• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.2
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• pp.93-97
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• 2015

Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is an autosomal recessive hereditary metabolic disorder of mitochondrial fatty acid beta-oxidation. Mutations in the ACADS gene cause short-chain acyl-CoA dehydrogenase deficiency, which is characterized by developmental delay, hypotonia, seizure, and hypoglycemia. Here, we describe one Korean pediatric case of SCAD deficiency, which was diagnosed during newborn screening by tandem mass spectrometry and confirmed by molecular analysis. The level of C4 was typically elevated 5.23 mg/dL (reference range <1.5 mg/dL). This patient had a homozygous mutation [c.1031A>G, p. E344G] in ACADS. Therefore, we present a case of SCAD deficiency in an otherwise healthy neonate and her subsequent development and growth over four years.

• Journal of The Korean Dental Society of Anesthesiology
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• v.14 no.4
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• pp.243-250
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• 2014

Generalized anaphylaxis is a most dramatic and acutely life-threatening allergic reaction and may cause death within a few minutes. Differential diagnosis of anaphylaxis is made by clinical signs, such as, mental change, respiratory distress, hypotension, hypoglycemia, urticaria and angioedema. Especially, insulin reaction, myocardial infarction and vasovagal syncope are considered as differential diagnosis. In cases of fatal anaphylaxis, respiratory and cardiovascular disturbances predominate and are evident early in the reaction. This is a case report of the intensive care of anaphylactic shock after intravenous injection of the penicillin in a old medically compromised patient with the maxillary osteonecrosis. The anaphylactic shock symptoms, such as, unconsciousness, respiratory disorder, no pulsation on carotid artery and cardiopulmonary arrest are occurred in intravenous injection of augmentin 1.2 g after the skin test. In spite of immediate emergency cares, such as intravenous injection of epinephrine, endotracheal intubation, cardiopulmonary resuscitation, and continuous intensive care, the patient is expired in 58 hours after anaphylactic shock attack.

• Journal of Yeungnam Medical Science
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• v.23 no.2
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• pp.252-257
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• 2006

Glycogen storage diseases are a heterogeneous group of metabolic disorder affecting multiple organ system: liver, skeletal muscle, heart and brain. Clinical features include: short status, hepatomegaly, hypoglycemia, dyslipidemia and rare involvement of the myocardium except in the case of type III, glycogen storage diseases with hypertrophic cardiomyopathy in adult, which is extremely rare. We treated a case of hypertrophic cardiomyopathy with hepatomegaly that was unknown etiology. The patient was diagnosed as having glycogen storage disease. This 46-year old women was transferred with dyspnea on exertion and abnormal LFTs. She was diagnosed with hypertrophic cardiomyopathy by echocardiography but there was no specific cause for hypertrophic cardiomyopathy. A liver biopsy was performed. The result showed glycogen storage disease possible type III, IV or IX. In conclusion, patients with hypertrophic cardiomyopathy of unknown etiology and abnormal LFTs should be evaluated for glycogen storage disease.

• Investigative Magnetic Resonance Imaging
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• v.18 no.4
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• pp.357-361
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• 2014

Purpose : In a previous report, it took several days for white matter lesions to regress in hypoglycemic encephalopathy. We present a case of rapid diffusion-weighted image (DWI) changes in hypoglycemic encephalopathy. Case Report: A 58-year-old male patient was found semi-comatous with the only abnormality in his laboratory tests showing hypoglycemia (44 mg/dL). After rapid correction of glucose level, immediate brain DWI showed bilateral subcortical white matter lesions. After about 5 hours, follow-up DWI showed resolved subcortical white matter lesions, with newly-appeared bilateral fronto-temporo-parietal cortical lesions. Conclusion: Both white matter and cortex involvement in hypoglycemic encephalopathy has been shown in several reports, but rapid regression of white matter changes in hypoglycemic encephalopathy has been rarely reported. It is important to know that MR imaging changes in hypoglycemic encephalopathy can be made as quick as just a few-hour-long.

• Asian-Australasian Journal of Animal Sciences
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• v.30 no.12
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• pp.1696-1701
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• 2017

Objective: In the present study, we examined whether the post-prandial reduction in plasma growth hormone (GH) levels is related to the increase in plasma insulin levels in ruminants. Methods: We performed two experiments: intravenous bolus injection of insulin (0.2 IU/kg body weight) or glucose (1.0 mmol/kg body weight) was administered to increase the plasma insulin levels in male Shiba goats. Results: In the insulin injection experiment, significant (p<0.05) increase in GH concentrations was observed, 15 to 20 min after the injection; it was accompanied with a significant (p<0.01) increase in cortisol concentrations at 45 to 90 min, when compared to the concentrations in the saline-injected controls. The glucose injection significantly (p<0.05) increased the plasma GH concentration at 20 to 45 min; this was not accompanied by significantly higher cortisol concentrations than were observed for the saline-injected control. Hypoglycemia induced by the insulin injection, which causes the excitation of the adrenal cortex, might be involved in the increase in insulin levels. Conclusion: Based on these results, we conclude that post-prandial increases in plasma insulin or glucose levels do not induce a decrease in GH concentration after feeding in the ruminants.

• Journal of the Korean Applied Science and Technology
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• v.1 no.1
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• pp.37-47
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• 1984

Effects of garlic on hypocholesterolemia, anticoagulation and hypoglycemia were studied in the present experiments using male rats. The results were summarized as follows. 1. The supplementation of $2{\sim}4%$ garlic to 2% cholesterol diets did not affect food intake and weight gain in male rats. 2. Rat's groups fed the diets supplemented with $2{\sim}3%$ garlic (C.D.E.F) to 2% cholesterol diet (B) decreased serum total cholesterol levels by $16{\sim}32%$, triglyceride levels by $18.6{\sim}26.8%$ and ${\beta}/{\alpha}-lipoportein$ rations by $42{\sim}58%$, but increased HDL-cholesterol levels by $29{\sim}65%$ as compared to B group, and so the author assumes that garlic supplementation exerts hypocholesterolemic effect on cholesterol - fed rats because of the increase of HDL and HDL - cholesterol levels. 3. Rat's groups fed the diets supplemented with $2{\sim}4%$ garlic (C.D.E.F) to 2% cholesterol plus 0.25% bile salt diet (B) increased whole blood coagulation times, prothrombin times and fibrinolytic activities, but decreased plasma fibrinogen levels as compared to B group, and so the author assumes that garlic supplementation exerts anticoagulative effect because of the inhibition of fibrinogen synthesis in male rat's liver. 4. Rat's groups fed the diets supplemented with $2{\sim}4%$ garlic (B.C.D.E) to standard diet (A) decreased serum glucose levels by $1{\sim}24%$, but increased serum insulin concentrations by $0.5{\sim}3.0$ times as compared to A group, and so the author assumes that garlic supplementation exerts hypoglycemic effect because of the increase of serum insulin levels by stimulation pancreatic secretion of insulin from ${\beta}-cells$ in the islets of Langerhans.

• Biomolecules & Therapeutics
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• v.4 no.4
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• pp.443-448
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• 1996

The effects of Taheebo on the diabetic-piegnant rats and their fetus was investigated. It has been reported that diabetic condition of the pregnant rats can affect the process of liver formation and damage the respiratory function in the fetus. Therefore we investigated the effects of Taheebo on the prevention of liver damage and respiratory failure in the fetus and those results were compared with that of dexamethasone (DXM). In pregnant rats, streptozotocin(STZ, 45 mg/kg, 0.01 M citrate buffer) was injected into the pregnant rats on the third day of pregnancy. Methanol extracts of Taheebo(500 mg/kg p.o.) was administered once daily during pregnancy. DXM (10 $\mu\textrm{g}$/g i.p.) was injected into the pregnant rats in 16th and 18th days of pregnancy. Body weights were measured and fetal number and abortion rate in pregnancy rats were determined. Lecithin/sphingomyelin ratio in amniotic fluid and malondialdehyde, glycogen, triglyceride, protein and cholesterol levels in the liver homogenate were determined. Also blood glucose level was analyzed. Body weights of maternal rats were increased in the all groups except the DXM group. Fetal number of the Taheebo treated group was similar to the control group, and a significant increase in the body weights of fetus was observed in the STZ treated group and the Taheebo treated group compared with the control group. Blood glucose of fetus produced hypoglycemia in the control group and hyperglycemia in the diabetic-pregnant rats. The protein and cholesterol levels in fetus liver were significantly increased in the DXM treated group compared with the control group. Triglyceride content was significantly increased in all groups compared with the control group. Liver malondialdehyde level of fetus in the STZ treated group was similar to the control group. Glycogen level was significantly increased in the all groups compared with the control group. Methanol extract of Taheebo showed hypoglycemic effect on the pregnant rats. However, we could not observe any hypoglycemic effect on the fetus. There's no difference between the control and Taheebo treated group in terms of the levels of triglyceride, cholesterol, protein and glycogen in the fetus liver. Further study to identify the effect of Taheebo on the fetus is under investigation.

• Korean Journal of Veterinary Research
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• v.36 no.3
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• pp.731-741
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• 1996

ELISA kit for cortisol was developed and then evaluated. Polyclonal antihydrocortisone-3-(o-carboxymethyl)oxime BSA rabbit serum was used to coat the 96-well microplates. The minimum detection limit of the kit was 250pg of cortisol per milliliter. The within-run variation and the day to day variation of the ELISA system were 2.0 and 5.9 at maximum, respectively. The kit was used to determine whether salivary cortisol concentration could replace blood cortisol concentration in dexamathasone suppression test of dogs. Changes of cortisol concentration were measured in serum or saliva after intravenous administration of 0.01mg of dexamethasone per kilogram of body weight. Blood alone, saliva alone or both were collected at 0, 30, 60, 120, 240, and 360 minutes after injection of dexamethasone. The change in blood cortisol concentration was found to be suitable in dexamathasone suppression test of dogs, but the change in salivary cortisol concentration was not. The kit was also used to determine whether salivary cortisol concentration could be a stress index as well as blood cortisol concentration in dogs. Two types of trial were performed to estimate the stress either by blood or salivary cortisol concentration. The first trial was stress experiment by intravenous injection of 0.2IU of PZI-insulin per kilogram body weight. Either blood alone or saliva alone was collected at 0, 30, 60, and 90 minutes after insulin administration. Both blood and salivary cortisol concentration were found to be suitable index in estimating stress from hypoglycemia by injection of insulin. The second trial was stress experiment by electrical irritation. The dogs were irritated with anti-bark device for 10 seconds. Blood was collected before and at 2 and 5 minutes after electrical irritation. Saliva was collected before and at 3 and 6 minutes after electrical irritation. The blood cortisol concentration, but not the salivary cortisol concentration was found to be suitable index in estimating stress from electrical irritation. Cushing syndrome in a dog was also successfully diagnosed with this kit.

• Journal of muscle and joint health
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• v.21 no.3
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• pp.184-194
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• 2014

Purpose: The purpose of this study was to develop and validate a diabetes management self-efficacy scale for older adults (DMSES-O). Methods: A preliminary DMSES-O of 22 items was derived from a literature review and seven domains of self-management behaviors. Content validity was confirmed by experts in diabetes self-management education. To test the reliability and validity of the DMSES-O, data were collected from 150 older adults with type 2 diabetes. The data were analyzed using exploratory factor analysis, and Cronbach's ${\alpha}$ and Pearson's correlation coefficients were calculated. Results: From the exploratory factor analysis, 17 significant items in six subscales were derived. Factors derived were named "problem solving for hypoglycemia and self-monitoring blood glucose," "problem solving for hyperglycemia," "coping with psychological distress and taking medication," "reducing risks of diabetes complications," "appropriate exercise," and "healthy eating." The criterion-related validity of the DMSES-O was established by its correlation with the Summary of Diabetes Self-care Activities Questionnaire. Cronbach's ${\alpha}$, a measure of internal consistency, was .84 for the overall scale and ranged from .54 to .80 for the subscales. Conclusion: The DMSES-O is a reliable and valid instrument to measure selfefficacy for diabetes self-management among older adults.

• 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).