• Title/Summary/Keyword: Failure analysis

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Primary Orbital Lymphoma : A Retrospective Analysis of Results of Radiation Therapy (원발성 안와 림프종의 방사선치료 성적에 관한 후향적 분석)

  • Kim Sussan;Ahn Seung Do;Chang Hyesook;Kim Kyoung Ju;Lee Sang-wook;Choi Eun Kyung;Kim Jong Hoon;Huh Jooryung;Suh Cheol Won;Kim Sung Bae
    • Radiation Oncology Journal
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    • v.20 no.2
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    • pp.139-146
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    • 2002
  • Purpose : This study evaluated the treatment outcomes, patterns of failure, and treatment related complications of primary lymphoma patients who received definitive radiation therapy. Materials and Methods : A retrospective analysis was undertaken for 31 patients with primary orbital lymphoma at the Asan Medical Center between February 1991 and April 2001. There were 18 males and 13 females with ages ranging from 3 to 73 years (median, 44 years). The involved sites were 9 conjunctivae, 12 eyelids and 10 other orbits. The histological types were 28 MALT lymphomas (low-grade B-cell lymphoma of mucosa-associated lymphoid tissue type), 1 diffuse large B-cell lymphoma, 1 anaplastic large cell lymphoma and 1 lymphoblastic lymphoma. The Ann Arbor stages were all IE $(100\%)$. Ann Arbor stage III or IV patients were excluded from this study, Bilateral orbital involvement occurred in 6 cases. Radiation therapy was given with one anterior port of high energy electrons $(6\~16\;MeV)$ for the lesions located at the anterior structures like the conjunctivae or eyelids. Lesions with a posterior extension or other orbital lesions were treated with 4 or 6 MeV photons with appropriately arranged portals. In particular, lens blocks composed of lead alloy were used in conjunctival or eyelid lesions. Twelve patients received chemotherapy. The median follow-up period was 53 months. Results : The 5-year overall, cause-specific, and disease-free survival was $91\%,\;96\%,\;and\;80\%$, respectively. The complete response rate 6 months after radiation therapy was $100\%$. Local recurrences were observed in 2 patients at 16 and 18 months after completion of radiation treatment. They were salvaged with additional radiation therapy. Two patients developed distant metastases. A MALT lymphoma patient with a lung relapse was successfully salvaged with radiotherapy, but the other lymphoblastic lymphoma patient with bone marrow relapse expired. There were no severe complications but 5 patients developed radiation-induced cataracts and 2 patients developed dry eye. Conclusion : Most primary orbital lymphomas consisted of MALT lymphomas. Radiation therapy was a successful treatment modality for orbital lymphoma without any severe complications. In cases of local relapses, radiation therapy is also a very successful salvage treatment modality.

The Etiologies and Initial Antimicrobial Therapy Outcomes in One Tertiary Hospital ICU-admitted Patient with Severe Community-acquired Pneumonia (국내 한 3차 병원 중환자실에 입원한 중증지역획득폐렴 환자의 원인 미생물과 경험적 항균제 치료 성적의 고찰)

  • Lee, Jae Seung;Chung, Joo Won;Koh, Yunsuck;Lim, Chae-Man;Jung, Young Joo;Oh, Youn Mok;Shim, Tae Sun;Lee, Sang Do;Kim, Woo Sung;Kim, Dong-Soon;Kim, Won Dong;Hong, Sang-Bum
    • Tuberculosis and Respiratory Diseases
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    • v.59 no.5
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    • pp.522-529
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    • 2005
  • Background : Several national societies have published guidelines for empirical antimicrobial therapy in patients with severe community-acquired pneumonia (SCAP). This study investigated the etiologies of SCAP in the Asan Medical Center and assessed the relationship between the initial empirical antimicrobial regimen and 30 day mortality rate. Method : retrospective analysis was performed on patients with SCAP admitted to the ICU between March 2002 and February 2004 in the Asan Medical Center. The basic demographic data, bacteriologic study results and initial antimicrobial regimen were examined for all patients. The clinical outcomes including the ICU length of stay, the ICU mortality rate, and 30 days mortality rates were assessed by the initial antimicrobial regimen. Results : One hundred sixteen consecutive patients were admitted to the ICU (mean age 66.5 years, 81.9 % male, 30 days mortality 28.4 %). The microbiologic diagnosis was established in 58 patients (50 %). The most common pathogens were S. pneumoniae (n=12), P. aeruginosae (n=9), K. pneumonia (n=9) and S. aureus (n=8). The initial empirical antimicrobial regimens were classified as: ${\beta}$-lactam plus macrolide; ${\beta}$-lactam plus fluoroquinolone; anti-Pseudomonal ${\beta}$-lactam plus fluoroquinolone; Aminoglycoside combination regimen; ${\beta}$-lactam plus clindamycin; and ${\beta}$-lactam alone. There were no statistical significant differences in the 30-day mortality rate according to the initial antimicrobial regimen (p = 0.682). Multivariate analysis revealed that acute renal failure, acute respiratory distress syndrome and K. pneumonae were independent risk factors related to the 30 day mortality rate. Conclusion : S. pneumoniae, P. aeruginosae, K. pneumonia and S. aureus were the most common causative pathogens in patients with SCAP and K. pneumoniae was an independent risk factor for 30 day mortality. The initial antimicrobial regimen was not associated with the 30-day mortality.

Factors Related with the Compliance and Treatment in Patients with Pulmonary Tuberculosis in Urban and Suburb Area (도시와 농촌지역의 폐결핵 환자 순응도 및 치료에 관련된 요인)

  • Kim, Sang-Soon;Kim, Yoon-Ock
    • Research in Community and Public Health Nursing
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    • v.7 no.1
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    • pp.69-79
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    • 1996
  • To investigate the factors related with the compliance and the treatment of the patients with pulmonary tuberculosis in urban and suburb area, we followed up all the 755 registered patients(at urban Public Health Office 544, at suburb 210) as follow from January 1,1992 to December 31, 1993. We describe the general characteristics and the characteristics related with the disease of the patients according to the area as follow. 150 patients(27.5%) were at their age of 20 to 29 years in the urban area, whereas 45 patients (21.4%) were 60 to 69 years and another 45 patients(21.4%) were 70 to 79 years in the suburb area. According to the first chest X -ray examination, 54.5% of all cases were proved to be mild in the urban area. But in the suburb area, moderate cases (44.3%) were more than mild cases(p<0.01). Follow-up X-ray's were performed more properly(p<0.05) in the urban area(94.3%) than in the suburb area(90.0%). Most cases were found in the chest X -ray examination performed by Public Health Office (p<0.01) : payable chest X-ray in the urban area (56.7%) and free chest X-ray in the suburb area(35.2%). More patients were cured in the urban area(90.8%) than in the suburb area(87.1%). The presence of supporting family member were significantly higher(p<0.05) in the urban area(79.1%) than in the suburb area(88.1%). In the analysis of the treatment efficacy, more cure ate were found in the patients cytologically confirmed to be culture (+). In the urban area, 201 culture (+) patients (93.5%) 294 culture (-) patients (89.1%) were cured. In the suburb area, 99 culture (+) patients(91.7%) and 84 culture (-) patients(82.4%) were cured. Age, the presence of supporting family member, and the socioeconomic status of the patient had significant association with the prescription compliance related with the general characteristics of the patients. Whereas, X-ray finding and AFB culture finding were the significant factors associated with the prescription compliance related with pulmonary tuberculosis (p<0.05). The cumulative compliance in the survival analysis was 92.5% in the urban area and 88.1% in suburb area, at sixth month of follow-up. Failure rate for regular drug receipt was highest at second month in the urban area(3.75%) and at fourth month in the suburb area(4.15%). In logistic regression of the factors related with the tratment result, first X-ray examination and prescription compliance were significantly associated in the urban area(p<0.05). However, there is no factor significantly associated with the treatment result in the suburb area. It could be explained by too small size of the sample. In logistic regression of the factors related with the prescription compliance, first chest X-ray, sputum culture outcome and the presence of supporting family member were significant variables in the urban area(p<0.05). Most patients with family member were proved to be compliant with the prescription. This shows that it is important for the patients with long-lasting ilnesses to have supporting family member. Therefore, to improve prescription compliance we should strengthen the health education before the initiation of treatment and take special interest in the patients without supporting family member.

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Pulmonary Resection in the Treatment of Multidrug-Resistant Tuberculosis (다제 내성 폐결핵환자의 폐절제술에 관한 연구)

  • Kwon, Eun-Soo;Ha, Hyun-Cheol;Hwang, Su-Hee;Lee, Hung-Yol;Park, Seung-Kyu;Song, Sun-Dae
    • Tuberculosis and Respiratory Diseases
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    • v.45 no.6
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    • pp.1143-1153
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    • 1998
  • Background : Recent outbreaks of pulmonary disease due to drug-resistant strains of Mycobacterium Tuberculosis have resulted in significant morbidity and mortality in patients worldwide. We reviewed our experience to evaluate the effects of pulmonary resection on the management of multidrug-resistant tuberculosis. Method : A retrospective review was performed of 41 patients undergoing pulmonary resection for multidrug-resistant tuberculosis between January 1993 and December 1997. We divided these into 3 groups according to the radiologic findings : (1) patients who have reasonably localized lesion (Localized Lesion Group ; LLG) (2) patients who have cavitary lesions after pulmonary resection on chest roentgenogram (Remained Cavity Group : RCG) (3) patients who have Remained infiltrative lesions postoperatively (Remained infiltrative group : RIG). We evaluated the negative conversion rate after resection and overall response rate of the groups. Then they were compared with the results of the chemotherapy on the multi drug-resistant tuberculosis which has been outcome by Goble et al. Goble et al reported that negative conversion rate was 65% and overall response rate, 56% over a mean period of 5.1 months. Results : Seventy five point six percent were men and 24.4% women with a median age of 31 years (range, 16 to 60 years). Although the patients were treated preoperatively with multidrug regimens in an effort to reduce the mycobacterial burden, 22 of 41 were still sputum culture positive at the time of surgery. 20 of 22 patients(90.9%, p<0.01) responded which is defined as negative sputum cultures within 2 months postoperative. Of 26 patients with the sufficient follow up data, 19 have Remained sputum culture negative for a mean duration of 25.7 months (73.1%, p<0.05). The bulk of the disease was manifest in one lung, but lesser amounts of contralateral disease were demonstrated in 15, consisted of 8 in RIG and 7 in RCG, of 41. 12 of 12 patients (100%, p<0.01) who were sputum positive at the time of surgery in LLG converted successfully. 14 of 15 patients (93.3%, p<0.05) with the follow up have completed treatment and not relapsed for a mean period of 25. 7 months. The mean length of postoperative drug therapy of LLG was 12.2 months. In RIG, postoperative negative conversion rate was 83.3% which was not significant statistically. There was a statistical significance in overall response rate (100%, p<0.05) of RIG for a mean period of 24.4 months with a mean length of postoperative chemotherapy, 11.8 months. In RCG a statistically lower overall response rate (14.3%, p<0.01) has been revealed for a mean duration of follow up, 24.2 months. A negative conversion rate of RCG was 75% which was not significant statistically. Conclusion : Surgery plays an important role in the management of patients with multidrug-resistant Mycobacterium tuberculosis infection. Aggressive pulmonary resection should be performed for resistant Mycobacterium tuberculosis infection to avoid treatment failure or relapse. Especially all cavitary lesions on preoperative chest roentgenogram should be resected completely. If all of them could not be resected perfectly, you should not open the thorax.

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A Study of Equipment Accuracy and Test Precision in Dual Energy X-ray Absorptiometry (골밀도검사의 올바른 질 관리에 따른 임상적용과 해석 -이중 에너지 방사선 흡수법을 중심으로-)

  • Dong, Kyung-Rae;Kim, Ho-Sung;Jung, Woon-Kwan
    • Journal of radiological science and technology
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    • v.31 no.1
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    • pp.17-23
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    • 2008
  • Purpose : Because there is a difference depending on the environment as for an inspection equipment the important part of bone density scan and the precision/accuracy of a tester, the management of quality must be made systematically. The equipment failure caused by overload effect due to the aged equipment and the increase of a patient was made frequently. Thus, the replacement of equipment and additional purchases of new bonedensity equipment caused a compatibility problem in tracking patients. This study wants to know whether the clinical changes of patient's bonedensity can be accurately and precisely reflected when used it compatiblly like the existing equipment after equipment replacement and expansion. Materials and methods : Two equipments of GE Lunar Prodigy Advance(P1 and P2) and the Phantom HOLOGIC Spine Road(HSP) were used to measure equipment precision. Each device scans 20 times so that precision data was acquired from the phantom(Group 1). The precision of a tester was measured by shooting twice the same patient, every 15 members from each of the target equipment in 120 women(average age 48.78, 20-60 years old)(Group 2). In addition, the measurement of the precision of a tester and the cross-calibration data were made by scanning 20 times in each of the equipment using HSP, based on the data obtained from the management of quality using phantom(ASP) every morning (Group 3). The same patient was shot only once in one equipment alternately to make the measurement of the precision of a tester and the cross-calibration data in 120 women(average age 48.78, 20-60 years old)(Group 4). Results : It is steady equipment according to daily Q.C Data with $0.996\;g/cm^2$, change value(%CV) 0.08. The mean${\pm}$SD and a %CV price are ALP in Group 1(P1 : $1.064{\pm}0.002\;g/cm^2$, $%CV=0.190\;g/cm^2$, P2 : $1.061{\pm}0.003\;g/cm^2$, %CV=0.192). The mean${\pm}$SD and a %CV price are P1 : $1.187{\pm}0.002\;g/cm^2$, $%CV=0.164\;g/cm^2$, P2 : $1.198{\pm}0.002\;g/cm^2$, %CV=0.163 in Group 2. The average error${\pm}$2SD and %CV are P1 - (spine: $0.001{\pm}0.03\;g/cm^2$, %CV=0.94, Femur: $0.001{\pm}0.019\;g/cm^2$, %CV=0.96), P2 - (spine: $0.002{\pm}0.018\;g/cm^2$, %CV=0.55, Femur: $0.001{\pm}0.013\;g/cm^2$, %CV=0.48) in Group 3. The average error${\pm}2SD$, %CV, and r value was spine : $0.006{\pm}0.024\;g/cm^2$, %CV=0.86, r=0.995, Femur: $0{\pm}0.014\;g/cm^2$, %CV=0.54, r=0.998 in Group 4. Conclusion: Both LUNAR ASP CV% and HOLOGIC Spine Phantom are included in the normal range of error of ${\pm}2%$ defined in ISCD. BMD measurement keeps a relatively constant value, so showing excellent repeatability. The Phantom has homogeneous characteristics, but it has limitations to reflect the clinical part including variations in patient's body weight or body fat. As a result, it is believed that quality control using Phantom will be useful to check mis-calibration of the equipment used. A value measured a patient two times with one equipment, and that of double-crossed two equipment are all included within 2SD Value in the Bland - Altman Graph compared results of Group 3 with Group 4. The r value of 0.99 or higher in Linear regression analysis(Regression Analysis) indicated high precision and correlation. Therefore, it revealed that two compatible equipment did not affect in tracking the patients. Regular testing equipment and capabilities of a tester, then appropriate calibration will have to be achieved in order to calculate confidential BMD.

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An Overview of the Rationale of Monetary and Banking Intervention: The Role of the Central Bank in Money and Banking Revisited (화폐(貨幣)·금융개입(金融介入)의 이론적(理論的) 근거(根據)에 대한 고찰(考察) : 중앙은행(中央銀行)의 존립근거(存立根據)에 대한 개관(槪觀))

  • Jwa, Sung-hee
    • KDI Journal of Economic Policy
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    • v.12 no.3
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    • pp.71-94
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    • 1990
  • This paper reviews the rationale of monetary and banking intervention by an outside authority, either the government or the central bank, and seeks to delineate clearly the optimal limits to the monetary and banking deregulation currently underway in Korea as well as on a global scale. Furthermore, this paper seeks to establish an objective and balanced view on the role of the central bank, especially in light of the current discussion on the restructuring of Korea's central bank, which has been severely contaminated by interest-group politics. The discussion begins with the recognition that the modern free banking school and the new monetary economics are becoming formidable challenges to the traditional role of the government or the central bank in the monetary and banking sector. The paper reviews six arguments that have traditionally been presented to support intervention: (1) the possibility of an over-issue of bank notes under free banking instead of central banking; (2) externalities in and the public good nature of the use of money; (3) economies of scale and natural monopoly in producing money; (4) the need for macro stabilization policy due to the instability of the real sector; (5) the external effects of bank failure due to the inherent instability of the existing banking system; and (6) protection for small banknote users and depositors. Based on an analysis of the above arguments, the paper speculates on the optimal role of the government or central bank in the monetary and banking system and the optimal degree of monetary and banking deregulation. By contrast to the arguments for free banking or laissez-faire monetary systems, which become fashionable in recent years, monopoly and intervention by the government or central bank in the outside money system can be both necessary and optimal. In this case, of course, an over-issue of fiat money may be possible due to political considerations, but this issue is beyond the scope of this paper. On the other hand, the issue of inside monies based on outside money could indeed be provided for optimally under market competition by private institutions. A competitive system in issuing inside monies would help realize, to the maxim urn extent possible, external economies generated by using a single outside money. According to this reasoning, free banking activities will prevail in the inside money system, while a government monopoly will prevail in the outside money system. This speculation, then, also implies that the monetary and banking deregulation currently underway should and most likely will be limited to the inside money system, which could be liberalized to the fullest degree. It is also implied that it will be impractical to deregulate the outside money system and to allow market competition to provide outside money, in accordance with the arguments of the free banking school and the new monetary economics. Furthermore, the role of the government or central bank in this new environment will not be significantly different from their current roles. As far as the supply of fiat money continues to be monopolized by the government, the control of the supply of base money and such related responsibilities as monetary policy (argument(4)) and the lender of the last resort (argument (5)) will naturally be assigned to the outside money supplier. However, a mechanism for controlling an over-issue of fiat money by a monopolistic supplier will definitely be called for (argument(1)). A monetary policy based on a certain policy rule could be one possibility. More importantly, the deregulation of the inside money system would further increase the systemic risk inherent in the current fractional banking system, while enhancing the efficiency of the system (argument (5)). In this context, the role of the lender of the last resort would again become an instrument of paramount importance in alleviating liquidity crises in the early stages, thereby disallowing the possibility of a widespread bank run. Similarly, prudential banking supervision would also help maintain the safety and soundness of the fully deregulated banking system. These functions would also help protect depositors from losses due to bank failures (argument (6)). Finally, these speculations suggest that government or central bank authorities have probably been too conservative on the issue of the deregulation of the financial system, beyond the caution necessary to preserve system safety. Rather, only the fullest deregulation of the inside money system seems to guarantee the maximum enjoyment of external economies in the single outside money system.

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The Role of NF-${\kappa}B$ in the TNF-$\alpha$-induced Apoptosis of Lung Cancer Cell Line (폐암세포주의 TNF-$\alpha$ 유발 apoptosis에서 NF-${\kappa}B$의 역할)

  • Kim, J.Y.;Lee, S.H.;HwangBo, B.;Lee, C.T.;Kim, O.H.;Han, S.K.;Shim, O.S.;Yoo, C.G.
    • Tuberculosis and Respiratory Diseases
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    • v.48 no.2
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    • pp.166-179
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    • 2000
  • Background: The main reason for the failure of anti-cancer chemotherapy is the build up of resistance by cancer cells to apoptosis. The activation of NF-${\kappa}B$ in many cancer cell lines is reported to be underlying mechanism behind the build up of resistance of cancer cells to apoptosis. However, this relationship varied depending on the cells used in the experiments. In this study, the role of NF-${\kappa}B$ activation in the TNF-$\alpha$-induced apoptosis in lung cancer cell line was evaluated. Methods: NCI-H157 cells were used in all experiments. Cells were exposed to a high dose of TNF-$\alpha$(20 ng/ml) for 24 or 48 hours with or without blocking NF-${\kappa}B$ activation. TNF-$\alpha$-induced activation of NF-${\kappa}B$ was inhibited either by overexpression of $I{\kappa}B{\alpha}$-super repressor($I{\kappa}B{\alpha}$-SR) or by pre-treatment with proteasome inhibitor. Cell viability and apoptosis were evaluated with MTT assay and Western blot analysis for PARP fragment, respectively. Results: Cell viability of NCI-H157 cells was not affected by TNF-$\alpha$ treatment alone; however, combined treatment with TNF-$\alpha$ and cycloheximide reduced cell viability significantly, indicating that resistance to TNF-$\alpha$ is mediated by the new proteins synthesized after TNF-$\alpha$ stimulation. To evaluate the role of NF-${\kappa}B$ in the transcription of anti-apoptotic proteins. delete NF-${\kappa}B$ activation was inhibited before TNF-$\alpha$ stimulation. as described above. $AD5I{\kappa}B{\alpha}$-SR-transduction inhibited TNF-$\alpha$-induced nuclear translocation of p65. TNF-$\alpha$-induced cell death and apoptosis increased after inhibition of TNF-$\alpha$-induced activation of NF-${\kappa}$ by methods. Conclusion: These results suggest that TNF-$\alpha$-induced activation of NF-${\kappa}B$ may be closely related to the acquisition of the resistance to TNF-$\alpha$-induced apoptosis in lung cancer cells. Therefore. blocking of NF-${\kappa}B$ pathway can be a useful therapeutic modality in the treatment of lung cancer.

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Clinical Analysis of Children with Transitory Minimal Change Nephrotic Syndrome (MCNS) to Focal Segmental Glomerulosclerosis (FSCS) (미세변화형 신증후군(MCNS)으로부터 국소성 분절성 사구체 경화증(FSGS)으로 이행된 환아의 임상양상)

  • Lee Ji Eun;Yook Jinwon;Lee Eui Seong;Kim Ji Hong;Kim Pyung-Kil;Chung Hyun Joo
    • Childhood Kidney Diseases
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    • v.4 no.1
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    • pp.17-24
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    • 2000
  • Purpose: MCNS is found in approximately $85\%$ of the idiopathic nephrotic syndrome in children and shows good prognosis with initial steroid therapy. However in FSGS, there is poor prognosis with initial therapy and shows higher rate of progression to chronic renal failure and relapse after kindney transplantation. We have experienced 8 patients who were diagnosed as MCNS on initial renal biopsy and then progressed to FSGS on follow-up biopsy. So we have investigated their clinical course and risk factors for transition of MCNS to FSGS. Methods: We conducted a retrospective study with a review of histopathologic findings and clinical manifestations of 296 cases of MCNS and FSGS that were diagnosed from January 1988 to May 1999. We classified them into 3 groups according to the histopathologic finding; MCNS, FSGS, MCNS progressed to FSGS in follow-up biopsy. Results: The number of children was 296 cases comprising 241 cases($81.4\%$) showing MCNS, 8 cases($2.7\%$) transition group, 47 cases($15.9\%$) FSGS. The mean onset age was $6.0{\pm}2.6$years in MCNS, transition group $8.3{\pm}2.3$years, FSGS $7.2{\pm4.3$years, and the gender (M:F) ratio was 3.7:1 in MCNS, 3:1 in transition group, 1.8:1 in FSGS. Comparing the presence of initial hematuria, hypertension,24 hour urine protein, serum albumin, serum creatinine, there were significant difference between the transition group and the FSGS group in the following points; 24hour urine protein $684:342mg/m^2/hr$(P<0.05), serum albumin 1.92: 2.47g/dL(P<0.05), serum cholesterol 494:343mg/dL(P<0.05). Refractoriness to steroid therapy was 13.3$\%$ in MCNS. $12.5\%$ in transition group, $29.6\%$ in FSGS; significantly higher in FSGS(P<0.05). Immunosuppressant therapy was performed in $58.5\%$ of MCNS, $100\%$ in transition group, $80.8\%$ in FSGS; transition group showed significantly higher .ate(P<0.05) comparing with MCNS. Mean number of relapse and duration from onset to first relapse showed no significance difference between these groups. Conclusion: 249 patients with MCNS have been followed and $3.2\%$ (8 patients) of them has shown change in pathologic diagnosis from MCNS to FSCS. The risk factor for transition could not be found. Our results point to the need for a follow-up biopsy to certify the possibility of transition to FSCS in some MCNS cases with refractory cases to steroid therepy, frequent relapsing cases, or in case of no remission in spite of vigorous immunosuppressant therapy.

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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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Analysis of Childhood Rapidly Progressive Glomerulonephritis (소아 급속 진행성 사구체신염의 임상적 고찰)

  • Uhm Ji Hyun;Kim Mi Jin;Lee Young-Mock;Kim Ji Hong;Lee Jae Seung;Kim Pyung-Kil;Hong Soon Won;Jeung Hyeun Joo
    • Childhood Kidney Diseases
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    • v.5 no.2
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    • pp.78-86
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    • 2001
  • Purpose: Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than $50\%$ of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features Method: Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during tile last 10 years, from May 1990 to May 2000. Result: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean $10.9{\pm}3.8$). 3 had Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was $74.2{\pm}39.1\;mg/dL$ mean serum creatinin, $3.2{\pm}1.8\;mg/dL$ and mean creatinin clearance, $26.5{\pm}13.2\;mL/min/1.73m^2$. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function. Conclusion: Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 78-86)

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