Question 14

College answer

a)

Inborn error of metabolism
Sepsis (viral likely)
Cardiac disease- especially duct dependent disease  
Trauma (NAI)
Drugs / Toxins 
 

b)

History: 
Exposure to ill persons including siblings and parents.  
“Colds”, chicken pox and maternal herpes should be specifically solicited.
Maternal Group B Strep swab should be reviewed
Injury 
Cyanotic spells  Apnoeas 
Family history including infant deaths, inborn errors of metabolism (IEMs), cardiac disease, degree of consanguinity 
 
Examination: 
General exam - trauma, rash Liver edge (failure, hepatitis) Murmurs Femoral pulses 
 
Investigations: 
CXR
ECG
Ammonia
Urine amino and organic acids (if can’t be processed, take while acidotic and store) Cultures if not done
CMV, HSV PCR
Consider skeletal survey if any suggestion of injury  
Cranial ultrasound (widely available)
Echo if available 
 
c) Ongoing liaison with receiving centre.  

Restore then maintain BSL using 10% Glucose (2.5-5ml/kg 10% glucose bolus then 6mg/kg/min infusion.) 
Restore intravascular volume (even post FEAST fluid bolus reasonable)
Direct therapy if specific pathology found- e.g. alprostadil infusion if evidence of duct dependent cardiac disease

 Empiric antibiotics
Empiric antiviral given results above (acyclovir or ganciclovir)
Nil protein intake till initial metabolic results in- maintain on glucose as above

Lung protective ventilation

General ICU housekeeping. 
 
Examiners Comments: 
 
Reasonably well done. Part a) was answered better than b) and c). Some candidates did not read the question completely and described intubation of the baby. 
 

Discussion

This question, and the college answer, are weirdly identical to Question 10 from the first paper of 2015, except now they have capitalised the word "Paediatric", and in 2018 the bloods merely "show," instead of now being "shown below". What was the merit of making these changes without altering any other features of the question?  What is the significance of these changes? Surely there must be some reason behind them, because it would have required less effort to simply cut and paste the SAQ. However, this line of thinking is unproductive. Trying to get into the examiner's heads in the pursuit of some hidden eldritch meaning, there is some risk that a trainee might suddenly be confronted with the Lovecraftian cosmic horror of realising that nobody is carefully tending to the wording or syntax of these SAQs. Gibbering madness may ensue. 

In context of these matters, below one may see that the author has cut-and-pasted the entire discussion section from Question 10 from the first paper of 2015, with subtle changes which on the surface might appear random and cosmetic.

a) Differentials for this shock-like presentation:

b) Assessment of this shock state:

c) Approach to management, which is very generic:

1. Assess the need for endotracheal intubation.
   - At this stage, senior assistance from somebody expert in paediatric critical care is required, as the intubation may be difficult.
2. Administer 100% oxygen.
3. Establish venous access.
   - Give a 20ml/kg bolus, FEAST be damned.
   - Inotropes and vasopressors if no longer fluid-responsive
   - Parameters guiding resuscitation (eg. lactate, haemodynamic variables, urine output) differ little from adult standards
4. Sedation and analgesia to support tolerance of invasive therapies
   (also decreases demands on the cardiac output)
5. Electrolyte correction
6. Maintenance fluid:  As per college answer, "add 100 ml of 50% dextrose to 900 ml 0.9% NaCl and infuse this at 2/3 maintenance rate (16 ml/hr in this case) (accept 24 ml/hr for 1^st 48 hours)". On close inspection, this concoction resembles the premixed "4% and 1/5^th " bags.
   - A urinary catheter will also be required.
7. No protein in diet until metabolic screen is cleared
   - Maintain normoglycaemia with infusion of 10% dextrose of dextrose-rich maintenance fluid
8. Blood transfusion may not be warranted
9. Empiric antibiotics if sepsis is suspected, within 1 hour.
   - Cultures of blood and urine.
   - Consider antivirals if there is suspicion of viral meningitis or encephalitis