33 results about "Hyperammonemia" patented technology

The present invention relates to newborn screening kits, methods, stable isotopically-labeled internal standards or internal standard solution for high throughput screening and analysis of metabolic disorders using liquid chromatography mass spectrometry (LC-MS) are provided. The metabolic disorders can be amino acid, organic acid or fatty acid oxidation disorders, and particularly urea cycle disorders or deficiencies, hyperammonemia, Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH), and / or argininosuccinic aciduria. The newborn screening kits, methods, stable isotopically-labeled internal standards or internal standard solution are particularly useful for newborn screening (NBS) of metabolic disorders.

Viral vectors comprising engineered hOTC DNA and RNA sequences are provided which when delivered to a subject in need thereof are useful for treating hyperammonemia, ornithine transcarbamylase transcarbamylase deficiency and symptoms associated therewith. Also provided are methods of using hOTC for treatment of liver fibrosis cirrhosis in OTCD patients by administering hOTC.

The invention features the use of a defined microbial consortia for the replacement of a gut microbiome associated with disease. In particular, the invention provides for the treatment of hyperammonemia, Clostridium difficile colitis, hepatic encephalopathy associated with cirrhosis, and inflammatory bowel disease.

The present invention is directed to a liposome composition for use in the peritoneal dialysis of patients suffering from endogenous or exogenous toxicopathies, wherein the pH within the liposomes differs from the pH in the intraperitoneal cavity and wherein the pH within the liposome results in a liposome-encapsulated charged toxin. The invention also relates to a pharmaceutical composition comprising said liposomes. A further aspect of the present invention relates to a method of treating patients suffering from endogenous or exogenous toxicopathies, preferably selected from drug, metabolite, pesticide, insecticide, toxin, and chemical warfare toxicopathies, more preferably hyperammonemia, comprising the step of administering liposomes of the invention in a therapeutically effective amount into the peritoneal space of a patient in need thereof. Next to human, the present invention is particularly suitable to veterinary aspects.

The present invention provides active salts of ornithine and aspartate dipeptide compounds, preparation methods thereof, pharmaceutical compositions comprising the active salts, and preparation of the dipeptide compounds or their active salts for preventing or treating hyperemia Use in the medicament of ammonia disease or liver disease, especially hepatic encephalopathy. The test results clearly show that the ornithine and aspartate dipeptide compounds and their active salts of the present invention can significantly reduce blood ammonia concentration after administration, and can significantly improve the memory secondary to TAA-induced liver injury in rats. It means that the dipeptide compound and its active salt have a certain therapeutic effect on hyperammonemia or liver disease, especially hepatic encephalopathy.

The object of the present invention is to provide a new improvement capable of preventing or delaying the transfer to hemodialysis therapy or reducing the number of implementations of the therapy in the case of renal dysfunction, and preventing or reducing the occurrence of hyperammonemia in the case of liver dysfunction Disease with food. In order to achieve this object, the disease-modifying food of the present invention is characterized by containing indigestible polysaccharides as the main component and limiting the addition of protein components. Thus, by using the energy generated by the absorption of indigestible polysaccharides, the urea or ammonia secreted into the intestines is used as the N source to synthesize bacterial protein, and the proliferated bacterial cells are excreted as feces to reduce the amount of nitrogen in the blood. Low-molecular nitrogen compounds, as a result, can improve renal dysfunction and liver dysfunction.

Some embodiments of the present application are directed to oral formulations of L- ornithine phenylacetate and methods of using the same. These oral formulations offer alternative administration route than the standard intravenous administration of L-ornithine phenylacetate for treating hyperammonemia in patients having various acute and chronic liver diseases and disorders, for example, acute liver failure, liver cirrhosis, liver decompensation, portal hypertension, hepatic encephalopathy, or patients with urea cycle disorders.

A compound containing o-naphthalene quinone structure for preparing medicines to prevent and treat hyperammonemia and hepatic encephalopathy is prepared from natural Masonate F through structure reformation and optimization.