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Revisiting the administration of biotin to children with biotin-responsive disorders

Barry Wolf1

  • 1Division of Genetics, Birth Defects and Metabolism, Department of Pediatrics, Ann and Robert H. Lurie, Children's Hospital of Chicago, Chicago, IL, USA.

Molecular Genetics and Metabolism
|July 17, 2022
PubMed

Insights

This commentary clarifies biotin administration for biotin-responsive disorders in children. It addresses common questions and misconceptions for healthcare providers and families regarding this essential vitamin therapy.

Area of Science:

  • Biochemistry
  • Genetics
  • Pediatrics

Background:

  • Inherited metabolic disorders can affect biotin utilization.
  • Biotin-responsive disorders present unique challenges in pediatric care.
  • Misconceptions regarding biotin supplementation persist among caregivers and clinicians.

Purpose of the Study:

  • To clarify the appropriate administration of biotin in children with inherited biotin-responsive disorders.
  • To address common questions and misconceptions surrounding biotin therapy.
  • To provide guidance for healthcare workers, parents, and affected individuals.

Main Methods:

  • This is a commentary, not an experimental study.
  • It synthesizes current understanding and clinical recommendations.
  • Expert consensus and literature review inform the discussion.

Main Results:

  • Biotin administration is crucial for managing biotin-responsive disorders.
  • Early and consistent biotin supplementation is vital for optimal outcomes.
  • Understanding the specific needs of infants and children is paramount.

Conclusions:

  • Clear guidelines on biotin administration are essential for effective treatment.
  • Addressing misconceptions can improve patient adherence and outcomes.
  • This commentary serves as a resource for managing biotin-responsive disorders in pediatric populations.

Keywords:
BiotinBiotin-responsiveBiotinidase deficiencyHolocarboxylase synthetase deficiencyTreatment

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