• Clinical and Experimental Pediatrics
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• 제62권2호
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• pp.43-47
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• 2019

Hyperammonemia can be caused by several genetic inborn errors of metabolism including urea cycle defects, organic acidemias, fatty acid oxidation defects, and certain disorders of amino acid metabolism. High levels of ammonia are extremely neurotoxic, leading to astrocyte swelling, brain edema, coma, severe disability, and even death. Thus, emergency treatment for hyperammonemia must be initiated before a precise diagnosis is established. In neonates with hyperammonemia caused by an inborn error of metabolism, a few studies have suggested that peritoneal dialysis, intermittent hemodialysis, and continuous renal replacement therapy (RRT) are effective modalities for decreasing the plasma level of ammonia. In this review, we discuss the current literature related to the use of RRT for treating neonates with hyperammonemia caused by an inborn error of metabolism, including optimal prescriptions, prognosis, and outcomes. We also review the literature on new technologies and instrumentation for RRT in neonates.

• 대한유전성대사질환학회지
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• 제23권1호
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• pp.1-11
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• 2023

Inborn errors of metabolism encompass a wide variety of disorders, frequently affecting bone. This review presents a comprehensive retrospect on the primary involvement of bone in inborn errors of metabolism. Primary involvement of bone in inborn errors of metabolism includes entities that primarily affect the bone marrow, mineral component or cartilage. These include lysosomal storage disorders, hypophosphatasia, and hereditary hypophosphatemic rickets. In this review, we discuss the primary involvement of bone in inborn errors of metabolism (hypophosphatasia, X-linked hypophosphatemic rickets, Gaucher disease, and mucopolysaccharidoses) along with the therapeutic agents used in clinical settings, diagnostic strategies, and general management. With the development of disease-specific targeted therapies and supportive care, more number of patients with these disorders live longer and survive into adulthood. Moreover, skeletal symptoms have become a more prominent feature of these disorders. This makes the awareness of these skeletal symptoms more important.

• Molecules and Cells
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• 제47권2호
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• pp.100010.1-100010.12
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• 2024

Recently, the incidence of metabolic dysfunction-associated steatotic liver disease (MASLD) is increasing due to the high prevalence of metabolic conditions, such as obesity and type 2 diabetes mellitus. Steatotic liver is a hotspot for cancer metastasis in MASLD. Altered lipid metabolism, a hallmark of MASLD, remodels the tissue microenvironment, making it conducive to the growth of metastatic liver cancer. Tumors exacerbate the dysregulation of hepatic metabolism by releasing extracellular vesicles and particles into the liver. Altered lipid metabolism influences the proliferation, differentiation, and functions of immune cells, contributing to the formation of an immunosuppressive and metastasis-prone liver microenvironment in MASLD. This review discusses the mechanisms by which the steatotic liver promotes liver metastasis progression, focusing on its role in fostering an immunosuppressive microenvironment in MASLD. Furthermore, this review highlights lipid metabolism manipulation strategies for the therapeutic management of metastatic liver cancer.

• 재활간호학회지
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• 제4권2호
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• pp.165-178
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• 2001

The purpose of this study is to determine the effect of diabetes education program on Glucose Metabolism(blood sugar, HbA1c) and Lipid Metabolism(total cholesterol, triglyceride, low density lipoprotein, high density lipoprotein), Self-efficacy in non-insulin independent diabetes mellitus. The study design was a non equivalent control group pre-test post-test design. Data for the study were collected from March 12 to June 19, 2001. Sixty-two research subjects were assigned to experimental(36) and control(26) groups. The collected data was analyzed using the Chi-Square test, t-test by spsswin program The results are as follows : 1. Experimental group had higher level of glucose metabolism than control group(FBS ; t=-3.317, p=.002, HbA1c; t=-4.956, p=.000). 2. Level of lipid metabolism were partly a significant different between experimental group and control group(Triglyceride ; t=-2.108, p=.039). 3. Experimental group had higher efficacy score than control group(t=4.651, p=.000). In conclusion, the study supported the effects of diabetes education program to increase metabolism an d self-efficacy. Further study with a longitudinal design is suggested to verify the effect of diabetes education program in NIDDM and standardized diabetes education program.

• 대한유전성대사질환학회지
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• 제6권1호
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• pp.89-93
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• 2006

• Plant Biotechnology Reports
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• 제3권2호
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• pp.167-174
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• 2009

To identify genes involved in rice Pi5-mediated disease resistance to Magnaporthe oryzae, we compared the proteomes of the RIL260 rice strain carrying the Pi5 resistance gene with its susceptible mutants M5465 and M7023. Proteins were extracted from the leaf tissues of both RIL260 and the mutant lines at 0, 24, and 48 h after M. oryzae inoculation and separated by two-dimensional polyacrylamide gel electrophoresis (2-DE). Matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) analysis identified eight proteins that were differently expressed between the resistant and susceptible plants (three down- and five up-regulated proteins in the mutants). The down-regulated proteins included a triosephosphate isomerase (spot no. 2210), a 2,3-bisphosphoglycerate-independent phosphoglycerate mutase (no. 3611), and an unknown protein (no. 4505). In addition, the five up-regulated proteins in the mutants were predicted to be a fructokinase I (no. 313), a glutathione S-transferase (no. 2310), an atpB of chloroplast ATP synthase (no. 3616), an aminopeptidase N (no. 3724), and an unknown protein (no. 308). These results suggest that proteomic analysis of rice susceptible mutants is a useful method for identifying novel proteins involved in resistance to the M. oryzae pathogen.

• Archives of Pharmacal Research
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• 제26권1호
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• pp.89-94
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• 2003

In vivo clearance of BMS-182874 was primarily due to metabolism via stepwise N-demethylation. Despite in vivo clearance approached ca 50％ of the total liver plasma flow, BMS-182874 was completely bioavailable after oral administration in rats. Saturable first-pass metabolism and the role of extrahepatic tissue were evaluated as possible reasons for complete oral bioavailability despite extensive metabolic clearance. Pharmacokinetic parameters were obtained after an intravenous and a range of oral doses of BMS-182874 in rats. Bile and urine were collected from bile-duct cannulated (BDC) rats and the in vivo metabolic pathways of BMS-182874 were evaluated. Pharmacokinetics of BMS-182874 were also compared in nephrectomized (renally impaired) vs. sham-operated control rats. Oral bioavailability of BMS-182874 averaged 100％, indicating that BMS-182874 was completely absorbed and the first-pass metabolism (liver or intestine) was negligible. The AUC and C/sub max/ values increased dose-proportionally, indicating kinetics were linear within the oral dose range of 13 to 290 mmole/kg. After intravenous administration of BMS-182874 to BDC rats, about 2％ of intact BMS-182874 was recovered in excreta, indicating that BMS-182874 was cleared primarily via metabolism in vivo. The major metabolite circulating in plasma was the mono-N-desmethyl metabolite and the major metabolite recovered in excreta was the di-N-desmethyl metabolite. In vivo clearance of BMS-182874 was significantly reduced in nephrectomized rats. These observations suggest saturable first-pass metabolism is unlikely to be a mechanism for complete oral bioavailability of BMS-182874. Reduced clearance observed in the nephrectomized rats suggests that extrahepatic tissues (e.g., kidneys) may play an important role in the in vivo clearance of xenobiotics that are metabolized via N-demethylation.

• Journal of Yeungnam Medical Science
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• 제31권2호
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• pp.103-108
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• 2014

Thyroglossal duct cyst (TGDC) carcinoma generally shows a favorable prognosis. If metastasis is present latently, it may not threaten the patient's life immediately. It has been shown, however, that larger than 1 cm papillary carcinoma (PC), level VI metastasis to the lymph node (LN), which is the nearest to the thyroid, independently predicts a worse prognosis. In the case presented herein, a 61-year-old female patient was diagnosed with an about 3 cm PC in the TGDC, particularly the columnar variant subtype, one of the aggressive variants. She had occult papillary thyroid microcarcinoma, but no LN metastasis. Even though she underwent the Sistrunk procedure and total thyroidectomy with central compartment neck dissection followed by high-dose radioactive iodine remnant ablation, however, the cancer cells spread to level IV neck LN, and finally to the lung. Therefore, when a patient is diagnosed with an aggressive histologic variant of PC in the TGDC, even without LN metastasis, the invasive surgical approach and close postoperative surveillance are necessary, with consideration of the risk of disease progression. Therefore, if it is possible to stratify the risk for patients, higher-risk patients can be offered a more invasive therapeutic approach.

• 운동영양학회지
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• 제23권3호
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• pp.1-12
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• 2019

[Purpose] The purpose of this study was to determine whether different types of carbohydrate diets with or without exercise changes energy metabolism at rest and during exercise. [Methods] To minimize differences in food and energy intake between experimental groups, mice were pairfed. After 1 week of adaptation, 40 male ICR mice (6 weeks old) were randomly divided into four groups: Sta. (high fat + high starch), Scu. (high fat + high sucrose), StaEX. (high fat + high starch + exercise), and SucEX. (high fat + high sucrose + exercise). StaEX. and ScuEX. groups underwent training by running on a treadmill five times a week. After 10 weeks of training, energy metabolism was measured for 24 h and during a 1 h exercise period. [Results] The final body weight showed no significant difference between the groups. However, the weight of abdominal tissues (epididymal, perirenal, and mesenteric adipose tissue) in training groups was markedly decreased following 10 weeks of training. Results of all energy metabolism (24 h at rest and during 1 h of exercise) showed no significant interactions between diet and exercise. A brief summary of the results of the energy metabolism is that the metabolism related indicators over 24 h were more affected by the dietary pattern than the exercise but during the 1 h of exercise, training had more effect on energy metabolism than diet. [Conclusion] Our findings confirm that: (a) the type of carbohydrates included in the diet influence the metabolic responses over 24 h, (b) training had more effect on energy metabolism than diet during 1 h of exercise, (c) both results; abdominal adipose tissue weight and fat oxidation during exercise are suggestive for a beneficial effect of moderate physical activity on weight maintenance.

• Applied Biological Chemistry
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• 제46권1호
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• pp.66-68
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• 2003