Nephrotic syndrome in adults: symptoms and management

doi:10.1211/pj.2021.1.43065

Cited by 3 publications

(1 citation statement)

References 748 publications

(1,217 reference statements)

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“…The most prominent symptom, hypoalbuminemia, is frequently accompanied by dyslipidemia and edema. Renal function may be reduced, and in some cases, an immunological disorder is an underlying condition. − …”

Section: Introductionmentioning

confidence: 99%

Profound Changes in Functional Structure and Dynamics of Serum Albumin in Children with Nephrotic Syndrome: An Exploratory Research Study

Haeri,

Eisermann,

Schimm

et al. 2023

J. Med. Chem.

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Patients with nephrotic syndrome (NS) suffer from urinary loss of albumin. As a cause, previous studies focused on the glomerular filter rather than analyzing the molecular properties of albumin itself. Later one was initiated by clinical observations indicating unexplained molecular alterations of human serum albumin (HSA) in an NS pediatric patient. Therefore, we examined serum from eight pediatric patients with steroidsensitive and -resistant NS and compared it with serum from healthy subjects as well as commercial HSA. We used dynamic and electrophoretic light scattering to characterize the protein size and effective surface charge and electron paramagnetic resonance spectroscopy to measure the local environment and binding dynamics of up to seven fatty acids associated with HSA. Our findings suggest that pronounced differences in binding behavior and surface charge of HSA could enhance their filtration through the GBM, leading to direct toxicity of HSA to podocytes.

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Section: Introductionmentioning

confidence: 99%

Profound Changes in Functional Structure and Dynamics of Serum Albumin in Children with Nephrotic Syndrome: An Exploratory Research Study

Haeri,

Eisermann,

Schimm

et al. 2023

J. Med. Chem.

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Efficacy and Safety of Mizoribine for the Treatment of Refractory Nephrotic Syndrome: Protocol for a Multicenter, Controlled, Open-label, Randomized Controlled Trial

Dong¹,

Zhou²,

Xu³

et al. 2023

JMIR Res Protoc

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Background Nephrotic syndrome that is resistant to steroid therapy is termed refractory nephrotic syndrome (RNS), a condition that is associated with an increased risk of end-stage renal disease. Immunosuppressants are used to treat RNS; however, prolonged use may lead to significant adverse effects. Mizoribine (MZR) is a novel agent used in long-term immunosuppressive therapy, which has few adverse effects, but data on its long-term use in patients with RNS are unavailable. Objective We propose a trial to examine the efficacy and safety of MZR compared with cyclophosphamide (CYC) in Chinese adult patients with RNS. Methods This is a multicenter, randomized, controlled interventional study with a screening phase (1 week) and a treatment phase (52 weeks). This study has been reviewed and approved by the Medical Ethics Committees of all 34 medical centers that are participating. Patients with RNS consent to participation, and are enrolled and randomized to an MZR group or a CYC group (1:1 ratio), with each group receiving tapering doses of oral corticosteroids. Participants are assessed for adverse effects, and laboratory results are collected at 8 visits during the treatment phase (weeks 4, 8, 12, 16, 20, 32, 44, and 52 [exit visit]). Participants are able to withdraw voluntarily, and investigators are required to remove patients when there are safety concerns or deviations from the protocol. Results The study started in November 2014 and was completed in March 2019. A total of 239 participants from 34 hospitals in China have been enrolled. Data analysis has been completed. The results are being finalized by the Center for Drug Evaluation. Conclusions This study examines the safety and efficacy of MZR as a long-term treatment approach for Chinese adults with RNS. It is the longest lasting and largest randomized controlled trial to examine MZR in Chinese patients. The results can help determine whether RNS should be considered as an additional indication for MZR treatment in China. Trial Registration ClinicalTrials.gov NCT02257697; https://clinicaltrials.gov/ct2/show/NCT02257697 International Registered Report Identifier (IRRID) RR1-10.2196/46101

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Efficacy and Safety of Mizoribine for the Treatment of Refractory Nephrotic Syndrome: Protocol for a Multicenter, Controlled, Open-label, Randomized Controlled Trial (Preprint)

Dong¹,

Zhou²,

Xu³

et al. 2023

Preprint

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BACKGROUND Nephrotic syndrome that is resistant to steroid therapy is termed refractory nephrotic syndrome (RNS), a condition that is associated with an increased risk of end-stage renal disease. Immunosuppressants are used to treat RNS; however, prolonged use may lead to significant adverse effects. Mizoribine (MZR) is a novel agent used in long-term immunosuppressive therapy, which has few adverse effects, but data on its long-term use in patients with RNS are unavailable. OBJECTIVE We propose a trial to examine the efficacy and safety of MZR compared with cyclophosphamide (CYC) in Chinese adult patients with RNS. METHODS This is a multicenter, randomized, controlled interventional study with a screening phase (1 week) and a treatment phase (52 weeks). This study has been reviewed and approved by the Medical Ethics Committees of all 34 medical centers that are participating. Patients with RNS consent to participation, and are enrolled and randomized to an MZR group or a CYC group (1:1 ratio), with each group receiving tapering doses of oral corticosteroids. Participants are assessed for adverse effects, and laboratory results are collected at 8 visits during the treatment phase (weeks 4, 8, 12, 16, 20, 32, 44, and 52 [exit visit]). Participants are able to withdraw voluntarily, and investigators are required to remove patients when there are safety concerns or deviations from the protocol. RESULTS The study started in November 2014 and was completed in March 2019. A total of 239 participants from 34 hospitals in China have been enrolled. Data analysis has been completed. The results are being finalized by the Center for Drug Evaluation. CONCLUSIONS This study examines the safety and efficacy of MZR as a long-term treatment approach for Chinese adults with RNS. It is the longest lasting and largest randomized controlled trial to examine MZR in Chinese patients. The results can help determine whether RNS should be considered as an additional indication for MZR treatment in China. CLINICALTRIAL ClinicalTrials.gov NCT02257697; https://clinicaltrials.gov/ct2/show/NCT02257697 INTERNATIONAL REGISTERED REPORT RR1-10.2196/46101

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Nephrotic syndrome in adults: symptoms and management

Cited by 3 publications

References 748 publications

Profound Changes in Functional Structure and Dynamics of Serum Albumin in Children with Nephrotic Syndrome: An Exploratory Research Study

Profound Changes in Functional Structure and Dynamics of Serum Albumin in Children with Nephrotic Syndrome: An Exploratory Research Study

Efficacy and Safety of Mizoribine for the Treatment of Refractory Nephrotic Syndrome: Protocol for a Multicenter, Controlled, Open-label, Randomized Controlled Trial

Efficacy and Safety of Mizoribine for the Treatment of Refractory Nephrotic Syndrome: Protocol for a Multicenter, Controlled, Open-label, Randomized Controlled Trial (Preprint)

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