The urea cycle functions to incorporate ammonia, generated by normal metabolism, into urea. Urea cycle disorders (UCDs) are caused by loss of function in any of the enzymes responsible for ureagenesis, and are characterized by life-threatening episodes of acute metabolic decompensation with hyperammonemia (HA). A prospective analysis of interim HA events in a cohort of individuals with ornithin...

In the title thio-urea derivative, C(14)H(20)N(4)S, the non-ring non-H atoms are approximately planar, with an r.m.s. deviation of 0.0720 Å. The pyridine ring is twisted out of this plane and makes a dihedral angle of 16.85 (13)° with it. The mean plane passing through the cyclo-hexyl ring is almost normal to the central plane [dihedral angle = 69.23 (8)°]. An intra-molecular N-H⋯N(imine) hydro...

In the title compound, C(16)H(17)N(3)S, the amino-thio-urea unit is nearly planar (r.m.s. deviation = 0.0425 Å), and is twisted with respect to the tolyl and phenyl rings by 57.84 (7) and 15.88 (14)°, respectively; the tolyl and phenyl rings are twisted by 65.64 (11)° to each other. Inter-molecular N-H⋯S and weak C-H⋯S hydrogen bonds are present in the crystal structure.

Conformationally mobile oligomers with helical structures, or 'dynamic foldamers', may populate a mixture of screw-sense conformers whose relative proportion has been used as a means of communicating information on a molecular scale. The dibenzazepinyl urea provides a means of quantifying both the sense and degree of this screw-sense preference through a combination of circular dichroism (CD) a...

Background: The diagnosis of inborn errors of metabolism is generally challenging. We aimed to explore various types of urea cycle disorders (UCDs), and their clinical presentations and biochemical findings among Egyptian pediatric patients. Materials and Methods: This case-control study was conducted on 86 participants categorized into ...

The application of herbicides to agricultural soil is a well established and effective practice to control weed growth. Another areas of herbicide application are roads and railways where herbicides are used to mantain the quality of the track and a safe working environment for railway personnel (Torstenson, 2001). Some of total herbicides are used in urban areas, or as algicides in paints and ...

Purpose. A 66-year-old man who presented with coma was found to have isolated severe hyperammonemia and diagnosed with a late-onset urea-cycle disorder. He was treated successfully and had full recovery. Methods. We report a novel case of noncirrhotic hyperammonemia and review the literature on this topic. Selected literature for review included English-language articles concerning hyperammonem...

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...

Citrullinemia type I (CTLN1) is an inherited urea cycle disorder, now included in most newborn screening panels in the US and Europe. Due to argininosuccinate synthetase deficiency, CTLN1 can lead to recurrent hyperammonemic crisis that may result in permanent neurologic sequelae. Vomiting in patients with urea cycle disorders may either be the result or cause of acute hyperammonemia, particula...