Enhanced urinary excretion of leukotriene E4 in patients with mevalonate kinase deficiency

In patients with mevalonate kinase deficiency, urinary excretion of the leukotriene LTE 4 was found to be elevated. A positive linear relationship between increased urinary excretion of mevalonate and LTE 4 (n=5) suggests that increased cysteinyl leukotriene synthesis is involved in the pathomechani...

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Veröffentlicht in:	The Journal of pediatrics 1993-07, Vol.123 (1), p.96-98
Hauptverfasser:	Mayatepek, Ertan, Hoffmann, Georg F., Bremer, Hans J.
Format:	Artikel
Sprache:	eng
Schlagworte:	Biological and medical sciences Child Child, Preschool Chromatography, High Pressure Liquid Errors of metabolism Female Gas Chromatography-Mass Spectrometry Humans Leukotriene E4 Lipids (lysosomal enzyme disorders, storage diseases) Male Medical sciences Metabolic diseases Mevalonic Acid - urine Phosphotransferases (Alcohol Group Acceptor) Phosphotransferases - deficiency SRS-A - analogs & derivatives SRS-A - urine Urine - cytology
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Beschreibung
Zusammenfassung:	In patients with mevalonate kinase deficiency, urinary excretion of the leukotriene LTE 4 was found to be elevated. A positive linear relationship between increased urinary excretion of mevalonate and LTE 4 (n=5) suggests that increased cysteinyl leukotriene synthesis is involved in the pathomechanisms of this disease.
ISSN:	0022-3476 1097-6833
DOI:	10.1016/S0022-3476(05)81548-3