• Clinical and Experimental Pediatrics
• /
• v.58 no.6
• /
• pp.234-237
• /
• 2015

Hepatic glycogenosis in type 1 diabetes mellitus (DM) can be caused by poor glycemic control due to insulin deficiency, excessive insulin treatment for diabetic ketoacidosis, or excessive glucose administration to control hypoglycemia. Mauriac syndrome, which is characterized by hepatomegaly due to hepatic glycogenosis, growth retardation, delayed puberty, and Cushingoid features, is a rare diabetic complication. We report a case of hepatic glycogenosis mimicking Mauriac syndrome. A 14-year-old girl with poorly controlled type 1 DM was admitted to The Catholic University of Korea, Seoul St. Mary's Hospital for abdominal pain and distension. Physical examination revealed hepatomegaly and a Cushingoid face. The growth rate of the patient had decreased, and she had not yet experienced menarche. Laboratory findings revealed elevated liver enzyme levels. A liver biopsy confirmed hepatic glycogenosis. Continuous glucose monitoring showed hyperglycemia after meals and frequent hypoglycemia before meals. To control hyperglycemia, we increased insulin dosage by using an insulin pump. In addition, we prescribed uncooked cornstarch to prevent hypoglycemia. After strict blood glucose control, the patient's liver functions and size normalized. The patient subsequently underwent menarche. Hepatic glycogenosis is a complication of type 1 DM that is reversible with appropriate glycemic control.

• Asian-Australasian Journal of Animal Sciences
• /
• v.15 no.11
• /
• pp.1591-1598
• /
• 2002

The effect of different dietary structural carbohydrate (SC) to nonstructural carbohydrate (NSC) ratios on fiber degradation, digestion, flow, apparent digestibility and rumen fluid characteristics was studied with a design using 18 wethers fitted with permanent rumen and duodenum cannulae. All sheep were divided into six groups randomly, receiving six diets with varying SC to NSC ratios. All diets contained the same proportion of wheat straw and concentrate. The dietary SC to NSC ratios were adjusted by adding cornstarch to the concentrate supplements. The duodenal and fecal flows of dry matter (DM), neutral detergent fiber (NDF), acid detergent fiber (ADF), hemicellulose (HC) and cellulose (CEL) were estimated using chromium-mordanted wheat straw as a flow marker. The degradation parameters of wheat straw DM, NDF, ADF, HC and CEL were determined by incubating the ground wheat straw in nylon bags in the rumen for different periods of time. There was no effect (p>0.05) of the different dietary SC to NSC ratios on rumen pH or $NH_3$-N, but acetate, propionate and butyrate concentrations were significantly affected (p<0.05 or p<0.01) by dietary SC to NSC ratios in the rumen fluid. When the dietary SC to NSC ratio was 2.86, the highest rumen degradability of wheat straw DM, NDF, ADF and CEL was found, but the highest apparent rumen digestibilities of DM, NDF, ADF, HC and CEL occurred at a 2.64 SC to NSC ratio. However, because of compensatory digestion in the hindgut, the apparent digestibilities of DM, NDF, ADF, HC and CEL were highest when the dietary SC to NSC ratio was 2.40. In conclusion, there is a optimal range of dietary SC to NSC ratios (between 2.86 and 2.40) that is beneficial to maximize wheat straw fiber degradation and apparent digestibility.

• Journal of Microbiology and Biotechnology
• /
• v.23 no.6
• /
• pp.785-793
• /
• 2013

We studied the effects of 2 different dosages of high-molecular-weight poly-${\gamma}$-glutamic acid (hm ${\gamma}$-PGA) derived from Bacillus subtilis chungkookjang on lipid metabolism in a high-fructose diet-induced hypertriglyceridemic animal model. For 4 weeks, rats were fed either AIN-93 diet (normal control, NC; n = 10) or modified AIN-93 diet in which cornstarch was substituted with 63% fructose (n = 30) to induce hypertriglyceridemia. After 4 weeks, the hypertriglyceridemic rats were treated with daily oral doses of 0 mg (hypertriglyceridemic control, HC), 2.5 mg (hypertriglyceridemic, low hm ${\gamma}$-PGA, HL), or 5 $mg{\cdot}kg{\cdot}bw^{-1}{\cdot}d^{-1}$ (hypertriglyceridemic, high hm ${\gamma}$-PGA, HH) hm ${\gamma}$-PGA for 4 weeks. The HL and HH groups exhibited significantly lower levels of serum triglyceride, total cholesterol, LDL cholesterol, and free fatty acids than the HC group. The administration of hm ${\gamma}$-PGA reduced serum ALT and AST levels. The activities of lipogenic enzymes such as hepatic malic enzyme and glucose-6-phosphate dehydrogenase as well as glucose-6-phosphate dehydrogenase mRNA expression were significantly decreased by hm ${\gamma}$-PGA administration (p < 0.05). These results indicate that hm ${\gamma}$-PGA has an anti-hypertriglyceridemic effect in high-fructose diet-induced hypertriglyceridemic rats.

• The Korean Journal of Mycology
• /
• v.15 no.3
• /
• pp.169-174
• /
• 1987

The studies on the screening and properties of Raw Starch Saccharifying Microorganism were as follows;Apotent mold strain was selected and screened to digest raw starch, which was classified as a strain of Aspergillus sp. SN-871. The crude enzyme production was maximized when grown on wheat bran media for 5 days at $30^{\circ}C$ and pH 4.0. The stable range of pH was 2 to 5.

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

• Animal Bioscience
• /
• v.37 no.3
• /
• pp.492-499
• /
• 2024

Objective: The objective was to determine the influence of amino acid (AA) supplementation during the adaptation period on the ileal digestibility of crude protein and AA in corn and soybean meal (SBM) fed to pigs. Methods: Six barrows with an initial body weight of 30.9±2.6 kg fitted with a T-cannula at the distal ileum were assigned to a 6×6 Latin square design with 6 dietary treatments and 6 periods. Two experimental diets contained corn or SBM as the sole AA source and an N-free diet was additionally prepared. For AA supplementation groups, an AA mixture consisted of Gly, Lys, Met, Thr, Trp, Ile, Val, His, and Phe was added to the corn diet and the N-free diet at the expense of cornstarch, and an AA mixture of Lys, Met, and Thr was added to the SBM diet. All diets contained 0.5% of chromic oxide. The 6 experimental diets were fed to the pigs for four and half days, and the 3 diets containing an AA mixture were switched to the respective diets without AA mixture during the following two and half days. Ileal digesta were collected on days 6 and 7. Results: The addition of an AA mixture during the adaptation period increased apparent ileal digestibility of Arg and Trp in corn (p<0.05) but did not affect that in SBM. The addition of an AA mixture during the adaptation period increased apparent ileal digestibility of Pro and Gly regardless of feed ingredient (p<0.05) but did not affect that of other AA. All AA except Pro in corn and SBM were unaffected by the addition of the AA mixture during the adaptation period. Conclusion: The addition of amino acids to a low-protein diet during the adaptation period does not affect the standardized ileal digestibility of indispensable amino acids in pigs.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.6 no.2
• /
• pp.129-139
• /
• 2003

Purpose: The aim of the study was to evaluate the long-term outcome of glycogen storage disease (GSD) type 1 with particular reference to hepatic adenoma and hepatocellular carcinoma, and to analyze risk factors affecting the development of hepatic adenoma in GSD type 1. Methods: Forty-three GSD type 1 patients (31 males and 12 females, mean age $13.9{\pm}6.4$ years) were analyzed retrospectively. Hepatic adenoma was detected on abdominal USG and diagnosed on histologic examination. Clinical profiles were compared between patients with hepatic adenoma (n=16) and age-matched controls without hepatic adenoma (n=16). Results: 1) Of 43 GSD type 1 patients, 16 (37.2%) had hepatic adeoma. Hepatic adenoma was detected at the age of mean $14.2{\pm}4.1$ years (range: 7.9~25.7 years). Fourteen (87.5%) adenomas were multiple at detection. 2) Comparison of the clinical profiles between adenoma group and non-adenoma group revealed that age at first introduction of uncooked cornstarch treatment was significantly late in adenoma group compared with non-adenoma group ($9.1{\pm}5.2$ years vs. $3.0{\pm}1.8$ years, p=0.003). Portocaval shunt surgery was performed in 11 (68.8%) patients in adenoma group and 3 (18.8%) in non-adenoma group (p=0.004). Hepatic adenoma developed mean $5.8{\pm}4.2$ years after shunt operation. 3) One patient was diagnosed as hepatocellular carcinoma at the age of 25.7 years. Conclusion: Hepatic adenoma is an important late complication of GSD type 1 with the risk of malignant transformation. Early introduction of cornstarch therapy with strict metabolic control is needed to prevent the development of hepatic adenoma in GSD type 1.

• Asian-Australasian Journal of Animal Sciences
• /
• v.25 no.12
• /
• pp.1748-1758
• /
• 2012

Two experiments were conducted to evaluate the effects of chemical composition of wheat shorts and red dog on energy and amino acid digestibility in growing pigs and to establish prediction models to estimate their digestible (DE) and metabolizable (ME) energy content and as well as their standardized ileal digestible (SID) amino acid content. For Exp. 1, sixteen diets were fed to thirty-two growing pigs according to a completely randomized design during three successive periods. The basal diet was based on corn and soybean meal while the other fifteen diets contained 28.8% wheat shorts (N = 7) or red dog (N = 8), added at the expense of corn and soybean meal. Over the three periods, each diet was fed to six pigs with each diet being fed to two pigs during each period. The apparent total tract digestibility (ATTD) of energy in wheat shorts and red dog averaged 75.1 and 87.9%. The DE values of wheat shorts and red dog averaged 13.8 MJ/kg (range 13.1 to 15.0 MJ/kg) and 15.1 MJ/kg (range 13.3 to 16.6 MJ/kg) of dry matter, respectively. For Exp. 2, twelve growing pigs were allotted to two $6{\times}6$ Latin Square Designs with six periods. Ten of the diets were formulated based on 60% wheat shorts or red dog and the remaining two diets were nitrogen-free diets based on cornstarch and sucrose. Chromic oxide (0.3%) was used as an indigestible marker in all diets. There were no differences (p>0.05) in SID values for the amino acids in wheat shorts and red dog except for lysine and methionine. Apparent ileal digestibility (AID) and SID values for lysine in different sources of wheat shorts or red dog, which averaged 78.1 and 87.8%, showed more variation than either methionine or tryptophan. A stepwise regression was performed to establish DE, ME and amino acid digestibility prediction models. Data indicated that fiber content and amino acid concentrations were good indicators to predict energy values and amino acid digestibility, respectively. The present study confirms the large variation in the energy content and amino acid digestibility in wheat shorts and red dog, and describes the factors that influence this variation and presents equations based on chemical composition that could probably be used to predict the DE and ME values as well as the amino acid digestibility of wheat shorts and red dog.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.20 no.1
• /
• pp.24-28
• /
• 2020

Type III Glycogen storage disease (Type III GSD, OMIM#232400) is a genetic metabolic disorder in which undigested glycogen accumulates in the organs due to lack of glycogen debranching enzyme caused by AGL mutation. The clinical symptoms of type III GSD include hepatomegaly, delayed growth, hypoglycemia and muscle weakness. These clinical symptoms are similar to those of other types of GSD, making it difficult to distinguish clinically. The authors report a case of type III GSD diagnosed by gene panel sequencing. A 11-month old male patient was presented with hepatomegaly. In liver biopsy, glycogen was accumulated in hepatocytes, suggesting GSDs. For differential diagnosis of types of GSD, gene panel sequencing for GSDs was performed. As a result, two novel pathogenic compound heterozygous variants: c.311_312del (p.His104Argfs*15) and c.3314+1G>A in AGL were detected and the patient was diagnosed as type III GSD. After diagnosis, he started dietary treatment with cornstarch, and has been free from complications. After two years, two same variants were also identified in the chorionic villous sampling of the pregnant mother, and the fetus was diagnosed as type III GSD. Gene panel sequencing is useful for diagnosis of disease which is indistinguishable by clinically and has high genetic heterogeneity, such as GSD. After diagnosis, familial genetic analysis can provide adequate genetic counseling and rapid diagnosis.

• Asian-Australasian Journal of Animal Sciences
• /
• v.26 no.5
• /
• pp.691-699
• /
• 2013

Three experiments were conducted to evaluate the metabolizable energy (ME) value, standardized ileal digestibility (SID) of amino acids (AA) of soybean meal (SBM), soy protein concentrate (SPC) and fermented soybean meal (FSBM), and the application of these products in early-weaned piglets. In Exp. 1, four barrows with initial body weight (BW) of $14.2{\pm}1.4$ kg were used in a $4{\times}4$ Latin square design. The diet 1 contained corn as the only energy source. The other three diets replaced 25% of corn in diet 1 with one of the three soybean products, and the digestable energy (DE) and ME contents were determined by difference. In Exp. 2, four barrows (initial BW of $18.2{\pm}1.5$ kg) were fitted with ileal T-cannulas and allotted to a $4{\times}4$ Latin square design. Three cornstarch-based diets were formulated using each of the soybean products as the sole source of AA. A nitrogen-free diet was also formulated to measure endogenous losses of AA. In Exp. 3, ninety six piglets (initial BW of $5.6{\pm}0.9$ kg) weaned at $21{\pm}2$ d were blocked by weight and assigned to one of three treatments for a 21-d growth performance study. The control diet was based on corn and SBM, the two treatments' diets contained either 10% SPC or FSBM and were formulated to same SID lysine to ME ratio of 3.6 g/Mcal. The results showed that the ME content of SPC was greater than SBM (p<0.05). The SID of most AA in SPC was greater than the SID of AA in SBM (p<0.05). For the essential AA, the SID of histidine, isoleucine, leucine, lysine and threonine in FSBM were greater than in SBM (p<0.05). Even though they were fed same SID lysine to ME ratio of 3.6 g/Mcal diets, pigs fed SPC and FSBM diets had greater weight gain, G:F (p<0.05) and better fecal score (p<0.05) than pigs fed SBM diet. In conclusion, SPC showed a higher ME content and SID of AA than the SBM. SID of some essential AA in FSBM was higher than SBM and was similar with SPC. But the lower antigenic proteins and anti-nutritional factors content in SPC and FSBM may be the main factors affecting the performance of early-weaned piglets rather than the increased ME content and SID of AA.