• Childhood Kidney Diseases
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• v.13 no.2
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• pp.229-234
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• 2009

Purpose : The aim of this study was evaluating the efficacy of endoscopic $Deflux^{(R)}$ submucosal injection in children with primary vesicoureteral reflux (VUR). Methods : Retrospective analysis of medical record was conducted on 38 children (59 ureters) who underwent endoscopic $Deflux^{(R)}$ injection due to primary VUR. Data were collected from March 2000 to February 2006. Mean infused amount of $Deflux^{(R)}$ was 0.77 cc. After $Deflux^{(R)}$ injection, patients were reassessed by voiding cystourethrogram (VCUG) 6 months later. Results : The success rate of endoscopic $Deflux^{(R)}$ submucosal injection 6 months later by VCUG was 100% for grade 1 VUR, 87.5% for grade 2, 60% for grade 3, 26.6% for grade 4, 16.6% for grade 5, respectively and there was negatively significant correlation between success rate and grade of VUR (P<.01). Degree of improvement of VUR by endoscopic $Deflux^{(R)}$ submucosal injection was not related to age at diagnosis, time to operation, existence of voiding dysfunction or constipation and infused amount of $Deflux^{(R)}$. However, group with anticholinergics medication had significantly lower success rate than non-medication group (P<0.047). Conclusion : Endoscopic $Deflux^{(R)}$ submucosal injection is effective therapy in patient with primary VUR, especially low grade VUR. It can be not only a useful substitute for prophylaxis with antibiotics, but also an effective management prior to ureteroneocystostomy in children with primary VUR.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.215-221
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• 2009

Purpose : Thymus is a lymphoproliferative organ that changes size in various physiological states in addition to some pathological conditions. Thymus is susceptible to involution, and shows a dramatic response to severe stress. Thymic measurements may be helpful in various diseases. UTI (urinary tract infection) is most common bacterial infection in infants and VUR (vesicoureteral reflux) is a common abnormality associated with UTI. In our study, the size of thymus was compared on the premise that a greater stress is exerted on the body when UTI is accompanied by VUR, than when occurs on its own. Methods : Thymic size was measured on standard chest anteroposterior radiographs and expressed as the ratio between the transverse diameter of the cardiothymic image at the level of the carina and that of the thorax (CT/T). The medical records of 99 febrile urinary tract infection infants without other genitourinary anomalies except VUR were reviewed retrospectively. Results : Among 99 patients with febrile UTIs, 25 were febrile UTI without VUR and 74 with VUR. For the UTI with VUR group, there was a significant decrease in the thymic size compared to the those without VUR group ($0.382{\pm}0.048$ vs $0.439{\pm}0.079$, P<0.05). However, there were no differences in the duration of fever and WBC, CRP between the UTI with VUR and UTI without VUR. In addition, there were no differences in the cardiothymic/thoracic ratios between renal defects and renal scars in febrile UTI patients. Conclusion : The results of this study show that the shirinkage of thymus was more frequently found in the UTI patients with VUR. Therefore, awareness of the risks associated with thymic size is important for the appropriate work up and management of UTI patients.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.207-214
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• 2009

Purpose : To evaluate the cause of asymptomatic bacteriuria (AB) or aseptic pyuria (AP) on physiologic phimosis and to evaluate the effect of topical steroid therapy and preputial hygiene on the resolution of AB and AP. Methods : Ninety uncircumcised boys (age 1-72 month, median 16 month) with AB or AP were examined for physiologic phimosis and allocated by the preputial retractibility into the non-retractile group (n=59) or the retractile group (n=31). Topical steroid therapy [topical application of hydrocortisone (0.1%) cream with physiotherapy] were prescribed (three times a day) and the method of preputial hygiene (gentle retraction of prepuce and water cleansing) was instructed to the non-retractile group. After 2-4 weeks, the preputial retractibility was reevaluated and urine examination was repeated. To the retractile group, only the method of preputial hygiene was instructed and urine examination was repeated two weeks later. Results : Among 90 boys with AB and AP, 65.6% (59/90) had the nonretractile prepuces and nonperformed preputial hygiene. In the nonretractile group, the prepuces became retractile in 81.4% (48/59) after topical steroid therapy. Among boys (n=48) whose prepuces became retractile after topical steroid therapy, AB or AP resolved in 77.1%, decreased in 18.7% and persisted in 4.2%, which were significantly different to 18.2%, 2.37%, 54.5% in boys (n=11) whose prepuces were persistently nonretractile (P=0.0114). In the retractile group (n=31), 65.2% was compliant to preputial hygiene. In boys (n=23) who were compliant to preputial hygiene, AB or AP resolved in 65.2%, decreased in 26.0% and persisted in 8.2%, which were significantly different to 12.5%, 50%, 37.5% in boys (n=8) who were not compliant (P=0.0457). Conclusion : Physiologic phimosis was an important cause of AB or AP. Simple topical steroid therapy on the nonretractile prepuces and good preputial hygiene could improve AB or AP.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.189-196
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• 2009

Purpose : The pathophysiologic mechanism of nephrotic syndrome is not yet known clearly. At least in some cases, certain 'circulating factors' are thought to increase the glomerular protein permeability. Considering the systemic effect of the circulating factor on peritoneal membrane, we evaluated the loss of protein through peritoneal membrane in patients on peritoneal dialysis due to the end stage renal disease (ESRD) caused by steroid resistant nephrotic syndrome (SRNS). Methods : We retrospectively reviewed the medical records of 26 pediatric patients on peritoneal dialysis ensued during the period from 2001 to 2007 at our clinic. Twelve patients had SRNS, while 14 patients had ESRD caused by the congenital anomalies of urinary system. Results : While the other parameters including nPNA indicating the adequacy of protein intake were similar between the two groups, serum albumin was lower in SRNS patients than the non-SRNS patients ($3.7{\pm}0.3$ g/dL vs. $4.0{\pm}0.4$ g/dL, P=0.021). Peritoneal protein loss was higher in SRNS patients than in non-SRNS patients ($3,044.4{\pm}837.6\;mg/m^2$/day vs. $1,791.6{\pm}1,244.0\;mg/m^2$/day, P=0.007). The protein permeability of the peritoneal membrane measured by the ratio of total protein concentration in dialysate to plasma was twice as high in SRNS patients as the non-SRNS ($1.06{\pm}0.46%$ vs. $0.58{\pm}0.43%$, P=0.010). After 1 year, peritoneal protein loss increased in both patient groups, but to a significantly greater degree in non-SRNS patient (P=0.023). Conclusion : The results of our study support the notion that in nephrotic syndrome there are some 'circulating factors' with the systemic effect. Since the greater protein loss through peritoneal membrane in SRNS was confirmed in this study, more meticulous nutritional support and close monitoring on the nutrition are required in these patients.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.176-188
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• 2009

Purpose : Peritoneal dialysis (PD) is the major form of dialysis in use for infants and children with end-stage renal disease (ESRD). The aim of this study was to gain insight into the current status of children on PD in Korea. Methods : In May 2008, questionnaires were sent to the pediatric nephrologists via e-mail. Four centers replied and those data were reviewed. Results : A total of 103 patients were included in this study. Male to female ratio was 1.6:1. Mean age was $11.5{\pm}4.9$ years (0-19 years). Primary renal diseases diagnosed were as follows: primary glomerular disease (34%), chronic pyelonephritis-reflux nephropathy (14.6%), systemic disease (9.7%), renal hypoplasia/dysplasia (8.7%), heredofamilial disease (6.8%), vascular disease (3.9%), drug-induced nephropathy (1.0%), and unknown (12.6%). PD modalities were as follows: CAPD (42.7%), CCPD (27.2%), NIPD (11.7%), and Hybrid (18.4%). Weekly total Kt/V was $2.1{\pm}0.7$ (0.3-4.1). Results of peritoneal equilibrium test were as follows: low 36.8%, low average 31.6%, high average 19.7%, and high 11.8%. Z-score for weight was $-1.00{\pm}1.20$ (-4.54~+2.50). Z-score for height was $-1.55{\pm}1.65$ (-9.42~+1.87). Growth hormone was administered in 24.3% of patients. Anti-hypertensive drugs were administered in 64.0% of patients. Laboratory findings were as follows: hemoglobin $10.5{\pm}1.4$ g/dL, calcium $9.7{\pm}0.7$ mg/dL, phosphorus $5.4{\pm}1.4$ mg/dL, and parathyroid hormone $324.2{\pm}342.8$ pg/mL. Conclusion : Primary glomerular disease was the most common cause of ESRD. CAPD was the most prevalent PD modality. Low and low average peritoneal transport type were common. Growth disturbance were noted in many patients. Some patients had hypertension even with anti- hypertensive drugs. Calcium-phosphorus levels were maintained adequately, but many patients had secondary hyperparathyroidism.

• 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.