• Clinical and Experimental Pediatrics
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• 제58권6호
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• pp.234-237
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• 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
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• 제15권11호
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• pp.1591-1598
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• 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
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• 제23권6호
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• pp.785-793
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• 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.

• 한국균학회지
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• 제15권3호
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• pp.169-174
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• 1987

생전분 당화효소 생산능이 우수한 균주를 얻기위하여 방앗간의 폐수, 주정회사의 폐수와 산성막걸리 공장의 토양 등에서 생전분 당화효소 생산능이 있는 효모 29종, 세균 35종, 곰팡이 40종을 순수분리하고 이들중에서 가장 생전분 당화력이 강한 곰팡이 1종을 선정하여 동정한 후 효소생산 조건과 pH 안정성을 측정한 결과는 다음과 같다. 1. 생전분 당화효소 생산능이 가장 강한 분리균주는 Aspergillus sp. SN-871로 동정되었다. 2. 효소생산 조건은 pH 4.0, 온도 $30^{\circ}C$와 배양기간이 5일 일때 가장 좋았다. 3. 탄소원으로 생전분 종류에서는 1% raw cornstarch 첨가시, 생전분 대신의 첨가에서는 0.5% frutose 첨가시 효과가 가장 좋았다. 4. 질소원으로는 0.1%의 $NaNO_3$ 첨가가 가장 효과가 좋았다. 5. 조효소의 pH 안정범위는 pH 2.0에서 pH 5.0까지가 비교적 안정하였다.

• 대한유전성대사질환학회지
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• 제23권2호
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• pp.8-14
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• 2023

간 당원축적병 0형은 glycogen synthase 2 유전자에 부호화되어 있는 간 당원 합성효소의 결핍으로 비정상적으로 당원 생성이 되는 상염색체 열성 유전 질환이다. 당원축적병 0형의 임상 양상은 공복시에 고케톤혈증 저혈당증을 나타내고 식사후 고혈당과 고젖산혈증을 보인다. 당원축적병 0형은 현재까지 적은 수만 보고되었는데 증상이 경하거나 심한 저혈당이 드물고 또는 무증상이거나 나이가 듦에 따라 점차 증상이 사라지는 양상을 보이기 때문에 진단을 놓치는 경우가 있을 것으로 생각된다. 필수적 치료 전략은 포도당신생성을 자극하기 위해 고단백 식사, 낮동안 저혈당을 방지하기 위해서 잦은 식사 횟수, 밤 동안 천천히 포도당을 방출하기 위해 생옥수수전분가루 같은 복합 탄수화물을 먹는 것이다. 당원축적병 0형은 예후는 좋고 적절한 치료를 하면 정상적으로 성장하며 합병증도 발생하지 않는다. 성인이 될수록 심한 저혈당은 보이지 않게 되지만 지속적인 식사 관리는 필요하다.

• Animal Bioscience
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• 제37권3호
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• pp.492-499
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• 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
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• 제6권2호
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• pp.129-139
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• 2003

목 적: 당원병 제1형은 어린 소아연령에서 발현하는 가장 심한 형태의 당원병이다. 장기간 관찰시 일부 환자에서 간선종이 발생하고 드물게 간암이 합병되어 예후에 심각한 영향을 미칠 수 있다고 알려져 있지만, 아직까지 이에 대한 연구가 거의 이루어지지 못하였다. 본 연구에서는 한국의 당원병 제1형 환자들의 추적관찰을 통해 간선종과 간암 발생의 측면에서 당원병 제1형의 장기적인 예후를 살펴보고자 하였다. 방 법: 1982년 3월에서 2003년 2월까지 서울대병원 소아과에서 간 조직 검사 및 효소검사를 통해 당원병 제1형으로 진단된 43명의 환자(제1a형 40명, 제1b형 3명)를 대상으로 하였다. 복부 초음파, 전산화단층촬영 또는 자기공명영상을 시행하여 간선종의 발생여부를 관찰하였으며, 간의 종괴가 확인된 경우 가능하면 조직검사를 시행하여 간선종 및 간암을 확진하였다. 간선종 발생에 관여하는 위험요인을 알아보기 위하여 간선종이 발생한 환자를 연령에 대해 짝짓기를 시행하여 선종군과 비선종군에서 여러 임상 지표를 비교하였다. 결 과: 1) 전체 43명 중 16명(37.2%)에서 간선종이 발생하였는데, 간선종이 처음 발견된 나이는 평균 $14.2{\pm}4.1$세(범위: 7.9~25.7세)였다. 처음 발견시 다발성 선종인 경우는 14례(87.5%)였다. 2) 간선종이 발생하였던 선종군(n=16)과 비선종군(n=16)을 비교하였을 때, 신장질환이 선종군에서는 7명(43.8%)에서 동반되고, 비선종군에서는 1명(6.3%)에서 병발하여 두 군 간에 유의한 차이를 보였다(p=0.037; 교차비 11.667, 95% 신뢰구간=1.227~110.953). 저신장은 선종군 6명(37.5%)과 비선종군의 10명(62.5%)에서 관찰되어 두 군 간에 유의한 차이가 없었다(p=0.157). 당원병 제1형 환자의 대부분에서 생옥수수 전분 치료를 시행하였는데, 생옥수수 전분 치료를 시작한 연령은 선종군이 $9.1{\pm}5.2$세인 반면, 비선종군은 $3.0{\pm}1.8$세로서 통계적으로 유의하게 선종군의 치료 시작 연령이 늦었다(p=0.003). 문맥하공정맥 문합술 시행 당시의 연령 차이가 없는 상태에서(p=0.170), 선종군의 11명(68.8%)과 비선종군의 3명(18.8%)에서 단락수술을 시행 받아 두 군간에 유의한 차이를 보였다(p=0.004; 교차비 9.533, 95% 신뢰구간=1.847~49.204). 고콜레스테롤혈증, 저혈당, 고요산혈증 등의 여부는 두 군 간에 유의한 차이를 보이지 않았다(p=0.833, p=0.273, p=0.550). 3) 25.7세에 간선종이 발견되었던 1명은 26.5세에 간암으로 진단되어 간동맥 항암 화학 색전술을 시행 받았다. 결 론: 간선종은 당원병 제1형 환자들을 장기간 추적관찰 하였을 때 비교적 흔하게 발생하는 합병증으로서, 일부에서 악성화 될 가능성이 있어 중요성이 매우 크다. 당원병 제1형 환자들의 장기적 예후를 위해서는 생옥수수 전분 치료의 조기 도입과 규칙적인 추적관찰을 통한 간선종의 조기 발견이 필요하다.

• Asian-Australasian Journal of Animal Sciences
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• 제25권12호
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• pp.1748-1758
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• 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.

• 대한유전성대사질환학회지
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• 제20권1호
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• pp.24-28
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• 2020

당원병 III형은 GDE 결핍으로 분해되지 않은 당원이 간 또는 근육에 축적되는 유전대사이상질환이다. AGL 유전자 변이에 의해 발생하고 높은 유전적 이질성을 가지고 있다. 당원병 III형의 임상증상은 간 비대, 성장 지연, 저혈당 등이 있다. 이러한 임상 증상은 다른 타입의 당원병의 증상과 비슷하여 임상적으로는 구분하기가 어렵다. 저자들은 간 비대 주소로 내원한 11개월 환아에서 유전자 패널 검사로 진단된 당원병 III형 증례를 보고하고자 한다. 간 조직검사결과 간세포에 글리코겐이 축적되어 있어 당원병을 의심하였으며, 당원병 아형의 감별 진단을 위해 유전자 패널 검사를 시행하였다. 그 결과, AGL 유전자에서 이전에 보고된 바 없는 c.311_312del와 c.3314+1G>A 변이가 이형접합체로 발견되어 당원병 III형으로 진단하였다. 진단 후 생옥수수 전분가루를 복용하는 식이요법 시작하였고, 생후 35개월인 현재까지 급·만성 합병증 없는 상태이다. 또한, 가족 검사를 통해 부모가 각각 보인자임을 확인하였고, 임신된 동생의 융모막 검사에서도 동일한 변이가 확인되어 출산 후 재검 및 조기 식이요법을 시행할 예정이다. 유전자 패널 검사법은 당원병과 같이 임상적으로 구분이 어려우며 높은 유전적 이질성을 가진 질환의 감별 진단 시 시간과 비용을 아낄 수 있어 유용하다. 또한 정확한 분자 유전학적 진단은 환아와 가족에게 질환에 대한 정확한 정보 및 치료 예방, 산전 유전상담을 제공하는 데 도움이 된다.

• Asian-Australasian Journal of Animal Sciences
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• 제26권5호
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• pp.691-699
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• 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.