Galactose metabolites in blood from neonates with and without hypergalactosaemia detected by mass screening

Mizoguchi, Nobuyuki; Ono, Hiroaki; Eguchi, Takaatsu; Sakura, Nobuo

doi:10.1007/pl00008352

Cited by 6 publications

(5 citation statements)

References 13 publications

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“…In 55 normal infants receiving more than 80 kcal/kg/day as breast milk or lactose rich formula, blood galactose levels in the 20 min after feeding were 0.08 8 0.01 mmol/l (1.5 8 0.2 mg/dl), ranging from 0.044 mmol/l (0.8 mg/dl) to 0.23 mmol/l (4.2 mg/dl) [4] . And in eighteen 18-28-dayold normal neonates, levels were 0.05 8 0.039 mmol/l (0.96 8 0.71 mg/dl), with galactose determined by enzymatic methods [5] . Galactose levels in our 48-hour-old newborns are similar to those of Mizoguchi et al [5] and lower than those found by Siegel et al [4] ; however, differences in protocols must be considered, such as infant age, the time after milk ingestion when samples were collected, and type of milk.…”

Section: Discussionmentioning

confidence: 99%

“…And in eighteen 18-28-dayold normal neonates, levels were 0.05 8 0.039 mmol/l (0.96 8 0.71 mg/dl), with galactose determined by enzymatic methods [5] . Galactose levels in our 48-hour-old newborns are similar to those of Mizoguchi et al [5] and lower than those found by Siegel et al [4] ; however, differences in protocols must be considered, such as infant age, the time after milk ingestion when samples were collected, and type of milk. Galactose and glucose concentrations are higher in preterm or full-term formulas than breast milk [27] .…”

Section: Discussionmentioning

confidence: 99%

“…Immediately after full-term delivery, mean maternal blood galactose level was 0.08 8 0.03 mmol/l. For normal adults, value is 0.05 mmol/l (0.96 mg/dl) [5] .…”

Section: Discussionmentioning

confidence: 99%

“…Therefore galactose, through hepatic glycogen, may be an important store of glucose in newborns. The few references to galactose blood levels in newborns are mostly related to overload tests for identifying galactosemia and are determined by less precise methods [5] . Some information is available on clinically using galactose instead of glucose to treat hypoglycemia [6] .…”

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confidence: 99%

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Blood Galactose and Glucose Levels in Mothers, Cord Blood, and 48-Hour-Old Breast-Fed Full-Term Infants

Bossolan

Trindade²,

Barreiros³

2006

Neonatology

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Background: Although galactose is an important component in human lactose, there are few reports of its role in the newborn metabolism. Objective: To determine the relationship of blood galactose and glucose levels in mothers, cord blood, and breast-fed full-term newborn infants. Methods: Maternal and cord vein blood samples were obtained from 27 pregnant women at delivery, and from their breast-fed, full-term newborns 48 h later. Galactose and glucose were determined by HPLC. Statistical analysis used ANOVA and Pearson correlation with p < 0.05. Results: Maternal galactose concentrations (0.08 ± 0.03 mmol/l) were similar to cord blood galactose (0.07 ± 0.03 mmol/l; p = 0.129). However, newborn blood galactose (0.05 ± 0.02 mmol/l) was significantly lower than both cord (p = 0.042) and maternal blood (p = 0.002). Maternal blood glucose levels (4.72 ± 0.86 mmol/l) were higher than cord blood (3.98 ± 0.57 mmol/l; p < 0.001), and cord blood concentrations were higher than newborn blood levels (3.00 ± 0.56 mmol/l; p < 0.001); all values expressed as mean ± SD. Significant correlation was only seen between maternal and cord blood galactose levels (r = 0.67; p < 0.001) and glucose levels (r = 0.38; p = 0.047). Conclusion: The association and similarity between maternal and cord blood galactose levels suggest that the fetus is dependent on maternal galactose. In contrast, the lower galactose levels in newborn infants and a lack of association between both suggest self-regulation and a dependence on galactose ingestion.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Immediately after full-term delivery, mean maternal blood galactose level was 0.08 8 0.03 mmol/l. For normal adults, value is 0.05 mmol/l (0.96 mg/dl) [5] .…”

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Blood Galactose and Glucose Levels in Mothers, Cord Blood, and 48-Hour-Old Breast-Fed Full-Term Infants

Bossolan

Trindade²,

Barreiros³

2006

Neonatology

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“…12 Galactose, glucose, and glucitol were increased in most patients with citrin deficiency 10,22,25 but not in BA, 11 argininosuccinate synthetase deficiency or other hyperammonemias associated with defects of the urea cycle. 22 Galactose was also elevated in the blood of patients with hypergalactosemia, such as, patients with RBC galactose-1-phosphate uridyltransferase (Gal-1-P), RBC uridine diphosphate glucose epimerase deficiency (UDP-Glc), portacaval shunts, and congenital BA, 26 in which Cit is not elevated. A portosystemic shunt is one of the main causes of persistent hypergalactosemia without enzyme deficiency.…”

Section: Discussionmentioning

confidence: 99%

Neonatal Intrahepatic Cholestasis Caused by Citrin Deficiency Differentiated from Biliary Atresia

Tian

Zhang

et al. 2015

Eur J Pediatr Surg

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Purpose The aim of this article is to differentiate neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) from biliary atresia (BA) by total hexose. Methods A total of 11 patients with NICCD, 29 patients with BA, and 4,898 children as controls were involved in this study. The blood concentration of amino acids, carnitine, acylcarnitines, and total hexose were measured in dry blood spots (DBS) using tandem mass spectrometry (MS/MS). Results In the patients with NICCD, the blood concentration of the total hexose (15.3 ± 9.0 mmol/L vs. 7.3 ± 2.7 mmol/L; p < 0.001), citrulline (Cit) (197.9 ± 93.7 µmol/L vs. 17.5 ± 7.4 µmol/L; p < 0.001) were higher than those of patients with BA. Using total hexose (> 10 mmol/L), Cit (> 55 µmol/L) to diagnose NICCD, the sensitivity and specificity were 66.7 and 97.8% and 90.0 and 99.1%, respectively, and all of the areas under the receiver-operating characteristic curves were greater than 0.85. Conclusion Elevated total hexose in DBS measured by MS/MS associated with elevated amino acids, especially Cit can be used to diagnose NICCD and differentiate it from BA.

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Development of a new diagnostic method for galactosemia by high-performance anion-exchange chromatography with pulsed amperometric detection

Jeong

Yoon

Hong

2007

Journal of Chromatography A

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Galactose metabolites in blood from neonates with and without hypergalactosaemia detected by mass screening

Abstract: In neonates, uridine diphosphate galactose concentrations were higher and uridine diphosphate glucose concentrations were lower than previously reported paediatric values. Patients with high plasma galactose concentrations should be investigated by hepatic imaging.

Cited by 6 publications

References 13 publications

Blood Galactose and Glucose Levels in Mothers, Cord Blood, and 48-Hour-Old Breast-Fed Full-Term Infants

Blood Galactose and Glucose Levels in Mothers, Cord Blood, and 48-Hour-Old Breast-Fed Full-Term Infants

Neonatal Intrahepatic Cholestasis Caused by Citrin Deficiency Differentiated from Biliary Atresia

Development of a new diagnostic method for galactosemia by high-performance anion-exchange chromatography with pulsed amperometric detection

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