6,951 results match your criteria Glomerulosclerosis Focal Segmental


The elusive podocyte crossmatch for recurrent focal segmental glomerulosclerosis.

Kidney Int 2019 Mar;95(3):498-500

Peggy and Harold Katz Family Drug Discovery Center, University of Miami, Miller School of Medicine, Miami, Florida, USA; Katz Family Division of Nephrology and Hypertension, Department of Medicine, University of Miami, Miller School of Medicine, Miami, Florida, USA. Electronic address:

Focal segmental glomerulosclerosis is a glomerular disorder with a high rate of recurrence. Although kidney biopsies remain the gold standard to diagnose recurrent focal segmental glomerulosclerosis, non-invasive prognostic assays could facilitate preventive treatments and offer insight into disease pathogenicity and heterogeneity. Srivastava et al. Read More

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http://dx.doi.org/10.1016/j.kint.2018.11.032DOI Listing

Successful Treatment With Abatacept in Recurrent Focal Segmental Glomerulosclerosis After Kidney Transplant.

Exp Clin Transplant 2019 Jan;17(Suppl 1):178-180

From the Department of Adult Kidney and Pancreas Transplantation, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.

Primary focal segmental glomerulosclerosis recurrence occurs in 10% to 50% of recipients after kidney transplant and may affect both children and adults. Treatment after recurrence with plasma exchange and immunosuppression is quite variable and challenging, and those who do not respond usually progress to allograft failure. Podocyte injury and B7-1 expression and subsequently its blockade (abatacept) have been reported to be associated with complete remission of proteinuria in 4 patients with focal segmental glomerulosclerosis recurrence after kidney transplantation and in 1 patient with focal segmental glomerulosclerosis in native kidney. Read More

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http://dx.doi.org/10.6002/ect.MESOT2018.P53DOI Listing
January 2019

Repository Corticotropin Versus Glucocorticoid for Nephrotic Syndrome: When Will We See the Evidence?

Am J Kidney Dis 2019 Feb 11. Epub 2019 Feb 11.

VA Portland Health Care System, Research Service, Portland, OR; Division of Nephrology and Hypertension, Department of Medicine, Oregon Health & Science University, Portland, OR. Electronic address:

Despite little evidence supporting its superiority to glucocorticoid therapy, use and expenditures for repository corticotropin (rACTH) injection (H.P. Acthar Gel; Mallinckrodt) have increased dramatically in the last 5 years, particularly among a small number of nephrologists, rheumatologists, and neurologists. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S02726386193000
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http://dx.doi.org/10.1053/j.ajkd.2018.12.025DOI Listing
February 2019
4 Reads

Tocilizumab for focal segmental glomerulosclerosis secondary to multicentric Castleman's disease.

Ann Hematol 2019 Feb 13. Epub 2019 Feb 13.

Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo Bunkyo-ku, Tokyo, 113-8655, Japan.

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http://dx.doi.org/10.1007/s00277-019-03616-yDOI Listing
February 2019
1 Read

Identification of a Novel Variant in Compound-Heterozygous State in a Patient With Alport Syndrome and Histological Findings Similar to Focal Segmental Glomerulosclerosis (FSGS).

Front Genet 2018 28;9:748. Epub 2019 Jan 28.

Department of Nephrology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Alport syndrome (AS) is a rare and inherited renal disorder with an autosomal recessive mode of inheritance. AS patients usually manifest with hematuria and progressive renal disorder also occasionally accompanied by hearing loss and ophthalmic disease. Germline variants in collagen type IV α-4 () gene lead to autosomal recessive Alport syndrome. Read More

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http://dx.doi.org/10.3389/fgene.2018.00748DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360158PMC
January 2019
1 Read

The first identified heterozygous nonsense mutations in podocalyxin offer new perspectives on the biology of podocytopathies.

Clin Sci (Lond) 2019 Feb 8;133(3):443-447. Epub 2019 Feb 8.

The Biomedical Research Centre, University of British Columbia, Vancouver, Canada

In the last two decades, our understanding of the genetic underpinnings of inherited podocytopathies has advanced immensely. By sequencing the genomes of a large pool of families affected by focal segmental glomerulosclerosis (FSGS), researchers have identified a common theme: familial podocytopathies are frequently caused by genes selectively expressed in podocytes. Podocalyxin is a podocyte-specific surface sialomucin that has long been known to play important roles in podocyte morphogenesis and function. Read More

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http://dx.doi.org/10.1042/CS20181067DOI Listing
February 2019
1 Read

Treatment with 2,4-Dihydroxybenzoic Acid Prevents FSGS Progression and Renal Fibrosis in Podocyte-Specific Knockout Mice.

J Am Soc Nephrol 2019 Feb 8. Epub 2019 Feb 8.

Division of Nephrology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts;

Background: Although studies have identified >55 genes as causing steroid-resistant nephrotic syndrome (SRNS) and localized its pathogenesis to glomerular podocytes, the disease mechanisms of SRNS remain largely enigmatic. We recently reported that individuals with mutations in COQ6, a coenzyme Q (also called CoQ, CoQ, or ubiquinone) biosynthesis pathway enzyme, develop SRNS with sensorineural deafness, and demonstrated the beneficial effect of CoQ for maintenace of kidney function.

Methods: To study function in podocytes, we generated a podocyte-specific knockout mouse ( ) model and a transient siRNA-based knockdown in a human podocyte cell line. Read More

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http://dx.doi.org/10.1681/ASN.2018060625DOI Listing
February 2019
2 Reads

Clinical course & management of childhood nephrotic syndrome in Germany: a large epidemiological ESPED study.

BMC Nephrol 2019 Feb 7;20(1):45. Epub 2019 Feb 7.

Department of Radiology, Pediatric Radiology, University Hospital of Bonn, Bonn, Germany.

Background: Nephrotic syndrome (NS) is one of the most frequent occurring chronic kidney diseases in childhood, despite its rarely occurrence in the general population. Detailed information about clinical data of NS (e.g. Read More

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http://dx.doi.org/10.1186/s12882-019-1233-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367765PMC
February 2019
5 Reads

The Role of Extracellular Phosphate Levels on Kidney Disease Progression in a Podocyte Injury Mouse Model.

Nephron 2019 Feb 7:1-12. Epub 2019 Feb 7.

Department of Molecular Nutrition, Institute of Biomedical Sciences, Tokushima University Graduate School, Tokushima, Japan.

Background: Hyperphosphatemia is a major accelerator of complications in chronic kidney disease and dialysis, and phosphate (Pi) binders have been shown to regulate extracellular Pi levels. Research on hyperphosphatemia in mouse models is scarce, and few models display hyperphosphatemia induced by glomerular injury, despite its relevance to human glomerular disease conditions. In this study, we investigated the involvement of hyperphosphatemia in kidney disease progression using a mouse model in which hyperphosphatemia is induced by focal segmental glomerulosclerosis (FSGS). Read More

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http://dx.doi.org/10.1159/000497118DOI Listing
February 2019
1 Read

uPAR isoform 2 forms a dimer and induces severe kidney disease in mice.

J Clin Invest 2019 Feb 7. Epub 2019 Feb 7.

Soluble urokinase plasminogen activator receptor (suPAR) is an immune-derived circulating signaling molecule that has been implicated in chronic kidney disease such as focal segmental glomerulosclerosis (FSGS). Typically, native uPAR (isoform 1) translates to a three-domain protein capable of binding and activating integrins, yet the function of additional isoforms generated by alternative splicing is unknown. Here, we characterized mouse uPAR isoform 2 (msuPAR2), encoding domain I and nearly one-half of domain II, as a dimer in solution, as revealed by 3D electron microscopy structural analysis. Read More

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http://dx.doi.org/10.1172/JCI124793DOI Listing
February 2019
1 Read
13.215 Impact Factor

The effects of add-on corticosteroids on renal outcomes in patients with biopsy proven HIV associated nephropathy: a single centre study from South Africa.

BMC Nephrol 2019 Feb 6;20(1):44. Epub 2019 Feb 6.

Division of Nephrology and Hypertension, Groote Schuur Hospital, University of Cape Town, Cape Town, South Africa.

Background: The aim of this study was to assess, the efficacy and safety of add-on corticosteroids to antiretroviral therapy [ART] in patients with biopsy proven HIV associated nephropathy.

Methods: All included patients had histological evidence of either collapsing or non-collapsing focal segmental glomerulosclerosis (FSGS) or podocyte and/or parietal cell hypertrophy or hyperplasia. All patients had evidence of tubulointerstitial inflammation with microcysts. Read More

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http://dx.doi.org/10.1186/s12882-019-1208-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6366071PMC
February 2019
1 Read

Endogenous IL-22 is dispensable for experimental glomerulonephritis.

Am J Physiol Renal Physiol 2019 Feb 6. Epub 2019 Feb 6.

III. Medizinische Klinik und Poliklinik, Universitaetsklinikum Hamburg-Eppendorf, Germany.

In recent years, the cytokine IL-22 attracted considerable attention due to its important immunoregulatory function in barrier tissues, such as the gut, lung and skin. While a regenerative role of IL-22 in renal tubular damage has been demonstrated, the role of IL-22 in the immunopathogenesis of glomerular injury is still unknown. Here, we demonstrate that the IL-22 receptor is expressed in the glomerular compartment of the kidney and that IL-22 expression increases in the renal cortex after induction of glomerular injury in a mouse model for crescentic glomerulonephritis (cGN, nephrotoxic nephritis). Read More

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http://dx.doi.org/10.1152/ajprenal.00303.2018DOI Listing
February 2019
2 Reads

The spectrum of kidney biopsy findings in patients with morbid obesity.

Kidney Int 2019 Mar 31;95(3):647-654. Epub 2019 Jan 31.

Department of Pathology and Cell Biology, Division of Renal Pathology, Columbia University Medical Center, New York, New York, USA. Electronic address:

Morbid obesity, defined as body mass index (BMI) ≥40 kg/m, affects approximately 8% of United States adults and is a recognized risk factor for chronic kidney disease (CKD). We present the first focused biopsy-based study exploring the range of kidney diseases in this population. Among 3263 native kidney biopsies interpreted at Columbia University in 2017, we identified 248 biopsies from morbidly obese patients. Read More

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http://dx.doi.org/10.1016/j.kint.2018.11.026DOI Listing
March 2019
1 Read

Mutational spectrum and novel candidate genes in Chinese children with sporadic steroid-resistant nephrotic syndrome.

Pediatr Res 2019 Feb 2. Epub 2019 Feb 2.

Molecular Immunology Laboratory, Capital Institute of Pediatrics, YaBao Road 2, 100020, Beijing, China.

Background: Approximately 10-20% of children with idiopathic nephrotic syndrome (NS) fail to respond to steroid therapy. NS is divided into steroid-sensitive NS (SSNS) and steroid-resistant NS (SRNS). Over 45 recessive and dominant genes have been found to be associated with SRNS and/or focal segmental glomerulosclerosis (FSGS). Read More

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http://dx.doi.org/10.1038/s41390-019-0321-zDOI Listing
February 2019
1 Read

Development of a novel cell-based assay to diagnose recurrent focal segmental glomerulosclerosis patients.

Kidney Int 2019 Mar 29;95(3):708-716. Epub 2019 Jan 29.

Department of Medicine, Nephrology Division, Medical University of South Carolina, Charleston, South Carolina, USA. Electronic address:

Definitive diagnosis of glomerular disease requires a kidney biopsy, an invasive procedure that may not be safe or feasible to perform in all patients. We developed a noninvasive, accurate, and economical diagnostic assay with easy commercial adaptability to detect recurrent focal segmental glomerulosclerosis (rFSGS) after kidney transplant. Since FSGS involves podocyte damage and death, our approach involved mRNA profiling of cultured podocytes treated with plasma from patients with rFSGS to identify upregulated genes involved in podocyte damage. Read More

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http://dx.doi.org/10.1016/j.kint.2018.10.030DOI Listing
March 2019
1 Read

[Advances in research on gene and cell therapy for type IV collagen related hereditary kidney diseases].

Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2019 Feb;36(2):179-182

Ruijin Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200025, China.

Type IV collagen is a component of the extracellular matrix in the basement membrane. Abnormal secretion or assembly of type IV collagen may lead to kidney lesions resulting in numerous nephropathy symptoms, e.g. Read More

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http://dx.doi.org/10.3760/cma.j.issn.1003-9406.2019.02.022DOI Listing
February 2019
1 Read

Nephrological aspects of surgical weight correction in morbid obesity.

Ter Arkh 2018 Jun;90(6):98-104

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Moscow, Russia.

Obesity, including morbid obesity, is a growing worldwide problem. The adverse effect of obesity on the kidneys is associated with the development of comorbid conditions, such as insulin resistance (IR), metabolic syndrome (MS), diabetes mellitus (DM), arterial hypertension (AH), which are the recognized risk factors of chronic kidney disease (СKD). Obesity also causes direct kidney damage with the development of non-immune focal segmental glomerulosclerosis. Read More

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http://ter-arkhiv.ru/en/archive/2018/vol-90-6-2018/15_2731/?
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http://dx.doi.org/10.26442/terarkh201890698-104DOI Listing
June 2018
10 Reads

FAM40A alters the cytoskeleton of podocytes in familial focal and segmental glomerulosclerosis by regulating F-actin and nephrin.

Arch Med Sci 2019 Jan 2;15(1):165-173. Epub 2018 Feb 2.

Division of Nephrology, Shanghai Changzheng Hospital of Second Military Medical University, Kidney Institute of Chinese People's Liberation Army, Shanghai, China.

Introduction: Familial focal and segmental glomerulosclerosis (FFSGS) was found in a large cohort of patients in our previous study. Under the sponsorship of the National Natural Science Foundation of China, we conducted linkage analysis and full exon sequencing on the genomes of 54 patients diagnosed with FFSGS. The results revealed a FAM40A gene signature in those patients. Read More

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http://dx.doi.org/10.5114/aoms.2018.73138DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6348344PMC
January 2019
2 Reads

Kidney Injury by Variants in the Gene Aggravated by Polymorphisms in Slit Diaphragm Genes Causes Focal Segmental Glomerulosclerosis.

Int J Mol Sci 2019 Jan 26;20(3). Epub 2019 Jan 26.

Clinic of Nephrology and Rheumatology, University Medical Center Goettingen, 37075 Goettingen, Germany.

Kidney injury due to focal segmental glomerulosclerosis (FSGS) is the most common primary glomerular disorder causing end-stage renal disease. Homozygous mutations in either glomerular basement membrane or slit diaphragm genes cause early renal failure. Heterozygous carriers develop renal symptoms late, if at all. Read More

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http://dx.doi.org/10.3390/ijms20030519DOI Listing
January 2019
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Identifying a potential biomarker for primary focal segmental glomerulosclerosis and its association with recurrence after transplantation.

Clin Transplant 2019 Jan 28:e13487. Epub 2019 Jan 28.

Kidney Transplant Service, University of California, San Francisco, California.

Background: We investigated circulating levels of individual soluble urokinase plasminogen activation receptor (suPAR) forms to determine if specific circulating fragments of suPAR (II-III) and (I) can better serve as clinical biomarkers for focal segmental glomerulosclerosis (FSGS) and the risk of recurrence after transplantation.

Materials And Methods: Serum levels of intact suPAR and its cleaved forms were measured with two assays, ELISA and TR-FIA.

Results: suPAR levels in healthy controls were significantly lower than those who had glomerular diseases but were not significantly different between FSGS patients and glomerular controls. Read More

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http://dx.doi.org/10.1111/ctr.13487DOI Listing
January 2019
1 Read

Primary coenzyme Q10 Deficiency-6 (COQ10D6): Two siblings with variable expressivity of the renal phenotype.

Eur J Med Genet 2019 Jan 22. Epub 2019 Jan 22.

Istanbul University, Istanbul Faculty of Medicine, Pediatric Nephrology Department, Istanbul, Turkey.

Primary coenzyme Q10 deficiency-6 (COQ10D6) is a rare autosomal recessive disorder caused by COQ6 mutations. The main clinical manifestations are infantile progressive nephrotic syndrome (NS) leading to end-stage renal disease and sensorineural deafness. A 7-year-old girl was diagnosed with steroid-resistant NS (SRNS) and an audiological work-up revealed bilateral sensorineural deafness. Read More

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http://dx.doi.org/10.1016/j.ejmg.2019.01.011DOI Listing
January 2019
2 Reads

A cryptic splicing mutation in the INF2 gene causing Charcot-Marie-Tooth disease with minimal glomerular dysfunction.

J Peripher Nerv Syst 2019 Jan 24. Epub 2019 Jan 24.

Centre de Biologie et de Pathologie Est, Hospices Civils de Lyon, Bron, France.

Heterozygous mutations in the inverted formin-2 (INF2) gene provoke focal segmental glomerulosclerosis (FSGS) and intermediate Charcot-Marie-Tooth (CMT) disease with FSGS. Here, we report four patients from a three-generation family with a new cryptic splicing INF2 mutation causing autosomal dominant intermediate CMT with minimal glomerular dysfunction. Three males and one female with a mean age of 51 years (26-87) presented with a slowly progressive sensorimotor polyneuropathy, pes cavus, and kyphoscoliosis. Read More

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http://doi.wiley.com/10.1111/jns.12308
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http://dx.doi.org/10.1111/jns.12308DOI Listing
January 2019
6 Reads

Upregulated long noncoding RNA LOC105375913 induces tubulointerstitial fibrosis in focal segmental glomerulosclerosis.

Sci Rep 2019 Jan 24;9(1):716. Epub 2019 Jan 24.

National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, 210002, China.

Tubulointerstitial fibrosis impacts renal prognosis of focal segmental glomerulosclerosis (FSGS). Based on transcriptomic analysis, we found that the level of LOC105375913 was increased in tubular cells of FSGS patients. C3a induced the expression of LOC105375913, which promoted the expression of fibronectin and collagen I in tubular cells. Read More

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http://dx.doi.org/10.1038/s41598-018-36902-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6345783PMC
January 2019
2 Reads

Infliximab-associated focal segmental glomerulosclerosis in a patient with ankylosing spondylitis.

Rheumatol Int 2019 Mar 23;39(3):561-567. Epub 2019 Jan 23.

Division of Rheumatology, Department of Internal Medicine, Dokuz Eylul University School of Medicine, Izmir, Turkey.

The introduction of tumor necrosis factor-alpha (TNF-α)-targeting drugs has given new opportunities in the treatment of various inflammatory rheumatic diseases and has been the most important development in the treatment of spondyloarthritis (SpA). However, the increasing use and longer follow-up periods of treatment also pose risks of developing various adverse effects ranging from common ones including infections to uncommon renal complications. This report describes a case of infliximab-induced focal segmental glomerulosclerosis (FSGS) in a 40-year-old female patient with ankylosing spondylitis (AS) who presented with asymptomatic proteinuria and microscopic hematuria. Read More

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http://link.springer.com/10.1007/s00296-019-04241-8
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http://dx.doi.org/10.1007/s00296-019-04241-8DOI Listing
March 2019
5 Reads

Noninvasive diagnosis of primary membranous nephropathy using phospholipase A2 receptor antibodies.

Kidney Int 2019 Feb;95(2):429-438

Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota, USA. Electronic address:

Kidney biopsy is the gold standard to diagnose membranous nephropathy (MN). Approximately 70%-80% of patients with primary MN have anti-phospholipase A2 receptor (PLA2R) antibodies. We hypothesized that PLA2R antibody testing without kidney biopsy may be a valid strategy to make a non-invasive diagnosis of MN in patients with a negative work-up for secondary causes. Read More

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http://dx.doi.org/10.1016/j.kint.2018.10.021DOI Listing
February 2019
1 Read

Knockout of TRPC6 promotes insulin resistance and exacerbates glomerular injury in Akita mice.

Kidney Int 2019 Feb;95(2):321-332

Division of Nephrology, Department of Medicine, Duke University Health System, Durham, North Carolina, USA; Durham VA Medical Center, Durham, North Carolina, USA. Electronic address:

Gain-of-function mutations in TRPC6 cause familial focal segmental glomerulosclerosis, and TRPC6 is upregulated in glomerular diseases including diabetic kidney disease. We studied the effect of systemic TRPC6 knockout in the Akita model of type 1 diabetes. Knockout of TRPC6 inhibited albuminuria in Akita mice at 12 and 16 weeks of age, but this difference disappeared by 20 weeks. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00852538183078
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http://dx.doi.org/10.1016/j.kint.2018.09.026DOI Listing
February 2019
3 Reads

Management and treatment of glomerular diseases (part 2): conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference.

Kidney Int 2019 Feb;95(2):281-295

Division of Nephrology, Rheinisch-Westfälische Technische Hochschule, University of Aachen, Aachen, Germany. Electronic address:

In November 2017, the Kidney Disease: Improving Global Outcomes (KDIGO) initiative brought a diverse panel of experts in glomerular diseases together to discuss the 2012 KDIGO glomerulonephritis guideline in the context of new developments and insights that had occurred over the years since its publication. During this KDIGO Controversies Conference on Glomerular Diseases, the group examined data on disease pathogenesis, biomarkers, and treatments to identify areas of consensus and areas of controversy. This report summarizes the discussions on primary podocytopathies, lupus nephritis, anti-neutrophil cytoplasmic antibody-associated nephritis, complement-mediated kidney diseases, and monoclonal gammopathies of renal significance. Read More

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http://dx.doi.org/10.1016/j.kint.2018.11.008DOI Listing
February 2019
1 Read

Participation of the AngII/TRPC6/NFAT axis in the pathogenesis of podocyte injury in rats with type 2 diabetes.

Mol Med Rep 2019 Mar 17;19(3):2421-2430. Epub 2019 Jan 17.

Department of Special Medicine, School of Medicine, Qingdao University, Qingdao, Shandong 266071, P.R. China.

The canonical transient receptor potential channel 6 ion channel is expressed in podocytes and is an important component of the glomerular slit diaphragm. Focal segmental glomerulosclerosis is closely associated with TRPC6 gene mutations, and TRPC6 mediates podocyte injury induced by high glucose. Angiotensin II (AngII) has been revealed to enhance TRPC6 currents in certain types of cells, including podocytes and ventricular myocytes. Read More

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http://dx.doi.org/10.3892/mmr.2019.9871DOI Listing
March 2019
7 Reads
1.484 Impact Factor

Changes in primary glomerulonephritis in Singapore over four decades
.

Clin Nephrol 2019 Mar;91(3):155-161

This review of 3,289 native kidney biopsies over the past four decades in Singapore documents the changing pattern of biopsy-proven glomerulonephritis (GN)from that of a third world country to that of a developed nation. In the 1st decade, mesangial proliferative GN was the most common form of primary GN, similar to the Asian region. In the 2nd decade, the percentage of mesangial proliferative GN decreased, but membranous GN became more common, as was seen in China and Thailand. Read More

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http://dx.doi.org/10.5414/CN109577DOI Listing
March 2019
8 Reads

TRPC6 inactivation does not affect loss of renal function in nephrotoxic serum glomerulonephritis in rats, but reduces severity of glomerular lesions.

Biochem Biophys Rep 2019 Mar 8;17:139-150. Epub 2019 Jan 8.

Department of Biology and Biochemistry, University of Houston, Houston, TX, USA.

Canonical transient receptor potential-6 (TRPC6) channels have been implicated in a variety of chronic kidney diseases including familial and acquired forms of focal and segmental glomerulosclerosis (FSGS) and renal fibrosis following ureteral obstruction. Here we have examined the role of TRPC6 in progression of inflammation and fibrosis in the nephrotoxic serum (NTS) model of crescentic glomerulonephritis. This was assessed in rats with non-functional TRPC6 channels due to genomic disruption of an essential domain in TRPC6 channels ( rats) and wild-type littermates ( rats). Read More

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http://dx.doi.org/10.1016/j.bbrep.2018.12.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6325086PMC
March 2019
1 Read

TBC1D8B Loss-of-Function Mutations Lead to X-Linked Nephrotic Syndrome via Defective Trafficking Pathways.

Am J Hum Genet 2019 Feb 17;104(2):348-355. Epub 2019 Jan 17.

Laboratory of Hereditary Kidney Diseases, Imagine Institute, INSERM U1163, Paris Descartes University, 75015 Paris, France; Department of Genetics, Reference center for Hereditary Kidney Diseases (MARHEA), Necker Hospital, APHP,75015 Paris, France. Electronic address:

Steroid-resistant nephrotic syndrome (SRNS) is characterized by high-range proteinuria and most often focal and segmental glomerulosclerosis (FSGS). Identification of mutations in genes causing SRNS has improved our understanding of disease mechanisms and highlighted defects in the podocyte, a highly specialized glomerular epithelial cell, as major factors in disease pathogenesis. By exome sequencing, we identified missense mutations in TBC1D8B in two families with an X-linked early-onset SRNS with FSGS. Read More

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http://dx.doi.org/10.1016/j.ajhg.2018.12.016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6369567PMC
February 2019
3 Reads
10.931 Impact Factor

MeSsAGe risk score: tool for renal biopsy decision in steroid-dependent nephrotic syndrome.

Pediatr Res 2019 Jan 15. Epub 2019 Jan 15.

Department of Paediatrics, Yong Loo Lin School of Medicine, National University of Singapore, Level 12 NUHS Tower Block, 1E Kent Ridge Road, Singapore, 119228, Singapore.

Background: A lack of consensus exists as to the timing of kidney biopsy in children with steroid-dependent nephrotic syndrome (SDNS) where minimal change disease (MCD) predominates. This study aimed at examining the applicability of a biomarker-assisted risk score model to select SDNS patients at high risk of focal segmental glomerulosclerosis (FSGS) for biopsy.

Methods: Fifty-five patients with SDNS and biopsy-proven MCD (n = 40) or FSGS (n = 15) were studied. Read More

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http://www.nature.com/articles/s41390-019-0277-z
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http://dx.doi.org/10.1038/s41390-019-0277-zDOI Listing
January 2019
6 Reads
2.314 Impact Factor

Utility of Columbia classification in focal segmental glomerulosclerosis: renal prognosis and treatment response among the pathological variants.

Nephrol Dial Transplant 2019 Jan 11. Epub 2019 Jan 11.

Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

Background: The utility of the Columbia classification (Col-class) for focal segmental glomerulosclerosis (FSGS) has not yet been fully proven.

Methods: We extracted 201 FSGS patients from 10 nephrology centers in Japan and investigated the difference of a composite renal endpoint, defined as doubling of serum creatinine and/or development of end-stage renal disease, in pathological variants. Sensitivity analysis was used to prove the utility of the Col-class to predict renal outcomes. Read More

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http://dx.doi.org/10.1093/ndt/gfy374DOI Listing
January 2019
1 Read

An Unusual Association of Renal Cell Carcinoma and Renal Malakoplakia with Focal Segmental Glomerulosclerosis in an Elderly Patient.

Indian J Nephrol 2018 Nov-Dec;28(6):485-487

Department of Nephrology Madras Medical Mission Hospital, Chennai, Tamil Nadu, India.

The association of malignancy and glomerulonephritis may be missed, especially in elderly patients. Here, we report a case of eosinophilic variant of renal cell carcinoma and renal parenchymal malakoplakia discovered on renal biopsy in a patient with steroid-dependent nephrotic syndrome. The presence of malakoplakia in our biopsy was probably due to systemic steroid therapy for glomerulonephritis, presence of concomitant asymptomatic urinary tract infection, and/or history of diabetes mellitus. Read More

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http://www.indianjnephrol.org/text.asp?2018/28/6/485/236568
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http://dx.doi.org/10.4103/ijn.IJN_289_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6309395PMC
January 2019
4 Reads

microRNAs in chronic kidney disease.

Clin Chim Acta 2019 Apr 11;491:59-65. Epub 2019 Jan 11.

Department of Nephrology, Baoji Central Hospital, No. 8 Jiangtan Road, Baoji, Shaanxi 721008, China. Electronic address:

Chronic kidney disease (CKD) results in high morbidity and mortality worldwide causing a huge socioeconomic burden. MicroRNA (miRNA) exert critical regulatory functions by targeting downstream genes and have been associated with many pathophysiologic processes including CKD. In fact, many studies have shown that the expression of various miRNAs was significantly changed in CKD. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00098981193002
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http://dx.doi.org/10.1016/j.cca.2019.01.008DOI Listing
April 2019
5 Reads

Successful management of post-transplant focal segmental glomerulosclerosis with therapeutic plasma exchange and rituximab.

Clin Exp Nephrol 2019 Jan 14. Epub 2019 Jan 14.

Imperial College Kidney and Transplant Centre, London, UK.

Background: Post-transplant focal segmental glomerulosclerosis (FSGS) is associated with renal allograft loss. Currently, optimal treatment remains controversial.

Methods: The aim of our study was to examine the efficacy and safety of therapeutic plasma exchange (TPE), and rituximab (RTX), in the management of post-transplant FSGS. Read More

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http://link.springer.com/10.1007/s10157-019-01690-0
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http://dx.doi.org/10.1007/s10157-019-01690-0DOI Listing
January 2019
8 Reads

One Actor, Many Roles: Histopathologies Associated With APOL1 Genetic Variants.

Adv Anat Pathol 2019 Jan 7. Epub 2019 Jan 7.

Kidney Diseases Branch, NIDDK, NIH, Bethesda.

Genetic variants in APOL1, encoding apolipoprotein L1, are major drivers of glomerular disease in peoples of sub-Saharan African descent. APOL1-associated primary glomerular diseases include focal segmental glomerulosclerosis, human immunodeficiency virus-associated nephropathies, and arterionephrosclerosis. Other conditions where APOL1 variants affect outcomes include membranous nephropathy, lupus nephritis, diabetic nephropathy, preeclampsia, and kidney transplant. Read More

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http://dx.doi.org/10.1097/PAP.0000000000000221DOI Listing
January 2019
3 Reads

Mesangial C4d deposition is independently associated with poor renal survival in patients with primary focal segmental glomerulosclerosis.

Clin Exp Nephrol 2019 Jan 7. Epub 2019 Jan 7.

Department of Pathology, Dokuz Eylul University, Izmir, Turkey.

Background: C4d deposition is defined as the footprint of immune injury and it is associated with unfavorable renal outcomes in patients with IgA nephropathy. We searched whether mesangial C4d deposition is associated with poor renal survival in patients with primary focal segmental glomerulosclerosis (FSGS).

Methods: Biopsy specimens were stained with anti-C4d antibody. Read More

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http://dx.doi.org/10.1007/s10157-018-01688-0DOI Listing
January 2019
1 Read
1.708 Impact Factor

Recessive mutation in CD2AP causes focal segmental glomerulosclerosis in humans and mice.

Kidney Int 2019 Jan 26;95(1):57-61. Epub 2018 Oct 26.

Research Institute, McGill University Health Centre, Montreal, Quebec, Canada; Department of Pediatrics, Montreal Children's Hospital, Montreal, Quebec, Canada. Electronic address:

Although sequence variants in CD2-associated protein (CD2AP) have been identified in patients with focal segmental glomerulosclerosis (FSGS), definitive proof of causality in human disease is meager. By whole-exome sequencing, we identified a homozygous frame-shift mutation in CD2AP (p.S198fs) in three siblings born of consanguineous parents who developed childhood-onset FSGS and end stage renal disease. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00852538183059
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http://dx.doi.org/10.1016/j.kint.2018.08.014DOI Listing
January 2019
7 Reads

C4d in Native Glomerular Diseases.

Authors:
Preeti Chandra

Am J Nephrol 2019 4;49(1):81-92. Epub 2019 Jan 4.

Complement activation occurs in many glomerular diseases, the exact pathway(s) of activation has been studied in detail in some diseases but not in all. C4d is generated by the activation of classical and lectin pathways, and its presence can point to the activation of either of these pathways. This review aims to summarize the available data with regard to the deposition of glomerular C4d in native kidney biopsies in different glomerular pathologies that may be useful for future research into the role of complement activation in glomerular diseases. Read More

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http://dx.doi.org/10.1159/000496059DOI Listing
January 2019
2 Reads

C-C chemokine receptor type 2 mediates glomerular injury and interstitial fibrosis in focal segmental glomerulosclerosis.

Nephrol Dial Transplant 2018 Dec 28. Epub 2018 Dec 28.

Nephrologisches Zentrum, Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, Ludwig-Maximilians-Universität München, Munich, Germany.

Background: Glomerulosclerosis and tubulointerstitial fibrosis are hallmarks of chronic kidney injury leading to end-stage renal disease. Inflammatory mechanisms contribute to glomerular and interstitial scarring, including chemokine-mediated recruitment of leucocytes. In particular, accumulation of C-C chemokine receptor type 2 (CCR2)-expressing macrophages promotes renal injury and fibrotic remodelling in diseases like glomerulonephritis and diabetic nephropathy. Read More

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http://dx.doi.org/10.1093/ndt/gfy380DOI Listing
December 2018
1 Read

Calcineurin Inhibitors With Reduced-Dose Steroids as First-Line Therapy for Focal Segmental Glomerulosclerosis.

Kidney Int Rep 2019 Jan 31;4(1):40-47. Epub 2018 Aug 31.

Department of Nephrology and Mineral Metabolism, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

Introduction: High-dose corticosteroids remain the first-line therapy for focal and segmental glomerulosclerosis (FSGS), whereas calcineurin inhibitors (CNIs) are reserved for those patients resistant to corticosteroid therapy.

Methods: This is a retrospective cohort analysis in patients with primary FSGS diagnosed between 2007 and 2014. According to the administered treatment, patients were segregated into 3 groups: high-dose prednisone, first-line CNIs plus low-dose prednisone, and rescue CNIs. Read More

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http://dx.doi.org/10.1016/j.ekir.2018.08.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6308907PMC
January 2019
1 Read

TRPC6 Mutational Analysis in Iranian Children With Focal Segmental Glomerulosclerosis.

Iran J Kidney Dis 2018 Nov;12(6):341-349

Isfahan Kidney Diseases Research Center, Isfahan University of Medical Sciences, Isfahan, Iran.

Introduction: Focal segmental glomerulosclerosis (FSGS) ranks among nephrotic syndromes. Research shows that FSGS is brought about by several genes including transient receptor potential cation channel subfamily c member 6 (TRPC6). This study aimed to investigate TRPC6 gene in Iranian FSGS children. Read More

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November 2018
1 Read
0.979 Impact Factor

Genetic studies of focal segmental glomerulosclerosis: a waste of scientific time?

Pediatr Nephrol 2018 Dec 27. Epub 2018 Dec 27.

Department of Pathology, Department of Cellular Pathology, Royal Free Hospital, University College London, London, NW3 2QG, UK.

Many genetic causes of focal segmental glomerulosclerosis (FSGS) have been described. A paradox is that the science in the molecular biology, which generally appears of high quality, is not mirrored by a similarly critical analysis of the renal pathology. FSGS has been applied to such a wide range of conditions that it can reasonably be said to have no useful meaning. Read More

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http://dx.doi.org/10.1007/s00467-018-4161-6DOI Listing
December 2018
1 Read

Flt3 inhibition alleviates chronic kidney disease by suppressing CD103+ dendritic cell-mediated T cell activation.

Nephrol Dial Transplant 2018 Dec 26. Epub 2018 Dec 26.

Centre for Transplant and Renal Research, Westmead Institute for Medical Research, The University of Sydney, Sydney, NSW, Australia.

Background: Chronic kidney disease (CKD) is a global public health problem, which lacks effective treatment. Previously, we have shown that CD103+ dendritic cells (DCs) are pathogenic in adriamycin nephropathy (AN), a model of human focal segmental glomerulosclerosis (FSGS). Fms-like tyrosine kinase 3 (Flt3) is a receptor that is expressed with high specificity on tissue resident CD103+ DCs. Read More

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https://academic.oup.com/ndt/advance-article/doi/10.1093/ndt
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http://dx.doi.org/10.1093/ndt/gfy385DOI Listing
December 2018
7 Reads
3.577 Impact Factor

Pattern of glomerular disease and clinicopathological correlation: A single-center study from Eastern Nepal.

Saudi J Kidney Dis Transpl 2018 Nov-Dec;29(6):1410-1416

Department of Internal Medicine, B.P. Koirala Institute of Health Sciences, Dharan, Nepal.

The pattern of glomerular disease varies worldwide. In the absence of kidney disease/kidney biopsy registry in Nepal, the exact etiology of different forms of glomerular disease is primarily unknown in our country. We analyzed 175 cases of renal biopsies performed from September 2014 to August 2016 in Internal Medicine Ward at B. Read More

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http://www.sjkdt.org/text.asp?2018/29/6/1410/248302
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http://dx.doi.org/10.4103/1319-2442.248302DOI Listing
December 2018
7 Reads

Clinicopathological profile of pediatric renal biopsies at a tertiary care hospital, Pakistan.

Saudi J Kidney Dis Transpl 2018 Nov-Dec;29(6):1403-1409

The Kidney Center, Karachi, Pakistan.

Renal biopsy is an important tool for the diagnosis of acute and chronic glomerular diseases in children. We aimed to analyze the spectrum of clinical indications and histopathological patterns (HPP) in children who underwent renal biopsy (RB). This is a retrospective review of case records of 108 renal biopsies carried out from January 2010 to December 2015 at the Pediatric Nephrology Department, National Institute of Child Health Karachi, Pakistan. Read More

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http://www.sjkdt.org/text.asp?2018/29/6/1403/248290
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http://dx.doi.org/10.4103/1319-2442.248290DOI Listing
December 2018
13 Reads

A single-center 4-year experience with 47 pediatric renal transplants: Evolving trends.

Saudi J Kidney Dis Transpl 2018 Nov-Dec;29(6):1303-1310

Department of Surgery, Section of Transplantation, Renal Transplant Unit, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia.

Outcome of pediatric kidney transplantation (KT) has improved over the last several decades. We retrospectively reviewed the outcomes pediatric KT in King Faisal Specialist Hospital and Research Center-Jeddah, Saudi Arabia. Between May 2013 and November 2016, we performed renal transplantation in 47 children, 30 (64%) males, and 17 (36%) females. Read More

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http://www.sjkdt.org/text.asp?2018/29/6/1303/248297
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http://dx.doi.org/10.4103/1319-2442.248297DOI Listing
December 2018
7 Reads

Spectrum of glomerular diseases in Arab countries: A systematic review.

Saudi J Kidney Dis Transpl 2018 Nov-Dec;29(6):1256-1266

Department of Allied Health Sciences, College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, Sultanate of Oman.

According to the best of our knowledge, there is no review compiling incidence of glomerular disease in all Arab countries. Most of the Arab countries do not have a national renal biopsy registry. In addition, there is scanty data available on the epidemiology of glomerular diseases in Arab countries. Read More

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http://www.sjkdt.org/text.asp?2018/29/6/1256/248285
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http://dx.doi.org/10.4103/1319-2442.248285DOI Listing
December 2018
8 Reads

Persistent CD-19 Depletion by Rituximab is Cost-effective in Maintaining Remission in Calcineurin-inhibitor Dependent Podocytopathy.

Nephrology (Carlton) 2018 Dec 26. Epub 2018 Dec 26.

Department of Nephrology, PGIMER, Chandigarh.

Aim: A significant proportion of patients with minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are either steroid dependent (SD) or resistant (SR), requiring long-term calcineurin inhibitors (CNIs) use. Rituximab has more favourable safety profile. The present study was undertaken to evaluate the efficacy and safety of rituximab in CNI dependent patients. Read More

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http://dx.doi.org/10.1111/nep.13554DOI Listing
December 2018
1 Read