Question 28

A two-week-old baby is brought to your general intensive care unit in extremis, pending transfer to a paediatric centre. The baby was born at term and discharged well on day 5 of life. For the past three days the baby has had progressive tachypnoea, lethargy and failure to feed, and has now presented after a seizure. The baby has been intubated in the emergency department.

Blood gas results taken breathing room air prior to intubation are below:

Parameter

Patient

Reference

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

Lactate

8 mmol/L*

<2

Glucose

0.9 mmol/L*

3.5-6.1

White cell count

14.7 x 109/L*

4.0-11.0

Alanine aminotransferase (ALT)

1600 U/L*

10-55

Aspartate aminotransferase (AST)

2200 U/L*

10-40

a) List the likely differential diagnoses for this patient. (2 marks)

b) Outline your assessment to differentiate between these diagnoses. (3 marks)

c)  Outline the management of this patient. (5 marks)

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College answer

Syllabus topic/section:

2.1.17    Paediatrics: L2


Discussion: 

Candidates who did well on this question were able to formulate a list of differentials, provide an assessment to help differentiate between the answers listed in part a), and then provide a general and specific plan of management. Some candidates were able to efficiently tabulate appropriate parts of their answer. Answers that included structure and a clear management plan of the listed abnormalities (e.g. hypoglycaemia) received more marks, as did those that referenced frequent liaison with a paediatric centre and specific treatments such as prostaglandin for potential duct dependent cardiac lesions.

Answers that did not attract as many marks omitted detail in part c) (50% of total marks for the question) Candidates should be aware of the marks allocated to different parts of each question and prioritise their timing accordingly for maximum time value. Some answers omitted important details such as omission of non- accidental injury (NAI) as a DDx in part a) and/or liaison with a paediatric centre. Some candidates also wrote detailed intubation specifics which did not attract marks as the stem outlined the intubation was already complete.
 

Discussion

This is almost identical to Question 10 from the first paper of 2015, except in 2015 it was still ok to instruct the exam candidates to "list, in broad terms", whereas these days we use more meaningful language. 

a) Systematically:

  • There is acidaemia
  • The CO2 is compensated well: the expected is (1.5 × 5 + 8) = 15.5; so the acidosis is mainly metabolic
  • There is reasonable oxygenation, considering this is a room air gas
  • The lactate is severely elevated
  • The BSL is desperately low
  • the LFTs are severely deranged

What could this mean?

  • Septic shock (probably the most common)
  • Non-accidental injury (trauma)
  • Congenital cardiac disease
  • Drugs, eg. paracetamol
  • Congenital metabolic disorders

b) Assessment:

History

  • Fever, feeding, urine output, diarrhoea, vomiting
  • Irritability, lethargy
  • Trauma
  • Potential for ingestion 
  • Vaccination history
  • Unwell contacts

Examination

  • Temperature
  • Level of consciousness
  • Peripheral perfusion, capillary refill
  • Rash
  • Skin turgor
  • Mucous membranes
  • Pulses
  • Heart rate, rhythm
  • Blood pressure
  • Respiratory rate

Investigations

  • CXR
  • ECG
  • ABG for lactate
  • FBC and blood film
  • BSL
  • EUCs, LFTs
  • Blood cultures
  • TTE

c) Management:

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
    - 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

References

Steiner, Michael J., Darren A. DeWalt, and Julie S. Byerley. "Is this child dehydrated?." Jama 291.22 (2004): 2746-2754.

Levine, Adam C., et al. "Empirically Derived Dehydration Scoring and Decision Tree Models for Children With Diarrhea: Assessment and Internal Validation in a Prospective Cohort Study in Dhaka, Bangladesh.Global Health: Science and Practice 3.3 (2015): 405-418.

Freedman, Stephen B., et al. "Diagnosing clinically significant dehydration in children with acute gastroenteritis using noninvasive methods: a meta-analysis." The Journal of pediatrics 166.4 (2015): 908-916.

Friedman, Jeremy N., et al. "Development of a clinical dehydration scale for use in children between 1 and 36 months of age." The Journal of pediatrics 145.2 (2004): 201-207.

Gorelick, Marc H., Kathy N. Shaw, and Kathleen O. Murphy. "Validity and reliability of clinical signs in the diagnosis of dehydration in children." Pediatrics 99.5 (1997): e6-e6.

Holliday, Malcolm A., and William E. Segar. "The maintenance need for water in parenteral fluid therapy." Pediatrics 19.5 (1957): 823-832.

Meyers, Rachel S. "Pediatric fluid and electrolyte therapy." The Journal of Pediatric Pharmacology and Therapeutics 14.4 (2009): 204-211.

Wang, Jingjing, Erdi Xu, and Yanfeng Xiao. "Isotonic versus hypotonic maintenance IV fluids in hospitalized children: a meta-analysis." Pediatrics (2013): peds-2013.

Neilson, Julie, et al. "Intravenous fluids in children and young people: summary of NICE guidance." BMJ: British Medical Journal (Online) 351 (2015).