• Childhood Kidney Diseases
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• v.9 no.1
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• pp.56-63
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• 2005

Purpose : Development of renal scarring is associated with delayed diagnosis and treatment of urinary tract infection(UTI). This study was performed to clarify how soon treatment should be started to Inhibit renal scarring after onset of UTI and the factors associated with renal scarring in children with a first episode of febrile UTI. Methods : We retrospectively reviewed 163 patients with a first episode of febrile UTI under the age of 2 years from April 2000 to Ap,il 2004. All patients had a DMSA renal scan and voiding cystourethrogram done in the diagnostic period, 6 months after which a follow-up renal scan was done. After patients wet-e divided into 2 groups according to the duration of fever prior to start of treatment, the duration of fever after start of treatment, and total duration of fever, initial and follow-up DMSA scan findings were analyzed among the different groups. We compared the factors associated with renal scars between the groups with and without renal scars. Results : The initial DMSA renal scan identified abnormal finding in 23% of the patients who were treated $\leq$24 hr from the onset of disease and in 43% of those with fever more than 24 hr. Renal scars developed in 33% of patients who were treated $\leq$24 hr and 38% of those with fever >24 hr prior to treatment. Renal scars developed in 34% of patients with remission of fever $\leq$48 hr after treatment and ill 50% of those with fever >48 hr after treatment. The risk for renal scars was significantly higher in children who had total duration of feyer >72 hr(67%) than in those with shorter duration(19%). In children with renal scars, VUR was most highly associated with an increased risk of renal scar formation. Conclusion : Although children with a first episode of febrile UTI are treated within 24 hr after onset of the fever, renal damage cannot be prevented completely and it is mainly associated with VUR. (J Korean Soc Pediatr Nephrol 2005;9:56-63)

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.64-68
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• 2005

Purpose : Renal abscess is very rare in children and its diagnosis is difficult because symptoms are often nonspecific. In previous studies, on]y 15% to 25% of patients were reported to be diagnosed at the time of admission. Early diagnosis and treatment are important be cause mortality rate correlates positively with the time of diagnosis. The purpose of this study is to clarify the clinical features of children with renal abscess and to investigate the possible indicators of this disease for early diagnosis and Proper treatment. Methods : Twelve children diagnosed with renal abscess from Jan. 1996 to Jul. 2004 were included. The age of patients ranged from S months to 15 years. We retrospectively analyzed the demographics of patients, their symptoms, predisposing factors, diagnostic methods and causative organisms and the treatment modalities. Results : Fever was the most common manifestation, Five children(42%) had vesicoureteral reflux. Renal ultrasonography and computerized tornography were the most frequently used imaging tools to detect renal abscess. Gram negative bacteria were isolated in 7 patients and Staphylococcus aureus grew in 2 patients. All patients received intravenous antibiotics and 4 patients underwent aspiration or drainage of renal abscess. The average admission duration was 30 days. Conclusion : Renal abscess should be included in the differential diagnosis of prolonged fever in children, especially when flank pain is combined. For early diagnosis and a better prognosis, patients should be promptly investigated with ultrasonography or computerized tomography.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.8-14
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• 2005

Purpose : This study was peformed to study normative blood pressure data in full-term neonates that may be used to facilitate Identification of neonatal hypertension.Methods : 383 newborns born in our hospital from May 2003 to January 2004 were enrolled in this study Using an oscillometric device(BP-88 NEXT, COLIN Corp.), their blood pressures were measured more than one time within a week after birth. According to each clinical variable such as sex, delivery mode, birth weight, gestational age and presence of maternal disease or perinatal problems, we divided the population into groups and calculated the mean blood pressures of each group. We compared mean blood pressures between the divided groups according to each clinical variable statistically.Results : Mean systolic and diastolic blood pressure of the Population was 70.8$\pm$ 10.9 mmHg and 43.4 $\pm$ 8.0 mmHg, respectively. There was no satistically significant difference in blood pressure according to clinical variables. Mean systolic pressure showed positive correlation with birth weight and gestational age(r=0.1420, 0.03130).Conclusion : Our results are almost in agreement with Zubrow's data from 695 newborns in U.S.A, 1995. Our data may be helpful for early detection and management of neonatal hypertension, thereby maintaining renal function ,and preventing possible complications of renal disease.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.15-20
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• 2005

Purpose : Nutcracker syndrome must be considered when hematuria or proteinuria occurs in a healthy child. The purpose of this study is to investigate the prevalence of nutcracker syndrome among children with asymptomatic hematuria or proteinuria, and to obtain the ratios of the peak velocity of the left renal vein between the aortomesenteric portion and the hilar portion in children with asymptomatic hematuna or protelnuria in which nutcracker syndrome was excluded and to observe whether the ratios are affected by sex, age or urinalysis findings. Methods : Using Doppler ultrasonography, we measured the flow velocity and obtained the peak velocity ratios of the left renal vein at the aortornesenteric portion and at the hilar Portion of the left kidney in children with asymptomatic hematuria or proteinuria who visited the Division of Pediatric Nephrology, Severance Hospital from May 2001 to March 2004. Results : Of 304 children with asymptomatic hematuna or proteinuria, 107 children(35.2%) were diagnosed with nutcracker syndrome. For 197 children with asymptomatic hematuria or proteinuria excluding nutcracker syndrome, the mean ratio of the peak velocity was 2.54 $\pm$0.73, which was not affected by sex, age or urinalysis findings. Conclusion : Nutcracker syndrome was the major cause of asymptomatic hematuria or proteinurla In children, comprising 35% of all cases. Doppler ultrasonography was helpful in the screening of nutcracker syndrome and prevention of its complications. For children with asymptomatic hematuria or proteinuria excluding nutcracker syndrome, the peak velocity ratio of the left renal vein did not differ from that of normal children and was not affected by sex, age or urinalysis findings. (J Korean Soc Pediatr Nephrol 2005;9:15-20)

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.21-30
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• 2005

Purpose : Metabolic bone diseases have been mai or problems in children with renal diseases and steroid treatment is the main precipitating factor reducing bone mineral density(BMD). This study was performed to assess the prevalence of osteoporosis and to evaluate the clinical factors associated with decreased BMD in children with renal diseases. Methods : Forty-four children with renal diseases who were diagnosed at the Pediatric no phrology division of Ajou University hospital since Oct. 1994 were included. Using a new quantitative ultrasound device, BMD and the prevalence of osteoporosis were evaluated. The clinical and serological data were analyzed in association with decreased BMD. Results : A total of 44 patients were evaluated. The age at initial diagnosis was 6.7$\pm$4.2 years. At the time of evaluation, the chronological and bone age was 9.3$\pm$4.2 years and 8.2 $\pm$ 4.6 years, respectively. The renal diseases included nephrotic syndrome 24(54.5%), Henoch Schonlein purpura nephritis 7(15.9%), IgA nephropathy 6(13.9%), reflux nephropathy(RN) 2 (4.5%), and other renal disease 5(%). The prevalence of osteoporosis was 11%. There was no difference in the clinical factors between the long-term and the short-term treated steroid groups. Conclusion : The prevalence of osteoporosis was 12% in 44 children with renal diseases No significant factor was found in association with decreased BMD and there was no relationship between osteoporosis and steroid usage duration or cumulative dose. A new quantitative ultrasound, which is relatively easy to perform, especially in children, is expected to be in common use and will enable clinicians to evaluate metabolic bone disorders with ease.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.31-37
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• 2005

Puruose : Thin glomerular basement membrane disease(TGBMD) is found in patients with family history of hematuria. TGBMD is autosomal dominant and is known to be one of the commonest causes of asymptomatic hematuria. This study was conducted to evaluate the histological and clinical features of patients with TGBMD. Methods : 150 cases diagnosed with TGBMD by renal biopsy while admitted in the department of pediatrics, Kyungpook National University Hospital between January 1999 and December 2003 comprised the study group. The following parameters were retrospectively anaIyzed age of onset, hematuria pattern, existence of proteinuria, process of diagnosis, laboratory findings, thickness and character of basement membrane and family history. Results : The mean age at the time of diagnosis was 7.9 years. The male to female ratio was 65:77. 94 patients or 66% visited the hospital with a chief complaint of persistent microscopic hematuria. Gross hematuria accounted for 13 cases or 9%. 78 cases(55%) were found to have hematuria for the first time from a routine school urinalysis screening. The renal biopsy showed the thickness of basement membrane to be 186$\pm$36 nm. Focal lamellation of the basement membrane was found in eight cases. In the family history, hematuria was shown in 10 cases on the Paternal side, 13 on The maternal side and none on both sides. In seven cases, hematuria was shown among siblings. No significant differences were found among the laboratory test results which were conducted at an average interval of fifteen months. Conclusion : TGBMD is one of the major causes of asymptomatic hematuria in children, which was diagnosed in increasing numbers since school urinary mass screening test started in 1998. In cases with familial progressive renal disease or focal duplication in the basement membrane Alport syndrome should be considered.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.38-45
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• 2005

Purpose : The purpose of this study was to analyze the therapeutic effect of plasmapheresis in various pediatric diseases. Methods : Therapeutic plasmapheresis was performed by COBE Spectra centrifugation. Nine cases were included in this study. The number an[;. method of plasmapheresis, together with the progress and prognosis of each case were retrospectively reviewed. Results : The patients' ages ranged from 26 mont]Is to 16 years of age, and the mean age was 9.9 years. There were S males and 4 females. The underlying diseases requiring plasmapheresis included 2 cases of hemolytic uremic svndrome(HUS), 1 case of lupus nephritis, 2 cases of rapidly Progressive glomerulonephritis(RPGN), 1 case of focal segmental glomorulosclerosis(FSGS), 1 case of systemic vasculitis after pulmonary hemorrhage, 1 case of acute renal failure associated with pulmonary hemoIThage, and 1 case of acute rejection after renal transplantation. The average number of plasmapheresis performed was 6.2 times with a range of 3 to 13 times. The patients with HUS, lupus nephritis, ANCA positive systemic vasculitis induced by pulmonary hemorrhage and ARF-associated pulmonary hemorrhage showed a good response to therapeutic plasmapheresis, but the patients with RPGN, refractory FSGS, and acute rejection after renal transplantation were not responsive to treatment. The most common side effect was hypocalcemia which was rarely symptomatic. Vital signs were not compromised. Conclusion : Although it is presumptuous to generalize the therapeutic effects of plasma pheresis in different diseases due to the small number of study subjects, this study shows that plasmapheresis may be an effective therapeutic modality in various pediatrics diseases and should be considered as a therapeutic option.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.62-70
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• 2010

Purpose : The voiding cystourethrogram (VCUG) is the investigation of choice in detecting the vesicoureteral reflux in urinary tract infections in children. As it is a potentially distressing and invasive test, most of the parents are so concerned about the child's stress. In this study, we compared the difference of the state of anxiety of parents before and after the VCUG. Methods : We divided 68 parents whose children underwent VCUG into 2 groups; who have given an explanation about VCUG in detail using pictures (group 1) Vs. who have given an oral explanation only (group 2). All the parents submitted the same questionnaire 2 times before and after the VCUG, which consisted of State-Trait-Anxiety-Inventory X-I (STAI-X-I) and visual analog scale (VAS) on the perception of worry, anxiety, confusion and pain. Results : Before VCUG, the perception of pain was higher in group 1 (P<0.05). After VCUG, the anxiety and confusion were significantly higher in group 2 than group 1 (P<0.05). In group 1, STAI-X-I scores, the perception of worry and anxiety were significantly decreased after the VCUG (P<0.05). In group 2, the confusion and pain were increased after VCUG (P<0.05). Conclusion : It showed that doctor's explanation on the procedure in advance may raise the perception of pain and the possibility of refusal by parents. But the STAI-X-I, worry, anxiety after VCUG were significantly decreased in group 1, while the confusion and pain were increased in group 2. Therefore we suggest that prior and sufficient explanation about invasive procedure like VCUG can be helpful in ameliorating the anxiety of the parents.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.42-50
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• 2010

Purpose : The clinical characteristics and associated anomalies in children with solitary kidney (SK) were analyzed retrospectively. Methods : Total 38 children diagnosed to have SK at our hospital between December 1989 and December 2009 were recruited, and the clinical records including imaging studies were retrospectively reviewed. SK was defined as unilateral renal agenesis by imaging studies only, and patients with regression of unilateral dysplastic kidney were excluded. Results : Among total 38 patients, 12 were male. The median age at the diagnosis of SK was 6.5 months (at birth-13 years). SK was detected by prenatal ultrasonography in 14 patients and during work-up for renal or urinary tract diseases in 13 (including urinary tract infection in 7). In 10 patients, SK was detected incidentally. Anomalies in the SK were noted in 17 patients including vesicoureteral reflux in 11. Other anomalies in the genitourinary tract were present in 16 patients, and multi-organ-involving syndromes or chromosomal anomalies were detected in 9. The mean duration of follow-up was 9 years (9 months-20 years). Two patients developed chronic renal failure during follow-up, and the median serum creatinine concentration of the remaining 36 at their last follow-up was 0.6 mg/dL. Conclusion : SK may be isolated and clinically asymptomatic; it is frequently accompanied by other anomalies in genitourinary tract and other organs, some of which can induce progressive renal dysfunction. Early recognition of associated anomalies with SK and regular follow-up is recommended to reduce long-term risk.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.22-31
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• 2010

Purpose : This study was performed to evaluate the relationship between glomerular basement membrane (GBM) alterations to epithelial cell (EpC) structure and renal function in Alport Syndrome (AS) patients. Methods : Fifteen patients diagnosed with AS (4-26yrs) were examined. The GBM in AS was categorized as : C1) normal, C2) minor alterations (widening of lamina rara interna or externa without lamina densa change), C3) nonspecific splitting of lamina densa, C4) basket-weaving pattern of lamina densa splitting. The length of each GBM portion along the epithelial side was measured on the systematically obtained electron microscopic photographs. Furthermore to obtain an objective assessment of the degree of glomerular EpC foot process change, the number of slit pores along $10\;{\mu}m$ of peripheral GBM in each category was obtained. Results : The percentage of normal GBM portion (C1) correlated inversely with daily protein excretion (g/day/$m^2$, P<0.05) and sum of the percentage of abnormal GBM portion (C2+C3+C4) had direct correlation with daily protein excretion (g/day/$m^2$, P<0.05). There were no significant relationships between the percentages of other categories of GBM alterations and creatinine clearance or protein excretion. There were no significant relationships between of creatinine clearance in relation to normal GBM(C1) portion as well as that in relation to sum of the percentage of abnormal GBM portion (C2+C3+C4). GBM abnormality did not correlate with age at biopsy. Conclusion : The extent of GBM structural abnormality is related to proteinuria in AS but the epithelial response is uniform even though the GBM ultrastructural lesions are not.