7,013 results match your criteria Glomerulosclerosis Focal Segmental


C1q nephropathy in adults is a form of focal segmental glomerulosclerosis in terms of clinical characteristics.

PLoS One 2019 19;14(4):e0215217. Epub 2019 Apr 19.

Korean GlomeuloNephritis Study Group, Korean Society of Nephrology, Seoul, Republic of Korea.

Although C1q nephropathy (C1qN) was introduced three decades ago, the clinical significance and renal outcomes of C1qN remain unclear. This study aimed to evaluate the clinical characteristics of C1qN, including renal outcomes, by performing a matched comparison within a multicenter cohort. We enrolled 6,413 adult patients who underwent kidney biopsy between January 2000 and January 2018 at three tertiary hospitals in Korea. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0215217PLOS
April 2019
1 Read

Dominant PAX2 mutations may cause steroid-resistant nephrotic syndrome and FSGS in children.

Pediatr Nephrol 2019 Apr 17. Epub 2019 Apr 17.

Division of Nephrology, Department of Medicine, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA, USA.

Background: Heterozygous PAX2 mutations cause renal coloboma syndrome (RCS) [OMIM no. 120330]. RCS is a renal syndromic disease encompassing retinal coloboma and sensorineural hearing loss. Read More

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http://link.springer.com/10.1007/s00467-019-04256-0
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http://dx.doi.org/10.1007/s00467-019-04256-0DOI Listing
April 2019
1 Read

CRISPR-Cas9-Mediated Correction of the G189R-PAX2 Mutation in Induced Pluripotent Stem Cells from a Patient with Focal Segmental Glomerulosclerosis.

CRISPR J 2019 Apr;2:108-120

1 Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Centro Anna Maria Astori, Science and Technology Park Kilometro Rosso, Bergamo, Italy; University of Milan, Milan, Italy.

Focal segmental glomerulosclerosis (FSGS) is defined by focal (involving few glomeruli) and segmental sclerosis of the glomerular tuft that manifests with nephrotic syndrome. Mutations in genes involved in the maintenance of structure and function of podocytes have been found in a minority of these patients. A family with adult-onset autosomal dominant FSGS was recently found to carry a new germline missense heterozygous mutation (p. Read More

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http://dx.doi.org/10.1089/crispr.2018.0048DOI Listing
April 2019
1 Read

Survival Advantage of African American Dialysis Patients with End-Stage Renal Disease Causes Related to APOL1.

Cardiorenal Med 2019 Apr 17;9(4):212-221. Epub 2019 Apr 17.

Kidney Disease Branch, NIDDK, Bethesda, Maryland, USA,

Background: Observational studies show that African American (AA) dialysis patients have longer survival than European Americans. We hypothesized that apolipoprotein L1 (APOL1) genetic variation, associated with nephropathy in AAs, contributes to the survival advantage in AA dialysis patients.

Methods: We examined the association between race and mortality among 37,097 adult dialysis patients, including 54% AAs and 46% European Americans from a large dialysis organization (entry period from July 2001 to June 2006, follow-up through June 2007), within each cause of end-stage renal disease (ESRD) category associated with APOL1 renal risk variants using Cox proportional hazard models. Read More

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https://www.karger.com/Article/FullText/496472
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http://dx.doi.org/10.1159/000496472DOI Listing
April 2019
7 Reads

Effect of LDL apheresis for nephrotic idiopathic membranous nephropathy as initial induction therapy.

Ther Apher Dial 2019 Apr 17. Epub 2019 Apr 17.

Department of Nephrology, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Aichi, Japan.

Low-density lipoprotein apheresis (LDL-A) has been used for nephrotic syndrome (NS) by focal segmental glomerulosclerosis in Japan. Idiopathic membranous nephropathy (iMN) can also cause treatment-resistant NS. Therefore we investigated the effect of LDL-A during initial induction for it. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/1744-9987.12
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http://dx.doi.org/10.1111/1744-9987.12811DOI Listing
April 2019
2 Reads

Nephrotic Syndrome in South African Children: Changing Perspectives in the New Millennium.

Kidney Int Rep 2019 Apr 12;4(4):522-534. Epub 2019 Feb 12.

Department of Paediatrics and Child Health, College of Health Sciences, Nelson R Mandela School of Medicine, University of KwaZulu-Natal, Durban, South Africa.

The epidemiological landscape of nephrotic syndrome (NS) in South Africa has changed drastically in the New Millennium. Although the pattern of disease in the 3 main non-Black racial groups (White, Indian, and Mixed race) mirror that seen in Western countries, Black African children show a pattern of disease that is at variance with these 3 racial groups. The incidence of infectious diseases, particularly hepatitis B virus associated nephropathy has sharply declined to being almost extinct in Black children in the New Millennium whereas HIV-related nephropathy surfaced. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S24680249193004
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http://dx.doi.org/10.1016/j.ekir.2019.01.019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6451079PMC
April 2019
2 Reads

C1q Nephropathy in a Patient of Neurofibromatosis Type 1: A Rare Case Report.

Indian J Nephrol 2019 Mar-Apr;29(2):125-127

Department of Nephrology and Transplantation Medicine, G.R. Doshi and K.M. Mehta Institute of Kidney Diseases and Research Centre - Dr. H.L. Trivedi Institute of Transplantation Sciences, Ahmedabad, Gujarat, India.

C1q nephropathy is a rare glomerular disease defined by the presence of characteristic mesangial dominant or codominant C1q deposition on immunofluorescence microscopy. Neurofibromatosis type 1 (NF-1) is an autosomal dominant syndrome caused by a mutation of a gene located on chromosomal segment 17q11.2. Read More

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http://www.indianjnephrol.org/preprintarticle.asp?id=252208
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http://dx.doi.org/10.4103/ijn.IJN_353_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6440327PMC
April 2019
2 Reads

Loss of Subpodocytic Space Predicts Poor Response to Tacrolimus in Steroid-Resistant Calcineurin Inhibitor-Naïve Adult-Onset Primary Focal Segmental Glomerulosclerosis.

Indian J Nephrol 2019 Mar-Apr;29(2):90-94

Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Focal segmental glomerulosclerosis (FSGS) is the most common cause of adult-onset nephrotic syndrome, but its pathophysiology is poorly understood. The question as to why only a subset of patients responds to treatment in unanswered. In the past few years, change of podocytic phenotype from stationary type in health to migratory type in disease has been described, of which loss of subpodocytic space is a surrogate marker. Read More

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http://dx.doi.org/10.4103/ijn.IJN_422_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6440328PMC
April 2019
1 Read

APOL1 risk genotype in Europe: Data in patients with focal segmental glomerulosclerosis and after renal transplantation.

Nephrol Ther 2019 Apr;15 Suppl 1:S85-S89

Inserm U1163, 24, boulevard du Montparnasse, 75015 Paris, France; Institut Imagine, 24, boulevard du Montparnasse, 75015 Paris, France; Université Paris Descartes, 24, boulevard du Montparnasse, 75015 Paris, France; Genetic Department, hôpital Necker-Enfants-malades, AP-HP, 149, rue de Sèvres, 75015 Paris, France.

Apolipoprotein L1 (APOL1) risk variants are strongly associated with sporadic focal segmental glomerulosclerosis in populations with African ancestry. We determined the frequency of G1/G2 variants in patients with steroid-resistant nephrotic syndrome/focal segmental glomerulosclerosis with African or French West Indies origin in France and its relationships with other steroid-resistant nephrotic syndrome genes. In a cohort of 152 patients (139 families), the APOL1 risk variants were genotyped: the two risk allele (high risk) genotype was found in 43. Read More

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http://dx.doi.org/10.1016/j.nephro.2019.02.005DOI Listing
April 2019
1 Read

Continuous B-cell depletion in frequently relapsing, steroid-dependent and steroid-resistant nephrotic syndrome.

Clin Kidney J 2019 Apr 24;12(2):224-231. Epub 2018 Jul 24.

Vasculitis and Glomerulonephritis Center, Division of Nephrology, Massachusetts General Hospital, Boston, MA, USA.

Background: Patients with frequently relapsing (FR), steroid-dependent (SD) and steroid-resistant (SR) nephrotic syndrome are a therapeutic challenge with limited treatment options. Here, we retrospectively analyze the efficacy and safety of rituximab-induced continuous B-cell depletion in these populations.

Methods: Patients were included if they were at least 18 years of age and had FR, SD or SR minimal change disease (MCD) or primary focal segmental glomerulosclerosis (FSGS) and were treated with a strategy of continuous B-cell depletion. Read More

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http://dx.doi.org/10.1093/ckj/sfy067DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6452201PMC
April 2019
1 Read

Ofatumumab in post-transplantation recurrence of focal segmental glomerulosclerosis in a child.

Pediatr Transplant 2019 Apr 11:e13413. Epub 2019 Apr 11.

Children's Hospital at Montefiore, Bronx, New York.

FSGS is a potentially devastating form of nephrotic syndrome. Treatment of SRNS can be difficult, especially post-transplantation. The current therapy of post-transplant SRNS includes plasmapheresis, ACE-I, CNI, and monoclonal antibodies (rituximab). Read More

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http://dx.doi.org/10.1111/petr.13413DOI Listing
April 2019
1 Read

Renal tubular P-glycoprotein expression is reduced in plasma cell disorders.

Kidney Res Clin Pract 2019 Apr 8. Epub 2019 Apr 8.

Division of Nephrology, Department of Internal Medicine, Dokuz Eylül University, Izmir, Turkey.

Background: : P-glycoprotein (P-gp) transports many chemicals that vary greatly in their structure and function. It is normally expressed in renal proximal tubular cells. We hypothesized that P-gp expression influences light chain excretion. Read More

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http://dx.doi.org/10.23876/j.krcp.18.0134DOI Listing
April 2019
1 Read

Open-Label Clinical Trials of Oral Pulse Dexamethasone for Adults with Idiopathic Nephrotic Syndrome.

Am J Nephrol 2019 Apr 9;49(5):377-385. Epub 2019 Apr 9.

Kidney Disease Section, National Institute of Diabetes and Digestive and Kidney Disease, Bethesda, Maryland, USA,

Background: In adults with primary focal segmental glomerulosclerosis (FSGS), daily prednisone may induce complete remissions (CR) and partial remissions (PR), but relapses are frequent and adverse events are common.

Methods: We carried out 2 open-label, uncontrolled trials to explore the efficacy and tolerability of pulse oral dexamethasone as an alternative to daily prednisone. We enrolled adult patients with proteinuria > 3. Read More

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http://dx.doi.org/10.1159/000497064DOI Listing
April 2019
1 Read

A Case of Rheumatoid Arthritis Presenting with Renal Thrombotic Microangiopathy Probably due to a Combination of Chronic Tacrolimus Arteriolopathy and Severe Hypertension.

Case Rep Nephrol 2019 6;2019:3923190. Epub 2019 Mar 6.

Department of Internal Medicine, Teikyo University School of Medicine, Itabashi-ku, Tokyo, Japan.

A 51-year-old woman with rheumatoid arthritis presented with mild hypertension 20 months after tacrolimus treatment and developing proteinuria 24 months after the treatment. Tacrolimus was discontinued 27 months after the treatment, followed by heavy proteinuria, accelerated hypertension, and deteriorating renal function without ocular fundus lesions as a clinical sign of malignant hypertension. Renal biopsy revealed malignant nephrosclerosis characterized by subacute and chronic thrombotic microangiopathy (TMA), involving small arteries, arterioles, and glomeruli. Read More

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http://dx.doi.org/10.1155/2019/3923190DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6431373PMC
March 2019
1 Read

A complex containing lysine-acetylated actin inhibits the formin INF2.

Nat Cell Biol 2019 Apr 8. Epub 2019 Apr 8.

Department of Biochemistry and Cell Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, USA.

Inverted formin 2 (INF2) is a member of the formin family of actin assembly factors. Dominant missense mutations in INF2 are linked to two diseases: focal segmental glomerulosclerosis, a kidney disease, and Charcot-Marie-Tooth disease, a neuropathy. All of the disease mutations map to the autoinhibitory diaphanous inhibitory domain. Read More

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http://dx.doi.org/10.1038/s41556-019-0307-4DOI Listing

Genetics of Nephrotic Syndrome Presenting in Childhood: Core Curriculum 2019.

Am J Kidney Dis 2019 Apr 4. Epub 2019 Apr 4.

Department of Pediatrics-Nephrology, 4 Center for Computational Medicine and Bioinformatics, University of Michigan School of Medicine, Ann Arbor, MI. Electronic address:

Nephrotic syndrome (NS) is one of the most challenging conditions to manage and treat, partly because we lack a specific molecular understanding of its pathogenesis and progression. This limits our ability to provide targeted therapy or precise prognostications. Fortunately, genomic discovery in NS and its translation to genomic-informed medicine is allowing us to improve our understanding of the molecular anatomy of NS and our ability to care for patients with NS. Read More

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http://dx.doi.org/10.1053/j.ajkd.2019.01.033DOI Listing
April 2019
4 Reads

Urine exosomal ceruloplasmin: a potential early biomarker of underlying kidney disease.

Clin Exp Nephrol 2019 Apr 6. Epub 2019 Apr 6.

Division of Nephrology, John H. Stroger, Jr. Hospital of Cook County, 1950 West Polk Street, Suites 5-56, Professional Building, Chicago, IL, 60612, USA.

Background: Previously we found that kidney tissue and urinary exosomes from patients of diabetic kidney disease showed high levels of ceruloplasmin (CP). Because CP is an acute-phase protein of kidney origin, it could be an early marker of many other kidney diseases. To investigate this hypothesis, we first measured urine exosomal and kidney expression of CP in non-diabetic chronic kidney disease (CKD) patients (membranous nephropathy, focal segmental glomerulosclerosis, lupus nephritis and IgA nephropathy) followed by a longitudinal study in rat passive Heymann nephritis (PHN), a model of human membranous nephropathy. Read More

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http://dx.doi.org/10.1007/s10157-019-01734-5DOI Listing
April 2019
2 Reads

Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study.

Pediatr Nephrol 2019 Apr 3. Epub 2019 Apr 3.

Department of Pediatrics, Division of Nephrology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, USA.

Background: Renal damage is a progressive complication of sickle cell disease (SCD). Microalbuminuria is common in children with SCD, while a smaller number of children have more severe renal manifestations necessitating kidney biopsy. There is limited information on renal biopsy findings in children with SCD and subsequent management and outcome. Read More

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http://dx.doi.org/10.1007/s00467-019-04237-3DOI Listing
April 2019
2 Reads

C3a and suPAR drive versican V1 expression in tubular cells of focal segmental glomerulosclerosis.

JCI Insight 2019 Apr 4;4(7). Epub 2019 Apr 4.

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

Chronic tubulointerstitial injury impacts the prognosis of focal segmental glomerulosclerosis (FSGS). We found that the level of versican V1 was increased in tubular cells of FSGS patients. Tubular cell-derived versican V1 induced proliferation and collagen synthesis by activating the CD44/Smad3 pathway in fibroblasts. Read More

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http://dx.doi.org/10.1172/jci.insight.122912DOI Listing
April 2019
5 Reads

Contribution of Electron Microscopy to the Clinicopathologic Diagnosis in Childhood Glomerular Renal Diseases.

Fetal Pediatr Pathol 2019 Apr 3:1-8. Epub 2019 Apr 3.

a Department of Pediatrics , Division of Nephrology, Dokuz Eylul Universitesi Tip Fakultesi , Izmir 35210 , Turkey.

Background: Electron microscopy (EM) provides another diagnostic assessment of glomerular lesions in addition to light and fluorescent microscopy.

Objectives: We evaluated the contribution of diagnostic EM in childhood glomerular diseases.

Patients And Methods: Forty-eight renal biopsies which were assessed by EM between 2000 and 2014 were evaluated. Read More

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http://dx.doi.org/10.1080/15513815.2019.1587119DOI Listing
April 2019
5 Reads
0.398 Impact Factor

c-Src is in the effector pathway linking uPAR and podocyte injury.

J Clin Invest 2019 Apr 2;130. Epub 2019 Apr 2.

The role of urokinase-type plasminogen activator receptor (uPAR) in kidney physiology and pathology has attracted considerable attention. The protein uPAR has dual functions: as a key regulator of plasmin generation and a component of the innate immune system. In the current issue, Wei and colleagues describe a transgenic mouse expressing Plaur RNA in glomerular podocytes. Read More

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https://www.jci.org/articles/view/127927
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http://dx.doi.org/10.1172/JCI127927DOI Listing
April 2019
5 Reads

[Clinical features and MYH9 gene variant in two Chinese siblings with Fechtner syndrome].

Zhonghua Er Ke Za Zhi 2019 Apr;57(4):286-290

Department of Nephrology, Children's Hospital of Nanjing Medical University, Nanjing 210008, China.

To summarize the clinical data and molecular characteristics of two siblings with Fechtner syndrome. A retrospective analysis was made on the clinical data, laboratory tests and genetic test results of two siblings with Fechtner syndrome in a family who were followed up in the Department of Nephrology, Children's Hospital Affiliated to Nanjing Medical University from April 2018 to August 2018. Both siblings showed proteinuria, microscopic hematuria and thrombocytopenia. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2019.04.011DOI Listing
April 2019
4 Reads

A systems pharmacology workflow with experimental validation to assess the potential of anakinra for treatment of focal and segmental glomerulosclerosis.

PLoS One 2019 28;14(3):e0214332. Epub 2019 Mar 28.

Epsilon 3 Gmbh, Vienna, Austria.

Focal and Segmental Glomerulosclerosis (FSGS) is a severe glomerulopathy that frequently leads to end stage renal disease. Only a subset of patients responds to current therapies, making it important to identify alternative therapeutic options. The interleukin (IL)-1 receptor antagonist anakinra is beneficial in several diseases with renal involvement. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0214332PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6438574PMC
March 2019
1 Read

Cardiovascular disease risk among children with focal segmental glomerulosclerosis: a report from the chronic kidney disease in children study.

Pediatr Nephrol 2019 Mar 22. Epub 2019 Mar 22.

Division of Pediatric Nephrology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Background: The aims were to compare the cardiovascular disease (CVD) risk among children with chronic kidney disease (CKD) secondary to focal segmental glomerulosclerosis (FSGS) with the CVD risk of children with CKD due to other diagnoses.

Methods: Casual blood pressure (BP), ambulatory blood pressure monitoring (APBM), echocardiogram, lipids, carotid intima medial thickness (cIMT), and uric acid obtained from participants in the Chronic Kidney Disease in Children (CKiD) cohort were analyzed longitudinally. Seventy-nine children with FSGS (FSGS-CKD) were compared to 196 children with non-FSGS glomerular disease (GDO-CKD) and 616 children with non-glomerular disease (NG-CKD). Read More

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http://dx.doi.org/10.1007/s00467-019-04229-3DOI Listing
March 2019
1 Read

Analysis of the genomic architecture of a complex trait locus in hypertensive rat models links to kidney damage.

Elife 2019 Mar 22;8. Epub 2019 Mar 22.

Institute of Clinical Pharmacology and Toxicology, Charité - Universitätsmedizin Berlin, Berlin, Germany.

Unraveling the genetic susceptibility of complex diseases such as chronic kidney disease remains challenging. Here, we used inbred rat models of kidney damage associated with elevated blood pressure for the comprehensive analysis of a major albuminuria susceptibility locus detected in these models. We characterized its genomic architecture by congenic substitution mapping, targeted next generation sequencing, and compartment-specific RNA sequencing analysis in isolated glomeruli. Read More

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https://elifesciences.org/articles/42068
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http://dx.doi.org/10.7554/eLife.42068DOI Listing
March 2019
3 Reads
8.519 Impact Factor

Health-related quality of life in glomerular disease.

Kidney Int 2019 Feb 27. Epub 2019 Feb 27.

Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA.

There is scant literature describing the effect of glomerular disease on health-related quality of life (HRQOL). The Cure Glomerulonephropathy study (CureGN) is an international longitudinal cohort study of children and adults with four primary glomerular diseases (minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, and IgA nephropathy). HRQOL is systematically assessed using items from the Patient-Reported Outcomes Measurement Informative System (PROMIS). Read More

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http://dx.doi.org/10.1016/j.kint.2018.12.018DOI Listing
February 2019
3 Reads

Clinical features and outcomes of kidney transplant recipients with focal segmental glomerulosclerosis recurrence (FSGSr).

Nephrology (Carlton) 2019 Mar 19. Epub 2019 Mar 19.

Nephrology Division, Hospital do Rim, Universidade Federal de Sao Paulo, Sao Paulo, Brazil.

Aim: FSGS recurs in up to 30% and up to 80% of adult and pediatric kidney transplant recipients, respectively. There is no standard of care treatment. The purpose of this study was to evaluate clinical characteristics, treatments and outcomes of patients with FSGSr. Read More

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http://dx.doi.org/10.1111/nep.13589DOI Listing
March 2019
4 Reads

Signal regulatory protein α protects podocytes through promoting autophagic activity.

JCI Insight 2019 Mar 19;5. Epub 2019 Mar 19.

High autophagic activity in podocytes, terminally differentiated cells which serve as main components of the kidney filtration barrier, is essential for podocyte survival under various challenges. How podocytes maintain such a high level of autophagy, however, remains unclear. Here we report that signal regulatory protein α (SIRPα) plays a key role in promoting podocyte autophagy. Read More

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http://dx.doi.org/10.1172/jci.insight.124747DOI Listing
March 2019
1 Read

[Minimal change disease and focal segmental glomerulosclerosis].

Internist (Berl) 2019 Mar 18. Epub 2019 Mar 18.

Medizinische Klinik IV, Friedrich-Alexander-Universität Erlangen-Nürnberg, Ulmenweg 18, 91054, Erlangen, Deutschland.

Minimal change disease (MCD) or minimal change glomerulonephritis and focal segmental glomerulosclerosis (FSGS) are the two major causes of nephrotic syndrome in children and young adults. Both disease entities resemble each other and can sometimes only be discriminated on the basis of their clinical courses. MCD and FSGS display two classical examples that share a common pathophysiology in which the glomerular podocyte and the cytoskeleton of its foot processes play important roles. Read More

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http://dx.doi.org/10.1007/s00108-019-0590-yDOI Listing
March 2019
1 Read

Rituximab as a Preemptive Treatment to Prevent Recurrence of Primary Focal Segmental Glomerulosclerosis: A Novel Approach.

Exp Clin Transplant 2019 Mar 14. Epub 2019 Mar 14.

From the Department of Internal Medicine and Nephrology, Cairo University, Cairo, Egypt.

Objectives: Primary idiopathic focal segmental glomerulo-sclerosis is a serious disease, frequently progressing to end-stage kidney failure. Management of recurrence after kidney transplant is challenging despite multiple proposed therapeutic approaches. Available treatment for focal segmental glomeru-losclerosis recurrence is plasma exchange, intravenous cyclosporine, and rituximab. Read More

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http://dx.doi.org/10.6002/ect.2018.0027DOI Listing
March 2019
1 Read

Successful Treatment of Recurrent Focal Segmental Glomerulosclerosis After Transplantation in Children: A Single-Center Experience.

Transplant Proc 2019 Mar 4;51(2):517-521. Epub 2019 Jan 4.

Organ Transplant Center, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia.

Objective: We aim to report our experience managing cases of recurrent focal segmental glomerulosclerosis (FSGS) in a group of pediatric renal transplant recipients.

Methods: This study was a retrospective chart review of pediatric patients who had their first kidney transplant at King Faisal Specialist Hospital & Research Center between 2014 and 2016.

Results: We reviewed the files of 6 patients, 3 of whom were male. Read More

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http://dx.doi.org/10.1016/j.transproceed.2019.01.004DOI Listing
March 2019
2 Reads

Anti-LRP2 Nephropathy With Abundant IgG4-Positive Plasma Cells: A Case Report.

Am J Kidney Dis 2019 Mar 12. Epub 2019 Mar 12.

Department of Pathology, Oregon Health & Science University, Portland, OR. Electronic address:

In older adults, the most common kidney biopsy diagnoses include pauci-immune crescentic glomerulonephritis, membranous nephropathy, and focal segmental glomerulosclerosis. Recently, investigators described a small series of older patients (aged 66-80 years) with acute kidney injury and a kidney biopsy demonstrating tubular basement membrane (TBM) immune deposits of polytypic immunoglobulin G (IgG) and C3, acute tubular injury, and tubulointerstitial inflammation. They identified a circulating antibody against kidney tubular low-density lipoprotein (LDL) receptor-related protein 2 (LRP2; also known as megalin) in patients' sera and colocalization of LRP2 with IgG in TBM deposits. Read More

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http://dx.doi.org/10.1053/j.ajkd.2018.12.039DOI Listing
March 2019
1 Read
5.900 Impact Factor

Prevalence of low molecular weight proteinuria and Dent disease 1 CLCN5 mutations in proteinuric cohorts.

Pediatr Nephrol 2019 Mar 10. Epub 2019 Mar 10.

Rare Kidney Stone Consortium, Rochester, USA.

Background: Dent disease type 1 (DD1) is a rare X-linked disorder caused mainly by CLCN5 mutations. Patients may present with nephrotic-range proteinuria leading to erroneous diagnosis of focal segmental glomerulosclerosis (FSGS) and unnecessary immunosuppressive treatments.

Methods: The following cohorts were screened for CLCN5 mutations: Chronic Kidney Disease in Children (CKiD; n = 112); Multicenter FSGS-Clinical Trial (FSGS-CT) (n = 96), and Novel Therapies for Resistant FSGS Trial (FONT) (n = 30). Read More

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http://dx.doi.org/10.1007/s00467-019-04210-0DOI Listing
March 2019
2 Reads

Thrombus risk versus bleeding risk: a clinical conundrum.

BMJ Case Rep 2019 Mar 8;12(3). Epub 2019 Mar 8.

School of Medicine, University of Notre Dame, Fremantle, Western Australia, Australia.

A 62-year-old man presented to the Emergency Department with dyspnoea and central pleuritic chest pain radiating posteriorly to between the scapulae. His medical history included hypertension, osteoporosis and chronic kidney disease secondary to focal segmental glomerulosclerosis with relapsing nephrotic syndrome. Significant examination findings included a loud palpable P2 and a displaced apex beat. Read More

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http://dx.doi.org/10.1136/bcr-2018-228344DOI Listing
March 2019
1 Read

Salidroside Attenuates Adriamycin-Induced Focal Segmental Glomerulosclerosis by Inhibiting the Hypoxia-Inducible Factor-1α Expression Through Phosphatidylinositol 3-Kinase/Protein Kinase B Pathway.

Authors:
Guoyong Liu Liyu He

Nephron 2019 Mar 6:1-10. Epub 2019 Mar 6.

Department of Nephrology, The Second Xiangya Hospital, Central South University, Hunan Key Laboratory of Kidney Disease and Blood Purification, Changsha, China,

Background: Focal segmental glomerulosclerosis (FSGS) is a common histologic pattern of kidney injury, which may eventually lead to end-stage renal disease.

Objectives: Salidroside (SAL, p-hydroxyphenyl-β-D-glucoside) is an active component isolated from Rhodiolarosea, which has various pharmacological properties including anti-inflammation and antioxidation. SAL may attenuate FSGS, but the underlying mechanism is not clear. Read More

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http://dx.doi.org/10.1159/000497821DOI Listing
March 2019
2 Reads

Epigallocatechin-3-Gallate Attenuates Adriamycin-Induced Focal Segmental Glomerulosclerosis via Suppression of Oxidant Stress and Apoptosis by Targeting Hypoxia-Inducible Factor-1α/ Angiopoietin-Like 4 Pathway.

Authors:
Guoyong Liu Liyu He

Pharmacology 2019 6;103(5-6):303-314. Epub 2019 Mar 6.

Department of Nephrology, The Second Xiangya Hospital, Central South University, Key Lab of Kidney Disease and Blood Purification in Hunan, Changsha, China,

Background: Focal and segmental glomerular sclerosis (FSGS) is a common cause of nephrotic syndrome and end-stage renal disease. It has been reported that overproduction of reactive oxygen species (ROS) and cell apoptosis are associated with the development of FSGS. Epigallocatechin-3-gallate (EGCG) is a bioactive constituent accounting for more than 50% of the total catechins in green tea, which have anti-oxidative and anti-apoptotic effects. Read More

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http://dx.doi.org/10.1159/000496799DOI Listing
March 2019
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Mitochondrial Disease (MELAS Syndrome) Discovered at the Start of Pregnancy in a Patient with Advanced CKD: A Clinical and Ethical Challenge.

J Clin Med 2019 Mar 4;8(3). Epub 2019 Mar 4.

Department of Clinical and Biological Sciences, University of Torino, 10100 Torino, Italy.

Pregnancy is a challenge in the life of a woman with chronic kidney disease (CKD), but also represents an occasion for physicians to make or reconsider diagnosis of kidney disease. Counselling is particularly challenging in cases in which a genetic disease with a heterogeneous and unpredictable phenotype is discovered in pregnancy. The case reported regards a young woman with Stage-4 CKD, in which "Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes" (MELAS syndrome), was diagnosed during an unplanned pregnancy. Read More

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http://dx.doi.org/10.3390/jcm8030303DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6462991PMC
March 2019
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Electron microscopic findings suggestive of focal and segmental glomerulosclerosis in patients with steroid-resistant nephrotic syndrome.

Ultrastruct Pathol 2019 Mar 5:1-7. Epub 2019 Mar 5.

b Department of Pathology , Shahid Hasheminejad Hospital, Iran University of Medical Sciences , Tehran , I.R. Iran.

Distinction between minimal change disease and unsampled Focal Segmental Glomerulosclerosis is a challenging concept in kidney biopsy of patients with nephrotic syndrome with minimal histopathological findings. This study was performed to compare electron microscopic findings in patients with steroid-resistant nephrotic syndrome with minimal histopathological abnormalities and cases with Focal Segmental Glomerulosclerosis. This Cohort study was conducted in Cancer Institute, Imam Khomeini Hospital Complex, Tehran, Iran. Read More

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http://dx.doi.org/10.1080/01913123.2019.1584258DOI Listing
March 2019
2 Reads

Spectrum of biopsy proven renal disease in northern India: a single centre study.

Nephrology (Carlton) 2019 Mar 4. Epub 2019 Mar 4.

Department of Nephrology, All India Institute of Medical Sciences, New Delhi, India-110029.

Aim: Pattern of kidney diseases varies across geographies due to multiple factors. There is a paucity of information from South Asia due to absence of nationwide/regional biopsy registries. This study aimed to delineate the spectrum of renal parenchymal diseases in our region. Read More

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http://doi.wiley.com/10.1111/nep.13582
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http://dx.doi.org/10.1111/nep.13582DOI Listing
March 2019
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Outcomes of Older Patients (≥60 years) with New-Onset Idiopathic Nephrotic Syndrome Receiving Immunosuppressive Regimen: A Multicentre Study of 116 Patients.

J Clin Med 2019 Mar 2;8(3). Epub 2019 Mar 2.

Département de Néphrologie et Transplantation d'Organes, Centre de Référence des Maladies Rénales Rares, Centre Hospitalier Universitaire de Toulouse, 31000 Toulouse, France.

Because of its rarity, renal presentation and outcomes of idiopathic nephrotic syndrome (INS; minimal changes disease or focal and segmental glomerulosclerosis) has poorly been described in elderly patients, precluding an individualized therapy procedure. Whether immunosuppressive regimens formerly designed in children and young adults are safe and efficient in elderly remains elusive. In a large multicentric retrospective study that included 116 patients with INS and onset ≥ 60 years of age, we showed that cumulative incidence of renal response was 95% after frontline therapy, with an age-dependent median time-to-response (60 days before 70 years of age at the onset vs. Read More

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https://www.mdpi.com/2077-0383/8/3/298
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http://dx.doi.org/10.3390/jcm8030298DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6463053PMC
March 2019
8 Reads

Evaluation of kidney biopsies in elderly patients.

Int Urol Nephrol 2019 Mar 4. Epub 2019 Mar 4.

Department of Nephrology, Haydarpasa Numune Training and Research Hospital, Istanbul, Turkey.

Objective: The renal parenchymal disease spectrum in geriatric patients is similar to that in younger patients and can be controlled by appropriate treatment. We evaluated the clinicopathological features of kidney biopsies from geriatric patients.

Materials And Methods: One hundred nine native kidney biopsies from older patients (> 65 years old) obtained from 2005 to 2014 were evaluated retrospectively. Read More

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http://dx.doi.org/10.1007/s11255-019-02109-1DOI Listing
March 2019
5 Reads

European Society of Pediatric Nephrology survey on current practice regarding recurrent focal segmental glomerulosclerosis after pediatric kidney transplantation.

Pediatr Transplant 2019 Mar 1:e13385. Epub 2019 Mar 1.

Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

Introduction: Primary FSGS is an important cause of ESRD in children. FSGS recurrence after kidney transplantation is associated with early graft loss. No guidelines for treatment of FSGS recurrence exist. Read More

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http://dx.doi.org/10.1111/petr.13385DOI Listing
March 2019
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Renal-Protective Effects of the Peroxisome Proliferator-Activated Receptor-γ Agonist Pioglitazone in ob/ob Mice.

Med Sci Monit 2019 Mar 1;25:1582-1589. Epub 2019 Mar 1.

Tianjin Key Laboratory of Ionic-Molecular Function of Cardiovascular Disease, Department of Cardiology, Tianjin Institute of Cardiology, Second Hospital of Tianjin Medical University, Tianjin, China (mainland).

BACKGROUND This study investigated the therapeutic effects of the peroxisome proliferator-activated receptor-γ (PPARγ) agonist pioglitazone in ob/ob mice with obesity-related glomerulopathy (ORG). MATERIAL AND METHODS A total of 24 mice were divided into 3 groups: wild-type C57BL/6 mice (n=8), ob/ob mice (n=8), and ob/ob mice receiving pioglitazone treatment (n=8). Body mass, blood glucose, serum adiponectin (ADP), and urine microalbumin (mALB) levels were determined. Read More

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http://dx.doi.org/10.12659/MSM.913461DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6407328PMC
March 2019
2 Reads

Focal and Segmental Glomerulosclerosis and Membranous Nephropathy overlapping in a patient with Nephrotic Syndrome: a case report.

J Bras Nefrol 2019 Feb 25. Epub 2019 Feb 25.

Universidade Federal do Triângulo Mineiro, Instituto de Ciências Biológicas e Naturais, Uberaba, MG, Brasil.

Introduction: Some cases of membranous nephropathy (MGN) present focal segmental glomerulosclerosis (FSGS) typically associated with disease progression. However, we report a case of a patient who seemed to have MGN and FSGS, both primary.

Case Presentation: A 17-year-old female, Caucasian, presenting lower extremity edema associated with episodes of foamy urine and high blood pressure, had physical and laboratorial exams indicating nephrotic syndrome. Read More

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http://dx.doi.org/10.1590/2175-8239-JBN-2018-0239DOI Listing
February 2019
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Widening spectrum of renal involvement in psoriasis: First reported case of C3 glomerulonephritis in a psoriatic patient.

Saudi J Kidney Dis Transpl 2019 Jan-Feb;30(1):258-260

Department of Medicine, Northern Railway Central Hospital, New Delhi, India.

Renal involvement in psoriasis is usually seen as mesangioproliferative glomerulonephritis (GN), IgA nephropathy, and focal segmental glomerulosclerosis. Microscopic hematuria is not uncommon in a patient of psoriasis with above-mentioned disorders. We found C3 GN as a cause when evaluated for macroscopic and persistent microscopic hematuria in a patient of psoriasis. Read More

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February 2019

Incidence of pediatric glomerular diseases in Arab world: A systematic review.

Saudi J Kidney Dis Transpl 2019 Jan-Feb;30(1):15-23

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

Despite the high number of Arab children, there are a scanty available data about different types of pediatric glomerular diseases in all Arab countries. Very few Arab countries have a national renal biopsy registry. In this review, we performed a systematic review analyzing the incidence of pediatric glomerular disease in all Arab countries. Read More

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February 2019
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HCV-Associated Nephropathies in the Era of Direct Acting Antiviral Agents.

Front Med (Lausanne) 2019 8;6:20. Epub 2019 Feb 8.

Division of Nephrology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, United States.

Hepatitis C virus (HCV) infection is a systemic disorder that frequently associates with extrahepatic manifestations, including nephropathies. Cryoglobulinemia is a typical extrahepatic manifestation of HCV infection that often involves kidneys with a histological pattern of membranoproliferative glomerulonephritis. Other, less common renal diseases related to HCV infection include membranous nephropathy, focal segmental glomerulosclerosis, IgA nephropathy, fibrillary and immunotactoid glomerulopathy. Read More

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http://dx.doi.org/10.3389/fmed.2019.00020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6376251PMC
February 2019
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CD44-Positive Glomerular Parietal Epithelial Cells in a Mouse Model of Calcineurin Inhibitors-Induced Nephrotoxicity.

Nephron 2019 Feb 20:1-11. Epub 2019 Feb 20.

Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

Background/aims: Calcineurin inhibitors (CNIs) such as cyclosporine A (CsA) and tacrolimus are immunosuppressants that are frequently used as "key drugs" in the fields of transplantation, steroid resistance, refractory nephrotic syndrome, and autoimmune disease. However, long-term CNI use causes nephrotoxicity, the features of which are arteriolar hyalinosis, tubular atrophy, striped interstitial fibrosis, and focal segmental glomerulosclerosis (FSGS). We investigated whether FSGS in CNI-induced nephrotoxicity is associated with CD44-positive glomerular parietal epithelial cells (PECs), which play a significant role in experimental and human FSGS pathogenesis. Read More

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http://dx.doi.org/10.1159/000497325DOI Listing
February 2019
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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
March 2019
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Successful Treatment With Abatacept in Recurrent Focal Segmental Glomerulosclerosis After Kidney Transplant.

Exp Clin Transplant 2019 01;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
3 Reads