释义 |
vitamin B6 -dependency syndromes vitamin B6 -dependency syndromes A group of functional or structural enzyme defects that respond to a megadoses–50-100-fold > minimum daily requirements of pyridoxine Examples Vitamin B6-dependent convulsions, vitamin B6-responsive anemia, xanthurenic aciduria, cystathioninuria, homocystinuria, possibly due to a defective structure of the apoenzyme, its coenzyme binding site or coenzyme synthesis Clinical Predominantly neurologic–eg, mental retardation, psychiatric Sx, seizures, convulsions, ataxia, spasticity, peripheral neuropathy |