A two-week-old baby is brought to your general ICU in extremis pending transfer to a paediatric centre. Born at term, she had been discharged well on day 5 of life. For three days she has had progressive tachypnoea, lethargy and failure to feed, and has now presented after a seizure. She has been intubated in the Emergency Department.
Blood tests taken on air prior to intubation show:
|Parameter||Patient Value||Normal Adult range|
|pH||7.04*||7.35 – 7.45|
|PCO2||14 mmHg (1.9 kPa)*||35 – 45 (4.6 – 6.0)|
|PO2||80 mmHg (10.5 kPa)|
|Bicarbonate||5 mmol/L*||22 – 28|
|Glucose||0.9 mmol/L*||3.5 – 6.1|
|White Cell Count||9 14.7 x 10 /L*||4.0 – 11.0|
|Alanine aminotransferase (ALT)||1600 U/L*||10 – 55|
|Aspartate aminotransferase (AST)||2200 U/L*||10 – 40|
a) List, in broad terms, the key differential diagnoses for this presentation. (20% marks)
b) Outline your approach to differentiating between these diagnoses. (30% marks)
c) Outline principles of early management pending transfer. (50% marks)
- Inborn error of metabolism
- Sepsis (viral likely)
- Cardiac disease- especially duct dependent disease
- Trauma (NAI)
- Drugs / Toxins
- 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
- Cyanotic spells
- Family history including infant deaths, inborn errors of metabolism (IEMs), cardiac disease, degree of consanguinity
- General exam – trauma, rash Liver edge (failure, hepatitis) Murmurs Femoral pulses
- 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
- 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.
Pass rate: 34%
Highest mark: 7.0