• Clinical and Experimental Pediatrics
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• v.51 no.7
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• pp.736-741
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• 2008

Purpose : The incidence of thromboembolic episodes in children with nephrotic syndrome (NS) is low; however, these episodes are often severe. Moreover, both pulmonary thromboembolism (PTE) and renal vein thrombosis (RVT) rarely show clinical symptoms. This study was performed to determine the benefits of routine screening in the detection of thrombosis in childhood NS. Methods : Among 62 children with nephrotic syndrome, a total of 54 children (43 males, 11 females) were included in this study. When the patients experienced their first NS episode, we performed renal Doppler ultrasonography in order to detect RVT. To rule out the possibility of PTE, a lung perfusion scan was performed. Computed tomographic (CT) pulmonary angiography was recommended to patients who showed possible signs of PTE. All patients were evaluated for clinical signs of thrombosis, biochemical indicators of renal disease, as well as clotting and thrombotic parameters. Results : RVT or related clinical symptoms were not observed in any children. Based on the findings of the lung perfusion scans, 15 patients (27.8%) were observed with as a high probability of PTE. We were able to perform a CT pulmonary angiography only on 12 patients, and 5 patients were diagnosed with PTE (prevalence 8.1%). The serum fibrinogen level in the group with PTE was significantly higher ($776.7{\pm}382.4mg/dL$, P<0.05) than that in the group without PTE, and other parameters were not significantly different between each group. Conclusion : Further studies are required for clarifying the role of renal Doppler ultrasonography for the detection of RVT in NS. Children with NS who developed non-specific respiratory symptoms should be evaluated for the diagnosis of PTE. In the management of NS, a lung perfusion scan should be performed at the time of the initial episode of NS regardless of the pulmonary symptoms, since patients having PTE are either often asymptomatic, or present with nonspecific symptoms.

• Journal of radiological science and technology
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• v.31 no.2
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• pp.161-169
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• 2008

Since measuring the size of kidney with sonography becomes an important index for diagnosis, treatment, and prognostic prediction in kidney disease, the accurate measurement and evaluation on this are clinically very important. Accordingly, the purpose of this study was to increase reproducibility and objectivity in measuring the size of kidney by enumerating factors that have an impact for measurement. It targeted 44 adults in Korea at the age of 21-27. It measured in order for both kidneys to be seen most largely while changing a subject-examiner's position in a state of fasting for 8 hours and a transducer's approaching direction. It compared a size of kidney by measuring, respectively, with the same method in 30 minutes and in 1 hour after drinking water in 700-1,000cc. In case of the lateral approach scan in decubitus position, the average length of the kidney both to the right and the left and the deviation of measurement to be the largest. In NPO(None Per Oral) state, the average length in the right kidney was 10.19cm, and the average length in the left kidney was 10.33cm. In 60 minutes after taking moisture, the average length in the right kidney was 10.94cm, and the average length in the left kidney was 11.13cm. In comparing the average length of the kidney in NPO state and its average length in 60 minutes after taking moisture, the size swelled by 7.3% for the length in the right kidney and by 7.7% in the left, thereby having been indicated to be statistically significant(P<0.003). The measurement in a size of kidney by using ultrasound may be measured differently depending on a patient's state of taking moisture and a transducer's approaching direction. It is thought that when the measurement in a size of kidney is especially important clinically, the intake and intake time in moisture need to be considered and that measuring with the posterior approach in prone position is a good method aiming to increase reproducibility in measuring length of the kidney.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.167-174
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• 2005

Purpose : Relapses are a major problem in children with steroid responsive nephrotic syndrome(SRNS). This study has been performed to determine the predictive factors for relapse in children with SRNS. Methods : The study group consisted of 7,3 children with SRNS who had been admitted to the Department of Pediatrics, Kyungpook National University Hospital, over 6 years from 1996 to 2001. The medical records were reviewed retrospectively and analyzed to determine significant relationships between selected variables[age at onset, sex, laboratory data, the rapidity of response(days to remission), interval to first relapse] and the frequency of relapse. Results : The age($mean{\pm}SD$) of patients was $4.53{\pm}2.53$ years old. The male to female ratio was 52:21. In 95$\%$, 39 out of the 41 children had a renal biopsy, and the final diagnosis was minimal change nephrotic syndrome. There was no significant correlation between the frequency of relapse and the following variables age at onset, sex, and presence of hematuria. However, the rapidity of response correlated well with the frequency of relapse, especially during the first year after the onset of the disease(P=0.005). Conclusion : The rapidity of response is expected to be one of the predictive (actors for relapse in children with SRNS. (J Korean Soc Pediatr Nephrol 2005;9:167-174)

• Journal of Chest Surgery
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• v.39 no.12 s.269
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• pp.900-905
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• 2006

Background: Homografts and bioprostheses are most commonly used for Rastelli operation in congenital heart disease, but the limited durability is responsible for multiple reoperations associated with increased morbidity This study evaluated long-term results after Rastelli operation with a mechanical valved conduit. Material and Method: A total of 20 patients underwent Rastelli operation with mechanical valved conduit from January 1990 to July 1992. Operative mortality was 1 of 20 patients, and a retrospective review of 19 patients(10 males, 9 females) was done. Initial diagnosis was congenitally corrected transposition of great arteries(cc-TGA, n=4), complete TGA (n=2), ventricular septal defect with pulmonary atresia(VSD with PA, n=9), truncus arteriosus(n=2), double outlet right ventricle with pulmonary stenosis(DORV with PS, n=2). The mean age at Rastelli operation was $4.6{\pm}3.4$ years, and mean follow-up period was $12.8{\pm}2.7$ years. Patients underwent Rastelli opearation using 16 CarboMedics mechanical valve, and 3 Bjork-Shiley mechanical valve($17{\pm}2$ mm). Result: There were 15 reoperations for failed mechanical valved conduit. The freedom from reoperation at 5 and 10 years was 53% and 37%. Most patients were received oral anticoagulation with warfarin, and maintained the international normalized ratio(INR) of 1.5 to 2.0. There was no anticoagulation or thromboembolism related complication. There was a significant difference in the causes of a conduit failure between early(within 3 years) and late(after 3 years) failure groups. The six patients reported early prosthetic valve failure, mainly due to valvular dysfunction by thrombosis or pannus formation. The other nine patients reported late prosthetic valve failure, mainly due to dacron conduit stenosis at anastomosis sites, whereas their valvar motion was normal except 1 patient. Conclusion: To avoid early prosthetic valve failure, strict anticoagulation therapy would be helpful. About the late development of obstructive intimal fibrocalcific peels within the Dacron conduit, an improvement of conduit material is necessary to reduce late prosthetic valve failure. In selected patients, the long term results were satisfactory.

• Journal of Chest Surgery
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• v.30 no.3
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• pp.282-286
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• 1997

In the era of coronary artery bypass grafting, the intraaortic balloon pump (IABP) is more widely used and its indication has been ex anded. We perf'orbed retrospective clinical analysis on the patients who have received IABP pre andfor postoperatively during the course of CABG. From January 1981 to June 1995, total 322 patients have received CABG at the Seoul National University Hospital and among them 50 patients (15.5%) were supported by IABP during the course of the operation. The mean age at the time. of the operation was 57.2 years (39∼ 75 years) and the male to female ratio was 33 : 17. The preoperative diagnosis was unstable angina in 33 (66%), stable angina in 7 (14%) and postinfarct angina in 8 patients(16%). As for the indications of the IABP, there were 13 cases(26%) with left main disease, 13 (26%) with class IV angina, 12 (24%) with difficulty in CPB weaning, 6 (12%) with postinfarct angina and 3 (6%) with severe LV dysfunction. In the remaining 3 cases, one patient was operated on after PTCA failure in emergency basis, another was a patient with AMI, and the other was one who had postoperative low c rdiac output syndrome. All IABPS were introduced via femoral artery and among them 45 cases (90%) percutaneously. The mean postoperative assist time was 22.3 hours (0.5 ∼ 168 hours) and IABP could be removed within 48 hours in most of them (44150). The operative mortality was 6.1% (3 cases) and postoperative morbidity was only one with lower extremity ischemia. The more general application of the IABP during the course of the CABG ,especially in patients with high preoperative risk factors or difficulty in CPB weaning is a good measure of protecting and recovering myocardial function with minimal risk.

• Journal of Chest Surgery
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• v.42 no.1
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• pp.72-78
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• 2009

Background: Mediastinal neurogenic tumors are generally benign lesions and they are ideal candidates for performing resection via video-assisted thoracoscopic surgery (VATS). However, benign neurogenic tumors at the thoracic apex present technical problems for the surgeon because of the limited exposure of the neurovascular structures, and the optimal way to surgically access these tumors is still a matter of debate. This study aims to clarify the feasibility and safety of the VATS approach for performing surgical resection of benign apical neurogenic tumors (ANT). Material and Method: From January 1996 to September 2008, 31 patients with benign ANT (15 males/16 females, mean age: 45 years, range: 8~73), were operated on by various surgical methods: 14 VATS, 10 lateral thoracotomies, 6 cervical or cervicothoracic incisions and 1 median sternotomy. 3 patients had associated von Recklinhausen's disease. The perioperative variables and complications were retrospectively reviewed according to the surgical approaches, and the surgical results of VATS were compared with those of the other invasive surgeries. Result: In the VATS group, the histologic diagnosis was schwannoma in 9 cases, neurofibroma in 4 cases and ganglioneuroma in 1 case, and the median tumor size was 4.3 cm (range: 1.2~7.0 cm). The operation time, amount of chest tube drainage and the postoperative stay in the VATS group were significantly less than that in the other invasive surgical group (p<0.05). No conversion thoracotomy was required. There were 2 cases of Hornor's syndrome and 2 brachial plexus neuropathies in the VATS group; there was 1 case of Honor's syndrome, 1 brachial plexus neuropathy, 1 vocal cord palsy and 2 non-neurologic complications in the invasive surgical group, and all the complications developed postoperatively. The operative method was an independent predictor for postoperative neuropathies in the VATS group (that is, non-enucleation of the tumor) (p=0.029). Conclusion: The VATS approach for treating benign ANT is a less invasive, safe and feasible method. Enucleation of the tumor during the VATS procedure may be an important technique to decrease the postoperative neurological complications.

• Journal of Chest Surgery
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• v.42 no.1
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• pp.46-52
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• 2009

Background: Vascular injuries to the extremities are potentially devastating and they can lead to limb loss and mortality if they are not appropriately managed. The vascular trauma caused by traffic and industrial accidents has recently increased according to the developing industry and transport system in Korea. Early recognition and treatment of these injuries are mandatory to achieve satisfactory outcomes. Material and Method: We retrospective reviewed 43 patients with vascular injuries that were due to blunt and penetrating trauma and they underwent emergency operations from January of 1998 to December of 2006. Result: There were 38 men and 5 women patients with a mean age of $42.0{\pm}16.8$ years (range: 17~77). The cause of vascular injuries were 28 traffic accidents (65%), 6 industrial accidents (14%), 6 glass injuries (14%) and 3 knife injuries (7%). The average time from admission to the operating room was $319.0{\pm}482.2$ minutes (range: 27~2,400 minutes). The average time from admission to discharge was $53.1{\pm}56.0$ days (range: 2~265 days). The anatomic injuries included the femoral artery in 16 cases (37%), the popliteal artery in 8 cases (19%), the brachial artery in 8 cases (19%), and the subclavian and axillary arteries in 7 cases (16%). The associated injuries were 23 bone fractures (53%), 18 muscle injuries (42%) 5 nerve injuries (12%) and 11 vein injuries (26%). The operation methods were 20 end to end anastomoses (46%), 16 interposition grafts (36%), 2 repairs with using patches (5%) and 5 others (12%). The number of amputations and cases of mortality were 3 cases (7%) and 4 cases (9%), respectively. Conclusion: Minimizing ischemia is an important factor for maximizing salvage of extremities. Prompt diagnosis and treatment can reduce the amputation and mortality rates.

• Clinical and Experimental Pediatrics
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• v.48 no.8
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• pp.871-876
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• 2005

Purpose : Recently, it was reported that tumor necrosis factor(TNF) and $lymphotoxin-{\alpha}$($LT-{\alpha}$) gene regions might be a susceptible loci to type 1 diabetes in Japanese. The purpose of this study was to investigate the association of TNF and $LT-{\alpha}$ gene polymorphisms with disease susceptibility in Korean children with type 1 diabetes. Methods : Forty-nine Korean children with type 1 diabetes(29 girls and 20 boys) and 94 healthy Koreans were investigated in this study. Genotyping for -857T/C polymorphism in the TNF promoter region and $LT-{\alpha}$ gene polymorphism were performed by PCR-RFLP(restriction fragment length polymorphism). TNF promoter -1031C/T polymorphism was detected by allele-specific PCR. Results : The distribution of the -857T/C and -1031C/T genotype in the TNF promoter region was not different between diabetic children and the controls. The frequency of TT genotype in the distribution of TNF -1031C/T polymorphism in diabetic children with diabetic ketoacidosis(DKA) at diagnosis was significantly lower than those without DKA(P<0.05). No significant difference in the distribution of $LT-{\alpha}$ gene polymorphism was observed between diabetic children and the controls. There was no association between clinical characteristics of type 1 diabetes and $LT-{\alpha}$ gene polymorphisms. Conclusion : These results suggest that TNF promoter -857T/C and $LT-{\alpha}$ gene polymorphisms are not associated with susceptibility to type 1 diabetes in Korean children. TNF promoter -1031C/T polymorphism might be related to clinical manifestations(DKA) of type 1 diabetes.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.299-306
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• 2008

Purpose : Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by fever, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow and other tissues. In this study, we investigated the clinical manifestations and prognostic factors in patients with HLH. Methods : We retrospectively analyzed the data from 29 patients who were diagnosed whit HLH in the Severance Children's Hospital from Jan. 1996 to Feb. 2007. Results : The median age at diagnosis was 3.8 years (range 0.1-12.2). The ratio of male to female patients was 1.1:1. The 5-year overall survival rate was 55.2% with a median follow-up duration of 32 months. In a multivariate analysis, the duration of fever before admission (survival vs. non-survival, 6.5 days vs. 14 days, P=0.010), the interval from the day of fever onset to the day of initiation of etoposide (survival vs. non-survival, 10 days vs. 35 days, P=0.002) and the presence of neurologic symptoms (survival vs. non-survival, 1 case vs. 7 cases, P=0.010) were independent, poor prognostic factors of HLH. EBV infection, gender, and the level of serum ferritin had no relations to the poor prognosis of the disease. Conclusion : This study showed that the presence of neurologic symptoms and a longer duration of fever were related to a poor prognosis. Therefore, if a patient develops neurologic symptoms and the duration of fever is prolonged, a prompt diagnostic approach and aggressive treatment for HLH are necessary.

• Clinical and Experimental Pediatrics
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• v.50 no.9
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• pp.891-895
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• 2007

Purpose : Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system and mostly develops after viral illness or vaccinations. We investigated the clinical differences and neurologic outcomes according to the distribution of the lesions on brain MRI. Methods : The study group was composed of 21 patients from January 1995 to August 2003 in Kyunghee University hospital. We grouped the patients according to the MRI findings as follows. Group I (14 cases): Multi- or unifocal lesions only in the cerebral white matter. Group II (7 cases): lesions in the gray matter with or without white matter involvement. Results : 1. Preceding events were as follows: no defined prodrome (38.1%), upper respiratory tract infection (28.6%), nonspecific febrile illness (19.0%), gastointestinal disturbance and vaccination. 2. Presenting symptoms were as follows: seizures (76.2%), headache/vomiting (47.6%), altered consciousness (38.1%), hemiparesis, cerebellar ataxia, visual disturbance and facial nerve palsy. 3. Laboratory findings were as follows: CSF pleocytosis (76.2%), leucocytosis (38.1%) and elevated CSF protein (28.6%). 4. Fifteen patients were recovered completely without neurological sequelae. Three patients in group I and 1 patient in group II had intractable seizures. Two patients in group I and 2 patients in group II had motor disturbance. Conclusion : There were no statistically significant differences in preceding events, presenting symptoms, and neurological outcomes according to the distribution of the lesions on brain MRI. However, the ADEM have quite diverse clinical manifestations and neuroimage findings. MRI plays an important role in making diagnosis of the patients who are suspected of ADEM.