• Childhood Kidney Diseases
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• v.3 no.2
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• pp.203-208
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• 1999

Purpose: The information on the change of the relative renal function after operation is essential to set the initial treatment plan in unilateral hydronephrosis. So we analyzed the preoperative and postoperative relative renal function, and observed the functional changes by operation and reliability of the various factors in those changes. Materials and Methods: A retrospective trial was done of 81 patients, 35 ureteropelvic junction(UPJ) obstruction and 46 vesicoureteral reflux(VUR), among 393 patients undergoing operations at our institution from March. 1992 to February. 1997. The patients who had infravesical abnormalities, abnormal contralateral kidney and insufficient research data were excluded. We determined the relative renal functions using DTPA and MAG3 scan in UPJ obstruction and DMSA scan in VUR. The mean observation period after operation was 2.01(0.25-4) years. Results: A. UPJ Obstruction 1. The relative renal function was improved significantly after operation(P=0.0007). 2. The kidneys which have preoperative functions between 20% and 40% improved significantly in relative renal function comparing to the kidneys of the other functions(P=0.0046). B. VUR 1. The renal functions didn't improve significantly after operation(P>0.05). 2. There was no significant factor affecting the prognosis in renal functions. Conclusions: 1. The kidneys with UPJ Obstruction show the difference in functional improvement after operation according to the degree of the preoperative renal function, but more investigations about the exact value are needed. 2. In VUR there was no difference between preoperative and postoperative functions, the theoretical basis on objectives of the operation could be the symptoms or factors except the operation.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.48-56
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• 2000

Purpose: Alport SD., the most common herectitary rephriris, is a renal disease with rapid progression. Deafness, ocular abnormalities and a specific EM finding may be associated in addition to a family history. We have aralyged retrospectively. Methods: We observed 12 children with Alport syndrome who were diagnosed at Dept. of pediatrics in Kyunghee Univ., College of Medicine, from Apr. 1991 until Jun. 1999. We used four criteria for diagnosis: renal disease, family history, deafness or eye abnormalities, and a specific finding in electron microscopy Results: 2 of 12 patients had all features of the four diagnostic criteria. We could not trace an exact family history in 3 patients, and 6 patients did not exhibit deafness or eye abnormality. One could not have renal biopsy because offer chronic renal failure. Other three criteria were observed in her. The ratio of male to female observed was 1:2 respectively and the mean age of initial renal symptom was 5.6 years. 9 of 12 patients had a family history of renal disease. In the audiogram and ocular examination for 11 of 12 cases, sensorineural hearing loss was observed in 6 and ocular abnormality in 2 cases. In electron microscopic finding, irregular thickness of the capillary basement membranes with lamination of lamina densa and foot process obliteration was noted in 9 of 11 and thin basement membrane with splitting and foot process obliteration was noted in the other 2. The mean period of follow-up was 3 6/12 years. And one patient developed the chronic renal failure until now and had kidney transplantation. Conclusion: For the diagnosis of Alport syndrome, the following four diagnostic criteria are very important : renal disease, family history, deafness or eye abnormalities, and a specific finding on electron microscopy. We expect that more patients can be detected through the analysis of these characteristics.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.63-68
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• 2000

Purpose: MCDK is regarded as a common cause of abdominal masses in children. And the presentation of the MCDK is usually a unilateral flank mass in the a newborn. Bialteral disease results in either fetal demise or necessity fer renal replacement therapy at birth. This study is designed to assess the clinical features and natural history of the unilateral multicystic dysplastic kidney. Patients and Methods: From January 1987 to January 2000 data were obtained retrospectively on 57 patients (28 boys and 29 girls, age ranged 1day-11years) who had a diagnosis of multicystic dysplastic kidney. The diagnosis of multicystic dysplastic kidney was confirmed by a combination of ultrasonography and radionuclide scan. Voiding cystourethrogram study in 31 patients were done to determine the condition of the contalateral kidney. Restllts: $84\%$ of the patients were diagnosed before birth by antenatal ultrasonography Clinical manifestations of children with postnatal diagnoses were palpable abdominal mass($3.5\%$), abdominal distension($17\%$), and incidental($10.5\%$). The abnormalities in contralateral kidney were hydronephrosis($21\%$), compensatory hypertrophy($12\%$), simple cyst($2\%$), bifid pelvis($2\%$). Surgical management was performed in 20 patients($35\%$) due to recurrent infection, for diagnostic purpose to differentiate from malignancy and abdominal distention. Follow-up in the remaining 37 patients continued (mean 18 months) and results of sonogram findings were involution change in 23 patients($40\%$) and no interval changes in 13 patient($23\%$). Conclusions : The apparent tendency to regression of the dysplastic kidney and no difference in the number of complications justify a conservative management rather than operative intervention except in associated severe complications such as urinary tract infection or rupture of cysts.

• 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.3 no.2
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• pp.180-186
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• 1999

Purpose : It has been well known that urinary tract infection(UTI) in infants and children is frequently associated with vesicoureteral reflux(VUR). However, the publishied papers dealing with congenital anomalies associated with UTI emphasized the importance of VUR only. The aim of our study was to evaluate the type, incidence and spectrum of urologic anomalies associated with UTI. Methods : Medical records of clinical, bacteriologic and radiologic study were assessed retrospectively in 65 infants or children with documented UTI who were admitted to the Department of Pediatrics, Samsung Seoul Hospital from March 1996 to February 1998. Results : Spectrum of anomalies were associated with UTI as follows: VUR(n=23), both ectopic kidney(n=1), ureterovesical junction(UVJ) obstruction(n=1), multicystic dysplastic kidney(n=1), ureteropelvic junction(UPJ) obstruction with hydronephrosis(n=1), hutch diverticulum(n=1), UPJ stenosis(n=1), posterior urethral valve(n=1), urachal remnant(n=1) and bladder diverticula(n=1). Congenital urinary anomalies other than VUR were detected in 9 children among 65 patients with UTI(13.8%). 4 children among 9 congenital urinary anomalies other than VUR were combined with VUR. Sex distribution with congenital urinary anomalies other than VUR was more prevalent in male than female (7 males : 2 females). Age distribution at the time of UTI was less than 5 years in most patient (under 1 year in 1 patient, 1-2 year in 5 patients, 3-5 year in 1 patient, and above 5 year in 2 patients). And age distribution at the time of UTl associated with VUR was less than 5 years in most patient (under 1 year in 6 patients, 1-2 year in 8 patients, 3-5 year in 5 patients, and above 5 year in 4 patients), too Conclusion : Because congenital urinary anomalies other than VUR are seen in as high as 13.8% of patients, more careful evaluation of all possible congenital urinary anomalies as well as VUR is mandatory in pediatric patient with UTI.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.187-195
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• 1999

Purpose : VUR is state where urine regurge from bladder to ureter and kidney. It is shown in about 1/3 of urinary tract infection patients and it is classified as grade I to V. We compared results from RI VCUG(Radiisotope voiding cystourethrography) and X-ray VCUG which used in diagnosing VUR in children, to evaluate which is better in diagnosing VUR in children. Methods : 41 Patients(19 males, 21 females), who visited Pediatric department, Soonchunhyang university Hospital from peroid of 1991. January to 1998. July for recurrent urinary tract infection or abnormalities in ultrasonogams, were enrolled in the study. The age ranged from 9 months to 17 years and mean age was 5 1/2 years. Both RI VCUG and X-ray VCUG were done and follow-up test of urine culture, renal ultrasonogram and RI VCUG were done every month, every 3 month and every 6 month, respectively to observe the disappearance of VUR and evaluated the prognosis. Results : 24 patients had taken RI VCUG and 17(70.1%) patients showed positive result. 22 patients had taken X-ray VCUG and 9(40.1%) patients showed findings of VUR. 17 patients had taken both tests and 14 patients showed positive result in RI VCUG and 6 of these patients also showed reflux in X-ray VCUG. 3 patients who showed negative in RI VCUG, showed negative also in X-ray VCUG. For prognosis, resolution and scar formation was shown in 8 patients each. Persistent VUR was shown in 6 patients and 2 of these patients VUR was corrected by operation, 1 patient showed decreased renal function, and 1 patient was not follwed up. 8 of 9 patients who showed findings of VUR on DMSA scan formed a scar and 8 patients who showed no findings of VUR didn't form a scar. Urine culture was positive in 17 of 19 patients with VUR. Positive rate in urine culture was higher than that of patients with no VUR who showed positivity in 15 of 21 patients for urine culture. E. coli was most common organism and the period free of UTI was 14 months in VUR patients and it was shorter compared to patients without VUR which was 26 months. Conclusion : In diagnosing VUR in children, the positive rate was higher in RI VCUG than X-ray VCUG. Therefore, in early diagnosis when VUR is suspicious but not shown in X-ray VCUG, RI VCUG should be done and it will help to make accurate diagnosis.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.196-202
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• 1999

Purpose : When the mature kidney fails to reach its norml location in the renal fossa, the condition is known as ectopic kidney. Presenting symtoms can be various and it generally depend on the associated anomaly. Beside urologic anomalies such as hydronephrosis and vesicoureteral reflux, various anomalous vascular net work, skeletal anomaly or genital anomaly can be observed in this condition. Methods : Sixteen children with ectopic kidney was studied retrospectively to analyse initial presentation, accompanied anomaly and prognosis. Results : 56% of the children were accompanied with other urologic anomalies such as true incontinence and vesicoureteral reflux that required surgical treatment. 31% of children were either diagnosed incidentally during evaluation of other non-urologic disease or during follow-up evaluation of abnormal antenatal renal sonogram. Conclusion : Ectopic kidney can be often misdiagnosed as tumorous condition or as a surgical condition depend on the abnormal location of the kidney. Careful evaluation using abdominal sonogram, DMSA, VCUG and abdominal CT scan should be performed in order to search for associated anomalous condition and for proper management.