• Childhood Kidney Diseases
• /
• v.6 no.1
• /
• pp.48-55
• /
• 2002

Purpose: Thin glomerular basement membrane nephropathy (TGBMN) is recognized as the leading cause of microscopic hematuria in both children and adults. However thinning of glomerular basement membrane (TCBM) has been found in healthy adult and also is known to be associated with various renal diseases such as Alport syndronh, IgA nephropathy and mesangial proliferative glomerulonephritis. The association of TGBM with minimal change nephrotic syndrome (MCNS) has been very rare so that the present study was undertaken to determine the relationship between TGBM and MCNS. Methods: The study population consisted of 49 children with biopsy- proven MCNS who have been admitted to the pediatric department of Kyungpook University Hospital during the past 5 years from 1997 to 2001. Group I consisted of 8 children associated with TGBM and Group II 41 children without TCBM. Various parameters such as age of illness, duration from discovery of illness to the time of biopsy, family history of hematuria and other laboratory tests were compared between these two groups and the following results were obtained. Results: Age distribution showed slightly older age in Group I ($7.1{\pm}3.5$ years) compared to Group II ($4.8{\pm}2.9$ years). However this was not statistically different (P=0.056). Family history of hematuria was noted in 2 cases in Group II. Though statistically not significant, hematuria was seen in 2 out of 8 cases ($25\%$) in MCNS children with TGBM, compared to 7 out of 41 cases ($17\%$) with MCNS children without TGBM. Other parameters such as BUN, creatinine, 24 hours urine protein excretion, serum protein, albumin, cholesterol, and T4/T8 ratio, showed no difference. Also renal biopsy finding showed no significant difference and the thickness of glomerular basement membrane in Croup I was $188{\pm}30nm$. Conclusion: TGBM was found in 8 out of 49 children with MCNS ($16.3\%$). And this high frequency of occurrence indicates that these association is not an incidental findings. Typical clinical findings of TCBMN was not noted in all of the 8 children with MCNS associated with TGBM, suggesting that thinning of glomerular basement membrane (TCBN) is secondary to rather than the cause of MCNS. (J Korean Soc Pediatr Nephrol 2002;6: 48-55)

• Childhood Kidney Diseases
• /
• v.2 no.1
• /
• pp.20-25
• /
• 1998

Purpose : It has been reported that endothelin-1 can be associated with the development of cyclosporine A nephrotoxicity. Levels of endothelin-1 was assayed to determine the effect of cyclosporine A on the plasma and urine levels of this peptide. Method : Nine patients who were less than 15 years of age and were also diagnosed as steroid dependent minimal change nephrotic syndrome were included in this study. Assay of endothelin-1 was done before and 3 months after cyclosporine A treatment. The mean age of the patients was $8.3{\pm}4.6$ years. Result : There was no significant differences in the plasma levels of endothelin-1 before and after 3 months of cyclosporine A treatment ($3.22{\pm}0.39$ pg/mL vs. $3.84{\pm}1.52$ pg/mL, P=0.64). Also there was no changes in the urine levels of endothelin-1 ($21.8{\pm}5.8$ pg/mL vs. $20.3{\pm}3.1$ pg/mL, P=0.30). Conclusion : No significant changes of endothelin-1 levels were found with 3 months of cyclosporine A treatment. Further studies with longer durations will help clarifying the effect.

• Childhood Kidney Diseases
• /
• v.8 no.1
• /
• pp.1-9
• /
• 2004

Purpose : Minimal Change Disease (MCD) is the most common primary nephrotic syndrome in children. Some suggested that tumor necrosis $factor-{\alpha}$ ($TNF-{\alpha}$) are involved in the pathogenesis of MCD. Methods : This study was done to see the changes of plasma and urinary $TNF-{\alpha}$, and its effect on the determination of permeability of the glomerular basement membrane (BM) contributed by heparan sulfate proteoglycan (HSPG). Study patients consisted of 19 biopsy-proven MCD children aged 2-15 years old. Both plasma and urinary $TNF-{\alpha}$ were measured. Employing the Millicell system, $TNF-{\alpha}$ was screened for the permeability factors. We examined whether $TNF-{\alpha}$ regulated BM HSPG gene expression and HS synthesis in the glomerular epithelial cells (GECs). Results : Urinary $TNF-{\alpha}$ during relapse was significantly increased when compared with that of during remission or controls ($364.4{\pm}51.2$ vs $155.3{\pm}20.8,\;36.0{\pm}4.5$ ng/mg cr) (P<0.05). However, negative results were obtained in the permeability assay using the Millicell system. No difference was seen in the BM HSPG gene expression and HS synthesis in the GECs. Conclusion : It seems that $TNF-{\alpha}$ may not play a disease-specific role in the pathogenesis of MCD.

• Childhood Kidney Diseases
• /
• v.2 no.1
• /
• pp.26-33
• /
• 1998

This retrospective study was designed to evaluate the prognostic value and histologic correlation of sonography in childhood nephrotic syndrome. Sixty-nine patients with proteinuria over 2g per day at the time of presentation who were treated at the Korea University Hospital were included in this review. They were 1 to 15 years old(mean age, 7.8 years) with 49 males and 20 females. In each patient an ultrasound examination was done using SPA 1000(Diasonics, C.A., U.S.A.) on admission. Tissue specimens were obtained from 46 patients. The paraffin-embedded specimens were reviewed with special reference to interstitial edema, interstitial fibrosis, tubular atrophy, global sclerosis or inflammatory cell infiltrates. Biopsy proven renal disease were minimal change disease(n=20), focal segmental glomerulosclerosis(n=7), membranous glomerulonephritis(n=2), membranoproliferative glomerulonephritis(n=1), $Henoch-Sch\"{o}nlein$ purpura nephritis(n=6), IgA nephropathy(n=5), poststreptococcal glomerulonephritis(n=2), systemic lupus erythematosus(n=1) and Alport syndrome(n=2). There was a significant relationship between increased cortical echogenicity and global sclerosis or tubular atrophy(P<0.05). But no significant relationship was found between increased cortical echogenicity and interstitial fibrosis, interstitial edema, or inflammatory cell infiltration. In biopsy-proven primary nephrotic syndrome(n=30), no significant relationship was found between the increased conical echogenicity and the interstitial edema, interstitial fibrosis, global sclerosis, tubular atrophy or inflammatory cell infiltration. But there was a significant relationship between increased cortical echogenicity and resistance to corticosteroid (P<0.05). These results suggest that increased cortical echogenicity may be due to tubular atrophy or global sclerosis in patients with proteinuria and may be an effective indicator of resistance to corticosteroid in primary nephrotic syndrome.(J Korean Soc of Pediatr Nephrol 2:26-33, 1998)

• Childhood Kidney Diseases
• /
• v.9 no.2
• /
• pp.159-166
• /
• 2005

Purpose : Recently the merits of 6 weeks of initial prednisolone treatment for pediatric primary nephrotic syndrome have been reported, and the use of the 6 week regimen is increasing. We compared our experiences with the 6 week treatment versus the 4 week treatment for Korean patients. Methods : We conducted a retrospective analysis of 69 children who had primary nephrotic syndrome and who were followed up for at least 12 months in the 4 major medical centers in Daegu. The remission rate, the relapse rate, the frequency of relapse and complication of steroid treatment were compared between the 4 weeks and 6 weeks treatment group. Results : Of the 69 children, 42 were in the 4 week treatment group and 27 were in the 6 week group. The median age, blood pressure, serum total protein, serum albumin, cholesterol, creatinine, estimated creatinine clearance, 24 hour urine protein and 12 month cumulative dose did not differ between the two groups. Among the children who relapsed after steroid treatment, the relapse time was significantly later for the 6 week treatment group. The relapse rate after 1 year of treatment was 62$\%$ in the 4 week treatment group and 52$\%$ in the 6 week treatment group; however, there was no statistically significant difference between the two groups. The frequency of relapse at 12 months was $1.5{\pm}1.2$ times in the 4 week treatment group and $1.1{\pm}1.2$ times in the 6 week treatment group, and there was not different between the two groups. The most common side effects of steroid treatment were an increase of appetite and a cushingoid appearance, and there was no statistical difference between the two groups. Among the 27 children who had kidney biopsies performed, 21 suffered from minimal change nephrotic syndrome. Conclusion : The first relapse time after steroid treatment was significantly later in the 6 week steroid treatment group. The frequency of relapse and the 12 month cumulative dose of steroid were lower in the 6 week treatment group, but there was no statistical significance between the two groups. The side effects of steroid treatment did not differ between the two groups. We need to study the long term side effects and the advanced regimens of steroid treatment in the future.(J Korea Soc Pediatr Nephrol 2005;9:159-166)

• Childhood Kidney Diseases
• /
• v.9 no.2
• /
• pp.167-174
• /
• 2005

Purpose : Relapses are a major problem in children with steroid responsive nephrotic syndrome(SRNS). This study has been performed to determine the predictive factors for relapse in children with SRNS. Methods : The study group consisted of 7,3 children with SRNS who had been admitted to the Department of Pediatrics, Kyungpook National University Hospital, over 6 years from 1996 to 2001. The medical records were reviewed retrospectively and analyzed to determine significant relationships between selected variables[age at onset, sex, laboratory data, the rapidity of response(days to remission), interval to first relapse] and the frequency of relapse. Results : The age($mean{\pm}SD$) of patients was $4.53{\pm}2.53$ years old. The male to female ratio was 52:21. In 95$\%$, 39 out of the 41 children had a renal biopsy, and the final diagnosis was minimal change nephrotic syndrome. There was no significant correlation between the frequency of relapse and the following variables age at onset, sex, and presence of hematuria. However, the rapidity of response correlated well with the frequency of relapse, especially during the first year after the onset of the disease(P=0.005). Conclusion : The rapidity of response is expected to be one of the predictive (actors for relapse in children with SRNS. (J Korean Soc Pediatr Nephrol 2005;9:167-174)

• Childhood Kidney Diseases
• /
• v.4 no.1
• /
• pp.17-24
• /
• 2000

Purpose: MCNS is found in approximately $85\%$ of the idiopathic nephrotic syndrome in children and shows good prognosis with initial steroid therapy. However in FSGS, there is poor prognosis with initial therapy and shows higher rate of progression to chronic renal failure and relapse after kindney transplantation. We have experienced 8 patients who were diagnosed as MCNS on initial renal biopsy and then progressed to FSGS on follow-up biopsy. So we have investigated their clinical course and risk factors for transition of MCNS to FSGS. Methods: We conducted a retrospective study with a review of histopathologic findings and clinical manifestations of 296 cases of MCNS and FSGS that were diagnosed from January 1988 to May 1999. We classified them into 3 groups according to the histopathologic finding; MCNS, FSGS, MCNS progressed to FSGS in follow-up biopsy. Results: The number of children was 296 cases comprising 241 cases($81.4\%$) showing MCNS, 8 cases($2.7\%$) transition group, 47 cases($15.9\%$) FSGS. The mean onset age was $6.0{\pm}2.6$years in MCNS, transition group $8.3{\pm}2.3$years, FSGS $7.2{\pm4.3$years, and the gender (M:F) ratio was 3.7:1 in MCNS, 3:1 in transition group, 1.8:1 in FSGS. Comparing the presence of initial hematuria, hypertension,24 hour urine protein, serum albumin, serum creatinine, there were significant difference between the transition group and the FSGS group in the following points; 24hour urine protein $684:342mg/m^2/hr$(P<0.05), serum albumin 1.92: 2.47g/dL(P<0.05), serum cholesterol 494:343mg/dL(P<0.05). Refractoriness to steroid therapy was 13.3$\%$ in MCNS. $12.5\%$ in transition group, $29.6\%$ in FSGS; significantly higher in FSGS(P<0.05). Immunosuppressant therapy was performed in $58.5\%$ of MCNS, $100\%$ in transition group, $80.8\%$ in FSGS; transition group showed significantly higher .ate(P<0.05) comparing with MCNS. Mean number of relapse and duration from onset to first relapse showed no significance difference between these groups. Conclusion: 249 patients with MCNS have been followed and $3.2\%$ (8 patients) of them has shown change in pathologic diagnosis from MCNS to FSCS. The risk factor for transition could not be found. Our results point to the need for a follow-up biopsy to certify the possibility of transition to FSCS in some MCNS cases with refractory cases to steroid therepy, frequent relapsing cases, or in case of no remission in spite of vigorous immunosuppressant therapy.

• The Journal of Korean Medicine
• /
• v.21 no.3
• /
• pp.40-50
• /
• 2000

Objectives : The progression of renal disease can be identified as a glomerulosclerosis by histological examination, and the basic mechanism of glomerulosclerosis is mesangial cell proliferation and mesangial matrix accumulation. ICAM-1, ${\beta}1-integrin$ and MHC-class II are known to attribute to the progression of glomerulosclerosis. They mediate cell-cell or cell-matrix interactions and are expressed in response to injury and inflammation. Up to now, there have been few satisfactory regimens to treat glomerular diseases except minimal change nephrotic syndrome, which can be improved by steroid therapy. Studies were performed in order to investigate whether Jinmu-tang has suppressive effects on some factors associated with the progression of glomerular disease, mesangial cell proliferation, fibronectin synthesis, ICAM-1, ${\beta}1-integrin$ and MHC-class II expression. Methods : Studies were performed with the method of surface enzyme immunoassays or flow cytometry after addition of peripheral blood mononuclear cells(PBMC) supernatants treated with Jinmu-tang, using the cultured human mesangial cells. Results : 1. The suppressive effect of Jinmu-tang on mesangial cell proliferation was higher than that of hydrocortisone. 2. Jinmu-tang has some suppressive effects on fibronectin synthesis, ICAM-1, expression, ${\beta}1-integrin$ expression and MHC-class II expression of mesangial cells, but was lower than hydrocortisone. Conclusions : Jinmu-tang generally shows some immunosuppressive effects. We carefully suggest that the above prescription may be applied to prevent the progression of renal disease or can be used as an adjuvant of or a substitute for steroid therapy.

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

• Journal of the Korea Computer Graphics Society
• /
• v.11 no.2
• /
• pp.40-46
• /
• 2005

본 논문에서는 스윕곡면(sweep surface)을 기반으로 복잡한 3차원 물체의 형상을 변형하는 새로운 자유형상 변형(free-form deformation)기법을 제시한다. 본 논문에서 제시한 방법은 기존의 격자점(control lattices)을 조정하는 체적기반의 방법에 비해 스윕기반의 절차적 (procedural) 모델링 기법의 직관성을 활용하여 사용자에게 보다 효율적인 형상변형 기법을 제공한다. 3차원 물체의 정점들은 스윕곡면의 단면(cross-section)에 바인딩(binding) 되어 곡면의 변화에 따라 형상변형을 한다. 본 논문에서 제시한 스윕기반 형상변형 기법은 3차원 물체를 부분적으로 변형하는 기법과 계층구조를 기반으로 전반적인 형상을 변형하는 기법으로 구분된다. 부분적인 형상변형 기법은 스윕곡면을 구성하는 성분의 다단계(multi-level) 표현을 통하여 사용자에게 다중해상도(multi-resolution) 형상변형 기능을 제공한다. 계층구조에 기반한 형상변형 기법은 복잡한 3차원 물체를 위상구조에 따라 여러 계층으로 분할하고, 각 부분의 형상을 근사하는 스윕곡면들을 생성한다. 분할된 각 부분의 정점(vertices)들은 해당 스윕곡면에 바인딩 되어 스윕곡면의 단면의 변화를 따르게 된다. 이러한 상황하에서 스윕곡면들은 3차원 물체의 계층구조에 따라서 서로 상호작용(interaction)을 하게 되고, 결과적으로 3차원 물체의 각부분은 상호반응적 형상변형을 한다. 이러한 계층구조에 기반한 상호반응적 형상변형 기법은 사용자의 의도에 부합하는 보다 사실적이고 기능적인 형상변형을 가능하게 한다. 본 논문에서는 다양한 3차원 물체에 대한 실험을 통해 제시된 방법의 효율성을 입증한다. 결과 막성 사구체병증과 같은 신질환이 발견될 수 있으므로 신조직검사의 적응증에 합당한 경우 적극적인 신조직검사를 시행하여 원인 질환을 찾는 노력이 필요할 것으로 사료된다.. 남아가 75명으로 남녀비는 3.6:1을 보였다. 임상병리학적 분류상 일차성 신증후군이 89명(92.7%), 이차성 신증후군이 7명(7.3%)이었으며 일차성 신증후군에서 minimal change nephrotic syndrome이 71명으로 79.8%, focal segmental glomerulosclerosis 11.2%, mesangial proliferation 4.5%, membranoprolifrative glomeulonephritis 3.4%, membranous nephropathy 1명 1.1%이었으며 2차성 신증후군은 $Henoch-Sch\"{o}nlein$ nephritis가 3례로 가장 많았다. 미세변화 신증후군 71명 중 비재발군이 16명으로 22.5%, 비빈발 재발군 49.3%, 빈발 재발군 18.3%, 스테로이드 의존군 9.9%를 보였다. 결론 : 대전시에서의 신증후군 환아의 발생빈도는 15세이하 소아 10만명당 약 5명으로 추정되었으며 10여 년전과 비교하여 큰 변화를 보이지는 않았다. 또한 저자들의 임상병리학적 연구결과가 다른 문헌에서 보고된 소아 신증후군의 연구결과와 큰 차이를 보이지 않음을 알 수 있었다. 자극에 차이가 있지 않나 추측되며 이에 관한 추후 연구가 요망된다. 총대장통과시간의 단축은 결장 분절 모두에서 줄어들어 나타났으나 좌측결장 통과시간의 감소 및 이로 인한 이 부위의 통과시간 비율의 저하가 가장 주요하였다. 이러한 결과는 차가운 생수 섭취가 주로 결장 근위부를 자극하는