What is the treatment for Vitamin B7 (biotin) deficiency?

Treatment of Biotin (Vitamin B7) Deficiency

Oral biotin is the first-line treatment for biotin deficiency, with doses of 10 mg/day for patients with malabsorption or short bowel syndrome, while intravenous biotin up to 200 mg/day for 2-3 weeks is reserved exclusively for parenteral nutrition-dependent patients. 1

Treatment Algorithm Based on Intestinal Function

The European Society for Clinical Nutrition and Metabolism (ESPEN) provides a clear treatment pathway that prioritizes route of administration based on gastrointestinal function 1:

For Patients with Functional GI Tract

• Oral biotin at 10 mg/day is recommended for rapid replenishment in deficiency states, particularly in malabsorption or short bowel syndrome 1
• This represents a substantial increase from maintenance doses (30 mcg/day for enteral nutrition) 1
• No upper limit for biotin intake has been established, and no adverse effects have been reported even with prolonged use at pharmacological doses up to 5 mg/day 1, 2

For Parenteral Nutrition-Dependent Patients

• IV biotin up to 200 mg/day for 2-3 weeks is the appropriate treatment 1
• Standard PN maintenance dosing is 60 mcg/day 1
• Intramuscular administration is not mentioned in guidelines and should not be considered a standard route 1

Clinical Manifestations Requiring Treatment

Biotin deficiency presents with a characteristic constellation of symptoms that should prompt immediate supplementation 3, 4:

• Dermatologic: Alopecia, dermatitis, pallor, glossitis 3
• Neurologic: Lethargy, hypotonia, irritability, seizures, ataxia, mental retardation, hyperaesthesia 3, 4
• Metabolic: Recurrent lactic acidosis (especially in short bowel syndrome), ketolactic acidosis, organic aciduria 3, 4
• Other: Anorexia, nausea, muscle pain, elevated serum cholesterol and bile pigments 3

High-Risk Populations Requiring Vigilance

Certain patient populations are at substantially increased risk and may require proactive supplementation 3, 1:

• Long-term parenteral nutrition patients, especially when biotin-free formulations are used 3
• Patients on prolonged broad-spectrum antibiotics (which eliminate biotin-producing intestinal bacteria) 3
• Children with short bowel syndrome (at risk for recurrent lactic acidosis) 3
• Patients on renal replacement therapy (may require additional biotin supplementation) 1

Pediatric Dosing Considerations

For infants and children on parenteral nutrition, specific weight-based dosing applies 3:

• Preterm and term infants up to 12 months: 5-8 mcg/kg/day 3
• Older children: 20 mcg/day 3

Special Populations

• Pregnancy: May require up to 300 mcg/day based on metabolic markers 1
• Breastfeeding: At least 35 mcg/day orally 1

Critical Safety Consideration

While biotin toxicity is essentially nonexistent, high-dose biotin supplementation can cause false laboratory results in biotin-streptavidin-based immunoassays, potentially leading to misdiagnosis of endocrine disorders, particularly thyroid dysfunction 2, 5, 6. This interference does not reflect actual drug interactions (biotin does not interact with levothyroxine absorption) 2, but clinicians must be aware of this analytical interference when interpreting laboratory results in patients taking biotin supplements.

Genetic Deficiency Disorders

For inborn errors of metabolism including biotinidase deficiency and holocarboxylase synthetase deficiency, high-dose biotin supplementation is the definitive treatment and can successfully manage these conditions 7, 4. These patients require lifelong supplementation at pharmacological doses.