Biotinidase Deficiency (BIO) is caused by the lack of biotinidase enzyme to liberate bound biotin. Without sufficient free biotin several other critical enzyme systems are unable to function properly. It occurs in about 1 in 60,000 newborns and can result in developmental delay, seizures, eczema, hearing loss and death in severe cases. Treatment is simple and involves daily doses of biotin supplement.

Product Info

Enzyme Deficiency Disorders

GAL      Galactosaemia

G6PD    Glucose-6P Dehydrogenase Deficiency

BIO       Biotinidase Deficiency