• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.2
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• pp.55-62
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• 2017

Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by beta-glucosidase deficiency. An 18 month-old male with hepatosplenomegaly, anemia, thrombocytopenia, and growth retardation referred to our hospital. The patient showed neurological symptoms, such as supranuclear gaze palsy and developmental delay. Bone marrow biopsy performed to rule out malignancy and the results revealed no malignant cell; however, abnormal histiocytes suggesting storage disease was noted. Based on hepatosplenomegaly, bicytopenia and unexplained neurologic manifestations, enzyme activity and genetic analysis were conducted emergently with a strong suspicion of GD. Beta-glucosidase activity in leukocyte was decreased. GBA sequencing to confirm the diagnosis revealed compound heterozygous pathogenic variants (i.e., c.754T>A, c.887G>A), both previously reported as the cause of neuronopathic GD. Under the diagnosis of type 3 GD, the patient immediately received enzyme replacement therapy (ERT). After 17 months of ERT, the size of spleen decreased, and hemoglobin and platelet count returned to normal. In addition, the activity of chitotriosidase and angiotensin converting enzyme decreased. However, myoclonic movement and generalized seizure occurred at the age of 19 months and antiepileptic drug was started. Other neurological deterioration including supranuclear gaze palsy and developmental delay also persisted. A new therapy to overcome neurologic problems should be developed for patients with type 3 GD.

• Parasites, Hosts and Diseases
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• v.38 no.2
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• pp.51-57
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• 2000

Follow-up studies have been conducted every three years on the endemicity of Gymnophalloides seoi infection in a small coastal village of Chollanam-do (Province), Korea, since it was first known as an endemic area in 1994. Special attention was given to its egg laying capacity in the human host. In fecal examinations, the overall helminth egg and/or cyst positive rate was 78.7% (74/94) in 1997 and 76.6% (82/107) in 2000. Among them G. seoi eggs showed the highest rate; 71.3% (67/94) in 1997 and 72.0% (77/107) in 2000. The average number of eggs per gram of feces (EPG) was 1,015 in 1997, while a reduced rate of 353 was observed in 2000. In 1997, total of 320,677 adult flukes of G. seoi (av. 10,344/person, 94-69,125 in range) were collected from the diarrheic stools of 31 treated patients. The EPG/worm obtained from 21 cases ranged from 0.04 to 0.77 (av. 0.23), suggesting density-dependent constraints on the worm fecundity. The relationship between the worm burden (X) and EPG/worm (Y) can be expressed as $Y=0.42{\cdot}e{\cdot}^{-l.2x}(r=0.49)$. The results showed that G. seoi infection is persistently endemic in this village.

• Science of Emotion and Sensibility
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• v.20 no.1
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• pp.95-104
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• 2017

This study aims to develop a reliable and valid Geriatric Reaction Inventory-Simplified Form (GRI-SF). Reaction inventory for measuring anger (Evans & Stangelang, 1971) is an assessment tool in a view of stimuli for measuring the level of anger invoked from the anger-triggered experiences. Yet, the inventory, comprised of 76 items, is sometimes incapable of finishing up the lengthy questionnaire. In the sense, a simplified form of RI is necessary for those lacks cognitive ability such as stroke patients or the elderly. In study 1, a full version of Reaction Inventory was given for the elder who are above 60, and ten items out of 76 was selected to form GRI-SF based on the psychometric theory. In study 2, the reliability and validity of GRI-SF was tested by another sample group of elderly with some additional examinations. The reliability was good with Cronbach's alpha of 0.79. Based on the fact that GRI-SF measures the personal trait anger, GRI-SF should not be only strong correlation with the scores of trait anger strongly, but be less correlated with scores representing other aspects of anger. As a result, GRI-SF score showed strong correlations with trait anger among other sub-categories of State-Trait Expression Inventory (STAXI), and was less correlated with anger-out score which reflects behavioral aspect of anger. Moreover, it is less correlated (r=.41) with hostility representing attitudinal or cognitive aspects of anger. Consequently, GRI-SF, constructed by this research, is verified to be a reliable and valid tool for anger measurement.

• Molecules and Cells
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• v.24 no.2
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• pp.200-209
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• 2007

We generated gene expression data from the tissues of 50 gastric cancer patients, and applied meta-analysis and gene set analysis to this data and three other stomach cancer gene expression data sets to define the gene expression changes in gastric tumors. By meta-analysis we identified genes consistently changed in gastric carcinomas, while gene set analysis revealed consistently changed biological themes. Genes and gene sets involved in digestion, fatty acid metabolism, and ion transport were consistently down-regulated in gastric carcinomas, while those involved in cellular proliferation, cell cycle, and DNA replication were consistently up-regulated. We also found significant differences between the genes and gene sets expressed in diffuse and intestinal type gastric carcinoma. By gene set analysis of cytogenetic bands, we identified many chromosomal regions with possible gross chromosomal changes (amplifications or deletions). Similar analysis of transcription factor binding sites (TFBSs), revealed transcription factors that may have caused the observed gene expression changes in gastric carcinomas, and we confirmed the overexpression of one of these, E2F1, in many gastric carcinomas by tissue array and immunohistochemistry. We have incorporated the results of our meta- and gene set analyses into a web accessible database (http://human-genome.kribb.re.kr/stomach/).

• Korean Journal of Clinical Pharmacy
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• v.20 no.1
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• pp.9-16
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• 2010

Purpose: 본 연구는 한국인 성인 조혈모세포이식환자를 대상으로 경구용 사이클로스포린의 집단약동학 분석을 통하여 사이클로스포린의 약동학적 파라미터에 영향을 미치는 요인 분석을 실시하고자 하였다. Methods: 2000년 12월부터 2006년 8월까지 서울대학교병원에서 동종조혈모세포이식을 받고 면역억제제로 사이클로스포린을 복용한 성인 백혈병환자를 대상으로 후향적으로 자료를 수집하였다. 사이클로스포린의 약동학에 영향을 미치는 인자로는 연령, 성별, 이식 후 날짜, 신기능, 공여자와의 관계, 질병의 종류, 혈중 빌리루빈 농도, 사이클로스포린의 대사를 유도하는 프레드니솔론의 투여량, 헤마토크리트, 사이클로스포린의 대사를 저해하는 약물의 병용여부 등을 검토하였다. 분석은 NONMEM$^{(R)}$ VI 프로그램을 이용하였으며, 변수를 추가하지 않은 기본 모형을 만든 후에 단계적인 요인의 추가와 제거를 통해 최종모형을 제작하였다. Results: 최종 상관 모형은 다음과 같다; CL/F (L/h) = $85.6{\times}e^{(0.646\;{\times}\;HCT/28.9\;+\;0.0464\;{\times}\;Gender)}$. 사이클로스포린의 겉보기 클리어런스는 환자의 성별이 남자일 때 또는 헤마토크릿이 감소할수록 증가하였다. 그 외 파라미터는 다음과 같이 계산되었다; $K_{\alpha}=0.0787\;(h^{-1})$; Q=57.1(L/kg/h); $V_{d-central\;compartment}$=1,100 (L); $V_{d-peripheral\;compartment}$ = 213,000(L). 개체간 편차는 40% 미만이었으며, 개체내 편차를 포함하는 잔차는 24.02%였다. Conclusions: 사이클로스포린의 약동학적 특징과 그 클리어런스에 영향을 끼칠 수 있는 임상적 요인을 이해하는 것은 환자 개개인의 용량과 용법의 결정 및 이상반응 발생의 예방에 유용할 수 있다. 한국인 조혈모세포이식환자에서 사이클로스포린의 약동학에 영향을 미치는 최종 파라미터를 구한 본 연구의 결과는 조혈모세포이식을 받은 한국인 성인환자에서 사이클로스포린의 모니터링 및 용량조절에 유용할 것으로 전망된다.

• Proceedings of the Korean Society of Fisheries Technology Conference
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• 2000.05a
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• pp.71-73
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• 2000

Campylobacter jejuni is most frequently identified cause of cause of acute diarrhoeal infections in developeed countries, exceeding rates of illness caused by both salmonella and shigilla(Skirrow, 1990 ; Lior 1994). Previous studies on campylobacter jejuni contamination of commercial broiler carcasses in u.s.(Stern, 1992). Most cases of the disease result from indirect transmission of Campylobactor from animals via milk, water and meat. In addition to Campylobactor jejuni. the closely relates species Campylobactor coli and Campylobactor lari have also been implicated as agents of gastroenteritis in humans. Campylobactor coli represented only approximately 3% of the Campylobactor isolates from patients with Campylobactor enteritis(Griffiths and Park, 1990) whereas Campylobactor coli is mainly isolated from pork(Lmmerding et al., 1988). Campylobactor jejuni has also been isolated from cases of bacteremia, appendicitis and, recently, has been associated with Guillai-Barre syndrome(Allos and Blaser, 1994; von Wulffen et al., 1994; Phillips, 1995). Studies in volunteers indicated that the infectious dose for Campylobactor jejuni is low(about 500 organisms)(Robinson, 1981). The methods traditionally used to detect Campylobactor ssp. in food require at least two days of incubation in an enrichment broth followed by plating and two days of incubation on complex culture media containing many antibiotics(Goossens and Butzler, 1992). Finnaly, several biochemical tests must be done to confirm the indentification at the species level. Therfore, sensitive and specific methods for the detection of small numbers of Campylobactor cells in food are needed. Polymerase chain reaction(PCR) assays targeting specific DNA sequences have been developed for the detection of Campylobactor(Giesendorf and Quint, 1995; Hemandex et al., 1995; Winter and Slavidk, 1995). In most cases, a short enrichment step is needed to enhance the sensitivity of the assay prior to detection by PCR as the number of bacteria in the food products is low in comparison with those found in dinical samples, and because the complex composition of food matrices can hinder the PCR and lower its sensitivity. However, these PCR systems are technically demanding to carry out and cumbersome when processing a large number of samples simutaneously. In this paper, an immunomagnetic method to concentrate Campylobactor cells present in food or clinical samples after an enrichment step is described. To detect specifically the thermophilic Campylobactor. a monoclonal antibody was adsorbed on the surface of the magnetic beads which react against a major porin of 45kDa present on the surface of the cells(Huyer et al., 1986). After this partial purification and concentration step, detection of bound cells was achieved using a simple, inexpensive microtitre plate-based hybridization system. We examined two alternative detection systems, one specific for thermophilic Campylobactor based on the detection of 23S rRNA using an immobilized DNA probe. The second system is less specific but more sensitive because of the high copy number of the rRNA present in bacterial cell($10^3-10^4$). By using specific immunomagnetic beads against thermophilic Campylobactor, it was possible to concentrate these cells from a heterogeneous media and obtain highly specific hybridization reactions with good sensitivity. There are several advantages in using microtitre plates instead of filter membranes or other matrices for hybridization techniques. Microtitre plates are much easier to handle than filter membranes during the adsorption, washing, hybridization and detection steps, and their use faciilitates the simultanuous analysis of multiple sample. Here we report on the use of a very simple detection procedure based on a monoclonal anti-RNA-DNA hybrid antibody(Fliss et al., 1999) for detection of the RNA-DNA hybrids formed in the wells.

• Tuberculosis and Respiratory Diseases
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• v.48 no.5
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• pp.766-772
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• 2000

Background : Pressure rise time (PRT) is the time in which the ventilator aclieves the set airway pressure in pressure-targeted modes, such as pressure control ventilation (PCV). With varying PRT, in principle, the peak inspiratory flow rate of the ventilator also varies. And if PRT is set to a shorter duration, the effective duration of target pressure level would be prolonged, which in turn would increase inspiratory tidal volume(Vti) and mean airway pressure (Pmean). We also postulated that the increase in Vti with shortening of PRT may relate inversely to the patients' basal airway resistance. Methods : In 13 paralyzed patients on PCV (pressure control 18$\pm$9.5 cm $H_2O$ $FIO_2\;0.6\pm0.3$, PEEP 5$\pm$3 cm $H_2O$, f 20/min, I : E1 : 2) with Servo 300 (Siemens-Elema, Solna, Sweden) from various causes of respiratory failure, PRT of 10 %, 5 % and 0 % were randomly applied. At 30 min of each PRT trial, peak inspiratory flow (PIF, L/sec), Vti (ml), Pmean (cm $H_2O$) and ABGA were determined. Results : At PRT 10%, 5%, and 0%, PIF were 0.69$\pm$0.13, 0.77$\pm$0.19, 0.83$\pm$0.22, respectively (p<0.001). Vti were 425$\pm$94, 439$\pm$101, 456$\pm$106, respectively (p<0.001), and Pmean were 11.2$\pm$3.7, 12.0$\pm$3.7, 12.5$\pm$3.8, respectively (p<0.001). pH were 7.40$\pm$0.08, 7.40$\pm$0.92, 7.41$\pm$0.96, respectively (p=0.00) ; $PaCO_2$ (mm Hg) were 47.4$\pm$15.8, 47.2 $\pm$15.7, 44.6$\pm$16.2, respectively (p=0.004) ; $PAO_2-PaO_2$ (mm Hg) were 220$\pm$98, 224$\pm$95, 227$\pm$94, respectively (p=0.004) ; and $V_n/V_T$ as determined by ($PaCO_2-P_E-CO_2$)/$PaCO_2$ were 0.67$\pm$0.07, 0.67$\pm$0.08, 0.66$\pm$0.08, respectively (p=0.007). The correlation between airway resistance and change of Vti from PRT 10% to 0% were r= -0.243 (p=0.498). Conclusion : Shortening of pressure rise timee during PCV was associated with increased tidal volume, increased mean airway pressure and lower $PaCO_2$.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.11 no.2
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• pp.209-220
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• 2000

The purpose of this study is to find out the characteristics of depressive episode about major depression and bipolar disorder in child and adolescent. The subjects of this study were 34 major depression patients and 17 bipolar disorder patients hospitalized at child and adolescent psychiatry in OO university children's hospital from 1st March 1993 to 31st October 1999. The method of this study is to review socio-demographic characteristics, diagnostic classification, chief problems and symptoms at admission, frequency of symptoms, maternal pregnancy problem history, childhood developmental history, coexisting psychiatric disorders, family psychopathology and family history and therapeutic response through their chart. 1) The ratio of male was higher than that of female in major depressive disorder while they are similar in manic episode, bipolar disorder. 2) Average onset age of bipolar disorder was 14 years 1 month and it was 12 years 8 months in the case of major depression As a result, average onset age of major depression is lower than that of bipolar disorder. 3) The patients complained of vegetative symptoms than somatic symptoms in both bipolar disorder and depressive disorder. Also, the cases of major depression developed more suicide idea symptom while the case of bipolar disorder developed more aggressive symptoms. In the respect of psychotic symptoms, delusion was more frequently shown in major depression, but halucination was more often shown in bipolar disorder. 4) Anxiety disorder coexisted most frequently in two groups. And there coexisted symptoms such as somartoform disorder, mental retardation and personality disorder in both cases. 5) The influence of family loading was remarkable in both cases. Above all, the development of major depression had to do with child abuse history and inappropriate care of family. It is apparent that there are distinctive differences between major depression and bipolar disorder in child and adolescent through the study, just as in adult cases. Therefore the differences of clinical characteristics between two disorders is founded in coexisting disorders and clinical symptoms including onset age, somatic symptoms and vegetative symptoms.

• Journal of Korean Academy of Nursing
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• v.11 no.1
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• pp.65-87
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• 1981

The study is aimed at exploring concept held by clinical nurses of nursing. The data were collected from 225 nurses conviniently selected from the population of nurses working in Kang Won province. Findings include. 1) Nurse's Qualification. The respondents view that specialized knowledge is more important qualification of the nurse. Than warm personality. Specifically, 92.9% of the respondents indicated specialized knowledge as the most important qualification while only 43.1％ indicated warm personality. 2) On Nursing Profession. The respondents view that nursing profession as health service oriented rather than independent profession specifically. This suggests that nursing profession is not consistentic present health care delivery system nor support nurses working independently. 3) On Clients of Nursing Care The respondents include patients, family and the community residents in the category of nursing care. Specifically, 92.0％ of the respondents view that patient is the client, while only 67.1％ of nursing student and 74.7％ of herself. This indicates the lack of the nurse's recognition toward their clients. 4) On the Priority of Nursing care. Most of the respondents view the clients physical psychological respects as important component of nursing care but not the spiritual ones. Specially, 96.0% of the respondents indicated the physical respects, 93% psychological ones, while 64.1% indicated the spiritual ones. This means the lack of comprehensive conception on nursing aimension. 5) On Nursing Care. 91.6% of the respondents indicated that nursing care is the activity decreasing pain or helping to recover illness, while only 66.2% indicated earring out the physicians medical orders. 6) On Purpose of Nursing Care. 89.8% of the respondents indicated preventing illness and than 76.6% of them decreasing 1;ai of clients. On the other hand, maintaining health has the lowest selection at the degree of 13.8%. This means the lack of nurses' recognition for maintaining health as the most important point. 7) On Knowledge Needed in Nursing Care. Most of the respondents view that the knowledge faced with the spot of nursing care is needed. Specially, 81.3% of the respondents indicated simple curing method and 75.1%, 73.3%, 71.6% each indicated child nursing, maternal nursing and controlling for the communicable disease. On the other hand, knowledge w hick has been neglected in the specialized courses of nursing education, that is, thinking line among com-w unity members, overcoming style against between stress and personal relation in each home, and administration, management have a low selection at the depree of 48.9%,41.875 and 41.3%. 8) On Nursing Idea. The highest degree of selection is that they know themselves rightly, (The mean score measuring distribution was 4.205/5) In the lowest degree,3.016/5 is that devotion is the essential element of nursing, 2.860/5 the religious problems that human beings can not settle, such as a fatal ones, 2,810/5 the nursing profession is worth trying in one's life. This means that the peculiarly essential ideas on the professional sense of value. 9) On Nursing Services. The mean score measuring distribution for the nursing services showed that the inserting of machine air way is 2.132/5, the technique and knowledge for surviving heart-lung resuscitating is 2.892/s, and the preventing air pollution 3.021/5. Specially, 41.1% of the respondents indicated the lack of the replied ratio. 10) On Nurses' Qualifications. The respondents were selected five items as the most important qualifications. Specially, 17.4% of the respondents indicated specialized knowledge, 15.3% the nurses' health, 10.6％ satisfaction for nursing profession, 9.8% the experience need, 9.2% comprehension and cooperation, while warm personality as nursing qualifications have a tendency of being lighted. 11) On the Priority of Nursing Care The respondents were selected three items as the most important component. Most of the respondents view the client's physical, spiritual: economic points as important components of nursing care. They showed each 36.8%, 27.6%, 13.8% while educational ones showed 1.8%. 12) On Purpose of Nursing Care. The respondents were selected four items as the most important purpose. Specially,29.3% of the respondents indicated curing illness for clients, 21.3% preventing illness for client 17.4% decreasing pain, 15.3% surviving. 13) On the Analysis of Important Nursing Care Ranging from 5 point to 25 point, the nurses' qualification are concentrated at the degree of 95.1%. Ranging from 3 point to 25, the priorities of nursing care are concentrated at the degree of 96.4%. Ranging from 4 point to 16, the purpose of nursing care is concentrated at the degree of 84.0%. 14) The Analysis, of General Characteristics and Facts of Nursing Concept. The correlation between the educational high level and nursing care showed significance. (P < 0.0262). The correction between the educational low level and purpose of nursing care showed significance. (P < 0.002) The correlation between nurses' working yeras and the degree of importance for the purpose of nursing care showed significance (P < 0.0155) Specially, the most affirmative answers were showed from two years to four ones. 15) On Nunes' qualification and its Degree of Importance The correlation between nurses' qualification and its degree of importance showed significance. (r = 0.2172, p< 0.001) 0.005) B. General characteristics of the subjects The mean age of the subject was 39 ; with 38.6% with in the age range of 20-29 ; 52.6% were male; 57.9％ were Schizophrenia; 35.1% were graduated from high school or high school dropouts; 56.l％ were not have any religion; 52.6% were unmarried； 47.4% were first admission; 91.2% were involuntary admission patients. C. Measurement of anxiety variables. 1. Measurement tools of affective anxiety in this study demonstrated high reliability (.854). 2. Measurement tools of somatic anxiety in this study demonstrated high reliability (.920). D. Relationship between the anxiety variables and the general characteristics. 1. Relationship between affective anxiety and general characteristics. 1) The level of female patients were higher than that of the male patient (t = 5.41, p < 0.05). 2) Frequencies of admission were related to affective anxiety, so in the first admission the anxiety level was the highest. (F = 5.50, p < 0.005). 2, Relationship between somatic anxiety and general characteristics. 1) The age range of 30-39 was found to have the highest level of the somatic anxiety. (F = 3.95, p < 0.005). 2) Frequencies of admission were related to the somatic anxiety, so .in first admission the anxiety level was the highest. (F = 9.12, p ＜ 0.005) 0. Analysis of significant anxiety symptoms for nursing intervention. 1. Seven items such as dizziness, mental integration, sweating, restlessness, anxiousness, urinary frequency and insomnia, init. accounted for 96％ of the variation within the first 24 hours after admission. 2. Seven items such as fear, paresthesias, restlessness, sweating insomnia, init., tremors and body aches and pains accounted for 84% of the variation on the 10th day after admission.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.7 no.1
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• pp.77-91
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• 1996

In order to elucidate the biological etiology and the effects of comorbidity on biological variables in tic disorders, plasma serotonin (5-hydroxlfryptamine, 5-HT) and 5-hydroxy- indoleacetic acid (5-HIAA) we.e measured in 87 tic disorders and 30 control subjects. The 87 tic disorder were composed of 45 Tourette's disorder(TS), 22 chronic motor tic disorders (CMT) and 20 transient tic disorders (TTD). Among these patients,43 patients were pure tic disorder (PT), 28 subject also had attention deficit hyperactivity disorder (T+ADHD) and 16 subjects had obsessive compulsive disorders (T+ OCD) as comorbid disorders. The results are summarized as follows : 1) Plasma 5-HT levels showed significant positive correlations with plasma 5-HIAA levels (Pennon r=0.77, p<0.05). 2) Plasma 5-HT and 5-HIAA levels showed no significant correlation with age in tic disorders. 3) Plasma 5-HIAA and 5-HT levels showed no significant correlations with age in control subjects. 4) There was significant difference in plasma 5-HT levels among TS, CMT, TTD and control groups (ANOVA F=34.48, df=3, 113, p<0.01), and post-hoc test using Scheffe method showed significant differences between control and TS, control and CMT, control and ITD groups. But, post-hoc test showed no significant differences between TS and CMT, TS and TTD, CMT and TTD groups. 5) There was significant difference in plasma 5-HIAA levels among TS, CMT, TTD and control groups (ANOVA F=26.48, df=3, 113, p<0.01), and post-hoc test using Scheffe method showed significant differences between control and TS, control and CMT, control and TTD groups. But, post-hoc test showed no significant differences between TS and CMT, TS and TTD, CMT and TID groups.f) There was significant difference in plasma 5-HT and 5-HIAA levels among PT, T+ADHD, T+OCD and contol groups (ANOVA 5-HT, F=37.59, df=3, 113, p<0.01, 5-HIAA, F=27.37, df=3, 113, p<0.01), and post-hoc test using Scheffe method showed signiscant differences between control and PT, control and T+ADHD and control and T+OCB. But, post-hoc test showed no significant differences between PT and T+ADHD, PT and T+ OCD and T+ADHD and T+ OCD. These results show that decreased 5-HT and 5-HIAA levels may play a role in the genesis of tic disorders, but these findings have no significant correlations with the severity of tic disorders. And the comorbid disorders of tics may have minimal effects on the biochemical abnormalities. Future studies must be focused on the effects of serotonin agonists and antagonists on tic disorders and molecular biological methodology may enhance to elucidate the mechanisms of these abnormal findings.