Simple and fast quantification of nitisone (NTBC) using liquid chromatography-tandem mass spectrometry method in plasma of tyrosinemia type 1 patients.

Tyrosinemia type 1, which is caused by a deficiency in fumarylacetoacetate hydrolase, is successfully treatable with nitisone (NTBC), an inhibitor of 4-hydroxyphenyl pyruvate dioxygenase. The recommended average dose of NTBC is 1 mg/kg per day. A rapid liquid chromatography (LC) coupled with negative electrospray ionization tandem mass spectrometry method was developed and validated for the quantification of NTBC in heparinized human plasma.

Glycine and ammonia plasma concentrations during sedation with remifentanil in critically ill patients.

Objectives: To investigate glycine and ammonia plasma concentrations during a 72-h remifentanil infusion and the relationship between glycine concentration and remifentanil infusion rate.

Design And Setting: A prospective open-label observational clinical trial in a trauma and a neurosurgical intensive care unit in a university teaching hospital.

Patients: Nine consecutive patients requiring sedation and ventilatory support for at least 72 h.