GALS Examination - Revision Card

WIPER

GAIT
Observe gait (3)
Observe patient in anatomical position (8)

SPINE
Inspect spine (3)
Assess lateral flexion of neck (1)
Assess lumbar spine movement (2)

ARMS
Observe shoulder movement (3)
Observe back of hands and wrists (2)
Observe palms (2)
Assess power grip & grip strength (4)
Assess fine precision pinch (1)
Squeeze MCPs (1)

LEGS
Assess full flexion and extension (3)
Assess internal rotation of hip (1)
Patellar tap (1)
Inspect feet (3)
Squeeze MTPs (1)

GALS Examination

- Screening examination
- Examines Gait, Arms, Legs and Spine
... but we usually examine them in the order of Gait, Spine, Arms, Legs, for simplicity.
... however, 'GSAL' is less catchy.

WIPER

"Hi, my name is Anamika Basu, I'm a final year student.

- Is it alright if I do a quick examination of your joints and general movement?

- For this examination we need to have in your underwear, is that okay?

- And we'll need to have you standing up at the start of it."

GAIT

"Can you walk a few steps away from me, turn around and walk back to me?"

I'm observing the patient's gait for symmetry, smoothness and the ability to turn quickly.

"Can you stand like this (imitate anatomical position)?"

I'm looking from 

- behind, from the sides, and from the front,

all the time assessing for 

- the bulk and symmetry of the shoulder, gluteal, quadriceps and calf muscles,

- limb alignment,

- alignment of the spine,

- level of iliac crests,

- ability to extend elbows and knees,

- any popliteal swelling,

- and any abnormalities of feet.

SPINE

As the patient is standing, I am also inspecting the spine

- behind for scoliosis

- and from the side to observe any lordosis or kyphosis

"Can you tilt you head to each side, with the ear towards the shoulder?"

I'm assessing the lateral flexion of the neck

"Can you bend down to touch your toes, as much as you can?"

I'm assessing functional movement

"Okay, now I'm just placing two fingers on your back. Can you straighten up for me?"

I placed my fingers on the lumbar vertebrae to see if they move apart on flexion and back together on extension. This also helps to distinguish the patient's movement from compensatory hip flexion.

ARMS

"Would you like to sit on the couch?

- Can you put you hands behind your head?"

I'm assessing

- shoulder abduction,

- external rotation

- and elbow flexion

"Okay, relax, now can you put you hands out like this, with the palm face down, and fingers outstretched?"

I'm looking for any 

- joint swelling

- or deformity

"Can you turn your hands over?"

I'm assessing the palms for

- muscle bulk

- and any abnormalities

"Can you make a fist for me?"

I'm assessing

- power grip,

- hand and wrist function

- and range of movement in fingers

"Can you squeeze my fingers for me?"

I'm assessing grip strength

"Can you bring each finger in turn to meet the thumb?"

I'm assessing fine precision pinch, which has functional importance

"Do you have any pain in your hands?"

I'm gently squeezing across the MCP joints to check for tenderness, which might suggest inflammatory joint disease, remembering to look at the patient's face for discomfort.

LEGS

"Can you lie down on the couch for me? Are you comfortable? Do you have any pain in your legs? I'm just going to have a feel of your knee joints."

I'm assessing for 

- full flexion and extension of the knees

- and feeling for ant crepitus 

- x2

I'm flexing the hip and knee to 90 degrees, holding the knee and ankle. This is to assess the

- internal rotation of each hip in flexion

- x2

"I'm going to tap on your knee."

I'm performing a patella tap on each knee to check for patellar effusion

This involves me sliding down my hand down the thigh and push over the suprapatellar pouch, forcing any effusion behind the patella. Keeping the pressure on that hand, I use the first two fingers of my other hand to push the patella down gently. If the patellar bounces and 'taps' this is positive for the present of an effusion.
- x2

I'm inspecting the feet for

- swelling,

- deformity

- and calloses on the soles

"Is there any pain in your toes?"

I am squeezing across the MTP joints to check for tenderness

GALS is written down a column with two additional columns headed appearance and movement.

A tick is given for normal findings, and a cross for abnormal ones, with extra notes noting the details.

Lung Tumours

Normal Respiratory Epithelium

- Ciliated columnar cells
- Goblet mucin-producing cells
- Kulchitsky cells (diffuse/dispersed endocrine system)
- Reserve Cells (stem cells)

Tumour-Like Lesions
Pulmonary Hamartoma
Developmental disorder of cell growth - excessive growth of normal cells & tissues at a site

1ry Tumours
Lymphoid
Lymphoma
Epithelial
Benign - Bronchial Adenoma
Borderline - Carcinoid
Malignant - Bronchogenic Carcinoma
Connective Tissue

Benign - Leiomyoma/Lipoma
Malignant - Leiomyosarcoma/Liposarcoma

2ry tumours
Metastasis

Epithelial
Connective Tissue
Lymphoid

Benign Tumours
Well-circumscribed
*single masses of 1 cell type

Well-differentiated
Little cytological atypia

Interstitial Lung Disease - Goodpasture's Syndrome & Wegener's Granulomatosis

Interstitial Lung Disease


Goodpasture's Syndrome
Autoantibodies to alveolar basement membrane
= pulmonary Hg & fibrosis

Autoantibodies to glomerular basement membrane
= proliferative, crescentic glomerulonephritis

Ix
serum anti-BM (basement membrane) antibodies


Wegener's Granulomatosis
Systemic vasculitis
Necrotising, granulomatous inflammation in upper and lower respiratory tract
= pulmonary Hg & fibrosis

*assoc/ w/ proliferative, crescentic glomerulonephritis

Ix
serum C-ANCA (anti-neutrophil cytoplasmic antibody)

Composition of the Normal Respiratory Tract

Trachea
C-shaped plates of cartilage anteriorly & smooth muscle posteriorly
Pseudo-stratified, columnar 'respiratory' epithelium

Mucous glands

Bronchus
Discontinuous foci of cartilage and continuous smooth muscle
Pseudo-stratified, columnar 'respiratory' epithelium
Mucous glands

Non-respiratory Bronchiole
No cartilage
Continuous smooth muscle
Pseudo-stratified, columnar 'respiratory' epithelium
No mucous glands

Respiratory Bronchiole & Alveolar Ducts
No cartilage
Discontinuous smooth muscle
Flat single layer epithelium
No mucous glands/goblet cells

Alveoli

Flat single layer epithelium
Type I & Type II pneumocytes

Interstitial Lung Disease - Pneumoconiosis

Pneumoconiosis

Inorg/org/chemical fumes

Inorganic = direct interstitial fibrosis

Organic = inflammation = fibrosis

Fumes = ARDS = fibrosis

NB Org & fumes also = asthma

Inorganic Pneumoconiosis

*clinically insignificant
Progressive massive fibrosis when significant respiratory compromise
Caplan's syndrome if assoc/ w/ RA

*Coal dust - anthracosis (coal-mining, *hard coal)
*Silica - silicosis (foundry work, sandblasting, stone cutting, hardrock mining)
Asbestos - asbestosis (mining, milling & fabrication, insulation - NB also causes non-isttl pulm & extra-pulm diseases)
Beryllium - Beryllium granulomatosis (mining, fabrication)
Iron Oxide - Siderosis (welding)
Barium Sulphate - Baritosis (mining)
Tin Oxide - Stannosis (mining)

Pathology

Nodules of fibrosis & chronic inflammation in Isttm


Organic Pneumoconiosis
aka extrinsic allergic alveolitis/hypersensitivity pneumonitis
Sensitisation to organic inhaled antigen
Init - Type III reaction

Late - Type IV reaction

Farmer's lung - actinomycetes in mouldy hay
Bird fancier's lung - animal proteins from faeces
Bagassosis - bagasse & paper (manufacturing of wallboard)

Pathology
Isttl inflammation by lymphocytes & granulomata
Later = fibrosis

Tx
Stop exposure = stop progression
Any damage irreversible


Organic pneumoconioses that produce asthma
Byssinosis - cotton, flax, hemp (textile manufacturing)
'Asthma' - red cedar dust, nitrous oxide, sulphur dioxide (lumbering, carpentry)
'Asthma' - ammonia, benzene, insecticides exposure (occupational/accidental)

Interstitial Lung Disease - Sarcoidosis & Idiopathic Pulmonary Fibrosis

Interstitial Lung Disease

Group of diseases
Med by immune mechanisms
Interstitial inflammation & fibrosis
Defined by lung function tests
Restrictive pattern (reduced lung compliancy & gas exchange)

Causes
Pneumoconiosis
Systemic CT (autoimm) disease
Sarcoidosis
Wegener's granulomatosis
Goodpasture's Syndrome
Post-RTx/CTx
Post ARDS
Idiopathic pulmonary fibrosis


Sarcoidosis
Systemic disease of unknown aetiology
F>M
Black>>White>>Asian
Prob response to Ag
Activated T-Cells & macrophages
Hilar LN/lung involvement in 90%
Skin/LN/Kidney involvement*
Raised serum ACE
*High calcium
Pathology
Granulomatous inflammation w/o necrosis


Idiopathic Pulmonary Fibrosis
aka
cryptogenic fibrosing alveolitis/usual interstitial pneumonitis/Hamman-Rich syndrome when rapidly progressive
M>F
60+ yrs
Interstitial pneumonitis & fibrosis
?cause
Pathology
End stage 'Honeycomb' lung (cystic air-spaces from interstitial fibrosis)
Extensive interstitial fibrosis with alveolar distortion & interstitial expansion


Tx
Steroid/further imm-supp
Variable response
*Progress to significant fibrosis


Cx
Respiratory failure
Cor pulmonale
Pulmonary HT

Tuberculosis

Mycobacterium tuberculosis

Atypical mycobacteria produce different disease
Course dependant on host immunity & organism virulence
Histology always same

- Worldwide epidemic
- Incidence rising (w/ HIV & drug resistance)
- 1ry infection *lung
- Gut/skin/lymph nodes rare 1ry sites

Classification
1ry Pulm TB
2ry Pulm TB
Progressive Pulm TB
 - cavitatory fibrocaseous TB
 - tuberculous bronchopneumonia
Extra-Pulm TB
 - single organ TB
 - miliary TB

1ry Pulmonary TB
1st contact with bacillus

Initial lesion parenchymal & sub-pleural
Involvement of draining hilar lymph nodes

Usually asymptomatic

Most cases heal by scar formation

Occ progression - * infants/imm-supp

Pathology

Ghon complex - yellow, necrotic areas in parenchyma & nodes

2ry pulmonary TB
Occurs in 5% of 1ry TB cases
Usu due to reactivation

Assoc/ with debilitation
Occ re-inf/post-BCG

Apices due to high O2 tension

Often symptomatic
May heal by scar formation

Progression more common than 1r TB

Pathology

Assmann focus - more ext parenchymal involvement of upper lobes

• Progressive pulmonary TB

Cavitatory fibrocaseous TB
Usu seen with 2ry TB

Drainage into bronchus/bronchiole
Formation of cavity
Extension of local inflammation
Spread of inflammation w/i lungs
Spread into upper airways & gut = open TB

Gd prognosis w/ Tx

Pathology
Large parenchymal lesion
W/ central cavity & surrounding necrosis

Tuberculous bronchopneumonia
Usu seen with 2ry TB

Rapid parenchymal spread via airways = open TB

Rare with Tx h/e prognosis poor

Pathology
Scattered but ext parenchymal inflammation

• Extra-pulmonary TB

Single organ TB

Usu seen with 2ry TB

Single episode of Hg spread
*Bone/kidney/adrenals/memnges/genital tract

Prognosis gd with Tx

Pathology
Single focus of destruction
eg spine = pott's disease

Miliary TB
Usu seen with 1ry TB

Widespread Hg spread
Assoc/ w/ reduced immunity
*Lungs/spleen/bone marrow/liver

Prognosis poor despite Tx

Pathology
Widespread small necrotic foci


Histology
Epitheloid cell granulomata with central necrosis & Langhans giant cells
Lymphocytes & plasma cells
Coalescence of granuloma w/ extensive central caseous necrosis
Use of ZN (Ziehl-Neelsen)staining

COPD

Chronic obstructive airways/pulmonary disease

Defined by lung function tests
Combination of bronchitis & empysema
2ry to smoking
Most = mixture of function, h/e 'pure' disease at each end of spectrum

Treatment & Prognosis

Prevention & supportive therapy
5-10% of all deaths in UK

Cx

Pneumothorax
Polycythaemia
Cor pulmonale
Pneumonia
Resp failure
Pulmonary hypertension

Pure empysema

'pink puffer'
Middle-aged to elderly
Dyspnoea early & severe
Cough minimal with scanty sputum
Infections rare
Hyperinflation as low recoil
O2 and CO2 maintained at muscular cost
Type I resp failure & cor pulmonale = terminal events

Pure chronic bronchitis
'blue bloater'

Middle-aged
Dyspnoea mild
Cough productive
Infections common
Increased airways resistance
Low O2 & CO2 levels tolerated
Repeated type II resp failure & cor pulmonale

Emphysema

Emphysema

Definition

 - Abnormal permanent increase in the size of air spaces beyond the terminal bronchioles

 - With destruction of their walls
 - Without fibrosis

Pathogenesis
Imbalance between protease & anti-protease activity

... leads to...

Neutrophils & macrophages = released proteases

Alpha-1-antitrypsin most common protease inhibitor
h/e...
Neutrophils = also release O2 free radicals

+ oxidants from cigarette smoke
Leads to inhibition of alpha-1-antitrypsin

Classification

Centriacinar emphysema
 - assoc with cig smoking & CB
 - increased protease activity
 - affects upper zone of lungs
 - 2ry to airway inflammation

Panacinar emphysema
 - assoc with alpha-1-antitrypsin deficiency

 - decreased anti-protease activity
 - affects lower zones of lungs
 - homozygotes (PiZZ) = emphysema <40
 - heterozygotes = emphysema if they smoke

Pathology
Macro
Cystic alveolar spaces (end-stage emphysema)
Micro
Destruction & dilatation of alveolar walls

Effects
Functional obstruction
 - loss of elastic recoil
 - premature closure of small airways

Chronic Bronchitis

Definition:
- Chronic sputum production, usually accompanied by chronic cough
- Most days for 3 months of year
- 2/more consecutive years
(when not due to a specific disease e.g. TB)

Pathogenesis:
Chronic irritation
Cigarette smoke & air pollution
 = Death of ciliated cells
 = Reduced mucociliary action

 = Increased mucous production
 = 2ry infection

 = Inflammation & fibrosis

Partially reversible on x Smoking

Classification
1) Simple chronic bronchitis

 - cough, no obstruction
2) Chronic asthmatic bronchitis

 - bronchospasm & wheezing
3) Obstructive chronic bronchitis 

 - COPD

Pathology

Thickened walls, increased size in mucous glands & fibrosis

Increased no of goblet cells = increased mucin production

Squamous metaplasia of respiratory epithelium

Effects
Physical obstruction

• Thickened wall

Submucous gland hyperplasia
Measured in Reid index
Normal = 0.14 - 0.36 (mean = 0.26)
Chronic Bronchitis = 0.41 - 0.79 (mean = 0.59)

• Mucus accumulation

Increased production

 - From increased goblet cells & mucous gland size
Impaired clearance

 - From loss of ciliated epithelium & thicker mucus

Obstructive Airway Disease

Obstructive Airways Disease

Acute
- Bronchiolitis
- Foreign Body

Recurrent acute
- Asthma

Chronic
- Chronic Bronchitis
- Emphysema
- Bronchiectasis

Ultimate Finals List - Advice

Ultimate Finals List

Random advice garnered from the askdoctorclarke website

Writtens
 - They are all RANDOM but not too bad. For instance we had this questions on J curves on an ECG, apparently re Hypothermia. I’d just happened to see it in the Cheese and Onion that morning, very lucky. Some questions were ok, eg. practical things like how long someone should fast before an operation etc

 - The med school repeat questions they have put up on Moodle, so learn those well and do them the day before exams for easy marks. I also think they use questions that are similar to the commercial SBA question books and websites out there, so it is worth doing those. I didn’t do many SBA books, although I did pastest and onexam websites religiously- not sure if they helped much. My friend who I revised with kept asking me questions she had struggled with from books and lots of similar questions came up- although in the exam I could never quite remember what the answer was! Either question setters are unable to be original, or they are purposefully copying questions cos they are lazy or want to give us a better chance of passing. Another possible explanation is that maybe its just core curriculum that examiners set questions on which is somewhat limited! It really helps to speed through questions if you have seen similar situations before as they are often assessing principles rather than factual recall.

 - There were lots of data interpretation questions and a fair bit of orthopaedics. Pharm was generally ok. Microbiology was horrible, especially the pictures but it wasn’t worthwhile revising all of communicable diseases for a few picture questions. I’d also ignore the pathology books and read a decent path book with colour photos  as they are very dull and you won’t remember anything from them anyway. I studied mine for weeks and it didn’t seem to help at all, I would have been better off reading the cheese and onion. Some pictures, an ecg, an x-ray come up although we did have one text definition of what chronic inflammation in the stomach looks like which was lifted directly from the pathology book but that was really just common sense re the inflammatory cell types expected.

 - The pictures in the exam were pretty impossible. One slide was nicked off UTAH path quizzes. That’s it, the rest were just random guess work! Some questions were really tricky, perhaps relying on epidemiology? Stats came up, more easy marks.

 - My recommendations for the writtens would be check the common conditions in the log book, rare conditions are not just small print but barely feature AT ALL, so don't waste your time, and don't forget to look at common ENT and ophthalmology.

OSCEs
 - The exam in no way tested the depth of my knowledge, it just wanted to check I had the basics needed to be an FY1, ie. Simple practical stuff like filling in drugs charts or giving a referral!! I must have done ok with these as I passed but it wasn’t because I’d prepared well for them, I just had to adlib in the exam, which made me believe my answers were incorrect. Examiners also give you clues. Ie. If they repeat a question, its not cos they are hard of hearing, its due to them giving you a second chance to change your answer which is Wrong! Also, they try to help and will say “are you sure there is nothing else you want to add.” This makes you feel like a wally if you know you’ve missed something, but can’t remember what it is- but you will still probably pass the station, so don’t get disheartened. It might even jog your memory.

 - My advice would be to complete your history and examinations fully and then look the examiner in the eye and tell him/her what you think without making anything up. Then you have most of the marks and it doesn't matter if you have got the diagnosis wrong.

 - Got told off for running commentary - don’t do it to a surgeon.

 - The neuro / MSK stations had a box of neuro based toys in them which i didn’t think i needed to use.

 - Time was very short on most of them so by the time you'd finished the examination you barely had time to present and that was it. One thing that is quite odd about the long stations is that some examiners let you talk through the exam, and in fact encourage it because you're short of time, and others absolutely refuse to let you. This can be very off-putting as it's not standardised and everyone seems to make up their own rules... Hmm.

 - Take a FULL history, you have 5 minutes before station starts to read the scenario and write whatever questions you may want to ask so use this time efficiently to write down differentials etc before you have even taken the history.

- The examiner jumped over and covered the patient when I had finished and was washing my hands - I felt really bad as I was going to do it in about 10 seconds, but he got there first and gave me a raised eyebrow so I just apologised to the patient.

Causes of Hyperthyroidism

• Graves' disease (thyroid-stimulating autoantibodies)
• Toxic adenoma
• Toxic multinodular goitre
• Amiodarone
• Viral (de Quervain's) thyroiditis
• Postpartum thyroiditis
• T4 overdose
• Pituitary TSH-secreting adenoma (rare)

Let us bid adieu to de Quervain,

As he is lowered into the pit of his grave.
Post the tears, we'll nod amid beers,
Over pity he couldn't be saved.

Let us bid adieu (toxic adenoma) to de Quervain (viral de Quervain's thyroiditis), 

As he is lowered into the pit (pituitary TSH-secreting adenoma) of his grave (Graves' disease).
Post  (postpartum thyroiditis) the tears, we'll nod (toxic multinodular goitre) amid (amiodarone) beers,
Over (T4 overdose) pity he couldn't be saved.

Bone Scans

Bone Scans or Skeletal Scintigraphy

We start with a bone-loving agent - Tc99, a radioactive tracer which is followed by 007, our special gamma camera that can trace our tracer, no matter where it hides.

Indications
Spread of cancer
Unexplained bone pain/musculoskeletal pain

How to Read a Bone Scan
"This is a bone scan of [NAME], date of birth [DOB], taken on [DATE]. It shows increased uptake in [HOT AREAS], while osteopenic, or reduced uptake in [COLD AREAS]. This is suggestive of [DIAGNOSIS]."

Dark or "cold" spots
- areas that absorb little or no amount of tracer
Lack of blood supply to the bone/bone infarction
Certain types of cancer eg multiple myeloma

Bright or "hot" spots
- areas that absorb increased amounts of tracer
Rapid bone growth or repair/abnormal bone metabolism eg Paget's disease
Arthritis
Tumour
Fracture eg hip fractures, stress fractures - poorly seen on X-Ray
Infection eg osteomyelitis
Malignancy

Contra-indications
Pregnancy, due to possible radiation exposure to foetus

Contamination/Artefacts
Urinary contamination - the kidneys clear the radioisotope. If patient wets themselves, or urine spots leak onto clothing or the body, these can be recorded as false positives. Can be confirmed as contamination on removal of hospital gowns and a second scan
Injection sites, where the radioisotope can 'leak'
Barium - bone scans should be performed before the use of any barium
Patient motion
Full bladder, which blocks the view of the pelvic bones
Jewellery/Metal accessories

Other Ix necessary for Dx

- as bone scans cannot distinguish between different pathology
X-ray tests
CTs
MRIs
Blood tests
Biopsy

Thrombocytosis

Plts > 500
1ry vs 2ry

1ry
'Essential thrombocythaemia'
Myeloproliferative D/O
Dx of exclusion
25% prolonged BT with abn plt function
25% vascular thromboses (thrombotic and bleeding probs)

Tx
Cytotoxic agents = decreases plt count
(hydroxyurea/radioactive 32P)

2ry
Rarely plt count >1000

Chronic bleeding
Low Fe
Trauma
Inflammation - neoplasm/chronic infection (TB)/Chronic collagen disorders (RA)

Abnormal Platelet Function

Platelets count = normal
BT = prolonged

CFx
Purpura/bleeding mucosa

Aetiology
Congenital
Acquired

Acquired
1) *Drugs (asp/NSAIDS) - NB - stop for 2 weeks and repeat BT
2) Membrane defects (CRF/polycythaemia/raised FDPs)
3) Acquired storage pool disease (myeloproliferative D/Os eg essential thrombocythaemia)

Congenital
Rare
1) Membrane abnormal (Bernard-Soulier Syndrome = deficiency in glycoprotein Ib)
2) Glanzmann's Thromboasthenia (deficiency of gp IIb/IIIa)
3) Arachadonic acid metabolism enzyme defects (cycloxygenase deficiency)
4) Storage Pool Disease (Abn/decreased alpha &/ dense granules)

Ix
i) Plt aggregation studies (ristocetin, ADP, collagen)
ii) ADP & ATP (contents of alpha and dense granules) release post max-stim
iii) Quantify specific membrane protein receptors (*gp Ib & gp IIb/IIIa)
iv) Ix arachadonic acid pathway & Ca flux
v) exclude immunological destruction (plt a/ Ig)

Tx
1) Underlying cause
2) Congenital = plt concentrates (operations/bleeding)
OR DDAVP infusion *storage PD
3) ITP
- Steroids (pred) - suppress immune reaction & inhibit splenic sequestration
- Splenectomy - decrease plt destruction
- HD IV human gammaglobulin (temporary ret-endothelial blockade)
Clinical Course
Post-splen = 60% remission
NB Proph. pneumococc. vacc & long-term penicillin
If splenectomy/steroidx ineffective - other immune suppressants eg azathioprine

Thrombocytopenia

Low Platelet Count

1) Decreased production from bone marrow

2) Peripheral destruction/consumption

- autoimmune (reticuloendothelial system)

- massive clotting process (DIC)

Ix of Choice
Bone marrow examination

Underproduction
1) BM infiltration - leukaemia/secondary malignancy
2) Decrease in megakaryocyte numbers/abnormal differentiation - aplastic anaemia/severe megaloblastic anaemia/excess alcoholism

Peripheral Destruction/Consumption
BM: megakarocytes no. normal or raised

1) Raised peripheral destruction

Pathology
Immunological
aB adh to Plt membranes
Premature ret-end Plt clearance

Aetiology
*ITP (idiopathic thrombocytopenia purpura)
2ry -
SLE
Lymphoid malignancies (B cell chronic lymphocytic leukaemias)
Drugs (quinine, rifampicin)

Ix
Plt a/ Ig (aBs bound to plt surface antigen)

2) Increased consumption

Haemostasis

DIC
Tcytopen assoc with consumption of all clotting Fx
Prolonged coag time
Intravascular haemolytic An
RBCs chopped up by fibrin strands

3) Pooling

1/3 plts pooled in normal spleen
Mod-severe splenomegaly = 'circulating thrombocytopenia'
eg myelofibrosis + mass spleen

4) Massive Transfusion
Bank blood = no functional platelets
Clumps of dead WCCs + plts = microaggregates
Plt adh (before ret-end/lung filtration)
Solution: microaggregate filter in giving set

NB + plt consumption in bleeding site/wound loss

Platelets & Investigations

Platelets
Fragments of megakaryocyte cytoplasm

Megakaryocytes
- giant cells
- multilobulated nuclei
- fr red bone marrow

Platelet Structure
Discus with surface openings on the membrane that connect to the canalicular system
Contains 2 granules
- dense (rich in ADP)
- alpha (contain fibrinogen/factor V, vWF & PA1-1)
and lysosomes
Cytoplasm = Coag Fx - a/w/ the surf membranes

Normal function
BV damage
Plts attach to exposed collagen (underneath endothelial vessel lining)
Plts shape change & degranulate
= ADP release
= activates nearby Plts
Phospholipid released from plts = coagulation system

Defects - can be in no. or function

Tests
Test of number
Electronic particle counters
Blood film

Function test
Bleeding time
 - cuff and cut
Norm <10minutes
New: In-vitro BTs

or

Lab aggregating agents (ADP/collagen) + light transmittance through plasma

Two Basic Initial Tests
1) Plt Count (n = 150-400)
NB if init. low - *artefactual, clot in sample therefore repeat
2) Bleeding Time (n = 2-10 minutes)

The Bleeding Patient

Haemostasis
1) BV constriction
2) Plt adh/activation/aggregation
3) Coagulation factors, leading to a fibrin clot

Bleeding D/Os
2 Types

Platelet Dysf
- superficial bruising
- skin/mucous membrane purpura

Coag Fx Dysfunction
- Deep muscular haemotomas
- Haemarthroses

GIT Viral Infections

Virus types
RotaV (2/RNA)
AdenoV (2/DNA)
AstroV
SRSVs/CaliciV

With Non-GI Symptoms
HepA
EnteroVs (polioV)

Characteristics
ALL unsegmented 1 stranded/RNA
Except...
RotaV - segmented 2-stranded/RNA
AdenoV 2-stranded/DNA

Signs and symptoms
D/N/Vg
Abdo cramps
Fever
D&V = severe dehydration & hypoNa

Epidemiology
*infant mortality in developing world
*children (also more severe)
a/w/ overcrowding/poor sanitation/poverty

Transmission
* F/O
Contaminated food/H20
Vomitus - SRSV-a/ GE (caliciV)

Dx
Stool samples
E-Micro/ELISA/Latex agglutination
*Useful in outbreaks
PCR
Research only
Tissue Culture
Not helpful (Viruses fastidious)

Tx
Symptomatic
Rehydration
Replace Na loss

Prevention
Hygiene
Food preparation
Clean H20
Good sewage disposal
Hospitals - hand washing, source isolation
Vaccines - polio, hep A

Food Infections of the GIT

Food Poisoning


Beware! > 50% food-related infections come from chicken


GI Defences
Acid (biocide)
Motility
Normal flora (colonisation resistance)
MALT - Peyers patches - sample lumen Ags - seed LProp with T & B cells
B Cells pass through enterocytes & attach to glycoprotein called the secretory piece
Mature secretory IgA = immune exclusion = pathological binding


Organisms


Ingestion of Toxins
Tx + receptor (GIT)

V&D

Central absoption = CNS symptoms

Staph aureus

Vomiting
2-6h
Creams
Cold meats


Bacillus Cereus
V&D
1-10h
Contam rice


Clostridium perfringens
Abdo pain & D
10-20 h
Contam meat stews


Clostridium botulinum
Paralysis
1-3d
Canned foods
Yoghurts


Enterotoxic Organisms
Organism + receptor (GIT)
Secrete Txs

Disrupt enterocyte cell signalling

Loss of Na and H20 from cell

Vibrio cholera

Water-spread

Cholera

Large volume fluid loss

Rapid dehydration

Enterotoxigenic E. coli

Food

Toxin similar to cholera

Not as severe

E. coli O157

Poorly cooked meats
Verotoxin - similar to Shigella

Toxins spread to blood stream

Affects platelets and kidneys
Haemolytic-uraemic sydrome
Enterocyte necrosis = dysentry

Enteroinvasive Bacteria

Invasive organisms = cell necrosis/apoptosis

Txs = binding/penetration/membrane damage/cell death

Erosion and ulceration = dysentry & 'bystander damage'

(acute inflamm = accumulated polymorphs in LPp = releases pore-forming toxins)

Campylobacter jejuni

Chicken

Dysentry

Salmonella enteritidis

Meat & eggs

Diarrhoea

Enteroinvasive E. coli

Dysentry

Shigella sp

Dysentry

F/O

Food/water


Enteroinvasive Viral/Protozoa

Transmitted by water

Watery diarrhoea

Protozoa

Giardia

Cryptosporidia

Entamoeba histolytica (dys)

Virus

RotaV

SRSV

Norwalk

Organisms transmitted by food/water, but without GI symptoms


Listeria

Most foods

*Cheese

Septicaemia

Meningitis

Salmonella typhi

Water-sp

Systemic illness

Some GI symptoms

Polio

Water-sp

Neuro Sys

Hepatitis A

Water

Reaction/Tx

LHA

Prevention

Adequate food preparation

Safe portable water supplies

Good sewage disposal

Vaccination - polio/typhoid

Small Bowel Tumours

Benign
Lipoma
Leiomyoma
Adenoma

Malignant
Carcinoid tumours
Carcinoma
Lymphoma
Sarcoma (GI stromal tumours)


Carcinoid Tumours
Neuroendocrine cells
Secrete gastrin/5HT/somatostatin/VIP/Insulin/other endocrine things

Site
Malignancy depends on site of origin
Appendix/Rectum
Locally invasive
Resectable
SmI/St/Col
Highly invasive
Metastatic

NB - also occur in lungs

Symptoms and Cx - Carcinoid Syndrome
Flushing
Diarrhoea
Wheezing
Abdo fibrosis
Cardiac valve fibrosis/stenosis/regurgitation

If metastasises to liver:
5HT bypasses portal system = CS
 - otherwise metab in first past metabolism
NB therefore lung tumours drain directly into system = CS


Gastrointestinal Stromal Tumours
*Stomach/SmI
SmM/neural differentiation
Ben/Malig
Malignant = higher mitotic rate/necrosis/neural differentiation


Gastrointestinal Tract Lymphomas
*GIT extranodal lymphoma

1) MALT lymphoma
2) Enteropathy-associated lymphomas
3) Other B Cell lymphomas (mantle cell Lys & follicular Lys)

1) MALT
B Cell neoplasm
Fr mucosa associated lymphoid tissue
*SmI & Col - Tx difficult
St with H.Py
H.Py = chronic inflammation = chronic inflammatory cell infiltrate
Tx of H.Py can Tx Lymphoma (therefore ABs)

2) Enteropathy-associated Lymphomas
eg a/w/ Coeliac disease
* T cell

Ulcerative Colitis

Pancolitis

Starts at rectum

Retrograde into colon

Continuous

x Skip lesions

10% Backwash ileitis (mild mucosal inflamm of distal ileum)

Epidemiology
*White
*20-25

Symptoms
Worse Pn than Crohn's
Acute appendicitis
Diarrhoea - Haem/Mel/Mucus
Toxic megacolon (toxin damage of Nplexus and MPp = shutdown of neuromuscular function = distension & gangerene. W/o intervention = perforation & sepsis)

Pathology
Limited to mucosa
Pseudopolyps (extensive superficial ulceration = small islands of residual mucosa resembling polyps)
x transmural
x serosal inflammation
x fat wrapping
x fissuring
x fistulae
x thickening

Micro
Diffuse mucosal ulceration
Cryptitis (lim to muc)
Crypt abscesses (lim to muc)
Crypt distortion (long-standing inflammation)
x Granulomas

Cx
Increased risk - dysplasia & colonic carcinoma
Up risk with time
10yrs = x20R
35yrs = 30%

Prevention
10yrly colonoscopies (NB if init BP -ve  or dysp = lower risk of Ca)

Crohn's Disease

Epidemiology
*White
*20-30yrs (any age)

RFx
SMOKING!!!

Symptoms
Insid O/S
Abdo pain
Diarr
WL
Perianal disease
Skin tags
Fisulae
Fissures
Abscesses
Appendicitis

Pathology
Sharply delimited & transmural
Mucosal damage & inflammation
Non-caeseating granulomas
Deep fissuring (rose thorn ulcers)
Cobblestoning (islands of residual mucosa surrounded by deep ulcers)
Fat wrapping of mesentery (serosal - the external surface of bowel - inflammation)
Adhesions to another bowel loop/bladder/uterus

Fistulas (fissuring ulcers extend between serosa & bowel loop/bladder/uterus) =
Pneumouria/faecesuria/air or faeces per vagina

Transmural inflamm of muscPp (main muscle coat of bowel) =
Neuromusc Hplasia & thickening of bowel wall =
Stricture =
Sx Tx

Ix
Micro
Full thickness ulceration w/ granuloma formation (50% biopsies)
Lp inflammation & cryptitis (containing Nps)
Crypt abscesses (Nps destroying crypts)

Cx
Increases ColCa x5/6

Ischaemic Bowel Disease

Any part of bowel

Causes

• Atheroma
• Emboli
• Thrombosis
• Vasculitis
• Radiation

Diverticular Disease

Diverticulosis
Outpouchings of mucosa through bowel wall
Diverticulitis
Inflamed diverticula
Diverticular Disease
Symptomatic diverticulosis
Solitary & multiple diverticula poss throughout colon/smI
*sigmoid colon

Epidemiology
Affects Western, elderly population
>60% >80
Rare in Asia & Africa

Pathology
60% only in sigmoid
Never rectum
SmI diverticula
*duodenum
Incidental
Rarely Sy

NB
Diverticulosis - if Right-sided colon = ?different disease (*younger; oriental)

Pathogenesis
Fibre diet has inverse relationship with disease
Fibre = large 'residue' in bowel
Muscularis propria thickened (raised intraluminal pressure with empty colon)

Cx
Diverticulitis (leads to perforation/Abscesses)
Fistulae (*colovesical; ut/vaginal/abdo wall)
Intestinal obstruction fr inflammatory mass
Haemorrhage - diverticula *form where arteries enter muscPp

Inflammatory Bowel Disease

Chronic relapsing inflammation of the GIT with unknown origins

2 Types

• Crohn's
• UC

Crohn's

Granulomatous disease

Can affect any part of GI tract

*smI & colon

Ulcerative Colitis

Nongranulomatous disease

Limited to colon

Both have extra intestinal inflammatory sites

Eyes
(uveitis/episcleritis/conjunctivitis)
Joints
(Monoarticular arthritis/Ank Spond/Sacroileitis)
Skin
(Erythema nodosum/vasculitis/pyoderma gangrenosum)
Liver
(fatty change/sclerosing cholangitis/hepatitis/cirrhosis)
Calculi
(higher incidence of calculi - urinary tract & GB)

Aetiology
?cause
Suggested mechanisms:
1) Inflammatory reactions
Mycobacterium
Measles
Viruses
2) Autoimmune
3) Multifocal infarction of GIT

Comparison of the Crohn's and UC

Crohn's

Mouth 2 anus

Skip lesions

Thickened bowel wall

Transmural inflammation

Granulomas

Deep fissuring ulcers

Fistula form

Cancer rare
Non-surveillance

Ulcerative Colitis

Colon only

Continuous lesion

Normal BW thickness

Mucosal inflammation

x Granulomas

Pseudopolyps

x Fistula formation

Cancer common
Surveillance

Tropical Sprue & Whipple's Disease

Tropical Sprue
Coeliac-like disease
Tropics

Aetiology
?Spec cause
Poss bacterial overgrowth (eg Ecolab/Haemophilic)
Small intestine (entire)

Presentation/CFx
*folate/B12 deficiency - pernicious anaemia

Ix
Micro
Variable

Tx
B-Sp ABs

Cx
x a/w/ T Cell lymphoma


Whipple's Disease
Rare
Systemic
*Intestine/CNS/Joints

Epidemiology
*White
M:F=10:1
*30-40yrs


Aetiology
G+ve Actinomycete - Tropheryma whippelii

Ix
Biopsy/Micro
PAS +ve macrophages within LProp =
SmI distension
E- Micro
Macrophages contain rod shaped bacilli

Coeliac Disease

Pathology affects mucosa of small intestine
Impaired nutrient absorption
Improves on withdrawal of wheat gliadins & related grain proteins from diet

3 Features

1. Small Intestine
2. Malabsorption
3. Iimproves with removal of wheat gliadins from diet.

Aetiology
*Caucasian (Irish = 1/100)
Genetic: HLA DQw2 locus
Imm-med I inj with malab

Ix
Serum
Antiendomysial antibodies
Antigliadins
Biopsy
Confirm positive aBs
Macro
Mucosa smooth
Changes prominent in proximal > distal sm I
Micro
Flattening of villi (villous atrophy)
Lengthening of crypts
Resembles colonic mucosa
Intraepithelial lymphocytosis (*cytotox T cells & up nos. of T helper cells within Lprop)

Tx
Remove wheat gliadins & related grain proteins from diet
Confirm Dx
 - 2nd biopsy post diet (n villous architecture)/gluten challenge
 - decrease in symptoms to confirm

Cx
Up risk - T cell lymphoma
Malignancies of GI Tract & Breast

Malabsorption

Malabsorption

Suboptimal absorption

Fats

Vitamins

Proteins

Carbohydrates

Electrolytes

Minerals

Water

Symptoms

Abdo pain

Bloating
Flatus
Diarrhoea
Steatorrhea
WL
Failure to thrive
Vitamin & metal deficiencies

Pathology
1) xIntraluminal digestion (digestion enzyme dysfunction)
2) xTerminal digestion (luminal brush border dysf)
3) xTransepithelial transport (epith transp dysf)

Causes
Coeliac D (coeliac sprue)
Tropical sprue
Lactose intolerance
Pancreatic insufficiency (CF/ChrPi in alcoholics)
Whipple's disease
Parasitic infection (eg G lam)
Sx (short gut syndrome, gastrectomy)
Crohn's disease

Infectious Enterocolitis

Inflammation of small/large bowel
Bact/Para/Prot/Viral

Bacterial

Pathology

1) Ingestion of toxin (Staph aureus, Clostridium perfringens)

2) Enterotoxic organisms - ingestion of organism and toxin secreted within lumen = mucosal damage (Vibrio cholera, E coli, Cl difficile)
3) Enteroinvasive organisms - ingestion of organism, which invades and damages mucosa (Salmonella, Shigella, some E coli)

 - Clostridium Difficile
Normal commensal of gut

1) Common - Antibiotic-associated colitis

Broad-spectrum ABs =
Overgrowth =
ClDiff acts as a pathogen

2) Rarer - w/o ABs
Post-surgery/Chronic illness

Ix
Macro
< inflammatory exudate

 Adheres to surface mucosa

= Pseudomembrane

Micro

< inflammation of mucosa
Neutrophils erupting from crypts - 'volcano appearance'

Parasitic & Protozoal Enterocolitis

 - Round worms
Strongyloides
Ascaris
Hookworms

 - Flatworms

Tapeworms

Flukes

 - Protozoa (seen on H&E)

Entamoeba Histolytica

Giardia lamblia

 - Entamoeba Histolytica

Faecal/Oral
Amoebas burrow into crypts
Colonic mucosa through muscularis mucosa =
Acute Dysentry

Macro

Numerous ulcers in colonic mucosa
Micro
Penetration of muscularis mucosa
Spreading out
Narrow neck
Broad base
Flask-shaped ulcers
Approx 4% of patients, parasites penetrate portal vessels
Spread haematogenously to liver
= Hepatic abscesses

*Solitary

 Up to 10cm diameter

Other abscess sites:

Lungs

Heart

Renal

Brain

Remain long after dysentry gone
Hard to Tx

 - Giardia Lamblia
Spreads via water
Attaches to small intestine mucosa
x Invasion

Pathology
Normal to blunting villi
Mixed inflamm infiltrate of Lpropria
= Malabsorptive diarrhoea


Viral Enterocolitis

Young Pts
Rotavirus

Adults
Norwalk virus (winter vomiting virus)

Immunocompromised
CMV
Acquired Immunodeficiency Virus
 - 30-60% HIV Pts = diarrhoeal illness
 - Exclusion of other pathogens
 - ?unk path/HIV

Tumours of Exocrine Pancreas

Benig/Mal/Solid/Cyst
>80% Ductal Adenocarcinoma

Ductal Adenocarcinoma
From dysplastic ductal epithelium
10/100 000
*5th cancer mortality in Western Society

Cystic
<5% pancreatic tumours
Cystadenoma (Ben)
Cystadenocarcinoma (Malig)
Dx by micro

Symptoms
*Very late
Insidiously, non-specific O/S
Abdo pain
WL
Anorexia
Vomiting
>50% develop jaundice, h/e, <20% J at Pn
Migratory thrombophlebitis (Trousseau's Sign)
 - w/ appearing/disappearing Tbs in 10%
Painless distended GB (Courvoisier's Sign)

Aetiology
Unknown
A/w/ smoking with chronic pancreatitis and DM
Inconsistent a/w/ alcohol & Hlipid
Familial clustering

Pathology
Head (60%)
Body (15%)
Tail (5%)
Diffuse (20%)

Gritty/hard/pale masses
Poorly defined infiltrating edges
Invades adjacent structures

Micro
*poorly-moderately differentiated adenoCa
Infiltrating pattern of cell clusters
10% adenosquamous/giant cell/sarcomatoid type

Cx
*Very late with pain 2ry to nerve compression/invasion
Invasion = x Cure

Mets
Local
Peritoneum
Spine
Liver
Distant
Lungs
Bone

*Death w/i 18months
<15% resectable at Dx
Sx = palliative

Pseudocysts & Pancreatic Abscesses

Pseudocysts
Localised collection of secretions
After pancreatic inflammation/trauma

Pancreatic abscesses
Sterile
Filled with liquefactive necrosis of pancreas

Both: lack epithelial lining of true cysts

Symptoms
Abdominal pain

DDx
?malignancy

Pseudocysts

Pathology
Solitary lesions (up to 10cm in diam)
Within/adj to pancreas
Secretion from damaged ducts walled off by fibrotic tissue =
Fluid-filled pseudocysts
*Calcified = Seen on CT


Cx
Haemorrhage
Infection w/ peritonitis

Chronic Pancreatitis

"sustained inflammation of the pancreas associated with irreversible morphological changes & loss of function"

Epidemiology
10/100 000 Western population
50s

Presentation
Abdominal pain
Maldigestion
DM
Jaundice

Aetiology
40% unknown
Chronic alcoholism
Hupercalcaemia
Huperlipidaemia
CF
Biliary disease
Genetic Fx (rare AD hereditary pancreatitis) - predisposes to pancreatic cancer

Pathogenesis
Duct plugging from abnormal secretion/repeated inflammation & repair fibrosis =
Gland distortion =
Increased risk pancreatitis
(necrosis-fibrosis-cycle)
*stimulus remains (e.g. alcoholism)

Pathology
Pancreas enlarged
Septal fibrosis
Intra-P fat necrosis
Advanced Disease
Shrunken, firm pancreas
Extensive fibrosis
Calcification
Variable dilatation of Pc ducts
Pseudocysts common

Micro Fx
Fibrosis
Loss of exocrine glands
Chronic inflammation (lymphocytes/plasma cells & monocytes)

Cx
70% Local
Biliary/duodenal/colonic obstruction
Pseudocysts
Haemorrhage
Pancreatic ascites
Gastric varices

*2ry DM (islet damage)
*Malabsorption (acinar damage)

Increased risk of pancreatic carcinoma (confounded by alcohol & smoking)

Cholecystitis

Inflammation of the gallbladder
Acute/chronic/acute on chronic
Almost always from calculous cholecystitis (gallstones)
H/e - acalculous cholecystitis


Acute


Aetiology
90% gallstones - neck of GB/cystic duct obstruction
Acute acalculous - severely ill pt (Post major (non-biliary) surgery/Trauma/Burns/Organ failure/Sepsis/Postpartum)


Symptoms

Mild/transient/surg emerg

RUQ pain

Fever

Nausea

Vomiting

Less * = Jaundice

Pathology
Mucosal defences imbalanced with effects of bile salts
Detergent bile salts damage mucosal surface = inflammation
Initially - no infection - may supervene
Calculous
Stone impaction = bile stasis = increased intraluminal pressure = GB distention = decreased blood flow to mucosa = mucosal defences damaged
Acalculous
Ischaemic mucosal damage


Ix

Increased bilirubin = common bile duct obstruction

Increased WCC

Increased liver enzymes ('cholestatic')

GB: enlarged/tense/mottled
Wall: thickened/oedema/hyperaemic or green/black (gangrenous chol.)
*Gallstones
Pus
Mucosal perforation
Micro
Non-specific acute inflammation
Oedema
Vascular congestion
Neutrophils
Abscess formation
Necrosis

Tx
Spontaneously resolves: 1-10 days
* Recurrence
25% worsen - Sx
Acalculous
Insidious o/s
Possibly obscured by primary illness
Lack of diagnosis = fatal
Onset possible indolent in outpatients with chronic gallbladder ischaemia


Chronic Cholecystitis


*Asymptomatic


Aetiology
Recurr symptomatic acute episodes
90% Gallstones


Ix
Fibrotic serosa (dulled)
Wall: thickened/opaque/stiff
Mucosa: Well preserved
Variable subepithelial chronic inflammation and fibrosis
Diverticulas - with epithelium protruding through layers of GB wall (Rokitansky-Ashoff sinuses)

Cx
Cholangitis
Sepsis
Perf with abscess formation
Peritonitis
Cholecystenteric fistula
Gallstone ileus
Bacterial infection
Bile drainage to adjacent organs
...also - decompensation (of pre-existing disease - renal/hep/card/pulm)

IMP - Tx early & avoid Cx

Gallstones

Cholelithiasis
Accretion of bile constituents & mucus
Forms solid 'stones'
Within biliary tract

Epidemiology
20% western adults
Caucasian Male:Female  = 1:2
Up risk with age
>50% prevalence >80years

Symptoms & Signs
10-20% = Symptomatic
Depends upon location of stone
*RUQ pain
Severe/spasmodic (rhythmic contraction of gallbladder against the impacted stone aka biliary colic)

If in common bile duct
1) Cholestasis
2) Jaundice (conjugated hyperbilirubinaemia)
3) Pancreatitis
* Attack 30minutes after a bacon sandwich

Pathology
2 types of stone
 - cholesterol 80%
 - pigment 20%

Formed from:

• Bile supersaturation
• Gallbladder hypomobility
• Mucus hypersecretion

Cholesterol Stones
50% - 100% cholesterol monohydrate
Sm amounts of calcium salts +/- bilirubin

Aetiology
Obesity
Rapid weight loss
High oestrogen states (OCP, pregnancy)
FHx
Congenital errors of metabolism
Clofibrate Tx

Pigment Stones
Insoluble Ca salts of unconjugated bilirubin & inorganic molecules

Aetiology
Haemolysis
Severe ileal dysfunction
Bacterial contamination of biliary tract

Ix
Cholesterol Stones
Multiple
Sm, yellow surface
Lamellar cut surface
15% radio-opaque (if sufficient Ca carbonate)
Pigment
Multiple
Black, facetted surface
50-75% radio-opaque

Cx
*Cholecystitis - *indication for emergency cholecystectomy
Empyema
Perforation
Filstulae
Cholangitis
Cholestasis
Pancreatitis
Increased gallbladder carcinoma risk