Histopathology Flashcards

Question

What does NAFLD look like? What causes it?

Answer 1

Histologically looks like alcoholic liver disease Due to insulin resistance associated with raised BMI and DM

Answer 2

Primary biliary cirrhosis Bile duct loss associated with chronic inflammation: granulomas AMAs (anti-mitochondrial)

Answer 3

Primary biliary cirrhosis Bile duct loss with granulomas

Answer 4

Primary sclerosing cholangitis Periductal bile duct fibrosis leading to loss Associated with UC Increased risk of cholangiocarcinoma ERCP Dxic

Answer 5

Primary sclerosing cholangitis

Answer 6

Genetically determined increased gut iron absorption Gene on chromosome 6 Parenchymal damage to organs secondary to Fe deposition-\> "bronzed diabetes"

Answer 7

Haemochromatosis

Answer 8

Accumulation of Fe in macrophages Seen following blood transfusion

Answer 9

Accumlation of Cu due to feailure of excretion by hepatocytes Genes on chromosome 13 Accumulates in the liver and CNS-\> hepatolenticular degenration

Answer 10

Wilson's wth a rhodanine stain.

Answer 11

Keyser-Fleischer rings

Answer 12

Interface hepatitis with plasma cells. Anti-SMA Abs Responds to steroids.

Answer 13

Failure to secrete alpha-one antitrypsin Leads to intra-cytoplasmic inclusions, hepatitis and cirrhosis

Answer 14

Alpha-1 antitrypsin

Answer 15

Specific: PBC, drugs General: TB, sarcoid

Answer 16

Liver cell adenoma bile duct adenoma haemangioma

Answer 17

Secondary \> primary

Answer 18

HCC Hepatoblastoma Cholangiocarcinoma Haemangiosarcoma

Answer 19

PSC, helminth infection, cirrhosis, congenital liver abnormalities, Lynch syndrome Type II Intrahepatic ducts, extrahepatic ducts (including GB) Poor

Answer 20

Cholangiocarcinoma

Answer 21

Associated with OCP Presents with abdo pain/ intraperitoneal bleeding Resection if symptomatic, \>5cm or if no shirnkage when stopping OCP

Answer 22

Most common benign lesion No Rx

Answer 23

Hepattis B + C, ETOHic cirrhosis. Haemocrhomatosis, NAFLD, aflatoxin, androgenic steroids. Ix: alpha fetoprotein, USS

Answer 24

MMR defect Type 1: HNPCC Type 2: Extracolonic

Answer 25

GI, breast or bronchus

Answer 26

ALD NAFLD Chronic viral hepatitis (B+/-D, C) Autoimmune Biliary causes:PBC and PSC Genetic: haemochromatosis (HFE gene), Wilsons (ATP7B gene), A1AT, galactosaemia, glycogen storage disease Drugs e.g. methotrexate

Answer 27

Uniform liver involvement (\<3mm) Alcoholic hepatitis, biliary tract disease

Answer 28

Variable nodule size (\>3mm) Viral hepatitis, Wilson's, A1AT

Answer 29

Chronic inflammation causes stellate cell activation in the space of Disse They become myofibroblasts that initiate fibrosis by deposition of collagen in space of Disse Myofibroblasts contract, constricting sinusoids and increasing vascular resistance. Undamaged hepatocytes regenerate in nodules between fibrous septa

Answer 30

Modified Child's Pugh Ascites Encephalopathy Bilirubin Albumin Prothrombin time

Answer 31

\<7: 45% 5 year survival 7-9: 20% \>10: \<20%

Answer 32

Increased vascular resistance in liver Hyperdynamic circulation Sodium retention and plasma volume expansion When portal pressure \>10-12 mmHG, venous system dilates and collateral vessels form

Answer 33

GO junction Rectum L renal vein Diaphragm Retroperitoneum Anterior abdominal:umbilical vein

Answer 34

Pre-hepatic: Portal vein thrombosis: Factor V leiden _Hepatic_ Pre-sinusoidal: Schistosomiasis, PBC, sarcoid Sinusoidal: cirrhosis Post-sinusoidal: veno-occlusive disease Post-hepatic: Budd-Chiari syndrome

Answer 35

30% idiopathic Thrombophilia OCP Leukaemias Compression by renal tumours, HCC RTx Thrombolyse, treat underlying cause. TIPS

Answer 36

Transjugular Intrahepatic portosystemic shunt

Answer 37

Large, pale, yellow, greasy Accumulation of fat droplets in hepatocytes Fully reversible if ETOH avoided

Answer 38

Large, fibrotic liver Hepatocyte ballooning and necrosis due to accumulation of fat, water and proteins Mallory bodies Fibrosis Seen acutely after heavy night of drinking. Can range from asymptomatic to fulminant liver failure

Answer 39

Yellow-tan, fatty, enlarged, transforms into shrunken, non-fatty brown organ Micronodular cirrhosis (\<3mm)

Answer 40

Hepatic steatosis in non-ETOHs Most common cause of chronic liver disease in West NAFLD: simple steatosis NASH: steatosis and hepatitis, can progresss to cirrhosis

Answer 41

Common with other autoimmune disease e.g. coeliac, SLE\< RA, thyroiditis, Sjogrens, UC 78% female, young and postmenopausal HLA-DR3

Answer 42

ANA, anti-SMA\< anti-actin, anti-soluble liver Ag

Answer 43

Anti-LKM (liver, kidney, microsomal)

Answer 44

Immunosuppression until transplant. Disease recurs in 40%

Answer 45

Auotimmune inflammatory destruction of medium sized intra-hepatic bile ducts-\> cholesstasis-\>slow development of crrhosis F\>M 10:1 Peak incidence 40-50y/o raised ALP, cholesterol, IgM, hyperbilirubinaemia (late) AMA in \>90% US scan shows no bile duct dilatation Histology: bile duct loss with granulomas Fatigue, pruritus and abdo discomfort. Skin pigementation, anthelasma, steatorrhoea, Vit D malabsorption, inflammatory arthropathy Ursodeoxycholic acid in early phase

Answer 46

Inflammation and obliterative fibrosis of extrahepatic and intrahepatic bile ducts-\> multi-focal stricture formation with dilation of preserved semgnets M\>F Peak incidence at 40-50y/o IBD associated (UC) Raised ALP\< several associated auto-Ig (p-ANCA) US: bile duct dilatation ERCP: shows beding of the bile ducts due to multifocal strictures Increased incidence of cholangiocarcinoma

Answer 47

Genetic 40-50y/o **Autosomal recessive:** mutated HFE gene 6p21.3-\> fe absorption which deposits in liver, heart, pancreas, adrenals, pituitarry, joints, skin-\> fibrosis Fe deposits in liver stains with Prussian blue Skin bronzing (melanin deposition), DM, hepatomegaly, cardiomyopathy, hypogonadism, pseudogout Ix: raised Fe, ferritin, transferrin sa \>45%, decreased TIBC Venesection Desferrioxamine 30% with cirrhosis -\> HCC

Answer 48

v. rare 11-14y **Autosomal recessive**: mutated ATP7B (Chr13) encodes Cu transporting ATPase-\> decreased biliary Cu excretion and deposition in liver, CNS, iris. Cu stains with Rhodanine stain, Mallory bodies and fibrosis on microscopy. Liver disease: acute hepatitis, fulminant liver failure or cirrhosis. Neuro disease: parkinsonism, psychosis, dementia Decreased serum caeruloplasmin, decreased serum Cu, increased urinary Cu Lifelong penicillinamine

Answer 49

**Autosomal dominant:** A1AT accumulates in hepatocytes -\> intracytoplasmic inclusions -\> hepatitis. lack of A1AT in lungs -\> emphysema Intracytoplasmic inclusions of A1AT which stain with periodic acid Schiff Kids: neonatal jaundice Adults: emphysema and liver disease Ix: redcued A1AT absent alpha globulin band on electrophoresis.

Answer 50

Mechanical: support and site of muscle attachment Protective: vital organs and bone marrow Metabolic: Ca reserve

Answer 51

Inorganic: (65%) Ca hydroxyapatite (10Ca 6PO4 OH2) body store for 99% of body Ca 85% of P, 65% Na and Mg Organic: bone cells and protein matrix

Answer 52

Medulla, Cortex, Periosteum

Answer 53

Diaphysis Metaphysis Epihpyseal line Epiphysis Subchondral bone Articular cartilage

Answer 54

Long bones 80% of bony skeleton Appendicular 80-90% calcified Mainly mechanical and protective

Answer 55

Vertebrae and pelvis 20% of skelton Axial 15-25% calcified Mainly metabolic Large surface

Answer 56

Cancellous

Answer 57

Woven/lamellour Anatomically: flat/long bones - intramembranous and anedochondral ossification Trabecular (cancellous)/compact (cortical)

Answer 58

Build bone by laying down osteoid

Answer 59

Multinucleate cells of macrophage family Resorb bone

Answer 60

Osteoblast like cells which sit in lacunae in bone

Answer 61

In histology, a lacuna is a small space containing an osteocyte in bone or chondrocyte in cartilage. The Lacunae are situated between the lamellae, and consist of a number of oblong spaces.

Answer 62

RANK is expressed on the surface of osteoclast lineage cells RANKL expressed on MSCs of ostebolast lineage and on B and T Ls When RANKL binds to RANK this causes osteoclast precursor cell to differentiate, increasing bone resorption OPG competed with RANK for RANKL

Answer 63

Oncogene products produced by tumour cells metastasising to bone influence the bone cells to resorb bone and promote local growth of the tumour. This is mediated by the RANK /OPG signalling pathway.

Answer 64

Prostate carcinoma

Answer 65

Disordered bone turnover due to imblanace of chemicals in body: vitamins, hormones, minerals Net effect: reduced bone mass-\> pathological #

Answer 66

Non-endocrine e.g. age-related Endocrine: e.g. Vit D, PTH Disuse osteopenia

Answer 67

Static parameters: cortical thickness and porosity, trabecular bone volume, thickness, number & separation of tranbeculae Biopsy from iliac crest

Answer 68

Trabecular (cancellous) bone The trabecular bone, the grey is the marrow

Answer 69

Fluorescent tetracycline labelling

Answer 70

1o: age, post-menopause 2o: drugs, systemic disease

Answer 71

Pathogenesis: low intiial bone mass or accelerated bone loss can reduce bone mass below # threshold 90% of cases due to insufficient Ca intake and post-menopausal oestrogen deficiecny High turnover: increased bone resorption Low turnover: decreased bone formation

Answer 72

Nutrition and social practices: ETOH, smoking, malabsorption, Vit C & DD Endocrine Immobilisation Iatrogenic: corticosteroids, long term heparin or phenyotin therapy, casstration, XS thyroid therapy

Answer 73

Advanced age Female Smoking ETOH Early menopause LT immobility Low BMI Poor Diet/malabsorption Thyroid disease Low testosterone Chronic renal disease Steroids

Answer 74

50% of patients cannot live independently post # 20% die

Answer 75

Back pain and # Classic #s: wrist (Colle's) hip: NOF and intertrochanteric pelvis \>60% vertebral #s are asymptomatic with compresion # usually in T11-L2

Answer 76

Lab: Serum Ca, P and ALP (usually N) Urinary Ca Collagen breakdown products Imaging Bone densitometry

Answer 77

T score: 1-2.5SD below normal peak bone mass= osteopenia \>2.5SD= osteoporosis

Answer 78

Defective bone mineralisation: either Vit DD or PO4 deficiency

Answer 79

Bone pain/tenderness # Proximal weakness Deformity

Answer 80

Osteomalacia (Rickets) ## Footnote There is a widening of the growth plate which is also irregular and the femur and tibia become bowed as the child starts to walk and the legs have to weight bear.

Answer 81

Horizontal # in Looser's zone Osteomalacia

Answer 82

* Excess PTH * increased Ca + PO4 excretion in urine * hypercalcaemia * hypophosphataemia * skeletal changes of osteitis fibrosa cystica

Answer 83

1o: parathyroid adenoma (85-90%) or chief cell hyperplasia 2o: chronic renal deficiency, Vit DD, malabsorption

Answer 84

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

Answer 85

Comprises all the skeletal changes of chronic renal disease Increased bone resorption: osteitis fibrosa cystica Osteomalacia Osteosclerosis Growth retardation Osteoporosis

Answer 86

Osteitis fibrosa cystica: increase bone resorption

Answer 87

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

Answer 88

Disorder of bone turnover Osteolytic Osteolytic-osteosclerotic Quiescent osteosclerotic

Answer 89

Paget's disease of bone

Answer 90

Onset \>40y M=F Rare in asians/africans Mono-ostotic in 15%, remained polyostotic Aetiology is unknown Familial pattern shows autosomal pattern with incomplete penetrance Parvomyxovirus type particles have been seen in Pagetic bone

Answer 91

Vertebrae Skull Pelvis Femur Tibia

Answer 92

Pain micro# Nerve compression Skull changes may put medulla at risk +/- haemodynamic chanes, HF Development of sarcoma in area of involvement in 1%

Answer 93

Paget's disease affecting tibia

Answer 94

Suspected osteomalacia Diagnostic classification of renal osteodystrophy Osteopaenia: in young patients (\<50) or associated with abnormal Ca metabolism Classification of hereditary childhood bone disease

Answer 95

No XR Loss of cancellous bone N Ca, N PO4, N ALP

Answer 96

Looser's zones: pseudo#s, splaying of metaphysis Excess of unmineralised bone: osteoid N/L Ca, L PO4, H ALP

Answer 97

Brown's tumours, Salt and pepper skull, Subperiosteoal bone resorption in phalanges Osteitis fibrosa cystica: marrow fibrosis and cysts aka Brown tumour Raised Ca,, Low/N PO4, Raised/N ALP

Answer 98

The brown tumor is a bone lesion that arises in settings of excess osteoclast activity, such as hyperparathyroidism. It is not a true neoplasm, as the term "tumor" suggests; however, it may mimic a true neoplasm.[1]Brown tumours are radiolucenton x-ray.

Answer 99

Hyperparathyroidism: Brown's tuour of the hands

Answer 100

Salt and pepper sign of the calvaria refers to multiple tiny hyperlucent areas in the skull vault caused by resorption of trabecular bone in hyperparathyroidism. There is loss of definition between the inner and outer tables of the skull and a ground-glass appearance as well as spotty deossification.

Answer 101

Mixed lytic and sclertoic. Skull: osteoprorosis circumscripta, cotton wool. Vertebrae: picture frame, ivory vertebrae Pelvis: sclerosis and lucency Huge osteclosts w \>100 nuclei, mosaic (like jigsaw) pattern of lamellar bone N Ca + PO4, +++ALP

Answer 102

Osteoporosis circumscripta cranii (also known as osteolysis circumscripta) refers to discrete radiolucent regions of the skull on plain radiographs. They are often seen in context of the lytic (incipient-active) phase of Paget's disease of the skull, but may be observed in other circumstances as well, e.g. hyperparathyroidism, leontiasis ossea 9.

Answer 103

Cotton wool skull- Pagets

Answer 104

Picture frame vertebra Paget's

Answer 105

Ivory vertebra Paget's

Answer 106

Most abundant Anchor neurones by numerous projections, regulate environment e.g. ions, neurotransmitters and form BBB

Answer 107

Coat axons with their cell membranes forming myelin

Answer 108

Line the cavities of the CNS, make up walls of the ventricles, create and secrete CSF and beat cilia to help move the CSF. Act as neuronal stem cells

Answer 109

Arise from the neuroepithelial cells in embrogenes, act as the scaffold for new neurones

Answer 110

Provide myelination to PNS

Answer 111

Small cells that surround the neurones in sensory sympathetic and parasympathetic ganglia, helping to regulate the environment

Answer 112

Intrinsic ganglia of the GIT

Answer 113

Act as macrophages

Answer 114

Astrocytes Oligodendrocytes Microglia

Answer 115

Ependyma Choroid plexus epithelium Meninges

Answer 116

Endothelium and pericytes

Answer 117

Metastatic neoplasm

Answer 118

Leukaemias and lymphomas, more so in young. Lung, breast and malignant menaloma

Answer 119

May involve the meninges as well as the parenchyma Well demarcated solitary or multiple lesions with surrounding oedema Neurological efffects and raised ICP

Answer 120

Breast Lung Large Bowel Prostate Bladder

Answer 121

Astrocytomas

Answer 122

Any age, elderly usually worse An infiltrative growth pattern in the cerebral hemispheres Grading is based on the degree of dy/dx with histological grade an important predictor of behaviour Raised ICP and focal neuro signs Depends on site, grade and age of the patient

Answer 123

Pilocytic astrocytomas: more common in children but can occur any age. Anaplastic astrocytoma Glioblastoma multiforme: necrotic, poorly differentiated tumour

Answer 124

Most common in adulthood Usually in the cerebral hemisphere and are soft and gelatinous Better demarcated than infiltrating astroctomas Calcifcaition common Px less predictable, dependant on grade site, patient age, cytogenetics etc

Answer 125

Occur at any age Most offen within the ventricular cavities or within the canal of the SC Usually well demarcated Symptoms depend on site: intracranial: hydrocephalus or raised ICP Anaplastic astrocytomas/variants often also disseminate through the sub arachnoid space

Answer 126

In te ventricular cavities In the SC

Answer 127

Composed of embryonal primitive cells Most common in children Undifferentiated lesions Px: most survive 5y or more

Answer 128

Lesion of the cerebellum in the first 2 decades of life Leads to raised ICP and cerebellar signs

Answer 129

Increased since AIDs

Answer 130

Derive from meningioethlial cells Most occur in the brain parenchyma but can occur in the cranial vault and cord. Usually adults Increased in NF2 Usually lobulated lesions, sharp interface between tumour and parenchyma Overlying skull may be thickened or invaded by the tumour Symptoms: raised ICP with focal neurological signs

Answer 131

Meningioma

Answer 132

Pilocytic astrocytoma

Answer 133

Oligodendroma

Answer 134

VHL NF 1 + 2 Li-Fraumeni Gorlin Turcot syndrome

Answer 135

Focal neurological deficit Seizure Altered mental status Headache

Answer 136

Cerebellar ataxia Long tract signs CN palsy

Answer 137

Stereotacic biopsy Open biopsy Craniotomy for debulking

Answer 138

LT survival Causes death in \>5y Death in \<5y Deaith within 6m-1y

Answer 139

Account for 80% of gliomas. Astrocytomas- Oligodendrogliomas and mixed

Answer 140

De novo glioblastoma

Answer 141

From progression of a lower grade astrocytoma

Answer 142

Oligodendroglioma Resection

Answer 143

Pilocytic astrocytoma

Answer 144

Medulloblastoma

Answer 145

Meningioma

Answer 146

Mitotic activity

Answer 147

Pseudoinvasion along Virchow-Robin's space

Answer 148

Infarction Cerebral atherosclerosis

Answer 149

Atherosclerosis affects the larger extracerebral vessels worse, often near the carotid bifurcation or in the basilar artery

Answer 150

Intracerebral atery, usually from the heart or atherosclerotic plaques Usually occurs in the MCA branches

Answer 151

Occurs on border zone, hypoperfusion of the most distal edges of the blood supply. Not necessarily occlusive. ACA and MCA most at risk Site: distally is affected more than proximal Dependant on the anastamoses around the zone

Answer 152

Between ACA and MCA

Answer 153

Between LCA and MCA

Answer 154

Between MCA and PCA

Answer 155

Under 25hrs, self-limiting vascular obstruction, emboli and or platelet-fibrin aggregates

Answer 156

1/3rd will get a significant infarct within 5 years

Answer 157

Haemorrhage into the brain substance, usually rupture of one of the small intraparenchymal vessels Raised ICP, rapid LOC Basal ganglia, abrupt onset

Answer 158

50% HTN Weakening of the walls, accelerated atheroscleroris in LV, hyaline atherosclerosis in smaller vesseles. Clotting disorders, neoplasms, amyloid, vasculitis and vascular malformations also contribute

Answer 159

Rupture of Berry aneurysm (present in 1% of the population) 80% ICA bifurcation, 20% vertebrobasilar circulation 30% of patients they are multiple

Answer 160

Greatest risk of rupture Mass lesion

Answer 161

Sudden onset, severe headache, vomiting, LOC 50% die within a few days, worse Px if there were warning bleeds

Answer 162

AVM Capillary telangectasias Venous Angiomas Cavernous angiomas Important cause of ICH and developmental abnormalities

Answer 163

Meningeal artery rupture which lies between the dura mater. Associated with skull fractures

Answer 164

Lucid interval followed by progressive LOC. Bleeding is arterial and there is subsequent mass effect

Answer 165

Between the dura and the arachnoid mater. Disruption of the bridging veins. Associated with rapid change of head velocity which leads to tearing of the veins. Usually clear Hx of tumour caused by raised ICP. Venous bleeding therefore slower development than EDH.

Answer 166

There is a less good history of trauma and it is associated with brain atrophy. Often causes vague alteration in mental state rather than classical features of raised ICP

Answer 167

Occurs due to sudden acceleration or deceleration with sufficient force to tear nerve cell process in the cerebral white matter

Answer 168

Concussion, transient LOC and neurological deficit. Sometimes with seizrues with recovery over hours or days. Diffuse axonal injury: causes most post-traumatic dementia and with hypoxic ischaemic injury is the leading cause of persistent vegetative state

Answer 169

Contustion at site vs away from site of impact Need to know the MOI

Answer 170

Bleeding in the ventral and paramedian part of the upper brainstaim (pons and midbrain)

Answer 171

Gyral flat and te sulci are obliterated

Answer 172

Anything that can damage the BBB Can be classified as Vasiogenic: where the integrity of the BBB is disrupted which is aggravated by the lack of lymphatic CNS drainage Cytotoxic: secondary to cellular injury e.g. due to general hypoxic ischaemic injury.

Answer 173

Due to raised ICp, can force the brain against unyielding bony wall

Answer 174

Cingulated gyrus displaced under falx cerebri

Answer 175

Uncal gyral, medial temporal lobe compressed against the free margin of the tentorium cerebelli Results in compression of the PCA and oculomotor nerve

Answer 176

Cerebellar tonsils herniate through the foramen magnum causing brainstem compression

Answer 177

Sudden onset, FAST, numbness, loss of vision, dysphagia MCA most common CT/MRI (infarct vs haemorrhage) Ix for vascular risk: BP, FBC, ESR, U&E, glucose, lipids, CXR, ECG, carotid doppler Aspirin +/- dipyridamole Thrombolytics (\<3h) +/'- caroit dendarterectomy LT: treat HTN, reduce lipids, anticoagulate

Answer 178

\<24hrs, amaurosis fugax, carotid bruit Any however characteristically embolic atherogenic debris from the carotid artery travels to the opthalmic branch of the internal caroitd Cartoid USS IX for vascular risk as in stroke As for stroke except no thrombolysis

Answer 179

85% PKD, Ehler's Danlos and patients with coarctation Also associated with AVMs, capillary telangectasias, venous and cavrnous angiomas

Answer 180

Non traumatic: ICH SAH Traumatic: EDH SDH

Answer 181

Uncal Central (transtentorial) Cingulate (subfalcine) Transcalvarial Upward Tonsilar

Answer 182

Cerebral oedema

Answer 183

Communicating/non-obstructive Non-communicating/obstructive

Answer 184

Impaired reabsorption of CSF: Normal pressure hydrocephalus Hydrocephalus ex-vacuo

Answer 185

Associated with elevated CSF causing increased ICP and increased ventricular size Thought to be scondary to an increase in fluid levels. Infection, tumours, trauma and haemorrhage

Answer 186

Atrophy causing secondary enlargement of the ventricle and subarachnoid space Not the result of increased CSF production pressure but a compensatory enlargement of ventricle due to loss of brain parenchyma

Answer 187

Actual obstruction to CSF flow Adhesion, external compression, interventricular cysts and tumours

Answer 188

A stroke is a clinical syndrome characterised by rapidly developing clinical symptoms and / or signs of focal, and at times global loss of cerebral function, with symptoms lasting more than 24 hours or leading to death, with no apparent cause other than that of vascular origin (Hatano, 1976). This definition includes stroke due to cerebral infarction, primary intracerebral haemorrhage, intraventricular haemorrhage, and most cases of subarachnoid haemorrhage It excludes subdural haemorrhage, epidural haemorrhage, or intracerebral haemorrhage (ICH) or infarction caused by infection or tumour.

Answer 189

Focal: defined bascular territory Global: failure of systemic circulation

Answer 190

Atypical refers to odd anatomy – not classical vascular territory Clinical features and neuroimaging can be misleading. “Atypical brain infarct may mimic tumors”

Answer 191

Surgery, embolisation, Radiosurgery

Answer 192

* Occur anywhere in the CNS * Becomes symptomatic between 2nd and 5th decade (mean age 31.2 years) * Present with haemorrhage, seizures, headache, focal neurological deficits * High pressure – MASSIVE BLEEDING!!! * Seen at angiography * Risk of bleeding 1.3-3.9% yearly * Risk of re-bleeding 6.0-6.9% during the first year * Morbidity after rupture 53-81% - high in eloquent areas * Mortality 10-17.6%

Answer 193

“Well-defined malformative lesion composed of closely packed vessels with no parenchyma interposed between vascular spaces” Anywhere in the CNS Usually symptomatic after age 50 Pathogenesis unknown Rarely multiple – familial (linkage chrom. 7p, 7q, 3q) Present with headache, seizures, focal deficits, haemorrhage A few feeding vessels Low pressure – RECURRENT BLEEDINGS Angiographically usually negative Therapy: surgery

Answer 194

INFARCT 1. Tissue necrosis (stains) 2. Rarely haemorrhagic 3. Permanent damage in the affected area (except for penumbra) 4. No recovery HAEMORRHAGE 1. Bleeding 2. Dissection of parenchyma 3. Less macrophages 4. Limited tissue damage (periphery) 5. Partial recovery

Answer 195

Primary Skull fracture 75% Surface contusions 95% Diffuse axonal injury (DAI) 30% Intracranial haematoma 60% Secondary Brain swelling 53% Ischaemic brain damage 55% Infection 4% Due to raised ICP 75%

Answer 196

66% of fatal non-missile head injury 10% extradural 56% subdural, subarachnoid, intracerebral or burst lobe surgical evacuation

Answer 197

Functional conective areas in the brain

Answer 198

GABA: inhibitory Glutamate: excitatory Ach, dopamine, serotonin

Answer 199

Resident macrophage population constituting 20% CNS cells. Have motile processes. Involved in immune surveillance and tissue reomdelling (synaptic stripping) Cytotoxic in neurodegeneration and neuroinflammation

Answer 200

Progressive, irreversible condition leading to neuronal loss Often caused by intra or extracellular accumulation of a misfolded protein. Usually sporadic resulting in dementia. Common in the ageing population

Answer 201

Conditions characterised by an innate and/or adaptive inflammatory response. Can be reveersible, progressive irreversible

Answer 202

A serious loss of global cognitive ability in a previously unimpaired person beyond what might be expected from normal ageing. Can be a static event e.g. from TBI or due to neurodegeneration.

Answer 203

Difficult to diagnose on symptoms alone, some have a distinct clinical phenotype which gives a high probability of a dx e.g. PD. Based on neuroimaging, brain biopsy or PM examniation

Answer 204

Tau, beta-amyloid, alpha-synuclein, Huntingtin, PrP, Fus

Answer 205

Dense deposits around neurones (neuritic plaques) Twisted bands of fibre (NFTs) Begins after 5th-6th decade, pathogenesis unclear.

Answer 206

Senile plaques: complex spherical structures involving the grey matter. Diffuse plaques are amorphous and seen in ageing brains. Neuritic plaques consist of clusters of radially orientated abnormal axons and dendrites= dystrophic dendrites

Answer 207

Ubiquitin abnormally accumulates inside cells, indicates a disease process. These protein accumulations= inclusion bodies NFTs are aggregates of hyperphosphorylated Tau proteins.Intracellular structures composed of two filaments wound in a double helix

Answer 208

AD, progressive SNP, some other uncommon conditions

Answer 209

Normal component of the neuronal cytoskeleton, stabilises MTs. 6 isoforms. Function is regulated by kinases. Unphosphorylated or hyperphosphorylated Tau fails to bind to MTs, accumulating in NFTs and neuritic plaques

Answer 210

Senile plaque

Answer 211

Age FHx T21 Head trauma PD and depression

Answer 212

Clinical suspicion MRI and PET scans helpful Role of biopsy unclear Dx and PM is definitive

Answer 213

Derived from APP which is a ubiquitously expressed membrane molecule. Cleaved by secretases which are regulated by Presenilin 1 Amyloid accumulation results from defective APP cleavage

Answer 214

Beta amyloid

Answer 215

Fragment 39-42

Answer 216

Produced by astrocytes and mediates phospholipid and cholesterol mobilisation ApoE4 has a high affinity to beta amyloid and less activity. ApoE4 is a risk factor for late onset sporadic AD

Answer 217

Acetylcholinesterases: Tacrine (Cognex(), Donepezil, Rivastigmine. Produce mild benefits without influencing progression nAChRs: galantamine Glutamate antagonist: memantine (also used in vascular dementia)

Answer 218

Parkinsonian syndromes: Primary: PD Secondary Parkinson-plus syndrome Familial Neurodegenerative diseases

Answer 219

PD (sporadic, familial)

Answer 220

Drug induced: dopamine antags and depletor Hemiatryophy-hemiparkinsonism Normal pressure hydrocephalus Hypoxia Infectious: postencephalitic Toxin: e.g. MPTP Trauma Tumour Vascular: multiinfarct state

Answer 221

Cortical-basal ganglionic degeneration Dementia sydnromes: AD, LBD, frontotemporal dementia Lytico-Bodig (ALS) Multiple system atrophy syndromes: Shy Drager, OPCA, MND parkinsonism Progressive pallidal atrophy PSN Palsy

Answer 222

Hallervoden-Spatz Huntingoton Lubag Mitochondrial cytopathies with Striatal Necrosis Neuroacanthocytosis. WIlsons

Answer 223

Usually sporadic, develops after 50y Pathogenesis unclear

Answer 224

Attributed to cell death of the dopamine producing neurones in the SN Need to lose 80-85% of the dopaminergic neurones and deplete dopamine levels by 70% before they appear. Cell death in substantita nigra results in depigmentation Lewy Bodies found in cell bodies and Lewy neurites in neuronal projections Alpha-synuclein and ubiquitin positive

Answer 225

Resting tremor, rigidity, bradykinesia, autonomic dysfunction, dysphagia Psychiatric: hallucinations, anxiety and dementia L-DOPA responsiveness

Answer 226

Small protin associated with synaptic membrane, accumulates and has toxic effect. Mutations reported in instances of familial parkinson's

Answer 227

Lewy Bodies

Answer 228

Viral infections: PML (progressive multifocal leukoencephalopathy caused by JC virus in immune deficiencies Genetic: leukodystrophies Autoimmune: MS, acute haemorrhagic encephalomyeltisi, acute disseminated encephalomyelitis Nutritional: central pontine myleniosis

Answer 229

Produced by oligodendrocytes, fundamental for axon conduction, highly susceptible to damage. Lipid rich insulating membrane

Answer 230

Peak age: 20-40y Assocaited with some HLAs Usually presents with focal symptoms, optic neuritis and poor coordination

Answer 231

Relapsing remitting: recovery less severe, evolves into secondary progressive agter years Primary progressive (10%): no recovery after episodes of demyelination: severe Progressive relapsing

Answer 232

Rare variant of MS Aka Devic disease Autoimmune disease against the SC and optic nerves with auqaporin 4 attacked

Answer 233

Rare variant of MS Rapidly progressive with concentric scoliosis

Answer 234

Loss of myelin, lesions centred by veins with sharpy margins Axonal preservation in early plaques Active plaques- glial scar Remyleinating shadow plaques Infiltration of macrohpages. Cortical involvement is the leading cause of disability, CI leading to dementia, related to inflamm rather than demyelination

Answer 235

Tau, beta-amyloid

Answer 236

Alpha synuclein, ubiquitin

Answer 237

Generalised atrophy of the brain Widened sulci Narrowed Gyri Enlarged ventricles (most marked in the temporal and frontal lobes with loss of cholinergic neurones)

Answer 238

Psychological disturbances occur early, day to fluctuations in cogntiive performance Vsiual hallucinations Spontaneous motor signs of Parkinsonism Recurrent falls and syncope

Answer 239

Degenerative neurological disorder characterised by features that can present in a similar manner to Parkinson's but show a poort response to parkinson's medication Shy Drager Stratonigral Olivopontocerebellar

Answer 240

MSA: autonomic dysfunction

Answer 241

Difficulty with movement

Answer 242

Difficulty with balance and coordination

Answer 243

vSporadic neuropsychiatric disorder vPatients \<45 yrs old vCerebellar ataxia vDementia vLonger duration than CJD vLinked to BSE

Answer 244

* Extracellular plaques * Neurofibrillary tangles * Cerebral amyloid angiopathy (CAA) * Neuronal loss (cerebral atrophy)

Answer 245

Cortical atrophy- AD

Answer 246

alpha-synuclein immunostaining

Answer 247

Excretion of metabolic waste products and foreing chemicals Regulation of fluid and electrolyte balance, acid base balance Hormone secretion: EPO, Renin and 1,25 cholecaliferol

Answer 248

Failure to: Filter an adequate amount of blood resulting in a lack of waste product excretion Failure to maintain a barrier function leading to the loss of protein and/or blood cells in the urine

Answer 249

Syndrome: e.g. ARF, nephrotic etc. Morphological changes: glomerulonephritis, thrombotic microangiopathy Aetiologies: SLE, amyloidosis, drugs and infetions

Answer 250

Composed of a lattice work of Ab and Ag, may become deposited in te glomerulus and lead to an inflammatory response-\> complement activation and stimulation of inflammatory cells through Fc receptors May be endogenous e.g. SLE or exogenous e.g. derived from an infective organism Rate: occur at different rates: rapidly progressive glomerulonephritis vs slow onset Site may bary but glomerular injury is determined by the immune complex location

Answer 251

Bilateral/unilateral agenesis Ectopic e.g. pelvic Horseshoe (usually fused at the lower pole)

Answer 252

Causes 10% of ESRF Cysts arise from all portions of the nephron Renal failure develops from 40-70y Genes: PKD1 and PKD2

Answer 253

Cysts develop in the kidneys of ESRF on dialysis Carcinoma can develop in 7% in 10y

Answer 254

Rapid loss in golmerular filtration and tubular function Results in an abnormal water and electrolyte balance Reduced GFR manifestated as rasied serum creatinine (Normal= \<1.3) and urea (10-20mg/dL) May r3esult in acidosis, hyperkalaemia and fluid overload

Answer 255

According to the site of the nephron it affects 1. Glomerulus Nephrotic syndrome Nephritic syndrome 2. Tubules and interstitium ATN Tubulointerstitial nephritis: acute phyelonephritis, chronic pyelonephritis & refulx nephropathy, interstitial nephritis 3. BVs: Thombotic microangiopathies (HUS, TTP)

Answer 256

Proteinuria (\>3g/24h) Hypoalbuminaemia Oedema (+hyperlipidaemia) "*Swelling"* facial in children, peripheral in adults *"frothy urine"*

Answer 257

Minimal change disease Membranous glomerular disease Gocal segmental glomerluosclerosis

Answer 258

Primary cause of nephrotic syndrome Most common in children (75%) with second peak in elderly No changes Loss of podocyte foot processes No immune deposits \<5% ESRF 90% respond

Answer 259

Primary cause of nephrotic syndrome Common in adults (30%) Diffuse glomerular BM thickening Loss of podocyte foot processes. Subepithelial deposits **spikey** Ig and complements in granular deposits along entire GBM 40% ESRF at 2-20y Can be 1o or 2o to SLE, infections, drugs and malignancy Poor response to steroids

Answer 260

Common in adults (30%), Afrocarribean Focal and segmental glomerular consolidation and scarring. Hyalinosis Loss of podocyte foot processes Ig and complement in scarred areas 50% ESRF in 10y 1o but can 2o to obesity and HIV nephropathy 50% respond

Answer 261

DM Amyloidosis

Answer 262

Diffuse GBM thickening Messangial matric nodules aka **Kimmelstiel Wilson nodules** Asian

Answer 263

**Apple green birefringence** with congo Red May have chronic inflammation: RA, chronic infection. Causes AA protien depositions May have Ig light chain deposition most commonly from MM Clinical clues of amyloidosis: Macroglossia, HF, hepatomegaly

Answer 264

Manifestation of glomerular inflammation i.e. glomerulonephritis Haematuria Dysmorphic RBCs and RBC casts in urine May also have: Oliguria Raised urea and creatinine HTN Proteinuria (not in nephrotic range)

Answer 265

Acute postinfectious IgA Nephropathy (Berger Disease) Rapidly progressive (crescenteric) GN Hereditary nephritis (Alport's) Thin BM Disease (Bening familal haematuria)

Answer 266

Occurs 1-3w post **streptococcal throat infection or impetigo** (usually Group A S= Strep pyogenes) Glomerular damage due to immune complex deposition Haematuria (red cell casts), proteinuria, oedema, HTN Bloods: raised ASOT titre, decreased C3

Answer 267

Findings in Acute Postinfectous GN

Answer 268

IgA Nephropathy Most common GN worldwide IgA complex deposition in glomeruli Presents 1-2d after URTI with Frank haematuria Main symptoms are persistent or recurrent frank haematuria or asymptomatic macroscopic haematuria Can rapidly prgoress to ESRF Biopsy: granular deposition of IgA and complement in mesangium

Answer 269

IgA Nephropathy (Berger)

Answer 270

Acute Postinfectious GN

Answer 271

Most aggressive GN causing ESRF within weeks Presents as nephritic syndrome but oliguria and renal failure are more pronounced

Answer 272

Based on immunological findings: Type 1: Anti-GBM Ab Type 2: Immune complex Type-3: pauci-immune/ANCA associated Regardless of cause all are characterised by crescenets in glomeruli on light micrscopy

Answer 273

Anti-GBM Ab Goodpasture's. HLA-DRB1 association Crescents Linear deposition of IgG in GBM Lungs: pulmonary haemorrhage

Answer 274

Immune complex mediated SLE, IgA nephropathy, postinfectious GN Crescents Granular IgG immune complex deposition on GBM/mesangium Often limited, except in SLE

Answer 275

Pauci-immune i.e. lacking anti-GBM or immune complex c-ANCA: Wenger's granulomatosis p-ANCA: microscopic polyangitis Cresecents Lack/scanty immune complex deposition Vasculitis: skin rashes or pulmonary haemorrhage

Answer 276

Hereditary Nephritis Caused by mutation in Type IV collagen alpha 5 chain X-linked Nephritic syndrome + sensorineural deafness and eye disorders Presents at 5-20y with nephritic syndrome progressing to ESRF

Answer 277

Thin BM disease nephirits Rarely causes nephritic syndrome, normally exclusively asymptomatic haematuria Diffuse thinning of the GBM caused by autosomal dominant mutation in type 4 collagen alpha 4 chain. Usually asymptomatic and diagnosed incidentally Usually normal renal function

Answer 278

Benign Familal Haematuria Berger Alport NB: IgA and Thin BM are more common causes of asymptomatic haematuria than of nephritic syndrome. IgA more likely to cause frank haematuria and change in renal funciton and slighlty more common in Asian pop

Answer 279

Damage to tubular epithelial cells-\> blockage of tubules by casts-\> reduced flow and haemodynamic changes-\> acute renal failure Necrosis of short segments of tubules

Answer 280

Ischaemia: burns, septicaemia Nephrotoxins: drugs (gentamicin, NSAIDs, radiographic contrast agents, mygolobin, heavy metals)

Answer 281

A group of renal inflammatory disorders involving the tubules and interstitium Acute pyelonephritis Chronic pyelonephritis and reflux nephropathy Acute interstitial nephritis Chronic interstitial nephritis/analgesic nephropathy

Answer 282

Bacterial infection of the kidney, usually as a result of ascending infection, most commonly by E. Coli Presents with fever, chills, sweats, flank pain, renal angle tenderness and leukocytosis +/- frequency, dysuria, haematuria Leukocytic casts seen in the urine

Answer 283

Chronic inflammation and scarring of the renal parenchyma caused by recurrent and persistent bacterial infection Can be due to: Chronic obstruciton: posterior urethral valves, renal calculi Urine reflux

Answer 284

A hypersensitivity reaction usually to a drug (NSAID, Abx, diuretics) Usually begins days after drug exposure Presents with fever, skin rash, haematuria, proteinuria, eosinophilia

Answer 285

Seen in elderly with long term analgesic consumption e.g. NSAIDs, paracetamol Symptoms occur late in disease: HTN, anaemia, proteinuria and haematuria

Answer 286

HUS and TTP

Answer 287

Thrombosis generally renal in HUS and widespread in TTP Triad of: Microangiopathic haemolytic anaemia Thrombocytopenia Sometimes renal failure (HUS)

Answer 288

Widespread fibrin deposition in vessels forming platelet fbirin thrombi, damages passing platelets and RBCs Leads to platetel and RBC destruction Resulting in thrombocytopenia and MAHA

Answer 289

Usually affects children Associated with dairrhoea caused by E. Coli 0157:H7 Can be non-diarrhoea associated due to abnromal protines in the complement pathway Thrombi confined to kidney Decreased platelet count-\> bleeding, haematemesis, melena MAHA-\> pallor and jaundice Usually involves renal failure Dx: Anaemia, thrombocytopaenia Signs of haemolyiss: raised bilirubin, LDH and reticulocytes Fragmented RBCs on blood smear Coomb's negative as not AIHA

Answer 290

Usually affects adults Thrombi occurs throughout circulation with CNS involvement particularlr Reduced platelets-\> bleeding MAHA-\> pallor and jaundice Usually no renal involvement, neuro symptoms predominate (headache, altered consciousness, seizures, coma) Dx: As for HUS

Answer 291

A rapid loss of renal funciton manifesting as increased serum creatinine and urea Complications include metabolic acidosis hyperkalaemia, fluid overload, HTN, hypocalcaemia, uraemia

Answer 292

Pre-renal (Most cmmon): renal hypoperfusion e.g. hypovolaemia, sepsis, burns, acute pancreatitis and RAS Renal: ATN commonest renal cause of ARF Acute GN Thrombotic microangiopathy Post-renal: obstruction to urine flow Stones, tumour, prostatic hypertrophy and retroperitoneal fibrosis

Answer 293

Progressive irreversible loss of renal function characterised by prolonged symptoms and signs of uraemia

Answer 294

Fatigue, itching, anorexia, eventually confusion

Answer 295

DM (20%) GN (15%) HTN and vascular disease (15%) Reflux nephropathy (10%) PKD (10%)

Answer 296

E Coli O157: H1

Answer 297

5 stages based on GFR: Stage 1: kidney damage with normal renal function (proteinuria) \>90 Stage 2: mildly impaired 60-89 Stage 3: moderately impaired 30-59 Stage 4: severely impaired 15-29 Stage 5: renal failure (RRT required) \<15 or if being treated with RRT

Answer 298

Autosomal dominant inheritance, 85% due to mutations in PKD1 remained in PKD2, both encode polycystin Pathological features: large multicystic kidneys with destroyed renal parenchyma, liver cysts (PKD1) and Berry aneurysms. Clinical features: haematuria, flank pain, UTI. Clinical features are often due to cysts cxs e.g. rupture, infection, haemorrhage

Answer 299

Depends on site and intensity of immune complex deposition Presentation may be with Acture renal failure Urinary abnromalities Nephrootic syndrome or progressive CRF

Answer 300

Minimal mesangial lupus nephirits Immune complexes with no structural alteration

Answer 301

Mesangial proliferative LN: immune complexes and mild/moderate increase in mesangial matrix and cellularity

Answer 302

Focal lupus nephritis: active swelling and proliferation in less than half the glomeruli

Answer 303

Diffuse LN involving more than half of the glomeruli

Answer 304

Membarnous LN with subiepithalil complex deposition?

Answer 305

Advanced sclerosing: complete sclerosis of \>90% of the glomeruli

Answer 306

Through inhibition of prostaglandins

Answer 307

Proximal: squamous epithelium Distal: columnar (about 1.5-2cm below the diaphragm) LOS: 2-cm segment proximal to the OGJ OGJ: point where tubular oesophagus joins saccular stomach Squamo-columnar junction/ Z line: irregular serrated margin usually at +/- 40cm from incisors but may lie anywhere within distal 2cm

Answer 308

+/- 40cm from the incisors in distal 2cm of oesoophagus

Answer 309

GORD Reflux of acidic gastric contents into the oesophagus Ulceration, necrotic slough, inflammatory exudates, granulation tissue, fibrosis

Answer 310

Haemorrhage, perforation, stricture, Barret's oesophagus

Answer 311

Los Angeles Classificaiton Grade A: mucosal breaks confined to the mucosal fold, each no longer than 5mm Grade B: at least one mucosal break longer than 5mm coninfed to the mucosal fold but not continuous between two folds Grade C: mucosal breaks that are continuous between the tops of the mucosal folds but not circumferential Grade D: extensive mucosal breaks engaging at least 75% of the oesophageal circumference

Answer 312

Re-epithelialisation by metaplastic olumner epithelium with goblet cells Barret's: columnar lined oesophagus Goblet cell/intestinal metaplasia Precancerous form: metaplastic galndular epithelium-\> dysplasia-\> Adenocarcinoma Surveillance: repeat endoscopy and biopsy to detect early neoplastic changes

Answer 313

30-40% of primary oesophageal carcinoma Associated with Barrett's Other Cas: squamous or combined form

Answer 314

Dysphagia, anorexia, weight loss 90% in the mid and lower oesophagus Invasion into the muscularis propria 30-40% have invasion of the mediastinum, 50% have LN mets Cytology + biopsy Can spread directly, by LNs or liver mets

Answer 315

Extremely dilated submucosal veins in the lower third othe oesophagus. A consequence of portal HTN commonly due to cirrhosis

Answer 316

Made of the cardia, body and antrum Lined by gastric mucosa: columnar epithelium (mucin secreting) and glands, then lamina propria and muscularis mucosa

Answer 317

Acute insult Neutrophil infiltration Caused by chemicals e.g. Aspirin/NSAIDs, ETOH or corrosives or infection (H. Pylori)

Answer 318

Persistent insult H. pylori associated Lymphocytic infiltration May also by neutrophils and MALT induction. Also associated with chemical, autoimmune and others

Answer 319

Reactive or reflux, caused by NSAIDs. Pattern: foveolar hyperplasia (mucus producing cells), smp and sparse chronic inflammation +/- neutrophils

Answer 320

Acute: neutrophil infiltration Chronic: lymphocyte

Answer 321

H. pylori or Heilmanni Chronic gastritis +/- activity, severity varias Outcome variable: Persistence Intestinal metaplasia Dysplasia Ca and lymphoma Eradication willl reduce the risk of Ca but will not eliminate cancer due to [redetermined pathways

Answer 322

Parietal cell Abs (90%) IF Abs (60%) Chronic gastritis and body atrophy Outcome variable: Vit B12, atrophy and Ca

Answer 323

Infection: CMV, Herpes, Strongyloides (immunosuppresion) IBD: Crohn's

Answer 324

Chronic gastritis/ulcer Intestinal metaplasia Dysplasia Cancer

Answer 325

To exclude malignancy Bleeding (anaemia), perforation, peritonitis

Answer 326

IM in gastric mucosa is a response to LT damage e.g. H. Pylori and Bile There is a Ca risk

Answer 327

An abnormal pattern of growth in which some of the histological features of malignancy are present but a non or pre-invasive stage

Answer 328

High incidence in Japan, Chile, Italy, CHina, Portual and Russia M\>F 90% are carcinomas Environmental factors: smoking, diet

Answer 329

Do a gastric Bx to assess H. pylori status, the principal cause is acid in the presence of gastric metaplasia

Answer 330

Villus:crypt 2:1 No increase in lamina propria cellularity No evdience of epithelial damage, no neutrophils Brunner's glands in 1st part

Answer 331

Immunosuppressed CMV: microsporidois, crytposporidiosis in immunosuppression Giarda Lambila Infection Whipples disease: Tropheryma Whippeli

Answer 332

Show scalloping with a smooth shiny mucosa (or normal) Bx: early changes are hard to see on biopsy. There is normal variation in villous height May be crypt hyperplasia or intraepithelial lymphocytes

Answer 333

H. pylori Coeliacs: 10% will get primary lymphoma (less often carcinoma of the gut) if not adeuqately treated.

Answer 334

lifestyle changes (smoking, weight loss), PPI/H2R antag

Answer 335

Seen in 10% of those with symptomatic GORD Upwards migration of the SCJ

Answer 336

Lower 1/3rd of oesophagus due to association with Barrett's

Answer 337

ETOH, smoking Achalasia of cardia Plummer-Vinson Nutritional deficiencies Nitrosamines HPV

Answer 338

Progressive dysphagia, odynopgagia, anorexia, severe weight loss

Answer 339

Emergency endoscopy-\> sclerotherapy/banding

Answer 340

Breach through muscularis mucosa into submucosa Epigastric pain +/- weight loss

Answer 341

Gastric ulcer is WORSE with food Duodenal ulcer is RELIEVED by food

Answer 342

Biopsy for H. pylori status: punched outm lesion with rolled margins

Answer 343

Triple therapy PPI Clarithromycin Amoxicillin or metronidazole

Answer 344

T-cell mediated autoimmune disease: DQ2, DQ8 HLA status Villous atrophy and malabsorption Presents in young children and Irish women (EMQs)

Answer 345

Malabsorption: steatorrhoea, abdo pain, N+V, weight loss, fatigue, IDA, failure to thrive, rash (dermatitis herpetiformis)

Answer 346

Anti-endomysial Ab (best sensitivity and specificity) Anti-TTG Anti-gliadin (poor marker of disease control)

Answer 347

Upper GI endoscopy and duodenal biopsy (villous atrophy, crypt hyperplasia, lymphocyte infiltrate 10% progress to duodenal T-cell lymphoma if not adequately treated

Answer 348

Stomach (body) lined by gastric mucosa columnar epithelium (foveolar, mucin secreting) specialised glands in the lamina propria muscularis mucosa

Answer 349

Stomach (antrum) lined by gastric mucosa columnar epithelium (fovelolar, mucin secreting) Non-specialised glands in the lamina propria (gastric pits) mucularis mucosa

Answer 350

Duodenum Glandular epithelium with goblet cells (intestinal type epithelium) Villous architecture villous:crypt ratio of \>2:1

Answer 351

Metaplastic glandular epithelium (intestinal type) Dysplasia changes showing some of the cytological and histological features of malignancy but no invasion through the basement membrane Adenocarcinoma invasion through the basement membrane

Answer 352

Intestinal: well differentaited Diffuse: poorly differentiated Linitis plastica, includes signet ring cell caricnoma

Answer 353

VIllous atrophy Coeliac disease

Answer 354

Duodenal MALToma

Answer 355

Epidermis: tratum corneum Stratum lucidum Stratum granulosum Stratum spinosum Stratum Basale DEF Dermis: Paipllary Dermis Reticular dermis Subutis

Answer 356

Corneum Lucidum Granulosum Basale DEJ

Answer 357

Horny layer Granular layer Squamous cell layer Basal layer

Answer 358

Basalae: mitosis, cells bound to BM by hemidesomosomes Spinosum (prickle layer): cells linked by desmosomes S granulosum: nuceli disintegrate Corneum: non-nucleated, contains keratin

Answer 359

Increase in S. corneum/ kertain

Answer 360

Nuclei in S corneum

Answer 361

Increases in spinosum

Answer 362

Decreased cohesions between keratinocytes

Answer 363

Intercellular oedema

Answer 364

Linear pattern of melanocyte proliferation within epidermal basa cell layer

Answer 365

All have same histpoapthology Spongiosis of the epidermis, perivascular chronic inflammatory infiltrate in the dermis Dilated dermal capillaries Chronic: acanthosis, crusting/scaling

Answer 366

Atopic (eczma) Contact Seborrhoeic

Answer 367

Infants: face, scalp Older: flexural areas If chronic may lead to lichenification, persists into adulthood in those with FHx of atopy

Answer 368

Type IV hypersensitivity e.g. to Nickel, rubber Erythema, swelling, pruritus Commonly affects ear lobes and neck (from jewellery), wrst (leather watch straps), feet (from shoes)

Answer 369

Inflammatory reaction to a yeast- Malassezia Infants: cradle cap Young adults: mild erythema, fine scaling, mildly pruritic affecting face, eyebrow, eyelid, anterior chest, external ear

Answer 370

Lesions are pruritic, purple, polygonal, papules and plaques Mother of pearl sheen and fine network on surface called Wickam's striae Usually on inner surfaces of wrists, can also affect oral mucous membranes. Aetiology unknown Hyperkeratosis with saw toothing of rete ridges and basal cell degeneration with chornic inflammatory infiltrate

Answer 371

Lichen planus

Answer 372

Lichen planus

Answer 373

2% Commonest form is plaque is chronic plaque psoriasis with salmon pink plaques and a silver scale Rubbing them causing pin-point bleeding Koebner phenomenon: lesions form at sites of trauma Cells have increased proliferation rate Parakeratosis, loss of granular layer, clubbing of rete ridges giving test tubes in a rack appearance. Munro's microabscesses

Answer 374

Plaque psoriasis

Answer 375

Rubbing of psoriatic plaques causing pin-point bleeding

Answer 376

Psoriatic plaques forming at trauma sites

Answer 377

Chronic plaque psoriasis Flexural psoriasis: seen in later lift, usually groin, natal cleft and sub mammary areas Guttate psoriasis: rain drop plaque distribution seen 2 week post Strep-throat Erthrodermic/pustular psoriasis: severe widespread disease often systemic symptoms

Answer 378

Pitting Onycholysis Subungual hyperkeratosis Arthritis

Answer 379

Salmon pink scaly eruption on the trunk extending outwards Herald patch May be assocaited with a virus Pathology: non specific dermatitis

Answer 380

Causes annular target lesions on hands and feet. Pleomorphic lesions that can be a combination of macules, papules, urticarial weals, vesicles, bullae and petechiae Subepidermal bulllae on histology

Answer 381

Infections e.g. HsV, mycoplasma or drug reactions e.g. penicllin, salicylates, anti-malarials

Answer 382

Vesicles \<0,5cm Bullae \>0.5cm Can be sub-epidermal, intra-epidermal and subcorneal

Answer 383

Dermatitis herpetiformis Pemphigoid Pemphigus

Answer 384

Itchy vesicles on extensor surfaces of elbows, buttocks. Associated with coeliac IgA Abs bind to the BM leading to subepidermal bulla Microabscesses which coalesce to form subepidermal bullae. Neutrophil and IgA deposts at the tips of the dermal papillae

Answer 385

Dermatitis herpetiformis

Answer 386

Dermatitis herpetiformis

Answer 387

Large tense bullae on erythematous ase. Often on forearms, groin and axillae, seen in the elderly Bullae do not rupture as early as pemphigus IgG Abs bind to demidesomosomes of BM-\> subepidermal bullae PemphigoiD: Bullae are deep Subepidermal bullae with eosinophils. Linear deposition of IgG along BM

Answer 388

Pemphiogid Deep: subepidermal bulla

Answer 389

Bullae are easily rupturd and found on skin AND mucous membranes IgG Abs bind to desmosomal protines-\> intraepidermal bulla PemphiguS- superficial Intraepidermal bulla. Netlike pattern of IgG deposits Acantholysis

Answer 390

Chronic, discoid LE, buttterfly rash, alopecia

Answer 391

Benign: Seborrhoeic keratosis Premalignant: Actinic Keratosis Keratoachthoma Bowen's Disease Malignant: SCC BSS Melanocytic: Melanocytic naevia Lentigines MM

Answer 392

Rough, waxy plaques, stuck on appear in middle age/elderly

Answer 393

(Solar keratosis) Rough, sandpiper like lesions on sun-exposed areas SPAIN: Solar elastosis Parakeratosis Atypia/dysplasia Inflammation Not full thickness

Answer 394

Actinic keratosis

Answer 395

Rpaidly growing dome shaped nodule which may develop a necrotic crusted centre. Grows over 2-3w and clears spontaenously May be difficult to differentiate from SCC hsitologically

Answer 396

Intra-epidermal SCC in situ Flat, red, scaly patches on sun-exposed areas Full thickness atypia/dysplasia with BM intact

Answer 397

Bowen's disease

Answer 398

When Bowen's has spread to involve dermis. Similar clinical features to Bowen's but may ulcerate Atypia/dysplasia throughout epdiermis, nuclear crowding and spreading through BM into dermis

Answer 399

Aka: rodent ulcer Slowly growing tumour, rarley metastatic but locally destructive Pearly surfact often with telangectasia Mass of basal cells pushing down into dermis. Nuclei align in outermost layer (pallisading)

Answer 400

Proliferation in the basal layer, nesting into dermis

Answer 401

Melanocyte proliferation limited to the epidermis

Answer 402

Atypical melanocytes which intiially grow horizontally in the epidermis (radial growth phase), then grow vertically into dermis (vertical growth phase). Vertical growth produces Buckshot appearance (=pagetoid cells)

Answer 403

Breslow Thickness

Answer 404

Asymmetry Border irregularity Colour Diamete \>6mm

Answer 405

Lentigo MM Superficial spreading MM Nodular MM Acral lentiginous MM

Answer 406

Sun exposed areas of elderly caucasians: flat, flowly growing black lesions. MM can develop in a lentigo MM

Answer 407

Irregular borders with variation in colour. Not associated with any special site

Answer 408

Can occur at all sites and seen in younger age group

Answer 409

Palms, soles and subungual areas.

Answer 410

Erythema multiforme

Answer 411

Dermatological emergency: sheets of skin detachment Nikolsky sign positive: mucosal involvement prominent Commonly caused by drug e.g. sulfonamide antibiotics

Answer 412

\<10% body surface vs \>30%

Answer 413

Hair follicles and seat glands can produce specialised tumours of skin

Answer 414

Capillary haemangioma- strawberry mark Flat haemangioma- port wine stain Kaposi's sarcoma

Answer 415

Most common form of cutaenous T-cell lymphoma generally affects skin. Is a NHL Has a muschroom like appearance

Answer 416

Cluster of inflamed pustules and vesicles

Answer 417

Viral infection

Answer 418

Pemphigoid

Answer 419

Aka Compact bone Facilitaties mane bone functions: suppors, protection and elverage Forms cortex, dense hard bone to which muscles are attached

Answer 420

Also called cancellous bone Higher SA, less dense, softer, weaker and less stiff, High vascular and contains red bone marrow where haematopoesis. Vertebrae Has role in homeostasis of Ca

Answer 421

Trauma Infeciton Degeneration Inflammation Metabolic

Answer 422

Complete or incomplete Closed Comminuted Compound

Answer 423

Organisation of a haematoma at # site (pro-callus) Formation of fibrocartilaginous callus Mineralisation of fiberocartilaginous callus Remodelling of bone along weight bearing lines

Answer 424

Type Presence of infection Pre-existing conditions e.g. neoplasm, metabolic, drugs (steroids), Vit deficiency

Answer 425

Sites: direct spread, secondary to diabetic skin ulcer, dental abscess etc. S. aureus (90%) E coli Kleb Salmonella (associated with SCD) Pseudomonas IVDU

Answer 426

Haematgoenous spread to long bones, usually metaphytic Neonates: Haemophils influenza, Group B strep

Answer 427

Fever, malaise, chills, leucocytosis Local: pain, swelling and tenderness Blood test +ve in 60% XR: mixed picture eventually lytic, 10d post onset Periosteal reaction Mottled rarefaction (increased osseus vascularity and lifting up of the periosteum) Involcrum: after 1w irregular sub-periosteal bone formation Lytic destruction (10-14d) Seqeustra: after 3-6w, some areas of cortex may become detached

Answer 428

Periosteal reaction Involcrum (after 1w: sub-periosteal bonefrmation) Irregular lytic destruction Sequestra: 3-6w, necrotic areas of cortex may become detached.

Answer 429

Cierny-Mader Staging Anatomical Type: 1- medullary, 2- superficial, 3- localised, 4- diffuse Physiologic: Host A-normal, B- local or systemic compromise, C- treatment worse than disease

Answer 430

Biopsy: culture 6w IV antibiotics

Answer 431

Rare cause mainly affecting immunocompromised More destructive and resistant to contral Spinal disease may result in psoas abscess: inguinal lump and severe skeletal deformity: Pott's Systemic amyloidosis may result in protracted cases as an inflammatory response to chornic TB infection Marrow replaced by inflammatory response and a granuloma forms. There are Langerhan's type giant cells which have a peripheral rim of nuceli

Answer 432

TB osteomyelitis

Answer 433

Caused by Traponema pallidum Can be congenital or acquired Congenital: skeletal lesions: osteochondritis, osteoperiostitis, diaphyseal ostomyelitis Acquired: sekeltal lesions are late onset, require lack of Rx: non gummatous periosteitis gummatous inflammation of bone and joints neuropathic joints (tabes dorsalis) neuropathic shaft fractures

Answer 434

Syphillitic OM

Answer 435

Inflammatory arthropathy as part of a complex multisystem illness resulting from tick bite Caused by Borrelia burgdorferi: ixodes dammini (tick spp.) Can be mistaken for OM Associated with erythema chronicam migrans

Answer 436

1: early localisation, selling + rash (90%) usually within 7-10d and in groin, axilla, earlobe or on thigh Stage 2: early dissemination, affects multiple organ systems Stage 3: low grade, late and persistent, dominated by arthritis, mimics RA

Answer 437

Prevention: long trousers, vaccines Antibiotics for proven disease Dx is purely clinical

Answer 438

Subacute OM

Answer 439

Degenerative joint disease: Disease of cartilage, cartialge is lost and osteophytes formed, pain due to friction Eboination of underlying bone Avascular necrosis of the femoral head can occur Cartilage degernation Fissuring Abnormal matrix calcification Osteophytes

Answer 440

Primary is age related Secondary is to a prveiously damaged joint or congenitally abnormal joint

Answer 441

Loss of joint space Osteophytes Subchondral sclerosis Subchondral cysts

Answer 442

Big weight bearing joints, vertebrae and kness PIPJ: Bouchards' DIPJ: Herbeden May also affect MCP Synovial disease: synovium Bx is non-specific with increased vessels and chronic inflammation

Answer 443

Loss of joint space Erosions Soft Bones Soft tissue swelling

Answer 444

Disease of the synovium/severe chronic relapsing synovitis Slow and progressive. F\>M RF +ve in 80%, immunocomplexes are more associated with extra-articular disease

Answer 445

HLA-DR4 and DR1 TNF, STAT

Answer 446

Hand: radial deviation of wrist and ulnar deviation of fingers Swan neck deformity: extension of POPJ and flexion of DIPJ Boutonniere: flexion PIPJ Z shaped thumb Symmetrical pain and swelling DIPJ swelling Small joints of hands and feet, wrists, elbows, ankles and knees Mild anaemia, raised ESR, RF +ve, rheumatoid nodules?

Answer 447

Extension of PIPJ and flexion of DIPJ

Answer 448

Flexion PIPJ, extension of DIPJ

Answer 449

Proliferative synovitis with thickening of the synovial membranes, hyperplasia of surface synoviocytes, intense inflammatory cell infiltrate, fibrin deposition on the joint and encrosis Pannus: exuberant inflamed synovium of the articular surface, hyperplastic synovium. Proliferative synovitis Grimley-Sokoloff cells

Answer 450

Hyperplastic synovial cells

Answer 451

Unknown antigen reaches the synovial membrane T-cell proliferation associated with increased B cells and angiogenesis Chronic inflammation with inflammatory cytokines Pannus Cartilage and bone destruction

Answer 452

RA spares DIPJ

Answer 453

Male, middle aged Increased dietary purin intake, ETOH, diuretics, inherited metabolic abnormalities Great toe: MTP (podagara), lower extremities Urate crystals: needle shaped Negatively birefringent crystals

Answer 454

Hot, swollen, red, painful joint. Tophus is the pathognomic lesion (on pinna and hands)

Answer 455

Urate GOUT

Answer 456

\>50y Idiopathic, hyperPTH, DM, hypothyroid, Wilsons Knee and shoulder Hot, swollen joint with effucion Calcium pyrophosphate crystals, rhomboid shaped Positively birefringent

Answer 457

Pseudogout

Answer 458

Acute attack: colchicine LT: allopurinol Conservative: ETOH and purine intake (sardines/liver) reduction

Answer 459

NSAIDs or intra-articular steroids

Answer 460

Sporadic Metabolic: haemachromatosis, primary HPT, mypo Mg, low PO4 Hydroxylapatite crystal deposition Hereditary: AD, ANKH mutation, transmembrane protein Traumatic: e.g. bad OA of the knee

Answer 461

Calcium pyrophosphate (mainly knees) or calcium hydroxyapatite (knees and shoulders) Crystals survive processing, whereas crystals of gout dissolve in aqueous solution and need to be fixed in alcohol or sent unfixed. Crystals are not needle shaped but found in polyhonal or rhomboid islands

Answer 462

AVN of femoral head with characteristic wedge shaped infarct

Answer 463

Swan neck deformity

Answer 464

Boutonniere

Answer 465

RA vs OA synovium RA= proliferative

Answer 466

Pain, swelling, deformity and fracture

Answer 467

XR: evaluate site, margin and size. Number of lesions and soft tisse extension. Any associated disease or fratcure Biopsy: performed by radiologist with a Jamshidi needle to produce a core lesion. Can be CT or USS duiged. Open Bx for sclerot or inaccessible lesions

Answer 468

Osteosarcoma Chondrosarcoma Eqing's sarcoma Giant cell (borderline malignancy)

Answer 469

Secondary metastatic

Answer 470

Breast Prostate Lung Kidney Thyroid

Answer 471

Neuroblastoma, Wilm's, osteosarcoma, Ewings, rhabdomyosarcoma

Answer 472

Adolescents. M\>F. 75% 10-30y. Site: knee\>tibia\>humerus\>skull\>femur Originates from cells of mesenchymal origin, osteoblastic differentiation differentiation and produce malignant osteoid Commonest primary bone tumour Malignant mesenchymal cells +/- bone and cartilage formation. H&E stain of osteosarcoma will show mixed malignant bone in a backgrown of less cellular chondroid tumour. ALP +VE

Answer 473

Osteosarcoma

Answer 474

osteosarcoma

Answer 475

Osteosarcoma ALP +ve

Answer 476

Usually metaphyseal Lytic Permeative Elevated periosetum (Codman's triangle)

Answer 477

Site within bone: intramedullary, cortical or surface Degree of differentiation: grade Multicentricity: synchronous or metachronous Primary or secondary

Answer 478

CTx and limb salvage surgery

Answer 479

Malignant cartilage producing sarcoma \>40 M\>F Pelvis, axial skeleton, proximul femur, proximal tibia XR: ltyic lesions with fluffy calcification involving axial skelton Malignant chondrocytes +/- chondroid matrix may dedifferentiate to high grade sarcoma Px: 70% 5y but depends on grade and size

Answer 480

Chondrosarcoma

Answer 481

Chondrosarcoma

Answer 482

Site: intramedullary or juxtacortical Histologically: conventional (myxoid or hyaline), clear cell, dedifferentiated or mesenchymal

Answer 483

AKA PNET (primitive peripheral neuroectodermal tumour) Highly malignant small round cell tumour found in bone and soft tissue \<20y/0 Long bones and pelvis: diaphysis or metaphsis XR: Onion skinning of periosteum Sheets of small round cells. CD99+ve, T 11.22 translocation Px: 75% 5y

Answer 484

Ewing's Sarcoma

Answer 485

Ewing's Sarcoma

Answer 486

Ewing's Sarcoma Onion skinning of the periosteum

Answer 487

Ewing Sarcoma

Answer 488

Ewing Small cell osteosarcoma Rhabdomyosarcoma Neuroblastoma Lymphoma SCC

Answer 489

Borderline malignancy 20-40y F\>M Epiphysis with metaphyseal extension Knee epiphysis, either femur or tibia, wrist, pelvis, sarcum XR: lytic lesions right up to articular surface Osteoclast-type multinucleate giant cells on background of spindle/ovoid cells Usually benign with unpredicatble behaviour Locally aggreswsive, may recur and can metastasise

Answer 490

Giant Cell Tumour

Answer 491

Giant cell tumour Lytic lesion right up to articular surface

Answer 492

60 times rarer than lung Ca Primary malignant bone tumours are most cmmon in children and young adults On H&E appear red and destroy local bone

Answer 493

Osteid osteoma Osteoma Enchondroma Osteochondroma Fibrous dysplasia Simple bone cysts Osteoblastoma

Answer 494

Normal bone replaced with fibrous bone tissue causing abnromal sweeling and growth of bone. F\>M, after 3rd decade Any bone: ribs and proximal femur commonest. Monosototic\>polyostotic XR: soap bubble osteolysis. Shepher'd crook deformity Irregular trabeculae of woven bone in bland spindle cell stroma. Bone trabeculae shapes= chinese letters

Answer 495

Fibrous dysplasia

Answer 496

Shepherds Crook Fibrous dysplasia

Answer 497

Chinese letters Fibrous Dysplasia

Answer 498

Polyostotic fibrous dysplasia Cafe au lait spots Precocious puberty (endocrine problems)

Answer 499

Fibrous dysplasia Metaphyseal fibrous cortical defect/non-ossifying fibroma Reparative giant cell granuloma Ossifying fibrome Simple bone cyst

Answer 500

Found in humer or femur Fluid filled and unilocular, lystic well defined margins

Answer 501

Cartilaginous differentiation: Osteochondroma Endochrondroma Chondroblastoma Bone forming tumours: Osteoid osteoma Osteoblastoma Osteoma

Answer 502

Adolescent M\>F Arise from osteoblasts, tend to be \<1.5cm, most common in long bones (tibia and femur) Night pain relieved by aspirin Radiolucent nidus with sclerotic rim Normal bone

Answer 503

Osteoid osteoma

Answer 504

Middle age A new piece of bone usually growing on another piece of bone, tpically inthe head and neck Normal bone Assocaited with Gardner syndrome

Answer 505

GI polyps + multiple osteomas + epidermoid cysts

Answer 506

Similar to osteoid osteomas. Osteoblastoma is a giant osteoid osteoma XR: speckled mineralisation

Answer 507

Osteoblastoma

Answer 508

Osteoid osteoma Radiolucent nidus with sclerotic rim

Answer 509

Osteoblastoma Speckled mineralisation

Answer 510

Benign tumours of cartilage. 55% of pts 10-40y Hand\>humerus\>knee\>foot XR: lytic lesion. Cotton wool calcifcation. Expansile O-ring sign

Answer 511

Enchondroma

Answer 512

50%pts 10-20y. M:F Knee, humerus, tibia and femur. Growth plates of long bones A benign cartilage capped bony outgrowth. XR: well defined bony protruberance from bone Cartilage capped Most common neoplsam of the skeletonl. If there is irritation it can be removed by surgery

Answer 513

Osteochondroma

Answer 514

Rare, originating from chondroblasts, most common epiphyseal tumour in children

Answer 515

Multiple endochondromas

Answer 516

Multiple endochondromas with haemangioma

Answer 517

Multiple exostoses (osteochondromas) + short stature + bone deformity

Answer 518

Mesenchymal proliferation which occur in the extraskeletal non-epithelial tissues of the body excluding the meninges and the lymphoreticular system Anywhere but the majority occur in the large muscles of extremities, chest wall and retroperitoneum. Mostly older. M\>F but varies by histological type

Answer 519

Myxoid liposarcoma Spindle cell sarcoma Pleomorphic tumours Synovial sarcoma: biphasic patter with epithelial and spindle cell areas.

Answer 520

Atresia and stenosis Duplication imperforate anus Hirschprung's

Answer 521

Absence of ganlgion cells in myenteric plexus Distal colon fails to dilate 80% male Constipation, abdominal distension, vomiting and overlow diarrhoea in young babies. Associated with DS Genetics: proto-oncogene Cr10+

Answer 522

Clinical impression, biopsy of affected segment: hypertrophied nerve fibres but no ganglia Mx: resection of affected: constricted segment

Answer 523

Mechanical Inflammatory Ischaemic Malignant

Answer 524

Constipation Diverticular disease Adhesions Herniation External mass Volvulus Intussuception

Answer 525

Complete twisting of bowel loop at mesenteric base around a vascular pedicle. Intestinal obstruction +/- infarciton. Infants: commonly small bowel Elderly: sigmoid\>caecal

Answer 526

High incidence in West due to low fibre diet and high intraluminal pressure Herniation occurs at weak points in the wall of the bowel, 90% in left colon. Seen as outpouchings on endoscopy. Cxs: diverticulitis, gross perforation, fistula to bowel, bladder or vagina, or obstruction

Answer 527

Acute: Infection Toxins (abx) CTx/RTx Chronic: IBD TB

Answer 528

Infection, durgs and toxins, CTx

Answer 529

Secretory diarrhoea (toxin) Exudative diarrhoea (invasion and muscoal damage) Severe damage and perforation Systemic illness

Answer 530

Abx associated colitis. Acute colitis with pseudomembrane formation. Caused by protein exotoxins of C. diff Dx; histology, biopsy, C diff toxin or stool assay Rx: metronidazole or vancomycin

Answer 531

Can be acute or chronic Most common vascular disorder of the GIT Usually occurs in "watershed" zones e.g. splenic flexure (SMA/IMA) and the rectosigmoid (IMA/IIA) Can cause damage in tissue layers leading to perfroation: muscoal, mural, transmural

Answer 532

Arterial: atheroma, thrombosis and embolism Venous: thrombus, hypercoagulable states Small vessel disease: DM, cholesterol emboli, vasculitis Low flow state: CCF, haemorrhage, shock Obstruction: hernia, intussuception, volvuluis and adhesions

Answer 533

Diarrhoea +/- blood Fever Abdo pain Acute abdomen Weight loss Extra-intestinal manifestations

Answer 534

Western populations F\>M White\>non-white Smoking aggravates

Answer 535

More common than Crohn's Whites\>non-whites 20-25y

Answer 536

Genetic predispotiion (familial aggregation, twin studies, HLA0 Infection (MTB, measles) Abnormal host immunoreactivity

Answer 537

Affects whole GIT Most common in terminal ileum and caecam Skip lesions: patchy distribution Cobblestone appearance: areas of health mucose lie above diseased mucosa Apthous ulcer. Rosethorn ulcers can join together to form serpentine granulomas Transmural inflammation Non-caseasting granulomas Fistula/fissure formation more common

Answer 538

First lesion in Crohn's

Answer 539

Extends proximally from rectum Continuous Small bowel not affected unless v. severe colitis casuses backwash ileitis Extensive superficial broad ulcers confined to the mucosa No granulomas/fissures/fistulae/strictures Islands of regenerating muscoa bulge into lumen to cause pseudopolyps

Answer 540

Severe pancolitic UC

Answer 541

Malabsorption and Fe def: stomatitis Eyes: uveitis, conjuncitvitis Skin: erythena nodosum, pyoderma gangrenosum, erythema multiforme, digital clubbing Joints: migratory asymmetrical polyarthropathy of large joints, sacroiliitis, myositis, ankylosing spondylitis Pericholangitis, primary sclerosing cholangitis, steatosis

Answer 542

UC Pancolitis= increased

Answer 543

Uveitis, conjunctivitis

Answer 544

Eythema nodosum Pyoderma gangrenosum Erythema multiforme Clubbing

Answer 545

Migratory asymmetrical polyarthritis Sacroilitis Anky spond Myositis

Answer 546

Pericholangitis PSC Steatosis

Answer 547

Strictures (recurrent) Fistulae Abscess fromation Perforation

Answer 548

Severe haemorrhage Toxic megacolon 30% require colectomy within 3y for uncontrollable symptoms Adenocarcinoma (20-30x risk)

Answer 549

Markers of inflammation: ESR, CRP, Ba contrast, endoscopy

Answer 550

Rectal biopsy Flexible sig/colonoscopy AXR Stool culture

Answer 551

Mild: prednisolone Severe: IV hydrocortisone, metronidazole Additional therapies: Azathioprine, methotrexate, infliximab

Answer 552

Mild: prednisolone and 5 ASA (meslalzine) Moderate: prednisolone, 5ASA and steroid eneme BD Severe: admit, nbm IV fluids and IV hydrocortisone, rectal steroids For remission: 5ASA 1st line, azathioprine 2nd line

Answer 553

Diverse group of tumours of enterochromaffin cell origin Produce 5-HT Commonly found in the bowel but also lung, ovaries, testes, usually slow growing

Answer 554

Bronchoconstriction Flushing Diarrhoea

Answer 555

Life threatening vasodilation, hypotension, tachycardia. Bronchoconstriction Hyperglycaemia

Answer 556

24h urine 5-HIAA- main 5HT metabolite Octreotide: somatostatin analgoue

Answer 557

Hyperplastic Inflammatory: pseudopolyps Hamartomotous (juvenile, Peutz Jeghers)

Answer 558

Benign dysplastic lesions that are the precursor to most adenocarcinomas Mostly asymptomatci so need surveillance if \>3.4cm Classified based on architecture as tubular, tubulovillous or villous adenoma Size is most important risk factor for malignancy, in addition to degree of dysplasia and increased villous component

Answer 559

Hypoproteinaemic hypokalaemia because they leak large amounts of protein and K

Answer 560

Normal colon-\> at risk mutation after first hit mutation in APC At risk\_\> adenoma after second hit to APC Progression to APC follows activation of KRAS, LOF in p53

Answer 561

Found sporadically in some genetically acquired syndromes

Answer 562

Focal malformations of mucosa and lamina propria Generally \<5y/o. Mostly in rectum leading to bleeding Usually solitary but \>100= juvenile polyposis which may require colectomy to stop haemorrhage

Answer 563

Multiple polyps, mucocutaneous hyperpigmentation, freckles around mouth, palms and soles Increased risk of intussucception and malignancy. Require regular surveillance of GIT, pelvis and gonds

Answer 564

Seen at 50-60y and though to be caused by shedding of epithelium-\> cell build up

Answer 565

Stromal lesions Lipoma Sarcoma

Answer 566

Villous adenoma

Answer 567

Hyperplastic polyps

Answer 568

2nd commonest cause of cancer death in UK 60-79y/o \<50y= ?familial syndrome 98% are adenocarcinomas, 45% in rectum

Answer 569

Diet low in fibre high in fat Lack of exercise Obesity Familaly syndromes Chronic IBD NSAID protective (COX2 overexpressed in 90%)

Answer 570

Fe def, anaemia. Weight loss

Answer 571

Change in bowel habit, crampy LLQ pain

Answer 572

Sigmoidoscopy, solonoscopy, proctoscopy Barium enem Bloods: FBC CT/MRI CEA to moniotr disease

Answer 573

Grade: level of differentiation Staging: Duke's

Answer 574

A: confined to the wall of the bowel \>95% B: Through wall of bowel 1: extending into muscularis propria 67% 2: transmural invasion without LN involvement 54% C: LN metastases 1: Extending into muscularis propria with LN involvement 43% 2: Transmural with involved LNs 23% D: Distant mets \<10%

Answer 575

Sx: Rectal cancer/ low sigmoid cancer: \<1-2cm above anal sphincter (lower 1/3rd of rectum): abdomino-perineal resection \>1-2cm above anal sphincter: anterior resection Sigmoid cancer: sigmoid colectomy Descending colon and distal transverse: left hemicolectomy Caecum, ascending colon and proximal transverse: right hemicolectomy Transverse colon: extended right hemicolectomy RTx to reduce local recurrence post-Sx CTx in pallliation: 5 fluorouracil

Answer 576

70% AD mutations in APC gene with 30% AR mutation in MMR genes Presents 10-15y with \>100 adenomatous polyps required for Dx All will progress to adenoarcinoma if left untreated thereefore most have prophylactic colectomy Increased risk of neoplasia elsewheree.g. ampulla of Vater and stomach At birth hypertrophy of retinal pigment epithelium

Answer 577

Same clinical, pathological and aetiological features as FAP Distinctive extra-intestinal manifestations: Multiple osteomas of skull and mandible Epidermoid cysts Desmoid tumours Dental caries Post-surgical mesenteric fibromatoses

Answer 578

Malignant tumours of the CNS associated with FAP

Answer 579

AD mutation in MMR gene 3-5% of colorectal cancer at early age of onset Cacrinomas usually in right colon, few polyps but fast preogression Also assocaited with endometrial, ovarian, small bowel, transitional cell and stomach carcinoma

Answer 580

Familial colon cancer

Answer 581

Associated with other cancers of the GIT or reproductive system

Answer 582

Used to determine testing of individuals at risk of HNPCC Amsterdam Criteria: Three or more family members with a confirmed diagnosis of colorectal cancer, one of whom is a first degree (parent, child, sibling) relative of the other two Two successive affected generations One or more colon cancers diagnosed under age 50 years Familial adenomatous polyposis (FAP) has been excluded Amsterdam Criteria II: Three or more family members with HNPCC-related cancers, one of whom is a first degree relative of the other two Two successive affected generations One or more of the HNPCC-related cancers diagnosed under age 50 years Familial adenomatous polyposis (FAP) has been excluded

Answer 583

Gynae: NHSCSP Non gynae: exfoliative samples

Answer 584

Women invited from 25-65y Samples taken are liquid based cytology Viewed microscopically Also test for HPV and STDs Reduces incidence of invasive squamous cell carcinoma

Answer 585

EGFR, ALK-1

Answer 586

BRAC1/2 CERB-B2

Answer 587

Metabolic disorder characterised by chronic hyperglycaemia due to lack of insulin.

Answer 588

Autoimmune destruction of insulin producing beta cells by CD4 and CD8 T lymphocytes Strongly linked to insulin resistance and obesity

Answer 589

Polyuria (osmotic diuresis) Polydipsia (raised plasma osmolality) Hyperglycaemia predisposes to recurrent UTI and skin infections T1 can present as DKA

Answer 590

Fasting glucose \>7mmol/l Random \>11.1mmol/l

Answer 591

IHD CKD Blindness PVD Foot ulceration

Answer 592

Pancreatic tissue located in the duodenum , stomach or Meckel's 25-50% asymptomatic in adulthood

Answer 593

Failure of fusion of the ventral and dorsal panceatic duct system Higher risk of pancreatitis

Answer 594

Partial or complete obstructionof the duodenum by the pancres. Duodenum surrounded by a ring of pancreatic tissue which is continuous with the head of the pancreas

Answer 595

Produces 2l/d of enzymic HCO3- rich fluid, stimulated by secretin and CCL

Answer 596

Produced by s-cells of the duodenum Controls gastric acid secretion and buffering with HCO3

Answer 597

Responsible for stimulating digestion of fat and protein Made by I-cells in the duodenum Causes release of digestive enzymes

Answer 598

Glucagon-\> increases blood glucose

Answer 599

Somatostatin to regulate alpha and beta cells

Answer 600

Vasoactive peptide stimulating the secretion of H2O into the pancreatic system

Answer 601

Pancreatic polypeptide Self-regulates secretion activities

Answer 602

Cardiac- MI Renal- GN, pyelonephritis Cerebral- CVA

Answer 603

Diabetic retinopathy Claudidcation

Answer 604

Idiopathic Gallstones Ethanol Trauma Steroids Mumps Autoimmune Scorpion Venom Hyperlipidaemia ERCP Drugs e.g. thiazides

Answer 605

Severe epigastric pain radiating to back, relived by sitting forward. Vomiting prominent Hypoetnsive shock in severe cases A signficantly raised plasma amylase in clinical setting is virutally diagnostic Can result in the formation of a pseudocyst, associated with alcoholic pancreatitis

Answer 606

Acinar cells

Answer 607

Amylase is only transiently raised Lipase is more sensitive

Answer 608

Severe cases may lead to shock and multiorgan failure Infection of necrosed pancreas worsens prognosis

Answer 609

Coagulative necrosis

Answer 610

Alcoholism CF Hereditary Pancreatic duct obstruction e.g. stone/tumour Autoimmune IgG4 sclerosing

Answer 611

Autoimmune chronic pancreatitis

Answer 612

Can very closely mimic pancreatic cancer clinically, radiologically and pathologically

Answer 613

Chornic upper abdominal pain Weight loss Steatorrhoea and diarrhoea occur when most of the gland has been destroyed.

Answer 614

Very similar to pancreatic carcinoma: Grossly replaced with fibrous tissue, loss of exocrine tissue, duct dilatation with thick secretions, calcification

Answer 615

Rare (1%) malingnant epithelial neiplasm of the pancreas demonstrating evidence of enzyme production by the neoplastic cells Older adults.

Answer 616

Non specific symptoms: abdominal pain, weight loss, N+D 10% get multifocal fat necrosis and polyarthralgia due to lipase secretion

Answer 617

Neoplastic epithelial cells gowing in sheets Some have abundnant eosinophilic granular cytoplasm Positive immunoreactivity for lipase, trypsin, chymotrypsin.

Answer 618

5YSR= \<10%

Answer 619

Acinar cell carcinoma neoplastic epithelial cells growing in sheets, trabeculae and acini, some have abundant eosinophilic granular cytoplasm, positive immunoreactivity for lipase, trypsin and chymotrypsin.

Answer 620

Ductal cell adenocarcinoma of the pancreas M\>F \>60y/o

Answer 621

Smoking is the main Diet Genetic e.g. FAP, HNPCC

Answer 622

Cachexia and anorexia Upper abdominal and back pain that is persistent and severe Painless jaundice, pruritis, steatorrhorea DM Trousseau's syndrome Ascites Abdominal mass Vrichow's node Corvosier's sign

Answer 623

The Trousseau sign of malignancy or Trousseau's Syndrome is a medical sign involving episodes of vessel inflammation due to blood clot (thrombophlebitis) which are recurrent or appearing in different locations over time (thrombophlebitis migrans or migratory thrombophlebitis). The location of the clot is tender and the clot can be felt as a nodule under the skin.[1] Trousseau's syndrome is a rare variant of venous thromboembolism (VTE) that is characterized by recurrent, migratory thrombosis in superficial veins and in uncommon sites, such as the chest wall and arms. This syndrome is particularly associated with pancreatic and lung cancer. [2] Trousseau's Syndrome can be an early sign of gastric or pancreatic cancer,[3] typically appearing months to years before the tumor would be otherwise detected.[4] Heparin therapy is recommended to prevent future clots.[5] The Trousseau sign of malignancy should not be confused with the Trousseau sign of latent tetany caused by hypocalcemia.

Answer 624

Bloods: decreased Hb, raised bilirbuin, hypercalcaemia CT/MRI/ERCP CA19.9 \>70IU

Answer 625

CTx palliative (5FU) Whipple's procedure Px v poor \<5% 5YSR

Answer 626

Ductal cell carcinoma

Answer 627

Group of epithelial tumours of the pancreas showing endocrine differentiation May be functioning or non-functioning 2% of all pancreatic tumours 30-60y/o Associated with MEN1

Answer 628

Present with symptoms related to hormone excess e.g. Insulinoma: hypoglycaemic attacks Gastrinoma: Zollinger-Ellison syndrome: recurrent ulceration VIPoma: diarrhoea Glucagonoma: necrolytic migrating erythemia

Answer 629

Parathyroid hyperplasia/adenoma Pancreatic endocrine tumour Pituitary adenoma

Answer 630

Parathyroi Thyroid Phaeo

Answer 631

Medullary thyroid Phaeo Neuroma Marfanoid phenotype

Answer 632

Intraductal papillary mucinous neoplasm Mucinous cystic neoplasm Serous cystic neoplasms Solid pseudopapillary neoplasm

Answer 633

Pancreatic neuroendocrine tumour

Answer 634

A stereotypic arterial response to injury caused by an excessive and harmful inflammatory and fibroproliferative response Chronic inflammation in the intima of large arteries

Answer 635

Branch points of arteries, bifurcations and curvatures

Answer 636

Sideways force on the endothelium representing the tendency to be dragged along with blood flow. Turbulence is atherogenic

Answer 637

Damage to smooth endothelium LDL enters hte endothelium, becomes trapped and undergoes oxidative modification Monocyte recruitment via chemokines (MCP-1) Adhere to endothelium via ICAM-1 and VCAM-1 Transendothelial migration Oxidised lipoproteins are taken up by monocytes, recognised by macrophage scavenger receptors and PRRs (can stain for macrophages with CD68) These form foam cells which excrete inflammatory mediators e.g. IL-1 Collagenolysis via MMP occurs Foam cells die by apoptosis releasing cholesterol into a lipid core. Fatty streaks are hte earliest lesions seen in atherosclerosis Platelets stick to the damaged tissue Endothelium proliferates: fibrous cap forms on top of the endothelium and more cholesterol is deposited in the core The plaque enlarges blocking arteries

Answer 638

Fatty streaks

Answer 639

Cholesterol rich, carries cholesterol from liver to tissues Oxidised LDL causes inflammation, endothelial damage and VSMC death

Answer 640

Two phenotypes: contractile and synthetic: Synthesise collagen and strengthen the fibrous cap

Answer 641

Biologically active lining of BVs Portal of LDL emigration Modualted by shear stress Express adhesion molecules

Answer 642

AR Extreme cholesterol levels \>20mmol/L which is resistant to lipid lowering agents Xanthomas in skin and tendons Rapid atherosclerosis

Answer 643

Intermediate lesions: 20y Advanced lesions: 40y Cx \>40y

Answer 644

Stenosis: carotids Rupture: leading to sudden thrombosis and or emoblism Erosion: can lead to thrombosis Intraplaque haemorrhage Aneurysm

Answer 645

Large lipid core, fibrous cap with a prominent inflammatory infiltrate Inflammation has a lytic effect and there are fewer synthetic VSMCs There is a structural breakdown of the superficial cap which tears causing intraplaque haemorrhage and superficial endothelial erosion

Answer 646

Insufficient blood supply leads to cellular dysfunction which is often experienced as pain on exertion: angina pectoris, intermitten claudication Critical iscahemia results in cell death: MI, cerebral infarction, gangrene Cell death occurs, then micrsscopic changes, then gross cahnges

Answer 647

Loss of nuclei, homogenous cytoplasm and necrotic cell death Neutrophil infiltration occurs first

Answer 648

Infiltration of polymorphs Then macrophages- clear debris

Answer 649

Removal of debris

Answer 650

Granulation tissue, new blood vessels, myofibroblasts and collagen synthesis Weeks-months-\> strengthening and decellularising scar

Answer 651

Arrythmias Congestive heart failure Fibrionous pericarditis Cardiac rupture Papillary muscle dysfunction or rupture Aneurysm PE Dressler syndrome Cardiogenic schock M

Answer 652

Rupture of the LV, rupture of the interventricular septum and MR

Answer 653

3 types: Early infarct associated pericarditis Pericardial effusion (+/- tamponade) Dressler's

Answer 654

The damaged myocardium acts as a substrate for re-entrant circuits due to changes in tissue refractoriness: Generalised autonomic dysfunction resulting in enhanced automaticity of the myocardium and conduction system There is an electrolyte imbalance: hypokalaemia and hypomagnesia Transmural infarction can interrupt afferent and efferent limbs of the SNS

Answer 655

Greatest immediately after, declines afterwards

Answer 656

Can cause mitral valve incompetence Usually occurs in the first few days

Answer 657

\>1w due to mural thrombi overlying infarcted tissue

Answer 658

End-stage scars can form large transmural infarcts which result in large bulging aneurysms \>4w

Answer 659

Triad of fever, pleuritic pain and pericardial effusion

Answer 660

IHD Valve disease Myocarditis HTN Cardiomyopathy

Answer 661

Dilated heart Scarring and thinning of the walls Pleural effusion Pulmonary oedema Hepatomegaly Ascites Peripheral oedema

Answer 662

Neurohumoral imbalance, increased cytokine expression, immune and inflammatory changes and altered fibrinolysis. Leads to multiple effects

Answer 663

Diastolic heart failure

Answer 664

Systolic heart failure

Answer 665

nutmeg liver Venous congestion and fatty changes Hepatic cirrhosis with firosis

Answer 666

Intrinsic disease of heart muscle

Answer 667

Progressive loss of myocytes, characterised by a dilated heart, HF, arrythmias and sudden death

Answer 668

Myocytes replaced with fine interstitial fibrosis

Answer 669

Idiopathic Infective Toxic: ETOH, CTx, cobalt Fe Hormonal: hyper, hypothyroid, DM, peri-partum Genetics: haemochromatosis, Fabry's, Mc ARdle's Immunological: myocarditis

Answer 670

LV hypertrophy, characteristic fibre dissaray Familial in 50% (AD with variable penetrance) Thickening of the septum narrows the LV outflow tract

Answer 671

Systolic murmur Arrhythmias Hx of sudden death

Answer 672

Sarcomere mutations (myosin heavy chain)

Answer 673

Walls are rigid, heart is prevented from stretching and filling with blood

Answer 674

Priamry: Loffler's endocarditis Endocardial fibroelastosis Secondary: Infiltrative: amyloidosis, sarcoidosis, haemochromatosis Interstitial: postradiation fibrosis

Answer 675

Loeffler endocarditis is a form of restrictive cardiomyopathy which affects the endocardium and occurs with white blood cell proliferation, specifically of eosinophils.[1] Restrictive cardiomyopathy is defined as a disease of the heart muscle which results in impaired filling of the heart ventricles during diastole.[1][2]

Answer 676

Sequaelae of earlier rheumatic fever, predominantly left sided Mitral\>aortic\>tricuspid\>pulmonic

Answer 677

Leaflets thicken Fusion of the commissures Chordae tendinae also thicken, shorten and fuse

Answer 678

Nodules found in hearts of individuals with rheumatic fever which resulted from inflammation of the heart muscle

Answer 679

Rigid: rheumatic and degenerative Destructive: microbial endocarditis Collapse: Prolapse through a VSD or due to myxomatous degeneration Disease of the arotic valve ring Mecahnical: if the heart dilates the valve may become insufficient to cover the dilated area, can occur in Marfan's dissecting aneurysms, ank spond, syphillitic aortitis and cystic medial degeneration of the aortic media

Answer 680

Middle aged woman with SOC, chest pain, mid systolic click, late systolic murmur

Answer 681

Increased glycosaminoglycans which are prominent on Alvian blue stain

Answer 682

Fibrinous: MI and uraemia Purulent: staphylococcus Granulomatous: TB Haemorrhagic: tumour, TB, uraemia Fibours aka constrictive

Answer 683

Chronic heart failure

Answer 684

Damming of blood within pulmonary circulation\_\> dyspnoea, orthopnoea, PND, wheeze, fatigue Eventually leads to reduced peripheral BP and flow

Answer 685

BNP, CXR, ECG, Echo

Answer 686

Heart: pancraditis Joints: arthritis and synovitis Skin: erythema marginatum, subcut nodules Neurological: Encephalopathy, Sydenham's chorea

Answer 687

Clinical features develop 2-4w post strep throat

Answer 688

Lancefield GAS

Answer 689

Regenerating myocytes seen in acute rheumatic fever

Answer 690

Rheumatic fever

Answer 691

Benpen Erythromycin if penallergic

Answer 692

Rheumatic heart disease Infective endocarditis Non-bacterial thrombotic endocarditis (marantic) Libman Sacks endocarditis

Answer 693

Infective endocarditis

Answer 694

Libman=Sacks Endocarditis

Answer 695

SLE and anti-phsopholipid

Answer 696

Rheumatic heart disease Mitral valve prolapse Calcified valves Bicuspid aortic valve Prosthetic valve Congential defects

Answer 697

Bacteraemia as a consequence of poor dentalo hygiene IVDU Soft tissue infection Dental treatments Cannulae Cardiac Sx/pacemeakers

Answer 698

Staph aureus Strep pyogenes

Answer 699

Larger and more localised vegetations spreading into the aorta

Answer 700

Strep viridans Staph epidermis HACEK Coxiella Mycoplasma Candida

Answer 701

Friable, soft thrombi, small Extending into chordae

Answer 702

Fever, malaise, anaemia, rigors, splenomegaly, new murmur Roth spots Splinter haemorrhages Janeway lesions Osler's nodes Usually mitral/aortic unless IVDU

Answer 703

Duke's criteria

Answer 704

Benpen + gentamicin

Answer 705

Bronchus Dilatation nof the airways and excess mucus production Tobacco smoke, air pollution

Answer 706

Bronchus Airway dilatation and scarring

Answer 707

Bronchus SM cell hyperplasia, excess mucus, inflammation Immunologic

Answer 708

Acinus Airspace enlargement, wall destruction Tobacco smoke, alpha-1 anti-trypsin deficiency

Answer 709

Bronchiole Inflammatory scarring, obliteration Tobacco smoke, air pollutants

Answer 710

Cough and sputum on most days for 3m over 2 years

Answer 711

Chronic bonchitis

Answer 712

Reuccrent infections Chronic hypoxia Pulmonary HTN

Answer 713

Bronchiectasis

Answer 714

Curschmann spirals

Answer 715

Pneumothorax Respiratory failure Pulmonary HTN

Answer 716

Recurrent infections Haemoptysis Pulmonary HTN Amyloidosis

Answer 717

Cough Purulent sputum Fever

Answer 718

Inflammatory: Post-inefctious Abnromal host defence: hypogammaglobinaemia, CTx Ciliary dyskinesia: Kartageners and 2o Obstruction Post-inflammatory (aspiration) Secondary to bronchiolar disease and interstitial fibrosis Systemic disease Asthma Congenital: CF Primary ciliary dyskinesia Hypogammaglobulinaemia

Answer 719

Group of \>200 disease characterised by inflammation and fibrosis of the pulmonary connective tissue, particulary the most peripheral and delivate interstitium of the alveolar wall

Answer 720

Restrictive features on spirometry: Decreased CO diffusion capacity Decreased lung volume Decreased compliance

Answer 721

SOB End-inspiratory crackles Cyanosis, pulmonary HTN and cor pulmonale All have honey-comb lung in end stage

Answer 722

Interstitial lung disease

Answer 723

Fibrosing: Idiopathic pulmoanry fibrosis Pneumoconiosis Crpyotgenic organising pneumonia Associated with CTD Drug-induced Radiation pneumonitis Granulomatous: Sarcoid EAA Associated with vasculitides e.g. Wegener's, Churg-Staruss, microscopic polyangitis Eosinophilic Smoking related

Answer 724

M\>F Unknown causative agents Usual intersitital pneumonia required for Dx (also seen in CTD, asbestosis and EAA)

Answer 725

Usual Interstitial fibrosis

Answer 726

Increasing exertional dyspnoea and non-productive cough 40-70y/o Hypoxaemia-\> cyanosis and pulmonary HTN +/- cor pulmonale and clubbing

Answer 727

Steroids Cyclophosphamide Azathioprine (Limited impact on survival)

Answer 728

RA, systemic sclerosis, SLE

Answer 729

Typically and occupational lung disease: non-neoplastic reaction to inhalation of mineral dusts or inorganic particles Majority affect the upper lobe e.g. coal workers penumoconiosis, silicosis, asbsestosis (tends to affect the lower lobe)

Answer 730

TB, fungal (histoplasma, cryptococcus, coccidioides, aspergillus, pneumoystitsi) parasites

Answer 731

Sarcoid Foreign body Drugs or occupational lung disease

Answer 732

EEA Hypersensitivity pneumonitiis Crypotgenic organisng pneumonia Bronchiolitis obliterans organising pneumonia

Answer 733

Feature of granuloma formation and organisn pneumonia

Answer 734

Inhalation of antigenic dust in sensitised individual leading to systemic symptoms (fever, chills, chest pain, SOB, cough) within hours of exposure Usually settles by following day Progresses to EEA

Answer 735

Farmers lung: moudly hay - Saccharopolyspora rectivirgula Pigeon fanciers lung - Proteins in excreta/feathers Humidifiers lung - heated reservoirs Malt-workers lung: - germinating barley Cheese washers loung: - fungi

Answer 736

Bronchopneumonia: patchy bronchial/peri-bronchial distribution. Low virulence organisms Lobar pneumonis: fibrinosuppurative consolidation Atypical: interstitial pneumonitiis without intra-alveolar inflammation

Answer 737

Consolidation Red hepatisation (neutrophilia) Grey hepatisation (fibrosis) Resolution

Answer 738

PE Pulmonary HTN

Answer 739

Squamous CC Adenocarcinoma Small CC Large CC Mesothelioma

Answer 740

Squamous CC

Answer 741

Squamous cell Small cell

Answer 742

Closely correlated with smoking M\>F Usually proximal bronchi, local spread with late metastasis Less responsive to chemo Variety of subtypyes: papillary, basaloid, Associated with caviation and hypercalcaemia

Answer 743

Epithelium Hyperplasia Squamous metaplasia Angiosquamous dysplasia Carcinoma in situ Invasive carcinoma

Answer 744

Squamous cell lung carcinoma

Answer 745

Most common in womena dn non smokers Malignant epithelial tumour with glandular differntiation or mucin production Occurs peripherally and metastasises early

Answer 746

Adenocarcinoma

Answer 747

Atytpical adenomatous hyperplasia-\> non mucinous BAC-\> mixed pattern adenocarcinoma

Answer 748

Usually occurs centrally in the proximal bronchi Arises from neuroendocrine cellls Associated with ectopic ACTH secretion, Lambert-Eaton, cerebellar degeneration Highly malignant with early metastasis usually by diagnosis Poor prognosis p53 and RB1 mutations common

Answer 749

Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disease — a disease in which the immune system attacks the body's own tissues. The attack occurs at the connection between nerve and muscle (the neuromuscular junction) and interferes with the ability of nerve cells to send signals to muscle cells.

Answer 750

Paraneoplastic syndromes are a group of rare disorders that are triggered by an abnormal immune system response to an underlying (usually undetected) malignant tumor. Patients with paraneoplastic neurological syndrome (PNS) most often present with neurologic symptoms before an underlying tumor is detected. Paraneoplastic neurologic syndromes include many neurologic disorders including paraneoplastic cerebellar degeneration (PCD) caused by an immune-mediated mechanism other than a metastatic complication in patients with an underlying malignancy. Any part of the nervous system can be involved depending on the type of primary malignancy. These syndromes affect 1-3% of all cancer patients.[1] These syndromes are difficult to diagnose and respond poorly to treatment. However, the oncologic outcome of patients with antibody-associated paraneoplastic syndromes does not significantly differ from that of patients who do not have the antibodies or a paraneoplastic syndrome.

Answer 751

Paraneoplastic cerebellar degeneration

Answer 752

Poorly differentiated malignant epithelial tumour: large cells, large nuclei with prominent nuceloli No evidence of glandular or squamous differentiation Associated with paraneoplastic syndromes: ADH, ACTH, PTH, PTHrP, Calcitonin, serotonin, bradykinin

Answer 753

Poorer response to cisplatin

Answer 754

Target for anti-EGFR TKI therapy

Answer 755

Poor prognosis- no response to TKI

Answer 756

Usualy adenocarcinoma= no benefit from TKI

Answer 757

T1-4 based on size, invasion of pleura and pericardium LN metas: N0-2 (N0- no LNs, N1-N2 LNs involved, dependant on extent) M0-1: mets or na

Answer 758

Arises from either parietal or visceral pleura Spreads within pleural space Associated with pleural effusion, chest pain and dyspnoea Latent period of 25-45 following asbestos exposure

Answer 759

Impact in the main pulmonary arteries leading to acute cor pulmonale, cardiogenic shock, and death if \>60% of pulmonary bed occluded

Answer 760

Occluding the pulmonary trunk

Answer 761

May be silent or cause peripheral wedge infarctions Repeated infarctions can lead to pulmonary HTN

Answer 762

BM, amniotic fluid, tumour, air, foreign body

Answer 763

Mean pulmonary arterial pressure \>25mmHg at rest

Answer 764

Can be primary More often secondary due to: Chronic lung disease Heart diseases Recurrent thrombo-emboli Autoimmune disorders

Answer 765

Women aged 20-40

Answer 766

Pre-capillary: Chronic hypoxia/embolus Capillary: Fibrosis Post-capillary: Left heart disease, veno-occlusive disease

Answer 767

Intra-alvolar fluid accumulation leading to poor gas exchange, main aetiology is left heart failure

Answer 768

Heart failure cells Seen in pulmonary oedema due to heart failure

Answer 769

ARDS in adults Neonates: hyaline membrane disease of newborn, insufficient surfactant production

Answer 770

Lung agenesis or hypoplasia Tracheal and bronchial stenosis Congeital cysts

Answer 771

Infection Aspiration Trauma Inhaled irritant gases Shock Blood transfusion DIC Drug overdose Pancreatitis Idiopathic

Answer 772

Mucus plug Overinflated lungs

Answer 773

Paracinar: caused by anti-trypsin deficiency Centrilobular: caused by smoking, loss of the centre of the bronchiole

Answer 774

Lung GIT: meconium ileus and malabsroption Pancreas: pancreatitis and malabsorption Liver: cirrhosis Male reproductive system: infertility

Answer 775

Normal response of lung is to reduce blood flow to hypoxic areas Results in chronic vasoconstriction of the pulmonary arterioles which leads to morphological changes e.g. eccentric intimal fibrosis, thickening of the muscle wall

Answer 776

Fibrotic occlusion of the pulmonary veins can be caused by: Idiopathic Herbal teas and diet pills CTx RTx BM transplant Renal transplant HIV Systemic sclerosis

Answer 777

HRCT +- biopsy

Answer 778

Some patients develop fatal haemorrhages with some durgs as it is an angiogenesis inhibitor

Answer 779

CK5/6, P63, TTF-1

Answer 780

Bronchial obstruction Invasion of local structures Extension through pleura or pericardium Diffuse lymphatic spread within lungs Metabolic spread Paraneoplastic ssyndromes

Answer 781

ADH: SIADH causing hyponatraemia ACTH: Cushings PTHrP: hypercalcaemia Calcitonin; hypocalcaemia Gonadotrophin: gynaecomastia Serotonin: carcinoid syndrome Nonednocrine: haematological/coagulation defects

Answer 782

SVCO Oesophagus: dysphagia Horner's syndrome

Answer 783

Leptomeninges and sometimes dura thickened Arachnoid adhesions may be present Lymphocyte, plasma cell and epitheloid macrophages seen in the infiltrate Caseous necrosis and granulomatous inflammation may be present in TB Adhesions can cause a non-communicating hydrocephalus

Answer 784

Pia and arachnoid mater and the subarachnoid space Inflammation usually due to infection.

Answer 785

Can be acute or chronic 85% caused by gram negative bacilli that are normally resident in GIT e.g. E Coli etc

Answer 786

Cyclophosphamide with haemorrhagic cystitis

Answer 787

Female Calculi Urinary obstruction DM Sexually active Instrumentation (Cyclophosphamide)

Answer 788

Transitional cell Squamous cell Adenocarcinoma

Answer 789

Transitional cell

Answer 790

90% of all bladder tumours Male\>Female 50-80y/o

Answer 791

Cigarette smoking Aromatic amines

Answer 792

Painless haematuria, freuqency, urgency, pyelonephritis or hydronephrosis if ureteral orifices involved

Answer 793

Cystoscopy + biopsy

Answer 794

Schistosomiasis

Answer 795

Rare, arising from extensive intestinal metaplasia or from urachal remnants

Answer 796

The urachus is a remnant of a channel between the bladder and the umbilicus (belly button) where urine initially drains in the fetus during the 1st trimester of pregnancy. The channel of the urachus usually seals off and obliterates around the 12th week of gestation and all that is left is a small fibrous cord between the bladder and umbilicus called the median umbilical ligament.

Answer 797

DHT-mediated hyperplasia of prostatic stromal and epithelial cells resulting in the formation of large nodules

Answer 798

Difficulty urinating Retention Frequency Nocturia Overflow dribbling

Answer 799

Tubulo-interstitial inflammation causing scars, leads to blunting of the renal calcyes and deformation, Xanthogranulomatous PN can result from Gram -ve infections

Answer 800

TURP 5 alpha reductase inhibitors

Answer 801

Adenocarcinoma Commonly seen in older gentlemen

Answer 802

Gleason scoring: Pattern 1- well differentiated tumours, uniform round glands packed into circumscribed nodules Pattern 5: no glandular differentiation Final score= predominant pattern plus worst pattern

Answer 803

Grade 1 \<6 Grade 2 6+7 Grade 3 \>8

Answer 804

Hx Ex PSA (.4ng/ml)

Answer 805

Stage T1/T2: RTx and Sx T3: Extraprostatic spread: external beam radiation

Answer 806

E Coli and other gram -ve rods Fever, chills and dysuria

Answer 807

Manifestation similar to chronic bacterial but without hx of recurrent UTI Culture negative Dysuria or mild suprapubic discomfort

Answer 808

Symptoms of recurrent UTI

Answer 809

Bladder BCG for treatment of carcinoma in situ Non-specific granulomatous prostatitis Symptoms of urinary incontinence and pelvic discomfort

Answer 810

Germ cells

Answer 811

Prostatic intraepithelial neoplasia Common in young men 33% risk of carcinoma Doesn't cause an elevated PSA but examine for carcinoma

Answer 812

AFp beta HCG LDH

Answer 813

Beta HCG (AFP and ALP not raised)

Answer 814

Embryonal carcinoma and yolk sac tumour Aggressive and malignant AFP

Answer 815

Genital wart

Answer 816

Squamous cell carcinoma HPV-related

Answer 817

Costovertebral pain Palpable mass Haematuria Paraneoplastic syndromes: Polycythaemia, hypercalcaemia, HTN, Cushing's, amyloidosis

Answer 818

Cryptorchidism Testicular dysgenesis Klinfelters Testicular feminisation

Answer 819

Clear cell Papillary: commonest in dialysis-associated cystic disease Chromophobe renal carcinoma: pale eosinophilic cells

Answer 820

Chlamydia Gonorrhoea

Answer 821

Lower abdo pain Dyspareunia Vaginal bleeding/discharge Fever Adnexal tenderness Cervical excitation

Answer 822

RUQ from perihepatitis Violin string peri-hepatic adhesions

Answer 823

FHC syndrome Infertility Increased risk of ectopic Intestinal obstruciton-\> bacteraemia Tubo-ovarian abscess Peritonitis Plical fusion

Answer 824

Presence of endometrial glands or stroma outside of uterus 10% of women

Answer 825

Metaplasia of pelvic peritnoum Implanatation of endometrium through retrograde menstruation Induction of metaplasia

Answer 826

Endometriosis

Answer 827

Endometriosis Endometriomas

Answer 828

Ectopic endometrial tissue deep within the myometrium causing dysmenorrhoea May cause menorrhagia and deep dyspareunia Globular uterus

Answer 829

Oestrogen stimulation: Enlarge during pregnancy Regress post-menopausally

Answer 830

Sharp, circumscribed, discrete, round, firm, grey-white tumours of variable size Bundles of smooth muscle cells

Answer 831

Menorrhagia Dysmenorrhoea Pressure effects: urinary frequency, tenesmus Subfertility Red degeneration in pregnancy May rarely transform to leiomyosarcoma

Answer 832

May be due to transformation of leiomyoma More common post-menopausally

Answer 833

FIGO Stage 1: confined to uterus Stage 2: involves cervix Stage 3: spread to adenezae, serosa, positive peritoneal cytlogy, LNs (pelvic or para-aortic) Stage 4: pelvic organs and distal spread

Answer 834

Look similar to normal endometrial glands Associated with oestrogen excess, uusally in peri-menopausal women

Answer 835

E2 excess: OBESITY Anovulatory amenorrhoea (PCOS) Nulliparity Early menarche Late menopause Tamoxifen DM HTN

Answer 836

Non-endometroid

Answer 837

Dysplasia of epithelium associated with HPV I, II and III Progression to invasive disease is lower than for CIN

Answer 838

Adenocarcinoma in situ, rarely associated with invasive adenocarcinoma

Answer 839

Generally squamous cell

Answer 840

Serous epithelial tumour

Answer 841

Mucinous tumour of ovary

Answer 842

Clear cell epithelial tumour of ovary

Answer 843

Dysgerminoma

Answer 844

Dermoid cyst Contains skin, hair, teeth, etc.

Answer 845

Germ cell tumours of the ovary Malignant and usually solid, contain immature, embryonal tissues

Answer 846

Fibroma of ovary

Answer 847

Granulosa-Theca cell tumour

Answer 848

Sertoli-Leydig tumour

Answer 849

Endometroid, serousus, clear cell carcinomas: may be uterine primary Krukenbergy tumours: metastatic mucin producing adenocarcinomas from stomach or breast

Answer 850

Krukenberg tumour

Answer 851

Commonly 16 and 18

Answer 852

Where the squamous cell transforms into columnar epithelium

Answer 853

I: dysplasia confined to lower 1/3 II: lower 2/3 III: full thickness but with bm intact

Answer 854

I: reverts to normal 30% of 3 progresses to cervical cancer over 10 yeras

Answer 855

Less common, treatment requires excision of entire endocervix

Answer 856

Squamous cell although 20% can be adenocarcinomas etc.

Answer 857

Invasion through basement membrane

Answer 858

PCB IMB Post MB Pain

Answer 859

Autoimmune multi-system disorder Type III hypersensitivity reaction Increased in classical complement deficiciencies. Can be drug-induced Higher prevalence in Afrocarribean and women

Answer 860

HLA DR3 (or 2)

Answer 861

ANA (95%) Anti dsDNA Anti-Sm Drug-induced Anti-histone

Answer 862

Serositis Oral ulcers Arthritis Photosensitivity Blood disorders (AIHA, ITP, leucopenia) Renal involvement ANA +ve Immune phenomena (dsDNA, anti-SM, antiphospholipid Ab) Neuro symptoms Malar rash Discoid rash

Answer 863

Limited cutaneous (CREST) Diffuse scleroderma

Answer 864

Autoimmune multi-system disorders SKin fibrosis

Answer 865

HLA DR5 and DRw8

Answer 866

Anti-centromere

Answer 867

CREST (limited cutaenous)

Answer 868

Skin changes on face and distal to elbows and knees Calcinosis Raynauds Esophageal dysmotility Sclerodactyly Telangiectasia Associated with pulmonary HTN

Answer 869

Sclerodactyl CREST

Answer 870

CREST= pulmonary HTN Diffuse= pulmoanry fibrosis

Answer 871

Anti Scl-70 Fibrillarin RNA pol I, II, III PM-SCl

Answer 872

Diffuse scleroderma

Answer 873

Diffuse scleroderma

Answer 874

Skin changes can occur anywhere Widespread organ involvement Associated with pulmonary fibrosis

Answer 875

Autoimmune inflammatory disorder of muscle +/- skin Associated with underlying malignancy

Answer 876

Anti-Jo1 Anti-Mi2

Answer 877

Dermatomyositis

Answer 878

Anti-signal recognition peptide Ab (anti-Jo1/ anti-Mi2 (D\>P))

Answer 879

Dermatomyositis

Answer 880

Dermatomyositis

Answer 881

Proximal limb, anterior neck weakness and oesophageal and respiratoy muscle involvement. Elevated skeletal muscle enzymes Abnormal EMG Positive muscle biopsy Associated with pulmonary fibrosis

Answer 882

Proximal limb, anterior neck weakness and oesophageal and respiratoy muscle involvement. Elevated skeletal muscle enzymes Abnormal EMG Positive muscle biopsy + skin changes: Heliotrope rash Gottron Papules Assoc c. pulmonary fibrosis

Answer 883

Gottron papules Dermatomyositis

Answer 884

Heliotrope rash Dermatomyositis

Answer 885

Defects in the classical pathway

Answer 886

Hydralazine Pocainamide

Answer 887

\>4 of the ACR criteria

Answer 888

CRp ESR Test for ANA Complement

Answer 889

C3 (common): Normal C4 (classical): low i.e. undetectable

Answer 890

C3 (common): Low C4 (classical): low

Answer 891

C3: normal C4: normal

Answer 892

Generally normal

Answer 893

High Can be used for disease monitoring

Answer 894

Sometimes high

Answer 895

Anti-SCl70

Answer 896

Takayasu's Temporal arteritis

Answer 897

Polyarteritis Nodosa Kawasaki's Buerger's disease

Answer 898

Wegener's Churg Strauss Microscopic polyangitis HSP

Answer 899

Pulseless disease Increased in Japanese women Vascular symptoms: absent pulse, bruits, claudication Granulomatous vasculitis with massive intimal fibrosis

Answer 900

Takayasu's

Answer 901

Elderly Scalp tenderness, temporal headache Jaw claudication, blurred vision **ESR** raised Overlap with polymyalgia rheumatica

Answer 902

Temporal arteritis

Answer 903

Temporal arteritis

Answer 904

Renal involvement is main feature Can involve other ograns Spares lungs 30% have underlying Hep B

Answer 905

Heavy smokers, usually men \<35 Inflammation of arteries of extremities: usually tibial and radial Pain, ulceration of toes, feet, fingers Corkscrew appearance on angiogram

Answer 906

Buerger's disease

Answer 907

Upper resp tract: sinusitis, epistaxis, saddle nose Lower resp tract: cavitation, pulmonary haemorrhage Kidneys: cresecenteric glomerulonephritis cANCA +ve

Answer 908

Upper RT Lower RT Kidneys

Answer 909

Asthma, allergic rhinitis, eosinophilia Later systemic involvement pANCA (anti-PR3) +ve

Answer 910

Churg-Strauss

Answer 911

Churg Strauss Microscopic polyarteritis

Answer 912

Churg Strauss

Answer 913

Microscopic polyangitis Wegener's Churg Strauss

Answer 914

Pulmonary renal syndrome: Pulmonary haemorrhage Glomerulonephritis

Answer 915

IgA mediated vasculitis Affects children URTI precedes Palpable purpuric rash, abdo pain, GN, arthritis, Orchitis

Answer 916

Presence of abnormal proteins in the blood which becomes thick or gel-like in cold temperatures Cryoglobulins are Abs Cause a range of Sx from skin rashes to kidney failure

Answer 917

Multisystem disorder caused by abnormal protein folding that are subsequently deposited as amyloid fibrils in tissues disrupting their function

Answer 918

Primary and Secondary

Answer 919

AL Derived from light chains therefore more common in patients with myeloma

Answer 920

AA Derived from serum AA which is an acute phase protein Therefore secondary to chronic infection/inflammation

Answer 921

Most common form Most associated with plasma cell dyscrasias Most have Bence Jones protein in urine and increased plasma cells

Answer 922

Autoimmune disease: RA, Ank Spond, IBD Chronic infections: TB, osteomyelitis, IVDU Non-imune: RCC, HL

Answer 923

Deposition of beta2-microglobulin

Answer 924

Familial Mediterranean Fever

Answer 925

++++IL-1 production leading to attacks of fever and inflammation of serosal surfaces AA amyloid, with predominant renal deposition

Answer 926

Caused by amyloid deposits in various organs: Kidney: nephrotic syndrome Heart: conduction defects, heart failure Liver/spleen: hepatosplenomegaly Tongue: macroglossia Neuropathies: carpal tunnel

Answer 927

Amyloidosis If not polarised light appears pink/red

Answer 928

Multisystemic disease of unknown cause Commonly affects young adults Characterised by non-caseating granulomas in many tissues

Answer 929

Sarcoidosis

Answer 930

Most severe disease in Afro-Carribeans Lungs most commonly involved Often detected at routine CXR Most seek help with pulmonary symptoms

Answer 931

Bilateral hilar lymphadenopathy May also see fine nodular shadowing in mid zones Sarcoidosis

Answer 932

Skin: erythema nodosum, lupus pernio, skin nodules LNs: lymphadenopathy, painless and rubbery Joints: arthritis, bone cysts Eyes: anterior uveitis-\> misting of vision and painful red eye Posterior uveitis-\> progressive visual loss Hepatosplenomegaly Leukopaenia/anaemia Hypercalcaemia/hpercalcuria Myocardial involvement CNS involvement Keratoconjuncitvits sicca and lacrimal gland enlargement

Answer 933

Misting of vision and painful red eye

Answer 934

Progressive visual loss

Answer 935

Dx of exclusion Raised Ca Raised ESR Raised ACE Transcbronchial biopsy

Answer 936

Lupus pernio

Answer 937

ngiotensin converting enzyme (ACE) participates in the renin cascade in response to hypovolemia. Its peptidase action on the decapeptide angiotensinogen I results in the hydrolysis of a terminal histidyl leucine dipeptide and the formation of the octapeptide angiotensin II, a potent vasoconstrictor that increases blood pressure. The primary source of ACE is the endothelium of the lung. ACE activity is increased in sarcoidosis, a systemic granulomatous disease that commonly affects the lungs. In sarcoidosis, ACE is thought to be produced by epithelioid cells and macrophages of the granuloma. Currently, it appears that ACE activity reflects the severity of sarcoidosis: 68% positivity in those with stage I sarcoidosis, 86% in stage II sarcoidosis, and 91% in stage III sarcoidosis. Serum ACE also appears to reflect the activity of the disease; there is a dramatic decrease in enzyme activity in some patients receiving prednisone. Other conditions such as Gaucher disease, leprosy, untreated hyperthyroidism, psoriasis, premature infants with respiratory distress syndrome, adults with amyloidosis, and histoplasmosis have been associated with increased levels of ACE.

Answer 938

Painful red breast, fever Almost always occur during lactation and are due to staphylococcal infection via cracks in the nipples Involved breast tissue is necrotic and infiltratred by neutrophils

Answer 939

Continued expression of milk Abx +/- surgical drainage

Answer 940

Mostly in smokers and is not associated with lactation

Answer 941

Periductal mastitis

Answer 942

Inflammation and dilatation of the large breast ducts Poorly defined palpable periarolar mass with thick white nipple secretions Caused by granulomatous inflammation and dilation of large breast ducts Mimics mammographic appearance of cancer

Answer 943

Inflammatory reaction to damaged adipose tissue Presents as painless breast mass/skin thickening/mammographic lesion Caused by trauma, surgery, radiotherapy

Answer 944

Fibrocystic disease/ fibroadenosis Gynaecomastia

Answer 945

Cystic change: small cysts form by dilation of lobules, contain fluid and often calcified Fibrosis: inflammation and fibrosis secondary to cyst rupture Adenosis: increased number of acini seen per lobule Exagerrated response to hormonal influence

Answer 946

Fibrocystic disease

Answer 947

Unilateral or bilateral enlargement of the male breast Indicative of hyperestrinism: ETOH, age, liver cirrhosis, functioning testicular tumour Epithelial hyperplasia with finger-like projections into ducts

Answer 948

Fibroadenoma Duct papilloma Radial scar (Phyllodes tumour)

Answer 949

Most common benign tumour, from stroma, often multiple and bilateral Occuring at any age within the reporudcitve period Epithelial response to size therefore increase during pregnancy and calcify after menopause

Answer 950

Fibroadenoma

Answer 951

Benign papillary tumour arising within the duct system of the breast Can be peripheral (within small terminal ductules) or central (larger lactiferous ducts) Causes bloody discharge, not seen on mammogram, Ix with galactogram

Answer 952

Excision of the affected duct is curative

Answer 953

Benign sclerosing lesion of the breast Central scarring surrounded by proliferating glandular tissue in stellate pattern Resembles cancer on mammogram

Answer 954

Radiat scar

Answer 955

A diverse group of intraductal proliferative lesions of the breast associated with an increased risk of the development of subsequent invasvie breast carcinoma Usually microscopic asymptomatic lesions Usual epithelail hyperplasia Fat epithelial atypia: low grade precursor to DCIS In situ lobular neoplasia

Answer 956

Susceptiblity genes Hormone exposure Age FHx Race: caucasian\>Afrocarribean\>asian\>hispanic Obesity, tobacco, alcohol

Answer 957

BRCA1/BRCA2 Increased risk of ovarian, prostate and pancreatic malignancy BRCA mutations cause a lifetime risk of breast carcinoma of up to 85%

Answer 958

Early menarche Late menopause Late 1st live birth (pregnancy leads to terminal differentiation of molk-producing luminal cells removing these from the potential cancer pool) OCP/HRT

Answer 959

Hard fixed lump Paget's disease of breast Peau d'orange Nipple retraction

Answer 960

Paget's disease of breast

Answer 961

47-73 y/o women invited every 3 years for mammography

Answer 962

Neoplastic intraductal epithelial proliferation with an inherent but not inevitable risk of progression to breast cancer Limited to ducts/lobules by basement membranes Increased incidence since mammography Appear as areas of microcalficiation

Answer 963

Always incidental finding on biopsy as no microcalcifications or stromal reactions 20-40% bilateral Cells lack adhesion protein

Answer 964

Malignant epithelial tumours which infiltrate hte breast with capacity to metastasise

Answer 965

Arises from low grade DICS or in situ lobular neoplasia and show 16q loss

Answer 966

Ductal Lobular Tubular Mucinous

Answer 967

Carcinoma that cannot be classified into another group, most common

Answer 968

Cells aligned in single file chains/strands

Answer 969

Well-formed tubules with low grade nuclei, rarely palpable as \<1cm

Answer 970

Cells produce abundant quantities of extracellular mucin which dissects into surrounding stroma

Answer 971

Examination Radiological examination (mammography, FNA, USS) FNA and cytology

Answer 972

Nuclear pleomorphism, tubule formation, mitotic activity

Answer 973

Investigated for receptor status: ER/PR: good prognosis- tamoxifen Her-2: bad prognosis

Answer 974

Axillary LN status

Answer 975

Mixed agonist/antagonist of ER

Answer 976

Herceptin Monoclonal Ab vs Her2

Answer 977

Has a direct toxic effect on myocardium Must monitor LVEF

Answer 978

Sheets ot atypical cells with lymphocytic infilrtate Stains positive for CK5/6/14

Answer 979

Arise from interlobular stroma with increased cellularity and mitosies \>50y with a palpable mass Low or high grade lesions. Most benign but can be aggressive Therefore excised with wide margins to limit local recurrence

Answer 980

•Hyperpituitarism –Excess secretion of trophic hormones –Usually due to functional adenoma •Hypopituitarism –Deficiency of trophic hormones •Local mass effects

Answer 981

Prolactinoma

Answer 982

Amenorrhea, galactorrhoea, loss of libido, infertility

Answer 983

Prepubertal children- gigantism Adults- acromegaly DM, muscle weakness, HTN, congesitve cardiac failure

Answer 984

Nonsecretory pituitary adenomas Ischaemic necrosis: Sheehan's DIC, SCA, elevated ICP, Shock Pituitary ablation by sx or irradiation

Answer 985

* 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

Answer 986

ADH Oxytocin

Answer 987

Compression of optic chiasm leading to bitemporal hemianopia Signs and symptoms of raised ICP Obstructive hydrocephalus

Answer 988

Anterior pituitary

Answer 989

Responds to TSH from ant pit Follicular cells convert thyroglobulin into T3 and T3 Effect is to increase BMR Also contain a population of C -cells (parafollicular) that promote Ca absorption by the skeletal system

Answer 990

Enlargement of the thyroid Common if there is impaired synthesis of thyroid hormone, most often due to I deficiency Can be: Seen at puberty in females Due to ingestion of substances that interfere with thyroid hormone synthesis Due to hereditary enzyme defects

Answer 991

* With time simple thyroid enlargement may be transformed to a multinodular pattern * May reach massive size * May lead to mechanical effects including dysphagia and airways obstruction * A hyperfunctioning nodule may develop leading to hyperthyroidism

Answer 992

•Hypermetabolic state caused by elevated circulating levels of free T3 and T4

Answer 993

Primary: Grave's Hyperfunctioning multinodular goitre Hyperfunctioning adenoma Thyroidits Secondary: TSH secreting pituitary adenoma (rare) Not associated with thyroid disease: Struma ovarii (ovarian teratoma with ectopic thyroid) Factitious thyrotoxicosis (exogenous thyroid intake)

Answer 994

Thyrotoxicosis Infiltrative opthalmoapthy with exopthalmos Infiltrative dermopathy (myxoedema) F\>M

Answer 995

Exopthalmos

Answer 996

Pretibial myxoedema

Answer 997

Autoimmune disorder caused by autoantibodies including those vs TSHR and thyroglobulin Abs vs TSHR most important-\> stimulate release of thyorid hormones Associated with other autoimmune diseases (hunt in packs)

Answer 998

Primary: Postablative Autoimmune- Hashimoto's Iodine deficiency Congenital biosynthetic defect Secondary: Pituitary or hypothalamic failure

Answer 999

Hashimotos'

Answer 1000

Hashimoto's thyroiditis

Answer 1001

Number- solitary Consistency- solid Age of patient- younger Sex- Male Iodine uptake- Cold

Answer 1002

* Usually solitary * Well circumscribed lesion that compresses the surrounding parenchyma * Well formed capsule * Small proportion cause thyrotoxicosis * Important to examine the capsule for invasion to exclude follicular carcinoma

Answer 1003

Nonfunctional Painless mass in neck Cervical mets 10y survival up to 90% * May occur at any age * May have papillary architecture * BUT diagnosis is based on nuclear features –Optically clear nuclei –Intranuclear inclusions •May be psammoma bodies

Answer 1004

Papillary carcinoma

Answer 1005

* Peak incidence in middle age * Follicular morphology * May be well demarcated with minimal invasion or clearly infiltrative * Usually metastasise via bloodstream to lungs bone and liver

Answer 1006

* Neuroendocrine neoplasm derived from parafollicular C cells * 80% sporadic - adults 5-6th decade * 20% familial - MEN - younger patients

Answer 1007

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

Answer 1008

* Activity controlled by level of free calcium in blood * Decreased calcium stimulates release of PTH * PTH –Activates osteoclasts –Increases renal tubular reabsorption of calcium –Increases conversion of vitamin D to its active form –Increases urinary phosphate excretion –Increases intestinal calcium absorption

Answer 1009

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

Answer 1010

* Surgical ablation * Congenital absence * Autoimmune

Answer 1011

–Neuromuscular irritability - tingling, muscle spasms, tetany –Cardiac arrhythmias –Fits –Cataracts

Answer 1012

Adrenal gland

Answer 1013

Aldosterone

Answer 1014

Androgens and GCs

Answer 1015

Nadr + Adr

Answer 1016

* Cushing’s syndrome – excess glucocorticoids * Hyperaldosteronism * Virilising syndromes – excess androgens

Answer 1017

* Hypertension and weight gain * Truncal obesity * ‘moon’ facies * ‘buffalo hump’ * Cutaneous striae

Answer 1018

Administration of exogenous GCs Atrophic adrenal glands

Answer 1019

* \>50% due to primary hypothalamic-pituitary disease with increased ACTH – Cushing’s disease * Most associated with ACTH-producing adenoma in the pituitary * Some have hyperplasia of ACTH secreting cells in pituitary * Adrenal glands show nodular cortical hyperplasia * 30% of cases – primary adrenal * Most cases are due to a solitary neoplasm –Adenoma –Carcinoma •Less commonly due to bilateral hyperplasia

Answer 1020

Small Cell Carcinoma Adrenals show bilateral hyperplasia

Answer 1021

•Primary –35 % aldosterone secreting adenoma – Conn’s syndrome –60 % bilateral adrenal hyperplasia –Clinical manifestations are hypertension and hypokalaemia –Accounts for \<1% of causes of hypertension but important to recognise as surgically correctable

Answer 1022

Primary hyperaldosteronism

Answer 1023

More commonly associated with carcinoma than adenoma

Answer 1024

–Group of autosomal recessive disorders –Hereditary defects in enzymes involved in cortisol biosynthesis –Decreased cortisol results in increased ACTH, adrenal stimulation and increased androgen synthesis –May present in childhood or less commonly in adults

Answer 1025

Primary Secondary due to reduced ACTH

Answer 1026

–Sudden withdrawal of corticosteroid therapy –Haemorrhage (neonates) –Sepsis with DIC (Waterhouse-Friderichson syndrome)

Answer 1027

Waterhouse–Friderichsen syndrome (WFS), hemorrhagic adrenalitis or fulminant meningococcemia is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection: Typically the pathogen is the meningococcus Neisseria meningitidis.

Answer 1028

Chronic primary adrenal insufficiency

Answer 1029

–Autoimmune (75-90%) –TB –HIV –Metastatic tumour (lung and breast particularly) –Rarely amyloid, fungal infections, haemochromatosis, sarcoidosis

Answer 1030

•Adenomas –most non-functional –May be associated with Cushing’s syndrome or Conn’s syndrome •Carcinomas –Rare –Usually large –More commonly associated with virilizing syndrome than adenoma

Answer 1031

Phaeo Neuroblastoma

Answer 1032

–10% arise in association with a familial syndrome inc. MEN 2A and 2B, von Hippel-Lindau disease and Sturge-Weber syndrome –10% are bilateral –10% are malignant –In addition 10% of catecholamine-secreting tumours arise outside the adrenal (paragangliomas)

Answer 1033

Myomalacia cordis

Answer 1034

Myxomatous mitral valve

Answer 1035

Myomalacia cordis (weakness of cardiac muscle due to disintegration of dead myocytes): Rupture of weakened cardiac muscle may result in: · Mitral regurgitation: Apical thrill, systolic murmur and pulmonary oedema · Ventricular septal defect: Left sternal edge thrill, murmur, hypotension · External rupture: Cardiac tamponade

Answer 1036

Carcinoma head of pancreas

Answer 1037

Carcinoma of the head causes jaundice, tail does not

Answer 1038

Carcinoma tail of the pancreas

Answer 1039

VIPoma | (Werner Morrison syndrome)

Answer 1040

VIPoma Werner Morrison Syndrome

Answer 1041

A VIPoma (also known as Verner–Morrison syndrome, after the physicians who first described it)[1] is a rare (1 per 10,000,000 per year) endocrine tumor,[2] usually (about 90%) originating from non-β islet cell of the pancreas, that produce vasoactive intestinal peptide (VIP). It may be associated with multiple endocrine neoplasia type 1. The massive amounts of VIP in turn cause profound and chronic watery diarrhea and resultant dehydration, hypokalemia, achlorhydria (hence WDHA-syndrome, or pancreatic cholera syndrome), acidosis, vasodilation (flushing and hypotension), hypercalcemia and hyperglycemia.[3]

Answer 1042

Cystadenoma

Answer 1043

Carcinoma of the Pancreas

Answer 1044

Pancreatic pseudocyst is a fluid filled collection contained within a well-defined capsule of fibrous or granulation tissue or a combination of both. It does not possess an epithelial lining. A cystadenoma has an epithelial wall or capsule that contains a fluid collection.

Answer 1045

Duodenal ulceration

Answer 1046

Cryptosporidios

Answer 1047

Whipple's disease

Answer 1048

Gastroenteritis- staph

Answer 1049

Size: Massive, moderate, mild

Answer 1050

CML, myelofibrosis (UK) Malaria, Kala-Azar (worldwide)

Answer 1051

Portal HTN Lymphoma CLL Thalassaemia Metabolic diseases e.g. Gaucher's

Answer 1052

Infection e.g. EBV, hepatitis, bacterial CTD e.g. RA, PAN, SLE, Felty's Infiltrative disorders: amyloidosis, sarcoidosis

Answer 1053

Secondary to inflammatory conditions e.g. RA, Crohn's etc

Answer 1054

Post surgery

Answer 1055

Intestinal metaplasia describes the transformation of an epithelium to the columnar epithelium found in the intestines. The distinguishing feature of intestinal epithelium is the presence of goblet cells. (NB. the stomach epithelium is also columnar, but it doesn't contain the distinctive goblet cells. It contains foveolar cells which form glands and secrete mucin) Intestinal metaplasia is a response to chronic inflammation. It is commonly associated with chronic gastritis - H. pylori-induced or autoimmune. The presence of intestinal metaplasia is a strong risk-factor for the development of adenocarcinoma.

Answer 1056

Barrett's oesophagus is another example of intestinal metaplasia due to chronic inflammation. This time chronic GORD is the cause. Again, the intestinal metaplasia is strongly associated with the development of adenocarcinoma. GORD and Barrett's is the reason that oesophageal adenocarinoma tends to affect the distal third of the oesophagus while squamus cell oesophageal cancer affects the upper two thirds.

Answer 1057

DQ2 most strongly DQ8

Answer 1058

Chronic gastritis is usually caused by H. pylori which would result in an antral gastritis. This patient had erosions throughout the gastric mucosa. Chronic gastritis would be have lymphocytes and plasma cells on biopsy - this patient had a neutrophilic infiltrate which is typical of an acute gastritis. Focal, acute mucosal defects are a well-known complication of NSAIDs which this patient was probably taking.

Answer 1059

Adenocarcinoma

Answer 1060

Squamous cell carcinoma

Answer 1061

Staphylococcus aureus is a leading cause of gastroenteritis resulting from the consumption of contaminated food. Staphylococcal food poisoning is due to the absorption of staphylococcal enterotoxins preformed in the food. The onset of symptoms is rapid (from 30 min to 8 h) and usually spontaneous remission is observed after 24 h. As the presentation is so acute, the WCC often have not had a chance to increase (lag-time). The symptoms of staphylococcal food poisoning are abdominal cramps, nausea, vomiting, sometimes followed by diarrhea (never diarrhea alone).

Answer 1062

Crohn's disease

Answer 1063

olonic angiodysplasia is a common cause of acute or chronic rectal bleeding and iron deficiency anaemia. Angiodysplasias are tiny - 1-5 mm in diameter - hamartomatous capillary lesions in the colonic wall which produce bleeding out of proportion to their size. They are believed to be acquired, possibly as a result of tension on the veins where they pass through the muscularis. Diagnosis: subtraction mesenteric arteriography may demonstrate bleeding if rapid colonscopy: may visualise lesion Treatment: electrical coagulation via the colonoscope resection of segment of colon if the above is unsuccessful

Answer 1064

Chronic lymphocytic leukaemia

Answer 1065

Alcoholic liver disease

Answer 1066

Hepatocellular carcinoma

Answer 1067

Bladder cancer

Answer 1068

. Renal cell carcinoma

Answer 1069

Endometritis

Answer 1070

Fever RUQ pain Jaundice with rigors etc Bacterial cholangitis

Answer 1071

Primary sclerosing cholangitis is a rare disease of unknown aetiology characterised by chronic inflammation and fibrosis of the bile duct. - usually men - associated with IBD, particularly ulcerative colitis - may be complicated by strictures, cholangitis, cholangiocarcinoma - autoantibodies are less frequent than in autoimmune chronic active hepatitis and primary biliary cirrhosis: in an exam, ANCA positivity might be a clue as P-ANCA positive in more than 50% of cases. Antimitochondrial antibodies almost never occur. - Liver Bx: fibrous obliterating cholangitis loss of interlobular and adjacent septal bile ducts - ERCP shows multiple annular strictures, separated by round or slightly dilated duct segments and the intrahepatic and extrahepatic bile ducts have a beaded appearance

Answer 1072

Primary biliary cirrhosis is a chronic, progressive, cholestatic liver disease which classically affects middle aged women (rare under 30 yrs). 95% of patients have anti-mitochondrial autoantibodies, especially E2 component of the pyruvate dehydrogenase complex. Pruritus and fatigue common, but also remember xanthelasma, hyperlipidaemia, feats of chronic liver disease.

Answer 1073

Although you may classically think of the pain of endometriosis as cyclical - responding to the hormonal influence of menstruation, the history is very variable and may be progressively worse to even continuous pelvic pain. Constant pain is often due to adhesions (which this patient may have now developed). In the history - nulliparity, due to subfertility secondary to the endometriosis itself or abstinence due to deep dyspareunia is common. Rare, but possible...are symptoms such as cyclical haematuria or haemopytsis....(which is important to keep in your differential diagnosis, albeit at the bottom of your list...) or painful, cyclical, expanding masses in a pelvic scar.

Answer 1074

Ewing's sarcoma

Answer 1075

Primary osteosarcoma

Answer 1076

Familial hypocalcuric hypercalcaemia

Answer 1077

Secondary hyperparathyroidism with chronic renal osteodystrophy

Answer 1078

Osteoid osteoma

Answer 1079

Osteochondroma

Answer 1080

Simple bone cyst

Answer 1081

Osteosarcoma

Answer 1082

Ewing's tumour

Answer 1083

Small cell carcinoma

Answer 1084

Subacute thyroiditis

Answer 1085

Papillary carcinoma

Answer 1086

Mucinous ovarian neoplasms account for 10-15% of all ovarian tumours and are rarely malignant (~10-15%). They are rarely bilateral, but can be very large. In contrast, whilst serous tumours are also usually benign (30% malignant) and common - accounting for 30% of all ovarian tumours - they have a strong tendency to be bilateral.

Answer 1087

Mature cystic teratoma

Answer 1088

.Thecoma Feedback: In a fibroma, there are intersecting bundles of spindle cells. Thecomas in addition contain lipid.

Answer 1089

Serous cystadenocarcinoma

Answer 1090

Dysgerminoma

Answer 1091

radioSensitive, present in the 30s ; 15% secrete HCG ; 0% secrete AFP.

Answer 1092

Seminomatous germ cell tumours

Answer 1093

present in the 20s, and may secrete AFP and/or HCG.

Answer 1094

Embryonal carcinoma

Answer 1095

Choriocarcinoma

Answer 1096

Acute lymphoblastic leukaemia/lymphoma (ALL)

Answer 1097

Diffuse large B cell lymphoma (DLBCL)

Answer 1098

Gardnerella vaginalis

Answer 1099

Treponema pallidum (syphilis)

Answer 1100

Candida albicans

Answer 1101

Glandular to squamous epithelium

Answer 1102

Mild dyskaryosis

Answer 1103

Core biopsies give HISTOLOGY Fine needle aspirates give CYTOLOGY - cannot assess basement membrane penetration (ie invasion) FNA

Answer 1104

Familial hypocalciuric hypercalcaemia (FHH) is a dominantly inherited condition caused by a mutation of the calcium sensing receptor. The result is that the kidney reabsorbes calcium very avidly as a primary problem. This results in hypocalciuria and hypercalcaemia. Because the parathyroid gland calcium receptor also does not work, the parathyroids continue to secrete excess PTH despite the high calcium, so it looks just like primary hyperparathyroidism. However removing the parathyroids does not help the calcium, as the primary cause of trouble is in the kidney. In true primary hyperparathyroidism however, the calcium that is filtered hugely exceeds the tubular maximum for calcium, so there is no chance of reabsorption, so the urinary calcium is high, with resulting renal stones (which NEVER happens in FHH). There are many surgeons who have operated wrongly on FHH patients, removing the parathyroid only to find the calcium does not get better. As a consequence, we have written guidelines to surgeons, reminding them of the importance of measuring urine calcium in such a patient before removing the parathyroid gland.

Answer 1105

This guy has Myasthenic Syndrome Eaton-Lambert syndrome which occurs in ~3% of people with small cell l ung cancer. In contrast with myasthenia gravis, the fatigue gets BETTER with repeated use of the muscle.

Answer 1106

Autoimmune tend to be painless

Answer 1107

Treponema pallidum

Answer 1108

Brain stem infarction

Answer 1109

Ductal Carcinoma in situ

Answer 1110

Lobular Carcinoma in situ

Answer 1111

Invasive Carcinoma

Answer 1112

Pagets Disease of the nipple

Answer 1113

Fibroadenoma

Answer 1114

Monckeberg arteriosclerosis

Answer 1115

Sparing of the ocular muscles

Answer 1116

Peptic ulcer disease (I) can either be duodenal or gastric. Ulcers differ from erosions as they extend to the submucosa (sometimes to the muscularis mucosa) and take weeks to heal, whereas erosions breach the mucosa only and take days to heal. The main causes of peptic ulcers are Helicobacter pylori, NSAIDs, Zollinger–Ellison syndrome and smoking. These factors disrupt the balance between protective (mucus layer and bicarbonate secretion) and damaging (acid and enzymes) elements leading to ulceration.

Answer 1117

Haemochromatosis (F) is an autosomal recessive condition that is due to a mutation in the HFE gene. The HFE protein regulates iron absorption that is stored as haemosiderin. Histological features of haemochromatosis include a golden-brown haemosiderin deposition in the parenchyma of many organs. Haemosiderin eventually leads to inflammation and subsequent fibrosis. Histological samples of affected tissue will stain blue with Perl’s Prussian blue. Organs affected include the liver (cirrhosis), pancreas (diabetes), skin (bronzed pigmentation), heart (cardiomyopathy) and gonads (atrophy and impotence).

Answer 1118

Actinic keratosis

Answer 1119

Pityriasis rosea

Answer 1120

Nephritic syndrome

Answer 1121

Cellular casts

Answer 1122

Duct ectasia

Answer 1123

Lobular carcinoma in situ

Answer 1124

Phylloides tumour

Answer 1125

Infiltrating ductal carcinoma

Answer 1126

Giant cell tumour (GCT; I) is a borderline malignant tumour of giant osteoclast cells. The cells are similar to those found in Paget’s disease as they have multiple nuclei (\>20). The osteoclastic cells cause lytic lesions in the epiphyses (especially around the knee) that are visible on X-ray and may give a characteristic ‘soap bubble’ appearance. Histological features include multinucleated giant osteoclasts with surrounding ovoid and spindle cells.

Answer 1127

Giant cell tumour

Answer 1128

Fibrous dysplasia (B) occurs due to the developmental arrest of normal bone structures secondary to an osteoblast maturation defect. The most common sites affected are the proximal femur and ribs. On X-ray, fibrous dysplasia may cause a ground-glass or soap bubble appearance. Histological investigation reveals trabeculae that lack osteoblastic rimming. Two possible syndromes can arise: 1) Mono-ostotic (70 per cent) affecting femurs more than ribs occurring in patients under 30 years of age, and 2) McCune–Albright syndrome (30 per cent) that is poly-ostotic and causes café-au-lait spots and precocious puberty.

Answer 1129

C In the first 6 hours (A) in the evolution of an acute myocardial infarction (MI), the histology is normal. Necrotic cell death takes place between 6 and 24 hours (B). Pathologically, there is contraction band necrosis (dark red/pink wavy lines extending across the myocardial fibres), loss of nuclei in the myocardial cells and the start of a homogeneous appearing cytoplasm. At 1–4 days (C) following an acute MI, the start of an extensive acute inflammatory response takes place with cell infiltration. Debris is left by the necrosis in the previous stage and the cytoplasm is homogeneous so that it is difficult to see the outlines of the myocardial fibres. Infiltration of polymorphs, and later macrophages, takes place. Removal of the debris takes place at about 5–10 days. At approximately 2 weeks (D), the area undergoes repair and a classic young scar with new capillaries is seen with early collagenization. Macrophages and myofibroblasts are present in large numbers. Some granulation tissue and collagen synthesis will continue on to the next stage. In the months (E) following an acute MI, the area starts to strengthen with the formation of a decellularizing scar. This established scar will be seen as a pale white collagenized area within the interstitium between myocardial fibres.

Answer 1130

Cystic medial necrosis is a disorder particularly affecting the aorta, causing focal degeneration of the elastic tissue and muscle fibres in the media, with accumulation of basophilic ground substance. This leads to cyst-like pools between the fibres disrupting the normally parallel arrays. Clinically, aneurysm formation becomes more likely. It is more frequent after 40 years of age and is twice as common in males. There is evidence that links cystic medial necrosis to aortic dissection in patients with a variety of syndromes, the most common of which is Marfan’s syndrome (E). These patients are characteristically tall with long limbs and long thin fingers. All other syndromes are inconsistent with this patient. The most common murmur in Ortner’s syndrome (A) is mitral stenosis, associated with an enlarged left atrium and recurrent laryngeal nerve palsy. Turner’s syndrome (D) is only present in females. Congenital heart defects include: aortic coarctation, aortic stenosis, ventricular septal defect and atrial septal defect, but aortic dissection is uncommon. In Ehlers–Danlos syndrome (B), cystic medial necrosis can cause fragile blood vessels but aortic regurgitation is not commonly present. Down syndrome (C) patients may have congenital atrioventricular septal canal defects. Other causes of cystic medial necrosis in patients without Marfan’s syndrome are advanced age and chronic hypertension.

Answer 1131

Multiple sclerosis (MS) is the leading cause of disability in Western countries among young individuals between 20 and 40 years of age. The pathological hallmark of MS is the presence of demyelinating plaques on MRI scanning of the central nerves (spinal cord and brain). Particular things to look for are myelin loss, destruction of oligodendrocytes, and reactive astrogliosis, often with relative sparing of the axon cylinder until later stages. The number of plaques, which range from a few to several hundreds, is indicative of the severity of the disease. According to the activity, the International Classification of Diseases classifies plaques as follows; Acute plaque (A): Minor changes (e.g. oedema) and often difficult to recognize Early chronic active plaque (B): Oedema and macrophages, indicative of an inflammatory disorder of the central nervous system, with some myelin breakdown. Reactive astrocytosis is present Late chronic active plaque (C): Complete loss of myelin. Some macrophages will contain myelin debris and there will be often very mild perivascular inflammation at this stage with enlarged perivascular spaces Chronic inactive plaque (D): Complete loss of myelin with the absence of macrophages Shadow plaque (E): Nearly complete remyelination as a thin myelin with some scattered macrophages and a mild microglial up-regulation.

Answer 1132

Parkinson's disease

Answer 1133

Huntingtons

Answer 1134

Autoimmune chronic gastritis

Answer 1135

Reactive gastritis

Answer 1136

H pylori associated gastritis

Answer 1137

Menetrier's disease

Answer 1138

18 D Hereditary non-polyposis colorectal cancer (HNPCC) (D) is an uncommon autosomal dominant disease but the cancers are poorly differentiated and highly aggressive, therefore screening for identification of carriers for surveillance is necessary. Familial adenomatous polyposis (FAP) (A) is also a rare autosomal dominant condition that is caused by a mutation in the FAP gene on chromosome 5. It is characterized by the presence of adenomata in the large bowel, which this patient did not have, yet also carries a 90 per cent risk of developing into carcinoma by the age of 45. Gardner’s syndrome (B) is similar clinically, pathologically and aetiologically to FAP and also carries a high carcinoma risk. However, there are very distinct extra-intestinal manifestations of Gardner’s syndrome, including multiple osteomas of the skull and mandible, epidermoid cysts and desmoid tumours. Colorectal carcinoma (C) of the sporadic type is inconsistent with the history and age of the patient, as it is more commonly present in patients over the age of 60. Hamartomatous polyps (E) often present in childhood or adolescence as part of Peutz–Jeghers syndrome and these carry a small risk of carcinomas. Patients also tend to have pigmented lesions around the mouth that are characteristic of this condition.

Answer 1139

C Features of iron deficiency anaemia are hypochromic (pale) and microcytic (small) red blood cells. Poikilocytes are red blood cells that are abnormally shaped. When there are variations in shape and size, it is known as anisopoikilocytosis. Basophilic stippling (aggregation of ribosomal material) is absent in iron deficiency and present in b- thalassaemia trait and lead poisoning. In megaloblastic anaemia, there is impaired DNA synthesis and this can be caused by B12 deficiency, folate deficiency and drugs. Here the features are the characteristic hypersegmented neutrophils and macrocytic red blood cells. In hyposplenism, there is presence of target cells known as codocytes (red blood cells that have a high surface area:volume ratio). Acanthocytes (spiculated blood cells/spur cells) and Howell–Jolly bodies (nuclear remnants visible in red cells) are also present in the hyposplenism picture.

Answer 1140

24 B Fibrous dysplasia (B) is a benign disorder of children and young adults, whereby lesions composed of fibrous and bony tissue develop usually in the ribs, femur, tibia or skull. It mostly presents as bone pain and weakness in female patients under 30 years of age and results from congenital dysplasia of bone, consistent with the patient’s history. There are three forms: the more common monostotic form (lesions localized to only one bone), polyostotic (multiple lesions) and McCune–Albright syndrome, which also has endocrine manifestations. Shepherd’s crook deformity refers to a varus angulation of the proximal femur commonly seen in the femoral involvement of polyostotic fibrous dysplasia. Histologically, it is characterized by loose fibrous tissue with metaplastic immature or woven bone trabeculae arranged in a ‘Chinese letters’ formation. Non-ossifying fibroma (A) is often asymptomatic and merely an incidental finding on X-ray and occurs in an even younger group of patients. Ossifying fibroma (D) is a benign, fibrous tumour with reactive bone formation that shows local aggressive behaviour. Lesions are found in the mandible for adults, and the tibia for children. Giant-cell reparative granuloma (C) is an uncommon benign reactive intraosseous lesion and can occur in the skull, jaw, hand and foot. The histology resembles giant cell tumour of bone (see below). A simple bone cyst (E) is common in the proximal metaphysis of the humerus and asymptomatic unless there has been a fracture.

Answer 1141

25 E Enchondroma (E) is a benign intramedullary cartilage tumour usually found in the central mature hyaline cartilage of the short tubular bones of the hands and feet. It may present at any age (average is 40 years) and is often asymptomatic, but some patients present with pain, fracture or swelling of the affected area. There are lytic lesions on X-rays that usually contain variably calcified chondriod matrix and histopathological findings showing bluish-grey lobules of hyaline cartilege. There may be a thin layer of lamellar bone surrounding the cartilage nodules but no permeation of pre-existing host bone which is a positive sign that the lesion is benign. Each potential enchondroma needs to be evaluated on imaging and histology to distinguish it from low-grade chondrosarcoma. Osteochondroma (A) is the most common skeletal neoplasm, often affecting boys in their teens and often found in the long bones. When the growth plates close in late adolescence, there is usually no further growth of the osteochondroma. There is a low risk of malignancy in osteochondromas associated with syndromes that involve multiple lesions such as Ollier’s disease or Maffucci’s syndrome. Osteoid osteoma (C) is a small, benign lesion surrounded by a zone of reactive bone. It mostly occurs in the long bones, with a very distinct clinical picture in that pain is dull, worse at night and relieved by aspirin. Similar radiology and histology are seen in osteoblastomatomours that are larger than 1 cm diameter and that commonly affect the vertebrae. Pathological fracture is a common presentation of giant cell tumour (D), which is more common in females, is locally aggressive and can metastasize. It does not occur in the immature skeleton. It is more common in the metaphysics of the long bones, especially the distal femur and proximal tibia. There is no calcification on X-ray and, histologically, there would be numerous multinucleated giant cells. The mono-nuclear cell population is the neoplastic cells. Multiple myeloma (B) is highly unlikely as it is a different classification of bone tumours altogether and is considered as a blood cell malignancy rather than a primary bone tumour. It is a malignant tumour of plasma cells that causes widespread osteolytic bone damage. It may be in are bone only when it is known as plasmacytoma rather than myeloma.

Histopathology Flashcards

(1289 cards)