• Childhood Kidney Diseases
• /
• v.5 no.2
• /
• pp.164-175
• /
• 2001

Purpose : Alport syndrome is a hereditary nephrotic disease characterized by progressive nephrotic symptom, sensorineural hearing loss, ophthalmic abnormality, typical microscopic findings, and familial occurrence. In this study, we tried to find the risk factors related with its prognosis by taking a close observation on clinical symptoms of children with Alport syndrome reviewing retrospectively. Materials & methods : We chose children diagnosed as Alport syndrome in renal biopsy during 20 years(from 1980, Jan. until 1999, Dec.) who could receive follow up studies in tile department of pediatrics. They were divided into two groups by comparing renal function at the time of diagnosis and at current status. We compared several clinical aspects in them, and applied nonparametric test for statistical analysis. Results : The sex ratio(male:female) of 24 children was 3:1. The most common clinical symptom presented at their first visit was gross hematuria. Among those 24 children, 11 cases($46\%$) of progressing into chronic renal failure(Group II) were observed. Hypertension, proteinuria and edema were seen much frequently in group II. The level of serum protein, albumin, and creatinine clearance were decreased while BUN, creatinine were relatively increased. All the results were statistically significant. Conclusion Clinically significant risk factors related to prognosis in Alport syndrome were the presence of hypertension, edema, and proteinuria at the time of diagnosis. Also, the level of serum protein, albumin, BUN, creatinine, and glomerular filtration rate were proved to be important factors in predicting prognosis. We believe that studies on these possible risk factors would be of great help in treating and predicting prognosis of children suffering with Alport syndrome. (J Korean Soc Pediatr Nephrol 2001;5 : 164-75)

• Childhood Kidney Diseases
• /
• v.6 no.1
• /
• pp.48-55
• /
• 2002

Purpose: Thin glomerular basement membrane nephropathy (TGBMN) is recognized as the leading cause of microscopic hematuria in both children and adults. However thinning of glomerular basement membrane (TCBM) has been found in healthy adult and also is known to be associated with various renal diseases such as Alport syndronh, IgA nephropathy and mesangial proliferative glomerulonephritis. The association of TGBM with minimal change nephrotic syndrome (MCNS) has been very rare so that the present study was undertaken to determine the relationship between TGBM and MCNS. Methods: The study population consisted of 49 children with biopsy- proven MCNS who have been admitted to the pediatric department of Kyungpook University Hospital during the past 5 years from 1997 to 2001. Group I consisted of 8 children associated with TGBM and Group II 41 children without TCBM. Various parameters such as age of illness, duration from discovery of illness to the time of biopsy, family history of hematuria and other laboratory tests were compared between these two groups and the following results were obtained. Results: Age distribution showed slightly older age in Group I ($7.1{\pm}3.5$ years) compared to Group II ($4.8{\pm}2.9$ years). However this was not statistically different (P=0.056). Family history of hematuria was noted in 2 cases in Group II. Though statistically not significant, hematuria was seen in 2 out of 8 cases ($25\%$) in MCNS children with TGBM, compared to 7 out of 41 cases ($17\%$) with MCNS children without TGBM. Other parameters such as BUN, creatinine, 24 hours urine protein excretion, serum protein, albumin, cholesterol, and T4/T8 ratio, showed no difference. Also renal biopsy finding showed no significant difference and the thickness of glomerular basement membrane in Croup I was $188{\pm}30nm$. Conclusion: TGBM was found in 8 out of 49 children with MCNS ($16.3\%$). And this high frequency of occurrence indicates that these association is not an incidental findings. Typical clinical findings of TCBMN was not noted in all of the 8 children with MCNS associated with TGBM, suggesting that thinning of glomerular basement membrane (TCBN) is secondary to rather than the cause of MCNS. (J Korean Soc Pediatr Nephrol 2002;6: 48-55)

• Childhood Kidney Diseases
• /
• v.6 no.1
• /
• pp.61-67
• /
• 2002

Purpose: The urinary mass screening program in school aged population has been performed since 1981, but the consensus on the follow-up schedule and the management of isolated proteinuria has not been reached yet. The aim of this study was to investigate the cause of isolated proteinuria and to propose a guideline for the treatment and follow-up afterwards Methods: The medical records of 114 cases of isolated proteinuria detected through the analysis of urinary mass screening and evaluated at the pediatric outpatient clinic of Asan Medical Center from January 1990 to July 2001 have been reviewed. Results: The classification of isolated proteinuria was as follows. Transient proteinuria $32\%$, orthostatic proteinuria $65\%$, persistent proteinuria $3\%$, In orthostatic proteinuria group, daytime and nighttime proteinuria were $319.2{\pm}189.1\;mg/dL$ and $56.5{\pm}56.1\;mg/dL$. In persistent proteinuria group, daytime and nighttime proteinuria were $1140{\pm}540.5\;mg/dL$ and $289{\pm}58\;mg/dL$. After 30 month follow-up, 2 cases of persistent proteinuria were needed renal biopsy and 1 case revealed focal segmental glomerular sclerosis. In all cases, serum creatinine, albumin and complements levels were normal. In the orthostatic proteinuria group, no significant renal diseases were detected. Conclusion: Since most of the isolated proteinuria detected through the school urinary mass screening were orthostatic proteinuria or transient proteinuria, initially aggressive diagnostic method such as renal biopsy is not needed and regular follow- up with quantitation of proteinuria is warranted.(J Korean Soc Pediatr Nephrol 2002 ; 6 61-7)

• Childhood Kidney Diseases
• /
• v.6 no.1
• /
• pp.68-74
• /
• 2002

Purpose: Suprapubic bladder aspiration(SBA) of urine is the most reliable method to obtain urine avoiding contamination in non-toilet trained infants. Ultrasonography is a useful tool for guiding the anatomic location as well as for direct visualization during procedure. We evaluated the success rate and complication of ultrasound(US) assisted SBA Methods. Sixty infants who visited Ewha Womans University Mokdong Hospital, with suspected urinary tract infection were randomly divided into the US assisted (n=32) and blind SBA(control, n=28) group. In US assisted SBA group, the anteroposterior(AP), transverse, and sagittal diameters and the volume of the bladder were measured. In the blind SBA group, urine was blindly aspirated when the urinary bladder was palpated at the suprapubic area. The rate of successful urine aspiration, the number of attempts until successful aspiration, aspirated urine volume were compared between the two groups. Results: The success rate was $100\%$(32/32) in the US assisted group, which was significantly higher than $85.7\%$(24/28) of the control group (P<0.05). The aspirated urine volume in the US assisted group was $7.4{\pm}3.7\;mL$, which was significantly higher than $4.5{\pm}3.4\;mL$ of the control group (P<0.05), The diameters and volume of bladder in successful aspiration were $2.1{\pm}0.7\;cm$ in AP diameter, $3.1{\pm}0.6\;cm$ in transverse diameter, $4.2{\pm}1.0\;cm$ in sagittal diameter and $15.2{\pm}10.4\;mL$ in volume, which were significantly higher than those ($1.7{\pm}0.3\;cm,\;1.7{\pm}0.3\;cm,\;1.8{\pm}0.7\;cm,\;2.4{\pm]0.6\;cm,\;3.9{\pm}2.5$) of the control group (P<0.05) The correlations between the AP(r=0.78), transverse (r=0.72), sagittal(r=0.91) diameter and bladder volume were significant (P<0.05). SBA was $100\%$ successful in the AP diameter >3 cm, transverse diameter >4 cm, depth >4 cm and bladder volume >5 mL. Conclusion: US assistance can significantly improve the success rate of SBA in infant with suspected urinary tract infection. (J Korean Soc Pediatr Nephrol 2002 ; 6 : 68-74)

• Childhood Kidney Diseases
• /
• v.5 no.2
• /
• pp.100-108
• /
• 2001

Purpose : We investigated the incidence and predisposing factors of pulmonary embolism in minimal change nephrotic syndrome(MCNS). Methods : Lung perfusion scan using 99mTC-MAA were done on 14 patients who were diagnosed to minimal change nephrotic syndrome. Group h: Five patients who had perfusion defects on scan, Group B; Nine patients who had no perfusion defect on scan. Between the two groups, the differences of platelet number, hematocrits, albumin, cholesterol, triglyceride, proteinuria were evaluated. Results : Five patients were found to have perfusion defect consistent with pulmonary embolism($35.7\%$). However, there were minimal or no respiratory symptoms and signs. In our laboratory studies, the mean proteinuria on admissions was $676{\pm}31\;mg/m2/hr$ in the group with pulmonary embolism, and $313{\pm}28\;mg/m2/hr$ in the group without pulmonary embolism. There were more severe proteinuria in group with pulmonary embolism(P<0.05). The mean platelet count at early stage of remission after steroid treatment was $746,600{\pm}280,000/mm3$ in the group with pulmonary embolism, $511,890{\pm}90,000/mm3$ in the group without pulmonary embolism. There were significant difference of platelet count between the two groups(P<0.01). In patients with pulmonary embolism, there were more higher and sustained increasement of platelet count. All cases of pulmonary embolism were treated with dipyridamole(5 mg/kg). In 4 cases the perfusion defects were improved in two weeks, however, one case showed persistent perfusion defect after 1 month. Conclusion : Our study suggested that pulmonry embolism might be one of tile major complications in childhood MCNS The occurrence rate was correlated with severity of proteinuria before treatment and sustained increasement of platelet counts in early remission state after steroid treatment. Therefore, the scintigraphic pulmonary perfusion study is mandatory in childhood MCNS, especially in the high risk patients, such as the patients with severe proteinuria and sustained increasement of platelet count. (J Korean Soc Pediatr Nephrol 2001;5 : 100-8)

• Childhood Kidney Diseases
• /
• v.6 no.1
• /
• pp.75-84
• /
• 2002

Purpose Acute pyelonephritis of growing kidneys may result in renal scarring. TGF-${\beta}1$, inflammatory cytokine, has been suggested to play an important role in promoting renal scarring through apoptosis, suppression of cellular proliferation and fibrosis. We observed the effects of a potent anti-inflammatory agent, methylprednisolone on apoptosis and renal scarring in experimentally induced acute pyelonephritic weaning rats. Materials and Methods: To induce ascending pyelonephritis a saline solution containing Escherichia coli type ATCC No. 25922, pili- form (107 bacteria/mL) was infused into the bladder through the 16-guage silicone cannula for 48 hours to 102 three-week-old Sprague-Dawley rats (50-60g). Experimental groups were divided into three groups according to the treatment protocols, group I (ceftriaxone only, n=3l), group II (methylprednisolone+ceftriaxone n=28), control group (n=43) was not treated. Histopathologic scores of inflammatory changes, fibrosis and tubular atrophy, the apoptosis index and TGF-${\beta}1$ expression score were observed at post-infection 1 and 3 week. Datas were analysed using ANOVA test and P value below 0.05 was interpreted as significant. Results: The mortality rate ($21.4\%$) of group II was not different to the control group ($41.9\%$) and group I ($32.3\%$). The inflammatory score of group II ($0.8{\pm}0.87$) at week 1 was significantly lower than those of the control group ($2.3{\pm}0.87$) and Group I ($1.7{\pm}0.79$) (P<0.05). Apoptosis index of group II ($2.9{\pm}2.15$) at week 1 was significantly lower than those of the control group ($10.0{\pm}1.95$) and group 1 ($8.3{\pm}2.53$) (P<0.05). TCF-${\beta}1$ expression score of group II ($0.8{\pm}0.72$) at week 1 was significantly lower than those of the control group ($1.9{\pm}0.68$) and group I ($1.8{\pm}0.60$) (P<0.05). The fibrosis score of group II ($1.1{\pm}1.10$) at week 3 was significantly lower than that of the group I ($1.8{\pm}0.83$) (P<0.05) Conclusion: Conclusion Combined treatment with methylprednisolone and ceftriaxone reduced inflammation, fibrosis, apoptosis and TGF-${\beta}1$ expression in acute pyelonephritic weaning rats, compared to ceftriaxone alone. Anti-inflammatory agent supplemented to antibiotics could prevent renal scarring more effectively. (J Korean Soc Pediatr Nephrol 2002 ; 6 : 75-84)

• Childhood Kidney Diseases
• /
• v.5 no.2
• /
• pp.109-116
• /
• 2001

Purpose Long- term use of steroid, cyclophosphamide and cyclosporin, which are frequently used in the therapy of SDNS, might cause severe side effects. Recently, the immune-modulator levamisole has been tried as a substitute therapy and it has been reported as a method with less side effects and more effectiveness. We started this research in order to observe the effects of levamisole and compare it to other therapy results. Patients and Methods : We chose 16 steroid dependent nephrotic syndrome children, those who had shown frequent relapse during the immunocompromised therapy period. Mean age was $9.1{\pm}1.4$ years in children and the male to female ratio was 15:1. All of subjects were diagonized with MCNS and had received cyclophosphamide or cyclosporin before receiving levamisole. Levamisole at a dose of 2.5mg/kg was used every other day for 1 year and the relapse rate was observed. Results : On average of 14 days after treatment, complete remission was visible in all of the children, and the relapse percentage was $50\%$, which represents 8 children, while remaining 8 children representing $50\%$ of the cases showed no relapse during treatment. During the levamisole therapy period, tile average relapse rate was reduced significantly from $2.18{\pm}0.9/year\;to\;0.77{\pm}0.9/year$(P=0.027). Also the average relapse rate after the therapy was reduced to $1.34{\pm}1.1/year$, which was a significant level compared to the level before treatment(P=0.003). There was no significant difference in terms of duration of remission maintenance. Duration of remission maintenance showed an average of $12.2{\pm}9.1$ months before the use of levamisole, but it was also $10.1{\pm}6.9$ month after therapy. No other side effects such as leukopenia, skin disease and other clinically significant symptoms appeared at all during therapy. Conclusion : The long-term medication of levamisole for the therapy of SDNS children is thought to be able to maintain stable remission by reducing the relapse frequency without causing severe side effects. Further study with a broader range of subjects is required to eluccidate the long-term effects of this treatment. (J. Korean Soc Pediatr Nephrol 2001;5 : 109-16)

• Childhood Kidney Diseases
• /
• v.5 no.2
• /
• pp.125-135
• /
• 2001

Purpose : Efforts to predict long-term outcome of focal segmental glomerulosclerosis(FSCS) have been made but have yielded conflicting results. Reports are rare especially in Pediatric patients. In this study, we reviewed the predictable prognostic factors in patients of FSGS Method : Fifty children who diagnosed as biopsy-proven FSGS at department of pediatrics at Yonsei university were studied retrospectively. Based on medical records, response to treatment and pathologic slides, we compared normal renal function group and decreased renal function group, assessed the factors affecting renal survival and progression to renal failure. Results : The mean age at onset was 8 1/12 years, sex ratio was 2.3 : 1, and the mean duration of follow-up was 7 1/12 years. The overall renal survival rate was $34\%$ at 5 years, $8\%$ at 10 years Five-year survival rate was $74\%$ in normal renal function group and $27\%$ in decreased renal function group. Between the two groups, there were no significant differences in age at onset, sex ratio, amount of proteinuria, incidence of hematuria and hypertension, mesangial hypercellularity. Decreased renal function group showed higher serum creatinine level, poor response to treatment, higher percent of glomeruli with sclerosis, moderate to severe tubulointerstitial change and vascular change(P<0.05). The prognostic factors of renal survival rate were same as above and incidence of hypertension also affected renal survival( P<0.05). The progression rate to renal failure did not show statistically significant factor. Conclusion : We reviewed the factors affecting long-term outcome of FSGS. Serum creatinine level, steroid responsiveness, and the degree of glomerulosclerosis were significant prognostic factors. (J Korean Soc Pediatr Nephrol 2001 ;5 : 125-35)

• Childhood Kidney Diseases
• /
• v.5 no.2
• /
• pp.156-163
• /
• 2001

Purpose We performed urinary mass screening(UMS) program for 2,804 children of second grade elemantary school 8 years of age in Paju city with cooperation of Paju City Health Center to determine the prevalence of asymptomatic proteinuria and hematuria, and to estimate the risk of incipient renal diseases. Also we attempted to evaluate the significance of hematuria in UMS in addidtion to proteinuria. Methods : 2,804 children of the 2nd grade of elementary school who lived in Paju city were included to our UMS program in 2000. They were constituted with 1,428 boys and 1,376 girls. The screening program was carried out in 3 steps The 1st screenig test was performed at schools and then students with abnormal results were examined repeatedly at Paju City Health Center and our hospital. Those students who showed proteinuria and/or hematuria in the 1st and 2nd test were referred to our hospital to undertake the 3rd close examination including physical examination, laboratory tests and radiologic tests. Results : (I) The prevalence of urinary abnormality in the 1st screening test was $8.3\%$(233 students), comprised of $5.9\%$ of boys, $10.8\%$ of girls. (2) Among 2,804 children tested in the first screening, prevalences of asymptomatic proteinuria and isolated hematuria were 64($2.3\%$), 163($5.8\%$) respectively, and the prevalence of proteinuria with hematuria was 6($0.2\%$). (3) Among 233 students with urinary abnormalities at the 1st screening test, 102 students applied to the 2nd test. 32 children, about one third of them, were also found to have abnormal urinary findings ; isolated hematuria 30, proteinuria with hematuria 2. (4) Those findings of clinical evaluation for children with isolated hematuria at the hospital showed as follows: idiopathic isolated microscopic hematuria 21, normal 6, urinary tract infection 1, idiopathic hypercalciuria 1 and simple renal cyst 1. Those 2 students with proteinuria and hematuria seemed to have chronic glomerulonephritis. Conclusion : (1) The clinical evaluation for children who showed positive results at the 1st screening test should be done judiciously. Because of high false positive rate, almost who showed positive results was normal, only a few of them had pathologic conditions. In this study, actual incidence of incipient renal diseases in children of 8 year old was calculated to be $0.4\%$. (2) The definite conclusion whether a urinary mass screening test can alter the prognosis of incipient renal diseases could not be drawn with this study. Further study must be necessary (3) We could acknowledge the significance of hematuria in UMS, but it is necessary that one should be judicious in managing and follow-up those that show abnormal results. (J Korean Soc Pediatr Nephrol 2001;5 : 156-63)

• Childhood Kidney Diseases
• /
• v.5 no.2
• /
• pp.196-205
• /
• 2001

Purpose : Membranous glomerulopathy is a glomerular disease characterized by the presence of subepithelial immune deposits with thickening of the capillary wall of the glomerulus without inflammatory change. The pathogenesis of membranous glomerulopathy is still unknown. Its incidence is higher in males, and it is rarely found in infants and adolescents. Among the clinical manifestations proteinuria is most common, while edema and hematuria are present. According to reports from other countries, among few patients diagnosed with membranous glomerulopathy by renal biopsy, show isolated microscopic hematuria without the clinical manifestations. Little research in this area has been performed in Korea, and so we conducted retrograde studies on membranous glomerulopathy associated with isolated microscopic hematuria. Materials and Methods : We analyzed retrogradely 109 cases of asymptomatic isolated microscopic hematuria that were diagnosed as membranous glomerulopathy by renal biopsy at Yonsei University Severance hospital from January, 1992 to July, 2001. Results : In 87 of the 109 cases patients were over 15 years old while in 22 cases patients were under 15 at the time of dignosis. Only three patients showed isolated microscopic hematuria without the clinical manifestations and abnormal laboratory findings and they were all male patients under 15 years old. Conclusion : Few cases of the membranous glomerulopathy show only asymptomatic isolated microscopic hematuria However, since membranous glomerulopathy can be found in patients who present with asymptomatic isolated microscopic hematuria only, if adequate indication for renal biopsy is present, we conclude that renal biopsy must be aggresively pursued in order to find the underlying disease. (J Korean Soc Pediatr Nephrol 2001 ; 5 : 196-205)