MRCP revision battle 51.2: Hypothermia

Mild hypothermia is defined as a core body temperature less than 35C.
Severe hypothermia is core body temperature less than 28C


Signs of hypothermia include:

• bradycardia
• hypoventilation
• hypotension
• muscle stiffness
• fixed and dilated pupils

Metabolic acidosis is common and may predispose to pancreatitis.


ECG changes in hypothermia include:

• J waves
• long PR, QT and QRS
• under 28C increasing risk of VF

Primary hypothermia is hypothermia due to exposure.
Secondary hypothermia is hypothermia due to a medical illness, for example hypothyroidism or hypoglycaemia


Treatment is passive rewarming for mild to moderate hypothermia and core rewarming (eg peritoneal lavage) for severe hypothermia.


Next - dilated cardiomyopathy

MRCP revision battle 51.3: Dilated cardiomyopathy

Dilated cardiomyopathy is exactly what it says it is: a dilated heart.


It is associated with:

• alcohol excess
• hypertension
• coxsackie virus
• HIV
• doxorubicin
• haemochromatosis
• sarcoidosis

Possible presentations include fatigue, dyspnoea or AF.


Clinically there may be a displaced apex, S3 gallop, TR or MR


The ECG may show poor R wave progression


Treatment is as per heart failure.  ?cardiac transplant


Now on to coeliac disease...

MRCP revision battle 51.4: Coeliac's Disease

Coeliac disease is a T cell mediated autoimmune disease of the small bowel.

There is intolerance to prolamins (proteins found in wheat, barley, rye) which results in villous atrophy and malabsorption.

Gliadin (found in gluten) is a form of prolamin.


Presentation may be with:

• abdominal pain
• weight loss
• nausea and vomiting
• steatorrhoea
• bloating

Patients must eat gluten for 6 weeks prior to testing.

NICE recommends diagnosis is by tissue transglutaminase (TTG) antibodies (IgA)
Endomysial antibodies and anti gliadin antibodies may also be found but are not recommended by NICE.


Jejenal biopsy may show:

• villous atrophy
• crypt hypoplasia
• raised intraepithelial lymphocytes
• lamina propria infiltrates with lymphocytes

Associations with coelic disease include:

• dermatitis herpetiformis
• type 1 diabetes
• autoimmune hepatitis

Management is by careful diet.


Now on to some botulism...

MRCP revision battle 51.5: Botulism

Botulism is caused by clostridium botulinum (gram positive rod)

The toxin causes a descending flaccid paralysis by binding irreversibly to the presynaptic membranes of the neuromuscular junction, blocking acetylcholine release.


Signs include:

• flaccid paralysis
• dysarthria
• ptosis
• fixed/dilated pupils
• dry mouth
• respiratory arrest

Botulism may be caused by food or by wound infection.  Heroin users are at high risk.
All commericial canned food has to undergo a 'botulum cook' at 121C.
Honey can contain botulum and as a result it is not recommended to give honey to infants under 1 yr of age.


Botulism may cause a false positive tensilon result.


Treatment is with an antitoxin and ITU support.



Next up: hypertrophic cardiomyopathy

MRCP revision battle 51.6: Hypertrophic cardiomyopathy

Hypertrophic obstructive cardiomyopathy is a condition in which there is left ventricular outflow tract obstruction due to asymmetric septal hypertrophy.

It is inherited in an autosomal dominant fashion but 50% of cases are sporadic.
 
Presentation may be with:

• angina
• syncope
• shortness of breath
• sudden death
• palpitations

Signs include:

• jerky pulse
• ESM radiating to axilla
• increases with valsalva, decreases with squatting
• large a waves
• double apical beat
• ?MR
• ?split S2

On ECG look for:

• LVH
• TWI
• Q waves
• possibly AF

Associations include:

• WPW
• phaechromocytoma
• Friedreichs ataxia

Poorer prognosis if:

• younger
• family history of sudden death
• syncope

No correlation between degree of LVOT obstruction and prognosis.


On angio look for a difference between the LV and aortic systolic pressures - in a normal patient they should be the same, in HCOM aortic pressure will be lower than LV pressure.


Management is:

• avoid 
• nitrates
• ACE-i
• inotropes
• atropin
• give beta blockers
• ?amiodarone
• ?ICD
• ?surgery

Annual mortality is 2.5% in adults, 6% in children.



Lets finish on the smelly topic of bacterial vaginosis...

MRCP revision battle 51.7: Bacterial vaginosis

Bacterial vaginosis is caused by an overgrowth of bacteria.  Lactobacillus tend to predominate.


It produces a thin white-grey discharge with a fishy odour.
It is not normally itchy or sore.


Diagnosis is made by Amsel's criteria, which requires 3 of the following 4:

• thin grey-white discharge
• fishy odour on adding an alkali
• pH >4.5 (normal pH 3.8-4.2)
• clue cells on microscopy

Treatment is given to symptomatic ladies.
Options include metronidazole and clindamycin.

MRCP revision battle 50.1: Gangrene and Necrotising Fasciitis

A day dedicated to dermatology...


MRCP revision battle 50.1: Gangrene and necrotising fasciitis
MRCP revision battle 50.2: Acne Rosacea
MRCP revision battle 50.3: Seborrhoeic dermatitis
MRCP revision battle 50.4: Alopecia
MRCP revision battle 50.5: Discoid lupus erythematosus
MRCP revision battle 50.6: Bullous Pemphigoid and Pemphigus Vulgaris
MRCP revision battle 50.7: Erythrasma




MRCP revision battle 50.1: Gangrene and necrotising fasciitis


A couple of grim topics: gangrene and necrotising fasciitis.


Gangrene

Gangrene is death of tissue due to ischaemia.  It can be subdivided into:

• dry gangrene = ischaemia only
• wet gangrene = ischaemia plus infection

Management is surgical with debridement and IV antibiotics



Necrotising fasciitis 




Necrotising fasciitis is a rapidly spreading infection of the deep fascia.


Type 1 is caused by mixed anaerobes and aerobes, classically occuring in diabetes and post surgery
Type 2 is usually caused by streptococcus pyrogenes, which is a group A beta haemolytic strep.



Fournier's gangrene is a subtype of necrotising fasciitis which affects male genitalia/the perineal area.
Meleney's gangrene is a subtype of necrotising fasciitis which occurs after an operation


Treatment of necrotising fasciitis is with debridement.



Mortality is around 70%.



Onwards to acne rosacea...

MRCP revision battle 50.2: Acne Rosacea

Acne rosacea is a skin condition that affects the face.

The first sign is often facial flushing.


Other features of acne rosacea include:

• erythema of face
• papules/pustules
• telangiectasia
• eye involvement - eg dry eyes
• rarely rhinophyma 

The cause is not known although  demodex follicularum is hypothesised to be involved as it occurs in larger numbers on suffers of acne rosacea than the general population.  Overuse of topical steroids can cause a condition like acne rosacea.


If the condition is mild topical metronidazole is used.
In severer cases oral oxytetracycline is given.




On to some flakey skin....

MRCP revision battle 50.3: Seborrhoeic dermatitis

Seborrhoeic dermatitis, AKA seborrhoeic eczema, is a skin condition characterised by dandruff and sometimes dry, erythematous flakey skin.


It is associated with a fungus called Malassezia furfur (previously known as Pityrosporum ovale).  However, it is not contagious.


Treatment is with ketoconazole 2% shampoo and anti-fungal cream.


Severe forms of seborrhoeic dermatitis are associated with Parkinson's disease and HIV.

Complications include otitis externa and blepharitis.


Now for some hair loss...

MRCP revision battle 50.4: Alopecia

This is a topic close to many men's hearts - alopecia (=hair loss).

We will consider alopecia in 3 sections:

1. autoimmune
2. scarring
3. non-scarring

Alopecia areata

• Alopecia areata is thought to be an autoimmune condition in which there are 'patches' of hair loss.
• hair regrows in 50% of sufferers within a year
• hair regrows in 80-90% of sufferers eventually
• it is associated with:
• autoimmune thyroid disease
• pernicious anaemia
• addisons
• vitiligo
• nail dystrophy
• cataracts
• alopecia totalis refers to complete loss of all hair on scalp
• alopecia universalis refers to loss of all body hair

Scarring alopecia

Causes of scarring alopecia include:

• infections: TB, syphyllis
• radiotherapy
• sarcoidosis
• lichen planus

Non-scarring alopecia

Causes of non-scarring alopecia include:

• areata
• hypopituitarism
• hypo/hyper thyroidism
• pregnancy
• OCP
• carbimazole
• thiouracil
• lithium
• iron deficiency
• chronic illness

 Next up... some discoid lupus...

MRCP revision battle 50.5: Discoid lupus erythematosus

Discoid lupus erythematosus is a skin condition characterised by:

• erythematous plaques with 
• slight scaling with the 
• inside hypopigmented compared to the edge.  As they age they develop
• keratin plugs in the centre.

Young females tend to be most commonly affected.

The lesions are photosensitive and tend to occur on the face, neck and scalp.

It is associated with SLE but less than 5% of cases progress to SLE.


First line treatment is topical steroids.
Second line treatment is hydroxychloroquine.



Now to a pair of blistering diseases that have always confused me...

MRCP revision battle 50.6: Bullous Pemphigoid and Pemphigus Vulgaris

Personally I have always mixed up bullous pemphigoid and pemphigus vulgaris so I'm hoping by tackling them in the same battle the differences will become apparent...





Note that pemphigus vulgaris can be drug-induced, with culprits including penicillamine, captopril and cephalosporin.

Pemphigus vulgaris is more common in Ashkenazi Jews.


On to something that glows coral red under woods lamp...

MRCP revision battle 50.7: Erythrasma

Erythrasma is a skin condition characterised by asymptomatic initially pink patches that become brown as the skin sheds.


Erythrasma is caused by corynebacterium, a gram positive bacteria.


If a woods lamp is held near a patch of erythrasma the erythrasma glows coral red due to porphyrins released by the bacteria.


It is commonest amongst diabetics and the obese.


Treatment is with topical fusidic acid/clindamycin or oral erythromycin if extensive.

MRCP revision battle 49.1: Homocystinuria

Today is back to being a mixed set of battles with some familiar topics and some utterly unusual ones...


MRCP revision battle 49.1: Homocystinuria
MRCP revision battle 49.2: Nail-patella syndrome
MRCP revision battle 49.3: Golfers and Tennis Elbow
MRCP revision battle 49.4: Whipples disease
MRCP revision battle 49.5: Hypermagnesaemia
MRCP revision battle 49.6: Osteoporosis
MRCP revision battle 49.7: Osteogenesis Imperfecta




MRCP revision battle 49.1: Homocystinuria


Homocystinuria is an autosomally recessive inherited condition in which there is decreased activity of cystathionine beta synthase resulting in accumulation of homocysteine and methionine which interferes with collagen cross-linkage.


Features include:

• Marfanoid body habitus
• spontaneous retinal detachment
• downwards lens dislocation
• osteoporosis
• venous and arterial thrombosis
• low IQ

Diagnosis is with the cyanide nitroprusside test which detects elevated urinary homocysteine.


Treatment is:

• methionine restriction
• cysteine supplements
• pyridoxine supplements

Now to nail-patella syndrome...

MRCP revision battle 49.2: Nail-patella syndrome

Nail-patella syndrome occurs as a possible answer in a fair few MRCP questions so an insight into it is helpful...


Nail-patella syndrome affects 1 in 50,000

It is characterised by:

• nail abnormalities, classically loss of the ulnar half of the thumb nail
• small patella
• boney spines over posterior iliac crests (70%)
• renal abnormalities (30-50%)
• over-extension of joints

On to some elbows...

MRCP revision battle 49.3: Golfers and Tennis Elbow

Golfer's Elbow

• medial epicondylitis
• pain worse on pronating forearm/ wrist flexion
• may have an assocaited ulnar neuropathy

Tennis Elbow

• lateral epicondylitis
• pain worse on elbow/wrist extension
• usually lasts 6 to 12 weeks
• commoner than Golfer's elbow

Both are treated with:

• ice
• NSAIDs
• ? corticosteroid injection
• ?physio

Back to the world of esoteric medicine...

MRCP revision battle 49.4: Whipples disease

Whipple's disease is a rare cause of GI malabsorption.  It is caused by a gram positive bacterium Tropheryma whippelei.


Presentation is:

• weight loss
• arthralgia
• diarrhoea
• malaise
• fever

Other symptoms may occur in relation to the malabsorption, for example signs of hypocalcaemia or vitamin C deficiency.


Possible clinical signs include:
• lymphadenopathy
• perioral hyperpigmentation


Cardiac involvement can result in an endocarditis, and CNS involvement can cause a reversible dementia.


It is commonest in males (9:1) and is associated with HLA B27.


Treatment is 2 weeks IV ceftriaxone then oral co-trimoxazole for one year.


Now for some excess magnesium...

MRCP revision battle 49.5: Hypermagnesaemia

Hypermagnesaemia generally becomes clinically relevant above 4mmol


Symptoms and signs include:

• lethargy
• nausea
• areflexia
• hypotension
• heart block
• bradycardia
• double vision

Treatment is with IV calcium.


Next up: osteoporosis

MRCP revision battle 49.6: Osteoporosis

Osteoporosis literally means 'porous bone'.


It is diagnosed by DEXA scan:

• T to -1 SD = normal
• T -1 to -2.5 SD = osteopenia
• T below -2.5  SD = osteoporosis

Fracture risk is increased by 2x with each standard deviation of the T score.

Common fracture sites include:

• vertebrae and neck of femur = trabecular bone
• distal radius and humerus = cortical bone

Factors increasing the risk of OP include:

• being slim
• smoker
• alcoholic
• inactive
• old
• steroid use
• early menopause
• thyrotoxicosis
• primary biliary sclerosis
• cushings

Primary prevention of OP is with alendronate.



Now to the final battle of the day, osteogenesis imperfecta

MRCP revision battle 49.7: Osteogenesis Imperfecta

Osteogenesis Imperfecta is an autosomal dominant inherited condition in which there is abnormal and or insufficient type 1 collagen synthesis.


This results in:

• bone fractures
• blue sclerea
• deafness secondary to otosclerosis
• muscle weakness

The photo below demonstrates the classical blue sclera  (from wiki commons, posted by Dr Fred)

There is no cure, so treatment is trying to prevent bone fractures.