• Clinical and Experimental Pediatrics
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• v.52 no.11
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• pp.1260-1266
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• 2009

Purpose : Idiopathic nephrotic syndrome (NS) can be clinically classified as steroid-sensitive and steroid-resistant. The detailed mechanism of glucocorticoid action in NS is currently unknown. Methods : In this study, we investigated 3 known single nucleotide polymorphisms (SNPs) (ER22/23EK, N363S, and BclI) of the glucocorticoid receptor gene (the NR3C1 gene) in 190 children with NS using polymerase chain reaction-restriction fragment length polymorphism and analyzed the correlation between the genotypes and clinicopathologic features of the patients. Results : Eighty patients (42.1%) were initial steroid nonresponders, of which 31 (16.3% of the total) developed end-stage renal disease during follow-up. Renal biopsy findings of 133 patients were available, of which 36 (31.9%) showed minimal changes in NS and 77 (68.1%) had focal segmental glomerulosclerosis. The distribution of the BclI genotypes was comparable between the patient and control groups, and the G allele frequencies in both the groups were almost the same. The ER22/23EK and N363S genotypes were homogenous as ER/ER and NN, respectively, in all the patients and in 100 control subjects. The BclI genotype showed no correlation with the NS onset age, initial steroid responsiveness, renal pathologic findings, or progression to end-stage renal disease. Conclusion : These data suggested that the ER22/23EK, N363S, and BclI SNPs in the NR3C1 gene do not affect the development of NS, initial steroid responsiveness, renal pathologic lesion, and progression to end-stage renal disease in Korean children with NS.

• Childhood Kidney Diseases
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• v.16 no.2
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• pp.73-79
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• 2012

Purpose: The association of mitochondrial DNA (mtDNA) mutations, deletions and copy number with progressive changes in patients with some glomerular disease and end-stage renal disease have been reported. In this study, we performed mtDNA mutation analysis in children with IgA nephropathy to investigate its role in progressive clinical course. Methods: Seven children with IgA nephropathy were involved in this study. MtDNA isolated from platelet was amplified by PCR and sequenced entirely. Results: The mean age at renal biopsy was $11.5{\pm}2.2$ year and the mean age at latest evaluation was $17.9{\pm}3.2$ year. The mean follow-up period were $7.8{\pm}3.1$ years. Patients was divided into 2 groups according to the amount of proteinuria at presenting manifestation. Group 2 patients were nephrotic syndrome. Renal function reveals within normal range in all patients. In group 2 patients, the mean serum albumin level was significantly lower than those of group 1 ($3.7{\pm}0.6g/dL$ vs. $4.7{\pm}0.2g/dL$, P=0.0241) and the mean total cholesterol level was significantly higher than those of group 1 ($222.7{\pm}35.7mg/dL$ vs. $148.3{\pm}29.1mg/dL$, P=0.0283). In Group 2 patients, total amount of protein of 24 hour collected urine also significantly higher than those of group 1 ($1,466.0{\pm}742.5mg$ vs. $122.5{\pm}48.1mg$, P=0.0135). Pr/Cr ratio in random urine sample was also higher in group 2 than those of group 1 but the statistical significance was not noted ($1.8{\pm}1.6$ vs. $0.2{\pm}0.2$, P=0.0961). Deletion of mtDNA nt 8272-8281 were observed in two patients, one patient in each groups, respectively. This is noncoding lesion. No patients demonstrated the mtDNA mutations. Conclusions: We have identified a deletion of mtDNA nt 8272-8281 in two children with IgA nephropathy. Further studies are needed to clarify the role of mitochondrial function in the progressive change of IgA nephropathy.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.64-68
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• 2005

Purpose : Renal abscess is very rare in children and its diagnosis is difficult because symptoms are often nonspecific. In previous studies, on]y 15% to 25% of patients were reported to be diagnosed at the time of admission. Early diagnosis and treatment are important be cause mortality rate correlates positively with the time of diagnosis. The purpose of this study is to clarify the clinical features of children with renal abscess and to investigate the possible indicators of this disease for early diagnosis and Proper treatment. Methods : Twelve children diagnosed with renal abscess from Jan. 1996 to Jul. 2004 were included. The age of patients ranged from S months to 15 years. We retrospectively analyzed the demographics of patients, their symptoms, predisposing factors, diagnostic methods and causative organisms and the treatment modalities. Results : Fever was the most common manifestation, Five children(42%) had vesicoureteral reflux. Renal ultrasonography and computerized tornography were the most frequently used imaging tools to detect renal abscess. Gram negative bacteria were isolated in 7 patients and Staphylococcus aureus grew in 2 patients. All patients received intravenous antibiotics and 4 patients underwent aspiration or drainage of renal abscess. The average admission duration was 30 days. Conclusion : Renal abscess should be included in the differential diagnosis of prolonged fever in children, especially when flank pain is combined. For early diagnosis and a better prognosis, patients should be promptly investigated with ultrasonography or computerized tomography.

• Clinical and Experimental Pediatrics
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• v.50 no.1
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• pp.74-78
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• 2007

Purpose : Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease with complex clinical manifestations. It probably involves genetic, environmental and immunologic factors. In this study, we investigated the clinical manifestations, laboratory findings and prognosis of pediatric SLE to aid clinical care of pediatric SLE. Methods : The data of 45 patients who were diagnosed as pediatric SLE in Severance Children's Hospital from Jan. 1996 to Dec. 2005 were analysed retrospectively. Results : The mean age at diagnosis was 10.8 (0-15) years old. And the ratio of male to female patients was 1:4. The initial manifestations were facial edema (51.1 percent), malar rash (44.4 percent), and fever (28.9 percent). The ANA (97.8 percent), anti-ds DNA antibody (82.2 percent), lupus nephritis (71.1 percent), malar rash (71.1 percent), and cytopenia (66.7 percent) were the most common findings among the classification criteria by ACR (American College of Rhematology, 1997). Conclusion : Clinical manifestations and prognosis are various in pediatric SLE. Intensive studies of SLE in children should be continued for more effective treatment.

• Journal of Digital Convergence
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• v.14 no.7
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• pp.309-319
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• 2016

There is a need to develop a data quality management algorithm in order to improve the quality of health care data. In this study, we developed a data quality control algorithms associated diseases related to diabetes in patients with hypertension. To make a data quality algorithm, we extracted hypertension patients from 2011 and 2012 discharge damage survey data. As the result of developing Data quality management algorithm, significant factors in hypertension patients with diabetes are gender, age, Glomerular disorders in diabetes mellitus, Diabetic retinopathy, Diabetic polyneuropathy, Closed [percutaneous] [needle] biopsy of kidney. Depending on the decision tree results, we defined Outlier which was probability values associated with a patient having diabetes corporal with hypertension or more than 80%, or not more than 20%, and found six groups with extreme values for diabetes accompanying hypertension patients. Thus there is a need to check the actual data contained in the Outlier(extreme value) groups to improve the quality of the data.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.176-188
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• 2009

Purpose : Peritoneal dialysis (PD) is the major form of dialysis in use for infants and children with end-stage renal disease (ESRD). The aim of this study was to gain insight into the current status of children on PD in Korea. Methods : In May 2008, questionnaires were sent to the pediatric nephrologists via e-mail. Four centers replied and those data were reviewed. Results : A total of 103 patients were included in this study. Male to female ratio was 1.6:1. Mean age was $11.5{\pm}4.9$ years (0-19 years). Primary renal diseases diagnosed were as follows: primary glomerular disease (34%), chronic pyelonephritis-reflux nephropathy (14.6%), systemic disease (9.7%), renal hypoplasia/dysplasia (8.7%), heredofamilial disease (6.8%), vascular disease (3.9%), drug-induced nephropathy (1.0%), and unknown (12.6%). PD modalities were as follows: CAPD (42.7%), CCPD (27.2%), NIPD (11.7%), and Hybrid (18.4%). Weekly total Kt/V was $2.1{\pm}0.7$ (0.3-4.1). Results of peritoneal equilibrium test were as follows: low 36.8%, low average 31.6%, high average 19.7%, and high 11.8%. Z-score for weight was $-1.00{\pm}1.20$ (-4.54~+2.50). Z-score for height was $-1.55{\pm}1.65$ (-9.42~+1.87). Growth hormone was administered in 24.3% of patients. Anti-hypertensive drugs were administered in 64.0% of patients. Laboratory findings were as follows: hemoglobin $10.5{\pm}1.4$ g/dL, calcium $9.7{\pm}0.7$ mg/dL, phosphorus $5.4{\pm}1.4$ mg/dL, and parathyroid hormone $324.2{\pm}342.8$ pg/mL. Conclusion : Primary glomerular disease was the most common cause of ESRD. CAPD was the most prevalent PD modality. Low and low average peritoneal transport type were common. Growth disturbance were noted in many patients. Some patients had hypertension even with anti- hypertensive drugs. Calcium-phosphorus levels were maintained adequately, but many patients had secondary hyperparathyroidism.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.130-137
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• 2009

Purpose : Stem cell transplantation (SCT) has gained worldwide acceptance as a treatment for hematologic disorders. This study was performed to evaluate the clinical characteristics and outcomes of the acute kidney injury after SCT in children. Methods : The records of 53 patients who were treated with SCT at the pediatric department of Yeungnam University Hospital between January, 1996 and April, 2009 were used as subjects. Their were divided into two groups ; 'Early renal insufficiency' (ERI, n=18) and 'Non-early renal insufficiency' (NERI, n=35). ERI had greater than 25% of drop in GFR after SCT. Results: Total 53 patients were analyzed. In cord blood SCT (n=11), ERI was 4 (36.4%) and NERI was 7 (63.6%). In bone marrow SCT (n=16), ERI was 8 (50.0%) and NERI was 8 (50.5%). In autologous peripheral blood SCT (n=26), ERI was 6 (23.1%) and NERI was 20 (76.9%). There is no difference in both groups according to kinds of SCT. GVHD was developed in 22 patients, and there is no difference in each group. Twenty two of 53 patients died. ERI was 12 (66.7%) and NERI was 10 (28.6%). Acute renal failure is most important cause of the deaths. Conclusion : Out of 53 pediatric patients who were treated with SCT, 18 patients had greater than 25% of drop in GFR. There is no difference in both groups according to kinds of SCT. GVHD was found in 22 patients and there is no relation between GVHD development and acute kideney injury.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.161-167
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• 2007

Purpose : GFR(glomerular filtration rate) is a fundamental parameter in detecting renal impairment and predicts the progression of renal disease. Because serum creatinine has several disadvantages, serum cystatin C has been recently proposed as a new endogenous marker for GFR. We compared serum cystatin C with creatinine and creatinine clearance to investigate the clinical usefulness of cystatin C. Methods : We retrospectively analyzed 46 patients(60 case numbers) who had various renal diseases and classified them into 3 groups according to creatinine clearance(Group 1 : CrCl <40 mL/min/$1.73m^2$, Group 2 : CrCl 40-60 mL/min/$1.73m^2$, Group 3 CrCl >60 mL/min/$1.73m^2$). We measured serum creatinine, cystatin C and creatinine clearance and also analyzed the correlations among them. Results : Serum cystatin C and creatinine showed a similar correlation to creatinine clearance (r=0.685, r=0.640, respectively) and showed similar diagnostic accuracy in detecting decreased GFR(AUC, cystatin C 0.829 vs. creatinine 0.826, P=0.848). Serum cystatin C showed a greater sensitivity for detecting a decreased GFR than creatinine in Group 2 and 3(Group 1 : 100% vs. 100%, Group 2 : 70% vs. 35%, Group 3 : 46% vs. 15%). Conclusions : Serum cystatin C could be a useful endogenous marker for GFR and would be superior to serum creatinine in early detection of renal impairment in pediatric patients with renal diseases.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.144-150
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• 1997

Purpose : The kidney is one of the most common sites of cyst formation. Cystic diseases of the kidney are a diverse group of clinicopathologic entities and variable prognosis. They span a wide range of both age of presentation and severity of the renal disease. And many of them are systemic disorders, sharing similar process of cyst formation in other organs. Recently, development of imaging studies has been contributing widely to the diagnosis of the diseases. Treatment, however, is not established satisfactorily. We performed this study to evaluate the occurrence and treatment of cystic diseases of the kidney. Methods : We reviewed retrospectively the medical records of 44 patients with cystic diseases of the kidney in the Department of Pediatrics, during last 11 years. Results : In the 44 patients with cystic diseases of the kidney, 31 patients(71%) had multicystic dysplastic kidney and 11(35%) of them received nephrectomy due to differentiation from neoplasms or severe abdominal distension. Seven patients(16%) had polycystic kidney disease, and all of them were infantile type. Five patients(11%) were diagnosed as having a simple renal cysts. Progression to renal failure was noted in none of the cases. In 14(32%) out of total 44 patients, the diagnosis was made in neonatal or infantile pelted. Conclusion : The incidence of cystic diseases of the kidney appeared very low, but further investigation on their pathogenesis, classification, and indication of treatment is needed.

• Applied Microscopy
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• v.34 no.4
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• pp.241-254
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• 2004

Kidney had recovery functions against toxicants, ischemia, reperfusion-induced damage, acute-renal failure (ARF). Urinary epidermal growth factor (EGF) is produced by the juxtaglomerular apparatus. Kidney accumulates or excretes the EGF. In case of renal diseases, excreted EGF was decreased. The aim of this study is to evaluate the effects squalene (SQ) on the prevention of experimental acute renal failure induced by glycerol. In case of in vitro study, we investigated the expression of EGF by RT-PCR. After the proximal tubular cells was isolated, glycerol (1, 2, 4 mM) or glycerol plus squalene (0.1, 0.05 or 0.1%) was added. In case of in vivo study, we investigated the changes of BUN, creatine, and ultrastructure. Experimental groups were divided into four groups. Group 1 was normal mouse. Group 2 was injected with SQ only (180 mg/kg). Group 3 was not treated with squalene after intraperitoneal contamination of glycerol (50%, 8 ml/kg). And, Group 4 was treated with squalene (180 mg/kg) after intraperitoneal contamination of glycerol (50%, 8 ml/kg). All groups were used to 7 mice. In the results, we investigated the glycerol induced renal failure. The expression of EGF mRNA was decreased in renal proximal tubules when treated with only glycerol. SQ increased the mRNA expression of EGF in renal proximal tubules. SQ also quickly recovered the levels of BUN and creatine compared with those of mice treated with only glycerol (P<0.01). In case of ultrastructure, group 3 had heavily damaged mitochondria, but, mitochondria in group 4 had evidences of the recovery. It was concluded that SQ had the recovery effects for the glycerol-induced acute renal failure.