• Korean Journal of Veterinary Service
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• v.19 no.3
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• pp.227-237
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• 1996

In order to investigate the prevalence and pattern of renal lesions in sows, 250 kidneys collected from abattoir were examined grossly and histopathologically. The prevalence of renal lesions in sows was 46.8% (l17/250). Main gross findings were consisted of congestion and/or petechiation (21.6%), cortical enlargement (15.2%), renal cysts (6.0%), abscessation (4.4%), and infarction (1.5%). Principle microscopic lesions were composed of interstitial nephritis (25.6% ), glomerulosclerosis (13.6%), glomerular thrombosis (3.6%), amyloidosis (2.0%) and glomerulosclerosis (2.0%) Sixty four kidneys with interstitial nephritis was classified by 46 chronic and 18 acute cases. Among 34 kidneys with glomerulonephritis, there were divided into 18 membranous type, 9 proliferative type and 7 membranoproliferative type. For these results, it was confirmed that sows raised in Kwangju and Chonnam areas had been affected by a variety of pathological renal lesions.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1104-1109
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• 2007

Purpose : Since 1998, school urinary screening tests have been performed on Korean school children. We could detect and treat so many asymptomatic chronic renal disease in early stage. We investigated the efficacy of school urinary screening tests from children with membranoproliferative glomerulonephritis (MPGN) type I. Methods : We analyzed the characteristics and prognosis of 18 patients with MPGN type I who admitted after 1996 and received steroid therapy with or without cyclosporine. These patients were divided into two groups. Group A (asymptomatic patients detected by school urinary screening tests) consisted of 7 patients; Group S (symptomatic patients) consisted 11 patients. Results : Mean follow-up duration was 6.3 years (from 2 to 11 years). Urinary protein excretion was 1.1 g/day in group A and 6.6 g/day in group S. 24 hour creatinine clearance (mL/min/$1.73m^2$) was 134.3 in group A and 82.3 in group S. No patients in group A had renal insufficiency, but three patients in group S had renal insufficiency and one patient required peritoneal dialysis. Conclusion : Early detection by school urinary screening tests improves prognosis of MPGN type I.

• 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.5 no.2
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• pp.188-195
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• 2001

Type II membranoproliferative glomerulonephritis (Dense deposit disease) is an acquired primary glomerular disease characterized by electron microscopic evidence of a continuous dense membrane deposition replacing the lamina densa. It is a subtype of idiopathic membra- noproliferative glomerulonephritis, and was described as a separate entity by Berger and Galle in 1963. It frequently occurs in older chilren and young adults and the clinical course is variable, but is generally progressive. The presenting feature is nephrotic syndrome in many patients, and proteinuria and hematuria are also seen frequently. The purpose of this paper is to present a case of DDD (Dense deposit disease) from a 10 year old boy who was diagnosed as a acute poststreptococcal glomurulonephritis with protenuria, hematuria, and facial edema by renal biopsy 4 years ago. (J, Korean Soc Pediatr Nephrol 2001 ; 5 : 188-95)

• Clinical and Experimental Pediatrics
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• v.53 no.7
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• pp.766-769
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• 2010

Dense deposit disease (DDD) is a rare disorder characterized by the deposition of abnormal electron-dense material within the glomerular basement membrane of the kidneys. The diagnosis is made in most patients between 5 and 15 years of age, and within 10 years, approximately half of the affected patients progress to end-stage renal disease. We report a rare case of regressive DDD without C3 deposition after steroid therapy in an 11-year-old boy. The patient presented with edema, gross hematuria, and nephrotic-range proteinuria. Laboratory testing revealed a serum creatinine level of 1.17 mg/dL, albumin level of 2.3 g/dL, and serum C3 level of 125 mg/dL (range 90-180 mg/dL). The results of the renal biopsy were consistent with DDD without C3 deposition. After 6 weeks of steroid therapy, the nephrotic syndrome completely resolved. The follow-up renal biopsy showed a significant reduction in mesangial proliferation and disappearance of electron-dense deposits in the GBM.

• Korean Journal of Veterinary Research
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• v.29 no.2
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• pp.99-108
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• 1989

In order to observe the pathological changes of kidney in rabbits infected with the viral haemorrhagic disease, the kidney tissues from the 91 rabbits infected with the viral haemorrhagic disease were examined by light and electron microscopy. The results observed were as follows: 1. On light microscopic observation, the kidney lesions were identified as haemorrhagic glomerular necrosis(33.0%), membranous glomerulonephritis(20.9%), thrombotic glomerulopathy(19.8%), membranoproliferative glomerulonephritis(8.8%), mesangial proliferative glomerulonephritis(8.8%) ischemic acute tubular necrosis(7.7%), and acute serous glomerulitis(6.6%). 2. On electron microscopic observation, cytoplasmic degeneration of mesangial cells, and irregular thickening of basement membranes with electron dense granular materials were observed. In podocytes swelling of mitochondria, dilatation of endoplasmic reticulum and extensive fusion of foot processes were also observed. Nonenveloped round icosahedral picornaviral particles with a diameter of 28~33nm were detected in the cytoplasm of degenerative endothelial cells, polymorphonuclear leucoytes, and monocytes.

• Childhood Kidney Diseases
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• v.22 no.1
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• pp.17-21
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• 2018

Thymic stromal lymphopoietin (TSLP) is an interleukin-7-like cytokine that is an important trigger and initiator of many allergic diseases. TSLP promotes a T-helper type 2 (Th2) cytokine response that can be pathological. A relationship is formed both at the induction phase of the Th2 response through polarization of dendritic cells to drive Th2 cell differentiation and at the effector phase of the response, by promoting the expansion of activated T cells and their secretion of Th2 cytokines and TSLP. In transgenic mice with TSLP overexpression, it has been reported that TSLP leads to the development of mixed cryoglobulinemic membranoproliferative glomerulonephritis. In addition, TSLP can play an important role in the pathogenesis of IgA nephropathy and systemic lupus erythematosus-related nephritis. From our knowledge of the role of TSLP in the kidney, further studies including the discovery of new therapies need to be considered based on the relationship between TSLP and glomerulonephritis.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.186-194
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• 2004

Purpose: Membranoproliferative glomeulonephritis(MPGN) has been diagnosed in an increasing number of asymptomatic cases. These cases have been detected by school urinary screening test even though the total cases of MPGN show a decreasing trend. We have analyzed the clinical and pathological characteristics of children with MPGN according to the clinical manifestations at the time of disease presentation. Methods: A total of 18 patients who had been diagnosed with idiopathic MPGN by percutaneous renal biopsy from January 1990 to February 2004 were involved in our study. The patients were divided into 2 groups as the school urinary screening(A) group and the symptomatic(S) group according to the clinical manifestations at the time of disease presentation. Results: Out of the total 18 patients, 8(44.4%) were in the S group and 10(55.6%) were in the A group. The mean serum total protein, albumin and $C_3$ levels in the S group were significantly lower than those levels of the A group, respectively($4.9{\pm}1.2\;g/dL,\;vs\;7.0{\pm}0.5\;g/dL\;P=0.002,\;2.8{\pm}0.9\;g/dL\;vs.\;4.1{\pm}0.3\;g/dL\;P=0.002,\;63.9{\pm}36.4\;mg/dL\;vs.\;100.8{\pm}39.5\;g/dL\;P=0.041$). The mean total protein amount of 24 hour collected urine in the S group were significantly higher than that of the A group($3684.0{\pm}2601.3\;mg/m^2\;vs.\;559.4{\pm}4.6.9\;mg/m^2$, respectively, P=0.001). Hypocomplementemia was observed in 11(61.1%) out of 18 patients at the time of disease onset, 7(87.5%) in the S group and 4(40%) in the A group. However the hypocomplementemia was decreased in 6(33.3%) out of 18 patients at the time of final follow-up, 3(37.5%) in the S group and 3(30%) in the A group. According to the pathologic type, hypocomplementemia was observed 8 patients(61.5%) with type I disease, 1 patients (100%) with type II disease, 2 patients(50%) in type III disease at the disease onset, but 4 patients(30.8%) in type I disease, 1 patient(100%) in type II disease, 1 patient(33.3%) with type III disease at the time of last follow-up. The incidence of cellular crescent formation and tubular atropy. as observed on light microscopy, were higher in the S group compared to the A group. Mean grade of capillary wall thickening and, mesangial proliferation were significantly higher in the S group. Conclusion: MPGN, as diagnosed in patients with only asymptomatic urinary abnormalities, has been increasing, it is more frequent in asymptomatic patients than in patients with presenting symptoms. Our result suggests that MPGN should be considered in the renal biopsy diagnosis regardless of serum $C_3$ level when urinary abnormalities are found by school urinary screening test.

• Korean Journal of Veterinary Research
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• v.40 no.3
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• pp.587-599
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• 2000

This study was conducted to observe the severity of the disease and pathogenecity of Babesia gibsoni parasite on the splenectomized dogs(SPD) and nonsplenectomized(intact) dogs (NSPD) experimentally infected with B gibsoni. The average prepatent period was 4 days in the SPD and 8 days in the NSPD, respectively. Peak parasitaemia(PE) ranged from 26% to 34% of erythrocytes infected in the SPD and from 4% to 5% in the NSPD. Latent parasitaemia was still detectable 40 days as low as under 1.0% of erythrocytes infected after the initial parasitaemia in the SPD. Blood packed cell volume(PCV) decreased to as little as 6.4% to 6.9% in the SPD. The clinical signs were mild fever and anemia in the NSPD, remissions and exacervations of temperature, intermittent or spike-like increases of temperature, progressive polychromatophilic macrocytic anemia with anisocytosis, icterus, marked loss of appetite, rarely haemoglobinuria, and deep brown-yellowish urine in the SPD. Gross pathologic changes mainly involved slightly enlargement of liver and spleen in the NSPD and marked enlargement of liver in the SPD. Anatomic changes associated with the disease included diffuse periportal and centrilobular hephatitis, and membranoproliferative glomerulonephritis. Hyaline droplets, resulting protein metabolic alterations, were found in the convoluted ephithelium of the kidney. The density of lymphocytes within the liver sinusoids was markedly increased. Aggregates of large monocytes and macrophages were demonstrated in the centrilobular veins of the liver. The density of these cells in the centrilobular veins were greatest in the SPD. The forms of B gibsoni parasite found in the acute stage of SPD were large signet ring form, small signet ring form, pyriform, elongated form, comma form, head-phone form, oval form, peared form, racket-like form, amoeboid form, triangle form, quartered form, dot form, band form and multiple, and rosette form, et al. The severity of the disease and pathogenecity of B gibsoni parasite were mild in the NSPD but fatal in the SPD.

• Childhood Kidney Diseases
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• v.26 no.1
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• pp.40-45
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• 2022

Purpose: Chronic kidney disease (CKD) has various underlying causes in children. Identification of the underlying causes of CKD is important. Genetic causes comprise a significant proportion of pediatric CKD cases. Methods: In this study, we performed whole-exome sequencing (WES) to identify genetic causes of pediatric CKD. From January to June 2021, WES was performed using samples from pediatric patients with CKD of unclear etiology. Results: Genetic causes were investigated using WES in 37 patients (17 males) with pediatric CKD stages 1 (n=5), 2 (n=7), 3 (n=2), 4 (n=2), and 5 (n=21). The underlying diseases were focal segmental glomerulosclerosis (n=9), congenital anomalies of the kidney and urinary tract including reflux nephropathy (n=8), other glomerulopathies (n=7), unknown etiology (n=6), and others (n=7). WES identified genetic causes of CKD in 12 of the 37 patients (32.4%). Genetic defects were discovered in the COL4A4 (n=2), WT1 (n=2), ACTN4, CEP290, COL4A3, CUBN, GATA3, LAMA5, NUP107, and PAX2 genes. WT1 defects were found in patients whose pathologic diagnosis was membranoproliferative glomerulonephritis, and identification of CUBN defects led to discontinuation of immunosuppressive agents. Genetic diagnosis confirmed the clinical diagnosis of hypoparathyroidism, sensorineural deafness, and renal disease; Alport syndrome; and Joubert syndrome in three of the patients with CKD of unknown etiology (COL4A4 [n=2], CUBN [n=1]). Extrarenal symptoms were considered phenotypic presentations of WT1, PAX2, and CEP290 defects. Conclusions: WES provided a genetic diagnosis that confirmed the clinical diagnosis in a significant proportion (32.4%) of patients with pediatric CKD.