• Clinical and Experimental Pediatrics
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• v.49 no.7
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• pp.737-744
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• 2006

Purpose : To assess the usefulness of $^{99m}Tc-DISIDA$ scanning in the early evaluation of neonatal cholestasis and to verify the diagnostic value of this test in the differential diagnosis of biliary atresia. Methods : DISIDA scannings were performed and analyzed in 87 children(58 males and 29 females; age, 18-139 days, mean, 59.1 days) with neonatal cholestasis. Five groups according to the final diagnosis and the results of DISIDA scanning were analyzed by scatter plots using the parameters of age and the level of liver function tests(direct bilirubin, AST, ALT, ALP, GGT). The diagnostic sensitivity, specificity and accuracy of DISIDA scanning in the diagnosis of biliary atresia were compared between a higher bilirubin group and a lower bilirubin group(direct bilirubin level >5 mg/dL vs. <5 mg/dL) decided by the pattern of scatter plots. Results : DISIDA scannings in the diagnosis of biliary atresia were analyzed by high sensitivity(100 percent, 16/16) but lower specificity(70.4 percent, 50/71) and accuracy(75.9 percent, 66/87). False positivity(29.6 percent, 21/71) was higher in patients with a higher direct bilirubin level(42.5 percent for >5 mg/dL vs. 9.7 percent for <5 mg/dL, P<0.01). The age and the level of liver function tests(AST, ALT, ALP, GGT) analyzed by scatter plots revealed neither diagnostic value in predicting final diagnosis nor estimated the accuracy rate of DISIDA scanning in the evaluation of neonatal cholestasis. Conclusion : We suggest that DISIDA scannings should not be routinely used in evaluating neonatal cholestasis with elevated direct bilirubin level(>5 mg/dL), especially if it delays early diagnosis and surgical intervention.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.46-55
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• 2005

Purpose : Recurrent urinary tract Infection(UTI) in primary vesicoureteral reflux(VUR) may lead to serious renal scarring, a major cause of childhood hypertension and end-stage renal disease. To prevent recurrent UTI, low-dose long--term antibiotic prophylaxis has been recommended. However, recurrent UTI still develops during antibiotic prophylaxis, the efficacy of which is now being disputed. The emergence of resistant bacteria has also raised concerns. To evaluate the effect of antibiotic prophylaxis, we investigated recurrent UTI during prophylactic antibiotic use in children with primary VUR Materials : The incidence and risk factors of recurrent UTI were retrospectively evaluated in ninety-one children with primary VUR on trimethoprim- sulfamethoxazole(TMP/SMX) prophylafis during the year following their index febri]e UTI. Results : Recurrent UTI occurred in 31.9%(29/91) children and comprised 0.32 episodes/patient year. Febrile UTI was 0.26 episode/patient year and afebrile UTI was 0.07 episodes/patient year. The recurrent rate of UTI in male patients with phimosis was 37.2%(19/51), which was significantly higher than in males without phimosis 0%(0/5)(P=0.025). In the logistic regression analysis for recurrent UTI, renal scar was the significant risk factor for recurrent UTI [RR 3.8(95% CI 1.0-14.1) P=0.04]. For other well-known risk factors such as sex, age, degree of VUR, APN, and voiding dysfunction, the differences were not significant. Conclusion : TMP/SMX prophylaxis did not prevent recurrent UTI in children with primary VUR. Phimosis and renal scars were the risk factors for recurrent UTI but the grade of primary VUR was not. In VUR without phlmosis and renal scar, a randomized controlled study without antibiotic prophylaxis is required. (J Korean Soc Pediatr Nephrol 2005;9:46-55)

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.33-39
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• 2006

Purpose : Neonatal hydronephrosis is diagnosed with an incidence of 4.5-7% of pregnancies. Recently, early detection of neonatal hydroneprosis with antenatal ultrasonography has be-come possible. But consensus about its management has not been reached, especially concerning surgical intervention. The purpose of this study is to analyze the natural course of neonatal hydronephrosis and to determine the guideline of surgical intervention and follow up study. Methods : Sixty nine hydronephrotic kidneys were confirmed from April 2001 to April 2005. All cases were rechecked by ultrasonography once at least and had a minimum follow-up of 6 months. We classified the patients into 4 groups according to the anterior posterior pelvic diameter(APPD) on perinatal ultrasonography. Ultrasonography to measure the APPD diameter and Society for Fetal Urology(SFU) grade, $^{99m}Tc-MAG3$ scan were done to a set protocol. Pyeloplasty was peformed according to the protocol. Results : Pyeloplasty was not needed in most cases where the APPD was below 10 mm and where the SFU grade were less than grade 3. Many cases with APPD 10 mm above or SFU grade III above had undergone pyeloplasty. We found a correlation between obstruction grade on MAG3 scan and whether surgery was performed or not. Conclusion : If APPD is above 10 mm, SFU grade is above grade 3 or urinary tract obstruction is suspected by MAG3 scan, pyeloplasty must be considered. In cases where APPD is below 10mm and SFU grade is less than grade 3, we can observe the natural course of neonatal hydronephrosis with consecutive follow-up. (J Korean Soc Pediatr Nephrol 2006;10:33-39)

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.150-156
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• 2003

Purpose : Efforts to predict the clinicopathological outcome of IgA nephropathy have been made but have yielded conflicting results and have not helped in deciding the appropriate timing of the renal biopsy. In this study, we reviewed the predictive factors of clinicopathological outcome for finding out the criteria of renal biopsy timing of IgA nephropathy. Methods : Forty children diagnosed with biopsy proven IgA nephropathy at Wonju Christian Hospital were studied retrospectively, based on medical records. Results : Among 39 patients, 2 children progressed to higher serum creatinine level. One of them reached to the end stage renal disease within 2 year 7 months. According to WHO histopathological classification, there were 15 cases of class I, 14 cases of class II, 7 cases of class III, and 3 cases of class IV. In the mild histological classes(class I, II), gross hematuria was shown in 23 out of 29 children(P=0.02). In the severe histological classes(class III, IV), gross hematuria was noted in 4 out of 10(P>0.05). The tubulointerstitial changes were grade 1 in 24 cases, grade 2 in 4 cases, grade 3 in 8 cases, and grade 4 in 3 cases. With an increase in the tubulointerstitial grade, the 24 hour urine protein/albumin ratio increased. Serum creatinine less than 0.79 mg/dL could predict the lower grade(grade 1 and 2) of tubulointerstitial changes. But serum creatinine greater than 1.13 mg/dL could predict the higher grade(grade 3 and 4) of tubulointerstitial changes. In children with gross hematuria(n=27), serum creatinine was lower(0.78 vs 1.09 mg/dL, P=0.027), serum IgA was higher(316.3 vs 198.8 mg/dL), and the cases of lower WHO classification(I and II) were more common(23 vs 4, P=0.029) than the children with microscopic hematuria. Conclusion : Serum creatinine less than 0.79 mg/dL, macroscopic hematuria, and higher 24 hour urine protein/albumin ratio would predict the lower grade glomerulo tubulointerstitial lesion in IgA nephropathy and could be used as the criteria delaying the renal biopsy.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.161-169
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• 1999

Purpose : The urinary mass screening program for the detection of proteinuria in school aged population has been performed in Seoul since 1981. Systematic evaluation in corporation with the Seoul School Health Center for students with proteinuria identified in the mass screening has been performed from 1987. The results of urinary mass screening up to 1994 was reported. I report here the results of urinary mass screening from 1995 to 1997 and compare them with previous results and attempt to reveal the significance of urinary mass screening. Objects and Methods : In the 3-year period between 1995 and 1997, annually about 460,000 students comprising 3 different age groups; 5th grade of elementary school, 2nd grade of middle school and 2nd grade of high school were chosen, corresponding to the approximate ages of 11, 14, and 17 years, respectively. These subjects accounted for 26% of total school aged children in Seoul. The screening program was carried out in 3 steps. The 1st test was performed with dipstick at school and the 2nd at the Seoul School Health Center. Those students who showed proteinuria in the 1st and 2nd tests were referred to the hospital. Laboratory examinations including renal biopsies were performed to those students with pathologic proteinuria to clarify the incipient renal diseases. Results : 1) The prevalence of asymptomatic proteinuria was 0.28% in the 1st test. It peaked at the group of 14 years old as 0.34%, compared with 0.26% at the group of 11 years old and 0.24% at the group of 17 years old. It reached to 0.26% in male and 0.30% in female. 2) 25 percent of those having proteinuria at the first test were positive at the second test. 3) The proportion of patients with proteinuria by 3rd test were as follows; 25% of transient proteinuria, 55% of orthostatic proteinuria, 6% of constant proteinuria, 12% of proteinuria with hematuria, and 2% of transient proteinuria with isolated hematuria. Pathologic proteinuria were totaled as 20%. The prevalence of renal diseases among the age group of 7-18 years old was estimated to be 1.4 per 10,000. 4) Renal biopsy performed on 38 children with proteinuria at the third test revealed IgA nephropathy in 17(44%), focal segmental glomerusclerosis in 5(13%), minimal change disease in 4(11%), membranoproliferative glomeronephritis in 3(8%), $Henoch-Sch\"{o}nlein$ purpura nephritis in 3(8%), and others in 6(16%). Therefore, the prevalence of IgA nephropathy among the age group of 7-18 years old was estimated to be 0.64 per 10,000. 5) The prevalence of chronic renal failure was estimated to be 5.7 per 1 million of 7 to 18 years age group. Conclusions : 1) The prevalence of proteinuria in the first screening test was 0.28% and finally only 5% of them showed the pathologic proteinuria at the third test. 2) The prevalence of IgA nephropathy and chronic renal failure were 0.63 per 10,000 and 5.7 per 1 million, respectively among school-aged children in Seoul.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.117-124
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• 2001

Purpose : Since Mendoza(1990)'s report that long term methylprednisolone pulse therapy by Mendoza protocol (MP therapy) is a good treatment option in focal segmental glomerulosclerosis(FSGS), there have been reports of the effects of this therapy in steroid-resistant nephrotic syndrome. However, no studies have been performed on the effects of MP therapy in steroid- dependent nephrotic syndrome and secondary nephrotic syndrome. In this study, we investigated the effects of long term MP therapy in primary and secondary nephrotic syndrome in which previous treatment options were not effective. Methods : We chose 10 children who were diagnosed with steroid-dependent minimal change nephrotic syndrome(SD-MCNS), who had shown frequent relapse during the immunocompromised or cytotoxic therapy Period, and 6 children with FSGS and 5 children with secondary nephrotic syndrome children, who had shown no response during the previous therapy period. We treated these patients according to Mendoza protocol involving infusions of high doses of methylprednisolone, often in combination with oral cyclophosphamide for 82 weeks. Results : In all the 10 children with SD-MCNS, complete remission was visible on average of $18{\pm}9$ days after MP therapy was started. However, all these children relapsed during or after MP therapy. In these children, the mean relapse rate prior to MP therapy was $2.1{\pm}1.0$ relpases/year, which was reduced to $1.4{\pm}0.9$ relapses/year during MP therapy(P>0.05) and rose to $2.7{\pm}1.0$ relapse/year after MP therapy. Of the 6 children with FSGS, 4 children($67\%$) showed complete remission, of whom 3 children($50\%$) remained in the remission status during the follow up period, $1.2{\pm}0.7$ years, after the end of MP therapy. 2 children($33\%$) showed no response. All of the 5 children with secondary nephrotic syndrome showed remission and remained in the remissiom status during the follow up period, $1.7{\pm}0.6$ years The only side effect of MP therapy was transient hypertension in 10 children of ail subjects during the intravenous infusion of methylprednisolone. Conclusion : We conclude that although long term MP therapy is not effective in the treatment of SD-MCNS, it is an effective therapy against intractable FSGS and secondary nephrotic syndrome. (J Korean Soc Pediatr Nephrol 2001 ; 5 : 117-24)

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.76-82
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• 2003

Purpose : The aim of this study was to determine whether differences exist in the presentation, clinical course, and outcome of Graves' disease between prepubertal children and adolescents. Methods : A retrospective chart review of 14 prepubertal(PREPUB, $7.2{\pm}0.9yr) and 38 pubertal (PUB, $12.4{\pm}1.5yr$) children with Graves' disease between January 1989 and November 1995 at St. Mary's Hospital and Kangnam St. Mary's Hospital was undertaken. Results : There were no significant differences in $T_3$, $T_4$, TSH between two the groups at diagnosis. The PUB group had significantly higher titers of antimicrosomal antibody(positive dilution factor $11,727.3{\pm}22,888.4$) than did the PREPUB group($2,111.5{\pm}2,285.0$, P<0.001). The PREPUB group had significantly higher titers of TSH-binding inhibitory immunoglobulin(TBII, $62.5{\pm}39.6$) than did the PUB group($44.9{\pm}10.4$, P<0.05) before treatment started. The duration(months) of medical therapy before thyroid function tests were normalized was longer in the PREPUB group than in the PUB group($T_3:6.8{\pm}5.0$ vs. $5.4{\pm}13.2$, $T_4:2.3{\pm}1.9$ vs. $2.1{\pm}2.2$, $TBII:26.7{\pm}24.0$ vs. $20.8{\pm}12.1$), especially that of TSH was significantly longer in the PREPUB group($14.6{\pm}11.0$ vs. $6.8{\pm}7.8$, P< 0.05). Total length of medical therapy was significantly longer in the PREPUB group than the PUB group($52.3{\pm}19.3$ vs. $37.9{\pm}16.3months$, P<0.01). During three years of antithyroid drug therapy, in the PREPUB group, the remission rate was lower and the relapse rate was higher than in the PUB group. Total length of treatment correlated negatively with chronological age(P=0.03). Conclusion : Prepubertal children require longer medical therapy to achieve a remission than do pubertal children. But there is an obvious need for more studies because of the small number of patients and the short duration of the follow-up.