high yield Flashcards

(736 cards)

1
Q

triad of nephrotic syndrome

A

1) proteinuria
2) hypoalbuminaemia
3) peripheral oedema
+/- high cholesterol, thrombosis (loss of antithrombin 3)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

causes of nephrotic syndrome

A

primary
1) minimal change disease
2) membranous glomerular disease
3) focal segmental glomerulosclerosis (FSGS)

secondary
1) diabetes
2) amyloidosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

minimal change disease
-demographic
-histology/microscopy
-treatment

A

-kids
-loss of podocyte foot processes on electron microscopy
-good steroid response

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

membranous glomerular disease
-demographic
-associations
-histology/microscopy
-treatment

A

-adults
-SLE + anti-phospholipase A2 antibodies
-light micrscopy: diffuse GBM thickening
-electron: loss of podocyte foot processes
-immunofluorescence: immune complex deposition of entire GBM
-poor steroid response

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

focal segmental glomerulosclerosis
-epidemiology
-microscopy/histology
-treatment

A

-adults / afro-carribean
-light microscopoy: focal + glomerular scarring
-electron: loss of podocyte foot processes
-poor response to steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

diabetetic nephropathy
-first feature
-histology/microscopy

A

-microalbuminuria
-diffuse glomerular basement thickening
-Kimmelstein Wilson nodules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

amyloidosis
-stain
-causes

A

-congo red stain: apple green birefringence
-AA: chronic inflammation
-AL: multiple myeloma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

nephritic syndrome triad

A

1) haematuria (red cell casts)
2) oedema
3) hypertension

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

causes of nephritic syndrome

A

1) post-streptococcal glomerulonephritis
2) IgA nephropathy
3) Rapidly progressive (crescenteric) glomerulonephritis

4) Alprorts syndrome
5) benign familial haematuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

post-strepotoccal glomerulonephritis
-presentation
-microscopy/histology

A

-1-3 weeks after strep infection
-light microscopy: increases cellularity of glomerulus
-immunofluorescene: granular deposits IgG on GBM
-electron microscopy: subendothelial humps

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

IgA nephropathy (bergers)
-epidemiology
-presentation
-histology
-prognosis

A

-most common
-1-2 days after URTI or sterp - increased IgA
-granular deposition of IgA in mesangium
-1/3 asymptomatic 1/3 CKD 1/3 dialysis/transplant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

rapidly progressive (crescenteric) glomerulonephritis
-epidemiology
-features
-types + histology

A

-most aggressive
-oligouria + renal failure
-Type 1 = goodpastures - anti-GBM antibodies: +pulmonary haemorrhages = linear deposition of IgG
-Type 2: immune complex mediated (SLE, IgA): granular immune deposition
-Type 3: pauci-immune (ANCA associated) - scanty immune depostion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Alporst syndrome
-inheritance
-mutation
-features

A

-x-linked
-type 4 collagen on BM
1) nephritic 2) sensorineural deafness 3) lens disclocation

(kidney, ears, eyes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

benign familial haematuria
-Inheritance
-mutation
-features

A

-autosomal dominant
-type 4 collagen of BM
-rarely nephritis, usually asymptomatic haematuria
-good prognosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

lupus nephritis
-staging
-histology

A

-6 stages
-wire loop capillaries & lumpy bumpy granular immune complex deposition

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Tubular causes of kidney disease

A

1) Acute tubular necrosis (ATN)
2) tubointersitial nephritis
-pyelonephritis
-chronic pyelonephritis + reflux nephropathy
-acute interstitial nephritis
-chronic interstitial nephritits

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

acute tubular necrosis (ATN)
-epidemiology
-cause
-feature
-histology

A

-most common cause of AKI
-drugs or toxins
-muddy brown casts
-necrosis of tubules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

acute interstitial nephritis (AIN)
-cause
-features
-histology

A

-allergic picture: post-drugs (eg. NSAIDS, abx)
-eosinophilia + rash
-inflammatory infiltrate (eosinophils)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

vessel causes of kidney problems

A

TTP
HUS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

causes of AKI

A

1) pre-renal (sepsis, renal artery stensois, CCP, hypovolaemia)
2) renal (ischameia, glomeurlonephritis etc)
3) post-renal (obsturction: stones, tumour, prostate, blocked catheter)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

most common cause of AKI

A

ATN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

common causes of CKD

A

1) diabetes
2) HTN
3) glomerulonephritis
4) chronic pyelonephritis
5) APCKD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

staging of CKD

A

1) >90 (normal)
2) 60-89 (mild)
3) 30-59 (moderate)
4) 15-29 (severe)
5) <15 (renal failure)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

waxy casts in urine

A

CKD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
thickened basement membrane with spike and dome appearance due to electron dense deposits in the subepithelial layer
membranous glomerulonephritis
26
What condition is the presence of fatty casts in urine associated with?
nephrotic syndrome
27
What type of bladder cancer is associated with chronic cystitis?
squamous cell carcinoma
28
What condition is the presence of epithelial casts in urine associated with?
ATN
29
adult polycystic kidney disease -inheritance -mutation -features -what is diagnosis based on
-AD -2 types (most comonly PKD1, other PKD2) - mutation in polycystin -liver cysts + berry anaeurysms (SAH) -depends on how many cysts (in each age group)
30
stroke and TIA definition
focal neurological deficit of presumed vascular origin that lasts >24hrs (stroke) or that resolves within 24hrs (TIA)
31
stroke -types -most important risk factor
-ischaemic (80%) - RF: atheroscleosis -haemorrhagic (20%) - RF: HTN
32
difference between ischaemia & infraction?
ischaemia = lack of oxygen supply to tissue infarction = tissue death due to lack of o2 supply
33
what kind of necrosis happens in the brain (stroke)?
liquefactive necrosis
34
signs of a TBI
straw-coloured fluid (otorrhea, rhinorrhea), battle sign
35
what is difusse axonal injury? what is it the most common cause of?
shear tensile forces tearing axona apart in midline structures eg. corpus callosum, rotral brainstem, septum pellucidum most common cause of traumatic coma
36
what are contusions and their pathophysiology?
-collisions between brain and skull -coup: impact of brain on skull -countercoup: injury to the opposite side of brain
37
subarachnoid haemorrhage -most common cause -location -CT -investigation if CT negative
-ruptured berry aneurysms -bifurcation of internal carotid, posterior communicating arteries -hyperattenuation around circle of willis (star sign) -LP at 12 hours to see xanthrocromia
38
extradural haemorrhage -pathophysiology -CT
-rupture of middle menigneal artery (fracture of pterion) -"lemon" shape
39
subdural haemorrhage -pathophysiology -RF -CT
-rupture of bridging veins -alcoholics, elderly, anti-coagulants -"banana" "crescent shape"
40
5As of neurodegenerative disorders
Amnesia apraxia aphasia agnosia anomia
41
most to least common neurodegenerative conditions
alz > vascular > lewy > FTD
42
alzheimers -key pathology -histopathology -affected areas -staging
-accumulation of B-amyloid plaques around neurons interferes with neurone communication. hyperphosphorylation of tau forms neurofibrillary tangles -amyloid plaques & tau tangles -medial temporal lobes & hippocampus (but global atrophy) -BRAAK staging
43
vascular dementia key words
mini strokes, step-wise deterioration
44
lewy body dementia key words
parkinsons, fluctuating course, visual hallucinations of little people an animals
45
FTD key words + histopath
younger patients, strong FH, personality changes & disinhibition -pick bodies (hyperphosphorylated TAU)
46
parkinsons disease -pathophysiology -histopathology
-depletio of dopamingeric neurons projecting from basal ganglia to substantial nigra (nigrostriatal pathway). alpha synuclein mutation --> lewy bodies that deposit in nigrostriatal pathway -lewy bodies
47
Multi system atrophy - key feature - histopathology
-PD + autonomic dysfunction - alpha synucleinopathy - glial cytoplasmic inclusions
48
Progressive supranuclear palsy (PSP) -key faeture -histopathology
-vertigal gaze dysnfunction -tauopathy
49
corticobasal degeneration (CBD) -key feature -histopathology
-alien limb phenomenon -tauopathy
50
types of herniation?
-subfalcine -transtenorial/uncal -tonsillar (compresses brainstem, risk if high ICP and LP)
51
types of oedema in high ICP?
excess accumulation of fluid in the brain parenchyma 1) Vasogenic – disruption of the blood-brain-barrier permeability 2) Cytotoxic – secondary to cellular injury (e.g. ischaemic or hypoxic)
52
types of hydrocephalus and features?
1) Communicating – reduced absorption of CSF into sinus veins e.g. in meningitis the meninges can become fibrous and this reduces absorption. 2) Non-communicating – obstruction in outflow of CSF e.g. in neonates, the lateral ventricles obstruct the cerebral aqueduct causing build-up of CSF in the lateral ventricles 3)normal pressure hydrocephalus (type of communicating hydrocephalus but normal CSF pressure): *urinary incontinence, cognitive impairment, gait apraxia (‘wet, wacky, wobbly’), Treated with a ventriculoperitoneal shunt (drains excess CSF into abdo)
53
single largest cause of death in under 45s
TBI
54
commonest cause of adult brain tumours?
secondary tumours (metastasis) - most commonly lung. very poor prognosis
55
classification of brain tumours?
1) intra-axial: glial, neurones, neuroendocrine cells (normally malignant) 2) extra-axial: cranium, soft tissue, meninges, nerves (normally benign)
56
supratentorial vs infratentorial tumour features?
1) supratentorial: -Focal neurological deficit -Seizures -Personality changes 2) infratentorial -Ataxia -Long tract signs – spasticity, hyperreflexia -Cranial nerve palsies
57
brain tumour grading?
-Grade 1 – benign -Grade 2 – > 5 years survival -Grade 3 – 1-5 years survival -Grade 4 – < 1 year survival NO staging
58
pilocytic astrocytoma -type -age -mutation -grade -histology
-astrocytoma -kids (0-20) -BRAF -grade 1 -"hairy cells" "rosenthal fibres" "slow mitotic divisions"
59
diffuse gliomas -type -age -mutation -grade -histology
-astrocytoma -20-40 -IDH -grade 2-3 -"low-moderate cellularity" "Low mitotic activity" "No vascular proliferation"
60
glibolastoma multiforme -type -age -mutation -grade -histology
MOST COMMON AGGRESSIVE PRIMARY BRAIN TUMOUR IN ADULTS -astrocytoma -50+ -IDH wildtype -grade 4 - "high cellularit" "high mitotic activity" " vascular proliferation/necrosis)
61
meningioma -type -age -mutation -histology -grading
-meningeal -increases with age -NF2 -psammoma bodies -grading determined by mitotic activity
62
medulloblastoma -type -age
-embryonal -2nd most common brain tumour in children
63
oligodendrocytoma histology?
"fried egg appearance"
64
ependymoma histology?
"ventricular tumour" "hydrocephalus)
65
neutrophils raised in
* acute inflammation * corticosteroid use
66
macrophages, lymhocytes & plasma cells raised in?
chronic inflammation
67
characteristic features of squamous cell carcinoma
* keratin production * intracellular bridges
68
characteristic features of adenocarcinomas
* from glandular epithelium * form glands that secrete mucin
69
stain for melanin/melanoma
fontana
70
stain for amyloid/osis
congo red stain (apple green birifrengence)
71
stains for iron/haemochromatosis
* prussian blue * Perl's stain
72
stain for epithelial cells
cytokeratin
73
stain for lymphoid cells
CD45
74
stains for TB
* ziehl-neelson stain (red against blue background): acid fast bacilli * rhodamine-auramine stain (bright yellow)
75
stain for copper (wilsons disease)
rhodanine
76
stain for pneumocystis jiroveci (PCP)
Gomori's methanamine silver stain (flying saucer shaped cysts)
77
stain for cryptosporidium parvum
modified kimyoun stain
78
stain for cryptococcus neoformans
india ink stain (yeast cells surrounded by HALOs)
79
stain for chalmydia psittaci
giemsa stain
80
stain for mycobacterium leprae (leptosy)
Fite stain
81
pericarditis * most common cause * ECG * features
* viral and idiopathic * diffuse concave ST elevation + PR depression * pleuritic chest pain (recent viral illness)
82
cardiac tamponade * causes * features
* chronic heart failure, inflammatory, infectious, malignant, autoimmune * Becks triad: 1) muffled heart sounds 2) raised JVP 3) hypotension
83
atherosclerosis steps
1. endothelial injury causes LDL to accumulate 2. LDL enters tunica intima and becomes trapped in sub-intimal space 3. LDL converted into modified & oxidised LDL 4. macrophages take this LDL up (via scavenger receptors), becoming foam cells 5. apoptosis of foam cells leads to inflammation & cholesterol core of plaque 6. Increase in adhesion molecules so more inflammatory cell infiltrate 7. vascular smooth muscle cells form fibrous cap (segregates thrombogenic core from lumen)
84
which part of aorta is affected more in atherosclerosis + sequelae?
* abdominal aorta > thoracic aorta * leads to AAA
85
which arteries are most affected by atherosclerosis & why
* origins (ostia) of major branches * turbulent flow: low/oscillartory shear stress = atherogeneic (laminar flow = protective)
86
what is prinzmetal angina
angina due to coronary artery spasm rather than atherosclerosis (rare)
87
myocardial infarction pathogenesis + type of necrosis
* coronary atherosclerosis * plaque rupture * platelet activation * thrombosis & vasospasm * myocardial necrosis seconary to ischaemia * COAGULATIVE NECROSIS
88
histological findings at each stage of MI? (sequence)
* <6 hrs: **normal** * 6-24hrs: loss of nuclei, homogenous cytoplasma, necrotic cell death * 1-4 d: polymorph infiltration --> macropahge infiltration (clears up debris) * 5-10 d: removal of debris * 1-2 wks: granulation tissue, angiogenenesis & myofibroblasts lay down collagen * weeks/months: strenghtening decellularising SCAR TISSUE
89
right heart failure * most common cause * histopathology
* secondary to LVF * nutmeg liver
90
hypertrophic cardiomyopathic myopathy * inheritance * mutated gene * apperance of heart * histology
* autosomal dominant * βMHC (β-myosin) gene or Topronin T (less common) * thick-walled, heavy & hyper-contracting heart * histology: myocyte disarray
91
acute rheumatic fever * cause * valve affected * pathogenesis * histology * treatment
* group A streptococcus (pyogenes) * mitral. less commonly aortic * antigen mimicry: antibodies against streptococcal antigen cross-react with myocardial antigens * beady vegetations (verrucae), aschoff bodies (granulomas) + anitschkov myocytes (regenerating) * benzylpenicillin
92
rheumatic fever * Criteria name * Criteria * Criteria needed for diagnosis
* Jones criteria * Major (CASES) 1) carditis 2) arthritis 3) syndenhams chorea 4) erythema marginatum 5) subcutaneous nodules * Minor 1) fever 2) high inflammatory markers 3) migratory arthralgia 4) PR prolgonation 5) previous RF 6) malaise 7) Tachycardia * group A strep + 2 major * 1 major + 2 minor
93
vegetation apperance * rheumatic heart disease * infective endocarditis * non-bacterial thrombotic endocarditits * libman-Sacks endocarditis
* small warty vegeations on line of closure (verrucae) * large irregular masses extendiing to chordae * small bland vegetations formed of thrombi (DIC/hypercoag) * small warty vegetations (sterile + platelet rich) - assoc with SLE
94
Chronic rheumatic heart disease: most common to least common valve affected
mitral > aortic > tricuspid > pulmonary
95
histological features of chronic bronchitis
* dilataion of airways * goblet cell hyperplasia * hypertrophy of mucuous glands
96
histological features of bronchiectasis
* permanent fibrotic dilatation of bronchi
97
histological features of asthma
* smooth muscle hyperplasia * goblet cell hypertrophy * curschmann spirals (shed epithelium) * Charcot-Leyden crystals (eosinophil infiltration)
98
histological features of emphysema
loss of alveolar parenchyma distal to terminal brochiole
99
Bronchiectasis * cause * pathophysiology * signs
* post-infectious most commonly. others: obstruction, CF, primary ciliary dyskinesia, asthma, abnormal host defect * recurrent infection/inflammation leads to bronchial wall oedema & excess mucus with inflammatory cells --> bronchial wall damage (more prone to infection + inflammation) * Xray: tram-track sign * CT: signet ring sign
100
cystic fibrosis * inheritance * mutation * organism susceptible
* autosomal recessive * CFTR gene (delta F508 deletion) * pseudomonas aureginosa --> bronchiectasis
101
Restricitive lung disease * feature * causes
* fine end-inspiratory crackles * reduced FEV1 and FVR but ratio >70% * fibrosing, granulomatous, eosinophilic, smoking-related
102
idiopathic pulmonary fibrosis * pattern of fibrosis * histological changes * lobe
* usual interstitial pneumonia * honeycomb change (begins at periphy) - usually sub-pleural * lower lobe | may have clubbing
103
pneumoconiosis * cause * lobe affected
* inhaling mineral dusts (coal-miners) * upper lobes
104
asbestosis * sequlae * lobe
* pleural plaques (benign, fibrosis). malignant lesions (adenocarcinoma > mesothelioma) * lower lobe
105
extrinsic allergic alveolitis * cause * histology * niche examples * lobe
* immune-mediated - against organic antigens causing widespread alvoelar inflammation * polypoid plugs of loose connective tissue with granuloma formation * farmers lung, pigeon fanciers, humidifiers lung, malt workers lung, cheese washers lung
106
steps of lobar pneumonia
1) consolidation 2) red hepatisation (neutrophilis) 3) grey hepatisatio (fibrosis) 4) resolution | typically strep.pneumoniae - rust coloured sputum
107
Lung cancers: which are found centrally and which peripherally + association with smoking?
small cell + squamous = central + smoking
108
most to least common types of lung cancer
squamous > adenocarcinoma > small cell > large cell
109
squamous cell carcinoma (lung) * risk factors * mutations * location * histology * paraneoplastic syndrome
* strongest smoking correlation * p53/c-myc mutations * central (proximal bronchi) * keratin + intracellular bridges * hypercalcaemia (PTHrP secretion)
110
lung adenocarcinoma * risk factors * location * histology * mutation
* women, non-smokers * peripheral * gland formation / mucin production * EGFR mutations
111
small cell carcinoma (lung) * risk factors * mutations * location * histology * paraneoplastic featues
* smoking * p53 + RB1 * central * oat cells (small, non-differentiated * SIADH, ectopic ACTH (chsings), Lambert-eaton syndrome | metastasise early - highly malignant
112
large cell carcinoma * features * histology
* large cells + large nuclei + prominent nucloli (no gland/mucin)
113
which lung cancer mutation responds well to tyrosine kinase inhibitor
EGFR (adenocarcinoma)
114
mesothelioma * origin * CT finding
* parietal/visceral pleura * plerual thickening on CT | bronchoenic carcinoma is still the most common lung cancer with asbestos
115
pulmonary hypertension * definition * classes
* mean pulmonary arterial pressure > 25mmHg at rest * classes 1) pulmonary arterial hypertension 2) due to left heart disease 3) due to lung dissease 4) due to clots 5) unclear multifactorial mechanisms
116
pulmonary oedema * main cause * histology (acute + chronic) * CXR finding
* left heart failure * acute: intra-alveolar fluid. chronic: IRON-LADEN macrophages * batwing, kerley B lines, fluid in horizontal fissure
117
ARDS * cause in adults * cause in kids * histology * CXR
* adults: infection, aspiration, trauma, pancreatitis * hylaine membrane disease (nRDS) * explaned, firm, plum-coloured, airless lungs * CXR: white out of all lung fields
118
normal histology of oesophagus
* squamous stratified epithelium * separated from columnar epithelium of stomach via Z line (squamo-columnar junction) | NO GOBLET CELLS
119
most common cause of oesophagitis
reflux oesophagitis = GORD
120
Barrett's oesophagus * pathophysiology * complication * bad prognostic factor
* metaplasia of squamous mucosa to columnar epithelium due to chronic GORD * complication: adenocarcinoma * prescence of goblet cells (intestinal metaplasia)
121
Oesophageal adenocarcinoma * associations * location * epidemiology
* barrett's, smoking, obesity, radiation, M>F, white * distal 1/3 * most common type in UK/US (developed)
122
squamous cell oesophageal carcinoma * RF * epidemiology * location * presentation
* alcohol & smoking, achalasia, nitrosamines, HPV * afro-carribean, men * most common in developing countries * upper 2/3 of oesophagus * progressive dysphagia (solids then fluids)
123
acute periosteal reaction on X-ray indicates?
malignant bone tumour
124
thick endosteal reaction, regular bone formation & intraosseus and regular calficifcation indicates?
benign bone tumour
125
4 malignant bone tumours
* osteosarcoma * chondrosarcoma * ewing's sarcoma * giant cell tumour (borderline malignant)
126
osteosarcoma * epidemiology * common bone * histology * x-ray appearance
* children (very rare) * knee * malignant mesenchymal cells, **ALP positive** * **"Codman's triangle"** (elevated periosteum), **"sunburst appearance"**
127
chondrosarcoma * epidemiology * common bone * histology * X-ray
* >40 yrs * pelvis + axial skeleton * malignant **chondrocytes** * lytic lesion with **fluffy calcification**
128
ewing's sarcoma * epidemiology * common bone * histology * x-ray * mutation
* <20 yrs, highly malignant * pelvis + long bones * **small round blue cells, CD99+** * **onion skinning** * **t (11;22)**
129
giant cell bone tumour * epidemiology * bone * histology * x-ray
* 20-40yrs, **F>M** * knee-epiphysis * **soap bubble appearance**, **giant multi-nucleate osteoclasts** * lytic lucent lesions up to articular surface
130
most malignant bone cancer
ewings sarcoma
131
malignant bone tumour with best prognosis (excluding giant cell)
chondrosarcoma
132
5 benign bone tumours
* osteoid osteoma * osteoma * enchondroma * osteochondroma * fibrous dysplasia
133
osteoid osteoma * epidemiology * bone * special feature * histology * x-ray
* adolescents M>F * tibia, femur * **night pain releived by aspirin** * normal bone, arises from osteoblasts * **"central nidus with sclerotic rim", "Bulls-eye"**
134
osteoma * epidemioplogy * bone * special features * histology
* middle age * **head & neck** * **bony outgrowths** attached to normal bone. part of **gardners syndrome**: GI polyps + multiple osteomas + epidermoid cysts * normal bone
135
enchondroma * epidemiology * bone * feature * histology * x-ray
* middle age * hands * benign cartilage tumours * normal cartilage * **cotton wool calcification, popcorn calcification, O ring sign** | enchondroma = ends = hands
136
osteochondroma * epidemiology * bone * histology * x-ray
* adolescent - **most common benign tumour** * metaphysis of long bones * cartilage capped **"mushroom" bony growth** * well defined bony protuberance from bone
137
fibrous dysplasia * epidemuology * bone * special feature * histology * x-ray
* middle age, F>M * bone replaced by fibrous tissue * **McCune Albright syndrome:** polyostotic dysplasia + cafe au lait spots + precocious puberty * **chinese letters** (misshapen bone trabeculae) * **"soap bubble osteolysis"** **"shepherds crook deformity"**
138
most common benign bone tumour
osteochondroma
139
osteoporosis * disease feature * sx * biochemistry * histology
* **decreased bone mass** * low impact features: NOF, vertebrae, wrists (colles) * normal biochemistry * loss of cancellous bone
140
osteomalacia (adults) * disease feature * sx * x-ray fefature * histology * biochemistry
* **decreased bone mineralisation** * bone pain, tenderness, proximal muscle weakness * **"looser's zones"** (pseudofractures), splaying of metaphysis * excess unmineralised bone (osteoid) * low calcium, low phosphate, high ALP
141
primary hyperparathyrodisim * disease features * sx * x-ray features * histology
* bone changes of osteitis fibrosa cystica * hypercalcaemia features (moans, stones, bones, groans) * **"brown tumours"** (collection of multinucleate giant cells. **"salt & pepper skull"**, **"subperiosteal bone resorption in phalanges"** * osteitis fibrosa cystica (brown tumour)
142
pagets dsiease * disaease features * sx * RF * x-ray features * histology * biochemistry
* mixed lytic & sclerotic lesions: osteolytic --> mixed --> osteosclerotic * bone pain, fractures, **sensori-neural & conductive deafness**, skull changes, heart failure * >50, M, caucasian * **mixed lytic & sclerotic lesions**. "cotton wool", "picture frame" "ivory vertebrae" * **huge osteoclasts** with >100 nuclei, **mosaic pattern** of lamellar bone * isolated high ALP
143
renal osteodystrophy * disease features
* all skeletal changes assoc with CKD: * osteitis fibrosa cystica (2ndary PTH) * osteomalacia * osteosclerosis * osteoporosis * adynamic bone disease | histology, x-ray features depend on subtype
144
gout * features of chronic tophaceous gout
* polyarticular arthritis * tophi deposits in ear lobes, fingers, and elbows * urate kindey stones * tophus is pathognomonic
145
gout * x-ray feature * crystal type
* rat bite erosions * monosodium urate
146
pseudogout aetiology?
* idiopathic * electrolytes: hyperPTH, hypophosphataemia, hypomagnesemia * metabolic: DM, hypothyroid, wilsons, haemochromatosis
147
pseudogout * joint affected in acute * x-ray feature
* knee & shoulder * white lines of chondrocalcinosis
148
osteomyelitis * X-ray changes (eaerly & post-10 days)
* early: subperiosteal new bone formation * 10 days after: lytic bone destruction
149
osteomyelitis organism * adults (+ bone) * children (+ bone) * sickle cell * immunocompromised * congenital
* S.aureus (vertebrae, jaw - dental abscess, toe - diabetic ulcer) * haemophilus influenzae, GBS * salmonella * TB * syphillis
150
examination findings osteoarthritis
* heberdens nodes (DIP) * bouchard nodes (PIP) * squaring at base of thumb | bouch my peen
151
x-ray features osteoarthritis
LOSS * loss of joint space * osteophytes * subchondral sclerosis * subchondral cysts
152
RA - most sensitive/specific ab
anti-CCP
153
joint spared in RA
DIP
154
characteristic deformities in RA
* radial deviation of wrist * ulnar deviation of fingers * swan neck: hyperextension of PIP & felxion of DIP * boutonierre: flexion of FIP, hyperextension of DIP * z-shaped thumb * synovial swelling
155
hyperextension of PIP & flexion of DIP
swan neck deformity (RA) | swans help people
156
hyperextension of DIP & flexion of PIP
boutonniere deformity (RA)
157
histopathology of RA?
* thickening of synovial membrnae * hyperplasia of surface synoviocytes * intense inflammatory infiltrate * fibrin deposition & necrosis
158
where are kidney stones formed
renal collecting ducts
159
most common type of kidney stone
calcium oxalate
160
calcium oxalate stones: cause?
* too much calcium absorption in gut * renal issues: impaired calcium reabsorption from PCT * anti-freeze consumption
161
magnesium ammonium phosphate stones form what?
staghorn calculi (large and painful)
162
cause of magnesium ammonium phosphate stones
urease producing organisms (alkalinise urine) and precipitate magnesium phosphate salts eg. proteus mirabilis
163
uric acid stones - cause
hyperuricaemia (gout / rapid cell turnover eg. TLS)
164
points of impaction of kidney stones
* pelvic-ureteric junction * pelvic brim * vesico-ureteric junction
165
name 3 bening renal tumours
* papillary adenoma * oncocytoma * angiomyolipoma
166
papillary adenoma * feature * histology
* renal epithelium tumour with papillary architecture * bland epithelial cells growing in papillary or tubopapillary pattern - well circumscribed | <15mm
167
oncocytoma * feature * histology
* oncocytic renal epithelial neoplasm * macroscopic: **majogany brown** * microscopic: sheets of oncolytic cells with pink cytoplasm forming **nests of cells**
168
angiomyolipoma * feautre * histology
* **mesenchymal** tumour: composed of fat, blood vessels & muscle * fat spaces, thick blood vessels & spinde cells
169
3 malignant renal tumours
* renal cell carcinoma * nephroblastoma (wilms tumour) * transitional cell carcinoma
170
most common malignant renal tumour
renal cell carcinoma
171
RF for renal cell carcinoma
* smoking, HTN, obsity, long-term dialysis, Von hippel lindau syndrome
172
Presentation + biochemistry of renal cell carcinoma
* High EPO & high Hb * triad of renal mass + painless haematuria + loin pain
173
most common subtype of renal cell carcinoma
clear cell
174
subtypes of renal cell carcinoma
* clear cell * papillary * chromophobe
175
clear cell renal carcinoma * macrosopic * microsopic
* **golden yellow** with haemorrhagic areas * nests of epithelium with **clear cytoplasm**
176
papillary renal carcinoma * macroscopic * microscopic
* **fragile, friable brown tumour** * papillary/tubopapillary growth pattern * >15mm
177
chhromophobe renal carcinoma * macroscopic * microscopic
* **well circumscribed, solid brown tumour** * sheets of large cells with distinct cell borders
178
nephroblastoma * epidemiology * presetation
* children * 2nd most common childhood malignancy * abdominal mass with high BP
179
histology of nephroblastoma
* small round blue cells (undifferentiated) with an epithelial combonent
180
transitional cell renal carcinoma: RF
* smoking / dyes
181
subtypes of renal transitional cell carcinoma
* non-invasive papillary urothelial carcinoma * invasive urothelial carcinoma
182
non-invasive papillary urothelial carcinoma: histology
* frond-like growths projecting from bladder wall * papillart fronds lined by uroepithekial | can be low or high grade (high can progress to invasive)
183
invasive urothelial carcinoma - features
* invasive behaviour * grows as solid masses, fixed to tissue
184
paraneoplastic syndromes of renal tumours
* polycythameia * hypercalcaemia * HTN * cushings * amyloidosis
185
pathophysiology of benign prostatic hyperplasia (BPH)
* dihydrotestosterone - mediated hyperplasia of prostatic stromal & epithelial cells - forming large nodules * nodules compress urethra leading to outflow obstruction
186
sx of BPH
* difficulty urinating, retention, frequency, nocturia, dribbling
187
histology of BPH
* nodule formation, prostatic epithelial ducts + duct spaces
188
management of BPH
* TURP * 5-a reductase inhibitors (finasteride) * alpha blockers (tamsulosin)
189
side effect of alpha blockers (tamsulosin)
hypotension
190
does BPH increase risk of prostate cancer
no
191
most common type of prostate cancer
adenocarcinoma
192
pathophysiology of prostate cancer
* arises from precursor lesion: PIN (prostatic intraepithelial neoplasia) * arises in peripheral zone of gland with firm neoplastic tissue * local spread to bladder, haematogenous spread to bone
193
risk factors for prostate cancer
age, afro-carribean, FH, hormonal factors
194
grading system for prostate cancer
gleason score | based on degree of differentiation & glandular patterns
195
PSA over what is indicative of prostate cancer
>4ng/ml
196
how is gleason score calculated
add the 2 most common grades | result out of 10
197
most common type of testicular tumour
germ cell tumour
198
most common subtpe of germinal tumour (testicular)
* seminoma
199
age group for testicular cancer
20-45
200
risk factors for testicular tumour
* cryptorchidism * testicular dysgenesis * klinefelters * testicular feminisation
201
precursor lesion for testicular cancer
intratubular germ cell neoplasia
202
seminoma (testicular) features
* most common type * peak age 30 * radiosenstivie
203
teratoma (testicular) features + markers
* at any age (infancy onwards) * malignant when in post-pubertal male * chemo-sensitive * markers: AFP, hCG, LDH | (mr gonzalez one)
204
embryonal carcinoma (testicular) feature
resembles embryonic tissue
205
yolk sac tumour (testis) secretes?
AFP
206
feature of testicular cancer
painless lump
207
non-germ cell tumours (testicular) * examples * prognosis
* leydig cell tumour (from stroma) * sertoli cell tumour (from sex cord) * poor prognosis
208
management of testicular cancers
* orchidectomy +/- LN dissection * grade I and above - BEP (bleomycin, eoposide and cisplatin)
209
prognosis of testicular cancers
good, even with mets (90% 5-year survival)
210
Most common bladder tumour
* transitional cell
211
squamous cell carcinoma of bladder: RF
* shistosomiasis * smoking
212
transitional cell carcinoma of bladder: RF
* smoking (most important) * aniline dyes (textile industry) * rubber manufacture * cyclophosphamide
213
adenocarcinoma of bladder : features
* rare * arises from extensive intestinal metaplasia or urachal remnant
214
structure of liver lobule
* hepatic lobule is structural unit: hexagonal * at the centre is hepatic vein (central vein) * corners formed by portal tracts: portal triad - bile ducts, hepatic artery & portal vein
215
components of portal triad + direction
* portal vein (out to in) * hepatic artery (out to in) * bile duct (in to out) | blood flows towards centre, bile flows away
216
How many zones in liver
3
217
Zone 1 is closest to?
portal triad | zone 3 closest to central vein
218
Zone 1 is susceptible to?
* viral hepatitis * recieves more oxygen (periportal hepatocytes)
219
Zone 3 is most susceptible to?
* metabolic toxins & hypoxia (ischaemia) * perivenular hepatocytes * has the most enzymes & is furthest from oxygen supply
220
metabolic roles of liver
* glyclosis * glycogen storage * glucose syntehsis * amino acid syntehsis * fatty acid synthesis * lipoprotein etabolism * drug metabolism
221
protein synthesis of liver?
* all circulating proteins (except gamma globulins) - albumin, fibrinogen, coagulation factors
222
storage role of liver?
* glycgoen * vitamin A, D, B12 (a lot) & a little vit K, folate, iron, copper
223
hormone metabolism role of liver?
* activates vitamin D (25-hydroxylase) * conjugation/excretion of steroid hormones * peptide hormone metabolism (insulin, GH, PTH)
224
role of liver in bile?
bile synthesis (600-1000ml daily)
225
immune function of liver
* phagocytosis by kuppfer cells
226
normal liver structure
* hepatocytes with microvilli * stellate cells in space of disse (space between hepatocytes and sinusoid)
227
what does chronic inflammation do to the liver?
* loss of microvilli * activated stellate cells become myofibroblasts and lay down collagen in space of disse (fibrosis) * myofibroblasts contrast and constrict sinusoits (+ increase vascular resistance) * undamaged hepatocytes regenerate in nodules
228
causes of acute hepatitis
* hepatitis A & E * drugs
229
acute hepatitis histopathology
spotty necrosis
230
chronic hepatitis causes
* viral (hep B & C) * genetic: PBS/PSC, wilsons, haemochromatosis
231
how is chronic hepatitis graded and staged?
* grade: severity of inflammation * state: severity of fibrosis | liver is part of GI (grade = inflammation)
232
histopathology of chronic hepatitis
1. portal infalmmation 2. interface hepatitis (piecemeal necrosis) 3. lobular inflammation 4. bridge formed from portal vein to central vein
233
what is interface hepatitis (piecemeal necrosis)
cannot see border between portal tract & parenchyma
234
what does bridge from portal vein to central vein in chronic hepatitis cause?
* intrahepatic shunting (blood bypasses hepatocytes and reduces liver function) * loss of liver detox (and thus ammonia build up)
235
definition of cirrhosis
* DIFFUSE abnormality in liver architecture (interferes with blood flow & function)
236
histopathology of cirrhosis
* hepatocyte necrosis * fibrosis * **nodules of regenerating hepatocytes** * disturbance of vascular architecture
237
consequences of cirrhosis
* fibrotic bridges form between portal triad & central vein (intrahepatic shunting) * extrahepatic shunting (due to portal hypertension) --> eg. varices (oesophageal, anorectal, caput madusae)
238
features of reduced detoxification due to cirrhosis
* hepatic encephalopathy (build up of ammonia)
239
features of decreased synthesis in cirrhosis
* bleeding * hypogonadism * osteodystrophy * ascites
240
features of portla hypertension
* oesophageal varices * anorectal varices * caput medusae
241
major causes of cirrhosis
1. alcoholic liver disease 2. non-alcoholic fatty liver disease (NAFLD) 3. chronic viral hepatitis (B +/- D, & C) 4. autoimmune hepatitis 5. PBC, PSC 6. genetic: haemachromatosis, wilsons, alpha-1 antitrypsin, galactosaemia, glycogen storage diseases 7. drugs - methotexate
242
top 3 most common causes of cirrhosis
* alcoholic * NAFLD * chronic viral hepatitis
243
how can cirrhosis be classified
* size of regenerating nodules (micronodular or macronodular)
244
micronodular cirrhosis measurement
nodules <3mm
245
micronodular cirrhosis: causes
**alcoholic hepatitis**, biliary tract disease
246
macronodular cirrhosis: causes
* viral hepatitis * wilsons disease * alpha-1 antitrypsin deficiency
247
prognostic score for cirrhosis
modified child's pugh score
248
what does modified childs pugh score take into account
* albumin (lower = worse) * bilirubin (higher = worse * prothrombin time (higher = worse) * ascites * encephalopathy
249
how to calculate childs pugh score
* albumin: 1 (>35), 2 (28-35), 3 (↓28) * bilirubin: 1 (↓34), 2 (34-50), 3 (↑50) * PT: 1 (↓4), 2 (4-6), 3 (↑6) * ascites: 1 (none), 2 (mild), 3 (moderate/severe) * encephalopathy: 1 (none), 2 (mild), 3 (marked) | albumin: 35, 28 bilirubin: 34, 50 PT: 4, 6
250
interpretation of modified childs pugh score?
* <7: A (45% 5-year survival) * 7-9: B (20% 5-year survival) * 10+: C (<20% 5 year survival)
251
hepatic steatosis (fatty liver) * macroscopic * microscopic
* large, pale, yellow, greasy liver * accumulation of fat droplets in hepatocytes (statetosis), fibrosis in late stage
252
is fatty liver reversible
fully reversible if alcohol avoided
253
alcoholic hepatits * macroscopic * microscopic
* large, fibrotic liver * **hepatocyte ballooning**, **mallory denk bodies** (clumped cytoskeleton) | can be seen acutely after night of heavy drinking
254
alcoholic cirrhosis * macroscopic * microscopic
* yellow, tan, fatty, big. transforms into shrunken, non-fatty and brown * **micronodular cirrhosis**
255
NAFLD looks histologically similar to?
alcoholic hepatitis
256
most common cause of chronic liver disease in the west
NAFLD | obesity, hyperlipidaemia, metabolic syndrome, DM
257
NAFLD includes?
* **simple steatosis**: fatty infiltration, relatively benign * **non-alcoholic steatohepatitis (NASH)**: statosis + hepatosis (can progress to cirhhosis)
258
autoimmune hepatitis * demographic * HLA association
* women - young & post-menopausal * HLA-DR3
259
autoimmune hepatitis * histology
plasma cells
260
types of autoimmune hepatitis
* Type 1: ANA, **anti-smooth muscle antibody**, anti-actin, anti-soluble liver antigen * Type 2: **anti-LMK (anti-liver-kidney-microsomal)**
261
treatment of autoimmune heptaitis
* steroids (good response) * until transplant | but disease returns in up to 40%
262
primary biliary cholangitis (PBC) * pathophysiology * epidemiology
* autoimmune destruction of small/medium sized **INTRAHEPATIC bile ducts** causing cholestasis and **slow** development of cirrhosis * females: 10:1 - 40-50 years
263
primary billiary cholangitis * biochemistry * antibodies
* high ALP, high cholesterol, high IgM, high bilirubin (late) * **anti-mitochondrial antibodies**
264
primary biliary cholangitis * US scan
no bile duct dilation
265
histology of PBC
* bile duct loss with granulomas
266
presentation of PBC
* pruritus, fatigue, abdominal discomfort
267
treatment of PBC
ursodeoxycholic acid
268
primary sclerosing cholangitis (PSC) * pathophysiology * epideiomogy
* inflammation &** fibrosis of EXTRAHEPATIC & INTRAHEPATIC bile ducts **leading to **stricutre formation and dilatation** of preserved segments * M>F
269
PSC * association * biochemistry * antibody
* IBD (mostly **UC**) * increased ALP, several auto-Ig mainly **p-ANCA**
270
PSC * US scan * ERCP * histology
* US: bile duct **dilatation ** * ERCP: strictures look like **necklace beads** * histology: **onion skinning fibrosis**
271
risk of PSC
cholangiocarcinoma
272
haemochromatosis inheritance
autosomal recessive
273
haemochromatosis gene
* mutated **HFE gene** (6p21.3) * causes increased iron gut absorption (deposits in tissues/organs)
274
histology of haemochromatosis + stain
iron deposits in liver - prussian blue
275
sx of haemochromatosis
* skin bronzing * diabetes * hepatomegaly (micronodular cirrhosis)
276
biochemistry of haemochromatosis
* high ferritin and iron * low TIBC * transferrin saturation >45%
277
treatment of haemochromatosis
* venesection * desferrioxamine | RF for HCC
278
wilsons disease inheritance
autosomal recessive
279
wilsons disease gene
* ATP7B (chr13) * endoes copper transporting ATPase - decreased biliary copper excretion & deposits in liver, CNS & iris
280
histology of wilsons disease
* stains with rhodanine * mallory bodies & fibrosis
281
sx of wilsons disease
* liver disease * neuro disease ( psychosis, dementia, parkinsonism (basal ganglia) * kayser fleischer rings (copper deposit in descemet's membrane in cornea)
282
managment of wilsons disease
lifelong penicillamine (copper chelator) | neuro damage is permanent, may need liver transplant
283
alpha-1 antitrypsin deficiency inheritance
autosomal dominant | all the others are recessive !!
284
pathophysiology of alpha-1 antitrypsin deficiency
* failure to secrete A1AT in blood - accumulates in hepatocytes causing hepatitis * lack of A1AT in lungs causes emphysema
285
histology of alpha-1 antitrypsin deficiency
**intracytoplasmic globules** of A1AT staining for **PAS (periodic acid Schiff)**
286
features of alpha-1 antitrypsin deficiency
* kids: neonatal jaundice * adults: emphysema & chronic liver disease
287
biochemistry of alpha-1 antitrypsin deficiency
* low serum A1AT * absent a-globulin band on electrophoresis
288
2 benign liver tumours
* hepatic adenoma * haemangioma
289
hepatic adenoma * association * management
* **OCP** * resection if symptomatic, >5cm or no shrinkage once stopped OCP
290
most common benign liver lesion
haemangioma | no treatment
291
most common malignant liver lesion
secondary tumour (met from GI, breast or bronchus) | usually multiple
292
types of malignant liver tumours
* hepatocelular carcinoma * cholangiocarcinoma * haemangiosarcoma * hepatoblastoma * secondary tumour
293
hepatocellular carcinoma * RF
* chronic liver disease * viral hepatitis * alcoholic cirrhosis * haemochromatosis * NAFLD * **aflatoxin-B1 (in grains)** * androgenic steroids
294
afltatoxin-B1 associated with?
HCC
295
screening for HCC?
6 monthly USS in cirrhotic patients
296
marker for HCC
AFP (alpha-fetoprotein)
297
what are cholangiocarcinomas
adenocarcinomas arising from bile ducts | intra or extra hepatic
298
cholangiocarcinomas: assoication
gallstones
299
causes of cholangiocarcinoma
* **PSC** !! * parasitic liver disease * chronic liver disease * congential liver abnormalities * lynch syndrome type 2
300
what is haemangiosarcoma
* cancer of vascular epithelium * highly invasive
301
what is hepatoblastoma
* from immature liver precursor cells * occurs in kids / infants - presents with abdo mass
302
most to least common malignant liver tumours
* secondary tumours * hepatocellular * cholangiocarcinoma * haemangiosarcoma * hepatoblastoma
303
normal histology of stomach
lined by gastric mucosa, columnar epithelium (mucin secreting) & glands | NO GOBLET CELLS
304
what do parietal cells secrete
HCL & intrinsic factor
305
what do chief cells produce
pepsinogen
306
Histology of acute vs chronic gastritis
* acute = neutrophils * chronic = lymphocytes & plasma cells
307
H.pylori complication?
MALT lymphoma
308
peptic ulcer made worse with food
gastric ulcer
309
peptic ulcer made better with food
duodenal ulcer
310
Breach through muscularis mucosa into submucosa
ulcer | otherwise an erosion
311
histology of gastric ulcer
punched out lesion with rolled margins
312
most common type of gastric cancer
adenocarcinoma
313
Subtypes of gastric adenocarcinoma
* intestinal * diffuse
314
histology of intestinal gastric cancer?
* well differentiated * golbet cells present following intestinal metaplasia
315
diffuse gastric adenocarcinoma histology
poorly differentiated, no gland formation
316
subtypes of diffuse gastric adenocarcioma
* signet ring cell carcinoma * linitis plastica
317
pathognomonic mets for gastric cancer
* left supraclavicular node (Virchows node) * umbilicus (Sister Mary Joseph nodule)
318
which peptic ulcer is more common
duodeal ulcer
319
which population has higher incidence of gastric cancer
* japan, china * eat fermented, pickled, smoked foods
320
coeliac disease HLA associations
DQ2 DQ8
321
which ethnicity has coeliac disease
irish women
322
rash associated with coeliac
dermatitis herpetiformis (itchy rash on extensor)
323
association of coeliac
hyposplenism | recomend penumococcal vaccine
324
Differential for coeliac
tropical sprue
325
serological tests for coeliac
* IgA anti-tissue trasglutaminase (TTG) * IgA anti-endomysial antibody (EMA) * anti-gliadin (poor marker of disease control)
326
poor marker of disease control in coeliac
anti-gliadin antibodies
327
gold standard for coeliac
distal duodenal biopsy (while on gluten diet for at least 6 wks before)
328
biopsy findings of coeliac?
* villous atrophy * crypt hyperplasia * increased intraepithelial lymphocytes | normal villous: crypt ratio = 2:1
329
malignancy associated with coeliac
enteropathy-associated T cell lymphoma (EATL) | 10% risk
330
what is carcinoid syndrome
group of tumours of **enterochrommafin origin** - produce **5-HT (serotonin)**
331
where are carcinoid tumours found
commonly small bowel other: lung, ovaries, testes
332
features of carcinoid syndrome
* bronchoconstriction * flushing * diarrhoea
333
features of carcinoid crisis
* life threatening vasodilation * hypotension * tachycardia * bronchoconstriction * hyperglycaemia
334
investigation for carcinoid syndrome
24hr urinary 5-HIAA
335
managmenet of carcinoid syndrome
ocretotide (somatostatin analogue)
336
sign seen in duodenal atresia
double bubble sign
337
coffee bean sign
sigmoid volvulus
338
ischarmic colitis occurs in what areas?
* **watershed areas**: eg. splenic flexure & rectosigmoid * areas most distal to arteries
339
IBD where smoking worsens symptoms
crohns | improves sx in UC
340
which IBD is slightly more common
UC
341
crohns disease: pathognomonic features
* whole GI tract * transmural * terminal ileum most common * skip lesions * cobblestone mucosa * non-caseaeting granulomas * fistula * 1st lesion: aphthous ulcer * deep rosethorn ulcers * can join to form serpentine ulcers
342
UC: pathognomonic features
* rectum most common * backwash ileitis (when it affects small intestine) * superfirical lesions confined to mucosa * crypt abscesses * pseudopolyps (islands of regereating mucosa)
343
IBD most likely to cause bloody diarrhoea
UC
344
mouth sign associated with IBD
angular stomatitis (iron deficiency)
345
eye sign associated with IBD
anterior uveitis
346
skin sign associated with IBD
* erythema nodosum * erythema multiforme * pyoderma gangrenosum * digital clubbing
347
joint sign of IBD
migratory assymetrical polyarthropathy of large joints
348
severe complication of UC (acute)
toxic megacolon
349
severe risk of UC (chronic)
adenocarcinoma
350
induce remission in crohns
corticosteroids
351
induce remission in UC
5-ASA +/- steroids
352
maintain remission in crohns
* 1st line: azathioprine, mercaptopurine * 2nd line: methotrexate
353
maintain remission in UC
* 1st line: 5-ASA * 2nd line: azathioprine
354
hirschprungs disease * association * red flag * gold standard investigation * treatment
* downs syndrome * no meconium >48hrs * rectal biopsy * resection of affected bowel & ano-rectal pull through
355
finding of biopsy in hirschprungs
hypertrophied nerve fibres, no ganglia
356
C.diff complication
pseudomembranous colitis
357
prescence of diverticula =
diverticulosis
358
infected diverticula =
diverticulitis
359
colorectal neoplastic polyp
adenoma
360
how are colorectal adenomas classified?
architecture * tubular * tubulovillous * villous
361
feature and prongosis of villous adenoma
* worst prognosis * hypoproteinaemic hypokalameia (leak a lot of protein & potassium) | VILLAINOUS = bad prognosis
362
most important RF for malignancy in adeoma? + others
most important = **large size** other: * degree of dysplasia * increased villous component
363
explain formation of adenocarcinoma & adenoma (colorectal)?
* normal colon * first mutation in 1st **APC **gene --> at risk * 2nd mutation to remaining APC gene --> adenoma * progresses to carcinoma after activation of **KRAS, LOF mutations of p53**
364
non neoplastic colorectal polyps
* hamartomatous polyp * hyperplastic polyp * inflammatory polyp
365
condition where there are 100s of hamartomatous polyps?
juvenile polyposis (autosomal dominant)
366
condition with multiple hamartomatous polyps + mucocutaneous hyperpigmentation + preckles around moutg, palms & soles
peutz-jeugers syndrome
367
peutz jeugers syndrome: * mutation * inheritance
autosomal dominant **LKB1**
368
where might inflammatory polyps be seen?
IBD (pseudopolyps)
369
2nd commonest cause of cancer death in UK
colorectal cancer
370
majority of colorectal cancers are?
adenocarciomas
371
used to monitor disease & therapy response in colorectal cancer?
carcinoembryonic antigen (CEA)
372
right sided colorectal tumours are more likely to?
bleed
373
staging for colorectal cancer
Duke's staging (to determine treatment)
374
Dukes staging for colorectal carcinoma?
* A = confined to mucosa (5yr >95%) * B1: extending into muscularis propria * B2: transmural invasion * C1: into muscularis propria + LN met * C2: transmural invasion + LN met * D = distant met (5yr <10%)
375
management of tumour <1-2 cm above anal sphincter (lower 3rd of rectum)?
abdominoperineal resection
376
management of tumour >1-2cm above anal sphincter?
anterior resection
377
management of sigmoid cancer?
sigmoid colectomy
378
managemenet of descending colon & distal transverse?
left hemicolectomy
379
management of tumour in caecum, ascending colon & proximal transverse?
right hemicolectomy
380
management of tumour in trasnverse colon?
extended right hemicolectomy
381
familial adenomatous polyposis (FAP) inheritance?
autosomal dominant
382
familial adenomatous polyposis (FAP) mutation?
APC tumour suppressor gene (C5q1)
383
how does FAP present?
>100 adenomatous polyps - all will progress to adenocaricnoma if left untreated | prophylactic colectomy
384
Gardners syndrome?
subtype of FAP with +++ skull osteomas + dental caries
385
hereditary non polyposis colorectal cancer (HNPCC)/Lynch syndrome inheritance
autosomal dominant
386
hereditary non polyposis colorectal cancer (HNPCC)/Lynch syndrome mutation
in DNA miscmatch repair genes
387
cancers caused by HNPCC/lynch?
* colorectal * endometrial * ovarian * gastric * small bowel | usually in right colon, very few polyps
388
most common familial colorectal cancer syndrome
HNPCC / lynch
389
secretin produced by
S cells of duodenum | causees release of gastric acid
390
CCK produced by?
I cells of duodenum | causes release of digestive enzyme
391
exocrine function of pancreas
digestive enzymes - amylases, proteases, lipases into ducts
392
alpha cells produce
glucagon
393
beta cells produce
insulin
394
delta cells produce
somatostatin
395
D1 stimulates secretion of what into pancreatic system?
H20
396
polypeptide secretes by pancreas that self-regulates secretion activities
pancreatic polypeptide
397
what area of pancreas controls endocrine function
islets of langerhans
398
what area of pancreas controls exocrine function
acini
399
what score indicates severe acute pancreatitis
glasgow scale 3 and above
400
most sensitive test for acute pancreatitis
serum lipase
401
pathological collection of fluid in acute pancreatitis
pseudocyst
402
what necrosis in acute pancreatitis
coagulative necrosis
403
periductal damage is due to?
obstructive causes
404
perilobular damage is due to?
ischaemic causes
405
panlobular damage is due to?
combo of obstructive & ischaemic causes
406
hypocalcaemia in acute pancreatitis?
due to fat necrosis (enzmes react with fat causing precipitation fo calcium soaps)
407
most common cause of chronic pancreatitis
alcoholism
408
autoimmune cause of chronic pancreatitis
IgG4 disease
409
how might chroic pancreatitis present different to acute?
malabsorption and secondary DM
410
Histology of chronic pancreatitis
fibrosis and loss of exocrine parenchyma with calficiation
411
complications of chronic pancreatitis
* pseudocysts * diabetes * pancreatic cancer
412
most common pancreatic carcinoma
ductal adenocarcinoma
413
usual site of pancreatic adenocarcinoma
head of pancreas
414
common presenting feature of pancreatitic cancer
painless jaundice + painless enlargement of gallbladder (courvoisiers sign)
415
recurrent superficial thrombophebilits in context of pancreatic cancer?
trousseaus syndrome
416
cancer marker in pancreatic cancer
Ca19-9 (>70)
417
chemotherapy used for pancreatic cancer?
Palliative (5-FU)
418
surgery used for pancreatic cancer?
whipples procedure
419
location of islet cell tumours (NET)
body or tail of pancreas
420
histology of islet cell tumours (NET)?
cells arranged in nests or trabeculae with granular cytoplasm
421
genetic assoication of islet cell tumours?
MEN1
422
how are islet cell tumours of pancreas classified
functional or non-functional
423
examples of functional islet cell tumours?
* insulinoma * gastrinoma * VIPoma * glucagonoma
424
features of insulinoma
Whipple's triad * hypoglycaemia, neurogylcopenic sx, prompt relief when given glucose)
425
syndrome causing gastrinoma
Zollinger-Ellison syndrome | leads to recurrent ulceration
426
what does VIPoma cause
diarrhoea
427
what does glucagonoma cause
necrolytic migrating erythema
428
marker for islet cell tumours of pancreas
chromogranin
429
Features of MEN1
* parathyroid hyperplasia/adenoma * pancreatic endocrine tumour * pituitary adeoma
430
features of MEN2a
* parathyroid adenoma/hyperplasia * medullary thyroid cancer * phaeochromocytoma
431
features of MEN2b?
* phaeochromocytoma * medullary thyroid * acoustic neuroma * +++ marfanoid habitus
432
histopathology of acinar cell carcinoma
neoplastic epithelial cells with eosinophilic granular cytoplasm. positive immunoreactivitiy for lipase, trypsin & chymotrypsin | BAD prognosis, + for enzymes, eosinophils
433
RF for gallstones
FORTY, FERTILE, FAT, FEMALES * increasing age * Females * OCP * disorders of bile matabolism
434
what is cholelithiasis
gallstones in gall bladder
435
what is acute cholecystitis
acute inflammation of gallbladder
436
sign in acute cholecystits
murphy's sign + (pain on palpation of RUQ on inspiration)
437
where is obstruction in acute cholecystitis
cystic duct
438
chronic cholecystitis feature
porcelain gallbladder (calcification & fibrosis)
439
where is obstruction in acute cholangitis (ascending)
common BD
440
feature of acute cholangitis
Charcot's triad (fever, RUQ pain, jaundice)
441
most common cause of cholangiocarcinoma
adenocarcinoma
442
cholangioarcinoma associated with?
PSC
443
most common ovarian cyst
follicular
444
cause of follicular cyst
non-rupture of dominant follicle
445
corpus luteal cyst common in?
early pregnancy
446
cause of corpus luteal cyst?
corpus luteum doesnt break down, and instead filled with blood/fluid | may present with intraperitoneal bleed
447
RF for ovarian cancer
* nulliparity * early menarche * obesity * HRT * BRCA1/2
448
genetic mutation in ovarian cancer
BRCA 1/2
449
most common type of ovarian cancer
epithelial
450
most common benign epithelial tumour
serous cystadeoma
451
Types of benign epithelial ovarian tumours
* serous cystadenoma * mucinous cystadenoma | 1st and 2nd most common benign ovarian tumours
452
serous cystadenoma histology
columnar epithelium, **psamomma bodies**
453
mucinous cystadenoma histology
mucin secreting cells
454
rare complication of mucinous cystadenoma
pseudomyxoma peritonei
455
mutation in mucinous cystadenoma
K-ras
456
most common oestrogen secreting tumour
mucinous cystadenoma
457
Malignant epithelial ovarian tumours?
* endometrioid * clear cell
458
Endometroid ovarian tumour histology
tubular glands | endometriosis is RF
459
clear cell ovarian tumour histology
clear cells, clear cytoplasm, hobnail appearance | also assoc with endometriosis
460
benign germ cell ovarian tumour
* dysgerminoma * teratoma
461
dysgerminoma is like?
* testicular seminoma * radiotherapy sensitive
462
most common ovarian maliginancy in young women
dysgerminoma
463
pattern of malignancy in dysgerminoma
benign in adults, malignant in kids
464
most common bengin ovarian tumour in younger women
teratoma
465
mature teratoma (dermoid cyst) histology
* usually cystic * contains mature tissues (skin, hair, teeth)
466
immature ovarian teratoma featre?
* solid * malignant * contains immature, embryonal tissues * secretes AFP
467
malignant germ cell ovarian tumour
choriocarcinoma
468
what does choriocarcinoma secrete
hcg
469
types of sex cord / stromal tumours (ovarian)
* fibroma * granulosa-theca * sertoli-leydig
470
ovarian fibroma features
* no hormone production * associated with Meig's syndrome ( fibroma + ascites + R sided pleural effusion)
471
granulosa-theca ovarian tumours feature + histology
* produces **E2** (oestrogen) * histology: **call-exner bodies**
472
sertoli-leydig ovarian tumour secretes?
androgens
473
metastatic ovarian tumour?
krukenberg tumour | from gastric/coloic cancer (signet cells)
474
krukernberg tumour histology
mucin prodicing **signet ring cells**
475
staging for ovarian tumours
FIGO
476
FIGO stages?
* 1: only in ovaries * 2: spread to pelvis * 3: spread to abdomen (inc. regional LN) * 4: met ouside abdominal cavity
477
risk of malignancy index formula
RMI = U X M X Ca-125 | US finding x menopausal status x ca-125
478
cut-off for RMI
200
479
most common causes of PID in UK
chlamydia & gonorrhea
480
most common causes of PID in other parts of the world
TB and shistostomiasis
481
organisms causing PID secondary to abortion / TOP
* S.aureus * Streptococcfus * C.perfringens * Coiliforms
482
Fitz-Hugh Curtis syndrome histology
"violin string" peri-hepatic adhesions
483
endometriosis theories
1. retrograde menstrual flow of endometrial cells 2. metaplastic transformation of coelomic epithelial cells 3. vascular/lymphatic dissemination of endometrial cells
484
macroscopic appearance of endometriosis
red-blue to brown vesicles - "powder burns"
485
histology of endometriomas
blood filled "chocolate cysts"
486
microscopic histology of endometiosis
endometrial glands & stroma
487
adenomyosis buzzwords
"bulky uterus" "globular uterus" "subendothelial linear striations"
488
Complications of adenomyosis
* malignant transformation * red degeneration in pregnancy
489
fibroid =
leiomyoma
490
most common tumour of female genital tract
fibroid (leiomyoma)
491
most common location of fibroid
intramural
492
macroscopic appearance of fibroids
sharply circumscribed, discrete, round, firm, grey-white tumour
493
microscopic appearance of fibroid
bundles of smooth muscle cells
494
endometial cancers subdivided into
* endometrioid (80%) * non-endometrioid (20%)
495
Types of endometrioid endometrial cancers?
* secretory * endometrioid * mucinous
496
which type of endometrial cancer is related to oestrogen excess
endometrioid
497
obesity related to which endometrial cancer
endometrioid (E2 excess)
498
types of non-endometrioid endometrial cancer
* papillary * sesous * clear cell
499
serous endometrial cancer mutation
p53
500
clear cell endometrial cancer mutation
PTEN mutation p53 HER-2 amplifications
501
FIGO staging for endometrial cancer
* stage 1: only in uterus * 2: spread to cervix * 3: to pelvic area * 4: met to rectum/bladder/distal organs
502
normal cervical histology
* outer cervix covered by squamous epithelium * endocervical cancal by columnar glandular epithelium * squamocolumnar junction separates them
503
area where columnar epithelium of cervix transforms into squamous cells (metaplasia)
transformation zone
504
why is transformation zone susceptible to malignant change
high rate of cell turnover
505
HPV trains causing CIN
HPV 16 & 18
506
grading of CIN from histology
1: dysplasia confined to deepest 1/3 of epithelium 2: lower 2/3 3: full thickness, but basement membrane intact
507
what is CGIN
cervical galndular intraepithelial neoplasia * less common * requires excision of entire endocervix
508
2nd most common cancer in women worldwide
cervical cancer
509
most common type of cervical cancer
adenocarcinoma -rest are squamous cell
510
what marks change from CIN3 to cancer
invasion through basement membrane
511
how does HPV cause cervical cancer
* HPV encodes E6 and E7 proteins * E6 inactivated p53 leading to proliferation * E7 inactivated retinoblastoma (Rb) gene --> proliferation | HPV proteins inactivate tumour suppressor genes
512
FIGO staing of cervical cancer
* 0= CIN * 1 = only cervix * 2 = spread to upper 1/3rd vagina * 3 = spread to pelvic side wall or/and lower 1/3 vagina * 4 = met beyong pelvic to bladder/bowel
513
normal vulval histology
squamous epithelium
514
vulval cancer orginiates from?
VIN
515
VIN classified into?
* usual type * differentiated type
516
usual type VIN: * association * histology
* HPV 16/18, smoking, immunosuppresion * warty, basaloid, mixed | younger women
517
differentiated type VIN * association * histology
* lichen sclerosus * keratinised squamous epithelium | more likely to progress to cancer
518
vulval carcinoma types
* mainly squamous cell * clear cell adenocarcinoma - teenagers, associated with diethylstilbestrol * primary vaginal carcinoma - older women usual squamous carcinoma
519
triple assessment of breast cancer comprises?
1) clinical examination 2) Imaging: USS or mammography (cut-off 35) 3) cytology & histology
520
how is cytopathology of a breast tissue done?
* fine needle aspirate * C1 (inadequate), C2 (benign), C3 (atypia) C4 (suspicious of malignancy) C5 (malignant)
521
how is histopathology of a breast tissue done?
* core biopsy * B1 (normal), B2 (benign) B3 (uncertain) B4 (suspicious) B5 (malignant) B5a (DCIS), B5b (invasive carcinoma)
522
what is gold standard for diagnosis fo breast cancer
histopathology
523
normal breast histology
ductal-lobular system lined by inner glandular epithelium
524
lactational acute mastitis organism
Staph aureus
525
FNA cytology of acute mastitis
abundance of neutrophils
526
non-lactational acute mastitis - cause
keratinising squamous metaplasia blocks lactiferous ducts (smoking association)
527
what is fat necrosis
inflammatory reaction to damaged adipose tissue (usually obese)
528
fat necrosis presentation
painless breast mass / skin thickening
529
cytology of fat necrosis
empty fat spaces, histiocytes & giant cells | giant cells cos fat
530
most common lump in women ages 20-40
fibroadenoma
531
definition of firboadenoma
benign neoplasm of a lobule arising from fibro (stromal) and glandular (adenomal) epithelium
532
presentation of fibroadenoma
painless, mobile "breast mouse" firm smooth mass
533
cytology of fibroadenoma
braching sheets of epithelium, bare bipolar nuclei & stroma
534
breast cyst presentation
painless transillumimable lump in peri-menopausal women that is mobile and correlates with menstraul cycle
535
duct ectasia definition
dilatation of milk ducts due to blockage
536
important risk factors for duct ectasia
smoking
537
presentation of duct ectasia
thick yellow-green-white nipple discharge
538
complication of duct ectasia
if ducts get infected can lead to inflammatory symtpoms and abscess formation
539
cytology of duct ectasia
nipple discharge - proteinacious material and macrophages
540
histology of duct ectasia
duct dilatation, proteinacious material and periductal inflammation
541
intraductal papilloma definition and types
* benign papillary tumour from duct system * peripheral papilloma = small terminal ductules (clinically silent) * central papilloma = larger lactiferous ducts (nipple discharge)
542
presentation of intraductal papilloma
sub-areolar mass +/- bloody nipple discharge
543
is intraductal papilloma seen on mammography
no
544
histology of intraductal papilloma
**papillary mass** with dilated duct lined by epithelium
545
what is radial scar
benign slceroing lesion - central scarring surrounded by proliferating glandular tissue (stellate pattern)
546
mammography finding of radial scar
stellate mass
547
histology of radial scar
central, fibrous, stellate area
548
phyllodes tumour definition
arises from interlobular stroma (like fibroadenoma) with increased cellularity/mitoses
549
how are phyllodes tumours classified
* low grade or high grade * most benign but can be agressive so excised
550
breat mass that can be benign or malignant
phyllodes tumour
551
histology of phyllodes tumour
**branching**/**leaf** like fronts/**artichocke** appearance
552
fibrocystic disease presentation
lumpy breasts changes according to mentstrual cycle
553
histology of fibrocystic disease?
**dilated large ducts** that may become **calcified**
554
3 proliferative breast conditions
* usual epithelial hyperplasia * flat epithelial atypia (atypical ductal carcinoma) * in situ lobular neoplasia | intraductal epithelial proliferations with risk of invasive disease
555
usual epithelial hyperplasia * histology * cancer risk
* growth of glandular tissue & epithelial cells forming **fronds** * 1-2%
556
flat epithelial atypica * histology * cancer risk
* multiple layers of epithelial cells & lumens more **regular & round** with **punched out areas** * 4x risk
557
in situ lobular neoplasia * histology * cancer risk
* solid proliferation of **aplastic cells** with little space where you can still see lumens * 7-12x cancer risk
558
most common cancer in women
breast
559
susceptibility genes for breast cancer
* BRCA1/2
560
screening program for breast cancer
47-73 y/o every 3 years for mammography
561
non-invasive form of breast cancer
ductal carcinoma in situ (DCIS)
562
invasive form of breast cancer
* invasive ductal carcinoma * invasive lobular carcinoma * pagets disease of nipple (eczematous)
563
how is lobular carcinoma in situ found
* always incidentally * no microcalcifications or stromal reactions
564
lobular carcinoma in citu cells lack?
E-cadherin
565
ductal carcinoma in situ - mammography?
microcalficiation
566
histology of DCIS
ducts filled with **atypical epithelial cells**
567
invasive ductal carcinoma - buzzwords
Big, pleiomorphic cells
568
invasive lobular carcinoma - buzzword
cells aligned in single file chains / strands
569
tubular carcinomas of breast - buzzword
well-formed tubules
570
mucinous carcinoma of breast - buzzword
cells produce extracellular mucin
571
what is taken into consideration in breast cancer grading
* nuclear polymorphism * tubule formation * mitotic activity
572
grades of breast cancer
* grade 1: well differentiated <5 * grade 2: moderately 6-7 * grade 3: poorly differentiated 8-9
573
good prognosis of breast cancer
ER/PR + | predicts good response to tamoxifen
574
bad prognosis in breast cancer
HER-2 +
575
most important prognostic factor of breast cancer
status of axillary LN
576
ER/PR +, HER2 - responds to
tamoxifen | low grade
577
ER/PR - HER2 + responds to
herceptin | high grade
578
which breast cancer is triple negative (ER/PR/HER2 - )
basal cell carcinoma
579
tamoxifen mechanism
mixed agonist/antagonist of estrogen
580
herceptin/trastuzumab mechanism
monoclonal antibody to HER2
581
caution of herceptin/trastuzumab?
* toxic to myocardium * moitor LVEF
582
Basal- like carcinoma of breast histology
sheets of markedly atypical cells with lymphocytic infiltrate - stains + for CK5/6/14 | BRCA association
583
breast cancer type that commonly has vascular invasion & distant met spread
basal-like carcinoma
584
layers of the skin from superficial to deep?
* (stratum) corneum * lucidum * granulosum * spinosum * basale | Come lets get some beers
585
increase in stratum corneum/keratin
hyperkeratosis
586
nuclei in stratum corneum
parakeratosis
587
increase in stratum spinosum
acanthosis
588
decreased cohesions between keratinocytes
acantholysis
589
intercellular oedema
spongiosis
590
linear pattern of melanocyte proliferation with epidermatl basal cell layer
lentiginous
591
shiny plaque apperance on surface of skin
lichenoid
592
thickened skin
psoriasiform
593
dermatitis/eczema presentation
* dry itchy rashes * often history of atopy
594
theories surrounding dermatitis
1) inside out theroy: immune system --> IgE sensitisation --> skin barrier dysfunction 2) outside in theory: defective skin barrier --> allergen exposure --> IgE sensitisation
595
which conditions are under dermatitis
* atopic dermatitis * contact dermatitis * seborrheic dermatitis
596
acute histology of dermatitis (all)
* fluid collection in dermis (**spongiosis**) * **eosinophil infiltrate** in dermis * **dilated thermal capillaries**
597
chronic histology of dermatitis
* acanthosis * crusting, scaling
598
atopic dermatitis presentation in different ages
* infants: face scalp, extesnor * older: flexural areas
599
cause of atopic dermatitis
IgE-mediated
600
Contact dermatitis presentation
* erythema, swelling itching * commonly ear lobes and neck (jewellery), wrist (watch), feet (shoes)
601
cause of contact dermatitis
type IV HSN rxn
602
seborrheic dermatitis cause
inflammatory reaction to yeast malasseiza furfur
603
presentation of seborrheic dermatitis in each age group
* infants: cradle cap & nappy sites (large yellow scales) * adults: erythema, fine scaling, mildly pruritic (face, eyebrow, ear)
604
psoriasis feature
erythematous, well demarcated scaly plaques
605
pathophysiology of psoriasis
**Type IV T cell HSN rxn** in epidermis causes further T cell recuitment and release of pro-inflammatory TNF-a & IFN-γ leading to **keratinocyte hyperproliferation** & epidermal thickening
606
Histology of psoriasis
* **Parakeratosis** * neutrophilia * **test tubes in a rack** apperance (clubbing of rete ridges) * **munro's microabscesses**
607
Types of psoriasis
* chronic plaque * flexudral * guttate * ertyhrodermic/pustular * koebner phenomenon
608
most common type of psoriasis
chronic plaque psoriasis
609
chronic plaque psoriasis affects?
extensor surfaces
610
flexural psriasis affects?
groin, natal cleft, sub-mammary area
611
Guttate psoriasis features
"rain drop" plaques often in children on trunk - 2 kws post-group A strep infection
612
what is erythrodermic/pustular psoriasis
* emergency * severe disease + systemic sx
613
what is koebners phenomenon
plaques forming at/along sites of trauma
614
rubbing psoriasis plaques may cause?
pin-point bleeding (**Auspitz' sign**)
615
nail changes in psoriasis
* Pitting * onycholysis * subungual hyperkeratosis
616
arthritis in psoriasis
* DIP * **Arthritis mutilans (telescoping)** * Spondylopathy * Symmetrical polyarthritis
617
Lichen planus appearance
* Pruritic * pruple * polyglonal * papules * plaques | 5 Ps
618
fine white lines in mouth in lichen planus
wickham's striae
619
location of lichen planus
usually inner survfaces of wrists + oral mucous membrane
620
cause of lichen planus
T cells attack BM
621
histology of lichen planus
hyperkeratosis with **saw-toothing of rete ridges** &** civette bodies **(dead cells releasing nucleus)
622
erythema multiforme appearance
annular target lesions commonly on extensor surface of hands & feet
623
Infective causes of erythema multiforme
* HSV * mycoplasma
624
drug causes of erythema multiforme
SNAPP * sulphonamides * NSAIDs * Allopurinol * Penicllin * Phenytoin
625
Erythema multiforme spectrum of disease?
erythema multiforme --> steven johnsons syndrome --> toxic epidermal necrolysis
626
ertyhema multiforme, SJS and TEN have waht mechanism?
lichenoid inflammation
627
Steven Johnsons syndrome presentation
* sheets of skin detatment (<10% body surface vs >30% in TEN)
628
Sign in SJS
**Nikolsky's sign positive** - rubbing skin results in pealing of outer layer (separation of demis) | mucosal invovlement prominent
629
drugs causing SJS
sulphonamide abx, anti-convulsants
630
dermatitis herpetiformis: association & cause
* coeliac diseaase * **IgA** binds to basement membrane causing **subepidermal bulla**
631
feature of dermatitis herpetiformis
itchy vesicles on extensor surfaces of elbows
632
histology of dermatitis herpetiformis
* subepidermal bullae * IgA deposits at tips of demal papillae
633
Bullous pemphigoid: cause
* **IgG & C3** complement bind to **hemidesmosomes of BM** * causes epidermis to lift off and fluid to accumulate in space * SUBepidermal bullae
634
clinical feature bullous pemphigoid
* **large tense bullae** * do not rupture as easily
635
Histology of bullous pemphigoid
* **eosinophilia** * **linear deposition of IgG** along BM
636
pemphigus vulgaris cause
* **IgG** binds to **desmoglein 1 & 3** between keratinocytes in SS causing acantholysis * INTRAepidermal bulla
637
Clinical features of pemphigus vulgaris
* bullae **rupture easily** (flaccid bullae) leaving red raw surface * skin & **mucosal surfaces** * **Nikolsky's sign +ve**
638
Histology of pemphigus vulgaris
* **intraepidermal bullae** * netlike pattern of **intercellular IgG deposits** * Acantholysis
639
pemphigus follaceus cause
IgG against desmoglein in epidermis causes detatchment of superficial keratinocytes
640
Benign cutaneous neoplasm
seborrheic keratosis
641
features of seborrheic keratosis
rough plaques, **waxy, stuck on apperance**
642
histology of seborrheic keratosis
keratin horns in epidermis
643
Pre-malignant neoplastic cutaneous neoplasms?
* acitinic keratosis * keratoacanthoma * Bowens disease (SCC in situ)
644
Acitinic keratosis appearance
rough sandpaper like texture lesions on sun-exposed areas
645
histology of acitinic keratosis
SPAIN * Solar elastosis * Parakeratosis * Atypical cells * Inflammation * Not full thickness
646
Appearance of keratoacanthoma
rapidly growing dome shaped nodule - may have necrotic crusted centre | grows every 2-3 wks and spontaneously clears
647
keratoacanthoma has similar histology to?
SCC
648
Bowens disease (SCC in situ) apperance
* flat, red, scaly patches on sun-exposed areas * can form horns on skin
649
Bowens disease histology
intact BM but full thickness & atypia/dysplasia
650
Squamous cell carcinoma appearance
Ulcerative, crusting, hyperkeratotic +/- rolled edges | can met and be locally invasive
651
histology of SCC
atypia/dysplasia spreading through BM into dermis
652
Basal cell carcinoma apperance
"rodent" ulcer, well defined rolled edges, pearly surface often with telangiectasis | rarely met but locally destructive
653
histology of BCC
mass of basal cells pushing down into dermis and palisading
654
Benign melanocytic ?
melanocytic nevi (moles)
655
Types of melanocytic naevi
* junctional * compound * intradermal
656
Melanoma histology
atypical melaoncutes, initially growing horizontally in epidermis (**radial growth phas**e), then vertically into dermis (**vertical growth phase**) producing **buckshot appearance** (pagetoid cells)
657
most important prognostic factor in melanoma
breslow thickness (depth of lesion)
658
Most common type of melanoma
superficial spreading
659
subtype of melanoma from most to least common
* superficial spreading * nodular * lentigo maligna * acral lentiginous
660
superifical spreading melanoma feature
irregualr morders with colour variation
661
lentigo maligna features
sun-exposed areas, elderly white people, flat slowly growing black lesion
662
acral lentiginous melanoma features
* palms, soles, subungual * more in asians & black
663
ABCDE of melanoma?
* Assymetry * Border * Colour * Diametes * Evolving
664
pytiriasis rosea appearance
**salmon pink rash** apperas first (**herals patch)** followed by oval macules in **christmas tree distribution**
665
cause of pytiriasis rosea
HHV-6 & 7
666
what HSN rxn is SLE
type III
667
what complement marker low in SLE
low C4
668
feature of severe SLE disease
low C3 as well
669
populations susceptible to SLE
* Females * Afro-carribean
670
HLA associations SLE
2,3, SLE DR3 (mostly) + DR2
671
autoantibodies in SLE
ANA, Anti-dsDNA, Anti-Sm (smith)
672
most specific SLE ab
Anti-Sm
673
antibody in drug-induced lupus
anti-histone
674
LE bodies (degraded nuclear material) found in what disease
SLE
675
kidney feature of SLE
"wire loop" glomeruli
676
CNS feature of SLE
small-vessel angiopathy
677
spleen feature of SLE
'onion skin' lesions
678
heart feature SLE
libman sacks endocarditis
679
HLA association in scleroderma
DR5 & DRw8
680
antibody in limited systemic sclerosis
anti-centromere
681
antibody in diffuse systemic sclerosis
anti-topoisomerase II (Scl-70)
682
Histology of limited systemic sclerosis
* increasec ollagen in skin & organs * **"onion skin" thickening of arterioles**
683
histology of diffuse systemic sclerosis
inflammation within or around muscle fibres
684
signs / sx in limited systemic sclerosis
* distal skin only (forearms) * CREST * calcinosis * raynauds * esophageal dysmotility * sclerodactyly * telangeictasisa
685
limited SS associated with what in old age?
pulmonary HTN
686
diffuse SS signs / sx
* skin changes anywhere (distal + proximal) * Tendon friction + Raynauds * Widespread organ involvement
687
diffuse SS associated with what?
pulmonary fibrosis
688
Definitive investigation for polymyositis / dermatomyositis
muscle biopsy
689
autoantibodies in polymyositis / dermatomyositis
Anti-Jo-1 (tRNA synthetase)
690
what might be raised / abnormal in polymyositis / dermatomyositis
* CK, LDH, myoglobin * abnormal EMG
691
underlying malignancy in dermatomyositis
ovarian, pancreatic, NHL
692
underlying malignancy in Polymyositis
NHL, lung, bladder
693
dermatomyositis histology
endomysial inflammatory infiltrate
694
polymyositis histology
"drop out" of capillaries & myofibre damage
695
main feature of polymyositis
proximal muscle weakness
696
cutaneous features of dermatomyositis
* Heliotrope rash (over eyelids) * Gottrons papules * Systemic V rash * Mechanics hands
697
polymyositis / dermatomyositis associated with?
pulmonary fibrosis
698
name 2 large vessel vasculites?
* takayasu's arteritis * Temporal arteritis (GCA)
699
Takayasu arteritis affects?
branches of aortic arch
700
sx of takayasu's arteriritis
* inflammatory phase: FLAWS * pulseless phase: **Pulseless**, claudication, cold hands | Japanese women
701
giant cell arteritis features and association
* scalp tenderness, headache, jaw claudication * polymyaglia rheumatica | age >50, high ESR
702
Investigations for GCA + definitive Ix
* ESR 1st * Definitive: temporal artery biopsy
703
HIstology of GCA
granulomatous transmural inflammation + giant cells + skip lesions
704
name 2 medium vessel vasculitis
* polyarteritis nodosa (PAN) * Buerger's diseaase
705
Polyarteritis nodosa underlying cause
Hep B
706
polyarteritis nodosa main feature
renal involvement (spares lungs)
707
angiography feature of PAN
microaneurysms (string of pearls / **rosary bead** appearance)
708
histology of PAN
fibrinoid necrosis & neutrophil infiltration
709
Buergers disease main RF
heavy smoking, men <35
710
angiogram feature of buerger's disease
corkscrew apperaance
711
4 small vessel vasculitis
* GPA (wegeners) * eGPA (churg-strauss) * microscopic polyangitis * HSP (IgA vasculitis)
712
GPA antibody
cANCA
713
GPA triad?
* URTI: **saddle nose**, sinusitis, epistaxis * LRTI: **pulmonary haemorrhage** * Kidney: **cresenteric glomerulonephritis**
714
eGPA featurese
asthma, allergic rhinitis, eosinophilia
715
eGPA ab
p-ANCA
716
microscopic polyangiitis features
pulmonary renal syndrome (pulmonary haemorrhage + rapidly progressive glomerulonephritis)
717
ab in microscopic polyangiitis
p-ANCA (but sometimees can be c-ANCA)
718
HSP mediated by?
IgA
719
Amyloidosis structure
beta-pleated sheet resistant to enzyme degradation
720
Which form of amyloidosis is most common
Primary (AL)
721
Primary (AL amyloidosis) * deposition of? * associated with?
* Ig light cahins * Multiple myeloma (bence jones proteins in urine and inceased BM plasma cells)
722
secondary (AA amyloidosis) * build up of what? * cause?
* serum amyloid A (acute phase protein) * due to chronic inflammation/infection * eg. autoimmune (RA), chronic infections (TB)
723
deposition of what in haemodialysis associated amyloidosis?
beta-2 microglobulin | assoc with carpal tunnel syndrome
724
most common cause of familial amyloidosis?
familial mediterranean fever (AR)
725
most common presentation of amyloid deposits?
nephrotic syndrome
726
clinical features of amyloidosis
* nephrotic syndrome * restrictive cardiomyopathy * heart failure * hepatosplenomegaly * macroglossia * carpal tunnel syndrome
727
Sarcoidosis histology
* non-caseating granulomas * Schauman and asteroid bodies (inclusion of protein & calcium)
728
sarcoidosis more severe disease in?
afro-carribeans
729
organ most commonly involved in sarcoidosis
lungs
730
cXR finding sarcoidosis
* bilateral hilar Lymphadenopathy * pulmonary infiltrates (fine nodular shadowing in mid zones)
731
skin features of sarcoidosis
* erythema nodosum * lupus pernio (red/purple lesion on nose) * skin nodules
732
eye features of sarcoidosis
* anterior uveitis * posterior uveitis * uveoparotid fever * keratoconjunctivitis * lacrimal gland enlargment
733
blood features of sarcoidosis?
* anaemia * leukopenia * hypercalcaemia * hypercalciuria (stones)
734
enzymes high in sarcoidosis
* ACE * 1-alpha hydroxylase
735
sarcoidosis on spirometry
restrictive
736
electrolyte high in sarcoidosis and why
* high calcium * ectopic 1-alpha hydroxylase from activated macrophages