
  • Deposition of extracellular proteinaceous material that stains with Congo red to give green bifrefringence.
  • May be derived from many precursor proteins – commonest in the kidney are:
    AA – derived from serum amyloid A protein which is elevated in chronic inflammation e.g. rheumatoid arthritis, chronic infections
    AL - derived from immunoglobulin light chains
380
Q

What is minimal change disease?

A

Primary disease of podocytes. Glomeruli look normal apart from effacement of podocyte foot processes on EM. Commonest in children. Generally responds to immunosuppression

381
Q

What is focal and segmental glomerulosclerosis?

A

Similar to minimal change disease but glomeruli develop segmental scars. Less likely to respond to immunosuppresssion

382
Q

What is membranous glomerulonephritis?

A

Associated with immune complex deposition on the outside of the GBM.
May be primary (idiopathic or auto-antibodies to phospholipase A2 receptor) or secondary to SLE, infection, malignancy or drugs

383
Q

An asymptomatic patient with microscopic haematuria may have what?

A
  • Thin basement membranes due to hereditary defect in type IV collagen
  • IgA nephropathy
384
Q

What is IgA nephropathy?

A
  • Commonest form of glomerulonephritis worldwide
  • Predominant IgA deposition in glomeruli
  • Often presents with microscopic or macroscopic haematuria
  • May cause proteinuria, acute renal failure
  • Up to 30% progress to end stage renal failure
385
Q

What are the commonest causes of chronic renal failure by prevalence?

A
  • Diabetes (19.5%)
  • Glomerulonephritis (15.3%)
  • Hypertension & Vascular disease (15%)
  • Reflux nephropathy (chronic pyelonephritis) (9.5%)
  • Polycystic kidney disease (9.4%)
386
Q

What are the main subtypes of brain oedema?

A
  • Vasogenic oedema – When integrity of blood brain barrier is disrupted, made worse due to lack of lymphatic drainage in brain, can be local or general
  • Cytotoxic oedema – Secondary to cellular injury e.g. due to general hypoxic-ischaemic injury
387
Q

What is the normal volume of CSF and normal pressure?

A

90-150 ml

7–15 mmHg for a supine adult

388
Q

How can hydrocephalus be classified?

A 
Non-communicating/obstructive:
- Actual obstruction to flow of CSF
Communicating/Non-obstructive:
- Impaired reabsorption
- No obstruction to flow
389
Q

What different types of brain herniation are there?

A
  • Transtentorial (uncal gyral, mesial temporal) – medial temporal lobe compressed against the free margin of the tentorium cerebelli
  • Subfalcine (cingulate gyrus) – cingulate gyrus displaced under the falx cerebri
  • Tonsillar – cerebellar tonsils through the foramen magnum, this causes brain stem compression
390
Q

What are the causes of intraparenchymal haemorrhage (non traumatic)?

A

» Hypertension accounts for 50% of clinically significant haemorrhages
» Other factors include clotting disorders, neoplasms, amyloid, vasculitis, vascular malformations

391
Q

What are Charcot–Bouchard aneurysms?

A

Microaneurysms most often located in the lenticulostriate vessels of the basal ganglia that are associated with chronic hypertension. They are a common cause of cerebral hemorrhage.

392
Q

What types of vascular malformation are there in the brain?

A
  • Arteriovenous (AV) malformations: high pressure massive bleeds
  • Capillary telangectases
  • Venous angiomas
  • Cavernous angiomas: low pressure recurrent bleeds
393
Q

What is the commonest cause of a subarachnoid haemorrhage?

A
  • Rupture of a Berry aneurism
  • present in 1% of general population
  • 80% occur at internal carotid artery bifurcation
  • 30% patients have multiple
  • Higher incidence in people with polycystic kidney disease, coarctation of the aorta, fibromuscular dysplasia and AV malformations in the brain
394
Q

What inflammatory skin reaction patterns are there?

A 
Vesiculobullous
Spongiotic
Psoriasiform
Lichenoid
Vasculitic
Granulomatous
395
Q

What is seen on immunofluorescence in bulous pemphigoid?

A

IgG and C3 deposition along dermoepidermal junction

396
Q

What is Pemphigus foliaceus?

A

look up

397
Q

What does a classical BCC look like?

A

A raised, smooth, shiny, pearly nodule which may have ulcerated

398
Q

What does a classical SCC look like?

A

A red, scaling, thickened patch on sun-exposed skin

399
Q

What congenital diseases of the lungs are there?

A
  • Lung agenesis or hypoplasia
  • Tracheal & bronchial stenosis
  • Congenital cysts
400
Q

What are the causes of acute respiratory distress syndrome?

A

Infection (local or generalised sepsis), aspiration, trauma, inhaled irritant gases, shock, blood transfusion, DIC, drug overdose, pancreatitis, idiopathic.

401
Q

What does insufficient surfactant production cause in premature babies?

A

Hyaline membrane disease of newborn

402
Q

What is the basic pathology in ARDS and hyaline membrane disease of newborn?

A

Diffuse alveolar damage

403
Q

What are the risk factors / associations with hyaline membrane disease?

A 
Prematurity
Maternal diabetes
Second twin
Caesarean section
Birth asphyxia
404
Q

What complications can hyaline membrane disease cause?

A
  • Respiratory failure and death within 48 hours
  • Pneumonia
  • Interstitial emphysema
  • Bronchopulmonary dysplasia: fibrous scarring
405
Q

What are Charcot–Leyden crystals?

A

Microscopic crystals found in people who have allergic diseases such as asthma or parasitic infections.

406
Q

What are Curschmann’s spirals?

A

Desquamated epithelium seen in biopsies from asthmatic patients.

407
Q

What is the definition of chronic bronchitis?

A

> Chronic cough productive of sputum

> Most days for at least 3 months over at least 2 consecutive years

408
Q

What are the histological features of chronic bronchitis?

A

> Dilatation of airways
Hypertrophic mucous glands
Goblet cell hyperplasia

409
Q

What main complicatiosn arise from chronic bronchitis?

A

> Repeated infections
Chronic hypoxia and reduced exercise tolerance
Chronic hypoxia results in pulmonary hypertension and right sided heart failure (cor pulmonale)
Increased risk of lung cancer independent of smoking

410
Q

What is the definition of emphysema?

A

Emphysema is a permanent loss of the alveolar parenchyma distal to the terminal bronchiole.

411
Q

How does the pattern of emphysema differ between a cause of smoking or alpha-1-antitrypsin deficiency?

A

Smoking > Loss centred on bronchiole - CENTRILOBULAR

Alpha-1-antitrypsin deficiency > Diffuse loss of alveolae - PANACINAR

412
Q

What main complicatiosn arise from emphysema?

A

Bullae > rupture = pneumothorax
Respiratory failure
Pulmonary hypertension > cor pulmonale

413
Q

What is the definition of bronchiectasis?

A

Permanent abnormal dilatation of bronchi - site depends upon cause.

414
Q

What are the causes of bronchiectasis?

A
  • Congenital
  • Post-infectious (esp children or cystic fibrosis)
  • Immunodeficiency: 1º [hypogammagl.] and 2º [chemotherapy, NG]
  • Ciliary dyskinesia: 1º [Kartagener’s] and 2º
  • Obstruction (extrinsic/intrinsic/middle lobe syn.)
  • Post-inflammatory (aspiration)
  • Secondary to bronchiolar disease (OB) and interstitial fibrosis (CFA, sarcoidosis)
  • Systemic disease (connective tissue disorders)
  • Asthma
415
Q

What are the main complications from bronchiectasis?

A
  • Recurrent infections
  • Haemoptysis
  • Pulmonary Hypertension > cor pulmonale
  • Amyloidosis
416
Q

What is the location of the cystic fibrosis gene?

A

CFTR gene (Cystic Fibrosis Transmembrane Conductance Regulator) = 7q3

417
Q

What are the main systemic effects of cystic fibrosis?

A

Lung -> airway obstruction, respiratory failure, recurrent infection
GI tract -> meconium ileus, malabsorption
Pancreas -> pancreatitis, malabsorption
Liver -> cirrhosis
Male reproductive system -> infertility

418
Q

What organisms do patients with cystic fibrosis get repeatabley infected with?

A

S.aureus, H. influenzae, P.aeruginosa, B.cepacia

419
Q

What are the main complications from cystic fibrosis?

A

Recurrent infections
Haemoptysis
Pneumothorax
Chronic respiratory failure and cor pulmonale
Allergic bronchopulmonary aspergillosis (ABPA)
Atelectasis
BRONCHIECTASIS

420
Q

What are the histological features of bronchopneumonia?

A
  • Patchy bronchial and peribronchial distribution
  • Often lower lobes
  • Acute inflammation surrounding airways and within alveoli
421
Q

What are the histological features of lobar pneumonia?

A
  1. Congestion: Hyperaemia, Intra-alveolar fluid
  2. Red hepatization: Hyperaemia, Intra-alveolar neutrophils
  3. Grey hepatization: Intra-alveolar connective tissue
  4. Resolution: Restoration normal architecture
422
Q

What are the main complications from pneumonia?

A
  • Abscess formation
  • Pleuritis and pleural effusion
  • Infected pleural effusion (EMPYEMA)
  • Fibrous scarring
  • Septicaemia
423
Q

What is the lung involvement in sarcoidosis?

A

Discrete epithelioid and giant cell granulomas, preferential distribution in upper zones with tendency to be perilymphatic, peribronchial.
Advanced disease may be fibrotic and cystic.

424
Q

What is the definition of pulmonary hypertension?

A

Mean pulmonary arterial pressure > 25mmHg at rest

425
Q

What are the morphological changes in pulmonary vessels with chronic hypoxia?

A

Eccentric intimal fibrosis,

Thickening of muscle wall.

426
Q

What is the effect of a small pulmonary embolus?

A
  • Small peripheral pulmonary arterial occlusion > haemorrhagic infarct
  • Repeated emboli cause increasing occlusion of pulmonary vascular bed > pulmonary hypertension
  • Patients present with pleuritic chest pain or chronic progressive shortness of breath
427
Q

What is the effect of a large pulmonary embolus?

A
  • Large emboli can occlude the main pulmonary trunk (saddle embolus)
  • Sudden death, acute right heart failure, or cardiovascular shock occurs in 5% of cases when >60% of pulmonary bed is occluded
  • If patient survives, the embolus usually resolves
  • 30% develop second or more emboli
428
Q

Examples of non-thrombotic emboli

A 
Bone marrow
Amniotic fluid
Trophoblast
Tumour
Foreign body (e.g. talc in IVDU)
Air
429
Q

What are the causes of pulmonary veno-occlusive disease?

A

Fibrotic/collagenous occlusion of pulmonary veins. Causes:

  • IDIOPATHIC
  • Some “herbal” teas and diet pills
  • Chemotherapy
  • Radiotherapy
  • Bone marrow transplantation
  • Renal transplantation
  • HIV infection
  • Systemic sclerosis
430
Q

What are the main complications of pulmonary hypertension?

A

Right sided heart failure:

  • Venous congestion of organs – “nutmeg liver”
  • Peripheral oedema
  • Pleural effusions and ascites
  • Poor lung perfusion and hypoxia
431
Q

What is idiopathic pulmonary fibrosis also know as?

A

Cryptogenic fibrosing alveolitis

432
Q

What is farmers lung?

A

Aka Extrinsic allergic alveolitis
Reaction to inhaled antigen, acute or chronic.
Responds well to avoiding antigen and steroids. Some develop fibrosis.

433
Q

What is dusty lung?

A 
Aka Pneumoconiosis = permanent alteration of lung structure by inhaled inorganic dust and the tissue reaction of the lung to its presence, excluding bronchitis and emphysema.
Coal workers’ lung 
Silicosis
Asbestosis 
Berylliosis, Kaolin, haematite
434
Q

What are the histological features of asbestosis?

A
  • Fine subpleural basal fibrosis with asbestos bodies in tissue
  • May also see pleural disease - fibrosis, pleural plaques
  • Increased risk of lung cancer in the presence of asbestosis
435
Q

What are the frequencies of the most common lung cancers?

A 
Small cell carcinoma 20%
Non-small cell lung cancer:
> Squamous cell carcinoma 35%
> Adenocarcinoma 27%
> Large cell carcinoma – uncommon 10%
436
Q

What types of lung cancer are most strongly associated with smoking?

A

Squamous cell carcinoma and small cell carcinoma

437
Q

What are the risk factors for lung cancer, other than smoking?

A

> Asbestos exposure (Asbestos + smoking = 50x risk)
Radiation (Radon, theraputic, uranium miners)
Heavy metals (Chromates, arsenic, nickel)
Family history (familial lung cancers rare)

438
Q

What is the pathway of development for squamous cell carcinoma of the lung?

A 
Normal epithelium
Hyperplasia
Squamous metaplasia
Dysplasia
Carcinoma in situ
Invasive carcinoma
439
Q

What is the pathway of development for adenocarcinoma of the lung?

A

Atypical adenomatous hyperplasia
Non-mucinous BAC
Mixed pattern adenocarcinoma

440
Q

What does histology of lung adenocarcinoma show?

A

Evidence of glandular differentiation, stains mucin positive

441
Q

What molecular pathways are involved in adenocarcinoma pathogenesis?

A

Smokers: K-ras mutation, DNA methylation and p53

Non-smokers: EGFR mutation/amplification

442
Q

What mutations are common in small cell carcinoma?

A

p53 and RB1

443
Q

What drug targets EGFR and is sometimes used to treat adenocarcinoma lung cancer?

A

Cetuximab

444
Q

Which paraneoplastic syndromes are strongly associated with small cell carcinoma?

A

SIADH and Cushing’s syndrome (ACTH)

445
Q

Which paraneoplastic syndromes are strongly associated with squamous carcinoma?

A

Parathyroid hormone-related peptide -> hypercalcaemia

K1s Path (16 decks)

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