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1

Lady with falciparum.  Other than doxycycline, what other abx would you give her?  

 

Options:

Chloroquine

Quinine

Proguanil

Pyrimethamine

Quinine

Non-artemisinin based combinations: Quinine plus tetracycline/doxycycline

Feature of severe malaria

  • schizonts on a blood film
  • parasitaemia > 2%
  • hypoglycaemia
  • acidosis
  • temperature > 39 °C
  • severe anaemia
  • complications:
    • cerebral malaria: seizures, coma
    • acute renal failure: blackwater fever, secondary to intravascular haemolysis, mechanism unknown
    • acute respiratory distress syndrome (ARDS)
    • hypoglycaemia
    • disseminated intravascular coagulation (DIC)

Uncomplicated falciparum malaria

  • strains resistant to chloroquine are prevalent in certain areas of Asia and Africa
  • the 2010 WHO guidelines recommend artemisinin-based combination therapies (ACTs) as first-line therapy
  • examples include artemether plus lumefantrine, artesunate plus amodiaquine, artesunate plus mefloquine, artesunate plus sulfadoxine-pyrimethamine, dihydroartemisinin plus piperaquine

Severe falciparum malaria

  • a parasite counts of more than 2% will usually need parenteral treatment irrespective of clinical state
  • intravenous artesunate is now recommended by WHO in preference to intravenous quinine
  • if parasite count > 10% then exchange transfusion should be considered
  • shock may indicate coexistent bacterial septicaemia - malaria rarely causes haemodynamic collapse

2

Which of the DMARDs cause retinopathy?  

Options

Hydroxychloroquine

Prednisolone

Sulfasalazine

Leflunomide

Hydroxychloroquine

Rheumatoid arthritis drug side effects

  • Hydroxychloroquine
    • Retinopathy 
    • Corneal deposits
  • Methotrexate
    • Myelosuppression
    • Liver cirrhosis
    • Pneumonitis
  • Sulfasalazine
    • Rashes
    • Oligospermia
    • Heinz body anaemia
    • Interstitial lung disease
  • Leflunomide
    • Liver impairment
    • Interstitial lung disease
    • Hypertension
  • Prednisolone
    • Cushingoid features
    • Osteoporosis
    • Impaired glucose tolerance
    • Hypertension
    • Cataracts
  • Gold
    • Proteinuria
  • Penicillamine
    • Proteinuria
    • Exacerbation of myasthenia gravis
  • Etanercept
    • Demyelination
    • Reactivation of tuberculosis
  • Infliximab
    • Reactivation of tuberculosis
  • Adalimumab
    • Reactivation of tuberculosis
  • Rituximab
    • Infusion reactions are common
  • NSAIDs (e.g. naproxen, ibuprofen)
    • Bronchospasm in asthmatics
    • Dyspepsia/peptic ulceration

3

Which of the DMARDs cause azoospermia and bone marrow suppression?  

Options

Sulfasalazine

Azathioprine

Methotrexate

Leflunomide

Sulfasalazine

  • Hydroxychloroquine
    • Retinopathy 
    • Corneal deposits
  • Methotrexate
    • Myelosuppression
    • Liver cirrhosis
    • Pneumonitis
  • Sulfasalazine
    • Rashes
    • Oligospermia
    • Heinz body anaemia
    • Interstitial lung disease
  • Leflunomide
    • Liver impairment
    • Interstitial lung disease
    • Hypertension
  • Prednisolone
    • Cushingoid features
    • Osteoporosis
    • Impaired glucose tolerance
    • Hypertension
    • Cataracts
  • Gold
    • Proteinuria
  • Penicillamine
    • Proteinuria
    • Exacerbation of myasthenia gravis
  • Etanercept
    • Demyelination
    • Reactivation of tuberculosis
  • Infliximab
    • Reactivation of tuberculosis
  • Adalimumab
    • Reactivation of tuberculosis
  • Rituximab
    • Infusion reactions are common
  • NSAIDs (e.g. naproxen, ibuprofen)
    • Bronchospasm in asthmatics
    • Dyspepsia/peptic ulceration

4

Lady with tremor, weight loss, proptosis and exophthalmos.  Has ophthalmoplegia.  What is likely cause?  

 

Options: 

Rectus muscle thickening

Retrobulbar tumour

Cavernous sinus syndrome

Rectus muscle thickening 

as seen in thyroid eye disease

Pathophysiology: it is thought to be caused by an autoimmune response against an autoantigen, possibly the TSH receptor → retro-orbital inflammation --> glycosaminoglycan and collagen deposition in the muscles

(frequently: inferior rectus > medial rectus  > superior rectus) sparing their tendinous insertions, and is usually bilateral and symmetrical. 

Features

  • the patient may be eu-, hypo- or hyperthyroid at the time of presentation
  • exophthalmos
  • conjunctival oedema
  • optic disc swelling
  • ophthalmoplegia
  • inability to close the eye lids may lead to sore, dry eyes. If severe and untreated patients can be at risk of exposure keratopathy

Prevention

  • smoking is the most important modifiable risk factor for the development of thyroid eye disease
  • radioiodine treatment may increase the inflammatory symptoms seen in thyroid eye disease. In a recent study of patients with Graves' disease around 15% developed, or had worsening of, eye disease. Prednisolone may help reduce the risk

Management

  • topical lubricants may be needed to help prevent corneal inflammation caused by exposure
  • steroids
  • radiotherapy
  • surgery

5

69 year old guy with two previous TIAs and AF.  What do you start him on?  

 

Option

Warfarin

Aspirin

Clopidogrel

Aspirin + Modified release dipyridamol

 

Warfarin or NOAC (Dabigatran, Rivaroxaban, Apixaban)

 

nb Direct oral anticoagulants (direct thrombin/Xa inhibitors) are used in the prevention of stroke secondary to non-valvular AF 

Post ischaemic stroke prophylaxis:

  • clopidogrel is now recommended by NICE ahead of combination use of aspirin plus modified release (MR) dipyridamole in people who have had an ischaemic stroke
  • aspirin plus MR dipyridamole is now recommended after an ischaemic stroke only if clopidogrel is contraindicated or not tolerated, but treatment is no longer limited to 2 years' duration
  • MR dipyridamole alone is recommended after an ischaemic stroke only if aspirin or clopidogrel are contraindicated or not tolerated, again with no limit on duration of treatment
  • Stoke secondary to AF: 
    • following a stroke or TIA warfarin or a DOAC should be given as the anticoagulant of choice.
      • If valvular AF, Warfarin must be used
      • Aspirin/dipyridamole should only be given if needed for the treatment of other comorbidities
    • in acute stroke patients, in the absence of haemorrhage, anticoagulation therapy should be commenced after 2 weeks. If imaging shows a very large cerebral infarction then the initiation of anticoagulation should be delayed

6

Lady with hx of AF presents to A&E with fast AF, pulmonary oedema and peripheral oedema.  What do you give initially?  

 

Options:

Furosemide and warfarin

Furosemide and Ramipril

DC cardioversion (if an option) 

Cardioversion indicated in Peri-arrest rhythms: tachycardia

  • shock: hypotension (systolic blood pressure < 90 mmHg), pallor, sweating, cold, clammy extremities, confusion or impaired consciousness
  • syncope
  • myocardial ischaemia
  • heart failure

 

 

Furosemide and ?warfarin

nb.acute pulmonary edema may increase sensitivity to ACE inhibition or angiotensin blockade, including risks of hypotension and renal dysfunction

warfarin will initiall decrease the INR (increase risk of clotting) – it is often recommended that heparin should be administered in the ED if a AF patient is going to be admitted to hospital for expedited elective cardioversion (AF duration < 48 hours, or AF duration > 48 hours with a negative TEE study)

 

Management options in acute heart failure include:

  • oxygen
    • diuretics
    • opiates
    • vasodilators
    • inotropic agents
    • CPAP
    • ultrafiltration
    • mechanical circulatory assistance: e.g. intra-aortic balloon counterpulsation or ventricular assist devices

 

Drug management of heart failure: 

  1. first-line= ACE-inhibitor + beta-blocker
  2. second-line= aldosterone antagonist, angiotensin II receptor blocker or a hydralazine in combination with a nitrate
  3. third line= cardiac resynchronisation therapy or digoxin* or ivabradine (in pt on ACE-inhibitor, beta-blocker + aldosterone antagonist, with HR > 75/min and a left ventricular fraction < 35%)
  • diuretics should be given for fluid overload
  • offer annual influenza vaccine
  • offer one-off** pneumococcal vaccine

7

30 something man with joint pain - sacroilitis and distal interphalangeal joint pain?

 

Options:

Ank spond

Psoriatic arthritis

Rheumatoid arthritis.

Psoriatic arthropathy

correlates poorly with cutaneous psoriasis and often precedes the development of skin lesions. Around 10-20% percent of patients with skin lesions develop an arthropathy with males and females being equally affected

Types*

  • rheumatoid-like polyarthritis: (30-40%, most common type)
  • asymmetrical oligoarthritis: typically affects hands and feet (20-30%)
  • sacroilitis
  • DIP joint disease (10%)
  • arthritis mutilans (severe deformity fingers/hand, 'telescoping fingers')


Management

  • treat as rheumatoid arthritis
  • but better prognosis

 

nb ankylosing spondylitis: affects axial spine

8

Guy on prednisolone presents with darkened red reflex and problems with night vision.  What does he have?  

 

Cataracts

opacities in lens, cornea or vitreous results in loss of red reflex

Presentation

  • Increasing myopia
  • Blurred vision → gradual visual loss
  • Dazzling in sunshine / bright lights
  • Monocular diplopia

Cause

Majority

  • age related
  • UV light

Systemic

  • diabetes mellitus
  • steroids
  • infection (congenital rubella)
  • metabolic (hypocalcaemia, galactosaemia)
  • myotonic dystrophy
  • Down's syndrome

Ocular

  • trauma
  • uveitis
  • high myopia
  • topical steroids


Classification

  • Nuclear: change lens refractive index, common in old age
  • Polar: localized, commonly inherited, lie in the visual axis
  • Subcapsular: due to steroid use, just deep to the lens capsule, in the visual axis
  • Dot opacities: common in normal lenses, also seen in diabetes and myotonic dystrophy


 

9

Lady from some Pakistan or somewhere with HTN controlled on amlodipine and BP of 150/90 has retinal findings:  blot haemorrhages and yellow deposits (hard exudates I think) on macula.  What is this?

 

Options:

 Hypertensive retinopathy

Diabetic retinopathy

NOTE: Drusen seen in age related macular degeneration

 

Background diabetic retinopathy

  • microaneurysms (dots)
  • blot haemorrhages (<=3)
  • hard exudates

Pre-proliferative retinopathy

  • cotton wool spots (soft exudates; ischaemic nerve fibres)
  • > 3 blot haemorrhages
  • venous beading/looping
  • deep/dark cluster haemorrhages
  • more common in Type I DM, treat with laser photocoagulation

Proliferative retinopathy

  • retinal neovascularisation - may lead to vitrous haemorrhage
  • fibrous tissue forming anterior to retinal disc
  • more common in Type I DM, 50% blind in 5 years

Maculopathy

  • based on location rather than severity, anything is potentially serious
  • hard exudates and other 'background' changes on macula
  • check visual acuity
  • more common in Type II DM

 

 

 

10

Footballer inverts his ankle and presents with foot pain, surprised he has no ankle pain.  What does he have?  

 

Options: 

Fractured base of 5th metatarsal

Damaged ligaments

Fractured base of 5th metatarsal

is caused by forcible inversion of the foot in plantar flexion

 

Associated with Lisfranc injury

11

Guy jumps and lands on his knee (repeated question)

 

Options: 

medial meniscus tear

patella fracture

damage to ACL 

patella fracture

 

12

Pulmonary adenocarcinoma Ca met to liver via what route?  

 

Options: 

Transcoelomic

Direct invasion

Haem

Lymph

Heamatogenous 

Spread: 

  • local: to pleura, left recurrent laryngeal and phrenic nerve, pericardium, oesophagus, sympathetic chain, and brachial plexus
  • Lymphatic: to mediastinal and cervical nodes. Compression of the SVC by massive mediastinal node involvement produces gross oedema and cyanosis of the face and upper limbs
  • Blood: to bone, brain, liver and adrenals
  • Transcoelomic: pleural seeding and effusion

 

Cancers which matastasise heamatogenously = Folicular, Renal Cell Ca, Hepatocellular carcinoma

Hematogenous--Hepatoma
Spreading -- Sarcoma
Cancers --Choriocarcinoma
Reign -- Renal cell carcinoma
Foolishly -- Follicular carc. of the thyroid

 

13

Lady playing squash suddenly runs, hears a crack from behind ankle then pain whenever she tries to plantar flex.  What happened?  

 

options: 

Ruptured Achilles

Fractured talus

Fractured calcaneus 

Achilles tendon rupture

..suspected if the person describes the following whilst playing a sport or running; an audible 'pop' in the ankle, sudden onset significant pain in the calf or ankle or the inability to walk or continue the sport. 

An examination should be conducted using Simmond's triad, to help exclude Achilles tendon rupture. This can be performed by asking the patient to lie prone with their feet over the edge of the bed. The examiner should look for an abnormal angle of declination; Achilles tendon rupture may lead to greater dorsiflexion of the injured foot compared to the uninjured limb. They should also feel for a gap in the tendon and gently squeeze the calf muscles if there is an acute rupture of the Achilles tendon the injured foot will stay in the neutral position when the calf is squeezed.

An acute referral should be made to an orthopaedic specialist following a suspected rupture.

14

 

Middle aged lady presenting with atypical chest pain – what would be used to decide pre test probability she has ischaemic heart disease?  

 

Options

Epidemiological evidence of something

Case control study on atypical chest pain RFs

Clinical experience

Exercise ECG

Epidemiological evidence of something

Evidence based medicine works by deciding pre-test probability of something and then doing tests and changing the probability of that thing accordingly.

Pretest Probability

  • Definition
    • Pretest Probability is defined as the probability of a patient having the target disorder before a diagnostic test result is known. It represents the probability that a specific patient, say a middle-aged man, with a specific past history, say hypertension and cigarette smoking, who presents to a specific clinical setting, like Accident and Emergency, with a specific symptom complex, say retrosternal chest pressure, dyspnoea and diaphoresis, has a specific diagnosis, such as acute myocardial infarction.
  • Application
    • The pretest probability is especially useful for four things:
  1. interpreting the results of a diagnostic test,
  2. selecting one or more diagnostic tests
  3. choosing whether to start therapy:
    • * a) without further testing (treatment threshold);
    • * b) while awaiting further testing;
  4. deciding whether it’s worth testing at all (test threshold)
  • Calculation
    • The probability of the target disorder, usually abbreviated P(D+), can be calculated as the proportion of patients with the target disorder, out of all the patients with the symptoms(s), both those with and without the disorder:
      • P(D+) = D+ / (D+ + D-)
    • where D+ indicates the number of patients with target disorder, D- indicates the number of patients without target disorder, and P(D+) is the probability of the target disorder.
  • Example:
    • A group of investigators in North America studied the underlying diseases found in patients presenting to a primary care setting with persistent dizziness. They studied a total of 100 dizzy patients, finding 16 had dizziness from psychiatric conditions.
    • Thus, 16 / 100, or 16 per cent, is the estimate of of disease probability for psychiatric conditions from this study.
    • So, if this information proves valid and applicable to your practice, if IF one of your patients presents to a primary care setting with persistent dizziness, the P(D+) of a psychiatric cause might be estimated at 16 per cent.

15

Person with 4cm head of pancreas ca that has invaded mesenteric vessels.  How do you manage?  

 

options

ERCP and biliary stent

PTC drainage

Whipple’s

Vit k

Abx

ERCP and biliary stent

Management

  • less than 20% are suitable for surgery at diagnosis
  • Resectable:
    • a Whipple's resection (pancreaticoduodenectomy) is performed for resectable lesions in the head of pancreas.
      • Side-effects of a Whipple's include dumping syndrome and peptic ulcer disease
    • adjuvant chemotherapy is usually given following surgery
  • Palliation:
    • ERCP with stenting is often used for palliation

16

Son of 75 year old lady requests home visit for mother who has recent behavioural changes.  Sometimes gets confused and sees people in the room who aren’t there.  Recent loss of appetite.  Cause?

 

options: 

Lewy body dementia

Alzheimer’s

Acute Confusional State

Depression with psychosis

Acute confusional state

is also known as delirium or acute organic brain syndrome. It affects up to 30% of elderly patients admitted to hospital.

Features - wide variety of presentations

  • memory disturbances (loss of short term > long term)
  • may be very agitated or withdrawn
  • disorientation
  • mood change
  • visual hallucinations
  • disturbed sleep cycle
  • poor attention

 

 

(if not an option, potentially LBD)

17

Guy post-op with delirium and morphine epidural in situ, what is best initial management?  

 

Options:

Put in well lit side room

Haloperidol

Discontinue morphine epidural

Put in a well lit side room 

Management of Acute Confusional State

  • treatment of underlying cause
  • modification of environment
  • haloperidol 0.5 mg as the first-line sedative or olanzapine

18

Guy with short history of back pain and painless black lesions on his feet appear.  What ix do you do?  

 

Options: 

Arteriogram

CT abdo

CT abdo with contrast

 

Abdominal aortic aneurysms occur primarily as a result of the failure of elastic proteins within the extracellular matrix. Aneurysms typically represent dilation of all layers of the arterial wall. Most aneurysms are caused by degenerative disease. After the age of 50 years the normal diameter of the infrarenal aorta is 1.5cm in females and 1.7cm in males. Diameters of 3cm and greater, are considered aneurysmal. The pathophysiology involved in the development of aneurysms is complex and the primary event is loss of the intima with loss of elastic fibres from the media. This process is associated with, and potentiated by, increased proteolytic activity and lymphocytic infiltration. 
 

symptomatic

  • pain in the abdomen, loin or lower back
    • abdominal pain may spread to the back
    • require urgent referral for investigation and management because they may indicate impending rupture
  • patient may feel a pulsatile mass in the abdomen
  • features of limb ischemia - caused by distal embolisation
  • lower extremity oedema
    • rarely seen caused by compression of the inferior vena cava

Investigations

  • ultrasonography
    • non invasive, low cost method with the sensitivity and specificity close to 100%
    • measures the size of the aorta in longitudinal as well as in anteroposterior and transverse directions
    • modality of choice for initial assessment, follow up, screening and surveillance
    • should have a low threshold for arranging abdominal ultrasonography in patients at risk. 
  • CT
    • can visualise
      • the proximal neck (the transition between the normal and aneurysmal aorta)
      • any extensions of the aneurysm (to the iliac areries)
      • patency of visceral arteries
      • the presence of blood within the thrombus (crescent sign) - is considered a predictive marker of imminent rupture
    • helical CT and CT angiography can provide additional anatomical details - useful if an endovascular procedure is considered
  • resonance angiography (MRA)
    • safer than conventional arteriography since it does not use nephrotoxic contrast material
  • plain radiography
    • calcified aneurysmal wall may be seen in some cases

19

Guy presenting to GP with nocturia.  PSA 18, urinalysis trace blood and protein, urea and cr mildly elevated.  What do you do?  

 

Options: 

Refer routinely to uro

Refer urgently to uro

Refer routinely to renal

Refer urgently to renal

Refer urgently to uro

refer men using a suspected cancer pathway referral (for an appointment within 2 weeks) for prostate cancer if their PSA levels are above the age-specific reference range

Management Programme are as follows:

  • aged 50-59 >= 3.0 ng/ml;
  • aged 60-69 >= 4.0 ng/ml;
  • aged 70 and over > 5.0 ng/ml.
  • (Note that there are no age-specific reference ranges for men over 80 years. Nearly all men of this age have at least a focus of cancer in the prostate. Prostate cancer only needs to be diagnosed in this age group if it is likely to need palliative treatment.) 

exclude urinary infection before PSA testing. Postpone the PSA test for at least 1 month after treatment of a proven urinary infection (indicated by nitrite and leucocyte on urine dip)

20

?75 year old lady with 2.5cm firm breast lump not tethered to skin and no skin changes.  Daughter 40 year old just had benign breast cyst diagnosed.  What does the old lady have?  

 

Options: 

Ductal carcinoma

Breast cyst

Lobular carcinoma in situ

Ductal carcinoma

  • Age, solitary, firm lump – increases likelihood of cancer vs benign cyst. Ductal (70%) more common than lobullary, proliferative breast disease with atypia = risk factor

Breast cyst

  • 7% of all Western females will present with a breast cyst
  • Usually presents as a smooth discrete lump (may be fluctuant)
  • Small increased risk of breast cancer (especially if younger)

Commonest Single Breast Lumps

  • Fibroadenoma
  • Cyst
  • Fat necrosis
  • Cancer

Features of a Malignant Lump

  • Irregular, nodular surface
  • Poorly defined edge
  • Hard / scirrhous consistency
  • Painless
  • Fixation to skin or chest wall
  • Nipple involvement

In this patient AGE makes Ca more likely? 

21

45 year old lady with spontaneous dark brown nipple discharge. Examination reveals only one duct producing discharge. What does she have?  

 

options:

Intraductal papilloma

Duct ectasia

Intraductal papilloma

  • Commoner in younger patients
  • May cause blood stained discharge originating from a single duct
  • There is usually no palpable lump
  • NO risk of malignancy
  • Growth of papilloma in a single duct

Duct ectasia

  • Dilatation breast ducts.
  • Most common in menopausal women
  • Discharge typically thick and green in colour, which may be from single or multiple ducts (usually present age >50 years)
  • Most common in smokers

22

Tall 28 year old man with radiofemoral delay and hypertension. BP 210/110.  Has rib notching.  What is underlying diagnosis?  

 

Options:

Coarctation

Marfan’s

ED

Coarctation of the aorta

...describes a congenital narrowing of the descending aorta

more common in males (despite association with Turner's syndrome)
Features:

  • infancy: heart failure
  • adult: hypertension
  • radio-femoral delay
  • mid systolic murmur, maximal over back
  • apical click from the aortic valve
  • notching of the inferior border of the ribs (due to collateral vessels) is not seen in young children


Associations

  • Turner's syndrome
  • bicuspid aortic valve
  • berry aneurysms
  • neurofibromatosis

 

Marfans is associated with the following cardiac abnormalities: 

  • Aortic aneurysm and dissection
  • Aortic root dilatation → regurgitation
  • MV prolapse ± regurgitation

23

The post MI papillary muscle rupture question (repeated again)

Revision Qs: 

How will this present? 

List the complications of an MI

Acute mitral regurgitation

More common with infero-posterior infarction and may be due to ischaemia or rupture of the papillary muscle. An early-to-mid systolic murmur is typically heard and late systolic click. Patients are treated with vasodilator therapy but often require emergency surgical repair.

 

Death Passing PRAED st.

  • Death: VF, LVF, CVA
  • Pump Failure
  • Pericarditis
  • Rupture: myomalacia cordis
    • Cardiac tamponade
    • Papillary muscle / chordae → MR
    • Septum
  • Arrhythmias
    • Tachycardias
      • SVT
      • Ventricular T/F
    • Bradycardia
      • sinus brady 
      • AV block 
      • Ventricular brady 
  • Aneurysm: ventricular
  • Dressler’s Syndrome: pleuro-pericarditis

24

Young guy vomiting after a night out presents with chest and epigastric pain, left sided pleural effusion, subcutaneous emphysema.  What happened?  

 

 

Oesophageal rupture

Boerhaave syndrome: Severe vomiting → oesophageal rupture

Defined as: Complete disruption of the oesophageal wall in absence of per-existing pathology. Left postero-lateral oesophageal is commonest site (2-3cm from OG junction). Suspect in patients with severe chest pain without cardiac diagnosis and signs suggestive of pneumonia without convincing history, where there is history of vomiting. Erect CXR shows infiltrate or effusion in 90% of cases

Other: 

Mallory-Weiss syndrome: Severe vomiting → painful mucousal lacerations at the gastroesophageal junction resulting in haematemesis. Common in alcoholics

Plummer-Vinson syndrome: Triad of: dysphagia (secondary to oesophageal webs) + glossitis + iron-deficiency anaemia. Treatment includes iron supplementation and dilation of the webs

25

Sickle cell girl with severe back pain (?) What do you do first?

 

Options: 

Exchange transfusion

Epidural anaesthesia

Paracetamol/ibuprofen

Fluid bolus

Fluid bolus

 

Sickle cell anaemia is characterised by periods of good health with intervening crises
Four main types of crises are recognised:

  1. Thrombotic crises/Vaso-occlusive ‘painful’ crisis: 
    • precipitated by infection, dehydration, deoxygenation
    • infarcts occur in various organs including the bones (e.g. avascular necrosis of hip, hand-foot syndrome in children, lungs, spleen and brain
    • may cause mesenteric ischaemia, mimicking an acute abdomen
  2. Sequestration crises
    • sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia
    • acute chest syndrome: dyspnoea, chest pain, pulmonary infiltrates, low pO2 - the most common cause of death after childhood
  3. Aplastic crises
    • caused by infection with parvovirus
    • sudden fall in haemoglobin
  4. Haemolytic crises
    • rare
    • fall in haemoglobin due an increased rate of haemolysis

 

Give prompt, generous analgesia, eg IV opiates (see p576).
• Crossmatch blood. FBC, reticulocytes, blood cultures, MSU ± CXR if T° or chest signs.
• Rehydrate with IVI and keep warm. Give O2 by mask if Pa O2 or O2 sats <95%.
• ‘Blind’ antibiotics (eg cephalosporin, p382) if T° >38°, unwell, or chest signs.
• Measure PCV, reticulocytes, liver, and spleen size twice daily.
• Give blood transfusion if Hb or reticulocytes fall sharply. Match blood for the blood group antigens Rh(C, D, E) and Kell, to prevent alloantibody formation. This helps oxygenation, and is as good as exchange transfusion (reserved for those who are rapidly worsening: it is a process where blood is removed and donor blood is given in stages). 40 Indications: severe chest crisis, suspected CNS event or multiorgan failure—when the proportion of HbS should be reduced to <30%.

 

Mx Acute Crises

  • General
    • Analgesia: opioids IV
    • Good hydration
    • O2
    • Keep warm
  • Ix
    • FBC, U+E, reticulocytes, cultures
    • Urine dip
    • CXR
  • Rx
    • Blind Abx: e.g. ceftriaxone
    • Transfusion: exchange if severe

26

Old man with smear cells. Cause?

Options

CLL

Hodgkin’s

NHL

CLL

Chronic lymphocytic leukaemia is caused by a monoclonal proliferation of well-differentiated lymphocytes which are almost always B-cells (99%)

Features

  • often none
  • constitutional: anorexia, weight loss
  • bleeding, infections
  • lymphadenopathy more marked than CML

Complications

  • hypogammaglobulinaemia leading to recurrent infections
  • warm autoimmune haemolytic anaemia in 10-15% of patients
  • transformation to high-grade lymphoma (Richter's transformation)

Investigations

  • blood film: smudge cells (also known as smear cells)
  • immunophenotyping

27

Guy on ibuprofen started recently for some pain. Comes in with two episodes of vomiting bright red blood. No further episodes. Stable obs, low Hb. What do you do?

Options

Observe for 24 hours and discharge with omeprazole

OGD within 2 hours

OGD on next endoscopy list

OGD on next endoscopy list (<24hrs) 

Ideally all patients admitted with upper gastrointestinal haemorrhage should undergo Upper GI endoscopy within 24 hours of admission. In those who are unstable this should occur immediately after resuscitation or in tandem with it. The endoscopy department is a potentially dangerous place for unstable patients and it may be safer to perform the endoscopy in theatre with an anaesthetist present.

 

Management of GI bleed

ABCDE: CIRCULATION!

  • Resuscitate:
    • Head down
    • Airway: Early control of airway is vital (e.g. Drowsy patient with liver failure) -> protect the airway
    • Breathing: 100% O2
    • ​C 2x 14G cannulae: 
      • Bloods: cross match blood, check FBC, LFTs (ETOH abuse), U+E (protein meal) and Clotting and ABG (lactate + Hb) and glucose
      • IV crystalloid infusion up to 1L (avoid dilution)
    • D: blood glucose, GCS, Temperature, expose (PR; maleana)
  • Major H'gge protocol: on-going bleeding and haemodynamic instability are likely to require O negative blood pending cross matched blood 
    • ​keep Hb >10
  • Upper GI endoscopy (scoring systems) 
    • all within 24 hours of admission.
    • If unstable = immediately after resuscitation or in tandem with it.

28

Lady collapses on hospital ward. Had co-amox in A&E earlier and reports feeling breathless before collapse, 2 hours later is slightly tachy, slightly hypotensive, resp rate 34, sats 94% on 10L o2.  What do you give first?

Options:

 Adrenaline

Chlorephenamine

Steroids

IV fluid bolus

Adrenaline IM 500 micrograms (0.5ml 1 in 1,000)

hydrocortisone (200mg)

Chlorphenamine (10mg)

Salbutamol (5mg) 

29

PBC question:  antibody test for PBC

 

anti-mitochondrial antibodies (AMA) M2

subtype are present in 98% of patients and are highly specific

 

Primary biliary cirrhosis (now increasingly referred to as primary biliary cholangitis) is a chronic liver disorder typically seen in middle-aged females (female:male ratio of 9:1). The aetiology is not fully understood although it is thought to be an autoimmune condition. Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis which may eventually progress to cirrhosis. The classic presentation is itching in a middle-aged woman
Associations:

  • Sjogren's syndrome (seen in up to 80% of patients)
  • rheumatoid arthritis
  • systemic sclerosis
  • thyroid disease

Diagnosis

  • anti-mitochondrial antibodies (AMA) M2 subtype are present in 98% of patients and are highly specific
  • smooth muscle antibodies in 30% of patients
  • raised serum IgM

Management

  • pruritus: cholestyramine
  • fat-soluble vitamin supplementation
  • ursodeoxycholic acid
  • liver transplantation e.g. if bilirubin > 100 (PBC is a major indication) - recurrence in graft can occur but is not usually a problem

30

PBC questions: histology showing classic PBC

Granulomatous 

PBC: intrahepatic destruction by chronic granulomatous inflammation results in cirrhosis

PSC: inflammation, fibrosis and strictures and intra and extrahepatic ducts

 

Primary biliary cirrhosis (now increasingly referred to as primary biliary cholangitis) is a chronic liver disorder typically seen in middle-aged females (female:male ratio of 9:1). The aetiology is not fully understood although it is thought to be an autoimmune condition. Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis, which may eventually progress to cirrhosis. The classic presentation is itching in a middle-aged woman

Clinical features

early: may be asymptomatic (e.g. raised ALP on routine LFTs) or fatigue, pruritus

cholestatic jaundice

hyperpigmentation, especially over pressure points

xanthelasmas, xanthomata

also: clubbing, hepatosplenomegaly

late: may progress to liver failure


Complications

malabsorption: osteomalacia, coagulopathy

sicca syndrome occurs in 70% of cases

portal hypertension: ascites, variceal haemorrhage

hepatocellular cancer (20-fold increased risk)