background: organic acids refer to a family of compounds that are intermediates in a variety of metabolic pathways. many organic acids are present in urine from clinically normal individuals. elevated levels of urine organic acids cause to the organic acidurias, disorders in which some metabolic pathways in organic acid metabolism are blocked. the present work identified major and minor urinary...

Background: Organic acids refer to a family of compounds that are intermediates in a variety of metabolic pathways. Many organic acids are present in urine from clinically normal individuals. Elevated levels of urine organic acids cause to the organic acidurias, disorders in which some metabolic pathways in organic acid metabolism are blocked. The present work identified major and minor urinary...

clinical differential diagnosisthe organic acidemias are important in the differential diagnosis of metabolic and neurologic derangement in the neonate and of new-onset neurologic signs in the older child. a-organic aciduriaseveral disorders, not classified as primary disorders of organic acid metabolism, have a characteristic urinary organic acid profile that suggests the appropriate diagnosis...

organic acid occur as physiologic intermediates in variety of intracellular metabolic pathways, such as catabolism of aminoacid, mitochondrial β oxidation of fatty acids, tricarboxilic acid cycle, and cholestrol and fatty acid biosynthesis. the classical organic aciduria represent the pursuit of abnormalities of aminoacid degradation beyond deamination their diagnostic hallmark is an accumulati...

We combined column and partition chromatography to isolate, purify, and quantify biological organic acids in urine and cerebrospinal fluid (CSF). Urine and CSF samples were introduced onto a preconditioned cation-exchange column (Dowex 50 W x 8 resin) to remove the biological interferences. The effluent with water was extracted with ethyl acetate two times (pH 1 and 3) and the organic acids wer...

We describe a method for adsorbing organic acids from amniotic fluid and urine specimens onto a column of silica gel before analysis by gas chromatography and by combined gas chromatography/mass spectrometry. Analytical recoveries of individual organic acids by our adsorption method compare favorably with those obtained by a more laborious manual extraction with ethyl acetate. Results for speci...

For some years now, urinary amino acid screening for inborn errors of metabolism has been routine in many institutions. In the early 1960’s, Cox and White (1) as well as Barness et a!. (2) showed the importance of measuring methylmalonate excretion in the urine of patients suffering from pernicious anemia. Since that time, detection of non-nitrogenous organic acids has taken on an importance si...

The simple titration method for the determination of organic acids in urine, described by Van Slyke and Palmer (I), has in many circumstances proved satisfactory. A more extended employment of the method to determine organic acids in pathological urines has brought to light limitations and sources of error which need consideration. The organic acids present in urine, both free and as salts, are...