• Childhood Kidney Diseases
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• v.4 no.1
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• pp.48-56
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• 2000

Purpose: Alport SD., the most common herectitary rephriris, is a renal disease with rapid progression. Deafness, ocular abnormalities and a specific EM finding may be associated in addition to a family history. We have aralyged retrospectively. Methods: We observed 12 children with Alport syndrome who were diagnosed at Dept. of pediatrics in Kyunghee Univ., College of Medicine, from Apr. 1991 until Jun. 1999. We used four criteria for diagnosis: renal disease, family history, deafness or eye abnormalities, and a specific finding in electron microscopy Results: 2 of 12 patients had all features of the four diagnostic criteria. We could not trace an exact family history in 3 patients, and 6 patients did not exhibit deafness or eye abnormality. One could not have renal biopsy because offer chronic renal failure. Other three criteria were observed in her. The ratio of male to female observed was 1:2 respectively and the mean age of initial renal symptom was 5.6 years. 9 of 12 patients had a family history of renal disease. In the audiogram and ocular examination for 11 of 12 cases, sensorineural hearing loss was observed in 6 and ocular abnormality in 2 cases. In electron microscopic finding, irregular thickness of the capillary basement membranes with lamination of lamina densa and foot process obliteration was noted in 9 of 11 and thin basement membrane with splitting and foot process obliteration was noted in the other 2. The mean period of follow-up was 3 6/12 years. And one patient developed the chronic renal failure until now and had kidney transplantation. Conclusion: For the diagnosis of Alport syndrome, the following four diagnostic criteria are very important : renal disease, family history, deafness or eye abnormalities, and a specific finding on electron microscopy. We expect that more patients can be detected through the analysis of these characteristics.

• Journal of Chest Surgery
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• v.35 no.4
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• pp.261-266
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• 2002

In an attempt to avoid the adverse effects of the cardiopulmonary bypass, off pump coronary artery bypass grafting(Off pump CABG) that has recently been rediscovered and refined. We compared the preoperative risk factors and in-hospital outcomes of patients done Off pump with those done On pump CABG. Material na Method: One hundred seventy eight patients was underwent CABG between January 2001 and July 2001 12 patients whom underwent associated valvular or left ventricular volume reduction surgery were excluded in this study Data were collected for 52 Off pump CABG and 114 On pump CABG for patient and disease risk factors, extent of coronary disease, and in-hospital outcomes. Result: Off pump CABG and On pump CABG groups did not show any differences in their patient and disease risk factors, and extent of coronary disease. Off pump CABG group had significantly lower mean operation time(234 $\pm$ 37 min vs 290 $\pm$ 48 min, p<0.001), lower mean CK-MB level(10.1 $\pm$ 13.5 IU/L vs 33.1 $\pm$ 18.2 IU/L, p<0.001) and mean ventilation time(14.8 $\pm$ 3.5 hours vs 16.2 $\pm$ 4.9 hours, p=0.048) than On pump CABG groups. On pump CABG group had significantly more distal grafts(3.4 $\pm$ 0.9 vs 2.6 $\pm$ 0.8, p<0.001) than Off pump CABG groups. There were no operative mortality in two groups. Off pump) CABG and On pump CABG groups did not show any differences in their postoperative complications and outcomes including perioperative myocardial infarction, stroke, respiratory failure, renal failure, reoperation, the amount of bleeding, the need of intraaortic balloon pump, the need of inotropics, and the stay of intensive care unit and hospital. Two patients were converted to On pump CABG. Conclusion: This study showed that patients having Off pump CABG are not exposed to a greater risks of adverse outcomes and also provided evidence that patients having Off pump CABG have significantly lower operation time, CK-MB, ventilation time and less distal grafts. Although there may be potential benefits to Off pump CABG, further studies must be directed to determine those patients who would benefit from Off pump CABG.

• THE JOURNAL OF KOREAN ORIENTAL ONCOLOGY
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• v.2 no.1
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• pp.25-42
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• 1996

In order to study the effect of Sikbuntang on the in vitro Cell-cytotoxicity, this study had put through MTT Assay. And to investigate the effects of Sikbuntang on the ICR mice which had Abdominal tumor induced by Sarcoma-180 cell line, C57Bl/6 mice which had pulmonary melanoma induced by B16 cell line. After Sarcoma-180 cell line and B16 cell line were transplanted, the extract of Sikbuntang was orally administered to the mice to observe the extension of survival time of the mice, inhibition of solid tumor, inhibition of pulmonary melanoma metastasis, productivity of Interleukin-2, NK-Activity. The results were summarized as follows : 1. On the MTT assay, in case of $100{\mu}g/ml$ and $10{\mu}g/ml$ of Sikbuntang concentration were inhibited cell viability significantly. But $1{\mu}g/ml$ of Sikbuntang concentration just had tend to inhibit cell viability. 2. In the effect of life extension, Sikbuntang treated group appeared to survive longer than the control group, but which were not significant. 3. In the effect of inhibition solid tumor, Sikbuntang treated group appeared to decrease than the control group, but which were not significant. 4. In the effect of inhibition melanoma pulmonary metastasis, Sikbuntang treated group appeared to inhibit than the control group significantly. 5. In the productivity of Interleukin-2, on 7 and 14 day, Sikbuntang treated group increased than control group significantly. But on 21 day, Sikbuntang treated group had tend to increase with no significance. 6. In the NK-Activity, the ratio of effector cell and target cell. In case which the ratio was 50:1, Sikbuntang treated group showed increase than control group significantly. But in another cases, they showed increase with no significance.

• Journal of Chest Surgery
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• v.38 no.11 s.256
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• pp.761-772
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• 2005

Background: Following the implantation of heart valve prostheses, it is important to maintain therapeutic INR to reduce the risk of thromboembolism. The objective of this study was to suggest a practical dosing guideline for Korean outpatients with prosthetic heart valves managed by a pharmacist-run anticoagulation service (ACS). Material and Method: A retrospective chart review was completed for all patients enrolled in the ACS at Seoul National University Hospital from March, 1997 to September, 2000. Patients who were at least 6 months post-valve replacement and had nontherapeutic INR value (less than 2.0 or greater than 3.0) were included. The data on 688 patients (1,782 visits) requiring dosing adjustment without any known drug or food interaction with warfarin were analyzed. The amount of adjusted dose and INR changes based on the INR at the time of the event were calculated. Aortic valve replacements (AVR) patients and mitral or double valve replacement (MVR/DVR) patients were evaluated separately. Result: Two methods for the warfarin dosage adjustment were suggested: Guideline I (mg-based total weekly dose (TWD) adjustment), Guideline II (percentage-based TWD adjustment). The effectiveness of Guideline 1 was superior to Guideline II overall in patients with both AVR and MVR/DVR. Conclusion: The guideline suggested in this study could be useful when the dosage adjustment of wafarin is necessary in outpatients with mechanical heart valves.

• Journal of Chest Surgery
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• v.40 no.5 s.274
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• pp.341-350
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• 2007

Background: We present here the early and midterm surgical results for infective endocarditis and we especially focus on the effect of aggressive reconstruction or root implantation after wide debridement. Material and Method: Between January 1995 and Jun 2006, we enrolled 79 adult infective endocarditis patients who underwent surgical treatment. There were 63 and 16 native and prosthetic valve endocarditis cases, respectively. They included 27 cases of culture negative endocarditis. With performing valve replacement or repair, 28 of the patient underwent a more aggressive surgical option, for example, aortic root replacement or reconstruction, or heart base reconstruction etc. Result: There were statistical relationships between the in-hospital mortality and staphylococcal infection, urgent-based operation and operation during the active phase of endocarditis. Wide debridement and aggressive reconstruction were not related to either the post operative mortality or the early morbidity. Culture negative endocarditis was not related to the postoperative mortality and morbidity. Conclusion: Physicians must pay attention to patients' medical treatment during the preoperative period of the infective endocarditis. If surgery is considered for treating infective endocarditis, it should be performed before the downhill course of the disease so that the surgical outcome is improved. Wide debridement and more aggressive reconstruction are also warranted.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.247-254
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• 2007

Purpose : Continuous renal replacement therapy(CRRT) has been the first choice for the treatment of acute renal failure in critically ill children not only in western countries but also in Korea. However, there are very few studies that have analyzed the outcome and prognosis of this modality in Korean children. We performed this study to evaluate the factors associated with the outcome and prognosis of patients treated with CRRT. Methods : We retrospectively reviewed the medical records of 32 children who had received CRRT at Severance hospital from 2003 to 2006. The mean age was 7.5 years(range 4 days-16 years) and the mean body weight was 25.8 kg (range 3.2-63 kg). Results : Eleven(34.4%) of the 32 patients survived. Bone marrow transplantation and malignancy were the most common causes of death and underlying disease leading to the need for CRRT Mean patient weight, age, duration of CRRT, number of organ failures, urine output, estimated glomerular filtration rate(eGFR), C-reactive protein, and blood urea level did not differ significantly between survivors and nonsurvivors. (1) Pediatric risk of mortality(PRISM) III score at CRRT initiation($9.8{\pm}5.3$ vs. $26.7{\pm}7.6$, P<0.0001), (2) maximum pressor number ($2.1{\pm}1.2$ vs. $3.0{\pm}1.0$, P=0.038), and (3) the degree of fluid overload($5.2{\pm}6.0$ vs. $15.0{\pm}8.9$, P=0.002) were significantly lower in survivers than in nonsurvivors. Multivariate analysis revealed that fluid overload was the only independent factor reducing survival rate. Conclusion : CRRT was successfully applied to the treatment of acute renal failure in a wide range of critically ill children. To improve survival, we suggest the early initiation of CRRT to prevent the systemic worsening and progression of fluid overload in critically ill children with acute renal failure. (J Korean Soc Pediatr Nephrol 2007;11:247-254)

• Journal of Chest Surgery
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• v.30 no.4
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• pp.383-389
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• 1997

From March, 1992 to March, 1996, a total of 279 patients underwent coronary bypass surgery at the Sejong General Hospital, Puchon. We selected 22 patients with severe left ventricular(LV) dysfunction from them. The criteria were the presence of global or segmental abnormalities of left ventricular contraction and LV ejection fraction(EF) less than 35% based on biplane LV angiography by planimetry method. The mean age of 17 male and 5 female patients was 60$\pm$5.6years(range:47~73 years). All had the anginas, which were Canadian class II in 6, class 111 in 12 and class IV in 4. All patients except one had the history of previous myocardial infarction more than once. Seven of them had the symptoms and signs of congestive heart failure, such as dyspnea on excertion and increased pulmonary vascular markings. Their mean LVEF was 29.4$\pm$4 5%(range : 18~35%) and mean LV end-diastolic pressure was 18.7 $\pm$8. 2mmHg(range:10~42mmHg). 21 patients had 3 vessel-disease and 1 had 2 vessel-disease. Complete revascularization was tried with the use of 16 internal mammary arteries and 60 sapheuous veins and 3 radial arteries grafts. The mean number of distal anastomosis was 3.5$\pm$ 1.1. Concomitantly, one mitral valvuloplasty and annuloplasty was performed in the patient with moderate mitral regurtigation. The hospital mortality was 4.5%. During the follow-up, there were 3 late deaths. Of 18 survivors, 2 patients were lost in follow-up 24 and 27 month respectively after operation and the remaining 16 patients have bcen followed up with an average of 30.4 $\pm$ 13.4 months.15 patients had improvement with respect to angina but 8 patients still have the continuing or progressing heart failure. The 1-year, 2-year and 3-year actuarial survival rate was 85.2, 69.1, 46.1%, respectively. This study indicates that coronary artery bypass sur ery can be performed in the patients with severe LV dysfunction at acceptable risk but does not greatly contribute to the improvement of congestive heart failure.

• Journal of Chest Surgery
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• v.34 no.12
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• pp.901-908
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• 2001

Background : Coronary artery bypass graft(CABG) in patients with advanced left ventricular dysfunction has often been regarded as having high mortality rate, despite the great improvement in operative result of CABG. With recent advances in surgical technique and myocardial protection, surgical revascularization improved the symptom and long-term survival of these high risk patients more than the medical conservative treatment. Material and Methold : Clinical data of 31(4.1％) patients with preoperative ejection fraction less than 30% among 864 CABGs performed between January 1995 and March 1999 were retrospectively analyzed and pre- and postoperative changes of the ejection fraction on echocardiography were analyzed. There were 26 men and 5 women. The mean age was 60.7 years(range 41 to 72 years). History of myocardial infarction(30 cases, 98％) was the most common preoperative risk factor. There were seven irreversible myocardial infarction on thallium scan. Most patients had triple vessel diseases(26 cases, 84％) and first degree of Rentrop classification(16 cases, 52％) on coronary angiography. The mean number of distal anastomosis during CABG was per patient was 4.9${\pm}$0.8 sites in each patient. In addition to long saphenous veins, the internal mammary artery was used in 20 patients. Total bypass time was 244.7${\pm}$3.7 minutes(range, 117 to 567 minutes), and mean aortic cross-clamp time was 77.9 ${\pm}$ 1.6 minutes(range, 30 to 178 minutes). There were five other reparative procedures such as two left ventricular aneurysrmectomy, two mitral repair, and one aortic valve replacement. There were twelve postoperative complications such as three cardiac arrhythmia, two bleeding(re-operation), one delayed sternal closure, eleven usage of intra-aortic balloon counterpulsation for low cardiac output. Two patients died, postoperative mortality was 6.5% . Twenty-nine patients were relieved of chest pain and left ventricular ejection fraction after operation was significantly higher(38.5${\pm}$11.6%, p 0.001) as compared with preoperative left ventricular ejection fraction(25.3${\pm}$2.3%). The follow up period of out patient was 25. 3 months. Conclusion: In patients with coronary artery disease and advanced left ventricular dysfunction, coronary artery bypass grafting can be performed relatively safely with improvement in left ventricular function, but it will be necessary to study long term results.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.31-37
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• 2017

Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is an autosomal recessive disorder of the urea cycle that causes hyperammonemia. Two forms of CPS1D are recognized: a lethal neonatal type and a less severe, delayed-onset type. Neonatal CPS1D cases often present their symptoms within the first days of life. Delayed-onset cases are predominantly adolescents or adults, and infantile delayed-onset cases are rare. Severe hyperammonemia in the neonatal period leads to serious brain damage, coma, and death if not treated promptly. Therefore, early diagnosis and acute treatment are crucial. Despite the improvement of treatments, including continuous hemodialysis, ammonia-lowering agents, and a low-protein diet, the overall outcome of severe forms of hyperammonemia often remains disappointing. As the liver is the only organ in which ammonia is converted into urea, liver transplantation has been considered as an elegant and radical alternative therapy to classical dietary and medical therapies. However, liver transplantation has many disadvantages, such as a considerable risk for technical complications and perioperative metabolic derangement, especially in neonates. Additionally, there is a lack of suitable donor organs in most countries. According to recent studies, liver cell transplantation is a therapeutic option and serves as a bridge to liver transplantation. Here, we report a Korean CPS1D patient with novel mutations in CPS1 who was treated by liver cell transplantation after being diagnosed in the neonatal period and showed a good neurodevelopmental outcome at the last follow-up at six months of age.

• Journal of Embryo Transfer
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• v.19 no.2
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• pp.81-87
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• 2004

This study was designed to determine whether repeated superovulation is beneficial for recovery and quality of oocytes in Korean native goats. Seventy-six mature goats, maintained in a pen under natural day length and fed hay ad libitum, were pretreated with progestagen impregnated CIDR for 10 days and then the goats were divided into two groups. One group of the goats received a single intramuscular injection of 1,000 IU PMSG on Day 8 of CIDR insertion. The other group of the goats received twice daily intramuscular injections of a total of 70 mg FSH for 3 days from Day 8 of CIDR. All the gonadotropin treated goats were injected with 10 mg $PGF2{\alpha}on$ Day 8 and 400 IU hCG in the afternoon on Day 10. For oocyte recovery, donor goats were fasted 24 h before operation. Anesthesia was induced by intravenous injection of 2% xylazine(0.2 mg/kg body weight) and ketamin(11 mg/kg body weight). In vivo oocytes were recovered by follicle aspiration or oviduct flushing at 35 to 40 hours after hCG injection through mid-ventral incision. The mean number of CL and oocytes recovered and recovery rate of oocytes by oviduct flushing were greater(P<0.05) in the first treatment than those in the second treatment. Contrary to our assumption, PMSG treatment significantly (P<0.05) increased the number of CL formed and recovery rate of oocytes compared to FSH. However, the same effect was not observed in recovery of follicular oocytes. There was no significant difference in oocyte quality between FSH and PMSG or first and second treatments. The present results indicate that repeated superovulation and repeated use of donor animals may be inefficient for obtaining oocytes in good qualities.