• Childhood Kidney Diseases
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• v.3 no.2
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• pp.161-169
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• 1999

Purpose : The urinary mass screening program for the detection of proteinuria in school aged population has been performed in Seoul since 1981. Systematic evaluation in corporation with the Seoul School Health Center for students with proteinuria identified in the mass screening has been performed from 1987. The results of urinary mass screening up to 1994 was reported. I report here the results of urinary mass screening from 1995 to 1997 and compare them with previous results and attempt to reveal the significance of urinary mass screening. Objects and Methods : In the 3-year period between 1995 and 1997, annually about 460,000 students comprising 3 different age groups; 5th grade of elementary school, 2nd grade of middle school and 2nd grade of high school were chosen, corresponding to the approximate ages of 11, 14, and 17 years, respectively. These subjects accounted for 26% of total school aged children in Seoul. The screening program was carried out in 3 steps. The 1st test was performed with dipstick at school and the 2nd at the Seoul School Health Center. Those students who showed proteinuria in the 1st and 2nd tests were referred to the hospital. Laboratory examinations including renal biopsies were performed to those students with pathologic proteinuria to clarify the incipient renal diseases. Results : 1) The prevalence of asymptomatic proteinuria was 0.28% in the 1st test. It peaked at the group of 14 years old as 0.34%, compared with 0.26% at the group of 11 years old and 0.24% at the group of 17 years old. It reached to 0.26% in male and 0.30% in female. 2) 25 percent of those having proteinuria at the first test were positive at the second test. 3) The proportion of patients with proteinuria by 3rd test were as follows; 25% of transient proteinuria, 55% of orthostatic proteinuria, 6% of constant proteinuria, 12% of proteinuria with hematuria, and 2% of transient proteinuria with isolated hematuria. Pathologic proteinuria were totaled as 20%. The prevalence of renal diseases among the age group of 7-18 years old was estimated to be 1.4 per 10,000. 4) Renal biopsy performed on 38 children with proteinuria at the third test revealed IgA nephropathy in 17(44%), focal segmental glomerusclerosis in 5(13%), minimal change disease in 4(11%), membranoproliferative glomeronephritis in 3(8%), $Henoch-Sch\"{o}nlein$ purpura nephritis in 3(8%), and others in 6(16%). Therefore, the prevalence of IgA nephropathy among the age group of 7-18 years old was estimated to be 0.64 per 10,000. 5) The prevalence of chronic renal failure was estimated to be 5.7 per 1 million of 7 to 18 years age group. Conclusions : 1) The prevalence of proteinuria in the first screening test was 0.28% and finally only 5% of them showed the pathologic proteinuria at the third test. 2) The prevalence of IgA nephropathy and chronic renal failure were 0.63 per 10,000 and 5.7 per 1 million, respectively among school-aged children in Seoul.

• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.311-319
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• 2002

Purpose : We have performed this study to obtain reference data for the distribution of chromosomal aberrations in Korea. Methods : We analyzed 1,180 chromosomal study cases from Kwang ju Christian Hospital during the past 25 years. 756 cases suspected of characteristic chromosomal aberration syndromes and 424 cases with hermaphroditism, mild sexual abnormalities, multiple anomalies, or mental & growth retardation were included. Results : The male to female ratio of autosomal aberration syndromes was 1.2 : 1. 78.6% of autosomal aberrations were diagnosed under 1 year of age, whereas 89.8% of sex chromosomal aberrations were diagnosed over 12 years of age. Among 1,180 cases, 612 ones had chromosomal aberrations(51.9%) : 590 of 756 cases suspected of chromosomal aberration syndromes had aberrations( 78.0%), whereas 22 of 424 showing the above other features had aberrations(5.2%). Autosomal aberrations appeared in 514 cases(83.8%) and sex chromosomal aberrations appeared in 98 cases(16.2%). The most frequently observed abberation in autosomal aberrations was Down syndrome, followed by E, D, B, A and C group aberrations. The most common abberation in sex chromosomal aberrations was Turner syndrome, followed by Klinefelter syndrome and Fragile X syndrome. Conclusion : It is of vital importance that patients suspected of chromosomal aberrations undergo chromosomal analysis. Further advanced chromosomal staining and molecular genetic methods will raise the detection rate of chromosomal aberrations.

• Clinical and Experimental Pediatrics
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• v.45 no.9
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• pp.1075-1082
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• 2002

Purpose : Obesity is known to be associated with hypertension, dyslipidemia, and fatty liver and is thought to be associated with increased levels of free fatty acids. One of the strategies for decreasing free fatty acid levels is stimulation of hepatic lipid oxidation with L-carnitine. Carnitine is an essential cofactor for transport of long-chain fatty acid into mitochondria for oxidation. This study was designed to evaluate the changes of serum fatty acids and carnitine levels after exogenous injection of L-carnitine. Methods : Sprague Dawley rats were divided into two groups. Group A was control. Group B was given intraperitoneal injection with L-carnitine(200 mg/kg) daily for two weeks. Serum lipid (total cholesterol, triglyceride, HDL-cholesterol, LDL-cholesterol) and fatty acid levels were analyzed on the first day of the first and second weeks after injection of L-carnitine. Total, free, and acyl carnitine levels also were performed by a enzymatic cycling techniques at the same day intervals. Results : There was no significant difference between the two groups in total cholesterol, HDL-cholesterol, LDL-cholesterol levels before and after the administration of L-carnitine. But triglyceride levels were significantly decreased at the first week in group B compared with group A. Among free fatty acids, linoleic acid showed significant decrement(A group : $131.3{\pm}31.3mg/dL$ vs B group : $90.0{\pm}7.0mg/dL$) at the first week. Total, free, and acyl carnitine levels showed significant increments at all days intervals, but only free carnitine showed significant increments according to cumulative doses of carnitine. Conclusion : Plasma linoleic acid, a long-chain fatty acid, showed significant decrement after administration of L-carnitine in the first week. This may suggest that L-carnitine can be used as an antilipidemic agent for obese patients. A prospective study will investigate obese children in the future.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.2
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• pp.151-159
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• 2000

Purpose: Recently, a wide application of gastrofiberscopy in the pediatric group have revealed that nodular duodenitis is not an uncommon disease in children and is suspected to be associated with H. pylori infection. The aim of this retrospective study was to investigate the clinical and histopathologic features in children with nodular duodenitis, and to assess the correlations beween both. Methods: During a period of 5 years (Jan. 1995~Dec. 1999), we investigated clinical, endoscopic and histopathologic features of 39 consecutive patients diagnosed as having nodular duodenitis at Pediatric department of Seoul Red Cross Hospital. In 35 children with nodular duodenitis endoscopic biopsy specimens were stained with Hematoxylin & Eosin and Giemsa's stain, and were graded according to the criteria outlined by Triadafilopoulos, Whitehead et al., and Prieto et al.. Statistical analyses were performed with Graph PAD InStat. Results: The prevalence rate of nodular duodenitis was 17.1% and the most frequent chief complaint was abdominal pain (69.2%). Endoscopically grade 1 was the most common (45.7%) and nodular gastritis was coexistent in 28.3%. The most common histology of the duodenum was grade 2 (54.3%), and the most common histologic score of the stomach was 2 (42.9%). H. pylori was found in the duodenum in 37.1%, and in the stomach in 31.4%. The correlation coefficient between the endoscopic grade and the histologic grade of nodular duodenitis was 0.3983 (p=0.0178). And the correlation coefficient between the histologic grade and the grade of H. pylori colonization in the duodenum was 0.5154 (p=0.0018). Conclusion: There was significant correlation between the endoscopic grade and the histologic grade of nodular duodenitis, and was also significant correlation between the histologic grade and the grade of H. pylori colonization in the duodenum. Therfore H. pylori infection should be regarded as an etiologic factor of nodular duodenitis.

• Pediatric Infection and Vaccine
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• v.7 no.1
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• pp.83-93
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• 2000

Purpose : Urabe AM-9 strain was known to be associated with increased aseptic meningitis. The reason for high incidence of vaccine-associated meningitis was known that nucleotide(nt) substituted form G to A at position 1081 of the hemagglutinin-neuraminidase(HN) gene and therefore, glutamic acid changed to lysine at amino acid 335. We assessed by comparing nt sequence of the HN gene form Urabe AM-9 strain with wild strain and documented the correlation between nt substitution and vaccine-associated meningitis. Methods : Two lots of Urabe AM-9 vaccine distributed in Korea and mumps wild strains isolated from 1998 through 1999 were analysed. Analysis was made by nt sequencing following amplification of HN gene by RT-PCR. Results : Nucleotide substitution at position 343, 1476, 1570 was not found in both Urabe AM-9 vaccines and wild strains. But analysis of vaccine strains and wild strains isolated from patients revealed substitution from G to A at nt 1081 of the HN gene. Therefore, it encodes lysine instead of glutamic acid at amino acid 335. There was no mixture from of G and A at nt 1081. Nt at 1470 of one lot of Urabe AM-9 vaccines changed from C to A after Vero cell passage. Nt at 1727 of vaccines and wild strains was substituted A to G, so it encodes glycine instead of aspartic acid. Conclusion : Nucleotide analysis of HN gene revealed that nt 1081 of Urabe AM-9 vaccines and wild strains had wild type AAA($Lys^{335}$) instead of variant type GAA($Glu^{335}$). The results of this study suggest that there was a probability of vaccine-associated meningitis due to Urabe AM-9 in Korea before. But incidence of actual side effect was not evaluated because there was no reporting system in Korea.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.142-154
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• 2002

Purpose : One of the most important adverse effects of long-term cyclosporine therapy is nephrotoxicity, the morphologic changes of which include interstitial fibrosis and arteriolar hyalinization. Recently, several authors have shown that osteopontin plays an important role in the development of interstitial fibrosis by acting as a macrophage chemoattractant and stimulating the production of $TGF-{\beta}$ in experimental cyclosporine nephrotoxicity. However, the relationship between osteopontin and $TGF-{\beta}$ in humans has not been clearly documented so far. We studied the expression of osteopontin and $TGF-{\beta}$ in children with minimal change nephrotic syndrome treated with cyclosporine to demonstrate whether there is a relationship between cyclosporine toxicity and osteopontin expression as previously shown in animal models. Materials and methods : Nineteen children (15 males and 4 females) were the subject of this study. Renal biopsies had been performed before and after the cyclosporine therapy (mean duration: 15.9 months). In 5 patients, additional biopsies were performed after completing the cyclosporine treatment (mean; 26 months). The expressions of osteopontin and $TGF-{\beta}$ were evaluated by immunohistochemistry in the glomeruli and tubulointerstitium. Results : Osteopontin expression was significantly increased in the glomerular mesangium and tubules after cyclosporine treatment. But there was no statistically significant increase of $TGF-{\beta}$ in the interstitium. There was no significant increase in tubular osteopontin and interstitial $TGF-{\beta}$ expression in those cases developing interstitial fibrosis after cyclosporine treatment compared with cases those not developing interstitial fibrosis. No significant changes in osteopontin or $TGF-{\beta}$ expression were observed in subsequent 5 biopsy samples after discontinuation of cyclosporine compared with the first follow up biopsies. Conclusion : These results suggest that osteopontin is a nonspecific marker of renal injury rather than a mediator of interstitial fibrosis in cyclosporine nephrotoxicity of human.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.88-94
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• 1999

Purpose : Malformation of urinary tract is among the most common of all congenital anomalies and can progress to irreversible renal damage before diagnosis due to difficulty of early diagnosis. Present study was undertaken to determine the clinical characteristics of urinary tract anomaly and to find out the most appropriate diagnostic and therapeutic measures for children with these anomalies. Methods : During the past 10 years from 1987 to 1998, review of medical records revealed 65 children with congenital anomaly of urinary tract and the following results were obtained. Results : The most common anomalies were ureteropelvic junction obstruction occuring in 26 cases ($36\%$), followed by ureteral duplication in 11 cases, renal agenesis in 10 cases and ureterovesical function obstruction in 7 cases. Complex anomaly of urinary tract was found in 8 cases and anomaly of other systems such as congenital heart disease was detected in 11 cases. The most frequent age group at the time of diagnosis was below 1 year of age constituting 39 cases ($60\%$) and male preponderance was noted as male to female ratio being 2.25:1. Presenting symptoms were urinary tract infection in 25 cases, followed by hematuria, abdominal mass, abdominal pain and voiding difficulty, etc, and in 11 cases, the anomaly was picked up by routine prenatal ultrasonography. Azotemia was noted in 9 cases and the underlying anomaly was obstructive uropathy in 4 out of these 9 cases. Surgical correction was undertaken in 38 cases (most frequently in cases of obstructive uropathy) and in 2 out off cases with obstructive uropathy in whom surgical correction was done, azotemia disappeared during follow up period of 1-5years. No new cases of deteriorating renal function appeared during follow-up period. Conclusion : In spite of high incidence of congenital malformation of urinary tract, early diagnosis is still hampered by nonspecific symptoms and signs. Therefore, in patients with symptoms such as urinary tract infection, abdominal pain and voiding problems, etc, it Is advisable to take various diagnostic tests promptly to pick up any urinary tract anomaly and to apply proper therapy in order to avoid progression to irreversible renal damage. In this regard, prenatal ultrasonography should be utilized more widely as a routine procedure to detect any urinary tract anomalies before birth.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.95-103
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• 1998

Purpose : To find and solve the common problems of peritoneal dialysis(PD) by analysing the clinical data of pediatric PD performed in Korea. Methods : 264 cases of CAPD and acute PD had been performed from Nov.1987 to Oct. 1997 in 17 institutions of pediatric nephrology in Korea. Results : CAPD was performed in 114 cases. The mean age of the patients was $10.5{\pm}6.6$ years and male to female ratio was 1.4:1. The original renal diseases of ESRD were proven in 92 cases($80\%$). The common renal disease of ESRD were FSGS($17\%$), reflux nephropathy ($11\%$), chronic glomerulonephritis($9.6\%$). Mean duration of CAPD was $20{\pm}16.9$ months. Peritonitis was the most common complication and incidence was one episode/18.2 patient-months. Other complications were exit site infection in 10 cases, obstruction in 7 cases, leakage of dialysate in 6 cases. The most common etiologic organism of peritonitis was staphylococcus aureus and the next was staphylococcus coagulase(-). Acute PD was performed in 150 cases. Most common underlying causes were congenital heart disease, hemolytic uremic syndrome, sepsis and dehydration. The mean duration was $10.3{\pm}11.3$ days. The most common complication was peritonitis($20.6\%$). The most common etiologic organism was staphylococcus aureus and coagulase(-), acinetobactor and pseudomonas. Conclusion : Reflux nephropathy should be emphasized in early diagnosis and treatment to prevent ESRD. Incidence of congenital anomaly($7\%$) as a original disease of ESRD was relatively low in Korea. Growth status was not significantly improved after CAPD. In acute PD, the incidence of peritonitis was rapidly increased at 2weeks after beginning of dialysis.

• Journal of Life Science
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• v.24 no.12
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• pp.1339-1344
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• 2014

Inflammatory bowel disease is an immune disorder associated with chronic mucosal inflammation and severe ulceration in the gastrointestinal tract. Antibodies against proinflammatory cytokines, including TNF${\alpha}$, are currently used as promising therapeutic agents against the disease. Stabilization of the transcript is a crucial post-transcriptional process in the expression of proinflammatory cytokines. In the present study, we assessed the expression and histological distribution of the HuR protein, an important transcript stabilizer, in tissues from experimental animals and patients with Crohn's disease. The total and cytosolic levels of the HuR protein were enhanced in the intestinal epithelia from dextran sodium sulfate (DSS)-treated mice compared to those in control tissues from normal mice. Moreover, the expression of HuR was very high only in the mucosal and glandular epithelium, and the relative localization of the protein was sequestered in the lower parts of the villus during the DSS insult. The expression of HuR was significantly higher in mucosal lesions than in normal-looking areas. Consistent with the data from the animal model, the expression of HuR was confined to the mucosal and glandular epithelium. These results suggest that HuR may contribute to the post-transcriptional regulation of proinflammatory genes during early mucosal insults. More mechanistic investigations are warranted to determine the potential use of HuR as a predictive biomarker or a promising target against IBD.

• Journal of Chest Surgery
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• v.34 no.8
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• pp.597-603
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• 2001

Background: The purpose of writing this article is to get better clinical results and further clinical improvement based on subject to 110 cases of cardiac surgery which were performed and clinically analyzed. Material and Method: Since January 1995, the patent ductus arteriosus surgery had started in our hospital. In February 1999, an open heart surgery had started and up to September 2000, total of 110 cases were performed as of double ligation of patent ductus arteriosus(10 cases) and open heart surgery(100 cases). Result: Among the patients, Korean-Chines was 74(67.3%) and Han-Chinese was 35(31.8%). Congenital heart disease was 95 cases and acquired valvular heart disease was 15 cases. 83 cases of acyanotic congenital heart disease consisted of ventricular septal defect(VSD) with associated anomaly(45 cases), atrial septal defect(ASD) with associated anomaly(20 cases), patent ductus arteriosus(PDA) with associated anomaly(11 cases), cogenital aortic stenosis(5 cases), double chamber right ventrical(1 case) and Ebstein's anomaly(1 case). Among the 12 cases of cyanotic congenital heart disease, 11 cases of tetralogy of Fallot underwent total correction. Among the 15 cases of acquired valvular heart disease, valvular replacement(7 cases), double valve replacement (3 cases), mitral valve replacement(3 cases) and aortic valve replacement(1 case) were performed. And 8 cases of valvuloplasty were performed by using of commissurotomy, chordal plasty, plasty of papillary muscle, ring type annuloplasty, repair of leaflet. Conclusion: on cogenital heart disease, short term results of surgery for acyanotic cogenital heart disease was good. Among the cyanotic congenital heart disease, tetralogy of Fallot showed a little difference of recovery according to the surgery method so that further follow up observation was needed for long term result. On acquired valvular heart disease, especially, in terms of short term result of valvuloplasty, was relatively good, but further follow up observation was also needed for long term result. There wasn's any operative mortality.