• Journal of The Korean Society of Inherited Metabolic disease
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• v.1 no.1
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• pp.23-27
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• 2001

Fanconi-Bickel Syndrome (FBS) is a rare autosomal recessive disorder of carbohydrate metabolism recently demonstrated to be caused by mutations in the GLUT 2 gene for the glucose transporter protein 2 expressed in liver, pancreas, intestine, and kidney. This disease is characterized by hepatorenal glycogen accumulation, both fasting hypoglycemia as well as postprandial hyperglycemia and hyperglactosemia, and generalized proximal renal tubular dysfunctions. We report the first Korean patient with FBS diagnosed based on clinical manifestations and identification of a novel mutation in the GLUT 2 gene. She was initially diagnosed having a neonatal diabetes mellitus due to hyperglycemia and glycosuria at 3 days after birth. In addition, newborn screening for galactosemia revealed hypergalactosemia. Thereafter, she has been managed with lactose free milk, insulin therapy. However, she failed to grow and her liver has been progressively enlarging. Her liver functions were progressively deteriorated with increased prothrombin time. Liver biopsy done at age 9 months indicated micronodular cirrhosis with marked fatty changes. She succubmed to hepatic failiure with pneumonia at 10 months of age. Laboratory tests indicated she had generalized proximal renal tubular dysfuctions; renal tubular acidosis, hypophosphatemic rickets, and generalized aminoaciduria. Given aforementioned findings, the diagnosis of FBS was appreciated at age of 2 months. The DNA sequencing analysis of the GLUT 2 gene using her genomic DNA showed a novel mutation at 5th codon; Lysine5 Stop (K5X).

• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.2
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• pp.8-14
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• 2023

The hepatic glycogen storage disease type 0 (GSD type 0) is an autosomal recessive disorder caused by a deficiency of hepatic glycogen synthase encoded by the glycogen synthase 2 (GYS2) gene, leading to abnormal synthesis glycogen. The clinical findings of GSD type 0 are hyperketotic hypoglycemia at fasting state and accompanying postprandial hyperglycemia and hyperlactatemia. GSD type 0 has only been reported in a very small number so far, and the diagnosis is likely to be missed because symptoms are mild, severe hypoglycemia is rare or asymptomatic, or symptoms gradually disappear with age. Essential management strategies include feeding high-protein meals to stimulate gluconeogenesis, frequent meals to prevent hypoglycemia during the day and feeding complex carbohydrates such as uncooked cornstarch to slowly release glucose during nignt. GSD type 0 has a good prognosis, with appropriate treatment, normal growth can be achieved and no complications occur. Significant hypoglycemia occurs less common in adulthood, but ongoing dietary management may be necessary.

• International Journal of Industrial Entomology and Biomaterials
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• v.31 no.2
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• pp.48-55
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• 2015

1-Deoxynojirimycin (DNJ) has been extensively investigated for its applications as an a-glucosidase inhibitor in postprandial hyperglycemia, and has been applied to nutraceuticals and medicines to prevent or delay the progression of type 2 diabetes. However, the amount of DNJ in mulberry leaves is low (approximately 0.1%), therefore, a more effective extraction method is needed. In this study, microbial DNJ production was developed as an alternative to chemical methods. We identified fermented sericultural products and bacteria that produce DNJ in large quantities using high performance liquid chromatography and thin layer chromatography. The inhibition of a-glucosidase activity was examined with respect to DNJ production or non-production. Crude DNJ from the isolated strains exhibited greater than 70% a-glucosidase activity. An investigation of the effect of mulberry leaf powder concentration (1~5%), using high DNJ producing bacteria, provided evidence for microbial mass production of DNJ.

• International Journal of Industrial Entomology and Biomaterials
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• v.33 no.1
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• pp.24-30
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• 2016

1-Deoxynojirimycin (DNJ) have been extensively investigated for their α-glucosidase inhibitor on postprandial hyperglycemia, and applied in nutraceuticals and medicine for preventing or delaying progression of type 2 diabetes. However, the amount of DNJ in mulberry leaves is low (about 0.1%), therefore, more effective extraction method is needed. This study was performed to develop microbial DNJ for biological methods of DNJ as an alternative to the chemical methods. In this study, we obtained evidence for Bacillus subtilis that produce DNJ in large quantities by high performance liquid chromatography. Inhibition of α-glucosidase activity was determined to DNJ production or non-production. Investigation of the effect of mulberry leaves powder concentration (1~5%), using the DNJ high-production bacteria, provided evidence for microbial mass production of DNJ. When the 4% mulberry leaf powder for 9 days was used, the α-glucosidase inhibitory activity was over the 85%. Also, the results presented in this study confirm DNJ yield's increasement in microbes using the various of nutrients and provide insight of ways to improve DNJ yields in microorganisms.

• Archives of Pharmacal Research
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• v.28 no.9
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• pp.1027-1030
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• 2005

The antidiabetic-activity-guided fractionation and isolation of the $80\%$ EtOH extracts obtained from cultivated Korean Rhubarb rhizomes (Rheum undulatum, Polygonaceae) led to the isolation and characterization of one stilbene, desoxyrhapontigenin(1) and two anthraquinones, emodin (2) and chrysophanol (3). Their structures were established by chemical and spectroscopic methods. Compounds 1, 2, and 3 inhibited postprandial hyperglycemia by 35.8, 29.5, $42.3\%$, respectively.

• Preventive Nutrition and Food Science
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• v.19 no.3
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• pp.164-169
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• 2014

The purpose of the present study was to investigate the short-term effects of a 12-day, soft pellet (SP) diet with a 3-h restricted feeding schedule on caloric intake, body weight, lipid metabolism, and insulin sensitivity. Glucose and insulin levels were measured pre-, mid-, and post-feeding. The SP rats exhibited postprandial hyperglycemia compared to rats fed control pellets (CP). The insulin response of SP rats during a meal was significantly higher than that of CP rats. There were no significant differences in the hepatic triacylglycerol contents and lipogenesis gene mRNA levels of SP and CP rats. However, the hepatocytes of SP rats were slightly hypertrophic. In addition, histological analysis revealed that the pancreases of SP rats had more islet areas than those of CP rats. This study demonstrated that feeding an SP-only diet for 12 days induces glucose intolerance, suggesting that the consumption of absorbable food, like a soft diet, may trigger glucose metabolism insufficiency and lead to life-threatening diseases.

• Korean Journal of Adult Nursing
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• v.17 no.3
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• pp.444-451
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• 2005

Purpose: The purpose of this study was to evaluate whether the effect of the Internet diabetic education on plasma glucose in people with hyperglycemic diabetes. Method: A randomized design with control and experimental groups being assessed pre- and post-intervention was used. Twenty-two patients were randomly assigned to an intervention group and 21 to a control group. Participants were requested to input the blood glucose level weekly in http://www.biodang.com by cellular phone or wire Internet for 3 months. The researcher sends optimal recommendations to each patient using short message service(SMS) of cellular phone and wire Internet weekly. Results: Patients in the intervention group had a mean decrease of 1.3% in glycosylated haemoglobin ($HbA_1c$) levels and those in the control group had no significant difference. There was a significant mean change in 2-hour postprandial blood glucose(2HPPG) for the intervention group, with a mean change of-75.2mg/dl. The mean change in the control group was, however, not significant. Conclusion: These findings indicated that a web-based intervention using SMS of cellular phone improved $HbA_1c$ and 2HPPG.

• The Journal of Internal Korean Medicine
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• v.43 no.2
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• pp.283-293
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• 2022

A 42-year-old male patient with prolonged throat pain and discomfort, dry mouth, and general weakness and recently diagnosed with tonsillitis, pulmonary embolism, and venous thrombosis was admitted to the internal Korean medicine department. A sudden onset of diabetic ketoacidosis with hyperammonemia was diagnosed on the second day of treatment. During admission, the patient received insulin therapy, hydration, and traditional Korean medicine treatment, including herbal medicine. Subjective symptom change was evaluated daily and blood glucose level checked five times per day. At discharge, the patient's fasting and postprandial blood sugar levels were adequate. After an additional two weeks of herbal treatment, the symptoms were significantly ameliorated. Thus, having been admitted with dysregulated glucose metabolism leading to a hyperglycemic crisis after a series of inflammatory events, the patient showed symptomatic improvements and decreased blood glucose after 18 days of hospitalization and treatment.

• Journal of the Korean Society of Food Science and Nutrition
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• v.32 no.6
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• pp.921-925
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• 2003

To control blood glucose level as close to normal is the major goal of treatment of diabetes mellitus. $\alpha$-glucosidase is the enzyme to digest dietary carbohydrate and inhibition of $\alpha$-glucosidase could suppress postprandial hyperglycemia. The methanol extract of soybean sprout was tested for the inhibitory activities against $\alpha$-glucosidase in vitro. Soybean sprout extract inhibited yeast $\alpha$-glucosidase activity by 24.5% at the concentration of 5 mg/mL. The methanol extract of soybean sprout was subsequently subjected to sequential fractionation with hexane, ethyl acetate, butanol and water. Among the fractions tested ethyl acetate-soluble fraction showed relatively strong inhibition against $\alpha$-glucosidase by 36.3% at the concentration of 5 mg/mL. Acarbose, standard $\alpha$-glucosidase inhibitor, inhibited $\alpha$-glucosidase activity by 40.1%. The ability of soybean sprout extract to lower postprandial glucose was studied in streptozotocin-induced diabetic rats. Starch solution (1 g/kg) with and without the methanol extract of soybean sprout (500 mg/kg) was administered to diabetic rats after an overnight-fast by gastric intubation. A single oral dose of soybean sprout extract inhibited the increase in blood glucose levels significantly at 60, 90, 120, 180 min (p<0.05) and decreased incremental response areas under the glycemic response curve significantly (p<0.05). These results suggest that soybean sprout might exert hypoglycemic effect by inhibiting $\alpha$-glucosidase activity.

• Food Science and Biotechnology
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• v.17 no.1
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• pp.130-134
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• 2008

Hypoglycemic effect of onion skin extract in vitro and in vivo was investigated. A methanol extract of onion skin inhibited yeast $\alpha$-glucosidase with an $IC_{50}$ of 0.159 mg/mL. A single oral administration of the onion skin extract (500 mg/kg) significantly lowered the postprandial area under the glucose response curve to starch (1 g/kg, p<0.05). Three-week-old db/db mice were fed an AIN-93G diet or a diet supplemented with a 0.5% onion skin extract for 7 weeks after a 1-week adaptation period. Consumption of onion skin extract significantly reduced the levels of plasma glucose, insulin, and blood glycated hemoglobin as compared with the control group (p<0.05). These findings suggest that onion skin is effective in controlling hyperglycemia in animal models of type 2 diabetes mellitus, at least in part by inhibiting $\alpha$-glucosidase activity.