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ACUTE MANAGEMENT OF PRIMARY HYPERAMMONEMIA

Ammonia level (µmol/l)

Undiagnosed case

Known urea cycle disorders

Above upper limit of normal
  • Stop protein intake
  • Give IV glucose at an appropriate dosages to prevent catabolism (10mg/kg/min)+/- insuline

Same

>100 but less than 250( in neonate >150 but <250)
  • Start drug treatment with nitrogen scavengers (l-arginine and ammonul)
  • Start carnitine, biotin, vitamine B12
  • Start Carbaglu®
  • Start  lipid IV 2-3 gram/kg to give higher calories
  • Start medications and nitrogen scavengers according to the protocol of each disorder

250-500
  • Same as above
  • Prepare for hemodialysis
  • Begin hemodialysis  if no rapid drop of ammonia within 3–6 h

Same

> 500

Start hemodialysis with above measure

Same

 

Recommended dosages for medications used in acute management of urea cycle disorders

 

 

Arginine HCl

Ammonul®

N-carbamylglutamate (Carbaglu®)

Kg

<20kg

>20kg

<20 kg

>20kg

Only oral/enteral drug)

Pending diagnosis

250-400mg/kg.  Up to 600mg/kg was recommended

250-400 mg/kg

Up to 600mg/kg was recommended

250mg/kg

 

5.5gram/m2

100mg/kg bolus per NG tube then 25–62.5mg/kg every 6h

NAGS deficiency

250mg/kg

250mg/kg

Not indicated

Same as above

CPS or OTC deficiency

250mg/kg

 

250mg/kg or 4000mg/m2/day

250mg/kg

Maintenance dose up to 500mg/kg/day

5.5gram/m2

Not indicated

ASL deficiency

200-400mg/kg. Up to 600mg/kg was recommended.

200-400mg/kg or  12000mg/m2/day

Same as above

Not indicated

Arginase deficiency

Not indicated

Same as above

Not indicated

 

Normal ammonia level by  age

 

Age

Upper limit (umol/l)

0-7 day

94

8-30 days

80

1 m-15yr

48

>16

26

 

Secondary hyperammonemia:

Can be due to inborn errors of metabolism such as organic acidemia and fatty acids oxidation defects or drugs or other metabolites that may interfere with urea cycle function, or severe liver disease. Laboratory studies can help to distinguish the underlying primary defect and cause of hyperammonemia and guide appropriate treatment.

Caution:

False positive hyperammonemia is not uncommon, therefore, several precaution should be taken in consideration when collecting blood sample to measure ammonia:

  • A free-flowing venous (or arterial) blood sample without tourniquet should be collected into green-top tube (containing lithium or heparin).
  • The sample should be transported on ice to the laboratory, separated within 15 minutes of collection and analyzed immediately.

 

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