• Title/Summary/Keyword: metabolic disorder

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HYPOPHOSPHATASIA : CASE REPORT (Hypophosphatasia 환아의 치료 증례)

  • Park, Soo-Jung;Lee, Jae-Ho;Choi, Hyung-Jun;Kim, Kee-Deog;Choi, Byung-Jai
    • Journal of the korean academy of Pediatric Dentistry
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    • v.25 no.3
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    • pp.555-561
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    • 1998
  • Hypophosphatasia is a rare metabolic disorder which manifests characteristics such as abnormal mineralization of bone and dental tissues, diminished serum and tissue alkaline phosphatase, and increased urinary secretion of PEA. It inherited as an autosomal recessive or dominant trait and occurs in all races. In general, hypophosphatasia can be classified in 4 subtypes which are the perinatal, infantile, childhood, adult type depending upon the age at presentation and severity. In young children with Hypophosphatasia the long bones show irregular defects, and the skull showes poor calcification. In older children with premature closure of the skull sutures there may be multiple lucent area called gyral or convolutional markings, described as resembling beaten copper, presumably resulting from increased intracranial pressure. Examination of the jaws reveals a generalized lucency of the maxilla and mandible. the cortical bone and lamina dura are thin, and the alveolar bone may be deficient. Clinical features of Hypophosphatasia include premature loss of deciduous teeth, especially incisors, hypoplasia or aplasia of root cementum, enamel hypoplasia, irregular calcification of dentin, large pulp chamber, and resorption of marginal alveolar bone and roots. Our report involves a patient with a chief complaint of early loss of both Mx. and Mn. deciduous incisors. After conducting a through clinical and radiographic examination this patient was referred to pediatrics under the suspicion of hypophosphatasia, the diagnosis proved to be correct and successful results were accomplished through a denture made to improve esthetics and function.

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The Inhibitory Effects of So-yangin Biman-bang on the Obese-Mice Induced by High-Fat Diet (소양인(少陽人) 비만방(肥滿方)이 생쥐의 비만 억제에 미치는 영향)

  • Park, In-Sun;Yoon, Il-Ji
    • Journal of Korean Medicine Rehabilitation
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    • v.20 no.2
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    • pp.35-50
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    • 2010
  • Objectives : In order to investigate the effects of So-yangin Biman-bang(here in after referred to BM) on the obese gene and obese inhibitory, C57BL/6 mice were distinctively induced by high fat diet. Methods : C57BL/6 mice were divided into four groups(normal, high fat diet with control, high fat diet with Reductil(here in after referred to RDT), high fat diet with BM extract) and have been fed for 8 weeks. Through observing the process, the weight change of the adipocytes in body and liver, total cholesterol, LDL-cholesterol, HDL-cholesterol, triglyceride, glucose, leptin change in the serum, the expression of ${\beta}3AR$ and leptin gene in 3T3-L1 cell and primary adipocyte cell. Results : The following results have been found in this study. 1. Losing weight have significantly been shown. 2. The weight change of the body adipocyte and liver have been decreased but no significance has been found. 3. With close observation on the change of blood, HDL-cholesterol has positively been increased in numbers but the level of glucose, LDL-cholesterol, triglyceride have distinctively been decreased. 4. The manifestation of ${\beta}3AR$ in 3T3-L1 cell and primary adipocyte cell have shown a definite increase in numbers but the manifestation of leptin has shown certain decrease in its amount. Conclusions : As results of this, So-yangin Biman-bang is thought to be effective in metabolic syndrom and lipid metabolism disorder that can possibly be occurred by obesity control and treatment. The clinical research will be needed in order to apply to So-yangin constitution in the future.

Hershberger Assays for Bisphenol-A and Its Substitute Candidates

  • Kim, Hee-Su;Kim, Yong-Bin;Choi, Donchan;Cheon, Yong-Pil;Lee, Sung-Ho
    • Development and Reproduction
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    • v.21 no.4
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    • pp.441-448
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    • 2017
  • Bisphenol-A(BPA) is a member of alkylphenol family, and shows adverse effects including reduced fertility, reproductive tract abnormalities, metabolic disorder, cancer induction, neurotoxicity and immunotoxicity. In the present study, we conducted Hershberger assay to evaluate whether the two candidates to replace BPA have androgenic or antiandrogenic activity. The assay was carried out using immature castrated Sprague-Dawley male rats. After 7 days of the surgery, testosterone propionate (TP, 0.4 mg/kg/day) and test materials (low dose, 40 mg/kg/day; high dose, 400 mg/kg/day) were administered for 10 consecutive days by subcutaneous (s.c.) injection and oral gavage, respectively. Test materials were BPA, isosorbide (ISO) and cyclohexanedimethanol (CHDM). The rats were necropsied, and then the weights of five androgen-dependent tissues [ventral prostate, seminal vesicle, levator ani-bulbocavernosus (LABC) muscle, paired Cowper's glands, and glans penis] and three androgen-insensitive tissues (kidney, spleen and liver) were measured. All test materials including BPA did not exhibit any androgenic activity in the assay. On the contrary, antiandrogen-like activities were found in all test groups, and the order of the intensity was CHDM > BPA > ISO in the five androgen-sensitive tissues. There was no statistical difference between low dose treatment and high dose treatment of BPA group as well as ISO group. In CHDM group, high dose treatment exhibited most severe weight reduction in all measured tissues. There was no statistical difference in androgen-insensitive tissue measurements, except BPA groups. Since the effects of ISO treatment on the accessory sex organs were much less or not present at all when compared to those of BPA, ISO could be a strong candidate to replace BPA. CHDM treatment brought most severe weight reduction in all of androgen-sensitive tissues, so this material should be excluded for further screening of BPA substitute selection.

Altered Gene Expression Profiles in the Lungs of Streptozotocin-induced Diabetic Mice

  • Kim, Jung-Hyun;Rasaei, Roya;Park, Sujin;Kim, Ji-Young;Na, Sunghun;Hong, Seok-Ho
    • Development and Reproduction
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    • v.24 no.3
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    • pp.197-205
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    • 2020
  • Diabetes mellitus is a common heterogeneous metabolic disorder, characterized by deposition of extracellular matrix, oxidative stress, and vascular dysfunction, thereby leading to gradual loss of function in multiple organs. However, little attention has been paid to gene expression changes in the lung under hyperglycemic conditions. In this study, we found that diabetes inuced histological changes in the lung of streptozotocin-induced diabetic mice. Global gene expression profiling revealed a set of genes that are up- and down-regulated in the lung of diabetic mice. Among these, expression of Amigo2, Adrb2, and Zbtb16 were confirmed at the transcript level to correlate significantly with hyperglycemia in the lung. We further evaluated the effect of human umbilical cord-derived perivascular stem cells (PVCs) on these gene expression in the lung of diabetic mice. Our results show that administration of PVC-conditioned medium significantly suppressed Amig2, Adrb2, and Zbtb16 upregulation in these mice, suggesting that these genes may be useful indicators of lung injury during hyperglycemia. Furthermore, PVCs offer a promising alternative cell therapy for treating diabetic complications via regulation of gene expression.

A Case Report of Holoprosencephaly (전전뇌증(Holoprosencephaly)의 증례보고)

  • Song, Seung Han;Kang, Nak Heon
    • Archives of Plastic Surgery
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    • v.34 no.4
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    • pp.528-530
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    • 2007
  • Purpose: Holoprosencephaly(HPE) is a rare developmental defect due to incomplete cleavages of the prosencephalon during the third week of fetal development. Chromosomal anomalies, genetic syndrome, teratogen, or genetic disorder of non-syndromic HPE are usually accepted as etiology. The consequences of prechordal mesoderm defect are varying degrees of deficit of midline facial development, especially the median nasal process(premaxilla), and incomplete morphogenesis of the forebrain. We experienced a case of lobar HPE with complete cleft lip and palate. Methods: A female newborn infant was born at $38^{+6}$ weeks' gestational age via NSVD. The infant's birth weight was 3.6 kg, height 52 cm, and head circumference 32.5 cm, showing microcephaly, flat nose, median complete cleft lip & palate, and hypotelorism, along with defects of midfacial development including losses of premaxilla, philtrum, nasal septum, and columella. Results: There were no specific findings noted from the head and neck X-ray and tests for endocrine and metabolic disorders, but clinical characteristics of midface and dysgenesis corpus callosum on brain MRI were seen, so that this case was diagnosed with HPE. Conclusion: HPE is divided into three categories of alobar, semilobar, and lobar prosencephaly according to the degree of cerebral hemisphere separation. Assesment of patient's brain abnormality and malformation is essential in determining the extent and benefit of surgical intervention. This case was included in the lobar type HPE which shows relatively good prognosis compared with other types and reconstruction of median complete cleft lip & palate and midfacial defects will be performed.

Involvement of a Novel Organic Cation Transporter in Paeonol Transport Across the Blood-Brain Barrier

  • Gyawali, Asmita;Krol, Sokhoeurn;Kang, Young-Sook
    • Biomolecules & Therapeutics
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    • v.27 no.3
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    • pp.290-301
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    • 2019
  • Paeonol has neuroprotective function, which could be useful for improving central nervous system disorder. The purpose of this study was to characterize the functional mechanism involved in brain transport of paeonol through blood-brain barrier (BBB). Brain transport of paeonol was characterized by internal carotid artery perfusion (ICAP), carotid artery single injection technique (brain uptake index, BUI) and intravenous (IV) injection technique in vivo. The transport mechanism of paeonol was examined using conditionally immortalized rat brain capillary endothelial cell line (TR-BBB) as an in vitro model of BBB. Brain volume of distribution (VD) of [$^3H$]paeonol in rat brain was about 6-fold higher than that of [$^{14}C$]sucrose, the vascular space marker of BBB. The uptake of [$^3H$]paeonol was concentration-dependent. Brain volume of distribution of paeonol and BUI as in vivo and inhibition of analog as in vitro studies presented significant reduction effect in the presence of unlabeled lipophilic compounds such as paeonol, imperatorin, diphenhydramine, pyrilamine, tramadol and ALC during the uptake of [$^3H$]paeonol. In addition, the uptake significantly decreased and increased at the acidic and alkaline pH in both extracellular and intracellular study, respectively. In the presence of metabolic inhibitor, the uptake reduced significantly but not affected by sodium free or membrane potential disruption. Similarly, paeonol uptake was not affected on OCTN2 or rPMAT siRNA transfection BBB cells. Interestingly. Paeonol is actively transported from the blood to brain across the BBB by a carrier mediated transporter system.

Intracutaneous Delivery of Gelatins Reduces Fat Accumulation in Subcutaneous Adipose Tissue

  • An, Sung-Min;Kim, Min Jae;Seong, Keum-Yong;Jeong, Jea Sic;Kang, Hyeon-Gu;Kim, So Young;Kim, Da Som;Kang, Da Hee;Yang, Seung Yun;An, Beum-Soo
    • Toxicological Research
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    • v.35 no.4
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    • pp.395-402
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    • 2019
  • Subcutaneous adipose tissue (SAT) accumulation is a constitutional disorder resulting from metabolic syndrome. Although surgical and non-surgical methods for reducing SAT exist, patients remain non-compliant because of potential adverse effects and cost. In this study, we developed a new minimally-invasive approach to achieve SAT reduction, using a microneedle (MN) patch prepared from gelatin, which is capable of regulating fat metabolism. Four gelatin types were used: three derived from fish (SA-FG, GT-FG 220, and GT-FG 250), and one from swine (SM-PG 280). We applied gelatin-based MN patches five times over 4 weeks to rats with high-fat diet (HD)-induced obesity, and determined the resulting amount of SAT. We also investigated the histological features and determined the expression levels of fat metabolism-associated genes in SAT using hematoxylin and eosin staining and western blotting, respectively. SAT decreased following treatment with all four gelatin MN patches. Smaller adipocytes were observed in the regions treated with SA-FG, GT-FG 250, and SM-PG 280 MNs, demonstrating a decline in fat accumulation. The expression levels of fat metabolism-associated genes in the MN-treated SAT revealed that GT-FG 220 regulates fatty acid synthase (FASN) protein levels. These findings suggest that gelatin MN patches aid in decreasing the quantity of unwanted SAT by altering lipid metabolism and fat deposition.

Role of n-3 long-chain polyunsaturated fatty acids in human nutrition and health: review of recent studies and recommendations

  • Dael, Peter Van
    • Nutrition Research and Practice
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    • v.15 no.2
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    • pp.137-159
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    • 2021
  • Long-chain (LC) n-3 polyunsaturated fatty acids (n-3 PUFAs), in particular docosahexaenoic acid (DHA) and eicosapentaenoic acid (EPA), are nutrients involved in many metabolic and physiological processes, and are referred to as n-3 LCPUFA. They have been extensively studied for their effects in human nutrition and health. This paper provides an overview on metabolism, sources, dietary intake, and status of n-3 LCPUFA. A summary of the dietary recommendations for n-3 LCPUFAs for different age groups as well as specific physiological conditions is provided. Evidence for n-3 LCPUFA in cardiovascular diseases, including new studies, is reviewed. Expert recommendations generally support a beneficial effect of n-3 LCPUFA on cardiovascular health and recommend a daily intake of 500 mg as DHA and EPA, or 1-2 servings of fish per week. The role of n-3 LCPUFA on brain health, in particular neurodegenerative disorders and depression, is reviewed. The evidence for beneficial effects of n-3 LCPUFA on neurodegenerative disorders is non-conclusive despite mechanistic support and observational data. Hence, no definite n-3 LCPUFA expert recommendations are made. Data for the beneficial effect of n-3 LCPUFA on depression are generally compelling. Expert recommendations have been established: 200-300 mg/day for depression; up to 1-2 g/day for major depressive disorder. Recent studies support a beneficial role of n-3 LCPUFAs in reducing the risk for premature birth, with a daily intake of 600-800 mg of DHA during pregnancy. Finally, international experts recently reviewed the scientific evidence on DHA and arachidonic acid (ARA) in infant nutrition and concluded that the totality of data support that infant and follow-on formulas should provide both DHA and ARA at levels similar to those in breast milk. In conclusion, the available scientific data support that dietary recommendations for n-3 LCPUFA should be established for the general population and for subjects with specific physiological conditions.

Oral administration of Grifola frondosa affect lipid metabolism and insulin signaling pathway on BKS. Cg-+Leprdb/+Leprdb/OlaHsd mouse

  • Yun, Seong-Bo;Kim, Dae-Young
    • Journal of Animal Reproduction and Biotechnology
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    • v.36 no.4
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    • pp.203-211
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    • 2021
  • Diabetic mellitus (DM) is a carbohydrate metabolic disorder that involves high blood sugar because insulin works abnormally. Type 2 diabetes accounts for most of them. However, diabetes treatments such as GLP-1 and DPP-4 inhibitors commonly caused side effects including gastrointestinal disorders. Grifola frondosa (G. frondosa) revealed various pharmacological effects in recent studies. It has a variety of anti-cancer polysaccharides through host-mediated mechanisms. D-fraction in G. frondosa has apoptotic effects, promoting myeloid cell proliferation and differentiation into granulocytes-macrophages. It has also been shown to reduce the survival rate of breast cancer cells. Though, no further study has been conducted on the specific effects of G. frondosa in the db/db mouse. Therefore, we would like to research the blood glucose improving effect of G. frondosa, a natural material, in type 2 diabetes model mouse, in this study. G. frondosa was administered to the disease model mouse (BKS.Cg-+Leprdb/+Leprdb/OlaHsd) for 8 weeks to monitor weight and blood glucose changes every week. And we evaluated anti-diabetes effects by checking biomarker changes shown through blood. Experiment did not show statistically significant weight differences, but control groups showed significantly higher weight gain than G. frondosa administered groups. We collected blood from the tail veins of the db/db mouse each week. As a result, the lowest blood sugar level was shown in the 500 mg/kg group of G. frondosa. Glucose in the blood was examined with HBA1c, and 7.8% was shown in the 500 mg/kg administration group, lower than in other groups. These results suggest the potential improvements of diabetes in G. frondosa.

Remedy effects of dandelion and milk thistle on fatty liver hemorrhagic syndrome in laying hens

  • Young-Joon Cho;Jeong-Ran Min;Jeong-Hee Han;Sang-Hee Jeong
    • Korean Journal of Veterinary Service
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    • v.46 no.1
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    • pp.1-13
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    • 2023
  • Fatty liver hemorrhagic syndrome (FLHS) is a metabolic disorder found in caged layer hens and causes reduced egg production and sudden death. Dandelion (Taraxacum coreanum, TC) and milk thistle (Cirsium japonicum var. ussuriense, CJ) are well known wild herbs inhabiting Korean peninsula and presenting antioxidative effects. This study investigated alleviate effects of these herbal mixture (6:4, w/w) composed of dried powder of TC and CJ against fatty liver in laying hens. The herbs mixture 5.0, 10.0, 20.0 or 40.0 g/kg feed was provided via feed admixture for 3 weeks to laying hens having FLHS. FLHS was induced by intramuscular injection of β-estradiol (2 mg/kg bw) 2 times per week for 3 weeks and supply with high caloric feed. Egg production rate was reduced from 76.2% at pre-treatment to 49.4% at 1 week and further decreased according to β-estradiol treatment. Increment of serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), total cholesterol (T-Chol) and total bilirubin (T-Bil) and decrement of serum superoxide dismutase (SOD) and glutathione peroxidase (GPX) with fatty liver were found by the treatment of β-estradiol. Supplementation of TC and CJ mixture via feed admixture recovered the reduction of egg production and attenuated serological changes and gross and pathological lesions of fatty liver with the best amelioration effects at 5 and 10 g TC and CJ mixture per kg feed. In conclusion, TC and CJ mixture attenuates FLHS by means of antioxidative effects. Further mechanistic study is required to explain TC and CJ's amelioration effects against FLHS in laying hens.