Description
This assay is intended for use in diagnostic laboratories (not self‑testing). It uses dried blood samples on blotting paper (903® or 226). In the test, biotinidase from the sample acts on an artificial substrate to release 4‑aminobenzoic acid (PABA), which is then converted into a diazotized compound. The purple coloration generated is measured by absorbance and is directly proportional to the biotinidase activity.
A deep deficiency is defined as residual activity < 10% of the normal mean, and partial deficiency between 10–30%. Clinical symptoms of untreated deficiency include neurological abnormalities (seizures, hypotonia, ataxia), skin disorders, optic atrophy, hearing loss, and hair loss. Early treatment with daily oral biotin is highly effective when started early.
The kit is offered in multiple packaging formats to suit different laboratory needs, with manufacturing under European ISO / CE standards
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