Elsevier

Analytical Biochemistry

Volume 168, Issue 2, 1 February 1988, Pages 374-379
Analytical Biochemistry

Measurement of total plasma cysteamine using high-performance liquid chromatography with electrochemical detection

https://doi.org/10.1016/0003-2697(88)90332-6Get rights and content

Abstract

Cysteamine is currently used to treat children with the inherited disorder nephropathic cystinosis. A method for the quantitative determination of this aminothiol in human plasma is presented. Whole plasma was reduced with sodium borohydride to convert disulfides to thiols. Cysteamine was then separated by high-performance liquid chromatography and detected electrochemically. The recovery of standard cysteamine added to plasma was 96.6 ± 1.9%. In a patient with cystinosis, an oral dose of cysteamine was absorbed rapidly, with plasma cysteamine reaching a maximum of 56 μm 1 h after the dose. By 1.8 h the plasma cysteamine concentration had decreased to one-half the maximum value.

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