• Childhood Kidney Diseases
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• v.6 no.2
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• pp.131-141
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• 2002

Purpose : Renal transplantation which allows children normal growth and development and a return to normal life. is now proven to be the best modality for children with ESRD Up to Recently, the number of renal transplantations in Asia has rapidly increased and the outcome has also improved. This investigation was planned to estimate the current status of pediatric renal transplantation in Asia and to find the keys for better improvement of outcome in pediatric renal allograft in Asian countries. Material and methods : The participating countries and institutions for this investigation were China, Hong Kong, India, Indonesia, Japan, Malaysia, Pakistan, Philippines, Singapore, Thailand, Korea, KSPN (Korean Society of Pediatric Nephrology), KONOS (Korean Network for Organ Sharing). Results : Many countries in Asia still do not have a well organized nation wide renal transplantation registration system independently in the pediatric field. So it's very difficult to evaluate the real state of pediatric transplantation among Asian countries. According to the estimation with fragmented data from each countries, in the front running countries of pediatric renal transplantation in Asia, about 40 or more transplants were performed in each country per year and the five year actuarial renal allograft survival was around 80% which is similar to that of western countries. But there were large gaps among the behind groups. Conclusion : Vigorous attempts to perform renal transplantation for children especially younger than 5 years old would be encouraged as well as organ donation from brain dead donor and non heart beating cadaveric donor also should be activated to cope effectively with the shortage of living donor supply. Large number of recent reports shows the favorable outcome of pre-emptive renal transplantation, we should make more efforts toward pre-emptive renal transplantation. First of all, in order to improve the outcome and to narrow the gap between Asian countries in pediatric renal transplantation, effective and continuous efforts to establish nationwide pediatric renal transplantation registration program as well as official, nation-to-nation data sharing program should be needed.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.188-197
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• 2002

Purpose : Nowadays, drinks, foods and snacks have frequently been intensified with calcium and the insights into the importance of calcium-intake in general has developed in Korea. In this decade, we found the numbers of children who was visited to our hospital for evaluation of hematuria defined with hypercalciuria were increased. So we tried to compare the mean levels of serum calcium, alkaline phosphate, sodium, potassium, chloride, BUN, creatinine, bicarbonate and urinary pH who visited our hospital in 1991, 1992 with in 2000, 2001. Materials and methods : Between January 1991 to December 1992, and between January 2000 to December 2001, each 366 children and 488 children, aged 1 month to 15 years, who presented in our hospital for tonsilectomy and adenoidectomy or for inguinal herniorrhaphy were enrolled in the study, The children in the study were checked the level of serum calcium, alkaline phosphate, sodium, potassium, chloride, BUN, creatinine, bicarbonate and urinary pH with the machine which was corrected the similar levels of practical chemical levels in serum. We compared each mean levels in 1991s' group with in 2001s' group totally and separately through the age and sex. We used t-test to analysis data. Results : The levels of serum calcium, alkaline phosphate, creatinine, sodium, potassium, and urinary pH of 2001s' group were significantly higher than the levels of 1991s' group(P<0.05). The each level was $9.91{\pm}0.50\;mg/dL,\;248.58{\pm}94.98\;U/L,\;0.61{\pm}0.14\;mg/dL,\;138.64{\pm}2.22\;mM/L,\;4.35{\pm}0.40\;mM/L,\;6.18{\pm}0.86$ in 2001s' and $9.13{\pm}0.68;mg/dL,\;198.26{\pm}79.34\;U/L,\;0.433{\pm}0.18\;mg/dL,\;137.86{\pm}2.67\;mM/L,\;4.22{\pm}0.36\;mM/L,\;5.83{\pm}0.95$ in 1991s'. And the levels of serum bicarbonate, $23.64{\pm}2.57\;mM/L$ in 2001s' was significantly lower than the 1991s', $24.60{\pm}2.23\;mM/L$(P<0.05). The similar results were detected each age and sex group. Conclusion : The levels of serum calcium increase in this decade. The results will be used as a basic data for the national health plan in the years to come.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.209-217
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• 2002

Purpose : Corticosteroid has been used as the mainstay therapy of childhood NS. But SIO is one of the serious complications of long-term steroid therapy, especially in growing children. Recently calcium, calcitonin, PTH, vitamin D and bisphosphonate has been used to treat or prevent SIO in adult, which is rare in children with NS. We studied the effect of $1{\alpha}-(OH)D_3$ and Pamidronate on SIO using dual energy X-ray absorptiometry (DEXA). Patients and methods : We studied thirty patients who admitted in the Dept. of Pediatrics of Kyung Hee Medical Hospital with NS. All patients was received longterm steroid therapy. There was no history of bone, liver, or endocrine disease. The samples, serum protein, albumin, BUN, creatinine, calcium, phosphorus, and BMD were obtained before and the six months after the dose of $1{\alpha}-(OH)D_3$ and Pamidronate, respectively Results : The mean age was $6.9{\pm}3.3\;and\;6.5{\pm}2.5$ years old. The mean duration of steroid therapy was $28.8{\pm}1.8\;and\;27.6{\pm}1.0$ months. The changes of serum protein, albumin, BUN, creatinine, calcium and phosphorus level between pre-treatment and post-treatment did not show statistical significance in both $1{\alpha}-(OH)D_3$ and Pamidronate treatment group. However, BMD was increased in both from $0.472{\pm}0.12\;and\;0.457{\pm}0.10\;g/cm^2\;to\;0.533{\pm}0.12$ and $0.529{\pm}0.09\;g/cm^2$ after treatment. (P<0.05) Conclusion : Both $1{\alpha}-(OH)D_3$ and Pamidronate appears to be effective in treating and preventing SIO in children with nephrotic syndrome requiring long-term steroid therapy.

• 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.3 no.1
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• pp.80-87
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• 1999

Purposes : Renal involvement is a potentially serious complication of systemic lupus erythematosus (SLE). There have been only few studies of lupus nephritis in pediatric age. In this study, the clinical manifestations, pathologic findings, response to treatment, and clinical course of lupus nephritis in children were analyzed. And the results will provide basic data for future nation-wide prospective multi-center study. Methods . The medical records of 46 children clinically and pathologically diagnosed to have lupus nephritis at Seoul National University Children's Hospital during 1986 to 1997 were analyzed retrospectively. Results : 1) The median age of diagnosis of lupus nephritis was 12.8 years ($2\;years\~\;15year$ 8months), and the sex ratio was 1:2.5. 2) FANA($85.7\%$), anti-ds-DNA antibody ($78.0\%$), and malar rash ($60.8\%$) were the most common findings among the classification criteria by ARA Decreased C3 was detected in $88.9\%$ of patients. 3) Hematuria ($87.0\%$) was the most common renal symptom, and WHO class IV lupus nephritis was identified in 41 cases by renal biopsy. 4) In most of patients, the disease activity was controlled relatively well with a single or combined therapy of prednisolone, azathioprine, or cyclophosphamide. The response revealed no difference according to the mode of treatment. 5) Infection, especially of Varicella-Zoster virus and candida, was the most common complication during the disease course. Conclusion : The renal involvement was noted in $87.0\%$ of childhood SLE, and $89.1\%$ of renal lesions was WHO class IV lupus nephritis known to associated with poor long-term prognosis. So, aggressive treatment using immunosuppressants in the early disease course may be helpful to increase long-term prognosis of lupus nephritis. A prospective multi-center study is necessary to analyze the therapeutic efficacy of various treatment modalities.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.27-34
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• 1999

Purpose: This study was designed to investigate the changes in soluble interleukin-2 receptor (sIL-2R) level in sera and urines of children with primary nephrotic syndrome, eliminating the confounding effects of age, proteinuria, and steroid treatment. Methods: Soluble IL-2R was measured by ELISA in sera and urines from patients with minimal change nephrotic syndrome or focal segmental glomerulosclerosis as well as from healthy controls. The serum levels and urinary sIL-2R/creatinine ratios were compared between control group and the 12 patient groups divided by their ages (0-1, 2-4, over 5 years), and presence or absence of proteinuria and/or steroid treatment (PU+Tx-, PU+Tx+, PU-Tx+, PU-Tx-). Results: Though the differences were not statistically significant probably because of the small numbers, serum sIL-2R levels seemed to be higher in younger age groups both in patients and control group. Nephrotic children did not show higher serum levels than normal children. Among the patients, proteinuric condition seemed to raise and steroid treatment tended to suppress the serum sIL-2R levels. Urinary sIL-2R/creatinine ratios were higher in younger age groups, more significantly in patients (P<0.001). Proteinuria and steroid treatment affected the urinary sIL-2R/creatinine ratios by the same way as the serum sIL-2R levels. Serum sIL-2R levels and urinary sIL-2R/creatinine ratios were not different between groups of different histologic findings or steroid responsiveness (P>0.05). Conclusion: Serum sIL-2R levels and the urinary sIL-2R/creatinine ratios were higher in younger age, and they were not higher in nephrotic patients compared to control group. The patients in relapse showed higher levels, while the levels were suppressed with steroid treatment. In proteinuric state, urinary sIL-2R/creatinine ratios reflected serum sIL-2R levels.

• 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.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.152-160
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• 1998

Purpose : Vesicoureteral reflux (VUR), which can result in renal failure in children, is expected to resolve spontaneously in mild cases, but severe cases require surgical correction, posing dilemma in deciding the right measures in some cases. Therefore, to delineate the factors affecting spontaneous resolution of VUR and to apply them in assessing the prognosis, we reviewed the patients with VUR who had been treated medically. Patients and Methods : 32 children (49 renal units) with reflux who had been admitted during the 5-year period from Jan '92 to Dec '96 were classified into 4 groups (Resolved, Improved, Unchanged, and Worsened) and analyzed. Results : 1) 25 were boys and 7 girls. The age at diagnosis ranged from 13 days to 9 years (mean $24.6{\pm}11.4$ mo) 2) Among 49 refluxing ureters, 4 were below 1 me of age,21 between 1 me to 1 yr, 16 between 1 yr to 6 yr, 8 beyond 6 yr. Two belonged to Grade I, 16 to Crade II, 17 to Grade III, 12 to Grade IV, and 2 to Grade V. 3) Spontaneous resolution rates of reflux were 100, 81, 47, 8, 0$\%$ for each Grade, respectively. Resolution plus improvement rates in Grade III and IV were 71 and $50\%$, each. 4) Resolution rates in relation to the age at diagnosis were $100\%$ for below 1 mo, $48\%$ between 1 mo to 1 yr, $56\%$ between 1 to 6 yr, and $13\%$ beyond 6 yr. 5) Of 15 unilateral refluxing ureters, 14 ($93\%$) resulted in resolution. Of 34 bilateral refluxing ureters, 11 ($32\%$) resolved spontaneously 6) Resolution occurred within 1 year in 20 units of 24 renal units in regular follow-up. Conclusion: The lower the initial grade and the younger the patient, the sooner the reflux resolved. Resolution was better when VUR was unilateral than bilateral. Long-term regular follow-up is essential because even the cases falling in Grades III and IV have high rates of spontaneous resolution and improvement with medical treatment.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.20-26
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• 1999

Purpose : Several modulatory factors for renal peripheral benzodiazepine receptor (PBR) has been reported, but their physiological significance remains elusive. Tissue-specific, stress-induced down-regulation of renal PBR coupled with the pharmacological stimulation of these effects by angiotensin II suggested that physiological significance of renal PBR may be related to the pathophysiology of stress-induced hypertension. The boderline hypertensive rat (BHR) has been used extensively to study the interaction of environmental factors, such as stress and blood pressure. The BHR is the first-generation progeny of a cross between the spontaneously hypertensive rat and the control Wistar-Kyoto rat. The pathogenesis of stress induced hypertension in this model is not demonstrated well. Methods In this study, BHR (male, 150-200 g) and Sprague-Dawley (SD, male, 150-200 g) rats were treated by repeated immobilization to induce anxiety. We used plus-maze performance to observe the level of anxiety by measuring percent open crosses and percent time in open. Results : Percent open crosses and percent time in open in BHR were lower than in SD rats (P<0.05). Receptor densities of renal PBR in BHRs were significantly lower than those of SDs (P<0.05). We also observed that the renal PBR was upregulated in the repeatedly stressed (immobilization, 2 hours daily, for 2 weeks) rats, both in the BHR and SD. However, the density of renal PBR in the stressed BHR was still lower than that of stressed SD. Renal PBR has been suggested to be an important organs which Is responsible for the production of cholesterol-derived products during stress. Conrlusion : From these results, it can be summarized that the lowed density of renal PBR may be involved in the pathogeneis of stress-induced hypertension.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.35-41
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• 1999

Purpose The single administration of PAN(Puromycin-Aminonudeoside) to rats results in nephropathy that are similar to human minimal change nephrotic syndrome. Recently several studies indicate the pathophyslological importance of oxygen free radicals in rats with PAN-induced nephrosis. This study was conducted to evaluate the effect of $\alpha$-tocopherol, an oxygen free radical scavenger, on the histologic and biochemical changes of PAN-induced nephrosis in rats. Methods : Twenty-one Sprague-Dawley rats weighing 180-300 gm were divided into 3 groups. In group I (control group), the rats were given saline intraperitoneally for 12 days, in group II the rats were given PAN 7.5mg/100g of body weight intravenously one time and group III PAN intravenously, followed by $\alpha$-tocopherol 0.5 mg/100g of body weight jntramuscularly for 12 days. Twenty four hour urinary protein and creatinine excretion were measured on day 0, 5, 11 and 18. On the 18th day, rats were sacrificed for the determination of total serum protein, albumin and cholesterol levels. To estimate renal injuries by oxygen free radical, lipid peroxide concentration and reduced glutathione were measured in renal cortex. Histological examination in rat glomerular lesions were performed. Results : From the 5th days of PAN administration, urine protein/creatinine of group II and III were significantly increased compared the group I (P<0.05). But, urine protein/creatinine of group III was significantly lower than group II at 18th days (P<0.05). Total serum protein and albumin of group II were significantly lower than those of group III (P<0.05). Serum cholesterol of group II was significantly higher than that of group III (P<0.05). Lipid peroxide and reduced glutathione in renal cortex of group II were significantly higher than that of group I and III (P<0.05). Electron microscopic strudies of group II showed the loss of epithelial foot processes, but in group III showed preservation of epithelial foot processes. Conclusion : PAN-induced nephropathy was ameliorated significant recovery of foot process change and reduction of the urinary protein excretion by antioxidant, $\alpha$-tocopherol.