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Hydroxocobalamin 1mg is the standard UK formulation, typically administered every two to three months for maintenance therapy Self-administration requires GP prescription, competency training from practice nurses, and demonstration of proper injection technique Common injection sites include the thigh (vastus lateralis) for both intramuscular and subcutaneous administration with site rotation essential Serious side effects are rare but anaphylaxis requires immediate 999 call

Mutations in the BTD gene decrease biotin activity and reduce the ability of the intestine to absorb biotin, resulting in biotin deficiency