Section 4 Flashcards
You are asked to review a 13-year-old male by the paediatric team.
He has been an inpatient for 10 days and was admitted with lethargy, unsteady feet, slurred speech, and general unwellness. Over the past 24 hours he has developed weakness in all four limbs and has started to drool saliva. His parents are Jehovah’s Witnesses.
summarise the case.
• Complex critically ill paediatric patient
• Severe neurological pathology with threatened airway and failure of
ventilation needing urgent multidisciplinary input from senior clinicians
• Parents are Jehovah’s Witnesses
What abnormality do you note
in the blood results?
• Normocytic anaemia
• Platelets are high, depicting inflammation
• Low magnesium
What are the causes of normocytic anaemia?
• Decreased production of normal-sized red blood cells (e.g. anaemia of
chronic disease, aplastic anaemia)
• Increased production of HbS as seen in sickle cell disease
• Increased destruction or loss of red blood cells (e.g. haemolysis,
post-haemorrhagic anaemia)
• An uncompensated increase in plasma volume (e.g. pregnancy, fluid
overload)
• B2 (riboflavin) and B6 (pyridoxine) deficiency
What are the causes of
hypomagnesaemia?
• Decreased magnesium intake
° Starvation
° Alcohol dependence
° Total parenteral nutrition
• Redistribution of magnesium from extracellular to intracellular space
° Treatment of diabetic ketoacidosis
° Alcohol withdrawal syndrome
° Refeeding syndrome
° Acute pancreatitis
• Gastrointestinal magnesium loss
° Diarrhoea, vomiting, and nasogastric suction
° Hypomagnesaemia with secondary hypocalcaemia (HSH)
• Renal magnesium loss
° Renal tubular defects
interpret the arterial blood gas.
• Compensated respiratory acidosis
• Type 2 respiratory failure
• Normal glucose and lactate
• Anion gap 21
What is anion gap? How do you
calculate it?
• Anion gap is the difference in the measured cations and the anions in
serum.
° Measured cations: Na+, K+, Ca2+, and Mg2+
° Unmeasured cations: serum proteins (normal) and paraproteins
(abnormal)
° Measured anions: Cl−, H2Po4
−, HCo3
−
° Unmeasured anions: sulphates and some serum proteins
• Used to determine the cause of metabolic acidosis
• Expressed as mEq/L
• Anion gap = (Na + K) − (Cl + HCo3)
What are the reasons for an
increased anion gap?
• Lactic acidosis/ketoacidosis/alcohol abuse
• Toxins: methanol/aspirin/cyanide
• Renal failure causes high anion gap acidosis by decreased acid excretion
and decreased HCo3
− reabsorption. Accumulation of sulphates,
phosphates, urate, and hippurate accounts for the high anion gap.
What is the significance of a high anion gap?
• The anion gap is affected by changes in unmeasured ions.
- A high anion gap indicates acidosis (e.g. in uncontrolled diabetes, there is an increase in ketoacids i.e. an increase in unmeasured anions) and a resulting increase in the anion gap.
• Bicarbonate concentrations decrease in response to the need to buffer
the increased presence of acids (as a result of the underlying condition).
-The bicarbonate is consumed by the unmeasured anion (via its action as
a
buffer), resulting in a high anion gap.
comment on the csF analysis.
Increase protein in the absence of organisms is called albumin cytological dissociation
Why is there increase in csF protein?
The increase in CSF protein is due to widespread inflammation of the nerve roots.
What would you expect the csF
glucose to be?
Normal—i.e. approx. 2/3 of plasma glucose
What is the differential
diagnosis?
• Guillain Barre Syndrome (GBS)
• Myasthenia Gravis
• Multiple Sclerosis
• Transverse myelitis
• Encephalitis
• Meningitis
• Space-occupying lesion
• Sepsis
What is the likely diagnosis?
What is the pathogenesis?
My diagnosis is Guillain Barre Syndrome.
- The history of prodromal infection and the course of presentation favour my diagnosis.
- There is often history of campylobacter or cytomegalovirus infections or vaccinations (influenza, polio, rabies, and rubella).
- It is postulated that the immune responses directed towards the infecting
organisms cross-react with neural tissues resulting in widespread segmental
demyelination of peripheral nerves
How will you differentiate this from myasthenia gravis?
Differences in Myasthenia (the following are features of myasthenia):
• Early involvement of muscle groups including extra-ocular, levator,
pharyngeal jaw, neck, and respiratory muscles. Sometimes presents
without limb weakness
• Excessive fatigability and variation of symptoms and signs throughout the day are common
• Reflexes are preserved and sensory features, dysautonomia, and bladder dysfunction are absent
• Electrophysiological study shows normal nerve conduction and presence
of decremental response to repetitive nerve stimulation
• EMG shows abnormal jitter and blocking
• Edrophonium test is normally positive
How will you manage this case guillien barre?
General—icU care
• ABC approach: 30% cases require ventilation
• Temperature control
• VTE prophylaxis
• Pain relief
• Feeding
specific
• Steroids
• Immunoglobulin G (IgG)
• Plasma exchange
• CSF filtration
Discuss autonomic dysfunction in these patients with Guillan B syndrome.
• Autonomic dysfunction is a major cause of morbidity and mortality,
particularly in ventilated patients
• May cause refractory orthostatic hypotension, paroxysmal hypertension, bradycardia, ventricular tachyarrhythmias, ileus and urinary retention
• Autonomic dysfunction is of particular importance at the induction of anaesthesia. Careful consideration should be given to the use of suxamethonium and inotropic and vasopressor agents may produce markedly atypical responses in heart rate and blood pressure. Even tracheal suction may lead to significant cardiovascular instability
How do you decide when to
intubate this patient?
• Intubation should be performed on patients who develop any
degree of respiratory failure
• Clinical indicators for intubation include hypoxia, rapidly
declining respiratory function, poor or weak cough, and
suspected aspiration
• Typically, intubation is indicated when the FVC is less than
15 mL/kg
How would you intubate
this child?
• Avoid suxamethonium
• Size 7 cuffed tube (Age/2 +12 cm at lips)
What are the specific treatment options for guillien Barre syndrome?
• Plasma exchange: removes auto antibodies, immune complexes, etc., and has shown to halve the recovery time
• Immunoglobulins: easier and safer than, and equally effective as plasma exchange. Useful in unstable patients
• Steroids: ineffective as monotherapy
• Complement inhibitors: e.g. Eculizumab has been trialed.
• CSF filtration
What is the problem with igG
use in this patient?
• Jehovah’s parents may refuse this.
How do you deal with this
situation?
• Assess the child’s capacity—Gillick competence.
• Involve hospitals Jehovah liaison group/legal service/social
services.
This child is now transferred to ICU
What are the problems encountered during his ICU stay? Gillian B
This condition needs long-term care and ventilatory support. This makes
way for the usual and common ICU-related problems on top of the
pathology-related complications.
Non-pathology-related
• Infections—lines, chest, UTI, septicaemia, etc.
• DVT, pressure sores, and contractures
• Nutritional deficits
• Psychological
Pathology-related
• Autonomic neuropathy
• Pain
The ICU team has tried to do an early percutaneous tracheostomy, which
was unsuccessful due to technical reasons. An X-ray is done after the
procedure. See Figure 4.1
comment on the chest X-ray
• Pneumomediastinum
Cause of pneumomediastinum: Traumatic intubation or tracheostomy/NG
insertion as children have fragile soft tissue in trachea and oesophagus
should a percutaneous
tracheostomy procedure be
followed by X-ray to look for
complications?
Immediate CXR after uncomplicated percutaneous tracheostomy performed
under bronchoscopic guidance rarely reveals unexpected radiological
abnormalities. The role of CXR appears to be restricted to those patients
undergoing technically difficult and complicated procedures.