Urinary Excretion of Pentose Phosphate Pathway-Associated Polyols in Early Postnatal Life

Koy, Anne; Waldhaus, Anne; Hammen, Hans-Werner; Wendel, U.; Mayatepek, Ertan; Schadewaldt, Peter

doi:10.1159/000167789

Cited by 6 publications

(3 citation statements)

References 30 publications

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“…High polyol concentrations have been observed during fetal life, possibly playing a protective role against the low-oxygen environment [ 27 ] and regulating the fluid balance by acting as organic osmolytes [ 28 ]. High urinary excretion of pentose phosphate pathway-associated polyols was reported after birth in both preterm and term neonates [ 29 ], although other studies showed significantly decreased polyols (mannitol/sorbitol arabitol/xylitol and xylose) in the urine of neonates with necrotizing enterocolitis [ 10 ] and sepsis [ 30 ]. Interestingly, increased polyols including arabitol in the urine and other body fluids have been described in inborn errors of the pentose phosphate pathway involving deficiencies in ribose 5-phosphate isomerase or transaldolase [ 26 , 31 ].…”

Section: Discussionmentioning

confidence: 99%

A Cohort Study of Gastric Fluid and Urine Metabolomics for the Prediction of Survival in Severe Prematurity

et al. 2023

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Predicting survival in very preterm infants is critical in clinical medicine and parent counseling. In this prospective cohort study involving 96 very preterm infants, we evaluated whether the metabolomic analysis of gastric fluid and urine samples obtained shortly after birth could predict survival in the first 3 and 15 days of life (DOL), as well as overall survival up to hospital discharge. Gas chromatography–mass spectrometry (GC-MS) profiling was used. Uni- and multivariate statistical analyses were conducted to evaluate significant metabolites and their prognostic value. Differences in several metabolites were identified between survivors and non-survivors at the time points of the study. Binary logistic regression showed that certain metabolites in gastric fluid, including arabitol, and succinic, erythronic and threonic acids, were associated with 15 DOL and overall survival. Gastric glyceric acid was also associated with 15 DOL survival. Urine glyceric acid could predict survival in the first 3 DOL and overall survival. In conclusion, non-surviving preterm infants exhibited a different metabolic profile compared with survivors, demonstrating significant discrimination with the use of GC-MS-based gastric fluid and urine analyses. The results of this study support the usefulness of metabolomics in developing survival biomarkers in very preterm infants.

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Section: Discussionmentioning

confidence: 99%

A Cohort Study of Gastric Fluid and Urine Metabolomics for the Prediction of Survival in Severe Prematurity

et al. 2023

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“…According to previously reported data, the normal value for galactose in whole blood was set at ≤ 240 μmol/l (Rommel et al 1968). Galactose-1-phosphate and galactitol in red blood cells (RBC) as well as galactose and galactitol in urine were measured by stable-isotope dilution analyses as recently described (Koy et al 2009;Schadewaldt et al 2003a, b). According to previously published data, the upper cut-off level for galactitol in RBC was set at 0.8± 0.2 μmol/l (Schadewaldt et al 2003a, b).…”

Section: Methodsmentioning

confidence: 99%

“…According to previously published data, the upper cut-off level for galactitol in RBC was set at 0.8± 0.2 μmol/l (Schadewaldt et al 2003a, b). Age-related reference values for galactitol in urine were determined by random sampling in healthy neonates and infants, and by postabsorptive sampling in healthy children, adolescents, and adults in a total of 225 samples (Koy et al 2009;Schadewaldt et al 2003a, b).…”

Section: Methodsmentioning

confidence: 99%

Features and outcome of galactokinase deficiency in children diagnosed by newborn screening

Hennermann

Schadewaldt

Vetter

et al. 2011

J of Inher Metab Disea

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Galactokinase deficiency (GALK-D), an autosomal recessive disorder in the Leloir pathway, results in accumulation of galactose, galactitol, and galactonate and leads to early onset of juvenile bilateral cataract. Highest incidence of GALK-D is found in Romani populations. The migration wave due to the Yugoslavian civil war has changed the spectrum of inborn errors of metabolism within Europe. Hence, newborn screening (NBS) in the Berlin region, performed from 1991 until 2010 in 683,675 neonates, revealed an increased incidence of GALK-D of 1:40,000, comparable to that of galactose-1-phosphate-uridyltransferase deficiency. A total of 44% of GALK-D patients were of Romani origin. All patients of Bosnian or Serbian origin were homozygous for the Romani founder mutation p.P28T. Detection of GALK-D by NBS and early start of galactose-restricted diet resulted in regression or prevention of cataracts. Slight cataracts without visual impairment occurred in 50% of the patients, 56% of whom were noncompliant. Further clinical symptoms, e.g., hypoglycemia, mental retardation, microcephaly, and failure to thrive, were associated with noncompliance. With treatment, galactose in blood decreased from 8,892 ± 5,243 to 36.5 ± 49.3 μmol/l, galactose in urine from 31,820 ± 32,103 to 30.0 ± 36.1 μmol/mmol creatinine, galactitol in RBC from 1,584 ± 584 to 12.3 ± 9.4 μmol/l, and galactitol in urine from 11,724 ± 4,496 to 236 ± 116 μmol/mmol creatinine. This is the first presentation of outcome and clinical features in GALK-D patients diagnosed by NBS. As our data suggest, GALK-D should be considered for inclusion in NBS in populations expected to have substantial numbers of GALK-D carriers, e.g., Yugoslavian immigrants.

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Direct analysis of polyols using 3-nitrophenylboronic acid in capillary electrophoresis: thermodynamic and electrokinetic principles of molecular recognition

Fei

Britz‐McKibbin

2010

Anal Bioanal Chem

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The design of boronic acid sensors for photometric detection of carbohydrates has relied on exploiting differences in the thermodynamic stability of complex formation for molecular recognition. Herein, we introduce a direct method for analysis of sugar alcohols using 3-nitrophenylboronic acid (NPBA) as an electrokinetic probe in capillary electrophoresis (CE). Dynamic complexation of neutral polyols by NPBA during electromigration allows for their simultaneous resolution and UV detection based on formation of an anionic ternary boronate ester complex in phosphate buffer. Unlike conventional boronic acid sensors, thermodynamic and electrokinetic processes in CE allow for improved selectivity for the resolution of sugar alcohol stereoisomers having different vicinal polyol chain lengths even in cases when binding affinity is similar due to differences in their complex mobility. Three complementary approaches were investigated to compare the thermodynamics of polyol chelation with NPBA, namely direct binding assays by CE, UV absorbance spectroscopy and an indirect pK (a) depression method. Overall, CE offers a convenient platform for characterization of reversible arylboronic acid interactions in free solution while allowing for direct analysis of complex mixtures of neutral/UV-transparent polyols without complicated sample handling.

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Urinary Excretion of Pentose Phosphate Pathway-Associated Polyols in Early Postnatal Life

Cited by 6 publications

References 30 publications

A Cohort Study of Gastric Fluid and Urine Metabolomics for the Prediction of Survival in Severe Prematurity

A Cohort Study of Gastric Fluid and Urine Metabolomics for the Prediction of Survival in Severe Prematurity

Features and outcome of galactokinase deficiency in children diagnosed by newborn screening

Direct analysis of polyols using 3-nitrophenylboronic acid in capillary electrophoresis: thermodynamic and electrokinetic principles of molecular recognition

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