Humans ; Glomerulonephritis, Membranoproliferative/pathology* ; Fibronectins

Glomerulonephritis, Membranoproliferative ; Humans ; Kidney Glomerulus ; pathology

OBJECTIVETo analyze the pathological and clinical features of IgA nephropathy (IgAN) in west Guangdong province.

METHODSThe pathological type and clinical features of 120 patients with IgAN were retrospectively analyzed.

RESULTSMesangial proliferative glomerulonephritis and focal segmental glomerulosclerosis were the most frequent features of IgAN. IgM deposit could be found in half of the IgAN patients, especially in the IgAN patients with focal segmental glomerulosclerosis.

CONCLUSIONThe incidence of IgAN may vary between different regions. Clinically, misdiagnosis of other renal diseases as IgAN may often occur. The nature and severity of glomerular immunoglobulin deposition can be related to the pathogenesis and progression of IgAN.

Adult ; China ; Female ; Glomerulonephritis, IGA ; diagnosis ; pathology ; Glomerulonephritis, Membranoproliferative ; pathology ; Glomerulosclerosis, Focal Segmental ; pathology ; Humans ; Male ; Retrospective Studies ; Young Adult

OBJECTIVETo analysis the clinical and pathological characteristics of children with dense deposit disease (DDD).

METHODS12 Children diagnosed as DDD by electron microscope were enrolled in this study. The clinical and pathological data were analyzed.

RESULTSOf the 12 cases, 7 were males and 5 females, mean age 9.1 +/- 3.9 (5-13) years at onset, the duration from onset to renal biopsy was 1 month to 5 years and the follow-up period was 1-9 years. All cases had heavy proteinuria >50 mg/(kg x d), and persistent microscopic hematuria with recurrent gross hematuria during the course. Seven cases had hypertension (> or = 140/100 mm Hg, 1 mm Hg =0. 133 kPa), 5 cases had transient or recurrent abnormal renal function, and mild to severe anemia were observed in 8 cases respectively. All the cases had lower serum C3 (0.15-0.55 g/L). Clinically, 10 cases were diagnosed as nephritic syndrome (one case had partial lipodystrophy at the same time), and 2 cases were diagnosed as acute nephritic syndrome. Immunofluorescence study showed intense deposition of C3 along GBM, TBM and the wall of Bowman's capsule in a ribbon-like pattern and in the mesangial regions as coarse granules in all the cases. Under light microscopy, 9 cases showed the feature of membrane proliferative glomerulonephritis (MPGN), 1 case with focal segmental glomerulosclerosis (FSGS), 1 case with endocapillary proliferative glomerulonephritis (EnPGN) and 1 case with proliferative sclerosis (PSGN). Crescents were seen in 3 cases. Under electron microscopy, ribbon-like or linear electron-dense intramembranous deposits were identified in the lamina dense of GBM, and often along TBM and the wall of Bowman's capsule. All patients showed steroid resistance. After methylprednisone treatment, some patients showed transient remission. During the follow- up stage of 1-9 years, 3 cases showed normal urinalysis, 5 cases showed partial remission, 2 cases progressed to end stage renal disease (ESRD) and 2 cases were lost.

CONCLUSIONDDD is an in dependently rare disease with pathological-clinical varieties. Children with DDD presented with persistently lower C3, heavy proteinuria, recurrent gross hematuria and anemia. The characteristic immunopathologic finding is intense deposition of C3 along the GBM. Under electron microscopy, ribbon-like or linear electron-dense deposits in the lamina dense of the GBM, TBM and the wall of Bowman's capsule. Electron microscopic examination to demonstrate the intramembranous dense deposits is definitive diagnosis, regardless of the finding of light microscopy. All of them showed steroid resistant. Patients with steroid and CTX treatment showed some clinical improvement of their urinalysis.

Adolescent ; Child ; Child, Preschool ; Female ; Glomerular Basement Membrane ; pathology ; Glomerulonephritis, Membranoproliferative ; diagnosis ; pathology ; therapy ; Humans ; Male

PURPOSE: There have been numerous researches on urine beta(2)-microglobulin (beta(2)-M) concerned with primary nephrotic syndrome and other glomerular diseases, but not much has been done in relation to pediatric age groups. Thus, our hospital decided to study the relations between the analysis of the test results we have conducted on pediatric patients and renal functions. METHODS: Retrospective data analysis was done to 102 patients of ages 0 to 4 with renal diseases with symptoms such as hematuria, edema, and proteinuria who were admitted to Chung-Ang Yongsan Hospital and who participated in 24-hour urine and urine beta(2)-M excretion test between January of 2003 and January of 2006. Each disease was differentiated as independent variables, and the statistical difference of the results of urine beta(2)-M excretion of several groups of renal diseases was analyzed with student T-test by using test results as dependent variables. RESULTS: Levels of urine beta(2)-M excretion of the 102 patients were as follows:52 had primary nephrotic syndrome [MCNS (n=45, 72+/-45 microgram/g creatinine, microgram/g-Cr), MPGN (n=3, 154+/-415 microgram/g-Cr), FSGS (n=4, 188+/-46 microgram/-Cr)], six had APSGN (93+/-404 microgram/g-Cr), seven had IgA nephropathy (3,414+/-106 microgram/g-Cr), 9 had APN (742+/-160 microgram/g-Cr), 16 had cystitis (179+/-168 microgram/g-Cr), and 12 had HSP nephritis (109+/-898 microgram/g-Cr). IgA nephropathy (P<0.05) and APN (P<0.05) were significantly higher than in other renal diseases. Among primary nephrotic syndrome, FSGS with higher results of beta(2)-microglobulin test had longer treatment period (P<0.01) when compared to the lower groups, but no significant differences in Ccr, BUN, or Cr were observed. CONCLUSIONS: IgA nephropathy and APN groups showed significantly higher level of beta(2)-M excretion value than other groups. Although beta(2)-microglobulin value is not appropriate as an indicator of general renal function and pathology, it seems to be sufficient in the differential diagnosis of the UTI and in the prediction of the treat-ment period of nephrotic syndrome patients.
Creatinine ; Cystitis ; Diagnosis, Differential ; Edema ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Hematuria ; Humans ; Nephritis ; Nephrotic Syndrome ; Pathology ; Proteinuria ; Retrospective Studies ; Statistics as Topic

C3 glomerulopathy is a renal disorder involving dysregulation of alternative pathway complement activation. In most instances, a membranoproliferative pattern of glomerular injury with a prevalence of C3 deposition is observed by immunofluorescence microscopy. Dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are subclasses of C3 glomerulopathy that are distinguishable by electron microscopy. Highly electron-dense transformation of glomerular basement membrane is characteristic of DDD. C3GN should be differentiated from post-infectious glomerulonephritis and other immune complex-mediated glomerulonephritides showing C3 deposits.
Complement Activation ; Complement Pathway, Alternative ; Dichlorodiphenyldichloroethane ; Glomerular Basement Membrane ; Glomerulonephritis ; Glomerulonephritis, Membranoproliferative ; Microscopy, Electron ; Microscopy, Fluorescence ; Pathology ; Prevalence

Animals ; Biopsy ; Disease Models, Animal ; Fluorescent Antibody Technique ; Glomerulonephritis, Membranoproliferative ; pathology ; Humans ; Kidney ; pathology ; Kidney Diseases ; pathology ; Plastic Embedding

OBJECTIVE: To discuss the pathologic features, treatment and prognosis of the children with isolated proteinuria (IP). METHODS: Twenty-one children with IP were enrolled according to their renal biopsy and were followed up for 0.5 to 10 years. RESULTS: Renal biopsy was performed in all children. Among them 13 were mesangial proliferation glomerulonephritis (MsPGN) (including 3 minor, 6 moderate, and 4 severe ones), 2 minimal change nephritis (MCN), 3 IgA nephropathy (IgAN) (1 in Grade I and 2 in Grade II), 2 focal segmemtal glomerulosclerosis (FSGS) and 1 endocapillary proliferative glomerulonephritis (EnPGN). Interstitial changes could be found in MsPGN and FSGS mostly, presenting interstitial fibrosis, infiltration of inflammatory cells, atrophy of renal tubule, and the vacuolar degeneration of epithelia. All children accepted the medical treatment except the EnPGN case. Fifteen children recovered with no relapse; proteinuria persisted in 3 severe MsPGN and FSGS cases; 2 got the impaired renal function accompanied by persistent proteinuria; and 1 had hypertension. CONCLUSION The different degrees of renal damage can be found in all IP children who have persistent proteinuria. Most patients can get good outcome after aggressive therapies. However, the prognosis of those with severe MsPGN and FSGS was not so optimistic, and some reno-protective treatments should be given to postpone the deterioration of the renal function.
Adolescent ; Biopsy, Needle ; Child ; Child, Preschool ; Female ; Glomerulonephritis, Membranoproliferative ; pathology ; Humans ; Kidney ; pathology ; Male ; Prognosis ; Proteinuria ; etiology ; pathology

BACKGROUND: Even though there have been many case reports on nephrotic syndrome in patients with malignancy, an overall study on malignancy- associated nephrotic syndrome is rare in Korea. The purpose of this study was to explore the clinical and pathologic findings and clinical course of malignancy-associated nephrotic syndrome. METHODS: From January, 1986 to December, 2003, the medical records of patients with nephrotic syndrome and concomitant malignancy were retrospectively reviewed. RESULTS: Forty-eight patients (2.3%) out of 2, 085 patients with nephrotic syndrome had concomitant malignant disease during the study period. The mean age of patients was 57.9+-1.6 years with sex ratio of 2.4: 1. The most common primary origin of malignancy was liver (8 patients, 16.7%) and lungs (8 patients, 16.7%), and adenocarcinoma (17 patients, 35.4 %) was the leading histologic type of malignancy. There was no significant difference in 24-hour urinary protein excretion among patients grouped by TNM stage. Percutaneous renal biopsy was performed in 26 patients (54.2%), renal pathology revealed membranous nephropathy and minimal change disease in 9 patients (34.6%) each, membranoproliferative glomerulonephritis in 6 (23.1%), and IgA nephropathy in 2 patients (7.7%). When the patients were divided into progression and remission group based on the clinical course of underlying malignancy, there were significantly more patients with improved nephrotic syndrome in the remission group than the progression group (55.0% vs. 0%, p<0.05). CONCLUSION: Malignancy should be considered as a cause of nephrotic syndrome in adults, and the treatment of underlying malignancy may affect the outcome of nephrotic syndrome in patients with malignancy.
Adenocarcinoma ; Adult ; Biopsy ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Humans ; Korea ; Liver ; Lung ; Medical Records ; Nephrosis, Lipoid ; Nephrotic Syndrome* ; Pathology ; Retrospective Studies ; Sex Ratio

Chronic HCV infection has been reported to be associated with several extrahepatic conditions such as cryoglobulinemia, lymphoma, lichen planus, porphyria cutanea tarda, autoimmune thyroiditis, and renal disease. Glomerular disease may occur in patients with chronic HCV infection. The most common patterns are membranoproliferative glomerulonephritis, and less frequently, membranous nephropathy, fibrillary glomerulonephritis and immunotactoid glomerulopathy. Few crescentic glomerulonephritis has been reported in association with HCV infection, and no case was reported in Korea yet. We experienced one case of rapidly progressive glomeruloinephritis with the pathology of crescentic glomerulonephritis complicated in membranoproliferative glomerulonephritis in a patient with HCV infection.
Cryoglobulinemia ; Glomerulonephritis* ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Hepacivirus* ; Hepatitis C* ; Hepatitis* ; Humans ; Korea ; Lichen Planus ; Lymphoma ; Pathology ; Porphyria Cutanea Tarda ; Thyroiditis, Autoimmune