Abstract
The oral administration of L-5-HTP and carbidopa to children with the Lesch-Nyhan syndrome has been reported to reduce the rate of self-mutilative behavior. In experimental animals, drugs which decrease brain serotonin induce aggressive behavior which is reversed by serotonin's immediate precursor L-5-HTP. A 6 m. old male with a complete deficiency of HGPRT was placed on L-5-HTP initially 24 mg twice a day and carbidopa 12.5 mg twice a day. Prior to therapy, CFS was obtained for 5-HIAA, HVA and amino acids, and repeated at 24 and 60 days of therapy. Thereafter, L-5-HTP was increased to 24 mg four times a day. CFS 5-HIAA, the metabolite of serotonin and HVA, the major metabolite of dopamine, were assayed by spectrophotofluorimetry, and amino acids by column chromatography. An increase in 5-HIAA was detected during therapy e.g., 45.9 ng/ml prior, and 170, and 123 ng/ml on therapy. After 12 m. of therapy, a one-day probenecid turnover study (100 mg/kg, divided into four doses) was done with the patient on and repeated off therapy. On therapy, CFS 5-HIAA pre-probenecid was 24.0 and after probenecid was 365.7 ng/ml, while off therapy after probenecid it was 77.6 ng/ml. CFS HVA and amino acids were not effected by therapy. This study indicated that L-5-HTP and carbidopa given orally specifically increase the turnover of serotonin.
Supported by NIH Grant RR-318
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Castella, S., Chakrabarti, C., Winsberg, B. et al. MONOAMINE AND AMINO ACID TURNOVER IN THE LESCH-NYHAN SYNDROME: EFFECTS OF L-5-HYDROXYTRYPTOPHAN (L-5-HTP). Pediatr Res 11, 453 (1977). https://doi.org/10.1203/00006450-197704000-00501
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DOI: https://doi.org/10.1203/00006450-197704000-00501
