Quantitative LC-MS/MS analysis kit for Organic Acids in human urine samples.

Organic acid disorders (organic acidemia) are a group of inherited metabolic conditions. Most of the organic acidemias caused by defective autosomal genes for various enzymes for amino acid metabolism. Neurological and physiological harms are caused by this impaired ability to synthesize a key enzyme required to break down a specific amino acid, or a group of amino acids, resulting in acidemia and toxicity to specific organs systems. Most are inherited as autosomal recessive diseases. Organic acid related disorders may affect many metabolic pathways including amino acid, lipid metabolism, fatty acid oxidation and the Krebs cycle.

Organic acidemias are usually diagnosed in infancy, characterized by urinary excretion of abnormal amounts or types of organic acids. The diagnosis is usually made by detecting an abnormal pattern of organic acids in a urine sample. In some conditions, the urine is always abnormal, in the others the characteristic substances are only present intermittently. Many of the organic acidemias are detectable by newborn screening with Tandem Mass spectrometry. These disorders vary in their prognosis, from manageable to fatal, and usually affect more than one organ system, especially the central nervous system.

Quantitative analysis of the organic acids in urine samples have clinical importance for determining organic acidemias and monitoring the organic acid concentrations in human urine. ZIVAK Quantitative Organic Acid LC-MS/MS kit was developed for quantitative detection of the  major organic acids in human urine samples. LC-MS/MS is a influential device for the study of metabolic disorders and LC-MS/MS analysis gives results in 20 minutes with minimal sample preparation. Main methods and procedures that we selected are based on EN ISO 13485 and 98/79/EC.