• Childhood Kidney Diseases
• /
• v.9 no.2
• /
• pp.159-166
• /
• 2005

Purpose : Recently the merits of 6 weeks of initial prednisolone treatment for pediatric primary nephrotic syndrome have been reported, and the use of the 6 week regimen is increasing. We compared our experiences with the 6 week treatment versus the 4 week treatment for Korean patients. Methods : We conducted a retrospective analysis of 69 children who had primary nephrotic syndrome and who were followed up for at least 12 months in the 4 major medical centers in Daegu. The remission rate, the relapse rate, the frequency of relapse and complication of steroid treatment were compared between the 4 weeks and 6 weeks treatment group. Results : Of the 69 children, 42 were in the 4 week treatment group and 27 were in the 6 week group. The median age, blood pressure, serum total protein, serum albumin, cholesterol, creatinine, estimated creatinine clearance, 24 hour urine protein and 12 month cumulative dose did not differ between the two groups. Among the children who relapsed after steroid treatment, the relapse time was significantly later for the 6 week treatment group. The relapse rate after 1 year of treatment was 62$\%$ in the 4 week treatment group and 52$\%$ in the 6 week treatment group; however, there was no statistically significant difference between the two groups. The frequency of relapse at 12 months was $1.5{\pm}1.2$ times in the 4 week treatment group and $1.1{\pm}1.2$ times in the 6 week treatment group, and there was not different between the two groups. The most common side effects of steroid treatment were an increase of appetite and a cushingoid appearance, and there was no statistical difference between the two groups. Among the 27 children who had kidney biopsies performed, 21 suffered from minimal change nephrotic syndrome. Conclusion : The first relapse time after steroid treatment was significantly later in the 6 week steroid treatment group. The frequency of relapse and the 12 month cumulative dose of steroid were lower in the 6 week treatment group, but there was no statistical significance between the two groups. The side effects of steroid treatment did not differ between the two groups. We need to study the long term side effects and the advanced regimens of steroid treatment in the future.(J Korea Soc Pediatr Nephrol 2005;9:159-166)

• Childhood Kidney Diseases
• /
• v.5 no.2
• /
• pp.125-135
• /
• 2001

Purpose : Efforts to predict long-term outcome of focal segmental glomerulosclerosis(FSCS) have been made but have yielded conflicting results. Reports are rare especially in Pediatric patients. In this study, we reviewed the predictable prognostic factors in patients of FSGS Method : Fifty children who diagnosed as biopsy-proven FSGS at department of pediatrics at Yonsei university were studied retrospectively. Based on medical records, response to treatment and pathologic slides, we compared normal renal function group and decreased renal function group, assessed the factors affecting renal survival and progression to renal failure. Results : The mean age at onset was 8 1/12 years, sex ratio was 2.3 : 1, and the mean duration of follow-up was 7 1/12 years. The overall renal survival rate was $34\%$ at 5 years, $8\%$ at 10 years Five-year survival rate was $74\%$ in normal renal function group and $27\%$ in decreased renal function group. Between the two groups, there were no significant differences in age at onset, sex ratio, amount of proteinuria, incidence of hematuria and hypertension, mesangial hypercellularity. Decreased renal function group showed higher serum creatinine level, poor response to treatment, higher percent of glomeruli with sclerosis, moderate to severe tubulointerstitial change and vascular change(P<0.05). The prognostic factors of renal survival rate were same as above and incidence of hypertension also affected renal survival( P<0.05). The progression rate to renal failure did not show statistically significant factor. Conclusion : We reviewed the factors affecting long-term outcome of FSGS. Serum creatinine level, steroid responsiveness, and the degree of glomerulosclerosis were significant prognostic factors. (J Korean Soc Pediatr Nephrol 2001 ;5 : 125-35)

• Childhood Kidney Diseases
• /
• v.10 no.2
• /
• pp.152-161
• /
• 2006

Purpose : In Korea, the school urine screening program is a useful tool for screening urine abnormalities. It is particularly useful in early detection of membranoproliferative glomerulonephritis(MPGN) I, which frequently progresses to chronic renal failure. In this study, we studied the medical history, laboratory findings, and histologic findings of MPGN to gain helpful information on early detection and treatment. Methods : The subjects were 19 children, who were diagnosed with MPGN from kidney biopsies that were performed in ten nationwide university hospitals because of abnormal urine findings from school urine screening programs conducted from July 1999 to April 2004. We divided the patients into 2 groups, a nephrotic range proteinuria group(n=8) and a non-nephrotic proteinuria group(n=11), and retrospectively analyzed the clinical features, laboratory findings, histologic findings, treatment, and clinical course. Results : The mean age at the first abnormal urinalysis was $10.6{\pm}2.2$ years in the nephrotic proteinuria group and $9.6{\pm}3.2$ years in the non-nephrotic proteinuria group. The mean age at the time of kidney biopsy was $11.3{\pm}2.3$ years in the nephrotic range proteinuria group and $10.4{\pm}3.2$ years in the non-nephrotic proteinuria group respectively. There was no significant difference in the mean age and sex between the two groups. In the nephrotic proteinuria group, 6 children had a low plasma C3 level and in the non-nephrotic proteinuria group, 8 children had a low plasma C3 level, but there was no significant difference between the 2 groups. There was no significant difference in the laboratory test results(including WBC count, RBC count, platelet count and other serologic tests) between the 2 groups except for 24 hour urine protein secretion. There was no difference between the 2 groups with regard to the acute and chronic changes in the glomerulus on light microscopic findings, IgG, IgA, Ig M, C1q, C3, C4, fibrogen deposition on immunofluoroscence findings, and mesangial deposits, subendothelial deposits, and subepithelial deposits on electron microscopic findings. The children were treated with corticosteroids, ACE(angiotensin-converting enzyme) inhibitors, dipyridamole and other immunosuppressive agents. During the course of treatment, there were no children whose clinical condition worsened. Among 19 children, 3 children went into remission(2 in the nephrotic proteinuria group, 1 in the non-nephrotic proteinuria group) and 9 children went into a partial remission(4 in the nephrotic proteinuria group, 5 in the non-nephrotic proteinuria group) on urinalysis. There was no significant difference in the treatment results between the two groups. Conclusion : The 73.7% of children who were incidentally diagnosed with MPGN by the school urine screening program had reduced C3. 42.1% of the children had nephrotic range proteinuria. There were no significant differences in clinical features, laboratory test results, light microscopic, immunofluorescence microscopic, and electron microscopic findings between the nephrotic proteinuria group and the non-nephrotic proteinuria group except for the 24 hour urine protein secretion. Therefore, for early detection of MPGN during the school urine screening program, we strongly recommend a kidney biopsy if children have abnormal urine findings such as persistent proteinuria and persistent hematuria, or if the serum C3 is reduced.

• Childhood Kidney Diseases
• /
• v.13 no.2
• /
• pp.229-234
• /
• 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
• /
• v.13 no.2
• /
• pp.215-221
• /
• 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
• /
• v.13 no.2
• /
• pp.189-196
• /
• 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
• /
• v.13 no.2
• /
• pp.146-152
• /
• 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
• /
• v.13 no.2
• /
• pp.138-145
• /
• 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.

• Development and Reproduction
• /
• v.12 no.1
• /
• pp.57-66
• /
• 2008

Changes in luteinizing hormone (LH), serum testosterone (T), and salivary T levels with age were examined in Korean men. Serum was obtained from 167 Korean men of different ages ($20{\sim}69\;y$), and the serum LH and T levels were measured. Saliva samples were also obtained, and the salivary T level was determined. The LH levels did not change considerably until 40 y of age (20s, $2.5{\pm}1.0$; 30s, $2.7{\pm}1.5$; and 40s, $2.5{\pm}1.8\;mIU/mL$) but increased significantly around 50 y (50s, $3.7{\pm}1.8$ and 60s, $3.1{\pm}1.7\;mIU/mL$). Further, the serum T levels also did not change until 40 y of age (20s, $5.3{\pm}2.6$, 30s, $4.4{\pm}1.4$, 40s, $4.1{\pm}1.5\;ng/mL$) but decreased significantly at 50 y (50s, $3.4{\pm}1.5$; 60s, $2.6{\pm}0.8\;ng/mL$). The salivary T levels also showed small changes until the age of 40 y ($20s{\sim}40s$, $0.11{\pm}0.015\;ng/mL$) but decreased significantly at 50 y ($0.08{\pm}0.03\;ng/mL$). Thus, the relative ratio of salivary T to serum T levels did not change significantly in all the ages examined ($2.4{\pm}0.9%$). Linear regression analysis predicted that the LH levels increased 1.5%/y while the serum and salivary T levels decreased 1%/y and 0.8%/y, respectively. The serum T/LH ratio did not change considerably until the age of 40 y ($20s{\sim}40s$, $2.27{\pm}0.14$) but decreased significantly ($1.2{\pm}1.0$) at 50 y. Age-related changes in the salivary T/LH ratio were very similar to those in the serum T/LH ratio. These results demonstrated that LH and T levels in serum or saliva did not change considerably until 40 y of age; instead, in Korean men, from 50 y of age, the LH level increased, while the T level decreased. This suggests that primary testicular failure that occurred due to aging (approximately 50 y) and caused this phenomenon. The present study also shows that the salivary T level can be an indicator of the free T level in serum although the salivary T level correlates weakly with the total T level in serum (r=0.53). Thus, information regarding salivary T levels may be useful for studying the age-related changes occurring in male testicular physiology.

• Clinical and Experimental Pediatrics
• /
• v.48 no.9
• /
• pp.953-959
• /
• 2005

Purpose : The administration of total parenteral nutrition(TPN) has become a standard procedure in the management of nutritionally deprived and critically low birth weight neonates. Sepsis remains the most frequent serious complication during TPN, resulting in increased morbidity, mortality and health care costs. This study was performed to evaluate the clinical efficacy and complications of percutaneous central venous catheterization(PCVC) in very low birth weight infants. Methods : A total of 56 very low birth weight infants below 1,500 g during the period from January 1998 to December 2003 were enrolled and their medical records reviewed. Study group(n=32) included the babies who had undergone PCVC and a control group(n=24) included babies who had not undergone PCVC. We compared the study group with the control group for factors such as subject characteristics and catheter-related complications. Results : There was no difference in subject characteristics, such as birth weight, gestational week, respiratory distress syndrome, duration of ventilator therapy, duration from tube to complete oral feeding, days at TPN and its total duration, body weight at discontinuation of TPN and the days taken to reach to 2,000 g. However, the morbidity rate due to patent ductus arterious, chronic lung disease, necrotizing enterocolitis, osteopenia, cholestasis, and sepsis showed no difference. The study group with infants below 1,000 g showed a higher incidence of sepsis compared to the control group of the same weight group. The study group with infants between 1,000 to 1,500 g showed significantly higher incidences of intraventricular hemorrhage and took longer reach the a body weight of 2,000 g. Conclusion : Considering the high incidence of sepsis in the PCVC group, every attempt should be made to minimize the length of TPN therapy and encourage early enteral feeding. We also recommend the use of PCVC carefully in patients requiring prolonged nutritional support.