Biotin deficiency can affect 5 carboxylases, which interferes with a range of metabolic processes.
Biotin deficiency results in deficiency of 5 carboxylases that are biotin dependent.
(1) pyruvate carboxylate
(2) propionyl-CoA carboxylase
(3) 3-methylcrotonoyl-CoA carboxylase
(4) acetyl-CoA carboxylase 1
(5) acetyl-CoA carboxylase 2
Conditions associated with biotin deficiency:
(1) acquired biotin deficiency
(2) congenital biotinidase deficiency (autosomal recessive)
(3) congenital holocarboxylase synthetase deficiency
Causes of acquired biotin deficiency:
(1) chronic alcohol use
(2) therapy with isotretinoin, antibiotics, phenytoin, carbamazepine or primidone
(3) partial gastrectomy
(4) achlorhydria
(5) burns
(6) elderly
(7) athletes
(8) inflammatory bowel disease
(9) parenteral nutrition without biotin
(10) severe malnutrition
(11) diet high in raw egg whites
Biotin deficiency may be partial or profound.
Most patients clinically respond to <= 10 mg per day oral biotin. Some patients may require much higher replacement.
