Specific diseases Flashcards
What are the criteria of the STOP-BANG assessment?
Snoring
Tiredness during the day
Observed cessation in breathing
Pressure (BP) high
BMI >35 kg/m2
Age >50
Neck circumference >40cm
Gender male
≤ 2 - excludes OSA
3-4 - intermediate risk of OSA
≥ 5 - high risk of OSA
What is the AHI?
Apnoea-Hypopnoea Index
The number of apnoeas or hypopnoeas lasting >10s recorded in 1 hour
≥5 mild
≥15 moderate
≥30 severe
What constitutes a significant desaturation in OSA?
Desaturations >4% if ≥5 in 1 hour
Which features of OSA does nocturnal CPAP most improve? (3)
CCF
Platelet aggregation
Dysrhythmias
Which features of OSA should prompt to postpone surgery and optimise?
Untreated CCF
Hypercapnoea with PaCO₂ >6.5
What is the most common and most severe muscular dystrophy?
Duchenne Muscular Dystrophy (DMD)
What is the incidence of DMD?
1 in 3500 male live births (LB)
Define muscular dystrophy
Inherited disorder characterised by progressive muscle weakness, without evidence of denervation
Caused by mutations in genes (usually those involved in making muscle proteins)
X-linked recessive
Post-junctional neuromuscular disorder
Dystrophy = tissue degeneration
Muscular dystrophy = muscular tissue degeneration
Progressive muscular weakness, pseudohypertrophy (muscle contractures), poor balance, progressive inability to walk, scoliosis, waddling gait, Gowers’ sign
What are the cardiac effects of DMD? (3)
Cardiomyopathy
Arrhythmias
Conduction defects
Heart failure
What are the respiratory effects of DMD? (4)
Respiratory muscle weakness
Swallowing impairment
Improper secretion management
Recurrent chest infections
Scoliosis resulting in restrictive lung defects
Failure to wean from ventilation
What are the neurological effects of DMD?
Learning disability
What is the typical life expectancy in DMD?
15-25 years
What is pseudohypertrophy?
False enlargement of muscle
Dystrophin absence renders sarcolemma weak → muscle fibres inadequately tethered → replaced with fibrous connective tissue → pseudohypertrophy
What are the biochemical abnormalities found in DMD?
Raised CK
What are the common operations in DMD?
Orthopaedic:
Scoliosis corrections
Cardiac:
Angiography
Valve replacement
Ophthalmic:
Cataracts
Incidental:
Dental work 2° to low IQ
Why should you perform lung function tests in DMD?
FVC <20ml/kg associated with increased mortality
List 3 acquired, non-drug related causes of Long QT
Subarachnoid haemorrhage
Anorexia Nervosa
Hypothermia
Hypocalcaemia
Hypokalaemia
How do you diagnose long QT?
QTc greater than 450 ms men, 460 ms women
What is your perioperative anaesthetic management of Long QT?
Seek cardiologist opinion
Check electrolytes
Perform ECG with valsalva
Stop any drugs that increase QTc
Continue beta blockers
Avoid suxamethonium
Obtund pressor response to laryngoscopy
Avoid use of reversal (use sugammadex)
Consider transvenous pacing
Pathophysiology of long QT?
Depolarisation abnormality
Malfunction in cardiac ion channels
What is porphyria?
Group of disorders characterised by an inherited or acquired abnormality in the enzymes relating to haem synthesis