Metabolic emergencies (peds): Difference between revisions
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== Hypoglycemia ==
*[[Hypoglycemia (peds)]]
=== 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===
 
#Inadequate oral intake
#Excess insulin
##Newborns of diabetic mothers
#Deficient hyperglycemic hormones (GH or adrenal hormone deficiency)
#Fatty acid oxidation or carbohydrate metabolism disorders
#Prematurity (inadequate glycogen stores)
#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  ===
 
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|- style="font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12"
! bgcolor="#ffffff" align="left" valign="top" rowspan="1" | Patient Age
! bgcolor="#ffffff" align="left" valign="top" rowspan="1" | Dextrose Bolus Dose
! bgcolor="#ffffff" align="left" valign="top" rowspan="1" | Dextrose Maintenance Dosage
! bgcolor="#ffffff" align="left" valign="top" rowspan="1" | Other Treatments to Consider
|- style="font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12"
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" rowspan="2" class="font12" | Neonate
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" rowspan="2" class="font12" | D10 5 mL/kg PO/NG/IV/IO
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" rowspan="2" class="font12" | 6 mL/kg/h D10
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12" | Glucagon, 0.3 milligram/kg IM
|- style="font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12"
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12" | Hydrocortisone, 25 grams PO/IM/IV/IO
|- style="font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12"
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" rowspan="4" class="font12" | Infant
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12" | D10 5 mL/kg PO/NG/IV/IO
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" rowspan="4" class="font12" | 6 mL/kg/h D10
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12" | Glucagon, 0.3 milligram/kg IM
|- style="font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12"
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12" | ''or''&nbsp;
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" rowspan="3" class="font12" | Hydrocortisone, 25 grams PO/IM/IV/IO
|- style="font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12"
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12" | D25 2 mL/kg
|- style="font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12"
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12" | &nbsp;
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| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" rowspan="2" class="font12" | D25 2 mL/kg PO/NG/IV/IO
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" rowspan="2" class="font12" |
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 &gt;20 kg
 
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12" | Glucagon, 0.3 milligram/kg/IM
|- style="font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12"
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12" | Hydrocortisone, 50 grams PO/IM/IV/IO
|- style="font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12"
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" rowspan="2" class="font12" | Adolescent
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" rowspan="2" class="font12" | —
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" rowspan="2" class="font12" |
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 &gt;20 kg
 
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12" | Glucagon, 0.3 milligram/kg IM
|- style="font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12"
| bgcolor="#ffffff" align="left" valign="top" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin: 0px 0px 9px;" class="font12" | Hydrocortisone, 100 grams PO/IM/IV/IO
|}


==Inborn Errors of Metabolism==
==Inborn Errors of Metabolism==

Revision as of 07:33, 1 May 2015

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)


See Also

Source

Tintinalli

Kwon KT, Tsai VW. Metabolic emergencies. Emerg Med Clin N Am. 2007;25:1041-1060.

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