Methylprednisolone pulse therapy was performed for 8 patients of childhood nephrotic syndrome who showed resistance to conventional prednisolone therapy of 4 to 8 weeks. The pathological diagnosis of the patients were: 1 case of membranous nephropathy, membrano-proliferative glomerulonephritis, Menbranoproliferative glomerulonephritis with epithelial crescent (70%), sclerosing glomerulonephritis, mesangial proliferative glomerulonephritis, focal and global glomerulonephritis, and 2 cases of focal and segmental glonerulosclerosis. Creatinine clearance was above 50% of the normal in 7 cases, and less than 20% in crescentic glomerulonephritis. 20% in crescentic glomerulonephritis. 30gm/kg/D. of methylprednisolone was administered intravenously over 1~2hours, which was repeated 2 to 9 times on every other day. Thereafter, alternate day prednisolone therapy was continued. The results were as follows: Remission was attained in membranous nephropathy and MPGN, within 9 weeks and 13 weeks respectively. Marked improvement was noted in crescentic glomerulonephritis. Ccr increased from 18.5ml/min/1.73mm(2) to 59.1ml/min/1.73mm(2) 10 days later after pulse? Sclerosing glomerulonephritis showed significant improvement in clinical finding and serum albumin. There was no improvement in mesangial proliferative glomerulonephritis, focal and global glomerulonephritis, and 2 cases of focal and segmental glomerulosclerosis. These findings suggest that methylprednisolone pulse?therapy may benefit the childhood nephrotic syndrome with resistance to conventional prednisolone therapy.
Creatinine ; Diagnosis ; Glomerulonephritis ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Humans ; Methylprednisolone ; Nephrotic Syndrome* ; Prednisolone* ; Serum Albumin

BACKGROUND: Since the introduction of cyclosporine, the short-term renal allograft survival has significantly improved. However, the long-term success is still limited by the development of chronic rejection and recurrent disease. Post-transplant glomerulonephritis (post-Tx GN) including recurrent disease is becoming an important cause of graft dysfunction. METHODS: From November 1988 to June 2004, a total of 629 renal transplants involving 588 patients were performed at our medical center. RESULTS: The prevalence rate of post-Tx GN was 11.9% in 629 renal transplant. Among 75 transplants diagnosed as post-Tx GN, IgA nephropathy (62.7%) was the most common histologic diagnosis, followed by focal segmental glomerulosclerosis (26.7 %), membranous glomerulonephritis (8.0%), membranoproliferative glomerulonephritis (1.3%) and diabetic nephropathy (1.3%). Documented histologic recurrence occurred in only 24.2% of patients with prior biopsy-proven glomerulonephritis of their native kidneys. The actuarial allograft survival at 5 and 10 years posttransplantation with post-Tx GN was 80.5 % and 27.9%, respectively; and the corresponding graft survival for patients without post-Tx GN was 74.9% and 52.3%, respectively (p<0.05). However, there was no significant difference in the graft survival according to type of post-Tx GN. The 5 and 10 year graft survival for patients with proteinuria over than 3.5 g/24 hr were 62.5% and 0%, which is significantly lower compared with 85.3% and 28.7% for patients with proteinuria less than 3.5 g/24 hr (p<0.01). CONCLUSION: In conclusion, post-Tx GN is associated with decreased long-term graft survival and nephrotic range proteinuria is most important prognostic factor for graft survival. A prospective study with rigorous efforts to make pretransplant diagnosis and standardized criteria for allograft biopsy will more accurately characterize the natural history of post-Tx GN and may provide insight regarding treatment.
Allografts* ; Biopsy ; Cyclosporine ; Diabetic Nephropathies ; Diagnosis ; Glomerulonephritis* ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Graft Survival ; Humans ; Kidney ; Natural History ; Prevalence ; Proteinuria ; Recurrence ; Transplants

Child ; Early Diagnosis ; Evidence-Based Medicine ; Glomerulonephritis ; diagnosis ; therapy ; Glomerulonephritis, Membranous ; diagnosis ; therapy ; Humans ; Kidney Diseases ; diagnosis ; therapy ; Kidney Glomerulus ; pathology ; Nephrosis, Lipoid ; diagnosis ; therapy ; Renal Replacement Therapy

PURPOSE: We examined the clinical characteristics and incidence of adults idiopathic nephrotic syndrome (NS) according to pathologic diagnosis, age, sex. METHODS: We retrospectively reviewed the clinical and pathological characteristics of primary glomerular lesions in adults idiopathic NS taken a renal biopsy from 1978 to 2005 at the Dongsan Medical Center. We compared the prevalence of adults idiopathic NS according to the pathologic diagnosis between two time intervals 1978 to 1990 and 1991 to 2005. RESULTS: The patients had mean age of 36.7+/-16.3 years and male to female ratio was 1.7:1 with male predominance. The frequency of histopathologic diagnoses were minimal change nephrotic syndrome (MCNS) 51.6%, membranous glomerulonephritis (MGN) 21.3%, focal segmental glomerulosclerosis (FSGS) 12.1%, IgA nephropathy 9.1%, membranoproliferative glomerulonephritis (MPGN) 4.2% in decreasing order of frequency. The mean age was youngest in MCNS (32.9+/-15.1) and oldest in MGN (46.2+/-16.6). Between 1978 to 1990 period and 1991 to 2005 period, the prevalence of MGN was significantly increased, whereas the prevalence of MPGN was decreased significantly. The prevalence of MCNS had a tendency to decrease and that of IgA nephropathy had a tendency to increase, however, both didn't reach statistical significance. The incidence of FSGS didn't show a significant change during the both study periods. CONCLUSION: MCNS was the most common disease among adults idiopathic NS. MGN was the most frequent etiology in patients older than 45 years. The incidence of MGN was increased over the 28-year period, and that of MPGN decreased significantly. There was no change in the frequency of FSGS.
Adult* ; Biopsy ; Diagnosis ; Female ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Humans ; Incidence ; Korea* ; Male ; Nephrosis, Lipoid ; Nephrotic Syndrome* ; Prevalence ; Retrospective Studies

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.
Amyloidosis ; Diagnosis ; Glomerulonephritis, Membranous* ; Hodgkin Disease ; Humans ; Kidney ; Lung Neoplasms* ; Lung* ; Nephrotic Syndrome ; Paraneoplastic Syndromes ; Renal Veins ; Thrombosis

BACKGROUND: The natural history of idiopathic membranous nephropathy (IMN) is heterogeneous with some patients showing spontaneous remission while others show a progressive course leading to end-stage renal failure. We tried to assess quantitatively alpha-smooth muscle actin (alpha-SMA) and vimentin expression as markers to predict the outcome of membranous nephropathy. METHODS: This study included 24 patients with biopsy proven IMN. We measured the volume of the positive area for alpha-SMA and vimentin within the glomeruli and compared the results with 5 patients in the normal control group. We evaluated glomerular alpha-SMA and vimentin expression in correlation with BUN and serum creatinine level at the time of diagnosis and after treatment. RESULTS: Glomerular alpha-SMA and vimentin in IMN were higher than in the control group. Glomerular alpha-SMA was significantly higher in progressive IMN than in non-progressive IMN. The glomerular alpha-SMA was sifnificantly correlated with BUN and serum creatinine at last follow-up (p<0.05), but there was no statistically significant correlation at diagnosis. The glomerular vimentin was not different between progressive and non-progressive groups. CONCLUSION: These data suggest that the expression of glomerular alpha-SMA may be a useful prognostic indicator and may be able to differentiate between patients with membranous nephropathy who respond well to treatment and those who continue to progress.
Actins* ; Biopsy ; Creatinine ; Diagnosis ; Follow-Up Studies ; Glomerulonephritis, Membranous* ; Humans ; Kidney Failure, Chronic ; Natural History ; Prognosis ; Remission, Spontaneous ; Vimentin*

Objective To investigate the clinical significance of thrombospondin type 1 domain-containing 7A (THSD7A) and neural epidermal growth factor-like 1 protein (NELL1) in phospholipase A2 receptor (PLA2R)-negative membranous nephropathy (MN). Methods A total of 116 PLA2R-negative MN patients treated in Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University from 2014 to 2021 were enrolled in this study.Immunohistochemistry was employed to detect THSD7A and NELL1 in the renal tissue.The pathological characteristics,treatment,and prognosis were compared between positive and negative groups. Results The 116 PLA2R-negative MN patients included 23 THSD7A-positive patients and 9 NELL1-positive patients.One patient was tested positive for both proteins.The THSD7A-positive group showed higher positive rate of IgG4 (P=0.010),more obvious glomerular basement membrane (GBM) thickening (P=0.034),and higher proportion of stage Ⅱ MN and lower proportion of stage I MN (P=0.002) than the THSD7A-negative group.The NELL1-positive group had lower positive rates of C1q and IgG2 (P=0.029,P=0.001),less obvious GBM thickening (P<0.001),more extensive inflammatory cell infiltration (P=0.033),lower proportion of deposits on multi-locations (P=0.001),and lower proportion of atypical MN (P=0.010) than the NELL1-negative group.One patient with THSD7A-positive MN was diagnosed with colon cancer,while none of the NELL1-positive patients had malignancy.Survival analysis suggested that THSD7A-positive MN had worse composite remission (either complete remission or partial remission) of nephrotic syndrome than the negative group (P=0.016),whereas NELL1-positive MN exhibited better composite remission of nephrotic syndrome than the negative group (P=0.015).The MN patients only positive for NELL1 showed better composite remission of nephrotic syndrome than the MN patients only positive for THSD7A (P<0.001). Conclusions THSD7A- and NELL1-positive MN is more likely to be primary MN,and there is no significant malignancy indication.However,it might have a predictive value for the prognosis of MN.
Humans ; Autoantibodies ; Clinical Relevance ; Colonic Neoplasms ; EGF Family of Proteins ; Glomerulonephritis, Membranous/diagnosis* ; Nephrotic Syndrome ; Receptors, Phospholipase A2/metabolism* ; Thrombospondins/metabolism*

The paraneoplastic nephrotic syndrome can be diagnosed by clinical and immunologic features. We have had a case of paraneoplastic nephrotic syndrome in the patients with aadeno-carcinoma of the lung, whose diagnosis was made by excluding other causes of nephrotic syndrome. The type of renal lesion was membranous glomerulopathy which commonly occurs in carcinoma. The quantity of proteinuria in this patient had decreased according to the improvement of lung cancer with combination chemotherapy. After fourth chemotherapy he was refractory to treatment, and unfortunately he had passed away with cardiac tamponade.
Adenocarcinoma* ; Cardiac Tamponade ; Diagnosis ; Drug Therapy ; Drug Therapy, Combination ; Glomerulonephritis, Membranous* ; Humans ; Lung Neoplasms ; Lung* ; Nephrotic Syndrome ; Paraneoplastic Syndromes ; Proteinuria

BACKGROUND: Asymptomatic urinary abnormalities are one of the most frequent abnormalities in clinical nephrology. However, there are few large-scaled studies about the clinical manifestations and the pathologic findings of the disease. The aim of present study was to evaluate the clinicopathologic nature of the patients with asymptomatic urinary abnormality proven by renal biopsy. METHODS: Between January 1998 and July 2002, two hundred and eight patients with asymptomatic urinary abnormality at three hospitals in Daegu were studied for age, sex, initial urinary findings, serum creatinine, daily urine protein and pathologic findings by renal biopsy. RESULTS: Mean age was 28.0 years (range 14-60 years) at diagnosis of 208 patients and sex ratio of male to female was 141:67. One hundred and two patients (49.0%) had hematuria and proteinuria, 94 (45.2%) had pure microscopic hematuria and the remaining 12 (5.8%) had isolated proteinuria. Pure microscopic hematuria was the dominant urinary abnormality in younger patients. In pathologic findings, 120 patients (57.7%) were IgA nephropathy, 35 (16.8%) thin glomerular basement membrane disease, 8 (3.8%) minimal change disease, 6 (2.9%) membranous glomerulonephropathy and 22 (10.6%) showed no histologic abnormality. The most common pathologic diagnosis in all three groups was IgA nephropathy. In pure microscopic hematuria group, 38 patients (40.4%) were IgA nephropathy and 27 patients (28.7%) were thin glomerular basement membrane disease. There were no significant difference in pathologic findings depending on the severity of proteinuria (p>0.05). CONCLUSION: In our study, the most common cause of asymptomatic urinary abnormalities was IgA nephropathy. In patients with pure microscopic hematuria, IgA nephropathy and thin glomerular basement membrane disease were two leading causes.
Biopsy* ; Creatinine ; Daegu ; Diagnosis ; Female ; Glomerular Basement Membrane ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranous ; Hematuria ; Humans ; Male ; Nephrology ; Nephrosis, Lipoid ; Proteinuria ; Sex Ratio

Microscopic polyangiitis (MPA) is a distinct type of systemic small vessel vasculitis affecting small sized vessels with few or no immune deposit and no granulomatous inflammation. Cause or pathogenetic mechanism of MPA has been unknown but association with silicon or silica exposure or pulmonary silicosis has been reported rarely and supports hypothesis that environmental factors are important modulating or triggering factors of the vasculitis in the indivisual who may be genetically predisposed. We report a case of microscopic polyangiitis with underlying pulmonary silicosis in 43 year-old male. He was admitted due to hemoptysis, dyspnea, fever and bilateral pulmonary infiltration with underlying small nodular densities in whole lung field and egg-shell calcification of both hilar areas. Laboratory findings showed hematuria, proteinuria and rapid deterioration of renal function. Renal biopsy revealed focal segmental necrotizing glomerulonephritis with early cellular crescents accompanied with membranous glomerulonephropathy and perinuclear-antineutrophil cytoplasmic antibody was positive. Under the diagnosis of MPA, he has been managed with high dose steroid, cyclophosphamide and hemodialysis. Chest infiltration decreased and hemoptysis and hypoxia was improved but renal function was not recoverd and he needed regular hemodialysis continuously.
Adult ; Anoxia ; Biopsy ; Cyclophosphamide ; Cytoplasm ; Diagnosis ; Dyspnea ; Fever ; Glomerulonephritis ; Glomerulonephritis, Membranous ; Hematuria ; Hemoptysis ; Humans ; Inflammation ; Lung ; Male ; Microscopic Polyangiitis* ; Proteinuria ; Renal Dialysis ; Silicon ; Silicon Dioxide ; Silicosis* ; Thorax ; Vasculitis