23,542 results match your criteria Nephrotic Syndrome


Treatment-resistant PLA2R-negative membranous nephropathy responsive to low-density lipoprotein apheresis.

J Clin Apher 2018 Dec 10. Epub 2018 Dec 10.

Department of Transfusion Medicine, National Institutes of Health Clinical Center, Bethesda, Maryland.

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. Read More

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December 2018

Efficacy and safety of rituximab therapy for membranous nephropathy: a meta-analysis.

Eur Rev Med Pharmacol Sci 2018 Nov;22(22):8021-8029

Department of Nephrology, China-Japan Union Hospital of Jilin University, Changchun, China.

Objective: Membranous nephropathy (MN) is a leading cause of nephrotic syndrome in adults, but the treatment of MN remains controversial. Rituximab, a possible alternative treatment option, represented a new therapeutic hope for the treatment of membranous nephropathy (MN). We performed a meta-analysis to perform the efficacy and safety of rituximab therapy. Read More

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November 2018
1 Read

Immunoglobulin E and G Levels in Predicting Minimal Change Disease before Renal Biopsy.

Biomed Res Int 2018 11;2018:3480309. Epub 2018 Nov 11.

Kidney Research Center, Department of Nephrology, Chang Gung Memorial Hospital, Taoyuan, Taiwan.

Purpose: The diagnosis of minimal change disease in adults relies mainly on renal biopsy, but this procedure is not without complications. Despite the advancements in technique of percutaneous renal biopsy, biopsy-related complications still occur. Bleeding is one of the major complications, which may lead to hemodynamic instability and, sometimes, even death. Read More

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November 2018

Minimal change disease: A case report.

Nursing 2018 Dec 10. Epub 2018 Dec 10.

At California State University, Chico, Fay Mitchell-Brown is an associate professor and Tiernan Veisze is a senior nursing student. Dr. Mitchell-Brown teaches pathophysiology and is a practicing critical care nurse.

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. Read More

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December 2018

Bioinformatic Analysis Reveals Novel Immune-Associated Hub Genes in Human Membranous Nephropathy.

Genet Test Mol Biomarkers 2018 Dec 8. Epub 2018 Dec 8.

Department of Nephrology, West China Hospital, Sichuan University , Chengdu, China .

Background: Membranous nephropathy (MN) is one of the most common pathologies of the nephrotic syndrome. MN is closely associated with the autoimmune response but its molecular mechanism remains unclear. Bioinformatic network analysis can be used to identify disease-related hub genes. Read More

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December 2018

Nanoscale Imaging of Kidney Glomeruli Using Expansion Pathology.

Front Med (Lausanne) 2018 21;5:322. Epub 2018 Nov 21.

Department of Biological Sciences, Mellon College of Science, Carnegie Mellon University, Pittsburgh, PA, United States.

Kidney glomerular diseases, such as the minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS), and other nephrotic syndromes, are typically diagnosed or confirmed via electron microscopy. Although optical microscopy has been a vital tool to examine clinical specimens for diagnoses in pathology for decades, the optical resolution is constricted by the physical diffraction limit of the optical microscope, which prevents high-resolution investigation of subcellular anatomy, such as of the podocyte tertiary foot processes. Here, we describe a simple, fast, and inexpensive protocol for nanoscale optical imaging of kidney glomeruli. Read More

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November 2018
1 Read

SGPL1 Deficiency: A Rare Cause of Primary Adrenal Insufficiency.

J Clin Endocrinol Metab 2018 Dec 3. Epub 2018 Dec 3.

Section on Endocrinology and Genetics, Program on Developmental Endocrinology and Genetics & Pediatric Endocrinology Inter-Institute Training Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.

Context: Multiple autosomal recessive genes have been etiologically linked to Primary Adrenal Insufficiency (PAI). Recently, SGPL1 gene mutations were recognized as causes of steroid-resistant nephrotic syndrome type 14 (NPHS14), a sphingolipidosis with multisystemic manifestations including PAI. Interestingly, this is the only monogenetic form of nephrotic syndrome (NS) and the sole sphingolipidosis causing adrenal disease. Read More

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December 2018
5 Reads

3D organoid-derived human glomeruli for personalised podocyte disease modelling and drug screening.

Nat Commun 2018 12 4;9(1):5167. Epub 2018 Dec 4.

Murdoch Children's Research Institute, Flemington Rd, Melbourne, 3052, VIC, Australia.

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. Read More

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December 2018

Evidence for a role of Angiotensin Converting Enzyme 2 in proteinuria of idiopathic nephrotic syndrome.

Biosci Rep 2018 Dec 4. Epub 2018 Dec 4.

UFMG, Belo Horizonte, Brazil

Introduction: Renin Angiotensin System (RAS) plays a role in idiopathic nephrotic syndrome (INS). Most studies investigated only the classical RAS axis. Therefore, the aims of this study were to evaluate urinary levels of RAS molecules related to classical and to counter-regulatory axes in pediatric patients with INS, to compare the measurements with levels in healthy controls and to search for associations with inflammatory molecules, proteinuria and disease treatment. Read More

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December 2018

Morphological Processes of Foot Process Effacement in Puromycin Aminonucleoside Nephrosis Revealed by FIB/SEM Tomography.

J Am Soc Nephrol 2018 Dec 4. Epub 2018 Dec 4.

Department of Anatomy and Life Structure and.

Background: Foot process effacement is one of the pathologic indicators of podocyte injury. However, the morphologic changes associated with it remain unclear.

Methods: To clarify the developmental process, we analyzed puromycin nephrotic podocytes reconstructed from serial focused-ion beam/scanning electron microscopy (FIB/SEM) images. Read More

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December 2018

Predicting risk of pulmonary infection in patients with primary membranous nephropathy on immunosuppressive therapy: the AIM-7C score.

Nephrology (Carlton) 2018 Nov 29. Epub 2018 Nov 29.

MRI Division, Department of Radiology, HeBei General Hospital, ShiJiaZhuang P.R. China.

Aim: Pulmonary infection (PI) is the leading cause of death in patients with primary membranous nephropathy on immunosuppressive therapy. A rating score was thus developed to foresee the risk of PI in such patients.

Methods: We reviewed the charts of the pertinent patients treated during the past three years either with (n=29) or without PI (n=304). Read More

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November 2018
2 Reads

Efficacy and safety of mycophenolate mofetil versus levamisole in frequently relapsing nephrotic syndrome: an open-label randomized controlled trial.

Kidney Int 2018 Nov 22. Epub 2018 Nov 22.

Division of Nephrology, Department of Pediatrics, Indian Council of Medical Research Advanced Center for Research in Nephrology, India Institute of Medical Sciences, New Delhi, India. Electronic address:

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/m daily) or levamisole (2-2. Read More

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November 2018
1 Read

"Neuro-renal syndrome" related to anti-contactin-1 antibodies.

Muscle Nerve 2018 Nov 29. Epub 2018 Nov 29.

Institute for Neurosciences of Montpellier, INSERM U1051, Montpellier University, Hopital Gui de Chauliac, 80 avenue Augustin Fliche, 34295, Montpellier Cedex 5, France.

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November 2018
1 Read

Unraveling the Molecular Mechanisms Underlying Complement Dysregulation by Nephritic Factors in C3G and IC-MPGN.

Front Immunol 2018 15;9:2329. Epub 2018 Oct 15.

Clinical Research Center for Rare Diseases Aldo e Cele Daccò and Centro Anna Maria Astori, Science and Technology Park Kilometro Rosso, Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Bergamo, Italy.

Membranoproliferative glomerulonephritis (MPGN) was recently classified as C3 glomerulopathies (C3G), and immune-complex (IC) mediated MPGN. Dysregulation of the complement alternative pathway, driven by acquired and/or genetic defects, plays a pathogenetic role in C3G. However, alternative pathway abnormalities were also found in IC-MPGN. Read More

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October 2018

Efficacy of Semiannual Single Fixed Low-Dose Rituximab Therapy in Steroid-Dependent Minimal Change Nephrotic Syndrome: A Case Series.

Case Rep Nephrol Dial 2018 Sep-Dec;8(3):230-237. Epub 2018 Oct 22.

Division of Nephrology, Department of Medicine, Yachiyo Medical Center, Tokyo Women's Medical University, Chiba, Japan.

The frequency of using rituximab to treat refractory nephrotic syndrome has recently been increasing, and the conventional dose of rituximab used to treat it, 375 mg/m body surface area once weekly for 4 weeks, has been modelled on the chemotherapy regimen for B-cell non-Hodgkin's lymphoma. The dose and intervals of rituximab in refractory nephrotic syndrome remain controversial. Clear lymphoma cell hyperplasia is seen in lymphoma patients, but not in nephrotic syndrome patients. Read More

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October 2018
9 Reads

The utility of urinary CD80 as a diagnostic marker in patients with renal diseases.

Sci Rep 2018 Nov 23;8(1):17322. Epub 2018 Nov 23.

Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan.

CD80, which regulates T cell activation, may provide a differential diagnostic marker between minimal change disease (MCD) and other renal diseases, including focal segmental glomerular sclerosis (FSGS). However, recent reports show contrasting results. Therefore, we evaluated the utility of urinary CD80 as a diagnostic biomarker. Read More

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November 2018
4 Reads

Increased urinary exosomal microRNAs in children with idiopathic nephrotic syndrome.

EBioMedicine 2018 Nov 19. Epub 2018 Nov 19.

Department of Clinical Laboratory, Jinling Hospital, Nanjing School of Clinical Medicine, Southern Medical University, State Key Laboratory of Analytical Chemistry for Life Science, Nanjing, China; State Key Laboratory of Pharmaceutical Biotechnology, Jiangsu Engineering Research Center for MicroRNA Biology and Biotechnology, Advance Research Institute of Life Sciences, Nanjing University, School of Life Sciences, Nanjing, China. Electronic address:

Background: Urinary exosomal miRNAs are gaining increasing attention for their potential as ideal non-invasive biomarkers for kidney diseases; however, little is known about their diagnostic ability for paediatric nephrotic syndrome (NS). This study explored the clinical value of urinary exosomal miRNAs for paediatric idiopathic NS.

Methods: Urine samples were collected from 129 NS children and 126 age-/sex-matched healthy controls. Read More

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November 2018
9 Reads

Constitutional absence of epithelial integrin α3 impacts the composition of the cellular microenvironment of ILNEB keratinocytes.

Matrix Biol 2018 Dec 3;74:62-76. Epub 2018 Jul 3.

Department of Dermatology, Medical Center - University of Freiburg, Faculty of Medicine, Freiburg, Germany. Electronic address:

Integrin α3β1, a major epidermal adhesion receptor is critical for organization of the basement membrane during development and wound healing. Integrin α3 deficiency leads to interstitial lung disease, nephrotic syndrome and epidermolysis bullosa (ILNEB), an autosomal recessive multiorgan disease characterized by basement membrane abnormalities in skin, lung and kidney. The pathogenetic chains from ITGA3 mutation to tissue abnormalities are still unclear. Read More

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December 2018

Significance of histological crescent formation in patients with IgA vasculitis (Henoch-Schönlein purpura)-related nephritis: a cohort in the adult Chinese population.

BMC Nephrol 2018 Nov 22;19(1):334. Epub 2018 Nov 22.

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

Background: IgA vasculitis (IgAV, formerly Henoch-Schönlein purpura) is a type of systemic vasculitis. This study aimed to explore the clinicopathological features, treatment and renal outcomes of adult IgAV-related nephritis (Henoch-Schönlein purpura nephritis) patients with different degrees of crescent formation.

Methods: Adult patients with biopsy-proven IgAV-related nephritis in Nanjing Jinling Hospital were enrolled and divided into three groups as follows: control (no crescents, n = 257), group 1 (crescents < 25%, n = 381), and group 2 (crescents ≥25%, n = 60). Read More

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November 2018
1 Read
1.520 Impact Factor

Targeted gene panel for genetic testing of south Indian children with steroid resistant nephrotic syndrome.

BMC Med Genet 2018 Nov 20;19(1):200. Epub 2018 Nov 20.

Division of Molecular Medicine, St. John's Research Institute, Bangalore, India.

Background: Steroid resistant nephrotic syndrome (SRNS) is a genetically heterogeneous disease with significant phenotypic variability. More than 53 podocyte-expressed genes are implicated in SRNS which complicates the routine use of genetic screening in the clinic. Next generation sequencing technology (NGS) allows rapid screening of multiple genes in large number of patients in a cost-effective manner. Read More

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November 2018

Minimal change nephrotic syndrome in patients infected with human immunodeficiency virus: a retrospective study of 8 cases.

BMC Nephrol 2018 Nov 20;19(1):331. Epub 2018 Nov 20.

AP-HP (Assistance Publique-Hôpitaux de Paris), Service de Néphrologie et de Transplantation, Centre de Référence Maladie Rare Syndrome Néphrotique Idiopathique, Hôpital Henri-Mondor/Albert-Chenevier, F-94000, Créteil, France.

Background: Human immunodeficiency virus (HIV) is associated with diverse glomerular diseases. Characteristics of minimal change nephrotic syndrome (MCNS) in this setting have been little studied, and the specific features of this uncommon association remain to be determined.

Methods: We conduct a retrospective study. Read More

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November 2018
6 Reads

A rare cause of AA amyloidosis and end-stage kidney failure: Answers.

Pediatr Nephrol 2018 Nov 19. Epub 2018 Nov 19.

Division of Pediatric Nephrology, Department of Pediatrics, Koç University School of Medicine, Davutpaşa cad no: 4 Topkapı, 34010, Istanbul, Turkey.

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November 2018
1 Read

A rare cause of AA amyloidosis and end-stage kidney failure: Questions.

Pediatr Nephrol 2018 Nov 19. Epub 2018 Nov 19.

Division of Pediatric Nephrology, Department of Pediatrics, Koç University School of Medicine, Davutpaşa cad no: 4 Topkapı, 34010, Istanbul, Turkey.

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November 2018
1 Read

Membranous or membranous-like GN: A case report of massive proteinuria, positive serum with negative PLA2R on biopsy.

Clin Case Rep 2018 Nov 9;6(11):2198-2201. Epub 2018 Oct 9.

Department of Pathology Penn State Milton S. Hershey Medical Centre Hershey Pennsylvania.

This case report represents primary membranous glomerulonephritis (MGN) with positive serum anti-PLA2R antibodies, 2+ positivity for IgG4 on immunofluorescence with routine fresh-frozen sections and negative PLA2R stain on biopsy. He was treated as primary MGN based on positive serum PLA2R and the absence of clinical symptoms or signs suggestive of any secondary MGN. Read More

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November 2018
2 Reads

Interleukin-7 stimulation inhibits nephrin activation and induces podocyte injury.

Biochem Biophys Res Commun 2018 Dec 16;507(1-4):100-105. Epub 2018 Nov 16.

Department of Pediatric Nephropathy, The First Hospital of Jilin University, China. Electronic address:

The glomerular podocytes control filtration barrier permeability in the kidney, and their disturbance underlies the pathogenesis of idiopathic nephrotic syndrome (INS), a kidney disease that predominantly occurs in children. In this study, we found that the interleukin-7 receptor (IL-7R) was induced in the glomeruli of adriamycin (ADR)-induced mouse nephropathy, a rodent model of nephrotic syndrome. In addition, IL-7R was also induced by ADR in mouse podocytes cultured in vitro. Read More

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December 2018
4 Reads

Duodenal Schwannoma as a Rare Association With Membranous Nephropathy: A Case Report.

Am J Kidney Dis 2018 Nov 16. Epub 2018 Nov 16.

Department of Medicine, University of Hawaii John A. Burns School of Medicine, Honolulu, HI.

Membranous nephropathy (MN) associated with malignancies is a well-known entity. However, its association with benign neoplasm is not broadly recognized. A 69-year-old man with recurrent nephrotic syndrome presented with pedal edema and proteinuria of 5 months' duration. Read More

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November 2018
5 Reads

Nephrotic Syndrome.

Pediatr Clin North Am 2019 Feb;66(1):73-85

Division of Pediatric Nephrology, Department of Pediatrics, Emory University School of Medicine and Children's Healthcare of Atlanta, 2015 Uppergate Drive Northeast, Atlanta, GA 30322-1015, USA.

Nephrotic syndrome is characterized by edema, proteinuria, hypoalbuminemia, and hyperlipidemia. Minimal change disease, the most common cause in childhood, generally responds to corticosteroids, although most patients experience disease relapses. Focal segmental glomerulosclerosis is usually resistant to corticosteroids and carries a significant risk of kidney failure, necessitating renal transplantation. Read More

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February 2019
1 Read

Management of Intra-Abdominal Biloma in the Setting of Chronic Steroid Use for Nephrotic Syndrome.

Am Surg 2018 Apr;84(4):e156-e157

Department of Surgery, University of South Alabama Medical Center, Mobile, Alabama, USA.

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Parental Whole-Exome Sequencing Enables Sialidosis Type II Diagnosis due to an Missense Mutation as an Underlying Cause of Nephrotic Syndrome in the Child.

Kidney Int Rep 2018 Nov 29;3(6):1454-1463. Epub 2018 Jul 29.

Center for Pediatrics and Adolescent Medicine, University Hospital Freiburg, Freiburg University, Faculty of Medicine, Freiburg, Germany.

Introduction: Monogenetic renal diseases, including recessively inherited nephrotic syndromes, represent a significant health burden despite being rare conditions. Precise diagnosis, including identification of the underlying molecular cause, is especially difficult in low-income countries and/or if affected individuals are unavailable for biochemical testing. Whole-exome sequencing (WES) has opened up novel diagnostic perspectives for these settings. Read More

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November 2018
3 Reads

V260E Is a Frequent Cause of Steroid-Resistant Nephrotic Syndrome in Black South African Children.

Kidney Int Rep 2018 Nov 29;3(6):1354-1362. Epub 2018 Jul 29.

Basic Research Laboratory, Center for Cancer Research, National Cancer Institute, Leidos Biomedical Research, Inc., Frederick National Laboratory, Frederick, Maryland, USA.

Introduction: In South Africa (SA), steroid-resistant nephrotic syndrome (SRNS) is more frequent in black than in Indian children.

Methods: Seeking a genetic basis for this disparity, we enrolled 33 Indian and 31 black children with steroid-sensitive nephrotic syndrome (SSNS) and SRNS from KwaZulu-Natal, SA; SRNS children underwent kidney biopsy. We sequenced and genotyped in 15 SSNS and 64 SRNS unrelated patients and 104 controls and replicated results in 18 black patients with steroid-resistant focal segmental glomerulosclerosis (SR-FSGS). Read More

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November 2018
2 Reads

Management of Denys-Drash syndrome: A case series based on an international survey.

Clin Nephrol Case Stud 2018 12;6:36-44. Epub 2018 Nov 12.

Department of Pediatrics and Department of Epidemiology, Biostatistics and Occupational Health, Faculty of Medicine, McGill University, Montreal, QC, Canada.

Denys-Drash syndrome (DDS), a condition caused by mutations in the tumor-suppressor gene WT-1, is associated with a triad of disorders: ambiguous genitalia, nephrotic syndrome leading to end-stage renal disease (ESRD), and Wilms' tumor. Given the variable disease course, management is challenging. We aimed to describe the evolution of DDS and the range of management strategies by summarizing the clinical courses of cases collected from a questionnaire sent to the international pediatric nephrology community. Read More

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November 2018
6 Reads

Correction to: Recurrence of nephrotic syndrome following kidney transplantation is associated with initial native kidney biopsy findings : A Midwest Pediatric Nephrology Consortium (MWPNC) study.

Pediatr Nephrol 2018 Nov 15. Epub 2018 Nov 15.

Department of Pediatrics, Divisions of Nephrology and Critical Care, Duke University Medical Center, Durham, NC, 27710, USA.

The original version of this article unfortunately contained a mistake. The subtitle "A Midwest Pediatric Nephrology Consortium (MWPNC) study" was missing. The correct title including subtitle is given above. Read More

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November 2018
6 Reads

Adrenocorticotropic Hormone for Childhood Nephrotic Syndrome: The ATLANTIS Randomized Trial.

Clin J Am Soc Nephrol 2018 Dec 15;13(12):1859-1865. Epub 2018 Nov 15.

Department of Pediatrics, Emory and Children's Pediatric Institute, Atlanta, Georgia.

Background And Objectives: There is renewed interest in adrenocorticotropic hormone (ACTH) for the treatment of nephrotic syndrome. We evaluated the efficacy and safety of ACTH in children with frequently relapsing or steroid-dependent nephrotic syndrome in a randomized trial.

Design, Setting, Participants, & Measurements: Participants aged 2-20 years old with frequently relapsing or steroid-dependent nephrotic syndrome were enrolled from 16 sites in the United States and randomized 1:1 to ACTH (repository corticotropin injection) or no relapse-preventing treatment. Read More

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December 2018
9 Reads

Characterization of autoantibodies in primary membranous nephropathy and their clinical significance.

Expert Rev Clin Immunol 2018 Nov 15:1-11. Epub 2018 Nov 15.

a III. Department of Medicine , University Medical Center Hamburg-Eppendorf , Hamburg , Germany.

Introduction: Membranous nephropathy (MN) is the most common cause of a nephrotic syndrome in Caucasian adults. The identification of target antigens in MN in the last decade has had a major impact on the clinical approach to these patients. Areas covered: Since the discoveries in animal models in the 1980s that circulating autoantibodies induce disease upon in situ binding to glomerular podocytes, many attempts have been undertaken to define the human antigens responsible for disease induction. Read More

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November 2018
2 Reads

Efficacy and safety of rituximab in childhood-onset, difficult-to-treat nephrotic syndrome: A multicenter open-label trial in Korea.

Medicine (Baltimore) 2018 Nov;97(46):e13157

Department of Pediatrics, Seoul National University Children's Hospital, Seoul.

Background: The anti-CD20 monoclonal antibody rituximab (RTX) has been proposed as a rescue therapy for difficult-to-treat nephrotic syndrome (NS). We conducted a clinical trial to evaluate the efficacy and safety of RTX in children with difficult-to-treat NS dependent on or resistant to steroids and calcineurin inhibitors (CNIs).

Methods: A multicenter open-label trial was performed at 8 major pediatric nephrology centers in Korea. Read More

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November 2018
4 Reads

Nephrotic Syndrome as a Presenting Feature in a Child With NLRP3 Mutation.

J Clin Rheumatol 2018 Nov 14. Epub 2018 Nov 14.

Departments of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, National Amyloidosis Centre, Division of Medicine, UCL, Royal Free Hospital, London, United Kingdom. Departments of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India Department of Pediatrics Postgraduate Institute of Medical Education and Research Chandigarh, India Departments of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

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November 2018
1 Read

Primary glomerulonephritis with predominant mesangial Immunoglobulin G deposits.

Tunis Med 2018 Jul;96(7):442-444

Background: Glomerular deposits are a major pathologic feature of a wide range of human glomerulonephritis and may be located in the mesangial, subepithelial, and subendothelial regions. Rare cases of primary glomerulonephritis definied by exclusive or predominant mesangial IgG deposits were reported. We reviewed the pathologic findings for the 848 renal biopsies examined in our department between 2007 and 2016, one case of primary mesangial IgG glomerulonephritis (MIG) in the absence of any evidence of systemic lupus erythematous (SLE), of other systemic diseases or of Infections. Read More

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July 2018
2 Reads

Homozygous splicing mutation in NUP133 causes Galloway-Mowat syndrome.

Ann Neurol 2018 Nov 14. Epub 2018 Nov 14.

Department of Human Genetics, Yokohama City University Graduate School of Medicine, Yokohama 236-0004, Japan.

Objective: Galloway-Mowat syndrome (GAMOS) is a neural and renal disorder, characterized by microcephaly, brain anomalies, and early-onset nephrotic syndrome. Biallelic mutations in WDR73 and the four subunit genes of the KEOPS complex are reported to cause GAMOS. Furthermore, an identical homozygous NUP107 (nucleoporin 107 kDa) mutation was identified in four GAMOS-like families, although biallelic NUP107 mutations were originally identified in steroid-resistant nephrotic syndrome. Read More

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November 2018
6 Reads

Childhood onset steroid-sensitive nephrotic syndrome continues into adulthood.

Pediatr Nephrol 2018 Nov 13. Epub 2018 Nov 13.

Department of Pediatric and Adolescent Medicine, Aarhus University Hospital, Skejby, Palle Juul-Jensens Boulevard 99, DK-8200, Aarhus N, Denmark.

Background: Childhood steroid-sensitive nephrotic syndrome (SSNS) has previously been assumed to be a disease of childhood. This has been challenged by few studies reporting that some patients with childhood SSNS may continue to relapse into adulthood. The aim of this study was to investigate the long-term outcome of childhood SSNS presenting data from an unselected well-defined cohort of Danish patients. Read More

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November 2018
8 Reads

Long-term outcomes after early treatment with rituximab for Japanese children with cyclosporine- and steroid-resistant nephrotic syndrome.

Pediatr Nephrol 2018 Nov 13. Epub 2018 Nov 13.

Division of Nephrology, Saitama Children's Medical Center, 1-2 Shintoshin, Chuo-ku, Saitama city, Saitama, 330-8777, Japan.

Background: Although rituximab (RTX) may be effective treatment in children with nephrotic syndrome who are resistant to cyclosporine A and steroid (CsA-SRNS), long-term outcomes after B cell depleting therapy remain unclear.

Case-diagnosis/treatment: We retrospectively reviewed the clinical courses (median follow-up, 5.1 years) of six CsA-SRNS children (three boys; median age at RTX, 4. Read More

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November 2018
2 Reads

Postpartum nephrotic syndrome related to new onset of systemic lupus erythematosus: A case report.

Case Rep Womens Health 2018 Oct 15;20:e00083. Epub 2018 Oct 15.

Second Department of Obstetrics and Gynecology, Aretaieio University Hospital, Vasilissis Sofias 76, Greece.

Postpartum nephrotic syndrome in a pregnant woman with rheumatoid arthritis in long-standing remission is rare. Systemic lupus erythematosus can remain undiagnosed, especially in the absence of clinical manifestations. We present the case of a 34-year-old woman (gravida 2, para 1) who underwent a lower-segment cesarean section at 34 weeks and 6 days of gestation because she had developed preeclampsia and nephrotic syndrome. Read More

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October 2018
6 Reads

Pathogenesis, histopathologic findings and treatment modalities of lipoprotein glomerulopathy: A review.

J Bras Nefrol 2018 Nov 8. Epub 2018 Nov 8.

Departamento de Patologia, Santa Casa de Misericórdia de Porto Alegre, Porto Alegre, RS, Brasil.

Lipoprotein glomerulopathy (LPG) is an uncommon cause of nephrotic syndrome and/or kidney failure. At microscopy, LPG is characterized by the presence of lipoprotein thrombi in dilated glomerular capillaries due to different ApoE mutations. ApoE gene is located on chromosome 19q13. Read More

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November 2018
2 Reads

Recurrent venous thromboembolism in primary membranous nephropathy despite direct Xa inhibitor therapy.

J Nephrol 2018 Nov 12. Epub 2018 Nov 12.

Division of Nephrology and Hypertension, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.

Clinically apparent venous thromboembolism (VTE) occurs in approximately 7% of patients with membranous nephropathy. Hypoalbuminemia at diagnosis is an independent risk factor for VTE, and risk increases significantly as albumin falls. Optimal prophylactic and treatment anticoagulation regimens in the nephrotic syndrome remain unproven but novel oral anti-coagulants have become attractive therapeutic options. Read More

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November 2018
6 Reads

IgA-dominant glomerulonephritis with a membranoproliferative pattern of injury.

Hum Pathol 2018 Nov 3;81:272-280. Epub 2018 Jul 3.

University of Washington, Department of Pathology, Seattle, WA 98195.

Immunoglobulin A (IgA)-dominant membranoproliferative glomerulonephritis (MPGN) is a descriptive term for renal biopsies in which differential diagnoses of unusual IgA nephropathy (IgAN), infection-related GN, or other etiologies are considered. We sought to understand clinical and pathologic features of this finding. Native kidney biopsies with IgA-dominant immune deposits and diffuse MPGN features without significant exudative features or subepithelial deposits were retrospectively reviewed. Read More

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November 2018
4 Reads
2.770 Impact Factor

The Role of Glucocorticoid Receptors in Podocytes and Nephrotic Syndrome.

Nucl Receptor Res 2018 24;5. Epub 2018 Apr 24.

Department of Biochemistry, School of Medicine, Case Western Reserve University, 10900 Euclid Avenue, Cleveland, Ohio 44106, USA.

Glucocorticoid receptor (GC), a founding member of the nuclear hormone receptor superfamily, is a glucocorticoid-activated transcription factor that regulates gene expression and controls the development and homeostasis of human podocytes. Synthetic glucocorticoids are the standard treatment regimens for proteinuria (protein in the urine) and nephrotic syndrome (NS) caused by kidney diseases. These include minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), membranous nephropathy (MN) and immunoglobulin A nephropathy (IgAN) or subsequent complications due to diabetes mellitus or HIV infection. Read More

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April 2018
1 Read

Correlation between endocapillary proliferative and nephrotic-range proteinuria in children with Henoch-Schönlein purpura nephritis.

Pediatr Nephrol 2018 Nov 10. Epub 2018 Nov 10.

Department of Anesthesia, Stanford University School of Medicine, Stanford, CA, USA.

Background: The endocapillary proliferative (EP) lesion is not included in the International Study of Kidney Disease in Children (ISKDC) pathological classification of Henoch-Schönlein purpura nephritis (HSPN). The main objective of the study was to determine the pathological importance of EP in the development of proteinuria in children with Henoch-Schönlein purpura nephritis (HSPN).

Methods: The pathological features of 148 HSPN children with nephrotic-range proteinuria were investigated retrospectively. Read More

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November 2018
7 Reads
2.860 Impact Factor

The Accumulation of Heparan Sulfate S-Domains in Kidney Transthyretin Deposits Accelerates Fibril Formation and Promotes Cytotoxicity.

Am J Pathol 2018 Nov 7. Epub 2018 Nov 7.

Department of Biochemistry, Wakayama Medical University, Wakayama, Japan; Department of Pathology and Laboratory Medicine, Institute of Biomedical Sciences, Tokushima University Graduate School, Tokushima, Japan.

The highly sulfated domains of heparan sulfate (HS), also called HS S-domains, are made up of repeated trisulfated disaccharide units [iduronic acid (2S)-glucosamine (NS, 6S)-] and are selectively remodeled by extracellular endoglucosamine 6-sulfatases (Sulfs). Although HS S-domains are critical for signal transduction of several growth factors, their roles in amyloidoses are not yet fully understood. Here, we found HS S-domains in the kidney of a patient with transthyretin amyloidosis (ATTR amyloidosis). Read More

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November 2018
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Retroperitoneal abscess with subcutaneous extension: case report of a rare complication of percutaneous renal biopsy.

BMC Nephrol 2018 Nov 9;19(1):319. Epub 2018 Nov 9.

Department of Clinical Medicine, Faculty of Medicine, University of Colombo, Colombo, Sri Lanka.

Background: Infective complications following percutaneous renal biopsy are rare, even among immunocompromised. However it is important to be vigilant about such complications, to detect them early and prevent morbidity and mortality. We report a case of retroperitoneal abscess with extension to subcutaneous plane after a renal biopsy. Read More

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November 2018
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