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Emergency treatment

Caveat: The general recommendation for emergency measures as mentioned in the paragraph on emergency management have to be carefully recalculated and evaluated for the individual patient and his situation. Metabolic center Heidelberg will not assume responsibility for any adverse outcome in any patient treated following the suggestions on its websites.

A. Endogenous intoxication triggered by catabolism

General approach – promote anabolism!

Provide energy with i.v. glucose

AgeGlucose infusion (rate)
0-12 months10-15 g/kg/d
1-3 years10-12 g/kg/d
4-10 years7-10 g/kg/d
11-15 years4-7 g/kg/d
> 16 years3-5 g/kg/d
  • Add insulin starting with 0,1 I.E./kg h.  Increase when blood glucose concentration exceeds 120 mg/dl (8 mmol/l).
  • consider fat i.v. at a rate of 1-2 g/kg day (only when fatty acid oxidation defects are excluded/highly unlikely).
  • Assess lactic acid concentraion and blood gases every hour. In case of rising lactic acid levels reduce glucose infusion and contact metabolic specialist.

B: Disorders of glucose homeostasis without endogenous intoxication

General approach – provision of glucose i.v. or p.o.!

 

AgeGlucose infusion (rate)
0-12 months10-12 g/kg/d
1-3 years8-10 g/kg/d
4-10 years6-8 g/kg/d
11-15 years4-6 g/kg/d
> 16 years3-5 g/kg/d
  • assess lactic acid concentrations and blood sugar frequently!
  • In hyperinsulinism > 15 g/kg/d may be required

C: Disorders of energy supply from glucose indicated by lactic academia and ketosis

General approach – promote anabolism, restrict glucose!  

 

AgeGlucose infusion (rate)
0-12 months7-10 g/kg/d
1-3 years5-7 g/kg/d
4-10 years4-5 g/kg/d
11-15 years3-4 g/kg/d
> 16 years1-3 g/kg/d
  • assess lactic acid concentrations and blood glucose concentration every hour!
  • Add fat i.v. at 2-4 g/kg/d when fatty acid oxidation defects are excluded

Recommended drug doses

Doses of drugs that should be available as „metabolic emergency drugs” in every NICU/ PICU. 

 

Drug (Generic)
Recommended doses
Indication
Arginine hydrochloride

Bolus: 1 mmol/kg in 1-2 hours i.v.

Maintenance: 1 mmol/kg/day
(up to 3 mmol/kg/24 h in ASL deficiency)

Hyperammonaemia, most probably due to urea cycle defects
Sodium benzoate

Bolus: 250 mg/kg in 1-2 hours i.v,

Maintenance:  250-500 mg/kg/day.

Hyperammonaemia, most probably due to urea cycle defects or organic aciduria
Sodium phenylacetate  Bolus: 250 mg/kg in 1-2 hours i.v, (only available as compound preparation with sodium benzoate (Ammonul(R)

Maintenance: 250-500 mg/kg day p.o.

Hyperammonaemia, most probably due to urea cycle defects
 L- Carnitine

Bolus: 50 mg/kg in 1-2 hours i.v.

Maintenance: 100 mg/kg/day

only when a defect of long-chain fatty acid oxidation is excluded

 



Doses of the vitamines/co-enzymes may be used within the first 3 years of life without any weight adjustment. In general doses may be doubled after the 3rd year.

DrugRecommended dose Indication
Hydroxocobalamin (Vitamin B12)1 mg/day i.m.Methylmalonic aciduria
Biotin (Vitamin H)3 x 5 mg/day p.o.Lactic acidosis
Thiamine (Vitamin B1)3 x 50 mg/day p.o./i.v.Lactic acidosis
Riboflavin (Vitamin B2)3 x 50 mg/day p.o./i.v.Lactic acidosis
Coenzyme Q10 (Ubichinon)3 x 25 mg/day p.o.Lactic acidosis
Vitamin C3 x 200 mg/day p.o./i.v.Lactic acidosis

 



In (refractory) neonatal seizures:

DrugRecommended dose
Pyridoxin (Vitamin B6) Repeatedly 100 mg i.v., up to 500 mg total; continue with 30 mg/kg/d p.o. for 3-7 days
Pyridoxal phosphate30-50 mg/kg/d p.o. for 3 days
Folinic acid 3-5 mg/kg day i.v. for 3 days


 Caveat: After administration of pyridoxine or pyridoxal phosphate apnea is possible!



Suspicion of congenital hyperinsulinism:

DrugRecommended dose
Glukagon

Bolus: 30 µg/kg i.v./i.m.

Maintenance 5-10 µg/kg/h i.v.

Diazoxide3 x 5 mg/kg/ d p.o.
Somatostatin5-30 µg/kg/d continously i.v.
Octreotide5-20 µg/kg/d s.c. in 4-6 doses
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Address

Metabolic Center Heidelberg
Metabolic Laboratory
Im Neuenheimer Feld 669
D-69120 Heidelberg

Metabolic Laboratory:
Phone: +49 (0) 6221 56-8276
Fax: +49 (0) 6221 56-5565
 
Emergency Phone:
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Phone: +49 (0) 6221 56-4002

Emergency contacts