• Childhood Kidney Diseases
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• v.10 no.2
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• pp.152-161
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• 2006

Purpose : In Korea, the school urine screening program is a useful tool for screening urine abnormalities. It is particularly useful in early detection of membranoproliferative glomerulonephritis(MPGN) I, which frequently progresses to chronic renal failure. In this study, we studied the medical history, laboratory findings, and histologic findings of MPGN to gain helpful information on early detection and treatment. Methods : The subjects were 19 children, who were diagnosed with MPGN from kidney biopsies that were performed in ten nationwide university hospitals because of abnormal urine findings from school urine screening programs conducted from July 1999 to April 2004. We divided the patients into 2 groups, a nephrotic range proteinuria group(n=8) and a non-nephrotic proteinuria group(n=11), and retrospectively analyzed the clinical features, laboratory findings, histologic findings, treatment, and clinical course. Results : The mean age at the first abnormal urinalysis was $10.6{\pm}2.2$ years in the nephrotic proteinuria group and $9.6{\pm}3.2$ years in the non-nephrotic proteinuria group. The mean age at the time of kidney biopsy was $11.3{\pm}2.3$ years in the nephrotic range proteinuria group and $10.4{\pm}3.2$ years in the non-nephrotic proteinuria group respectively. There was no significant difference in the mean age and sex between the two groups. In the nephrotic proteinuria group, 6 children had a low plasma C3 level and in the non-nephrotic proteinuria group, 8 children had a low plasma C3 level, but there was no significant difference between the 2 groups. There was no significant difference in the laboratory test results(including WBC count, RBC count, platelet count and other serologic tests) between the 2 groups except for 24 hour urine protein secretion. There was no difference between the 2 groups with regard to the acute and chronic changes in the glomerulus on light microscopic findings, IgG, IgA, Ig M, C1q, C3, C4, fibrogen deposition on immunofluoroscence findings, and mesangial deposits, subendothelial deposits, and subepithelial deposits on electron microscopic findings. The children were treated with corticosteroids, ACE(angiotensin-converting enzyme) inhibitors, dipyridamole and other immunosuppressive agents. During the course of treatment, there were no children whose clinical condition worsened. Among 19 children, 3 children went into remission(2 in the nephrotic proteinuria group, 1 in the non-nephrotic proteinuria group) and 9 children went into a partial remission(4 in the nephrotic proteinuria group, 5 in the non-nephrotic proteinuria group) on urinalysis. There was no significant difference in the treatment results between the two groups. Conclusion : The 73.7% of children who were incidentally diagnosed with MPGN by the school urine screening program had reduced C3. 42.1% of the children had nephrotic range proteinuria. There were no significant differences in clinical features, laboratory test results, light microscopic, immunofluorescence microscopic, and electron microscopic findings between the nephrotic proteinuria group and the non-nephrotic proteinuria group except for the 24 hour urine protein secretion. Therefore, for early detection of MPGN during the school urine screening program, we strongly recommend a kidney biopsy if children have abnormal urine findings such as persistent proteinuria and persistent hematuria, or if the serum C3 is reduced.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.53-59
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• 1997

There have been many recent reports that unilateral renal agenesis and multicystic dysplastic kidneys are accompanied by contralateral vesicoureteral reflux leading to its injury. We grouped the children with unilateral renal agenesis, renal hypoplasia, multicystic dysplastic kidney into abnormal unilateral renal development and investigated whether it was accompanied with contralateral vesicoureteral reflux. We retrospectively reviewed 96 pediatric cases of unilateral renal agenesis, hypoplasia, multicystic dysplastic kidney diagnosed at Shinchon Severance Hospital, Yongdong Severance Hospital from 1987 to 1996 and Ajou University Hospital from 1994 to 1996. Diagnosis was based on radiological findings, renal hypoplasia being defined as small renal size with no apparent renal scarring and no irregularity of the calyceopelvic system on abdominal sonography or intravenous pyelography. Among the 96 cases,48 cases carried out voiding cystourethrography. 58 cases were male(60%) and 38 cases were female(40%). The cases of abnormal unilateral development on the left side were 45(47%) and that on the right side were 51(53%). Although there were diverse reasons leading to diagnosis, the major ones included were prenatal sonography, urinary tract infection, and other congenital anomalies. In cases of unilateral renal agenesis & hypoplasia the leading factors were urinary tract infection & other congenital anomalies and in cases of multicystic dysplastic kidney that was prenatal sonography. There was a chronological gap between the mean age of diagnosis(1.8 year) and voiding cystouerthrography(2.5 year, P < 0.01). 9 of the 18 unilateral renal agenesis cases, 5 of the 11 unilateral renal hypoplasia cases, and 3 of the 19 unilateral multicystic dysplastic kidney cases showed contralateral vesicoureteral reflux. Average reflux grade was above G III.Among the 17 children who had contralateral vesicoureteral reflux, 3 children had chronic renal failure and ureteroneocystostomy was carried out in 6 children. From the above results we conclude that screening voiding cystourethrography should be performed in children with abnormal unilateral renal development for early detection of vesicoureteral reflux in the contralateral kidney.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.170-179
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• 1999

Purpose: To evaluate whether or not sodium restriction had its own beneficial effect and increased the efficiency of the anti-hypertensive drugs on the progression of renal failure. Methods: We studied using the excision remnant kidney model. Treatment groups were as follows: 5/6 nephrectomy and a 0.49% (normal-high) sodium diet (NN); 5/6 nephrectomy and a 0.25% (normal-low) sodium diet (LN); 5/6 nephrectomy, a 0.49% sodium diet and enalapril (NNE); 5/6 nephrectomy, a 0.49% sodium diet and nicardipine (NNN); 5/6 nephrectomy, a 0.25% sodium diet and enalapril (LNE); 5/6 nephrectomy, a 0.25% sodium diet and nicardipine (LNN). Both diets were isocaloric and had the same content of protein, phosphorus and calcium. Proteinuria, remnant kidney weight, mesangial expansion scores, and glomerular volume were assessed. Results: Blood pressure tended to be lower in LN compared to NN (P<0.05). NN developed progressive hypertension. LNE, LU, NNE, and NNN reduced blood pressure. LNE, LNN, NNE, NNN, and LN had significantly less proteinuria than NN at 16 weeks (P<0.05). At 24 weeks, LN developed proteinuria (82 mg/day), which were lessened in LNE (54 mg/day) and not lessened in LNN (76 mg/day). Mesangial expansion scores were significantly less in LN rats compared to those in NN rats. Glomerular volumes at 24 weeks in LN rats were significantly less compared to those at 16 weeks in NN rats. Mesangial expansion scores and glomerular volumes at 4, weeks, 12 weeks, and 24 weeks were not different among LN, LNE, and LNN groups. Conclusion: Dietary salt restriction lessens renal damage, at least in part, by inhibiting compensatory renal growth and reducing blood pressure. Enalapril was particularly successful in reducing proteinuria and glomerular injury when combined with dietary salt restriction.

• Journal of Cardiovascular Imaging
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• v.26 no.4
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• pp.217-225
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• 2018

BACKGROUND: In patients with acute heart failure (AHF), diastolic dysfunction, especially pseudonormal (PN) or restrictive filling pattern (RFP) of left ventricle (LV), is considered to be implicated in a poor prognosis. However, prognostic significance of diastolic dysfunction in patients with ischemic heart disease (IHD) has been rarely investigated in Korea. METHODS: We enrolled 138 patients with IHD presenting as AHF and sinus rhythm during echocardiographic study. Diastolic dysfunction of LV was graded as ${\geq}2$ (group 1) or 1 (group 2) according to usual algorithm using E/A ratio and deceleration time of mitral inflow, E'/A' ratio of tissue Doppler echocardiography and left atrial size. RESULTS: Patients in group 1 showed higher 2-year mortality rate ($36.2%{\pm}6.7%$) than those in group 2 ($13.6%{\pm}4.5%$; p = 0.008). Two-year mortality rate of patient with LV ejection fraction (LVEF) < 40% ($26.8%{\pm}6.0%$) was not different from those with LVEF 40%-49% ($28.0%{\pm}8.0%$) or ${\geq}50%$ ($13.7%{\pm}7.4%$; p = 0.442). On univariate analysis, PN or RFP of LV, higher stage of chronic kidney disease (CKD) and higher New York Heart Association (NYHA) functional class were poor prognostic factors, but LVEF or older age ${\geq}75$ years did not predict 2-year mortality. On multivariate analysis, PN or RFP of LV (hazard ratio [HR], 2.52; 95% confidence interval [CI], 1.09-5.84; p = 0.031), higher stage of CKD (HR, 1.57; 95% CI, 1.14-2.17; p = 0.006) and higher NYHA functional class (HR, 1.81; 95% CI, 1.11-2.94; p = 0.017) were still significant prognostic factors for 2-year mortality. CONCLUSIONS: PN or RFP of LV was a more useful prognostic factor for long-term mortality than LVEF in patients with IHD presenting as AHF.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.144-153
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• 2000

Purpose : Effects to predict tile progression of chronic renal failure (CRF) in children, using mathematical models based on transformations of serum creatinine (Scr) concentration, have failed. Error may be introduced by age-related variations in creatinine production rate. Height (Ht) is a reliable reference for creatinine production in children. Thus, Scr, factored for Ht, could provide a more accurate predictive model. We examined this hypothesis. Methods : The progression of of was detected in 63 children who proceeded to end-stage renal disease. Derivatives of Scr, including 1/Scr, log Scr & Ht/Scr, were defined fir the period Scr was between 2 and 5 mg/dl. Regression equation were used to predict the time, in months, to Scr > 10 mg/dl. The prediction error (PE) was defined as the predicted time minus actual time for each Scr transformation. Result : The PE for Ht/Scr was lower than the PE for either 1/Scr or log Scr (median: -0.01, -2.0 & +10.6 mos respectively; P<0.0001). For children with congenital renal diseases, the PE for Ht/Scr was also lower than for the other two transformations (median: -1.2, -3.2 & +8.2 mos respectively; P<0.0001). However, the PEs for children with glomerular diseases was not as clearly different (median: +0.9, +0.5 & +9.9 respectively). In children < 13 yrs, PE for Ht/Scr was tile lowest, while in older children, 1/Scr provided the lowest PE but not significantly different from that for Ht/Scr. The logarithmic transformation tended to predict a slower progression of CRF than actually occurred. Conclusion : Scr, floored for Ht, appears to be a useful model to predict the rate of progression of CRF, particularly in the prepubertal child with congenital renal disease.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.78-86
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• 2001

Purpose: Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than $50\%$ of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features Method: Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during tile last 10 years, from May 1990 to May 2000. Result: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean $10.9{\pm}3.8$). 3 had Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was $74.2{\pm}39.1\;mg/dL$ mean serum creatinin, $3.2{\pm}1.8\;mg/dL$ and mean creatinin clearance, $26.5{\pm}13.2\;mL/min/1.73m^2$. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function. Conclusion: Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 78-86)

• Animal cells and systems
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• v.7 no.4
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• pp.309-315
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• 2003

Renal dipeptidase (RDPase, membrane dipeptidase, dehydropeptidase 1, EC 3.4.13.19) has been widely studied since it was first purified from porcine kidney brush border membrane. It was reported that RDPase activity in urine samples of acute and chronic renal failure patients decreases. Nitric oxide (NO) is a highly reactive free radical involved in a number of physiological and pathological processes. NO is able to act in a dual mode, leading either to induction of apoptosis or to blunted execution of programmed cell death. NO inhibited the RDPase release from porcine renal proximal tubules, which could be blocked by L-NAME. Chitosan, the linear polymer of D-glucosamine in $\beta$(1\longrightarrow4) linkage, not only reversed the decreased RDPase release by NO but also increased NO production in the proximal tubule cells. The stimulatory effect of NO on RDPase release from proximal tubules in the presence of chitosan must be different from the previously proposed mechanism of RDPase release via NO signaling pathway. Chitosan stimulated the RDPase release in the proximal tubules and increased RDPase activity to 220% and 250% at 0.1% and 1%, respectively. RDPase release was decreased to about 40% in the injured proximal tubules and was recovered in proportion to the increase of chitosan. Chitosan may be useful in recovery of renal function from $HgCl_2$injury.

• Journal of Physiology & Pathology in Korean Medicine
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• v.28 no.5
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• pp.486-493
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• 2014

Adipocytes are endocrine cells that release bioactive mediators called adipokines. In condition of obesity characterized by low-grade chronic inflammation, adipocytes release inflammatory adipokines, which is related to insulin resistance. Bojungchiseub-tang (BJCST) has been used in symptoms and signs of edema, dampness-phlegm, kidney failure, and so on in Korean medicine. BJCST is also expected to have anti-obesity activities. In the present study, we examined whether BJCST modulate the production of inflammatory adipokines and the activations of the mitogen-activated protein kinases (MAPK) signaling pathway related to induce adipocyte inflammation to elucidate the effects and its mechanism of BJCST on lowering the content of inflammatory adipokines in 3T3-L1 adipocytes. As a result, BJCST suppressed the production of proinflammatory cytokines, tumor necrosis factor (TNF) $-{\alpha}$, interleukin (IL) $-1{\beta}$, IL-6, interferon (IFN) -${\gamma}$, granulocyte-macrophage colony-stimulating factor (GM-CSF), monocyte chemoattractant protein-1 (MCP-1), and the production of other inflammatory mediators, prostaglandin $E_2(PGE_2)$ and nitric oxide(NO)viadownregulationofcyclooxygenase-2(COX-2)andinducible NO synthase (iNOS) gene expressions. In addition, BJCST decreased the phosphorylation of MAPK that promotes the production of inflammatory adipokines in 3T3-L1 mature adipocytes. In conclusion, BJCST could regulate the production of inflammatory adipokines and MAPK signaling pathway related to induction of adipose inflammation.

• Childhood Kidney Diseases
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• v.19 no.2
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• pp.184-189
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• 2015

Poststreptococcal glomerulonephritis (PSGN) is one of the most well-known and important infectious renal diseases resulting from a prior infection with group A ${\beta}$-hemolytic streptococcus. The typical clinical characteristics of the disease reflect acute onset with gross hematuria, edema, hypertension and moderate proteinuria after the antecedent streptococcal infection. In children, usually PSGN is healed spontaneously but if it combines with fast progressing glomerulonephritis, it would be developed to chronic renal failure. Therefore, it is important to make a fast diagnosis and treatment by simple tools to predict the course and the prognosis of disease. Sonography is a simple tool for diagnosis but there is no typical renal sonographic finding in PSGN, so it is difficult to predict the course and the prognosis of disease by sonographic findings. In comparison between two cases of renal sonographic findings in PSGN, a patient who showed more increased echogenicity in more extended area of renal sonography had the severe results of renal pathology, prolonged treatment period and low serum C3 level. Here, we report the different findings of renal sonography and pathology depending on the degree of severity between two patients. Thus, it is necessary to gather more information from further studies to make a consensus about the relationship between the renal sonography and the prognosis of disease in PSGN.

• Clinical and Experimental Pediatrics
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• v.54 no.5
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• pp.212-218
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• 2011

Purpose: The diagnosis of acute pyelonephritis (APN) is often difficult, as its clinical and biological manifestations are non-specific in children. If not treated quickly and adequately, however, APN may cause irreversible renal damage, possibly leading to hypertension and chronic renal failure. We were suspecting the diagnostic value of $^{99m}Tc$-dimercaptosuccinic acid (DMSA) scan by experiences and so compared the results of DMSA scan to those of multi-detector row computed tomography (MDCT). Methods: We retrospectively selected and analyzed 81 patients who were diagnosed as APN by MDCT during evaluation of their acute abdomen in emergency room and then received DMSA scan also for the diagnostic work-up of APN after admission. We evaluated the results of imaging studies and compared the diagnostic value of each method by age groups, <2 years (n=45) and ${\geq}$2 years (n=36). Results: Among total 81 patients with MDCT-proven APN, DMSA scan was diagnostic only in 55 children (68%), while the remaining 26 children (32%) showed false negative normal findings. These 26 patients were predominantly male and most of them, 19 (73.1%) were <2 years of age. Conclusion: DMSA scan holds obvious limitation compared to MDCT in depicting acute inflammatory lesions of kidney in children with APN, especially in early childhood less than 2 years of age. MDCT showed hidden lesions of APN, those were undetectable through DMSA scan in children.