• Clinical and Experimental Pediatrics
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• v.45 no.7
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• pp.896-901
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• 2002

Purpose : The aim of this study was to determine the epidemiology of Kawasaki disease in the Kyung Nam area and to evaluate whether the results of this epidemiological study could support infectious etiology. Methods : We sent a questionnaire to three training hospitals in the Kyung Nam area and retrospectively reviewed their medical records of Kawasaki disease from Jun. 1995 to Dec. 1999. Results : The total number of patients was 717 cases, with little differences of annual prevalence during the five years. In all cases, the monthly prevalence of Kawasaki disease was high in Apr. and Jul. At the eastern of Kyung Nam, the monthly prevalence was high in Apr. and Jul. in 1995 and 1996, Jul. in 1997, Apr. in 1998 and Apr. and Jul. in 1999. In the central area of Kyung Nam, the monthly prevalence was high in Apr. in 1995 and 1996, Apr. and Jul. in 1997 and Jul. in 1998 and 1999. In the western Kyung Nam, the monthly prevalence was high in Nov. in 1995, Aug. in 1996, Oct. in 1997, Dec. in 1998 and Nov. in 1999. Conclusion : In the eastern and central areas of Kyung Nam, the monthly prevalence of Kawasaki disease was similarly high in Apr. and Jul. However, in the western district, the prevalence was high in late fall and winter. We could not prove the hypothesis that Kawasaki disease occurred with the spread of single infectious agent, but the a nnually similar prevalence in eastern and central Kyung Nam supported the infection theory for the etiology of the disease.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.34-40
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• 1998

Present study was conducted to determine the frequency, clinical characteristics and long-term outcome of children with idiopathic hypercalciuria. Study patients consisted of 150 children with isolated hematuria (recurrent gross or persistent microscopic), and hypercalciuria was defined as urinary calcium excretion over 4 mg/kg/day. During follow-up period up to $6{\sim}8$ years, serial check-up of renal sonogram for stone formation and Dipstick examination for hematuria were done. Forty-four (29%) out of 150 cases were diagnosed as idiopathic hypercalciuria, and in hypercalciuric children compared to normocalciuric children boys were more common than girls (9:35) and gross hematuria was more common than microscopic hematuria (37:7) (P<0.05). Oral calcium loading test showed renal type in 29 cases, absorptive type in 8 cases and in 7 cases type could not be definable. Among 3 types no differences could be found in 24 hour urinary calcium excretion and in clinical or laboratory data. Urolithiasis developed in 4 out of 44 cases (2 at the time of initial diagnosis and 2 within $1{\sim}2$ years of follow-up periods) and these children showed lower chronologic age ($3.7{\pm}2.7\;vs\;7.2{\pm}2.9\;yr$) and more girl than boys (3:1 vs 6:34) (P<0.05) compared to the rest of the hypercalciuric children. Follow-up urinalysis showed disappearance of hematuria in 56, 50, 66 and 75% of children at $1{\sim}2,\;2{\sim}4,\;4{\sim}6$ and $6{\sim}8$ years after initial diagnosis respectively. In conclusion, present study demonstrates that idiopathic hypercalciuria is a major cause of isolated hematuria in children so that in these children 24 hour urinary calcium excretion test seems to be an essential test to be performed. And serial renal sonography should be done to detect development of nephrolithiasis. However, clinical significance of dividing hypercalciuric children into two pathophysiologically distinct subtypes by oral calcium loading test seems to be in doubt and further study is needed to solve this problem.

• 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.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.3 no.2
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• pp.145-152
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• 1999

Purpose: The prevalence of childhood renal diseases including the nephrotic syndrome in Korea has not been well established. In priority to this point, we analyzed ninety-six nephrotic children of the annual incidence, the ratio of them to all inpatients and estimated incidence of childhood nephrotic syndrome under 15 years of age in Taejon, Korea. Also we classified them by clinical and histopathological findings. Methods: we analyzed the admission and outpatient records retrospectively between June 1986 and December 1998. For the estimation of incidence, we were assisted with three other general hospitals in Taejon Results: The mean annual number and the ratio to inpatients were $7.4{\pm}2.1,\;0.3{\pm}0.11%$ respectively. The latter showed a tendency to decrease during the recent three years. The estimated annual incidences of childhood nephrotic syndrome in Taejon were 5.6 in 1988, 5.5 in 1993 and 4.8 in 1998 per 100,000 for children aged up to 15 years. The mean age of our patients was $6.8{\pm}3.5$ tears, 1-7 years of age was 55 cases(57.3%), and male to female ratio was 3.6:1. By the clinicopathologic classification, 89 cases(92.7%) were classified as primary nephrotic syndrome, and 7 cases(7.3%) as secondary nephrotic syndrome. Among the primary nephrotic syndrome, there was 79.8% of minimal change nephrotic syndrome, focal segmental glomerulosclerosis 11.2%, mesangial proliferation 4.5%, membranoproliferative glomerulonephritis 3.4%, and membranous nephropathy 1.1%. $Henoch-Sch\"{o}nlein$ nephritis was the most frequent of 3 cases in the secondary nephrotic syndrome. Respones to steroid therapy of 71 cases minimal change were classified as non-relapse 22.5%, non-frequent relapse 49.3%, frequent relapse 18.3% and steroid-dependence 9.9%. Conclusion: The estimated incidence of childhood nephrotic syndrome was about 5 per 100,000 for children aged up to 15 years and it showed little changes during 10 years in Taejon, Korea. Our results of clinicopathologic study was little difference from the results reported in other literatures.

• Childhood Kidney Diseases
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• v.13 no.1
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• pp.56-62
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• 2009

Purpose : The aim of this study was to evaluate the clinical usefulness of measurement of ${\beta}_2$ microglobulin (${\beta}_2$ MG), N-acetyl-$\beta$-D-glucosaminidase (NAG) of spot urine samples as indices of renal tubular damage and microalbumin of spot urine samples as a parameter of glomerular damage in children with vesicoureteral reflux (VUR) or renal defects. Methods : We studied 91 children with previous UTI. The children were classified as 62 children without VUR and renal defects (group I), 10 children with VUR, without renal defects (group II), and 19 children with VUR and renal defects (group III). Patients having VUR were separated according to the degree of VUR (mild VUR: VUR grade I-III, severe VUR: VUR grade IV-V). Urinary excretion of ${\beta}_$ microglobulin (${\beta}_2$ MG), microalbumin, N-acetyl-$\beta$-D-glucosaminidase (NAG), creatinine were measured in samples of morning urine specimens. Children with VUR or renal defects detected by voiding cystourethrography (VCUG) and DMSA renal scan were investigated. Results : Microalbumin/Cr ratio of spot urine was significantly increased in group III compared group I ($42.3{\pm}27.2\;mg/gCr$ vs $25.2{\pm}10.9\;mg/gCr$, P<0.05). NAG/Cr ratio of spot urine was significantly increased in group II compared group I ($3.70{\pm}23.4\;mg/gCr$ vs $18.7{\pm}12.7\;mg/gCr$, P<0.05). There was no statistically significant difference of ${\beta}_2$ MG/Cr ratio among three groups. Conclusion : Urinary microalbumin excretion of morning urine sample may be a simple and reliable clinical indicators for early identification of renal damage in children with VUR and renal defects. Urinary microalbumin excretion may be useful marker to predict the the severity of VUR.

• Clinical and Experimental Pediatrics
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• v.49 no.12
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• pp.1275-1281
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• 2006

Purpose : Cerebral edema in meningitis is a potentially complication. Hypertonic saline of various concentrations are frequently used to treat cerebral edemas in meningitis. We analyzed the safety and efficacy of osmotic therapy in cerebral edema by comparison of various hypertonic saline concentrations and mannitol. Methods : The medical records of 42 patients, who were followed up in the Department of Pediatrics, Busan Medical Center, from Jan. 2002 to Oct. 2005, were analyzed retrospectively. We measured intracranial pressure, mean flow velocity, and various laboratory parameters. Results : In cerebral edema developed in meningitis, intracranial pressure and symptoms were improved in treatment of hypertonic saline and mannitol. Serial bolus infusion of 3 percent hypertonic saline resulted in the best outcome. There was not a statistically considerable difference on the mean values of the intracranial pressure gap. On transcranial doppler, mean flow velocity was increased and pulsatilty index was decreased. Laboratory findings (osmolarity, Na, Cl, pH, lactic acid, Ca) were diffenent during the treatment period as opposed to K, Hb, bicarbonate, base excess. There was not a specific form of hypertonic saline used in meningitis treatment with cerebral edema. Conclusion : The therapy for cerebral edema in meningitis remains largely empirical. Serial bolus infusion of 3 percent hypertonic saline is better than other hypertonic salines. Various concentrations and different infusion methods of hypertonic saline statistically does not influence the result of treatment. More research aimed at improving cerebral edema treatment is needed to identify new, effective forms of treatment.

• Clinical and Experimental Pediatrics
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• v.49 no.6
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• pp.653-658
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• 2006

Purpose : Because voiding cystourethrography(VCUG) is an invasive method, we studied whether VCUG could be postponed through evaluation of alternative non-invasive tests including renal ultrasonography and $^{99m}Tc$-DMSA renal scan. Methods : We reviewed the medical records of 175 patients initially diagnosed with febrile urinary tract infection during the one year period of 1999, and compared 3-tests : renal ultrasongraphy, $^{99m}Tc$-DMSA renal scan, and VCUG. Results : Renal ultrasonography didn't contribute to the prognostication of pyelonephritis(photopenic areas) or vesicoureteral reflux(VUR). Presentation of photopenic areas in $^{99m}Tc$-DMSA renal scan was related to VUR. If both findings of renal ultrasonography and $^{99m}Tc$-DMSA renal scans were normal, this condition was closely related to normal results in VCUG. And if both examinations were abnormal, the condition was closely related to VUR. But this state could not always guarantee the normal result from VCUG because of low sensitivity in finding VUR. Conclusion : In cases in which acute phyelonephritis is demonstrated by $^{99m}Tc$-DMSA renal scan, VCUG is required. In addition to this, if the conditions of hydronephrosis, vesicoureteral dilatation, increases of renal volume, and changes of echogenesity are shown by renal ultrasonography, VCUG should be performed. If a patient has difficulty undergoing VCUG, temporary postponement of VCUG can be taken into consideration, but only in cases where both examinations of renal ultrasonography and $^{99m}Tc$-DMSA renal scan are normal. Nevertheless, close observation is be advised even in this case.

• Clinical and Experimental Pediatrics
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• v.48 no.11
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• pp.1244-1251
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• 2005

Purpose : $Henoch-Sch{\ddot{o}}nlein$ purpura(HSP) is the most common and benign systemic vasculitis in children. Few reports have focused on worse outcomes of HSP in adults. The age of onset is suggested as a main risk factor. We assessed the characteristics of adolescent-onset HSP. Methods : We retrospectively analyzed 205 cases presented from Aug. 1993 to Oct. 2003. Patients were classified as children(<10 years of age), adolescents(10-20 years of age), and adults(>20 years of age). Results : The mean age was $5.7{\pm}1.8years$ in 149 children, $13.5{\pm}2.4years$ in 38 adolescents, and $44.9{\pm}14.5years$ in 18 adults. The male to female ratio was 1.2 : 1 in children and adolescents, and 2 : 1 in adults. Previous upper respiratory infections were found in 53.4 percent of children, 32.4 percent of adolescents, and 33.3 percent of adults. Positivity of stool occult blood was more frequent in adults(50.5 percent) than in children(23.0 percent)(P<0.05). Renal involvement was found in 46 cases (30.9 percent) of children, 23 cases(60.5 percent) of adolescents, and 15 cases(83.3 percent) of adults. Recurrences occurred in 23 cases(15.4 percent) of children, nine cases(23.7 percent) of adolescents, and three cases(16.7 percent) of adults. Among the cases with renal involvement, 97.8 percent of children and 87.0 percent of adolescents improved to normal or asymptomatic urinary abnormalities. 60.0 percent of adults persisted with severe nephropathy and 13.3 percent progressed to renal insufficiency. Conclusion : Although the outcome of adolescent HSP was as good as children, the clinical manifestations were similar to those of adults. Adolescents had the highest rate of recurrences. Thus long term observations may be needed in adolescent onset HSP.

• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1241-1246
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• 2007

Purpose : Recently, there has been several studies to clarify the pathogenesis of Kawasaki disease (KD) and the relations of VEGF and endostatin that act on vascular endothelial cells to the coronary artery complications. In this report, we measured serum levels of VEGF and endostatin in acute and subacute phases of KD to assess the change of these levels and the relations to the development of coronary artery lesions (CAL). Methods : Twenty six patients were diagnosed and treated for KD between January, 2001 and July, 2005 at Kangnam St. Mary's Hospital, the Catholic University of Korea. They were divided into those with and without CAL. Serum levels of VEGF and endostatin were measured during acute and subacute phases and compared to those measured in healthy and disease control groups. Results : Serum levels of VEGF were increased in KD but no differences were noted in KD with and without CAL. Serum levels of endostatin were decreased in the acute phase of KD, however they were recovered in the subacute phase of KD, regardless of CAL. The VEGF/endostatin ratio was increased in KD. KD without CAL showed a relative decrease in this ratio during the subacute phase. Significant positive correlations were found between serum VEGF and WBC count, VEGF and ESR, VEGF/endostatin ratio and ESR in the acute phase of KD. Conclusion : Analysis of factors influencing the vascular endothelium such as VEGF and endostatin will help to clarify the etiology of KD and the pathogenesis of CAL.