• 학교보건
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• s.26
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• pp.31-34
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• 2006

사구체 질환들은 주로 면역학적 기전에 의해 일어나며 그밖에 대사장애, 혈역학, 독성, 침착, 감염, 유전성 등의 비 면역학적 기전으로도 일어난다.

• 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)

• Pediatric Infection and Vaccine
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• v.26 no.2
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• pp.118-123
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• 2019

Postinfectious glomerulonephritis (PIGN) is most commonly caused by Streptococcus pyogenes in children, but PIGN associated with other pathogens has been described in the literature. A previously healthy 6-year-old boy was admitted with complaints of cough, fever, and right chest pain. The patient was diagnosed with pneumococcal bacteremia and influenza A virus infection and treated with antibiotics and antiviral agent. During hospitalization, generalized edema, hematuria, proteinuria, and increased blood pressure were observed; therefore, we started administering diuretics. The boy was discharged with gross hematuria, and even microscopic hematuria disappeared 14 weeks after discharge. We report a case of PIGN associated with bacteremic pneumococcal pneumonia and influenza A virus infection in children. A urine test and blood pressure measurement should be considered for the early detection of PIGN in children with pneumococcal or influenza A virus infection when they present with nephritic symptoms.

• Clinical and Experimental Pediatrics
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• v.48 no.11
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• pp.1201-1205
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• 2005

Purpose : The purpose of this study is to evaluate the clinical characteristic changes of acute poststeptococcal glomerulonephritis(APSGN) in recent. The cases of APSGN occurred for the last ten years were divided into group I and II by the unit of 5 years and clinical manifestations and laboratory findings of each groups were analyzed. Methods : Total of 74 cases of APSGN, diagnosed at the department of Pediatrics, Chungnam National University Hospital from January 1995 to August 2004 were divided into group I(31 cases from January 1995 to December 1999) & II(43 cases from January 2000 to August 2004). In addition, the clinical records of each group were reviewed retrospectively and compared. Results : In both groups, the prevalence rate was much higher in males than females, the seasonal prevalence rate was higher in winter, the upper respiratory infection was most frequent predisposing illness, and one to two weeks of latent period was the highest. However, the average age for group II were younger than group I. The prevalence rate of gross hematuria was higher in group II, but there was no significant difference between two groups. In the course of clinical symptoms, edema and hypertension were relieved within two weeks in both groups. In group II, the prevalence rate of proteinuria was higher, and the duration of proteinuria and gross hematuria were longer than group I. Conclusion : In this comparative study, the average age became younger. Clinically the duration of gross hematuria and proteinuria became longer, and the prevalence rate of proteinuria increased in recent 5 years. However, there was no difference between two groups in clinical courses and prognosis.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.50-59
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• 1998

Purpose : Hepatitis B virus (HBV) infection has been involved in several forms of immune-related glomerulopathy but the pathogenic role of HBV infection is not clear. To evaluate the clinicopathological features of HBV-associated glomerulopathy, a clinicopathological analysis and immunohistochemical stain for HBs Ag and HBe Ag were done. Methods : Clinicopathological features of HBV-associated glomerulopathy were analyzed with renal biopsies in 28 HBsAg seropositive patients from April 1990 to February 1997 at Pusan Paik Hospital, and immunohistochemical evaluation for HBsAg and HBeAg was done in renal tissues. Light microscopic, immunofluorescent and electron microscopic examination and immunohistochemical staining for HBsAg (DAKO) and HBeAg (BIONIKE) of renal tissue were performed. Result ; 1. The age distribution was 6 to 73 years old, and eight were children and 20 were adults. Male : female ratio was 3:1. Nineteen (67.9%) and 21 (75.0%) of 28 cases showed hematuria and proteinuria, respectively at the time of biopsy. Sixteen (57.2%) of them had nephrotic syndrome. 2. Liver function test was performed in 11 patients and seven (63.6%) of them showed increased AST and ALT levels. Liver biopsy was done in three patients and revealed findings of chronic active hepatitis. 3. HBV-associated glomerulopathy was membranous glomerulonephritis (MGN) in 10 (35.7%), mesangiopathy in 8 (28.6%), membranoproliferative glomerulonephritis (MPGN) in 7 (25.0%) and minimal change disease in 3 (10.7%) out of 28 cases. 4. Ultrastructurally HBV-associated MGN showed conspicuous subepithelial deposits with intramembranous, mesangial and subendothelial deposits and proliferation of mesangial cells and matrix, which were suggestive of MPGN. In HBV-associated MPGN, intramembranous and subepithelial deposits were scattered. 5. Immunohistochemical staining revealed no expression for HBsAg, but positive reaction for HBeAg along capillary wall in 8 cases (28.6%), of which 3 cases were checked for serum HBeAg, all showed positivity. Conclusion : HBV-associated glomerulopathy showed a wide morphologic spectrum and overlapping ultrastructural features in MGN and MPGN, and the activity of hepatitis B virus may be related to the development of HBV-associated glomerulopathy but further studies are recommended to confirm this relationship.

• The Journal of the Korean dental association
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• v.9 no.11
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• pp.665-671
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• 1971

• 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.8 no.2
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• pp.159-165
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• 2004

Purpose: This study aimed to compare the clinical outcome of acute poststreptocaccal glomerulonephritis (APSGN) between patients who had presented with gross hematuria and those with microscopic hematuria. Methods: Thirty-nine patients with acute poststreptococcal glomerulonephritis, who were diagnosed from January 2000 to April 2003 were enrolled. Results: The mean age was 8.85 years and the male to female ratio was 1.1:1 Seventeen patients presented with gross hematuria at diagnosis(group A) and twenty-two patients had microscopic hematuria only(group B). There were no significant differences between the two groups in the incidence of edema, fever or history of respiratory infection and oliguria. But hypertension was more frequent in group B. Laboratory data showed decreased C3 and C4 level in group B. Spot urine protein/creatinine ratio and 24hr urine protein showed higher levels in group A. Conclusion; Patients with gross hematuria at diagnosis had lower incidence of hypertension and a higher rate of nephrotic range of proteinuna than patients with microscopic hematuria. However, no difference in the duration of admission or complication rate was observed. All patients had clinical improvement during follow-up. We conclude that gross hematuria is not a significant prognostic factor for poststreptococcal glomerulonephritis.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.32-41
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• 2010

Purpose : Glomerulonephropathy (GN) often manifests as proteinuria and progresses to chronic renal failure without specific therapy. Mesenchymal stem cell (MSC) has been tried as a therapeutic agent in experimental GN, and previous studies showed that administration of MSC concomitantly to the insult inducing GN or via intra-renal administration ameliorated proteinuria. The purpose of this study was to test the therapeutic potential of MSC administered via intravenous route at the time of clinically evident proteinuria. Methods : MSCs were administered intravenously via tail vain into the mice with adriamycin (ADR) induced nephropathy (ADR-GN), two weeks after ADR injection when massive proteinuria was evident. To test the capacity of MSC modulate the cytokine production in the inflammatory milieu, the concentrations of IFN-$\gamma$ and IL-10 were measured in the supernatant of in vitro mixed lymphocyte culture (MLC) with or without additional MSC. Results : MSCs administered intravenously into the proteinuric mice with ADR-GN accelerated the recovery of this experimental GN with disappearance of proteinuria in two weeks when the saline treated (control) mice still showed significant proteinuria. The mice treated with MSC also had a tendency of better survival. Addition of MSC decreased IFN-$\gamma$ and increased IL-10 in the supernatant of MLC. Conclusion : This study showed that MSC had a therapeutic potential even when administered in a more clinically relevant setting into a proteinuric glomerulonephropathy model. Further study to verify the mechanism and long-term safety of this phenomenon is required.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.239-243
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• 2004

Acute poststreptococcal glomerulonephritis(APSGN) is the most common form of postinfectious glomerulonephritis, and acute pyelonephritis(APN) is the most severe form of urinary tract infection in childhood. However, the concurrence of two diseases is uncommon in the literature. We describe a case of APSGN accompanied with APN in a 5-year-old female who presented with fever, left flank pain, headache and facial edema. Urinalysis showed pyuria, microscopic hematuria, and mild proteinulra. Serial urine cultures grew Escherichia coli. ${^99m}$Tc-DMSA renal scan revealed a cortical defect in the upper pole of left kidney. She had a history of preceding pharyngitis, in addition, showed high blood pressure, high anti-streptolysin 0 titer, and low serum complement levels. The patient improved completely with supportive treatment, Including antibiotic and antihypertensive therapy. These findings suggested that APSGN and APN could be manifested simultaneously or be .superimposed on each other.