• Title/Summary/Keyword: Defects

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Somatic Cell Analysis and Cobalamin Responsiveness Study in Ten Korean Patients with Methylmalonic Aciduria (한국 메틸말로닌산혈증 환아 10례에서 Somatic Cell 분석과 cobalamin 반응성 연구)

  • Lim, Han Hyuk;Song, Wung Joo;Kim, Gu-Hwan;Watkins, David;Rosenblatt, David S.;Kim, Yoo-Mi;Chang, Mea Young;Kil, Hong Ryang;Kim, Sook Za
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.19 no.1
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    • pp.12-19
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    • 2019
  • Purpose: Isolated methylmalonic acidemia (MMA) is an autosomal recessive inherited disorder of propionate metabolism. There are two subtypes of MMUT gene defects. $Mut^0$ represents complete loss of methylmalonyl-CoA mutase (MCM) activity while mut- is associated with residual MCM activity, which can be stimulated by hydroxocobalamin (OHCbl) supplementation. The objective of this study is to investigate cobalamin responsiveness and mutations present in Korean MMA population. Methods: We evaluated 10 MMA patients using somatic cell complementation analysis on their fibroblasts to measure MCM activity and vitamin B12 responsiveness for the optimal treatment. MMUT gene was sequenced to identify the MMA mutations. Results: For all patients, the incorporation of $[^{14}C]-propionate$ was low, and there was no response to OHCbl. The incorporation of $[^{14}C]-methyltetrahydrofolate$ and $[^{57}Co]-CNCbl$ fell within the normal range. There was adequate synthesis of methylcobalamin while the synthesis of adenosylcobalamin was low. The complementation analysis showed all patients were $mut^0$. The sequence analysis identified 12 different MMUT mutations, including 2 novel mutations, p.Gln267Ter and p.Ile697Phe, were identified. All the patients in this study had neonatal onset of symptoms, belonged to $mut^0$ complementation class, and as a result, showed no cobalamin responsiveness. Conclusion: No Korean MMA patient showed cobalamin responsiveness.

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Clinical Evaluation of Guided Bone Regeneration Using 3D-titanium Membrane and Advanced Platelet-Rich Fibrin on the Maxillary Anterior Area (상악 전치부 3D-티타늄 차폐막과 혈소판농축섬유소를 적용한 골유도재생술의 임상적 평가)

  • Lee, Na-Yeon;Goh, Mi-Seon;Jung, Yang-Hun;Lee, Jung-Jin;Seo, Jae-Min;Yun, Jeong-Ho
    • Implantology
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    • v.22 no.4
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    • pp.242-254
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    • 2018
  • The aim of the current study was to evaluate the results of horizontal guided bone regeneration (GBR) with xenograf t (deproteinized bovine bone mineral, DBBM), allograf t (irradiated allogenic cancellous bone and marrow), titanium membrane, resorbable collagen membrane, and advanced platelet-rich fibrin (A-PRF) in the anterior maxilla. The titanium membrane was used in this study has a three-dimensional (3D) shape that can cover ridge defects. Case 1. A 32-year-old female patient presented with discomfort due to mobility and pus discharge on tooth #11. Three months after extracting tooth #11, diagnostic software (R2 GATE diagnostic software, Megagen, Daegu, Korea) was used to establish the treatment plan for implant placement. At the first stage of implant surgery, GBR for horizontal augmentation was performed with DBBM ($Bio-Oss^{(R)}$, Geistlich, Wolhusen, Switzerland), irradiated allogenic cancellous bone and marrow (ICB $cancellous^{(R)}$, Rocky Mountain Tissue Bank, Denver, USA), 3D-titanium membrane ($i-Gen^{(R)}$, Megagen, Daegu, Korea), resorbable collagen membrane (Collagen $membrane^{(R)}$, Genoss, Suwon, Korea), and A-PRF because there was approximately 4 mm labial dehiscence after implant placement. Five months after placing the implant, the second stage of implant surgery was performed, and healing abutment was connected after removal of the 3D-titanium membrane. Five months after the second stage of implant surgery was done, the final prosthesis was then delivered. Case 2. A 35-year-old female patient presented with discomfort due to pain and mobility of implant #21. Removal of implant #21 fixture was planned simultaneously with placement of the new implant fixture. At the first stage of implant surgery, GBR for horizontal augmentation was performed with DBBM ($Bio-Oss^{(R)}$), irradiated allogenic cancellous bone and marrow (ICB $cancellous^{(R)}$), 3D-titanium membrane ($i-Gen^{(R)}$), resorbable collagen membrane (Ossix $plus^{(R)}$, Datum, Telrad, Israel), and A-PRF because there was approximately 7 mm labial dehiscence after implant placement. At the second stage of implant surgery six months after implant placement, healing abutment was connected after removing the 3D-titanium membrane. Nine months after the second stage of implant surgery was done, the final prosthesis was then delivered. In these two clinical cases, wound healing of the operation sites was uneventful. All implants were clinically stable without inflammation or additional bone loss, and there was no discomfort to the patient. With the non-resorbable titanium membrane, the ability of bone formation in the space was stably maintained in three dimensions, and A-PRF might influence soft tissue healing. This limited study suggests that aesthetic results can be achieved with GBR using 3D-titanium membrane and A-PRF in the anterior maxilla. However, long-term follow-up evaluation should be performed.

A Study on Differentiation and Improvement in Arbitration Systems in Construction Disputes (건설분쟁 중재제도의 차별화 및 개선방안에 관한 연구)

  • Lee, Sun-Jae
    • Journal of Arbitration Studies
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    • v.29 no.2
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    • pp.239-282
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    • 2019
  • The importance of ADR(Alternative Dispute Resolution), which has the advantage of expertise, speed and neutrality due to the increase of arbitration cases due to domestic and foreign construction disputes, has emerged. Therefore, in order for the nation's arbitration system and the arbitration Organization to jump into the ranks of advanced international mediators, it is necessary to research the characteristics and advantages of these arbitration Organization through a study of prior domestic and foreign research and operation of international arbitration Organization. As a problem, First, education for the efficient promotion of arbitrators (compulsory education, maintenance education, specialized education, seminars, etc.). second, The effectiveness of arbitration in resolving construction disputes (hearing methods, composition of the tribunal, and speed). third, The issue of flexibility and diversity of arbitration solutions (the real problem of methodologies such as mediation and arbitration) needs to be drawn on the Arbitration laws and practical problems, such as laws, rules and guidelines. Therefore, Identify the problems presented in the preceding literature and diagnosis of the defects and problems of the KCAB by drawing features and benefits from the arbitration system operated by the international arbitration Institution. As an improvement, the results of an empirical analysis are derived for "arbitrator" simultaneously through a recognition survey. As a method of improvement, First, as an optimal combination of arbitration hearing and judgment in the settlement of construction disputes,(to improve speed). (1) A plan to improve the composition of the audit department according to the complexity, specificity, and magnification of the arbitration cases - (1)Methods to cope with the increased role of the non-lawyer(Specialist, technical expert). (2)Securing technical mediators for each specialized expert according to the large and special corporation arbitration cases. (2) Improving the method of writing by area of the arbitration guidelines, second, Introduction of the intensive hearing system for psychological efficiency and the institutional improvement plan (1) Problems of optimizing the arbitration decision hearing procedure and resolution of arbitration, and (2) Problems of the management of technical arbitrators of arbitration tribunals. (1)A plan to expand hearing work of technical arbitrator(Review on the introduction of the Assistant System as a member of the arbitration tribunals). (2)Improved use of alternative appraisers by tribunals(cost analysis and utilization of the specialized institution for calculating construction costs), Direct management of technical arbitrators : A Study on the Improvement of the Assessment Reliability of the Appraisal and the Appraisal Period. third, Improvement of expert committee system and new method, (1) Creating a non-executive technical committee : Special technology affairs, etc.(Major, supports pre-qualification of special events and coordinating work between parties). (2) Expanding the standing committee.(Added expert technicians : important, special, large affairs / pre-consultations, pre-coordination and mediation-arbitration). This has been shown to be an improvement. In addition, institutional differentiation to enhance the flexibility and diversity of arbitration. In addition, as an institutional differentiation to enhance the flexibility and diversity of arbitration, First, The options for "Med-Arb", "Arb-Med" and "Arb-Med-Arb" are selected. second, By revising the Agreement Act [Article 28, 2 (Agreement on Dispute Resolution)], which is to be amended by the National Parties, the revision of the arbitration settlement clause under the Act, to expand the method to resolve arbitration. third, 2017.6.28. Measures to strengthen the status role and activities of expert technical arbitrators under enforcement, such as the Act on Promotion of Interestments Industry and the Information of Enforcement Decree. Fourth, a measure to increase the role of expert technical Arbitrators by enacting laws on the promotion of the arbitration industry is needed. Especially, the establishment of the Act on Promotion of Intermediation Industry should be established as an international arbitration agency for the arbitration system. Therefore, it proposes a study of improvement and differentiation measures in the details and a policy, legal and institutional improvement and legislation.

A Study on World University Evaluation Systems: Focusing on U-Multirank of the European Union (유럽연합의 세계 대학 평가시스템 '유-멀티랭크' 연구)

  • Lee, Tae-Young
    • Korean Journal of Comparative Education
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    • v.27 no.4
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    • pp.187-209
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    • 2017
  • The purpose of this study was to highlight the necessity of a conceptual reestablishment of world university evaluations. The hitherto most well-known and validated world university evaluation systems such as Times Higher Education (THE), Quacquarelli Symonds (QS) or Academic Ranking of World Universities (ARWU) primarily assess big universities with quantitative evaluation indicators and performance results in the rankings. Those Systems have instigated a kind of elitism in higher education and neglect numerous small or local institutions of higher education, instead of providing stakeholders with comprehensive information about the real possibilities of tertiary education so that they can choose an institution that is individually tailored to their needs. Also, the management boards of universities and policymakers in higher education have partly been manipulated by and partly taken advantage of the elitist ranking systems with an economic emphasis, as indicated by research-centered evaluations and industry-university cooperation. To supplement such educational defects and to redress the lack of world university evaluation systems, a new system called 'U-Multirank' has been implemented with the financial support of the European Commission since 2012. U-Multirank was designed and is enforced by an international team of project experts led by CHE(Centre for Higher Education/Germany), CHEPS(Center for Higher Education Policy Studies/Netherlands) and CWTS(Centre for Science and Technology Studies at Leiden University/Netherlands). The significant features of U-Multirank, compared with e.g., THE and ARWU, are its qualitative, multidimensional, user-oriented and individualized assessment methods. Above all, its website and its assessment results, based on a mobile operating system and designed simply for international users, present a self-organized and evolutionary model of world university evaluation systems in the digital and global era. To estimate the universal validity of the redefinition of the world university evaluation system using U-Multirank, an epistemological approach will be used that relies on Edgar Morin's Complexity Theory and Karl Popper's Philosophy of Science.

1H Solid-state NMR Methodology Study for the Quantification of Water Content of Amorphous Silica Nanoparticles Depending on Relative Humidity (상대습도에 따른 비정질 규산염 나노입자의 함수량 정량 분석을 위한 1H 고상 핵자기 공명 분광분석 방법론 연구)

  • Oh, Sol Bi;Kim, Hyun Na
    • Korean Journal of Mineralogy and Petrology
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    • v.34 no.1
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    • pp.31-40
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    • 2021
  • The hydrogen in nominally anhydrous mineral is known to be associated with lattice defects, but it also can exist in the form of water and hydroxyl groups on the large surface of the nanoscale particles. In this study, we investigate the effectiveness of 1H solid-state nuclear magnetic resonance (NMR) spectroscopy as a robust experimental method to quantify the hydrogen atomic environments of amorphous silica nanoparticles with varying relative humidity. Amorphous silica nanoparticles were packed into NMR rotors in a temperature-humidity controlled glove box, then stored in different atmospheric conditions with 25% and 70% relative humidity for 2~10 days until 1H NMR experiments, and a slight difference was observed in 1H NMR spectra. These results indicate that amount of hydrous species in the sample packed in the NMR rotor is rarely changed by the external atmosphere. The amount of hydrogen atom, especially the amount of physisorbed water may vary in the range of ~10% due to the temporal and spatial inhomogeneity of relative humidity in the glove box. The quantitative analysis of 1H NMR spectra shows that the amount of hydrogen atom in amorphous silica nanoparticles linearly increases as the relative humidity increases. These results imply that the sample sealing capability of the NMR rotor is sufficient to preserve the hydrous environments of samples, and is suitable for the quantitative measurement of water content of ultrafine nominally anhydrous minerals depending on the atmospheric relative humidity. We expect that 1H solid-state NMR method is suitable to investigate systematically the effect of surface area and crystallinity on the water content of diverse nano-sized nominally anhydrous minerals with varying relative humidity.

Possibility of Combined Meningitis in Under 90-Day-Old Infants With Urinary Tract Infection (생후 90일 미만 영아의 요로 감염에서 세균성 수막염의 동반 가능성)

  • Hwang, Jun Ho;Kim, Su Yeong;Lee, Na Mi;Yi, Dae Yong;Yun, Sin Weon;Chae, Soo Ahn;Lim, In Seok;Park, Ji Young
    • Pediatric Infection and Vaccine
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    • v.29 no.2
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    • pp.84-95
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    • 2022
  • Purpose: Urinary tract infections (UTIs) are the most common serious bacterial infections in young infants. Lumbar puncture (LP) has been used to diagnose coexisting meningitis in infants under 90 days of age with suspected UTI in many hospitals. However, the incidence of bacterial meningitis associated with UTIs is low. We aimed to describe the prevalence of concomitant bacterial meningitis in young infants with UTIs. Methods: The medical records of infants with the first episode of UTI admitted to the Chung-Ang University Hospital from January 2010 to December 2019 were retrospectively reviewed. Infants aged < 90 days who underwent LP with initial evaluation were included. Demographic and clinical features, laboratory findings, and imaging findings were collected and analyzed. Results: Eighty-six infants with UTIs were enrolled in the study. The median age was 61.5 days (interquartile range, 42.3-73.8 days) and boys (90.7%) were predominant. Escherichia coli was the most common pathogen (n=80, 93.0%) and followed by Klebsiella species (n=5, 5.8%). Fifteen (18.1%) specimens produced extended spectrum β-lactamase (ESBL). Five (5.8%) infants had positive blood culture results. Seven (8.1%) infants showed pleocytosis in the cerebrospinal fluid, but none had coexisting bacterial meningitis. Twenty-four (30.8%) infants showed renal dilatation or hydronephrosis on ultrasonography. Dimercaptosuccinic acid (DMSA) scans revealed cortical defects in 17 (21.3%) infants while voiding cystourethrography revealed vesicoureteral reflux in 6 (46.2%) infants. Conclusion: Co-existing bacterial meningitis was not observed in young infants with UTIs. LP could not be routinely performed considering the clinical condition of <90 days old UTI patients.

An event-related potential study of global-local visual perception in female college students with binge drinking (폭음 여자대학생의 전체-세부 시지각 처리에 관한 사건관련전위 연구)

  • So-yeon Lim;Myung-Sun Kim
    • Korean Journal of Cognitive Science
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    • v.34 no.2
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    • pp.111-151
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    • 2023
  • It is reported that binge drinkers show cognitive impairment similar to alcohol use disorder patients. A previous studies using neuropsychological tests and brain imaging techniques to investigate the visual perception of alcohol use disorder patients reported that they had global-local visual perception defects. Although the neurological basis for the global-local visual perception deficit in the heavy drinking group has been presented, there are no studies to date that have investigated the global-local visual perception in the heavy drinking group. This study investigated local-biased visual perception in female college students with binge drinking (BD) using event-related potentials (ERPs). Based on the scores of the Korean version of Alcohol Use Disorder Identification Test and the Alcohol Use Questionnaire, participants were assigned into BD (n=25) and non-BD (n=25) groups. Local-global visual processing was assessed using a local-global paradigm, in which large stimuli (global level) composed of small stimuli (local level) were presented. The stimuli presented at global and local levels were either congruent or incongruent. The behavioral results exhibited that the BD and non-BD groups did not differ in terms of accuracy and response time. In terms of ERPs, the BD and non-BD groups did not show difference in N100, P150 and N200 amplitude. However, the BD group showed significantly smaller P300 amplitude than non-BD group especially in the local condition. In addition, a negative correlation between P300 amplitude and binge drinking score was observed, i.e., severer binge drinking smaller P300 amplitude. The P300 is known to reflect cognitive inhibition and attentional allocation. In the global-local paradigm, the local condition required to attend to local target while ignoring global non-target. Therefore, the present results indicate that female college students with BD do not have local-biased visual processing, instead they seem to have difficulties in inhibition of irrelevant stimuli.

Alveolar ridge preservation using granulation tissue for esthetic implant restoration on maxillary anterior tooth (상악 전치부의 심미적 임플란트 수복을 위한 육아 조직(Granulation tissue)을 이용한 치조제 보존술)

  • Lee Chang Kyun
    • Journal of the Korean Academy of Esthetic Dentistry
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    • v.32 no.1
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    • pp.16-22
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    • 2023
  • Esthetic factors are very important in the success of maxillary anterior implant restoration. However, achieving esthetic results is difficult, especially in cases where periodontitis has resulted in severe alveolar bone loss. In the case of maxillary anterior teeth, the alveolar ridge resorption that begins immediately after tooth extraction interferes with the esthetic implant restoration. Therefore immediate implant placement can be performed to minimize the alveolar ridge resorption. However, in severe bone loss cases, immediate implant placement could result in esthetic failure, and this result might cause irreparable problems. We can also perform alveolar ridge preservation and then place implants later. On JCP published in 2019, there is the consensus of European academy of periodontology on the extraction socket management and the timing of implant placement. This consensus states that alveolar ridge preservation should be considered when there is severe labial bone loss in an esthetically important area such as maxillary anterior region. On performing the alveolar ridge preservation, we cannot obtain the primary wound closure, so secondary wound healing is induced with open membrane technique or soft tissue grafting should be performed for primary wound closure. However, the secondary wound healing can have a negative impact on bone regeneration, and soft tissue grafting such as FGG or CT graft can be burdensome for both patients and dentists. On the other hand, by using the granulation tissue in the extraction socket, primary closure can be achieved without soft tissue grafting. Also some studies have shown that granulation tissue in periodontal defects contains stem cells that may help in tissue regeneration. Based on this, implant restorations were performed on maxillary anterior teeth with severe alveolar bone loss by alveolar ridge preservation using granulation tissue. In spite of the severe bone defect of the extraction socket, relatively esthetic results could be obtained in implant restorations.

Optimum Management Plan for Soil Contamination Facilities (특정토양오염관리대상시설의 최적 관리방안에 관한 연구)

  • Park, Jae-Soo;Kim, Ki-Ho;Kim, Hae-Keum;Choi, Sang-Il
    • Korean Journal of Soil Science and Fertilizer
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    • v.45 no.2
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    • pp.293-300
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    • 2012
  • This study was to investigate the unsuitable rate of the storage facilities, the changes in corrosion process over time after installation according to the status, the time to install the facilities, years elapsed after facilities installation, inspection of methods and motivation, and so on, based on the results of the inspection at the petroleum storage facilities conducted by domestic soil-relate specialized agency to derive optimal management plans which meet the status of soil contamination facilities. The results showed that the facilities more than 5 years after the initial leak test at the time of the installation need to be inspected periodically by considering costs of leak test and remediation of polluted soil. The inspection period can be decided by cost and leak test methods showing discrepancies for the results obtained from individual test whether it was direct or indirect. To compensate these matters, we suggested that the direct inspection method on regular schedule is recommended. On the other hand, the inspection can be voluntarily completed to ease burden of the results by inspection or equivalent level to this inspection method. Also, it may need improved construction supervision and performance test system to minimize the occurrence of the nature defects in installing the facilities as well as the upgrade program for the facilities during intervals of inspection period.

Diffuse Panbronchiolitis : Clinical Significance of High-resolution CT and Radioaerosol Scan Manifestations (미만성 범세기관지염에서 흉부 고해상도 전산화 단층촬영의 임상적의의 및 폐환기주사 소견)

  • Song, So Hyang;Kim, Hui Jung;Kim, Young Kyoon;Moon, Hwa Sik;Song, Jeong Sup;Park, Sung Hak;Kim, Hak Hee;Chung, Soo Kyo
    • Tuberculosis and Respiratory Diseases
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    • v.44 no.1
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    • pp.124-135
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    • 1997
  • Background : Diffuse panbronchiolitis(DPB) is a disease characterized clinically by chronic cough, expectoration and dyspnea; and histologically by chronic inflammation localized mainly in the region of the respiratory bronchiole. It is prevalent in Japanese, but is known to be rare in Americans and Europians. Only a few cases in Chinese, Italians, North Americans and Koreans have been reported. It is diagnosed by characteristic clinical, radiological and pathologic features. High-resolution CT(HRCT) is known to be valuable in the study of the disease process and response to therapy in DPB. To our knowledge, there has been no correlation of its appearance on HRCT with the severity of the disease process, and radioaerosol scan(RAS) of the lung has not previously been used for the diagnosis of DPB. Method : During recent two years we have found 12 cases of DPB in Kangnam St. Mary's Hospital, Catholic University Medical College. We analysed the clinical characteristics, compared HRCT classifications with clinical stages of DPB, and determined characteristic RAS manifestations of DPB. Results : 1. The ages ranged from 31 to 83 years old(mean 54.5 years old), and male female ratio was 4:8. 75%(9/12) of patients had paranasal sinusitis, and only one patient was a smoker. 2. The patients were assigned to one of three clinical stages of DPB on the basis of clinical findings, sputum bacterology and arterial blood gas analysis. of 12 cases, 5 were in the first stage, 4 were in the second stage, and 3 were in the third stage. In most of the patients, pulmonary function tests showed marked obstructive and slight restrictive impairments. Sputum culture yielded P.aeruginosa in 3 cases of our 12 cases, K.pneumoniae in 2 cases, H.influenzae in 2 cases, and S.aureus in 2 cases. 3. Of 12 patients, none had stage I characteristics as classified on HRCT scans, 4 had slage II findings, 5 had stage III findings, and 3 had stage IV characteristics. 4. We peformed RAS in 7 of 12 patients With DPB. In 71.4% (5/7) of the patients, RAS showed mottled aerosol deposits characteristically in the transitional and intermediary airways with peripheral airspace defects, which contrasted sharply with central aerosol deposition of COPD. 5. There were significant correlations between HRCT stages and clinical stages(r= 0.614, P < 0.05), between HRCT types and Pa02(r= -0.614, P < 0.05), and between HRCT types and ESR(r= 0.618, P < 0.01). Conclusion : The HRCT classifications correspond well to the clinical stage. Therfore in the examination of patients with DPB, HRCT is useful in the evaluation of both the location and severity of the lesions. Also, RAS apears to be a convenient, noninvasive and useful diagnostic method of DPB.

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