Ornithine carbamoyltransferase (OTC) deficiency is a urea cycle disorder that causes the accumulation of ammonia, which can lead to encephalopathy. Adults presenting with hyperammonemia who are subsequently diagnosed with urea cycle disorders are rare. Herein, we report a case of a late-onset OTC deficient patient who was successfully treated with arginine, benzoate and hemodialysis. A 59-yr-ol...

Succinic semialdehyde dehydrogenase enzyme (SSADH) deficiency is a rare autosomal disorder involving the catabolism of the neurotransmitter γ-amino butyric acid (GABA). In the absence of SSADH, GABA is converted to 4-hydroxybutyrate. 4-hydroxybutyrate, which is regarded as a biochemical hallmark, accumulating in serum, urine and cerebrospinal fluid. We reported three brothers whose parents were...

THE LOSS OF MERCURY due to volatilization and incomplete removal of oxidizing agents which destroy dithizone are among the major problems in determining mercury in urine. This paper describes a method by which these difficulties are reduced to a minimum. In actual use, this method has been found efficient in screening large numbers of workers exposed to a mercury hazard. Most methods require a ...

Modern instrumentation allows the measurement of organic acids in urine in their physiological concentration ranges. Eight of the compounds that are reported can serve as markers for specific toxicant exposure or detoxification challenges. Xylene exposure causes elevation of 2-methylhippurate, and orotic acid elevation reveals ammonia challenge that exceeds the capacity of the urea cycle. Gener...

exposure to mercury (hg) as a heavy metal can cause health effects. the objective of this study was to assess occupational exposure to hg in a chlor-alkali petrochemical industry in iran by determining of hg concentrations in air, blood and urine samples.the study was performed on 50 exposed subjects and 50 unexposed controls. air samples were collected in the breathing zone of exposed subjects...

In this study, a newly developed nitrogen (N) and phosphorus (P) recovery process using membrane contactor termed NPHarvest was tested with different liquid waste streams in large-scale trials. Ammonia captured ammonium sulfate form hydrophobic membrane, while precipitated as sludge alkali precipitation coagulation processes. The were selected to cover the potential for included two mesophilic ...

inherited disease, 7th ed. New York: McGraw-Hill, 1995:791–801. 5. Tanaka K, Orr JC, Isselbacher KJ. The identification of -hydroxyisovaleric acid in the urine of a patient with isovaleric acidemia. Biochem Biophys Acta 1968;152:638–41. 6. Tanaka K, Ikeda Y, Matsubara Y, Hyman D. Molecular basis of isovaleric acidemia in the study of the acyl-CoA dehydrogenase family. Adv Neurol 1988;48:107–31....

background & aim: this study evaluated prevention effect of hydro-alcoholic extract of punica granatum peel ethylene glycol-induced nephrolithiasis in wistar rats. experimental: 20 male wistar rats were randomly divided into 4 equal groups. group i animals served as normal control (a) and received distilled water for 28 days. group ii to iv animals received 1% v/v ethylene glycol (eg) in d...

4-chloro-2-methylphenoxy acetic acid (mcpa) is one of the most important pesticides which is extensively used to control weeds in arable farmland. exposure to this compound occurs in general population and persons who occupationally handle it. the aim of this present work was the preparation of mcpa imprinting polymer and its application as a selective sample preparation technique for trace det...

Cu BA, T. Serum and Urine Amino Acid Analysis in Children with Biliary Atresia. Tohoku J. exp. Med., 1983, 139 (1), 61-66 Serum and urine amino acid levels were measured preoperatively and at varying time following successful hepatic portoenterostomy. All of the essential amino acids measured returned to normal levels during the late postoperative period over five years, whereas many of the non...