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Nefrologia 2019 Apr 20. Epub 2019 Apr 20.

Renal Division, Department of Medicine, Peking University First Hospital, Institute of Nephrology, Peking University, Beijing 100034, PR China; Key Laboratory of Renal Disease, Ministry of Health of China, Beijing 100034, PR China; Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing 100034, PR China; Peking-Tsinghua Center for Life Sciences, PR China.

Background: C3 glomerulonephritis is a rare, chronic disease characterized by C3c-dominant staining on renal biopsy and is caused by inherited or acquired alternative complement pathway dysregulation.

Case Presentation: Here, we reported a 36-year-old man presenting with nephritic syndrome and normal renal function. Secondary causes were excluded by detailed clinical history and laboratory tests. Read More

J Med Chem 2019 Apr 17. Epub 2019 Apr 17.

Complement Factor D (FD), a highly specific S1 serine protease, plays a central role in the amplification of the alternative complement pathway (AP) of the innate immune system. Dysregulation of AP activity predisposes individuals to diverse disorders such as age-related macular degeneration (AMD), atypical hemolytic uremic syndrome (aHUS), membranoproliferative glomerulonephritis type II (MPGNII) and paroxysmal nocturnal hemoglobinuria (PNH). Previously, we have reported the screening efforts and identification of reversible benzylamine-based FD inhibitors (1 and 2) binding to the open active conformation of FD. Read More

Case Rep Nephrol 2019 12;2019:5390235. Epub 2019 Mar 12.

Department of Renal Medicine, Norfolk and Norwich University Hospital, Norwich, UK.

We describe a patient who had chronic lymphocytic leukaemia (CLL) Binet stage A at presentation with further evidence of disease at multiple sites but who initially required no treatment. However, several years later, her peripheral blood lymphocyte count started to increase, and soon after that she suffered an acute myocardial infarct (in the absence of coronary atheroma) together with proteinuric renal failure due to membranoproliferative glomerulonephritis. Her renal function improved markedly following anti-CLL chemotherapy. Read More

Clin Nephrol 2019 Apr 9. Epub 2019 Apr 9.

The kidney is among the various anatomical sites involved in mucosa-associated lymphoid tissue (MALT) lymphoma. A variety of renal pathological types, including membranous glomerulopathy, membranoproliferative glomerulonephritis, crescentic IgA nephropathy, minimal change disease, and cryoglobulinemic glomerulopathy, have been reported in MALT lymphoma patients. However, cast nephropathy is extremely rare in MALT lymphoma. Read More

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

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

Am J Kidney Dis 2019 Mar 28. Epub 2019 Mar 28.

Istituto di Ricerche Farmacologiche Mario Negri IRCCS; L. Sacco Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy. Electronic address:

Rationale & Objective: Primary membranoproliferative glomerulonephritis (MPGN) is a rare glomerulopathy characterized by complement dysregulation. MPGN progresses rapidly to kidney failure when it is associated with nephrotic syndrome. We assessed the effects of C5 convertase blockade in patients with MPGN and terminal complement activation. Read More

CEN Case Rep 2019 Mar 29. Epub 2019 Mar 29.

Division of Nephrology, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume City, Fukuoka, 830-0011, Japan.

A 65-year-old male patient with nephrotic syndrome was admitted to our hospital due to worsening systemic edema and purpura on the limbs. He had an impaired renal function, low serum complement level, and elevated rheumatoid factor level. He was positive for cryoglobulin (monoclonal IgM-κ and polyclonal mixed-type IgG), and the results of his kidney biopsy showed a tissue profile of membranoproliferative glomerulonephritis (MPGN). Read More

Pan Afr Med J 2018 20;31:44. Epub 2018 Sep 20.

Centre Hospitalier Universitaire Mohammed VI, Oujda, Maroc.

Renal needle biopsy (RNB) is the gold standard in the diagnosis of kidney diseases. It is an invasive technique causing several complications, in particular hemorrhagic events. This study aims to evaluate our RNB practice, to update the current understanding of the technique for percutaneous renal biopsy, to assess complications and to determine the prevalence of kidney diseases diagnosed in our region. Read More

Ren Fail 2019 Nov;41(1):126-130

a Department of Nephrology , the First Hospital of Jilin University , Changchun , China.

Background: Kimura's disease is a rare disease and its etiology is still unclear. Here we reported a case with lymphadenopathy complicated with secondary membranoproliferative glomerulonephritis.

Case Presentation: A 46-year-old Chinese man presented with bilateral tumor-like nodules over his neck during the past 6 months and developed edema for 15 days. Read More

Nephrol Dial Transplant 2019 Mar 16. Epub 2019 Mar 16.

Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Crescentic glomerulonephritis (cGN) comprises three main types according to the pathogenesis and immunofluorescence patterns: anti-glomerular basement membrane antibody cGN, vasculitis-associated cGN and post-infectious immune complex cGN. In this brief review of the immune-pathogenesis of cGN, the focus is mainly on the role of CD8+ T cells in the progression of cGN. Under control conditions, Bowman's capsule (BC) provides a protected immunological niche by preventing access of cytotoxic CD8+ T cells to Bowman's space and thereby podocytes. Read More

Internist (Berl) 2019 Mar 11. Epub 2019 Mar 11.

Klinik für Nieren- und Hochdruckerkrankungen, Medizinische Hochschule Hannover, Hannover, Deutschland.

Based on an increasingly better pathophysiological understanding over the last 10 years, in 2010 a new classification of glomerulonephritis with dominant or codominant C3 deposits was introduced and the predominant subgoup was termed C3 glomerulopathy (C3G). In the current classification, immune complex mediated membranoproliferative glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G) form a disease spectrum which is very heterogeneous in terms of pathophysiology and the clinical course. Recent evidence suggests that IC-MPGN and C3G share more pathophysiological aspects with respect to secondary causes, autoantibodies and genetic aspects than had been suggested with the creation of the new classification. Read More

Clin Exp Nephrol 2019 Mar 11. Epub 2019 Mar 11.

Department of Medicine, Kidney Center, Tokyo Women's Medical University, 8-1 Kawada-Cho, Shinjuku-ku, Tokyo, 162-8666, Japan.

Background: Recently, a new classification has been established for membranoproliferative glomerulonephritis (MPGN). However, the effect of the new classification on MPGN treatment is not fully understood.

Methods: We conducted a retrospective study of 87 patients with biopsies diagnosed as MPGN. Read More

CEN Case Rep 2019 Feb 25. Epub 2019 Feb 25.

Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Proliferative glomerulonephritis with monoclonal immunoglobulin G (IgG) deposits (PGNMID) is a rare disease that recently became recognized. Its pathological findings are characterized by the deposition of a single heavy chain subclass and a single light chain isotype. PGNMID has been proven to recur in renal allografts. Read More

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

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

Surg Case Rep 2019 Feb 19;5(1):34. Epub 2019 Feb 19.

Department of Thoracic Surgery, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.

Background: Hydrothorax due to pleuroperitoneal communication (PPC) is a rare complication of continuous ambulatory peritoneal dialysis (CAPD). Approximately 50% of the patients need to convert to hemodialysis.

Case Presentation: A 65-year-old man with chronic renal failure due to membranoproliferative glomerulonephritis underwent CAPD. Read More

BMC Nephrol 2019 Feb 14;20(1):53. Epub 2019 Feb 14.

Department of Pathology, Division of Renal and Transplant Pathology, Ohio State University Wexner Medical Center, M018 Starling-Loving Hall, 320 W 10th Ave, Columbus, OH, 43210, USA.

Background: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMIGD) is a disease entity classified under the group of "Monoclonal gammopathy-related kidney diseases", and can recur after transplant. Clinical remission of proteinuria in patients with PGNMIGD has been previously shown following anti-B cell and/or anti-plasma cell therapies. Our case is the first to show complete histologic resolution of the glomerular monoclonal IgG kappa deposits in a case of recurrent PGNMIGD in renal allograft after rituximab and steroid treatment. Read More

Rev Fac Cien Med Univ Nac Cordoba 2018 11 13;75(4):292-298. Epub 2018 Nov 13.

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The cryoglobulinemic syndrome is produced by precipitating immunoglobulins at low temperatures. Its production is associated with several causes, such as lymphoproliferative disorders, chronic infections and autoimmune disorders. However, the etiology is unknow. Read More

Joint Bone Spine 2019 Feb 4. Epub 2019 Feb 4.

Sorbonne universités, UPMC Université Paris 06, Inserm, UMR S 959, immunology immunopathology immunotherapy (I3), 75005, Paris, France; Biotherapy (CIC-BTi) and inflammation-immunopathology-biotherapy department (DHU i2B), hôpital Pitié-Salpêtrière, AP-HP, 75651, Paris, France; AP-HP, groupe hospitalier Pitié-Salpêtrière, department of internal medicine and clinical immunology, centre national de référence maladies autoinflammatoires et amylose inflammatoire, centre national de références maladies autoimmunes systémiques rares, 75013, Paris, France. Electronic address:

Cryoglobulinemia is defined as the persistent presence in serum of abnormal immunoglobulins (Igs) that precipitate at low temperatures and dissolve again upon warming. Cryoglobulins may be composed only of a monoclonal Ig (simple type I cryoglobulinemia), of a monoclonal Ig bound to the constant domain of polyclonal Ig heavy chains (mixed type II cryoglobulinemia), or only of polyclonal Igs (mixed type III cryoglobulinemia). The manifestations of type I cryoglobulinemia are often related to intravascular obstruction, whereas those seen in the mixed cryoglobulinemias often originate in true immune complex-mediated vasculitis. Read More

J Neurol Sci 2019 Mar 25;398:121-127. Epub 2019 Jan 25.

Department of Neurology, Cleveland Clinic Foundation, Cleveland, OH 44195, United States. Electronic address:

Introduction: Limited information is available describing the spectrum of neurological complications of cryoglobulinemia.

Methods: Single center retrospective review of patients with neurologic symptoms and elevated serum cryoglobulins, with their potential association being classified as definite, possible, or unlikely using defined criteria.

Results: Among 492 patients, 131 (87 classified as definite and 44 as possible) had neurologic symptoms associated with cryoglobulinemia. Read More

BMC Nephrol 2019 Jan 31;20(1):29. Epub 2019 Jan 31.

Department of Pathology and Laboratory Medicine, Cumming School of Medicine, University of Calgary, Foothills Medical Center, 1403 29 St NW, Calgary, AB, T2N 2T9, Canada.

Background: Anti-glomerular basement membrane (anti-GBM) disease is characterized by circulating IgG glomerular basement membrane antibodies and is clinically expressed as a rapidly progressive crescentic glomerulonephritis (GN), with 30-60% of patients also developing pulmonary hemorrhage. Classically, the renal biopsy shows glomerular crescent formation, bright linear staining of glomerular basement membranes (GBM) for IgG on direct immunofluorescence (IF), and the serologic presence of circulating anti-GBM antibodies. Recently, patients with linear IgG IF staining, undetectable circulating anti-GBM antibodies and glomerular changes atypical for anti-GBM disease have been described as "atypical anti-GBM disease", with a distinctly more benign clinical course than typical anti-GBM disease. Read More

Ter Arkh 2018 Dec;90(12):39-47

Research Institute of Nephrology, I.P. Pavlov First Saint Petersburg State Medical University of the Ministry of Health of the Russian Federation, Saint Petersburg, Russia.

Aim: Analysis of etiology, clinical and morphological manifestations, approaches to therapy and prognosis of membranoproliferative glomerulonephritis (MPGN).

Materials And Methods: Cases of MPGN were retrospectively identified in the period 2000-2017 with subsequent analysis of etiology, clinical data and morphology (including deposits of immunoglobulins (Ig) and C3 complement fractions). The achievement of complete and partial remissions (PR, CR), overall survival, progression (by composite endpoint: decrease in the estimated GFR (eGFR) ≥50% from the baseline or eGFR <15 ml/min/1. Read More

J Immunol Res 2018 30;2018:8051972. Epub 2018 Dec 30.

Rheumatology Group, School of Medicine, University of Western Australia, Perth, Australia.

While it is a common practice to monitor complement levels in patients with systemic lupus erythematosus to aid in flare prediction and detection, it is unclear if this strategy is helpful in preventing subsequent organ damage. We studied longitudinal complement levels in 102 SLE patients during a median follow-up of 13.8 years (IQR 7. Read More

Front Immunol 2018 9;9:3031. Epub 2019 Jan 9.

Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osaka-Sayama, Japan.

Myelodysplastic syndromes (MDS) are a heterogeneous group clonal disorders of hematopoietic stem cells (HSC) characterized by ineffective hematopoiesis that lead to variable grades of impaired blood cell production. Chromosomal aberrations are often detected in MDS patients and thus cytogenetic analysis is useful for the diagnosis of these disorders. Common recurring chromosomal defects, such as the -5/5q- and -7/7q- are relatively well characterized cytogenetic abnormalities in MDS, however, the biological significance of uncommon cytogenetic alterations is unknown. Read More

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

Retin Cases Brief Rep 2019 Jan 17. Epub 2019 Jan 17.

Department of Ophthalmology, Graduate School of Medicine, Tokyo University, Tokyo, Japan.

Purpose: To report three middle-aged cases with choroidal neovascularization (CNV) associated with early-onset drusen documented with optical coherence tomography angiography (OCTA).

Methods: Three patients with bilateral early-onset drusen were referred to our hospital. Fundus examination, fluorescein angiography, indocyanine green angiography, OCTA, and other multimodal imaging were performed. Read More

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

Indian J Nephrol 2018 Nov-Dec;28(6):462-464

Department of Nephrology, Institute of Liver and Biliary Sciences, New Delhi, India.

Hepatitis C virus (HCV) infection has been shown to affect kidneys with various histopathological pattern on the kidney biopsy. These commonly include a membranoproliferative glomerulonephritis (MPGN) pattern with mixed cryoglobulinemia (CG), thrombotic microangiopathy, membranous nephropathy, and vasculitis affecting medium and small vessels of the kidneys causing polyarteritis nodosa. It has been rarely associated with MPGN without detectable CG. Read More

Pathol Res Pract 2019 Mar 26;215(3):410-413. Epub 2018 Dec 26.

Department of Pathology and Laboratory Medicine, Kanazawa Medical University, Ishikawa, Japan.

Thrombocytopenia, anasarca, fever, renal failure or reticulin fibrosis, and organomegaly (TAFRO) syndrome, a poor prognostic clinical condition showing similar histopathological findings to idiopathic multicentric Castleman disease (iMCD), has been reported in Japan. In our previous report, a clinicopathological analysis was performed on 70 nodal cases of iMCD with/without TAFRO. iMCD is classified into three types based on histopathology: (i) plasmacytic (PC), (ii) mixed, and (iii) hypervascular (hyperV). Read More

Medicine (Baltimore) 2019 Jan;98(2):e14014

Department of Medicine Kidney Center.

Rationale: Adult-onset hepatitis B virus-associated membranoproliferative glomerulonephritis (HBV-MPGN) is generally refractory, and an effective treatment for this condition has not been established. The indications for steroids in HBV-MPGN are an important clinical concern.

Patient Concerns: A 28-year-old woman with a chronic hepatitis B virus infection developed nephrotic syndrome in her second month of pregnancy, with urinary protein levels of 3 to 10 g/d that continued into her postpartum period. Read More

Iran J Kidney Dis 2018 Nov;12(6):382-384

Urology and Nephrology Center, Mansoura University, Egypt.

Direct antivirals showed dramatic response in hepatitis C virus (HCV) eradication, but their effect on extrahepatic manifestations is still unclear. A 49-year-old woman was referred to us suffering from lower limb edema and frothy urine. Renal biopsy was done and she was diagnosed with HCV-related membranoproliferative glomerulonephritis with cryoglobulinemia. Read More

Autoimmunity 2018 Dec 28;51(8):386-398. Epub 2018 Dec 28.

a Laboratory of Anatomy, Faculty of Veterinary Medicine, Department of Basic Veterinary Sciences , Hokkaido University , Sapporo , Japan.

Toll-like receptors (Tlrs) are sensors of danger signals which promote the activation of immune cells and intrinsic renal cells. Podocytes, the intrinsic cells of glomerulus, are continuously exposed to various plasma solutes and danger signals due to their unique location in the glomerulus. Herein, we show that Tlr9 is overexpressed in podocytes and the mechanisms which cause its injury and development of membranoproliferative glomerulonephritis (MPGN) in model BXSB/MpJ-Yaa (Yaa) mice. Read More

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

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

BMJ Case Rep 2018 Dec 7;11(1). Epub 2018 Dec 7.

Nephrology, Allegheny Health Network, Pittsburgh, Pennsylvania, USA.

Proliferative glomerulonephritis with monoclonal immune deposits (PGNMID) is a newly described entity characterised by monoclonal IgG deposits consisting of single light chain isotype and single heavy chain subtype (IgG1-4) in the kidneys. We are presenting two cases of patients who presented with acute kidney injury and worsening proteinuria. Kidney biopsy showed membranoproliferative pattern. Read More

Mod Pathol 2018 Dec 14. Epub 2018 Dec 14.

Institute of Pathology and Nephropathology Section, University Hospital Hamburg Eppendorf, Hamburg, Germany.

Bevacizumab is a humanized monoclonal IgG1 antibody, which neutralizes vascular endothelial growth factor and is used for treating multiple cancer types. As a known and frequent adverse event, this therapy can lead to renal damage including proteinuria and nephrotic syndrome. In a retrospective approach, we analyzed 17 renal biopsies from patients receiving bevacizumab treatment. Read More

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

Clin Nephrol 2019 Feb;91(2):79-86

Introduction: C3 glomerulonephritis (C3GN) is a form of proliferative glomerulonephritis characterized by dominant glomerular C3 deposition. There is currently no consensus guideline on therapy for this disease. Experience with corticosteroids alone is scant in C3GN. Read More

Clin Case Rep 2018 Nov 23;6(11):2287-2288. Epub 2018 Sep 23.

Division of Pathology, Immunology and Laboratory Medicine University of Florida Gainesville Florida.

Idiopathic" membranoproliferative glomerulonephritis (MPGN) is rare compared to secondary forms and usually a progressive disease. Studies evaluating the role of steroids were primarily done in children, and the data are sparse in adults. Mycophenolate mofetil is effective and can be used as a steroid-sparing agent in the treatment of idiopathicMPGN. Read More

Kidney Int Rep 2018 Nov 7;3(6):1260-1266. Epub 2018 Jul 7.

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.

Immunofluorescence on frozen tissue is the gold standard immunohistochemical technique for evaluation of immune deposits in the kidney. When frozen tissue is not available or lacks glomeruli, immunofluorescence can be performed on paraffin tissue after antigen retrieval (paraffin immunofluorescence). Excellent results can be obtained by paraffin immunofluorescence in most immune complex-mediated glomerulonephritides and dysproteinemia-associated kidney lesions, and thus this technique has become a valuable salvage technique in renal pathology. Read More

Clin Nephrol 2019 Jan;91(1):52-58

Membranoproliferative glomerulonephritis is known to recur after kidney transplantation and may lead to allograft loss. Although an optimal treatment has not been determined, B-cell targeted therapies are now increasingly used as first-line agents, based on growing data showing antibodies as key players in the pathogenesis of membranoproliferative glomerulonephritis. Here, we report a case of recurrent immune complex-mediated membranoproliferative glomerulonephritis 3 years after a living-donor kidney transplant. Read More

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

Int J Nephrol 2018 11;2018:2751372. Epub 2018 Oct 11.

Department of Internal Medicine, School of Medicine, University of Jordan, Amman, Jordan.

Aim: To determine the prevalence and frequency of different pathological patterns of glomerulonephritis (GN) in adolescent (age ≥ 11 years) and adult Jordanian patients.

Materials And Methods: A retrospective analysis of all clinical and pathological reports of Jordanian patients who had native renal biopsies at the University of Jordan hospital between January 2007 and March 2018 to assess the prevalence and pathological pattern of GN. The data were analyzed statistically using descriptive statistics, the chi-squared test, and Fisher's exact tests. Read More

World J Transplant 2018 Oct;8(6):203-219

Faculty of Health and Science, University of Liverpool, Institute of Learning and Teaching, School of Medicine, Liverpool L69 3GB, United Kingdom.

For decades, kidney diseases related to inappropriate complement activity, such as atypical hemolytic uremic syndrome and C3 glomerulopathy (a subtype of membranoproliferative glomerulonephritis), have mostly been complicated by worsened prognoses and rapid progression to end-stage renal failure. Alternative complement pathway dysregulation, whether congenital or acquired, is well-recognized as the main driver of the disease process in these patients. The list of triggers include: surgery, infection, immunologic factors, pregnancy and medications. Read More

Case Rep Nephrol 2018 1;2018:4748357. Epub 2018 Oct 1.

Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo, Japan.

Glomerular immunoglobulin A (IgA) deposition is a common finding in hepatic glomerulosclerosis; thus, this disease is also called hepatic IgA nephropathy. However, only a small number of patients with hepatic IgA nephropathy have active glomerular lesions, so functional decline is slow in most cases. In this report, we describe a 60-year-old man who developed nephrotic syndrome and progressive renal impairment during follow-up for alcoholic liver cirrhosis. Read More

Nephron 2018 25;140(4):275-281. Epub 2018 Oct 25.

Department of Internal Medicine, Nephrology Unit, School of Medicine, Cairo University, Cairo, Egypt.

Introduction: The side effects profile of the new direct--acting antivirals for the treatment of hepatitis C virus (HCV) is not fully elucidated.

Objective: In this cross-sectional study, we aim to describe the incidence and characteristics of a novel observation of de novo renal cryoglobulinemic glomerulonephritis after successful treatment with DAA.

Methodology: A total of 12,985 Hepatitis C Patients (genotype IV) received the new DAA. Read More

J Nephrol 2018 Dec 17;31(6):907-918. Epub 2018 Oct 17.

Department of Pathology, Stanford University Medical Center, 300 Pasteur Drive, L235, Stanford, CA, 94305, USA.

IgM secreting myelomas or lymphomas, including Waldenström macroglobulinemia, are associated with a varied spectrum of renal pathology, including intracapillary hyaline deposits, cryoglobulin, membranoproliferative glomerulonephritis, amyloid, monoclonal immunoglobulin deposition disease, cast nephropathy, and lymphoma infiltration. We report our single institution experience, and describe five cases with distinctive glomerular pathology: intracapillary IgM pseudothrombi and thrombotic microangiopathic change, with glomerular intracellular crystals in two biopsies. Two patients were hypocomplementemic at presentation. Read More

J Clin Pathol 2019 Jan 12;72(1):31-37. Epub 2018 Oct 12.

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

Aim: To explore the clinicopathological characteristics of patients with anti-GBM antibody-negative anti-GBM disease.

Methods: The clinical and renal pathological findings were retrospectively studied in 19 patients. All patients met the following inclusion criteria: linear GBM IgG deposition on immunofluorescence(IF); and lack of serum anti-GBM antibodies by ELISA and indirect immunofluorescence assay. Read More

BMC Nephrol 2018 Oct 4;19(1):251. Epub 2018 Oct 4.

Department of Nephrology and Endocrinology, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-8513, Japan.

Background: The pathophysiologic role of exogenous granulocyte-colony stimulating factor (G-CSF) administration is reportedly linked to the progression of glomerulonephritis. However, the relationship between endogenous G-CSF overproduction and the progression of glomerulopathy has not been well investigated.

Case Presentation: A 76-year-old woman presented with neutrophilia at a medical check-up and thorough examination revealed a high level of serum G-CSF. Read More