• Childhood Kidney Diseases
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• v.8 no.1
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• pp.10-17
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• 2004

Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) nephritis has a variable range of prevalence from 25 to 50% among HSP patients and is a common cause of chronic glomerulonephritis in children. In our study, we evaluated the distribution and the association of the angioten-sinogen(AGT) M235T polymorphism with the clinical manifestations, particularly proteinuria in children with HSP with or without nephritis. Methods : The AGT M235T polymorphism was determined in children with HSP nephritis (n=33) or HSP without nephritis(n=28) who had been diagnosed at Busan Paik hospital from January 1996 to June 2001. The M235T polymorphism of the AGT gene was determined by PCR amplification of the genomic DNA. Results : The M235T polymorphism of AGT gene frequency was MM 75%, MT : 25%, TT : 0% in HSP and MM : 64%, MT : 36%, TT : 0% in HSP nephritis, there was no significant differences in the genotype and allele frequencies between the two groups. No significant differences in clinical manifestations at onset and last follow-up were seen between the two genotypes. When statistical analysis was done according to the presence of the M allele, the amount of 24-hour urinary protein excretion and the incidence of moderate to heavy proteinuria(>500 $mg/m^2/day$) at onset and at last follow-up were higher in the MT genotype than in those of in the MM genotype but these difference were not statistically significant. Conclusion : We suggest a lack of association between M235T polymorphism of the AGT gene and clinical manifestations in children with HSP nephritis. However, further follow-up studies based on sufficient number of patients and long term follow up periods are necessary to confirm the role of M235T polymorphism of AGT gene in children with HSP nephritis.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.33-42
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• 2004

Purpose : Children with nephrotic syndrome(NS) are under high risk for metabolic bone disease(MBD) as a complication of long-term glucocorticoid therapy. We prospectively evaluated the effect of oral bisphosphonate(alendronate) therapy in children with NS, which has proven efficacy in adult patients with glucocorticoid induced MBD. Methods : Among 58 children with NS, aged 5 to 8 years and haying a disease duration of more than 2 years, 30(51.7%) were enrolled to meet the selection criteria, less than -1.0 Z-scores of lumbar spine bone mineral density(BMD) by dual energy X-ray absorptiometry (DEXA). These 30 children were divided into three groups and each were assigned to receive alendronate, calcitriol, and no-medication, respectively for one year. Lumbar spine BMD was followed up every 6 months and the biochemical indexes were measured before and 1 year after the treatment. There were no significant difference among groups with respect to the average age, the initial BMD, and the cumulative steroid doses. Analysis of the treatment efficacy was done by the % change of BMD and by the changes in Z-scores of lumbar spine BMD. Results : Mean age and disease duration of patients at the initial lumbar spine BMD evaluation was $7.4{\pm}1.7$ years and $2.2{\pm}1.2$ years, respectively. Twenty-three of 30 children(76%) had osteopenia, and seven(23%) had osteoporosis. There was no difference in the biochemical values among the groups, before and 1 year after the treatment(P<0.05). Twenty two children(73.3%) with frequent relapsing or steroid dependant NS had more frequent MBD, compared to the 8 children(26.6%) with infrequent relapsing NS. The one year % changes of BMD were 8.56 in alendronate group, 5.79 in calcitriol group, and 1.9 in no-medication group. The changes in Z-score of lumbar spine BMD increased in the alendronate group and the calcitriol group, but not in the no-medication group. One year % changes of BMD were different among groups(P=0.0002). Significant differences were found between the alendronate and the no-medication group, and between the calcitriol and the no-medication group(P<0.05). There was no difference between the alendronate and the calcitriol group. No serious adverse effect was observed in the alendronate group. Conclusion : Children with NS receiving high dose steroids are under the high risk of BMD and should undergo regular BMD evaluation. Z-score of lumbar spine BMD was a useful parameter in diagnosing low bone mass in children. Alendronate weekly oral therapy was effective and relatively safe in increasing the lumbar spine BMD in children with NS having steroid induced MBD.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.60-66
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• 2003

Purpose : Neonatal hydronephrosis has been detected with increasing frequency with the widespread use of prenatal ultrasonography, but the consensus about its postnatal management has not yet been reached, especially about surgical intervention. We attempted to determine the guideline of follow-up study and surgical intervention of hydronephrosis by analyzing clinical outcomes of neonates with hydronephrosis. Materials and Methods : Between 1994 and 2000, 128 hydronephrotic kidneys were postnatally confirmed. Cases associated with other urologic anomalies were excluded and 90 unilateral hydronephrotic kidneys with a minimum follow-up of 12 months were enrolled in this study. We classified the patients into 6 groups according to the anterior posterior pelvic diameter(APPD) at initial ultrasonography(USG) within 1 month after birth. Renal USG and $Tc^{99m}-mercaptoacetyl$ triglycerine(MAG3) scan were done according to a set protocol, and pyeloplasty was performed when indicated according to our protocol. Results : Most cases whose APPD were below 10 mm improved or resolved. Only few cases with APPD above 20 mm showed spontaneous improvement and most(88%) had undergone operation. Those with initial APPD within 10-19 mm showed variable outcomes. When the risk factors for irreversible renal functional deterioration were analyzed, the age at pyeloplasty and pre-operative functional deficit were significant. Conclusion : We concluded that in infants with initial APPD below 10 mm, consideration of surgery is not needed, and in those with initial APPD above 20 mm, early operation is recommended. Our set protocol based on initial USG is useful, but the cut-off value of relative renal function(RRF) for operation might be increased to 40% to improve post operative RRF.

• 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.16 no.2
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• pp.115-120
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• 2012

Purpose: We aimed to evaluate the efficacy of Octyl-2-cyanoacrylate (Dermabond$^{TM}$) as a topical skin adhesive for pediatric urologic open surgery. Methods: From August 2010 to August 2011, we retrospectively evaluated pediatric patients who underwent urologic open surgery at our institution. A total of 128 pediatric patients with 210 incisions used Dermabond$^{TM}$ for skin closure. Results: We divided the 128 patients into 3 groups according to type of surgery. Group 1 underwent hydrocelectomy (55 cases, 41.3%), Group 2 underwent orchiopexy (43 cases, 32.3%), Group 3 underwent penoplasty (35 cases, 26.4%). One hundred and twenty eight patients who underwent 133 surgeries in total, with a total of 210 incisions visited our outpatient department postoperatively, and a total of 5 wound complications (2.3%) occurred, but were simple inflammations and no dehiscence was observed. When analyzed according to groups, no wound problems occurred in Group 1 (0/55, 0%), one occurred in Group 2 (1/43, 2.3%) and four cases occurred in Group 3 (4/35, 11.4%) respectively. When re-analyzed according to wound locations, one occurred in an inguinal wound (1/120, 0.83%), none occurred in scrotal wounds (0/55, 0%), and four occurred in penile wounds (4/35, 11.4%). In Group 3, the incidence of penile wounds was significantly increased compared to other groups (P=0.008). All 5 wound problems were inflammatory and healed at an average of 13.8 days (13-15 days) with antibiotic ointment application only. Conclusion: Dermabond$^{TM}$ is feasible and safe topical skin adhesive alternative to standard skin suture in pediatric urologic surgery. However, further research about its efficacy and safety could be valuable in the future.

• Childhood Kidney Diseases
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• v.16 no.2
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• pp.102-108
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• 2012

Purpose: We investigated the clinical presentation of febrile pediatric patients with acute pyelonephritis (APN) with a mixed urine culture from an aseptic urine sample, and compared with that of those with a single culture. Methods: We retrospectively reviewed the medical charts of 95 patients diagnosed as APN with fever between January 2008 and October 2010 at Korea University Medical Center. We classified the patients with APN into two groups with a positive single culture (S group) and a positive mixed culture (M group) from an aseptic urine sample of suprapubic bladder aspiration or urethral catheterization and compared the fever duration, laboratory markers such as serum white blood cell (WBC) counts and C-reactive protein (CRP) values in peripheral blood, and the presence of hydronephrosis, renal scar and vesicoureteral reflux (VUR) between the two groups (If presence of hydronephrosis, scar and VUR=1 and no=0). Results: Total pediatric patients with febrile APN were 95 patients, a positive S group was 89 patients and a positive M group was 6 patients. Fever duration (S vs. M, $4.7{\pm}3.1$ vs. $6{\pm}5.7$ days), serum WBC (S vs. M, $18,630{\pm}6,483$ vs. $20,153{\pm}7,660/{\mu}L$) and CRP (S vs. M, $100.6{\pm}2.46$ vs. $81.1{\pm}0.09\;mg/L$) values, and the presence of hydronephrosis, renal scar and VUR were not different between the two groups. Conclusion: Our data shows that there were no specific differences of clinical manifestation between a positive single urine culture and a positive mixed urine culture in pediatric APN. A mixed urine culture from an aseptic urine sample should be interpreted cautiously.

• Childhood Kidney Diseases
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• v.16 no.2
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• pp.80-88
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• 2012

Purpose: The aim of our study was to investigate the characteristics of the peritoneal dialysis (PD) - related peritonitis and to evaluate the effectiveness of the empirical antibiotics recommended by the International Society for Peritoneal Dialysis in Korean children. Methods: We retrospectively reviewed the medical records of 72 children on peritoneal dialysis at the Department of Pediatrics, Asan Medical Center over the period from March 2000 to February 2012. Results: Seventy-nine episodes of peritonitis occurred in 32 patients. The incidence of peritonitis was 0.43 episodes/patient year. There were no significant differences in the incidence of peritonitis in terms of dialysis modality (P=0.459). Twenty-one patients experienced 51 catheter exit-site infections (0.28 episode/patient year). There were no significant differences in the incidence of peritonitis between those with and without history of exit-site infections (P=0.721). Specific pathogens were isolated from 68.4% (54/79) of the patient with peritonitis episodes, including Gram-positive bacteria (n=34), Gram-negative bacteria (n=25) and fungus (n=1). Among Gram-positive bacteria, 85.3% of the isolates were susceptible to ${\beta}$-lactam antibiotics, among Gram-negative rods, 94.7% of the isolates were susceptible to ceftazidime. Among 25 cases with unknown etiologies, 92.0% of cases demonstrated satisfactory responses to cefazolin and ceftazidime. Conclusions: The incidence of peritonitis was 0.43 episodes/patient year. Initial empirical therapy consisting of cefazolin and ceftazidime was appropriate for 91.1% of the PD-related peritonitis treatment. Continuous monitoring for the emergence of the resistant organisms is an important part of the appropriate managements of PD-related peritonitis.

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.143-153
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• 2010

Purpose: Recently, massive proteinuria has been observed in some transplant patients after switching cyclosporine A (CsA) to sirolimus. To evaluate the pathogenesis of sirolimus-associated proteinuria, we investigated the early changes in slit diaphragm molecules by various administrative conditions of sirolimus and CsA. Methods: In vitro-Mouse podocytes were incubated with buffer (C), sirolimus ($10\;{\mu}g/mL$) after CsA ($10\;{\mu}g/mL$) (C-S), sirolimus only (S) and CsA and sirolimus simultaneously (C+S) for 12, 24, and 48 hours. In vivo- twenty four SPF female Wistar rats were divided into 4 groups buffer (C), sirolimus after 2 weeks of CsA (C-S), sirolimus only (S) and CsA and sirolimus simultaneously (C+S). All groups were treated by intraperitoneal injection every other day for 4 weeks (CsA: 25 mg/kg, sirolimus: 0.5 mg/kg). The changes in mRNA of slit diaphragm molecules were examined by RT-PCR. Results: The mRNA of nephrin was significantly decreased in group C-S and C+S in vitro. In vivo, the mRNA of nephrin in all groups using sirolimus and the mRNA of podocin in group C-S and C+S were decreased. Microscopically, group C-S and C+S showed small vacuolization and calcification in proximal tubular epithelial cells. Immunohistochemistry using nephrin and podocin antibodies did not show remarkable decrease of staining along the glomerular capillaries. Electron-microscopically, focal fusion of foot processes was seen in group C-S and C+S. Conclusion: This study suggests the decrease of slit diaphragm molecules (nephrin and podocin) in podocyte may be one of the causes of sirolimus associated proteinuria, and podocyte injury by sirolimus may need a primary hit by CsA to develop the proteinuria.

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.210-217
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• 2010

Purpose : Diabetes mellitus (DM) is known as one of the common causes of secondary enuresis in children. However, enuresis in diabetic children is overlooked only as a symptom of polyuria due to hyperglycemia. We evaluated the prevalence of nocturnal enuresis in children with diabetes mellitus in this paper. Methods : Among children with diabetes in three hospitals in Daegu area, 117 agreed to 'Tele research by means of a questionnaire'. Results : Diabetic patients were divided into two groups: Nocturnal enuresis and nonnocturnal enuresis group. thirty-two of 117 (27.0%) patients were in enuresis group, with more daytime urination than non-nocturnal enuresis group ($4.2{\pm}1.6/3.6{\pm}1.2$ times, P=0.016). HbA1c at diagnosis was $12.0{\pm}2.3%/12.0{\pm}2.5%$, and at follow-up $9.3{\pm}2.3%/8.3{\pm}2.3%$ (P =0.042). Insulin was administered at $1.1{\pm}0.5/1.1{\pm}0.4$ units/kg/day. Ten children of enuresis (31.2%) group were monosymptomatic (MNE) and 22 (68.8%) children were non-monosymptomatic enuresis (non-MNE). Fourteen (43.8%) of enuresis group had persistent symptoms, with 5 MNE and 9 non-MNE each. HbA1c at diagnosis was $11.1{\pm}2.5$, $12.4{\pm}2.1$, higher in non-MNE (P=0.144). Average arousal during sleep was step $3.3{\pm}1.2$, $2.5{\pm}1.0$, higher in improved enuresis group (P=0.059). Conclusion : Nocturnal enuresis among DM patients is underestimated. However, considering psychological and social effects of enuresis in children, extensive and long-term studies are needed in the future to clarify relationship between prevalence and DM control.

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.203-209
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• 2010

Purpose : It has been a common medical practice to use prophylactic antibiotics to prevent recurrent urinary tract infections (UTI) in high risk situations such as urinary tract obstruction, vesicoureteral reflux, neurogenic bladder, or urinary stones. But sometimes, we meet difficult situation of breakthrough infections (BI) which might cause new or progressive renal scarring. The clinical characteristics of children contracting breakthrough UTI experienced in a single center were studied. Methods : The study was done retrospectively through medical records of 150 pediatric patients who had been cared in pediatric and urologic clinics of Pusan National University Hospital from Jan. 2001 till June 2006 and had prophylactic antibiotics to prevent recurrent UTI. Results : The starting age of prophylactic antibiotics of 150 patient was 1-76 months, and median age was 5 months. The BI developed 61 times in 43 patients (28.7%), 1.5 times per 100 patient-months. The BI occurred more frequently in patients with higher grade of VUR, and in the cases with abnormal DMSA scan. Co-trimoxazole was more effective than 2nd and 3rd generation cephalosporins to prevent UTI. The distribution of causative organisms was more diverse than usual UTI. The causative organisms were sensitive to the antibiotics used for prophylaxis in 29.5%, and resistant in 59.1%. After experience of BI, 40 percents of patients went to the surgical treatment including endoscopic injection of Deflux, 35% to new antibiotics for prophylaxis, 26% remain on the same antibiotics as the previous one. Conclusion : Based on our study results, preexisting renal scar might be one of the factors which should be considered in favor of early surgical interventions of VUR. Poor compliance and wrong selection of antibiotics such as cephalosporins are important underlying causes of breakthrough UTIs.