• Childhood Kidney Diseases
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• v.18 no.1
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• pp.24-28
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• 2014

Purpose: The purpose of this study was to assess the therapeutic efficacy of methylprednisolone pulse therapy in children with IgA nephropathy and Henoch- Sch$\ddot{o}$nlein Purpura (HSP) nephritis combined with proteinuria. Methods: We retrospectively reviewed the clinical records of 21 patients who were diagnosed with IgA nephropathy and HSP nephritis based on percutaneous renal biopsy. Of the 21 patients, 15 were diagnosed with IgA nephropathy and 6 were diagnosed with HSP nephritis. They had mild to severe proteinuria at the time of diagnosis or during follow-up. Group 1 (n=7) received methylprednisolone pulse therapy three times every couple of months, and Group 2 (n=14) received oral steroid therapy. The follow-up periods for Group 1 and 2 were 14.0 (9-54) months and 26.5 (14-34) months, respectively. There was no significant difference in the follow-up duration between the two groups. Results: The average age at diagnosis and biopsy was lower in Group 1 compared to Group 2, but it was not significantly different. At admission, all patients in both groups had hematuria and 5 patients (71.4%) of Group 1 and 14 patients (100 %) of Group 2 had proteinuria. Before treatment, there was no significant difference of spot urine protein/creatinine ratio between the two groups. During followup, 7 patients of Group 1 (100%) and 10 patients of Group 2 (71.4%) showed complete improvement of proteinuria and the spot urine protein/creatinine ratio in Group 1 was significantly lower than Group 2. Conclusion: In patients with IgA nephropathy and HSP nephritis with proteinuria, methylprednisolone pulse therapy was more effective than oral steroid therapy in the reduction of proteinuria. To investigate the effects on long-term prognosis, large-scale prospective studies are needed.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.54-61
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• 2008

Purpose: This study was performed to assess necessity of voiding cystourethrography (VCUG) for infants with urinary tract infection (UTI) who had both normal renal sonography and normal DMSA renal scans. Methods: We reviewed 117 infants hospitalized for UTI between February 2002 and July 2007 at Sanggye Paik Hospital. The diagnosis of UTI was made by culture from a urine specimen obtained by suprapubic puncture(n=57), catheterization(n=58), or collection bag method (n=2, twice positive culture of the same organism). All patients had undergone renal sonography, DMSA renal scan and VCUG. Children with both normal renal sonography and normal DMSA renal scans were evaluated for the presence or severity of vesicoureteral reflux (VUR). Results: Of the 117 patients, 96 were boys and 21 were girls. 28 patients(23.9%) had VUR. 59(50.4%) showed both normal renal sonography and normal DMSA renal scans. Among these 59 patients, 7(11.9%) showed VUR. Three of them had grade I-II reflux, two grade III reflux, and the other two grade IV reflux. One of them showed bilateral VUR, grade IV reflux on the right and grade III on the left. Conclusion: Although the negative predictive value of both normal renal sonography and normal DMSA renal scan for VUR was 88.1%, 7 patients had VUR and two of them had high grade reflux(grade IV). So, we suggest that VCUG should be performed in infants with UTI despite both normal renal sonography and normal DMSA renal scans.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.47-53
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• 2008

Purpose: We often have patients who acquired incorrect medical information from the mass media. The purpose of this study was to evaluate credibility of articles in newspapers and medical counseling on websites about renal diseases. Methods: Kidney information was searched in 6 newspapers for the past 10 years, and 4 portal websites and 17 internet health counseling sites for the past 5 years. We classified them according to information providers and evaluated credibility by giving points 3, 2, 1 to correct, mostly correct but ambiguous, and incorrect contents, respectively. We compared the credibility of the groups with each other. Results: Sixty four articles from newspapers, and 789 and 506 medical counselings from portal websites and internet health counseling sites were selected, respectively. The kidney information providers in newspapers were medical journalists(doctors)(31.2%), kidney specialists(doctors)(23.4%) and so on. The consultants in the portal sites were doctors(49.1%)and anonymous reporters (49.9%). In internet health counseling sites, 91% of the consultants were doctors. All articles in the newspapers were credible. Doctors' answers were more credible than nonphysicians'(P=0.005) and anonymous contributors(P<0.001) in portal sites. In health counseling sites, doctors answered more reliably than nonphysicians. Conclusion: The kidney information in newspapers was credible. It is important for questioners to confirm the type of consultants in websites. We suggest that doctors, especially kidney specialists need to increase their roles in offering information to mass media.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.43-50
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• 2004

Background : Steroid-induced osteoporosis(SIO) is one of the serious complications of long-term steroid therapy, especially in growing children. Recently bisphosphonates have been used to treat or prevent SIO in adult, which is rare in children with glomerular diseases. We studied the effect of pamidronate on SIO using dual energy X-ray absorptiometry and biochemical markers of bone turnover. Methods : Forty four children receiving moderate-to-high doses of steroids were enrolled. They had no history of bone, liver, or endocrine disease. Patients were stratified by their baseline bone mineral density(BMD) findings. All patients received corticosteroids for 3 month and oral calcium supplementation(500 mg/day) daily. Among them, 28 patients were treated with placebo and 16 were treated with pamidronate(125 mg) for 3 months. Blood chemistry and bone mineral density(BMD) were measured at baseline, and 3months. In addition, parathyroid hormone(PTH), serum osteocalcin, and urinary dipyridinoline levels were evaluated. Results : In overall population, the mean lumbar spine BMD decreased from $0.754{\pm}0.211(g/cm^2)$ to $0.728{\pm}0.208(g/cm^2)$ in the placebo group(P<0.05) and increased from $0.652{\pm}0.194(g/cm^2)$ to $0.658{\pm}0.226(g/cm^2)$ in the pamidronate group(P>0.05). Conclusion : Pamidronate appears to be effective in preventing SIO in children with glomerular diseases requiring long-term steroids therapy. Further careful observation and follow-up might be needed for children receiving bisphosphonates such as pamidronate.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.52-59
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• 2003

Purpose : The renin-angiotensin system(RAS) plays an important role in renal growth and development. We have studied the prevalence of renal anomalies and documented the association between karyotype and renal anomalies using IVP and ultrasonography. Furthermore, to investigate the impact of RAS gene polymorphism on renal anomaly in Turner syndrome, we examined the ACE I/D genotype, angiotensinogen(AGT) gene M235T, angiotensin receptor type 1(ATR) gene A1166C. Methods : Cytogenetic analysis was performed in 33 Turner syndrome patients on peripheral blood lymphocytes. Ultrasonography(US) of the kidneys and collecting system and intravenous pyelography(IVP) were perfomed in all patients. Nuclear scintigraphy{Tc 99m dimercaptosuccinic acid(DMSA) scan} was also performed for the definite renal diagnosis if indicated. And, ACE I/D genotype, angiotensinogen(AGT) gene M235T, angiotensin receptor type 1(ATR) gene A1166C were examined by PCR amplification of genomic DNA samples. Results : The prevalence of renal anolmalies in Turner syndrome was 36.4%(12/33). The Karyotype 45, X was observed in 18 of the 33 girls(54.5%), of whom 8(44.4%) had renal anomalies. Mosaic karyotypes were observed in 11(33.3%) and four(12.2%) had a non-mosaic structural aberration of the X chromosome. In this group 4(25.7%) had renal anomalies. More renal anomalies were associated with the 45, X karyotype than those with mosaic/structural abnormalities of X chromosome, but the difference was not statistically significant(P>0.05). And, there was no significant differences in the RAS gene polymorphism and allele frequencies between renal anomaly group and normal group in Turner syndrome. Conclusion : The prevalence of renal anolmalies in Turner syndrome was 36.4%. There is no significant differences in the RAS gene polymorphism and allele frequencies between the renal anomaly group and the normal group in Turner syndrome.

• 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.14 no.2
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• pp.174-183
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• 2010

Purpose : The organisms causing peritonitis and their antibiotic sensitivities vary in different regions and centers, and these data are necessary to establish regional treatment guidelines. The aim of this study was to investigate the changes in incidence and characteristics of the organisms that cause peritonitis in children undergoing peritoneal dialysis (PD) during recent 10 years. Methods : We retrospectively collected and analyzed the data from medical records of 110 children on PD during the period from 2000 to 2010. Results : One hundred and forty episodes of peritonitis have occurred in 57 patients. The overall incidence of peritonitis was 0.43 episodes/patient year, and similar incidence have been maintained since 2003. Sixty percent of the patients experienced peritonitis within 1 year of PD, and all patients commencing PD in infancy experienced peritonitis. Gram positive (G (+)), gram negative (G (-)) organisms and fungi were cultured in 58%, 38%, and 4.1% respectively and cultures were negative in 13.6%. Staphylococcus was the most common G (+) organism, and Pseudomonas and Acinetobacter were 2 most frequent G (-) organisms isolated. Fifty-six percent of the G (+) organisms were sensitive to first generation cephalosporin and 91% of G (-) pathogens were sensitive to ceftazidime. Methicillin-resistance rate was not higher in children less than 2 years of age than in those more than 2 years. Conclusion : An additional breakthrough has to be made to further reduce the incidence of peritonitis. Treatment guideline customized for peritonitis in Korean children on PD need to be established through a nationwide co-work.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.143-149
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• 2008

Purpose : Urinary N-acetyl-beta-D-glucosaminidase(NAG) and beta 2-microglobulin(B2M) is considered to be a marker of tubulointerstitial injury. The aim of this study was to examine the urinary levels of NAG and B2M in children with various renal diseases. Methods : We studied 21 children(8.9$\pm$4.5 years, Male:Female=14:7) and they were divided into three groups: group I(steroid-sensitive nephrotic syndrome-4 patients), group II(various kinds of glomerulonephritis-4 patients), and group III(normal urinalysis or non-glomerular renal diseases-13 patients). Results : Urinary NAG levels in groups I and II were significantly higher than those in group III(19.4$\pm$11.5 and 30.0$\pm$30.1 vs. 4.7$\pm$3.9, P=0.01), while urinary B2M levels did not differ among the 3 groups, although urinary NAG levels were positively correlated with urinary B2M levels(r=0.49, P=0.03). Urinary NAG and B2M levels were all correlated with proteinuria(r=0.79, P<0.001 and r=0.68, respectively, P=0.001) serum albumin(r=-0.72, P<0.001 and r=-0.57, respectively, P=0.01) and cholesterol(r=0.58, P=0.006 and r=0.56, respectively, P=0.013) levels. Conclusions : Urinary excretions of NAG and B2M are increased in children with steroidsensitive nephrotic syndrome and various kinds of glomerulonephritis, suggesting tubular dysfunction might be present in these diseases.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.164-169
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• 2008

Purpose : There have been some reports on the prevalence of positive antineutrophil cytoplasmic antibody(ANCA) in Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP), but the results were conflicting. We performed this study to evaluate the clinical significance of ANCA(c-ANCA and p-ANCA) in Korean children with HSP. Methods : The medical records of 30 patients(13 boys and 17 girls) aged 6.0$\pm$1.9(5-12) years with a clinical diagnosis of HSP based on the EULAR/PReS criteria were reviewed retrospectively. From the years 2007 to 2008, the sera from children with acute HSP were tested for antineutrophil cytoplasmic antibodies(ANCA). The target antigens of these autoantibodies are proteinase 3(c-ANCA) or myeloperoxidase(p-ANCA). Results : Palpable purpura was seen in all 30 patients(100%), abdominal pain in 20(67%), arthralgia in 17(57%), and renal involvement in 11(37%). Laboratory findings showed leukocytosis in 4 patients(13%), thrombocytosis 18 in(60%), and elevated erythrocyte sedimentation rate in 18(60%). Anti-streptolysin O titers were elevated in 7% of the patients and no patient showed elevation of serum IgA level. The sera from 29 patients were negative for c-ANCA and p-ANCA by indirect immunofluorescence, but only one patient had weakly positive results, which became negative at follow-up. Conclusions : We conclude that c-ANCA or p-ANCA is not an important serologic marker in children with HSP, because it was neither diagnostically nor immunologically specific in children with HSP. These results suggest that ANCA are not involved in the pathogenesis of HSP in children.

• Clinical and Experimental Pediatrics
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• v.45 no.10
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• pp.1213-1218
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• 2002

Purpose : Hydronephrosis constitutes a great portion of fetal anomalies screened by prenatal sonogram. The present authors made an attempt to access its natural courses through follow up neonatal hydronephrosis diagnosed by prenatal sonogram. Methods : The study was composed of 23 neonates(36 renal units) who were diagnosed with hydronephrosis through prenatal sonogram screening and confirmed 3-7 days after birth with sonographic evaluation at Kyung Hee University Hospital. The neonates were closely monitored for 12-24 months with renal sonogram, diuretic renogram, intravenous pyelography(IVP) and voiding cystoureterography(VCUG). Results : The underlying diseases were composed of 16 cases(44%) of functional abnormalities, 14 cases(39%) of ureteropelvic junction(UPJ) obstruction, three cases(8%) of vesicoureteral reflux (VUR) and on case each of multicystic dysplastic kidney, UPJ obstruction with ureteral stenosis and ureterovesical junction(UVJ) obstruction with VUR. The degree of hydronephrosis was divided into three classes according to its severity. In 30 renal units with UPJ obstruction and functional abnormalities, 26(87%) showed mild hydronephrosis, while four(13%) were moderate. During the follow up period, six cases(20%) showed natural resolution of hydronephrosis, 15 cases(50%) showed improvement while two cases(7%) were aggravated with improvement only after surgery of the underlying disease. The cases which showed natural resolution were all mild hydronephrosis at diagnosis and the cases which underwent surgery due to continuous aggravation were mild one case and moderate one case. Conclusion : Those with cases of mild hydronephrosis show rapid natural improvement. On the other hand, in some cases, follow up monitoring reveal aggravation of the situation, emphasizing the necessity for thorough follow up for a long period of time.