caa a22 4500 469044225 CHVBK 20180323132819.0 cr unu---uuuuu 170328e19921101xx s 000 0 eng 10.1007/BF01800228 doi (NATIONALLICENCE)springer-10.1007/BF01800228 Tryptophan and serotonin metabolism in familial erythrophagocytic lymphohistiocytosis [Elektronische Daten] [D. Howells, K. Hyland, I. Smith, S. Strobel] Summary: Reduced concentrations of tryptophan and 5-hydroxyindoleacetic acid (the major CSF metabolite of serotonin) were found in the cerebrospinal fluid of two children with familial erythrophagocytic lymphohistiocytosis. This was associated with elevated cerebrospinal fluid neopterin concentrations indicating increased macrophage activity within the central nervous system. In one child, cytotoxic therapy induced a complete clinical remission and an increase of tryptophan and 5-hydroxyindoleacetic acid concentrations to normal; during a subsequent relapse, concentrations of these analytes again fell below normal. In the other child, in whom therapy produced only a transient improvement, tryptophan and 5-hydroxyindoleacetic acid concentrations remained low and the child died. It is likely that increased activity of indoleamine 2,3-dioxygenase induced by the activation of macrophages was responsible for the disturbance in serotonin and tryptophan homeostasis within the brain. Excessive tryptophan catabolism and the disturbance of serotonin turnover may play a role in the aetiology of the neurological symptoms seen in familial erythrophagocytic lymphohistiocytosis. SSIEM and Kluwer Academic Publishers, 1992 Howells D. Department of Child Health, Institute of Child Health, 30 Guilford Street, WC1, London, UK aut Hyland K. Department of Child Health, Institute of Child Health, 30 Guilford Street, WC1, London, UK aut Smith I. Department of Child Health, Institute of Child Health, 30 Guilford Street, WC1, London, UK aut Strobel S. Department of Immunology, Institute of Child Health, 30 Guilford Street, WC1, London, UK aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/6(1992-11-01), 891-897 0141-8955 15:6<891 1992 15 10545 https://doi.org/10.1007/BF01800228 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF01800228 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Howells D. Department of Child Health, Institute of Child Health, 30 Guilford Street, WC1, London, UK aut NATIONALLICENCE 700 1- Hyland K. Department of Child Health, Institute of Child Health, 30 Guilford Street, WC1, London, UK aut NATIONALLICENCE 700 1- Smith I. Department of Child Health, Institute of Child Health, 30 Guilford Street, WC1, London, UK aut NATIONALLICENCE 700 1- Strobel S. Department of Immunology, Institute of Child Health, 30 Guilford Street, WC1, London, UK aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/6(1992-11-01), 891-897 0141-8955 15:6<891 1992 15 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer