OBJECTIVE:To assess the clinical effectiveness and safety of Danshen injection(丹参注射液)following immunosuppressive therapy in primary nephrotic syndrome(PNS)with acute kidney injury(AKI)patients.METHODS:PNS with AK...OBJECTIVE:To assess the clinical effectiveness and safety of Danshen injection(丹参注射液)following immunosuppressive therapy in primary nephrotic syndrome(PNS)with acute kidney injury(AKI)patients.METHODS:PNS with AKI patients selected from nephrotic syndrome inpatients between 2012 and 2022 were divided into two groups according to the treatment regimen:the DS group who were treated with the Danshen injection and the SC group who received supportive care.Propensity score matching was used to balance the confounding factors between the two groups.The outcomes were complete and partial renal function recovery for effectiveness analysis and severe bleeding events for safety analysis.Kaplan-Meier curves and logrank tests were used to compare cumulative recovery rates.Outcome risks were assessed using Cox proportional hazard regression models.RESULTS:The study enrolled 441 patients with PNS and AKI from a population of 3873 inpatients with nephrotic syndrome.Among them,95 received Danshen injection,and 346 received supportive care.After propensity score matching,95 patients in the DS group and 285 patients in the SC group were analyzed.No significant difference was observed in the complete recovery rate for the DS group vs SC group(72.9%vs 65.1%,P=0.130)at 6 months.Compared with the SC group,the DS group was associated with a significantly higher partial recovery rate(71.0%vs 37.5%,P=0.011)at 6 months and all recovery rates(93.4%vs 79.1%,P=0.006)at 6 months.CONCLUSION:In this real-world retrospective study of PNS with AKI patients,Danshen injection following immunosuppressive therapy and prophylactic anticoagulation with low molecular heparin significantly increased 6-month partial renal function recovery and had a safety profile.Further randomized clinical trials are warranted to confirm the benefit of Danshen(Radix Salviae Miltiorrhizae)in these patients.展开更多
BACKGROUND The Wilms tumor 1(WT1)mutation is significantly associated with steroidresistant nephrotic syndrome(SRNS)in children.It causes a spectrum of disorders,including Frasier and Denys-Drash syndromes.The WT1 gen...BACKGROUND The Wilms tumor 1(WT1)mutation is significantly associated with steroidresistant nephrotic syndrome(SRNS)in children.It causes a spectrum of disorders,including Frasier and Denys-Drash syndromes.The WT1 gene encodes a transcription factor essential for renal,gonadal,and mesothelial development.The prevalence of Frasier syndrome is 1 in 1000000.Frasier syndrome presents with childhood onset of SRNS,progressing to end-stage kidney disease,has an XY female phenotype,delayed puberty,and gonadal tumor risk.Two siblings with identical WT1 mutations but differing phenotypes offer important clinical insights.CASE SUMMARY We report a rare familial occurrence of shared mutation of WT1(at intron 9 splice site:C.1432+5G>A)and COL4A5 genes in two siblings.The elder sibling(46,XY)presented with infantile steroid-resistant nephrotic syndrome and rapidly deteriorating and progressing to end-stage kidney disease with XY karyotype.The genetic testing confirmed the diagnosis of Frasier syndrome associated with a WT1 mutation in the elder sibling.She is currently on maintenance dialysis,awaiting renal transplantation.The younger sibling presented at age four with facial edema and SRNS with slower disease progression and no apparent gonadal anomalies.Her renal biopsy revealed minimal change disease,and she maintains stable renal function under antiproteinuric therapy.Genetic testing confirmed the diagnosis of WT1 mutation with 46,XX karyotype in the younger sibling.Both siblings also carried a COL4A5 gene variant of uncertain significance.Family history reveals consanguineous marriage and a similar presentation in a sibling.CONCLUSION Despite identical mutations,phenotypic differences highlight complex genotype-phenotype relations,stressing the need for research,genetic counseling,and family member screening.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
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
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
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
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
We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. ...We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. Each time the dose of steroid is tapered, a relapse of the nephrotic syndrome occurred. Eventually, the patient was complaining of dysphagia and difficulty swallowing. Hospital work-up with barium swallow, endoscopy, and CT of the chest, abdomen and pelvis, revealed a focal stenotic lesion with mild to moderate esophageal dysmotility 7/15/2022. A diagnosis of an ulcerating lesion with biopsy confirmed a neuro-endocrine carcinoma of the gastro-esophageal junction was entertained. The CT of the chest/abdomen/pelvis, 7/19/2022, has shown, an esophageal mass of 5.1 × 5.6 × 7 cm of the gastro-esophageal junction with ulceration. No evidence of spread beyond the esophagus and stomach. The histology revealed a poorly differentiated neuroendocrine tumor of the gastro-esophageal junction. The patient underwent several rounds of chemotherapy, radiation, and surgery culminating in tumor control. His nephrotic syndrome was resolved after the tumor has been controlled by surgery and chemotherapy.展开更多
BACKGROUND The interaction between the kidney and the thyroid is important for normal function of both organs.In nephrotic syndrome,proteinuria leads to loss of several proteins,which in turn causes hypothyroidism.AIM...BACKGROUND The interaction between the kidney and the thyroid is important for normal function of both organs.In nephrotic syndrome,proteinuria leads to loss of several proteins,which in turn causes hypothyroidism.AIM To assess the thyroid function in children with nephrotic syndrome.METHODS This cross-sectional study was conducted in a tertiary center,Bhopal,from February 2020 to January 2021.Consecutive children aged 1-15 years admitted with nephrotic syndrome(first-time diagnosed and all relapse cases)were included in the study.A thyroid profile was sent along with routine investigations,and thyroid hormone status was assessed in nephrotic syndrome children.RESULTS Of the 70 patients,39(55.7%)showed abnormal thyroid profiles;19(27.1%)had overt hypothyroidism,and 20(28.6%)had subclinical hypothyroidism.Overt hypothyroidism was seen in 16.1%of newly diagnosed cases,40%of second relapses,and 2.7%of frequently relapsed cases(P<0.001).The mean serum free T3 and free T4 levels in frequent relapses were 2.50±0.39 ng/dL and 0.78±0.12 ng/dL,respectively,which were significantly lower than in newly diagnosed cases(2.77±0.37 ng/dL and 0.91±0.19 ng/dL,respectively).The mean thyroidstimulating hormone(TSH)level was significantly higher in frequent relapses (5.86±1.56μIU/mL)and second relapse(5.81±1.78μIU/mL)than in newly diagnosed cases(4.83±0.76μIU/mL)and first relapse cases(4.74±1.17μIU/mL),(P<0.01).CONCLUSION An abnormal thyroid profile was commonly observed in children with nephrotic syndrome,and overt hypothyroidism was more common in frequent relapse cases.Therefore,thyroid screening should be a part of the management of nephrotic syndrome so that hypothyroidism can be detected and managed at an early stage.展开更多
Objective To investigate the efficacy and safety of rituximab(RTX) in the treatment of idiopathic membranous nephropathy(IMN) with nephrotic syndrome with a systematic review and meta-analysis.Methods Pub Med, Embase,...Objective To investigate the efficacy and safety of rituximab(RTX) in the treatment of idiopathic membranous nephropathy(IMN) with nephrotic syndrome with a systematic review and meta-analysis.Methods Pub Med, Embase, Cochrane Library and Clinical Trials(December 2016) were searched to identify researches investigating the treatment of RTX in adult patients with biopsy-proven IMN. Complete remission(CR) or partial remission was regarded as effective therapy, and the cumulated remission rate was calculated.Results Seven studies involved 120 patients(73% were men) were included in our systematic review and metaanalysis. All were prospective observation cohort studies or matched-cohort studies, mainly came from two medical centers, and one study was multi-centric(four nephrology units in northern Italy). The creatinine clearance was more than 20 ml/(min·1.73 m2) and persistent proteinuria higher than 3.5 g/d for at least 6 months. All patients received treatment previously [44(36.7%) had immunosuppressive treatment]. In 12-and 24-month, 56%(95%CI, 0.47-0.65) and 68%(95%CI, 0.41-0.87) patients could reach remission, while 15%(95%CI, 0.09-0.23) and 20%(95%CI, 0.12-0.32) patients could reach CR. The reduction in proteinuria was gradual and obvious, paralleled with upward trend of serum albumin level and decreasing serum cholesterol level. Renal functions were stable. Relapses happened in 24 months were around 8%. RTX related adverse events were mild and were mostly infusion-related reactions.Conclusions RTX treatment in IMN was efficient, well tolerated and safe. More than 60% patients can reach partial remission or CR in 24 months, and relapse is rare. Adverse events of RTX are mostly infusion-related reactions and generally mild.展开更多
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
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
Nephrotic syndrome is one of the most common childhood kidney diseases. It is mostly found in the age group of 2 to 8 years. Around 10%-15% of nephrotic syndrome cases are non-responders of steroid treatment(SRNS).Ang...Nephrotic syndrome is one of the most common childhood kidney diseases. It is mostly found in the age group of 2 to 8 years. Around 10%-15% of nephrotic syndrome cases are non-responders of steroid treatment(SRNS).Angiotensin converting enzyme(ACE)(I/D) gene association studies are important for detecting kidney disease and herein we assessed the association of ACE(I/D) polymorphism with nephrotic syndrome in South Indian children. We recruited 260 nephrotic syndrome(162 boys and 98 girls) and 218(140 boys and 78 girls) control subjects. ACE I/D polymorphism was analyzed by PCR using genotype allele specific primers. In ACE(I/D), we did not find significant association for the ungrouped data of nephrotic syndrome children and the control subjects. Kidney biopsies were done in 86 nephrotic syndrome cases(minimal change disease, n = 51;focal segmental glomerulosclerosis, n = 27;diffuse mesangial proliferation, n = 8). We segregated them into the minimal change disease/focal segmental glomerulosclerosis groups and observed that the ACE’D’ allele was identified with borderline significance in cases of focal segmental glomerulosclerosis and the ’Ⅰ’ allele was assessed as having very weak association in cases of minimal change disease. ’Ⅱ’ genotype was weakly associated with minimal change disease. Gender specific analysis revealed weak association of’ID’ genotype with female nephrotic syndrome in females. Dominant expression of DD genotype was observed in males with nephrotic syndrome. Our finding indicated that ACE(I/D) has moderate association with focal segmental glomerulosclerosis. However, due to the limited number of biopsy proven focal segmental glomerulosclerosis subjects enrolled, further studies are required to confirm these results.展开更多
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
THROMBOEMBOLISM is a well-known complicationof nephrotic syndrome. Deep vein thrombosis,pulmonary embolism and renal vein thrombosisare the most common venous thromboembolicdiseases in patients with nephrotic syndrome...THROMBOEMBOLISM is a well-known complicationof nephrotic syndrome. Deep vein thrombosis,pulmonary embolism and renal vein thrombosisare the most common venous thromboembolicdiseases in patients with nephrotic syndrome, while arterialthromboembolic complications are observed less frequently,and rarely when it occurs before the diagnosis of nephroticsyndrome.1 Here we report a case with minimal change ofnephrotic syndrome who presented initially as external iliacartery thrombosis, which may be associated with longsitting while playing Mah-Jong.展开更多
Thromboembolic complications have been frequently re- ported in patients with long-lasting nephrotic syndrome (NS). Although thrombotic complications in the venous system are common in patients with NS, arterial thr...Thromboembolic complications have been frequently re- ported in patients with long-lasting nephrotic syndrome (NS). Although thrombotic complications in the venous system are common in patients with NS, arterial thromboses associated with NS are much less common. However, coronary thromboses are extremely rarely observed. So, NS is a rare cause of acute coronary syndrome (ACS). As such, the incidence, pathogenesis, and treatment of these patients have yet to be clearly defined. In the current litera- ture, publications contain less than 15 patients, most of whom are young children.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
REVERSIBLE posterior leukoencephalopathy syn- drome (RPLS) is a rare neurological syndrome charac- terized by headache, altered mental status, seizures, and visual disturbance,associated with reversible white matter...REVERSIBLE posterior leukoencephalopathy syn- drome (RPLS) is a rare neurological syndrome charac- terized by headache, altered mental status, seizures, and visual disturbance,associated with reversible white matter cnanges,- n been commonly reported in patients with severe hypertension and pre-eclampsia. Here we report a case with nephrotic syndrome complicated by RPLS.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 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
基金Supported by Key Project of Science and Technology Program Coconstructed by the Science and Technology Department of the National Administration of Traditional Chinese Medicine of China and Administration of Traditional Chinese Medicine of Zhejiang Province:A Study on the Efficacy and Safety of Danshen(Radix Salviae Miltiorrhizae)in Treating Acute Kidney Injury in Patients with Primary Nephrotic Syndrome(GZY-ZJ-KJ-23085)Basic Research Project of Wenzhou Science and Technology Bureau:the Mechanism of Hypoxia Induicible Factor-1 alpha Down-regulating Angiotensinconverting Enzyme 2 in Proximal Renal Tubular Epithelial Cells Leading to Brush Border Shedding in Early Acute Kidney Injury Induced by Sepsis(Y20240071)。
摘要OBJECTIVE:To assess the clinical effectiveness and safety of Danshen injection(丹参注射液)following immunosuppressive therapy in primary nephrotic syndrome(PNS)with acute kidney injury(AKI)patients.METHODS:PNS with AKI patients selected from nephrotic syndrome inpatients between 2012 and 2022 were divided into two groups according to the treatment regimen:the DS group who were treated with the Danshen injection and the SC group who received supportive care.Propensity score matching was used to balance the confounding factors between the two groups.The outcomes were complete and partial renal function recovery for effectiveness analysis and severe bleeding events for safety analysis.Kaplan-Meier curves and logrank tests were used to compare cumulative recovery rates.Outcome risks were assessed using Cox proportional hazard regression models.RESULTS:The study enrolled 441 patients with PNS and AKI from a population of 3873 inpatients with nephrotic syndrome.Among them,95 received Danshen injection,and 346 received supportive care.After propensity score matching,95 patients in the DS group and 285 patients in the SC group were analyzed.No significant difference was observed in the complete recovery rate for the DS group vs SC group(72.9%vs 65.1%,P=0.130)at 6 months.Compared with the SC group,the DS group was associated with a significantly higher partial recovery rate(71.0%vs 37.5%,P=0.011)at 6 months and all recovery rates(93.4%vs 79.1%,P=0.006)at 6 months.CONCLUSION:In this real-world retrospective study of PNS with AKI patients,Danshen injection following immunosuppressive therapy and prophylactic anticoagulation with low molecular heparin significantly increased 6-month partial renal function recovery and had a safety profile.Further randomized clinical trials are warranted to confirm the benefit of Danshen(Radix Salviae Miltiorrhizae)in these patients.
摘要BACKGROUND The Wilms tumor 1(WT1)mutation is significantly associated with steroidresistant nephrotic syndrome(SRNS)in children.It causes a spectrum of disorders,including Frasier and Denys-Drash syndromes.The WT1 gene encodes a transcription factor essential for renal,gonadal,and mesothelial development.The prevalence of Frasier syndrome is 1 in 1000000.Frasier syndrome presents with childhood onset of SRNS,progressing to end-stage kidney disease,has an XY female phenotype,delayed puberty,and gonadal tumor risk.Two siblings with identical WT1 mutations but differing phenotypes offer important clinical insights.CASE SUMMARY We report a rare familial occurrence of shared mutation of WT1(at intron 9 splice site:C.1432+5G>A)and COL4A5 genes in two siblings.The elder sibling(46,XY)presented with infantile steroid-resistant nephrotic syndrome and rapidly deteriorating and progressing to end-stage kidney disease with XY karyotype.The genetic testing confirmed the diagnosis of Frasier syndrome associated with a WT1 mutation in the elder sibling.She is currently on maintenance dialysis,awaiting renal transplantation.The younger sibling presented at age four with facial edema and SRNS with slower disease progression and no apparent gonadal anomalies.Her renal biopsy revealed minimal change disease,and she maintains stable renal function under antiproteinuric therapy.Genetic testing confirmed the diagnosis of WT1 mutation with 46,XX karyotype in the younger sibling.Both siblings also carried a COL4A5 gene variant of uncertain significance.Family history reveals consanguineous marriage and a similar presentation in a sibling.CONCLUSION Despite identical mutations,phenotypic differences highlight complex genotype-phenotype relations,stressing the need for research,genetic counseling,and family member screening.
摘要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.
摘要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.
摘要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.
摘要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.
摘要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.
摘要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.
摘要BACKGROUND The interaction between the kidney and the thyroid is important for normal function of both organs.In nephrotic syndrome,proteinuria leads to loss of several proteins,which in turn causes hypothyroidism.AIM To assess the thyroid function in children with nephrotic syndrome.METHODS This cross-sectional study was conducted in a tertiary center,Bhopal,from February 2020 to January 2021.Consecutive children aged 1-15 years admitted with nephrotic syndrome(first-time diagnosed and all relapse cases)were included in the study.A thyroid profile was sent along with routine investigations,and thyroid hormone status was assessed in nephrotic syndrome children.RESULTS Of the 70 patients,39(55.7%)showed abnormal thyroid profiles;19(27.1%)had overt hypothyroidism,and 20(28.6%)had subclinical hypothyroidism.Overt hypothyroidism was seen in 16.1%of newly diagnosed cases,40%of second relapses,and 2.7%of frequently relapsed cases(P<0.001).The mean serum free T3 and free T4 levels in frequent relapses were 2.50±0.39 ng/dL and 0.78±0.12 ng/dL,respectively,which were significantly lower than in newly diagnosed cases(2.77±0.37 ng/dL and 0.91±0.19 ng/dL,respectively).The mean thyroidstimulating hormone(TSH)level was significantly higher in frequent relapses (5.86±1.56μIU/mL)and second relapse(5.81±1.78μIU/mL)than in newly diagnosed cases(4.83±0.76μIU/mL)and first relapse cases(4.74±1.17μIU/mL),(P<0.01).CONCLUSION An abnormal thyroid profile was commonly observed in children with nephrotic syndrome,and overt hypothyroidism was more common in frequent relapse cases.Therefore,thyroid screening should be a part of the management of nephrotic syndrome so that hypothyroidism can be detected and managed at an early stage.
基金Supported by the Key Projects in the National Science and Technology Pillar Program during the Twelfth Five-year Plan Period(2011BAI10B03)
摘要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.
基金supported by the National Research Foundation of Korea(NRF)grant funded by the Korean government(MSIP)(Nos.2017R1A5A2015805,2021R1A2C1010859,and 2021R1C1C2009542).
摘要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.
摘要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supported by Networking Resource Centre in Biological Sciences (NRCBS funded by UGC, Govt. of India)
摘要Nephrotic syndrome is one of the most common childhood kidney diseases. It is mostly found in the age group of 2 to 8 years. Around 10%-15% of nephrotic syndrome cases are non-responders of steroid treatment(SRNS).Angiotensin converting enzyme(ACE)(I/D) gene association studies are important for detecting kidney disease and herein we assessed the association of ACE(I/D) polymorphism with nephrotic syndrome in South Indian children. We recruited 260 nephrotic syndrome(162 boys and 98 girls) and 218(140 boys and 78 girls) control subjects. ACE I/D polymorphism was analyzed by PCR using genotype allele specific primers. In ACE(I/D), we did not find significant association for the ungrouped data of nephrotic syndrome children and the control subjects. Kidney biopsies were done in 86 nephrotic syndrome cases(minimal change disease, n = 51;focal segmental glomerulosclerosis, n = 27;diffuse mesangial proliferation, n = 8). We segregated them into the minimal change disease/focal segmental glomerulosclerosis groups and observed that the ACE’D’ allele was identified with borderline significance in cases of focal segmental glomerulosclerosis and the ’Ⅰ’ allele was assessed as having very weak association in cases of minimal change disease. ’Ⅱ’ genotype was weakly associated with minimal change disease. Gender specific analysis revealed weak association of’ID’ genotype with female nephrotic syndrome in females. Dominant expression of DD genotype was observed in males with nephrotic syndrome. Our finding indicated that ACE(I/D) has moderate association with focal segmental glomerulosclerosis. However, due to the limited number of biopsy proven focal segmental glomerulosclerosis subjects enrolled, further studies are required to confirm these results.
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)。
摘要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Funded by Obershi blood purification academician workstation of Guangdong Province(2013B090400004)
摘要THROMBOEMBOLISM is a well-known complicationof nephrotic syndrome. Deep vein thrombosis,pulmonary embolism and renal vein thrombosisare the most common venous thromboembolicdiseases in patients with nephrotic syndrome, while arterialthromboembolic complications are observed less frequently,and rarely when it occurs before the diagnosis of nephroticsyndrome.1 Here we report a case with minimal change ofnephrotic syndrome who presented initially as external iliacartery thrombosis, which may be associated with longsitting while playing Mah-Jong.
基金The program is supported by grants from the National Na tural Science Foundation of China (No.81400238).
摘要Thromboembolic complications have been frequently re- ported in patients with long-lasting nephrotic syndrome (NS). Although thrombotic complications in the venous system are common in patients with NS, arterial thromboses associated with NS are much less common. However, coronary thromboses are extremely rarely observed. So, NS is a rare cause of acute coronary syndrome (ACS). As such, the incidence, pathogenesis, and treatment of these patients have yet to be clearly defined. In the current litera- ture, publications contain less than 15 patients, most of whom are young children.
摘要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.
摘要REVERSIBLE posterior leukoencephalopathy syn- drome (RPLS) is a rare neurological syndrome charac- terized by headache, altered mental status, seizures, and visual disturbance,associated with reversible white matter cnanges,- n been commonly reported in patients with severe hypertension and pre-eclampsia. Here we report a case with nephrotic syndrome complicated by RPLS.
摘要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)。
摘要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.