The Usefulness of Measuring Urinary Proteins by SDS-PAGE in Children with Type I DM without Overt Proteinuria / 대한신장학회잡지

BACKGROUND: The present study was done to evaluate the usefulness of SDS-PAGE in measuring glomerular proteins and tubular proteins in patients with type I DM without overt proteinuria. METHODS: The study population consisted of 76 children with type I DM who have been participated in the Taegu Diabetic Camp from 1997 to 2000. We measured urine albumin, NAG, beta2-microglobulin, creatinine level in urine samples collected for 12 hours in 22 children and simultaneously we analyzed urinary proteins by SDS-PAGE. In remainder 54 children, we measured urine albumin, NAG, creatinine level in random morning urines and urinary proteins by SDS-PAGE. RESULTS: In 22 of 76 children, urinary albumin-to-creatinine ratio (mg/mg), NAG (U/g) and beta2-microglobulin (microgram/g) were 0.021, 2.99 and 170.2, respectively. Positive correlation between urine albumin and NAG was statistically significant (r=0.51, p< 0.05). No significant relations between urinary proteins, such as albumin, NAG, beta2-microglobulin, and clinical characteristics, such as age, duration of illness, HbA1c, were observed. In diagnosing `microalbuminuria', sensitivity of SDS-PAGE was 63% and band of tubular proteins was not detected in any children. CONCLUSION: We thought that SDS-PAGE could be one of useful methods in diagnosing early stage of diabetic nephropathy. The more large scale study is necessary.

Focal Segmental Glomerulosclerosis, FSGS / 소아과

No abstract available.

Asymptomatic Primary Hematuria in Children

PURPOSE: Present study has been undertaken to determine the distribution of various renal diseases causing asymptomatic hematuria in children and to evaluate the benefit of doing renal biopsy in these children. METHODS: Study population consisted of 146 children with asymptomatic primary hematuria who had been admitted to the pediatric department of Kyungpook National University Hospital for the past 4 years from 1999 to 2002. In 122 out of 146 cases, renal biopsy was performed percutaneously and in 24 out of 146 cases, diagnosed as idiopathic hypercalciuria, oral calcium loading test was performed. RESULTS: The age(mean+/-SD) at onset or discovery of hematuria of the 146 children included in this study was 8.0+/-3.2 years and the proportion of boys and girls was 54.8% and 45.2%, respectively. In 76 out of 146 cases(52%), asymptomatic hematuria was first diagnosed by school urinalysis screening. The proportion of histopathologic findings based on 122 biopsies was as follows : Thin Glomerular Basement Membrane(TGBM) 73 cases(50%); IgA nephropathy 20 cases(14%); Alport syndrome 6 cases(4%); Membranous Glomerulonephropathy(MGN) 4 cases(3%); Membranoproliferative Glomerulonephritis(MPGN) 2 cases(1%); IgA nephropathy with TGBM 3 cases(2%); "normal" glomeruli 14 cases(10%). Twenty four cases (16%) were diagnosed as idiopathic hypercalciuria. During follow-up periods, 15% of 146 cases became hematuria-free and renal function did not deteriorate in any cases. CONCLUSION: Unless hematuric children manifest poor prognostic indicators for renal survival, we would recommend long term regular follow-up prior to a renal biopsy.

Effect of Tumor Necrosis Factor-Alpha on Glomerular Epithelial Cells in Glomerular Permeability

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+/-51.2 vs 155.3+/-20.8, 36.0+/-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.

The Pattern of Differentially Expressed Genes in Biliary Atresia

Biliary atresia is a progressive obliterative cholangiopathy, but the etiology of this disorder remains uncertain. Identifying genes specifically expressed in biliary atresia and analyzing the pattern of expression may lead to a better understanding of the pathogenesis. Liver tissues were taken from a recipient with biliary atresia and a normal donor during liver transplantation. Total RNA was extracted from each sample and reversely transcribed to cDNA. Then radiolabeled cDNA probe pools were made by random primed DNA labeling method and used for screening of differentially expressed genes by hybridizing with expressed sequence tags (EST) dot blot panel. Northern blot hybridization was done to confirm that these genes are also differentially expressed in other liver tissues. Among 1,730 EST clones, 26 cDNA clones were significantly overexpressed in biliary cirrhosis, while 2 clones were significantly decreased in biliary atresia. By Northern blot hybridization, the results of tissue inhibitor of metalloproteinase (TIMP)-1 and IGFBP-2 were well correlated with differential EST screening (DES). This study identified the pattern of differentially expressed genes in the biliary cirrhosis due to biliary atresia using DES technique.

Henoch-Schonlein Nephritis in Korean Children: Renal Outcome and Prognostic Factors / 대한신장학회잡지

Purpose: This retrospective study has been undertaken to find out the clinical outcome of children with Henoch-Schonlein nephritis and its relationship with initial clinical presentations and/or renal pathologic findings. METHODS: Study population consisted of 54 children with HS nephritis who have been admitted to the Pediatric department of Kyungpook University Hospital from 1993 to 2003, and biopsy was done with indications of heavy proteinuria (1 g/m2/day) lasting over 1 month, nephrotic syndrome, and persistent hematuria and/or proteinuria over 1 years. Patients were clinically divided into 3 groups; isolated hematuria, hematuria with proteinuria and heavy proteinuria (including nephrotic syndrome). Biopsy findings were graded from I-VI according to International Study of Kidney Disease in Children. RESULTS: Mean age of presentation was 7.9+/-2.7 years and slight female predominance was noted (24 boys and 30 girls). Histopathologic grading showed grade I in 5, grade II in 17, grade III in 29, and grade VI in 3 cases. Clinical outcome at the follow- up period less than 4 years (45 cases) and more than 5 years (18 cases) showed normal urinalysis in 17 (38%) and 10 (56%), persistent isolated hematuria in 14 (31%) and 1 (5%), hematuria with mild proteinuria in 14 (31%) and 7 (39%), respectively. No patients persist heavy proteinuria more than 3 years. Clinical outcome according to histopathologic grading showed that normalization of urinalysis was significantly lower in grade III when compared to grade II (p<0.05). Disappearance of proteinuria takes significantly longer in children with crescent formation (p<0.05). CONCLUSION: The majority of children with mild HS nephritis had good prognosis. The severity of histopathologic grade was well correlated with improvement of urinalysis. Our study suggests that renal biopsy provides a valuable prediction of prognosis even in mild HS nephritis without renal functional impairment.

Changes of Plasma and Urinary TNF-alpha in Children with Minimal Change Nephrotic Syndrome and Its Role in Albumin Permeability

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. This study was done to see the changes of plasma and urinary TNF-alpha, and their effects on the permeability of glomerular basement membrane. METHODS: 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 were screened for the permeability factors. RESULTS: Urinary TNF-alpha during relapse was significantly increased(P<0.01). No significant change was seen in the plasma TNF-alpha during relapse when compared to those in remission and the healthy controls. Furthermore, in the in vitro Millicell system, TNF-alpha did not produce a significant change in albumin permeability. CONCLUSION: Therefore, it seems that TNF-alpha may not play a disease-specific role in the pathogenesis of MCD.

A Case of Minimal Change Nephrotic Syndrome Associated with D-penicillamine Therapy of Wilson's Disease / 대한소아소화기영양학회지

Wilson's disease is a treatable autosomal recessive inherited disorder of copper metabolism due to mutation of the copper transporting gene. The basic strategy of treatment is to reduce the amount of copper in the liver and other tissues by administering both a low copper diet and copper-chelating agents. D-penicillamine is the first choice as a copper-chelating agent. Some serious side effects could occur in 3~5% of all patients following D-penicillamine therapy. We report a 19 year-old male with Wilson's disease who developed nephrotic syndrome 6 months after the initiation of D-penicillamine therapy. Prednisolone was administered to control nephrotic syndrome and D-penicillamine was switched to trientine. Urinary remission was achieved within a week and maintained thereafter. Nephrotic syndrome was proven to be MCNS by kidney biopsy.

A Study on the Association of Thin Glomerular Basement Membrane Abnormality with Minimal Change Nephrotic Syndrome

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 (TGBM) has been found in healthy adult and also is known to be associated with various renal diseases such as Alport syndrome, 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 TGBM. 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+/-3.5 years) compared to Group II (4.8+/-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 Group I was 188 30 nm. 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 TGBMN was not noted in all of the 8 children with MCNS associated with TGBM, suggesting that thinning of glomerular basement membrane (TGBN) is secondary to rather than the cause of MCNS.

Changes of Blood and Urinary Interleukin-8 (IL-8) and Tumor Necrosis Factor-alpha (TNF-alpha) in Children with Minimal Change Nephrotic Syndrome, and Changes of Heparan Sulfate Proteoglycan mRNA Expression by IL-8 and TNF-alpha in Rats Glomerular Epitheli / 대한신장학회잡지

BACKGROUND: Minimal Change Nephrotic Syndrome (MCNS) is one of the most common primary nephrotic syndromes in children and its pathogenesis has not been exactly known. Interleukin-8 (IL-8) and tumor necrosis factor-alpha (TNF-alpha ) may be involved in the pathogenesis of this disese, because they are increased in blood and/or urine during relapse. This study was conducted to see changes of IL-8 and TNF-alpha in children with MCNS and to see the effects of IL-8 and TNF-alpha on the abundance of HSPG mRNA in rats glomerular epithelial cells (GECs). METHODS: Study patients consisted of 19 biopsy-proven MCNS children aged 2-15 years old. Ten age-matched healthy children were used as controls. Both blood and urinary IL-8 and TNF-alpha were measured using ELISA kit. GECs were cultured until confluent. IL-8 or TNF-alpha were added at various concentrations. Total RNA was extracted at 12 or 24 hours after adding IL-8 or TNF-alpha . RT-PCR using HSPG-specific primers and beta-actin as internal controls was done. Densities and areas of GBM HSPG corresponding bands to beta-actin bands were measured. RESULTS: Values of urinary IL-8 (ng/mg'cr) were 13,996+/-2,811, 2,811+/-3,734, and 5,331+/-6,403, during relapse, remission, and in control, respectively. Urinary IL-8 'during relapse' was significantly measured increased compared to 'during remission' and in controls (p<0.05). Values of urinary TNF-alpha (ng/mg'cr) were 364.4+/-512.1, 155.3+/-208.0, and 36.0+/-45.0, during relapse, remission, and in control, respectively. Urinary TNF-alpha during relapse was also significantly increased compared to 'during remission' and 'control' (p<0.05). Values of Blood IL-8 (ng/mL) were 1.19+/-1.23, 0.51+/-0.84, and 0.77+/-0.62, during relapse, remission, and in control, respectively. Blood IL-8 during relapse was significantly increased compared to 'during remission' and 'in control' (p<0.05). No significant change was seen in blood TNF-alpha. And no significant difference was seen in abdundance of HSPG mRNA after adding various concentraons IL-8 or TNF-alpha into rats GEC. CONCLUSION: The values of plasma and urinary IL-8 and TNF-alpha during relapse were increased compared to those of remission period and in control. but neither IL-8 nor TNF-alpha affect the abundance of HSPG-mRNA in rats GECs at various concentraions. So, it seems that both IL-8 and TNF-alpha do not play a direct role in GBM permeability and the elevated values of these cytokines in plasma and/or urine are secondary effects.

Long-term Cyclosporine A(Cypol(R)) Therapy in Children with Idiopathic Nephrotic Syndrome / 대한신장학회잡지

This multicenter collaboratory study was conducted to find out the long-term therapeutic efficacy and side effect of cyclosporine A(Cypol(R), Chong Kun Dang) on children with idiopathic nephrotic syndrome who experienced frequently relapsing(FR), steroid dependent(SD), or steroid resistant(SR) pattern. Forty-six children with SD/FR NS and 5 children with SR NS were enrolled in this study. After induction of remission(SD/FR NS) with steroid or after 4 weeks of steroid therapy(SR NS), cyclosporine A was started in a dose of 4-5mg/kg/day in two divided dose and steroid(prednisolone or equivalent dose of deflazacort) was tapered slowly. During 12 months of study period, monthly check up of physical examination and various laboratory tests including BUN, creatinine, Ccr and cyclosporine blood level were done. Out of 46 children with SD/FR NS, 28(60.9%) maintained sustained remission, 16(34.8%) showed 1 or 2 relapses during therapy and 2(4.3%) cases showed no response. At 4 weeks after therapy, values of serum protein, albumin, cholesterol, and 24 hours urinary protein excretion showed normal values. Four out of 5 children with SR NS showed complete or partial remission with cyclosporine A therapy and one child showed no response. Side reaction to cyclosporine A therapy showed hypertricosis in 14 cases, hyperuricemia in 8 cases and hypomagnesemia in 16 cases. However, other laboratory tests including CBC, liver profile, BUN, creatinine and GFR(creatinine clearance utilizing 24 hour urine) did not show any abnormalities during the 12 months of study period. We performed follow-up renal biopsy in 17 children after 12 months cyclosporine A treatment. Eight cases(47.1%) showed mild cyclosporine A nephrotoxicity like interstitial fibrosis and tubular atropy. In conclusion, present study shows that cyclosporine A(Cypol(R), Chong Kun Dang) can be used quitely effectively in maintaining remission and decreasing relapse rate on children with SD/FR or SR NS. However, because administration of cyclosporine A for 12 months is found to be associated with nephrotoxicity in a significant number of patients, we are planning further study using "smaller dosage" of cyclosporine A to reduce its nephrotoxic effect and for longer period of treatment(over 2 years).

Clinical Characteristics and Prognostic Factors of Hemolytic Uremic Syndrome in Korean Children / 대한신장학회잡지

PURPOSE: Hemolytic uremic syndrome is one of the most frequent cause of acute renal failure in children and can lead to progressive deterioration of renal function. Present nationwide study was undertaken to determine the clinical characteristcs and prognostic factors of hemolytic uremic syndrome in korean children during past 10 years(1990-1999). METHODS: Questionnaires(including clinical data, prodromal illness, lab data, treatment modality and prognosis) were mailed to all teaching hospitals in korea and 27 hospitals responded. During past 10 years, total 149 cases of HUS were diagnosed. Statistical analysis was done by chi-square test, using p<0.05 being "statistically significant". RESULTS: Sex distribution showed slight female preponderance(female 84 vs male 65 cases) and "under 5 years of age" comprised 71.8%(107 cases). Yearly distribution showed increasing number of HUS cases during past 3-4 years and the majority of cases occurred during summer months. Diarrhea was the most common prodromal illness comprising 75.2 % followed by URI 18.2% and in 3.5% of cases no prodromal illness was noted. Lab data (mean+/-SD) showed Hb 7.3+/-2.1g/dL, platelet 49+/-32 X 103cells/mm3, BUN 74+/-36mg/dL, and creatinine 3.7+/-2.8mg/dL. Hypertension was seen in 32.9%, convulsion in 16.7%, mental change in 15.4% and renal replacement therapy(PD or HD) was done in 49.7% of cases. Clinical outcome showed complete recovery in 75.5%, persisting abnormal urinalysis without renal failure in 11.1%, chronic renal failure(including ESRD) in 6.7% and death in 6.7%(9 cases). Poor prognosis was associated with older patients age, higher serum creatinine level, existence of mental change and longer duration of oligoanuria. Out of these, duration of oligoanuria was the most closely associated factor leading to poor outcome. Out of 119 cases with "oligoanuria under 2 weeks", CRF and death were seen in 2 and 7 cases respectively. Compared to this, out of 12 cases with "oligoanuria over 2 weeks", CRF and death were seen in 4 and 2 cases respectively(p<0.0005). CONCLUSION: The incidence of HUS is increasing recently in Korean children. And out of various prognostic factors(older age, higher serum creatinine, existence of mental change and duration of oligoanuria), duration of oligoanuria was the most significantly associated factor leading to poor outcome.

Clinical Characteristics and Prognostic Factors of Hemolytic Uremic Syndrome in Korean Children / 대한신장학회잡지

PURPOSE: Hemolytic uremic syndrome is one of the most frequent cause of acute renal failure in children and can lead to progressive deterioration of renal function. Present nationwide study was undertaken to determine the clinical characteristcs and prognostic factors of hemolytic uremic syndrome in korean children during past 10 years(1990-1999). METHODS: Questionnaires(including clinical data, prodromal illness, lab data, treatment modality and prognosis) were mailed to all teaching hospitals in korea and 27 hospitals responded. During past 10 years, total 149 cases of HUS were diagnosed. Statistical analysis was done by chi-square test, using p<0.05 being "statistically significant". RESULTS: Sex distribution showed slight female preponderance(female 84 vs male 65 cases) and "under 5 years of age" comprised 71.8%(107 cases). Yearly distribution showed increasing number of HUS cases during past 3-4 years and the majority of cases occurred during summer months. Diarrhea was the most common prodromal illness comprising 75.2 % followed by URI 18.2% and in 3.5% of cases no prodromal illness was noted. Lab data (mean+/-SD) showed Hb 7.3+/-2.1g/dL, platelet 49+/-32 X 103cells/mm3, BUN 74+/-36mg/dL, and creatinine 3.7+/-2.8mg/dL. Hypertension was seen in 32.9%, convulsion in 16.7%, mental change in 15.4% and renal replacement therapy(PD or HD) was done in 49.7% of cases. Clinical outcome showed complete recovery in 75.5%, persisting abnormal urinalysis without renal failure in 11.1%, chronic renal failure(including ESRD) in 6.7% and death in 6.7%(9 cases). Poor prognosis was associated with older patients age, higher serum creatinine level, existence of mental change and longer duration of oligoanuria. Out of these, duration of oligoanuria was the most closely associated factor leading to poor outcome. Out of 119 cases with "oligoanuria under 2 weeks", CRF and death were seen in 2 and 7 cases respectively. Compared to this, out of 12 cases with "oligoanuria over 2 weeks", CRF and death were seen in 4 and 2 cases respectively(p<0.0005). CONCLUSION: The incidence of HUS is increasing recently in Korean children. And out of various prognostic factors(older age, higher serum creatinine, existence of mental change and duration of oligoanuria), duration of oligoanuria was the most significantly associated factor leading to poor outcome.

Changes of Plasma and Urine IL-8 and TNF-alpha in Children with Minimal Change Nephrotic Syndrome / 대한신장학회잡지

PURPOSE: Minimal Change Nephrotic Syndrome (MCNS) is one of the most common primary nephrotic syndromes in children. T-cell dysfunction has been thought to be involved in the pathogenesis of MCNS. However, the exact pathogenesis of MCNS has not been proven yet in spite of many studies which support T-cell dysfunction being involved in the pathogenesis of MCNS. A present study was done to determine the role of IL-8 and TNF-alpha in the pathogenesis of MCNS. METHODS: Study patients consisted of 19 biopsy-proven MCNS children aged 2-15 years old. Ten age-matched healthy children were used as controls. Both plasma and urinary IL-8 and TNF-alpha were measured during relapse and remission period using ELISA kit. Urinary cytokine values were corrected for urinary creatinine. RESULTS: Each value of urinary IL-8 measured during relapse and remission period in MCNS and controls was 13,996+/-2,811, 2,811+/-3,734 and 5,331+/-6,403ng/mg cr, respectively, and we noted that the value of urinary IL-8 measured during relapse period in MCNS significantly increased compared to those measured during remission period and in controls(p< 0.05). And each value of urinary TNF-alpha measured in the same group was 364.4+/-512.1, 55.3+/-208.0 and 36.0+/-45.0ng/mg cr, respectively, and we also noted that the value of urinary TNF-alpha measured during relapse period in MCNS was signficantly increased compared to those measured during remission period and in controls. The plasma cytokine values measured during relapse and remission period and in controls were 1.19+/-1.23, 0.51+/-0.84 and 0.77+/-0.62ng/mL, respectively, in the case of IL-8 and 2.42+/-3.86, 1.95+/-3.24 and 2.25+/-3.50ng/mL, respectively, in the case of TNF-alpha, and we noted the value of plasma IL-8 measured during relpase was also significantly increased compared to those of remission period and in control(p<0.05), but the change of plasma TNF-alpha values was not significant. CONCLUSION: It can be said that both IL-8 and TNF-alpha play an important role in the pathogenesis of MCNS in children. The fact that changes of urinary IL-8 and TNF-alpha were more prominent than those of plasma suggests that immune dysregulation may occur intrarenally rather than systemically in MCNS.

Glomerular Hypertrophy and Sclerosis in Rats with Chronic Puromycin Aminonucleoside Nephropathy / 대한신장학회잡지

PURPOSE: This experimental study was conducted to determine serial morphological changes of rat's kidney with chronic puromycin aminonucleoside (PAN) nephropathy. Special emphasis was given to the occurrence of glomerular hypertrophy and its relationship to the subsequent development of focal segmental glomerulosclerosis(FSGS). METHODS: Sprague-Dawley rats weighing 200-230g were used and divided into control(n=9) and experimental group(n=15). Rats were given subcutaneous injections of PAN at a dose of of 2mg/100g body weight, or an equivalent volume of normal saline and six injection were given over a period of 9 weeks, at weeks 0, 1, 3, 5, 7 and 9. At weeks 4, 8 and 11, rats were sacrificed and kidney weight, kidney weight/body weight(%) and various laboratory tests including serum protein and albumin were determined. Renal tissues were prepared with Histochoice(R) fixative and paraffin embedding for morphologic study. RESULTS: Kidney weight and kidney weight/body weight(%) were increased significantly in experimental group compared to controls at 4, 8 and 11 weeks. Heavy proteinuria along with lowering of serum protein and albumin and elevation of serum cholesterol was seen in experimental group at week 4 and this change became more marked on weeks 8 and 11. The frequency of FSGS in experimental animal, at week 4, 8 and 11 were 0.6%, 10.6% and 26.2% respectively(p<0.05) and the development of FSGS was more marked in juxtamedullary glomeruli compared to cortical glomeruli. Glomerular surface area showed significant increase in experimental animals compared to controls(p<0.01), the percentage of increase being 12.0, 14.7 and 12.3% at week 4, 8 and 11. And the surface areas of juxtamedullary glomeruli were larger than those of cortical glomeruli throughout the study period. CONCLUSION: In summary, present study indicates that glomerular hypertrophy occurs and precedes the development of FSGS in rats with chronic PAN nephropathy and juxtamedullary glomeruli are more susceptible to developing FSGS compared to cortical glomeuli.

Magnetic Resonance Voiding Cystography in the Diagnosis of Vesicoureteral Reflux: Comparative Study with Voiding Cystourethrography

PURPOSE: To evaluate the availability of magnetic resonance (MR) voiding cystography for the diagnosis of vesicoureteral reflux (VUR) and to compare the sensitivity of MR voiding cystography (MRVC) with that of radiographic voiding cystourethrography (VCUG) in the detection of VUR. MATERIALS AND METHODS: MRVC was performed upon 20 children referred for investigation of VUR. Either coronal T1-weighted spin-echo or spoiled gradient-echo images were obtained before and after transurethral administration of a mixture of normal saline and gadopentetate dimeglumine, and immediately after voiding. The findings of MRVC were compared with those of VCUG performed within 6 months of MRVC. RESULTS: VUR was detected in 23 ureterorenal units (16 VUR's by both methods, five VUR's by VCUG, and two VUR's by MRVC). The sensitivity of VCUG and MRVC in detecting VUR was 91.3% (21/23) and 78.3% (18/23), respectively. MRVC detected renal scarring in 15 out of 17 kidneys with scintigraphically detected renal scarring. CONCLUSION: Although MRVC is slightly less sensitive than VCUG in the detection of VUR, it can be used for the diagnosis of VUR and renal scarring simultaneously, and thus will reduce the radiation hazard.

Asymptomatic Primary Hematuria in Children

Localized or solitary fibrous tumor (SFT) of the pleura has been classified as a type of mesothelioma, arising from the submesothelial connective tissue cells. The preoperative diagnosis of the tumor at the cytologic or histologic level is very important for the proper handling of the lesion. This preoperative diagnosis is now possible by means of the advance in the transthoracic fine needle aspiration biopsy (FNA) techniques and in the very experience of the cytopathologists. We describe FNA cytologic feature of two cases of SFT arising from the pleura. Cytologic, histologic, immunohistochemical, and electron microscopic characteristics of pleural SFT are discussed. The tumor cells of SFT are spindle or oval in shape with a variable amount of cytoplasm. They are arranged in irregular trabeculae intimately ass- ociated with capillaries. A unique cytologic feature observed in this tumor is that thick, eosinophilic, amorphous collagen bundles are scattered between tumor cells

Effect of Peripheral Blood Mononuclear Cells Isolated from Children with Minimal Change Nephrotir Syndrome to Glomerular Basement Mernbrane Heparan Sulfate Proteoglycan(GBM HSPG) in Rats Glomerular Epithelial Cell: Including Development of Quantitative RT / 대한신장학회잡지

Minimal Change Nephrotic Syndrome(MCNS) reflects a disorder of T-lymphocytes. These T-cells are thought to release a vascular permeability factor (UPF) that injures the glomerular epithelial cells (GECs). Glomerular epithelial cellular damage may lead to proteinuria in MCNS by decreasing the synthesis of polyanions such as heparan sulfate proteoglycan(HSPG) : these polyanions constitute most of the normal charge barrier to glomerular filtration of macromolecules such as albumin. This study evaluates the direct effect of supernatant of culture media of peripheral blood mono- nuclear cells(PBMC) which was isolated from children with MCNS to GBM HSPG mRNA expression in rats GEC. GEC were cultured until confluent. Supernatant of culture media of PBMC from each group of 3 chilren with MCNS, IgA nephropathy or normal healthy were added. Total RNA was extracted at 12, 24 and 72hrs after adding supernatant. RT-PCR using Rat Perlecan Domain-I(RPD- I) specific primers and beta-actin as internal controls was done. Densities and areas of GRM HSPG corresponding bands to beta-actin bands were measured. At 24 hrs, supernatant of culture media of PBMC from 3 children with MCNS caused 62, 70, and 75Vo reductions, respectvely, in GEC's GBM HSPG mRNA expression compared to normal children. However, supernatant of culture media of PRMC from 3 children with IgA nephropathy did not. In addition, reductions of GEC's GBM HSK' mRNA expressions caused by supernatant of culture media of PBMC from 3 children with MCNS were restored upto levels of normal children at 72hrs after adding supernatant. Mutant cDNA was synthesized as primers for competitive PCR to quantify GBM HSPG mRNA expression. Mutant template was 212 base pairs shorter than RPD-I, 497 base pairs. In conclusion, we found that supernatant of culture media of PBMC from children with MCNS reversibly suppressed GBM HSPG mRNA expression in rats GEC. This study suggests cytokines of PBMC from children with MCNS directly injures GEC and leads to decrease in synthesis of GBM HSPG by GEC in the pathogenesis of MCNS.

Henoch-Schonlein Nephritis Children

PURPOSE: This retrospective study has been undertaken to find out the clinical outcome of children with HS nephritis and its relationship with initial clinical presentation and/or renal pathologic finding. PATIENTS AND METHODS: Study population consisted of 59 children with HS nephritis who have been admitted to the Pediatric department of KyungPook University Hospital from 1987 to 1999, and biopsy was done with indications of heavy proteinuria ( > 1 g/m2/day ) lasting over 1 month, nephrotic syndrome, and persistent hematuria and/or proteinuria over 1 year. Patients were divided clinically into 3 groups ; isolated hematuria, hematuria with proteinuria and heavy proteinuria (including nephrotic syndrome). Biopsy findings were graded from I-V according to International Study of Kidney Disease in Children (ISKDC). RESULTS: Mean age of presentation was 8.1+/-3.0 years and slight male proponderance was noted ( 33 boys and 26 girls ). Histopathologic grading showed Grade I ; 2, Grade II ; 44, and Grade III ; 13 cases. Clinical outcome at the follow-up period of 1-2 years (49 cases) and 3-4 years (30 cases) showed normal urinalysis in 15 ( 30.6% ) and 18 cases ( 60.0%), persistent isolated hematuria in 20 ( 40.8% ) and 2 cases ( 6.7 % ), hematuria with proteinuria in 11 ( 22.5% ) and 8 cases ( 26.6% ), and persistent heavy proteinuria in 3 ( 6.1% ) and 2 cases ( 6.7% ) respectively. Clinical outcome according to histopathologic grading showed the frequency of normalization of urinalysis being lower in Grade III compared to grade I or II. Clinical outcome according to initial clinical presentation showed no relationship to the normalization of urinalysis at follow-up periods. However, 15-20% of children with initial heavy proteinuria showed persistent heavy proteinuria ( 3 out of 20 cases at 1-2 years, and 2 out of 10 case at 3-4 years of follow-up periods). CONCLUSION: The majority of children with HS nephritis (histopathologic grade I, II, III) improved within 3-4 years, and persistent heavy proteinuria was seen only in a few of children with initial clinical presentation of heavy proteinuria.

Cyclosporine A ( Cipol-N R ) Therapy in Children with Idiopathle Nephrotic Syndrome

A 30-year-old woman who was diagnosed as peripheral neuroblastoma by fine needle aspiration of a soft mass of the right upper arm is described. She presented a slowly growing, soft mass of the right upper arm for 1 month. The right humerus revealed no abnormal finding on X-ray. Ultrasonogram of the right upper arm revealed a well demarcated, smooth marginated solid mass without invasion of adjacent structures. Fine needle aspiration was done under the impression of soft tissue tumor with undetermined biologic behavior. The aspirates were highly cellular and the tumor cells were dispersed both singly and in clusters of varying size. The clusters occasionally showed a central capillary core and rosette-like structures. The tumor cells were small in size and had a small to medium amount of cytoplasm. Some of them revealed slender cytoplasmic processes. The nuclei showed distinct nuclear membranes, finely clumped chromatin and small conspicuous nucleoli. Cellular pleomorphism or mitotic figure was not definite. These cytologic findings were interpreted as a malignant, non-lymphomatous, small round cell tumor, most likely representing peripheral neuroblastoma or Ewing's sarcoma. Final diagnosis was confirmed by simple excision as peripheral neuroblastoma.