نتایج جستجو برای: pyridoxine dependent epilepsy

تعداد نتایج: 745226  

Journal: :Archives of disease in childhood 1967
P A Hughes B D Bower D N Raine N Syed

In a previous paper (Hughes, Bower, Raine, and Syed, 1966) it was shown that about one-third of patients with childhood epilepsy showed, on admission, an abnormality of tryptophan metabolism characterized by a high excretion of xanthurenic acid inthe urine followingan oral load oftryptophan. This is usually accompanied by high excretion of certain other metabolites of tryptophan having in commo...

2017
Izabella A Pena Yann Roussel Kate Daniel Kevin Mongeon Devon Johnstone Hellen Weinschutz Mendes Marjolein Bosma Vishal Saxena Nathalie Lepage Pranesh Chakraborty David A Dyment Clara D M van Karnebeek Nanda Verhoeven-Duif Tuan Vu Bui Kym M Boycott Marc Ekker Alex MacKenzie

Pyridoxine-dependent epilepsy (PDE) is a rare disease characterized by mutations in the lysine degradation gene ALDH7A1 leading to recurrent neonatal seizures, which are uniquely alleviated by high doses of pyridoxine or pyridoxal 5'-phosphate (vitamin B6 vitamers). Despite treatment, neurodevelopmental disabilities are still observed in most PDE patients underlining the need for adjunct therap...

2014
Zhixian Yang Xiaoling Yang Ye Wu Jingmin Wang Yuehua Zhang Hui Xiong Yuwu Jiang Jiong Qin

Pyridoxine-dependent epilepsy (PDE) is a rare autosomal recessive disorder that causes seizures in neonates and infants. Mutations of the ALDH7A1 gene are now recognized as the molecular basis PDE and help to define this disease. Three Chinese children with PDE were clinically analyzed, followed by treatment and examination of the ALDH7A1 mutations. The seizures of the 3 patients were all resis...

Journal: :Archives of Disease in Childhood 1984

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