Dosages
Side effects in children
Warnings & precautions in children
Contra-indications in children

Interactions
PK
Renal impairment
References

L-arginine

Generic name
L-arginine
Brand name
ATC Code
B05XB01

Pharmacokinetics in children

No information

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dose recommendation of formulary compared to licensed use (on-label versus off-label)

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Available formulations

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Dosages

Transient hyperammoniaemia in a neonate
  • Intravenous
    • Term neonate

      • Dose in consultation with a metabolic disorders paediatrician.
Hyperammoniaemia
  • Intravenous
    • 0 years up to 18 years
      [3] [4] [5] [6]
      • Initial dose: 350 mg/kg/dose, once only in 90 minutes.
      • Maintenance dose: 350 - 700 mg/kg/day, continuous infusion.
        • Ornithine transcarbamoylase (OTC) and carbamoyl phosphate synthetase (CPS) deficiency: up to 150 mg/kg/day
        • Argininosuccinate synthetase and argininosuccinate lyase deficiency: up to 700 mg/kg/day

        Treatment by or after consultations with a paediatric specialist (metabolic disorders) who has experience with arginine for this indication.

         

    • 0 years up to 18 years
      • Initial dose: 350 mg/kg/dose, once only in 90 minutes.
      • Maintenance dose: 350 - 700 mg/kg/day, continuous infusion.
        • Ornithine transcarbamoylase (OTC) and carbamoyl phosphate synthetase (CPS) deficiency: up to 150 mg/kg/day
        • Argininosuccinate synthetase and argininosuccinate lyase deficiency: up to 700 mg/kg/day

        Treatment by or after consultations with a paediatric specialist (metabolic disorders) who has experience with arginine for this indication.

         

    • 0 years up to 18 years
      • Initial dose: 350 mg/kg/dose, once only in 90 minutes.
      • Maintenance dose: 350 - 700 mg/kg/day, continuous infusion.
        • Ornithine transcarbamoylase (OTC) and carbamoyl phosphate synthetase (CPS) deficiency: up to 150 mg/kg/day
        • Argininosuccinate synthetase and argininosuccinate lyase deficiency: up to 700 mg/kg/day

        Treatment by or after consultations with a paediatric specialist (metabolic disorders) who has experience with arginine for this indication.

         
Growth hormone secretion test
  • Intravenous
    • 1 month up to 18 years
      • Arginine chloride, 10%: 0.5 g/kg/dose, once only. Max single dose: 30 g/dose. run in over 30 min..

      • A properly running infusion is needed because of necrosis in the event of extravasation.

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Renal impaiment in children > 3 months

No information available on dose adjustment in renal impairment.

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The complete list of all undesirable drug reactions can be found in the national Summary of Product Characteristics (SmPC) – click here

Side effects

No information is present at this moment.

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The complete list of all contra-indications can be found in the national Summary of Product Characteristics (SmPC) – click here

Contra-indications

No information available on specific contra indications in children.

The complete list of all warnings and precautions can be found in the national Summary of Product Characteristics (SmPC) – click here

Warnings & precautions in children

A properly running infusion is needed because of necrosis in the event of extravasation.

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Interactions

The complete list of all interactions can be found in the national Summary of Product Characteristics (SmPC) – click here

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References

  1. Rademaker C.M.A. et al., Geneesmiddelen-Formularium voor Kinderen, 2007
  2. Noordam C et al, Werkboek Kinderendocrinologie, digitale publicatie op www.nvk.nl (alleen leden), 2010
  3. Volwassenen, Kinderen en Stofwisselingziekten, Zorgpad Arginosuccinaat Lyase Deficientie (ASLD), http://www.stofwisselingsziekten.nl, Okt 2012, http://www.stofwisselingsziekten.nl/ziekte_informatie/zorgpaden_voor_stofwisselingsziekten
  4. Volwassenen, Kinderen en Stofwisselingziekten, Zorgpad Arginosuccinaat Synthetase Deficientie (ASSD), http://www.stofwisselingsziekten.nl/, Okt 2012, http://www.stofwisselingsziekten.nl/ziekte_informatie/zorgpaden_voor_stofwisselingsziekten
  5. Volwassenen, Kinderen en Stofwisselingziekten, Zorgpad Carbamoyl Fosfaat Synthetase 1 Deficientie (CPS1D), http://www.stofwisselingsziekten.nl/, Okt 2012, http://www.stofwisselingsziekten.nl/ziekte_informatie/zorgpaden_voor_stofwisselingsziekten
  6. Volwassenen, Kinderen en Stofwisselingziekten, Zorgpad Ornithine Transcarbamoylase Deficientie (OTCD), http://www.stofwisselingsziekten.nl/, Okt 2012, http://www.stofwisselingsziekten.nl/ziekte_informatie/zorgpaden_voor_stofwisselingsziekten

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Changes

Changes