• Title/Summary/Keyword: 혈액 질환

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Mutation Analysis of Korean Patients with Glycogen Storage Disease Type Ia (한국인 당원병 제 Ia형 환자의 돌연변이 분석)

  • Kim, Jong-Won;Park, Ji-Yeon;Seo, Jeong-Kee
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.4 no.2
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    • pp.213-217
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    • 2001
  • Purpose: Glycogen storage disease type Ia (GSD Ia) is an autosomal recessive disorder of glycogen metabolism caused by glucose-6-phosphatase (G6Pase) deficiency. The clinical manifestations of G6Pase deficiency include growth retardation, hepatomegaly, hypoglycemia, lactic acidemia, hyperlipidemia and hyperuricemia. Many mutations of this gene have been found worldwide in various ethnic groups, establishing the molecular basis of GSD Ia. To elucidate a spectrum of the G6Pase gene mutations in Korean, we analyzed mutations in Korean patients with GSD Ia. Methods: Both alleles of 9 unrelated GSD 1a patients were studied by PCR and direct DNA sequencing methods. In all patients, GSD 1a was diagnosed by the enzyme assay for the liver biopsy specimen. Results: In Korean, the most prevalent mutation was g727t substitution in exon 5, which has been reported to cause abnormal mRNA splicing: Sixteen out of 18 alleles were found to have this mutation. In addition, we identified one novel mutation, a c611g, converting a proline to an alanine at codon 178. Conclusion: Our findings suggest that a screening for the g727t mutation by noninvasive molecular method can detect most cases of GSD Ia in Korean patients.

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Splenic Irradiation in Chronic Myeologenous Leukemia (만성골수성백혈병 15예의 비장조사)

  • Oh Yoon Kyeong;Kwon Hyung Chul;Yoon Sei Chul;Bahk Yong Whee;Kim Choon Choo;Kim Dong Jip
    • Radiation Oncology Journal
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    • v.3 no.2
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    • pp.137-144
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    • 1985
  • Radiation therapy was the treatment of choice for CML in the past, in the form of SI or radioactive phosphorus. Its use has been replaced to a large extent by various chemotherapeutic agents. Recently SI in CML has been used, both to relieve painful splenomegaly and to take advantage of an indirect effect of SI on unirradiated bone marrow. We have treated 15 CML cases who had a huge spleen during chemotherapy or even after chemotherapy by 6 MV linear accelerator during the past two years at the Division of Radiation Therapy, Kang Nam St. Mary's Hospital, Catholic College. Response to SI has been rated according to the scoring system of Roger W. Byhardt, et al. which evaluated the splenic and hematologic response as well as the response of disease-related systems. According to this scoring system, most patients demonstrated a significant relief of splenomegaly along with improvement of hemogram. And we observed the change of Karnofsky Performance Status after SI, and survival after a confirmative diagnosis and SI.

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Secondary Acute Myeloid Leukemia after Chemotherapy in an Osteosarcoma Patient - A Case Report - (골육종 환자에서 항암화학요법후 발생한 이차성 급성 골수성 백혈병 - 증례 보고 -)

  • Kim, Jae-Do;Kim, Seong-Dae;Son, Jung-Hwan
    • The Journal of the Korean bone and joint tumor society
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    • v.6 no.2
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    • pp.98-105
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    • 2000
  • In the case of osteosarcoma, secondary acute myeloid leukemia which occurs as the consequences of the complication of chemotherapy, is rare. We are reporting the case that we have recently experienced in the laboratory. A case of secondary acute myeloid leukemia have been occurred to among 77 patients who have been diagnosed as osteosarcoma and received chemotherapy from 1995 to 1999. This case was compared with the cases of other reports for the analysis of its cause and results. A 17-year-old man was diagnosed as a osteosarcoma in the distal part of femur, and recieved chemotherapy. Within 28months, the hematological analysis of the case indicated the severe increase in the number of the white cell (over 200,000) and the profound decrease in the number of platelets. A test of bone marrow needle aspiration and peripheral blood smear showed a tremendous increase in the number of the monocytoid immature cell, which mostly are blasts and promonocytes. Due to this clinical results, the case was diagnosed as the secondary acute myeloid leukemia after the chemotherapy. The frequency of occurrence of secondary acute myeloid leukemia after chemotherapy is quite phenomenal. But the disease could be fatal leading to a high rate of morbidity and mortality without early diagnosis and treatment. Hence, an enough recognition of the possibility of its development, the periodical observation and inspection after chemotherapy and an immediate treatment in the case of occurrence are essential.

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The Association between Total Body Fat and Serum Lipids Concentrations in College Students (대학생에서 혈액 지질 수준과 체지방과의 관련성)

  • Lee, Jun-Ho
    • Korean Journal of Clinical Laboratory Science
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    • v.49 no.3
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    • pp.233-238
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    • 2017
  • This study was conducted to investigate the relationship between body fat content and serum lipids in college students. A total of 61 (15 males and 46 females) students of W university in Iksan, between June 2015 and July 2015, were enrolled for analysis. The relationship of serum lipid with BMI, and body fat percent measured by impedance fat meter (InBody 520) was analyzed. The study population was divided into three groups according to the InBody 520's guideline. Group I accounted for 26.7% of men and 10.9% of women. Weight and BMI were significantly different among the three groups in men and women (p<0.05); whereas height was not. In men, serum total cholesterol, triglyceride, low density lipoprotein cholesterol, and hemoglobin were significantly different between the three groups (p<0.05). Conversely, in women, serum total cholesterol, low density lipoprotein cholesterol, and alanine transferase were significantly different between the three groups (p<0.05). However, there were no differences in other variables between the three groups. BMI was significantly different among three groups in both men and women (p<0.05). In men and women, body fat percent was correlated with BMI, weight, total cholesterol, and low density lipoprotein cholesterol. Multiple regression analysis on total cholesterol indicates that body fat percent, BMI, and hemoglobin were not significantly independent variables in men and women. This study showed that body fat percent was significantly associated with other obesity markers and serum lipids.

The Effect of Polycan-Calcium Gluconate Complex on Inflammatory Mediators from Periodontitis Patients (Polycan-Calcium Gluconate 복합제에 의한 염증인자의 감소 효과)

  • Kim, Ji-Hye;Kim, Ki-Rim;Jin, Hye-Jung;Im, Sang-Uk;Song, Keun-Bae;Choi, Youn-Hee
    • Journal of dental hygiene science
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    • v.14 no.2
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    • pp.223-229
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    • 2014
  • The aim of this study was to examine the effect of polycan-calcium gluconate complex on the levels of immune and inflammatory mediators in gingival crevicular fluid and serum from patients with periodontitis. A total of 39 patients with periodontitis took placebo (placebo group) or polycan-calcium gluconate complex (treatment group) twice a day for 4 weeks. At baseline and 4 weeks, gingival crevicular fluid (GCF) and blood was collected from each subjects. The secretion level of interleukin $(IL)-1{\beta}$ in GCF was measured by enzyme-linked immunosorbent assay (ELISA). Western blot analysis was conducted to determine the expression of matrix metalloproteinase (MMP)-8 and tumor necrosis factor $(TNF)-{\alpha}$ in GCF. Serum samples were analysed for MMP-8 by ELISA and C-reactive protein (CRP) by turbidimetric immuno assay. MMP-8 and $TNF-{\alpha}$ were significantly decreased in the treatment group at 4 weeks. The level of $IL-1{\beta}$ in the treatment group was significantly lower than those of the placebo group. No differences were observed in CRP levels. Taken together, these data indicate that taking of polycan-calcium gluconate complex led to reduction of inflammatory biomarkers in serum and GCF of periodontitis patients.

Corticosteroids Add-on Therapy in the Acute Phase of Kawasaki Disease (고위험군 가와사끼병에서 스테로이드 추가 요법의 효과)

  • Kang, Seon-Mi;Moon, Eun-Kyung;In, Su-Mi;Kil, Hong-Ryang
    • Clinical and Experimental Pediatrics
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    • v.45 no.12
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    • pp.1571-1576
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    • 2002
  • Purpose : Recently, clinical trials of steroid add-on therapy were reported with variable results in Kawasaki disease. We analyzed the clinical outcomes of patients at high risk of with Kawasaki disease(${\geq}4$ points of Harada score) treated by three commonly used different treatment regimens, with or without corticosteroids. Methods : Medical records of 96 children with Kawasaki disease treated with one of the threee regimens were reviewed retrospectively. Regimen 1 was aspirin(100 mg/kg/day) plus intravenous gamma globulin 2 g/kg single dose; regimen 2, aspirin(100 mg/kg/day) plus intravenous gamma globulin 1 g/kg single dose; regimen 3, regimen 2 plus prednisolone(2 mg/kg/day), followed by tapering two weeks and pulse therapy of methyl prednisolone performed in cases of retreatment. Also low dose aspirin was given in all three regimens for eight weeks after the acute phase. The cardiovascular and laboratory evaluations were performed on acute phase, immediate after acute phase, and subacute phase, eight weeks after treatment. Results : The frequency of coronary artery lesions and laboratory findings in the three different regimens were similar. The more rapid control of fever after treatment was noted in regimen 3. Furthermore the frequency of retreatment was decreased in regimen 3 compared to the other two regimens. Conclusion : Steroid add-on therapy showed some beneficial outcome compared to conventional treatment regimens. The role of steroid in the treatment of Kawasaki disease should be reassessed in systemic manner.

Study on Production of Antibody in Milk Immunized Cows with Some Helicobacter pylori Antigen (Helicobacter pylori 항원을 이용한 면역우유 생산에 관한 연구)

  • Park, Chang-Ho;Kim, Soo-Jung;Yea, Eon-Ju;Bae, Man-Jong
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.34 no.4
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    • pp.484-488
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    • 2005
  • This study has been to produce of anti-H. pylori antibody in milk produced from cows immunized with antigen of Helicobacter pylori and to search the relationship between vaccine dosage and antibody formation and impact of vaccine dosage on cows. The content of anti-H. pylori antibody in serum and whey increased in accordance with vaccine dosage volume. It has been confirmed that the formation of high-quantified antibody was produced in all groups with vaccine dosages of 10 mL, 20 mL and 30 mL. It has been turned out that the antibody was formed most in 20 mL dosage. It was inclined to $12\%$ reduce caused by vaccine injection, but recovered after about maximum 1 week. In measurement of body temperature of cows after vaccine injected, it was inclined to rise with the normal scope in comparison with the controlled conditions.

Thrombin Inhibition Activity of Fructus Extract of Crataggus pinnatifida Bunge (산사자 추출물의 트롬빈 저해활성)

  • Ryu, Hee-Young;Kim, Yung-Kwan;Kwun, In-Sook;Kwon, Chong-Suk;Jin, Ing-Nyol;Sohn, Ho-Yong
    • Journal of Life Science
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    • v.17 no.4 s.84
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    • pp.535-539
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    • 2007
  • The fructus of Crataegus pinnatifida Bunge has been used as medicinal and food source in worldwide. In this study, a strong direct thrombin inhibition and antithrombosis activity were identified from the methanol extract of C. pinnatifida Bunge fructus. The solvent fractionation of fructus extract using hexane, ethylacetate, butanol revealed that the butanol fraction has a prominent antithrombin activity. Thrombin time(blood-clot formation time) and activated partial thromboplastin time(aPTT) extended to 835% and 315% by addition of the butanol fraction at concentration of 1.25 mg/mL, whereas thrombin time extended to 287% by addition of aspirin at concentration of 1,25 mg/mL. The butanol fraction showed anthrone-positive and weak ninhydrine-postive reaction. The thrombin inhibitory activity was not related to previously reported flavonoids or polyphenols. The activity was maintained against acid treatment(0.5 N HCl for 120 min), but rapidly lost by heat-treatment($100^{\circ}C$ for 30 min). Our results suggested that fructus of C. pinnatifida Bunge with non-heat treatment process could be developed as a natural source of antithrombosis.

Effect of Improve Obesity with Sprout Raw Grains and Vegetables (발아 생식을 이용한 비만개선 효과에 관한 연구)

  • 서정숙;방병호;여인법
    • The Korean Journal of Food And Nutrition
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    • v.14 no.2
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    • pp.150-160
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    • 2001
  • This study was carried out to investigate the effect of weight loss and the change of serum lipids composition for normal and obese women after sprout raw grains and vegetables diet intake. Diet were given to the subjects which were composed of 11 normal women and 7 obese women for 4 weeks. After diet intake, 4.6% in normal group and 3.5% in obese group were shown weight loss. And the both groups of normal and obese women showed a significant reduction in the thickness of subcutaneous fat and the body girth. Particularly, the waist measure was shown to be remarkably reduced in the normal group (p< 0.0001) and to be reduced in the obese group (p<0.017) significantly. After diet intake. the levels of total cholesterol and triglycerides were reduced in both groups significantly. And in the obese women, HDL-cholesterol level increased 5.7%. As those results, it could be seen that weight and the thickness of subcutaneous fat were reduced and the component of blood serum were improved after diet intake for 4 weeks. Specially, abdomen, waist measures and hip girth were reduced remarkably, so it is considered that the diet is more effective in the diet therapy of obese women with upper obese status.

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Effect of Soy Protein Hydrolyzate on Lipid Metabolism and Antioxidant Activity in the Rat (대두단백가수분해물이 흰쥐의 지질대사와 항산화에 미치는 영향)

  • Han, Yoon-Hee;Park, Sang-Kyu;Kim, Hye-Young
    • Journal of Nutrition and Health
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    • v.41 no.2
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    • pp.119-126
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    • 2008
  • This study was conducted to investigate the effect of soy protein hydrolyzate on lipid metabolism and antioxidant activity in the rat. Thirty-eight male rats of Sprague-Dawley strain were divided into five groups: casein, isolated soy protein (ISP), seoritae protein hydrolyzate (SH), soluble soy protein hydrolyzate (SS), and insoluble soy protein hydrolyzate (IS). The control diet (casein group) contained 20% casein protein and experimental diet contained 10% casein and 10% isolated soy-protein or soy-protein hydrolyzate. Fecal lipid content was increased and lipid apparent absorption rate was decreased significantly by the ISP group at the first week of experimental period. Blood triglyceride, total cholesterol, LDL-cholesterol and atherogenic index (AI) were decreased by soy protein hydrolyzate groups than casein group. Liver total lipid, triglyceride and cholesterol were not different among groups, but showed decreasing tendencies in soyprotein hydrolyzate groups. The lipid lowering effect was prominent in the IS group among soy protein hydrolyzate groups. Total antioxidant activity showed increasing tendency in the seoritae hydrolyzate group. Liver superoxide dismutase (SOD), glutathione peroxidase (GPx) and catalase activities also showed higher tendencies in the seoritae hydrolyzate group than other groups. In conclusion, insoluble soyprotein hydrolyzate was more effective in lowering body lipids and seoritae hydrolyzate had higher antioxidant capacity among soy protein hydrolyzates.