• Childhood Kidney Diseases
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• v.13 no.2
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• pp.146-152
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• 2009

Purpose : We performed this study to evaluate the incidence and clinical significance of antiphospholipid antibodies (aPL Ab) in Korean children with Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods : The medical records of 62 patients (31 boys and 31 girls) aged $46.0{\pm}3.1$ (1-16) years with a clinical diagnosis of HSP based on the EULAR/PReS criteria were reviewed retrospectively. From the years 2007 to 2009, the sera from children with acute HSP were tested for aPL Ab such as LA, anti-cardiolipin antibody and anti-${\beta}_2$ glycoprotein I antibody. Results : LA was positive in 18 (29%) of the 62 patients with HSP and We divided the patients into the two groups LA positive group (N=18) and LA negative group (N=44). There were no significant differences between the two groups with regard to abdominal pain, arthralgia and renal involvement, but LA positive group had significantly higher C-reactive protein ($4.3{\pm}7.2$ mg/dL vs. $1.3{\pm}1.8$ mg/dL, P=0.035), erythrocyte sedimentation rate ($37.5{\pm}26.2$ mm/hr vs. $25.1{\pm}22.6$ mm/hr, P= 0.039), IgM ($148.1{\pm}48.4$ mg/dL vs. $114.9{\pm}41.5$ mg/dL, P=0.024), C3 ($143.1{\pm}21.9$ mg/dL vs. $129.7{\pm}24.5$ mg/dL, P=0.048) and C4 levels ($30.9{\pm}6.3$ mg/dL vs. $24.9{\pm}7.8$ mg/dL, P=0.002) compared with LA negative group. Conclusion : We found that the incidence of positive aPL Ab tests was relatively higher in Korean children with HSP and the presence of aPL Ab was associated with acute inflammatory process of HSP. These results suggest that the aPL Ab are involved in the pathogenesis of HSP in children.

• Childhood Kidney Diseases
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• v.17 no.1
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• pp.19-24
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• 2013

Purpose: Recently, enterococcus spp. have become one of the most common nosocomial pathogens with increasing rates of multi-drug resistance. However, study on enterococcal urinary tract infections (UTIs) in children is very limited, especially community acquired UTIs. We studied the clinical characteristics of enterococcus spp. in community acquired UTIs and antibiotic resistance within our urban area. Methods: All children with first episode of community acquired UTIs due to enterococcus spp. and Echerichia coli who were admitted in Pusan National University Children's Hospital between January 2010 and January 2013 were included in our study. We retrospectively reviewed their medical records. Results: During the study period, 201 patients were identified to have first episode of community acquired UTIs. 154 cases were E.coli UTIs (76.6%) and 11 cases were enterococcal UTIs (5.5%) and all enterococcus spp. were Enterococcus feacalis. In enterococcal UTI group, voiding cystourethrogram(VCUG) was performed in 7/11 patients and demonstrated 4 vesicoureteral refluxes (VURs) with renal scar and 3 patients underwent corrective surgery. In E.coli UTI group, VCUG was performed in 121/154 patients and demonstrated 23 VURs and 11 patients underwent corrective surgery. Enterococcal group had significant high rate of underlying urinary abnormalities and surgical corrections compared with E. coli group. All enterococcus spp. were susceptible to ampicillin, vancomycin and linezolid, but all were resistant to tetracycline. They also showed 71.4% resistance to trimethoprim-sulfamethoxazole and 20% resistance to ciprofloxacin. Conclusion: Community acquired enterococcal UTIs in children were rare within our urban area. However, they could be indicative of severe underlying urinary tract abnormalities.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.83-91
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• 2007

Purpose : Childhood primary VUR is generally diagnosed after urinary tract infection, is more prevalent among girls and has a low spontaneous resolution rate in cases of severe VUR. The aim of the present study is to examine the age and gender-related characteristics and the spontaneous resolution rate of infantile primary VUR. Methods : The medical records of 96 infants with primary VUR, diagnosed after their first UTI, were retrospectively reviewed(1995-2004). The clinical characteristics including gender, the degree of VUR and presence of renal scars were evaluated. The spontaneous resolution rate and contributing factors were also analyzed Results : Infantile primary VUR was more prevalent in males than females. The percentage of atrophic scarred kidney was significantly higher in males than females(17.2% vs 3.4%) (P<0.05). The cumulative spontaneous resolution rate in 3 years was very high(89.1%), and was not significantly different between gender and among VUR grades. But in the first year, the spontaneous resolution rate of severe refluxing ureters was significantly higher in males than in females(46.2% vs 7.1%)(P<0.05) and the spontaneous resolution rate of refluxing ureters with no scarred kidneys was significantly higher than those associated with atrophic scarred kidneys(76.6% vs 20%)(P<0.05). Conclusion : Infantile Primary VUR was more prevalent among males and tends to be associated with atrophic scarred kidneys In male infants. The cumulative spontaneous resolution rate in 3 years was very high, even in high-grade VUR and associated atrophic scarred kidneys. In infantile primary VUR, surgery should be withheld even in infants with high-grade VUR with atrophic scarred kidneys.

• Journal of Chest Surgery
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• v.33 no.7
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• pp.547-551
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• 2000

Background; The purpose of this study was to analyze the early and midterm results of Cabrol technique to assist in making future decisions on a more adequate technique for repairing aortic root diseases. Material and Method; From August 1993 to July 1999, we performed Cabrol technique in 18 patients ; 12 annuloaortic ectasia, 6 Stanford type A aortic dissection. Male and female ratio was 11;7, mean age was 46.9$\pm$12.3 years and mean follow up period was 22.5$\pm$21.5 months. We analysed the factors influencing postoperative complications and early mortality. The factors were old age(>60 years), high NYHA(>III), preoporative concomitant disease, urgency of operation, concomitant procedure, long pump preoperative concomitant disease, urgency of operation, concomitant procedure, long pump time(>200 minute), and hospital stay time (>30 days). Result; Operative mortality was 11.1%, late mortality was 11.1%, and overall mortality was 22.2%. The causes of operative death were a heart failure and an arrhythmia. The causes of late death were an acute myocardial infarction and an unknown etiology. Postoperative complications were bleeding, wound infection, toxic hepatitis, acute renal failure, and cerebral infarction. The factors influencing postoperative complications were hihg MYHA Fc(>III) (p=0.044), concomitant disease (p=0.044), long pump time(>200 minute)(p=0.015), and concomitant procedure(p=0.004). There were no significant factors influencing early mortality. Conclusion; The lower postoperative bleeding rate and no complication related to tension of anastomosis after Cabrol technique warrant its consideration in patients requiring aortic root replacement, especially without feasible mobilization of coronary arteries. However, to confirm the graft thrombosis, a more detailed study including periodic angiography will be required.

• Pediatric Infection and Vaccine
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• v.12 no.1
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• pp.67-74
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• 2005

Purpose : The incidence of mortality associated with respiratory difficulties is decreasing nowadays contributed to the development of neonatology. However, complications associated with mechanical ventilator are increasing. This study is to determine clinical manifestations, diagnositc availability of the endotracheal tip culture in patients with Ventilator-Associated Pneumonia(VAP) in neonatal intensive care unit(NICU). Methods : A retrospective analysis of 50 neonates who were admitted to the NICU of Kangnam Sacred Heart Hospital and had given mechanical ventilator from 1 January 2000 to 30 June 2003. VAP group defined as neonates who had pneumonia with mechanical ventilation longer than 48 hours. They were classified into VAP group(n=13) and control group (n=37) and the prevalence, microorganisms cultured from the endotracheal tube tip and risk factors were investigated. Results : The prevalence of VAP was 26.0%(n=13) and the most dominant microorganism cultured in our NICU was methicillin-resistant coagulase negative staphylococcus(MR-CNS) in 4 cases. Other microorganisms were Pseudomonas, Enterobacter, methicillin-resistant Staphylococcus aureus(MRSA) and Klebsiella. Gestational age, birth weight, Apgar score, respiratory distress syndrome, retinopathy of prematurity, bronchopulmonary dysplasia, sepsis, renal failure, pulmonary hemorrhage, pneumothorax were not different significantly between two groups except intraventricular hemorrhage(P<0.001) and patent ductus arteriosus(P<0.05). Duration of hospital stay and mortality rate were also not different significantly. Conclusion : VAP occurred at a significant rate among mechanically ventilated NICU patients. Despite of limitation of encotracheal tip culture, the most common microorganism was MR-CNS. We should be aware of occurrence of VAP in NICU neonate who were with mechanical ventilator and should treat with great care.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.109-116
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• 1997

Purpose : Chronic renal failure is often accompanied by severe dyslipidemia, a known risk factor for cardiovascular disease. Lipoprotein(a) [Lp(a)] has recently been characterized as a risk factor for atherosclerosis and thrombosis. Cardiovascular disease is the leading cause of death in adult patients on dialysis. However, there are only limited data available concerning risk factors for atherosclerosis in uremic children. We have measured serum levels of lipids, lipoproteins, apolipoproteins and Lp(a) in uremic children with maintenance dialysis. Methods : Ten uremic children with hemodialysis (HD) and 14 with peritoneal dialysis (PD) in our dialysis unit were included in this study. The mean age of HD patients was $162{\pm}59$ months and the male to female ratio was 7:3. The mean age and sex ratio of PD patients were $123{\pm}63$ months and 6:8, respectively. The levels of cholesterol, triglyceride, lipoproteins, apolipoproteins and Lp(a) were measured from serum sampled after 14 hours of fasting. The normal control levels were cited from 2 articles presenting the normal blood lipid and lipoprotein levels of primary school and middle school children in Korea. Results : There was no difference in age, sex ratio, body mass index and duration of dialysis between the HD and the PD group. The serum concentration of the cholesterol, triglyceride and apolipoprotein B were significantly elevated in dialysis patients compared with normal subjects. The serum level of Lp(a) was significantly elevated in only PD group. The serum Lp(a) level was below 30 mg/dl in 13 and above 30 mg/dl in 11 patients. The serum albumin level was significantly decreased in high Lp(a) group than in low Lp(a) group. Conclusion : The uremic children receiving dialysis reveal abnormal serum lipid and lipoprotein profiles. These results suggest that they have a higher risk for coronary heart disease, although there has been no clinical evidence of coronary heart disease at present. A long-term follow-up study of these children to clarify the suggestion should be started now.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.178-184
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• 2007

Purpose : Since the first report by Mendoza in 1990, there have been several studies reporting that long-term intravenous methylprednisolone(MP) pulse therapy combined with cyclosporin A(CsA) or cyclophosphamide might be beneficial for the treatment of steroid resistant focal segmental glometulosclerosis(FSGS). We investigated the therapeutic effect of long-term MP pulse therapy without CsA or cyclophosphamide on steroid resistant FSGS. Methods : The medical records of the 10 steroid resistant FSGS patients who were treated with MP pulse therapy by the Mendoza protocol without CsA or cyclophosphamide in our hospital were retrospectively reviewed. Results : The median age at onset was 2.6 years(range 1.1-10.6 years) and the median age at the initiation of therapy was 5.7 years(range 1.8-20 years). The median duration of follow-up was 35 months(range 4-132 months). At the end of therapy, 5 patients achieved complete remission(50%) and 2 partial remission(20%), one of whom relapsed after the therapy. Three patients did not respond to the therapy, two of whom progressed to end-stage renal failure during the therapy eventually requiring kidney transplantation. Conclusion : Intravenous long-term MP pulse therapy without CsA or cyclophosphamide by the Mendoza protocol may be effective in a subset of patients with steroid-resistant FSGS.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.255-263
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• 2007

Purpose : A single center cross sectional retrospective study was performed to compare the outcomes of different peritoneal dialysis(PD) modalities in Korean children. Methods : Among children dialyzed with PD between the year 2004 and 2007, 35 children had reliable data on PD adequacy after 3 to 15 months of dialysis. Subjects were grouped by their modalities; 17, 13 and 5 children were on continuous ambulatory PD(CAPD), continuous cyclic PD(CCPD) and nightly intermittent PD(NIPD), respectively. Body weight and height, number of patients taking anti-hypertensives and laboratory data including biochemical and hemoglobin levels were compared. Dialysis adequacy including weekly Kt/Vurea, creatinine clearance (Ccr) and daily water removal were also compared. Patients were sub-grouped by their peritoneal permeability characteristics. Results : The percentage of patients taking anti-hypertensives, monthly change in Z-scores of body weight and height and laboratory data did not differ among the groups. Patients on CAPD and CCPD showed similar dialysis adequacies. Weekly dialytic Ccr was significantly lower in the NIPD group compared to the others. But total Ccr was not different when residual renal function was added. Weekly dialytic Ccr by CAPD was significantly higher than that of CCPD in low and low-average transporters. Conclusion : We propose that modality can be selected flexibly according to the patients' preferences. And peritoneal permeability characteristics provide valuable information for adjusting PD prescriptions in ultrafiltration failure or in inadequate dialysis. Further study of other clinical performance measures should be performed to clarify the comparable outcomes in different PD modalities.

• Childhood Kidney Diseases
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• v.18 no.1
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• pp.24-28
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• 2014

Purpose: The purpose of this study was to assess the therapeutic efficacy of methylprednisolone pulse therapy in children with IgA nephropathy and Henoch- Sch$\ddot{o}$nlein Purpura (HSP) nephritis combined with proteinuria. Methods: We retrospectively reviewed the clinical records of 21 patients who were diagnosed with IgA nephropathy and HSP nephritis based on percutaneous renal biopsy. Of the 21 patients, 15 were diagnosed with IgA nephropathy and 6 were diagnosed with HSP nephritis. They had mild to severe proteinuria at the time of diagnosis or during follow-up. Group 1 (n=7) received methylprednisolone pulse therapy three times every couple of months, and Group 2 (n=14) received oral steroid therapy. The follow-up periods for Group 1 and 2 were 14.0 (9-54) months and 26.5 (14-34) months, respectively. There was no significant difference in the follow-up duration between the two groups. Results: The average age at diagnosis and biopsy was lower in Group 1 compared to Group 2, but it was not significantly different. At admission, all patients in both groups had hematuria and 5 patients (71.4%) of Group 1 and 14 patients (100 %) of Group 2 had proteinuria. Before treatment, there was no significant difference of spot urine protein/creatinine ratio between the two groups. During followup, 7 patients of Group 1 (100%) and 10 patients of Group 2 (71.4%) showed complete improvement of proteinuria and the spot urine protein/creatinine ratio in Group 1 was significantly lower than Group 2. Conclusion: In patients with IgA nephropathy and HSP nephritis with proteinuria, methylprednisolone pulse therapy was more effective than oral steroid therapy in the reduction of proteinuria. To investigate the effects on long-term prognosis, large-scale prospective studies are needed.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.170-177
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• 2008

Purpose : The aim of this study was to evaluate whether the incidence of relapse or nephritis might be influenced by the duration of corticosteroid therapy in children with Henoch-Schonlein purpura(HSP). Methods : We retrospectively analyzed 186 children with a diagnosis of HSP in two major hospitals in Ilsan, Korea from the years 2000 to 2003. To evaluate whether renal involvement or relapse might be influenced by the duration of corticosteroid therapy in children with HSP, one pediatric nephrologist from hospital A, maintained corticosteroid therapy for at least 2 weeks(Group A, n=94). The other from hospital B used only during the symptomatic period(Group B, n=92). Results : There were no significant differences in age, sex, body weight, white blood cell count, hemoglobin, hematocrit, platelet count, serum protein and albumin levels between the two groups. The incidence of abdominal pain or arthralgia also did not differ between two groups. However, the duration of steroid therapy was significantly longer in Group A than in Group B and the cumulative dose of prednisolone was also higher in Group A than in Group B. The development of nephritis was more frequent in Group A. Conclusion : The longer duration of steroid use was not associated with the decreased rate of nephritis. Therefore, corticosteroids should be used carefully in a selected group of HSP children, and be tapered rapidly after control of the acute symptoms.