- Treatment-resistant PLA2R-negative membranous nephropathy responsive to low-density lipoprotein apheresis. [Case Reports]
- JCJ Clin Apher 2018 Dec 10
- Idiopathic membranous nephropathy is the most common cause of nephrotic syndrome in nondiabetic adults. The antibody most often implicated is the M-type phospholipase A2 receptor (PLA2R) antibody, fo...
Idiopathic membranous nephropathy is the most common cause of nephrotic syndrome in nondiabetic adults. The antibody most often implicated is the M-type phospholipase A2 receptor (PLA2R) antibody, found in >70% of primary membranous nephropathy cases. First-line therapy is immunosuppressive in nature, but for patients who are treatment-resistant there is a significant risk of end-stage renal disease and mortality. Hypercholesterolemia is not only a side effect of nephrotic syndrome, but also its presence may worsen renal function. A recent single-arm observational study in Japan found that low-density lipoprotein apheresis (LDL-A) was able to ameliorate nephrotic syndrome in half of patients who were resistant to medication. We present a case of treatment resistant PLA2R negative membranous nephropathy who had significant improvement following two courses of LDL-A. To our knowledge, this is the first such reported case in the United States.
- Efficacy and safety of rituximab therapy for membranous nephropathy: a meta-analysis. [Journal Article]
- EREur Rev Med Pharmacol Sci 2018; 22(22):8021-8029
- CONCLUSIONS: Rituximab had a beneficial effect and remissions of proteinuria on MN during follow-up times, but some adverse events were still unknown. Taking consideration of long-term therapeutic side effects and dose of the drug, we suggest that rituximab might replace cyclophosphamide and steroids as first-line immunosuppressive therapy in MN patients.
- Immunoglobulin E and G Levels in Predicting Minimal Change Disease before Renal Biopsy. [Journal Article]
- BRBiomed Res Int 2018; 2018:3480309
- CONCLUSIONS: Combining clinical model and this 2 Ig risk factors provides physicians simple and valuable clinical markers to diagnose MCD.
- Minimal change disease: A case report. [Journal Article]
- NNursing 2018 Dec 10
- Although minimal change disease (MCD) is a major cause of nephrotic syndrome in children, it's less common in adults. It develops from damage to the glomeruli with a loss of large amount of protein i...
Although minimal change disease (MCD) is a major cause of nephrotic syndrome in children, it's less common in adults. It develops from damage to the glomeruli with a loss of large amount of protein in the urine. Early recognition and treatment is the key to a good outcome. This article describes the diagnosis, treatment, and nursing care of an adult with MCD.
- Bioinformatic Analysis Reveals Novel Immune-Associated Hub Genes in Human Membranous Nephropathy. [Journal Article]
- GTGenet Test Mol Biomarkers 2018 Dec 08
- CONCLUSIONS: This study is the first to identify novel hub genes with transcriptome microarray data in MN using bioinformatics. The newly discovered hypothetical hub genes should be functionally tested to determine if they truly play an etiologic role in MN.
- SGPL1 Deficiency: A Rare Cause of Primary Adrenal Insufficiency. [Journal Article]
- JCJ Clin Endocrinol Metab 2018 Dec 03
- CONCLUSIONS: New genetic causes of PAI continue to be identified. We suggest that screening for SGPL1 mutations should not be reserved only for patients with nephrotic syndrome but may also include patients with PAI that lack other clinical manifestations of NPHS14 since, in certain cases, kidney disease and accompanying features might develop later on. Timely diagnosis of this specific sphingolipidosis, while the kidneys still function normally, can lead to prompt initiation of therapy and improve outcome especially, if a targeted NPHS14-treatment is available in the future.
- 3D organoid-derived human glomeruli for personalised podocyte disease modelling and drug screening. [Journal Article]
- NCNat Commun 2018 12 04; 9(1):5167
- The podocytes within the glomeruli of the kidney maintain the filtration barrier by forming interdigitating foot processes with intervening slit diaphragms, disruption in which results in proteinuria...
The podocytes within the glomeruli of the kidney maintain the filtration barrier by forming interdigitating foot processes with intervening slit diaphragms, disruption in which results in proteinuria. Studies into human podocytopathies to date have employed primary or immortalised podocyte cell lines cultured in 2D. Here we compare 3D human glomeruli sieved from induced pluripotent stem cell-derived kidney organoids with conditionally immortalised human podocyte cell lines, revealing improved podocyte-specific gene expression, maintenance in vitro of polarised protein localisation and an improved glomerular basement membrane matrisome compared to 2D cultures. Organoid-derived glomeruli retain marker expression in culture for 96 h, proving amenable to toxicity screening. In addition, 3D organoid glomeruli from a congenital nephrotic syndrome patient with compound heterozygous NPHS1 mutations reveal reduced protein levels of both NEPHRIN and PODOCIN. Hence, human iPSC-derived organoid glomeruli represent an accessible approach to the in vitro modelling of human podocytopathies and screening for podocyte toxicity.
- Evidence for a role of Angiotensin Converting Enzyme 2 in proteinuria of idiopathic nephrotic syndrome. [Journal Article]
- BRBiosci Rep 2018 Dec 04
- CONCLUSIONS: INS patients exhibited changes in RAS molecules and in chemokines. Proteinuria was associated with low levels of ACE2 and high levels of inflammatory molecules.
- Predicting risk of pulmonary infection in patients with primary membranous nephropathy on immunosuppressive therapy: the AIM-7C score. [Journal Article]
- NNephrology (Carlton) 2018 Nov 29
- CONCLUSIONS: Our AIM-7C score may therefore help predict the onset and facilitate the prevention of PI, a potentially life-threatening complication of the immunosuppressive therapy. This article is protected by copyright. All rights reserved.
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- Efficacy and safety of mycophenolate mofetil versus levamisole in frequently relapsing nephrotic syndrome: an open-label randomized controlled trial. [Journal Article]
- KIKidney Int 2018 Nov 22
- Both levamisole and mycophenolate mofetil (MMF) prevent relapses in patients with frequently relapsing nephrotic syndrome; however, their efficacy has not been compared prospectively. This single-cen...
Both levamisole and mycophenolate mofetil (MMF) prevent relapses in patients with frequently relapsing nephrotic syndrome; however, their efficacy has not been compared prospectively. This single-center, randomized, open-label trial enrolled 149 children ages 6-18 years with frequently relapsing or steroid-dependent nephrotic syndrome. Participants were randomized in a 1:1 ratio to receive therapy with MMF (750-1000 mg/m2 daily) or levamisole (2-2.5 mg/kg on alternate days) for 1 year; prednisolone was discontinued by 2-3 months. In intention-to-treat analyses, the frequency of relapse was similar between participants treated with MMF and levamisole (mean difference -0.29 relapses/patient-year; 95% confidence interval -0.65, 0.08). Relapse rates declined to almost one-third of baseline for both treatment groups. Therapy with MMF was not superior to levamisole in terms of the proportions of participants with sustained remission (40.8% vs. 34.2%), frequent relapses (14.5% vs. 16.4%), or treatment failure, a composite outcome of frequent relapses, steroid resistance, or significant steroid toxicity (15.8% vs. 20.6%). These outcomes were also similar in time to event analyses. Changes in anthropometry and blood pressure were similar between the groups, and the rates of adverse effects were low in both groups. Flow cytometry in 32 participants demonstrated similar proportions of B cells and CD4+, CD8+, T helper (Th)1, Th2, Th17, and T regulatory (Treg) cells during follow-up. Therapy with MMF was not superior to levamisole in the frequency of relapses, likelihood of sustained remission or corticosteroid sparing in children with frequently relapsing or steroid-dependent nephrotic syndrome. Registration CTRI/2012/02/002394.