Objective:To evaluate the value of rehabilitation nursing based on mind mapping model combined with psychological intervention for patients with nephrotic syndrome(NS).Methods:A total of 60 patients with NS who visite...Objective:To evaluate the value of rehabilitation nursing based on mind mapping model combined with psychological intervention for patients with nephrotic syndrome(NS).Methods:A total of 60 patients with NS who visited our hospital from January 2024 to December 2024 were selected as samples and randomly divided into groups.The observation group received rehabilitation nursing based on the mind mapping model combined with psychological intervention,while the control group received routine intervention.The differences in emotional scores,self-care ability scores,compliance,and complications were compared between the two groups.Results:The anxiety(SAS)and depression(SDS)scores of the observation group were lower than those of the control group,while the self-care ability scale(ESCA)score was higher than that of the control group(P<0.05).The compliance rate of the observation group was higher than that of the control group(P<0.05).The complication rate of NS in the observation group was lower than that in the control group(P<0.05).Conclusion:Rehabilitation nursing based on the mind mapping model combined with psychological intervention can enhance self-care ability,reduce negative emotions,and reduce complications in NS nursing,which is efficient and feasible.展开更多
The underlying molecular changes that result in minimal change disease(ne-phrotic syndrome)require an in-depth analysis.Current molecular studies have shown the involvement of zinc fingers and homeobox transcriptional...The underlying molecular changes that result in minimal change disease(ne-phrotic syndrome)require an in-depth analysis.Current molecular studies have shown the involvement of zinc fingers and homeobox transcriptional factors in its pathogenesis.The application of therapeutic drugs relies on understanding the cascade of molecular events to determine their efficacy in managing the clinical condition.展开更多
Glucocorticoids(GCs)such as prednisolone are widely used in conditions like nephrotic syndrome,asthma,and autoimmune diseases.However,prolonged or high-dose use may suppress the hypothalamic-pituitary-adrenal(HPA)axis...Glucocorticoids(GCs)such as prednisolone are widely used in conditions like nephrotic syndrome,asthma,and autoimmune diseases.However,prolonged or high-dose use may suppress the hypothalamic-pituitary-adrenal(HPA)axis,leading to secondary adrenal insufficiency(AI).This condition occurs when the adrenal glands fail to produce adequate cortisol,which is essential for regulating metabolism,immune response,and stress adaptation.Corticotropin-releasing hormone(CRH)from the hypothalamus stimulates the pituitary to release adrenocorticotropic hormone(ACTH),which then triggers cortisol production in the adrenal glands.Prolonged GC use disrupts this system by inhibiting CRH and ACTH secretion,leading to adrenal atrophy and reduced cortisol production.HPA axis suppression is primarily diagnosed through dynamic tests.Early morning cortisol levels above>18 ng/mL typically indicate normal function,while levels<3 ng/mL suggest AI.Intermediate values require additional testing,such as the insulin tolerance test,ACTH stimulation test,and metyrapone test.Prednisolone in nephrotic syndrome suppresses the HPA axis,heightening AI risk,influenced by dose,duration,and timing of administration.Careful GC management is essential to balance disease control with risks of HPA axis suppression.Early recognition and timely intervention can prevent adrenal crises and improve outcomes in pediatric patients.展开更多
Objective:Mycophenolate mofetil(MMF)is frequently prescribed to pediatric patients with nephrotic syndrome(NS);however,information on the active metabolite mycophenolic acid(MPA)in this population is limited.Method:Us...Objective:Mycophenolate mofetil(MMF)is frequently prescribed to pediatric patients with nephrotic syndrome(NS);however,information on the active metabolite mycophenolic acid(MPA)in this population is limited.Method:Using ultra-high performance liquid chromatography–tandem mass spectrometry(UHPLC–MS/MS),we validated a rapid and simple approach to measure MPA in accordance with the Bioanalytical Method Validation Guidance issued by the relevant regulatory authorities.Statistical analyses were performed on 69 pediatric patients with frequently relapsing/steroid-dependent nephrotic syndrome(FRNS/SDNS)who received corticosteroids plus MMF.Results:Forty-two patients achieved the endpoints and reported adverse events(AEs).The area under the concentration–time curve of MPA in the AE group was further distributed.Monitoring blood MPA concentrations is critical to prevent AEs during MMF administration in children with FRNS/SDNS.Conclusion:UHPLC–MS/MS offers a more accurate reference than immunoassays.展开更多
BACKGROUND Childhood nephrotic syndrome(NS)outcomes vary widely based on steroid responsiveness and complications.AIM To evaluate steroid response,outcomes,and the use of steroid-sparing medications in children with n...BACKGROUND Childhood nephrotic syndrome(NS)outcomes vary widely based on steroid responsiveness and complications.AIM To evaluate steroid response,outcomes,and the use of steroid-sparing medications in children with nephrotic syndrome in Jordan.METHODS This retrospective study evaluated the demographics and outcomes of 122 children aged 1-18 years with NS between 2011 and 2021 across three centers in Jordan.The outcomes assessed included steroid sensitivity rates,dependence,frequent relapses,complications[chronic kidney disease(CKD),end-stage kidney disease(ESKD)],infections,and need for steroid-sparing treatment.RESULTS Of 64%were boys;median age of disease onset was 4 years.Steroid-sensitive and steroid-resistant NS(SRNS)were observed in 81.1%and 18.9%of patients,respectively;28.7%and 9%had steroid-dependent and frequently relapsing NS,respectively.Kidney biopsies were conducted in 46.7%,the most common finding was minimal change disease in 56.1%;82.6%of biopsied SRNS cases showed focal segmental glomerulosclerosis.The median time to first relapse was 9 months.41.8%of patients required steroidsparing medications,with cyclosporine and mycophenolate mofetil being the most frequently used.Despite these treatments,relapse occurred in 11.5%of cases.Infections,primarily urinary tract infections,affected 24.6%of patients,7.4%progressed to high-grade CKD,and 6.6%required dialysis.SRNS was significantly associated with hematuria,hypertension,and ESKD.CONCLUSION Most patients were steroid sensitive,with minimal change being the most common.Focal segmental glomerulosclerosis was the predominant histopathology in the steroid-resistant cases.SRNS patients had worse outcomes,with more infections,CKD,and ESKD.展开更多
We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. ...We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. Each time the dose of steroid is tapered, a relapse of the nephrotic syndrome occurred. Eventually, the patient was complaining of dysphagia and difficulty swallowing. Hospital work-up with barium swallow, endoscopy, and CT of the chest, abdomen and pelvis, revealed a focal stenotic lesion with mild to moderate esophageal dysmotility 7/15/2022. A diagnosis of an ulcerating lesion with biopsy confirmed a neuro-endocrine carcinoma of the gastro-esophageal junction was entertained. The CT of the chest/abdomen/pelvis, 7/19/2022, has shown, an esophageal mass of 5.1 × 5.6 × 7 cm of the gastro-esophageal junction with ulceration. No evidence of spread beyond the esophagus and stomach. The histology revealed a poorly differentiated neuroendocrine tumor of the gastro-esophageal junction. The patient underwent several rounds of chemotherapy, radiation, and surgery culminating in tumor control. His nephrotic syndrome was resolved after the tumor has been controlled by surgery and chemotherapy.展开更多
BACKGROUND The interaction between the kidney and the thyroid is important for normal function of both organs.In nephrotic syndrome,proteinuria leads to loss of several proteins,which in turn causes hypothyroidism.AIM...BACKGROUND The interaction between the kidney and the thyroid is important for normal function of both organs.In nephrotic syndrome,proteinuria leads to loss of several proteins,which in turn causes hypothyroidism.AIM To assess the thyroid function in children with nephrotic syndrome.METHODS This cross-sectional study was conducted in a tertiary center,Bhopal,from February 2020 to January 2021.Consecutive children aged 1-15 years admitted with nephrotic syndrome(first-time diagnosed and all relapse cases)were included in the study.A thyroid profile was sent along with routine investigations,and thyroid hormone status was assessed in nephrotic syndrome children.RESULTS Of the 70 patients,39(55.7%)showed abnormal thyroid profiles;19(27.1%)had overt hypothyroidism,and 20(28.6%)had subclinical hypothyroidism.Overt hypothyroidism was seen in 16.1%of newly diagnosed cases,40%of second relapses,and 2.7%of frequently relapsed cases(P<0.001).The mean serum free T3 and free T4 levels in frequent relapses were 2.50±0.39 ng/dL and 0.78±0.12 ng/dL,respectively,which were significantly lower than in newly diagnosed cases(2.77±0.37 ng/dL and 0.91±0.19 ng/dL,respectively).The mean thyroidstimulating hormone(TSH)level was significantly higher in frequent relapses (5.86±1.56μIU/mL)and second relapse(5.81±1.78μIU/mL)than in newly diagnosed cases(4.83±0.76μIU/mL)and first relapse cases(4.74±1.17μIU/mL),(P<0.01).CONCLUSION An abnormal thyroid profile was commonly observed in children with nephrotic syndrome,and overt hypothyroidism was more common in frequent relapse cases.Therefore,thyroid screening should be a part of the management of nephrotic syndrome so that hypothyroidism can be detected and managed at an early stage.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
Objective: To explore better therapy and reduce the rate of re-relapse of primary nephritic syndrome in children who had been treated with corticosteroids but relapsed. Methods: Eighty relapsers were enrolled from Jan...Objective: To explore better therapy and reduce the rate of re-relapse of primary nephritic syndrome in children who had been treated with corticosteroids but relapsed. Methods: Eighty relapsers were enrolled from Jan. 1994 to Apr. 2000, who were randomly divided into two groups. The treatment group (n=39) had been treated with tripterysium glucosides for three months,with the control group (n=41) members were treated with cyclophosphmide (CTX) by intermission intravenous pulse, with total dose of CTX not being more than 150 mg/kg. Prednisone, meanwhile, was given to both groups. The total treatment period of prednisone was prolonged by 12-18 months. Results: After following up for 3-7 years, the re-relapse rates of both groups were observed. The re-relapse rate of the treatment group was 28.2% to 29.3% in the CTX-controlled group. The re-relapse rates between two groups were almost similar, and with no observed significant difference (P>0.05). The side effect of tripterysium glucosides was less than that of CTX. Conclusion: For the treatment of relapsing nephritic syndrome in children, the combination of tripterysium glucosides and prolonged corticosteroid therapy is as effective as the regimen of CTX plus prolonged use of prednisone.展开更多
This study aimed to investigate the effect and mechanisms of Ephedra Herb(EH)extract on adriamycin-induced nephrotic syndrome(NS),providing an experimental basis for the clinical treatment of NS.Hematoxylin and eosin ...This study aimed to investigate the effect and mechanisms of Ephedra Herb(EH)extract on adriamycin-induced nephrotic syndrome(NS),providing an experimental basis for the clinical treatment of NS.Hematoxylin and eosin staining,creatinine,urea nitrogen,and kidn injury molecule-1 were used to evaluate the activities of EH extract on renal function.The levels of inflammatory factors and oxidative stress were detected by kits.The levels of reactive oxygen species,immune cells,and apoptosis were measured by flow cytometry.A network pharmacological approach was used to predict the potential targets and mechanisms of EH extract in the treatment of NS.The protein levels of apoptosis-related proteins and CAMKK2,p-CAMKK2,AMPK,p-AMPK,mTOR and p-mTOR in the kidneys were detected by Western blot.The effective material basis of EH extract was screened by MTT assay.The AMPK pathway inhibitor(compound C,CC)was added to investigate the effect of the potent material basis on adriamycin-induced cell injury.EH extract significantly improved renal injury and relieve inflammation,oxidative stress,and apoptosis in rats.Network pharmacology and Western blot results showed that the effect of EH extract on NS may be associated with the CAMKK2/AMPK/mTOR signaling pathway.Moreover,methylephedrine significantly ameliorated adriamycin-induced NRK-52e cell injury.Methylephedrine also significantly improved the phosphorylation of AMPK and mTOR,which were blocked by CC.In sum,EH extract may ameliorate renal injury via the CAMKK2/AMPK/mTOR signaling pathway.Moreover,methylephedrine may be one of the material bases of EH extract.展开更多
Nephrotic syndrome(NS)is a kidney disease characterized by hypertriglyceridemia,massive proteinuria,hypo-albuminemia and peripheral edema.Sinkihwan-gamibang(SKHGMB)was recorded in a traditional Chinese medical book na...Nephrotic syndrome(NS)is a kidney disease characterized by hypertriglyceridemia,massive proteinuria,hypo-albuminemia and peripheral edema.Sinkihwan-gamibang(SKHGMB)was recorded in a traditional Chinese medical book named“Bangyakhappyeon(方藥合編)”and its three prescriptions Sinkihwan,Geumgwe-sinkihwan,and Jesaeng-sinkihwan belong to Gamibang.This study confirmed the effect of SKHGMB on renal dysfunction in an NS model induced by puromycin aminonucleoside(PAN).The experimental NS model was induced in male Sprague Dawley(SD)rats through injection of PAN(50 mg·kg^(-1))via the femoral vein.SKHGMB not only reduced the size of the kidneys increased due to PAN-induced NS,but also decreased proteinuria and ascites.In addition,SKHGMB significantly ameliorated creatinine clearance,creatinine,and blood urea nitrogen.SKHGMB relieved glomeruli dilation and tubules fibrosis in the glomeruli of the NS model.SKHGMB inhibited the protein and mRNA levels of the NLRP3 inflammasome including NLRP3,ASC,and pro-caspase-1 in NS rats.SKHGMB reduced the protein and mRNA levels of fibrosis regulators in NS rats.The results indicated that SKHGMB exerts protective effects against renal dysfunction by inhibiting of renal inflammation and fibrosis in NS rats.展开更多
BACKGROUND In paediatric patients with complicated nephrotic syndrome(NS), rituximab(RTX) administration can induce persistent IgG hypogammaglobulinemia among subjects showing low basal immunoglobulin G(IgG) levels.AI...BACKGROUND In paediatric patients with complicated nephrotic syndrome(NS), rituximab(RTX) administration can induce persistent IgG hypogammaglobulinemia among subjects showing low basal immunoglobulin G(IgG) levels.AIM To evaluate the effect of RTX on IgG levels and infections in patients with complicated NS and normal basal IgG levels.METHODS We consecutively enrolled all patients with complicated NS and normal basal IgG levels undergoing the first RTX infusion from January 2008 to January 2016. Basal IgG levels were dosed after 6 wk of absent proteinuria and with a maximal interval of 3 mo before RTX infusion. The primary outcome was the onset of IgG hypogammaglobulinemia during the follow-up according to the IgG normal values for age [mean ± standard deviation(SD)].RESULTS We enrolled 20 patients with mean age at NS diagnosis of 4.2 ± 3.3 years. The mean age at the first RTX infusion was 10.9 ± 3.5 years. Eleven out of twenty patients(55%) developed IgG hypogammaglobulinemia. None of these patients showed severe or recurrent infections. Only one patient suffered from recurrent acute otitis media and underwent substitutive IgG infusion. Three patients undergoing only the two "starting doses" experienced normalization of IgG levels. Using Kaplan-Meier analysis, the cumulative proportion of patients free of IgG hypogammaglobulinemia was 57.8% after the first RTX dose, 51.5% after the third dose, 44.1% after the fourth dose, and 35.5% after the fifth dose.CONCLUSION RTX can induce IgG hypogammaglobulinemia in patients with pre-RTX IgG normal values. None of the treated patients showed severe infections.展开更多
Nephrotic syndrome is one of the most common childhood kidney diseases. It is mostly found in the age group of 2 to 8 years. Around 10%-15% of nephrotic syndrome cases are non-responders of steroid treatment(SRNS).Ang...Nephrotic syndrome is one of the most common childhood kidney diseases. It is mostly found in the age group of 2 to 8 years. Around 10%-15% of nephrotic syndrome cases are non-responders of steroid treatment(SRNS).Angiotensin converting enzyme(ACE)(I/D) gene association studies are important for detecting kidney disease and herein we assessed the association of ACE(I/D) polymorphism with nephrotic syndrome in South Indian children. We recruited 260 nephrotic syndrome(162 boys and 98 girls) and 218(140 boys and 78 girls) control subjects. ACE I/D polymorphism was analyzed by PCR using genotype allele specific primers. In ACE(I/D), we did not find significant association for the ungrouped data of nephrotic syndrome children and the control subjects. Kidney biopsies were done in 86 nephrotic syndrome cases(minimal change disease, n = 51;focal segmental glomerulosclerosis, n = 27;diffuse mesangial proliferation, n = 8). We segregated them into the minimal change disease/focal segmental glomerulosclerosis groups and observed that the ACE’D’ allele was identified with borderline significance in cases of focal segmental glomerulosclerosis and the ’Ⅰ’ allele was assessed as having very weak association in cases of minimal change disease. ’Ⅱ’ genotype was weakly associated with minimal change disease. Gender specific analysis revealed weak association of’ID’ genotype with female nephrotic syndrome in females. Dominant expression of DD genotype was observed in males with nephrotic syndrome. Our finding indicated that ACE(I/D) has moderate association with focal segmental glomerulosclerosis. However, due to the limited number of biopsy proven focal segmental glomerulosclerosis subjects enrolled, further studies are required to confirm these results.展开更多
Dear editor,Nephrotic syndrome is defined by the presence of peripheral edema,heavy proteinuria(greater than 3.5 g/24h),and hypoalbuminemia(less than 3 g/dL).^[1]Nephrotic syndrome is relatively rare,with an incidence...Dear editor,Nephrotic syndrome is defined by the presence of peripheral edema,heavy proteinuria(greater than 3.5 g/24h),and hypoalbuminemia(less than 3 g/dL).^[1]Nephrotic syndrome is relatively rare,with an incidence of 3 new patients per 100,000 per year in adults.^[1]Despite being a known cause for new onset edema in patients at any age,nephrotic syndrome is often neglected in considering differential diagnoses for this presentation in primary care settings,and initial workups often focus on ruling out cardiac and hepatic causes of edema.^[1-3]In this case report,we describe a 25-year-old male patient who presented to the emergency department(ED)complaining of a 10-day history of anasarca.He was later diagnosed with nephrotic syndrome secondary to minimal change disease.This case served as a reminder to include the differential diagnosis of nephrotic syndrome early in the workup of an adult with peripheral edema presenting to the ED.展开更多
Objective:To explore the effect of Gushen tablet (固肾片,GST) in reducing the relapse of children's nephrotic syndrome and the possible mechanism of drugs used. Methods: Fifty children with primary nephrotic syndr...Objective:To explore the effect of Gushen tablet (固肾片,GST) in reducing the relapse of children's nephrotic syndrome and the possible mechanism of drugs used. Methods: Fifty children with primary nephrotic syndrome who had been induced and alleviated with regular glucocorticoid (GC) were randomly divided into two groups: the GST group used GST and standard middle-long term course of GC, and the control group adopted standard middle-long term course of GC and immunoinhibitory or immuno-modulatory agents for treatment. The 0.5,1 and 2 years after the treatment the relapse episodes, time for urinary protein negative conversion after relapse, the episodes of patient's infection and relapse after infection were evaluated. Before and after treatment the plasma cortisol and T lymphocyte subpopulation were determined. Results: The relapse rate of GST group: the rates after 0. 5, 1, 2 years were 20.0%, 30. 0% and 40. 9%, and the frequent relapse rate were 0, 6. 7% and 9. 2% respectively, which were lower than those of control group (60. 0%, 70. 0%, 69. 2% and 25. 0%, 15. 0%, 15. 4% respectively) ; in the GST group no relapse occurred within 0. 5 year, the relapse rate after 1 and 2 years reduced by 40. 0% and 28. 3%, compared with those of the control group (all P<0. 05) ; during the observation period, the mean infection/every child patient was 1. 86 episodes in GST group, after infection the nephrotic relapse rate was 28.3%, which was lower than that of the control group (2. 25 episodes, 71.1%, P<0. 05) > the relapse per patient in GST group was 0. 8 episodes, time for urinary protein negative conversion was 12. 00± 8. 98 days, lower than those of control group (1. 6 episodes, 20. 75±11. 95 days, P<0. 05) ; 3 months after GST treatment the plasma cortisol level normalized, and the CD4/CD8 ratio elevated (P<0. 05). Conclusion:GST could possibly reduce the relapse of children nephrosis, and the frequent relapse and relapse episodes, and the time for post-relaptic urinary protein negative conversion shortened, the plasma cortisol elevated, and the adjustment of cellular immunity disturbance promoted.展开更多
Nephrotic syndrome(NS) is the most common glomerular disease of childhood. Steroid-dependent and steroid-resistant nephrotic syndrome present challenges in their pharmaceutical management; patients may need several im...Nephrotic syndrome(NS) is the most common glomerular disease of childhood. Steroid-dependent and steroid-resistant nephrotic syndrome present challenges in their pharmaceutical management; patients may need several immunosuppressive medication for optimum control, each of which medication has its own safety profile. Rituximab(RTX) is a monoclonal antibody that targets B cells and has been used successfully for management of lymphoma and rheumatoid arthritis. Recent clinical studies showed that rituximab may be an efficacious and safe alternative for the treatment of complicated nephrotic syndrome. In this review article, we aim to review the efficacy and safety of RTX therapy in nephrotic syndrome. We reviewed the literature pertaining to this topic by searching for relevant studies on Pub Med and Medline using specific keywords. The initial search yielded 452 articles. These articles were then examined to ensure their relevance to the topic of research. We focused on multicenter randomized controlled trials with relatively large numbers of patients. A total of 29 articles were finally identified and will be summarized in this review. The majority of clinical studies of RTX in complicated pediatric NS showed that rituximab is effective in approximately 80% of patients with steroid-dependent NS, as it decreases the number of relapses and steroid dosage. However, RTX is less effective at achieving remission in steroid-resistant NS. RTX use was generally safe, and most side effects were transient and infusion-related. More randomized, double-blinded clinical studies are needed to assess the role of RTX in children with nephrotic syndrome.展开更多
BACKGROUND Waldenstr?m’s macroglobulinemia(WM) is a rare lymphoid neoplasia, which can have renal complications. These rarely occur, and most common renal manifestations are mild proteinuria and microscopic hematuria...BACKGROUND Waldenstr?m’s macroglobulinemia(WM) is a rare lymphoid neoplasia, which can have renal complications. These rarely occur, and most common renal manifestations are mild proteinuria and microscopic hematuria. Herein we describe a case of WM that presented with pseudothrombi depositing in capillaries associated with minimal change nephrotic syndrome and chronic kidney disease(CKD).CASE SUMMARY A 52-year-old man presented with features suggesting nephrotic syndrome.Extensive workups were done, and there were elevated serum levels of interleukin-6 and vascular endothelial growth factor(VEGF), capillary pseudothrombus accumulation associated with minimal change nephrotic syndrome, CKD, and WM. Treatment was directed at the patient’s WM with bortezomib, thalidomide, and dexamethasone whereby serum immunoglobulin M(IgM) decreased. The damage of IgM on the kidney was corrected; thus, the patient’s proteinuria and serum creatinine had improved. The patient is still under clinical follow-up.CONCLUSION It is essential for clinicians to promptly pay more attention to patients presenting with features of nephrotic syndrome and do extensive workups to come up with a proper therapy strategy.展开更多
Objective: To observe the clinical efficacy and safety of low dose tacrolimus (TAC) combined with tripterygium (TW) in treatment of steroid resistant nephritic syndrome (SRNS). Method: The patients, who were diagnosed...Objective: To observe the clinical efficacy and safety of low dose tacrolimus (TAC) combined with tripterygium (TW) in treatment of steroid resistant nephritic syndrome (SRNS). Method: The patients, who were diagnosed with mesangial proliferative glomerulonephritis (MesPGN) and focal segmental glomerulosclerosis (FSGS) by biopsy and failed to respond to a 3-month treatment with prednisone (1 mg/kg·d), were randomly divided into 2 groups (TAC + TW Group and TW Group). Initially TAC + TW group took TAC 0.05mg/(kg·d) 2 h after meal at 12 h interval. The plasma TAC level was examined after 3 days and was kept at 1.5 - 4 ng·ml;meanwhile, TW was given at 60 mg/d before meal. TW group only took TW (60 mg/d). The efficacy, adverse reactions and plasma TAC levels were observed in each group. Results: 1) Totally 20 SRNS patients completed the trial, 11 of TAC + TW Group and 9 of TW Group. There is no statistical difference between the two groups in terms of age, gender, duration since onset of the disease, blood pressure, 24 h UPQ, serum albumin, creatinine, cholesterol, triglyceride, FBG, kidney pathological categories, time of taking prednisone etc.;2) Urine protein started to decrease after 1 month treatment in both of TAC + TW and TW groups. By the 12th month of treatment, TAC + TW group showed 8 cases of complete remission (72.7%), 2 cases of partial remission (18.2%) and 1 case of no improvement (9.1%), while those of TW groups were 2 (22.2%), 4 (44.5%) and 3 (33.3%), respectively;3) With treatment, the TAC + TW Group patients’ plasma protein was significantly higher than that of pretreatment stage and recovered to normal level after 6 month of treatment. However, there was no significant plasma protein increase in TW Group. No obvious changes were observed on serum creatinine level of patients of both the two groups;4) The incidence of adverse reactions was not significantly different between the two groups. Conclusion: TAC + TW reduced proteinuria of SRNS patients, increased clinical remission rate and was tolerant to SRNS patients. We conclude that TAC + TW treatment is an effective way to treat patients with SRNS.展开更多
THROMBOEMBOLISM is a well-known complicationof nephrotic syndrome. Deep vein thrombosis,pulmonary embolism and renal vein thrombosisare the most common venous thromboembolicdiseases in patients with nephrotic syndrome...THROMBOEMBOLISM is a well-known complicationof nephrotic syndrome. Deep vein thrombosis,pulmonary embolism and renal vein thrombosisare the most common venous thromboembolicdiseases in patients with nephrotic syndrome, while arterialthromboembolic complications are observed less frequently,and rarely when it occurs before the diagnosis of nephroticsyndrome.1 Here we report a case with minimal change ofnephrotic syndrome who presented initially as external iliacartery thrombosis, which may be associated with longsitting while playing Mah-Jong.展开更多
BACKGROUND Hypereosinophilia(HE)is a heterogeneous disease of unknown etiology in which tissue and organ injury is inflicted by excess numbers of circulating or infiltrating eosinophils.Herein,we describe a patient wi...BACKGROUND Hypereosinophilia(HE)is a heterogeneous disease of unknown etiology in which tissue and organ injury is inflicted by excess numbers of circulating or infiltrating eosinophils.Herein,we describe a patient with rare organ damage due to HE and review the pertinent literature.CASE SUMMARY A 43 year-old Chinese man with a 13-year history of eosinophilia and shortness of breath for 7 d presented to our hospital.During the course of his illness,the patient variably presented with gastrointestinal symptoms,eczema,vitiligo,mastitis,joint symptoms,nephrotic syndrome,and interstitial pneumonia.The chronic mastitis proved burdensome,necessitating bilateral mastectomy.HE was diagnosed by repeat bone marrow biopsy,and a kidney biopsy showed focal segmental glomerulosclerosis.Intermittent steroidal therapy is typically initiated to relieve such symptoms,although relapse and organ involvement often ensue once treatment is withdrawn.We administered methylprednisolone sodium succinate(40 mg/d)intravenously for 3 d,followed by oral tablets at the same dose.Subsequent computed tomography(CT)of the chest CT showed relative improvement of the interstitial pneumonia.The patient is currently on a continuous regimen of oral steroid,and his condition is stable.CONCLUSION HE is heterogeneous condition.This is the first reported case of bilateral mastectomy in a male patient with longstanding HE.展开更多
文摘Objective:To evaluate the value of rehabilitation nursing based on mind mapping model combined with psychological intervention for patients with nephrotic syndrome(NS).Methods:A total of 60 patients with NS who visited our hospital from January 2024 to December 2024 were selected as samples and randomly divided into groups.The observation group received rehabilitation nursing based on the mind mapping model combined with psychological intervention,while the control group received routine intervention.The differences in emotional scores,self-care ability scores,compliance,and complications were compared between the two groups.Results:The anxiety(SAS)and depression(SDS)scores of the observation group were lower than those of the control group,while the self-care ability scale(ESCA)score was higher than that of the control group(P<0.05).The compliance rate of the observation group was higher than that of the control group(P<0.05).The complication rate of NS in the observation group was lower than that in the control group(P<0.05).Conclusion:Rehabilitation nursing based on the mind mapping model combined with psychological intervention can enhance self-care ability,reduce negative emotions,and reduce complications in NS nursing,which is efficient and feasible.
文摘The underlying molecular changes that result in minimal change disease(ne-phrotic syndrome)require an in-depth analysis.Current molecular studies have shown the involvement of zinc fingers and homeobox transcriptional factors in its pathogenesis.The application of therapeutic drugs relies on understanding the cascade of molecular events to determine their efficacy in managing the clinical condition.
文摘Glucocorticoids(GCs)such as prednisolone are widely used in conditions like nephrotic syndrome,asthma,and autoimmune diseases.However,prolonged or high-dose use may suppress the hypothalamic-pituitary-adrenal(HPA)axis,leading to secondary adrenal insufficiency(AI).This condition occurs when the adrenal glands fail to produce adequate cortisol,which is essential for regulating metabolism,immune response,and stress adaptation.Corticotropin-releasing hormone(CRH)from the hypothalamus stimulates the pituitary to release adrenocorticotropic hormone(ACTH),which then triggers cortisol production in the adrenal glands.Prolonged GC use disrupts this system by inhibiting CRH and ACTH secretion,leading to adrenal atrophy and reduced cortisol production.HPA axis suppression is primarily diagnosed through dynamic tests.Early morning cortisol levels above>18 ng/mL typically indicate normal function,while levels<3 ng/mL suggest AI.Intermediate values require additional testing,such as the insulin tolerance test,ACTH stimulation test,and metyrapone test.Prednisolone in nephrotic syndrome suppresses the HPA axis,heightening AI risk,influenced by dose,duration,and timing of administration.Careful GC management is essential to balance disease control with risks of HPA axis suppression.Early recognition and timely intervention can prevent adrenal crises and improve outcomes in pediatric patients.
文摘Objective:Mycophenolate mofetil(MMF)is frequently prescribed to pediatric patients with nephrotic syndrome(NS);however,information on the active metabolite mycophenolic acid(MPA)in this population is limited.Method:Using ultra-high performance liquid chromatography–tandem mass spectrometry(UHPLC–MS/MS),we validated a rapid and simple approach to measure MPA in accordance with the Bioanalytical Method Validation Guidance issued by the relevant regulatory authorities.Statistical analyses were performed on 69 pediatric patients with frequently relapsing/steroid-dependent nephrotic syndrome(FRNS/SDNS)who received corticosteroids plus MMF.Results:Forty-two patients achieved the endpoints and reported adverse events(AEs).The area under the concentration–time curve of MPA in the AE group was further distributed.Monitoring blood MPA concentrations is critical to prevent AEs during MMF administration in children with FRNS/SDNS.Conclusion:UHPLC–MS/MS offers a more accurate reference than immunoassays.
文摘BACKGROUND Childhood nephrotic syndrome(NS)outcomes vary widely based on steroid responsiveness and complications.AIM To evaluate steroid response,outcomes,and the use of steroid-sparing medications in children with nephrotic syndrome in Jordan.METHODS This retrospective study evaluated the demographics and outcomes of 122 children aged 1-18 years with NS between 2011 and 2021 across three centers in Jordan.The outcomes assessed included steroid sensitivity rates,dependence,frequent relapses,complications[chronic kidney disease(CKD),end-stage kidney disease(ESKD)],infections,and need for steroid-sparing treatment.RESULTS Of 64%were boys;median age of disease onset was 4 years.Steroid-sensitive and steroid-resistant NS(SRNS)were observed in 81.1%and 18.9%of patients,respectively;28.7%and 9%had steroid-dependent and frequently relapsing NS,respectively.Kidney biopsies were conducted in 46.7%,the most common finding was minimal change disease in 56.1%;82.6%of biopsied SRNS cases showed focal segmental glomerulosclerosis.The median time to first relapse was 9 months.41.8%of patients required steroidsparing medications,with cyclosporine and mycophenolate mofetil being the most frequently used.Despite these treatments,relapse occurred in 11.5%of cases.Infections,primarily urinary tract infections,affected 24.6%of patients,7.4%progressed to high-grade CKD,and 6.6%required dialysis.SRNS was significantly associated with hematuria,hypertension,and ESKD.CONCLUSION Most patients were steroid sensitive,with minimal change being the most common.Focal segmental glomerulosclerosis was the predominant histopathology in the steroid-resistant cases.SRNS patients had worse outcomes,with more infections,CKD,and ESKD.
文摘We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. Each time the dose of steroid is tapered, a relapse of the nephrotic syndrome occurred. Eventually, the patient was complaining of dysphagia and difficulty swallowing. Hospital work-up with barium swallow, endoscopy, and CT of the chest, abdomen and pelvis, revealed a focal stenotic lesion with mild to moderate esophageal dysmotility 7/15/2022. A diagnosis of an ulcerating lesion with biopsy confirmed a neuro-endocrine carcinoma of the gastro-esophageal junction was entertained. The CT of the chest/abdomen/pelvis, 7/19/2022, has shown, an esophageal mass of 5.1 × 5.6 × 7 cm of the gastro-esophageal junction with ulceration. No evidence of spread beyond the esophagus and stomach. The histology revealed a poorly differentiated neuroendocrine tumor of the gastro-esophageal junction. The patient underwent several rounds of chemotherapy, radiation, and surgery culminating in tumor control. His nephrotic syndrome was resolved after the tumor has been controlled by surgery and chemotherapy.
文摘BACKGROUND The interaction between the kidney and the thyroid is important for normal function of both organs.In nephrotic syndrome,proteinuria leads to loss of several proteins,which in turn causes hypothyroidism.AIM To assess the thyroid function in children with nephrotic syndrome.METHODS This cross-sectional study was conducted in a tertiary center,Bhopal,from February 2020 to January 2021.Consecutive children aged 1-15 years admitted with nephrotic syndrome(first-time diagnosed and all relapse cases)were included in the study.A thyroid profile was sent along with routine investigations,and thyroid hormone status was assessed in nephrotic syndrome children.RESULTS Of the 70 patients,39(55.7%)showed abnormal thyroid profiles;19(27.1%)had overt hypothyroidism,and 20(28.6%)had subclinical hypothyroidism.Overt hypothyroidism was seen in 16.1%of newly diagnosed cases,40%of second relapses,and 2.7%of frequently relapsed cases(P<0.001).The mean serum free T3 and free T4 levels in frequent relapses were 2.50±0.39 ng/dL and 0.78±0.12 ng/dL,respectively,which were significantly lower than in newly diagnosed cases(2.77±0.37 ng/dL and 0.91±0.19 ng/dL,respectively).The mean thyroidstimulating hormone(TSH)level was significantly higher in frequent relapses (5.86±1.56μIU/mL)and second relapse(5.81±1.78μIU/mL)than in newly diagnosed cases(4.83±0.76μIU/mL)and first relapse cases(4.74±1.17μIU/mL),(P<0.01).CONCLUSION An abnormal thyroid profile was commonly observed in children with nephrotic syndrome,and overt hypothyroidism was more common in frequent relapse cases.Therefore,thyroid screening should be a part of the management of nephrotic syndrome so that hypothyroidism can be detected and managed at an early stage.
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Objective: To explore better therapy and reduce the rate of re-relapse of primary nephritic syndrome in children who had been treated with corticosteroids but relapsed. Methods: Eighty relapsers were enrolled from Jan. 1994 to Apr. 2000, who were randomly divided into two groups. The treatment group (n=39) had been treated with tripterysium glucosides for three months,with the control group (n=41) members were treated with cyclophosphmide (CTX) by intermission intravenous pulse, with total dose of CTX not being more than 150 mg/kg. Prednisone, meanwhile, was given to both groups. The total treatment period of prednisone was prolonged by 12-18 months. Results: After following up for 3-7 years, the re-relapse rates of both groups were observed. The re-relapse rate of the treatment group was 28.2% to 29.3% in the CTX-controlled group. The re-relapse rates between two groups were almost similar, and with no observed significant difference (P>0.05). The side effect of tripterysium glucosides was less than that of CTX. Conclusion: For the treatment of relapsing nephritic syndrome in children, the combination of tripterysium glucosides and prolonged corticosteroid therapy is as effective as the regimen of CTX plus prolonged use of prednisone.
基金the National Key Research and Development Project(Nos.2019YFC1708802 and 2017YFC1702800)Henan province high-level personnel special support“ZhongYuan One Thousand People Pla”,Zhongyuan Leading Talent(No.ZYQR201810080)the Major Science and Technology Projects in Henan Province:Study on the key technology for quality control and the key characteristics of Rehmannia glutinosa,Dioscorea opposita Thunb.and Achyranthes bidentata Blume from Henan Province(No.171100310500).
文摘This study aimed to investigate the effect and mechanisms of Ephedra Herb(EH)extract on adriamycin-induced nephrotic syndrome(NS),providing an experimental basis for the clinical treatment of NS.Hematoxylin and eosin staining,creatinine,urea nitrogen,and kidn injury molecule-1 were used to evaluate the activities of EH extract on renal function.The levels of inflammatory factors and oxidative stress were detected by kits.The levels of reactive oxygen species,immune cells,and apoptosis were measured by flow cytometry.A network pharmacological approach was used to predict the potential targets and mechanisms of EH extract in the treatment of NS.The protein levels of apoptosis-related proteins and CAMKK2,p-CAMKK2,AMPK,p-AMPK,mTOR and p-mTOR in the kidneys were detected by Western blot.The effective material basis of EH extract was screened by MTT assay.The AMPK pathway inhibitor(compound C,CC)was added to investigate the effect of the potent material basis on adriamycin-induced cell injury.EH extract significantly improved renal injury and relieve inflammation,oxidative stress,and apoptosis in rats.Network pharmacology and Western blot results showed that the effect of EH extract on NS may be associated with the CAMKK2/AMPK/mTOR signaling pathway.Moreover,methylephedrine significantly ameliorated adriamycin-induced NRK-52e cell injury.Methylephedrine also significantly improved the phosphorylation of AMPK and mTOR,which were blocked by CC.In sum,EH extract may ameliorate renal injury via the CAMKK2/AMPK/mTOR signaling pathway.Moreover,methylephedrine may be one of the material bases of EH extract.
基金supported by the National Research Foundation of Korea(NRF)grant funded by the Korean government(MSIP)(Nos.2017R1A5A2015805,2021R1A2C1010859,and 2021R1C1C2009542).
文摘Nephrotic syndrome(NS)is a kidney disease characterized by hypertriglyceridemia,massive proteinuria,hypo-albuminemia and peripheral edema.Sinkihwan-gamibang(SKHGMB)was recorded in a traditional Chinese medical book named“Bangyakhappyeon(方藥合編)”and its three prescriptions Sinkihwan,Geumgwe-sinkihwan,and Jesaeng-sinkihwan belong to Gamibang.This study confirmed the effect of SKHGMB on renal dysfunction in an NS model induced by puromycin aminonucleoside(PAN).The experimental NS model was induced in male Sprague Dawley(SD)rats through injection of PAN(50 mg·kg^(-1))via the femoral vein.SKHGMB not only reduced the size of the kidneys increased due to PAN-induced NS,but also decreased proteinuria and ascites.In addition,SKHGMB significantly ameliorated creatinine clearance,creatinine,and blood urea nitrogen.SKHGMB relieved glomeruli dilation and tubules fibrosis in the glomeruli of the NS model.SKHGMB inhibited the protein and mRNA levels of the NLRP3 inflammasome including NLRP3,ASC,and pro-caspase-1 in NS rats.SKHGMB reduced the protein and mRNA levels of fibrosis regulators in NS rats.The results indicated that SKHGMB exerts protective effects against renal dysfunction by inhibiting of renal inflammation and fibrosis in NS rats.
文摘BACKGROUND In paediatric patients with complicated nephrotic syndrome(NS), rituximab(RTX) administration can induce persistent IgG hypogammaglobulinemia among subjects showing low basal immunoglobulin G(IgG) levels.AIM To evaluate the effect of RTX on IgG levels and infections in patients with complicated NS and normal basal IgG levels.METHODS We consecutively enrolled all patients with complicated NS and normal basal IgG levels undergoing the first RTX infusion from January 2008 to January 2016. Basal IgG levels were dosed after 6 wk of absent proteinuria and with a maximal interval of 3 mo before RTX infusion. The primary outcome was the onset of IgG hypogammaglobulinemia during the follow-up according to the IgG normal values for age [mean ± standard deviation(SD)].RESULTS We enrolled 20 patients with mean age at NS diagnosis of 4.2 ± 3.3 years. The mean age at the first RTX infusion was 10.9 ± 3.5 years. Eleven out of twenty patients(55%) developed IgG hypogammaglobulinemia. None of these patients showed severe or recurrent infections. Only one patient suffered from recurrent acute otitis media and underwent substitutive IgG infusion. Three patients undergoing only the two "starting doses" experienced normalization of IgG levels. Using Kaplan-Meier analysis, the cumulative proportion of patients free of IgG hypogammaglobulinemia was 57.8% after the first RTX dose, 51.5% after the third dose, 44.1% after the fourth dose, and 35.5% after the fifth dose.CONCLUSION RTX can induce IgG hypogammaglobulinemia in patients with pre-RTX IgG normal values. None of the treated patients showed severe infections.
基金supported by Networking Resource Centre in Biological Sciences (NRCBS funded by UGC, Govt. of India)
文摘Nephrotic syndrome is one of the most common childhood kidney diseases. It is mostly found in the age group of 2 to 8 years. Around 10%-15% of nephrotic syndrome cases are non-responders of steroid treatment(SRNS).Angiotensin converting enzyme(ACE)(I/D) gene association studies are important for detecting kidney disease and herein we assessed the association of ACE(I/D) polymorphism with nephrotic syndrome in South Indian children. We recruited 260 nephrotic syndrome(162 boys and 98 girls) and 218(140 boys and 78 girls) control subjects. ACE I/D polymorphism was analyzed by PCR using genotype allele specific primers. In ACE(I/D), we did not find significant association for the ungrouped data of nephrotic syndrome children and the control subjects. Kidney biopsies were done in 86 nephrotic syndrome cases(minimal change disease, n = 51;focal segmental glomerulosclerosis, n = 27;diffuse mesangial proliferation, n = 8). We segregated them into the minimal change disease/focal segmental glomerulosclerosis groups and observed that the ACE’D’ allele was identified with borderline significance in cases of focal segmental glomerulosclerosis and the ’Ⅰ’ allele was assessed as having very weak association in cases of minimal change disease. ’Ⅱ’ genotype was weakly associated with minimal change disease. Gender specific analysis revealed weak association of’ID’ genotype with female nephrotic syndrome in females. Dominant expression of DD genotype was observed in males with nephrotic syndrome. Our finding indicated that ACE(I/D) has moderate association with focal segmental glomerulosclerosis. However, due to the limited number of biopsy proven focal segmental glomerulosclerosis subjects enrolled, further studies are required to confirm these results.
文摘Dear editor,Nephrotic syndrome is defined by the presence of peripheral edema,heavy proteinuria(greater than 3.5 g/24h),and hypoalbuminemia(less than 3 g/dL).^[1]Nephrotic syndrome is relatively rare,with an incidence of 3 new patients per 100,000 per year in adults.^[1]Despite being a known cause for new onset edema in patients at any age,nephrotic syndrome is often neglected in considering differential diagnoses for this presentation in primary care settings,and initial workups often focus on ruling out cardiac and hepatic causes of edema.^[1-3]In this case report,we describe a 25-year-old male patient who presented to the emergency department(ED)complaining of a 10-day history of anasarca.He was later diagnosed with nephrotic syndrome secondary to minimal change disease.This case served as a reminder to include the differential diagnosis of nephrotic syndrome early in the workup of an adult with peripheral edema presenting to the ED.
基金The item was supported by the Science and Technology Department of Henan Province (No. 941200528)
文摘Objective:To explore the effect of Gushen tablet (固肾片,GST) in reducing the relapse of children's nephrotic syndrome and the possible mechanism of drugs used. Methods: Fifty children with primary nephrotic syndrome who had been induced and alleviated with regular glucocorticoid (GC) were randomly divided into two groups: the GST group used GST and standard middle-long term course of GC, and the control group adopted standard middle-long term course of GC and immunoinhibitory or immuno-modulatory agents for treatment. The 0.5,1 and 2 years after the treatment the relapse episodes, time for urinary protein negative conversion after relapse, the episodes of patient's infection and relapse after infection were evaluated. Before and after treatment the plasma cortisol and T lymphocyte subpopulation were determined. Results: The relapse rate of GST group: the rates after 0. 5, 1, 2 years were 20.0%, 30. 0% and 40. 9%, and the frequent relapse rate were 0, 6. 7% and 9. 2% respectively, which were lower than those of control group (60. 0%, 70. 0%, 69. 2% and 25. 0%, 15. 0%, 15. 4% respectively) ; in the GST group no relapse occurred within 0. 5 year, the relapse rate after 1 and 2 years reduced by 40. 0% and 28. 3%, compared with those of the control group (all P<0. 05) ; during the observation period, the mean infection/every child patient was 1. 86 episodes in GST group, after infection the nephrotic relapse rate was 28.3%, which was lower than that of the control group (2. 25 episodes, 71.1%, P<0. 05) > the relapse per patient in GST group was 0. 8 episodes, time for urinary protein negative conversion was 12. 00± 8. 98 days, lower than those of control group (1. 6 episodes, 20. 75±11. 95 days, P<0. 05) ; 3 months after GST treatment the plasma cortisol level normalized, and the CD4/CD8 ratio elevated (P<0. 05). Conclusion:GST could possibly reduce the relapse of children nephrosis, and the frequent relapse and relapse episodes, and the time for post-relaptic urinary protein negative conversion shortened, the plasma cortisol elevated, and the adjustment of cellular immunity disturbance promoted.
文摘Nephrotic syndrome(NS) is the most common glomerular disease of childhood. Steroid-dependent and steroid-resistant nephrotic syndrome present challenges in their pharmaceutical management; patients may need several immunosuppressive medication for optimum control, each of which medication has its own safety profile. Rituximab(RTX) is a monoclonal antibody that targets B cells and has been used successfully for management of lymphoma and rheumatoid arthritis. Recent clinical studies showed that rituximab may be an efficacious and safe alternative for the treatment of complicated nephrotic syndrome. In this review article, we aim to review the efficacy and safety of RTX therapy in nephrotic syndrome. We reviewed the literature pertaining to this topic by searching for relevant studies on Pub Med and Medline using specific keywords. The initial search yielded 452 articles. These articles were then examined to ensure their relevance to the topic of research. We focused on multicenter randomized controlled trials with relatively large numbers of patients. A total of 29 articles were finally identified and will be summarized in this review. The majority of clinical studies of RTX in complicated pediatric NS showed that rituximab is effective in approximately 80% of patients with steroid-dependent NS, as it decreases the number of relapses and steroid dosage. However, RTX is less effective at achieving remission in steroid-resistant NS. RTX use was generally safe, and most side effects were transient and infusion-related. More randomized, double-blinded clinical studies are needed to assess the role of RTX in children with nephrotic syndrome.
文摘BACKGROUND Waldenstr?m’s macroglobulinemia(WM) is a rare lymphoid neoplasia, which can have renal complications. These rarely occur, and most common renal manifestations are mild proteinuria and microscopic hematuria. Herein we describe a case of WM that presented with pseudothrombi depositing in capillaries associated with minimal change nephrotic syndrome and chronic kidney disease(CKD).CASE SUMMARY A 52-year-old man presented with features suggesting nephrotic syndrome.Extensive workups were done, and there were elevated serum levels of interleukin-6 and vascular endothelial growth factor(VEGF), capillary pseudothrombus accumulation associated with minimal change nephrotic syndrome, CKD, and WM. Treatment was directed at the patient’s WM with bortezomib, thalidomide, and dexamethasone whereby serum immunoglobulin M(IgM) decreased. The damage of IgM on the kidney was corrected; thus, the patient’s proteinuria and serum creatinine had improved. The patient is still under clinical follow-up.CONCLUSION It is essential for clinicians to promptly pay more attention to patients presenting with features of nephrotic syndrome and do extensive workups to come up with a proper therapy strategy.
文摘Objective: To observe the clinical efficacy and safety of low dose tacrolimus (TAC) combined with tripterygium (TW) in treatment of steroid resistant nephritic syndrome (SRNS). Method: The patients, who were diagnosed with mesangial proliferative glomerulonephritis (MesPGN) and focal segmental glomerulosclerosis (FSGS) by biopsy and failed to respond to a 3-month treatment with prednisone (1 mg/kg·d), were randomly divided into 2 groups (TAC + TW Group and TW Group). Initially TAC + TW group took TAC 0.05mg/(kg·d) 2 h after meal at 12 h interval. The plasma TAC level was examined after 3 days and was kept at 1.5 - 4 ng·ml;meanwhile, TW was given at 60 mg/d before meal. TW group only took TW (60 mg/d). The efficacy, adverse reactions and plasma TAC levels were observed in each group. Results: 1) Totally 20 SRNS patients completed the trial, 11 of TAC + TW Group and 9 of TW Group. There is no statistical difference between the two groups in terms of age, gender, duration since onset of the disease, blood pressure, 24 h UPQ, serum albumin, creatinine, cholesterol, triglyceride, FBG, kidney pathological categories, time of taking prednisone etc.;2) Urine protein started to decrease after 1 month treatment in both of TAC + TW and TW groups. By the 12th month of treatment, TAC + TW group showed 8 cases of complete remission (72.7%), 2 cases of partial remission (18.2%) and 1 case of no improvement (9.1%), while those of TW groups were 2 (22.2%), 4 (44.5%) and 3 (33.3%), respectively;3) With treatment, the TAC + TW Group patients’ plasma protein was significantly higher than that of pretreatment stage and recovered to normal level after 6 month of treatment. However, there was no significant plasma protein increase in TW Group. No obvious changes were observed on serum creatinine level of patients of both the two groups;4) The incidence of adverse reactions was not significantly different between the two groups. Conclusion: TAC + TW reduced proteinuria of SRNS patients, increased clinical remission rate and was tolerant to SRNS patients. We conclude that TAC + TW treatment is an effective way to treat patients with SRNS.
基金Funded by Obershi blood purification academician workstation of Guangdong Province(2013B090400004)
文摘THROMBOEMBOLISM is a well-known complicationof nephrotic syndrome. Deep vein thrombosis,pulmonary embolism and renal vein thrombosisare the most common venous thromboembolicdiseases in patients with nephrotic syndrome, while arterialthromboembolic complications are observed less frequently,and rarely when it occurs before the diagnosis of nephroticsyndrome.1 Here we report a case with minimal change ofnephrotic syndrome who presented initially as external iliacartery thrombosis, which may be associated with longsitting while playing Mah-Jong.
文摘BACKGROUND Hypereosinophilia(HE)is a heterogeneous disease of unknown etiology in which tissue and organ injury is inflicted by excess numbers of circulating or infiltrating eosinophils.Herein,we describe a patient with rare organ damage due to HE and review the pertinent literature.CASE SUMMARY A 43 year-old Chinese man with a 13-year history of eosinophilia and shortness of breath for 7 d presented to our hospital.During the course of his illness,the patient variably presented with gastrointestinal symptoms,eczema,vitiligo,mastitis,joint symptoms,nephrotic syndrome,and interstitial pneumonia.The chronic mastitis proved burdensome,necessitating bilateral mastectomy.HE was diagnosed by repeat bone marrow biopsy,and a kidney biopsy showed focal segmental glomerulosclerosis.Intermittent steroidal therapy is typically initiated to relieve such symptoms,although relapse and organ involvement often ensue once treatment is withdrawn.We administered methylprednisolone sodium succinate(40 mg/d)intravenously for 3 d,followed by oral tablets at the same dose.Subsequent computed tomography(CT)of the chest CT showed relative improvement of the interstitial pneumonia.The patient is currently on a continuous regimen of oral steroid,and his condition is stable.CONCLUSION HE is heterogeneous condition.This is the first reported case of bilateral mastectomy in a male patient with longstanding HE.
