Objective: To analyze the epidemiological characteristics of growth, as well as factors associated with growth retardation in children with primary nephrotic syndrome (PNS), and to investigate the effect of glucocorti...Objective: To analyze the epidemiological characteristics of growth, as well as factors associated with growth retardation in children with primary nephrotic syndrome (PNS), and to investigate the effect of glucocorticoid (GC) use duration on growth retardation in these children. Methods: Clinical and laboratory data of 353 PNS children treated at our hospital from July 2014 to June 2015 were collected through the medical record management system. Height, weight, and GC usage were recorded. Follow-up assessments were conducted in August 2022 for the original group, recording height, weight, and GC usage. Height and weight were evaluated using standard deviation scores (SDS). Categorical data were analyzed using chi-square test while continuous measurement data were analyzed using t-test or rank-sum test. Linear regression was used to assess the association between two single independent variables, and logistic regression analysis was used to screen for risk factors related to growth retardation in children with PNS. Results: Among the 353 PNS children enrolled in this study, male-to-female ratio of 2.64:1 (256 males vs 97 females). A total of 119 children exhibited growth retardation, incidence rate of 33.71%. The duration of GC usage among those with growth retardation was significantly longer compared to those without it (762.81 ± 934.50 days vs 263.77 ± 420.49 days;p Conclusion: PNS children treated with GC have a high incidence of growth retardation, and a high proportion of short stature in adulthood, especially in children with growth retardation in childhood, most of them have short stature after grown up. Time of GC usage is a risk factor for growth retardation in children with PNS.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 Hepatitis C infection not only damages the liver but also often accompanies many extrahepatic manifestations.Incidences of pulmonary hypertension(PH)caused by hepatitis C are rare,and incidences of concurre...BACKGROUND Hepatitis C infection not only damages the liver but also often accompanies many extrahepatic manifestations.Incidences of pulmonary hypertension(PH)caused by hepatitis C are rare,and incidences of concurrent nephrotic syndrome and polymyositis are even rarer.CASE SUMMARY Herein we describe the case of a 57-year-old woman who was admitted to our department for intermittent chest tightness upon exertion for 5 years,aggravated with dyspnea for 10 d.After relevant examinations she was diagnosed with PH,nephrotic syndrome,and polymyositis due to chronic hepatitis C infection.A multi-disciplinary recommendation was that the patient should be treated with sildenafil and macitentan in combination and methylprednisolone.During treatment autoimmune symptoms,liver function,hepatitis C RNA levels,and cardiac parameters of right heart catheterization were monitored closely.The patient showed significant improvement in 6-min walking distance from 100 to 300 m at 3-mo follow-up and pulmonary artery pressure drops to 50 mmHg.Long-term follow-up is needed to confirm further efficacy and safety.CONCLUSION Increasing evidence supports a relationship between hepatitis C infection and diverse extrahepatic manifestations,but it is very rare to have PH,nephrotic syndrome,and polymyositis in a single patient.We conducted a literature review on the management of several specific extrahepatic manifestations of hepatitis C.展开更多
Objective Idiopathic nephrotic syndrome(INS)is the most common glomerular disease in children.Toll-like receptors(TLRs)have been reported to be associated with response to steroid treatment in children with INS.Nevert...Objective Idiopathic nephrotic syndrome(INS)is the most common glomerular disease in children.Toll-like receptors(TLRs)have been reported to be associated with response to steroid treatment in children with INS.Nevertheless,the correlation between TLR genes and the progression of INS has not yet been clarified.The present study aimed to investigate the association of single-nucleotide polymorphisms(SNPs)in TLR2,TLR4,and TLR9 with susceptibility to INS as well as the clinical phenotyping of steroid responsiveness in Chinese children with INS.Methods A total of 183 pediatric inpatients with INS were included and given standard steroid therapy.Based on their clinical response to steroids,the patients were classified into three groups:steroid-sensitive nephrotic syndrome(SSNS),steroid-dependent nephrotic syndrome(SDNS),and steroid-resistant nephrotic syndrome(SRNS).A total of 100 healthy children were employed as controls.The blood genome DNA was extracted from each participant.Six SNPs(rs11536889,rs1927914,rs7869402,rs11536891,rs352140,and rs3804099)in TLR2,TLR4,and TLR9 were selected and detected by multiplex polymerase chain reaction with next-generation sequencing to assess TLR gene polymorphisms.Results Among the 183 patients with INS,89(48.6%)had SSNS,73(39.9%)had SDNS,and 21(11.5%)had SRNS.No significant difference was found in the genotype distribution between healthy children and patients with INS.However,the genotype and allele frequencies of TLR4 rs7869402 were significantly different between SRNS and SSNS.Compared with patients with the C allele and CC genotype,patients with the T allele and CT genotype had an increased risk of SRNS.Conclusion TLR4 rs7869402 affected the steroid response in Chinese children with INS.It might be a predictor for the early detection of SRNS in this population.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
BACKGROUND Non-alcoholic fatty liver disease(NAFLD) is a global health concern with a prevalence of about 25% amongst United States adults. Its increased prevalence is attributed to increase in patients with obesity a...BACKGROUND Non-alcoholic fatty liver disease(NAFLD) is a global health concern with a prevalence of about 25% amongst United States adults. Its increased prevalence is attributed to increase in patients with obesity and metabolic syndrome, partly due to similar mechanisms of injury. Nephrotic syndrome(NS) is a clinical entity resulting from extensive proteinuria leading to hypoalbuminemia, hyperlipidemia, edema, and other complications. Given its association with hyperlipidemia, there is concern that patients with NS may be at increased risk of NAFLD.AIM To perform a cross-sectional population-based study to investigate the prevalence and risk factors of NAFLD in patients with NS.METHODS A large multicenter database(Explorys Inc., Cleveland, OH, United States) was utilized for this retrospective cohort study. A cohort of 49700 patients with a diagnosis of “Non-Alcoholic fatty liver disease” using the Systematized Nomenclature of Medicine-Clinical Terms(SNOMED-CT) between 1999-2022 was identified. Inclusion criteria were age ≥ 18 years, presence of NAFLD, presence of NS. There were no specific exclusion criteria. Univariate and multivariate analysis were performed to adjust for multiple risk factors including age, gender, Caucasian race, NS, type Ⅱ diabetes mellitus, hypothyroidism, dyslipidemia, obesity, metabolic syndrome and chronic kidney disease. Statistical analysis was conducted using R, and for all analyses, a 2-sided P value of < 0.05 was considered statistically significant.RESULTS Among the 78734750 individuals screened in this database, there were a total of 49700 subjects with NAFLD. In univariate analysis, the odds of having NAFLD in patients with NS, type 2 diabetes mellitus, hypothyroidism, dyslipidemia, obesity, metabolic syndrome and chronic kidney disease were 14.84 [95% confidence interval(95%CI) 13.67-16.10], 17.05(95%CI 16.78-17.32), 6.99(95%CI 6.87-7.11), 13.61(95%CI 13.38-13.84), 19.19(95%CI 18.89-19.50), 29.09(95%CI 28.26--29.95), and 9.05(95%CI 8.88-9.22), respectively. In multivariate analysis, the odds of having NAFLD amongst patients with NS were increased to 1.85(95%Cl 1.70-2.02), while the odds were also remained high in patients that have type 2 diabetes mellitus [odds ratio(OR) 3.84], hypothyroidism(OR 1.57), obesity(OR 5.10), hyperlipidemia(OR 3.09), metabolic syndrome(OR 3.42) and chronic kidney disease(OR 1.33).CONCLUSION Patients with NS are frequently found to have NAFLD, even when adjusting for common risk factors. Hence, clinicians should maintain a high index of suspicion regarding presence of NAFLD in patients with NS.展开更多
[Objectives]To study the potential molecular mechanism of ginseng in treating nephrotic syndrome(NS)by using network pharmacology,molecular docking and experimental verification methods.[Methods]The active components ...[Objectives]To study the potential molecular mechanism of ginseng in treating nephrotic syndrome(NS)by using network pharmacology,molecular docking and experimental verification methods.[Methods]The active components and targets of ginseng were obtained through the network pharmacology database,and the potential targets for the treatment of NS were predicted.The STRING data platform and Cytoscape software were used to construct protein interaction network,and carry out GO and KEGG enrichment analysis.Molecular docking of active components of ginseng and core targets was performed.The in vitro experiment verified the improvement effect of kaempferol,a key active ingredient of ginseng,on podocyte injury.[Results]After screening,17 active components of ginseng and 38 key targets for treating NS were obtained.GO and KEGG enrichment analysis showed that NF-κB,MAPK and other inflammatory pathways were involved.Molecular docking results show that the core components had good binding activity to key targets.The results of in vitro experiments show that kaempferol can reduce the phosphorylation level of AKT1,down-regulate the expression levels of NF-κB p65 and p-NF-κB p65,play an anti-inflammatory effect by inhibiting the activation of NF-κB pathway,and improve podocyte injury.[Conclusions]Ginseng may play a role in the treatment of NS by regulating multiple targets and pathways such as inflammatory response,substance metabolism,and signal transduction.展开更多
[Objectives]This study was conducted to investigate the renal protective effects of Wuling Powder on mice with nephrotic syndrome(NS)based on Notch pathway.[Methods]Sixty KM mice were randomly divided into normal grou...[Objectives]This study was conducted to investigate the renal protective effects of Wuling Powder on mice with nephrotic syndrome(NS)based on Notch pathway.[Methods]Sixty KM mice were randomly divided into normal group,model group,prednisone acetate positive group,high-dose Wuling Powder group,medium-dose Wuling Power group and low-dose Wuling Power group,with 10 mice in each group.Three days after prophylactic administration,a comprehensive nephropathy model was prepared by injecting 1 mg/ml doxorubicin hydrochloride solution(7.5 mg/kg)into the tail vein.After successful modeling,prednisone acetate and Wuling SAN were given high,medium and low doses for intervention for 28 d,respectively.After that,urinary protein and creatinine contents of mice in each group were detected,and pathological damage of renal tissue was observed by HE and Masson staining.The mRNA levels of Notch1,Jagged1 and Hes1 in mouse kidney tissues were detected by RT-PCR,and the expression levels of Notch1,Jagged1 and Hes1 proteins were detected by Western blot.[Results]Wuling Powder could effectively reduce the contents of urine protein(P<0.01)and Scr(P<0.01)in NS mice,and alleviate the pathological injury of kidney.Compared with the model group,the prednisone acetate group and various Wuling Powder groups could down-regulate the expressions of Notch1,Jagged1 and Hes1 mRNA in the kidney tissue of mice(P<0.01),and the expression of Notch1 protein in the renal tissue of mice decreased(P<0.01).The contents of Hes1 in the prednisone acetate group and the high-and medium-dose Wuling Powder groups significantly decreased(P<0.05).[Conclusions]Wuling Powder could protect the kidneys in mice with NS through Notch pathway.展开更多
Background:The role of the patient’s medication literacy is crucial in ensuring the efficacy of the treatment for nephrotic syndrome(NS).It is imperative to identify and examine instances of inadequate medication lit...Background:The role of the patient’s medication literacy is crucial in ensuring the efficacy of the treatment for nephrotic syndrome(NS).It is imperative to identify and examine instances of inadequate medication literacy in order to effectively manage NS.This study aimed to detect the low medication literacy in Chinese NS children using the 22-item Medication Literacy Scale(MLS-22)and to further analyze its influencing factors.Methods:This study involved consecutive sampling of 157 Chinese children with NS.Interviewer-led questionnaires were used to collect data.Firstly,the MLS-22 was evaluated for reliability and validity.Secondly,the medication literacy level was assessed,and factors related to low scores were examined using a multivariate logistic regression model.Results:MLS-22 proved reliable and valid for detection at a low level in NS children.The tertile was divided into low-level and medium/high-level medication literacy.It was found that the scores of NS children averaged 13.06.Multivariate logistic regression analysis revealed that parents of children with primary NS and congenital NS had lower medication literacy scores,which were related to parents with less than high school education.Conclusions:Healthcare providers should develop tailored strategies to effectively assist Chinese children with limited medication literacy in managing chronic diseases.展开更多
Objective:To evaluate the efficacy and safety of Yishen-Huoxue Formula in the treatment of refractory nephrotic syndrome(RNS)using evidence-based medicine.Methods:Databases CNKI,WanFang,VIP,CBM,PubMed,EMbase,and Cochr...Objective:To evaluate the efficacy and safety of Yishen-Huoxue Formula in the treatment of refractory nephrotic syndrome(RNS)using evidence-based medicine.Methods:Databases CNKI,WanFang,VIP,CBM,PubMed,EMbase,and Cochrane Library were searched for randomized controlled trials(RCTs)on the treatment of RNS with the Yishen-Huoxue Formula from June 2008 to July 2020.The quality of the literature was evaluated by the Cochrane.Meta-analysis was performed using Review Manager 5.3 software.Results:A total of 18 articles and 1432 patients were included in this study.The results showed compared to the control group,Yishen-Huoxue Formula was much better in improving total effectiverate[OR=4.15,(95%CI:3.03,5.68),P<0.05],plasma albumin[MD=5.08,(95%CI:3.42,6.74),P<0.05]and decreasing 24-h urine protein quantitation[MD=-0.99,(95%CI:-1.30,-0,69),P<0.05],recurrence rate[OR=0.21,95%CI(0.11,0.40),P<0.05],and adverse reaction rate[OR=0.33,95%CI(0.21,0.52),P<0.05].However,the improvement of complete remission rate was affected by the course of treatment.The effects were similar in less than or equal to nine weeks[OR=1.52,(95%CI:0.74,3.13),P=0.25],whereas the experimental group was superior to the control group in 12-24 weeks[OR=2.47,(95%CI:1.63,3.74),P<0.05]and more than or equal to 26 weeks[OR=2.04,(95%CI:1.43,2.91),P<0.05].Conclusion:The efficacy and safety of Yishen-Huoxue Formula for refractory nephrotic syndrome is better than that of the Western medicine group,but the exact efficacy still needs to be confirmed by prospective studies of higher quality.展开更多
BACKGROUND Waldenstrom'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 hemat...BACKGROUND Waldenstrom'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
BACKGROUND Crumbs homolog 2(CRB2)is a recently discovered gene that is closely related to the maintenance of normal polarity in podocytes;mutations can directly lead to steroid-resistant nephrotic syndrome(SRNS).Howev...BACKGROUND Crumbs homolog 2(CRB2)is a recently discovered gene that is closely related to the maintenance of normal polarity in podocytes;mutations can directly lead to steroid-resistant nephrotic syndrome(SRNS).However,the characteristics of nephrotic syndrome(NS)caused by CRB2 mutations have not been described.CASE SUMMARY We report a novel compound heterozygous mutation of the CRB2 gene in two siblings with SRNS.The two siblings had edema,proteinuria,hypoproteinemia and hyperlipidemia.Both their father and mother had normal phenotypes(no history of NS).Whole exon sequencing(WES)of the family showed a novel compound heterozygous mutation,c.2290(exon 8)C>T and c.3613(exon 12)G>A.Glucocorticoid therapy(methylprednisolone pulse therapy or oral prednisone)and immunosuppressive agents(tacrolimus)had no effect.During a 3-year follow-up after genetic diagnosis by WES,proteinuria persisted,but the patient was healthy.CONCLUSION CRB2 mutations related to SRNS often occur in exons 7,10,and 12.Clinical manifestations of SRNS caused by CRB2 mutations are often less severe than in other forms of SRNS.展开更多
Nephrotic syndrome(NS)is a common refractory clinical disease that can develop into end-stage renal disease(ESRD)if cannot be controlled.At present,in Western medicine,glucocorticoid or immunosuppressant drugs with he...Nephrotic syndrome(NS)is a common refractory clinical disease that can develop into end-stage renal disease(ESRD)if cannot be controlled.At present,in Western medicine,glucocorticoid or immunosuppressant drugs with heavy dose and long course are used for the therapy of NS.There are still few reports on the combined treatment of traditional Chinese medicine and low-dose glucocorticoid for NS.Here,we reported a case of a 48-year-old man diagnosed with NS,accompanied by severe pulmonary infection,abundant proteinuria,and pleural effusion.Integrated traditional Chinese and Western medicine was used as the therapeutic regimen of this patient.Traditional Chinese medicine(TCM)mainly focused on invigorating spleen and kidney,promoting blood circulation,removing blood stasis and promoting diuresis,and Chinese patent medicines or TCM immunosuppressants were given to consolidate the treatment.In Western medicine,small dose and short course glucocorticoids combined with symptomatic treatment were adopted.Delightfully,proteinuria subsided after 5 months treatment with integrated TCM and Western medicine and all laboratory reports were negative after 10 months,which has been normal so far(July 2019).This case report indicates that the combination of TCM and low-dose glucocorticoid is effective in curing NS,with low recurrence rate and few side effects.展开更多
BACKGROUND In most cases of yellow nail syndrome(YNS),the classic triad of yellow nails,lymphedema and respiratory manifestations rarely manifest simultaneously.Therefore,diagnosis is delayed or frequently missed.CASE...BACKGROUND In most cases of yellow nail syndrome(YNS),the classic triad of yellow nails,lymphedema and respiratory manifestations rarely manifest simultaneously.Therefore,diagnosis is delayed or frequently missed.CASE SUMMARY We report a 62-year-old YNS patient presenting with bilateral pleural,pericardial and peritoneal effusions who,2 mo later,developed minimal-change nephrotic syndrome.After treatment with vitamin E,clarithromycin and prednisone for 3 mo,effusions in the chest,pericardium and abdominal cavity decreased while urine protein levels returned to within normal ranges.CONCLUSION Clinicians should consider the possibility of YNS for patients presenting with multiple serous effusions and nephrotic syndromes.展开更多
BACKGROUND Hematopoietic stem cell transplantation(HSCT)is widely used in the treatment of hematological diseases.However,complications after transplantation,such as acute and chronic graft-vs-host disease(GVHD),still...BACKGROUND Hematopoietic stem cell transplantation(HSCT)is widely used in the treatment of hematological diseases.However,complications after transplantation,such as acute and chronic graft-vs-host disease(GVHD),still seriously affect the quality of life and even threaten the lives of patients.There is evidence that glomerular diseases can manifest as GVHD.However,GVHD should not occur as a result of syngeneic HSCT.CASE SUMMARY A 20-year-old male diagnosed with T lymphoblastic lymphoma(stage IIIA,aaIPI 1)in September 2013 was treated with six cycles of hyper-CVAD and achieved complete remission.He underwent syngeneic HSCT in June 2014,and had no kidney disease history before the transplant.However,nephrotic syndrome occurred 24 mo later in the patient after syngeneic HSCT.Renal biopsy was performed,which led to a diagnosis of atypical membranous nephropathy.After treatment with glucocorticoids combined with cyclophosphamide and cyclosporine,the nephrotic syndrome was completely relieved.CONCLUSION We report a case of delayed nephrotic syndrome after syngeneic HSCT.Antibodymediated autoimmune glomerular disease may be the underlying mechanism.After treatment with immunosuppressive agents,the nephrotic syndrome was completely relieved but further long-term follow-up is still needed.展开更多
BACKGROUND Target therapy is licensed by United States Food and Drug Administration on certain cancers.Both sorafenib and lenvatinib are tyrosine kinase inhibitor and indicated on radioactive iodine(RAI)-refractory di...BACKGROUND Target therapy is licensed by United States Food and Drug Administration on certain cancers.Both sorafenib and lenvatinib are tyrosine kinase inhibitor and indicated on radioactive iodine(RAI)-refractory differentiated thyroid cancer(DTC).Lenvatinib is more effective in cancers'control than sorafenib,but causes more nephrotoxicity than sorafenib does.This case is the second published case about the serial adaptions from lenvatinib to sorafenib for improving the proteinuria and,meanwhile,achieving the therapeutic goal.CASE SUMMARY A 56-year-old man suffered from bilateral edematous lower extremities after 1-mo prescription of lenvatinib of 20 mg/d for RAI-refractory DTC.Aside from this symptom,he also developed hypertension.His laboratory showed grade-3 proteinuria(estimated 24-h urine protein:9993 mg),hypoalbuminemia and hypercholesterolemia.Anti-vascular endothelial growth factor(VEGF)therapyinduced nephrotic syndrome was impressed.After reduced dosage of lenvatinib of 10 mg/d and related symptomatic drugs,limited improvement was observed in both adverse effects and caner control.Under this condition,we substituted sorafenib of 400 mg/d for lenvatinib of 10 mg/d.After a 5-mo prescription,not only hypertension and peripheral edema were greatly improved,but also proteinuria was improved from grade three to grade one(estimated 24-h urine protein:962 mg).At the same time the cancer control was achieved,judged from computed tomography and laboratory evidence[thyroglobulin(Tg)before prescription of sorafenib:354.7 ng/m L;Tg after prescription of sorafenib:108.9 ng/m L].CONCLUSION Adaption from lenvatinib to sorafenib is a feasible method to improve the antiVEGF therapy-induced nephrotic syndrome and achieve the therapeutic goal at the same time.展开更多
Objective:To determine the therapeutic effect of Danggui Shaoyao San with Huangqi on rats with nephrotic syndrome(NS)was discussed,and the scientific connotation of TCM treatment principle was elaborated,so as to prov...Objective:To determine the therapeutic effect of Danggui Shaoyao San with Huangqi on rats with nephrotic syndrome(NS)was discussed,and the scientific connotation of TCM treatment principle was elaborated,so as to provide experimental basis for its clinical application.Methods:The NS rat model was established by iv administration of adriamycin;The rats were divided into model group,Danggui Shaoyao San group,Danggui Shaoyao San with Huangqi group,Huangqi group and positive drug group,with 10 rats in control group;The best dosage ratio of Danggui Shaoyao San and Huangqi was screened by Excel;24 h urine protein was measured by BCA method;The electrolyte level was detected by automatic biochemical analyzer;In serum Urea nitrogen(BUN),Creatinine(SCr),Total cholesterol(TC),Triglyceride(TG),Total protein(TP),Albumin(ALB),Immunoglobulin M(Ig-M)and Immunoglobulin G(Ig-G)were detected by the kit;hematoxylin-eosin staining was used to observe the renal morphology;The Total water content(TBW),Extracellular water content(ECF)and Intracellular water content(ICF)were measured by experimental animal body composition analyzer.Results:Compared with the model group,the urine protein in the modified Astragalus group of Danggui Shaoyao Powder was significantly decreased(P<0.01);Urine electrolytes were significantly decreased and serum electrolytes were significantly increased(P<0.01);Serum BUN,SCR,TC and TG were significantly decreased(P<0.01),while TP,ALB,TBW and ICF were significantly increased(P<0.01);The levels of Ig-M and Ig-G were significantly increased(P<0.01),TBW and ICF were significantly increased,and ECF was significantly decreased(P<0.01);Histopathology showed that the pathological changes of renal tissue were obviously alleviated;The improvement of each index was better than that of Danggui Shaoyao San group and Huangqi group.Conclusion:Danggui Shaoyao San with Huangqi group can significantly improve the edema,proteinuria,blood lipid and immune function of NS rats,and the effect is better than that of Danggui Shaoyao San group and Huangqi group.It has the best therapeutic effect on NS,which indicates that Huangqi plays a key synergistic effect on Danggui Shaoyao San,which verifies the scientific connotation of"Yiqi Huoxue Lishui"in the treatment of NS.展开更多
[Objectives]To observe the clinical efficacy of Shenling Baizhu Powder in the treatment of primary nephrotic syndrome(PNS)featuring damp abundance due to splenic asthenia.[Methods]40 patients with PNS were randomly di...[Objectives]To observe the clinical efficacy of Shenling Baizhu Powder in the treatment of primary nephrotic syndrome(PNS)featuring damp abundance due to splenic asthenia.[Methods]40 patients with PNS were randomly divided into treatment group(n=19)and control group(n=21).In the treatment group and the control group,methylprednisolone tablets were taken orally and cytoxan(CTX)was given intravenously for treatment;in the treatment group,based on oral administration of methylprednisolone tablets and intravenous drip of cytoxan(CTX),oral administration of traditional Chinese medicine(addition and subtraction of Shenling Baizhu Powder)was added.Urine routine,24-hour urinary protein,renal function and serum albumin were re-examined after 4 weeks of treatment.[Results]The total effective rate of the treatment group was significantly higher than that of the control group(89.47%vs 61.90%,P<0.05).After treatment,both groups could effectively reduce the levels of 24-hour urinary protein(P<0.05),serum creatinine(P<0.05)and urea nitrogen(P<0.05),and increase the level of serum albumin(P<0.05),but the degree of improvement in the treatment group was significantly higher than that in the control group(P<0.05),and the difference was statistically significant(P<0.05).[Conclusions]Methylprednisolone combined with addition and subtraction of Shenling Baizhu Powder could effectively reduce urinary protein,improve edema symptoms,shorten the course of hormone use and reduce adverse reactions in the treatment of PNS featuring damp abundance due to splenic asthenia.展开更多
BACKGROUND Cutaneous epithelioid angiomatous nodules(CEAN) are rare, benign, vascular lesions characterized by benign proliferation of endothelial cells with prominent epithelioid features, which can be easily confuse...BACKGROUND Cutaneous epithelioid angiomatous nodules(CEAN) are rare, benign, vascular lesions characterized by benign proliferation of endothelial cells with prominent epithelioid features, which can be easily confused with benign and malignant vascular tumors. However, the etiology of CEAN remains unclear, and no association with infection, trauma, or immunosuppression has been described.This case study indicated that CEAN is closely related to the patient’s impaired immune status and may be induced by cyclosporine.CASE SUMMARY A 19-year-old boy with nephrotic syndrome(NS) developed large CEAN on the left foot during treatment for NS. He had repeated relapses of edema in the past 6 years and different types of immunosuppressants were administered including methylprednisolone, mycophenolate mofetil, tacrolimus and cyclosporine;the dosages of these drugs were frequently adjusted. The patient had been receiving cyclosporine and methylprednisolone for 7 mo before he developed CEAN.Cyclosporine was discontinued due to its side effects on skin. After cessation of cyclosporine and 16 mo follow-up, the nodules gradually disappeared without any other treatment for the CEAN.CONCLUSION Impaired immune status is proposed to be a risk factor for CEAN, which may be induced by cyclosporine.展开更多
文摘Objective: To analyze the epidemiological characteristics of growth, as well as factors associated with growth retardation in children with primary nephrotic syndrome (PNS), and to investigate the effect of glucocorticoid (GC) use duration on growth retardation in these children. Methods: Clinical and laboratory data of 353 PNS children treated at our hospital from July 2014 to June 2015 were collected through the medical record management system. Height, weight, and GC usage were recorded. Follow-up assessments were conducted in August 2022 for the original group, recording height, weight, and GC usage. Height and weight were evaluated using standard deviation scores (SDS). Categorical data were analyzed using chi-square test while continuous measurement data were analyzed using t-test or rank-sum test. Linear regression was used to assess the association between two single independent variables, and logistic regression analysis was used to screen for risk factors related to growth retardation in children with PNS. Results: Among the 353 PNS children enrolled in this study, male-to-female ratio of 2.64:1 (256 males vs 97 females). A total of 119 children exhibited growth retardation, incidence rate of 33.71%. The duration of GC usage among those with growth retardation was significantly longer compared to those without it (762.81 ± 934.50 days vs 263.77 ± 420.49 days;p Conclusion: PNS children treated with GC have a high incidence of growth retardation, and a high proportion of short stature in adulthood, especially in children with growth retardation in childhood, most of them have short stature after grown up. Time of GC usage is a risk factor for growth retardation in children with PNS.
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 Hepatitis C infection not only damages the liver but also often accompanies many extrahepatic manifestations.Incidences of pulmonary hypertension(PH)caused by hepatitis C are rare,and incidences of concurrent nephrotic syndrome and polymyositis are even rarer.CASE SUMMARY Herein we describe the case of a 57-year-old woman who was admitted to our department for intermittent chest tightness upon exertion for 5 years,aggravated with dyspnea for 10 d.After relevant examinations she was diagnosed with PH,nephrotic syndrome,and polymyositis due to chronic hepatitis C infection.A multi-disciplinary recommendation was that the patient should be treated with sildenafil and macitentan in combination and methylprednisolone.During treatment autoimmune symptoms,liver function,hepatitis C RNA levels,and cardiac parameters of right heart catheterization were monitored closely.The patient showed significant improvement in 6-min walking distance from 100 to 300 m at 3-mo follow-up and pulmonary artery pressure drops to 50 mmHg.Long-term follow-up is needed to confirm further efficacy and safety.CONCLUSION Increasing evidence supports a relationship between hepatitis C infection and diverse extrahepatic manifestations,but it is very rare to have PH,nephrotic syndrome,and polymyositis in a single patient.We conducted a literature review on the management of several specific extrahepatic manifestations of hepatitis C.
基金This study was funded by the Science and Technology Projects of Zhejiang Province(No.LGC21H200004)the Key Research and Development Plan of Zhejiang Province(No.2019C03028)the Medical Scientific Projects from Health Department of Zhejiang Province(No.2018KY455)。
文摘Objective Idiopathic nephrotic syndrome(INS)is the most common glomerular disease in children.Toll-like receptors(TLRs)have been reported to be associated with response to steroid treatment in children with INS.Nevertheless,the correlation between TLR genes and the progression of INS has not yet been clarified.The present study aimed to investigate the association of single-nucleotide polymorphisms(SNPs)in TLR2,TLR4,and TLR9 with susceptibility to INS as well as the clinical phenotyping of steroid responsiveness in Chinese children with INS.Methods A total of 183 pediatric inpatients with INS were included and given standard steroid therapy.Based on their clinical response to steroids,the patients were classified into three groups:steroid-sensitive nephrotic syndrome(SSNS),steroid-dependent nephrotic syndrome(SDNS),and steroid-resistant nephrotic syndrome(SRNS).A total of 100 healthy children were employed as controls.The blood genome DNA was extracted from each participant.Six SNPs(rs11536889,rs1927914,rs7869402,rs11536891,rs352140,and rs3804099)in TLR2,TLR4,and TLR9 were selected and detected by multiplex polymerase chain reaction with next-generation sequencing to assess TLR gene polymorphisms.Results Among the 183 patients with INS,89(48.6%)had SSNS,73(39.9%)had SDNS,and 21(11.5%)had SRNS.No significant difference was found in the genotype distribution between healthy children and patients with INS.However,the genotype and allele frequencies of TLR4 rs7869402 were significantly different between SRNS and SSNS.Compared with patients with the C allele and CC genotype,patients with the T allele and CT genotype had an increased risk of SRNS.Conclusion TLR4 rs7869402 affected the steroid response in Chinese children with INS.It might be a predictor for the early detection of SRNS in this population.
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.
文摘BACKGROUND Non-alcoholic fatty liver disease(NAFLD) is a global health concern with a prevalence of about 25% amongst United States adults. Its increased prevalence is attributed to increase in patients with obesity and metabolic syndrome, partly due to similar mechanisms of injury. Nephrotic syndrome(NS) is a clinical entity resulting from extensive proteinuria leading to hypoalbuminemia, hyperlipidemia, edema, and other complications. Given its association with hyperlipidemia, there is concern that patients with NS may be at increased risk of NAFLD.AIM To perform a cross-sectional population-based study to investigate the prevalence and risk factors of NAFLD in patients with NS.METHODS A large multicenter database(Explorys Inc., Cleveland, OH, United States) was utilized for this retrospective cohort study. A cohort of 49700 patients with a diagnosis of “Non-Alcoholic fatty liver disease” using the Systematized Nomenclature of Medicine-Clinical Terms(SNOMED-CT) between 1999-2022 was identified. Inclusion criteria were age ≥ 18 years, presence of NAFLD, presence of NS. There were no specific exclusion criteria. Univariate and multivariate analysis were performed to adjust for multiple risk factors including age, gender, Caucasian race, NS, type Ⅱ diabetes mellitus, hypothyroidism, dyslipidemia, obesity, metabolic syndrome and chronic kidney disease. Statistical analysis was conducted using R, and for all analyses, a 2-sided P value of < 0.05 was considered statistically significant.RESULTS Among the 78734750 individuals screened in this database, there were a total of 49700 subjects with NAFLD. In univariate analysis, the odds of having NAFLD in patients with NS, type 2 diabetes mellitus, hypothyroidism, dyslipidemia, obesity, metabolic syndrome and chronic kidney disease were 14.84 [95% confidence interval(95%CI) 13.67-16.10], 17.05(95%CI 16.78-17.32), 6.99(95%CI 6.87-7.11), 13.61(95%CI 13.38-13.84), 19.19(95%CI 18.89-19.50), 29.09(95%CI 28.26--29.95), and 9.05(95%CI 8.88-9.22), respectively. In multivariate analysis, the odds of having NAFLD amongst patients with NS were increased to 1.85(95%Cl 1.70-2.02), while the odds were also remained high in patients that have type 2 diabetes mellitus [odds ratio(OR) 3.84], hypothyroidism(OR 1.57), obesity(OR 5.10), hyperlipidemia(OR 3.09), metabolic syndrome(OR 3.42) and chronic kidney disease(OR 1.33).CONCLUSION Patients with NS are frequently found to have NAFLD, even when adjusting for common risk factors. Hence, clinicians should maintain a high index of suspicion regarding presence of NAFLD in patients with NS.
基金Supported by College Students'Innovation Entrepreneurship and Training Program of Yantai University(X202211066143)。
文摘[Objectives]To study the potential molecular mechanism of ginseng in treating nephrotic syndrome(NS)by using network pharmacology,molecular docking and experimental verification methods.[Methods]The active components and targets of ginseng were obtained through the network pharmacology database,and the potential targets for the treatment of NS were predicted.The STRING data platform and Cytoscape software were used to construct protein interaction network,and carry out GO and KEGG enrichment analysis.Molecular docking of active components of ginseng and core targets was performed.The in vitro experiment verified the improvement effect of kaempferol,a key active ingredient of ginseng,on podocyte injury.[Results]After screening,17 active components of ginseng and 38 key targets for treating NS were obtained.GO and KEGG enrichment analysis showed that NF-κB,MAPK and other inflammatory pathways were involved.Molecular docking results show that the core components had good binding activity to key targets.The results of in vitro experiments show that kaempferol can reduce the phosphorylation level of AKT1,down-regulate the expression levels of NF-κB p65 and p-NF-κB p65,play an anti-inflammatory effect by inhibiting the activation of NF-κB pathway,and improve podocyte injury.[Conclusions]Ginseng may play a role in the treatment of NS by regulating multiple targets and pathways such as inflammatory response,substance metabolism,and signal transduction.
基金Supported by Natural Science Research of the Open Project of First-class Discipline Construction in Guangxi(2019XK120)Natural Science Research Project of Guangxi University of Chinese Medicine(2018MS008)College Students’Innovation and Entrepreneurship Training Program of Guangxi Autonomous Region in 2023(S202310600102).
文摘[Objectives]This study was conducted to investigate the renal protective effects of Wuling Powder on mice with nephrotic syndrome(NS)based on Notch pathway.[Methods]Sixty KM mice were randomly divided into normal group,model group,prednisone acetate positive group,high-dose Wuling Powder group,medium-dose Wuling Power group and low-dose Wuling Power group,with 10 mice in each group.Three days after prophylactic administration,a comprehensive nephropathy model was prepared by injecting 1 mg/ml doxorubicin hydrochloride solution(7.5 mg/kg)into the tail vein.After successful modeling,prednisone acetate and Wuling SAN were given high,medium and low doses for intervention for 28 d,respectively.After that,urinary protein and creatinine contents of mice in each group were detected,and pathological damage of renal tissue was observed by HE and Masson staining.The mRNA levels of Notch1,Jagged1 and Hes1 in mouse kidney tissues were detected by RT-PCR,and the expression levels of Notch1,Jagged1 and Hes1 proteins were detected by Western blot.[Results]Wuling Powder could effectively reduce the contents of urine protein(P<0.01)and Scr(P<0.01)in NS mice,and alleviate the pathological injury of kidney.Compared with the model group,the prednisone acetate group and various Wuling Powder groups could down-regulate the expressions of Notch1,Jagged1 and Hes1 mRNA in the kidney tissue of mice(P<0.01),and the expression of Notch1 protein in the renal tissue of mice decreased(P<0.01).The contents of Hes1 in the prednisone acetate group and the high-and medium-dose Wuling Powder groups significantly decreased(P<0.05).[Conclusions]Wuling Powder could protect the kidneys in mice with NS through Notch pathway.
文摘Background:The role of the patient’s medication literacy is crucial in ensuring the efficacy of the treatment for nephrotic syndrome(NS).It is imperative to identify and examine instances of inadequate medication literacy in order to effectively manage NS.This study aimed to detect the low medication literacy in Chinese NS children using the 22-item Medication Literacy Scale(MLS-22)and to further analyze its influencing factors.Methods:This study involved consecutive sampling of 157 Chinese children with NS.Interviewer-led questionnaires were used to collect data.Firstly,the MLS-22 was evaluated for reliability and validity.Secondly,the medication literacy level was assessed,and factors related to low scores were examined using a multivariate logistic regression model.Results:MLS-22 proved reliable and valid for detection at a low level in NS children.The tertile was divided into low-level and medium/high-level medication literacy.It was found that the scores of NS children averaged 13.06.Multivariate logistic regression analysis revealed that parents of children with primary NS and congenital NS had lower medication literacy scores,which were related to parents with less than high school education.Conclusions:Healthcare providers should develop tailored strategies to effectively assist Chinese children with limited medication literacy in managing chronic diseases.
基金Shanxi Province Science and Technology Research Project(2016ZD0307)Jinyao Comprehensive Development and Utilization Collaborative Innovation Center Project(2017-JYXT-32)+2 种基金The Key Project of Shanxi Province Key R&D Program(201603D3113023)The Key R&D Program(Guide)Project of Shanxi Province(201703D421024)Graduate Education Innovation Program of Shanxi University of Traditional Chinese Medicine(2020JG001)。
文摘Objective:To evaluate the efficacy and safety of Yishen-Huoxue Formula in the treatment of refractory nephrotic syndrome(RNS)using evidence-based medicine.Methods:Databases CNKI,WanFang,VIP,CBM,PubMed,EMbase,and Cochrane Library were searched for randomized controlled trials(RCTs)on the treatment of RNS with the Yishen-Huoxue Formula from June 2008 to July 2020.The quality of the literature was evaluated by the Cochrane.Meta-analysis was performed using Review Manager 5.3 software.Results:A total of 18 articles and 1432 patients were included in this study.The results showed compared to the control group,Yishen-Huoxue Formula was much better in improving total effectiverate[OR=4.15,(95%CI:3.03,5.68),P<0.05],plasma albumin[MD=5.08,(95%CI:3.42,6.74),P<0.05]and decreasing 24-h urine protein quantitation[MD=-0.99,(95%CI:-1.30,-0,69),P<0.05],recurrence rate[OR=0.21,95%CI(0.11,0.40),P<0.05],and adverse reaction rate[OR=0.33,95%CI(0.21,0.52),P<0.05].However,the improvement of complete remission rate was affected by the course of treatment.The effects were similar in less than or equal to nine weeks[OR=1.52,(95%CI:0.74,3.13),P=0.25],whereas the experimental group was superior to the control group in 12-24 weeks[OR=2.47,(95%CI:1.63,3.74),P<0.05]and more than or equal to 26 weeks[OR=2.04,(95%CI:1.43,2.91),P<0.05].Conclusion:The efficacy and safety of Yishen-Huoxue Formula for refractory nephrotic syndrome is better than that of the Western medicine group,but the exact efficacy still needs to be confirmed by prospective studies of higher quality.
文摘BACKGROUND Waldenstrom'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BACKGROUND Crumbs homolog 2(CRB2)is a recently discovered gene that is closely related to the maintenance of normal polarity in podocytes;mutations can directly lead to steroid-resistant nephrotic syndrome(SRNS).However,the characteristics of nephrotic syndrome(NS)caused by CRB2 mutations have not been described.CASE SUMMARY We report a novel compound heterozygous mutation of the CRB2 gene in two siblings with SRNS.The two siblings had edema,proteinuria,hypoproteinemia and hyperlipidemia.Both their father and mother had normal phenotypes(no history of NS).Whole exon sequencing(WES)of the family showed a novel compound heterozygous mutation,c.2290(exon 8)C>T and c.3613(exon 12)G>A.Glucocorticoid therapy(methylprednisolone pulse therapy or oral prednisone)and immunosuppressive agents(tacrolimus)had no effect.During a 3-year follow-up after genetic diagnosis by WES,proteinuria persisted,but the patient was healthy.CONCLUSION CRB2 mutations related to SRNS often occur in exons 7,10,and 12.Clinical manifestations of SRNS caused by CRB2 mutations are often less severe than in other forms of SRNS.
文摘Nephrotic syndrome(NS)is a common refractory clinical disease that can develop into end-stage renal disease(ESRD)if cannot be controlled.At present,in Western medicine,glucocorticoid or immunosuppressant drugs with heavy dose and long course are used for the therapy of NS.There are still few reports on the combined treatment of traditional Chinese medicine and low-dose glucocorticoid for NS.Here,we reported a case of a 48-year-old man diagnosed with NS,accompanied by severe pulmonary infection,abundant proteinuria,and pleural effusion.Integrated traditional Chinese and Western medicine was used as the therapeutic regimen of this patient.Traditional Chinese medicine(TCM)mainly focused on invigorating spleen and kidney,promoting blood circulation,removing blood stasis and promoting diuresis,and Chinese patent medicines or TCM immunosuppressants were given to consolidate the treatment.In Western medicine,small dose and short course glucocorticoids combined with symptomatic treatment were adopted.Delightfully,proteinuria subsided after 5 months treatment with integrated TCM and Western medicine and all laboratory reports were negative after 10 months,which has been normal so far(July 2019).This case report indicates that the combination of TCM and low-dose glucocorticoid is effective in curing NS,with low recurrence rate and few side effects.
文摘BACKGROUND In most cases of yellow nail syndrome(YNS),the classic triad of yellow nails,lymphedema and respiratory manifestations rarely manifest simultaneously.Therefore,diagnosis is delayed or frequently missed.CASE SUMMARY We report a 62-year-old YNS patient presenting with bilateral pleural,pericardial and peritoneal effusions who,2 mo later,developed minimal-change nephrotic syndrome.After treatment with vitamin E,clarithromycin and prednisone for 3 mo,effusions in the chest,pericardium and abdominal cavity decreased while urine protein levels returned to within normal ranges.CONCLUSION Clinicians should consider the possibility of YNS for patients presenting with multiple serous effusions and nephrotic syndromes.
基金the National Natural Science Foundation of China,No.8197039and 2017 Jiangsu Commission of Health Research Project,No.H2017023.
文摘BACKGROUND Hematopoietic stem cell transplantation(HSCT)is widely used in the treatment of hematological diseases.However,complications after transplantation,such as acute and chronic graft-vs-host disease(GVHD),still seriously affect the quality of life and even threaten the lives of patients.There is evidence that glomerular diseases can manifest as GVHD.However,GVHD should not occur as a result of syngeneic HSCT.CASE SUMMARY A 20-year-old male diagnosed with T lymphoblastic lymphoma(stage IIIA,aaIPI 1)in September 2013 was treated with six cycles of hyper-CVAD and achieved complete remission.He underwent syngeneic HSCT in June 2014,and had no kidney disease history before the transplant.However,nephrotic syndrome occurred 24 mo later in the patient after syngeneic HSCT.Renal biopsy was performed,which led to a diagnosis of atypical membranous nephropathy.After treatment with glucocorticoids combined with cyclophosphamide and cyclosporine,the nephrotic syndrome was completely relieved.CONCLUSION We report a case of delayed nephrotic syndrome after syngeneic HSCT.Antibodymediated autoimmune glomerular disease may be the underlying mechanism.After treatment with immunosuppressive agents,the nephrotic syndrome was completely relieved but further long-term follow-up is still needed.
文摘BACKGROUND Target therapy is licensed by United States Food and Drug Administration on certain cancers.Both sorafenib and lenvatinib are tyrosine kinase inhibitor and indicated on radioactive iodine(RAI)-refractory differentiated thyroid cancer(DTC).Lenvatinib is more effective in cancers'control than sorafenib,but causes more nephrotoxicity than sorafenib does.This case is the second published case about the serial adaptions from lenvatinib to sorafenib for improving the proteinuria and,meanwhile,achieving the therapeutic goal.CASE SUMMARY A 56-year-old man suffered from bilateral edematous lower extremities after 1-mo prescription of lenvatinib of 20 mg/d for RAI-refractory DTC.Aside from this symptom,he also developed hypertension.His laboratory showed grade-3 proteinuria(estimated 24-h urine protein:9993 mg),hypoalbuminemia and hypercholesterolemia.Anti-vascular endothelial growth factor(VEGF)therapyinduced nephrotic syndrome was impressed.After reduced dosage of lenvatinib of 10 mg/d and related symptomatic drugs,limited improvement was observed in both adverse effects and caner control.Under this condition,we substituted sorafenib of 400 mg/d for lenvatinib of 10 mg/d.After a 5-mo prescription,not only hypertension and peripheral edema were greatly improved,but also proteinuria was improved from grade three to grade one(estimated 24-h urine protein:962 mg).At the same time the cancer control was achieved,judged from computed tomography and laboratory evidence[thyroglobulin(Tg)before prescription of sorafenib:354.7 ng/m L;Tg after prescription of sorafenib:108.9 ng/m L].CONCLUSION Adaption from lenvatinib to sorafenib is a feasible method to improve the antiVEGF therapy-induced nephrotic syndrome and achieve the therapeutic goal at the same time.
基金National Natural Science Foundation Project(No.81573720,81974536)。
文摘Objective:To determine the therapeutic effect of Danggui Shaoyao San with Huangqi on rats with nephrotic syndrome(NS)was discussed,and the scientific connotation of TCM treatment principle was elaborated,so as to provide experimental basis for its clinical application.Methods:The NS rat model was established by iv administration of adriamycin;The rats were divided into model group,Danggui Shaoyao San group,Danggui Shaoyao San with Huangqi group,Huangqi group and positive drug group,with 10 rats in control group;The best dosage ratio of Danggui Shaoyao San and Huangqi was screened by Excel;24 h urine protein was measured by BCA method;The electrolyte level was detected by automatic biochemical analyzer;In serum Urea nitrogen(BUN),Creatinine(SCr),Total cholesterol(TC),Triglyceride(TG),Total protein(TP),Albumin(ALB),Immunoglobulin M(Ig-M)and Immunoglobulin G(Ig-G)were detected by the kit;hematoxylin-eosin staining was used to observe the renal morphology;The Total water content(TBW),Extracellular water content(ECF)and Intracellular water content(ICF)were measured by experimental animal body composition analyzer.Results:Compared with the model group,the urine protein in the modified Astragalus group of Danggui Shaoyao Powder was significantly decreased(P<0.01);Urine electrolytes were significantly decreased and serum electrolytes were significantly increased(P<0.01);Serum BUN,SCR,TC and TG were significantly decreased(P<0.01),while TP,ALB,TBW and ICF were significantly increased(P<0.01);The levels of Ig-M and Ig-G were significantly increased(P<0.01),TBW and ICF were significantly increased,and ECF was significantly decreased(P<0.01);Histopathology showed that the pathological changes of renal tissue were obviously alleviated;The improvement of each index was better than that of Danggui Shaoyao San group and Huangqi group.Conclusion:Danggui Shaoyao San with Huangqi group can significantly improve the edema,proteinuria,blood lipid and immune function of NS rats,and the effect is better than that of Danggui Shaoyao San group and Huangqi group.It has the best therapeutic effect on NS,which indicates that Huangqi plays a key synergistic effect on Danggui Shaoyao San,which verifies the scientific connotation of"Yiqi Huoxue Lishui"in the treatment of NS.
基金Natural Science Research Project of Guangxi University of Chinese Medicine(2015LX037).
文摘[Objectives]To observe the clinical efficacy of Shenling Baizhu Powder in the treatment of primary nephrotic syndrome(PNS)featuring damp abundance due to splenic asthenia.[Methods]40 patients with PNS were randomly divided into treatment group(n=19)and control group(n=21).In the treatment group and the control group,methylprednisolone tablets were taken orally and cytoxan(CTX)was given intravenously for treatment;in the treatment group,based on oral administration of methylprednisolone tablets and intravenous drip of cytoxan(CTX),oral administration of traditional Chinese medicine(addition and subtraction of Shenling Baizhu Powder)was added.Urine routine,24-hour urinary protein,renal function and serum albumin were re-examined after 4 weeks of treatment.[Results]The total effective rate of the treatment group was significantly higher than that of the control group(89.47%vs 61.90%,P<0.05).After treatment,both groups could effectively reduce the levels of 24-hour urinary protein(P<0.05),serum creatinine(P<0.05)and urea nitrogen(P<0.05),and increase the level of serum albumin(P<0.05),but the degree of improvement in the treatment group was significantly higher than that in the control group(P<0.05),and the difference was statistically significant(P<0.05).[Conclusions]Methylprednisolone combined with addition and subtraction of Shenling Baizhu Powder could effectively reduce urinary protein,improve edema symptoms,shorten the course of hormone use and reduce adverse reactions in the treatment of PNS featuring damp abundance due to splenic asthenia.
文摘BACKGROUND Cutaneous epithelioid angiomatous nodules(CEAN) are rare, benign, vascular lesions characterized by benign proliferation of endothelial cells with prominent epithelioid features, which can be easily confused with benign and malignant vascular tumors. However, the etiology of CEAN remains unclear, and no association with infection, trauma, or immunosuppression has been described.This case study indicated that CEAN is closely related to the patient’s impaired immune status and may be induced by cyclosporine.CASE SUMMARY A 19-year-old boy with nephrotic syndrome(NS) developed large CEAN on the left foot during treatment for NS. He had repeated relapses of edema in the past 6 years and different types of immunosuppressants were administered including methylprednisolone, mycophenolate mofetil, tacrolimus and cyclosporine;the dosages of these drugs were frequently adjusted. The patient had been receiving cyclosporine and methylprednisolone for 7 mo before he developed CEAN.Cyclosporine was discontinued due to its side effects on skin. After cessation of cyclosporine and 16 mo follow-up, the nodules gradually disappeared without any other treatment for the CEAN.CONCLUSION Impaired immune status is proposed to be a risk factor for CEAN, which may be induced by cyclosporine.
