نتایج جستجو برای: urea cycle disorders

تعداد نتایج: 967162  

Journal: :Journal of neurology, neurosurgery, and psychiatry 2004
S Rimbaux C Hommet D Perrier J P Cottier A Legras F Labarthe L Lemarcis A Autret F Maillot

Ornithine transcarbamylase deficiency (OTCD) is the most common urea cycle disorder. This condition usually presents in neonates or children. This report describes the clinical case of a 21 year old woman who was diagnosed in adulthood during the course of an unexplained coma. After recovery from the coma, she presented very unusual neuropsychological disorders involving memory and the meaning ...

2016
Patrick C Lee Brian Truong Agustin Vega-Crespo W Blake Gilmore Kip Hermann Stephanie AK Angarita Jonathan K Tang Katherine M Chang Austin E Wininger Alex K Lam Benjamen E Schoenberg Stephen D Cederbaum April D Pyle James A Byrne Gerald S Lipshutz

Urea cycle disorders are incurable enzymopathies that affect nitrogen metabolism and typically lead to hyperammonemia. Arginase deficiency results from a mutation in Arg1, the enzyme regulating the final step of ureagenesis and typically results in developmental disabilities, seizures, spastic diplegia, and sometimes death. Current medical treatments for urea cycle disorders are only marginally...

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