Introduction: Glomerular damage during Gougerot-Sjgren syndrome is much rarer than interstitial damage, and is essentially extra-membranous and membrano-proliferative glomerulonephritis. Observation: We report the cas...Introduction: Glomerular damage during Gougerot-Sjgren syndrome is much rarer than interstitial damage, and is essentially extra-membranous and membrano-proliferative glomerulonephritis. Observation: We report the case of a 44-year-old woman with primary Sjgrens syndrome, confirmed by clinical dryness syndrome, positive anti-SSA and anti-SSB antibodies, and a salivary gland biopsy revealing grade 4 lymphocytic sialadenitis according to CHISHOLMs classification. Later, the patient developed nephrotic syndrome, along with hypertension. Renal function remained normal with a creatinine level of 9.3 mg/l, and hematuria was absent. Only antinuclear antibodies tested positive, while anti-PLA2R antibodies were negative. A renal biopsy was performed, which was complicated on the same day by hemodynamic instability with hematuria. Renal CT scan with contrast injection revealed a posterior perirenal hematoma without contrast extravasation. Additionally, bilateral renal vein thrombosis was incidentally discovered, suggesting extramembranous glomerulonephritis. The patients hemodynamic status stabilized after fluid resuscitation with isotonic saline solution (0.9%), without the need for blood transfusion. Renal biopsy confirmed extramembranous glomerulonephritis with interstitial fibrosis and minimal tubular atrophy. The initial etiological assessment was negative. The patient was started on oral corticosteroids, angiotensin-converting enzyme inhibitors, and therapeutic anticoagulation for renal vein thrombosis. The patients condition improved, with the disappearance of the syndrome and spontaneous regression of the hematoma. Discussion: The association of nephrotic syndrome and renal vein thrombosis primarily suggests glomerulopathy, in particular extra-membranous glomerulonephritis. Sjgrens syndrome can be associated with extra-membranous glomerulonephritis without being its direct cause. Like, it is possible that it is a cause of glomerulonephritis, essentially extra membranous and membrano-proliferative. Conclusion: Sjgrens syndrome is generally underestimated cause of glomerulonephritis, which should be considered in cases of extra-membranous glomerulonephritis.展开更多
Dynamic changes in gut dysbiosis and metabolomic dysregulation are associated with immune-complex glomerulonephritis(ICGN).However,an in-depth study on this topic is currently lacking.Herein,we report an ICGN model to...Dynamic changes in gut dysbiosis and metabolomic dysregulation are associated with immune-complex glomerulonephritis(ICGN).However,an in-depth study on this topic is currently lacking.Herein,we report an ICGN model to address this gap.ICGN was induced via the intravenous injection of cationized bovine serum albumin(c-BSA)into Sprague-Dawley(SD)rats for two weeks,after which mycophenolate mofetil(MMF)and losartan were administered orally.Two and six weeks after ICGN establishment,fecal samples were collected and 16S ribosomal DNA(rDNA)sequencing and untargeted metabolomic were conducted.Fecal microbiota transplantation(FMT)was conducted to determine whether gut normali-zation caused by MMF and losartan contributed to their renal protective effects.A gradual decline in microbial diversity and richness was accompanied by a loss of renal function.Approximately 18 genera were found to have significantly different relative abundances between the early and later stages,and Marvinbryantia and Allobaculum were markedly upregulated in both stages.Untargeted metabolomics indicated that the tryptophan metabolism was enhanced in ICGN,characterized by the overproduction of indole and kynurenic acid,while the serotonin pathway was reduced.Administration of losartan and MMF ameliorated microbial dysbiosis and reduced the accumulation of indoxyl conjugates in feces.FMT using feces from animals administered MMF and losartan improved gut dysbiosis by decreasing the Firmicutes/Bacteroidetes(F/B)ratio but did not improve renal function.These findings indicate that ICGN induces serous gut dysbiosis,wherein an altered tryptophan metabolism may contribute to its pro-gression.MMF and losartan significantly reversed the gut microbial and metabolomic dysbiosis,which partially contributed to their renoprotective effects.展开更多
Post infectious Glomerulonephritis (PIGN) in renal allograft is a rare entity. Only a few Cases have been described in the literature. The post streptococcal glomerulonephritis is the classic example in native kidney....Post infectious Glomerulonephritis (PIGN) in renal allograft is a rare entity. Only a few Cases have been described in the literature. The post streptococcal glomerulonephritis is the classic example in native kidney. A wide variety of organism has been associated with PIGN in renal allograft such as Staphylococcus, Cytomegalovirus and Polyomavirus. We describe one case of Infection associated glomerulonephritis due to Echericha Coli, developed 5 years after kidney transplantation, in 47 years old female patient. The Clinical presentation was characterized by a peripheral edema and high blood pressure, and biological tests showed a nephrotic syndrome, an acute kidney injury, a consumption of Complement fractions. The renal biopsy revealed a diffuse endocapillary cell proliferation with preponderant deposits of C3. Total recovery was achieved 4 months after Methyprednisolone pulse and Cyclophasphamid with antimicrobial treatment.展开更多
In this editorial,we comment on the article by Meng et al published in the World Journal of Clinical Cases.We comprehensively review immunoglobulin A nephro-pathy(IgAN),including epidemiology,clinical presentation,dia...In this editorial,we comment on the article by Meng et al published in the World Journal of Clinical Cases.We comprehensively review immunoglobulin A nephro-pathy(IgAN),including epidemiology,clinical presentation,diagnosis,and management.IgAN,also known as Berger's disease,is the most frequent type of primary glomerulonephritis(GN)globally.It is mostly found among the Asian population.The presentation can be variable,from microscopic hematuria to a rapidly progressive GN.Around 50%of patients present with single or recurring episodes of gross hematuria.An upper respiratory infection and tonsillitis often precede these episodes.Around 30%of patients present microscopic hematuria with or without proteinuria,usually detected on routine examination.The diagnosis relies on having a renal biopsy for pathology and immunofluorescence microscopy.We focus on risk stratification and management of IgAN.We provide a review of all the landmark studies to date.According to the 2021 KDIGO(kidney disease:Improving Global Outcomes)guidelines,patients with non-variant form IgAN are first treated conservatively for three to six months.This approach consists of adequate blood pressure control,reduction of proteinuria with renin-angiotensin system blockade,treatment of dyslipidemia,and lifestyle modifications(weight loss,exercise,smoking cessation,and dietary sodium restrictions).Following three to six months of conservative therapy,patients are further classified as high or low risk for disease progression.High-risk patients have proteinuria≥1 g/d or<1 g/d with significant microscopic hematuria and active inflammation on kidney biopsy.Some experts consider proteinuria≥2 g/d to be very high risk.Patients with high and very high-risk profiles are treated with immunosuppressive therapy.A proteinuria level of<1 g/d and stable/im-proved renal function indicates a good treatment response for patients on immu-nosuppressive therapy.展开更多
BACKGROUND Primary immunoglobulin(Ig)-associated mesangiocapillary glomerulonephritis(Ig-MCGN)is an immune complex glomerulonephritis of unknown etiology.It is a common cause of chronic kidney disease in developing co...BACKGROUND Primary immunoglobulin(Ig)-associated mesangiocapillary glomerulonephritis(Ig-MCGN)is an immune complex glomerulonephritis of unknown etiology.It is a common cause of chronic kidney disease in developing countries.There is limited data available on renal and patient outcomes of this disease from developing countries.AIM To determine the short-term renal and patient outcomes of adults with a tissueconfirmed diagnosis of primary Ig-MCGN at a single center in Pakistan.METHODS A retrospective cohort study of adult patients was conducted on biopsy-proven Ig-MCGN cases diagnosed between 1998 and 2019 at the Sindh Institute of Urology and Transplantation,Karachi,Pakistan.Secondary causes were excluded.The primary endpoint was renal survival without end-stage kidney disease(ESKD)or mortality.The secondary endpoint was the rate of remission during the 2-year follow-up period.Survival curves were made with the use of Kaplan-Meier estimates.RESULTS A total of 163 patients were included in the study and their mean follow-up duration was 29.45 months±21.28 months.Among baseline characteristics,young age,lower estimated glomerular filtration rate,requirement of kidney replacement therapy,presence of crescents,and severity of interstitial fibrosis and tubular atrophy were found to have a significant association with renal outcomes.The renal outcomes were negatively correlated with the presence of hypertension,level of complements,and degree of proteinuria.In all,63(37.4%)patients were treated with steroids and 21(13%)received combination therapy(cyclophosphamide with steroids).At 2 years,124(76.07%)patients were in complete remission or partial remission[56(34.3%)and 68(41.71%),respectively],while 32(19.63%)patients progressed to ESKD and 7(4.29%)patients died.CONCLUSION The outcomes of primary Ig-MCGN are guarded in Pakistan and require further prospective studies to improve our understanding of this relatively common disease so that more personalized treatment approaches can be developed.展开更多
Objective:To investigate the effect of the ethanolic extract of Rosa laevigata Michx.fruit on rats with mesangial proliferative glomerulonephritis based on the NLRP3 inflammasome pathway.Methods:Thirty Wistar rats wer...Objective:To investigate the effect of the ethanolic extract of Rosa laevigata Michx.fruit on rats with mesangial proliferative glomerulonephritis based on the NLRP3 inflammasome pathway.Methods:Thirty Wistar rats were divided into three groups,a blank control group,a diabetic nephropathy(DN)model group,and an ethanolic extract intervention group,according to the random number table method,with 10 rats in each group.One day before the experiment,basic feeding was initiated for all the rats;the changes in activity and weight of each group of rats were observed and recorded after 7 d,and a rat model of renal function injury was established after 1 d.Results:Compared with the control group,the model group had significantly higher kidney/body ratio,24 h urine protein,serum creatinine(SCr),blood urea nitrogen(BUN),glomerular mesangial cell(GMC)count,and extracellular matrix(ECM)positive area ratio(P<0.05);the same indicators were significantly lower in the intervention group than in the model group(P<0.05).The NLRP3 inflammasome pathway in renal intrinsic cells was activated in the intervention group.The overactivation of NLRP3 inflammasome is known to promote interleukin(IL)-1βrelease,which was inhibited in the intervention group.Conclusion:The ethanolic extract of Rosa laevigata Michx.fruit has a protective effect on renal intrinsic cells and may be related to NLRP3 inflammasome pathway,suggesting that the fruit of Rosa laevigata Michx.has a potential role in protecting renal intrinsic cells from inflammatory damage.NLRP3 inflammasomes are involved in the development of various chronic inflammatory diseases,such as acute and chronic glomerulonephritis and renal fibrosis.展开更多
文摘Introduction: Glomerular damage during Gougerot-Sjgren syndrome is much rarer than interstitial damage, and is essentially extra-membranous and membrano-proliferative glomerulonephritis. Observation: We report the case of a 44-year-old woman with primary Sjgrens syndrome, confirmed by clinical dryness syndrome, positive anti-SSA and anti-SSB antibodies, and a salivary gland biopsy revealing grade 4 lymphocytic sialadenitis according to CHISHOLMs classification. Later, the patient developed nephrotic syndrome, along with hypertension. Renal function remained normal with a creatinine level of 9.3 mg/l, and hematuria was absent. Only antinuclear antibodies tested positive, while anti-PLA2R antibodies were negative. A renal biopsy was performed, which was complicated on the same day by hemodynamic instability with hematuria. Renal CT scan with contrast injection revealed a posterior perirenal hematoma without contrast extravasation. Additionally, bilateral renal vein thrombosis was incidentally discovered, suggesting extramembranous glomerulonephritis. The patients hemodynamic status stabilized after fluid resuscitation with isotonic saline solution (0.9%), without the need for blood transfusion. Renal biopsy confirmed extramembranous glomerulonephritis with interstitial fibrosis and minimal tubular atrophy. The initial etiological assessment was negative. The patient was started on oral corticosteroids, angiotensin-converting enzyme inhibitors, and therapeutic anticoagulation for renal vein thrombosis. The patients condition improved, with the disappearance of the syndrome and spontaneous regression of the hematoma. Discussion: The association of nephrotic syndrome and renal vein thrombosis primarily suggests glomerulopathy, in particular extra-membranous glomerulonephritis. Sjgrens syndrome can be associated with extra-membranous glomerulonephritis without being its direct cause. Like, it is possible that it is a cause of glomerulonephritis, essentially extra membranous and membrano-proliferative. Conclusion: Sjgrens syndrome is generally underestimated cause of glomerulonephritis, which should be considered in cases of extra-membranous glomerulonephritis.
基金funds by the Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences(CIFMS),China(Grant No.:2022-I2M-1e014)the National Natural Science Foundation of China(Grant No.:82293684)+1 种基金Beijing Natural Science Foundation,China(Grant No.:L232084)the National Key R&D Program of China(Grant No.:2022YFA0806400).
文摘Dynamic changes in gut dysbiosis and metabolomic dysregulation are associated with immune-complex glomerulonephritis(ICGN).However,an in-depth study on this topic is currently lacking.Herein,we report an ICGN model to address this gap.ICGN was induced via the intravenous injection of cationized bovine serum albumin(c-BSA)into Sprague-Dawley(SD)rats for two weeks,after which mycophenolate mofetil(MMF)and losartan were administered orally.Two and six weeks after ICGN establishment,fecal samples were collected and 16S ribosomal DNA(rDNA)sequencing and untargeted metabolomic were conducted.Fecal microbiota transplantation(FMT)was conducted to determine whether gut normali-zation caused by MMF and losartan contributed to their renal protective effects.A gradual decline in microbial diversity and richness was accompanied by a loss of renal function.Approximately 18 genera were found to have significantly different relative abundances between the early and later stages,and Marvinbryantia and Allobaculum were markedly upregulated in both stages.Untargeted metabolomics indicated that the tryptophan metabolism was enhanced in ICGN,characterized by the overproduction of indole and kynurenic acid,while the serotonin pathway was reduced.Administration of losartan and MMF ameliorated microbial dysbiosis and reduced the accumulation of indoxyl conjugates in feces.FMT using feces from animals administered MMF and losartan improved gut dysbiosis by decreasing the Firmicutes/Bacteroidetes(F/B)ratio but did not improve renal function.These findings indicate that ICGN induces serous gut dysbiosis,wherein an altered tryptophan metabolism may contribute to its pro-gression.MMF and losartan significantly reversed the gut microbial and metabolomic dysbiosis,which partially contributed to their renoprotective effects.
文摘Post infectious Glomerulonephritis (PIGN) in renal allograft is a rare entity. Only a few Cases have been described in the literature. The post streptococcal glomerulonephritis is the classic example in native kidney. A wide variety of organism has been associated with PIGN in renal allograft such as Staphylococcus, Cytomegalovirus and Polyomavirus. We describe one case of Infection associated glomerulonephritis due to Echericha Coli, developed 5 years after kidney transplantation, in 47 years old female patient. The Clinical presentation was characterized by a peripheral edema and high blood pressure, and biological tests showed a nephrotic syndrome, an acute kidney injury, a consumption of Complement fractions. The renal biopsy revealed a diffuse endocapillary cell proliferation with preponderant deposits of C3. Total recovery was achieved 4 months after Methyprednisolone pulse and Cyclophasphamid with antimicrobial treatment.
文摘In this editorial,we comment on the article by Meng et al published in the World Journal of Clinical Cases.We comprehensively review immunoglobulin A nephro-pathy(IgAN),including epidemiology,clinical presentation,diagnosis,and management.IgAN,also known as Berger's disease,is the most frequent type of primary glomerulonephritis(GN)globally.It is mostly found among the Asian population.The presentation can be variable,from microscopic hematuria to a rapidly progressive GN.Around 50%of patients present with single or recurring episodes of gross hematuria.An upper respiratory infection and tonsillitis often precede these episodes.Around 30%of patients present microscopic hematuria with or without proteinuria,usually detected on routine examination.The diagnosis relies on having a renal biopsy for pathology and immunofluorescence microscopy.We focus on risk stratification and management of IgAN.We provide a review of all the landmark studies to date.According to the 2021 KDIGO(kidney disease:Improving Global Outcomes)guidelines,patients with non-variant form IgAN are first treated conservatively for three to six months.This approach consists of adequate blood pressure control,reduction of proteinuria with renin-angiotensin system blockade,treatment of dyslipidemia,and lifestyle modifications(weight loss,exercise,smoking cessation,and dietary sodium restrictions).Following three to six months of conservative therapy,patients are further classified as high or low risk for disease progression.High-risk patients have proteinuria≥1 g/d or<1 g/d with significant microscopic hematuria and active inflammation on kidney biopsy.Some experts consider proteinuria≥2 g/d to be very high risk.Patients with high and very high-risk profiles are treated with immunosuppressive therapy.A proteinuria level of<1 g/d and stable/im-proved renal function indicates a good treatment response for patients on immu-nosuppressive therapy.
文摘BACKGROUND Primary immunoglobulin(Ig)-associated mesangiocapillary glomerulonephritis(Ig-MCGN)is an immune complex glomerulonephritis of unknown etiology.It is a common cause of chronic kidney disease in developing countries.There is limited data available on renal and patient outcomes of this disease from developing countries.AIM To determine the short-term renal and patient outcomes of adults with a tissueconfirmed diagnosis of primary Ig-MCGN at a single center in Pakistan.METHODS A retrospective cohort study of adult patients was conducted on biopsy-proven Ig-MCGN cases diagnosed between 1998 and 2019 at the Sindh Institute of Urology and Transplantation,Karachi,Pakistan.Secondary causes were excluded.The primary endpoint was renal survival without end-stage kidney disease(ESKD)or mortality.The secondary endpoint was the rate of remission during the 2-year follow-up period.Survival curves were made with the use of Kaplan-Meier estimates.RESULTS A total of 163 patients were included in the study and their mean follow-up duration was 29.45 months±21.28 months.Among baseline characteristics,young age,lower estimated glomerular filtration rate,requirement of kidney replacement therapy,presence of crescents,and severity of interstitial fibrosis and tubular atrophy were found to have a significant association with renal outcomes.The renal outcomes were negatively correlated with the presence of hypertension,level of complements,and degree of proteinuria.In all,63(37.4%)patients were treated with steroids and 21(13%)received combination therapy(cyclophosphamide with steroids).At 2 years,124(76.07%)patients were in complete remission or partial remission[56(34.3%)and 68(41.71%),respectively],while 32(19.63%)patients progressed to ESKD and 7(4.29%)patients died.CONCLUSION The outcomes of primary Ig-MCGN are guarded in Pakistan and require further prospective studies to improve our understanding of this relatively common disease so that more personalized treatment approaches can be developed.
基金This work was supported by the Health Commission of Hebei Province under the project Chuanxiong Extract Improves Inflammatory Response in Rats with Pyelonephritis Through IL-6/STAT3 Signaling Pathway(Project Number:20231486).
文摘Objective:To investigate the effect of the ethanolic extract of Rosa laevigata Michx.fruit on rats with mesangial proliferative glomerulonephritis based on the NLRP3 inflammasome pathway.Methods:Thirty Wistar rats were divided into three groups,a blank control group,a diabetic nephropathy(DN)model group,and an ethanolic extract intervention group,according to the random number table method,with 10 rats in each group.One day before the experiment,basic feeding was initiated for all the rats;the changes in activity and weight of each group of rats were observed and recorded after 7 d,and a rat model of renal function injury was established after 1 d.Results:Compared with the control group,the model group had significantly higher kidney/body ratio,24 h urine protein,serum creatinine(SCr),blood urea nitrogen(BUN),glomerular mesangial cell(GMC)count,and extracellular matrix(ECM)positive area ratio(P<0.05);the same indicators were significantly lower in the intervention group than in the model group(P<0.05).The NLRP3 inflammasome pathway in renal intrinsic cells was activated in the intervention group.The overactivation of NLRP3 inflammasome is known to promote interleukin(IL)-1βrelease,which was inhibited in the intervention group.Conclusion:The ethanolic extract of Rosa laevigata Michx.fruit has a protective effect on renal intrinsic cells and may be related to NLRP3 inflammasome pathway,suggesting that the fruit of Rosa laevigata Michx.has a potential role in protecting renal intrinsic cells from inflammatory damage.NLRP3 inflammasomes are involved in the development of various chronic inflammatory diseases,such as acute and chronic glomerulonephritis and renal fibrosis.
