Dataset Information

Use of waxy maize heat modified starch in the treatment of children between 2 and 5?years with glycogen storage disease type I: A retrospective study.

ABSTRACT: Background:Glycogen storage disease type I (GSDI) is caused by deficiency of the enzyme glucose-6-phosphatase or glucose-6-phosphate transporter. Mainstay of treatment is provision of uncooked cornstarch (and/or continuous nocturnal pump feed (CNPF) to maintain normoglycemia). Waxy maize heat modified starch (WMHMS) is another treatment option to maintain normoglycemia overnight. Our objective was to describe our experience treating children 2-5?years of age with GSDI using WMHMS overnight. Method:This is a retrospective case series review (n?=?5) comparing the overnight feeding regimen and biochemical control one year before and after nocturnal WMHMS therapy. The WMHMS trial, in which blood glucose and lactate levels were monitored hourly, is reported in detail. Results:Most patients successfully transitioned to nocturnal WMHMS feeds. These patients had stable glucose and lactate throughout the overnight period, permitting a fasting period of 6.5-8?h overnight. Within the time period studied, WMHMS appeared to have improved overnight control of blood glucose levels with fewer reported episodes of hypoglycemia compared to CNPF. Conclusion:WMHMS can be an effective substitute treatment to achieve stable nocturnal glucose control in children younger than five years of age. A larger multicenter prospective study is recommended to establish stronger evidence of the efficacy and safety of using WMHMS in treatment of young children with GSDI.

SUBMITTER: Hijazi G

PROVIDER: S-EPMC6895741 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Use of waxy maize heat modified starch in the treatment of children between 2 and 5 years with glycogen storage disease type I: A retrospective study.

Hijazi Ghada G Pai Nisha N Nagy Laura L LL Herd Sarah S Dickson Jolynn J Ram Maya M Inbar-Feigenberg Michal M

Molecular genetics and metabolism reports 20191106

<h4>Background</h4>Glycogen storage disease type I (GSDI) is caused by deficiency of the enzyme glucose-6-phosphatase or glucose-6-phosphate transporter. Mainstay of treatment is provision of uncooked cornstarch (and/or continuous nocturnal pump feed (CNPF) to maintain normoglycemia). Waxy maize heat modified starch (WMHMS) is another treatment option to maintain normoglycemia overnight. Our objective was to describe our experience treating children 2-5 years of age with GSDI using WMHMS overnig ...[more]

PMID: 31844626

Similar Datasets

Project description:This work investigates the stability of emulsions prepared by using octenyl succinic anhydride (OSA)-modified waxy maize starch in the form of granules, dissolved starch, and non-solvent precipitated starch as Pickering emulsion stabilisers. The aim of this study was to investigate the effects of different forms of starches on the stability of emulsion using light microscopy, light scattering, and static multiple light scattering. All starch samples were hydrophobically modified with 3% (w/w) n-octenyl succinyl anhydride (OSA). Starch polymer solutions were prepared by dissolving OSA- modified starch in water in an autoclave at 140°C. Non-solvent precipitates were obtained through ethanol precipitation of dissolved waxy maize. The stability of the oil/water emulsions were different for the three forms of starches used. The granule-based emulsions were unstable, with only a small proportion of the granules adsorbed onto oil droplets, as viewed under a light microscope. The emulsions were observed to cream after 2 hours. The dissolved starch and non-solvent precipitate-based emulsions were stable towards creaming for months, and they had almost 100% emulsifying index (EI = 1) by visual observation and EI ~ 0.9 by multiple light scattering measurements. The results from light microscopy and multiple light scattering measurements indicated the occurrence of coalescence for all three types of emulsions. The coalescence was fastest within days for the granule stabilised system while it was slower both for the dissolved starch and non-solvent precipitate-based emulsions. The latter demonstrated the least degree of coalescence over time. Thus, it was concluded that differences in starch particle size and molecular structure influenced the emulsion droplet size and stability. A decreased particle size correlates to a decrease in droplet size, thus increasing stabilisation against creaming. However, stability towards coalescence was low for the large granules but was best for the non-solvent precipitate starch indicating that there is a window of optimal particle size for stability. Thus, best emulsifying properties were obtained with the non-solvent precipitates (~ 120 nm particle size) where the emulsions remained stable after one year of storage. In conclusion, this study illustrated the potentiality of non-solvent precipitated starch as emulsion stabilizers.

Project description:BackgroundKernel development and starch formation are the primary determinants of maize yield and quality, which are considerably influenced by drought stress. To clarify the response of maize kernel to drought stress, we established well-watered (WW) and water-stressed (WS) conditions at 1-30 days after pollination (dap) on waxy maize (Zea mays L. sinensis Kulesh).ResultsKernel development, starch accumulation, and activities of starch biosynthetic enzymes were significantly reduced by drought stress. The morphology of starch granules changed, whereas the grain filling rate was accelerated. A comparative proteomics approach was applied to analyze the proteome change in kernels under two treatments at 10 dap and 25 dap. Under the WS conditions, 487 and 465 differentially accumulated proteins (DAPs) were identified at 10 dap and 25 dap, respectively. Drought induced the downregulation of proteins involved in the oxidation-reduction process and oxidoreductase, peroxidase, catalase, glutamine synthetase, abscisic acid stress ripening 1, and lipoxygenase, which might be an important reason for the effect of drought stress on kernel development. Notably, several proteins involved in waxy maize endosperm and starch biosynthesis were upregulated at early-kernel stage under WS conditions, which might have accelerated endosperm development and starch synthesis. Additionally, 17 and 11 common DAPs were sustained in the upregulated and downregulated DAP groups, respectively, at 10 dap and 25 dap. Among these 28 proteins, four maize homologs (i.e., A0A1D6H543, B4FTP0, B6SLJ0, and A0A1D6H5J5) were considered as candidate proteins that affected kernel development and drought stress response by comparing with the rice genome.ConclusionsThe proteomic changes caused by drought were highly correlated with kernel development and starch accumulation, which were closely related to the final yield and quality of waxy maize. Our results provided a foundation for the enhanced understanding of kernel development and starch formation in response to drought stress in waxy maize.

Dataset Information

Use of waxy maize heat modified starch in the treatment of children between 2 and 5?years with glycogen storage disease type I: A retrospective study.

Publications

Use of waxy maize heat modified starch in the treatment of children between 2 and 5 years with glycogen storage disease type I: A retrospective study.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets

Dataset Information

Use of waxy maize heat modified starch in the treatment of children between 2 and 5?years with glycogen storage disease type I: A retrospective study.

Publications

Use of waxy maize heat modified starch in the treatment of children between 2 and 5 years with glycogen storage disease type I: A retrospective study.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets

Use of waxy maize heat modified starch in the treatment of children between 2 and 5 years with glycogen storage disease type I: A retrospective study.