CICM SAQ 2015.1 Q10

a)  List, in broad terms, the key differential diagnoses for this presentation. (20% marks)

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

b)  Outline your approach to differentiating between these diagnoses.  (30% marks)

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)  Outline principles of early management pending transfer.  (50% marks)

• 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.