Low Molecular Weight Organic Acids in the Urine of the Newborn

Gregersen, N.; Ingerslev, J.; Rasmussen, Karsten

doi:10.1111/j.1651-2227.1977.tb07812.x

Cited by 32 publications

(26 citation statements)

References 14 publications

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“…There are no major discrepancies between the results of these studies and the present one, but comparisons are complicated by differences in sampling periods and analytical technique. The study of Gregersen et al, (1977) is very similar in design to ours, and the results also agree closely except for an unexplained higher urine concentration of citrate in the present study.…”

Section: Discussionsupporting

confidence: 90%

“…Quantitative data on organic acids in urine from healthy newborns have recently been .published by other groups (Aksu et al, 1974;Bjorkman et al, 1976;Gregersen et al, 1977). There are no major discrepancies between the results of these studies and the present one, but comparisons are complicated by differences in sampling periods and analytical technique.…”

Section: Discussionmentioning

confidence: 50%

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Concentrations of Organic Acids in the Urine of Healthy Newborn Children

1978

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analysed as methoxime derivatives, and methylation is used to prepare volatile derivatives of the acids. Ether extraction of the organic acids is made from a small volume of acid water phase that has been adsorbed by anhydrous sodium sulphate to improve the extraction of polar compounds. Biochemistry 1978, 15, 245-249 Concentrations of organic acids in the urine of healthy newborn children SUMMARY Urine concentrations of organic acids were determined by a gas chromatographic technique in healthy, full-term neonates at the ages of 2 and 5 days. Significant reductions in the interindividual variation were obtained by relating the levels of organic acids to urine creatinine concentration or osmolality. Citrate was the predominant organic anion found. Urine concentrations of fumarate, 3-hydroxy-3-methylglutarate, adipate, and suberate decreased during the first week of life, and their concentrations were significantly intercorrelated. It is suggested that these findings reflect the predominantly fat-utilising, hyperketonaemic state during the first days of life. The urine concentrations of the aromatic end-products, p-hydroxyphenylacetate and hippurate, increased during the first week of life, probably as an effect of dietary factors. Annuals of ClinicalIt is concluded that analysis of intermediary organic acids in urine could be of value in the assessment of the metabolic state in neonates.Gas chromatography of urinary organic acids has been extensively used in the screening for hereditary metabolic diseases (Dalgliesh et al., 1966;Jellum et al., 1971; Marner et aI., 1971; Bjorkman et al., 1976). A genetic enzyme deficiency leads to the excretion of one or several metabolites in concentrations that are usually orders of magnitude greater than normal. Quantitative aspects of the methods Material and methods employed have therefore not been of primary interest, and there is a paucity of data regarding the SUBJECTS urine concentrations of organic acids in the healthy Urine specimens were obtained from 26 healthy newborn. A study of the excretion of organic acids newborns, 18 boys and 8 girls. The samples were in the neonatal period could also be of interest collected with the informed consent of the parents. from other points of view than the diagnosis of The children included in the study were selected genetic metabolic defects. The maturation of to meet the following criteria: (a) the gestational enzyme systems and the adaptation to oral feeding age at spontaneous delivery estimated from the are processes that may well be reflected in the levels mother's last menstrual period between 38 and of intermediary metabolites in the urine.42 weeks (observed 279 ± 7 days, mean ± SD); In the present study we have determined the urine (b) weight and crown-to-heel length within ± 2 concentrations of organic acids in healthy newborns standard deviations from the mean for the gestational at the ages of 2 and 5 days. These ages were chosen age (observed weight 3750 ± 290 g and crown-tobecause screening for pathological metabol...

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Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 50%

Concentrations of Organic Acids in the Urine of Healthy Newborn Children

1978

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“…To investigate this hypothesis in hypoglycemic infants, we measured the urinary excretion of dicarboxylic acids in hypoglycemic and normoglycemic infants of group II. The val ues detected in the present study are slightly lower than those previously reported in neo nates [18,19]; the differences may be ex plained by the early timing in the present study and by the influence of feeding in the other reports. In the hypoglycemic infants, we did not observe an elevated urinary ex cretion of dicarboxylic acids in comparison to the normoglycemic infants.…”

Section: Discussioncontrasting

confidence: 56%

Ketogenesis in Hypoglycemic Neonates

Sann

Divry²,

B³

et al. 1987

Neonatology

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Since hypoglycemic neonates do not exhibit compensative ketosis, we investigated the possible involvement of carnitine deficiency or Ω-oxidation in neonatal hypoglycemia. In a first group of 49 neonates, serum free fatty acid, acetoacetate and β-hydroxybutyrate concentrations were similar in hypoglycemic and normoglycemic neonates. Serum free carnitine concentrations did not show any difference in the hypoglycemic small-for-date infants (median 40 μmol/l, range 16–92 μmol/l) compared to the normoglycemic small-for-date infants (median 30 μmol/l, range 8–64 μmol/l). In a second group of 45 neonates, urinary excretion of dicarboxylic acids (adipic, suberic, sebaric and succinic acids) was similar in hypoglycemic infants compared to normoglycemic neonates. Despite the limitations of interpretaion of free carnitine determination, these data do not suggest an impaired β-oxidation by carnitine depletion or an enzymatic defect in hypoglycemic neonates.

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“…Lactic and 3-OH-butyric acids in d . , urine were determined by gas chromatography as tr~methylsilyl-derivatives after extraction with ethvlacetate (10). Measurement of glutaric and 3-OH-glutaric acidiin urine was performed by means of gas chromatography after ion exchange and trimethylsilylation, as described by Thompson and Markey (24).…”

Section: Methodsmentioning

confidence: 99%

Ketotic Episodes in Glutaryl-CoA Dehydrogenase Deficiency (Glutaric Aciduria)

Gregersen

Brandt

1979

Pediatr Res

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Summawthe ketoacidosis, a pronounced lactic acidosis and lactic aciduria A 7-yr-old boy with glutaryl-CoA dehydrogenase deficiency (glutaric aciduria), presenting periodic episodes of lethargy and ketosis, was studied during two such episodes. The urinary excretions of glutaric and 3-OH-glutaric acids were 3100-7900 and 460-660 &mg creatinine, respectively, during these episodes. Urine samples collected before and after the attacks contained 100-5300 and 230-370 &mg creatinine of glutaric acid and 3-OH-glutaric acids, respectively. During the episodes, glutaconic acid excretion rose from 14-89 to 93-630 pg/mg creatinine. Adipic acid and suberic acid excretions were increased from 17-100 and 2-13 to 74-224 and 16-32 &mg creatinine, respectively. The excretions of conjugates of propionic, isobutyric, 2-Me-butyric, and isovaleric acids, outside and during the attacks, respectively, were: propionic acid, 7-13 and 5-19 &mg creatinine isobutyric acid, 1-7 and 3-15 pg/mg creatinine; 2-Me-butyric acid, 2-9 and 4-34 pg/mg creatinine; isovaleric acid, 0-6 and 3-11 pg/mg creatinine. The excretion of total 2-0x0-acids was increased from 0-12 to 29-75 pg/mg creatinine during the episodes. It is argued that one or more compounds, accumulated as a result of the primary glutarylCoA dehydrogenase deficiency, inhibit the oxidation of the branched-chain amino acids and the fatty acids. During excessive catabolism of amino acids and fatty acids this inhibition results in accumulation of metabolites that are excreted in the urine as conjugates and C6-Clodicarboxylic acids, respectively. Short-and medium-chain monocarboxylic acids are known to affect energy metabolism, and it is, therefore, argued that the short-chain monocarboxylic acids, shown to be accumulated in the patient during the ketotic episodes, may have some pathophysiologic significance. SpeculationKetotic episodes with lethargy and severe hypotonia are another feature of glutaric aciduria due to glutaryl-CoAdehydrogenase deficiency. During such episodes inhibition of the metabolism of branched-chain amino acids and fatty acids, secondary to the inability to metabolize glutaric acid, causes accumulation of branched short-chain monocarboxylic acids and of short-and medium-chain dicarboxylic acids.Straight short-and medium-chain fatty acids influence the mitochondria1 energy metabolism, and it is, therefore, speculated that the short-chain monocarboxylic acids, indicated in the present report to be accumulated in the mitochondria of the patient, contribute to the pathophysiology of the ketotic states.Severe ketoacidotic episodes are a well known feature in a number of the organic acidurias. Patients with propionic, isovaleric, and methylmalonic acidurias are often subject to recurrent crises induced by high protein intake or by infections (13,15,21). The clinical and biochemical presentations of these crises resemble one another closely: vomiting and lethargy followed by neurologic symptoms eventually leading to coma, and often to death. Besides is seen in most cases. The ...

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Low Molecular Weight Organic Acids in the Urine of the Newborn

Cited by 32 publications

References 14 publications

Concentrations of Organic Acids in the Urine of Healthy Newborn Children

Concentrations of Organic Acids in the Urine of Healthy Newborn Children

Ketogenesis in Hypoglycemic Neonates

Ketotic Episodes in Glutaryl-CoA Dehydrogenase Deficiency (Glutaric Aciduria)

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