• Childhood Kidney Diseases
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• v.8 no.1
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• pp.10-17
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• 2004

Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) nephritis has a variable range of prevalence from 25 to 50% among HSP patients and is a common cause of chronic glomerulonephritis in children. In our study, we evaluated the distribution and the association of the angioten-sinogen(AGT) M235T polymorphism with the clinical manifestations, particularly proteinuria in children with HSP with or without nephritis. Methods : The AGT M235T polymorphism was determined in children with HSP nephritis (n=33) or HSP without nephritis(n=28) who had been diagnosed at Busan Paik hospital from January 1996 to June 2001. The M235T polymorphism of the AGT gene was determined by PCR amplification of the genomic DNA. Results : The M235T polymorphism of AGT gene frequency was MM 75%, MT : 25%, TT : 0% in HSP and MM : 64%, MT : 36%, TT : 0% in HSP nephritis, there was no significant differences in the genotype and allele frequencies between the two groups. No significant differences in clinical manifestations at onset and last follow-up were seen between the two genotypes. When statistical analysis was done according to the presence of the M allele, the amount of 24-hour urinary protein excretion and the incidence of moderate to heavy proteinuria(>500 $mg/m^2/day$) at onset and at last follow-up were higher in the MT genotype than in those of in the MM genotype but these difference were not statistically significant. Conclusion : We suggest a lack of association between M235T polymorphism of the AGT gene and clinical manifestations in children with HSP nephritis. However, further follow-up studies based on sufficient number of patients and long term follow up periods are necessary to confirm the role of M235T polymorphism of AGT gene in children with HSP nephritis.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.33-42
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• 2004

Purpose : Children with nephrotic syndrome(NS) are under high risk for metabolic bone disease(MBD) as a complication of long-term glucocorticoid therapy. We prospectively evaluated the effect of oral bisphosphonate(alendronate) therapy in children with NS, which has proven efficacy in adult patients with glucocorticoid induced MBD. Methods : Among 58 children with NS, aged 5 to 8 years and haying a disease duration of more than 2 years, 30(51.7%) were enrolled to meet the selection criteria, less than -1.0 Z-scores of lumbar spine bone mineral density(BMD) by dual energy X-ray absorptiometry (DEXA). These 30 children were divided into three groups and each were assigned to receive alendronate, calcitriol, and no-medication, respectively for one year. Lumbar spine BMD was followed up every 6 months and the biochemical indexes were measured before and 1 year after the treatment. There were no significant difference among groups with respect to the average age, the initial BMD, and the cumulative steroid doses. Analysis of the treatment efficacy was done by the % change of BMD and by the changes in Z-scores of lumbar spine BMD. Results : Mean age and disease duration of patients at the initial lumbar spine BMD evaluation was $7.4{\pm}1.7$ years and $2.2{\pm}1.2$ years, respectively. Twenty-three of 30 children(76%) had osteopenia, and seven(23%) had osteoporosis. There was no difference in the biochemical values among the groups, before and 1 year after the treatment(P<0.05). Twenty two children(73.3%) with frequent relapsing or steroid dependant NS had more frequent MBD, compared to the 8 children(26.6%) with infrequent relapsing NS. The one year % changes of BMD were 8.56 in alendronate group, 5.79 in calcitriol group, and 1.9 in no-medication group. The changes in Z-score of lumbar spine BMD increased in the alendronate group and the calcitriol group, but not in the no-medication group. One year % changes of BMD were different among groups(P=0.0002). Significant differences were found between the alendronate and the no-medication group, and between the calcitriol and the no-medication group(P<0.05). There was no difference between the alendronate and the calcitriol group. No serious adverse effect was observed in the alendronate group. Conclusion : Children with NS receiving high dose steroids are under the high risk of BMD and should undergo regular BMD evaluation. Z-score of lumbar spine BMD was a useful parameter in diagnosing low bone mass in children. Alendronate weekly oral therapy was effective and relatively safe in increasing the lumbar spine BMD in children with NS having steroid induced MBD.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.194-201
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• 2008

Purpose : Voiding cystourethrography(VCUG) is required to detect vesicoureteral reflux(VUR), which may manifest as urinary tract infection(UTI) in children. It is well known that VCUG can cause UTI(post-VCUG UTI). In this study, risk factors for post-VCUG UTI and the preventive effect of antibiotics against this complication of VCUG were explored. Methods : Medical records of 284 patients who underwent VCUG at our hospital in 2007 were reviewed retrospectively. The incidence of post-VCUG UTI and risk factors for post-VCUG UTI, and the impact of antibiotic use on prevention of post-VCUG UTI were evaluated. According to antibiotics usage, we divided the enrolled patients into 4 groups of noantibiotics group, prophylactic antibiotics group(prophylactic antibiotics having been used before), antibiotics-for-VCUG group(antibiotics added for VCUG) and antibiotics-for-treatment group(treatment dose of antibiotics). Results : Seven of 284 children(2.5%) developed UTI after they underwent VCUG. Highgrade(grade$\geq$III) VUR was the only statistically significant risk factor(odds ratio[OR] 6.266, P=0.026) for post-VCUG UTI, while sex, age, and other anomalies of urinary system were not significant. Five post-VCUG UTI cases belonged to prophylactic antibiotics group. Antibiotics use (three groups using antibiotics vs. no-antibiotics group) or addition of antibiotics for VCUG (antibiotics-for-VCUG vs. other groups) did not have any effect on prevention of post-VCUG UTI. Conclusion : The risk factor for post-VCUG UTI was high-grade VUR. Antibiotics use did not prevent post-VCUG UTI in this study.

• Clinical and Experimental Pediatrics
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• v.49 no.2
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• pp.181-186
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• 2006

Purpose : The hematologic change during the treatment of acute lymphoblastic leukemia(ALL) is critical as a prognostic determinant and a variable to determine the dose of chemotherapeutic agents. It is known that the dose of vincristine used in the maintenance phase of ALL is small enough to increase the count of platelet. To investigate the change of platelet count according to the vincristine administration in maintenance phase of ALL chemotherapy, we performed this study. Methods : Eleven patients eligible under the criteria of Children's Cancer Study Group(CCG)-1882 and who had completed chemotherapy were enrolled in this study. The count of platelets before vincristine administration was compared with those of vincristine administration 1, 2 and 3 weeks after the early and last periods of maintenance phases. The platelet count before vincristine administration was defined as 100 percent and that after vincristine were compared. In addition, we tentatively defined an enhancing effect of vincristine as positive when the relative count was more than 120 percent. Results : Platelet count did not differ according to the early and last periods of maintenance phase. Platelet count at first week after vincristine administration increased more significantly than that before vincristine in early and last periods. There was an enhancing effect in 10(90.9 percent) of 11 patients after 1 week vincristine administration both in the early and last periods of the maintenance phase. Conclusion : Vincristine, used in ALL maintenance phases as a low dose, increased platelet count 1 week after administration. The increased platelet count resumed to the previous level 2-3 weeks later. However, the thrombocytosis observed in the maintenance phase by vincristine was not high enough to induce thrombosis. In addition, vincristine is known to reduce the activity of platelets. Therefore, the risk of thrombosis in the maintenance phase of ALL chemotherapy would be low.

• Clinical and Experimental Pediatrics
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• v.45 no.10
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• pp.1219-1226
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• 2002

Purpose : To assess the clinical characteristics of the 2000-2001 measles outbreak in the Seoul metropolitan area, Korea, the clinical data of measles inpatients were analyzed. Methods : Three hundred and five children diagnosed with measles by clinical manifestation from July, 2000 to February, 2001, in Seoul, Ilsan and Ansan City were grouped according to their age and investigated for clinical manifestations, vaccination history and measles-specific IgM/IgG antibody positivity. Results : Thirty eight point seven percent of the subjects were less than 12 months of age, 27.5 % were 12-47 months of age and 33.8% were 48 months of age or older. There was no significant sexual difference(male : female=1.2 : 1). This epidemic started in June, 2000 and the number of patients increased abruptly in October, peaked in December and finally decreased after February, 2001. It started from the older age group and moved to the younger. Sixty five point two percent had a history of more than 1-dose vaccination and 13.6% of the patients equal or more than 48 months of age had a history of 2-dose vaccination. Primary vaccine failure rate was 59.4%(107/180) and secondary vaccine failure rate was 3.9%(7/180) in 1 dose vaccinees. Sixty one point six percent showed more than one complication and 38.4% had no complication. The most common complication was pneumonia(31.8 %), followed by bronchitis(11.5%) and acute otitis media(4.6%). Vaccination and dose were not related significantly with the occurrence of complications. Conclusion : Compared with previous outbreaks in Korea, clinical features showed no specific change in the 2000-2001 measles epidemic. However, primary vaccine failure rate was so high that the second vaccination at four to six years of age must be emphasized in Korea.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.358-364
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• 2010

Purpose : The aim of this study was to determine the efficacy of $Synagis^{(R)}$ (palivizumab) in reducing the respiratory syncytial virus (RSV) readmission rate in very low birth weight infants (VLBWI ) and the subgroup that showed the most effective vaccination. Methods : We enrolled 350 VLBWI who had been discharged alive from the neonatal intensive care unit of Samsung Medical Center from January 2005 to December 2007 and were followed up for at least one year. A retrospective study based on medical records was performed for a period of one year after discharge. RSV readmission rate was investigated according to BPD (bronchopulmonary dysplasia, requiring oxygen at postnatal day 28) and $Synagis^{(R)}$ prophylaxis. We categorized the subgroups by the severity of BPD gestational age, and birth weight and compared the RSV readmission rates between subgroups. Results : Eleven VLBWI were readmitted. $Synagis^{(R)}$ prophylaxis resulted in a 86% reduction in the rate of readmission due to RSV infection (prophylaxis group, 0.7% and no prophylaxis group, 5.0%; P =0.02). Readmission rate in BPD patients was also reduced in the prophylaxis group (0.7% in the prophylaxis group vs. 5.2% in the no prophylaxis group, P =0.03). The readmission rate in patients without BPD was reduced in the prophylaxis group (0% in the prophylaxis group vs. 4.9% in the no prophylaxis group, P =1.00), but this was not statistically significant.Conclusion : $Synagis^{(R)}$ prophylaxis was effective at reducing RSV readmission in VLBWI. Its efficacy was verified irrespective of BPD, gestational age, or birth weight.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.157-165
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• 2003

Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) is a systemic vasculitis that involves multiple organs, especially the kidney, which is the most important organ in determining the prognosis of the disease. The morbidity of HSP nephritis in adults is low and there have been little research done on its clinical course so far. Therefore, we have compared the clinical course of HSP nephritis in children and adults in Korea. Methods : We retrospectively analyzed 81 cases of HSP nephritis in children younger than 15 years of age, and 25 cases of adults older than 15 years of age who were admitted to Yonsei University Medical College Severance Hospital from Jan. 1986 to May 2003. Results : The male to female ratio was 1.5 : 1 in children and 1.3 : 1 in adults. The incidence of HSP nephritis for both age groups was found to be increased during the autumn and winter. Infection was the predisposing factor in 39 cases(48.1%) of children, 16 cases(64.0%) of adults, and drugs were the predisposing factor in 8 cases(9.9%) of children and 4 cases (16.0%) of adults. All patients initially presented with microscopic hematuria. Thirteen cases (16.0%) of children and 7 cases(28.0%) of adults initially showed proteinuria of nephrotic range. Thirty four cases(42.0%) of children and 4 cases(16.0%) of adults showed normal urinalysis after treatment. Asymptomatic urinary abnormalities were found in 41 cases(50.6%) of children and 18 cases(72.0%) of adults. Complications such as nephrotic syndrome and hypertension were found in 3 cases(3.7%) of children and 2 cases(8.0%) of adults. Three children(3.7%) and 1(4.0%) adult required dialysis or renal transplantation. Follow-up renal biopsies were performed on 21 children, of whom 10 cases(47.6%) did not show any histologic change, 9 cases(42.9%) showed low grade changes, and 2 cases(9.5%) showed high grade changes. Prognosis was gloomy when proteinuria of nephrotic range and high grade of abnormal histology were present at diagnosis, and there was no significant difference between the two groups(P<0.05) Conclusion : This study showed that there was no difference in terms of the clinical features and courses between the children and adults with HSP nephritis. Proteinuria of nephrotic range and the severity of abnormal histologic changes at diagnosis were found to be associated with a bad prognosis, therefore we recommend that patients with these features require long term follow-up and management.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.133-141
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• 2003

Purpose : Idiopathic Membranous Nephropathy(IMN) is a rare renal disease in children. To help better understanding of its clinical course and treatment strategies, we reviewed the clinical manifestations and pathological findings of children with IMN. Methods : Among 58 cases with MN, from 1977 to 2003, 42(72.4%) were hepatitis B virus (HBV) associated and 16(27.6%), 6 males and 10 females, were idiopathic. All cases diagnosed aster 2000 were IMN. Several clinicopathological findings(sex, onset age, proteinuria, serum albumin, cholesterol, creatinine clearance, tubulointerstitial changes, glomerular sclerosis, hypertension, renal vein thrombosis, the use of ACE inhibitor, and immunosuppressive therapy) were compared between the remission and the non-remission group of the patients with IMN. Results : The median onset age was 13.4 years. Clinical manifestations were nephrotic syn-drome(7 cases, 43.8%), gross hematuria(5 cases, 31.3%) and microscopic hematuria with proteinuria(3 cases, 18.8%). Hypertension, hypocalcemic tetany and renal vein thrombosis were accompanied in 2, 1 and 2 cases, respectively. In addition to the typical findings of MN, the kidney biopsies showed segmental sclerosis(5 cases, 31.3%) or global sclerosis(6 cases, 37.5 %), diffuse crescents(1 case), and mild(11 cases, 68.7%) or moderate tubulointerstitial changes(3 cases, 18.8%). Thirteen cases(86.7%) received oral steroid. Among them 2 cases received cyclophophamide and 1 received cyclosporin as well. Ten cases(62.5%) received ACE inhibitors. In the patients followed up, 7 cases(46.7%) became free from proteinuria (remission group) while 8(53.3%) presented continous proteinuria (non-remission group), two (13.3%) of which progressed to renal failure. Clinicopathological findings showed no significant differences between the two groups. Conclusion : With HBV vaccination, HBV associated MN decreased markedly and IMN has taken up most of MN in children. For better understanding of this rare disease, a prospective multicenter study of the clinical course and treatment strategies should be done.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.136-146
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• 2001

Purpose : One of the most difficult problems in the care of children with nephrotic syndrome remains the occurrence of relapses, despite initial response to steroids. Constantinescu reported that rapidity of initial response to steroid therapy could predict fewer relapses in the first year. So we evaluated the changes in serum lipid abnormalities in children with corticosensitive nephrotic syndrome before steroid treatment and the correlation between serum lipid levels and renal function, days to remission. Methods . We analyzed the Medical records of children who were managed by us between October 1994 and August 2000. In 33 patients with corticosensitive nephrotic syndrome, we evaluated the correlation between serum lipid levels and renal function [Creatinine clearance(Ccr)] and proteinuria before steroid treatment, and days to remission defined as the third day when the patient's urine becomes protein free. Results : There were 21 males and 12 females. Median age at presentation was 6.4 years (range: 1.8-17.3 years). Median days to remission were 15.4 days (range 4-42 days) on Prednisolone $60mg/m^2$ daily. The increased levels of triglyceride, total cholesterol, LDL cholesterol, apolipoprotein B, total cholesterol/HDL cholesterol, Lipoprotein(a) were observed. But the level of HDL cholesterol was not increased. Serum albumin was decreased a]id proteinuria was increased before steroid treatment. But Ccr was not decreased. There were negative correlation between serum albumin and total cholesterol (r = -0.5157, P<0.005), LDL cholesterol (r = -0.5543, P<0.005), total cholesterol/HDL cholesterol (r = -0.4506, P<0.01), lipoprotein(a) (r = -0.4570, P<0.025), apolipoprotein B (r = -0.5297, P<0.025), apolipoprotein B/apolipoprotein Al (r = -0.5851, P<0.01), apolipoprotein B/HDL cholesterol (r = -0.4961, P<0.05) before steroid treatment. There was no correlation between proteinuria and serum lipid profiles. Also Ccr and serum lipid profiles were not correlated. There was positive correlation between days to remission and HDL cholesterol (r = +0.4511, P<0.05), apolipoprotein B (r = +0.5190, P<0.05), apolipoprotein B/HDL cholesterol (r = +0.7169, P<0.005). Conclusions : This results reveal that HDL cholesterol, apolipoprotein B and apolipoprotein B/HDL cholesterol can be used as a predictive factor in corticosensitive nephrotic syndrome. We could not determine the significant level of these lipids for insufficient patients number, but these level may predict future relapses of corticosensitive nephrotic syndrome patients and thus may allow to better management and treatment protocols. More data and long term follow up studies should be needed. (J Korean Soc Pediatr Nephrol 2001;5 : 136-46)

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.17-24
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• 2000

Purpose: MCNS is found in approximately $85\%$ of the idiopathic nephrotic syndrome in children and shows good prognosis with initial steroid therapy. However in FSGS, there is poor prognosis with initial therapy and shows higher rate of progression to chronic renal failure and relapse after kindney transplantation. We have experienced 8 patients who were diagnosed as MCNS on initial renal biopsy and then progressed to FSGS on follow-up biopsy. So we have investigated their clinical course and risk factors for transition of MCNS to FSGS. Methods: We conducted a retrospective study with a review of histopathologic findings and clinical manifestations of 296 cases of MCNS and FSGS that were diagnosed from January 1988 to May 1999. We classified them into 3 groups according to the histopathologic finding; MCNS, FSGS, MCNS progressed to FSGS in follow-up biopsy. Results: The number of children was 296 cases comprising 241 cases($81.4\%$) showing MCNS, 8 cases($2.7\%$) transition group, 47 cases($15.9\%$) FSGS. The mean onset age was $6.0{\pm}2.6$years in MCNS, transition group $8.3{\pm}2.3$years, FSGS $7.2{\pm4.3$years, and the gender (M:F) ratio was 3.7:1 in MCNS, 3:1 in transition group, 1.8:1 in FSGS. Comparing the presence of initial hematuria, hypertension,24 hour urine protein, serum albumin, serum creatinine, there were significant difference between the transition group and the FSGS group in the following points; 24hour urine protein $684:342mg/m^2/hr$(P<0.05), serum albumin 1.92: 2.47g/dL(P<0.05), serum cholesterol 494:343mg/dL(P<0.05). Refractoriness to steroid therapy was 13.3$\%$ in MCNS. $12.5\%$ in transition group, $29.6\%$ in FSGS; significantly higher in FSGS(P<0.05). Immunosuppressant therapy was performed in $58.5\%$ of MCNS, $100\%$ in transition group, $80.8\%$ in FSGS; transition group showed significantly higher .ate(P<0.05) comparing with MCNS. Mean number of relapse and duration from onset to first relapse showed no significance difference between these groups. Conclusion: 249 patients with MCNS have been followed and $3.2\%$ (8 patients) of them has shown change in pathologic diagnosis from MCNS to FSCS. The risk factor for transition could not be found. Our results point to the need for a follow-up biopsy to certify the possibility of transition to FSCS in some MCNS cases with refractory cases to steroid therepy, frequent relapsing cases, or in case of no remission in spite of vigorous immunosuppressant therapy.