• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제11권sup1호
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• pp.72-82
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• 2008

Wilson disease (WD) is an autosomal recessive disorder of copper metabolism that results in accumulation of copper primarily in the liver, the brain and the cornea. Mutations in the WD gene, ATP7B cause failure of copper excretion from hepatocyte into bile and a defective synthesis of ceruloplasmin. More than 370 mutations are now recognized, scattering throughout the ATP7B gene. Since WD has protean clinical presentations, awareness of WD in clinical practice is important for the early diagnosis and prevention of accumulated copper toxicity. None of the laboratory parameters alone allows a definite diagnosis of WD. There are numerous pitfalls in the diagnosis of WD. Low serum ceruloplasmin concentrations, increased 24 hour urinary copper excretion, increased hepatic copper concentrations and the presence of Kayser-Fleischer rings in the cornea are major diagnostic points. A combination of any two of these 4 laboratory findings is strong support for a diagnosis of WD. Molecular methods are now being used to aid diagnosis. Molecular genetic testing has confirmed the diagnosis in individuals in whom the diagnosis is not clearly established biochemically and clinically. Siblings should be screened for WD once an index case has been diagnosed. Discrimination of heterozygotes from asymptomatic patients is essential to avoid inappropriate lifelong therapy for heterozygotes. Genetic testing, either by haplotype analysis or by mutation analysis, is the only reliable tool for differentiating heterozygote carriers from affected asymptomatic patients. Currently, genetic testing is of limited value in the primary diagnosis. However, genetic testing will soon play an essential role in diagnosing WD as rapid advancement of biomedical technology will allow more rapid, easier and less expensive mutation detection.

• 한국식품과학회지
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• 제25권5호
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• pp.571-575
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• 1993

대두 펩타이드의 표면소수도가 혈청 콜레스테롤의 농도에 미치는 영향을 조사하기 위하여, 대두 단백질(ISP), 카세인(CNP), 이들 단백질을 펩신으로 가수분해하여 pH에 따른 펩타이드 침전 획분들(SHT, SH8, SH6, SH4, CHT, CH6, CH5, CH4)을 흰쥐에 섭취시키고 혈청 콜레스테롤 농도 및 분변 스테로이드이 배설량을 측정하였다. 그리고 각 펩타이드의 표면소수도를 ANS 형광법 및 SDS 결합법으로 측정하여, 이들의 상관관계를 분석한 결과, 펩타이드의 ANS 표면소수도가 높아질수록 분변으로 배설된 스테로이드량은 증가하였으며(r=0.81), 혈청 콜레스테롤 농도는 낮아졌다.(r=-0.868). 그러나 SDS 표면소수도는 그들과 상관관계가 없었다. 또한 대두 단백질은 가수분해에 의하여 ANS 표면소수도가 증가하였다. 이상의 결과는 흰쥐의 담즙염이, 소화중 생성된 높은 표면소수도의 펩타이드와 결합하여 체외로 배출되므로서 대두단백질의 섭취에 의한 혈청 콜레스테롤 농도가 낮아짐을 시사하였다.

• Journal of Nutrition and Health
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• 제27권2호
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• pp.127-140
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• 1994

The effect of milk in low and high cholesterol diet was invesigated on serum cholesterol metabolism and lipid contents of serum, aorta, liver of rats. Weanling male Sprague-Dawley rats were divided into low(0.01% w/w) and high(1.01% w/w) cholesterol-diets groups. Low cholesterol groups subdivided into four groups ; control group was given water and three milk groups were given low heat milk(LM), ultra-high heat milk(HM), and powder milk(PM), respectivily, instead of water. High cholesterol groups were consisted of three groups ; control, LM, and HM groups. After feeding these experimental diets for six weeks, lipid levels were measured in serum and tissure and dried feces were analyzed for neutral and acidic sterols. Results obtained from this study are as follows : 1) Nutrient intakes, body weight gains and aorta weights did not differ among groups, but liver weights were higher in high cholesterol fed rats than low cholesterol fed rats. 2) Serum protein contents were increased independently by intakes of high cholesterol and milk. 3) Serum total cholesterol and triglyceride levels were increased but phospholipid levels and HDL-cholesterol/total-cholesterol ratios were decreased by high cholesterol in diet. And milk supplementation decreased serum total cholesterol and triglyceride levels but increased phospholipid levels and HDL-cholesterol/total-cholesterol ratios. 4) Contents of cholesterol and triglyceride in aorta and liver were elevated by dietary high cholesterol and lowered by consumption of all three types of milk. 5) Levels of cholesterol and triglyceride among serum, aorta and liver were highly correlated (r=0.7-0.9, p<0.001). 6) Fecal excretion of total sterols was three times high in high cholesterol group, compared with low cholesterol groups and were increased about 20% by milk consumption. 7) The effects of milk were more pronouncely shown in low cholesterol groups and mostly confined to LM and HM groups, rarely shown in PM group. It is concluded from the present study that milk had the hypolipidemic as well as hypocholes terolemic effect, which appears to be mediated through increased fecal bile acid excretion. But the effect is likely to be shaded by excess consumption of dietary cholesterol and was almost absent in powder milk.

• Journal of Nutrition and Health
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• 제21권2호
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• pp.89-98
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• 1988

율무쌀이 흰쥐의 혈장과 조직의 콜레스테롤, 중성지방, 이지질 및 총담즙산 수준에 미치는 영향을 알아보자 흰쥐에 1%의 콜레스테롤을 첨가시킨 실험식이를 자유급식하여 사양한 결과를 요약하면 다음과 같다. 1) 식이섭취량, 식이효율, 증체량은 각 군간에 유의한 차이가 없었다. 2)형장콜레스테롤 함량은 라이드 급여시 현저히 높았으며 라이드-ㅠ율무쌀을 급여시에는 가장 낮았다. 혈장의 중성지방 역시 라이드 급여시 높았다. 혈장의 인지질 역시 라아드 급여서 현저히 높았고 대두유군이 가장 낮았다. 혈장의 총담즙산 함량은 각군간에 차이가 없었으며 혈장 담즙산은 glycoursodeoxy cholicacid, tauroursodeoxy choilic acid와 cholic acid이었다. 3) 간의 단위무게당 총지질, 콜레스테롤, 중성지방역시 라이드- 율무쌀 급여시 현저히 낮았다. 4) 소장의 단위무게당 총지질, 콜레스테롤, 중성지방 함량도 역시 라이드-율무쌀을 급여시 낮았다. 5)실험식의 소화율을 모든식이 처리간에 차이가 없었으나 콜레스테롤 보유율은 라이드-전분군이 가장 높았고 대두유-율무쌀군이 가장 낮았다.

• Korean Journal of Radiology
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• 제23권4호
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• pp.389-401
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• 2022

Objective: This study aimed to determine a factor for predicting suboptimal image quality of the hepatobiliary phase (HBP) of gadoxetic acid-enhanced MRI in patients with extrahepatic bile duct (EHD) cancer before MRI examination. Materials and Methods: We retrospectively evaluated 259 patients (mean age ± standard deviation: 68.0 ± 8.3 years; 162 male and 97 female) with EHD cancer who underwent gadoxetic acid-enhanced MRI between 2011 and 2017. Patients were divided into a primary analysis set (n = 184) and a validation set (n = 75) based on the diagnosis date of January 2014. Two reviewers assigned the functional liver imaging score (FLIS) to reflect the HBP image quality. The FLIS consists of the sum of three HBP features, each scored on a 0-2 scale: liver parenchymal enhancement, biliary excretion, and signal intensity of the portal vein. Patients were classified into low-FLIS (0-3) or high-FLIS (4-6) groups. Multivariable analysis was performed to determine a predictor of low FLIS using serum biochemical and imaging parameters of cholestasis severity. The optimal cutoff value for predicting low FLIS was obtained using receiver operating characteristic analysis, and validation was performed. Results: Of the 259 patients, 140 (54.0%) and 119 (46.0%) were classified into the low-FLIS and high-FLIS groups, respectively. In the primary analysis set, total bilirubin was an independent factor associated with low FLIS (adjusted odds ratio per 1-mg/dL increase, 1.62; 95% confidence interval [CI], 1.32-1.98). The optimal cutoff value of total bilirubin for predicting low FLIS was 2.1 mg/dL with a sensitivity of 95.1% (95% CI: 88.9-98.4) and a specificity of 89.0% (95% CI: 80.2-94.9). In the validation set, the total bilirubin cutoff showed a sensitivity of 92.1% (95% CI: 78.6-98.3) and a specificity of 83.8% (95% CI: 68.0-93.8). Conclusion: Serum total bilirubin before acquisition of gadoxetic acid-enhanced MRI may help predict suboptimal HBP image quality in patients with EHD cancer.

• 한국식품영양과학회지
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• 제25권6호
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• pp.958-962
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• 1996

해조류인 참도박으로 부터 고지혈증 개선효과를 관찰하기 위하여 고지혈증을 유발시킨 흰쥐에 메탄올 추출물을 투여하고 혈액 및 간조직에서의 지방 함량을 측정하였다. 그 결과 참도박의 메탄올 추출물의 투여는 지방조직의 무게는 정상군에는 미치지 않으나 대조군에 비해 다소 감소하는 경향을 보였다. 고지방식이로 인위적으로 고지혈증을 유발시켰을때 정상군보다 total cholesterol, LDL-cholestero의 함량이 증가되던 것이 추출물의 투여로 total cholesterol의 함량이 현저히 감소되었으나 LDL-cholesterolt의 함량은 별다른 영향이 없었다. 또한 이들의 고지방식이군 보다 total lipid, triglyceride의 함량도 감소시켰다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제8권2호
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• pp.263-268
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• 2005

본 저자들은 심한 간 부전과 함께 복통, 구토, 의식 변화, 사지 마비, 배뇨 장애 및 호흡부전을 동반한 EPP 예를 경험하였기에 보고하는 바이다.

• Asian-Australasian Journal of Animal Sciences
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• 제21권6호
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• pp.890-895
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• 2008

The antioxidant activity of resveratrol in cholesterol-fed rats, along with its hypolipidemic effects was determined. Thirty two male Sprague-Dawley (SD) rats were randomly divided into three groups (Control, Res30 and Res70) and fed a hyperlipidemic diet for 4 weeks. Resveratrol was suspended in 0.3% carboxymethyl cellulose (CMC) solution and given to rats of the Res30 and Res70 groups once a day for 4 weeks by oral intubation at a dose of 30 and 70 mg/kg body weight, respectively. The control group received 0.3% CMC solution alone. Resveratrol significantly lowered serum lipid, hepatic cholesterol (TC) and triglyceride (TG) levels compared to the control. Excretion of bile acids was significantly enhanced by resveratrol. The overall potential of the antioxidant system was significantly enhanced by the resveratrol as plasma and hepatic thiobarbituric acid reactive substances (TBARS) levels were lowered while serum superoxide dismutase (SOD), glutathione peroxidase (GSH-Px) and catalase (CAT) activities were increased in the cholesterol-fed rats. These findings suggest that resveratrol maintains an antioxidant efficacy as well as its anti-hyperlipidemic effect.

• Preventive Nutrition and Food Science
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• 제2권1호
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• pp.35-41
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• 1997

The pancreas is an important organ in the maintenance of zinc homeostasis. Endogenous zinc is con-tinuously secreted via pancreatic exocrine fluid or to a lesser extent in bile. Much of the endogenous secretion must be reabsorbed to sustain zinc homeostasis. The objective of this study was to estimate the relative size of the pancreatic/biliary zinc pool in comparision to the dietary zinc intake, and to study the effect of the phytate and calcium on the zinc homeostasis using a rat model. At the termination of the experiment, pan-creatic/biliary fluid was collected from the rats. Both radioactivity and total zinc were measured and the relative size of the pancreatic/biliary zinc pool was estimated. To determine the effect of phytate and calcium on zinc homeostsis, dietary zinc intake, the amount of zinc in pancreatic.biliary fluid and fecal zinc excretion were measured. The flow rate of pancreatic/biliary fluid, as corrected for tubing constriction, gives the corrected zinc concentration in the pancreatic/biliary fluid was 2.2 times higher than dietary zinc intake. To maintain zinc homeostasis, zinc absorption/reabsorption was very efficient in the current model; 76%, 88% of absorption/reabsorption for low calcium group and high calcium group 81% for phytate group and non-phytate group, respectively.

• Food Science and Biotechnology
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• 제18권6호
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• pp.1330-1335
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• 2009

Although beneficial effects of dietary plant proteins on lipid metabolism are well documented, not much information exists on the influence of different seafood proteins on the lipid metabolism. The present study evaluated the effect of 2 marine proteins (tuna protein and scallop ovary proteins) in comparison to casein and soy protein in male Wistar rats. The concentration of total lipids in the plasma of rats fed experimental diets was significantly lower from that of control (278.2 mg/dL) group (p<0.05); and, the liver lipid content was not significantly different (p>0.05). Fecal excretion of cholesterol and bile acids was significantly higher in marine proteins and soy protein fed groups compared to casein only fed control (6.1 and 6.4 mg/day, respectively) group (p<0.05). No significant differences were observed in the mRNA concentrations of different transcriptional factors (p>0.05).