Reduced glutathione and glutathione disulfide in the blood of glucose-6-phosphate dehydrogenase-deficient newborns

Abstract Background Glucose-6-phosphate dehydrogenase (G6PD) deficiency is commonly detected during mass screening for neonatal disease. We developed a method to measure reduced glutathione (GSH) and glutathione disulfide (GSSG) using tandem mass spectrometry (MS/MS) for detecting G6PD deficiency. M...

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Main Authors: Zhen-hua Gong, Guo-li Tian, Qi-wei Huang, Yan-min Wang, Hong-ping Xu
Format: Article
Language:English
Published: BMC 2017-07-01
Series:BMC Pediatrics
Subjects:
Online Access:http://link.springer.com/article/10.1186/s12887-017-0920-y
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author Zhen-hua Gong
Guo-li Tian
Qi-wei Huang
Yan-min Wang
Hong-ping Xu
author_facet Zhen-hua Gong
Guo-li Tian
Qi-wei Huang
Yan-min Wang
Hong-ping Xu
author_sort Zhen-hua Gong
collection DOAJ
description Abstract Background Glucose-6-phosphate dehydrogenase (G6PD) deficiency is commonly detected during mass screening for neonatal disease. We developed a method to measure reduced glutathione (GSH) and glutathione disulfide (GSSG) using tandem mass spectrometry (MS/MS) for detecting G6PD deficiency. Methods The concentration of GSH and the GSH/GSSG ratio in newborn dry-blood-spot (DBS) screening and in blood plus sodium citrate for test confirmation were examined by MS/MS using labeled glycine as an internal standard. Results G6PD-deficient newborns had a lower GSH content (242.9 ± 15.9 μmol/L)and GSH/GSSG ratio (14.9 ± 7.2) than neonatal controls (370.0 ± 53.2 μmol/L and 46.7 ± 19.6, respectively). Although the results showed a significance of P < 0.001 for DBS samples plus sodium citrate that were examined the first day after preparation, there were no significant differences in the mean GSH concentration and GSH/GSSG ratio between the G6PD deficiency-positive and negative groups when examined three days after sample preparation. Conclusion The concentration of GSH and the ratio of GSH/GSSG in blood measured using MS/MS on the first day of sample preparation are consistent with G6PD activity and are helpful for diagnosing G6PD deficiency.
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spelling doaj.art-ba0daf0aaa58432e9f330b7d4ba9bd242022-12-22T03:08:42ZengBMCBMC Pediatrics1471-24312017-07-011711610.1186/s12887-017-0920-yReduced glutathione and glutathione disulfide in the blood of glucose-6-phosphate dehydrogenase-deficient newbornsZhen-hua Gong0Guo-li Tian1Qi-wei Huang2Yan-min Wang3Hong-ping Xu4Department of general surgery, Shanghai Children’s Hospital, Shanghai Jiao Tong UniversityNeonatal screening center, Shanghai Children’s Hospital, Shanghai Jiao Tong UniversityDepartment of neonatology, Shanghai Children’s Hospital, Shanghai Jiao Tong UniversityNeonatal screening center, Shanghai Children’s Hospital, Shanghai Jiao Tong UniversityNeonatal screening center, Shanghai Children’s Hospital, Shanghai Jiao Tong UniversityAbstract Background Glucose-6-phosphate dehydrogenase (G6PD) deficiency is commonly detected during mass screening for neonatal disease. We developed a method to measure reduced glutathione (GSH) and glutathione disulfide (GSSG) using tandem mass spectrometry (MS/MS) for detecting G6PD deficiency. Methods The concentration of GSH and the GSH/GSSG ratio in newborn dry-blood-spot (DBS) screening and in blood plus sodium citrate for test confirmation were examined by MS/MS using labeled glycine as an internal standard. Results G6PD-deficient newborns had a lower GSH content (242.9 ± 15.9 μmol/L)and GSH/GSSG ratio (14.9 ± 7.2) than neonatal controls (370.0 ± 53.2 μmol/L and 46.7 ± 19.6, respectively). Although the results showed a significance of P < 0.001 for DBS samples plus sodium citrate that were examined the first day after preparation, there were no significant differences in the mean GSH concentration and GSH/GSSG ratio between the G6PD deficiency-positive and negative groups when examined three days after sample preparation. Conclusion The concentration of GSH and the ratio of GSH/GSSG in blood measured using MS/MS on the first day of sample preparation are consistent with G6PD activity and are helpful for diagnosing G6PD deficiency.http://link.springer.com/article/10.1186/s12887-017-0920-yGlucose-6-phosphate dehydrogenase deficiencyBloodGlutathioneTandem mass spectrometry
spellingShingle Zhen-hua Gong
Guo-li Tian
Qi-wei Huang
Yan-min Wang
Hong-ping Xu
Reduced glutathione and glutathione disulfide in the blood of glucose-6-phosphate dehydrogenase-deficient newborns
BMC Pediatrics
Glucose-6-phosphate dehydrogenase deficiency
Blood
Glutathione
Tandem mass spectrometry
title Reduced glutathione and glutathione disulfide in the blood of glucose-6-phosphate dehydrogenase-deficient newborns
title_full Reduced glutathione and glutathione disulfide in the blood of glucose-6-phosphate dehydrogenase-deficient newborns
title_fullStr Reduced glutathione and glutathione disulfide in the blood of glucose-6-phosphate dehydrogenase-deficient newborns
title_full_unstemmed Reduced glutathione and glutathione disulfide in the blood of glucose-6-phosphate dehydrogenase-deficient newborns
title_short Reduced glutathione and glutathione disulfide in the blood of glucose-6-phosphate dehydrogenase-deficient newborns
title_sort reduced glutathione and glutathione disulfide in the blood of glucose 6 phosphate dehydrogenase deficient newborns
topic Glucose-6-phosphate dehydrogenase deficiency
Blood
Glutathione
Tandem mass spectrometry
url http://link.springer.com/article/10.1186/s12887-017-0920-y
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