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 |
Lactate | 8 mmol/L* | <2 |
Glucose | 0.9 mmol/L* | 3.5 – 6.1 |
WCC | 14.7 x 109 /L* | 4.0 – 11.0 |
Neutrophils | 27% | . |
Lymphocytes | 70% | . |
ALT | 1600 U/L* | 10 – 55 |
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)
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.
Discussion
a) Differentials for this shock-like presentation:
Domain | Neonate/infant age group | Children older than 12 months |
Vascular |
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Infectious |
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Neoplastic |
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Drug-induced |
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Congenital |
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Autoimmune |
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Trauma |
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Endocrine or metabolic |
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b) Assessment of this shock state:
Generic to the infant/paediatric population | ||
History
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Examination
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Investigations
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Specific for the neonatal population: | ||
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c) Approach to management, which is very generic:
- 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. - Administer 100% oxygen.
- 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 - Sedation and analgesia to support tolerance of invasive therapies
(also decreases demands on the cardiac output) - Electrolyte correction
- 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 1st 48 hours)".
- A urinary catheter will also be required. - No protein in diet until metabolic screen is cleared
- Maintain normoglycaemia with infusion of 10% dextrose of dextrose-rich maintenance fluid - Blood transfusion may not be warranted
- 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).