Hyperammonemic Encephalopathy Caused by Carnitine Deficiency
نویسندگان
چکیده
منابع مشابه
Encephalopathy caused by thiamine deficiency.
513 July 2015, Vol. 105, No. 7 Encephalopathy caused by thiamine deficiency To the Editor: Antel et al.[1] have drawn attention to the acute encephalopathy caused by thiamine deficiency in non-alcohol-related settings. I would like to highlight a different clinical presentation that ought to be readily recognised as a possible manifestation of this deficiency, namely rapidly progressive leg wea...
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Acute hypoglycemia in children is not an uncommon disease that can be encountered in the Emergency Department. Most cases of childhood hypoglycemia are caused by ketotic hypoglycemia due to missed meals. Often, hypoketotic hypoglycemia can also occur, which suggests hyperinsulinemia or a defect in fatty acid oxidation. Carnitine is essential for long chain fatty acids transfer into mitochondria...
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Ornithine transcarbamylase (OTC) deficiency is an X chromosome-linked disorder causing hyperammonemic encephalopathy with a very poor prognosis. We describe here two patients with OTC deficiency, one a late on-set female patient (case 1) and the other a neonatal-onset male patient (case 2), who were successfully treated with orthotopic liver transplantation (OLTx). The OTC activity in the excis...
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The use of valproic acid (VPA) (also known as Depakote, Depakene, and others) frequently results in elevated plasma ammonia. In some people, hyperammonemia may be clinically significant, resulting in hyperammonemic encephalopathy, which may be severe. Valproic acid-induced hyperammonemic encephalopathy may occur in people with normal liver function, despite normal doses and serum levels of VPA....
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Valproate (VPA)-induced hyperammonemic encephalopathy is an unusual, but serious, adverse effect of divalproex sodium (DVPX) treatment and if untreated can lead to raised intracranial pressure, seizures, coma, and eventually death. It can, however, be reversed if an early diagnosis is made. It is therefore extremely important to recognize it and discontinue DVPX treatment. Our patient developed...
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ژورنال
عنوان ژورنال: Journal of General Internal Medicine
سال: 2007
ISSN: 0884-8734,1525-1497
DOI: 10.1007/s11606-007-0473-0