• Title/Summary/Keyword: Deficiency syndrome

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8 Years Report of Urine Organic Acid Analysis - Comparison before and after Introduction of Neonatal Screening Test using Tandem Mass Spectrometry - (소변 유기산 분석 8년의 정리 -탠덤매스(Tandem mass spectrometry)를 이용한 신생아 선별검사 도입 전후의 비교-)

  • Ahn, Seok Min;Shin, Woo Chul;Jeong, Han Bin;Seo, Young Jun;Jeong, Hwal Rim;Yoon, Jong Hyung;Bae, Eun Ju;Lee, Hong Jin
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.18 no.1
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    • pp.1-12
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    • 2018
  • Purpose: Disorders of organic acid metabolism have various clinical manifestations and it may be life-threatening. The prognoses of affected children are dependent on early diagnosis and treatment. We report this study to find out detection rate of referred samples, clinical manifestations and age distribution after introduction of neonatal screening test using tandem mass spectrometry in Hallym University Chuncheon Sacred Heart Hospital during 8 years and 9 months. Methods: The 2,794 patients referred from Jan. 2007 to Sep. 2015 were divided into four groups according to age. We conducted organic acid analysis of urine samples of patients and analyzed clinical manifestations and distributions of age at the diagnosis. For patients with ambiguous results, reanalysis of urine organic acid after diet restriction, protein loading and restriction, has been done. Results: A total of 626 patients with 20 disorders were diagnosed. Mitochondrial disorders (482 patients) were the most common diagnosis, followed by ketolytic defects (67), 3-hydroxyisobutyric aciduria (32), EPEMA syndrome (8), 3-methylcrotonyl glycinuria (7), glutaric aciduria type II (6) and type I (4), methylmalonic aciduria (3), isovaleric aciduria (3) and medium chain acyl-CoA dehydrogenase deficiency (3). Conclusion: As neonatal screening test using tandem mass spectrometry is increasingly common and medical environment is changed, detection rate of disorders of organic acid metabolism in this study has decreased compared to previous report. Because the deterioration can be prevented by early diagnosis and treatment, many pediatricians have to pay special attention to possibility of the disorders and make an effort for early diagnosis in clinical setting.

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A Study on etiological factors, symptoms of a disease, and treatments of obesity based on Dong-Yi Bo Gam (동의보감(東醫寶鑑)을 위주(爲主)로한 비만의 원인(原因), 증상(症狀), 치료법(治療法) 연구)

  • Kim, Seok;Jin, Seng-Hee;Kim, Tae-Hee
    • The Journal of Korean Medicine
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    • v.19 no.2
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    • pp.125-136
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    • 1998
  • This study is on the etiological factors of the obesity, symptoms of a disease caused by the etiological factors and the method of medical treatment resulted from symptoms of a disease, it is based on Dong-Yi Bo Gam. The conclusion of this study is as follows. 1. The final metabolic of fat is depend on the defensive energy. The shape of fatty layer is changed by the point where the defensive energy is replenish or not. So 'Goin(暠人)' 'Bein(肥人)' and 'Yuckin(肉人)' is made a discriminated and there is defferent between 'Besudaeso(肥瘦大小)' and 'Herlqidaso(血氣多少)'. 2. Etiological factors of obesity is ; essential substance from cereals overcome the primodial energy, spleen and stomach are strong at once, spleen and stomach are weak at once, spleen is disturbed by evil energy, retention of phlegm and fluid, muscle is weak, blood is excess but energy (qi)is weak, hua-sid(華食) eating the sweat food very often, a rich fatty diet. 3. To investigate each syndrome as etiological factons, essential substance from cereals overcome primodial energy is divided into two case: one is the food increase itself because of the weakness of stomach energy (qi), the other is not to digest the food because liver overact spleen by seven emotion. The obesity because of strengthen of the spleen and stomach at once do not feel hungry very well and spleen and stomach do not damaged easily by overacting. The obesity because of weakness of spleen and stomach at once, and disturbace in spleen cause by evil energy, gain the weight even if they eat food a little. Also it is hard to move four limbs. Weakness of muscle is weakness of spleen and stomach. Weakness of muscle is caused by weakness of spleen and stomach. That is the case of faining the weight because of eating food with an empty stomach. The obsity caused by retention of fluid has intestine rumbling and gain the weight suddenly. 4. The method of medical treatment fallowing to the etiological factors: The food is inclined to increase itself caused by weakness of stomach energy, In case of the obseity caused by this phenomenon. We can use Decoction for Reinforcing Middle wanner and Replenishing Qi(補中益氣湯), five tastes il-going-san(左味異功散) and so on. The method of medical treatment on obsity caused by strengthen of spleen and stomach is not referred yet. But we must investigate about using peptic powder(平胃散), ji-chul-huan(枳朮丸) for strengthen of stomach qi (胃氣). If the obesity appear caused by weakness of spleen and stomach and disturbance in spleen as to evil energy, and in the same time stomach qi is weak, we can use li-gong san(異功散). If not only obesity but also indigestion, we can use Decoction for naurishing the stomach and promotion eating(養胃進食湯). If not only obesity but also weakness of spleen and stomach, we can use Decoction for Reinforcing Middle warmer and replenishing Qi(補中益氣湯). The energy being weak, we can use Decoction for six noble drugs(六君子湯). If the obesity appear because of retention of fluid, we can use sin-chul-huan(神朮丸). On the obesit, caused by excess of blood and weakness of qi(血管氣虛), there is not method of mectical treatment yet, but the method of medical treatment that we count of first important maybe to invigorate the spleen and the lung qi and to disperse and recitify the depressed liver energy(疏肝利氣). The method of medical treament about the obesity caused by a rich fatty diet is not mentioned yet, but it is important to improve the lifestyle of food and we think that invigorate the spleen, and the lung-qi(補疏肝氣) and disperse and recitify the depressed liver-energy (??肝利機) amy count of first important. 5.There is useful medicine for weight down for example Herb tea(茶), red bean, wax gourd, etc. 6.The etiological factors of the obesity that revive frequently is weakness of qi(氣虛), from 形成氣衰, deficiency of yang, hasty of qi, damp and so on. The symptoms and sign of a disease that revive frequently to the obesity is appoplexia(中風), leucorrhea, reddish and whitish turbid, feeling of fullness in the upper abdomen, diabetes and so on.

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The Prognostic Indicies of Pneumocystis Carinii Pneumonia in Immunocompromised Patients other than Acquired Immune Deficiency Syndrome (비 AIDS 면역 결핍 환자들에서 발생한 주폐포자충 폐렴의 예후인자)

  • Park, Wann;Kim, Yoo-Kyum;Lee, Jin-Seong;Ahn, Jong-Jun;Hong, Sang-Bum;Shim, Tae-Sun;Lim, Chae-Man;Lee, Sang-Do;Kim, Woo-Sung;Kim, Dong-Soon;Kim, Won-Dong;Koh, Youn-Suck
    • Tuberculosis and Respiratory Diseases
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    • v.45 no.4
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    • pp.805-812
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    • 1998
  • Background: Among the variety of opportunistic infections, pneumonia comprises the major morbidity in immunocompromised patients. Pneumocystis carnii pneumonia (PCP) and cytomegalovirus (CMV) pneumonia are common infectious illness of immunocompromised hosts. Although there are many reports regarding to the co-infection of PCP and CMV diagnosed by bronchoalveolar lavage (BAL) fluid examination, the effects of CMV co-infection on the outcome of PCP is still controversial. The purpose of this investigation is to evaluate the effects of CMV detected by BAL fluid examination on the clinical course of PCP in the immunocompromised patients other than human immunodeficiency virus infection. Method: Ten patients with PCP were enrolled and retrospective analysis of their medical records were done. HIV infected persons were excluded. The PCP was diagnosed by BAL fluid examination with Calcofluor-White staining. CMV was detected in BAL fluid by Shell-vial culture system. Chest radiographic findings were reviewed. We used Fisher's exact test and Mann-Whitney U test for statistical analysis of data. Results: The underlying disorders of patients were idiopathic pulmonary fibrosis (n=1), renal transplantation (n=4), necrotizing vasculitis (n=l), systemic lupus erythematosus (n=1), brain tumor (n=1), chronic myelogenous leukemia (n=1), unidentified (n=1). There were no difference in clinical course, APACHE III score, arterial blood gas analysis, white blood cell count, lymphocyte count, serum albumin concentration, chest radiographic findings and mortality between patients with PCP alone (n=4) and those with CMV co-infection (n=6). Univariate analysis regarding to the factors that associated with mortality of PCP were revealed that the application of mechanical ventilation (p=0.028), the level of APACHE III score (p=0.018) and serum albumin concentration (p=0.048) were related to the mortality of patients with PCP. Conclusion: The clinical course of PCP patients co-infected by CMV were not different from PCP only patients. Instead, accompanied respiratory failure, high APACHE III score and poor nutritional status were associated with poor outcome of PCP.

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Immunomodulatory effects of β-1,3/1,6-glucan and lactic acid bacteria in LP-BM5 murine leukemia viruses-induced murine acquired immune deficiency syndrome (면역결핍 모델에서 β-1,3/1,6-glucan과 유산균을 이용한 in vivo 면역 활성 조절 효과)

  • Kim, Min-Soo;Kim, JoongSu;Ryu, Min Jung;Kim, Ki hong;Hwang, Kwontack
    • Food Science and Preservation
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    • v.24 no.8
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    • pp.1158-1167
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    • 2017
  • In this study, ${\beta}$-1,3/1,6-glucan, lactic acid bacteria, and ${\beta}$-1,3/1,6-glucan+lactic acid bacteria were tested for 10 weeks using an immunodeficient animal model infected with LP-BM5 murine AIDS virus On the immune activity. Cytokines production, plasma immunoglobulin concentration, T cell and B cell proliferation were measured. As a result, the T cell proliferative capacity which was weakened by immunization with LP-BM5 murine AIDS virus increased significantly T cell proliferative capacity compared with the red ginseng control group. B cell proliferative capacity was significantly higher than the infected control group. Increased B cell proliferation was reduced. In the cytokine production, IL-2, IL-12 and IL-15 in the Th1-type cytokine increased the secretion of IL-2, IL-12 and IL-15 compared to the infected control. The proliferative capacity of the treated group was higher than that of the mixed treatment group. TNF-${\alpha}$ was significantly decreased compared with the infected control group. The IL-4, IL-6 and IL-10 levels were significantly inhibited in the infected control group and the Th1/Th2 type cytokine expression was regulated by immunohistochemistry. IgE, IgA, and IgG levels were significantly lower in the immunoglobulin secretion assay than in the control. As a result, the immunomodulatory effect of ${\beta}$-1,3/1,6-glucan+lactic acid bacteria was confirmed by mixing with LP-BM5 murine AIDS virus-infected immunodeficient animal model.

The Knowledge and Attitude of Unmarried Young Men on AIDS (젊은 미혼 남성에서의 에이즈에 대한 지식과 태도)

  • Yeom, Chang-Hwan;Lee, Hye-Ree;Choi, Youn-Seon
    • Journal of Hospice and Palliative Care
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    • v.4 no.1
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    • pp.4-13
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    • 2001
  • Purpose : Since the acquired immune deficiency syndrome (AIDS) was first recognized in the United States in the summer of 1981, the number of these patients has been increasing in the world. But do not find out a cure and a vaccine for ARS (5). And so, the best treatment for AIDS is the prevention. People can find out accurate knowledge about AIDS, and they can prevent themselves from AIDS approximately 100%. In this study, we investigate with AIDS knowledge and attitudes in unmarried young men (<24 age) and suggest accurate preventive education for AIDS and good sexual behaviors. Methods : Un-married young soldiers and college students who were not diagnosed as AIDS until June 30, 2000 were included in the study. The study included a total of 923 men. A self evaluation questionnaire, included questions on 36 items(the part of demographic data - 9; the part of knowledge - 20; the part of attitudes - 8), was drawn up by three physicians. The demographic data, AIDS knowledge and attitudes were analyzed by chi-square analysis, and the total score of AIDS knowledge - comparison according to demographic factors and attitudewere analyzed by one-way ANOVA test. Results : In demographic characteristics, as for the first recognized time of AIDS, most of men knew it when they were in their middle school, as for sources of information on AIDS, most of them knew it through the TV-media, and as for the educational need about AIDS, most of them agreed with it. In AIDS knowledge, mean scores were $14.0{\pm}1.8$ (70.3%). Items of the misconceptions concerning AIDS, reported as less than 50% correct answers, were 6 among 20 items (30%). In AIDS attitudes, as for the item about that if I will be an AIDS patient, I will have an AIDS treatment, it showed that the number of men agreed with 759 (82.2%), and as for the item about that I will help for AIDS patient even though I don't know him, it showed that the number of them agreed with 412 (45.8%). In correlation of AIDS knowledge and demographic factors, the mean scores of knowledge of men with higher than college degree were higher than them of others. The mean scores of knowledge of men with total income of family with more than US$1667 were higher than them of others. The mean scores of knowledge of men with sources of information on AIDS through the TV-media were higher than them of others. And the mean scores of knowledge of men with past medical history of STD(sexually transmitted disease) were higher than them of others. Conclusions : The higher the knowledge he has, the lower the possibility of risk and the more positive the attitude he has. And then we think that the education program for AIDS will be included as a regular part of the curriculum in high school, and young men must be effectively educated by it.

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『황제내경소문(黃帝內經素問)·칠편대론(七篇大論)』 왕빙 주본(注本)을 통(通)한 운기학설(運氣學說) 관(關)한 연구(硏究)

  • Kim, Gi-Uk;Park, Hyeon-Guk
    • The Journal of Dong Guk Oriental Medicine
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    • v.4
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    • pp.109-140
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    • 1995
  • As we considered in the main subjects, investigations on the theory of 'Doctrine on five elements' motion and six kinds of natural factors(運氣學說)' through 'Wang Bing's Commentary(王氷 注本)' of 'The seven great chapters in The Yellow Emperor's Internal Classic Su Wen' ("黃帝內經素問 七篇大論") are as follows. (1) In The seven great chapters("七篇大論")' Wang Bing supplement theory and in the academic aspects as a interpreter, judging from 'forget(亡)' character. expressed in the 'The missing chapters("素問遺篇")', 'Bonbyung-ron("本病論")' and 'Jabeob-ron(刺法論)', 'The seven great chapters("七篇大論")' must be supplementary work by Wang Bing. Besides, he quoted such forty books as medical books, taoist books, confucianist books, miscellaneous books, etc in the commentary and the contents quoted in the 'Su Wen(素問)' and 'Ling Shu("靈樞")' scripture nearly occupy in the book. As a method of interpreting scripiure as scripture, he edited the order of 'Internal Classic("內經")' ascended from the ancient time and when he compensated for commentary, with exhaustive scholarly mind and by observing the natural phenomena practically and writing the pathology and the methods of treatment. We knew that the book is combined with the study of 'Doctrine on five elements motion and six kinds of natural factors(運氣學說)' (2) When we compare, analyze the similar phrase of 'The seven great chapters in The Yellow Emperor's Internal Classic Su Wen'("黃帝內經素問ㆍ七篇大論") through 'Wang Bing's Commentary(王氷 注本)', he tells abouts organized 'five elements(五行)' and 'heaven's regularly movement(天道運行)' rather than 'Emyangengsangdae-ron("陰陽應象大論")' in 'The seven great chapters("七篇大論")'. Also the 'Ohanunhangdae-ron("五運行大論")' because the repeated sentences with 'Emyangengsangdae-ron("陰陽應象大論")' is long they are omitted. And in the 'Youkmijidae-ron("六微旨大論")', 'Cheonjin ideology(天眞四象)' based on the 'Sanggocheonjin- ron("上古天眞論")', 'Sagijosindae-ron("四氣調神大論")' is written and in the 'Gigoupyondae-ron("氣交變大論")', the syndrome and symptom are explained in detail rather than 'Janggibeobsi-ron("藏氣法時論")', 'Okgijinjang-ron ("玉機眞藏論")' and in the 'Osangieongdae-ron("五常政大論")', the concept of 'five element(五行)' of the 'Gemgwejineon-ron("金櫃眞言論")' is expanded to 'the five elements' motion concept(五運槪念)' and in the 'Youkwonjeonggidae-ron("六元正紀大論")', explanations of 'The five elements' motion and six kinds of natural factors(運氣)' function are mentioned mainly and instead systematic pathology is not revealed rather than 'Emyangengsangdae-ron("陰陽應象大論")'. And in the 'Jijinyodae-ron("至眞要大論")', explanations of the change of atmosphere which correspond to treatment principle by 'The three Yin and Yang(三陰三陽)' as a progressed concepts are revealed. Therefore there are much similarity between the phrase of 'Emyangengsangdae-ron("陰陽應象大論")' and 'chapters of addition(補缺之篇)'. Generally, the doctrine which 'The seven great chapters("七篇大論")' are added by Wang Bing(王氷) is supported because there are more profound concepts rather than the other chapter in 'The seven great chapters("七篇大論")'. (3) When we study Wang Bing's(王氷) 'Pattern on five elements motion and six kinds of natural factors(運氣格局)' in 'The seven great chapter("七篇大論")', in the 'Cheonwongi-dae-ron("天元紀大論")', With 'Cheonjin ideology(天眞思想)' and the concepts of 'Owang(旺)'${\cdot}$'Sang(相)'${\cdot}$'Sa(死)'${\cdot}$'Su(囚)'${\cdot}$'Hu(休)' and 'Cheonbu(天符)'${\cdot}$'Sehwoi(歲會)' are measured time-spacially to the concept of 'Three Sum(三合)' the concept of 'Taeulcheonbu(太乙天符)' is explained. In the 'Ounhangdae-ron("五運行大論")', 'The calender Signs five Sum(天干五合)' is compared to the concepts of 'couples(夫婦)', 'weak-strong(柔强)' and in the 'Youkmijidae-ron("六微旨大論")', 'the relationship of obedience and disobedience(順逆關係)' which conform to the 'energy status(氣位)' change and 'monarch-minister(君相)' position is mentioned. In the 'Gikyobyeondae-ron("氣交變大論")', the concept of 'Sang-duk(相得)', 'Pyungsang(平常)' is emphasized but concrete measurement is mentioned. In the 'Osangieongdae-ron("五常政大論")', the detailed explanation with twenty three 'systemic of the five elements' motion(五運體系)' form and 'rountine-contrary treatment(正治. 反治)' with 'chill-fever-warm-cold(寒${\cdot}$${\cdot}$${\cdot}$凉)' are mentioned according to the 'analyse and differentiate pathological conditions in accordance with the eight principal syndromes(八綱辨證)'. In the 'Youkwonjeonggidae-ron("六元正紀大論")', Wang Bing of doesn't mention the concepts of 'Jungwun(中運)' that is seen in the original classic. In the new corrective edition, as the concepts of 'Jungwun, Dongcheonbu, Dongsehae and Taeulcheonbu(中運, 同天符, 同歲會, 太乙天符)' is appeared, Wang Bing seems to only use the concepts of 'Daewun, Juwun, and Gaekwun(大運, 主運, 客運)'. In the 'Jijinyodaeron("至眞要大論")', Wang Bing added detailed commentary to pathology and treatment doctrine by explaining the numerous appearances of 'Sebo, sufficiency, deficiency(歲步, 有餘, 不足)' and in the relation of 'victory-defeat(勝復)', he argued clearly that it is not mechanical estimation. (4) When we observe the Wang Bing's originality on the study of 'the theory of Doctrine on five elements' motion and six kinds of natural factors(運氣學說)', he emphasized 'The idea of Jeongindogi and Health preserving(全眞導氣${\cdot}$養生思想)' by adding 'Wang Bing's Commentary(王氷 注本)' of 'The seven great chapters("七篇大論")' and explained clearly 'The theory of Doctrine on five elements' motion and six kinds of natural factors(運氣學說)' and simpled and expanded the meaning of 'man, as a microcosm, is connected with the macrocosm(天人相應)' and with 'Atmosphere theory(大氣論)' also explained the meaning of 'rising and falling mechanism(升降氣機)'. In the sentence of 'By examining the pathology, take care of your health(審察病機 無失氣宜)'. he explained the meaning of pathology of 'heart-kidney-water-fire(心腎水火)' and suggested the doctrine and management of prescription. In the estimation and treatment, by suggesting 'asthenia and sthenia(虛實)' two method's estimation, 'contrary treatment(反治)' and treatment principals of 'falling heart fire tonifyng kidney water(降心火益腎水)', 'two class of chill and fever(寒熱二綱)' were demonstrated. There are 'inside and outside in the illness and so inner and outer in the treatment(病有中外 治有表囊)'. This sentence suggests concertedly. 'two class of superfies and interior(表囊二綱)' conforming to the position of disease. Therefore Wang Bing as an excellent theorist and introduced 'Cheoniin ideology(天眞思想)' as a clinician and realized the medical science. With these accomplishes mainly written in 'The theory of Doctrine on five elements' motion and six kinds of natural factors(運氣學說)' of 'The seven great chapters("七篇大論")', he interpreted the ancient medical scriptures and expanded the meaning of scriptures and conclusively contributed to the development of the study 'Korean Oriental Medicine(韓醫學)'.

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