ID: NMedQ22.

089-V1-R27 Queensland Health

Clinical Excellence Queensland

PYRIDOXINE (vitamin B6)

• Treatment of:
o Pyridoxine dependent seizures1-3
Indication o Seizures unresponsive to conventional antiseizures medications (particularly if cause
unknown)4
o Pyridoxine dependent syndromes3, dietary deficiency2

Presentation • Tablet 25 mg
• 0–28 days of age maintenance dose 4-6
o 100 mg/day (NOT mg/kg dose) in three divided doses6
Dosage o Maximum: 300 mg/day (NOT per kg) for periods of intercurrent illness1,2,6,7
ORAL

(maintenance) • 29 days to 1 year of age maintenance dose3,6

o 30 mg/kg/day in three divided doses
o Maximum: 300 mg/day (NOT per kg) for periods of intercurrent illness1,2,6,7
• Crush 25 mg tablet and dissolve in 5 mL water for injection
Preparation o Concentration now equal to 5 mg in 1 mL
• Draw up prescribed dose into oral/enteral syringe
Administration • Oral/OGT/NGT without regard to feeds4

Presentation • Vial 100 mg in 2 mL (50 mg in 1 mL)

• Initial dose4-6 (all ages)
INTRAVENOUS

Dosage o 100 mg as a single dose (NOT mg/kg dose)

o If no response, may be repeated after discussion with paediatric neurologist
• Draw up double the prescribed dose and make up to 10 mL total volume with
compatible fluid
Preparation
• Prime infusion line and reduce total syringe volume to 5 mL (prescribed dose in
remaining 5 mL)
• IV injection over 5 minutes8, or
• IV infusion via syringe driver pump over 30 minutes4
Administration
o On completion, disconnect syringe and infusion line
o Flush access port at same rate as infusion

Special • Initial dose for pyridoxine dependent seizures via IV route, followed by maintenance dose
considerations via oral route
• Resuscitation facilities readily available8 as increased apnoea risk with treatment4-7
• During IV initial dosing, continuous cardiorespiratory and intermittent non-invasive BP7
• EEG monitoring (at neonatologist/sub-specialist discretion)4
• A pyridoxine level than less 20 nanomoles/L is indicative of deficiency7
Monitoring
• Diagnostic biomarkers include α-AASA and Δ1-P6C, measured in urine, blood, or
cerebral spinal fluid3
• Pipecolic acid elevated in urine, plasma, and cerebral spinal fluid in most patients with
PDE-ALDH7A13
• Fluids
o 5% glucose, 0.9% sodium chloride9
• Drugs via Y site
o Adrenaline (epinephrine hydrochloride)10, amikacin10, atropine sulfate10,
benzylypenicillin10, calcium chloride10, calcium gluconate10, cefotaxime10, ceftazidime10,
Compatibility ceftriaxone10, cefuroxime10, clindamycin10, dexamethasone sodium phosphate10,
digoxin10, dobutamine10, dopamine10, epoetin alfa10, erythromycin10, esmolol10,
fentanyl10, fluconazole10, gentamicin10, heparin sodium10, insulin regular10, lignocaine
(lidocaine hydrochloride)10, magnesium sulfate10, midazolam10, morphine sulfate10,
noradrenaline (norepinephrine)10, piperacillin sodium10, potassium chloride10,
propranolol10, protamine10, sodium bicarbonate10, succinylcholine chloride10, ticarcillin
disodium10, tobramycin10, vancomycin10, vasopressin10

Queensland Clinical Guidelines

Available from www.health.qld.gov.au/qcg
Queensland Clinical Guidelines: Neonatal monograph Pyridoxine Page 2 of 3

• Fluids
o No information8
Incompatibility • Drugs
o Azathioprine8, cefazolin8, folic acid8, furosemide8, hydrocortisone sodium succinate8,
imipenem-cilastatin8, indometacin8, methylprednisolone sodium succinate8,
phenobarbital8
• High doses may decrease levels of11:
Interactions o Barbiturates
o Phenytoin
• Vial
o Store below 25 °C (do not refrigerate or freeze)8. Protect from light8
Stability
• Oral solution (prepared from tablet)
o Discard unused remainder
• Hypersensitivity and anaphylactic reactions reported1,4
• Blood pathology: acidosis4
• Circulatory: bradycardia7, hypotension7
Side effects
• Nervous: seizure (following large IV doses)4, neuropathy2, paraesthesia4, neurologic
depression7
• Respiratory: respiratory depression7, apnoea7
• Precursor to pyridoxal that functions in the metabolism of proteins, carbohydrates, fats4
• Aids in the release of liver and muscle-stored glycogen and in the synthesis of GABA
within the CNS in addition to the haemoglobin syntheisis4
Actions • Pyridoxine-dependent seizures are a result of defective binding of pyridoxine in the
formation of GABA7
• PDE-ALDH7A1 is an autosomal recessive condition due to a deficiency of α-AASA
dehydrogenase, which is a key enzyme in lysine oxidation3
α-AASA: alpha-aminoadipic semialdehyde, CNS: central nervous system, EEG:
electroencephalogram, GABA: gamma aminobutyric acid, IV: intravenous, NGT:
Abbreviations
nasogastric, OGT: orogastric, PDE-ALDH7A1: pyridoxine dependent epilepsy aldehyde
dehydrogenase 7 Family Member A1
ALDH7A1, pyridoxine dependent epilepsy, vitamin B6, anticonvulsant, seizure, refractory
Keywords
seizure, AED, anti-epileptic drug, anti-seizure drug, refractory seizure

The Queensland Clinical Guideline Neonatal Medicines is integral to and should be read in conjunction with this
monograph. Refer to the disclaimer. Destroy all printed copies of this monograph after use.

References
1. Australian Medicines Handbook. Pyridoxine. [Internet]. Adelaide: Australian Medicines Handbook Pty Ltd; January 2022 [cited 2022 May 24].
Available from: https://amhonline.amh.net.au.
2. British National Formulary for Children (BNFC) online. Pyridoxine. [Internet]: Royal Pharmaceutical Society; October 2021 [cited 2022 April 1].
Available from: https://www.medicinescomplete.com.
3. Coughlin CR, 2nd, Tseng LA, Abdenur JE, Ashmore C, Boemer F, Bok LA, et al. Consensus guidelines for the diagnosis and management of
pyridoxine-dependent epilepsy due to α-aminoadipic semialdehyde dehydrogenase deficiency. J Inherit Metab Dis 2021;44(1):178-92.
4. UpToDate, Lexicomp Online. Vitamin B6 (pyridoxine): Drug information Topic 9839 Version 263.0. [Internet]. Waltham MA: UpToDate Inc;
2018 [cited 2022 May 27]. Available from: https://www.uptodate.com/.
5. van Karnebeek CD, Jaggumantri S. Current treatment and management of pyridoxine-dependent epilepsy. Curr Treat Options Neurol
2015;17(2):335.
6. Kaminiów K, Pająk M, Pająk R, Paprocka J. Pyridoxine-dependent epilepsy and antiquitin deficiency resulting in neonatal-onset refractory
seizures. Brain Sciences 2022;12(1).
7. IBM Micromedex®Neofax®. Pyridoxine In: IBM Micromedex® NeoFax®/Pediatrics (electronic version). IBM Watson Health, Greenwood
Village, Colorado, USA. 2022 [cited 2022 May 26]. Available from: http://neofax.micromedexsolutions.com/neofax.
8. Australian Injectable Drugs Handbook. Nicolette Burridge, Keli Symons, editors. Pyridoxine. 8th ed. [Internet]. New South Wales: Society of
Hospital Pharmacists of Australia (SHPA); March 2022 [cited 2022 April 1]. Available from: https://aidh.hcn.com.au.
9. Royal Children’s Hospital. Vitamin B6. In Paediatric Injectable Guidelines. [Internet]. 2020 [cited 2022 July 01]. Available from:
https://pig.rch.org.au/monographs/calcium-gluconate/.
10. IBM Micromedex®. Pyridoxine in IBM Micromedex® (electronic version). Greenwood Village, Colorado, USA. 2022 [cited 2022 May 27].
Available from: http://www.micromedexsolutions.com.
11. MIMS Online. Pyridoxine. [Internet]: MIMS Australia; May 2022 [cited 2022 May 27]. Available from: https://www.mimsonline.com.au.

Destroy printed copies after use. Refer to the disclaimer State of Queensland (Queensland Health) 2022
Queensland Clinical Guidelines: Neonatal monograph Pyridoxine Page 3 of 3

Document history
ID number Effective Review Summary of updates
Endorsed by Queensland Neonatal Services Advisory
NMedQ22.089-V1-R27 26/09/2022 26/09/2027
Group (QNSAG)

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Destroy printed copies after use. Refer to the disclaimer State of Queensland (Queensland Health) 2022