• 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.

• Tuberculosis and Respiratory Diseases
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• v.47 no.1
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• pp.90-96
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• 1999

Background : Osteoporosis has been reported in patients with chronic obstructive pulmonary disease, but this association is not well established. This study was undertaken to determine whether the prevalence of osteoporosis was increased in patients with chronic obstructive pulmonary disease and we examined the relationship of corticosteroid administration with osteoporosis. Method: Subjects were 23 patients with chronic obstructive pulmonary disease and 20 control patients. We reviewed hospital records and measured bone mineral density using dual-energy x-ray absorptiometry(Lunar. USA). Results: Mean bone mineral density(BMD) of spine in COPD group was $0.683{\pm}0.154 g/cm^2$ and $0.971{\pm}0.212g/cm^2$ in controls(p<0.01). But there was no significant difference in femoral neck BMD. There were seventeen cases of osteoporosis and six cases of osteopenia in COPD group and three patients of osteoporosis and one case of osteopenia in controls. But, there was no significant correlation between disease duration of COPD and spinal T score(r=-0.395, p>0.05). Ten patients were received corticosteroid in COPD group. Spinal T score in steroid receiving patients were $-3.82{\pm}0.94(SD)$ and $-2.82{\pm}0.97(SD)$ in not having steroid patients(p<0.01). Cumulative dose of corticosteroid was associated with spinal T score(r=-0.424, p<0.05) and duration of corticosteroid administration also associated with spinal T score(r=-0.457. p<0.05). Spinal BMD of patients not having corticosteroid in COPD group(n=13) were significantly lower than that of controls($0.71{\pm}0.13 g/cm^2$ and $0.97{\pm}0.21 g/cm^2$, p<0.01). Conclusion : Prevalence of osteoporosis is increased in patients with chronic obstructive pulmonary disease. Especially patients who are receiving corticosteroid have high risk of osteoporosis or osteopenia and need for preventive management.

• Clinical and Experimental Pediatrics
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• v.49 no.6
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• pp.630-634
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• 2006

Purpose : This study was conducted to assess the current(2003-2005) prevalence of anti-HBs and immunologic memory for Hepatitis B vaccine in children from the central area of Korea. Methods : Subjects were chosen from children and adolescents who received tests for hepatitis B surface antigen(HBsAg) and anti-HBs at Dankook University Hospital from March 2003 to May 2005. Among these, antibodies to hepatitis B core antigen(IgG anti-HBc) were checked. A single booster vaccination was performed on children whose anti-HBs titers were under 10 mIU/mL. One month after booster vaccination we rechecked the anti-HBs titer. Results : A total of 3,277 subjects were tested for HBsAg/anti-HBs, and 1,913(58.4 percent) of them were positive for anti-HBs. Of these, 29 subjects(0.9 percent) were positive for HBsAg. Positive results for anti-HBs by age were 78.6 percent for 6-12 months of age, 62.7 percent for 1-3 years of age, 51.9 percent for 4-6 years of age, 49.5 percent for 7-12 years of age, 63.4 percent for 13-15 years of age and 72.2 percent for 16-18 years of age. The 80 subjects who were tested negative for HBsAg/anti-HBs received a single booster vaccine, 71 subjects were tested positive for antibodies. IgG anti-HBc titer was checked for 169 of the subjects, 5 subjects were positive. Conclusion : In our study, a significant anamnestic response was observed in 88.8 percent of children. This is believed to be a result of the relatively long immunologic memory effect of the hepatitis B vaccination in children from the central area of Korea.

• Clinical and Experimental Pediatrics
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• v.46 no.5
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• pp.440-446
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• 2003

Purpose : The genetic disturbance of galactosemia is expressed as a cellular deficiency of either galactose-1-phosphate uridyltransferase(GALT) or galactokinase(GALK) or UDP galactose 4-epimerase(GALE). To find-out the pattern of galactosemia in Korea, we retrospectively analyzed cases of galactosemia detected by neonatal screening program. Methods : We analyzed medical records of patients who visited Soonchunhyang University Hospital at age of 1 month after showing abnormalities in neonatal screening of galactosemia. For accurate diagnosis, galactose was measured by enzyme immunoassay(EIA) and fluorophotometer, also galactose-1-phosphate by fluorophotometer. Enzyme activities of GALK, GALT and GALE in RBC and galactose-1-phosphate were measured by radioisotope assay(RIA). Beutler test were done. Patients went on a lactose-free diet and follow-up tests for galactose, galactose-1-phosphate level and enzyme activity were performed. Results : 10 patients(male : 6, female : 4) were diagnosed as galactosemia. Two patients had GALK deficiency and two had GALT deficiency. Six were GALE deficient showing the largest number. In two patients with GALK deficiency, GALT and GALE activities were normal but GALK activities showed respectively reduced activity. For GALT deficiency, two patients had low GALT activity in RBC and showed genotype of Duarte 2/G(galactosemia) in DNA analysis. In one patient, GALT activity was normal. Three patients seemed to be heterozygote state of GALE deficiency according to GALE activity levels. Four patients showed GALK hyperactivity. Conclusion : GALE deficiency provided the highest number. After lactose-free diet, galactose and galactose-1-phosphate were normaly maintained. Neonatal screening on galactosemia is essential for preventing life-threatening symptoms and an accurate diagnosis is needed for finding out the type of galactosemia which is important for prognosis.

• Clinical and Experimental Pediatrics
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• v.52 no.8
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• pp.881-887
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• 2009

Purpose : The survival rate of very low birth weight infant (VLBWI) had increased as a result of advances in neonatal intensive care. We evaluated the changes in outcomes of VLBWI who admitted to the neonatal care unit of Hallym University Kangnam Sacred Heart Hospital. Methods : Retrospective review of 339 VLBWI who were born from 1st January 1997 to 31th December 2008 were performed. Outcomes including survival rate, birth weight (BW), gestational age (GA), morbidities, and mortality between period I (1997- 2003) and period II (2004-2008) were compared. Results : Overall incidence of VLBWI was 2.3% and it was significantly higher in period II(3.3%). Mean BW and GA were significantly decreased in period II (P<0.001, P=0.01). The survival rate increased from period I (59.1%) to period II (74.2%). BW-specific survival rate increased in 1,000-1,249 gm and GA-specific survival rate significantly increased in 27-28 weeks and 29-30 weeks. The incidences of respiratory distress syndrome (RDS), retinopathy of prematurity (ROP), sepsis, bronchopulmonary dysplasia (BPD), intraventricular hemorrhage, periventricular leukomalacia, and necorotizing enterocolitis were same except patent ductus arteriosus. Conclusion : The survival rate of VLBWI was increased in period II, especially in less than 1,000 gm and below 27 weeks. This may be due to recent dramatic improvement of neonatal care. But more efforts are needed to improve outcome during initial phase and to reduce long term complication such as BPD and ROP.

• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1247-1251
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• 2007

Purpose : Pathologically, Kawasaki disease (KD) is associated with widespread vascular endothelial damage in the acute phase. The vasculitis induced endothelial injury leads to coagulation abnormalities. Abnormalities of endothelial function, platelet activation, and fibrinolysis are present during acute phase and long after the onset of KD. The aim of study is to evaluate the change of hemostatic markers in the clinical stages of KD and to assess the hemostatic markers to be a useful indicator of the development of coronary artery lesion (CAL). Methods : Seventy four KD patients diagnosed in Chungnam National University Hospital from November 2004 to June 2007. Eleven febrile control and eleven healthy children were selected for healthy control. All blood samples were collected before and after Intravenous gammaglobulin (IVGG), $2^{nd}$ week, and $4^{th}-8^{th}$ week of illness of KD. Results : Initial D-dimer level of Kawasaki disease showed meaningful difference compared to control group (P<0.05). D-dimer and fibrinogen degradation products (FDP) before IVGG increased compared with normal control group and decreased after IVGG administration. It is normalized until 2 weeks later, and continue to decreasing. D-dimer and FDP were significantly different according to the CAL before IVGG. Conclusion : The hemostatic markers may change to the clinical stage of KD, which may suggest the degree of endothelial injury. Increased some hemostatic markers may be the predictors for development of CAL.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.229-234
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• 2009

Purpose : The aim of this study was evaluating the efficacy of endoscopic $Deflux^{(R)}$ submucosal injection in children with primary vesicoureteral reflux (VUR). Methods : Retrospective analysis of medical record was conducted on 38 children (59 ureters) who underwent endoscopic $Deflux^{(R)}$ injection due to primary VUR. Data were collected from March 2000 to February 2006. Mean infused amount of $Deflux^{(R)}$ was 0.77 cc. After $Deflux^{(R)}$ injection, patients were reassessed by voiding cystourethrogram (VCUG) 6 months later. Results : The success rate of endoscopic $Deflux^{(R)}$ submucosal injection 6 months later by VCUG was 100% for grade 1 VUR, 87.5% for grade 2, 60% for grade 3, 26.6% for grade 4, 16.6% for grade 5, respectively and there was negatively significant correlation between success rate and grade of VUR (P<.01). Degree of improvement of VUR by endoscopic $Deflux^{(R)}$ submucosal injection was not related to age at diagnosis, time to operation, existence of voiding dysfunction or constipation and infused amount of $Deflux^{(R)}$. However, group with anticholinergics medication had significantly lower success rate than non-medication group (P<0.047). Conclusion : Endoscopic $Deflux^{(R)}$ submucosal injection is effective therapy in patient with primary VUR, especially low grade VUR. It can be not only a useful substitute for prophylaxis with antibiotics, but also an effective management prior to ureteroneocystostomy in children with primary VUR.

• 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.

• Radiation Oncology Journal
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• v.6 no.2
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• pp.183-194
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• 1988

Twenty five patients with histologically proven medulloblastoma received craniospinal radiotherapy (CSRT) at the Seoul National University Hospital from 1979 to 1984. The extent of tumor removal was biopsy only in 2 patients, partial in 18, and near total in 5. With orthogonal technique of CSRT, mainly 55Gy was delivered to the posterior fossa (PF), 40Gy to whole brain (WB), and 30Gy to whole spine (WS). And with AP; PA technique, 50Gy to PF, 45-50Gy to WB, and 36 Gy to WS. Complete remission was obtained in $84\%$ of patients. Among 21 CR's 10 failures were observed, thus total failure rate was $56\%$ (14/25). Of 14 faiure 13 had the primary failure, 11 failed in primary site alone, 1 failure was combined with ventricular seeding, and another 1 was combined with neck node metastasis. There was 1 isolated spinal failure. Actuarial overall survival rates at 3 and 5 years were $75\%$ and $54\%$, and disease-free survival rates were $58\%$ and $36\%$, respectively. Better 5 year disease-free survival was noted in patients with 55 Gy to the posterior fossa than those with 50Gy $(62\%\;vs\;17\%,\;p<0.05)$, in patients treated with orthogonal technique than those treated with AP:PA technique $(87\%\;vs\;12\%,\;p<0.05)$, and in patients with near total removal than those with partial or less removal of tumor $(56\%\;vs\;30\%,\;N.S.)$ Re-irradiation was not satisfactory No severe late sequelae was noted among the survivors. For the higher control of medulloblastoma, dose to posterior fossa should be at least 55Gy with orthogonal CSRT to small tumor burden. And dose reduction in the subarachnoidal spaces might be safe, but optimal dose to the subarchnoidal spaces should be determined by the thorough tumor staging before radiotherapy.

• Clinical and Experimental Reproductive Medicine
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• v.35 no.4
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• pp.247-265
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• 2008

Objectives: Many types of liver diseases can damage regenerative potential of mature hepatocytes, hepatic progenitor cells or oval cells. In such cases, a stem cell-based therapy can be an alternative therapeutic option. We examined whether human amnion-derived mesenchymal stem cells (HAM) and human umbilical cord-derived stem cells (HUC) could differentiate into hepatocyte-like cells as therapeutic cells for the liver diseases. Methods: HAM and HUC were isolated from the amnion and umbilical cord of the volunteers after a caesarean section with informed consent. In order to differentiate these cells into hepatocyte-like cells, cells were cultivated in hepatogenic medium using culture plates coated with fibronectin. Effects of hepatocyte growth factor, L-ascorbic acid 2-phosphate, insulin premixture fibroblast growth gactor 4, dimethylsulfoxide, oncostatin M and/or dexamethasone were examined on the hepatic differentiation. After differentiation, the cells were analyzed by RT-PCR, immunocytochemistry, immunoblotting, albumin ELISA, urea assay and periodic acid-schiffs staining. Results: Initial fibroblast-like appearance of HAM and HUC changed to a round shape during culture in the hepatogenic medium. However, in all hepatogenic conditions examined, HUC secreted more amounts of albumin or urea into medium than HAM. Expression of some of hepatocyte-specific genes increased and expression of new genes were observed in HUC following cultivation in hepatogenic medium. Results of immunocytochemistry and immunoblotting analyses demonstrated that HUC secreted albumin into the culture medium. PAS staining further demonstrated that HUC could store glycogen inside of the cells. Conclusions: Both HUC and HAM could differentiate into albumin-secreting, hepatocyte-like cells. Under the same hepatogenic conditions examined, HUC more efficiently differentiated into hepatocyte-like cells compared with the HAM. The results suggest that HUC and HAM could be used as sources of stem cells for the cell-based therapeutics such as in liver diseases.