• Childhood Kidney Diseases
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• v.13 no.1
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• pp.33-39
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• 2009

Purpose : Our aim is to reduce the rate of lumbar spinal tapping and voiding cystourethrography (VCUG) in febrile urinary tract infection (UTI) infants less than 3 months of age. Methods : We reviewed the prevalence of UTI, sepsis, meningitis and UTI with bacterial meningitis in febrile infants less than 3 months of age during the period from Jan. 2001 to Jun. 2008. Renal ultrasonography, Technetium-99m dimercaptosuccinic acid (DMSA) renal scan or VCUG were performed in infants with UTI. Infants with UTI were divided into two groups according to the presence of abnormal findings of ultrasonography and DMSA renal scan : group 1-Infants in whom both ultrasonography and DMSA were normal, group 2-Infants in whom ultrasonography or DMSA were abnormal. Prevalence of VUR was compared between the two groups. We followed up the clinical course of patients who had VUR in group 1. Results : Among 1962 Infants, UTI, sepsis and bacterial meningitis were diagnosed in 620 (31.6%), 63 (3.2%), 8 (0.4%) respectively. Lumbar puncture was performed in 413 (66.6%) infants with UTI and we did not detect a case of bacterial meningitis in association. 348 infants with proven UTI were undergone ultrasonography, DMSA, VCUG. In group 1 with 110 infants (31.6%), the presence of VUR was 4 (3.6%). In group 2 with 238 infants (68.4%), the presence of VUR was 51 (21.4%). Abnormal findings of ultrasonography or DMSA renal scan were closely related with high grade VUR. Most of patients with VUR in group1 had good prognosis. Conclusion : Lumbar puncture and VCUG are invasive procedures. Therefore we should decide whether to perform lumbar puncture or VCUG in infants less than 3 months. of age with their first febrile UTI.

• Clinical and Experimental Pediatrics
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• v.52 no.8
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• pp.944-952
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• 2009

Purpose : To investigate the effects of angiotensin II inhibition on the epithelial to mesenchymal transition (EMT) in the developing kidney, we tested the expression of EMT markers and nestin in angiotensin converting enzyme (ACE) inhibitor-treated kidneys. Methods : Newborn rat pups were treated with enalapril (30 mg/kg/d) or a vehicle for 7 days. Immunohistochemistry for the expression of ${\alpha}$-smooth muscle actin (SMA), E-cadherin, vimentin, and nestin were performed. The number of positively-stained cells was determined under 100 magnification in 10 random fields. Results : In the enalapril-treated group, ${\alpha}SMA-positive$ cells were strongly expressed in the dilated tubular epithelial cells. The number of ${\alpha}SMA-positive$ cells in the enalapril-treated group increased in both the renal cortex and medulla, compared to the control group (P<0.05). The expression of E-cadherin-positive cells was dramatically reduced in the cortical and medullary tubular epithelial cells in the enalapril-treated group (P<0.05). The number of vimentin- and nestin-positive cells in the cortex was not different in comparisons between the two groups; however, their expression increased in the medullary tubulointerstitial cells in the enalapril-treated group (P<0.05). Conclusion : Our results show that ACE inhibition in the developing kidney increases the renal EMT by up-regulating ${\alpha}SMA$ and down-regulating E-cadherin. Enalapril treatment was associated with increased expression of vimentin and nestin in the renal medulla, suggesting that renal medullary changes during the EMT might be more prominent, and ACE inhibition might differentially modulate the expression of EMT markers in the developing rat kidney.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.164-175
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• 2001

Purpose : Alport syndrome is a hereditary nephrotic disease characterized by progressive nephrotic symptom, sensorineural hearing loss, ophthalmic abnormality, typical microscopic findings, and familial occurrence. In this study, we tried to find the risk factors related with its prognosis by taking a close observation on clinical symptoms of children with Alport syndrome reviewing retrospectively. Materials & methods : We chose children diagnosed as Alport syndrome in renal biopsy during 20 years(from 1980, Jan. until 1999, Dec.) who could receive follow up studies in tile department of pediatrics. They were divided into two groups by comparing renal function at the time of diagnosis and at current status. We compared several clinical aspects in them, and applied nonparametric test for statistical analysis. Results : The sex ratio(male:female) of 24 children was 3:1. The most common clinical symptom presented at their first visit was gross hematuria. Among those 24 children, 11 cases($46\%$) of progressing into chronic renal failure(Group II) were observed. Hypertension, proteinuria and edema were seen much frequently in group II. The level of serum protein, albumin, and creatinine clearance were decreased while BUN, creatinine were relatively increased. All the results were statistically significant. Conclusion Clinically significant risk factors related to prognosis in Alport syndrome were the presence of hypertension, edema, and proteinuria at the time of diagnosis. Also, the level of serum protein, albumin, BUN, creatinine, and glomerular filtration rate were proved to be important factors in predicting prognosis. We believe that studies on these possible risk factors would be of great help in treating and predicting prognosis of children suffering with Alport syndrome. (J Korean Soc Pediatr Nephrol 2001;5 : 164-75)

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.196-205
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• 2001

Purpose : Membranous glomerulopathy is a glomerular disease characterized by the presence of subepithelial immune deposits with thickening of the capillary wall of the glomerulus without inflammatory change. The pathogenesis of membranous glomerulopathy is still unknown. Its incidence is higher in males, and it is rarely found in infants and adolescents. Among the clinical manifestations proteinuria is most common, while edema and hematuria are present. According to reports from other countries, among few patients diagnosed with membranous glomerulopathy by renal biopsy, show isolated microscopic hematuria without the clinical manifestations. Little research in this area has been performed in Korea, and so we conducted retrograde studies on membranous glomerulopathy associated with isolated microscopic hematuria. Materials and Methods : We analyzed retrogradely 109 cases of asymptomatic isolated microscopic hematuria that were diagnosed as membranous glomerulopathy by renal biopsy at Yonsei University Severance hospital from January, 1992 to July, 2001. Results : In 87 of the 109 cases patients were over 15 years old while in 22 cases patients were under 15 at the time of dignosis. Only three patients showed isolated microscopic hematuria without the clinical manifestations and abnormal laboratory findings and they were all male patients under 15 years old. Conclusion : Few cases of the membranous glomerulopathy show only asymptomatic isolated microscopic hematuria However, since membranous glomerulopathy can be found in patients who present with asymptomatic isolated microscopic hematuria only, if adequate indication for renal biopsy is present, we conclude that renal biopsy must be aggresively pursued in order to find the underlying disease. (J Korean Soc Pediatr Nephrol 2001 ; 5 : 196-205)

• 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.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.3 no.1
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• pp.88-94
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• 1999

Purpose : Malformation of urinary tract is among the most common of all congenital anomalies and can progress to irreversible renal damage before diagnosis due to difficulty of early diagnosis. Present study was undertaken to determine the clinical characteristics of urinary tract anomaly and to find out the most appropriate diagnostic and therapeutic measures for children with these anomalies. Methods : During the past 10 years from 1987 to 1998, review of medical records revealed 65 children with congenital anomaly of urinary tract and the following results were obtained. Results : The most common anomalies were ureteropelvic junction obstruction occuring in 26 cases ($36\%$), followed by ureteral duplication in 11 cases, renal agenesis in 10 cases and ureterovesical function obstruction in 7 cases. Complex anomaly of urinary tract was found in 8 cases and anomaly of other systems such as congenital heart disease was detected in 11 cases. The most frequent age group at the time of diagnosis was below 1 year of age constituting 39 cases ($60\%$) and male preponderance was noted as male to female ratio being 2.25:1. Presenting symptoms were urinary tract infection in 25 cases, followed by hematuria, abdominal mass, abdominal pain and voiding difficulty, etc, and in 11 cases, the anomaly was picked up by routine prenatal ultrasonography. Azotemia was noted in 9 cases and the underlying anomaly was obstructive uropathy in 4 out of these 9 cases. Surgical correction was undertaken in 38 cases (most frequently in cases of obstructive uropathy) and in 2 out off cases with obstructive uropathy in whom surgical correction was done, azotemia disappeared during follow up period of 1-5years. No new cases of deteriorating renal function appeared during follow-up period. Conclusion : In spite of high incidence of congenital malformation of urinary tract, early diagnosis is still hampered by nonspecific symptoms and signs. Therefore, in patients with symptoms such as urinary tract infection, abdominal pain and voiding problems, etc, it Is advisable to take various diagnostic tests promptly to pick up any urinary tract anomaly and to apply proper therapy in order to avoid progression to irreversible renal damage. In this regard, prenatal ultrasonography should be utilized more widely as a routine procedure to detect any urinary tract anomalies before birth.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.1-10
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• 1999

Purpose: Up to date there is no nationwide survey on epidemiological or clinical data of nephrotic syndrome, so we investigated about age of onset, sex, result of renal biopsy, treatment method, its results of treatment, its responsiveness, time of response to treatment and pattern of relapse in Korea. Methods: Between 1987 and 1997, 2193 patients with primary nephrotic syndrome diagnosed at 38 university hospital and general hospital in Korea were included. Of these 1655 were male and 538 were female. Incidence peaked at 1-5 years of age. Results: Results were as follows; 1) Among 2193 cases, male was 1655($75.5\%$), female was 538 cases($24.5\%$) and male to female ratio was about 3:1. Among 1752 patients with MCNS, male was 1338, female was 414 and male to female ratio was about 3.23:1. The most prevalent age group was 1-5 years of age. 2) Renal biopsy was done in 942 cases($43\%$), pathologic findings were as follows; MCNS 646 cases($68.6\%$), FSCS 149 cases($15.8\%$). 3) Regimen of treatment were as follows; prednisolone 1191 cases, Calcort 192 cases, cyclophosphamide 251 cases, cyclosporin A 223 cases, MPD pulse therapy 120 cases.4) Complete response to treatment were noted in 1597 cases($82.2\%$, n=1944). 5) Responsiveness according to result of renal biopsy were significantly different between MCNS and FSGS. Complete response were noted in $86.5\%$ among patients with MCNS, $35.8\%$ in patients with FSGS. 6) Time of response to treatment were noted between 1 and 4 weeks after treatment in 879 cases($67.4\%$, n=1305). 7) 994 cases($73.1\%$) relapsed during follow up, most frequently between 2 months and 6 months after response. Conclusion : Nationwide survey of epidemiological and clinical data were performed in childhood primary nephrotic syndrome. Most of the clinical and epidemiological data were similar to other reports from U.S.A. and from Europe, however male to female ratio is higher in Korean nephrotic syndrome(3:1 in contrast to 2:1).

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.72-79
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• 1999

Renal scarring associated with vesico-ureteral reflux(VUR) is one of the major causes of end stage renal failure and renal hypertension in children. Urinary ${\beta}_2$-microglobulin(MG) has been suggested as a potential marker for presence of renal tubular damage. This study was designed to evaluate the significance of random urine ${\beta}_2$-MG as a predictor of presence of vesico-ureteral reflux in children with urinary tract infection(UTI). 57 children with urinary tract infection were studied. Patients were devided into two groups; 35($78.9\%$) children have UTI without VUR and 12($21.1\%$) children have UTI and VUR. Beta2-MG and creatinine in random urine sample was measured to decide the excretion ratio(${\beta}_2$-MG/creatinine). Among the 57 children with UTI, 44 children were confirmed by urine culture study and 13 children suspected by compatible clinical feature. Random urine ${\beta}_2$-MG of VUR group ($2.2{\pm}5.91$ mg/L) were significantly higher than that of simple UTI group($0.19{\pm}0.16mg/L$)(P=0.03). The ${\beta}_2$-MG/creatinine ratio of VUR group($32.41{\pm}25.7$) were significantly higher than that of simple UTI group($3.93{\pm}3.44$)(P=0.007). In conclusion, random urine ${\beta}_2$-MG and excretion ratio deserved early predictor of presence of VUR in children with UTI. And this method was more simple and inexpensive than the method of measuring ${\beta}_2$-MG with 24 hour urine collection, so might be a useful screening test for VUR in children with UTI.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.32-40
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• 2007

Purpose : We performed this study to determine the incidence of acute renal failure(ARF) in birth asphyxia and to correlate the severity of asphyxia and hypoxic-ischemic encephalopathy (HIE) and ARF in asphyxiated neonates. Methods : Data was retrospectively collected from the medical records of 33 patients with neonatal asphyxia and of 33 neonates with no asphyxia. On the basis the 5-minute Apgar score, the asphyxiated neonates were further grouped into mild(6 or 7), moderate(4 or 5), and severe asphyxia(3 or less). Asphyxiated neonates with HIE were staged by the Sarnat and Sarnat scoring system. We compared serum creatinine, blood urea nitrogen, electrolytes, and urine output on day 3 of life and the incidence and severity of intraventricular hemorrhage(IVH) between each group. Results : ARF occurred in 8(24.2%) asphyxiated neonates. Of these, 3(37.5%) were oliguric, while 1(10.0%) patient with mild asphyxia, 2(18.2%) of moderate asphyxia, and 5(41.7%) with of severe asphyxia had ARF(P>0.05). One(25%) patient with stage I HIE, 4(50%) with stage II HIE, and 3(75%) of HIE with stage III HIE developed ARF(P<0.01). There was no statistical correlation between the severity of asphyxia and HIE stage. One(7.7%) patient with grade 1 IVE, 0(0.0%) with grade 2 IVH, 2(66.7%) with grade 3 IVH, and 2(100.0%) with grade 4 IVH had ARF(P<0.01). Mortality was higher in asphyxiated neonates with ARF(P<0.05). There was no significant difference between the oliguric and non-oliguric renal failure. Conclusion : We found that the greater the degree of HIE, the higher was the incidence of ARF. Asphyxiated neonates with ARF had a poorer prognosis.