eMedicine Specialties > Endocrinology > Metabolic Disorders

Pyridoxine Deficiency: Treatment & Medication

Author: Richard E Frye, MD, PhD, Assistant Professor, Departments of Pediatrics and Neurology, University of Texas Health Science Center at Houston
Coauthor(s): Serge A Jabbour, MD, Associate Professor, Department of Medicine, Division of Endocrinology, Thomas Jefferson University
Contributor Information and Disclosures

Updated: Dec 8, 2008

Treatment

Medical Care

Levels of pyridoxine hydrochloride supplementation in various medical conditions are as follows:

  • Cirrhosis - 50 mg/d
  • Hemodialysis - 5-50 mg/d
  • Peritoneal dialysis - 2.5-5 mg/d
  • Chronic renal failure - 2.5-5 mg/d
  • Sideroblastic anemia - 50-600 mg/d
  • Pyridoxine-dependent seizures - 100 mg/d
  • Homocystinuria - 100-500 mg/d
  • Homocystinemia - 100-500 mg/d
  • Gyromitra poisoning - 25 mg/kg IV

At one time, pyridoxine supplementation was given to people with sickle cell anemia; however, no changes were noted in these patients' hematologic indexes or disease activity.

Diet

  • Pyridoxine is widespread in foods. Rather robust quantities can be found in meats, particularly liver, fish, and chicken; vegetables, particularly beans, peas, and tomato; fruits, such as oranges, bananas, and avocados; and grains, such as enriched breads, cereals, and grains.
  • Some vegetables contain up to 70% biologically unavailable pyridoxine as pyridoxine-5-glucoside.
  • Some heat-treated foods may contain pyridoxine-lysine, which has antivitamin activity.
  • The minimum daily requirement of pyridoxine is approximately 1.5 mg; however, the recommended daily intake by the US National Research Council is 2 mg for adults and 0.3 mg for infants.

Activity

  • Vigorous exercise results in a transient increase in plasma PLP, probably from the release of muscular glycogen phosphate. Carbohydrate loading prior to exercise reduces this response. Within 30 minutes of discontinuing exercise, PLP levels return to normal.

Medication

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Supplemental vitamins

Essential for normal deoxyribonucleic acid (DNA) synthesis.


Pyridoxine, vitamin B-6 (Nestrex)

Necessary for normal metabolism of proteins, carbohydrates, and fats. Pyridoxine is also involved in the synthesis of GABA within the CNS.

Adult

Variable depending on indication
Gyromitra poisoning: 25 mg/kg IV over 15-30 min, repeat prn (total dose 15-20 g/d)
Cirrhosis: 50 mg/d PO
Hemodialysis: 5-50 mg/d PO
Peritoneal dialysis or chronic renal failure: 2.5-5 mg/d PO
Sideroblastic anemia: 50-600 mg/d PO
Homocystinuria or homocystinemia: 100-500 mg/d PO
Pyridoxine-dependent seizures: 100 mg/d PO

Pediatric

Variable depending on indication
Pyridoxine-dependent seizures:
Neonates with seizures: 50-100 mg IV/IM as a single dose
Infants with pyridoxine-responsive seizures: 50-100 mg PO qd (with no other identifiable cause)
Children without neuritis: 5-25 mg PO qd for 3 wk, then 1.5-2.5 mg PO qd
Children with neuritis: 10-50 mg PO qd for 3 wk, then 1-2 mg PO qd
Not established for cirrhosis, hemodialysis, peritoneal dialysis, chronic renal failure, sideroblastic anemia, homocystinuria, homocystinemia, or Gyromitra poisoning

May decrease levodopa, phenytoin, and phenobarbital serum levels

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

>200 mg/d may precipitate withdrawal effects when medication is discontinued

More on Pyridoxine Deficiency

Overview: Pyridoxine Deficiency
Differential Diagnoses & Workup: Pyridoxine Deficiency
Treatment & Medication: Pyridoxine Deficiency
Follow-up: Pyridoxine Deficiency
References
Further Reading
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References

  1. Bender DA. Vitamin B6 requirements and recommendations. Eur J Clin Nutr. May 1989;43(5):289-309. [Medline].

  2. Tierney LM, McPhee SJ, Papadakis MA, eds. Current Medical Diagnosis and Treatment. 40th ed. New York, NY: McGraw-Hill; 2001.

  3. Goetz CG. Vitamin deficiencies. In: Goetz CG, Pappert EJ, eds. Textbook of Clinical Neurology. Philadelphia, Pa: WB Saunders; 1999.

  4. Scriver CR, Gibson KM. Disorders of beta- and gamma-amino acids in free and peptide-linked forms. In: Scriver CR, Beaudet A, Sly W, et al, eds. The Metabolic Basis of Inherited Disease. 7th ed. New York, NY: McGraw-Hill; 1995:1349-68.

  5. Beutler E, Lichtman MA, Coller BS, eds. Williams Hematology. 6th ed. New York, NY: McGraw-Hill; 2001.

  6. Chiang EP, Smith DE, Selhub J, et al. Inflammation causes tissue-specific depletion of vitamin B6. Arthritis Res Ther. 2005;7(6):R1254-62. [Medline]. [Full Text].

  7. Kelly PJ, Kistler JP, Shih VE, et al. Inflammation, homocysteine, and vitamin B6 status after ischemic stroke. Stroke. Jan 2004;35(1):12-5. [Medline]. [Full Text].

  8. Kaczorowska M, Kmiec T, Jakobs C, et al. Pyridoxine-dependent seizures caused by alpha amino adipic semialdehyde dehydrogenase deficiency: the first Polish case with confirmed biochemical and molecular pathology. J Child Neurol. Oct 14 2008;[Medline].

  9. Striano P, Battaglia S, Giordano L, et al. Two novel ALDH7A1 (antiquitin) splicing mutations associated with pyridoxine-dependent seizures. Epilepsia. Aug 19 2008;[Medline].

  10. Khayat M, Korman SH, Frankel P, et al. PNPO deficiency: an under diagnosed inborn error of pyridoxine metabolism. Mol Genet Metab. Aug 2008;94(4):431-4. [Medline].

  11. Morris MS, Picciano MF, Jacques PF, et al. Plasma pyridoxal 5'-phosphate in the US population: the National Health and Nutrition Examination Survey, 2003-2004. Am J Clin Nutr. May 2008;87(5):1446-54. [Medline].

  12. Woolf K, Manore MM. Elevated plasma homocysteine and low vitamin B-6 status in nonsupplementing older women with rheumatoid arthritis. J Am Diet Assoc. Mar 2008;108(3):443-53; discussion 454. [Medline].

  13. Baggot PJ, Eliseo AJ, DeNicola NG, et al. Pyridoxine-related metabolite concentrations in normal and Down syndrome amniotic fluid. Fetal Diagn Ther. 2008;23(4):254-7. [Medline].

  14. Balasa VV, Kalinyak KA, Bean JA, et al. Hyperhomocysteinemia is associated with low plasma pyridoxine levels in children with sickle cell disease. J Pediatr Hematol Oncol. Jun-Jul 2002;24(5):374-9. [Medline].

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Further Reading

Related eMedicine topic:
 Vitamin B-6 Dependency Syndromes

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Keywords

pyridoxine deficiency, vitamin deficiency, vitamin B, vitamin B6, pyridoxine, vitamin B deficiency, pyridoxine 5'-phosphate, pyridoxal 5'-phosphate, PLP, vitamin B-6 deficiency, malnutrition, peripheral neuropathy, poor diet, pyridoxine-dependent seizures, hereditary sideroblastic anemia, cirrhosis, hemodialysis, peritoneal dialysis, chronic renal failure, homocystinuria, homocystinemia, Gyromitra poisoning, mushroom poisoning, fungus toxicity, mushroom toxicity

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Contributor Information and Disclosures

Author

Richard E Frye, MD, PhD, Assistant Professor, Departments of Pediatrics and Neurology, University of Texas Health Science Center at Houston
Richard E Frye, MD, PhD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, Child Neurology Society, and International Neuropsychological Society
Disclosure: Nothing to disclose.

Coauthor(s)

Serge A Jabbour, MD, Associate Professor, Department of Medicine, Division of Endocrinology, Thomas Jefferson University
Serge A Jabbour, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Medical Association, American Thyroid Association, Endocrine Society, and Pennsylvania Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Elena Citkowitz, MD, PhD, FACP, Clinical Professor of Medicine, Yale University School of Medicine; Director, Cholesterol Management Center, Director, Cardiac Rehabilitation, Department of Medicine, Hospital of St Raphael
Elena Citkowitz, MD, PhD, FACP is a member of the following medical societies: American College of Physicians, American Heart Association, National Lipid Association, and Sigma Xi
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Kent Wehmeier, MD, Professor, Department of Internal Medicine, Division of Endocrinology, Diabetes, and Metabolism, St Louis University School of Medicine
Kent Wehmeier, MD is a member of the following medical societies: American Society of Hypertension, Endocrine Society, and International Society for Clinical Densitometry
Disclosure: Nothing to disclose.

CME Editor

Mark Cooper, MBBS, PhD, FRACP, Head, Diabetes & Metabolism Division, Baker Heart Research Institute, Professor of Medicine, Monash University
Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD, Professor of Medicine, St Louis University School of Medicine
George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Medical Practice Executives, American College of Physician Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical Research, Endocrine Society, International Society for Clinical Densitometry, and Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.

 
 
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