• Childhood Kidney Diseases
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• v.9 no.2
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• pp.231-236
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• 2005

Juvenile rheumatoid arthritis(JRA) is the most common major connective tissue disease in children. Renal involvement in JRA is rare. Among the renal lesions that have been reported in JRA, amyloidosis and drug-induced nephropathy are the most common. Crescentic glomerulonephritis in JRA has rarely been reported. We report a case of ANCA-associated pauci-immune crescentic glomerulonephritis in JRA. The patient was a 15-year old boy with a 3-year history of JRA. He presented with gross hematuria, proteinuria, positive p-ANCA and elevation of BUN and creatinine. Pathologic findings revealed focal necrotizing and crescentic glomerulonephritis. There were no significant immunoglobulin or complement deposits. His renal function recovered after intravenous methylprednisolone pulse therapy and oral steroid use. In Korea, this is the first reported case of pauci-immune crescentic glomerulonephritis in JRA. (J Korean Soc Pediatr Nephrol 2005;9:231-236)

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.154-160
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• 2000

The pathogenesis of IgA nephropathy and acute poststreptococcal glomerulonephritis is not fully understood. In the past, acute poststreptococcal glumerulonephritis was the most common cause of gross hematuria in children, but now IgA nephropathy is the most common one. We experienced two cases of acute poststreptococcal glomerulonephritis superimposing to IgA nephropathy in boys Case 1 had upper respiratory infection before elevation of anti-streptolysin O, generalized edema, gross hematuria and proteinuria. The complement levels were normal. Electron microscopic findings of renal biopsy at ten days after onset showed a few big subepithelial 'humps' and localized heavy subendothelial and mesangial deposits. Immunofluoroscopic findings revealed predominant IgA deposition in the mesangium. The electron microscopic findings were diagnostic of acute poststreptococcal glomerulonephritis On the other hand, immunoflorescence microscopic findings were compatible to IgA nephropathy. In case 2, the renal biopsy which was done 2 years after onset showed only finding of IgA nephropathy. To our knowledges, there has been kw reports of acute poststreptococcal glomerulonephritis superimposing to IgA nephropathy which was confirmed by renal biopsy. We report two cases of acute poststreptococcal glomerulonephritis superimposing: to IgA nephropathy with a brief review of the literatures.

• 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.

• Journal of Yeungnam Medical Science
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• v.30 no.2
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• pp.149-151
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• 2013

Kimura's disease is an angiolymphoid-proliferative disorder that manifests with benign subcutaneous swelling predominantly in the head and the neck. Kidney involvement, including proteinuria, occurs in 12-16% of patients with the disease, and 60-78% of such cases is nephrotic syndrome. Reported etiologies of nephrotic syndrome in Kimura's disease include membranous glomerulonephritis, mesangial proliferative glomerulonephritis, minimal-change disease, focal segmental glomerulosclerosis, diffuse proliferative glomerulonephritis and immunoglobulin A (IgA) nephropathy. There have been only two case reports of IgA nephropathy in Kimura's disease, in 1998. In this report, we present a third case of IgA nephropathy associated with Kimura's disease.

• Tuberculosis and Respiratory Diseases
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• v.52 no.6
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• pp.633-639
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• 2002

Paraneoplastic nephrotic syndrome can be diagnosed from its clinical and immunological features. The development of several types of glomerular injury in patients with cancer have been recognized, and are considered as paraneoplastic syndrome. Most prominent are the occurrence of membranous glomerulonephritis in patients with carcinomas. We report a case of a 60-year-old-man with small cell lung cancer presenting as nephrotic syndrome. A renal biopsy revealed membranous glomerulonephritis. Six lots of chemotherapy were administered, which led to a complete tumor response with total resolution of the nephrotic syndrome following treatment.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.81-84
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• 2005

Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis that can affect multiple organs predominantly the skin, joints, gastrointestinal tract and kidney. Although the specific pathogenesis of HSP is not known, there are several hypotheses. Although the importance of the complement activation in glomerular injury in HSP has been suggested, the complement levels and the blood pressure in those patients are usually normal and massive proteinuria is not common. And pathologic renal changes also have been reported to show a large variety of glomerular changes. However, to our knowledge, a membranoproliferative glomerulo-nephritis (MPGN) is a rare renal clinicopathologic manifestation of HSP. We report a 6-year-old boy with HSP who developed MPGN with hypertension, massive proteinuria, and hypo-complementemia revealed activation of the classical complement pathway, although we could not exclude the possibility of other hypocomplementemic glomerulonephritis including post-streptococcal acute glomerulonephritis.

• Journal of Yeungnam Medical Science
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• v.3 no.1
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• pp.325-332
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• 1986

Since Combes' first description of hepatitis B virus associated membranous glomerulonephritis, many reports have shown a strong association of hepatitis B virus infection with various types of nephropathies, especially membranous glomerulonephritis and membranoproliferative glomerulonephritis. Recently, the authors experienced a case of membraoproliferative glomerulonephritis, type I in a 16-year-old male patient with persistent HBs and HBe antigenemia. One year prior to admission the patient was told of hepatitis at routine check, but he remained asymptomatic throughout. He was admitted to our hospital with chief complaints of proteinuria and microscopic hematuria found on routine urinalysis. Liver and kidney biopsy revealed chronic persistent hepatitis and membranoproliferative glomerulonephritis type I, respectively. We report a case of hepatitis B virus associated nephropathy with review of the literatures. Considering the endemic nature of hepatitis B virus infection in Korea, the incidence of hepatitis B viurs associated nephropathy would be proportionally high in comparison with those of other countries. So, the importance of this entity merits special consideration in our country. Further study concerning pathogenesis, epidemiology, and treatment may be needed.

• Childhood Kidney Diseases
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• v.17 no.1
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• pp.1-5
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• 2013

C3 glomerulonephritis (C3GN) is a recently described entity that shows a glomerulonephritis on light microscopy, bright C3 staining and the absence of C1q, C4, and immunoglobulins on immunofluorescence microscopy and mesangial and/or subendothelial electron-dense deposits on electron microscopy. The term 'C3 glomerulopathy' is often used to include C3GN and dense deposit disease (DDD), CFHR5 nephropathy, those of which result from dysregulation of the alternative pathway of complement. C3GN shares some aspects of atypical hemolytic uremic syndrome, MPGN, late stage of post infectious glomerulonephritis and other glomerulonephrtis. When C3GN is considered, measurement of serum complement proteins including C3, CFH, CFI, CFB and testing for the presence of C3 nephritic factor, anti-factor H autoantibodies are necessary. To screening for mutations, genes that encode complement regulators should be evaluated. This disorder equally affected all ages, both genders, and typically presented with hematuria and proteinuria. In both the short and long term, renal function remained stable in the majority of patients.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.192-195
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• 1998

[ $\beta$ ]-streptococci have been reported as an etiology of Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP), but the role of $\beta$-streptococci in HSP is not yet confirmed. Our report of two HSP patients is as follows : Although the two patients' clinical symptoms resembled those symptoms of HSP, whereas serum $C_3$ decreased in one case, ASO titer increased in both cases and a histological examination of renal biopsy specimens revealed the fetures commonly described in poststreptococcal glomerulonephritis.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.73-78
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• 1997

The hypertensive encephalopathy is one of sudden convulsive cause in patients with acute post-streptococcal glomerulonephritis, but commonly, after the episode we can observe only mild to moderately hypertension in APSGN at ER. So these remains some question of what causes the convulsion in patients with APSGN. In this article, we report 3 cases of convulsion in APSGN with only moderate hypertension, the etiology of which proved to be due to hypertensive encephalopathy by the brain MRI. We believe that the brain MRI is a very useful test to diagnose the etiology of convulsion in APSGN and if brain edema in occipital lobe is detected by brain MRI, the convulsion would be due to hypertensive encephalopathy and the prognosis will be very good.