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.
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container_title The Journal of pediatrics
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creator Mayatepek, Ertan
Hoffmann, Georg F.
Bremer, Hans J.
description 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.
doi_str_mv 10.1016/S0022-3476(05)81548-3
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source MEDLINE; Elsevier ScienceDirect Journals Complete
subjects 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
title Enhanced urinary excretion of leukotriene E4 in patients with mevalonate kinase deficiency
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