• Childhood Kidney Diseases
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• v.13 no.2
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• pp.161-169
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• 2009

Purpose : This study was performed to report the diagnosis and treatment of nephrotic syndrome manifesting in the first year of life. Methods : We retrospectively reviewed the clinical data with chart review in 7 patients who were diagnosed as nephrotic syndrome manifesting in the first year of life from 1996 to 2007. Results : Three patients had congenital nephrotic syndrome, the other 4 patients had infantile nephrotic syndrome. Their ages ranged from birth to 11 months and male to female ratio was 1 to 6. Renal biopsies were done in 6 patients. One patient had Finnish type congenital nephrotic syndrome, 2 patients had diffuse mesangial sclerosis, 2 patients had focal segmental glomerulosclerosis and 1 patient had minimal change disease. Genetic analyses of NPHS2, PLCE1, and WT1 were done in 4 patients and 2 of them had WT1 mutation. Among 3 patients with congenital nephrotic syndrome, 1 patient was diagnosed as congenital nephrotic syndrome of Finnish type and the other 2 patients were diagnosed as Denys-Drash syndrome. All of the patients with congenital nephrotic syndrome died due to sepsis. Among 4 patients with infantile nephrotic syndrome, 2 patients died and 1 had remission, another patient progressed to end stage renal disease. Conclusion : Most of nephrotic syndrome manifesting in the first year was hereditary renal disease. Patients with nephrotic syndrome manifesting in the 3 month of life had poorer prognosis and needed more aggressive management including early dialysis and renal transplantation might be considered compared with infantile nephrotic syndrome. Further genotype-phenotype correlation studies are needed.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.146-152
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• 2009

Purpose : We performed this study to evaluate the incidence and clinical significance of antiphospholipid antibodies (aPL Ab) in Korean children with Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods : The medical records of 62 patients (31 boys and 31 girls) aged $46.0{\pm}3.1$ (1-16) years with a clinical diagnosis of HSP based on the EULAR/PReS criteria were reviewed retrospectively. From the years 2007 to 2009, the sera from children with acute HSP were tested for aPL Ab such as LA, anti-cardiolipin antibody and anti-${\beta}_2$ glycoprotein I antibody. Results : LA was positive in 18 (29%) of the 62 patients with HSP and We divided the patients into the two groups LA positive group (N=18) and LA negative group (N=44). There were no significant differences between the two groups with regard to abdominal pain, arthralgia and renal involvement, but LA positive group had significantly higher C-reactive protein ($4.3{\pm}7.2$ mg/dL vs. $1.3{\pm}1.8$ mg/dL, P=0.035), erythrocyte sedimentation rate ($37.5{\pm}26.2$ mm/hr vs. $25.1{\pm}22.6$ mm/hr, P= 0.039), IgM ($148.1{\pm}48.4$ mg/dL vs. $114.9{\pm}41.5$ mg/dL, P=0.024), C3 ($143.1{\pm}21.9$ mg/dL vs. $129.7{\pm}24.5$ mg/dL, P=0.048) and C4 levels ($30.9{\pm}6.3$ mg/dL vs. $24.9{\pm}7.8$ mg/dL, P=0.002) compared with LA negative group. Conclusion : We found that the incidence of positive aPL Ab tests was relatively higher in Korean children with HSP and the presence of aPL Ab was associated with acute inflammatory process of HSP. These results suggest that the aPL Ab are involved in the pathogenesis of HSP in children.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.235-241
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• 2009

Purpose : The purpose of this study was to determine the clinical value of bicarbonate gap (BG) in acute gastroenteritis by comparison of clinical manifestations and laboratory findings. Methods : We retrospectively analyzed 135 patients who had acute gastroenteritis. We classified them into two groups: $BG{\leq}-5$ (group A), BG>-5 (group B). We made a comparative study for clinical manifestations, serum electrolyte, albumin, protein, blood urea nitrogen (BUN), creatinine, anion gap (AG), delta anion gap (${\Delta}AG$) and delta bicarbonate (${\Delta}HCO_3{^-}$) between the two groups. Results : The duration of hospitalization and diarrhea was significantly longer in group A than group B. In laboratory findings, serum sodium, serum total $CO_2$, total protein, $AG_{corrected}$ and ${\Delta}AG$ were lower in group A than group B. ${\Delta}HCO_3{^-}$ and chloride were higher in group A than group B. Conclusion : BG may be a sensitive predictor enough to access the severity of acute gastroenteritis.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.138-145
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• 2009

Purpose : This study was aimed to evaluate the changes of immunologic parameters during hospitalization, and the relationship between IgG and other laboratory or clinical indices in patients with acute poststreptococcal glomerulonephritis (APSGN). Methods : We reviewed the medical charts of 36 children with APSGN who showed ASO titer>250 Todd U/L and C3<70 mg/dL. We evaluated the levels of IgG and other laboratory parameters including C3 and ASO at admission and at discharge (14 cases). Results : The mean age of APSGN patients was $7.5{\pm}2.6$ year of age, and male-to-female ratio was 2.3:1. At presentation, hypertension (systolic blood pressure>125 mmHg), gross hematuria, and weight gain were observed in 27.8% (10/36), 80.1% (29/36), and 80% (24/30) of the patients, respectively. The mean IgG level was $1,432{\pm}322$ mg/dL ($1,025{\pm}234$ mg/dL in control group, P<0.001), and C3 and ASO levels were $26.1{\pm}16.1$ mg/dL and $1,068{\pm}730$ Todd U, respectively. There were no correlation between IgG level and the levels of any of the parameters analyzed (ASO, C3, BUN, creatinine and white blood cell count), and the severity of the disease assessed by the weight-change during admission. The patients aged<6 years of age (10 cases) had less degree of the weight-change, compared to those of the patients aged>8 years of age (15 cases) (-0.6% vs. -5.7%, P=0.01). The IgG and ASO levels did not change, but C3 (P=0.001) and IgM (P=0.02) levels increased during admission. Conclusion : Increased IgG and ASO levels in APSGN did not change, but C3 level increased during admission. IgG level was not correlated with other laboratory parameters (ASO and C3) and the severity of the disease. Younger children seem to have less severe clinical course compare to older children. With our hypothetic pathogenesis of APSGN, further studies are needed to resolve the pathogenesis of the disease including the increase of IgG.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.130-137
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• 2009

Purpose : Stem cell transplantation (SCT) has gained worldwide acceptance as a treatment for hematologic disorders. This study was performed to evaluate the clinical characteristics and outcomes of the acute kidney injury after SCT in children. Methods : The records of 53 patients who were treated with SCT at the pediatric department of Yeungnam University Hospital between January, 1996 and April, 2009 were used as subjects. Their were divided into two groups ; 'Early renal insufficiency' (ERI, n=18) and 'Non-early renal insufficiency' (NERI, n=35). ERI had greater than 25% of drop in GFR after SCT. Results: Total 53 patients were analyzed. In cord blood SCT (n=11), ERI was 4 (36.4%) and NERI was 7 (63.6%). In bone marrow SCT (n=16), ERI was 8 (50.0%) and NERI was 8 (50.5%). In autologous peripheral blood SCT (n=26), ERI was 6 (23.1%) and NERI was 20 (76.9%). There is no difference in both groups according to kinds of SCT. GVHD was developed in 22 patients, and there is no difference in each group. Twenty two of 53 patients died. ERI was 12 (66.7%) and NERI was 10 (28.6%). Acute renal failure is most important cause of the deaths. Conclusion : Out of 53 pediatric patients who were treated with SCT, 18 patients had greater than 25% of drop in GFR. There is no difference in both groups according to kinds of SCT. GVHD was found in 22 patients and there is no relation between GVHD development and acute kideney injury.

• Childhood Kidney Diseases
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• v.15 no.2
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• pp.163-171
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• 2011

Purpose : To evaluate the prevalence of vesicoureteral reflux (VUR) according to the timing of voiding cystourethrography (VCUG) in infantile urinary tract infection (UTI). Methods : The data of 134 infants (1-12 months) with renal cortical defect in $^{99m}Tc$-2, 3-dimercaptosuccinic acid ($^{99m}Tc$-DMSA) scan with a diagnosis of UTI in two hospitals from 2000 to 2010 were retrospectively analyzed. The VCUG was performed after 2 weeks from the diagnosis of UTI in Group I (n=68), and the VCUG was performed within 2 weeks from the diagnosis of UTI in Group II (n=66). Results : There were no significant differences between the two groups in the duration of fever, white blood cell count, C-reactive protein levels, and abnormalities in ultrasonography (P>0.05). There was no significant difference between the two groups in the prevelence of VUR, bilateral VUR, and severe VUR. VCUG-induced UTI was detected 16 (23.5%) of patients in whom the procedure was performed 2 weeks after the diagnosis, and none of VCUG-induced UTI occurred in those in whom the procedure was performed 2 weeks within the diagnosis. Conclusion : We conclude that the prevalence of VUR according to the timing of VCUG did not differ between the two groups in infantile UTI with renal cortical defect in DMSA scan. We also found that performing VCUG with antibiotics can decrease risk of VCUG-induced UTI.

• Childhood Kidney Diseases
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• v.15 no.2
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• pp.138-145
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• 2011

Purpose : To test whether the expression of ${\beta}$-catenin, a component of podocyte as a filtration molecule, would be altered by puromycin aminonucleoside (PAN) in the cultured podocyte in vitro. Methods : We cultured rat glomerular epithelial cells (GEpC) with various concentrations of PAN and examined the distribution of ${\beta}$-catenin by confocal microscope and measured the change of ${\beta}$-catenin expression by Western blotting and reverse transcriptase-polymerase chain reaction (RT-PCR). Results :We found that ${\beta}$-catenin relocalized from peripheral cytoplasm to inner cytoplasm, therefore, intercellular separations were seen in confluently cultured cells by high concentrations of PAN in immunofluorescence views. In Western blotting of GEpC, PAN ($50{\mu}g/mL$) decreased ${\beta}$-catenin expression by 34.9% at 24 hrs and 34.3% at 48 hrs, compared to those in without PAN condition (P<0.05). In RT-PCR, high concentrations ($50{\mu}g/mL$) of PAN also decreased ${\beta}$-catenin mRNA expression similar to protein suppression by 25.4% at 24 hrs and 51.8% at 48 hrs (P<0.05). Conclusion : Exposure of podocytes to PAN in vitro relocates ${\beta}$-catenin internally and reduces ${\beta}$-catenin mRNA and protein expression, which could explain the development of proteinuria in experimental PAN-induced nephropathy.

• Childhood Kidney Diseases
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• v.15 no.2
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• pp.146-153
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• 2011

Purpose : Hypercalciuria has been suggested to be involved in the pathogenesis of nocturnal enuresis associated with polyuria. We have measured the amount of calcium excretion in the urine from children with monosymptomatic nocturnal enuresis and studied the association with enuresis. Methods : Thirty-six children (20 males and 16 females, age 5 to 15.6 years) presenting with monosymptomatic nocturnal enuresis were recruited for this study. Results : Among 36 patients, 6 patients had hypercalciuria, providing a 16.7% frequency. The mean Ca/Cr ratio of hypercalciuria group showed $0.23{\pm}0.10$ on daytime, and $0.33{\pm}0.10$ on nighttime showing higher values in nighttime, but it was not statistically significant. Average 24 hour calcium excretion was 8.95 mg/kg. Night/day time urine volume ratio in hypercalciuria group was $0.87{\pm}0.20$, and that in normocalciuria group was $0.81{\pm}0.33$, also showing no difference. Daytime Ca/Cr ratio and nighttime Ca/Cr ratio from all enuresis patients showed a significant correlation (P=0.0001). However, Ca/Cr ratio between daytime and nighttime urine volume had no significant correlation, respectively (daytime P=0.08; nighttime P=0.07). Also, daytime and nighttime Na concentration, urea concentration, and osmolality also had no significant correlation with urine Ca/Cr ratio, respectively. Conclusion: Hypercalciuria shown in some of enuresis patients is not directly caused by primary monosymptomatic nocturnal enuresis.

• Childhood Kidney Diseases
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• v.15 no.2
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• pp.154-162
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• 2011

Purpose: With increasing prevalence of hypertension (HTN) in children and adolescent, pediatricians have become more interested in blood pressure (BP) measurements. The ambulatory blood pressure monitoring (ABPM) is known to be useful to differentiate true HTN and white coat HTN. The object of this study is to assess the clinical usefulness of ABPM in Korean children and adolescents. Methods: A retrospective review of 51 patients in Kyungpook National University Hospital from January 2002 to February 2010 was done. All patients were 6-18 years old and underwent ABPM. We calculated the mean value of ABP, BP load, nocturnal dip and compared the results with the patients' diagnosis and characteristics. Results: The mean age of the 51 patients was $17.8{\pm}1.8$ years and 19 children were obese. 37 patients (72.5%) were truly hypertensive and 1 patient was diagnosed as masked HTN and 7 children (14%) as white coat HTN. The rest of the patients were normotensive. Among patients with white coat HTN, 5 were in a prehypertensive state. Mean systolic and diastolic BP load of patients with true HTN were significantly higher than non-hypertensive children (P<0.001). Although the nocturnal dip of all patients were below 10%, there was no statistical significance. The obese patients showed higher systolic and diastolic BP. Their systolic and diastolic BP load were significantly higher than non-obese patients (P<0.001). Conclusion: ABPM in children and adolescents seems to be a valuable tool in the assessment of white coat HTN and in the confirmation of true HTN. A considerable number of white coat HTN patients are revealed to be in a prehypertensive state and need close follow-up.

• Childhood Kidney Diseases
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• v.17 no.1
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• pp.19-24
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• 2013

Purpose: Recently, enterococcus spp. have become one of the most common nosocomial pathogens with increasing rates of multi-drug resistance. However, study on enterococcal urinary tract infections (UTIs) in children is very limited, especially community acquired UTIs. We studied the clinical characteristics of enterococcus spp. in community acquired UTIs and antibiotic resistance within our urban area. Methods: All children with first episode of community acquired UTIs due to enterococcus spp. and Echerichia coli who were admitted in Pusan National University Children's Hospital between January 2010 and January 2013 were included in our study. We retrospectively reviewed their medical records. Results: During the study period, 201 patients were identified to have first episode of community acquired UTIs. 154 cases were E.coli UTIs (76.6%) and 11 cases were enterococcal UTIs (5.5%) and all enterococcus spp. were Enterococcus feacalis. In enterococcal UTI group, voiding cystourethrogram(VCUG) was performed in 7/11 patients and demonstrated 4 vesicoureteral refluxes (VURs) with renal scar and 3 patients underwent corrective surgery. In E.coli UTI group, VCUG was performed in 121/154 patients and demonstrated 23 VURs and 11 patients underwent corrective surgery. Enterococcal group had significant high rate of underlying urinary abnormalities and surgical corrections compared with E. coli group. All enterococcus spp. were susceptible to ampicillin, vancomycin and linezolid, but all were resistant to tetracycline. They also showed 71.4% resistance to trimethoprim-sulfamethoxazole and 20% resistance to ciprofloxacin. Conclusion: Community acquired enterococcal UTIs in children were rare within our urban area. However, they could be indicative of severe underlying urinary tract abnormalities.