Metabolic emergencies (peds)

Hypoglycemia

Diagnosis

• Glucose <45 in symptomatic neonate
• Glucose <35 in asymptomatic neonate
• Most common features: vomiting, AMS, poor feeding
  • May also see lethargy, apnea, sz

Etiology

1. Inadequate oral intake
2. Excess insulin
   1. Newborns of diabetic mothers
3. Deficient hyperglycemic hormones (GH or adrenal hormone deficiency)
4. Fatty acid oxidation or carbohydrate metabolism disorders
5. Prematurity (inadequate glycogen stores)
6. Sepsis

Work-Up

• Rapid glucose
• UA
  • If ketones: adrenal or GH deficiency, inborn errors of metabolism
  • If no ketones: Hyperinsulinemia, fatty acid oxidation defects
• Glucagon 0.3 mg/kg IM or IV
  • If BS corrects then likely due to hormonal deficiency (e.g. adrenal insufficiency)

Treatment

Patient Age	Dextrose Bolus Dose	Dextrose Maintenance Dosage	Other Treatments to Consider
Neonate	D10 5 mL/kg PO/NG/IV/IO	6 mL/kg/h D10	Glucagon, 0.3 milligram/kg IM
			Hydrocortisone, 25 grams PO/IM/IV/IO
Infant	D10 5 mL/kg PO/NG/IV/IO	6 mL/kg/h D10	Glucagon, 0.3 milligram/kg IM
	or		Hydrocortisone, 25 grams PO/IM/IV/IO
	D25 2 mL/kg
Child	D25 2 mL/kg PO/NG/IV/IO	6 mL/kg/h D10 for the first 10 kg + 3 mL/kg/h for 11–20 kg + 1.5 mL/kg/h for each additional kg >20 kg	Glucagon, 0.3 milligram/kg/IM
			Hydrocortisone, 50 grams PO/IM/IV/IO
Adolescent	—	6 mL/kg/h D10 for the first 10 kg + 3 mL/kg/h for 11–20 kg + 1.5 mL/kg/h for each additional kg >20 kg	Glucagon, 0.3 milligram/kg IM
			Hydrocortisone, 100 grams PO/IM/IV/IO

Inborn Errors of Metabolism

Background

• Clinical manifestations are due to accumulation of toxic metabolites
• Must rule-out sepsis (more common in these pts)

Diagnosis

• Encephalopathy
• Hypoglycemia
• Hepatic dysfunction
• Nonspecific complaints: lethargy, irritability, N/V

Work-Up

• Glucose level
• UA (ketones)
• Chemistry
  • Anion gap a/w organic acidemias
• LFT
• Ammonia
  • Should be <200 in normal neonate (higher suggests urea cycle disorders)
• Lactate
• VBG

Treatment

1. NS 20 mL/kg boluses
   1. Increase renal excretion of toxic metabolites
2. Keep NPO
   1. Removes potential inciting metabolic substrates
   2. Provide D10 at 2x usual maintenance rates
3. Hyperammonemia
   1. <500
      1. (Na phenylacetate & Na benzoate) 250mg/kg in D10 over 90min; then 250 mg/kg/d infusion
      2. Arginine 210mg/kg IV/IO in D10 over 90min; then 210 mg/kg/d infusion
   2. >600
      1. Dialysis
4. Cerebral edema
   1. Hyperammonemia is risk factor
      1. Give mannitol 0.5gm/kg IV/IO
      2. Do not give steroids (worsens hyperammonemia)

Source

Tintinalli