Nocardiosis manifests as an opportunistic infection,primarily affecting individuals who are immunocompromised and susceptible to the infection.We present a case study of one patient with nephrotic syndrome and membran...Nocardiosis manifests as an opportunistic infection,primarily affecting individuals who are immunocompromised and susceptible to the infection.We present a case study of one patient with nephrotic syndrome and membranous nephropathy,who underwent treatment with prednisone and cyclosporine in 2016.In early 2017,the patient was diagnosed with a"fungal infection"and discontinued the use of cyclosporine.After one month of anti-infection therapy,a cranial magnetic resonance imaging scan showed multiple abscesses in the right temporal region.The diagnosis of nocardiosis was confirmed based on the presence of metastatic abscess masses,multiple lung and brain lesions,and a positive culture of Nocardia in the drainage.We changed the anti-infection therapy to a combination of trimethoprim-sulfamethoxazole(TMP-SMX),minocycline,and voriconazole.However,the patient experienced a sudden cardiac arrest and subsequently recovered after cardiopulmonary resuscitation.During the five-month follow-up period following the discharge,the patient displayed an enhanced nutritional status and stable renal function.The focal infection ultimately resolved during the subsequent three years.Neuro-infection caused by Nocardia should be considered in immunocompromised patients,and TMP-SMX is the preferred initial therapy;however,because of the high mortality rate,a long-term combination therapy with imipenem,cefotaxime,amikacin,and TMP-SMX is suggested.展开更多
BACKGROUND Extragastrointestinal stromal tumors(EGIST)and gastrointestinal stromal tumors are of similar pathological type and form.Here we report a rare case of EGIST diffusely distributed in membranous tissue in abd...BACKGROUND Extragastrointestinal stromal tumors(EGIST)and gastrointestinal stromal tumors are of similar pathological type and form.Here we report a rare case of EGIST diffusely distributed in membranous tissue in abdominal cavity,the feature of which included diffuse tumors at membranous tissue in entire abdominal cavity and spontaneous bleeding of the tumors.CASE SUMMARY The patient was a 71-year man and hospitalized due to continuous pain at lower abdomen for more than 10 days.Upon physical examination,the patient had flat and tough abdomen with mild pressing pain at lower abdomen,no obvious abdominal mass was touchable,and shifting dullness was positive.Positron emission tomography-computed tomography(CT)showed that in his peritoneal cavity,there were multiple nodules of various sizes,seroperitoneum,multiple enlarged lymph nodes in abdominal/pelvic cavity and right external ilium as well as pulmonary nodules.Plain CT scanning at epigastrium/hypogastrium/pelvic cavity+enhanced three-dimensional reconstruction revealed multiple soft tissue nodules in abdominal/pelvic cavity,peritoneum and right groin.Tumor marker of carbohydrate antigen 125 was 808 U/mL,diffuse tuberous tumor was seen in abdominal/pelvic cavity during operation with hematocelia,and postoperative pathological examination confirmed EGIST.Imatinib was administered with better therapeutic effect.CONCLUSION Gene testing showed breast cancer susceptibility gene 1 interacting protein C-terminal helicase 1 and KIT genovariation,and the patient was treated with imatinib follow-up visit found that his clinical symptoms disappeared and the tumor load alleviated obviously via imageological examination.展开更多
Objective:To investigate the efficacy of rituximab in the treatment of idiopathic membranous nephropathy with varying levels of serum phospholipase A2 receptor antibodies.Methods:A total of 137 patients with idiopathi...Objective:To investigate the efficacy of rituximab in the treatment of idiopathic membranous nephropathy with varying levels of serum phospholipase A2 receptor antibodies.Methods:A total of 137 patients with idiopathic membranous nephropathy admitted to Beijing Sixth Hospital were selected.Based on their blood PLA2R antibody levels before rituximab treatment,patients were categorized into the PLA2R antibody positive group(n=94)and the PLA2R antibody negative group(n=43).They were followed up for at least 1 year,during which the efficacy,measured through 24-hour urine protein quantification and serum albumin levels,were compared between the two groups before and after treatment.Results:After 3 months of treatment,there was no significant difference in the quantitative levels of 24-hour urine protein between the two groups(P>0.05).However,after 6 and 12 months of treatment,there was a significant difference in the levels of 24-hour urine protein between the two groups(P<0.05).Additionally,after 3 months of treatment,there was a notable difference in the serum albumin levels between the two groups(P<0.05).However,after 6 and 12 months of treatment,there was no significant difference in serum albumin levels between the two groups(P>0.05).Analysis of complications in the two groups revealed that in the positive group,9 individuals experienced thrombosis,5 had infections,and 11 developed acute kidney injury(AKI).In contrast,in the negative group,5 individuals had thrombosis,2 had infections,and 3 developed AKI.There was no statistically significant difference in complications between the two groups(P>0.05).Conclusion:Serum anti-PLA2R antibody levels provide valuable insights into the clinical observation of rituximab treatment for idiopathic membranous nephropathy.They aid in understanding the disease’s pathogenesis,evaluating treatment efficacy,and predicting disease prognosis.展开更多
BACKGROUND The recognition of idiopathic membranous nephropathy(IMN)as an autoimmune disease has paved the way for the use of B-cell-depleting agents,such as Rituximab(RTX),which is now a first-line drug for treating ...BACKGROUND The recognition of idiopathic membranous nephropathy(IMN)as an autoimmune disease has paved the way for the use of B-cell-depleting agents,such as Rituximab(RTX),which is now a first-line drug for treating IMN with proven safety and efficacy.Nevertheless,the usage of RTX for the treatment of refractory IMN remains controversial and challenging.AIM To evaluate the efficacy and safety of a new low-dose RTX regimen for the treatment of patients with refractory IMN.METHODS A retrospective study was performed on refractory IMN patients that accepted a low-dose RTX regimen(RTX,200 mg,once a month for five months)in the Xiyuan Hospital of Chinese Academy of Chinese Medical Sciences’Department of Nephrology from October 2019 to December 2021.To assess the clinical and immune remission data,we performed a 24 h urinary protein quantification(UTP)test and measured the serum albumin(ALB)and serum creatinine(SCr)levels,phospholipase A2 receptor(PLA2R)antibody titer,and CD19+B-cell count every three months.RESULTS A total of nine refractory IMN patients were analyzed.During follow-up conducted twelve months later,the results from the 24 h UTP decreased from baseline[8.14±6.05 g/d to 1.24±1.34 g/d(P<0.05)]and the ALB levels increased from baseline[28.06±8.42 g/L to 40.93±5.85 g/L(P<0.01)].Notably,after administering RTX for six months,the SCr decreased from 78.13±16.49μmol/L to 109.67±40.87μmol/L(P<0.05).All of the nine patients were positive for serum anti-PLA2R at the beginning,and four patients had normal anti-PLA2R titer levels at six months.The level of CD19+B-cells decreased to 0 at three months,and CD19+B-cell count remained at 0 up until six months of follow-up.CONCLUSION Our low-dose RTX regimen appears to be a promising treatment strategy for refractory IMN.展开更多
BACKGROUND Primary membranous nephrotic syndrome with chylothorax as the first manifestation is an unusual condition.To date,only a few cases have been reported in clinical practice.CASE SUMMARY The clinical data of a...BACKGROUND Primary membranous nephrotic syndrome with chylothorax as the first manifestation is an unusual condition.To date,only a few cases have been reported in clinical practice.CASE SUMMARY The clinical data of a 48-year-old man with primary nephrotic syndrome combined with chylothorax admitted to the Department of Respiratory and Critical Care Medicine of Shaanxi Provincial People's Hospital were retrospec-tively analysed.The patient was admitted to the hospital for 12 d due to shortness of breath.Imaging showed pleural effusion,laboratory tests confirmed true chylothorax,and renal biopsy revealed membranous nephropathy.After primary disease treatment and early active symptom treatment,the prognosis of the patient was good.This case suggests that chylothorax is a rare complication of primary membranous nephrotic syndrome in adults,and early lymphan-giography and renal biopsy can assist in the diagnosis when there are no contrain-dications.CONCLUSION Primary membranous nephrotic syndrome combined with chylothorax is rare in clinical practice.We report a relevant case to provide case information for clinicians and to improve diagnosis and treatment.展开更多
Dear Editor,Drug rash with eosinophilia and systemic symptoms(DRESS)syndrome is a drug reaction featuring high fever,eosinophilia,skin rashes,and multi-organ involvement that may be fatal.Ocular manifestations,includi...Dear Editor,Drug rash with eosinophilia and systemic symptoms(DRESS)syndrome is a drug reaction featuring high fever,eosinophilia,skin rashes,and multi-organ involvement that may be fatal.Ocular manifestations,including cicatrizing conjunctivitis,corneal infiltration,and anterior uveitis,are rarely reported[1-6]but may cause serious complications if untreated.Therefore,it is important to diagnose DRESS syndrome in patients with ocular features promptly with appropriate treatment.This article reports a case diagnosed with DRESS syndrome associated with pseudomembranous conjunctivitis secondary to carfilzomib.展开更多
BACKGROUND About 70%-80%of patients with primary membranous nephropathy(MN)have phospholipase A2 receptor(PLA2R)in renal tissue.Systemic light-chain(AL)amyloidosis is the most common type of amyloidosis.MN complicated...BACKGROUND About 70%-80%of patients with primary membranous nephropathy(MN)have phospholipase A2 receptor(PLA2R)in renal tissue.Systemic light-chain(AL)amyloidosis is the most common type of amyloidosis.MN complicated with amyloidosis is rare.CASE SUMMARY A 48-year-old Chinese male presented with nephrotic syndrome,positive serum PLA2R antibody,and positive serum and urine IgG-lambda type M-protein,with a normal ratio of serum-free light-chain level.The patient was diagnosed with MN accompanied by AL amyloidosis.He was treated with rituximab with glucocorticoids and CyBorD regimen of chemotherapy.After 21 mo of follow-up,the patient achieved complete remission regarding nephrotic syndrome without adverse effects of chemotherapy.CONCLUSION We report a case of PLA2R-related MN complicated with primary AL amyloidosis only with renal involvement and successfully treated with rituximab,glucocorticoids and chemotherapy.展开更多
BACKGROUND The co-occurrence of Anti-phospholipase A2 receptor-associated membranous nephropathy(anti-PLA2R-MN)and human immunodeficiency virus(HIV)infection is a rare clinical scenario,presenting significant challeng...BACKGROUND The co-occurrence of Anti-phospholipase A2 receptor-associated membranous nephropathy(anti-PLA2R-MN)and human immunodeficiency virus(HIV)infection is a rare clinical scenario,presenting significant challenges in terms of management and treatment.CASE SUMMARY A 32-year-old Chinese male diagnosed with HIV infection presented with a clinical history of proteinuria persisting for over two years.A kidney biopsy demonstrated subepithelial immune complex deposition and a thickened glomerular basement membrane,indicative of stage I-II membranous nephro-pathy.Immunofluorescence staining revealed granular deposition of PLA2R(3+)along the glomerular capillary loops,corroborated by a strongly positive anti-PLA2R antibody test(1:320).Initial treatment involving losartan potassium,rivaroxaban,tacrolimus,and rituximab was discontinued due to either poor effec-tiveness or the occurrence of adverse events.Following a regimen of weekly subcutaneous injections of telitacicept(160 mg),a marked decline in the 24 h urine protein was observed within a three-month period,accompanied by a rise in serum albumin level.No significant reductions in peripheral blood CD3+CD4+T and CD3+CD8+T cell counts were detected.The patient's physical and psychological conditions showed significant improvements,with no adverse events reported during the treatment course.CONCLUSION Telitacicept might offer a potential therapeutic avenue for patients diagnosed with anti-PLA2R-MN concomitant with HIV infection.展开更多
Objective:Based on network pharmacology and molecular docking technology to explore the mechanism of Professor Cao Enze's application of Panax notoginseng in the treatment of membranous nephropathy.Methods:TCMSP d...Objective:Based on network pharmacology and molecular docking technology to explore the mechanism of Professor Cao Enze's application of Panax notoginseng in the treatment of membranous nephropathy.Methods:TCMSP database was used to obtain the effective components and corresponding target information of Panax notoginseng,and Gene Cards database was used to obtain the disease target genes of membranous nephropathy.The intersection targets of the two were taken and the Venn diagram was drawn.The STRING database was used to obtain the protein interaction relationship,and the PPI network diagram was constructed by Cytoscape 3.9.1 software to screen out the core targets of Panax notoginseng in the treatment of membranous nephropathy.GO function and KEGG pathway enrichment analysis were performed using the David database to obtain the potential pathway of Panax notoginseng in the treatment of membranous nephropathy.Finally,Autodock software was used to verify the molecular docking of the main active components of the drug with the core targets.Results:A total of 7 effective components such as quercetin,ginsenoside rh2,Mandenol and Stigmasterol were retrieved,and 127 potential targets of Panax notoginseng in the treatment of membranous nephropathy were screened out.By PPI network topology analysis,23 core targets such as JUN,TP53,RELA,AKT1 and MAPK1 were screened out.GO functional enrichment analysis contained 703 biological processes,55 cell components and 121 molecular functions,and KEGG signal pathway enrichment analysis enriched 171 signal pathways.The results of molecular docking showed that there was a strong binding ability between the main core targets and the main components of Panax notoginseng.Conclusion:Through network pharmacology,it is concluded that Panax notoginseng treats membranous nephropathy through multiple targets and multiple pathways,which provides a theoretical basis for subsequent basic research.展开更多
Objective:To further evaluate the efficacy and safety of low-dose glucocorticoids combined with tacrolimus in the treatment of adult idiopathic membranous nephropathy(IMN)in a clinical setting.Methods:We carried out a...Objective:To further evaluate the efficacy and safety of low-dose glucocorticoids combined with tacrolimus in the treatment of adult idiopathic membranous nephropathy(IMN)in a clinical setting.Methods:We carried out a single-center prospective study of 88 patients with IMN who were admitted into the Affiliated Hospital of Hebei University from January 2019 to December 2021,and the participants were divided into two groups based on their serum anti-PLA2R antibody levels:the negative group and the positive group.46 patients were positive for anti-PLA2R antibodies and 42 were negative.Results:After 6 months of treatment,the serum albumin,cholesterol,and 24h urine protein quantification in the anti-PLA2R negative group improved more significantly compared to the positive group(P<0.05);after 6 months of treatment,the remission rate of the positive group was significantly lower than that of the negative group,and(P<0.05);Conclusion:After treatment with tacrolimus combined with low-dose glucocorticoids,patients with idiopathic membranous nephropathy who were tested positive for anti-PLA2R antibodies had a higher overall remission rate compared those who were tested negative for serum anti-PLA2R antibodies.展开更多
Objective To investigate the efficacy and safety of rituximab(RTX) in the treatment of idiopathic membranous nephropathy(IMN) with nephrotic syndrome with a systematic review and meta-analysis.Methods Pub Med, Embase,...Objective To investigate the efficacy and safety of rituximab(RTX) in the treatment of idiopathic membranous nephropathy(IMN) with nephrotic syndrome with a systematic review and meta-analysis.Methods Pub Med, Embase, Cochrane Library and Clinical Trials(December 2016) were searched to identify researches investigating the treatment of RTX in adult patients with biopsy-proven IMN. Complete remission(CR) or partial remission was regarded as effective therapy, and the cumulated remission rate was calculated.Results Seven studies involved 120 patients(73% were men) were included in our systematic review and metaanalysis. All were prospective observation cohort studies or matched-cohort studies, mainly came from two medical centers, and one study was multi-centric(four nephrology units in northern Italy). The creatinine clearance was more than 20 ml/(min·1.73 m2) and persistent proteinuria higher than 3.5 g/d for at least 6 months. All patients received treatment previously [44(36.7%) had immunosuppressive treatment]. In 12-and 24-month, 56%(95%CI, 0.47-0.65) and 68%(95%CI, 0.41-0.87) patients could reach remission, while 15%(95%CI, 0.09-0.23) and 20%(95%CI, 0.12-0.32) patients could reach CR. The reduction in proteinuria was gradual and obvious, paralleled with upward trend of serum albumin level and decreasing serum cholesterol level. Renal functions were stable. Relapses happened in 24 months were around 8%. RTX related adverse events were mild and were mostly infusion-related reactions.Conclusions RTX treatment in IMN was efficient, well tolerated and safe. More than 60% patients can reach partial remission or CR in 24 months, and relapse is rare. Adverse events of RTX are mostly infusion-related reactions and generally mild.展开更多
BACKGROUND Post-transplant nephrotic syndrome(PTNS)in a renal allograft carries a 48%to 77%risk of graft failure at 5 years if proteinuria persists.PTNS can be due to either recurrence of native renal disease or de no...BACKGROUND Post-transplant nephrotic syndrome(PTNS)in a renal allograft carries a 48%to 77%risk of graft failure at 5 years if proteinuria persists.PTNS can be due to either recurrence of native renal disease or de novo glomerular disease.Its prognosis depends upon the underlying pathophysiology.We describe a case of post-transplant membranous nephropathy(MN)that developed 3 mo after kidney transplant.The patient was properly evaluated for pathophysiology,which helped in the management of the case.CASE SUMMARY This 22-year-old patient had chronic pyelonephritis.He received a living donor kidney,and human leukocyte antigen-DR(HLA-DR)mismatching was zero.PTNS was discovered at the follow-up visit 3 mo after the transplant.Graft histopathology was suggestive of MN.In the past antibody-mediated rejection(ABMR)might have been misinterpreted as de novo MN due to the lack of technologies available to make an accurate diagnosis.Some researchers have observed that HLA-DR is present on podocytes causing an anti-DR antibody deposition and development of de novo MN.They also reported poor prognosis in their series.Here,we excluded the secondary causes of MN.Immunohistochemistry was suggestive of IgG1 deposits that favoured the diagnosis of de novo MN.The patient responded well to an increase in the dose of tacrolimus and angiotensin converting enzyme inhibitor.CONCLUSION Exposure of hidden antigens on the podocytes in allografts may have led to subepithelial antibody deposition causing de novo MN.展开更多
The efficacy and safety of tacrolimus (TAC) and cyclophosphamide (CTX) in the treatment of idiopathic membranous nephropathy (IMN) were compared in Chinese adult patients using a meta-analysis of the available l...The efficacy and safety of tacrolimus (TAC) and cyclophosphamide (CTX) in the treatment of idiopathic membranous nephropathy (IMN) were compared in Chinese adult patients using a meta-analysis of the available literatures. Randomized controlled clinical trials (RCTs) of the treatment of primary IMN with TAC or CTX combined with corticosteroids in the English databases PubMed, Embase and Cochrane, as well as Chinese databases, were searched. Qualified studies were subjected to quality assessment and meta-analysis. A total of 8 RCTs, including 359 Chinese patients, were included in the meta-analysis. The complete remission rate and overall remission rate in the TAC treatment group after 6 months of treatment were higher than those in the CTX treatment group. No significant difference in remission rate was found after 12 months of treatment. There was no significant difference in the adverse reaction between the two groups at the 6th or 12th months. TAC-based treatment was associated with a faster response than CTX at the 6th month, but there was no significant difference between the two groups at 12th month in Chinese adults. Further study is needed to evaluate the long-term efficacy and safety of this treatment regimen.展开更多
The association of primary sclerosing cholangitis(PSC)and autoimmune hepatitis(AIH)is known as an overlap syndrome(OS).OS can also be described in the setting of concomitant presence of AIH and PSC.These diseases can ...The association of primary sclerosing cholangitis(PSC)and autoimmune hepatitis(AIH)is known as an overlap syndrome(OS).OS can also be described in the setting of concomitant presence of AIH and PSC.These diseases can in some cases be associated with ulcerative colitis.In this case report we describe,to our knowledge,the first case in the literature of a young Caucasian male suffering from ulcerative colitis and an overlap syndrome consisting of an association betweenPSC-AIH,with the concomitant presence of a membranous glomerulonephritis.展开更多
BACKGROUND Idiopathic membranous nephropathy(IMN)has a high incidence in the middleaged and elderly population,and poses a great threat to the physical and mental health and quality of life of patients.Nephritis Rehab...BACKGROUND Idiopathic membranous nephropathy(IMN)has a high incidence in the middleaged and elderly population,and poses a great threat to the physical and mental health and quality of life of patients.Nephritis Rehabilitation Tablets have many potential effects,such as clearing residual toxins,tumefying the kidney and spleen,replenishing qi,and nourishing yin,and have played an important role in the treatment of a variety of kidney diseases.AIM To investigate the efficacy and safety of Nephritis Rehabilitation Tablets combined with tacrolimus in the treatment of IMN.METHODS Eighty-four patients with IMN recruited from January 2017 to September 2020 were randomly divided into a study group(n=42)and a control group(n=42).On the basis of routine symptomatic treatment,both groups were treated with tacrolimus,and the study group was additionally treated with Nephritis Rehabilitation Tablets.Both groups were treated for 12 wk.The therapeutic effect,the levels of renal function indexes[serum creatinine(Scr),serum albumin,and 24-h urinary protein],urinary immunoglobulin(IgG4),membrane attack complex(C5b-9),and the incidence of adverse reactions were measured before and after 12 wk of treatment.RESULTS The total effective rate in the study group was significantly higher than that of the control group.Before treatment,there was no significant difference in Scr,serum albumin,or 24 h urinary protein between the two groups.After 12 wk of treatment,the levels of Scr and 24-h urinary protein in both groups were significantly lower and serum albumin was significantly higher than those before treatment(P<0.05),and the levels of Scr and 24-h urinary protein were significantly lower(P=0.003 and 0.000,respectively),and the level of serum albumin was significantly higher(P=0.00)in the study group than in the control group.Before treatment,there was no significant difference in urinary IgG4 and C5b-9 levels between the study group and the control group(P=0.336 and 0.438,respectively).After 12 wk of treatment,the levels of urinary IgG4 and C5b-9 in the two groups were lower than those before treatment,and the levels of urinary IgG4 and C5b-9 in the study group were significantly lower than those in the control group(P=0.000).There was no significant difference in the incidence of adverse reactions between the two groups(P=0.710).CONCLUSION Based on routine intervention,Nephritis Rehabilitation Tablets combined with tacrolimus in the treatment of IMN can effectively improve the renal function of patients and downregulate the expression of urinary IgG4 and C5b-9.In addition,they can improve the overall therapeutic effect while not increasing the risk of adverse reactions.展开更多
China is a higher-intermediate hepatitis B virus(HBV)endemicity country,and HBV is among the most important infectious diseases[1,2].Hepatitis-Bassociated glomerulonephritis(HBV-GN)is also one of the main forms of sec...China is a higher-intermediate hepatitis B virus(HBV)endemicity country,and HBV is among the most important infectious diseases[1,2].Hepatitis-Bassociated glomerulonephritis(HBV-GN)is also one of the main forms of secondary kidney diseases seen in China[3].The main pathological type is hepatitis-Bassociated membranous nephropathy(HBV-MN).In the recent years,it has been found that human renal podocyte membrane M-type phospholipase 2 receptor(PLA2R)is the main autoantigen in idiopathic membranous nephropathy(IMN)[4].At present,it is found that PLA2 R and its antibodies are.展开更多
Integrity of the membranous labyrinth barrier system is of critical importance, which promotes inner ear homeostasis and maintains its features. The membranous labyrinth barrier system is divided into several subsets ...Integrity of the membranous labyrinth barrier system is of critical importance, which promotes inner ear homeostasis and maintains its features. The membranous labyrinth barrier system is divided into several subsets of barriers which, although independent from each other, are interrelated. The same substance may demonstrate different permeability characteristics through different barriers and under different conditions, while different substances can have different permeability features even in the same barrier under the same condition. All parts of the mem-branous labyrinth barrier structure, including their morphology, enzymes and channel proteins, and theirs permeability characteristics under various physiological and pathological conditions are reviewed in this paper. Infections, noise exposure, ototoxicity may all increase perme-ability of the barriers and lead to disturbances in inner ear homeostasis. Copyright ? 2015 The Authors. Production & hosting by Elsevier (Singapore) Pte Ltd On behalf of PLA General Hospital Department of Otolaryngology Head and Neck Surgery. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).展开更多
Summary:Recent studies suggested that serum secretory phospholipase A2 group IB(sPLA2-IB)was increased in idiopathic membranous nephropathy(IMN).However,the interference of high lipemia on the sPL.A2-IB levels was not...Summary:Recent studies suggested that serum secretory phospholipase A2 group IB(sPLA2-IB)was increased in idiopathic membranous nephropathy(IMN).However,the interference of high lipemia on the sPL.A2-IB levels was not taken into account in these studies.The present study aimed to investigate the correlation between sPLA2-IB and lipemia,and the clinical merit of sPLA2-IB in the prediction of prognosis of IMN patients.A total of 64 IMN patients,39 immunoglobulin A nephropathy(IgAN)patients and 64 healthy controls were included in the study.The levels of serum sPLA2-IB,lipemia and proteinuria were measured.Fifty IMN patients were followed up for 6 months.Pathologic stages were made for all IgAN and IMN patients.The results showed that the levels of serum sPLA2-IB,cholesterol and low-density lipoprotein cholesterol(LDL-C)were significantly higher,and the levels of albumin and high-density lipoprotein cholesterol(HDL-C)were significantly lower in IMN patients than in healthy controls and IgAN patients.Serum sPLA2-IB levels were also found to be higher in IgAN patients than in heathy controls,but the association of serum sPLA2-IB levels with proteinuria,cholesterol and albumin was only shown in IMN patients.Antibody against M-type receptor for secretory phospholipase A2(PLA2R1)was positive in 813%IMN patients.Glomerular sPLA2-IB deposition,podocyte fused processes,and density deposition on thickened basement membrane were seen in IMN patients,but not in IgAN patients.IMN patients with lower sPLA2-IB and proteinuria levels were found to have better outcome after the 6-month follow-up.In IMN patients,sPLA2-IB levels were significantly increased in both serum and renal tissue.In conclusion,serum sPLA2-IB was closely correlated with proteinuria,albumin and cholesterol,and IMN patients with lower sPLA2-IB levels were more likely to achieve a better outcome.展开更多
We report a challenging case of a 81-year-old male with history of severe calcific aortic valve stenosis and aneurysmal membranous interventricular septum. The presence of anomalies in the sub-annular area can lead to...We report a challenging case of a 81-year-old male with history of severe calcific aortic valve stenosis and aneurysmal membranous interventricular septum. The presence of anomalies in the sub-annular area can lead to valve malpositioning and its consequences. Transcatheter aortic valve implantation(TAVR) in patients with aneurysm of the perimembranous interventricular septum extending into the left ventricular outflow tract has not been previously reported. This case describes a successful transfemoral TAVR with an Edwards SAPIEN XT valve(Edwards Lifesciences, Irvine, CA, United States) with such anomaly.展开更多
When the physiopathology of membranous nephropathy was first described,almost 30%of cases were recognized to be secondary to well-known diseases such as autoimmune diseases,tumors or infections.The remaining 70%cases ...When the physiopathology of membranous nephropathy was first described,almost 30%of cases were recognized to be secondary to well-known diseases such as autoimmune diseases,tumors or infections.The remaining 70%cases were called primary membranous nephropathy as the exact mechanism or pathogenic factor involved was unknown.The discovery of the M type phospholipase A2 receptor and thrombospondin type 1 domain containing 7A as causative antigens in these“so called”primary membranous nephropathies provided new insights into the effective causes of a large proportion of these cases.Novel techniques such as laser microdissection and tandem mass spectrometry as well as immunochemistry with antibodies directed against novel proteins allowed the confirmation of new involved antigens.Finally,using confocal microscopy to localize these new antigens and immunoglobulin G and Western blot analysis of serum samples,these new antigens were detected on the glomerular membrane,and the related antibodies were detected in serum samples.The same antigens have been recognized in some cases of secondary membranous disease due to autoimmune diseases,tumors and infections.This has allowed examination of the relationship between antigens in primary membranous nephropathy and their presence in some secondary nephropathies.The aim of this study is to describe the characteristics of the new antigens discovered and their association with other diseases.展开更多
基金funded by grants from the National Natural Science Foundation of China(Grant No.81570666)International Society of Nephrology Clinical Research Program(Grant No.18-01-0247)+3 种基金Program of Jiangsu Clinical Research Center(Grant No.BL2014084)Jiangsu Province Key Medical Personnel Project(Grant No.ZDRCA2016002)CKD Anemia Research Foundation from China International Medical Foundation(Grant No.Z-2017-24-2037)Outstanding Young and Middle-aged Talents Support Program of the First Affiliated Hospital of Nanjing Medical University(Jiangsu Province Hospital).
文摘Nocardiosis manifests as an opportunistic infection,primarily affecting individuals who are immunocompromised and susceptible to the infection.We present a case study of one patient with nephrotic syndrome and membranous nephropathy,who underwent treatment with prednisone and cyclosporine in 2016.In early 2017,the patient was diagnosed with a"fungal infection"and discontinued the use of cyclosporine.After one month of anti-infection therapy,a cranial magnetic resonance imaging scan showed multiple abscesses in the right temporal region.The diagnosis of nocardiosis was confirmed based on the presence of metastatic abscess masses,multiple lung and brain lesions,and a positive culture of Nocardia in the drainage.We changed the anti-infection therapy to a combination of trimethoprim-sulfamethoxazole(TMP-SMX),minocycline,and voriconazole.However,the patient experienced a sudden cardiac arrest and subsequently recovered after cardiopulmonary resuscitation.During the five-month follow-up period following the discharge,the patient displayed an enhanced nutritional status and stable renal function.The focal infection ultimately resolved during the subsequent three years.Neuro-infection caused by Nocardia should be considered in immunocompromised patients,and TMP-SMX is the preferred initial therapy;however,because of the high mortality rate,a long-term combination therapy with imipenem,cefotaxime,amikacin,and TMP-SMX is suggested.
文摘BACKGROUND Extragastrointestinal stromal tumors(EGIST)and gastrointestinal stromal tumors are of similar pathological type and form.Here we report a rare case of EGIST diffusely distributed in membranous tissue in abdominal cavity,the feature of which included diffuse tumors at membranous tissue in entire abdominal cavity and spontaneous bleeding of the tumors.CASE SUMMARY The patient was a 71-year man and hospitalized due to continuous pain at lower abdomen for more than 10 days.Upon physical examination,the patient had flat and tough abdomen with mild pressing pain at lower abdomen,no obvious abdominal mass was touchable,and shifting dullness was positive.Positron emission tomography-computed tomography(CT)showed that in his peritoneal cavity,there were multiple nodules of various sizes,seroperitoneum,multiple enlarged lymph nodes in abdominal/pelvic cavity and right external ilium as well as pulmonary nodules.Plain CT scanning at epigastrium/hypogastrium/pelvic cavity+enhanced three-dimensional reconstruction revealed multiple soft tissue nodules in abdominal/pelvic cavity,peritoneum and right groin.Tumor marker of carbohydrate antigen 125 was 808 U/mL,diffuse tuberous tumor was seen in abdominal/pelvic cavity during operation with hematocelia,and postoperative pathological examination confirmed EGIST.Imatinib was administered with better therapeutic effect.CONCLUSION Gene testing showed breast cancer susceptibility gene 1 interacting protein C-terminal helicase 1 and KIT genovariation,and the patient was treated with imatinib follow-up visit found that his clinical symptoms disappeared and the tumor load alleviated obviously via imageological examination.
文摘Objective:To investigate the efficacy of rituximab in the treatment of idiopathic membranous nephropathy with varying levels of serum phospholipase A2 receptor antibodies.Methods:A total of 137 patients with idiopathic membranous nephropathy admitted to Beijing Sixth Hospital were selected.Based on their blood PLA2R antibody levels before rituximab treatment,patients were categorized into the PLA2R antibody positive group(n=94)and the PLA2R antibody negative group(n=43).They were followed up for at least 1 year,during which the efficacy,measured through 24-hour urine protein quantification and serum albumin levels,were compared between the two groups before and after treatment.Results:After 3 months of treatment,there was no significant difference in the quantitative levels of 24-hour urine protein between the two groups(P>0.05).However,after 6 and 12 months of treatment,there was a significant difference in the levels of 24-hour urine protein between the two groups(P<0.05).Additionally,after 3 months of treatment,there was a notable difference in the serum albumin levels between the two groups(P<0.05).However,after 6 and 12 months of treatment,there was no significant difference in serum albumin levels between the two groups(P>0.05).Analysis of complications in the two groups revealed that in the positive group,9 individuals experienced thrombosis,5 had infections,and 11 developed acute kidney injury(AKI).In contrast,in the negative group,5 individuals had thrombosis,2 had infections,and 3 developed AKI.There was no statistically significant difference in complications between the two groups(P>0.05).Conclusion:Serum anti-PLA2R antibody levels provide valuable insights into the clinical observation of rituximab treatment for idiopathic membranous nephropathy.They aid in understanding the disease’s pathogenesis,evaluating treatment efficacy,and predicting disease prognosis.
基金Supported by National Key Research and Development Program of China,No.2019YFC1708503。
文摘BACKGROUND The recognition of idiopathic membranous nephropathy(IMN)as an autoimmune disease has paved the way for the use of B-cell-depleting agents,such as Rituximab(RTX),which is now a first-line drug for treating IMN with proven safety and efficacy.Nevertheless,the usage of RTX for the treatment of refractory IMN remains controversial and challenging.AIM To evaluate the efficacy and safety of a new low-dose RTX regimen for the treatment of patients with refractory IMN.METHODS A retrospective study was performed on refractory IMN patients that accepted a low-dose RTX regimen(RTX,200 mg,once a month for five months)in the Xiyuan Hospital of Chinese Academy of Chinese Medical Sciences’Department of Nephrology from October 2019 to December 2021.To assess the clinical and immune remission data,we performed a 24 h urinary protein quantification(UTP)test and measured the serum albumin(ALB)and serum creatinine(SCr)levels,phospholipase A2 receptor(PLA2R)antibody titer,and CD19+B-cell count every three months.RESULTS A total of nine refractory IMN patients were analyzed.During follow-up conducted twelve months later,the results from the 24 h UTP decreased from baseline[8.14±6.05 g/d to 1.24±1.34 g/d(P<0.05)]and the ALB levels increased from baseline[28.06±8.42 g/L to 40.93±5.85 g/L(P<0.01)].Notably,after administering RTX for six months,the SCr decreased from 78.13±16.49μmol/L to 109.67±40.87μmol/L(P<0.05).All of the nine patients were positive for serum anti-PLA2R at the beginning,and four patients had normal anti-PLA2R titer levels at six months.The level of CD19+B-cells decreased to 0 at three months,and CD19+B-cell count remained at 0 up until six months of follow-up.CONCLUSION Our low-dose RTX regimen appears to be a promising treatment strategy for refractory IMN.
基金Supported by the Shaanxi Provincial People’s Hospital Science and Technology Development Incubation Fund Project,No.2021YJY-33.
文摘BACKGROUND Primary membranous nephrotic syndrome with chylothorax as the first manifestation is an unusual condition.To date,only a few cases have been reported in clinical practice.CASE SUMMARY The clinical data of a 48-year-old man with primary nephrotic syndrome combined with chylothorax admitted to the Department of Respiratory and Critical Care Medicine of Shaanxi Provincial People's Hospital were retrospec-tively analysed.The patient was admitted to the hospital for 12 d due to shortness of breath.Imaging showed pleural effusion,laboratory tests confirmed true chylothorax,and renal biopsy revealed membranous nephropathy.After primary disease treatment and early active symptom treatment,the prognosis of the patient was good.This case suggests that chylothorax is a rare complication of primary membranous nephrotic syndrome in adults,and early lymphan-giography and renal biopsy can assist in the diagnosis when there are no contrain-dications.CONCLUSION Primary membranous nephrotic syndrome combined with chylothorax is rare in clinical practice.We report a relevant case to provide case information for clinicians and to improve diagnosis and treatment.
基金Supported by the SNUBH Research Fund(No.13-2020-007)TRC Research Grant of the Korea University Medicine and Korea Institute of Science and Technology and Korea University Ansan Hospital grant,by Korea University grants(No.K1625491,No.K1722121,No.K1811051,No.K1913161,No.K2010921)+3 种基金the Korea Medical Device Development Fund grant funded by the Korea government(the Ministry of Science and ICT,the Ministry of Trade,Industry and Energy,the Ministry of Health&Welfare,the Ministry of Food and Drug Safety)(No.1711174253,RS-2020-KD000296)Korea Environment Industry&Technology Institute(KEITI)through Technology Development Project for Safety Management of Household Chemical Products,funded by Korea Ministry of Environment(MOE)(No.2020002960007,NTIS-1485018521)the National Research Foundation of Korea(NRF)grant funded by the Korea government(MSIT)(NRF-2021R1F1A1062017)the Technology Development Program(S3127902,S3305836)funded by the Ministry of SMEs and Startups(MSS,Korea)。
文摘Dear Editor,Drug rash with eosinophilia and systemic symptoms(DRESS)syndrome is a drug reaction featuring high fever,eosinophilia,skin rashes,and multi-organ involvement that may be fatal.Ocular manifestations,including cicatrizing conjunctivitis,corneal infiltration,and anterior uveitis,are rarely reported[1-6]but may cause serious complications if untreated.Therefore,it is important to diagnose DRESS syndrome in patients with ocular features promptly with appropriate treatment.This article reports a case diagnosed with DRESS syndrome associated with pseudomembranous conjunctivitis secondary to carfilzomib.
文摘BACKGROUND About 70%-80%of patients with primary membranous nephropathy(MN)have phospholipase A2 receptor(PLA2R)in renal tissue.Systemic light-chain(AL)amyloidosis is the most common type of amyloidosis.MN complicated with amyloidosis is rare.CASE SUMMARY A 48-year-old Chinese male presented with nephrotic syndrome,positive serum PLA2R antibody,and positive serum and urine IgG-lambda type M-protein,with a normal ratio of serum-free light-chain level.The patient was diagnosed with MN accompanied by AL amyloidosis.He was treated with rituximab with glucocorticoids and CyBorD regimen of chemotherapy.After 21 mo of follow-up,the patient achieved complete remission regarding nephrotic syndrome without adverse effects of chemotherapy.CONCLUSION We report a case of PLA2R-related MN complicated with primary AL amyloidosis only with renal involvement and successfully treated with rituximab,glucocorticoids and chemotherapy.
文摘BACKGROUND The co-occurrence of Anti-phospholipase A2 receptor-associated membranous nephropathy(anti-PLA2R-MN)and human immunodeficiency virus(HIV)infection is a rare clinical scenario,presenting significant challenges in terms of management and treatment.CASE SUMMARY A 32-year-old Chinese male diagnosed with HIV infection presented with a clinical history of proteinuria persisting for over two years.A kidney biopsy demonstrated subepithelial immune complex deposition and a thickened glomerular basement membrane,indicative of stage I-II membranous nephro-pathy.Immunofluorescence staining revealed granular deposition of PLA2R(3+)along the glomerular capillary loops,corroborated by a strongly positive anti-PLA2R antibody test(1:320).Initial treatment involving losartan potassium,rivaroxaban,tacrolimus,and rituximab was discontinued due to either poor effec-tiveness or the occurrence of adverse events.Following a regimen of weekly subcutaneous injections of telitacicept(160 mg),a marked decline in the 24 h urine protein was observed within a three-month period,accompanied by a rise in serum albumin level.No significant reductions in peripheral blood CD3+CD4+T and CD3+CD8+T cell counts were detected.The patient's physical and psychological conditions showed significant improvements,with no adverse events reported during the treatment course.CONCLUSION Telitacicept might offer a potential therapeutic avenue for patients diagnosed with anti-PLA2R-MN concomitant with HIV infection.
基金2022 Anhui Provincial Health Research Project (No.AHWJ2022b041)2021 Anhui Provincial Key Medical and Health Specialty Construction Project (No.Anhui Health Letter 2021-273)。
文摘Objective:Based on network pharmacology and molecular docking technology to explore the mechanism of Professor Cao Enze's application of Panax notoginseng in the treatment of membranous nephropathy.Methods:TCMSP database was used to obtain the effective components and corresponding target information of Panax notoginseng,and Gene Cards database was used to obtain the disease target genes of membranous nephropathy.The intersection targets of the two were taken and the Venn diagram was drawn.The STRING database was used to obtain the protein interaction relationship,and the PPI network diagram was constructed by Cytoscape 3.9.1 software to screen out the core targets of Panax notoginseng in the treatment of membranous nephropathy.GO function and KEGG pathway enrichment analysis were performed using the David database to obtain the potential pathway of Panax notoginseng in the treatment of membranous nephropathy.Finally,Autodock software was used to verify the molecular docking of the main active components of the drug with the core targets.Results:A total of 7 effective components such as quercetin,ginsenoside rh2,Mandenol and Stigmasterol were retrieved,and 127 potential targets of Panax notoginseng in the treatment of membranous nephropathy were screened out.By PPI network topology analysis,23 core targets such as JUN,TP53,RELA,AKT1 and MAPK1 were screened out.GO functional enrichment analysis contained 703 biological processes,55 cell components and 121 molecular functions,and KEGG signal pathway enrichment analysis enriched 171 signal pathways.The results of molecular docking showed that there was a strong binding ability between the main core targets and the main components of Panax notoginseng.Conclusion:Through network pharmacology,it is concluded that Panax notoginseng treats membranous nephropathy through multiple targets and multiple pathways,which provides a theoretical basis for subsequent basic research.
文摘Objective:To further evaluate the efficacy and safety of low-dose glucocorticoids combined with tacrolimus in the treatment of adult idiopathic membranous nephropathy(IMN)in a clinical setting.Methods:We carried out a single-center prospective study of 88 patients with IMN who were admitted into the Affiliated Hospital of Hebei University from January 2019 to December 2021,and the participants were divided into two groups based on their serum anti-PLA2R antibody levels:the negative group and the positive group.46 patients were positive for anti-PLA2R antibodies and 42 were negative.Results:After 6 months of treatment,the serum albumin,cholesterol,and 24h urine protein quantification in the anti-PLA2R negative group improved more significantly compared to the positive group(P<0.05);after 6 months of treatment,the remission rate of the positive group was significantly lower than that of the negative group,and(P<0.05);Conclusion:After treatment with tacrolimus combined with low-dose glucocorticoids,patients with idiopathic membranous nephropathy who were tested positive for anti-PLA2R antibodies had a higher overall remission rate compared those who were tested negative for serum anti-PLA2R antibodies.
基金Supported by the Key Projects in the National Science and Technology Pillar Program during the Twelfth Five-year Plan Period(2011BAI10B03)
文摘Objective To investigate the efficacy and safety of rituximab(RTX) in the treatment of idiopathic membranous nephropathy(IMN) with nephrotic syndrome with a systematic review and meta-analysis.Methods Pub Med, Embase, Cochrane Library and Clinical Trials(December 2016) were searched to identify researches investigating the treatment of RTX in adult patients with biopsy-proven IMN. Complete remission(CR) or partial remission was regarded as effective therapy, and the cumulated remission rate was calculated.Results Seven studies involved 120 patients(73% were men) were included in our systematic review and metaanalysis. All were prospective observation cohort studies or matched-cohort studies, mainly came from two medical centers, and one study was multi-centric(four nephrology units in northern Italy). The creatinine clearance was more than 20 ml/(min·1.73 m2) and persistent proteinuria higher than 3.5 g/d for at least 6 months. All patients received treatment previously [44(36.7%) had immunosuppressive treatment]. In 12-and 24-month, 56%(95%CI, 0.47-0.65) and 68%(95%CI, 0.41-0.87) patients could reach remission, while 15%(95%CI, 0.09-0.23) and 20%(95%CI, 0.12-0.32) patients could reach CR. The reduction in proteinuria was gradual and obvious, paralleled with upward trend of serum albumin level and decreasing serum cholesterol level. Renal functions were stable. Relapses happened in 24 months were around 8%. RTX related adverse events were mild and were mostly infusion-related reactions.Conclusions RTX treatment in IMN was efficient, well tolerated and safe. More than 60% patients can reach partial remission or CR in 24 months, and relapse is rare. Adverse events of RTX are mostly infusion-related reactions and generally mild.
文摘BACKGROUND Post-transplant nephrotic syndrome(PTNS)in a renal allograft carries a 48%to 77%risk of graft failure at 5 years if proteinuria persists.PTNS can be due to either recurrence of native renal disease or de novo glomerular disease.Its prognosis depends upon the underlying pathophysiology.We describe a case of post-transplant membranous nephropathy(MN)that developed 3 mo after kidney transplant.The patient was properly evaluated for pathophysiology,which helped in the management of the case.CASE SUMMARY This 22-year-old patient had chronic pyelonephritis.He received a living donor kidney,and human leukocyte antigen-DR(HLA-DR)mismatching was zero.PTNS was discovered at the follow-up visit 3 mo after the transplant.Graft histopathology was suggestive of MN.In the past antibody-mediated rejection(ABMR)might have been misinterpreted as de novo MN due to the lack of technologies available to make an accurate diagnosis.Some researchers have observed that HLA-DR is present on podocytes causing an anti-DR antibody deposition and development of de novo MN.They also reported poor prognosis in their series.Here,we excluded the secondary causes of MN.Immunohistochemistry was suggestive of IgG1 deposits that favoured the diagnosis of de novo MN.The patient responded well to an increase in the dose of tacrolimus and angiotensin converting enzyme inhibitor.CONCLUSION Exposure of hidden antigens on the podocytes in allografts may have led to subepithelial antibody deposition causing de novo MN.
基金supported by the National Natural Science Foundation of China(No.30800523 and 81570657)a grant from Hubei Province Health and Family Planning Commission(No.WJ2015MB013)
文摘The efficacy and safety of tacrolimus (TAC) and cyclophosphamide (CTX) in the treatment of idiopathic membranous nephropathy (IMN) were compared in Chinese adult patients using a meta-analysis of the available literatures. Randomized controlled clinical trials (RCTs) of the treatment of primary IMN with TAC or CTX combined with corticosteroids in the English databases PubMed, Embase and Cochrane, as well as Chinese databases, were searched. Qualified studies were subjected to quality assessment and meta-analysis. A total of 8 RCTs, including 359 Chinese patients, were included in the meta-analysis. The complete remission rate and overall remission rate in the TAC treatment group after 6 months of treatment were higher than those in the CTX treatment group. No significant difference in remission rate was found after 12 months of treatment. There was no significant difference in the adverse reaction between the two groups at the 6th or 12th months. TAC-based treatment was associated with a faster response than CTX at the 6th month, but there was no significant difference between the two groups at 12th month in Chinese adults. Further study is needed to evaluate the long-term efficacy and safety of this treatment regimen.
文摘The association of primary sclerosing cholangitis(PSC)and autoimmune hepatitis(AIH)is known as an overlap syndrome(OS).OS can also be described in the setting of concomitant presence of AIH and PSC.These diseases can in some cases be associated with ulcerative colitis.In this case report we describe,to our knowledge,the first case in the literature of a young Caucasian male suffering from ulcerative colitis and an overlap syndrome consisting of an association betweenPSC-AIH,with the concomitant presence of a membranous glomerulonephritis.
基金the Hospital of No.80 Group Army Institutional Review Board(Approval No.63).
文摘BACKGROUND Idiopathic membranous nephropathy(IMN)has a high incidence in the middleaged and elderly population,and poses a great threat to the physical and mental health and quality of life of patients.Nephritis Rehabilitation Tablets have many potential effects,such as clearing residual toxins,tumefying the kidney and spleen,replenishing qi,and nourishing yin,and have played an important role in the treatment of a variety of kidney diseases.AIM To investigate the efficacy and safety of Nephritis Rehabilitation Tablets combined with tacrolimus in the treatment of IMN.METHODS Eighty-four patients with IMN recruited from January 2017 to September 2020 were randomly divided into a study group(n=42)and a control group(n=42).On the basis of routine symptomatic treatment,both groups were treated with tacrolimus,and the study group was additionally treated with Nephritis Rehabilitation Tablets.Both groups were treated for 12 wk.The therapeutic effect,the levels of renal function indexes[serum creatinine(Scr),serum albumin,and 24-h urinary protein],urinary immunoglobulin(IgG4),membrane attack complex(C5b-9),and the incidence of adverse reactions were measured before and after 12 wk of treatment.RESULTS The total effective rate in the study group was significantly higher than that of the control group.Before treatment,there was no significant difference in Scr,serum albumin,or 24 h urinary protein between the two groups.After 12 wk of treatment,the levels of Scr and 24-h urinary protein in both groups were significantly lower and serum albumin was significantly higher than those before treatment(P<0.05),and the levels of Scr and 24-h urinary protein were significantly lower(P=0.003 and 0.000,respectively),and the level of serum albumin was significantly higher(P=0.00)in the study group than in the control group.Before treatment,there was no significant difference in urinary IgG4 and C5b-9 levels between the study group and the control group(P=0.336 and 0.438,respectively).After 12 wk of treatment,the levels of urinary IgG4 and C5b-9 in the two groups were lower than those before treatment,and the levels of urinary IgG4 and C5b-9 in the study group were significantly lower than those in the control group(P=0.000).There was no significant difference in the incidence of adverse reactions between the two groups(P=0.710).CONCLUSION Based on routine intervention,Nephritis Rehabilitation Tablets combined with tacrolimus in the treatment of IMN can effectively improve the renal function of patients and downregulate the expression of urinary IgG4 and C5b-9.In addition,they can improve the overall therapeutic effect while not increasing the risk of adverse reactions.
基金funded by National Natural Science Foundation of China[81870494]Qingdao Outstanding Health Professional Development Fund[2020-2022]Chinese Society of Nephrology[20010080800]。
文摘China is a higher-intermediate hepatitis B virus(HBV)endemicity country,and HBV is among the most important infectious diseases[1,2].Hepatitis-Bassociated glomerulonephritis(HBV-GN)is also one of the main forms of secondary kidney diseases seen in China[3].The main pathological type is hepatitis-Bassociated membranous nephropathy(HBV-MN).In the recent years,it has been found that human renal podocyte membrane M-type phospholipase 2 receptor(PLA2R)is the main autoantigen in idiopathic membranous nephropathy(IMN)[4].At present,it is found that PLA2 R and its antibodies are.
文摘Integrity of the membranous labyrinth barrier system is of critical importance, which promotes inner ear homeostasis and maintains its features. The membranous labyrinth barrier system is divided into several subsets of barriers which, although independent from each other, are interrelated. The same substance may demonstrate different permeability characteristics through different barriers and under different conditions, while different substances can have different permeability features even in the same barrier under the same condition. All parts of the mem-branous labyrinth barrier structure, including their morphology, enzymes and channel proteins, and theirs permeability characteristics under various physiological and pathological conditions are reviewed in this paper. Infections, noise exposure, ototoxicity may all increase perme-ability of the barriers and lead to disturbances in inner ear homeostasis. Copyright ? 2015 The Authors. Production & hosting by Elsevier (Singapore) Pte Ltd On behalf of PLA General Hospital Department of Otolaryngology Head and Neck Surgery. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
基金This study was funded by the National Natural Science Foundation of China(No.81670635)Talents Training Program of Pudong Hospital Affiliated to Fudan University(No.PY202002)Natxiral Science Foundation of Fujian Province of China(No.2019J01441).
文摘Summary:Recent studies suggested that serum secretory phospholipase A2 group IB(sPLA2-IB)was increased in idiopathic membranous nephropathy(IMN).However,the interference of high lipemia on the sPL.A2-IB levels was not taken into account in these studies.The present study aimed to investigate the correlation between sPLA2-IB and lipemia,and the clinical merit of sPLA2-IB in the prediction of prognosis of IMN patients.A total of 64 IMN patients,39 immunoglobulin A nephropathy(IgAN)patients and 64 healthy controls were included in the study.The levels of serum sPLA2-IB,lipemia and proteinuria were measured.Fifty IMN patients were followed up for 6 months.Pathologic stages were made for all IgAN and IMN patients.The results showed that the levels of serum sPLA2-IB,cholesterol and low-density lipoprotein cholesterol(LDL-C)were significantly higher,and the levels of albumin and high-density lipoprotein cholesterol(HDL-C)were significantly lower in IMN patients than in healthy controls and IgAN patients.Serum sPLA2-IB levels were also found to be higher in IgAN patients than in heathy controls,but the association of serum sPLA2-IB levels with proteinuria,cholesterol and albumin was only shown in IMN patients.Antibody against M-type receptor for secretory phospholipase A2(PLA2R1)was positive in 813%IMN patients.Glomerular sPLA2-IB deposition,podocyte fused processes,and density deposition on thickened basement membrane were seen in IMN patients,but not in IgAN patients.IMN patients with lower sPLA2-IB and proteinuria levels were found to have better outcome after the 6-month follow-up.In IMN patients,sPLA2-IB levels were significantly increased in both serum and renal tissue.In conclusion,serum sPLA2-IB was closely correlated with proteinuria,albumin and cholesterol,and IMN patients with lower sPLA2-IB levels were more likely to achieve a better outcome.
文摘We report a challenging case of a 81-year-old male with history of severe calcific aortic valve stenosis and aneurysmal membranous interventricular septum. The presence of anomalies in the sub-annular area can lead to valve malpositioning and its consequences. Transcatheter aortic valve implantation(TAVR) in patients with aneurysm of the perimembranous interventricular septum extending into the left ventricular outflow tract has not been previously reported. This case describes a successful transfemoral TAVR with an Edwards SAPIEN XT valve(Edwards Lifesciences, Irvine, CA, United States) with such anomaly.
文摘When the physiopathology of membranous nephropathy was first described,almost 30%of cases were recognized to be secondary to well-known diseases such as autoimmune diseases,tumors or infections.The remaining 70%cases were called primary membranous nephropathy as the exact mechanism or pathogenic factor involved was unknown.The discovery of the M type phospholipase A2 receptor and thrombospondin type 1 domain containing 7A as causative antigens in these“so called”primary membranous nephropathies provided new insights into the effective causes of a large proportion of these cases.Novel techniques such as laser microdissection and tandem mass spectrometry as well as immunochemistry with antibodies directed against novel proteins allowed the confirmation of new involved antigens.Finally,using confocal microscopy to localize these new antigens and immunoglobulin G and Western blot analysis of serum samples,these new antigens were detected on the glomerular membrane,and the related antibodies were detected in serum samples.The same antigens have been recognized in some cases of secondary membranous disease due to autoimmune diseases,tumors and infections.This has allowed examination of the relationship between antigens in primary membranous nephropathy and their presence in some secondary nephropathies.The aim of this study is to describe the characteristics of the new antigens discovered and their association with other diseases.