• Title/Summary/Keyword: glomerulonephritis

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Clinicopathologic Features and HBsAg and HBeAg Expressions in Hepatitis B Virus-associated Glomerulopathy (B형 간염 바이러스 감염과 연관된 사구체신염의 임상병리학적 특성과 신 조직내에서 HBs Ag 과 HBe Ag의 발현)

  • Jung Soo-Jin;Kim Young-Joo;Yoon Hye-Kyoung;Chung Woo-Yeong;Kim Young-Hoon;Kim Su-Yung
    • 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.

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A Clinicopathological Study of Rapidly Progressive Glomerulonephritis in Children (소아 급속 진행성 사구체 신염의 임상-병리학적 고찰)

  • Cho Hee-Yeon;Chung Dae-Lim;Kang Ju-Hyung;Ha Il-Soo;Choi Yong;Cheong Hae-Il
    • Childhood Kidney Diseases
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    • v.8 no.2
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    • pp.176-185
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    • 2004
  • Purpose: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. Methods: The clinical manifestations and pathological findings were reviewed retrospectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. Results: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glomerular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. Conclusion: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.

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Clinical Patterns of Acute Poststreptococcal Glomerulonephritis: A Single Center's Experience (단일 기관 내의 급성 연쇄구균감염후 사구체신염 발생의 임상적 고찰)

  • Yu, Ri-Ta;Park, Se-Jin;Shin, Jae-Il;Kim, Kee-Hyuck
    • Childhood Kidney Diseases
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    • v.15 no.1
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    • pp.49-57
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    • 2011
  • Purpose: The aim of this study is to investigate the change in incidence and clinical characteristics of acute poststreptococcal glomerulonephritis (APSGN) through a single center's experience. Methods: We retrospectively analyzed the medical records of 53 children who were diagnosed with glomerular nephritis at National Health Insurance Corporation Ilsan Hospital between March 2000 and December 2009. Twenty-six of 53 patients falled to meet the dragnostic criteria of APSGN and were excluded. Chief complaints, physical examinations, urinalysis, urine culture, laboratory results, chest x-ray, and clinical manifestations were all retrieved from the remaining 27 patients. Results: The incidence of APSGN by age was highest in children between six to seven years of age, with similar distributions in both genders, and seasonal incidence was higher from November to February when compared to other months, which was similar to the previously reported studies. However, the annual number of APSGN patients did not show a significant decrease from 2000 to 2009. The incidences of edema and hypertension were 59.3% and 48.1% respectively, suggesting lower incidences than those from previous studies. Six patients (22.2%) experienced hematuria for more than 6 months. Hypertensive encephalopathy, one of APSGN critical complications occurred in one patient but resolved with conservative treatment. Conclusion: Our study shows that APSGN still occur as a common renal disease but the disease severity seems to decrease with milder clinical manifestations and less complications compared to past studies.

A Case of the Membranous Glomerulonephritis Associated with Squamous Cell Lung Cancer (편평상피세포 폐암과 동반된 막성사구체신염 1예)

  • Cho, You-Lee;Seo, Young-Jin;Kim, Moo-Gon;Lee, Jhun-Yeob;Jung, Seung-Hie;Ryoo, Hun-Mo;Yeun, Hyen-Dae;Lee, In-Hee;Ahn, Ki-Sung;Sin, Tae-Lim;Hyun, Dae-Sung;Lee, Sang-Chae;Cho, Chang-Ho
    • Tuberculosis and Respiratory Diseases
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    • v.51 no.2
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    • pp.178-183
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    • 2001
  • The nephrotic syndrome that occurs in the absence of renal vein thrombosis, amyloidosis, neoplastic infiltration of the kidneys is an unusual but a well recognized paraneoplastic syndrome. The most frequently reported neoplasms associated with nephrotic syndrome are Hodgkin's disease and various carcinomas. The most common renal lesions are membranous glomerulonephritis(MGN) associated with carcinomas and minimal change lesions associated with Hodgkin's disease. Approximately 40% to 45% of patients clinically manifest the MGN symptoms prior to the diagnosis of the tumor, 40% simultaneously with the tumor and the remaining 15% to 20% following the tumor. Therefore, evaluating the underlying malignancy in patients with MGN is important. Here we report a patient with squamous cell lung cancer, which was detected 12 months after a MGN had been diagnosed, with a review of the relevant literature.

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Case of Combined Acute Poststreptococcal Glomerulonephritis and Acute Invasive Meningococcal Disease (A군 연구균 감염 후 급성 사구체 신염과 동반된 침습성 수막구균감염증 1례)

  • Jeon, Hosang;Hahn, Wonho;Park, Dongkyun;Cha, Sungho
    • Pediatric Infection and Vaccine
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    • v.11 no.1
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    • pp.126-130
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    • 2004
  • Neisseria meningitidis is a leading cause of meningitis and septicemia in children and young adults. Meningococcemia, the most severe form of infection, represents a part of the various spectrum of the illness, and its clinical manifestations varied from mild fever and occult bacteriemia to fulminant catastrophic events(e.g., sepsis, shock, and death) within a few hours after onset of symptoms. Complement deficiencies, either congenital or acqired, increase the risk for invasive meningococcal disease. Since C3 plays a critical role in the complement cascade, congenital C3 deficiency or conditions that decrease C3(e.g., systemic lupus erythematosus, cirrhosis, nephritis, C3 nephritic factor) increase the risk for invasive disease due to pyogenic bacteria including N. meningitidis. We had experienced a case of meningococcemia and meningococcal meningitis presenting with mild fever, petechiae, and purpura. The patient was a 7-year old male. He had mild fever, vomiting, headache, nuchal rigidity, petechiae and purpura on both lower extremities, which spreaded to the whole body. His blood culture grew Neisseria meningitidis. The diagnosis of meningococcal meningitis was confirmed by examination of cerebrospinal fluid. The clinical symptoms of the illness were improved after treatment of antibiotics(cefotaxime) for 12 days. However the patient had developed generalized edema, microscopic hematuria & proteinuria on the third day after admission. High ASO titer and low serum C3 level were also noted. The microscopic hematuria returned to normal about 2 months after discharge. The C3 level returned to normal range about 3~4 months after discharge. we report a case of combined acute poststreptococcal glomerulonephritis and acute invasive meningococcal disease with reference review.

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A Case of Membranoproliferative Glomerulonephritis Associated with Complement Deficiency and Meningococcal Meningitis (수막구균 뇌수막염과 보체 결핍이 동반된 막증식성 사구체신염 1례)

  • Kwon Sang-Mi;Park Kwan-Kyu;Lee Gyeong-Hoon
    • Childhood Kidney Diseases
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    • v.10 no.1
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    • pp.45-51
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    • 2006
  • Hypocomplementemia is found in all types of membranoproliferative glomerulonephritis (MPGN) but not in all patients. Hypocomplementemia can be ascribed to at least two circulating complement reactive modalities. The activation of the classical pathway produced by circulating immune complexes and the presence in the blood of anticomplement autoantibodies, called 'nephritic factor'(NF). The activation of the classical pathway by circulating immune complexes is probably the major mechanism responsible for hypocomplementemia in idiopathic MPGN type I. Nephritic factors have been shown to be responsible for the hypocomplementemia in both MPGN type II and III. NFa is probably the major mechanism responsible for the hypocomplementemia of idiopathic MPGN type II. NFt appears to be solely responsible for the hypocomplementemia in MPGN type III. Judging from the complement profile, NFt also may be present in some patients with MPGN type I. Although infection by meningococcus has been associated with deficiency of any of the plasmatic proteins of complement, it more commonly involves deficiency of the terminal components of the complement pathway(C5-C9). We experienced a patient who had MPGN and meningococcal meningitis. We examined the complement level and significantly lower levels of C3, C5 were found persistently. C7 was low at first and it returned to normal range after 2 months. C9 was normal at first, and was low after 2 months. This is the first reported case in which MPGN with meningococcal meningitis occurred.

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A Case of Membranoproliferative Glomerulonephritis Type II(Dense-Deposit Disease) (막증식성 사구체신염 제 II 형(Dense-Deposit Disease) 1례)

  • Lee Suk-Jin;Moon Jae-Hoon;Kang Mi-Seon;Song Min-Seob;Chung Woo-Yeong
    • Childhood Kidney Diseases
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    • v.7 no.2
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    • pp.204-210
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    • 2003
  • Membranoproliferative glomerulonephritis type II(MPGN II), also called dense deposit disease, was first described by Berger and Galle in 1963. The diagnosis of MPGN II is based on electron-microscopic finding of an intensely electron-dense substance which replaces the lamina densa of the glomerular basement membrane. Although the etiology and pathogenesis of MPGN II are unknown, it frequently progresses to end-stage renal failure. Typically in MPGN II, hypocomplementemia due to activation of the alternative complement pathway is present. In addition, the association of MPGN II with partial lipodystrophy and complement abnormalities is well documented. The relationship between these associated features and the patient's renal functional outcome is not clear. With respect to the therapy for MPGN II, an alternate-day prednisolone regimen was shown to be effective. Various treatment modalities, including immunosuppression with corticosteroids, cytotoxic drugs and cyclosporin A, anticoagulants and antiplatelet therapies are used, either alone or in combination, with varying degrees of success. The purpose of this paper is to present a case of MPGN II from a 7 years old girl with paroxysmal supraventricular tachycardia(PSVT).

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Pathological observations of rabbit hemorrhagic disease (토끼 출혈병의 병리학적 관찰)

  • 문운경;하준일;인민권;김순복;곽수동
    • Korean Journal of Veterinary Service
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    • v.23 no.4
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    • pp.309-312
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    • 2000
  • Pathological findings of natural cases of rabbit hemorrhagic disease was investigated. Clinically inappentence, increase in body temperature, depression, bloody foam from nostrils, and sudden death were recognized. Characteristic anatomical findings were hemorrhages in the lungs, liver, kidneys, and heart. Intestinal catarrh and retention of turbid urine in urinary bladder were also often observed. Severe .necrosis of hepatic cells, massive hemorrhages in many organs and membranous glomerulonephritis with hyaline droplet formation were characteristic changes under the microscopy. Perivascular cuffing of brain and catarrhal enteritis were also seen in many cases.

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Osteopontin and Developing Kidney (Osteopontin과 신장 발달)

  • Yim Hyung-Eun;Yoo Kee-Hwan
    • Childhood Kidney Diseases
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    • v.10 no.1
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    • pp.1-7
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    • 2006
  • Osteopontin (OPN) is a glycosylated phosphoprotein which mediates cell adhesion and migration, and is produced by bone, macrophages, endothelial cells, and epithelial cells. The many regulatory functions of OPN include bone remodeling, tumor invasion, wound repair, and promotion of cell survival. It is produced by renal tubular epithelial cells, and expression is upregulated in glomerulonephritis, hypertension, ischemic acute renal failure, renal ablation, and UUO. In this review, we discuss about osteopontin in general aspect, expression, role on the development and pathologic condition of neonatal kidney.

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Clinical and Pathological Findings of Renal Biopsy in Children: Outcomes from a Single Center Over 27 Years

  • Lee, Shin Ae;Kim, Min Sun;Kim, Soon Chul;Lee, Dae-Yeol
    • Childhood Kidney Diseases
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    • v.21 no.1
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    • pp.8-14
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    • 2017
  • Purpose: To classify the results of renal biopsy in pediatric patients and to compare pathological findings with clinical features. Methods: This study included data of 318 children who underwent renal biopsy at our hospital between December 1987 and November 2014. Biopsy specimens were examined histopathologically using light, immunofluorescence, and electron microscopy. Results: Asymptomatic urinary abnormalities was the most common clinical diagnosis (35.9%), followed by nephrotic syndrome (29.3%), and acute glomerulonephritis (18.0%). Glomerular disease was identified in 98.1% of the renal biopsy specimens. The most common primary cause of glomerulonephritis was IgA nephropathy, with gross hematuria in 61.9% of the patients, hypertension in 14.2%, proteinuria >1.0 gm/24-hr in 33.3%, and impaired renal function in 3.6% patients. Conclusion: The most common clinical diagnosis was asymptomatic urinary abnormalities, with primary glomerular disease being the most common renal biopsy finding, and IgA nephropathy the most common histopathological lesion. This study provides a 27-year overview of pediatric renal disease at our center and underlines the importance of renal biopsy for accurate diagnosis and proper management.