Adult ; Glomerulonephritis, Membranous ; complications ; pathology ; Humans ; Hypertension, Malignant ; etiology ; Kidney ; pathology ; Male

Objective To investigate the feasibility of IgG4 as a biomarker of the activity and outcome of phospholipase A2 receptor (PLA2R)-associated membranous nephropathy (PLA2R-MN). Methods Serum and urine samples were collected from 56 patients with PLA2R-MN,13 patients with secondary membranous nephropathy (SMN),and 10 patients with primary IgA nephropathy (IgAN) when kidney biopsy was performed in the Department of Nephrology,Hangzhou Hospital of Traditional Chinese Medicine from April 2017 to January 2018.Sandwich enzyme-linked immunosorbent assay was employed to measure the serum and urinary IgG4 levels. Results The PLA2R-MN group had higher median serum IgG4/IgG ratio than the SMN group (P=0.009) and the IgAN group (P<0.001) and higher median urinary IgG4/creatinine ratio than the SMN group (P=0.008).In the patients with PLA2R-MN,the median serum IgG4/IgG ratio and urinary IgG4/creatinine ratio were significantly higher in the renal insufficiency group than in the normal renal function group (P=0.049,P=0.015).Moreover,the median serum IgG4/IgG ratio was higher in those with a serum albumin level<30 g/L than in those with a serum albumin level ≥30 g/L (P=0.005).Fifty-three patients with PLA2R-MN were followed up for at least 1 year,and the serum IgG4/IgG ratios of the patients in remission were lower than those of the patients without remission (P=0.005).The median serum IgG4/IgG ratio of 23 patients in remission decreased from 5.82% (4.54%,10.20%)(at initial enrollment) to 2.91% (2.11%,5.37%)(after 1-year follow up) in remission patients (P<0.001).The receiver operating characteristic curve showed that the patients with a serum IgG4/IgG ratio<10.24% had a higher possibility of remission (P=0.005). Conclusion Serum and urinary IgG4 levels may be an indicator of the activity in PLA2R-MN patients and thus may be a predictive biomarker of the outcomes.
Biomarkers ; Creatinine ; Glomerulonephritis, Membranous/pathology* ; Humans ; Immunoglobulin G ; Receptors, Phospholipase A2 ; Serum Albumin

OBJECTIVEHepatitis B virus-associated membranous nephropathy (HBV-MN) is a disease characterized by podocytopathy. Podocyte is a terminally differentiated cell with limited capability of proliferation. Thus, damage of podocyte might result in decreased cell number, and then lead to the development of marked proteinuria and glomerulosclerosis. The present study aimed to investigate the changes of glomerular podocyte number in the children with hepatitis B virus-associated membranous nephropathy (HBV-MN), and their significance in the pathogenesis of HBV-MN.

METHODSPodocytes were identified through specific immunohistological staining of Wilms tumor gene protein 1 (WT1), a characteristic marker for podocyte nuclei, and podocyte numerical density (Nv), mean glomerular tuft volume (V) and the podocyte number per glomerulus (Npodo) were estimated through Weibel-Gomez method in 19 children with biopsy-proven HBV-MN and 8 children with thin basement membrane disease (control group), and analyses were made for possible correlation with clinical, serological and pathological data.

RESULTSAmong the 19 cases with HBV-MN, 3 showed microvillus-like foot process of podocytes, granular degeneration of podocyte were found in 4 cases, vacuolization in 1 case and podocyte detachment in 2 cases. Nv and Npodo were significantly decreased in children with HBV-MN compared with control group (t = 12.851, P = 0.0002 and t = 6.433, P = 0.0002, respectively). Moreover, the number of podocytes decreased more significantly in patients with stronger HBsAg deposition (> ++) than those with weak HBsAg deposition (< or = ++), P = 0.004, but no significant difference was found between patients with phase III or IV of HBV-MN and those with phase Ior II in podocyte number per glomerulus (P = 0.5262) and podocyte numerical density (P = 0.3564). Podocyte numerical density decreased more significantly in patients with massive proteinuria (> or = 2 g/24 h) than those with moderate proteinuria (< 2 g/24 h), P = 0.0488. The numbers of podocyte correlated significantly with serum levels of C(3) (r = 0.548, P = 0.028), but did not correlate with serum levels of albumin (r = -0.037, P = 0.891).

CONCLUSIONAll patients with HBV-MN showed podocyte damage and decreased number per glomerulus, which may play an important role in the pathogenesis of HBV-MN in children.

Cell Count ; Child ; Glomerulonephritis ; pathology ; Glomerulonephritis, Membranous ; complications ; virology ; Hepatitis B ; complications ; pathology ; Hepatitis B virus ; Humans ; Kidney Diseases ; pathology ; Kidney Glomerulus ; pathology ; Podocytes ; pathology ; Proteinuria ; pathology

Female ; Glomerulonephritis, IGA ; metabolism ; pathology ; Glomerulonephritis, Membranous ; metabolism ; pathology ; Glomerulosclerosis, Focal Segmental ; metabolism ; pathology ; Humans ; Kidney ; metabolism ; pathology ; Male ; Renal Insufficiency, Chronic ; metabolism ; 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

Biopsy ; Endopeptidase K ; Fluorescent Antibody Technique ; Glomerulonephritis, IGA ; pathology ; Glomerulonephritis, Membranous ; pathology ; Humans ; Kidney ; pathology ; Lupus Nephritis ; pathology ; Paraffin Embedding ; Staining and Labeling

OBJECTIVETo investigate the clinicopathological feature and treatment of idiopathic membranous nephropathy (IMN) in children.

METHODA retrospective analysis of 25 cases of biopsy-proven IMN seen between January 2004 and December 2009.

RESULTThe incidence of IMN was 3.81% in all the children patients who underwent renal biopsy. Of 25 patients with IMN, nine were boys and sixteen were girls. The mean age at onset was (9.4 ± 3.4) years with a range of 2 - 14 years. Renal biopsies were performed at a median 2.5 months (range 0.4 - 11 months) after onset. The clinical manifestations included nephrotic syndrome (NS) nephritic type in 21 cases (84%) and glomerulonephritis in 4 cases. All patients presented with hematuria, and 7 had macroscopic hematuria. Hypertension was noted in 4 patients. Two patients were complicated with thrombosis. One patient was in a chronic renal insufficiency(CRI)state. According to the MN staging criteria, 21 cases were in stage II IMN (84%). Six patients showed moderate or severe tubulointerstitial lesion. Focal segmental glomerulosclerosis (FSGS) was found in two patients. Of the 22 patients with NS and nephrotic proteinuria, 21 cases were treated with prednisone initially and in 20 of them the efficacy of corticosteroid therapy was evaluated:one of them was steroid sensitive (became steroid-resistant after relapse) and all the others were steroid-resistant (95%). The subsequent treatment: eight of them were treated with prednisone followed by a taper to alternate-day therapy. Five of them had complete remission and three partial remission. Twelve cases were treated with combined therapy of prednisone and immunosuppressive agents. Of these 12 cases together with one case who received initially combined treatment with prednisone and immunosuppressive agent and one case treated with prednisone initially for five weeks then with combined therapy contained another immunosuppressive agent, totally 14 cases, 5 had complete remission, 2 partial remission, 3 did not achieve remission, and 3 had unknown response.

CONCLUSIONOf the patient cohort, the predominant presenting feature was nephrotic syndrome, and with different degree hematuria. Almost all of them were steroid resistant, but followed by a taper to alternate-day therapy, some could achieve remission. The effect of a combination of prednisone and immunosuppressive agent is needed to be further proven in children.

Adolescent ; Child ; Child, Preschool ; Female ; Glomerulonephritis, Membranous ; pathology ; therapy ; Humans ; Male ; Nephrotic Syndrome ; pathology ; therapy ; Retrospective Studies

Caspase 3 ; metabolism ; Child ; Female ; Glomerulonephritis, Membranous ; enzymology ; etiology ; pathology ; virology ; Hepatitis B ; complications ; pathology ; Hepatitis B virus ; Humans ; Male

Nephrotic syndrome has been described as one of the clinical forms of chronic graft-versus-host disease (cGVHD), but a limited number of cases have been described. We experienced a young female patient with nephrotic syndrome developed 22 months after allogeneic hematopoietic stem cell transplantation (HSCT) for severe aplastic anemia. She had been well after successful management for gut-limited cGVHD until she developed a clinical nephrotic syndrome with hypoalbuminemia of 2.0 g/dL and 24-hr urine protein of 6.88 g/dL. On physical examination and laboratory findings, there was no other evidence of cGVHD. Clinical and renal biopsy findings were consistent with cGVHD-related membranous nephropathy, and immunosuppressive agents with cyclosporine and prednisone were prescribed. After 3 month of treatment, the proteinuria decreased to normal range; and the patient from nephrotic syndrome nearly recovered. We recommend cGVHD-related glomerulonephritis should be considered in all patients with hypoalbuminemia following allogeneic HSCT, even if there is no other evidence of clinical GVHD.
Adult ; Anemia, Aplastic*/physiopathology ; Anemia, Aplastic*/therapy ; Female ; Glomerulonephritis, Membranous/etiology* ; Glomerulonephritis, Membranous/pathology ; Graft vs Host Disease/physiopathology ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Human ; Kidney Glomerulus/pathology