title: Decreased 6-keto prostaglandin F1 alpha (6-keto PGF1 alpha) in patients with Lesch-Nyhan syndrome.
abstract: The coagulation abnormality in patients with Lesch-Nyhan syndrome (LNS) prompted us to examine 6-keto prostaglandin F1 alpha (6-keto PGF1 alpha), a stable metabolite of prostacyclin (PGI2). Plasma levels of 6-keto PGF1 alpha were significantly low in 4 patients with LNS, but they were elevated after discontinuation of allopurinol. Other indicators of coagulation and fibrinolysis systems did not change after the discontinuation of allopurinol. PGI2 prevents the production of superoxide which is formed after cerebral ischemia. The potential source of superoxide is xanthine oxidase which is inhibited by allopurinol. It is assumed that plasma PGI2 increased in response to formed superoxide because xanthine oxidase inhibition was abolished after discontinuation of allopurinol.
authors.affiliation: Division of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, Tokyo, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Imamura
authors.forename: H
authors.initials: H
authors.lastname: Yamanouchi
authors.forename: M
authors.initials: M
authors.lastname: Arima
mesh_terms: D015121:6-Ketoprostaglandin F1 alpha / Q000097:blood*; D000293:Adolescent; D000328:Adult; D000493:Allopurinol / Q000009:adverse effects / Q000627:therapeutic use; D001777:Blood Coagulation; D002648:Child; D005344:Fibrinopeptide A / Q000378:metabolism; D006801:Humans; D007677:Kidney Function Tests; D007926:Lesch-Nyhan Syndrome / Q000097:blood* / Q000188:drug therapy; D008297:Male; D010978:Platelet Factor 4 / Q000378:metabolism; D013481:Superoxides / Q000378:metabolism; D001620:beta-Thromboglobulin / Q000378:metabolism
chemical_list: D001620:beta-Thromboglobulin; D013481:Superoxides; D005344:Fibrinopeptide A; D010978:Platelet Factor 4; D015121:6-Ketoprostaglandin F1 alpha; D000493:Allopurinol