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Children with nephrotic syndrome (NS) are at an increased risk of acute kidney injury (AKI) and the incidence of AKI in this population is reportedly increasing. This study aimed to investigate the incidence, clinical profiles, and risk factors of AKI in hospitalized children with NS through a nationwide study. This retrospective multicenter study included 14 pediatric nephrology centers in Korea. From 2013 to 2017, a total of 814 patients with idiopathic NS were cared for at participating centers. Among them, 363 patients were hospitalized for NS and investigated in this study. A total of 363 children with NS were hospitalized 574 times. AKI occurred in 93 admissions (16.2%) of 89 patients: 30 (32.3%) stage 1; 24 (25.8%) stage 2; and 39 (41.9%) stage 3. Multivariate logistic regression analysis showed that longer disease duration, lower albumin level, and methylprednisolone pulse treatment were significantly associated with AKI development in hospitalized children with NS. AKI was associated with a longer hospital stay than non-AKI (median 10 vs. 7 days, P = 0.001). Among 93 admissions, 85 (91.4%) episodes recovered from AKI without complication, whereas 6 (6.5%) progressed to advanced chronic kidney disease (CKD). AKI is not uncommon in hospitalized children with NS, and its incidence in this nationwide study was 16.2%. Risk factors for AKI in hospitalized children with NS include longer disease duration, lower albumin level, and methylprednisolone pulse therapy. Pediatric NS patients with these characteristics should be under more strict scrutiny for the occurrence of AKI.

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 forms of glomerulonephritis, while IgA vasculitis (IgAV) is the most common systemic vasculitis in children. Herein we aimed to uncover single nucleotide polymorphism (SNP) markers associated with these two related diseases by applying association tests and Sanger sequencing. Within the discovery stage, genomic DNA in blood samples from 101 enrolled patients were genotyped by the Korean Biobank Array. Association tests were performed with 397 Korean reference genomes. In the validation stage, 26 independent samples were genotyped by Sanger sequencing. Four SNPs were identified (P G) was found in as many as 15 out of 26 samples (minor allele frequency = 0.288), even though this minor allele is rare in East Asians (

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

Children with nephrotic syndrome (NS) are at an increased risk of acute kidney injury (AKI) and the incidence of AKI in this population is reportedly increasing. This study aimed to investigate the incidence, clinical profiles, and risk factors of AKI in hospitalized children with NS through a nationwide study.This retrospective multicenter study included 14 pediatric nephrology centers in Korea. From 2013 to 2017, a total of 814 patients with idiopathic NS were cared for at participating centers. Among them, 363 patients were hospitalized for NS and investigated in this study.A total of 363 children with NS were hospitalized 574 times. AKI occurred in 93 admissions (16.2%) of 89 patients: 30 (32.3%) stage 1; 24 (25.8%) stage 2; and 39 (41.9%) stage 3. Multivariate logistic regression analysis showed that longer disease duration, lower albumin level, and methylprednisolone pulse treatment were significantly associated with AKI development in hospitalized children with NS. AKI was associated with a longer hospital stay than non-AKI (median 10 vs. 7 days, P = 0.001). Among 93 admissions, 85 (91.4%) episodes recovered from AKI without complication, whereas 6 (6.5%) progressed to advanced chronic kidney disease (CKD).AKI is not uncommon in hospitalized children with NS, and its incidence in this nationwide study was 16.2%. Risk factors for AKI in hospitalized children with NS include longer disease duration, lower albumin level, and methylprednisolone pulse therapy. Pediatric NS patients with these characteristics should be under more strict scrutiny for the occurrence of AKI. Graphical abstract.

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.

The aim of this study was to evaluate the association of a urinary tubular marker, liver-type fatty acid binding protein (L-FABP) and an inflammatory marker, serum/urinary YKL-40, with albuminuria in patients with childhood-onset type 1 diabetes (T1D). Twenty-nine patients with childhood-onset T1D and 32 controls were enrolled. Serum and urinary concentrations of YKL-40 and urinary concentrations of L-FABP were measured. The serum levels of YKL-40 were not significantly different between the control group and the patient groups. However, the levels of urinary YKL-40/creatinine (Cr) were higher in the patients, even those with normoalbuminuria than in the controls (p

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.

Henoch-Schönlein purpura (HSP) is a leukocytoclastic vasculitis classically characterized by palpable purpura, arthritis, abdominal pain and renal disease. In this article, we summarize our current understanding of the pathogenesis of HSP and the implications for improving its diagnosis. Although the pathogenesis of HSP is not fully understood yet, exciting new information has emerged in recent years, leading to a better understanding of its pathogenesis. Here, we discuss genetic predisposition, immunoglobulins with a particular emphasis on IgA1, activated complements, cytokines and chemokines, abnormal coagulation and autoantibodies in the underlying pathogenic mechanisms. Finally, diagnostic criteria for HSP developed by institutions such as the American College of Rheumatology and the European League against Rheumatism/Paediatric Rheumatology European Society were proposed to improve early detection and diagnosis.

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.

Since 1998, urine screening tests have been performed on school children in Korea. We report the findings of the screening program that analyzed patients with proteinuria and/or hematuria. Between 1999 and 2008, 5,114 children were referred to pediatric nephrologists at seven nationwide hospitals. Renal biopsies were performed on 1,478 children [28.79 % of total subjects; 26.77 % for isolated hematuria (IH), 9.09 % for isolated proteinuria (IP), and 51.19 % for combined hematuria and proteinuria (CHP)] who showed abnormal renal function, persistent hematuria and/or proteinuria for more than 6 months, nephrotic-range proteinuria, or those with underlying systemic diseases. Chronic glomerulonephritis (GN) was detected in 25 % of all visiting subjects. The most common findings in renal biopsies were immunoglobulin A (IgA) nephropathy in 38.97 %, mesangial proliferative GN in 24.29 %, and thin basement membrane nephropathy in 13.13 %. Compared with the relative frequency of renal diseases associated with urinary abnormalities, CHP (46.90 %) and nephrotic-range proteinuria (69.96 %) groups had more frequent GN than the others. Abnormal findings on renal ultrasound with or without Doppler scan were noted in 462 cases (suspected nutcracker phenomenon, 159; increased parenchymal echogenicity, 92; hydronephrosis, 75; simple cyst, 47). Mass urine screening tests could detect asymptomatic GN in its early stages. Initial aggressive diagnosis and treatment for CHP and nephrotic-range groups may prove helpful as interventions that delay chronic kidney disease progression. These findings may assist in the development of diagnostic and management guidelines for relatively mild urinary abnormalities, such as IH or low-grade IP.

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

Many studies have suggested that CXCL8 and CXCR2 play an important role in the pathogenesis of several types of renal diseases. However, there is no prior study on the association between polymorphisms of these genes and IgA nephropathy (IgAN), especially in children. The genotyping data from 192 patients with childhood IgAN and 397 controls showed significant differences in the frequencies of the CXCL8 gene with rs2227306 (dominant, P = 0.019; overdominant, P = 0.009), rs2227543 (dominant, P = 0.01; overdominant, P = 0.0057), and rs4073 (codominant, P = 0.034; dominant, P = 0.011; overdominant, P = 0.022). In addition, 2 single-nucleotide polymorphism frequencies of the CXCR2 gene (rs4674257 and rs4674259) significantly differed between the patients with pathologically mild and patients with advanced disease. Further, 5 single-nucleotide polymorphisms of the CXCL8 and CXCR2 genes significantly differed in the patients with infiltration of inflammatory cells on the renal biopsy samples. The results of this study suggest that polymorphisms of CXCL8 are associated with increased susceptibility to IgAN, and polymorphisms of CXCR2 with the pathological progression of childhood IgAN. These genetic variations might provide insight into novel individualized antichemokine regimens for treatment.

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.

Previous studies have suggested that insulin-like growth factor-1 (IGF-1) signaling might play an important role in renal fibrosis and regulation of the proliferation of mesangial cells and podocytes. We conducted the present study to investigate association between single nucleotide polymorphisms (SNPs) of IGF-1 (IGF-1) and IGF-1 receptor (IGF-1R) genes and childhood immunoglobulin (Ig) A nephropathy (IgAN). We analyzed five SNPs of IGF-1 and IGF-1R in 188 pediatric IgAN patients and in 263 healthy controls. We compared variations in SNPs in several sets of IgAN subgroups that were designated based on the presence of nephrotic range proteinuria (>40 mg/m2 per h), podocyte foot process effacement, and pathological progression. Genotyping of IgAN patients and controls revealed differences in IGF-1R rs2229765. Moreover, the rs2195239, rs978458, and rs1520220 SNPs of IGF-1 showed significant association with pathological progression. Thus, in the present study, we observed associations between the IGF-1/1R pathway, susceptibility to IgAN, and the pathologic progression of IgAN.

We evaluated the effectiveness of multidetector computed tomography (MDCT) as a diagnostic tool for nutcracker syndrome (NS) and its association with proteinuria. The angle and distance between the aorta and the superior mesenteric artery (SMA), the degree of difference in corticomedullary enhancement (DCE) between kidneys in the nephrographic phase of computed tomography, peak velocity ratio (PVR), and anteroposterior diameter ratio (APDR) in the sonogram were measured. The MDCT results, sonogram results, and the ratio of protein:creatinine were significantly different between NS patients and the controls. The area under the curve for angle, distance, and DCE were 0.895 +/- 0.058, 0.876 +/- 0.063, and 0.942 +/- 0.036, respectively. The cutoff values for angle and distance had sensitivity and specificity values of 96.2 and 80% for22.4 degrees and 84.6 and 80% for4.9 mm, respectively. The DCE had a sensitivity of 88.5% and a specificity of 100% for the positive scores. There were significant correlations between the degree of DCE and the ratio of protein:creatinine (r = 0.337, p = 0.031), and between distance and the ratio of protein:creatinine (r = -0.419, p = 0.006). We conclude that MDCT has diagnostic value for NS in children and that MDCT findings are correlated with proteinuria.