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A Study on Effects of the vocal psychotherapy upon Self-Consciousness (성악심리치료활동을 통한 자기의식 변화에 관한 연구)

  • Lee, Hyun Joo
    • Journal of Music and Human Behavior
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    • v.4 no.2
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    • pp.66-83
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    • 2007
  • The purpose of this study is to learn both effects of the vocal psychotherapy on the self-consciousness and the variety of the self-consciousness on the vocal psychotherapy in return. The research for this study was performed to three subjects who were students of E university, Seoul, ten times for sixty minutes. The subjects were all volunteers for the advertisement on a music-therapy program searching for them on the web site of E university. The vocal psychotherapy program consists of four steps and each of them consists of two to four short terms again. Both before and after the experiment, examinations on self-consciousness were done to recognize the change of the subjects' self-consciousness which would be caused by the vocal psychotherapy activity. After every short term, the subjects were asked to write reports to closely analyze the change of self-consciousness according to the terms and the variety of the subjects. The effect of the vocal psychotherapy activity on the changes of scores in the self-consciousness examination is the first thing to point out on this study. There appeared some personal varieties on the total scores of the examination and scores of some sub-categories. Especially, there were different scores on the private self-consciousness, the public self-consciousness, and the social anxiety between before and after performing the vocal psychotherapy program. Subject A, who had got the best score of all on the scope of the private self-consciousness, showed the steepest decrease on the very scope. On the contrary, the subject showed decrease of scores of the public self-consciousness and the social anxiety in the relatively little rate. Subject B, who had got the highest score of the three on the public self-consciousness, showed the steepest decrease on that of all scopes and showed no difference on the social anxiety scope. In the case of the last one, subject C, who had relatively low scores on the private and public self-consciousness than the others, the private self-consciousness score increased but the public self-consciousness and the social anxiety scores decreased. The changes of the scores of each questions were examined in order to see possible other changes that had not been exposed on the changes of the total and sub-categories scores. As a result of that, of all twenty-eight questions, there were changes about one to two points. Subject A showed the difference with thirteen questions, subject B with sixteen and subject C with nineteen questions. The rate of change of subject C was relatively small but more questions changed and the change of score was wider than the others. Considering all those results, It can be possibly said that the vocal psychotherapy affects the changes of the scores of sub-categories in self-consciousness examination. The next thing to point out on this study is the change of recognition that was exposed on the subjects' report after every short term of the program. As a result of the close analyzing, according to the short terms and variety of self-consciousness, recognizing the way express subjects themselves by voice and recognizing their own voices appeared to be different. How much they cared about others and why they did so were also different. According to the self reports, subject A cared much about her inner thought and emotion and tended to concentrate herself as a social object. There appeared some positive emotional experiments such as emotional abundance and art curiosities on her reports but at the same time some negative emotions such as state-trait anxiety and neuroticism also appeared. Subject B, who showed high scores on the private and public self-consciousness like subject A, had a similar tendency that concentrates on herself as a social object but she showed more social anxiety than subject A. Subject C got relatively lower points in self-consciousness examination, tended to care about herself, and had less negative emotions such as state-trait anxiety than other subjects. Also, with terms going on, she showed changes in the way of caring about her own voice and others. This study has some unique significances in helping people who have problems caused by self-estimation activated with self-consciousness, using voices closely related to one's own self, performing the vocal skills discipline to solve the technical problems. Also, this study has a potentiality that the vocal psychotherapy activity can be effectively used as a way affects the mental health and developing personality.

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A Study for Developing Music Therapy Activity Program for Development of Rudimentary Movement Phase of Spastic Cerebral Palsied Infant : Applying the techniques of Neurological Music Therapy (경직형 뇌성마비 유아의 초보운동단계 발달을 위한 음악치료활동 프로그램 개발 - 신경학적 음악치료의 기법을 활용하여)

  • Lee, Yoon Jin
    • Journal of Music and Human Behavior
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    • v.4 no.2
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    • pp.84-105
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    • 2007
  • Cerebral palsy is a collection of motor disorders resulting from damage to the central nervous system that arise in multiple handicaps including cognitive disorders, speech disorders, epilepsy, perception disorders, and emotion disorders. Today spastic cerebral palsy has become more prevalent because intensive care for newborns has resulted in higher survival rates for very small premature babies. Since the children grow the fastest in order for a development during one year after birth, the therapeutic intervention is provided as early as possible to the children with cerebral palsy. After seven year old, there is no effect of intervention. So, the necessity of early intervention to spastic cerebral palsied infants is increasing. The purpose of this study is to develop the music therapy activity program using the techniques of neurological music therapy(NMT), the therapeutic application of music to dysfunctions due to neurologic disease of the human nervous system, for rudimentary movement phase of spastic cerebral palsied infant. This music therapy activity program was developed on the basis of the major developmental tasks of the rudimentary movement phase, the period that children can acquire the most basic movement function at the 0 to 2. Then the developmental characteristics of spastic cerebral palsy were applied to this music therapy activity program. This music therapy activity program was classified to three domains, those are stability, locomotion, and manipulation. This study has been consisted of three steps, those are the development of the activities, the evaluation of the activities by th panels, and the adjustment and complement of the activities. Reviewing literatures and interviews were done for the development of the activities, and the evaluation the activities was done by seven music therapists. In the evaluation steps, the questionnaire was used for estimating the content validity and application efficiency. The adjustment and complement of the activities were evaluated by the panels who were participating in the music therapy for cerebral palsied children in the clinical setting, and the results of the adjustment and complement were confirmed by the panels. The evaluation was presented in a mean value with the comment of the panels. In conclusion, the music therapy activity program for the spastic cerebral palsied infants using the techniques of NMT was developed on the basis of the major developmental tasks of the rudimentary movement phase. The program is comprised of 38 activities, those are 14 activities for developing the stability, 10 activities for developing the locomotion, and 14 activities for developing the manipulation. The programed activities would bring out the answers in the affirmative for the conformance with infants' development phase, the harmony between the objective and the activity, the conformance with the cerebral palsied infants, the properness of the music and the instruments, and the utility in the clinic field. This results mean that this developed music activity program is appropriate to help spastic cerebral palsied infants progress their movement development by stages.

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Second allogeneic hematopoietic stem cell transplantation in children to overcome graft failure or relapse after initial transplant (조혈모세포이식 후 생착 실패나 재발한 소아환자에서 2차 이식의 의의)

  • Kim, Dong-Yeon;Kim, Do Kyun;Kim, Soo Young;Kim, Seok Joo;Han, Dong Gyun;Baek, Hee Jo;Kook, Hoon;Hwang, Tai-Ju
    • Clinical and Experimental Pediatrics
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    • v.49 no.12
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    • pp.1329-1339
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    • 2006
  • Purpose : Failure of hematopoietic stem cell transplantation(HSCT) may be encountered in practice because of either relapse of the malignancy or dysfunction of the graft. Second HSCT may be the only option for some patients whose initial HSCT failed. Methods : From May, 1991 to December, 2004, 115 HSCTs were performed at the Pediatric Blood & Marrow Transplantation Center, Chonnam National University. This study was a retrospective analysis of the medical records of 15 patients who received the second HSCT after initial graft. Results : Among eight patients with nonmalignant diseases, two patients underwent the second HSCT because of primary graft failure and five because of late graft rejection. The remaining Fanconi anemia patient was re-transplanted due to development of AML. Two patients died and one experienced primary graft failure, but is still alive. The Kaplan-Meier 5-year overall survival rate was 75 percent and the disease free survival rate was 62.5 percent in nonmalignant diseases. All malignant patients underwent second transplants because of relapses. Four died of relapse and one of treatment-related complications. The Kaplan-Meier 2-year overall and event free survival rate was 28.6 percent each in malignant diseases. Conclusion : Second HSCT for graft dysfunction of nonmalignant disease seems to be feasible and should be considered as a standard practice. The relapse of malignant diseases remains a big obstacle even after the second HSCT, although a small portion of patients might be salvaged. Further investigation of novel therapeutic strategies, as well an the understanding of the biology should be explored.

A comparative study of the puberty suppression effect of gonadotropin-releasing hormone agonist in precocious or early puberty girls (중추성 성조숙증 및 조기 사춘기 여아에서 성선자극호르몬 방출호르몬작용제의 용량에 따른 사춘기 억제 효과 비교)

  • Shim, Kye-Shik;Bae, Chong-Woo;Yang, You-Jung
    • Clinical and Experimental Pediatrics
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    • v.51 no.6
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    • pp.634-639
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    • 2008
  • Purpose : There has been considerable disagreement regarding the most appropriate dosage of gonadotropin-releasing hormone agonist in cases of central precocious puberty. The aim of this study was to determine the appropriate dosage for suppression of the puberty in girls with central precocious or early puberty. Methods : Twenty-two girls with early puberty were randomly subjected to 3 types of dosages of leuprolide acetate for at least 6 months. The number of cases in groups 1, 2, and 3 were 7, 7, and 8, and dosages were 70, 90, and $110{\mu}g/kg/-month$, respectively. Height, weight, bone age, Tanner stage of breast development, and serum levels of LH, FSH, estradiol, and progesterone were measured before treatment and after 6 months of treatment. The number of cases of puberty suppression was compared using a modified puberty suppression score with a nonparametric chi-square test. Results : There were no significant differences of chronologic and bone ages among the groups. There was a significant decrease in height SDS gain after 6 months in group 3 (P<0.05) compared with groups 1 and 2. Serum levels of LH, FSH, estradiol and progesterone were all significantly decreased after treatment in all 3 groups (P<0.05). The number of cases of puberty suppression in each group were 4 (57%), 5 (71%), and 8 (100%). There was a significantly increased proportion of suppression of puberty in group 3 (P<0.05). Conclusion : It was necessary to use a higher dose of gonadotropin-releasing hormone agonist to suppress early puberty in girls; however further longitudinal study will be needed for their prognosis of final adult height.

A Study on the Factors Influencing Technology Innovation Capability on the Knowledge Management Performance of the Company: Focused on Government Small and Medium Venture Business R&D Business (기술혁신역량이 기업의 지식경영성과에 미치는 요인에 관한 연구: 정부 중소벤처기업 R&D사업을 중심으로)

  • Seol, Dong-Cheol;Park, Cheol-Woo
    • Asia-Pacific Journal of Business Venturing and Entrepreneurship
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    • v.15 no.4
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    • pp.193-216
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    • 2020
  • Due to the recent mid- to long-term slump and falling growth rates in the global economy, interest in organizational structures that create new products or services as a new alternative to survive and develop in an opaque environment both internally and externally, and enhance organizational sustainability through changes in production methods and business innovation is increasing day by day. In this atmosphere, we agree that the growth of small and medium-sized venture companies has a significant impact on the national economy, and various efforts are being made to enhance the technological innovation capabilities of the members so that these small and medium-sized venture companies can enhance and sustain their performance. The purpose of this study is also to investigate how the technological innovation capabilities of small and medium-sized venture companies correlate with the performance of knowledge management and to analyze the role of network capabilities to organize the strategic activities of enterprise to obtain the resources and organizational capabilities to be used for value creation from external networks. In other words, research was conducted on the impact of technological innovation capabilities of small and medium venture companies on knowledge management performance by using network capabilities as parameters. Therefore, in this study, we would like to verify the hypothesis that innovation capabilities will have a positive impact on knowledge management performance by using network capabilities of small and medium venture companies. Economic activities based on technological innovation capabilities should respond quickly to new changes in an environment where uncertainty has increased, and lead to macro-economic growth and development as well as overcoming long-term economic downturns so that they can become the nation's new growth engine as well as sustainable growth and survival of the organization. In addition, this study was conducted by setting the most important knowledge management performance within the organization as a dependent variable. As a result, R&D and learning capabilities among technological innovation capabilities have no impact on financial performance. In contrast, it was shown that corporate innovation activities have a positive impact on both financial and non-financial performance. The fact that non-financial factors such as quality and productivity improvement are identified in the management of small and medium-sized venture companies utilizing their technological innovation capabilities is contrary to a number of studies by those corporate innovation activities affect financial performance during prior research. The reason for this result is that research companies have been out of start-up companies for more than seven years, but sales are less than 10 billion won, and unlike start-up companies, R&D and learning capabilities have more positive effects on intangible non-financial performance than financial performance. Corporate innovation activities have been shown to have a positive (+) impact on both financial and non-financial performance, while R&D and learning capabilities have a positive (+) impact on financial performance by parameters of network capability. Corporate innovation activities have been shown to have no impact on both financial and non-financial performance, and R&D and learning capabilities have no impact on non-financial performance. It could be seen that the parameter effects of network competency are limited to when R&D and learning competencies are derived from quantitative financial performance. It could be seen that the parameter effects of network competency are limited to when R&D and learning competencies are derived from quantitative financial performance.

The Comparative Imaging Study on Mn-phthalocyanine and Mangafodipir trisodium in Experimental VX2 Animal Model (실험적으로 유발시킨 VX2 동물모델에서의 Mn-phthalocyanine과 Mangafodipir trisodium의 비교영상)

  • Park Hyun-Jeong;Ko Sung-Min;Kim Yong-Sun;Chang Yongmin
    • Investigative Magnetic Resonance Imaging
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    • v.8 no.1
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    • pp.32-41
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    • 2004
  • Purpose : To measure the NMR relaxation properties of MnPC, to observe the characteristics of liver enhancement patterns on MR images in experimentally implanted rabbit VX2 tumor model, and to estimate the possibility of tissue specific contrast agent for MnPC in comparison with the hepatobiliary agent. Materials and Methods : Phthalocyanine (PC) was chelated with paramagnetic ions, manganese (Mn). 2.01 g (5.2 mmol) of phthalocyanine was mixed with 0.37 g (1.4 nlmol) of Mn chloride at $310^{\circ}C$ for 36 hours and then purified by chromatography ($CHCl_3:\;CH_3OH=98:2$, volume ratio) to obtain 1.04 g $(46\%)$ of MnPC (molecular weight = 2000 daltons). The T1/T2 relaxivity (R1/R2) for MnPC were determined at a 1.5 T (64 MHz) MR spectrometer. VX2 tumor model was experimentally implanted in the liver parenchyma of rabbits. All MR studies were performed on 1.5 T. The human extremity radio frequency coil of a bird cage type was employed. MR images were acquired at 17 to 24 days after VX2 carcinoma implantation.4 mmol/kg MnPC and 0.01 mmol/kg Mn-DPDP were injected via the ear vein of rabbits. T1-weighted images were obtained with spin-echo (TR/TE=516/14 msec) and fast multiplanar spoiled gradient recalled (TR/TE : 80/4 msec, $60^{\circ}$ flip angle) pulse sequence. Fast spin-echo (TR/TE=1200/85 msec) was used to obtain the T2-weighted images. Results : The value of T1/T2 relaxivity (R1/R2) of MnPC was $7.28\;mM^{-1}S^{-1}$ and $55.56\;mM^{-1}S^{-1}$ respectively at 1.5 T (64 MHz). Because the T2 relaxivity of MnPC that bonded strongly, covalently manganese with phthalocyanine was very high, the signal intensity of liver parenchyma was decreased on postcontrast T2-weighted images and we could easily distinguish the VX2 carcinoma within the liver parenchyma. When MnPC was administrated intravenously, the tumor margin delineation was more remarkable than Mn-DPDP-enhanced images. The enhancement of liver parenchyma with MnPC persisted at relatively high levels over at least one hour after injection of the contrast agents. Conclusion : The hepatic uptake and biliary excretion of MnPC, which are similar to Mn-DPDP, suggest that this agent is a new liver-specific agent. Also, MnPC seems to be used as a dual contrast agent (T1 and T2) with high T2 relaxivity. However, it is warranted that MnPC needs further investigation as a potential contrast agent for MR imaging of the liver. That is, further characterizations of MnPC are needed in vivo and in vitro before clinical trials. The diagnostic potential of MnPC will also have to be examined more in the animal models of additional types.

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[ $^1H$ ] MR Spectroscopy of the Normal Human Brains: Comparison between Signa and Echospeed 1.5 T System (정상 뇌의 수소 자기공명분광 소견: 1.5 T Signa와 Echospeed 자기공명영상기기에서의 비교)

  • Kang Young Hye;Lee Yoon Mi;Park Sun Won;Suh Chang Hae;Lim Myung Kwan
    • Investigative Magnetic Resonance Imaging
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    • v.8 no.2
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    • pp.79-85
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    • 2004
  • Purpose : To evaluate the usefulness and reproducibility of $^1H$ MRS in different 1.5 T MR machines with different coils to compare the SNR, scan time and the spectral patterns in different brain regions in normal volunteers. Materials and Methods : Localized $^1H$ MR spectroscopy ($^1H$ MRS) was performed in a total of 10 normal volunteers (age; 20-45 years) with spectral parameters adjusted by the autoprescan routine (PROBE package). In all volunteers, MRS was performed in a three times using conventional MRS (Signa Horizon) with 1 channel coil and upgraded MRS (Echospeed plus with EXCITE) with both 1 channel and 8 channel coil. Using these three different machines and coils, SNRs of the spectra in both phantom and volunteers and (pre)scan time of MRS were compared. Two regions of the human brain (basal ganglia and deep white matter) were examined and relative metabolite ratios (NAA/Cr, Cho/Cr, and mI/Cr ratios) were measured in all volunteers. For all spectra, a STEAM localization sequence with three-pulse CHESS $H_2O$ suppression was used, with the following acquisition parameters: TR=3.0/2.0 sec, TE=30 msec, TM=13.7 msec, SW=2500 Hz, SI=2048 pts, AVG : 64/128, and NEX=2/8 (Signa/Echospeed). Results : The SNR was about over $30\%$ higher in Echospeed machine and time for prescan and scan was almost same in different machines and coils. Reliable spectra were obtained on both MRS systems and there were no significant differences in spectral patterns and relative metabolite ratios in two brain regions (p>0.05). Conclusion : Both conventional and new MRI systems are highly reliable and reproducible for $^1H$ MR spectroscopic examinations in human brains and there are no significant differences in applications for $^1H$ MRS between two different MRI systems.

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Synthesis of a Dopamine Transporter Imaging Agent, N-(3-[$^{18}F$]fluoropropyl)-$2{\beta}$-carbomethoxy-$3{\beta}$-(4-iodophenyl)nortropane (도파민운반체 방사성추적자 N-(3-[$^{18}F$Fluoropropyl)-$2{\beta}$-carbomethoxy-$3{\beta}$-(4-iodophenyl)nortropane의 합성)

  • Choe, Yearn-Seong;Oh, Seung-Jun;Chi, Dae-Yoon;Kim, Sang-Eun;Choi, Yong;Lee, Kyung-Han;Kim, Byung-Tae
    • The Korean Journal of Nuclear Medicine
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    • v.33 no.3
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    • pp.298-305
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    • 1999
  • Purpose: N-(3-[$^{18}F$]Fluoropropyl)-$2{\beta}$-carbomethoxy-$3{\beta}$-(4-iodophenyl)nortropane [$^{18}F$]FP-CIT) has been shown to be very useful for imaging the dopamine transporter. However, synthesis of this radiotracer is somewhat troublesome. In this study, we used a new method for the preparation of [$^{18}F$]FP-CIT to increase radiochemical yield and effective specific activity. Materials and Methods: [$^{18}F$]FP-CIT was prepared by N-alkylation of nor-${\beta}$-CIT (2 mg) with 3-bromo-1-[$^{18}F$]fluoropropane in the presence of $Et_3N$ (5-6 drops of $DMF/CH_3CN$, $140^{\circ}C$, 20 min). 3-Bromo-1-[$^{18}F$]fluoropropane was synthesized from $5{\mu}L$ of 3-bromo-1-trifluoromethanesulfonyloxypropane (3-bromopropyl-1-triflate) and $nBu_4N^{18}F$ at $80^{\circ}C$. The final compound was purified by reverse phase HPLC and formulated in 13% ethanol in saline. Results: 3-Bromo-1-[$^{18}F$]fluoropropane was obtained from 3-bromopropyl-1-triflate and $nBu_4N^{18}F$ in 77-80% yield. N-Alkylation of nor-${\beta}$-CIT with 3-bromo-1-[$^{18}F$]fluoropropane was carried out at $140^{\circ}C$ using acetonitrile containing a small volume of DMF as the solvents. The overall yield of [$^{18}F$]FP-CIT was 5-10% (decay-corrected) with a radiochemical purity higher than 99% and effective specific activity higher than the one reported in the literature based on their HPLC data. The final [$^{18}F$]FP-CIT solution had the optimal pH (7.0) and it was pyrogen-free. Conclusion: In this study, 3-bromopropyl-1-triflate was used as the precursor for the [$^{18}F$]fluorination reaction and new conditions were developed for purification of [$^{18}F$]FP-CIT by HPLC. We established this new method for the preparation of [$^{18}F$]FP-CIT, which gave high effective specific activity and relatively good yield.

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Effect of Repetitive Transcranial Magnetic Stimulation in Drug Resistant Depressed Patients (치료 저항성 우울증 환자에서 반복적 경두개 자기자극후 국소뇌혈류 변화)

  • Chung, Yong-An;Yoo, Ie-Ryung;Kang, Bong-Joo;Chae, Jeong-Ho;Lee, Hye-Won;Moon, Hyun-Jin;Kim, Sung-Hoon;Sohn, Hyung-Sun;Chung, Soo-Kyo
    • Nuclear Medicine and Molecular Imaging
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    • v.41 no.1
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    • pp.9-15
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    • 2007
  • Purpose: Repetitive transcranial magnetic stimulation (rTMS) has recently been clinically applied in the treatment of drug resistant depressed patients. There are mixed findings about the efficacy of rTMS on depression. Furthermore, the influence of rTMS on the physiology of the brain is not clear. We prospectively evaluated changes of regional cerebral blood flow (rCBF) between pre- and post-rTMS treatment in patients with drug resistant depression. Materials and Methods: Twelve patients with drug-resistant depression (7 male, 5 female; age range: $19{\sim}52$ years; mean age: $29.3{\pm}9.3$ years) were given rTMS on right prefrontal lobe with low frequency (1 Hz) and on left prefrontal lobe with high frequency (20 Hz), with 20-minute-duration each day for 3 weeks. Tc-99m ECD brain perfusion SPECT was obtained before and after rTMS treatment. The changes of cerebral perfusion were analyzed using statistical parametric mapping (SPM; t=3.14, uncorrected p<0.01, voxel=100). Results: Following areas showed significant increase in rCBF after 3 weeks rTMS treatment: the cingulate gyrus, fusiform gyrus of right temporal lobe, precuneus, and left lateral globus pallidus. Significant decrement was noted in: the precental and middle frontal gyrus of right frontal lobe, and fusiform gyrus of left occipital lobe. Conclusion: Low-frequency rTMS on the right prefrontal cortex and high-frequency rTMS on the left prefrontal cortex for 3 weeks as an add-on regimen have increased and decreased rCBF in the specific brain regions in drug-resistant depressed patients. Further analyses correlating clinical characteristics and treatment paradigm with functional imaging data may be helpful in clarifying the pathophysiology of drug-resistant depressed patients.

Clinical Characteristics of Arthrogryposis, Renal Tubular Dysfunction, Cholestasis(ARC) Syndrome in Korea (ARC(Arthrogryposis, Renal Tubular Dysfunction, Cholestasis) 증후군의 발병양상에 관한 연구)

  • Lee Soon Min;Kim Ji Hong;Lee Jae Seung;Han Suk Joo
    • Childhood Kidney Diseases
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    • v.9 no.2
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    • pp.222-230
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    • 2005
  • Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

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