The effect of cysteamine was studied in 6 children with nephropathic cystinosis. In 3 of them an in vitro study on fibroblasts was performed. The cystine content of fibroblasts was immediately diminished (about 90% of total cystine content) as soon as the concentration of cysteamine in the medium was greater than or equal to 0,1 mmole/l. In vivo, 50 to 89 mg/kg/day of cysteamine was administered for 9 to 37 months (mean 21,3). There was no adverse reaction. In all cases a dramatic decline in leukocyte cystine level was observed (in 5 cases the level was within the range seen in clinically unaffected heterozygotes). Growth was not improved. The renal function was stabilised in 3 cases. Photophobia which was present in 4 children decreased in 2 cases or disappeared in 2 cases.
The aim of this study was to evaluate the liquid chromatographic system Remedi (Biorad) in comparison with traditional immunological and colorimetric methods, for the diagnosis of acute drug overdose. 469 blood samples and 95 stomach cleaning liquid samples have been analysed during 1995. The usual toxicologic analysis was composed of the benzodiazepines, tricyclic antidepressants and barbiturates research. Ethanol, meprobamate and acetaminophen assays were performed only on physician's request. Only three pharmacological classes can be analysed both with immunological methods and Remedi: benzodiazepines, tricyclic antidepressants and barbiturates. Remedi has been found to be less sensitive than immunological method for benzodiazepines, it sometimes gave false negative results for barbiturates, but it was very efficient for antidepressants. Remedi often identified drugs other than the 3 previous classes: sedatives, antipsychotic, beta-blockers, antiarythmics. Furthermore these drugs are of clinical importance due to the fact that they are able to modify the symptomatology. In every case Remedi was able to give an estimation of the blood concentration of the toxic molecule matched. Remedi can not replace traditional methods but is a good complementary tool, available in emergency. This is particularly useful when clinical signs do not correspond to the toxics suspected by questioning the patient or relatives.
