• Childhood Kidney Diseases
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• v.3 no.2
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• pp.117-122
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• 1999

Purpose : We studied the correlation between urinalysis and radiologic findings in infants and children with urinary tract infection. Method : Urine Dipstick test and unstained urine microscopic examination were carried out in 56 infants and children who were hospitallized with the diagnosis of urinary infection by pocitive urine culture at Seoul Adventist from September 1996 through August 1998. Urine was collected by midsream, catheter, urine bag after cleansing or bladder puncture. Renal sonography and $^{99m}Tc-DMSA$ renal scan or voiding cystoureterography were studied. Results : 1)In dipstick analysis, leukocyte esterase(LE) were positive in 30 cases of 56 patients(54%) and Nitrite were positive in 20 cases of 56 patients(36%) and LE or Nitrite were positive in 38 cases of 56 children with UTI(68%). 2)In microscopic analysis, WBC were positive in 38 cases of patients(68%) and bacteria were positive in 23 cases of 56 patients(41%) and WBC or bacteria were positive in 41 cases of 56 children with UTI(71%). 3)Ten cases of 56 UTI patients(18%) showed negative finding in Dipstick and microscopic analysis. 4)There was no significant difference in positive rate of dipstick(71% vs 66%) and microscopia analysis(83% vs 66%) between two group with or without renal scar on $^{99m}Tc-DMSA$ scan (P=0.117). 5)There was no significnt difference in positive rate of dipstick(100% vs 91%) and microscopic analysis(100% vs 100%) between two groups with or without vesicoureteral reflux on VCUG. Conclusion : There was no specific relationships between the abnormal urinalysis and the abnormal findings on $^{99m}Tc-DMSA$ scan or VCUG.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.153-160
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• 1999

Purpose : Present study was undertaken to find out significance of clinical presentation, initial laboratory data and renal biopsy findings on subsequent clinical course of IgA nephropathy in children. Methods : Clinical and laboratory data were analysed retrospectively from 60 children who have been admitted to the Pediatric Department of Kyungpook National University Hospital for the past 11 years and diagnosed as IgA nephropathy. Renal biopsy findings were graded according to the pathologic subclass proposed by Haas. Results : Pathologic grading according to Haas subclassification showed 10 cases in subclass I, 36 in II, 12 in IV and 2 in V and none in subclass II. Sex distribution showed male predominance (male to female ratio = 3 : 1) and mean age at onset of disease was $10.4{\pm}2.8$ years. Episodes of gross hematuria was seen in 71.7% and IgA level increased in 28.3% of children and these were not associated with pathologic grading nor clinical outcomes. With increasing subclass grading, serum protein and albumin decreased and 24 hours urinary protein excretion increased. Normalization of urinalysis (disappearance of hematuria) was seen in 14% at 1-2 years and 37.1% at 3-4 years of follow up period. In 3 cases, renal function deteriorated progressively and they belonged one each to the Haas subclass III, IV and V. Conclusion : In children with IgA nephropathy, progression to chronic renal failure appears to be quite high and pathologic grading according to Haas' subclassification seems to predict patient's outcome faily well. However, firm conclusion cannot be drawn from present study due to the small numbers of patients and short follow-up period. Therefore further multicenter study involving larger numbers of patients and longer periods of follow-up over 10 years was to be undertaken.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.209-216
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• 1999

Purpose : Nephrogenic diabetes insipidus (NDI) is a rare X-linked disorder associated with renal tubule resistance to arginine vasopressin (AVP). The hypothesis that the defect underlying NDI might be a dysfunctional renal AVPR2 has recently been proven by the identification of mutations in the AVPR2 gene in NDT patients. To investigate the association of mutations in th AVPR2 gene with NDI, we analyzed the AVPR2 gene located on the X chromosome. Methods : We have analyzed the AVPR2 gene in a kindred with X-linked NDI. The proband and proband's mother were analyzed by polymerase chain reaction-single strand conformational polymorphism(PCR-SSCP) and DNA sequencing of the AVPR2 gene. We also have used restriction enzyme analysis of genomic PCR product to evaluate the AVPR2 gene. Results : C to T transition at codon 202, predictive of an exchange of tryptophan 202 by cysteine(R202C) in the third extracellular domain was identified. This mutation causes a loss of Hae III site within the gene. Conclusion : We found a R202C missense mutation in the AVPR2 gene causing X-linked NDI, and now direct mutational analysis is available for carrier screening and early diagnosis.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.84-91
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• 2000

Purpose: To improve the recovery of growth deficit after renal transplantation in children, we analysed the factors affecting height growth after renal transplantation. Methods: We reviewed medical records of fifty-six children in whom height data were available for three years after transplantation. All height data were converted into Z-scores. We analyzed the effects of sex, age at transplantation, cumulative mean steroid dose for 3 years, serum creatinine levels, height at transplantation, donor source and history of prior dialysis on patients' z-scores and delta Zs. Results: The Z-scores at transplantation were lower in patients of younger age (P=0.007). When baseline Z-scores were lower, the delta Zs were higher (P<0.01), but the Z-scores after transplantation were still lower (P<0.001). According to the analysis of the partial correlation coefficients, Z-scores and delta Zs at 1 year after transplantation were higher in groups of younger age and of lower steroid dosages (P<0.05). The delta Zs at 6 month and 1 year after transplantation were lower in the group with abnormally higher serum creatinine (P<0.05). There was no difference in Z-scores between groups of different genders, donor sources, and histories of previous dialysis. Conclusion: The children of younger age, on lower steroid dosage, with less growth retardation at transplantation, and with normal graft function had better height growth recoveries after renal transplantation.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.6-10
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• 2000

Purpose: To evaluate whether the urinary creatinine concentration is a reliable reference value to standardize urinary solute excretion in a spot urine sample during the first week of life. Methods: Spontaneously voided urine specimens were obtained in 49 healthy full term neonates, and in 33 healthy older children with the median ages of $5.7{\pm}4.3$ years, two urine samples were available with an interval of 2 to 3 days. Urine creatinine concentration was determined by the Jaffe test(CoBAS, Integra, Roche, Swiss). Uurine osmolality was determined by the freezing point depression test(Multi-osmette, Precision, USA). Results: Mean urinary creatinine and osmolality values of the first urine samples were not significantly different with the second urine samples in each group. Mean urinary creatinine and osmolality values in neonates were significantly different from the older children of the each urine sample(P<0.01). In neonates, the mean of the urinary oreatinine/osmolality ratios was higher than that of the older children(P<0.01). The urinary creatinine and the creatinine/osmolality values of the first urine samples were closely correlated with those of the second samples in both two groups(P<0.001). Conclusion: The urinary creatinine concentration during the first day of life is relatively stable, even when corrected for urinary osmolality The urinary creatinine and the urinary creatinine/osmolality ratio, therefore, can be used to standardize the urinary excretion of solutes in the neonate.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.69-76
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• 2000

Purpose: The Pathogenesis of primary noctllrnal enuresis(PNE) is still controversial. Genetic factor and maturational delay of micturition reflex including arousal disorder, lack of nocturnal Arginine Vasopressin(AVP) release and functional bladder capacity have been suggested. We analyzed the risk factors of PNE. Methods: Fifty five children with PNE (20 enuretics diagnosed at school physical examination of the first grade students at Mok-Dong Elementary School and 35 enuretics (Age 6-7 year) diagnosed at Ewha Womans University Mok Dong Hospital) and 221 control students without PNE at school physical examination were included. Genetic, stress and developmental factors, arousability, water intake, urine volume, maximun voiding volume and daytime voiding dysfunction were compared. Results: 1) There was no significant difference between PNE and control group in sex ratio, birth order, percentage of working mothers, parental and child personality, age to start walking, school record and duration of sleep. 2) Family history in the PNE group was significantly higher than control group ($20.0\%\;vs\;2.7\%$)(P<0.05). 3) The difficulty in arousal in the PNE group was significantly more common than the control group ($70.9\%\;vs\;54.3\%$)(P<0.05). 4) Nocturnal water intake in the PNE group was significantly greater than the control group ($330{\pm}158.2\;mL\;vs\;235{\pm}129.5\;mL$). Nocturnal urine volume in the PNE group was significantly greater than the control group ( $390{\pm}61.5\;mL\;vs\;140{\pm}43.2\;mL$)(P<0.05). Daily water intake and daily urine volume did not significantly differ between the two groups. 5) Maximum urine volume per void in the PNE group was significantly lower than the control group ($107{\pm}35.9\;mL\;vs\;236{\pm}41.3\;mL$). Daytime voiding dysfunction in the PNE group was significantly more common than the control group ($80.0\%\;vs\;57.9\%$). The voiding frequency in the PNE group was significantly greater than the control group ($7.0{\pm}3.6\;vs\;5.4{\pm}1.6$)(P<0.05). Conclusion: In addition to genetic factors and maturational delay of micturition reflex (difficulty in arousal, nocturnal polyuria and decreased functional bladder capacity) nocturnal polydypsia was found to be the important risk factors fur PNE. So nocturnal fluid restriction should be encouraged as the first-line management of PNE.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.40-47
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• 2000

Purpose: Renal involvement is the most important prognostic factor of HSP. Therefore, the pathogenesis and prognostic factors in renal involvement have been studied by many researchers. The aim of this study was to evaluate the clinical risk factors and the role of TNF-$\alpha$ in renal involvement of HS purpura. Methods: The subjects of this study were 12 patients of HS purpura, 7 patients of HS nephritis, and 5 age-matched controls. We have analysed the rist factors for renal involvement in clinical symptoms and collected the sera and urines of all subjects in acute and convalescent stage. The concentration of TNF-$\alpha$ in the collected sera and urines were measured by sandwich ELISA and compared with that of age-matched controls. Results: Statistical analysis showed that persistent purpura increased the risk of developing renal involvement (P=0.0018). and serum TNF-$\alpha$ levels in the acute stage of patients with renal involvement($11.45{\pm}7.01$ pg/ml) were significantly higher than those of without renal involvement($6.32{\pm}1.31$pg/m1) and of age-matched controls($5.99{\pm}1.34$pg/m1)(P=0.012, 0.027, respectively). However, urine TNF-$\alpha$ levels have no correlation with renal involvement. On investigation of serum TNF-$\alpha$ levels in acute stage of HS purpura, persistent purpura had a significantly higher increase(P=0.038). Conclusion: Serum concentration of TNF-$\alpha$ is a risk factor and has a predictable value along with clinical risk factors, such as, persistent purpura for renal involvement in HS purpura. Also, the effectiveness of the specific treatment fur antagonizing TNF-$\alpha$ in HS nephritis may need further study.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.129-137
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• 2004

Purpose : Nocternal enuresis is a common disorder. Tricyclic antidepressant and desmopressin have been accepted pharmacological treatment for this disorder We conducted a cooperative study to investigate the efficacy and adverse reactions of imipramine, desmopressin and combination treatment in children with primary monosymptomatic nocturnal enuresis(PMNE). Methods: Data from a large multicenter study were analysed. In the period of 8 months in 2002, the study comprised of 168 children(78 boys and 90 girls, 5 to 15 years old) with PMNE for imipramine, desmopressin or combination treatment. Before treatment a history, physical examination and laboratory tests were performed and the children were observed for 2 weeks. Response rate, adverse reactions and enuresis episodes after stopping drug administration were evaluated after 12-weeks of imipramine, desmopressin or combination of both. Results: After 4 weeks, the frequency of bed wetting in all treated patients decreased during treatment significantly Even though a 30-50%, reduction in the number of wet nights were 68.6%, 74.4% and 86.1% during 12 weeks treatment by imipramine, desmopressin and both of them respectively, there was no significant difference between them. The most common adverse reaction was decreased appetite from imipramine administration. But no serious drug-related adverse events were reported. Conclusion: Efficacy of the combination therapy of imipramine and desmopressin in PMNE appears not to be better than either drug alone. It is necessary to pay attention on account of adverse reactions during imipramine treatment even though imipramine and desmopressin were generally well tolerated.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.195-204
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• 2004

Purpose: Hypophosphatemic rickets is a hereditary disease, characterized by hypophosphatemia due to renal phosphate wasting, impaired renal production of 1,25-dihydroxyvitamin $D_3$, rachitic bone deformities and impaired growth. The purpose of this study is to provide clinical profiles of patients with hypophosphatemic rickets in our hospital. Methods: Between July 1983 and February 2004, 56 patients were diagnosed as having hypophosphatemic rickets. The medical records of these patients were reviewed retrospectively. Clinical manifestations, family histories, laboratory data, treatment outcomes were described. Results: Fifty six patients were enrolled in this study. The average age at symptom onset and diagnosis were 20 months and 5 years respectively. Fourteen patients had family histories. The main clinical manifestations were bow legs and short stature. There was a significant negative correlation between the ages and the height z-scores at the time of diagnosis(r=-0.47, P=0.005). Initial laboratory data showed normocalcemia, hypophosphatemia, elevated serum alkaline phosphatase, decreased tubular reabsorption of phosphate and a normal range of 1,25-dihydroxyvitamin $D_3$ Radiographic examinations of bone revealed fraying, widening and cupping of the metaphyseal ends. Treatment consisted of Joulie solution and vitamin D metabolites, and resulted in improved biochemical and radiographic findings. However, height z-scores remained essentially unchanged(P=0.224). Complications of treatment were frequently observed, including hyperparathyroidism, nephrocalcinosis, and hypercalciuria. Sixteen patients had corrective osteotomy and 4 of them underwent leg lengthening together. Conclusion: There was a gap of several years between the onset of symptoms and the diagnosis. Early treatment seems to be essential to growth. For the earlier treatment, the offsprings of affected parents should be followed up closely.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.149-158
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• 2004

Purpose : In order to evaluate the value of the renal expression of ICAM-1 as a marker of renal injury, we analyzed the relationship between abnormal tubular expression of ICAM-1 and histopathological features and clinical manifestations in children with IgA nephropathy (IgAN). Methods: The clinical data from 43 patients with IgAN were analyzed retrospectively and compared to the histopathologic subclassification proposed by Haas. ICAM-1 in tubular epithelium was assessed using the LSAB(Labeled streptavidine biotin) kit on the renal biopsy specimens. Results: In 43 patients with primary IgAN, 28 males and 15 females aged $12.2{\pm}2.2$ years were studied. There were no differences of renal tubular expression of ICAM-1 between patients with gross hematuria and without gross hematuria. But renal tubular expression of ICAM-1 in patients with proteinuria was significantly higher than that of in patients without proteinuria($78.2{\pm}14.19%\;vs\;55.8{\pm}32.20%,\;P<0.05$). Renal tubular expression of ICAM-1 was also associated with the severity of histopathological degree using Haas classification method. In subclass I, renal tubular expression of ICAM-1 was significantly lower than those of other subclasses. A significant correlation was found between the tubular expression of ICAM-1 and the total amount of protein in 24 hour collected urine$(r_s=0.47236,\;p<0.05)$. But there were no significant correlations between the renal tubular expression of ICAM-1 and interstitial cellular infiltration, tubular atrophy, and interstitial fibrosis respectively(F=0.89, P>0.05; F=0.31, p>0.05; F=0.21, p>0.05). Conclusion: Renal tubular expression of ICAM-1 can be a useful marker of renal injury in children with IgAN. (J Korean Soc Pediatr Nephrol 2004;8:149-158)