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Backgrounds Diabetic children especially who are obese lead to poor glycemic control thus are at higher risk for early microvascular complications. Renal tubulointerstitial markers play integral role in the evaluation of diabetic nephropathy and various biomarkers have been proposed. Yet, their role in obese pediatric population is uncertain. The aim of this study is to investigate renal injury markers in children with diabetes mellitus (DM) in relation with obesity and determine their role for early predictor of diabetic complications. Methods Fifty-three children and adolescents diagnosed with either type 1 or 2 DM and 44 controls aged 7-18 years were included. Clinical and laboratory characteristics including renal injury markers among subjects were compared with respective to body mass index (BMI), presence and type of DM. Results Urine neutrophil gelatinase associated lipocalin and kidney injury molecule-1 were lower whereas beta-2 microglobulin and n-acetylglucosamine (NAG) concentrations were higher in controls compared to diabetic children. None of renal injury markers showed significant difference between normoweight and obese children. In multiple regression analyses, urine NAG was associated with HbA1c and the presence of DM while high homeostatic model assessment of insulin resistance was identified as a risk factor of for increased urine NAG in type 2 diabetic children. Conclusion Urine NAG could be a marker associated not only with diabetic nephropathy, but also with insulin resistance regardless of BMI. Presentation: No date and time listed

Background: Vesicoureteral reflux (VUR) is an important urologic anomaly that causes renal injury in children with febrile urinary tract infection (UTI). The present study aimed to evaluate the associations of abnormalities detected on technetium-99m-labeled dimercaptosuccinic acid (DMSA) scans, focusing on the association with VUR of the levels of relative decrease in kidney function and cortical defects after a first febrile UTI in children. Methods: All 171 children underwent ultrasonography, DMSA scan and voiding cystourethrography (VCUG). The features of ultrasound and DMSA scans were compared between patients with (n = 48) and without VUR (n = 123). The relative uptake (RU) by each kidney was derived from the absolute value of the differences between the value for RU of radionuclide in the right kidney and that in the left kidney. The extent of cortical defects (ECD) was graded according to the number of compartments that contained cortical defect in both kidneys (right upper/right lower, left upper/left lower). Receiver operating characteristic curves were constructed to examine the diagnostic value of these parameters of ultrasound and DMSA scans for predicting VUR. Results: The ratio of patients having hydronephrosis on ultrasound or cortical defects on DMSA scan did not differ significantly between VUR and non-VUR groups. However, the absolute values of the RU and the scores for ECD were significantly higher in the VUR group than in the non-VUR group. The area under the curves for these two parameters were higher than those for the presence of hydronephrosis or the presence of cortical defects or both. Conclusion: Decreased relative function and increased extents of cortical defects on DMSA scan may be associated with the presence of VUR. These findings may assist pediatricians to decide whether febrile UTI children need to undergo VCUG. Key Words: children, DMSA scan, urinary tract infection, vesicoureteral reflux

Immunoglobulin A nephropathy (IgAN) is one of the most common primary glomerulopathies diagnosed in children and adolescents. This study aimed to evaluate the clinical features in and outcomes of pediatric IgAN over the last 30 years. Patients who were diagnosed before age of 18 at 20 centers in Korea were evaluated retrospectively. Of the 1154 patients (768 males, 386 females) with a median follow-up of 5 years, 5.6% (n = 65) progressed to stage 3&ndash, 5 chronic kidney disease (CKD). The 10- and 20-year CKD-free survival rates were 91.2% and 75.6%, respectively. Outcomes did not differ when comparing those in Korea who were diagnosed prior to versus after the year 2000. On multivariate analysis, combined asymptomatic hematuria and proteinuria as presenting symptoms and decreased renal function at the time of biopsy were associated with progression to CKD, while remission of proteinuria was negatively associated with this outcome. Patients who presented with gross hematuria or nephrotic syndrome tended toward positive outcomes, especially if they ultimately achieved remission. While remission of proteinuria might imply that the disease is inherently less aggressive, it also can be achieved by management. Therefore, more aggressive management might be required for pediatric-onset IgAN.

Background: Cancer risk has been associated with certain gene variations in microRNA (miRNA), but conflicting evidence warrants re-assessing of significant results in meta-analyses. We summarized published meta-analyses that assess the associations between miRNA polymorphism and cancers to show the validity of the findings. Method: We searched PubMed and investigated the results of meta-analyses published through November 2018. We re-assessed the results based on false-positive report probability (FPRP) to test the noteworthiness of the associations. Results: Sixty-eight miRNA polymorphisms in 45 meta-analyses associated with cancer were included. Four (7.4%) and sixteen (25.0%) single nucleotide polymorphisms (SNPs) were noteworthy (FPRP

Background Given that YKL-40 is a known marker of inflammation, we sought to determine its association with urinary tract infection (UTI) in febrile children. Methods In total, 44 children aged 0 to 24 months with febrile UTI and 35 age- and sex-matched controls with fever from other causes were enrolled in the study. ELISA was performed to determine the level of YKL-40 in urine collected from each child. Results The ratio of urinary YKL-40 to creatinine (Cr) was higher in the children with a UTI than in the controls (P

Diabetic nephropathy (DN) is a serious microvascular complication in childhood diabetes and microalbuminuria has been a solid indicator in the assessment of DN. Nevertheless, renal injury may still occur in the presence of normoalbuminuria (NA) and various tubular injury biomarkers have been proposed to assess such damage. This case-controlled study aimed to evaluate plasma and urinary neutrophil gelatinase-associated lipocalin and kidney injury molecule-1 (KIM-1) levels in diabetic children particularly in those with normo- and high-NA stages and determine their role in predicting DN. Fifty-four children/adolescents with type 1 and 2 diabetes and forty-four controls aged 7–18 years were included. The baseline clinical and laboratory characteristics including plasma and urinary biomarkers were compared. The plasma KIM-1 levels were significantly higher in diabetic children than in the controls and in high-NA children than normo-NA children. Glycosylated hemoglobin (HbA1c) was identified as a significant risk factor for increased plasma KIM-1. The optimal cutoff for HbA1c when the plasma KIM-1 was &gt, 23.10 pg/mL was 6.75% with an area under the curve of 0.77. For diabetic children with mildly increased albuminuria, the plasma KIM-1 complementary to MA may help increase the yield of detecting DN. Our findings also suggested an HbA1c cutoff of 6.75% correlated with increased plasma KIM-1.

It is unknown whether epidemiological or clinical characteristics of Henoch-Schönlein purpura (HSP) have changed over time. This study aimed at evaluating the epidemiological and clinical changes of HSP during 3 decades.We retrospectively analyzed the data of 515 children with HSP (0-15 years of age) between 1987 and 2015. We compared the two HSP patient groups: those admitted from 1987 to 1996 (group A, 238 cases) and those admitted from 2006 to 2015 (group B, 98 cases), apart a decade.In total 515 patients, the mean age was 6.5 ± 3.0 years and the male-to-female ratio was 1.2:1 (278:237). The age distribution showed a peak at age 5 with a bell-shaped distribution pattern. The annual number of cases varied in each year with a trend of reduced cases in the recent decade. There were less cases during the summer season. Purpura, gastrointestinal involvement, joint involvement, and renal involvement were found in 100%, 56%, 38%, and 18% of the patients, respectively. In comparison between the two groups, there were similar findings in mean age, age distribution, and seasonal distribution. However, the hospitalization stay was longer, and the proportion of recurrent cases (14 cases vs. 0 case) and proteinuria (15% vs. 3%) were higher in the group A than in the group B.Long-term epidemiologic features of HSP were similar to those in other countries. Clinical manifestations of HSP showed a trend towards a less severe clinical phenotype over time in Deajeon, Korea. Key Points • It is unknown whether epidemiological and clinical traits of Henoch-Schönlein purpura (HSP) have changed over time. • We reported that clinical manifestations of HSP have changed to milder phenotype through a long-term observation of three decades at a single hospital in Daejeon, South Korea. • Clinical phenotype of infection-related diseases, including HSP, may be changed over time, and the etiology and the reason of clinical changes over time remain to be solved.

Background Dysmorphic red blood cells (dRBCs) are first-line biomarkers for detecting glomerulonephritis (GN) in patients with hematuria. The UF-1000i system (Sysmex, Kobe, Japan), based on flow cytometry, provides small red blood cell (RBC) values (UF-1000i [UF]-%sRBCs). We evaluated the clinical application of UF-%sRBCs for detecting %dRBCs and GN. Methods Urine samples of 103 patients (47 with GN; 56 without GN [NGN]) were analyzed using UF-1000i urinalysis, phase-contrast microscopy (PCM), and urine chemistry. Serum creatinine (mg/dL), serum albumin (g/dL), serum protein (mg/dL), urine protein (mg/dL), and urea nitrogen (mg/dL) levels were measured using an automated chemical analyzer. To determine the cut-off level of predicting GN, ROC curve was analyzed. Results UF-%sRBCs, %dRBCs, urine protein, serum creatinine, and estimated-glomerular filtration rate differed between the GN and NGN groups, with the greatest differences detected for UF-%sRBCs and %dRBCs (P40.5% and >6.7%, respectively. %dRBCs (P=0.0001) and UF-%sRBCs (P=0.0006) differed between the GN and NGN groups in patients with isolated hematuria but without proteinuria. Conclusions UF-%sRBCs had similar diagnostic power to %dRBCs determined by PCM for identifying patients with GN. UF-%sRBCs may be more useful for diagnosing GN in patients with isolated hematuria. Predicting %dRBCs using UF-1000i will provide information on possible GN in patients presenting with asymptomatic hematuria.

Purpose: The incidence of acute poststreptococcal glomerulonephritis (APSGN) in Korea has changed. This study aimed to evaluate the epidemiological and clinical changes of APSGN observed in a single Korean institution over two decades. Methods: We retrospectively analyzed the data of 99 children (0-15 years of age) who were admitted to our institution with APSGN between 1987 and 2013. The patients were selected based on the depression of serum complement 3 (C3, 250 IU/dL) as evidence of previous streptococcal infection. Results: In the 99 patients, the mean age was 8.3 ± 2.7 years, and the male-tofemale ratio was 2.2:1 (66:30). The annual number of cases fluctuated markedly, and most cases were observed during the late autumn and winter months. However, there have been few cases reported in the past 5 years. Clinical manifestations at presentation, including hypertension and generalized oedema, and the duration of hospitalization were higher and longer in patients admitted during the first half of the study period than during the most recent half-period, suggesting a more severe clinical course in the former group. Conclusions: APSGN has become a rare disease in Korea with a trend towards a less severe clinical course. This finding suggests that the prevalence of infection-related immune-mediated diseases could change over-time, together with environ mental and possibly pathogen-host relationship changes.

Recent studies suggest that dysregulated innate immunity plays an important role in the pathogenesis of immunoglobulin A nephropathy (IgAN). The interleukin-20 subfamily and its receptor, interleukin-22 receptor alpha-1 (IL-22R1), were recently identified as immunomodulators in human diseases, acting as mediators of mucosal host defense. However, the potential role of IL-22R1 in the pathogenesis of IgAN has not been explored. In the current study, 194 patients with IgAN and 287 normal controls were genotyped for coding polymorphisms of the IL-22R1 gene and the association between the polymorphisms and IgAN was investigated. Local expression of IL-22R1 was examined in patients with IgAN and healthy controls using immunohistochemistry. Our case-control analysis showed that genotypes of rs3795299 were associated with childhood IgAN. Individuals with the CC genotype of rs3795299 had about 3-fold reduced risk of IgAN compared with those with the GG genotype in the codominant model (P=0.0028) and those with the genotypes containing the G allele (GG or GC) in the recessive model (P=0.002). After Bonferroni correction, the association between the rs3795299 CC genotype and reduced risk of developing IgAN remained significant. Furthermore, the renal expression of IL-22R1 was significantly higher in healthy controls compared with subjects with IgAN. Our data suggest that the CC genotype of rs3795299 polymorphism in the IL-22R1 gene is associated with the reduced risk of IgAN, and this genetic association was supported by the higher renal expression of IL-22R1 in healthy controls compared with patients with IgAN.

Background The aim of this study is to evaluate the clinical significance of cystatin C(CysC) in the newborns who show normal serum creatinine (Cr) and who are in an intensive care unit. Methods From July 2009 to May 2010, a total of 106 patients (53 male and 53 female newborns) in a neonatal intensive care unit at Kyung Hee Medical Center were enrolled in this study. When clinicians ordered CysC, it was tested using HiSens Cystatin-C LTIA(HBi, An-yang, Korea) on a Toshiba chemical analyzer (Toshiba, Nasushiobara, Japan). Results The range of serum Cr and CysCwas from 0.1 to 0.8 mg/dL and from 1.0 to 2.3 mg/L, respectively. CysCpresented the wider amplitude of the changes in acute renal failure. Conclusion In this study, CysCwithout an increased Cr showed only a mild increase. However, CysCreflected more delicate changes in newborns than the serum Cr. This characteristic of CysCcould make it very appropriate for a pediatric population, especially for critically ill newborns. J. Clin. Lab. Anal. 26:267-271, 2012. © 2012 Wiley Periodicals, Inc.

Background: To evaluate the significance of serum cystatin C (Cys C) levels in pediatric patients with chronic kidney disease (CKD) diagnosed by renal biopsy and who show normal serum creatinine (Cr) levels. Methods: The patient group was composed of 167 males (M) and 119 females (F) with CKD enrolled from the Department of Pediatrics at Kyung Hee Medical Center from July 2009 to May 2010. The serum Cys C, Cr, and C-reactive protein (CRP) were tested using a chemical analyzer (Toshiba, Nasushiobara, Japan). Results: The mean age of the total patients was 11.72 years, and the mean body weight was 32.24 kg. The mean Cys C value was 0.84 mg/L, and 24 patients (3 Henoch-Schonlein purpura nephritis [HSPN], 3 IgA nephropathy [IgAN], 10 mesangial proliferative nephritis [MesPN], and 8 nephritic syndrome [NS]) showed increased Cys C levels (range: 1.0–1.6 mg/L). Conclusions: In this study, 95% of the patients showed only slightly increased Cys C levels (0.5∼1.1 mg/L) from the upper normal limit of the reference range (0.9 mg/L). Therefore, we carefully suggest that mildly increased Cys C without increased Cr might not have clinical significance. * Cr : creatinine CRP : C-reactive protein HSPN : Henoch-Schonlein purpura nephritis IgAN : IgA nephropathy MesPN : mesangial proliferative nephritis NS : nephritic syndrome C Cr : creatinine clearance GFR : glomerular filtration rate CysC : cystatin C CKD : chronic kidney disease BMI : body mass index

An abnormal T-cell response is involved in the pathogenesis of various renal diseases. Survival of naive T cells is dependent on interleukin 7 (IL7) and its receptor (IL7R). Thus, we investigated the association between IL7R single nucleotide polymorphisms (SNPs) and childhood IgA nephropathy (IgAN). We analyzed the genotypic distributions of two missense SNPs of IL7R, rs1494558 (Ile66Thr) and rs1494555 (Val138Ile), among 198 pediatric IgAN patients and 288 healthy controls. Haplotype analysis and measurement of pair-wise linkage disequilibrium were performed. In addition, the genotypes of patient subgroups, determined by the presence of nephrotic range proteinuria (>40 mg/m(2)/h) and pathological advancement, were analyzed. The genotyping data of IgAN patients and controls showed significant differences in rs1494558 (codominant, P=0.0003; dominant, P=0.0003) and rs1494555 (codominant, P=0.0038; dominant, P=0.0099). In the haplotype analysis, AC (codominant, P=0.0066) and GT (codominant, P=0.0005; dominant, P=0.0006) were significantly associated with susceptibility to IgAN. Furthermore, in the analysis of clinical subgroups of IgAN patients, rs1494558 was associated with nephrotic range proteinuria (codominant, P=0.027; recessive, P=0.023). Our results suggest that IL7R may be associated with disease susceptibility and proteinuria in childhood IgAN.

Bone morphogenetic proteins (BMPs) are multi-functional growth factors belonging to the transforming growth factor β (TGFB) superfamily and are important in both preservation of kidney function and resistance to injury. BMP2 is highly regulated in the kidney, and high affinity binding sites for BMP2 have been identified in kidney epithelial cells. BMP2 has been demonstrated to play various roles in the pathogenesis of renal diseases. However, the role of the BMP2 gene in glomerulonephritis has not been previously investigated. We aimed to evaluate the association of BMP2 gene polymorphisms with immunoglobulin A nephropathy (IgAN) in children. We evaluated 187 pediatric patients with biopsy-confimed IgAN and 262 healthy controls. Two coding single nucleotide polymorphisms (cSNPs) in the BMP2 gene [rs235768 (missense, Arg190Ser) and rs1049007 (synonymous, Ser87Ser)] were selected and genotyped by direct sequencing. Genotypes of rs1049007 were associated with childhood IgAN in the codominant model II (GG vs. AA) [p=0.02; OR (95% CI), 0.16 (0.04–0.70)] and in the recessive model [p=0.0023; OR (95% CI), 0.16 (0.04–0.69)]. We also found an association between rs235768 and IgAN in the codominant model II (TT vs. AA) [p=0.01; OR (95% CI), 0.08 (0.01–0.57)] and in the recessive model [p=0.0002; OR (95% CI), 0.07 (0.01–0.55)]. After Bonferroni correction, these associations of rs235768 and rs1049007 with IgAN risk remained significant. In the haplotype analysis, the TG haplotype [p=0.01; OR (95% CI), 6.76 (1.55–29.50) in the dominant model] and AA haplotype [p=0.01; OR (95% CI), 0.08 (0.01–0.59) in the recessive model] showed associations with IgAN. The BMP2 gene may contribute to susceptibility to IgAN in Korean children.

BACKGROUND: The aim of this study is to evaluate the clinical significance of cystatin C(CysC) in the newborns who show normal serum creatinine (Cr) and who are in an intensive care unit. METHODS: From July 2009 to May 2010, a total of 106 patients (53 male and 53 female newborns) in a neonatal intensive care unit at Kyung Hee Medical Center were enrolled in this study. When clinicians ordered CysC, it was tested using HiSens Cystatin‐C LTIA(HBi, An‐yang, Korea) on a Toshiba chemical analyzer (Toshiba, Nasushiobara, Japan). RESULTS: The range of serum Cr and CysCwas from 0.1 to 0.8 mg/dL and from 1.0 to 2.3 mg/L, respectively. CysCpresented the wider amplitude of the changes in acute renal failure. CONCLUSION: In this study, CysCwithout an increased Cr showed only a mild increase. However, CysCreflected more delicate changes in newborns than the serum Cr. This characteristic of CysCcould make it very appropriate for a pediatric population, especially for critically ill newborns. J. Clin. Lab. Anal. 26:267‐271, 2012. © 2012 Wiley Periodicals, Inc.

Neurotrophins (NTs) and their receptors (NTRs) are known to be important for pathogenesis of various inflammatory diseases that occur in not only neuronal but also nonneuronal tissues, including kidney. Here, we investigated association between childhood IgA nephropathy (IgAN) and single nucleotide polymorphisms (SNPs) of genes encoding NTs [nerve growth factor (NGF) and brain-derived neurotrophic factor (BDNF)] and NTRs [nerve growth factor receptor (NGFR) and neurotrophic tyrosine kinase receptor 1–3 (NTRK1–3)]. The genotyping data of 197 patients and 289 control subjects revealed significant association between NGF SNP rs11102930 and presence of IgAN. Patient subgroup analysis revealed that that the presence of nephrotic range proteinuria (>40 mg/m2/h) was associated with rs6334 of NTRK1 and rs11030104, rs7103411, rs7103873, and rs6484320 of BDNF. Significant genotype differences were observed in podocyte foot process effacement for rs1187321 and rs1187323 of NTRK2. Furthermore, some SNPs showed significantly different genotype distribution between patients with or without pathologically advanced disease markers, specifically in rs6334 of NTRK1. Our results suggest that SNPs of NTs and NTRs are associated with susceptibility, pathological advancement, podocyte foot process effacement, and development of proteinuria in childhood IgAN.

Diarrheal disease is one of the leading causes of worldwide morbidity and mortality, especially in children. It causes loss of body fluid, which may lead to severe dehydration, electrolyte imbalance, shock and even to death. The mortality rate from acute diarrhea has decreased over the last few decades. This decline, especially in developing countries is largely due to the implantation of the standard World Health Organization-oral rehydration solution (WHO-ORS). However, the use of standard ORS has been limited by its inability to reduce fecal volume or diarrhea duration. Subsequently, this has led to various attempts to modify its compositions. And these modifications include the use of reduced osmolarity ORS, polymer-based ORS and zinc supplementation. Some of these variations have been successful and others are still under investigation. Therefore, further trials are needed to progress toward the ideal ORS. In this article, we briefly reviewed the pathophysiologic basis of the ORS, followed by the standard WHO-ORS and several modifications to improve the ORS.

In several experimental studies, it has been suggested that discoidin domain receptor 1 (DDR1) plays an important role in the regulation of mesangial proliferation, glomerular basement membrane thickening, renal fibrosis, and in the development of inflammation in several tissue types, including renal tissues. The present study was conducted to investigate the association between single nucleotide polymorphisms (SNPs) of the DDR1 gene and childhood IgA nephropathy (IgAN). The genotyping data of 180 childhood IgAN patients and 336 controls showed significant differences in the frequency of rs1264319 (dominant model, P=0.040). Subgroup analysis revealed that development of proteinuria (>4 and

선천성 매독은 산전진찰로 예방할 수 있음에도 불구하고, 여전히 문제가 되고 있다. 최근에는 선천성 매독과 관련된 혈뇨나 폐렴이 보고된 증례가 없었으나 저자들은 적절한 산전치료를 받지 못한 산모에게서 태어난 22일된 남아에게서 발생한 선천성 매독에 동반된 신증후군과 폐렴의 증례를 보고한다. 선천 매독의 진단은...

Sjogren’s syndrome (SS) is an autoimmune disorder primarily affecting the salivary and lacrimal glands. In addition, extra-glandular manifestations involving the lungs, liver, kidneys, pancreas, skin and central nervous system were reported in patients with SS. These extra-glandular manifestations are not rare in adult patient, but are very rare in pediatric SS. Renal manifestations are relatively common in adult SS, but are rarely reported in childhood SS. We experienced a girl with primary SS manifested with nephrogenic diabetes insipidus and renal tubular acidosis.

The enabled homolog gene (ENAH, hMena) is abundantly expressed in mesangial tissue, and might play an important role in inflammatory processes of IgA nephropathy (IgAN). The present study was conducted to investigate the association between single nucleotide polymorphisms (SNPs) of the ENAH and childhood IgAN. We analyzed 12 SNPs of ENAH in 176 patients with childhood IgAN and 397 healthy controls. In addition, IgAN patients were dichotomized and compared with respect to several clinical and pathological parameters. Genotyping data showed significant differences between IgAN patients and controls in the frequency of rs2039620, rs12034829, and rs3795443. On comparison of patients with proteinuria to those without proteinuria (or = or4 mg/m(2)/h), rs12043633 was significantly different between the two groups. With regard to maximum proteinuria (or = or4 mg/m(2)/h), rs3795443, rs4653643, rs6751, rs10799319, rs7555139, rs576861, and rs487591 showed significant allele frequency differences. For patients with and without gross hematuria, rs4653643, rs6751, rs10799319, rs7555139, rs576861, and rs487591 showed significant allele frequency differences. The rs3795443 was found to be associated with progression of pathological findings. Our results suggest that ENAH polymorphisms are associated with increased susceptibility, development of proteinuria and gross hematuria, and pathological progression of childhood IgAN.