Question 10

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.

Discussion

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 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)".
   - 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