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
| العنوان: | 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 |
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| المؤلفون: | Laura Nagy, Ghada Hijazi, Sarah Herd, Maya Ram, Jolynn Dickson, Michal Inbar-Feigenberg, Nisha Pai |
| المصدر: | Molecular Genetics and Metabolism Reports Molecular Genetics and Metabolism Reports, Vol 21, Iss, Pp-(2019) |
| سنة النشر: | 2019 |
| مصطلحات موضوعية: | UCCS, uncooked cornstarch, GSD, Glycogen storage disease, Glucose control, BMI, body mass index, AST, aspartate aminotransferase, Hypoglycemia, Nocturnal, Modified starch, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Endocrinology, ALT, alanine aminotransferase, abdominal US, abdominal ultrasound, hrs, hours, Genetics, WMHMS, waxy maize heat modified starch, Medicine, cm, centimeter, Waxy maize heat modified starch (WMHMS), Prospective cohort study, Molecular Biology, lcsh:QH301-705.5, 0303 health sciences, Glycogen storage disease type I, lcsh:R5-920, TGs, triglycerides, business.industry, Continuous nocturnal pump feed (CNPF), 030305 genetics & heredity, Treatment options, Retrospective cohort study, Uncooked cornstarch (UCCS), medicine.disease, Glycogen storage disease (GSD), GI, gastrointestinal, chemistry, lcsh:Biology (General), Anesthesia, kg, kilogram, CNPF, continuous nocturnal pump feed, business, lcsh:Medicine (General), 030217 neurology & neurosurgery, Research Paper |
| الوصف: | 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. Keywords: Glycogen storage disease (GSD), Uncooked cornstarch (UCCS), Waxy maize heat modified starch (WMHMS), Continuous nocturnal pump feed (CNPF). |
| تدمد: | 2214-4269 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d381b396d8d3488437a03dbd2f704eb8 https://pubmed.ncbi.nlm.nih.gov/31844626 |
| Rights: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....d381b396d8d3488437a03dbd2f704eb8 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 22144269 |
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