• Tuberculosis and Respiratory Diseases
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• v.41 no.4
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• pp.339-353
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• 1994

Background : The p53 gene codes for a DNA-binding nuclear phosphoprotein that appears to inhibit the progression of cells from the G1 to the S phase of the cell cycle. Mutations of the p53 gene are common in a wide variety of human cancers, including lung cancer. In lung cancers, point mutations of the p53 gene have been found in all histological types including approximately 45% of resected NSCLC and even more frequently in SCLC specimens. Mutant forms of the p53 protein have transforming activity and interfere with the cell-cycle regulatory function of the wild-type protein. The majority of p53 gene mutations produce proteins with altered conformation and prolonged half life; these mutant proteins accumulate in the cell nucleus and can be detected by immunohistochemical staining. But protein overexpression has been reported in the absence of mutation. p53 protein overexpression or gene mutation is reported poor prognostic factor in breast cancer, but in lung cancer, its prognostic significance is controversial. Method : We investigated the p53 abnormalities by nucleotide sequencing, polymerase chain reaction-single strand conformation polymorphism(PCR-SSCP), and immunohistochemical staining. We correlated these results with each other and survival in 75 patients with NSCLC resected with curative intent. Overexpression of the p53 protein was studied immunohistochemically in archival paraffin- embedded tumor samples using the D07(Novocastra, U.K.) antibody. Overexpression of p53 protein was defined by the nuclear staining of greater than 25% immunopositive cells in tumors. Detection of p53 gene mutation was done by PCR-SSCP and nucleotide sequencing from the exon 5-9 of p53 gene. Result: 1) Of the 75 patients, 36%(27/75) showed p53 overexpression by immunohistochemical stain. There was no survival difference between positive and negative p53 immunostaining(overall median survival of 26 months, disease free median survival of 13 months in both groups). 2) By PCR-SSCP, 27.6%(16/58) of the patients showed mobility shift. There was no significant difference in survival according to mobility shift(overall median survival of 27 in patients without mobility shift vs 20 months in patients with mobility shift, disease free median survival of 8 months vs 10 months respectively). 3) Nucleotide sequence was analysed from 29 patients, and 34.5%(10/29) had mutant p53 sequence. Patients with the presence of gene mutations showed tendency to shortened survival compared with the patients with no mutation(overall median survival of 22 vs 27 months, disease free median survival of 10 vs 20 months), but there was no statistical significance. 4) The sensitivity and specificity of immunostain based on PCR-SSCP was 67.0%, 74.0%, and that of the PCR-SSCP based on the nucleotide sequencing was 91.8%, 96.2% respectively. The concordance rate between the immunostain and PCR-SSCP was 62.5%, and the rate between the PCR-SSCP and nucleotide sequencing was 95.3%. Conclusion : In terms of detection of p53 gene mutation, PCR-SSCP was superior to immunostaining. p53 gene abnormalities either overexpression or mutation were not a significant prognostic factor in NSCLC patients resected with curative intent. However, patients with the mutated p53 gene showed the trends of early relapse.

• Korean small business review
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• v.32 no.2
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• pp.209-223
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• 2010

Korea's business is composed of a few large-sized enterprises (which can be abbreviated as LSE) and a majority of small-sized enterprises (SSE). Although there has been a growing recognition of the need for the development of medium-sized enterprises (MSE) which can serve as a link between SSE and LSE, as yet there has not yet been a consensus on the definition, characteristics and the function of the MSE in Korea. Nowadays, the world is being globalized, and Japan and China are in competition to ne a great economic power. While East Asia is experiencing rapid changes, promoting MSE which can secure flexibility and efficiency through covering up the limitation of LSE and SSE is needed in order to respond the global market which is being specialized. The features of MSE in Japan can be listed as follows. First, the MSE in Japan is developing the company through getting into niche markets which are hard for major companies to enter rather than developing markets in order to compete against major companies directly. While MSEs are endeavoring to build the business firmly in the domestic market, they can possess special and competitive technical skills through trials and errors; so that they can get a chance develop their business through independent business system rather than putting their effort to compete against major companies. Second, from the MSEs with competitive edge in the market, there are many contributions to the national exportation. Those MSEs produce in domestic and maintain the quality of high price products which need cutting-edge technology, while they relocate the low and middle priced goods to the country where manufacturing costs are low, so that they can maintain the price competitiveness. Third, the industrial structure in Japan is formed from dual structure between major companies and small sized companies. In other words, in Japan's industrial structure which are composed of subcontract structure, this dual structure has taken a major role of small sized companies' growth and manufacturing businesses' international competitive power. Forth, MSE in Japan adopt a strategy of putting their value on qualitative scale growth rather than quantitative scale growth. In this paper, the case of Japanese MSE is analyzed. Along with its long history of Industrialization, Japan has a corporate environment where the SSEs can develop as a MSE and later a LSE through a full-support system. Among its SSEs, there are a number of world class corporations equipped with a large domestic market, win-win cooperation with the LSEs and an independent technology development. It can also be observed that these SSEs develop into MSEs with sustainable growth potentials. This study will focus on the condition under which the MSEs of Japan have been developed, and how they have survived the competition between SSEs and LSEs. Through this study, this paper attempts to offer solutions to Korea's polarization between the SSE and LSE, while providing the basis for SSEs revitalization. In general, if both extremities phenomenon deepen between LSE and SSE, there are possible fears of occurring disutility in national economy by the monopolization of LSE. For that reason, enterprise group, which can make SSE or MSE compete LSE in some area and ease the monopoly and oligopoly problem, is needed. This awareness has been shared for ages long. Nevertheless, there is no legal definition for MSE in Japan, and there is no definition about the enterprise size or unified view of MSE between scholars, but it is defined differently by each of academical person or research institution and study meeting. For that reason, this paper will organize the definition of MSE in Japan, and then will propose the characteristics of the background which has made MSE secure competitiveness and sustainable growth in global market. This study focus on that because through this process, the positive change to the awareness of MSE can be proposed in Korea and to seek the policy direction for building institutional framework which can make SSE become MES. Through this way, the fundamentals for SSE to become MSE can be managed and some appropriate suggestions which will be able to make MSE enter the global market in the future can also be proposed. Due to these facts, this study is very important and well timed task. In a sense of this way, this study will examine the definition and role of MSE in Japan. after this examination, this study will deal with the status, special feature, and promotion policy for MSE. Through this analysis of MSE in Japan, the foundation which be able to set the desirable role model for MSE in Korea can be proposed. Also, the political implication which is needed to push ahead to contribute to creating employment and economic growth through sustainable growth of MSEs in economic system of Korea can be offered through this study. It has been found that Japan's MSE functions as an indispensable link among various industrial structures by holding a significant position in employment rate, production and value added. Although the MSEs took up less than 1% of the entire number of businesses with 2700 manufacturing firms and 7000 non-manufacturing firms, its employment ratios are about 15%, while taking about 25% of the manufacturing industry's exports. In industries such as machinery and electronics which is considered Japan's major industry, the MSEs showed a higher than average ratio of manufacturing exports and employment rate. It can be analyzed that behind Japan's advantageous industries, close and deeply knit MSEs exist. Although there are no clearly stated policies geared towards the MSEs by the Japanese government, various political measures exist such as the R&D Project and the inducement of cooperation between enterprises which gives room for MSEs to participate in the SSE policies. In relation to these findings, the following practical measures can be considered in order to revitalize Korea's MSEs: First, there is a need for a legal definition of MSE and the incentives to provide legal support for its growth. Second, if a law to support the MSEs is established, it could provide a powerful inducement for the SSE to grow as a MSE, rather than stay as a SSE. Third, there is a need for a strategy of MSEs to establish a stable base in the domestic market and then advance to the global market with the accumulated trial and error and competitiveness. Fourth, the SSE themselves need the spirit of entrepreneurship in order to make the leap to a MSE. Because if nothing is to be changed about the system on the firms that grew, and the parts of the past custom was left to be managed alone, confusion and absence of management can take place. No matter how much tax favors the government will give and no matter how much incentive there could be through the policies, there are limits for industries to higher the ability to propagate. And because of that it is a period where industries need their own innovative skills to reform their firms.

• Radiation Oncology Journal
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• v.19 no.2
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• pp.118-126
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• 2001

Purpose : In this study, it was investigated whether dose response relation existed or not in local radiotherapy for primary hepatocellular carcinoma. Materials and Methods : From January 1992 to March 2000, 158 patients were included in present study. Exclusion criteria included the presence of extrahepatic metastasis, liver cirrhosis of Child's class C, tumors occupying more than two thirds of the entire liver, and performance status on the ECOG scale of more than 3. Radiotherapy was given to the field including tumor with generous margin using 6, 10-MV X-ray. Mean tumor dose was $48.2{\pm}7.9\;Gy$ in daily 1.8 Gy fractions. Tumor response was based on diagnostic radiologic examinations such as CT scan, MR imaging, hepatic artery angiography at $4\~8$ weeks following completion of treatment. Statistical analysis was done to investigate the existence of dose response relationship of local radiotherapy when it was applied to the treatment of primary hepatocellular carcinoma. Results : An objective response was observed in 106 of 158 patients, giving a response rate of $67.1\%$. Statistical analysis revealed that total dose was the most significant factor in relation to tumor response when local radiotherapy was applied to the treatment of primary hepatocellular carcinoma. Only $29.2\%$ showed objective response in patients treated with dose less than 40 Gy, while $68.6\%\;and\;77.1\%$ showed major response in patients with $40\~50\;Gy$ and more than 50 Gy, respectively. Child-Pugh classification was significant factor in the development of ascites, overt radiation induced liver disease and gastroenteritis. Radiation dose was an important factor for development of radiation induced gastroduodenal ulcer. Conclusion : Present study showed the existence of dose response relationship in local radiotherapy for primary hepatocellular carcinoma. Only radiotherapy dose was a significant factor to predict the objective response. Further study is required to predict the maximal tolerance dose in consideration of liver function and non-irradiated liver volume.

• Journal of the Korean Geographical Society
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• v.30 no.3
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• pp.269-295
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• 1995

This research is concerned with the social division of labor within the traditional industry district: Damyang bamboo ware industry district and Yeoju pottery industry district in South Korea, Damyang bamboo ware and Yeoju pottery are well known of the Korean traditional industry. The social division of labor in an industry district is considered as an important factor. The social division of labor helps the traditional industry to survive today. This summary shows five significant points from the major findings. First, Damyang bamoo ware industry and Yoeju pottery industry have experienced the growth stages until 1945, the stagnation in the 1960s, and the business recovery in the 1980s. Most Korean traditional industries had been radically declined under the Japanese colonization; while, Damyang bamboo ware industry and Yeoju pottery industry district have been developed during above all stages. The extended market to Japan helped the local government to establish a training center, and to provide financial aids and technical aids to crafts men. During the 1960s and 1970s, mass production of substitute goods on factory system resulted in the decrease of demand of bamboo ware and pettery. During the 1980s, these industries have slowly recovered as a result of the increased income per capita. The high rate of economic growth in the 1960s and 1970s was playing an important role in the emerging the incleased demand of the bamboo ware and pottery. Second the production-and-marketing system in a traditional industry district became diversified to adjust the demand of products. In Damyang bamboo ware industry district, the level of social division of labor was low until the high economic development period. Bamboo ware were made by a farmer in a small domestic system, The bamboo goods were mainly sold in the periodic market of bamboo ware in Damyang. In the recession period in the 1960s and 1970s, the production-and-marketing system were diversified; a manufacturing-wholesale type business and small-factory type business became established; and the wholesale business and the export traders in the district appeared. In the recovery period in the 1980s, the production-and-marketing systems were more diversified; a small-factory type business started to depend On subcontractors for a part of process of production; and a wholesale business in the district engaged in production of bamboo ware. In Yeoju pottery industry district, the social division of labor was limited until the early 1970s. A pottery was made by a crafts man in a small-business of domestic system and sold by a middle man out of Yeoju. Since the late 1970s, production-and-marketing system become being diversified as a result of the increased demand in Japan and South Korea. In the 1970s, Korean traditional craft pottery was highiy demanded in Japan. The demand encouraged people in Yoeju to become craftsmen and/or to work in the pottery related occupation. In South Korea, the rapid economic growth resulted in incline to pottery due to the development of stainless and plastic bowls and dishes. The production facilities were modernized to provide pottery at the reasonable price. A small-busineas of domestic system was transformed into a small-factory type business. The social division of labor was intensified in the pottery production-and-maketing system. The manufacturing kaoline began to be seperated from the production process of pottery. Within the district, a pottery wholesale business and a retail business started to be established in the 1980s. Third the traditional industry district was divided into "completed one" and "not-completed one" according to whether or not the district firms led the function of the social division of labor. The Damyang bamboo ware industry district is "completed one": the firm within the district is in charge of the supply of raw material, the production and the marketing. In the Damyang bamboo ware district, the social division of labor w and reorganized labor system to improve the external economics effect through intensifying the social division of labor. Lastly, the social division of labor was playing an important role in the development of traditional industry districts. The subdivision of production process and the diversification of business reduced the production cost and overcame the labor shortage through hiring low-waged workers such as family members, the old people and housewives. An enterpriser with small amount of capital easily joined into the business. The risk from business recession were dispersed. The accumulated know-how in the production and maketing provided flexiblility to produce various goods and to extend the life-cycly of a product.d the life-cycly of a product.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.2
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• pp.399-415
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• 1999

From bacteria to mammalian cells, one of the most important mediators of intracellular signal transduction mechanisms which regulate a variety of intracellular processes is free calcium. In salivary acinar cells, elevation of intracellular calcium concentration ($[Ca^{2+}]_i$) is essential for the salivary secretion induced by parasympathetic stimulation. However, in addition to $[Ca^{2+}]_i$, gap junctions which couple individual cells electrically and chemically have also been reported to regulate enzyme secretion in pancreatic acinar cells. Since the plasma membrane of salivary acinar cells has a high density of gap junctions, and these cells are electrically and chemically coupled with each other, gap junctions may modulate the secretory function of salivary glands. In this respect, I planned to investigate the role of gap junctions in the modulation of salivary secretion and $[Ca^{2+}]_i$, using mandibular salivary glands of rats. In order to measure the salivary flow rate, fluid was collected from the cannulated duct of the isolated perfused rat mandibular glands at 2 min intervals. $[Ca^{2+}]_i$, was measured from the cells loaded with fura-2 by spectrofluorometry. The results obtained were as follows: 1. CCh-induced salivary secretion was reversibly inhibited by 1 mM octanol, a gap junction blocker. 2. CCh-induced increase in $[Ca^{2+}]_i$, was also reversed by the application of 1 mM octanol. 3. Octanol did not block the initial increase in $[Ca^{2+}]_i$ caused by CCh, which suggested that the reduction of $[Ca^{2+}]_i$, caused by gap junction blockade was not resulted from the inhibition of $Ca^{2+}$ release from intracellular $Ca^{2+}$ stores. 4. Addition of octanol during stimulation with $1{\mu}M$ thapsigargin, a potent microsomal ATPase inhibitor, reduced $[Ca^{2+}]_i$, to the basal level. This suggested that inhibition of gap junction permeability closed plasma membrane $Ca^{2+}$ channels. 5. 2,5-di-tert-butyl-1,4-benzohydroquinone (TBQ) generated $[Ca^{2+}]_i$ oscillations resulting from periodic influx of $Ca^{2+}$ via plasma membrane. The TBQ-induced $[Ca^{2+}]_i$ oscillations were stopped by the application of 1mM octanol which implicated that gap junctions modulate the permeability of plasma membrane $Ca^{2+}$ channels. 6. Glycyrrhetinic acid, another well known gap junction blocker, also inhibited CCh-induced salivary secretion from rat mandibular glands. These results suggested that gap junctions play an important role in the modulation of fluid secretion from the rat mandibular glands and this was probably due to the inhibition of $Ca^{2+}$ influx through the plasma membrane $Ca^{2+}$ channels.

• The Korean Journal of Nuclear Medicine Technology
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• v.14 no.2
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• pp.203-207
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• 2010

Purpose: Hepatitis B is infection caused by Hepatitis B virus (HBV). Currently, there are several methods, Kits and equipments for conducting Hepatitis B test. Due to ununiformed methods, it would cause some differences. To manage these differences, it needs process evaluating function of test system and reagent using particular standard substance. The aim of this study is to investigate tendency of RIA method's reagent used in Asan Medical Center through comparing several other test reagents using national standard substance. Materials and Methods: The standard substance in National Institute of Food and Drug Safety Evaluation's biology medicine consists of 5 things, 4 antigens and 1 antibody. We tested reagents using A, B company's Kits according to each test method. All tests are measured repeatedly to obtain accurate results. Results: Test result of "HBs Ag Mixed titer Performance panel" is obtained match rate compared S/CO unit standard with RIA method and EIA 3 reagents, CIA 2 reagents is that company A's reagent is 94.4% (17/18), 83.3% (15/18), B is 88.9% (16/18), 77.8% (14/18). Test result of "HBs Ag Low titer Performance panel" is obtain that EIA 2 reagents is shown 7 posive results, CIA 3 reagents is 11, and RIA method's company A's reagent is 3, B is 2 of 13 in low panel. "HBV surface antigen 86.76 IU/vial" tested dilution. A is obtain positive results to 600 times(0.14 IU/mL), B is 300 times (0.29 IU/mL). Case of "HBV human immunoglobulin 95.45 IU/vial", A is shown positive result to 10,000 times (9.5 mIU/mL) and B is 4,000 times (24 mIU/mL). Test result of "HBs Ag Working Standards 0.02~11.52 IU/mL" is shown that Company A's kit concentration level was 0.38IU/mL, company B was 2.23 IU/mL and higher level of concentration was positive results. Conclusion: When comparing various test reagents and RIA method according to National Standard substances for Hepatitis B test, we recognized that there were no significant trends between reagents. For hepatitis B virus antigen-antibody titers even in parts of the test up to 600 times the antigen, antibodies to 10,000 times the maximum positive results could be obtained. Therefore, we confirmed that results from Asan Medical Center are performed smoothly by reagents and system for hepatitis B virus test.

• 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.

• The Korean Journal of Nuclear Medicine
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• v.4 no.2
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• pp.11-27
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• 1970

Cardiac output, plasma volume and renal plasma flow were determined to evaluate hemodynamic changes in 29 patients with cirrhosis of the liver. The results obtained were as follows. 1. The mean plasma volume was 3793+895ml and it was significantly higher than the normal controls. The mean blood volume ($5266{\pm}1222ml$) and blood volume per kg body weight ($95.7{\pm}23.41ml$) were also increased significantly. The mean plasma volume per kg body weight ($69.1{\pm}19.1ml$) showed increased tendency and the mean difference between blood volume and plasma volume per kg body weight ($26.4{\pm}7.05ml$) was in lower limit of normal range. 2. The mean cardiac output was $7708{\pm}2652ml/min$ and it was significantly increased. The mean cardiac index ($4924{\pm}1998ml/min/M^2$), stroke volume ($96.2{\pm}34.2ml/beat$), stroke index ($62.3{\pm}27.34ml/M^2$) and fractional cardiac index ($1.54{\pm}0.577$) were also increased significantly. The mean total -peripheral resistance was $1664{\pm}753.8\;dynes\;sec\;cm^{-5}M^2$ and it was significantly lower than the normal controls. 3. The mean renal plasma flow was $537{\pm}146.8ml/min/1.73M^2$ and it was normal to decreased tendency. The mean endogenous creatinine clearance ($66.7{\pm}23.0ml/min/1.73M^2$) was significantly decreased. Filtration fraction was variable, but it was slightly lower than normal in most cases. The mean renal fraction of cardiac output ($11.4{\pm}6.27%$) was relatively decreased. 4. Although renal plasma flow was normal or decreased in general, it was definitely diminished in patients with creatinine clearance less than $60ml/min/1.73M^2$, resistant ascites, and signs of azotemia (elevated BUN and serum creatinine). 5. Diminished glomrular filtration rate with low filtration fraction and decreased renal fraction of cardiac output observed strongly supported increased renal afferent arteriolar resistance. 6. Renal circulatory impairment preceded azotemia or oroliguria in cirrhosis. 7. Clinical findigns and liver function were not correlated with hemodynamic changes, except for esophageal varices associated with high cardiac output obsedved. 8. No definite correlation of renal hemodynamics with plasma volume or cardiac output was found.

• Journal of Chest Surgery
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• v.40 no.12
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• pp.817-824
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• 2007

Background: There are two choices for heart valve replacement-the use of a tissue valve and the use of a mechanical valve. Using a tissue valve, additional surgery will be problematic due to valve degeneration. If the risk of additional surgery could be reduced, the tissue valve could be more widely used. Therefore, we analyzed the risk factors and mortality of patients undergoing repeated heart valve replacement and primary replacement. Material and Method: We analyzed 25 consecutive patients who underwent repeated heart valve replacement and 158 patients who underwent primary heart valve replacement among 239 patients that underwent heart vale replacement in out hospital from January 1995 to December 2004. Result: There were no differences in age, sex, and preoperative ejection fraction between the repeated valve replacement group of patients and the primary valve replacement group of patients. In the repeated valve replacement group, the previously used artificial valves were 3 mechanical valves and 23 tissue valves. One of these cases had simultaneous replacement of the tricuspid and aortic valve with tissue valves. The mean duration after a previous operation was 92 months for the use of a mechanical valve and 160 months for the use of a tissue valve. The mean cardiopulmonary bypass time and aortic cross clamp time were 152 minutes and 108 minutes, respectively, for the repeated valve replacement group of patients and 130 minutes and 89 minutes, respectively, for the primary valve replacement group of patients. These results were statistically significant. The use of an intra aortic balloon pump (IABP) was required for 2 cases (8%) in the repeated valve replacement group of patients and 6 cases (3.8%) in the primary valve replacement group of patients. An operative death occurred in one case (4%) in the repeated valve replacement group of patients and occurred in nine cases (5.1%) in the primary valve replacement group of patients. Among postoperative complications, the need for mechanical ventilation over 48 hours was different between the two groups. The mean follow up period after surgery was $6.5{\pm}3.2$ years. The 5-year survival of patients in the repeated valve replacement group was 74% and the 5-year survival of patients in the primary valve replacement group was 95%. Conclusion: The risk was slightly increased, but there was little difference in mortality between the repeated and primary heart valve replacement group of patients. Therefore, it is necessary to reconsider the issue of avoiding the use of a tissue valve due to the risk of additional surgery, and it is encouraged to use the tissue valve selectively, which has several advantages over the use of a mechanical valve. In the case of a repeated replacement, however, the mortality rate was high for a patient whose preoperative status was not poor. A proper as sessment of cardiac function and patient status is required after the primary valve replacement. Subsequently, a secondary replacement could then be considered.

• 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)