• Journal of mucopolysaccharidosis and rare diseases
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• v.4 no.1
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• pp.21-25
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• 2018

Fabry disease is a hereditary lysosomal storage disorder caused by the reduction or absence of lysosomal enzyme alpha-galactosidase A and the accumulation of glycosphingolipids, such as globotriaosylceramide (Gb3), in various organs, including the heart. The prevention of cardiac involvement in Fabry disease can only be achieved by enzyme replacement therapy (ERT), and the method of assessing the efficacy of ERT should be confirmed. Changes in the electrocardiogram, such as the shortening of PQ interval, prolongation of QTc and repolarization abnormalities as well as left ventricular hypertrophy in voltage criteria, can be used to identify Fabry disease patients; however, the usefulness of electrocardiograms for evaluating the efficacy of ERT is limited. The assessment of left ventricular hypertrophy using echocardiography has been established to evaluate the efficacy of ERT during long-term period. A new technique involving speckled tracking method might be useful for detecting early cardiac dysfunction and identifying the effect of ERT for a relatively short period. The estimation of left ventricular hypertrophy using cardiac magnetic resonance (CMR) is also useful for assessing the efficacy of ERT. Identifying late gadolinium enhancement in CMR may affect the effectiveness of ERT, and the new technique of T1 mapping might be useful for monitoring the accumulation of Gb3 during ERT. Histopathology in cardiac biopsy specimens is another potentially useful method for identifying the accumulation of GB3; however, the use of histopathology to evaluate of the efficacy of ERT is limited because of the invasive nature of an endomyocardial biopsy.

• Journal of Drive and Control
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• v.9 no.2
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• pp.39-45
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• 2012

The NAS 1638 cleanliness classification system was originally developed in 1966 by the US Aircraft Industries of America to both simplify reporting of particle count data and to control the introduction of dirt during the assembly of aircraft fluid systems. The numbers of particles at stated sizes are represented by broad bands where the interval was generally a doubling of contamination. A number of systems have been introduced since this to suit differing requirements. NAS 1638 and AS4059 are used in other industrial sectors such as the Off-shore & Sub-Sea and the Primary Metal Industries. The changes to ISO contamination measurement standards controlled by ISO/TC131/SC6 in 1999 meant that a revision of most of these classification systems was necessary. The body responsible for NAS 1638 decided to withdraw it for new installations and replace it with an update of an existing standard, SAE AS 4059. This paper details the philosophy behind the contamination coding systems, the reasons for the changes to the ISO contamination standards and explains the workings of AS 4059, the replacement for NAS 1638. It goes on to detail the latest changes to this standard.

• Journal of Chest Surgery
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• v.29 no.12
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• pp.1337-1341
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• 1996

From September 1989 to March 1996, 13 patients with prosthetic valve thrombosis underwent reoperdtion on 16 occasions. The mean interval between implantation and reoperation was 27.8 months. The anticoagulation status was inadequate in 44% of th Instances. The majority(75%) were in NYHA functional class IV, 6 of them being in shock. Reoperation was performed for valve replacement(15 of 16 occasions) or thrombectomy(1 of 16 occasions) within 3 to 192 hours(mean 33.5 hours). Operative mortality at reoperation was 25%(4 patients). Re-thrombosis occurred In 3 patients. Long-term outcome was satisfactory in all survivors with a mean follow-up of 30.B months. The present results indicate that an early diagnosis and prompt surgical intervention is needed to decrease operative mortality.

• Journal of Chest Surgery
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• v.48 no.5
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• pp.328-334
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• 2015

Background: There have been a number of studies on mitral valve replacement and repeated percutaneous mitral balloon valvotomy for mitral valve restenosis after percutaneous mitral balloon valvotomy. However, studies on mitral valve repair for these patients are rare. In this study, we analyzed postoperative outcomes of mitral valve repair for mitral valve restenosis after percutaneous mitral balloon valvotomy. Methods: In this study, we assessed 15 patients (mean age, $47.7{\pm}9.7years$; 11 female and 4 male) who underwent mitral valve repair between August 2008 and March 2013 for symptomatic mitral valve restenosis after percutaneous mitral balloon valvotomy. The mean interval between the initial percutaneous mitral balloon valvotomy and the mitral valve repair was $13.5{\pm}7years$. The mean preoperative Wilkins score was $9.4{\pm}2.6$. Results: The mean mitral valve area obtained using planimetry increased from $1.16{\pm}0.16cm^2$ to $1.62{\pm}0.34cm^2$ (p=0.0001). The mean pressure half time obtained using Doppler ultrasound decreased from $202.4{\pm}58.6ms$ to $152{\pm}50.2ms$ (p=0.0001). The mean pressure gradient obtained using Doppler ultrasound decreased from $9.4{\pm}4.0mmHg$ to $5.8{\pm}1.5mmHg$ (p=0.0021). There were no early or late deaths. Thromboembolic events or infective endocarditis did not occur. Reoperations such as mitral valve repair or mitral valve replacement were not performed during the follow-up period ($39{\pm}16months$). The 5-year event-free survival was 56.16% (95% confidence interval, 47.467-64.866). Conclusion: On the basis of these results, we could not conclude that mitral valve repair could be an alternative for patients with mitral valve restenosis after percutaneous balloon mitral valvotomy. However, some patients presented with results similar to those of mitral valve replacement. Further studies including more patients with long-term follow-up are necessary to determine the possibility of this application of mitral valve repair.

• The Korean Journal of Pain
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• v.21 no.3
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• pp.211-216
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• 2008

Background: Postoperative pain after bilateral total knee replacement (TKR) is expected to be more severe than unilateral TKR. Intravenous patient-controlled analgesia (IV PCA) is less effective than other methods of pain management especially immediately after an operation even though it is an easily controlled method for managing pain. This study was designed to evaluate the effect of femoral nerve blocks combined with IV PCA after bilateral TKR for postoperative pain control. Methods: The patients in group I (n = 20) were given only IV PCA with morphine and group II (n = 20) were given bilateral femoral nerve blocks with 12 ml of 0.25% bupivacaine and epinephrine 1 : 400,000 before extubation followed by an IV PCA. Main outcome measures included numerical rating pain score, cumulative opioid consumption, hourly dose during each time interval, and side effects. Results: The pain score in group II was significantly lower than that in group I immediately after recovery of awareness and at 3, 6, 12 hours postoperatively. Cumulative opioid consumption was significantly decreased in group II during the first 48 hours postoperatively. The hourly dose in group II was also significantly lower than that in group I until 12 hours postoperatively. There was no difference in side effects between the groups. Conclusions: We concluded that bilateral femoral nerve blocks improve analgesia and decrease morphine use during IV PCA after bilateral TKR.

• Journal of Chest Surgery
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• v.47 no.4
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• pp.373-377
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• 2014

Background: This paper aimed to verify the effects of renal replacement therapy on changing the levels of serum creatinine for different veno-arterial and veno-venous configurations in prolonged extracorporeal membrane oxygenation (ECMO) patients. Methods: The subjects were chosen 71 patients who had undergone more than 1,440 minutes (24 hours) of the therapy from among 117 patients who had undergone ECMO insertion between January 2008 and December 2012. The patients were separated into the veno-arterial configuration group I (51 patients) and the veno-venous configuration group II (20 patients). The difference in the level of serum creatinine (${\Delta}Cr$) between before or just after ECMO insertion ($Cr_I$) and the level when the pump time was between 2,880 and 4,320 minutes ($Cr_F$) was checked (${\Delta}Cr=Cr_F-Cr_I$), and the average ${\Delta}Cr$ for each group was compared using a Student t-test at the confidence interval (CI) of 95%. Results: The change in the level of serum creatinine was an increase of 0.341 mg/dL (${\sigma}$=0.9202) for group I and a decrease of 0.120 mg/dL (${\sigma}$=1.5292) for group II. The change was significantly high for group I (p=0.011, CI=95%). Meanwhile, within group I, when renal replacement therapy was not done, there was a significant increase in the level of serum creatinine (p=0.009, CI=95%). Conclusion: For ECMO insertion patients whose pump time was more than 1,440 minutes, there was a significant change in the level of serum creatinine when renal replacement therapy was not done, for the veno-arterial configuration of group I.

• Journal of Korean Neurosurgical Society
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• v.29 no.5
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• pp.659-663
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• 2000

The motality rate of acute subdural hematoma(ASDH) associated with closed head injury is high in spite of recent advances in neurosurgery. Many variables in regard to outcome of ASDH have been assessed. But among them, intracranial pressure(ICP) control and the time interval between injury and operative evacuation are the only things that can be affected by doctor. We introduced a simple method to the management of ASDH for reducing the time interval between injury and operation. When the immediate decompressive operation of ASDH was impossible by any causes, we made a burr hole at the center of hematoma, usually on 2-3cm above temporal squama and 1-2cm behind coronal suture under local anesthesia before main operation. Partial hematoma evacuation was achieved through the burr hole and it was effective in preventing further worsening of patients neurological status before main operation. Prompt hematoma evacuation through the burr hole seemed to be effective in delaying secondary ischemic brain damage and made easy to closing the dura opening and replacement of the bone flap at the end of main decompressive operation. This easy method may reduce the time interval between injury and operation. We represent surgical technique with two cases of ASDH managed with this simple method.

• Journal of Chest Surgery
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• v.38 no.3 s.248
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• pp.204-213
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• 2005

In spite of the improvement in the quality of artificial heart valves and surgical techniques, the incidence of the complications following valve replacement is still high. We reviewed the clinical results of the valve replacements peformed in Korean University Anam Hospital during the last 26 years. Material and Method: The data of 571 patients who received valve replacement between December 1976 and December 2003 were reviewed. Result: There were 304 cases of MVR which was the most common procedure performed. There were 122 cases of AVR, and 111 cases of AVR with MVR. Among the 47 patients who received redo operation 38 cases were redo cases including 31 cases of MVR. $32.5\%$ of the patients who had tissue valve replacement had second valve replacement with 10.2 $\pm$ 3.9 years interval. $24.3\%$ (139/571) of the patients developed valve related complications and cerebral infarction was the highest in frequency. Atrial fibrillation was related with increased complication rates and the mechanical valve replaced group had higher hemorrhagic complication rate than tissue valve replaced group. The operative mortality was $3.68\%$ and the most common cause of the failure was low output syndrome. The operative mortality was higher in the patient group who had valve replacement before the year 1990. The patient group who had mechanical valve replacement had higher operative mortality rate th;3n the tissue valve group. The 5-year survival rate was $92.2\%$ and 10 year survival rate was $85.7\%$. Conclusion: The operative mortality of valve replacement has been improved. The mechnical valve replaced patients had higher hemorrhagic complication rate than the tissue valve replaced patients and more tissue valve replaced patients received redo valve replacement.

• Environmental and Resource Economics Review
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• v.18 no.3
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• pp.431-455
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• 2009

This study estimates the WTP for the plan of solving deforestation due to limestone mining and calculates that damage cost using contingent valuation method. As the results of analysing a dichotomous choice CV data, the yearly mean WTP per household for the plan is 5,045 won(95% confidence interval : 3,729~6,360 won) in single bounded CV model and is 4,361 won(95% confidence interval : 3,710~5,012 won) in double bounded CV model. The damage cost of deforestation due to limestone mining can be estimated as 55.3% of WTP for the plan which is assigned to restoring the deforestation area to the original or similar alternative state. The average yearly deforestation cost of 1 ha due to limestone mining is reached 20.90 million won(95% confidence interval : 16.53~25.27 million won). And the asset value of that 1 ha damage is 160.02 million won(95% confidence interval : 126.56~193.49 million won). The fact is found that the damage cost of deforestation using replacement cost method is likely to be underestimated. The total damage cost of deforestation because of limestone mining in 3 cities (Donghae, Samcheok, Yeongwol) is 204.0 billion won(95% confidence interval : 161.4~246.7 billion won) which is composed of 26.5% for Donghae, 28.9% for Samcheok, and 44.6% for Yeongwol according to the damage size of deforestation due to limestone mining in 3 cities.

• Journal of Chest Surgery
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• v.26 no.3
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• pp.186-190
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• 1993

During 6 year period from January, 1987 through December, 1992, a total of 17 valvular reoperations [14 mitral and 3 aortic valve reoperations] were performed in 15 patients with previously implanted prosthetic valves. There were 7 men and 8 women, mean age was 35.4 years. Mean time interval was 80 months. Causes for reoperations were prosthetic valve failure, prosthetic valve endocarditis, and periprosthetic leak.3 patients died in hospital. The cause of death were low cardiac output and acute renal failure.