Here two cases of renal failure occurring in pregnancy were reported,with biopsy-proven pauci-immune crescentic glomerulonephritis at postpartum.It is most rare that pauci-immune crescentic glomerulonephritis as the c...Here two cases of renal failure occurring in pregnancy were reported,with biopsy-proven pauci-immune crescentic glomerulonephritis at postpartum.It is most rare that pauci-immune crescentic glomerulonephritis as the cause of renal failure in pregnancy.Their renal function could remain stable for some time due to timely and appropriate treatment.Therefore,the pregnant and maternal who have the performance of renal impairment should be closely tracked their blood pressure,urinary protein and renal function,if necessary,execute renal biopsy,for a clear and accurate pathological diagnosis is of great significance to grasp the opportunity of treatment.But the long-term prognosis is still poor.展开更多
BACKGROUND Post-streptococcal acute glomerular nephritis(PSAGN)is mostly a benign condition.The usual sequelae of PSAGN include hypertension,its complications,and acute kidney injury.Severe PSAGN is associated with si...BACKGROUND Post-streptococcal acute glomerular nephritis(PSAGN)is mostly a benign condition.The usual sequelae of PSAGN include hypertension,its complications,and acute kidney injury.Severe PSAGN is associated with significant long-term morbidity,and histological abnormalities such as crescentic glomerulonephritis are infrequently reported.PSAGN has also been linked to late-onset chronic kidney disease in some populations due to high levels of proteinuria.METHODS This prospective observational study was conducted at Lady Ridgeway Hospital(Colombo,Sri Lanka)over 15 months.Children with PSAGN were enrolled based on clinical and laboratory criteria.Persistent proteinuria≥2+for 2 weeks and serum creatinine>100μmol/L warranted renal biopsy,assessed via light microscopy and immunofluorescence.Normalization of complement 3(C3)within 6 to 8 weeks was required for inclusion.Data on clinical features,urine protein levels,and renal function were collected from patient records,and potential associations were analysed using Statistical Package for the Social Sciences and R language for statistical computing.Ethical approval was obtained from the Ethical Review Committee,Lady Ridgeway Hospital for Children(Ref No:LRH/ERC/2021/60).RESULTS Forty-four patients were recruited.There were 27(61.4%)male patients and 17(38.6%)female patients.Thirty-seven(84%)of them were above 5 years of age.Twenty(45%)patients had a history of skin sepsis,and eighteen(41%)had a history of throat infection.Among patients with proteinuria≥2+,53%had serum creatinine>100µmol/L,while among those with proteinuria<2+,7%had serum creatinine>100µmol/L.The association of high-degree proteinuria with elevated serum creatinine was significant(χ²=7.8,P=0.005)in PSAGN.The odds ratio of the logistic regression model was 1.049(95%confidence interval:1.003-1.098),indicating a positive direction with statistically significant association(P=0.037).There was no significant association between proteinuria and the degree of hypertension or estimated creatinine clearance.Ten children underwent renal biopsy.Crescents(less than 50%)were demonstrated in five children,while three children had typical diffuse proliferative glomer-ulonephritis.One child had severe acute tubular necrosis,and another had crescentic glomerulonephritis(crescents>50%).The immunofluorescence studies revealed deposition of immunoglobulin G and C3 in all biopsy specimens.CONCLUSION High-degree proteinuria was significantly associated with elevated serum creatinine(>100μmol/L)in children with PSAGN.The majority of children with persistent proteinuria≥2+for more than 2 weeks and the highest recorded serum creatinine>100μmol/L had atypical renal histological findings.展开更多
Antineutrophil cytoplasmic antibodies(ANCA) are well known to be associated with several types of vasculitis, including pauci-immune crescentic glomerulonephritis, a form of rapid progressive glomerular nephritis(RPGN...Antineutrophil cytoplasmic antibodies(ANCA) are well known to be associated with several types of vasculitis, including pauci-immune crescentic glomerulonephritis, a form of rapid progressive glomerular nephritis(RPGN). ANCA vasculitis has also been reported after administration of propylthiouracil, hydralazine, cocaine(adulterated with levimasole), allopurinol, penicillamine and few other drugs. All previously reported cases of drug-associated ANCA glomerulonephritis were in native kidneys. Sofosbuvir is a new and effective drug for hepatitis C virus infection. Here, we report a case of ANCA vasculitis and RPGN following sofosbuvir administration in a kidney transplant recipient. It also represents the first case of drug-associated ANCA vasculitis in a transplanted kidney. Further drug monitoring is necessary to elucidate the degree of association and possible causal effect of sofosbuvir and perinuclear ANCA vasculitis.展开更多
A case of rapidly progressive glomerulonephritis with pathological changes to the crescent glomerulonephritis“C3 glomerulonephritis”in the Third Affiliated Hospital of Inner Mongolia Medical University was collected...A case of rapidly progressive glomerulonephritis with pathological changes to the crescent glomerulonephritis“C3 glomerulonephritis”in the Third Affiliated Hospital of Inner Mongolia Medical University was collected and its diagnosis,clinical manifestation,and pathological characteristics were explored.The experts analyzed the disease from the perspective of its causes,diagnosis,complications and treatment.Timely renal biopsy is required to clarify pathological diagnosis as the primary glomerular disease was newly recognized with a lower incidence.So misdiagnosis and delayed healing is more frequent.The paper aims to enhance the clinician’s understanding of“C3 glomerulonephritis”.展开更多
Objective To study the significance and distribution of renal infiltrating cells, including monocytes (CD68+ cells), proliferative cell nuclear antigen positive cells (PCNA+ cells), CD4+ and CD8+ cells in diffuse cres...Objective To study the significance and distribution of renal infiltrating cells, including monocytes (CD68+ cells), proliferative cell nuclear antigen positive cells (PCNA+ cells), CD4+ and CD8+ cells in diffuse crescent glomerulonephritis (DCGN). Methods Fifty-six patients with DCGN were studied, including 10 cases of anti-glomerular basement membrane (GBM)-type Ⅰ DCGN, 26 immune complex-type Ⅱ DCGN, and 20 cases of pauci-immune-type Ⅲ DCGN. Glomerular and interstitial infiltrates of CD68+ and PCNA+ cells, and interstitial infiltrates of CD4+ and CD8+ cells were detected by using four-layer PAP methods. Results There was a significant increase of renal infiltrating CD68+, PCNA+, CD4+ and CD8+ cells in patients with DCGN compared with that in normal controls. In patients with type Ⅰ DCGN, there was a higher number of renal infiltrating CD68+ and PCNA+ cells than that in patients with type Ⅱ and Ⅲ DCGN. A glomerular infiltrates of CD68+ and PCNA+ cells correlated with the interstitial infiltrates of CD4+ cells in type Ⅰ or Ⅲ DCGN patients. In lupus DCGN patients, the numbers of renal infiltrating CD68+ and PCNA+ cells were similar to vasculitis or type Ⅲ DCGN patients.Conclusion These findings demonstrate that the renal infiltrates of CD68+ and PCNA+ cells play an important role in patients with DCGN, that the infiltrates of CD4+ cells correlate with the infiltrates of CD68+ and PCNA+ cells may be an active marker of DCGN, and that cell-mediated immunity may contribute to crescent formation in lupus DCGN patients.展开更多
Infective endocarditis (IE) is associated with poor prognosis and higher mortality. IE-induced glomerulonephritis, especially crescentic glomerulonephritis (IE-CGN) with renal dysfunction, is an independent risk f...Infective endocarditis (IE) is associated with poor prognosis and higher mortality. IE-induced glomerulonephritis, especially crescentic glomerulonephritis (IE-CGN) with renal dysfunction, is an independent risk factor for mortality For the treatment of IE-CGN, many therapeutic strategies including antibiotics alone, antibiotics combined with corticosteroid, immunosuppressive agents, plasmapheresis, or cardiac surgery have been applied and obtained various results.C21 Here, we reported a case of CGN with progressive renal failure secondary to IE in which the renal function was dramatically improved by the treatment of surgical valve replacement and antibiotics.展开更多
Objective To investigate retrospectively the incidence, distribution of primary disease and clinicopathologic characteristics of diffuse crescentic glomerulonephritis (DCGN) in Chinese patients.Methods One hundred and...Objective To investigate retrospectively the incidence, distribution of primary disease and clinicopathologic characteristics of diffuse crescentic glomerulonephritis (DCGN) in Chinese patients.Methods One hundred and seventy-two consecutive patients diagnosed as having DCGN out of 9828 cases of non-transplanting renal biopsies over sixteen years, were studied. DCGN is categorized into three types according to immunopathologic characteristics. The incidence of this disease, its primary diseases, clinical characteristics and serum antineutrophil cytoplasmic antibodies (ANCAs) were analyzed.Results The distribution of patients among the three classifications was 8. 7% type Ⅰ , 68. 6% type Ⅱ and 22. 7% type Ⅲ. Clinically, the majority of patients (69.8%) presented rapidly progressive glomerulonephritis (RPGN), but 30. 2% manifested a chronic nephritic syndrome or chronic renal failure. In terms of related conditions, 93% were anemic, 61. 6% had hypertension, 50. 6% oliguria, 45.3% nephrotic syndrome, 43% uremic syndrome and 39.5% displayed gross hematuria. Those patients who were positive in serum for ANCAs had predominantly type Ⅲ DCGN. Two cases with anti-GBM-antibody crescentic glomerulonephritis and three with lupus nephritis were also positive for ANCAs in serum.Conclusion DCGN is not rare in Chinese patients. A majority of patients in our study presented with RPGN, but 30. 2% manifested a chronic renal failure. Lupus patients with DCGN that were positive for ANCAs had more severe vasculitic lesions.展开更多
Objective To evaluate the significance of serum anti neutrophil cytoplasmic antibodies (ANCAs) and the effects of immune suppressive treatments on its activity in patients with pauci immune crescentic glomeruloneph...Objective To evaluate the significance of serum anti neutrophil cytoplasmic antibodies (ANCAs) and the effects of immune suppressive treatments on its activity in patients with pauci immune crescentic glomerulonephritis (PICGN) Methods Serum ANCAs and myeloperoxidase (MPO) ANCA were detected by indirect immunofluorescence and enzyme linked immuno^sorbent assay (ELISA) methods respectively, and the renal tissues infiltrating cells including CD4 +, CD8 +, CD68 + and PCNA + cells were determined by four layer peroxidase antiperoxidase (PAP) method The clinical manifestations, pathologic features and immune pathologic changes in patients with positive ANCAs were compared with that in patients with negative ANCAs The effects of immune suppressive therapy on clinic and pathologic changes as well as ANCAs activity were also investigated in ANCAs positive and ANCAs negative patients Results Both of clinic active manifestations such as the degree of hematuria, rapidly progressive renal failure and pathologic active features including segmental capillary necrosis and vasculitis were much common in patients with positive ANCAs as compared with that in patients with negative ANCAs The number of infiltrating cells in renal tissue, especially CD4 + cells, was markedly higher in ANCAs positive patients than that in ANCAs negative patients The effects of immune suppressive therapy were also much better in patients with positive ANCAs than that in patients with negative ANCAs Conclusion Serum ANCAs is not only a marker for diagnosis of systemic vasculitis, but also a sensitive predictor for evaluation of diseases' activity and treatment in patients with PICGN The good effect of immune suppressive treatments on patients with PICGN is partially associated with the degree of ANCAs activity展开更多
文摘Here two cases of renal failure occurring in pregnancy were reported,with biopsy-proven pauci-immune crescentic glomerulonephritis at postpartum.It is most rare that pauci-immune crescentic glomerulonephritis as the cause of renal failure in pregnancy.Their renal function could remain stable for some time due to timely and appropriate treatment.Therefore,the pregnant and maternal who have the performance of renal impairment should be closely tracked their blood pressure,urinary protein and renal function,if necessary,execute renal biopsy,for a clear and accurate pathological diagnosis is of great significance to grasp the opportunity of treatment.But the long-term prognosis is still poor.
文摘BACKGROUND Post-streptococcal acute glomerular nephritis(PSAGN)is mostly a benign condition.The usual sequelae of PSAGN include hypertension,its complications,and acute kidney injury.Severe PSAGN is associated with significant long-term morbidity,and histological abnormalities such as crescentic glomerulonephritis are infrequently reported.PSAGN has also been linked to late-onset chronic kidney disease in some populations due to high levels of proteinuria.METHODS This prospective observational study was conducted at Lady Ridgeway Hospital(Colombo,Sri Lanka)over 15 months.Children with PSAGN were enrolled based on clinical and laboratory criteria.Persistent proteinuria≥2+for 2 weeks and serum creatinine>100μmol/L warranted renal biopsy,assessed via light microscopy and immunofluorescence.Normalization of complement 3(C3)within 6 to 8 weeks was required for inclusion.Data on clinical features,urine protein levels,and renal function were collected from patient records,and potential associations were analysed using Statistical Package for the Social Sciences and R language for statistical computing.Ethical approval was obtained from the Ethical Review Committee,Lady Ridgeway Hospital for Children(Ref No:LRH/ERC/2021/60).RESULTS Forty-four patients were recruited.There were 27(61.4%)male patients and 17(38.6%)female patients.Thirty-seven(84%)of them were above 5 years of age.Twenty(45%)patients had a history of skin sepsis,and eighteen(41%)had a history of throat infection.Among patients with proteinuria≥2+,53%had serum creatinine>100µmol/L,while among those with proteinuria<2+,7%had serum creatinine>100µmol/L.The association of high-degree proteinuria with elevated serum creatinine was significant(χ²=7.8,P=0.005)in PSAGN.The odds ratio of the logistic regression model was 1.049(95%confidence interval:1.003-1.098),indicating a positive direction with statistically significant association(P=0.037).There was no significant association between proteinuria and the degree of hypertension or estimated creatinine clearance.Ten children underwent renal biopsy.Crescents(less than 50%)were demonstrated in five children,while three children had typical diffuse proliferative glomer-ulonephritis.One child had severe acute tubular necrosis,and another had crescentic glomerulonephritis(crescents>50%).The immunofluorescence studies revealed deposition of immunoglobulin G and C3 in all biopsy specimens.CONCLUSION High-degree proteinuria was significantly associated with elevated serum creatinine(>100μmol/L)in children with PSAGN.The majority of children with persistent proteinuria≥2+for more than 2 weeks and the highest recorded serum creatinine>100μmol/L had atypical renal histological findings.
文摘Antineutrophil cytoplasmic antibodies(ANCA) are well known to be associated with several types of vasculitis, including pauci-immune crescentic glomerulonephritis, a form of rapid progressive glomerular nephritis(RPGN). ANCA vasculitis has also been reported after administration of propylthiouracil, hydralazine, cocaine(adulterated with levimasole), allopurinol, penicillamine and few other drugs. All previously reported cases of drug-associated ANCA glomerulonephritis were in native kidneys. Sofosbuvir is a new and effective drug for hepatitis C virus infection. Here, we report a case of ANCA vasculitis and RPGN following sofosbuvir administration in a kidney transplant recipient. It also represents the first case of drug-associated ANCA vasculitis in a transplanted kidney. Further drug monitoring is necessary to elucidate the degree of association and possible causal effect of sofosbuvir and perinuclear ANCA vasculitis.
文摘A case of rapidly progressive glomerulonephritis with pathological changes to the crescent glomerulonephritis“C3 glomerulonephritis”in the Third Affiliated Hospital of Inner Mongolia Medical University was collected and its diagnosis,clinical manifestation,and pathological characteristics were explored.The experts analyzed the disease from the perspective of its causes,diagnosis,complications and treatment.Timely renal biopsy is required to clarify pathological diagnosis as the primary glomerular disease was newly recognized with a lower incidence.So misdiagnosis and delayed healing is more frequent.The paper aims to enhance the clinician’s understanding of“C3 glomerulonephritis”.
文摘Objective To study the significance and distribution of renal infiltrating cells, including monocytes (CD68+ cells), proliferative cell nuclear antigen positive cells (PCNA+ cells), CD4+ and CD8+ cells in diffuse crescent glomerulonephritis (DCGN). Methods Fifty-six patients with DCGN were studied, including 10 cases of anti-glomerular basement membrane (GBM)-type Ⅰ DCGN, 26 immune complex-type Ⅱ DCGN, and 20 cases of pauci-immune-type Ⅲ DCGN. Glomerular and interstitial infiltrates of CD68+ and PCNA+ cells, and interstitial infiltrates of CD4+ and CD8+ cells were detected by using four-layer PAP methods. Results There was a significant increase of renal infiltrating CD68+, PCNA+, CD4+ and CD8+ cells in patients with DCGN compared with that in normal controls. In patients with type Ⅰ DCGN, there was a higher number of renal infiltrating CD68+ and PCNA+ cells than that in patients with type Ⅱ and Ⅲ DCGN. A glomerular infiltrates of CD68+ and PCNA+ cells correlated with the interstitial infiltrates of CD4+ cells in type Ⅰ or Ⅲ DCGN patients. In lupus DCGN patients, the numbers of renal infiltrating CD68+ and PCNA+ cells were similar to vasculitis or type Ⅲ DCGN patients.Conclusion These findings demonstrate that the renal infiltrates of CD68+ and PCNA+ cells play an important role in patients with DCGN, that the infiltrates of CD4+ cells correlate with the infiltrates of CD68+ and PCNA+ cells may be an active marker of DCGN, and that cell-mediated immunity may contribute to crescent formation in lupus DCGN patients.
文摘Infective endocarditis (IE) is associated with poor prognosis and higher mortality. IE-induced glomerulonephritis, especially crescentic glomerulonephritis (IE-CGN) with renal dysfunction, is an independent risk factor for mortality For the treatment of IE-CGN, many therapeutic strategies including antibiotics alone, antibiotics combined with corticosteroid, immunosuppressive agents, plasmapheresis, or cardiac surgery have been applied and obtained various results.C21 Here, we reported a case of CGN with progressive renal failure secondary to IE in which the renal function was dramatically improved by the treatment of surgical valve replacement and antibiotics.
文摘Objective To investigate retrospectively the incidence, distribution of primary disease and clinicopathologic characteristics of diffuse crescentic glomerulonephritis (DCGN) in Chinese patients.Methods One hundred and seventy-two consecutive patients diagnosed as having DCGN out of 9828 cases of non-transplanting renal biopsies over sixteen years, were studied. DCGN is categorized into three types according to immunopathologic characteristics. The incidence of this disease, its primary diseases, clinical characteristics and serum antineutrophil cytoplasmic antibodies (ANCAs) were analyzed.Results The distribution of patients among the three classifications was 8. 7% type Ⅰ , 68. 6% type Ⅱ and 22. 7% type Ⅲ. Clinically, the majority of patients (69.8%) presented rapidly progressive glomerulonephritis (RPGN), but 30. 2% manifested a chronic nephritic syndrome or chronic renal failure. In terms of related conditions, 93% were anemic, 61. 6% had hypertension, 50. 6% oliguria, 45.3% nephrotic syndrome, 43% uremic syndrome and 39.5% displayed gross hematuria. Those patients who were positive in serum for ANCAs had predominantly type Ⅲ DCGN. Two cases with anti-GBM-antibody crescentic glomerulonephritis and three with lupus nephritis were also positive for ANCAs in serum.Conclusion DCGN is not rare in Chinese patients. A majority of patients in our study presented with RPGN, but 30. 2% manifested a chronic renal failure. Lupus patients with DCGN that were positive for ANCAs had more severe vasculitic lesions.
文摘Objective To evaluate the significance of serum anti neutrophil cytoplasmic antibodies (ANCAs) and the effects of immune suppressive treatments on its activity in patients with pauci immune crescentic glomerulonephritis (PICGN) Methods Serum ANCAs and myeloperoxidase (MPO) ANCA were detected by indirect immunofluorescence and enzyme linked immuno^sorbent assay (ELISA) methods respectively, and the renal tissues infiltrating cells including CD4 +, CD8 +, CD68 + and PCNA + cells were determined by four layer peroxidase antiperoxidase (PAP) method The clinical manifestations, pathologic features and immune pathologic changes in patients with positive ANCAs were compared with that in patients with negative ANCAs The effects of immune suppressive therapy on clinic and pathologic changes as well as ANCAs activity were also investigated in ANCAs positive and ANCAs negative patients Results Both of clinic active manifestations such as the degree of hematuria, rapidly progressive renal failure and pathologic active features including segmental capillary necrosis and vasculitis were much common in patients with positive ANCAs as compared with that in patients with negative ANCAs The number of infiltrating cells in renal tissue, especially CD4 + cells, was markedly higher in ANCAs positive patients than that in ANCAs negative patients The effects of immune suppressive therapy were also much better in patients with positive ANCAs than that in patients with negative ANCAs Conclusion Serum ANCAs is not only a marker for diagnosis of systemic vasculitis, but also a sensitive predictor for evaluation of diseases' activity and treatment in patients with PICGN The good effect of immune suppressive treatments on patients with PICGN is partially associated with the degree of ANCAs activity