• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.1
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• pp.34-41
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• 2016

Adult-onset type II citrullinemia (CTLN2) is characterized by episodes of neurologic symptoms associated with hyperammonemia leading to disorientation, irritability, seizures, and coma. CTLN2 is distinct from classical citrullinemia, which is caused by a mutation of the argininosuccinic acid synthetase (ASS) gene. The serum citrulline level is elevated, while the activity of ASS in liver tissue is decreased. CTLN2 is known to have a poor prognosis if the proper treatment is not taken. We reported a female aged 37 years who developed recurrent attacks of altered consciousness, aberrant behavior, and vomiting. We initially suspected the patient had CTLN2 because of the signs of hyperammonemic encephalopathy, such as altered mentality, memory disturbance, and aberrant behaviors provoked by exercise-induced stress and excessive intravenous amino acid administration. Through her peculiar diet preferences and laboratory findings that included hyperammonemia and citrullinemia, we diagnosed the patient as CTLN2, and SLC25A13 sequencing revealed known compound heterozygous mutations (IVS11+1G>A, c.674C> A). Her parents were heterozygous carriers, and we identified that her older sister had the same mutations. The older sister had not experienced any episodes of hyperammonemia, but she had peculiar diet preferences. The patient and her sister have been well with conservative management. When considering the clinical course of CTLN2, it was meaningful that the older sister could be diagnosed early in an asymptomatic period and that preemptive treatment was employed. Through this case, CTLN2 should be considered in adults who present symptoms of hyperammonemic encephalopathy without a definite etiology. Because of its rare incidence and similar clinical features, CTLN2 is frequently misdiagnosed as hepatic encephalopathy, and it shows a poor prognosis due to the lack of early diagnosis and proper treatment. A high-carbohydrate diet, which is usually used to treat other urea cycle defects, can also exaggerate the clinical course of CTLN2, so proper metabolic screening tests and genetic studies should be performed.

• Nuclear Medicine and Molecular Imaging
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• v.40 no.3
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• pp.155-162
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• 2006

Purpose: Diabetes mellitus (DM) is a critical disease with higher rates of cardiovascular morbidity and mortality due to myocardial ischemia and infarction. There is glowing interest in how to determine high-risk patients who are candidates for screening testing. This study was performed to evaluate the incidence of coronary artery disease (CAD) in diabetic patients detected by Tc-99m MIBI myocardial perfusion SPECT (MPS) and to assess risk factors of CAD and cardiac hard events. Subjects and Methods: 203 diabetic patients (64 male, mean age $64.1{\pm}9.0$ years) who underwent MPS were included between Jan 2000 and July 2004. Cardiac death and nonfatal myocardial infarction (MI) were considered as hard events, and coronary angioplasty and bypass surgery >60 days after testing were considered as soft events. The mean follow-up period was $36{\pm}18$ months. Patients underwent exercise (n=6) or adenosine stress (n=197) myocardial perfusion SPECT. Results: Perfusion defects on MPS were detected in 28.6% (58/203) of the patients. There was no cardiac death but 11 hard events were observed. The annual cardiac hard event rate was 1.1%. In univariate analysis of clinical factors, typical anginal pain, peripheral vascular disease, peripheral polyneuropathy, and resting ECG abnormality were significantly associated with the ocurrence of hard events. Anginal pain, peripheral vascular disease, and resting ECG abnormality remained independent predictors of nonfatal MIs with multivariate analysis. Abnormal SPECT results were significantly associated with high prevalence of hard events but not independent predictors on uni- and multivariate analyses. Conclusion: Patients who were male, had longer diabetes duration (especially over 20 years), peripheral vascular disease, peripheral polyneuropathy, or resting ECG abnormality had higher incidence of CAD. Among clinical factors in diabetic patients, typical angina, peripheral vascular disease, peripheral polyneuropathy, and resting ECG abnormality were strong predictors of hard events.

• The Korean Journal of Nuclear Medicine
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• v.30 no.1
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• pp.130-138
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• 1996

Bone scan is known to be an effective tool for observing the state of soft tissues and bones of electric burn patients. It is also used for observing the progress of patients after debridement or skin graft as well as deforming to amputate specific body parts. To evaluate bone scan's role in electric burn, we analyzed bone scan 37 patients with electric burn. Among the 37 patients, 8 of 37 were injured in low voltage and 29 of them in high voltage. 27 patients received the electrical input through the hand, 6 through the scalp, 2 through the shoulder, 1 through the left chest wall and 1 through the left inguinal area. Among 29 patients received high voltage, 22 patients had the electrical output through the foot, 3 through the hand, 2 through the shoulder, 1 through the buttock and 1 through the left chest wall. Bone scans revealed cellulitis in 37 patients with 47 sites, osteomyelitis in 15 patients with 15 sites & bone defects in 4 patients with 4 sites. In 4 patients with skin graft or skin flap, follow up bone scan showed improvements of bony uptake in preoperatively bony defect area and all of them were healed without complication. There were 2 cases in which uptake increased in the myocardium, 1 in the liver and 6 in the kidney, however, serum calcium level, EKG, cardiac enzyme, liver and renal function tests were normal. In conclusion, bone scans are helpful in the assessment of injury sites after electrical insult and in differential diagnosis of cellulitis and osteomyelitis. It is also useful tool of assessment after skin graft or skin flap, however, it should be further evaluated about internal organ damage.

• The Korean Journal of Nuclear Medicine
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• v.37 no.4
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• pp.245-253
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• 2003

Puorpose: The purpose of this study was to evaluate the usefulness of $^{99m}Tc$ DMSA scintigraphy on the dignosis of a renal scar in children with urinary tract infections. Materials and Methods: Eighty three patients were included in this study, who were diagnosed as the urinary tract infection on the basis of symptom, urinalysis and urine culture. $^{99m}Tc$ DMSA scintigraphy and voiding cystoureterography were peformed within 7days before the treatment in all patients. We classified the scintigraphic findings as follow s : 1 ; a large hypoactive upper or lower pole. 2 ; a small hypoactive area. 3 ; single defect resulting in localized deformity of the outlines. 4 ; deformed outlines in a small or normal sized kidney. 5 ; multiple defects. 6 ; diffuse hypoactive kidney without regional impairment. Follow-up scintigraphy was done at least 6 months after the initial study. When the abnormality on the initial scintigraphy was not completely resolved on the follow-up scan, the lesion was defined as containing a scar. Results: One hundred and fifteen renal units of 166 units(69.3%) showed abnormal findings on the DMSA scintigraphy. 65 units(56.5%) was diagnosed as containing renal scars on follow-up scintigraphies. Incidences of renal scar among renal units showing pattern 3, 4 and 5 on the initial scan was 75%, 78% and 78%, respectively. Whereas many of renal units showing 1, 2 and 6 pattern were recovered(65%, 76%, 50%). Sensitivity, specificity and accuracy of pattern-based DMSA scintigraphic findings on the diagnosis of renal scar was 76.9%, 85.1% and 81.9%, respectively. VUR was significantly associated with the renal scar when the initial DMSA shows unrecoverable findings(pattern 3, 4, 5). Odds ratio of the renal scar in a kidney showing unrecoverable initial scintigraphic findings was 19.1. Odds ratio in a kidney with mild or moderate-to-severe VUR was 3.5 and 14.4 respectively. Conclusion: In the urinary tract infection, renal scar was significantly developed in a kidney showing unrecoverable findings on the initial DMSA scan and VUR on voiding cystoureterography.

• Journal of Chest Surgery
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• v.32 no.9
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• pp.781-789
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• 1999

Background: Pulmonary artery banding(PAB) accompanies some risks in the aspect of band complications and mortality in the second-stage operation. To assess these risks of the second-stage operation after PAB, we reviewed the surgical results of the second-stage operation in the pediatric patients who had undergone PAB in infancy. Material and Method: From May 1988 to June 1997, a total of 29 patients with preliminary PAB underwent open heart surgery. Ages ranged from 2 to 45 months(mean 20.6$\pm$9.0 months). Preoperative congestive heart failure conditions were improved after PAB(elective operation group) in 27 patients, but early second-stage procedures were required in the remaining 2 patients due to sustaining congestive heart failure(early operation group). Preoperative surgical indications included 2 double outlet right ventricles(DORV group) and 27 ventricular septal defects as the main cardiac anomaly(VSD group). Result: The mean time interval from PAB to the second-stage operation was 15.5$\pm$8.7 months(range 5 days to 45 months). One patient in the DORV group underwent intraventricular tunnel repair and modified Glenn procedure in the other. In the VSD group, the VSD was closed with a Dacron patch in all patients. Concomitant procedures included a right ventricular infundibulectomy in 4 patients and a valvectomy of the dysplastic pulmonary valve in 1 patient. At the second-stage operations, pulmonary angioplasty was required due to the stenotic banding sites in 18 patients. One patient underwent complete ligation of the main pulmonary artery with the modified Glenn procedure. The mortality at the second-stage operation was 17.2%(5 patients). Causes of death were 4 low cardiac output, and 1 autoimmune hemolytic anemia. Diagnosis with DORV and the early operative group were the risk factors for operative death in this series. There was 1 late death. Conclusion: This study revealed the second-stage operation for pulmonary artery debanding and closure of VSD in children was complicated by the correction of the acquired lesions with a significantly high incidence of morbidity and early postoperative deaths. Primary repair is recommended for isolated VSD, if possible.

• 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.

• 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.