Gas-chromatographic method of analysis for urinary organic acids. II. Description of the procedure, and its application to diagnosis of patients with organic acidurias.

1980 ◽  
Vol 26 (13) ◽  
pp. 1847-1853 ◽  
Author(s):  
K Tanaka ◽  
A West-Dull ◽  
D G Hine ◽  
T B Lynn ◽  
T Lowe
Keyword(s):  
Organic Acids ◽  
Chromatographic Method ◽  
Normal Subjects ◽  
Retention Indices ◽  
Organic Aciduria ◽  
Organic Acidurias ◽  
Urinary Organic Acids ◽  
Urinary Organic Acid Analysis ◽  
Gas Chromatographic Method ◽  
Extensive List

Abstract A gas-chromatographic method for urinary organic acid analysis is described, designed to be used routinely for the diagnosis of organic aciduria. It involves extraction of urine with ethyl acetate, dehydration of extract residues, and trimethylsilylation. Organic acids are identified by using an extensive list of retention indices published in the accompanying paper (this issue). Quantitative values are given for organic acids in urines from 50 ostensibly normal subjects. Typical chromatograms of urinary organic acids from patients with eight well-established organic acidurias are also shown.

Gas-chromatographic method of analysis for urinary organic acids. I. Retention indices of 155 metabolically important compounds.

1980 ◽  
Vol 26 (13) ◽  
pp. 1839-1846 ◽  
Author(s):  
K Tanaka ◽  
D G Hine ◽  
A West-Dull ◽  
T B Lynn
Keyword(s):  
Organic Acids ◽  
Metabolic Diseases ◽  
Retention Indices ◽  
Chromatographic Retention ◽  
Organic Acidurias ◽  
Gas Chromatographic Retention Indices ◽  
Chromatographic Retention Indices ◽  
Methylene Unit ◽  
Trimethylsilyl Derivatives ◽  
Gas Chromatographic Method

Abstract Gas-chromatographic retention indices are given, in terms of methylene units, for 155 metabolically important compounds (mostly organic acids) as trimethylsilyl derivatives on 10% OV-1 and 10% OV-17 columns. Comprehensive references on metabolic diseases that can be diagnosed by detection of these metabolites are cross-indexed to facilitate the use of the methylene-unit list. With the data presented here, it is now possible to diagnose more than 25 well-defined organic acidurias by use of gas chromatography alone.

Metabolic profiles of urinary organic acids recovered from absorbent filter paper.

1987 ◽  
Vol 33 (4) ◽  
pp. 572-576 ◽  
Author(s):  
B A Chamberlin ◽  
C C Sweeley
Keyword(s):  
Young Children ◽  
Organic Acids ◽  
Filter Paper ◽  
Metabolic Profiles ◽  
Sample Storage ◽  
Organic Acidurias ◽  
Urinary Organic Acids ◽  
Specimen Collection ◽  
Normal Urine

Abstract We evaluated for reliability and reproducibility a semiquantitative gas-chromatographic assay of organic acids in samples of normal urine recovered from absorbent filter paper. We also evaluated this method for use in diagnosis of some of the more common organic acidurias. Transfer of urine from diapers to absorbent filter paper eases the usual trauma of specimen collection from young children; it also simplifies sample storage and shipment.

scholarly journals Statistical Optimization of Urinary Organic Acids Analysis by a Multi-Factorial Design of Experiment

Analytica ◽  
2020 ◽  
Vol 1 (1) ◽  
pp. 14-23 ◽  
Author(s):  
Marco Pazzi ◽  
Sara Colella ◽  
Eugenio Alladio ◽  
M. Paola Puccinelli ◽  
Giulio Mengozzi ◽  
...  
Keyword(s):  
Principal Component Analysis ◽  
Linear Regression ◽  
Organic Acids ◽  
Design Of Experiment ◽  
Inborn Errors Of Metabolism ◽  
Principal Component ◽  
Inborn Errors ◽  
Organic Acidurias ◽  
Urinary Organic Acids ◽  
High Yields

The analysis of urinary organic acids is useful for patients suspected to have inborn errors of metabolism known as organic acidurias. These diseases cause an accumulation of organic acids in body fluids and their abnormal excretion in urines. By means of chemometrics tools, such as principal component analysis and multiple linear regression, it was concluded that the conditions used in our laboratory are really the most suitable to achieve high yields of analytes.

CONGENITAL ABNORMALITIES OF AMINO ACID TRANSPORT IN RENAL TUBULES

PEDIATRICS ◽  
1958 ◽  
Vol 21 (6) ◽  
pp. 1039-1046
Author(s):  
Charles U. Lowe
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Organic Acids ◽  
Congenital Abnormalities ◽  
Organic Aciduria ◽  
Elevated Concentration ◽  
Renal Tubules ◽  
Organic Acidurias

VARIETIES of syndromes have been described in which an abnormal amount of organic acid appears in the urine. In many of those syndromes which have been carefully studied, amino acids appear to constitute the major portion of organic acids. This probably is the result of the development of adequate methodology for the determination of amino acids. Nevertheless the author proposes classifying these diseases as organic acidurias, since it is his belief that there are organic acids, other than amino acid, present in the urine of these patients. Although the central theme of the Symposium of which this paper is a part is genetic defects, in the classification which is to be presented for consideration, some syndromes are included which are not genetic in origin. This is done for the purpose of completeness and to suggest the relationships between the syndromes with a genetic background and those in which the organic aciduria results from some nongenetically controlled aberrations. These syndromes may be roughly divided into two groups: Those in which the concentration of amino acids in the blood is elevated and those in which it is normal. The problem of classification of these syndromes has recently been reviewed in detail. In the group of syndromes with normal concentrations in the blood, further subdivision is necessary into four apparently different groups. In Table I are listed the diseases falling into these various categories. Among the diseases characterized by elevated concentration of organic acids in the blood, one may observe that only phenylpyruvic oligophrenia represents a congenital anomaly.

Urinary organic acids in man. I. Normal patterns.

1976 ◽  
Vol 22 (8) ◽  
pp. 1283-1287 ◽  
Author(s):  
A M Lawson ◽  
R A Chalmers ◽  
R W Watts
Keyword(s):  
Organic Acids ◽  
Human Urine ◽  
Organic Acidurias ◽  
Urinary Organic Acids ◽  
Qualitative Pattern

Abstract We studied qualitative pattern of urinary acidic metabolites excreted by normal persons. The results provide a basis on which to compare results for patients with potentially abnormal organic acidurias. A series of urinary polyhydroxy (aldonic and deoxyaldonic) acids has been identified. Most of these compounds have not been previously reported in human urine, except in connection with the present work, and are additional to the previously recognized urinary organic acids, which were also observed. Possible metabolic origins of some of the acids are briefly discussed.

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