keyword
https://read.qxmd.com/read/37312462/-short-term-efficacy-of-empagliflozin-in-children-with-glycogen-storage-disease-type-%C3%A2-b
#21
JOURNAL ARTICLE
J J Jiang, X Zheng, M S Ma, X G Cui, S Jian, X Y Tang, X D Bao, S M Zhang, J R Ma, H M Song, Z Q Qiu
Objective: To analyze the short-time efficacy of empagliflozin in the treatment of glycogen storage disease type Ⅰb (GSD Ⅰb). Methods: In this prospective open-label single-arm study, the data of 4 patients were collected from the pediatric department in Peking Union Medical College Hospital from December 2020 to December 2022. All of them were diagnosed by gene sequencing and had neutropenia. These patients received empagliflozin treatment. Their clinical symptoms such as height and weight increase, abdominal pain, diarrhea, oral ulcer, infection times, and drug applications were recorded at 2 weeks, 1 month, 2 months, 3 months, 6 months, 9 months, 12 months, and 15 months after treatment to assess the therapeutic effect...
June 2, 2023: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://read.qxmd.com/read/37275680/liver-transplantation-in-glycogen-storage-disease-type-ib-the-role-of-sglt2-inhibitors
#22
JOURNAL ARTICLE
Simona Murko, Manuela Peschka, Konstantinos Tsiakas, Sebastian Schulz-Jürgensen, Uta Herden, René Santer
We report on liver transplantation in two patients with GSD Ib on treatment with empagliflozin. The use of this SGLT2 inhibitor resulted in a marked decrease of 1,5-anhydroglucitol which has an important role in the development of neutropenia in this condition. As intended, this caused a significant rise of neutrophil numbers. Liver transplantation alone did not produce the desired effect and our observation argues for continuing SGLT2 inhibitor treatment after transplantation.
June 2023: Molecular Genetics and Metabolism Reports
https://read.qxmd.com/read/37266531/salivary-1-5-anhydroglucitol-and-its-correlation-with-postprandial-hyperglycemia-development-and-validation-of-a-novel-assay
#23
JOURNAL ARTICLE
Priyadarshini Subramaniam Kedarnath, S Sathasiva Subramanian, Emmanuel Bhaskar, Mohan Kasi, Vinod Pillai, Saravanan Subramanian, Venkat Manohar
BACKGROUND: Saliva has the potential to be used as a noninvasive sample for testing hyperglycemia in diabetes mellitus. Serum 1,5-anhydroglucitol (1,5-AG) decreases with an increase in blood sugar >180 mg/dl. We hypothesized that salivary 1,5-AG can be used to identify blood sugar higher than 180 mg/dl using a novel biochemical method. AIM: This study aimed to develop a novel biochemical method for serum and salivary assessment of 1,5-AG and assess its correlation with postprandial blood sugar (PPBS) >180 mg/dl...
2023: International Journal of Applied and Basic Medical Research
https://read.qxmd.com/read/37238286/treatment-of-the-neutropenia-associated-with-gsd1b-and-g6pc3-deficiency-with-sglt2-inhibitors
#24
REVIEW
Maria Veiga-da-Cunha, Saskia B Wortmann, Sarah C Grünert, Emile Van Schaftingen
Glycogen storage disease type Ib (GSD1b) is due to a defect in the glucose-6-phosphate transporter (G6PT) of the endoplasmic reticulum, which is encoded by the SLC37A4 gene. This transporter allows the glucose-6-phosphate that is made in the cytosol to cross the endoplasmic reticulum (ER) membrane and be hydrolyzed by glucose-6-phosphatase (G6PC1), a membrane enzyme whose catalytic site faces the lumen of the ER. Logically, G6PT deficiency causes the same metabolic symptoms (hepatorenal glycogenosis, lactic acidosis, hypoglycemia) as deficiency in G6PC1 (GSD1a)...
May 19, 2023: Diagnostics
https://read.qxmd.com/read/37178520/1-5-ag-suppresses-pro-inflammatory-polarization-of-macrophages-and-promotes-the-survival-of-b-all-in-vitro-by-upregulating-cxcl14
#25
JOURNAL ARTICLE
Min Wu, Shule Zhang, Cheng Chi, Huasu Zhu, Huixian Ma, Linghong Liu, Qing Shi, Dong Li, Xiuli Ju
B-lineage acute lymphoblastic leukemia (B-ALL) is one of the most common malignancies in children. Despite advances in treatment, the role of the tumor microenvironment in B-ALL remains poorly understood. Among the key components of the immune microenvironment, macrophages play a critical role in the progression of the disease. However, recent research has suggested that abnormal metabolites may influence the function of macrophages, altering the immune microenvironment and promoting tumor growth. Our previous non-targeted metabolomic detection revealed that the metabolite 1,5-anhydroglucitol (1,5-AG) level in the peripheral blood of children newly diagnosed with B-ALL was significantly elevated...
May 11, 2023: Molecular Immunology
https://read.qxmd.com/read/37152929/current-understanding-on-pathogenesis-and-effective-treatment-of-glycogen-storage-disease-type-ib-with-empagliflozin-new-insights-coming-from-diabetes-for-its-potential-implications-in-other-metabolic-disorders
#26
REVIEW
Arianna Maiorana, Francesco Tagliaferri, Carlo Dionisi-Vici
Glycogen storage type Ib (GSDIb) is a rare inborn error of metabolism caused by glucose-6-phosphate transporter (G6PT, SLC37A4 ) deficiency. G6PT defect results in excessive accumulation of glycogen and fat in the liver, kidney, and intestinal mucosa and into both glycogenolysis and gluconeogenesis impairment. Clinical features include hepatomegaly, hypoglycemia, lactic acidemia, hyperuricemia, hyperlipidemia, and growth retardation. Long-term complications are liver adenoma, hepatocarcinoma, nephropathy and osteoporosis...
2023: Frontiers in Endocrinology
https://read.qxmd.com/read/37085754/a-metabolomics-study-of-metabolites-associated-with-the-glomerular-filtration-rate
#27
JOURNAL ARTICLE
Hongquan Peng, Xun Liu, Chiwa Ao Ieong, Tou Tou, Tsungyang Tsai, Haibin Zhu, Zhi Liu, Peijia Liu
BACKGROUND: Chronic kidney disease (CKD) is a global public health issue. The diagnosis of CKD would be considerably enhanced by discovering novel biomarkers used to determine the glomerular filtration rate (GFR). Small molecule metabolites related to kidney filtration function that might be utilized as biomarkers to measure GFR more accurately could be found via a metabolomics analysis of blood samples taken from individuals with varied glomerular filtration rates. METHODS: An untargeted metabolomics study of 145 plasma samples was performed using ultrahigh-performance liquid chromatography tandem mass spectrometry (UPLC-MS/MS)...
April 21, 2023: BMC Nephrology
https://read.qxmd.com/read/37061006/dispersion-of-serum-1-5-anhydroglucitol-values-in-patients-with-type-2-diabetes-at-goal-of-hba1c
#28
JOURNAL ARTICLE
Jaime Eduardo Villena Chávez, Elsa Rosa Neira Sánchez, Luciano Francesco Poletti Ferrara
AIM: To investigate the relationship of 1,5 anhydroglucitol (1,5 AG) with HbA1c in patients with type 2 diabetes (T2D) with different ranges of glycemic control. METHODS: One hundred outpatients with T2D ≥ 18 years old were studied. In addition, HbA1c, glycemia, 1,5 AG, lipids, albuminuria, estimated glomerular filtration rate, and clinical data were registered. RESULTS: The patient's median age was 62.5 years, with a median of 10 years with T2D...
April 13, 2023: Diabetes Research and Clinical Practice
https://read.qxmd.com/read/37049081/an-efficient-electrochemical-biosensor-to-determine-1-5-anhydroglucitol-with-persimmon-tannin-reduced-graphene-oxide-ptpd-nanocomposites
#29
JOURNAL ARTICLE
Guiyin Li, Zhide Zhou, Zhongmin Wang, Shiwei Chen, Jintao Liang, Xiaoqing Yao, Liuxun Li
1,5-Anhydroglucitol (1,5-AG) is a sensitive biomarker for real-time detection of diabetes mellitus. In this study, an electrochemical biosensor to specifically detect 1,5-AG levels based on persimmon-tannin-reduced graphene oxide-PtPd nanocomposites (PT-rGO-PtPd NCs), which were modified onto the surface of a screen-printed carbon electrode (SPCE), was designed. The PT-rGO-PtPd NCs were prepared by using PT as the film-forming material and ascorbic acid as the reducing agent. Scanning electron microscopy (SEM), transmission electron microscopy (TEM), ultraviolet-visible spectroscopy (UV-vis), and X-ray diffraction (XRD) spectroscopy analysis were used to characterise the newly synthesised materials...
March 30, 2023: Materials
https://read.qxmd.com/read/37038850/changes-of-1-5-ag-in-vitreous-humor-of-rabbit-cadavers-with-hyperglycemic-metabolism
#30
JOURNAL ARTICLE
Fang-Fang Liu, Hui Wu, Wei Wang, Ying Xie
OBJECTIVES: To investigate the concentration and change characteristics of 1, 5-anhydroglucitol (1, 5-AG) in the vitreous humor of rabbit cadavers with hyperglycemic metabolism, and to explore the value of 1, 5-AG in forensic pathology identification of death caused by hyperglycemic metabolism disorders. METHODS: A diabetic hyperglycemic rabbit model was established by using alloxan. Eighteen rabbits with fasting glucose concentration ≥13.80 mmol/L (experimental group) and 18 healthy rabbits with fasting glucose concentration ≤6...
February 25, 2023: Fa Yi Xue za Zhi
https://read.qxmd.com/read/36900114/association-of-alternative-markers-of-carbohydrate-metabolism-fructosamine-and-1-5-anhydroglucitol-with-perioperative-characteristics-and-in-hospital-complications-of-coronary-artery-bypass-grafting-in-patients-with-type-2-diabetes-mellitus-prediabetes-and
#31
JOURNAL ARTICLE
Alexey N Sumin, Natalia A Bezdenezhnykh, Andrey V Bezdenezhnykh, Anastasiya A Kuzmina, Yuliya A Dyleva, Olga L Barbarash
UNLABELLED: Patients with type 2 diabetes make up 25 to 40% of those referred for coronary bypass surgery, and the impact of diabetes on the results of the operation is studied in various aspects. To assess the state of carbohydrate metabolism before any surgical interventions, including CABG, daily glycemic control, and the determination of glycated hemoglobin (HbA1c) is recommended. Glycated hemoglobin reflects the glucose concentration for the 3 months prior to the measurement, but alternative markers that reflect glycemic fluctuations over a shorter period of time may be useful in preoperative preparation...
March 3, 2023: Diagnostics
https://read.qxmd.com/read/36747147/1-5-anhydroglucitol-promotes-pre-b-acute-lymphocytic-leukemia-progression-by-driving-glycolysis-and-reactive-oxygen-species-formation
#32
JOURNAL ARTICLE
Huasu Zhu, Huixian Ma, Na Dong, Min Wu, Dong Li, Linghong Liu, Qing Shi, Xiuli Ju
BACKGROUND: Precursor B-cell acute lymphoblastic leukemia (pre-B ALL) is the most common hematological malignancy in children. Cellular metabolic reorganization is closely related to the progression and treatment of leukemia. We found that the level of 1,5-anhydroglucitol (1,5-AG), which is structurally similar to glucose, was elevated in children with pre-B ALL. However, the effect of 1,5-AG on pre-B ALL was unclear. Here, we aimed to reveal the roles and mechanisms of 1,5-AG in pre-B ALL progression...
February 6, 2023: BMC Cancer
https://read.qxmd.com/read/36516224/untargeted-metabolomic-profiling-identifies-serum-metabolites-associated-with-type-2-diabetes-in-a-cross-sectional-study-of-the-atbc-study
#33
JOURNAL ARTICLE
Yuzhao Liu, Lu Gan, Bin Zhao, Kai Yu, Yangang Wang, Satu Männistö, Stephanie J Weinstein, Jiaqi Huang, Demetrius Albanes
Type 2 diabetes (T2D) is a complex chronic disease with substantial phenotypic heterogeneity affecting millions of individuals. Yet, its relevant metabolites and etiological pathways are not fully understood. The aim of this study is to assess a broad spectrum of metabolites related to T2D in a large population-based cohort. We conducted a metabolomic analysis of 4,281 male participants within the Alpha-Tocopherol, Beta-Carotene Cancer Prevention (ATBC) Study. The serum metabolomic analysis was performed using an LC-MS/GC-MS platform...
December 14, 2022: American Journal of Physiology. Endocrinology and Metabolism
https://read.qxmd.com/read/36507137/favorable-outcome-of-empagliflozin-treatment-in-two-pediatric-glycogen-storage-disease-type-1b-patients
#34
JOURNAL ARTICLE
Zufit Hexner-Erlichman, Maria Veiga-da-Cunha, Yoav Zehavi, Zahava Vadasz, Adi D Sabag, Sameh Tatour, Ronen Spiegel
BACKGROUND: Glycogen storage disease type 1b (GSD1b) is an ultra-rare autosomal recessive disorder, caused by mutations in SLC37A4 gene. Affected patients present with episodes of fasting hypoglycemia and lactic acidosis, hepatomegaly, growth retardation, hyperlipidemia and renal impairment. In addition, patients present neutropenia, neutrophil dysfunction and oral, and skin infections as well as a significant predisposition to develop inflammatory bowel disease (IBD). Low neutrophil counts and function is related to the toxic accumulation of 1,5-anhydroglucitol-6-phosphate (1,5-AG6P)...
2022: Frontiers in Pediatrics
https://read.qxmd.com/read/36485107/genetics-of-severe-congenital-neutropenia-as-a-gateway-to-personalized-therapy
#35
JOURNAL ARTICLE
Jean Donadieu, Christine Bellanné-Chantelot
Severe congenital neutropenias (SCNs) are rare diseases, and to date about 30 subtypes have been described according to their genetic causes. Standard care aims to prevent infections and limit the risk of leukemic transformation; however, several subtypes may have additional organ dysfunction(s), requiring specialized care. Granulocyte colony-stimulating factor and hematopoietic stem cell transplantation are now the bedrock of standard care. Better understanding of SCN mechanisms now offers the possibility of adapted therapy for some entities...
December 9, 2022: Hematology—the Education Program of the American Society of Hematology
https://read.qxmd.com/read/36402758/metabolic-and-proteomic-signatures-of-type-2-diabetes-subtypes-in-an-arab-population
#36
JOURNAL ARTICLE
Shaza B Zaghlool, Anna Halama, Nisha Stephan, Valborg Gudmundsdottir, Vilmundur Gudnason, Lori L Jennings, Manonanthini Thangam, Emma Ahlqvist, Rayaz A Malik, Omar M E Albagha, Abdul Badi Abou-Samra, Karsten Suhre
Type 2 diabetes (T2D) has a heterogeneous etiology influencing its progression, treatment, and complications. A data driven cluster analysis in European individuals with T2D previously identified four subtypes: severe insulin deficient (SIDD), severe insulin resistant (SIRD), mild obesity-related (MOD), and mild age-related (MARD) diabetes. Here, the clustering approach was applied to individuals with T2D from the Qatar Biobank and validated in an independent set. Cluster-specific signatures of circulating metabolites and proteins were established, revealing subtype-specific molecular mechanisms, including activation of the complement system with features of autoimmune diabetes and reduced 1,5-anhydroglucitol in SIDD, impaired insulin signaling in SIRD, and elevated leptin and fatty acid binding protein levels in MOD...
November 19, 2022: Nature Communications
https://read.qxmd.com/read/36382190/advances-in-multi-omics-study-of-biomarkers-of-glycolipid-metabolism-disorder
#37
REVIEW
Xinyi Fang, Runyu Miao, Jiahua Wei, Haoran Wu, Jiaxing Tian
Glycolipid metabolism disorder are major threats to human health and life. Genetic, environmental, psychological, cellular, and molecular factors contribute to their pathogenesis. Several studies demonstrated that neuroendocrine axis dysfunction, insulin resistance, oxidative stress, chronic inflammatory response, and gut microbiota dysbiosis are core pathological links associated with it. However, the underlying molecular mechanisms and therapeutic targets of glycolipid metabolism disorder remain to be elucidated...
2022: Computational and Structural Biotechnology Journal
https://read.qxmd.com/read/36380614/prospective-test-performance-of-nonfasting-biomarkers-to-identify-dysglycemia-in-children-and-adolescents
#38
JOURNAL ARTICLE
Mary Ellen Vajravelu, Emily Hirschfeld, Acham Gebremariam, Charles F Burant, William H Herman, Karen E Peterson, Jennifer L Meijer, Joyce M Lee
INTRODUCTION: Test performance screening measures for dysglycemia have not been evaluated prospectively in youth. This study evaluated the prospective test performance of random glucose (RG), 1-h nonfasting glucose challenge test (1-h GCT), hemoglobin A1c (HbA1c), fructosamine (FA), and 1,5-anhydroglucitol (1,5-AG) for identifying dysglycemia. METHODS: Youth ages 8-17 years with overweight or obesity (body mass index, BMI, ≥85th percentile) without known diabetes completed nonfasting tests at baseline (n = 176) and returned an average of 1...
2023: Hormone Research in Pædiatrics
https://read.qxmd.com/read/36359120/dietary-and-animal-strategies-to-reduce-the-environmental-impact-of-pastoral-dairy-systems-result-in-altered-nutraceutical-profiles-in-milk
#39
JOURNAL ARTICLE
Cameron Joel Marshall, Konagh Garrett, Stephan Van Vliet, Matthew Raymond Beck, Pablo Gregorini
The objective of this study was to evaluate and provide further insights into how dairy cows genetically divergent for milk urea N breeding values [MUNBV, high (2.21 ± 0.21) vs. low (-1.16 ± 0.21); µ ± SEM], consuming either fresh cut Plantain ( Plantago lanceolata L., PL) or Ryegrass ( Lolium perenne L., RG) herbage, impacted the nutraceutical profile of whole milk by investigating amino and fatty acid composition and applying metabolomic profiling techniques. Both diet and MUNBV, and their interaction term, were found to affect the relative abundance of alanine, glycine, histidine, and phenylalanine in the milk ( p < 0...
October 31, 2022: Animals: An Open Access Journal From MDPI
https://read.qxmd.com/read/36241632/the-gut-microbe-bacteroides-fragilis-ameliorates-renal-fibrosis-in-mice
#40
JOURNAL ARTICLE
Wei Zhou, Wen-Hui Wu, Zi-Lin Si, Hui-Ling Liu, Hanyu Wang, Hong Jiang, Ya-Fang Liu, Raphael N Alolga, Cheng Chen, Shi-Jia Liu, Xue-Yan Bian, Jin-Jun Shan, Jing Li, Ning-Hua Tan, Zhi-Hao Zhang
Renal fibrosis is an inevitable outcome of various manifestations of progressive chronic kidney diseases (CKD). The need for efficacious treatment regimen against renal fibrosis can therefore not be overemphasized. Here we show a novel protective role of Bacteroides fragilis (B. fragilis) in renal fibrosis in mice. We demonstrate decreased abundance of B. fragilis in the feces of CKD patients and unilateral ureteral obstruction (UUO) mice. Oral administration of live B. fragilis attenuates renal fibrosis in UUO and adenine mice models...
October 14, 2022: Nature Communications
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