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The Impacts of Need for Cognitive Closure, Psychological Wellbeing, and Social Factors on Impulse Purchasing (인지폐합수요(认知闭合需要), 심리건강화사회인소대충동구매적영향(心理健康和社会因素对冲动购买的影响))

  • Lee, Myong-Han;Schellhase, Ralf;Koo, Dong-Mo;Lee, Mi-Jeong
    • Journal of Global Scholars of Marketing Science
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    • v.19 no.4
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    • pp.44-56
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    • 2009
  • Impulse purchasing is defined as an immediate purchase with no pre-shopping intentions. Previous studies of impulse buying have focused primarily on factors linked to marketing mix variables, situational factors, and consumer demographics and traits. In previous studies, marketing mix variables such as product category, product type, and atmospheric factors including advertising, coupons, sales events, promotional stimuli at the point of sale, and media format have been used to evaluate product information. Some authors have also focused on situational factors surrounding the consumer. Factors such as the availability of credit card usage, time available, transportability of the products, and the presence and number of shopping companions were found to have a positive impact on impulse buying and/or impulse tendency. Research has also been conducted to evaluate the effects of individual characteristics such as the age, gender, and educational level of the consumer, as well as perceived crowding, stimulation, and the need for touch, on impulse purchasing. In summary, previous studies have found that all products can be purchased impulsively (Vohs and Faber, 2007), that situational factors affect and/or at least facilitate impulse purchasing behavior, and that various individual traits are closely linked to impulse buying. The recent introduction of new distribution channels such as home shopping channels, discount stores, and Internet stores that are open 24 hours a day increases the probability of impulse purchasing. However, previous literature has focused predominantly on situational and marketing variables and thus studies that consider critical consumer characteristics are still lacking. To fill this gap in the literature, the present study builds on this third tradition of research and focuses on individual trait variables, which have rarely been studied. More specifically, the current study investigates whether impulse buying tendency has a positive impact on impulse buying behavior, and evaluates how consumer characteristics such as the need for cognitive closure (NFCC), psychological wellbeing, and susceptibility to interpersonal influences affect the tendency of consumers towards impulse buying. The survey results reveal that while consumer affective impulsivity has a strong positive impact on impulse buying behavior, cognitive impulsivity has no impact on impulse buying behavior. Furthermore, affective impulse buying tendency is driven by sub-components of NFCC such as decisiveness and discomfort with ambiguity, psychological wellbeing constructs such as environmental control and purpose in life, and by normative and informational influences. In addition, cognitive impulse tendency is driven by sub-components of NFCC such as decisiveness, discomfort with ambiguity, and close-mindedness, and the psychological wellbeing constructs of environmental control, as well as normative and informational influences. The present study has significant theoretical implications. First, affective impulsivity has a strong impact on impulse purchase behavior. Previous studies based on affectivity and flow theories proposed that low to moderate levels of impulsivity are driven by reduced self-control or a failure of self-regulatory mechanisms. The present study confirms the above proposition. Second, the present study also contributes to the literature by confirming that impulse buying tendency can be viewed as a two-dimensional concept with both affective and cognitive dimensions, and illustrates that impulse purchase behavior is explained mainly by affective impulsivity, not by cognitive impulsivity. Third, the current study accommodates new constructs such as psychological wellbeing and NFCC as potential influencing factors in the research model, thereby contributing to the existing literature. Fourth, by incorporating multi-dimensional concepts such as psychological wellbeing and NFCC, more diverse aspects of consumer information processing can be evaluated. Fifth, the current study also extends the existing literature by confirming the two competing routes of normative and informational influences. Normative influence occurs when individuals conform to the expectations of others or to enhance his/her self-image. Whereas informational influence occurs when individuals search for information from knowledgeable others or making inferences based upon observations of the behavior of others. The present study shows that these two competing routes of social influence can be attributed to different sources of influence power. The current study also has many practical implications. First, it suggests that people with affective impulsivity may be primary targets to whom companies should pay closer attention. Cultivating a more amenable and mood-elevating shopping environment will appeal to this segment. Second, the present results demonstrate that NFCC is closely related to the cognitive dimension of impulsivity. These people are driven by careless thoughts, not by feelings or excitement. Rational advertising at the point of purchase will attract these customers. Third, people susceptible to normative influences are another potential target market. Retailers and manufacturers could appeal to this segment by advertising their products and/or services as products that can be used to identify with or conform to the expectations of others in the aspiration group. However, retailers should avoid targeting people susceptible to informational influences as a segment market. These people are engaged in an extensive information search relevant to their purchase, and therefore more elaborate, long-term rational advertising messages, which can be internalized into these consumers' thought processes, will appeal to this segment. The current findings should be interpreted with caution for several reasons. The study used a small convenience sample, and only investigated behavior in two dimensions. Accordingly, future studies should incorporate a sample with more diverse characteristics and measure different aspects of behavior. Future studies should also investigate personality traits closely related to affectivity theories. Trait variables such as sensory curiosity, interpersonal curiosity, and atmospheric responsiveness are interesting areas for future investigation.

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LINAC-based Stereotactic Radiosurgery for Meningiomas (수막종에 대한 선형가속기형 정위방사선수술)

  • Shin Seong Soo;Kim Dae Yong;Ahn Yong Chan;Lee Jung Il;Nam Do-Hyun;Lim Do Hoon;Huh Seung Jae;Yeo Inhwan J;Shin Hyung Jin;Park Kwan;Kim BoKyoung;Kim Jong Hyun
    • Radiation Oncology Journal
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    • v.19 no.2
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    • pp.87-94
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    • 2001
  • Purpose : To evaluate the role of LINAC-based stereotactic radiosurgery (SRS) in the management of meningiomas, we reviewed clinical response, image response, neurological deficits for patients treated at our institution. Methods and materials : Between February 1995 and December 1999, twenty-six patients were treated with SRS. Seven patients had undergone prior resection. Nineteen patients received SRS as the initial treatment. There were 7 male and 19 female patients. The median age was 51 years (range, $14\~67\;years$). At least one clinical symptom presented at the time of SRS in 17 patients and cranial neuropathy was seen in 7 patients. The median tumor volume was $4.7\;cm^3\;(range,\;0.7\~16.5\;m^3)$. The mean marginal dose was 15 Gy (range, $10\~20\;Gy$), delivered to the $80\%$ isodose surface (range, $46\~90\%$). The median clinical and imaging follow-up periods were 27 months (range, 1-71 months) and 25 months (range, $1\~52\;months$), respectively. Results : Of 14 patients who had clinical follow-up of one year or longer, thirteen patients $(93\%)$ were improved clinically at follow-up examination. Clinical symptom worsened in one patient at 4 months after SRS as a result of intratumoral edema, who underwent surgical resection at 7 months. OF 14 patients who had radiologic follow-up of one year or longer, tumor volume decreased in 7 patients $(50\%)$ at a median of 11 months (range, $6\~25\;months$), remained stable in 6 patients $(43\%)$, and increased in one patient $(7\%)$, who underwent surgical resection at 44 months. New radiation-induced neurological deficits developed in six patients $(23\%)$. Five patients $(19\%)$ had transient neurological deficits, completely resolved by conservative treatment including steroid therapy. Radiation-induced brain necrosis developed in one patient $(3.8\%)$ at 9 months after SRS who followed by surgical resection of tumor and necrotic tissue. Conclusions : LINAC-based SRS proves to be an effective and safe management strategy for small to moderate sized meningiomas, inoperable, residual, and recurrent, but long-term follow-up will be necessary to fully evaluate its efficacy. To reduce the radiation-induced neurological deficit for large size meningioma and/or in the proximity of critical and neural structure, more delicate treatment planning and optimal decision of radiation dose will be necessary.

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Incidence of Chronic Pathologic Nephrotoxicity of Cyclosporine A in Pediatric Nephrotic Syndrome (소아 신증후군에서 Cyclosporine A에 의한 만성 조직학적 신독성의 발현빈도에 대한 연구)

  • Kim Ji-Hong;Jeong Hyun-Ju;Choi In-Jun;Kim Pyung-Kil
    • Childhood Kidney Diseases
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    • v.3 no.2
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    • pp.130-144
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    • 1999
  • Purpose : Long-term use of Cyclosporine(CsA) reduce renal blood flow by afferent arteriolar vasoconstriction and lead to chronic pathologic changes of CsA nephrotoxicity - 1) interstitial nephritis(IN); tubular atrophy (TA) and/or interstitial fibrosis(IF),2) arteriolopathy(AP). The Object of this study is to estimate the incidence of chronic pathologic CsA nephrotoxicity by duration of treatment and type of renal disease, relationship between histologic and clinical nephrotoxicity, and optimal duration of CsA therapy. Methods : 102 children with steroid resistant or dependent nephrotic syndrome confirmed by renal biopsy and treated with CsA from 1986 to 1997 were enrolled in this study(58 MCNS, 10 FSGS, 10 MGN, 15 $Henoch-Sch\"{o}nlein$ purpura nephritis with nephrotic syndrome (HSPN) and 9 IgA nephropathy with nephrotic syndrome(IgAN)). CsA was administered for 1yr, 1.5yr, 2yr in 24, 12, 22 MCNS patients and 2, 2, 6 FSGS patients respectively, 1yr, 2yr in MGN and 1yr in HSPN and IgAN. Sequential biopsies were done in all 102 patients after CsA treatment for evaluation of pathologic nephrotoxicity. Results : Complete remission rate was 92.2% (100% in MCNS and MGN, 80% in FSGS, 86.6% in HSPN and 55.5% in IgAN). Incidence of relapse during 6months after CsA treatment was significantly decreased compaed with relapsing spisodes during 6months before CsA treatment in MCNS(P<0.0001) and FSGS(P<0.0001). According to pathologic changes, 71 patients(69.6%) showed no pathological change, 24 patients(23.5%) showed IN and 7 patients(6.8%) showed AP. IN was 16.6%, 33.3%, 27.2% in 1, 1.5, 2 year of CsA treatment group in MCNS. AP was 0%, 16.6%, 9% in 1, 1.5, 2 year of CsA treatment group in MCNS. 14 out of 58 MCNS(24.1%) showed IN and 4 out of 58 MCNS(6.8%) showed AP. Incidence of pathologic change was significantly lower in CsA therapy of <1yr than >1yr(P=0.03). There were no significant difference of incidence of pathologic change in original renal disease, age and sex. Conclusion : Duration of CsA treatment was significant risk factor for nephrotoxicity and optimal duration seemed to be 1 year. Pathologic change due to nephrotoxicity did not correlate with deterioration of renal function and only detectable by renal biopsy.

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Asymptomatic Primary Hematuria in Children (소아의 무증상성 일차성 혈뇨에 관한 고찰)

  • Lee, Jung-Mi;Park, Woo-Saeng;Ko, Cheol-Woo;Koo, Ja-Hoon;Kwak, Jung-Sik
    • Childhood Kidney Diseases
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    • v.4 no.1
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    • pp.25-32
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    • 2000
  • Purpose: This retrospective study of 126 children with symptomless primary hematuria was undertaken to determine the distribution of various histologic types by renal biopsy, clinical outcome according to the biopsy findings and also to find out feasibility of performing renal biopsy in these children. Patients and Methods : Study population consisted of 126 children with symptom-less primary hematuria who have been admitted to the pediatric department of Kyung-poot National University Hospital for the past 11 years from 1987 to 1998 and renal biopsy was performed percutaneously. Hematuric children with duration of less than 6 months, evidences of systemic illness such as SLE or Henoch-Schonlein purpura, urinary tract infection, and idiopathic hypercalciuria were excluded from the study. Results : Mean age of presentation was 9.2${\pm}$3.3 years (range ; 1.5-15.3 years) and male preponderance was noted with male to female ratio of 2:1. IgA nephropathy was the most common biopsy finding occuring in 60 children ($47.6\%$), followed by MsPGN in 13 ($10.3\%$), MPGN in 5 ($3.9\%$), TGBM in 6 ($4.7\%$), Alport syndrome in 2 ($1.6\%$), FSGS in 1 ($0.8\%$), and in 39 children ($30.9\%$), 'normal' glomeruli were noted. Recurrent gross hematuria was more common than persistent microscopic hematuria (84 versus 42), and especially in IgA nephropathy, recurrent gross hematuria was the most prevalent pattern of hematuria. In 58 out of 126 cases ($46.0\%$), hematuria was isolated without accompa-nying proteinuria and this was especially true In cases of MsPGN and 'normal' glomer-uli by biopsy finding. Normalization of urinalysis (disappearance of hematuria) in IgA nephropathy, MsPGN and 'normal' glomuli group were similar and it was $14\%,\;27\%\;and\;21\%$ respectively during 1-2 years of follow-up period, and $37.1\%,\;40\%\;and\;35\%$ respectively during 3-4 years of follow-up periods. However, abnormal urinalysis persi-sted in the majority of children with MPGN, TGBM. Alport syndrome and FSGS. Renal function deteriorated progressively in 6 cases (3 with IgA nephropathy, 2 with Alport syndrome and 1 with TGBM). Conclusion : In summary, present study demonstrates that in 126 children with symptomless primary hematuria, IgA nephropathy was the most common biopsy findings followed by MsPGN, MPGN, TGBM, Alport syndrome and FSGS, and 'normal glomeruli' was also seen in 39 cases ($30.9\%$). Renal histology could not be predictable on the clinical findings, so that to establish appropriate long-term planning for these children, we would recommend to obtain precise histologic diagnosis by renal biopsy.

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An Epidemiological Study on the Industrial Injuries among Metal Products Manufacturing Workers in Young-Dung-Po, Seoul (일부 금속 및 기계제품 제조업체 근로자들의 산업재해($1980{\sim}1981$)에 관한 조사)

  • Lee, Jung-Hee
    • Journal of Preventive Medicine and Public Health
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    • v.15 no.1
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    • pp.187-196
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    • 1982
  • The followings are the results of the study on industrial accidents occurred at 12 factories manufacturing metal products during the period of 2 years from January 1980 to December 1981 in the area of Yong-Dung-Po in Seoul. The results of the study are as follows: 1. The incidence rate of industrial injuries was 45.7 per 1,000 workers of the sample group and the rate of male (54.0) was three times higher than that of female (17.5). 2. In age groups, the highest rate was observed in the group of under 19 years old with 83.5, while the lowest in the group of 40s. 3. It was found that those who had short term of work experience produced a higher rate of injuries, particularly, the group of workers with less than 1 year of experience showed the highest rate of it as 48.1%. 4. In working time, the highest incidence rate occurred 3 and 7 hours after the beginning of their working showing the rate of 6.0 and 6.1 per 1,000 workers, respectively. 5. The highest incidence rate was observed on Monday as 8.4 per 1,000 workers, and it was 18.3% in aspect of the days of a week. 6. In aspect of the months of a year, the highest incidence was observed on July 1,000 workers and the next was on March as 4.8. These figures account for 11.8% of total occurrence in respective month. as 5. 4 per and 10.5% 7. In causes of injuries, the accident caused by power driven machinery showed the highest rate with 37.5%, the second was due to handling without machinery with 17.2%, and the third was due to falling objects with 14.2%, and striking against objects with 10.2%, and so on. 8. By parts of the body affected, the most injuries 84.3% of them occurred on both upper and lower extremities with the rate of 58.8% for the former and 25.5% for the latter. Fingers were most frequently injured with a rate of 40.3%. Comparing the sites of extremities affected, rate of injuries on the right side was 55.0% and 45.0% on the left side. 9. In the nature of injury, laceration and open wound were the highest with 34. 0%, the next was fracture and dislocation with 31. 9%, and sprain was the third with 8.1%. 10. On the duration of treatment, it lasted less than one month in 68.9% of the injured cases, of which 14.5% of the cases were recovered within 2 weeks, and 54.4% of them were treated more than 2 weeks. And the duration of the treatment tended to be prolonged in larger industries. 11. The ratio of insured accidents to uninsured accidents was 1 to 4.7.

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Community-based Helicobacter pylori Screening and its Effects on Eradication in Patients with Dyspepsia (지역사회에서 소화불량 환자의 Helicobacter pylori 감염에 대한 집단검진 및 치료효과)

  • Kim, Seong-Ho;Hong, Dae-Yong;Lee, Kyeong-Soo;Kim, Seok-Beom;Kim, Sang-Kyu;Suh, Jeong-Ill;Kim, Mee-Kyung;Kang, Pock-Soo
    • Journal of Preventive Medicine and Public Health
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    • v.33 no.3
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    • pp.285-298
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    • 2000
  • Objectives : To investigate the positive rate of Helicobacter pylori in patients with dyspepsia; medical compliance and related factors; the eradication rate a year after screening and related factors; the relationship between the eradication of Helicobacter pylori and the improvement of symptoms; and the estimated cost of three alternative approaches to treat Helicobacter pylori in the community. Methods : A total of 510 subjects with dyspeptic symptoms were selected and given the serological test in March 1998. The subjects were all adults over 30 years of age residing in Kyongju city. Results : Of the 510 selected subjects, 375 (73.5%) subjects proved positive for Helicobacter pylori on serological testing. Of these 304 (81.1%) who consented to an endoscopic examination, underwent a Campylobacter-like organism (CLO) test. Of these 304 subjects, 204 (67.1%), who had positive CLO test results, were given the triple therapy - tripotassium dicitrato bismuthate, amoxicillin, and metronidazole. To determine the eradication rate of Helicobacter pylori, 181 (88.1%) out of the 204 subjects who were given the triple therapy completed a follow-up urea breath test one year later. Of these, the Helicobacter pylori of 87(48.1%) subjects was eradicated. Among the 122 subjects who were medication compliant, the Helicobacter pylori eradication rate was 57.4% (70 subjects), while the eradication rates was only 28.8% (17subjects) in the non-compliant group. The Helicobacter pylori eradication was significantly related to compliance (p<0.01), but not to other characteristics and habits. The symptom improvement rate tended to be higher 62.1%), in the Helicobacter pylori eradicated group than in the non-eradicated group (59.6%). Conclusions : When the advantages and disadvantages of each alternative treatment were considered in the light of cost, antibiotic tolerance and the number of patients to be treated, alternative II was favorable in terms of cost. Alternative III was favorable in terms of the number of patients to be treated, antibiotic tolerance and early detection of gastric cancer. Further long-term research analyzing the cost-benefit and cost-effectiveness of each treatment will be needed as supporting material in creating new policies.

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Operative Treatment of Congenitally Corrected Transposition of the Great Arteries(CCTGA) (교정형 대혈관 전위증의 수술적 치료)

  • 이정렬;조광리;김용진;노준량;서결필
    • Journal of Chest Surgery
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    • v.32 no.7
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    • pp.621-627
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    • 1999
  • Background: Sixty five cases with congenitally corrected transposition of the great arteries (CCTGA) indicated for biventricular repair were operated on between 1984 and september 1998. Comparison between the results of the conventional(classic) connection(LV-PA) and the anatomic repair was done. Material and Method: Retrospective review was carried out based on the medical records of the patients. Operative procedures, complications and the long-term results accoding to the combining anomalies were analysed. Result: Mean age was 5.5$\pm$4.8 years(range, 2 months to 18years). Thirty nine were male and 26 were female. Situs solitus {S,L,L} was in 53 and situs inversus{I,D,D} in 12. There was no left ventricular outflow tract obstruction(LVOTO) in 13(20%) cases. The LVOTO was resulted from pulmonary stenosis(PS) in 26(40%)patients and from pulmonary atresia(PA) in 26(40%) patients. Twenty-five(38.5%) patients had tricuspid valve regurgitation(TR) greater than the mild degree that was present preoperatively. Twenty two patients previously underwent 24 systemic- pulmonary shunts previously. In the 13 patients without LVOTO, 7 simple closure of VSD or ASD, 3 tricuspid valve replacements(TVR), and 3 anatomic corrections(3 double switch operations: 1 Senning+ Rastelli, 1 Senning+REV-type, and 1 Senning+Arterial switch opera tion) were performed. As to the 26 patients with CCTGA+VSD or ASD+LVOTO(PS), 24 classic repairs and 2 double switch operations(1 Senning+Rastelli, 1 Mustard+REV-type) were done. In the 26 cases with CCTGA+VSD+LVOTO(PA), 19 classic repairs(18 Rastelli, 1 REV-type), and 7 double switch operations(7 Senning+Rastelli) were done. The degree of tricuspid regurgitation increased during the follow-up periods from 1.3$\pm$1.4 to 2.2$\pm$1.0 in the classic repair group(p<0.05), but not in the double switch group. Two patients had complete AV block preoperatively, and additional 7(10.8%) had newly developed complete AV block after the operation. Other complications were recurrent LVOTO(10), thromboembolism(4), persistent chest tube drainage over 2 weeks(4), chylothorax(3), bleeding(3), acute renal failure(2), and mediastinitis(2). Mean follow-up was 54$\pm$49 months(0-177 months). Thirteen patients died after the operation(operative mortality rate: 20.0%(13/65)), and there were 3 additional deaths during the follow up period(overall mortality: 24.6%(16/65)). The operative mortality in patients underwent anatomic repair was 33.3%(4/12). The actuarial survival rates at 1, 5, and 10 years were 75.0$\pm$5.6%, 75.0$\pm$5.6%, and 69.2$\pm$7.6%. Common causes of death were low cardiac output syndrome(8) and heart failure from TR(5). Conclusion: Although our study could not demonstrate the superiority of each classic or anatomic repair, we found that the anatomic repair has a merit of preventing the deterioration of tricuspid valve regurgitations. Meticulous selection of the patients and longer follow-up terms are mandatory to establish the selective advantages of both strategies.

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Early Results of Heart Transplantaion: A Review of 20 Patients (심장이식술 20례의 조기성적)

  • Park, Chong-Bin;Song, Hyun;Song, Meong-Gun;Kim, Jae-Joong;Lee, Jay-Won;Seo, Dong-Man;Sohn, Kwang-Hyun
    • Journal of Chest Surgery
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    • v.30 no.2
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    • pp.164-171
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    • 1997
  • Heart transplantation is now accepted as a definitive therapeutic modality in patients with terminal hear failure. The first successful heart transplantation in humans was done in 1967 and the first case in Korea was performed in november, 1992. Since the first case in 1992, more than 50 cases have been performed in Korea. A total of 20 patients underwent orthotopic heart transplantation since November, 1992 in Asan Medicla Center. The purpose of this study is to evaluate the early results and the follow-up course of 20 cases of heart transplantation done in Asan Medical Center. The average age of 20 patients was 39.9$\pm$11.8 years old(20~58). The mean follow-up duration was 14.4$\pm$11.2 months(1~41). All patients are alive till now. The blood type was identical in 14 and compatible in 6 patients. ihe original heart disease was dilated cardiomyopathy in 16, valvular heart disease in 2, ischemic cardiomyopathy in 1, and giant cell myocarditis in 1 patient. HLA cross matching for recipient and donor was done in 18 cases and the results were negative for T-cell and B-cell in 16 patients, pos tive for warm B-cell in 2 patients. Among 6 loci of A, B, and DR, one locus was matched in 8 cases, 2 loci in 5 cases, and 3 loci matched in 1 case. The number of acute allograft rejection averaged 2.8$\pm$0.5 (0~6) per case and the number of acute allograft rejection requiring treatment averaged 1.0$\pm$0.9 (1~3) per case. The time interval from operation to the first acute rejection requiring treatment was 35.5$\pm$20.4 days (5~60). Acute humoral rejection was suspected strongly in 1 case and was successfully treated. The left ventricular ejection fraction measured by echocardiography and/or MUGA scan was dramatically increased from 17.5$\pm$6.8 (9~32)% to 58.9$\pm$2.0 (55~62)% after heart transplantation. Temporary pacing was needed in 5 patients over 24 hours but normal sinus rhythm appeared within 7 days in all cases. One patient has been taken permanent pacemaker implantation due to complete AV block appearing 140 days after heart transplantaion. One patient had cyclosporine-associated n urotoxicity during the immediate postoperative period and was recovered after 27 hours. The heart transplantation of Asan Medical Center is on a developing stage but the early result is comparable to that of well established centers in other countries, even though the long-term follow-up result must be reevaluated. We can conclude that the heart transplantion is a promising therapeutic option in patients with terminal heart failure.

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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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The Significance of Hyperlipidemia as a Predictive Factor of Relapse in Corticosensitive Nephrotic Syndrome (스테로이드에 반응을 보인 신증후군 환아에서 재발 예측인자로서 고지혈증의 중요성)

  • Jung, Soon-Pil;Hong, Soon-Cheul;Lim, Seong-Joon;Lim, In-Seok;Choi, Eung-Sang
    • Childhood Kidney Diseases
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    • v.5 no.2
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    • pp.136-146
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    • 2001
  • Purpose : One of the most difficult problems in the care of children with nephrotic syndrome remains the occurrence of relapses, despite initial response to steroids. Constantinescu reported that rapidity of initial response to steroid therapy could predict fewer relapses in the first year. So we evaluated the changes in serum lipid abnormalities in children with corticosensitive nephrotic syndrome before steroid treatment and the correlation between serum lipid levels and renal function, days to remission. Methods . We analyzed the Medical records of children who were managed by us between October 1994 and August 2000. In 33 patients with corticosensitive nephrotic syndrome, we evaluated the correlation between serum lipid levels and renal function [Creatinine clearance(Ccr)] and proteinuria before steroid treatment, and days to remission defined as the third day when the patient's urine becomes protein free. Results : There were 21 males and 12 females. Median age at presentation was 6.4 years (range: 1.8-17.3 years). Median days to remission were 15.4 days (range 4-42 days) on Prednisolone $60mg/m^2$ daily. The increased levels of triglyceride, total cholesterol, LDL cholesterol, apolipoprotein B, total cholesterol/HDL cholesterol, Lipoprotein(a) were observed. But the level of HDL cholesterol was not increased. Serum albumin was decreased a]id proteinuria was increased before steroid treatment. But Ccr was not decreased. There were negative correlation between serum albumin and total cholesterol (r = -0.5157, P<0.005), LDL cholesterol (r = -0.5543, P<0.005), total cholesterol/HDL cholesterol (r = -0.4506, P<0.01), lipoprotein(a) (r = -0.4570, P<0.025), apolipoprotein B (r = -0.5297, P<0.025), apolipoprotein B/apolipoprotein Al (r = -0.5851, P<0.01), apolipoprotein B/HDL cholesterol (r = -0.4961, P<0.05) before steroid treatment. There was no correlation between proteinuria and serum lipid profiles. Also Ccr and serum lipid profiles were not correlated. There was positive correlation between days to remission and HDL cholesterol (r = +0.4511, P<0.05), apolipoprotein B (r = +0.5190, P<0.05), apolipoprotein B/HDL cholesterol (r = +0.7169, P<0.005). Conclusions : This results reveal that HDL cholesterol, apolipoprotein B and apolipoprotein B/HDL cholesterol can be used as a predictive factor in corticosensitive nephrotic syndrome. We could not determine the significant level of these lipids for insufficient patients number, but these level may predict future relapses of corticosensitive nephrotic syndrome patients and thus may allow to better management and treatment protocols. More data and long term follow up studies should be needed. (J Korean Soc Pediatr Nephrol 2001;5 : 136-46)

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