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
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
BACKGROUND HDR syndrome is a rare genetic disease caused by variants in the GATA3 gene and is phenotypically defined by the triad of hypoparathyroidism(H),deafness(D),and renal disease(R).Renal disorders of HDR are ma...BACKGROUND HDR syndrome is a rare genetic disease caused by variants in the GATA3 gene and is phenotypically defined by the triad of hypoparathyroidism(H),deafness(D),and renal disease(R).Renal disorders of HDR are mainly developmental ab-normalities,although renal functional abnormalities can also be observed.Ne-phrotic syndrome or nephrotic-level proteinuria is rare in HDR syndrome.Here,we report a Chinese infant with HDR syndrome who presented with early-onset nephrotic syndrome.We suggest that variants in the GATA3 gene might be asso-ciated with nephrotic syndrome.(p.Pro235 Leu),in exon 3 of the GATA3 gene.CONCLUSION We report an infant with HDR syndrome who presented with early-onset nephrotic syndrome in China.We suggest that variants in the GATA3 gene might be associated with infant-onset nephrotic syndrome.展开更多
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
Objective Glucocorticoid(GC)-induced adverse reactions(ARs)have been extensively studied due to their potential impact on patients’health.This study aimed to examine the potential correlation between two polymorphism...Objective Glucocorticoid(GC)-induced adverse reactions(ARs)have been extensively studied due to their potential impact on patients’health.This study aimed to examine the potential correlation between two polymorphisms[adenosine triphosphate-binding cassette B1(ABCB1)C3435T and plasminogen activator inhibitor-1(PAI-1)4G/5G]and various GC-induced ARs in nephrotic syndrome(NS)patients.Methods In this study,513 NS patients who underwent GC treatment were enrolled.Then,the patients were divided into two groups based on ABCB1 C3435T and PAI-14G/5G genotyping,and intergroup comparisons of clinicopathological data and GC-induced ARs were performed.Univariate and multivariate logistic analyses were subsequently conducted to identify potential risk factors for GC-induced ARs,and a nomogram was subsequently established and validated via the area under the ROC curve(AUC),calibration curve and decision curve analysis(DCA).Results We identified ABCB1 C3435T as an independent risk factor for the development of steroid-associated avascular necrosis of the femoral head(SANFH)(OR:2.191,95%CI:1.258–3.813,P=0.006)but not as a risk factor for the occurrence of steroid diabetes mellitus(S-DM).On the other hand,PAI-14G/5G was identified as an independent risk factor for the development of both SANFH(OR:2.198,95%CI:1.267–3.812,P=0.005)and S-DM(OR:2.080,95%CI:1.166–3.711,P=0.013).Notably,no significant correlation was found between the two gene polymorphisms and other GC-induced ARs.In addition,two nomograms were established and validated to demonstrate strong calibration capability and clinical utility.Conclusion Assessing ABCB1 C3435T and PAI-14G/5G before steroid treatment in NS patients could be useful for identifying patients at a high risk of developing SANFH and S-DM.展开更多
Background: Idiopathic nephrotic syndrome (INS) is a frequent pathology in children. There is little data on the future of NS in children in sub-Saharan Africa, particularly Cameroon. The aim of our study is to report...Background: Idiopathic nephrotic syndrome (INS) is a frequent pathology in children. There is little data on the future of NS in children in sub-Saharan Africa, particularly Cameroon. The aim of our study is to report the prognosis of children treated for nephrotic syndrome in the city of Yaoundé. Method: This was an analytical cross-sectional study with retrospective collection in 4 reference hospitals in the Cameroonian capital over a period of five years from January 1, 2018 to December 31, 2022. We included all medical records of patients treated for idiopathic INS. We excluded incomplete records and those with a history of chronic kidney disease. The sociodemographic, clinical, paraclinical, and therapeutic data, as well as the short-term evolution were collected in the files. Data was analysed using the software statistical package for social sciences version 25.0. Statistical significance was set at a p-value Results: A total of 131 children (58% boys) were included in our study over a period of 5 years. The median age was 8 [6 - 11] years. Median proteinuria was 5 g/24h [3 - 8.4], median serum protein was 39 [34 - 46] g/l and median estimated glomerular filtration rate was 130.36 [68 - 174.6] ml/min/1.73m2. During steroid therapy, 45.07% were in partial remission at 2 months, 16.9% were in complete remission at 4 and 6 months, and 37.25% had relapsed. Steroid sensitivity was reported in 28.17% of cases, steroid resistance in 64.78% of cases and steroid dependent in 7.04% of cases. The mortality rate was 12.97%. Survival time averaged 48.2 months, with an overall crude survival rate of 99.2% at 3 and 6 months and 98.4% at 1 year. Regarding renal survival, renal function was impaired in 8.33% of patients at 6 months and 9% at 12 months. Conclusion: Idiopathic nephrotic syndrome is a common disease in children. Its evolution depends on corticosteroid therapy. The long-term prognosis is dominated by the risk of progression to end-stage kidney disease or even death. Rigorous and affordable follow-up is essential to reduce the number of patients lost to follow-up and the occurrence of complications.展开更多
Introduction: Studies have been conducted on nephrotic syndrome in Niger. The study aimed to determine the histological and etiological aspects of nephrotic syndrome. Patients and Method: This was a retrospective stud...Introduction: Studies have been conducted on nephrotic syndrome in Niger. The study aimed to determine the histological and etiological aspects of nephrotic syndrome. Patients and Method: This was a retrospective study from February 1st, 2018 to January 31st, 2024. All patients with nephrotic syndrome who underwent renal biopsy were included. Samples were analyzed at the anatomy-cytology pathology laboratory of the Faculty of Medicine in Dakar (Senegal). The variables studied included clinical, biological, histological and etiological characteristics. Data were analyzed using Excel 2013 and Epi-info 7.2.0 software. Results: The study included 119 patients with nephrotic syndrome. Prevalence of nephrotic syndrome was 11.24%. The male-to-female ratio was 2.25:1. The mean age at diagnosis was between 34.5 ± 18.84 years. Edema was the reason for admission in 40.34% of cases. The nephrotic syndrome was impure in 63.86% of cases. Nine histological lesions were identified. Focal and segmental glomerulosclerosis (40.09%), minimal change disease (23.53%), membranous nephropathy (13.45%), diabetic nephropathy (10.92%), membranous proliferative glomerulonephritis (3.36%), acute glomerulonephritis (3.36%), glomerular thrombotic microangiopathy (2.52%), non-IgA mesengial proliferative glomerulonephritis (1.68%) and amyloidosis (0.84%). Nephrotic syndrome was primary in 57.98% of cases. Secondary etiologies were dominated by diabetes (11.76%), followed by hepatitis B virus (9.24%), lupus, lymphoma, malaria, syphilis, cryoglobulinemia, sickle cell disease and HIV. Conclusion: Future studies should investigate the causes of glomerulopathy secondary to chronic tubulointerstitial lesions.展开更多
Objective:To analyze changes in liver and kidney function and lipid metabolism in patients with refractory nephrotic syndrome(RNS)after receiving different treatments.Methods:A total of 64 patients treated in the Wuwe...Objective:To analyze changes in liver and kidney function and lipid metabolism in patients with refractory nephrotic syndrome(RNS)after receiving different treatments.Methods:A total of 64 patients treated in the Wuwei Hospital of Traditional Chinese Medicine from January 2018 to January 2021 were included in this study.All subjects were diagnosed with RNS and randomly assigned to groups:a control group(32 cases)and an observation group(32 cases).The control group received cyclophosphamide+glucocorticoids,while the observation group received tacrolimus+glucocorticoids,both for six months.The various indicators of the two groups were compared.Results:After six months of treatment,the overall clinical efficacy rate of the observation group was significantly higher than that of the control group.Six months post-treatment,levels of serum ALT,AST,BUN,SCr,24 h UTP,TG,and TC were reduced in both groups compared to baseline levels,with reductions more pronounced in the observation group.Serum ALB levels increased in both groups,with a more significant increase in the observation group.Statistical analysis showed these differences were significant(P<0.05).There were no significant changes in FBG levels in either group(P>0.05).Conclusion:For RNS patients,treatment with tacrolimus combined with glucocorticoids significantly reduces liver function damage,improves kidney function and lipid metabolism,and enhances clinical efficacy.展开更多
Introduction: The nephrotic syndrome (NS) is the most common glomerular nephropathy in children. The aim of this work was to study the diagnostic and evolutionary aspects of NS in pediatrics at the national Hospital o...Introduction: The nephrotic syndrome (NS) is the most common glomerular nephropathy in children. The aim of this work was to study the diagnostic and evolutionary aspects of NS in pediatrics at the national Hospital of Zinder (Niger). Materials and methods: This descriptive cross-sectional study was carried out over an 11-month period from January 1, 2023 to November 30, 2023. Included were all children aged 5 to 15 years admitted during the study period for nephrotic syndrome in the Pediatric Department B of the National Hospital of Zinder (HNZ). Results: A total of 26 patients had fulfilled the inclusion criteria, i.e., a hospital frequency of 0.69%. The male sex was predominant with a frequency of 57.69% (sex ratio = 1.3). The 5 to 10 years age group was the most represented with a frequency of 57.69%. The mean age at diagnosis was 8 ± 2.9 years. A delay in consultation was observed in the majority of patients. The consultation time was longer than 14 days for 16 patients, i.e., 61.54%. Edema was found in all patients at the time of consultation, i.e., in 100% of the cases. The 24-hour proteinuria was between 50 to 100 mg/kg/day in 65% of cases with an average of 82.78 mg/kg/d. The protidemia and the average albuminemia were respectively 44.85 g/L and 18.78 g/L. The SN was pure in 15 patients (57.69% of cases) and impure in 11 patients (42.31% of cases). The treatment was essentially based on oral corticosteroid therapy (prednisolone: cotipred 20 mg). Corticosensitivity was observed in 22 patients, i.e., 84.61%, one (1) case of corticodependence (3.85%) and two (2) cases of corticoresistance, i.e., 7.69%. One (1) case of death was recorded, i.e., a frequency of 3.85%. Only one (1) patient had benefited from a renal biopsy puncture (RBP) and an anatomopathological examination. Conclusion: SN remains common in children at the HNZ. The improvement of the technical platform for PBR and anatomopathological examinations as well as the establishment of social security prove necessary for a better management of SN in children at the HNZ.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
Context + objective: Nephrotic syndrome accounts for 15 to 30% of glomerulopathies in adults;its evolution is difficult to predict. The objective of this work is to study the profile of patients suffering from idiopat...Context + objective: Nephrotic syndrome accounts for 15 to 30% of glomerulopathies in adults;its evolution is difficult to predict. The objective of this work is to study the profile of patients suffering from idiopathic nephrotic syndrome in a nephrological hospital environment at the University Hospital of Yopougon in Abidjan. Methods: We carried out a retrospective study with descriptive and analytical purposes including any patient aged 15 and over who had had a clean kidney biopsy between January 2018 and October 2019 concluding in a nephrotic syndrome with minimal glomerular lesions (MGL) or segmental and focal hyalinosis (SFH) at the Nephrology Department of the University Hospital of Yopougon in Abidjan. Results: A total of 28 patients were collected. Patients with SFH type lesions were older with a statistically significant p value (p = 0.002). The male gender predominated in MGL type lesions without a statistically significant link (p = 0.26). SFH-type lesions were dominant (57.14%), followed by MGL-type lesions (42.86%). Oedematous syndrome was the main physical sign (n = 9;56.25%) without significant difference (p = 0.742). ESRD with a GFR less than 15 mL/min/1.73 m2 was found in 8 patients, which is 28.6% without significant difference. Microscopic haematuria coexisted in 5 patients (17.9%) and hypertension in 7 patients (25%). The first-line treatment was oral corticosteroid therapy at usual doses (n = 28;100%), associated with immunosuppressants (n = 11.79;17%). Partial remission was noted in 9 MGL type patients, that is 69.2% and a total remission in SFH type patients that is 50%, without any statistically significant link (p = 0.568). The main complications were CRD in 9 patients, which is 45%, followed by dyslipidaemia that is 30%. Conclusion: Adult idiopathic nephrotic syndrome is common and dominated by SFH type of histological lesions. Its outcome is generally favourable.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
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.
文摘BACKGROUND HDR syndrome is a rare genetic disease caused by variants in the GATA3 gene and is phenotypically defined by the triad of hypoparathyroidism(H),deafness(D),and renal disease(R).Renal disorders of HDR are mainly developmental ab-normalities,although renal functional abnormalities can also be observed.Ne-phrotic syndrome or nephrotic-level proteinuria is rare in HDR syndrome.Here,we report a Chinese infant with HDR syndrome who presented with early-onset nephrotic syndrome.We suggest that variants in the GATA3 gene might be asso-ciated with nephrotic syndrome.(p.Pro235 Leu),in exon 3 of the GATA3 gene.CONCLUSION We report an infant with HDR syndrome who presented with early-onset nephrotic syndrome in China.We suggest that variants in the GATA3 gene might be associated with infant-onset nephrotic syndrome.
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.
基金supported by the General Project of Henan Natural Science Foundation(No.232300420034)the National Natural Science Foundation of China for the Youth(No.81600555)the General Project of China Postdoctoral Science Foundation(No.2018M640684)。
文摘Objective Glucocorticoid(GC)-induced adverse reactions(ARs)have been extensively studied due to their potential impact on patients’health.This study aimed to examine the potential correlation between two polymorphisms[adenosine triphosphate-binding cassette B1(ABCB1)C3435T and plasminogen activator inhibitor-1(PAI-1)4G/5G]and various GC-induced ARs in nephrotic syndrome(NS)patients.Methods In this study,513 NS patients who underwent GC treatment were enrolled.Then,the patients were divided into two groups based on ABCB1 C3435T and PAI-14G/5G genotyping,and intergroup comparisons of clinicopathological data and GC-induced ARs were performed.Univariate and multivariate logistic analyses were subsequently conducted to identify potential risk factors for GC-induced ARs,and a nomogram was subsequently established and validated via the area under the ROC curve(AUC),calibration curve and decision curve analysis(DCA).Results We identified ABCB1 C3435T as an independent risk factor for the development of steroid-associated avascular necrosis of the femoral head(SANFH)(OR:2.191,95%CI:1.258–3.813,P=0.006)but not as a risk factor for the occurrence of steroid diabetes mellitus(S-DM).On the other hand,PAI-14G/5G was identified as an independent risk factor for the development of both SANFH(OR:2.198,95%CI:1.267–3.812,P=0.005)and S-DM(OR:2.080,95%CI:1.166–3.711,P=0.013).Notably,no significant correlation was found between the two gene polymorphisms and other GC-induced ARs.In addition,two nomograms were established and validated to demonstrate strong calibration capability and clinical utility.Conclusion Assessing ABCB1 C3435T and PAI-14G/5G before steroid treatment in NS patients could be useful for identifying patients at a high risk of developing SANFH and S-DM.
文摘Background: Idiopathic nephrotic syndrome (INS) is a frequent pathology in children. There is little data on the future of NS in children in sub-Saharan Africa, particularly Cameroon. The aim of our study is to report the prognosis of children treated for nephrotic syndrome in the city of Yaoundé. Method: This was an analytical cross-sectional study with retrospective collection in 4 reference hospitals in the Cameroonian capital over a period of five years from January 1, 2018 to December 31, 2022. We included all medical records of patients treated for idiopathic INS. We excluded incomplete records and those with a history of chronic kidney disease. The sociodemographic, clinical, paraclinical, and therapeutic data, as well as the short-term evolution were collected in the files. Data was analysed using the software statistical package for social sciences version 25.0. Statistical significance was set at a p-value Results: A total of 131 children (58% boys) were included in our study over a period of 5 years. The median age was 8 [6 - 11] years. Median proteinuria was 5 g/24h [3 - 8.4], median serum protein was 39 [34 - 46] g/l and median estimated glomerular filtration rate was 130.36 [68 - 174.6] ml/min/1.73m2. During steroid therapy, 45.07% were in partial remission at 2 months, 16.9% were in complete remission at 4 and 6 months, and 37.25% had relapsed. Steroid sensitivity was reported in 28.17% of cases, steroid resistance in 64.78% of cases and steroid dependent in 7.04% of cases. The mortality rate was 12.97%. Survival time averaged 48.2 months, with an overall crude survival rate of 99.2% at 3 and 6 months and 98.4% at 1 year. Regarding renal survival, renal function was impaired in 8.33% of patients at 6 months and 9% at 12 months. Conclusion: Idiopathic nephrotic syndrome is a common disease in children. Its evolution depends on corticosteroid therapy. The long-term prognosis is dominated by the risk of progression to end-stage kidney disease or even death. Rigorous and affordable follow-up is essential to reduce the number of patients lost to follow-up and the occurrence of complications.
文摘Introduction: Studies have been conducted on nephrotic syndrome in Niger. The study aimed to determine the histological and etiological aspects of nephrotic syndrome. Patients and Method: This was a retrospective study from February 1st, 2018 to January 31st, 2024. All patients with nephrotic syndrome who underwent renal biopsy were included. Samples were analyzed at the anatomy-cytology pathology laboratory of the Faculty of Medicine in Dakar (Senegal). The variables studied included clinical, biological, histological and etiological characteristics. Data were analyzed using Excel 2013 and Epi-info 7.2.0 software. Results: The study included 119 patients with nephrotic syndrome. Prevalence of nephrotic syndrome was 11.24%. The male-to-female ratio was 2.25:1. The mean age at diagnosis was between 34.5 ± 18.84 years. Edema was the reason for admission in 40.34% of cases. The nephrotic syndrome was impure in 63.86% of cases. Nine histological lesions were identified. Focal and segmental glomerulosclerosis (40.09%), minimal change disease (23.53%), membranous nephropathy (13.45%), diabetic nephropathy (10.92%), membranous proliferative glomerulonephritis (3.36%), acute glomerulonephritis (3.36%), glomerular thrombotic microangiopathy (2.52%), non-IgA mesengial proliferative glomerulonephritis (1.68%) and amyloidosis (0.84%). Nephrotic syndrome was primary in 57.98% of cases. Secondary etiologies were dominated by diabetes (11.76%), followed by hepatitis B virus (9.24%), lupus, lymphoma, malaria, syphilis, cryoglobulinemia, sickle cell disease and HIV. Conclusion: Future studies should investigate the causes of glomerulopathy secondary to chronic tubulointerstitial lesions.
基金Wuwei City Science and Technology Plan Project“Efficacy Analysis of Tacrolimus Combined with Glucocorticoids in the Treatment of Refractory Nephrotic Syndrome”(Project No.WW2101161)。
文摘Objective:To analyze changes in liver and kidney function and lipid metabolism in patients with refractory nephrotic syndrome(RNS)after receiving different treatments.Methods:A total of 64 patients treated in the Wuwei Hospital of Traditional Chinese Medicine from January 2018 to January 2021 were included in this study.All subjects were diagnosed with RNS and randomly assigned to groups:a control group(32 cases)and an observation group(32 cases).The control group received cyclophosphamide+glucocorticoids,while the observation group received tacrolimus+glucocorticoids,both for six months.The various indicators of the two groups were compared.Results:After six months of treatment,the overall clinical efficacy rate of the observation group was significantly higher than that of the control group.Six months post-treatment,levels of serum ALT,AST,BUN,SCr,24 h UTP,TG,and TC were reduced in both groups compared to baseline levels,with reductions more pronounced in the observation group.Serum ALB levels increased in both groups,with a more significant increase in the observation group.Statistical analysis showed these differences were significant(P<0.05).There were no significant changes in FBG levels in either group(P>0.05).Conclusion:For RNS patients,treatment with tacrolimus combined with glucocorticoids significantly reduces liver function damage,improves kidney function and lipid metabolism,and enhances clinical efficacy.
文摘Introduction: The nephrotic syndrome (NS) is the most common glomerular nephropathy in children. The aim of this work was to study the diagnostic and evolutionary aspects of NS in pediatrics at the national Hospital of Zinder (Niger). Materials and methods: This descriptive cross-sectional study was carried out over an 11-month period from January 1, 2023 to November 30, 2023. Included were all children aged 5 to 15 years admitted during the study period for nephrotic syndrome in the Pediatric Department B of the National Hospital of Zinder (HNZ). Results: A total of 26 patients had fulfilled the inclusion criteria, i.e., a hospital frequency of 0.69%. The male sex was predominant with a frequency of 57.69% (sex ratio = 1.3). The 5 to 10 years age group was the most represented with a frequency of 57.69%. The mean age at diagnosis was 8 ± 2.9 years. A delay in consultation was observed in the majority of patients. The consultation time was longer than 14 days for 16 patients, i.e., 61.54%. Edema was found in all patients at the time of consultation, i.e., in 100% of the cases. The 24-hour proteinuria was between 50 to 100 mg/kg/day in 65% of cases with an average of 82.78 mg/kg/d. The protidemia and the average albuminemia were respectively 44.85 g/L and 18.78 g/L. The SN was pure in 15 patients (57.69% of cases) and impure in 11 patients (42.31% of cases). The treatment was essentially based on oral corticosteroid therapy (prednisolone: cotipred 20 mg). Corticosensitivity was observed in 22 patients, i.e., 84.61%, one (1) case of corticodependence (3.85%) and two (2) cases of corticoresistance, i.e., 7.69%. One (1) case of death was recorded, i.e., a frequency of 3.85%. Only one (1) patient had benefited from a renal biopsy puncture (RBP) and an anatomopathological examination. Conclusion: SN remains common in children at the HNZ. The improvement of the technical platform for PBR and anatomopathological examinations as well as the establishment of social security prove necessary for a better management of SN in children at the HNZ.
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Context + objective: Nephrotic syndrome accounts for 15 to 30% of glomerulopathies in adults;its evolution is difficult to predict. The objective of this work is to study the profile of patients suffering from idiopathic nephrotic syndrome in a nephrological hospital environment at the University Hospital of Yopougon in Abidjan. Methods: We carried out a retrospective study with descriptive and analytical purposes including any patient aged 15 and over who had had a clean kidney biopsy between January 2018 and October 2019 concluding in a nephrotic syndrome with minimal glomerular lesions (MGL) or segmental and focal hyalinosis (SFH) at the Nephrology Department of the University Hospital of Yopougon in Abidjan. Results: A total of 28 patients were collected. Patients with SFH type lesions were older with a statistically significant p value (p = 0.002). The male gender predominated in MGL type lesions without a statistically significant link (p = 0.26). SFH-type lesions were dominant (57.14%), followed by MGL-type lesions (42.86%). Oedematous syndrome was the main physical sign (n = 9;56.25%) without significant difference (p = 0.742). ESRD with a GFR less than 15 mL/min/1.73 m2 was found in 8 patients, which is 28.6% without significant difference. Microscopic haematuria coexisted in 5 patients (17.9%) and hypertension in 7 patients (25%). The first-line treatment was oral corticosteroid therapy at usual doses (n = 28;100%), associated with immunosuppressants (n = 11.79;17%). Partial remission was noted in 9 MGL type patients, that is 69.2% and a total remission in SFH type patients that is 50%, without any statistically significant link (p = 0.568). The main complications were CRD in 9 patients, which is 45%, followed by dyslipidaemia that is 30%. Conclusion: Adult idiopathic nephrotic syndrome is common and dominated by SFH type of histological lesions. Its outcome is generally favourable.
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.
