• Childhood Kidney Diseases
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• v.5 no.1
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• pp.1-8
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• 2001

Purpose : Clinical manifestations and pathologic findings of thin glumerular basement membrane disease, recognized as a common underlying disease of benign, familaiar and asymptomatic hematuria has not been reported systemically in Korera. We analyzed clinical and pathologic findings of patients who were diagnosed as thin glomerular basement membrane disease Methods : We analyzed clinical and pathologic findings of twenty-six patients who were diagnosed as thin glomerular basement membrane disease by renal biopsy among who complained asymptomatic hematuria from 1990 to 2000. Results : The subjects were aged 9.4${\pm}$3.2 (3.0-15.8) years-old at onset of hematuria, and 11.1${\pm}$2.2 (4.7-16.3) years-old at renal biopsy. Sexual discrepancy was more common in girls (eight boys and eighteen girls). A family history of hematuria was found in 8 patients(30.7$\%$). Major clinical manifestation on admission was microscopic hematuria according to the findings of 3case(11.5$\%$) of gross hematuria, 23cases(88.5$\%$9) of microscopic hematuria, and 1 case(3.8$\%$) of proteinuria. Microscopic hematuria persisted in all cases. Kidney biopsy showed few changes by light microscopy, but IgM, C3 and fibrinogen deposit in mesangium was found by immunofluorescent microscopy in a few cases. Electron microscopic findings have revealed thinning of the glomerular basement membrane varied from 180.9${\pm}$35.8nm. Conclusion : Thin glomerular basement membrane disease might be a common cause of microscopic hematuria of children and family history was revealed in about 30$\%$. Clinical progression was good in majorities.(J. Korean Soc Pediatr Nephrol 5 : 1-8, 2001)

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.153-160
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• 1999

Purpose : Present study was undertaken to find out significance of clinical presentation, initial laboratory data and renal biopsy findings on subsequent clinical course of IgA nephropathy in children. Methods : Clinical and laboratory data were analysed retrospectively from 60 children who have been admitted to the Pediatric Department of Kyungpook National University Hospital for the past 11 years and diagnosed as IgA nephropathy. Renal biopsy findings were graded according to the pathologic subclass proposed by Haas. Results : Pathologic grading according to Haas subclassification showed 10 cases in subclass I, 36 in II, 12 in IV and 2 in V and none in subclass II. Sex distribution showed male predominance (male to female ratio = 3 : 1) and mean age at onset of disease was $10.4{\pm}2.8$ years. Episodes of gross hematuria was seen in 71.7% and IgA level increased in 28.3% of children and these were not associated with pathologic grading nor clinical outcomes. With increasing subclass grading, serum protein and albumin decreased and 24 hours urinary protein excretion increased. Normalization of urinalysis (disappearance of hematuria) was seen in 14% at 1-2 years and 37.1% at 3-4 years of follow up period. In 3 cases, renal function deteriorated progressively and they belonged one each to the Haas subclass III, IV and V. Conclusion : In children with IgA nephropathy, progression to chronic renal failure appears to be quite high and pathologic grading according to Haas' subclassification seems to predict patient's outcome faily well. However, firm conclusion cannot be drawn from present study due to the small numbers of patients and short follow-up period. Therefore further multicenter study involving larger numbers of patients and longer periods of follow-up over 10 years was to be undertaken.

• 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.

• Pediatric Infection and Vaccine
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• v.23 no.1
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• pp.54-61
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• 2016

Purpose: Bacille Calmette-$Gu{\acute{e}}rin$ (BCG) lymphadenitis is a relatively frequent local adverse reactions after BCG vaccination. Its incidence rate is usually <1%. However, this rate may be different according to BCG strain, vaccination method or skill, etc. In the Republic of Korea, two BCG strains are used: intradermal Danish-1331 or percutaneous Tokyo-172. We surveyed the incidence rates of BCG lymphadenitis. Methods: This survey was performed in total 25 centers (5 general hospitals, 20 private pediatric clinics). Immunized type of BCG strain in study subjects was verified by directly observing the scar. The occurrence of BCG lymphadenitis was asked to their parent. In cases of BCG lymphadenitis, location, diameter size, progression of suppuration, and treatment method were investigated, as well. Results: The total number of study subjects was 3,342. Among these, the subjects suitable for enrollment criteria (total 3,222; Tokyo strain 2,501, Danish strain 721) were analyzed. BCG lymphadenitis regardless of its size developed in each five of subjects per strains, therefore, its incidence rate was 0.20% in Tokyo and 0.69% in Danish strain, respectively (P=0.086). However, when applying the WHO criteria - the development of lymph node swelling with diameter 1.5 cm or more, the incidence rate of BCG lymphadenitis was 0.16% (4 cases) in Tokyo and 0.42% (3 cases) in Danish strain, respectively. Conclusions: The incidence rate of lymphadenitis in two BCG types, percutaneous Tokyo and intradermal Danish strain BCG, is 0.20% and 0.69%, respectively. Both rates are acceptable.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.198-208
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• 2002

Purpose : There is no scientific basis for an immunization policy for children with renal disease who have increased risk of infection in Korea. As an initial step in approaching this problem, this survey of pediatric nephrologists was undertaken to determine the current recommendations of practicing pediatric nephrologists Methods : Questionnaires were sent to the members of Korean Society of Pediatric Nephrology via mail and E-mail. The questionnaire was designed to obtain information about the immunization practice of basic vaccination schedule for nephrotic syndrome, the side effects after vaccination and the immunization practice about recommended vaccines for children with renal disease. Results : Questionnaires were sent to 56 pediatric nephrologists. 35 replies were received (response rate: 62.5%). Almost of the respondents (82.8%) reported practicing at university hospital. All respondents reported modified vaccination schedule. 65.7% of the respondents immunized nephrotic children with live vaccines some time later after discontinuation of corticosteroids treatment and 57.1% of respondents immunized them with killed vaccines during medication of low doses of corticosteroids. Respondents experienced relapse of nephrotic syndrome after vaccination are nine, lack of vaccine efficacy are three and infection by organisms of live vaccines are two. 71.4% of respondents reported vaccinating children with renal disease for hepatitis B, pneumococcus and influenza during medication of low doses of corticosteroids. But There is few difference of the rates of respondents vaccinating them for Hemophilus influenzae type b between during medication of low doses of corticosteroids and after discontinuation of corticosteroids treatment (45.7% us 42.9%). Almost of respondents reported vaccinating renal failure children without immunosuppression for hepatitis B, pneumococcus, influenza and H. influenzae type b ($54.3{\sim}77.1%$). Conclusion : Pediatric nephrologists practiced modifying vaccination schedules for children with renal disease in Korea and there was variation according to the progression of disease and the doses of corticosteroids. It is necessary to establish the immunization guideline for children with renal disease through the prospective studies.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.169-177
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• 2002

Purpose: Hypertension accelerates the progression of chronic renal disease, whether it results from, or causes, the renal disease. Therefore, the control of hypertension is one of the important factors that retard the rate of renal deterioration. We compared the effects of different antihypertensive agents on renal function and glomerular morphology In subtotal nephrectomized rats. Materials and methods: After induction of chronic renal failure with 5/6 nephrectomy, the rats were divided into three groups; control group (Group C), enalapril group (Group E), and nicardipine group (Group N). Systolic blood pressure was measured by tail cuff method every 4 weeks until 12 weeks after nephrectomy. At 12 weeks after nephrectomy, all rats were placed in metabolic cages for 24 hour urine collections to measure urinary protein and creatinine excretion. After urine collection and blood sampling for serum creatinine, all rats were sacrificed. The renal tissue was processed for morphometric study with light microscope and electron microscope. Results: 1. The blood pressure of Group C increased progressively, but both enalapril and nicardipine prevented the development of hypertension, and the two drugs were equally effective in maintaining normal blood pressure throughout the study. 2. Twenty-four hour urinary protein excretion was lower in Group E compared to Group C and Group N 3. Mesangial expansion score in both treated groups were significantly lower than the control group. Mean glomerular volume in Group E was significantly reduced compared to Group C and Group N. There was no significant difference in mean glomerular volume between Group C and Group N. 4. There was no significant difference in podocyte structural changes, estimated by filtration slit length density, among control, enalapril and nicardipine treated groups. Conclusion: Control of hypertension with enalapril or nicardipine afforded considerable protection from mesangial expansion in the rat remnant kidney model. But protein excretion and glomerular growth were significantly reduced in Group E compared to Group N. There was no significant difference in podocyte structural changes among the 3 groups.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.88-94
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• 1999

Purpose : Malformation of urinary tract is among the most common of all congenital anomalies and can progress to irreversible renal damage before diagnosis due to difficulty of early diagnosis. Present study was undertaken to determine the clinical characteristics of urinary tract anomaly and to find out the most appropriate diagnostic and therapeutic measures for children with these anomalies. Methods : During the past 10 years from 1987 to 1998, review of medical records revealed 65 children with congenital anomaly of urinary tract and the following results were obtained. Results : The most common anomalies were ureteropelvic junction obstruction occuring in 26 cases ($36\%$), followed by ureteral duplication in 11 cases, renal agenesis in 10 cases and ureterovesical function obstruction in 7 cases. Complex anomaly of urinary tract was found in 8 cases and anomaly of other systems such as congenital heart disease was detected in 11 cases. The most frequent age group at the time of diagnosis was below 1 year of age constituting 39 cases ($60\%$) and male preponderance was noted as male to female ratio being 2.25:1. Presenting symptoms were urinary tract infection in 25 cases, followed by hematuria, abdominal mass, abdominal pain and voiding difficulty, etc, and in 11 cases, the anomaly was picked up by routine prenatal ultrasonography. Azotemia was noted in 9 cases and the underlying anomaly was obstructive uropathy in 4 out of these 9 cases. Surgical correction was undertaken in 38 cases (most frequently in cases of obstructive uropathy) and in 2 out off cases with obstructive uropathy in whom surgical correction was done, azotemia disappeared during follow up period of 1-5years. No new cases of deteriorating renal function appeared during follow-up period. Conclusion : In spite of high incidence of congenital malformation of urinary tract, early diagnosis is still hampered by nonspecific symptoms and signs. Therefore, in patients with symptoms such as urinary tract infection, abdominal pain and voiding problems, etc, it Is advisable to take various diagnostic tests promptly to pick up any urinary tract anomaly and to apply proper therapy in order to avoid progression to irreversible renal damage. In this regard, prenatal ultrasonography should be utilized more widely as a routine procedure to detect any urinary tract anomalies before birth.

• Asian Pacific Journal of Cancer Prevention
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• v.13 no.7
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• pp.3403-3408
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• 2012

The molecular mechanisms involved in the progression of clear cell renal cell carcinomas (ccRCCs) are still unclear. The aim of this study was to analyse the relationships between expression of RALYL and clinical characteristics. In 41 paired samples of ccRCCs and adjacent normal tissues, we used real-time qPCR to evaluate the expression of RALYL mRNA. RALYL protein levels were determined in 146 samples of ccRCC and 37 adjacent normal tissues by immunohistochemistry. Statistical analysis was used to explore the relationships between expression of RALYL and the clinical characteristics (gender, age, tumor size, T stage, N stage, M stage, survival times and survival outcome) in ccRCC. In addition, these patients were follow-up period 64 months (range: 4~116months) to investigate the influence on prognosis. We found significantly differences between ccRCC tissues and normal tissues (p<0.001, paired-sample t test) in mRNA levels of RALYL. Immunohistochemistry analyses in 146 ccRCC samples and 37 adjacent normal tissues showed significantly lower RALYL protein levels in ccRCC samples (${\chi}^2$-test, p<0.001), inversely correlating with tumour size (p=0.024), T stage (0.005), N stage (p<0.001) as well as M stage (p=0.019), but not age (p=0.357) and gender (p=0.348). Kaplan-Meier survival analysis demonstrated that people with lower level of RALYL expression had a poorer survival rate than those with a higher level of RALYL expression, significantly different by the log-rank test (p=0.011). Cox regression analysis indicated that RALYL expression (p=0.039), N stage (p=0.008) and distant metastasis (p<0.001) were independent prognosis factors for the overall survival of ccRCC patients. We demonstrated that the expression of RALYL was significantly low in ccRCC and correlated with a poor prognosis in a large number of clinical samples. Our findings showed that RALYL may be a potential therapeutic target as well as a poor prognostic factor.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.111-119
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• 2000

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a common pediatric discase presenting most frequently with skin, gastrointestinal, joint and renal manifestations. The prognosis of HSP is mainly determined by the involvement of the kidney, but prognostic markers have not been established. We evaluated the patients who have HSP nephritis with nephrotic syndrome. Method : Clinical manifestations and laboratory findings were observed and analyzed in 34 cases with HSP which were manifested by nephrotic syndrome hospitalized at Kyung Hee university Hospital during the period from Jan. 1990 to Dec. 1998. Results : 1) Male to female ratio was 1.3:1, and mean age at onset was 8.3 year. 2) Mean duration from symptom onset to renal biopsy was 10.5 weeks. 3) Proportion of patients presenting with acute nephritis was 32.4$\%$, gross hematuria 17.6$\%$, microscopic hematuria 50$\%$. 4) The findings of renal biopsy were 20 cases of grade II, 11 cases of grade III, 2 cases of grade I, 1 case of grade IV according to classification by ISKDC. 5) Patients with grade I were recovered with no residual defect, but patients with grade IV shows active renal disease(states C). Conclusion : Among the 디le patients with Henoch-$Sch{\ddot{o}}nlein$ purpura accompanying nephrotic syndrome, more aggressive treatment might be needed in patients showing crescents formation on renal biopsy. A prospective study will be needed to explore the progression of this disease.

• 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)