نتایج جستجو برای: lesch

تعداد نتایج: 4630  

2013
Edmund N. Santurri

[1] According to a recent assessment, it is now possible through medical procedures such as amniocentesis, fetoscopy and ultrasound, to diagnose in utero and with considerable accuracy over 280 abnormalities of pregnancy.1 These abnormalities include a range of fetal genetic disorders (e.g. Down's syndrome, Tay-Sachs disease, Lesch-Nyhan syndrome, spina bifida) which, if left unattended, will r...

Journal: :Brain : a journal of neurology 2006
H A Jinnah Jasper E Visser James C Harris Alfonso Verdu Laura Larovere Irene Ceballos-Picot Pedro Gonzalez-Alegre Vladimir Neychev Rosa J Torres Olivier Dulac Isabelle Desguerre David J Schretlen Kenneth L Robey Gabor Barabas Bastiaan R Bloem William Nyhan Raquel De Kremer Gary E Eddey Juan G Puig Stephen G Reich

Lesch-Nyhan disease (LND) is caused by deficiency of the purine salvage enzyme hypoxanthine-guanine phosphoribosyltransferase (HPRT). Affected individuals exhibit over-production of uric acid, along with a characteristic neurobehavioural syndrome that includes mental retardation, recurrent self-injurious behaviour and motor disability. Prior studies involving relatively small numbers of patient...

Journal: :Innovaciencia Facultad de Ciencias Exactas, Físicas y Naturales 2015

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